Beruflich Dokumente
Kultur Dokumente
IMAGING
T H I R D E D I T I O N
Donald Resnick, MD
Chief, Musculoskeletal Imaging
Professor of Radiology
University of California, San Diego
San Diego, California
Mark J. Kransdorf, MD
Chief, Musculoskeletal Imaging
Mayo Clinic
Jacksonville, Florida
Professor of Radiology
Mayo Clinic College of Medicine
Rochester, Minnesota
The Curtis Center
170 S Independence Mall W 300E
Philadelphia, Pennsylvania 19106
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NOTICE
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Resnick, Donald.
Bone and joint imaging/Donald Resnick, Mark J. Kransdorf. – 3rd ed.
p.; cm.
Includes bibliographical references and index.
ISBN 0-7216-0270-3
1. Bones—Imaging. 2. Joints—Imaging. 3. Bones—Diseases—Diagnosis.
4. Joints—Diseases—Diagnosis. I. Kransdorf, Mark J. II. Title.
[DNLM: 1. Bone Diseases—diagnosis. 2. Diagnostic Imaging—methods.
3. Joint Diseases—diagnosis. WE 141 R434b 2005]
RC930.5.R47 2005
616.7′10754–dc22 2003070436
Printed in USA
v
vi Contributors
Nine years after the publication of the second edition of niques, and postoperative imaging serve as introductory
Bone and Joint Imaging and a few years after the material; this material is then followed by sections deal-
publication of the fourth edition of the larger Diagnosis ing with imaging of most of the important diseases that
of Bone and Joint Disorders, the third edition of Bone affect the musculoskeletal system. Key images have been
and Joint Imaging is now ready for dissemination. In selected to illustrate the most important of the imaging
common with the first and second editions of this text, findings, and a short but appropriate bibliography is
the purpose of this book is to present in a logical manner included in each chapter. As before, we have included
and easy-to-read format the information that we, the shortened versions of many chapters written by experts in
authors, believe is essential for those learning muscu- the field that were part of the larger multivolume text-
loskeletal imaging for the first time or for those review- book. When compared with the second edition, however,
ing the subject one more time. The subject of muscu- there are significant changes in this third edition. Many
loskeletal imaging is ever changing and constantly subjects appear for the first time, countless new and
growing in scope. Much of this growth relates not to the improved illustrations are included, and references are
discovery of new processes or disorders but rather to the updated. And to do this properly and on time, two editors
development and refinement of advanced imaging methods rather than one have accomplished this task.
and techniques. Diagnostic methods now applied routinely Both of us are confident that we have succeeded in
to the analysis of musculoskeletal disorders include far condensing the essential material related to musculoskeletal
more than conventional radiography: CT scanning, MR imaging in a manageable textbook. But it is the readers
imaging, ultrasonography, radionuclide studies, and who are the ultimate judge. We are hopeful that whether
arthrography are among the additional methods that it is used for consultation on an intermittent basis or read
must be mastered by those interpreting images related to in its entirety, the readers will enjoy the experience and
bone, joint, and soft tissue disorders. To summarize ade- be wiser for it.
quately the many imaging techniques and findings in a
text any shorter than this, in our view, would not be DONALD RESNICK
appropriate or even possible. MARK J. KRANSDORF
The organization of the text follows that of the previous
edition. Basic anatomy and physiology, diagnostic tech-
ix
ACKNOWLEDGMENTS
We are greatly indebted to a number of individuals with- We would also like to acknowledge those individuals
out whom this project would not be possible. This includes whose dedication, commitment, and energy often go
our many contributing authors, all of whom are highly unnoticed but who keep the system running smoothly
regarded educators and experts in their respective fields. and on time: our administrative assistants Michael
Their efforts are very much appreciated. Holbrook, Debra Trudell, and Pamela J. Chirico.
A very special thanks must go to Allan Ross, Executive
Editor, and his associates at Elsevier: Janice M. Gaillard,
Senior Developmental Editor; Linda Lewis Grigg, Project
Manager, Book Production; and Walter Verbitski,
Illustration Specialist.
x
SECTION
I
Basic Science
CHAPTER 1
sheetlike arrangement of osseous tissue, termed parallel-
Histogenesis, Anatomy, and fibered, or lamellar, bone. As a connective tissue, bone is
Physiology of Bone highly specialized and differs from other connective tissue
by its rigidity and hardness, which relate primarily to the
inorganic salts that are deposited in its matrix. These
Donald Resnick, Stavros C. Manolagas, properties are fundamental to a tissue that must maintain
and Michael D. Fallon the shape of the human body, protect its vital soft tissues,
and allow locomotion by transmitting from one region of
the body to another the forces generated by the contrac-
tions of various muscles. Bone also serves as a reservoir
SUMMARY OF KEY FEATURES for ions, principally calcium, that are essential to normal
Bone is a unique tissue that is constantly undergoing fluid regulation; these ions are made available as a response
change. It develops through the processes of to stimuli produced by a number of hormones, particu-
endochondral and intramembranous ossification and is larly parathyroid hormone, vitamin D, and calcitonin.
subsequently modified and refined by the processes of
modeling and remodeling to create a structurally and
HISTOGENESIS
metabolically competent, highly organized architectural
marvel. Its cells, including osteoblasts, osteocytes, and Developing Bone
osteoclasts, reside in organic matrix, primarily collagen, Bone develops by the process of intramembranous bone
and inorganic material is deposited in a form that formation (transformation of condensed mesenchymal
resembles hydroxyapatite. The process of mineralization tissue), endochondral bone formation (indirect conver-
is complex and incompletely understood. sion of an intermediate cartilage model), or both. At some
Bone is essential in maintaining calcium homeostasis, locations, such as the bones of the cranial vault (frontal
or stabilization of the plasma level of calcium. Its cells and parietal bones, as well as parts of the occipital and
are highly responsive to stimuli provided by a number temporal bones), the mandible and maxilla, and the mid-
of humoral agents, the most important of which are portion of the clavicle, intramembranous (mesenchymal)
parathyroid hormone, thyrocalcitonin, and 1,25- ossification is detected; in other locations, such as the
dihydroxyvitamin D. Synthesis and resorption of bone, bones of the extremities, the vertebral column, the pelvis,
which normally continue in a delicate balance and the base of the skull, both endochondral and intra-
throughout life, are mediated by the action of such membranous ossification can be identified. The actual
humoral agents through processes that include processes of bone tissue formation are essentially the
stimulation of osteoblasts to form bone and stimulation same in both intramembranous and endochondral ossifi-
of osteoclasts to remove bone. cation and include the following sequence: (1) osteoblasts
differentiate from mesenchymal cells; (2) osteoblasts
deposit matrix, which is subsequently mineralized; (3)
bone is initially deposited as a network of immature
INTRODUCTION (woven) trabeculae, the primary spongiosa; and (4) the
Bone is a remarkable tissue. Although its appearance primary spongiosa is replaced by secondary bone,
on radiographs might be misinterpreted as indicating removed to form bone marrow, or converted to primary
inactivity, bone is constantly undergoing change. This cortical bone by the filling of spaces between trabeculae.
occurs not only in the immature skeleton, in which
growth and development are readily apparent, but also in
Intramembranous Ossification
the mature skeleton, through the constant and balanced
processes of bone formation and resorption. It is when Intramembranous ossification is initiated by the prolif-
these processes are modified such that one dominates, eration of mesenchymal cells about a network of capil-
that a pathologic state may be created. In some instances, laries. At this site, transformation of the mesenchymal
the resulting imbalance between bone formation and cells is accompanied by the appearance of a meshwork of
resorption is easily detectable on the radiograph. In others, collagen fibers and amorphous ground substance. The
a more subtle imbalance exists that may be identified primitive cells proliferate, enlarge, and become arranged
only at the histologic level. in groups, transforming into osteoblasts, which are inti-
The initial architecture of bone is characterized by mately involved in the formation of an eosinophilic matrix
an irregular network of collagen, termed woven-fibered within the collagenous tissue. As the osteoid matrix under-
bone, which is a temporary material that is either removed goes calcification with the deposition of calcium phosphate,
to form a marrow cavity or subsequently replaced by a some of the osteoblasts on the surface of the osteoid and
1
2 Section I Basic Science
woven-fibered bone become entrapped within the sub- sites of bone formation are called centers of ossification.
stance of the matrix in a space called a lacuna. These cells, In tubular bones, the primary center of ossification is
now osteocytes, maintain the integrity of the surround- located in the central portion of the cartilaginous model,
ing matrix and are not directly involved in bone forma- whereas later-appearing centers of ossification (second-
tion. Through the continued transformation of mesenchy- ary centers) are located at the ends of the models within
mal cells into osteoblasts, elaboration of an osteoid matrix, epiphyses and apophyses. Vascular mesenchymal tissue
and entrapment of osteoblasts within the matrix, the or perichondrium, whose deeper layers contain cells with
primitive mesenchyme is converted into osseous tissue. osteogenic potential, surrounds the cartilaginous model.
The ultimate characteristics of the tissue depend on its The initial changes in the primary center of ossifica-
location within the bone: in cancellous areas of the bone, tion are hypertrophy of cartilage cells, glycogen accumu-
the meshwork of osseous tissue contains intervening vas- lation, and reduction of intervening matrix. Subsequently,
cular connective tissue, representing the embryonic pre- these cells degenerate, die, and become calcified. Simulta-
cursor of the bone marrow; in compact areas of the bone, neously, the deeper or subperichondrial cells undergo
the osseous tissue becomes more condensed and forms transformation to osteoblasts, and through a process iden-
cylindric masses containing a central vascular channel, tical to intramembranous ossification, these osteoblasts
the haversian system. On the external and internal sur- produce a subperiosteal collar or cuff of bone that encloses
faces of the compact bone, fibrovascular layers develop the central portions of the cartilaginous tissue. Periosteal
(periosteum and endosteum) and contain cells that remain tissue is converted into vascular channels, and the aggres-
osteogenic and give bone its ever-changing quality. sive vascular tissue disrupts the lacunae of the cartilage
cells and creates spaces that fill with embryonic bone
marrow. Osteoblasts appear and transform the sites of
Endochondral Ossification
degenerating and dying cartilage cells into foci of ossifi-
In endochondral (intracartilaginous) ossification, carti- cation by laying down osteoid tissue in the cartilage
laginous tissue derived from mesenchyme serves as a matrix. Osteoblasts become trapped within the develop-
template and is replaced with bone (Fig. 1–1). The initial ing bone as osteocytes.
From the center of the tubular bone, ossification pro-
ceeds toward the ends of the bone. Similarly, the periosteal
collar, which is actively participating in intramembranous
ossification, spreads toward the ends of the bone, slightly
ahead of the band of endochondral ossification. Through
a process of subperiosteal deposition of bone, a cortex
becomes evident, grows thicker, and is converted into
a system with longitudinally arranged compact bone
surrounding vascular channels (haversian system). The
front of endochondral ossification that is advancing toward
the end of the bone becomes better delineated, and it
is this plate that ultimately becomes located between
the epiphysis and diaphysis of a tubular bone and forms
the growth plate (cartilaginous plate or physis). The plate
contains clearly demarcated zones: a resting zone of
flattened and immature cells on the epiphyseal aspect of
the plate, as well as zones of cell growth and hypertrophy
and of transformation, with provisional calcification and
ossification on the metaphyseal or diaphyseal aspect of
the plate.
The size and shape of the most recently formed por-
tion of the metaphysis of a tubular bone depend on the
effects of an encircling fibrochondro-osseous structure,
designated the periphysis, that consists of the zone of
Ranvier, the ring of La Croix, and the bone bark that they
produce (Fig. 1–2). In this setting of progressive ossifi-
cation of the diaphysis with longitudinal spread toward
the ends of the bone, characteristic changes appear
within the epiphysis. Epiphyseal invasion by vascular
channels is followed by the initiation of endochondral
bone formation, which creates secondary centers of ossifi-
Figure 1–1. Endochondral and intramembranous ossification
in a tubular bone: radius of a 41⁄2-month-old fetus. The large
cation. The process is again characterized by cartilage
and confluent cartilage cell lacunae are being penetrated by cell hypertrophy and death, followed by calcification.
vascular channels (solid arrow), thereby exposing intervening The epiphyseal ossification center at first develops
cores of calcified cartilage matrix. The osteoblasts are deposit- rapidly, although later the process slows. The epiphyseal
ing osseous tissue on these cartilage matrix cores (arrowhead). cartilage is thus converted to bone, although a layer on
Observe the subperiosteal bone formation (open arrows). its articular aspect persists and is destined to become the
Chapter 1 Histogenesis, Anatomy, and Physiology of Bone 3
Figure 1–2. Endochondral and intramembranous ossification in a tubular bone: periphysis and meta-
physeal collar. A, In this diagram, observe the periphysis (dashed boxes) and metaphyseal collar and spur. The
bone bark is indicated. B, In the distal portion of the radius of a normal infant, note the straight metaphyseal
margins (white arrowheads) forming the edges of the metaphyseal collar, in addition to the well-defined bone
bark (white arrow) at the medial margin of the ulnar physis.
(From Oestreich AE, Ahmad BS: The periphysis and its effect on the metaphysis. I. Definition and normal
radiographic pattern. Skeletal Radiol 21:283, 1992.)
fusion of the epiphyseal and diaphyseal ossification centers, Modeling and Remodeling of Bone
followed by cessation of endochondral bone formation The term intermediary organization has been used to des-
deep to the articular cartilage of the epiphysis and forma- cribe the control and regulation of coordinated cellular
tion of a subchondral bone plate. Although the growth events that occur in the living human skeleton. Inter-
plate has now ceased to function, a band of horizontally mediary organization is dependent on a number of bone
oriented trabeculae may persist and mark the previous cells, such as osteoblasts and osteoclasts, whose activity is
location of the plate as a transverse radiopaque fusion line. linked. Thus, the processes of bone formation and bone
Abnormalities of endochondral ossification in the physis resorption are intertwined.
are well recognized in a number of disorders and are
fundamental to the diagnosis of rickets (Fig. 1–5A).
Transient aberrations of such ossification lead to the Modeling
development of growth recovery lines (see Fig. 1–5B). It is the process of modeling that significantly alters the
shape and form of bone. Modeling, or sculpting, of the
skeleton is responsive to the mechanical forces placed
Developing Joint
on it. This process occurs continuously throughout the
An articulation eventually appears in the mesenchyme growth period at varying rates and involves all bone sur-
that exists between the developing ends of the bones. In faces. Classic examples of the modeling process are (1)
a fibrous joint, the interzonal mesenchyme is modified drifting of the midshaft of a tubular bone, (2) flaring of
to form the fibrous tissue that will connect the adjacent the ends of a tubular bone, and (3) enlargement of the
bones; in a synchondrosis, it is converted to hyaline carti- cranial vault and modification of cranial curvature. This
lage; and in a symphysis, the interzonal mesenchyme is form of modeling is a prerequisite to the normal develop-
changed to fibrocartilage. In a synovial joint, the central ment of tubular bones, ribs, and other osseous structures.
portion of the mesenchyme becomes loosely meshed and It is accomplished by resorption, which dominates in
is continuous in its periphery with adjacent mesenchyme one aspect of a bone, and apposition, which dominates in
that is undergoing vascularization (Fig. 1–6). The synovial another. In the long tubular bones of the extremity,
mesenchyme that is created will later form the synovial resorption is more evident on the side of the bone surface
membrane, as well as some additional intra-articular struc- that is nearer the body core, and apposition occurs on the
tures, whereas the central aspect of the mesenchyme under- opposite surface.
goes liquefaction and cavitation and thereby creates the The flaring that is normally evident in the end of a
joint space. Condensation of the peripheral mesenchyme long tubular bone exemplifies bone modeling (Fig. 1–7).
leads to joint capsule formation. As the bone grows in length, the wide metaphyseal
Remodeling
To produce and maintain biomechanically and meta-
bolically competent tissue, transformation of immature,
woven-type bone to more compact lamellar bone is
Figure 1–7. Modeling of bone: growth of a tubular bone.
required. This process of remodeling is normally most Note the changing shape of the epiphyseal ossification center,
prominent in the young but continues at reduced rates the altered organization of the growth plate, and the varying
throughout life. The linkage of resorption and formation zones of bone deposition and resorption (absorption).
of bone is very tight; formation follows resorption at the (From Warwick R, Williams PL [eds]: Gray’s Anatomy, 35th
resorption site, not at some other location, and the Br ed. Philadelphia, WB Saunders, 1973, p 230.)
amount of bone that is formed is almost always nearly
equal to the amount that is removed. The remodeling
process replaces aged or injured bone tissue with new In the endosteal and periosteal surfaces of the cortex,
bone tissue; over time, the repeated strain on skeletal osteoclastic resorption leads to a tube-shaped tunnel
tissue that occurs during ordinary physical activity results designated a resorption canal. Initially, this tunnel is
in microdamage that, if not repaired, would eventually oriented approximately perpendicular to the surface of
lead to structural failure. the bone and corresponds in position to Volkmann’s
6 Section I Basic Science
Figure 1–8. Remodeling of bone: cortical remodeling unit. Top, Diagram shows a longitudinal section
through a cortical remodeling unit, with corresponding transverse sections below (1 to 4). A, Multinucleated
osteoclasts in Howship’s lacunae advancing longitudinally from right to left and radially to enlarge a
resorption cavity; B, perivascular spindle-shaped precursor cells; C, capillary loops; D, mononuclear cells
lining reversal zones; E, osteoblasts depositing new bone centripetally; F, flattened cells lining the haversian
canal of a complete haversian system. Bottom, Transverse sections at different stages of development: 1,
resorption cavities lined with osteoclasts; 2, completed resorption cavities lined by mononuclear cells, the
reversal zone; 3, the forming haversian system, or osteons, lined with osteoblasts that had recently formed
three lamellae; 4, the completed haversian system with flattened bone cells lining the canal. Osteoid
(arrowheads) is present between osteoblast (O) and mineralized bone. G, cement line.
(Redrawn from Parfitt AM: The actions of parathyroid hormone on bone: Relation to bone remodeling
and turnover, calcium homeostasis, and metabolic bone disease. Part I of IV parts: Mechanisms of calcium
transfer between blood and bone and their cellular basis: Morphological and kinetic approaches to bone
turnover. Metabolism 25:809, 1976.)
Chapter 1 Histogenesis, Anatomy, and Physiology of Bone 7
TABLE 1–1
Adult Bone Surfaces (Envelopes)
Surface Area
Surface (×106 sq mm)
Cortical surfaces
Periosteal 0.5
Haversian (osteonal) 3.5
Corticoendosteal 0.5
Trabecular surfaces
Endosteal 7
Total surfaces 11.5
From Jee WSS: The skeletal tissues. In Weiss L, Lansing L (eds): Histology.
Cell and Tissue Biology, 5th ed. New York, Elsevier, 1983, p 221.
Figure 1–10. Cellular constituents of bone: osteoblasts, osteocytes, and osteoclasts. A, Prominent osteoblasts
(arrow) secreting osteoid matrix (O). Note the perinuclear clear zone, which represents the Golgi apparatus.
B, Multinucleated osteoclast (arrow) residing in a resorption bay or Howship’s lacuna (HL). Open arrow,
osteocyte; T, mineralized trabecular bone (trichrome stain, ×340).
8 Section I Basic Science
Osteoblasts. Osteoblasts are derived from cells that are Inorganic Mineral. The inorganic mineral of bone exists
probably components of the stromal system of bone and in a crystalline form that resembles hydroxyapatite—
marrow. Osteoblasts are intimately involved in the 3Ca(PO4)2 • Ca(OH)2; this mineral is distributed regularly
processes of intramembranous and endochondral bone along the length of the collagen fibers and is surrounded
formation. Indeed, any cell that forms bone—whether by ground substance.
during growth and modeling, remodeling, or fracture
healing—is defined as an osteoblast. The activity of the
precursor cells is directly governed by the principle of Bone Marrow
supply and demand; at times when new bone is required, Bone marrow is a soft, pulpy tissue that lies in the spaces
such as during the healing of a fracture, these cells are between the trabeculae of all bones and even in the larger
called to action in the generation of osteoblasts. haversian canals. It is one of the most extensive organs of
the human body. Its functions include the provision of a
Osteocytes. Osteocytes arise from preosteoblasts and continual supply of red cells, white cells, and platelets to
osteoblasts. Initially present at the surface of the bone, meet the body’s demand for oxygenation, immunity, and
some, but not all, of the osteoblasts subsequently become coagulation. A complex vascular supply relies mainly on
entrapped within the osseous tissue as osteocytes. Here, a nutrient artery that, in the long tubular bones, pierces
the osteocyte lies in a lacuna. They are unable to divide, the diaphyseal cortex at an angle and extends toward the
so only one cell is present in each lacuna. The osteocyte ends of the tubular bone by running parallel to its long
is concerned with proper maintenance of the bone matrix. axis. Branches from the nutrient artery enter the endosteal
surface of the cortex as capillaries and eventually form
Osteoclasts. Another cell, the osteoclast, is a multinucle- primary and collecting sinusoids in an extensive, anasto-
ated cell (2 to 100 nuclei) with a short life span that is mosing complex among the fat cells of the marrow.
intimately related to the process of bone resorption. The
origin of the osteoclast has been investigated, and it now
appears to be a product of one of the cell lines of the Marrow Composition
hematopoietic system and is derived from a hematopoi- The basic composition of bone marrow consists of miner-
etic stem cell (monocyte-phagocyte line). alized osseous matrix, connective tissue, and a variety
of cells. As described previously, the trabeculae in the
Bone-Lining Cells. The precise nature of the commonly medullary space represent the osseous component of the
identified flat, elongated cells with spindle-shaped nuclei bone marrow. The marrow itself occupies the spaces
that line the surface of the bone is not clear, although they between and around the plates and struts of trabecular
are generally believed to be derived from osteoblasts that (cancellous or spongy) bone, and it is held in place by a
have become inactive. Lining cells communicate with the network of fine fibrous tissue called a reticulum. The
syncytium of osteocytes, and although their function is also cancellous trabeculae provide architectural support and
unknown, it may include maintenance of mineral home- serve as a mineral depot. The interface between marrow
ostasis, control of the growth of bone crystals, or the abil- and trabecular bone, the endosteal envelope, is very
ity to differentiate into other cells, such as osteoblasts. active metabolically and has a complement of cells, such
as osteoblasts and osteoclasts, that are extremely sensitive
to metabolic stimuli.
Noncellular Constituents of Bone
The cells of the marrow consist of all stages of erythro-
Water is responsible for about 20% of the wet weight of cytic and leukocytic development, as well as fat cells and
bone tissue. The major cellular components—osteoblasts, reticulum cells. Under homeostatic conditions, the
osteocytes, and osteoclasts—account for a very small frac- production rate of hematopoietic cells precisely equals
tion of the total weight of bone. The other constituents the destruction rate. The average life span of a human
of bone include the remaining organic matrix (collagen red cell is approximately 120 days, and that of a platelet
and mucopolysaccharides), which accounts for approxi- is 7 to 10 days; the life span of leukocytes is more vari-
mately 20% to 30% of osseous tissue by dry weight, and able, being relatively short for granulocytes (6 to 12 hours)
inorganic material, which accounts for approximately and long for lymphocytes (months or even years). Fat
70% to 80% of osseous tissue by dry weight. It is these cells are also a major component of bone marrow.
constituents, in physiologic amounts, that create bone Although smaller than fat cells from extramedullary sites,
tissue that is both dynamic and uniquely capable of pro- marrow fat cells are active metabolically and respond to
viding the support the body requires. hematopoietic activity by changes in size. During periods
of decreased hematopoiesis, the fat cells in bone marrow
Organic Matrix. The organic matrix of bone, which surrounds increase in size and number, whereas during increased
the cellular components, is composed primarily of protein, hematopoiesis, the fat cells atrophy.
glycoprotein, and polysaccharide. Collagen (type I) is the Two forms of bone marrow are encountered, although
major constituent (90%) of the organic matrix of bone; at any given anatomic site an admixture of the two forms
the collagen is embedded in a gelatinous mucopolysaccha- often exists. Red marrow is hematopoietically active
ride material (ground substance). Although mucopolysac- marrow and consists of approximately 40% water, 40%
charides represent a minor quantitative part of the struc- fat, and 20% protein; yellow marrow is hematopoietically
ture of osseous tissue, they appear to be very important in inactive and consists of approximately 15% water, 80%
the process of bone matrix maturation and mineralization. fat, and 5% protein.
Chapter 1 Histogenesis, Anatomy, and Physiology of Bone 9
Marrow Conversion
Although the visualized patterns of signal intensity do
The amount of red marrow versus yellow marrow at any not precisely correspond to anatomic sites of red and
given time is dependent on the age of the person, the site yellow marrow, magnetic resonance imaging is an effec-
that is being sampled, and the health of the individual. At tive, albeit indirect, means of determining the cellular
birth, red marrow is present throughout the skeleton, but characteristics of bone marrow. Composed predominantly
with increasing age, because of the normal conversion of fat, yellow marrow displays the T1 and T2 relaxation
process of hematopoietic to fatty marrow, the proportion patterns of adipose tissue; containing considerable amounts
of hematopoietic marrow decreases. Fatty marrow of water and protein, as well as fat, red marrow has T1
represents approximately 15% of the total marrow and T2 relaxation patterns that differ from those of fatty
volume in a child but accounts for 60% of this volume by marrow. Although the major contributor to signal inten-
age 80 years. The conversion of red to yellow marrow sity for both types of marrow is fat, on standard T1-
that occurs during growth and development is predictable weighted spin echo sequences, red marrow demonstrates
and orderly (Fig. 1–11). This replacement commences lower signal intensity than yellow marrow does. Normal
earlier and is more advanced in the more distal bones of age-related conversion of red to yellow marrow in the
the extremities; further, in each bone, the conversion to vertebral bodies and femora is illustrated in Figures 1–12
yellow marrow proceeds from the distal to the proximal and 1–13, underscoring the variability of magnetic reso-
end, although some authors maintain that it commences nance imaging findings that characterize the normal,
in the center of the shaft and extends in both directions, orderly process of conversion from hematopoietic to
but more rapidly in the distal segment. Cartilaginous fatty marrow.
epiphyses and apophyses lack marrow until they ossify. When the body’s demand for hematopoiesis increases,
By the age of 20 to 25 years, marrow conversion is usually yellow marrow is reconverted to red marrow. The extent
complete. At this time, the adult pattern is characterized of reconversion depends on the severity and duration of
by the presence of red marrow only in portions of the the stimulus, and the process may be initiated or modu-
vertebrae, sternum, ribs, clavicles, scapulae, skull, and lated by such factors as temperature, low oxygen tension,
innominate bones and in the metaphyses of the femora hemoglobin blood level, and elevated levels of erythro-
and humeri. Minor variations in this distribution, poietin. The process of reconversion follows that of
however, are encountered. conversion, but in reverse. Initial changes occur in the
Figure 1–12. Marrow conversion: vertebral bodies. Appearance on T1-weighted spin echo magnetic reso-
nance images. Four patterns are observed. A, Pattern 1 is characterized by the presence of high-signal-
intensity fatty marrow confined to linear areas along the basivertebral vein. B, Pattern 2 is characterized by
bandlike and triangular areas of fatty marrow in a peripheral location. C, Pattern 3 is characterized by multiple
small regions of high-signal-intensity fatty marrow. D, Pattern 4 is characterized by multiple large regions
of fatty marrow. Pattern 1 is common in all regions of the spine in the first 2 or 3 decades of life, and patterns
2, 3, and 4 become more dominant after age 30 or 40 years, particularly in the thoracic and lumbar regions.
(From Ricci C, Cova M, Kang YS, et al: Normal age-related patterns of cellular and fatty bone marrow
distribution in the axial skeleton: MR imaging study. Radiology 177:83, 1990.)
10 Section I Basic Science
Figure 1–13. Marrow conversion: proximal portion of the femur. Appearance on T1-weighted spin echo
magnetic resonance images. Four patterns are observed. A, Pattern 1 is characterized by high-signal-intensity
fatty marrow confined to the capital femoral epiphysis and greater and lesser trochanters. B, Pattern 2 resem-
bles pattern 1, with the addition of fatty marrow in the medial portion of the femoral head and in the lateral
portion of the intertrochanteric region. C, Pattern 3 resembles pattern 1, with the addition of many small,
sometimes confluent areas of fatty marrow in the intertrochanteric region. D, Pattern 4 is characterized by
uniform high-signal-intensity fatty marrow throughout the proximal portion of the femur, with the excep-
tion of the regions of the major trabecular groups. Patterns 1 and 2 predominate in the first 3 decades of
life, pattern 3 predominates in the fifth and sometimes the fourth decades of life, and pattern 4 predominates
after age 50 or 60 years.
(From Ricci C, Cova M, Kang YS, et al: Normal age-related patterns of cellular and fatty bone marrow
distribution in the axial skeleton: MR imaging study. Radiology 177:83, 1990.)
axial skeleton and thereafter from a proximal to distal Bone Resorption and Formation
direction in the extremities. The processes of resorption and formation occur con-
tinuously in normal bone. These processes are prominent
PHYSIOLOGY in an immature skeleton, in which modeling leads to the
major changes in bone size and shape that are required
Mineralization of Bone
for normal osseous growth and development; in a mature
At present, no unified concept exists for the mechanism skeleton, these processes are less evident but nonetheless
of bone mineralization. The process of biologic calcifi- essential for the maintenance of biomechanically com-
cation, in which hydroxyapatite or some similar material petent tissue and calcium homeostasis.
is deposited within an organic matrix, is complex. The As indicated previously, resorption and apposition
ions essential to formation of the crystalline unit in bone dominate on the bone surfaces present in the cortex and
are calcium (Ca2+) and phosphate (PO43−). Initial inter- spongiosa. Four broad surface areas exist in the skeleton,
pretations of the calcification process emphasized pre- each of which is functionally distinct (Fig. 1–14). These
cipitation dynamics in which the unique milieu of the areas are often referred to as envelopes. The first of these
organic matrix of bone provided the specific conditions areas, related to the outer surface of the cortex, is the
required for the deposition of these ions. periosteal envelope (or periosteum), which consists of an
outer sheath of fibrous connective tissue and an inner, or
cambrian, layer of undifferentiated cells. These two
Calcium Homeostasis
distinct histologic layers are not present everywhere; they
The skeleton contains 99% of the body’s calcium and are absent in intra-articular locations such as the femoral
serves as the essential reservoir for the maintenance of neck, at entheses or sites of tendinous and ligamentous
stable plasma levels of calcium. Approximately 70% of attachments to bone, and about the sesamoid bones. The
plasma calcium is believed to be maintained by a con- second of the envelopes, the haversian envelope, lies
tinuous exchange of calcium ions between bone tissue within the bone cortex and surrounds the individual
and the extracellular fluid; this interchange occurs between haversian systems. The corticoendosteal envelope relates
the hydroxyapatite crystals of all bone surfaces and proceeds to the inner surface of the bone cortex and is therefore
independently of any change in bone volume (i.e., forma- the outermost boundary of the medullary bone. It is
tion and resorption). Hypocalcemia stimulates the release interrupted at sites where the trabeculae of the medullary
of calcium ions from the bone mineral into the extra- cavity are connected to the cortex. This envelope
cellular fluid, and conversely, hypercalcemia promotes an functions primarily as a bone resorptive surface, and it
inward flux of calcium ions from the extracellular fluid to accounts for the general thinning of the cortex that
the bone mineral. Maintenance of the remaining 30% of occurs with advancing age in adults. The fourth envelope
plasma calcium may be mediated by the actions of is the endosteal envelope, which represents the interface
parathyroid hormone and other hormones. of medullary bone and marrow. As indicated previously,
Chapter 1 Histogenesis, Anatomy, and Physiology of Bone 11
and tissue macrophages, are involved in bone resorption.
Surfaces of bone involved in extensive resorption are sites
of accumulation of multinucleated osteoclasts. The finely
striated (brush) border of the osteoclast is in contact with
the adjacent bone and is in a state of vigorous movement.
Osteoclasts play an active role in the resorption of
bone; however, the precise mechanism of the process,
including the participation of other cells, is not clear.
Osteoclasts appear to be the major cells responsible for
the skeletal contribution to regulating the serum concen-
tration of calcium; all the agents that have been shown to
increase the serum calcium concentration in vivo have
also been shown to increase osteoclastic activity, and the
hormones and drugs that lower this concentration have
been shown to inhibit osteoclastic activity. Among the
substances capable of directly or indirectly stimulating
existing osteoclasts, increasing the formation of new
osteoclasts, or both are parathyroid hormone, active
metabolites of vitamin D, prostaglandin E2, thyroid hor-
mone, heparin, and interleukin-1; among those substances
inhibiting resorption are calcitonin, glucocorticoid,
diphosphonates, glucagon, phosphate, and carbonic anhy-
drase inhibitors. Osteoclastic resorption plays a major
role in the pathogenesis of a variety of skeletal disorders,
including metabolic processes such as osteoporosis,
neoplastic and inflammatory conditions accompanied by
bone lysis, Paget’s disease, and osteopetrosis.
Bone Formation
Figure 1–14. Bone resorption and formation: available bone The principal cell involved in the formation of bone is
envelopes. Transverse sections of the metaphysis (A) and diaphysis the osteoblast. Osteoblasts are derived from mesenchymal
(B) of a tubular bone reveal the osseous envelopes involved in osteoprogenitor cells, or preosteoblasts; they are involved
the processes of resorption and apposition. In the cortex, they in the synthesis of bone matrix and subsequently become
are the periosteal (1), haversian or osteonal (2), and corticoen- either internal osteocytes or inactive bone-lining cells.
dosteal (3) envelopes; in the spongiosa, an endosteal or transi- New bone formation may result from the activation of
tional (4) envelope is present.
bone-lining cells, the proliferation and differentiation of
preosteoblasts, or both.
The formation of bone occurs in two phases: matrix
this envelope is characterized by a very large surface area formation and mineralization. Matrix formation precedes
and is primarily a bone-losing envelope. mineralization and occurs at the interface between osteo-
Thus, at any particular time, such surfaces may normally blasts and existing osteoid; mineralization occurs at the
be quiescent or, less commonly, actively involved in the junction of osteoid and newly mineralized bone, a region
synthesis or resorption of bone. Their cellular composi- designated the mineralization front. The layer of unmin-
tion varies according to their functional state. It is the eralized matrix is termed the osteoid seam. In adults, the
coupling of bone resorption to bone formation that con- usual interval between matrix production and mineral-
trols the volume of bone that is present at any particular ization is 10 days. In certain disease states, such as osteo-
time. It appears likely that the mechanisms responsible malacia, the thickness of the osteoid seam is increased.
for coupling are intrinsic to bone and that an increase
in bone resorption must subsequently be coupled to an
increase in bone formation if bone volume is to remain Humoral Regulation of Bone Metabolism
unchanged. Bone metabolism and calcium homeostasis are intimately
related to interactions among the skeleton, intestines,
and kidneys, and they are involved in the presence
Bone Resorption
of many chemical factors, of which three hormones—
Although it has long been held that the osteoclast is the parathyroid hormone, calcitonin, and 1,25-dihydroxyvitamin
principal cell involved in the degradation of organic bone D— are most important.
matrix and in the release of bone mineral, a potential (albeit
controversial) role for the osteocyte in removing at least Parathyroid Hormone. An important regulator of skeletal
a small amount of perilacunar bone has also been empha- metabolism is parathyroid hormone, the two main func-
sized, and accumulating evidence indicates that mononu- tions of which are to stimulate and control the rate of
clear phagocytes, including peripheral blood monocytes bone remodeling and to influence mechanisms control-
12 Section I Basic Science
ling the plasma level of calcium. This hormone is pro- scribed in detail in Chapter 42. The general term vitamin
duced by the chief cells of the four parathyroid glands. It D refers to both vitamin D2 (ergocalciferol), which origi-
has a direct effect on bone (enhancing the mobilization nates in plants and is obtained from dietary sources, and
of calcium from the skeleton) and on the kidney (stimu- vitamin D3 (cholecalciferol), which occurs naturally in
lating the absorption of calcium from the glomerular the skin. In humans, these two forms of vitamin D have
fluid) and has an indirect effect on the intestines (influ- similar potency, so considering them separately has little,
encing the rate of calcium absorption). if any, clinical significance. The classic biologic role of
The direct effect of parathyroid hormone on bone vitamin D is regulation of intestinal mineral absorption
(Fig. 1–15) may be either bone resorption or bone for- and maintenance of skeletal growth and mineralization.
mation. An immediate action of parathyroid hormone is It is now widely accepted that these functions are medi-
to promote the process of osteoclastic resorption, which ated through the actions of 1,25-dihydroxyvitamin D
is fundamental to calcium homeostasis; more prolonged (1,25[OH]2D) on the intestine, bone, and kidney.
effects of parathyroid hormone are influential on bone Additionally, accumulating evidence based on in vitro
remodeling. Thus, at the cellular level, parathyroid hor- observations indicates that 1,25(OH)2D has important
mone influences osteoclasts, osteoblasts, osteocytes, and regulatory effects on blood mononuclear cells and on
bone surface cells. A significant increase in the number of the immune system. There is also some evidence that
osteoclasts and in the ratio of osteoclasts to osteoblasts 1,25(OH)2D plays a significant role in the intrathymic
may occur within hours after administration of the hor- differentiation of lymphocytes. The clinical relevance of
mone. Osteoblast function is decreased initially; how- these experimental data regarding the immunoregulatory
ever, subsequent stimulation of osteoblasts results in an role of 1,25(OH)2D is not known.
increase in bone formation.
Calcitonin. Calcitonin is released from the thyroid gland, Metabolic Bone Disorders
and secretion of calcitonin is controlled by the circulating
levels of calcium. Calcitonin inhibits bone resorption Histologic Techniques
and may lead to significant hypocalcemia and hypophos- Because differentiation of the two major metabolic bone
phatemia. Data also indicate that calcitonin has a stimu- diseases, osteoporosis and osteomalacia, is based in part
latory effect on bone growth in vivo. The importance of on the quantity and quality of bone mineral, the ability to
calcitonin as a regulator of calcium metabolism in distinguish between calcified and uncalcified bone matrix
humans, however, is not clear at present. (osteoid) is critical. The traditional procedures for pro-
cessing bone require the removal of inorganic matrix to
Vitamin D. Vitamin D is one of the most potent humoral facilitate histologic sectioning; therefore, these proce-
factors involved in the regulation of bone metabolism. dures prevent subsequent determination of the degree of
The biochemistry and mechanisms of action are de- skeletal mineralization. Because of deficiencies in tradi-
tional histologic techniques, additional methods have been
used, including qualitative and quantitative microscopic
assessment of nondecalcified specimens and the use of in
vivo bone markers such as tetracycline. Typically, the iliac
crest is used as the biopsy site from which nondecalcified
tissue sections are prepared.
In nondecalcified bone sections, osteomalacia is usually
characterized by the accumulation of osteoid as a conse-
quence of a defect in the mineralization process. Excess
quantities of osteoid, however, may result not only from
a decreased rate of mineralization but also from an accel-
erated rate of bone matrix synthesis. Differentiation
between these states is based on a determination of miner-
alization rates, with tetracycline used as an in vivo bone
marker (Table 1–2). Tetracycline fluorescence is evalu-
ated on unstained, nondecalcified tissue sections by ultra-
violet light. The first course of tetracycline appears as a
discrete fluorescent band within the mineralized bone.
The second, more recently administered course of tetra-
cycline is located at the current mineralization front (i.e.,
mineralized bone–osteoid seam interface). The distance
between the two bands represents the amount of new bone
synthesized and mineralized over the drug-free interval.
Figure 1–15. Osseous effects of parathyroid hormone: hyper-
parathyroidism. Magnification radiographs of the phalanges in
a normal person (A) and in a patient with hyperparathyroidism Normal and Abnormal Histologic Appearance
(B) reveal the effects of parathyroid hormone on bone. In (B),
note the osteopenia, indistinct trabeculae, and prominent sub- Normally, the contour of the external cortical margins is
periosteal bone resorption. smooth. Subperiosteal osteoid deposits, as well as eroded
Chapter 1 Histogenesis, Anatomy, and Physiology of Bone 13
TABLE 1–2
Tetracycline-Labeling Regimen for Bone Biopsy
Day Regimen
1, 2, 3 Tetracycline hydrochloride, 250 mg orally
four times daily or 500 mg orally twice
daily*†
4–17 Hiatus (no tetracycline)‡
18, 19, 20 Tetracycline hydrochloride, 250 mg orally
four times daily or 500 mg orally twice daily†
24 Bone biopsy§
*Tetracycline is given 1 hour before or 2 hours after meals. A larger dose is used
if malabsorption or severe osteomalacia is suspected; up to 3 g/day may be
necessary for patients after intestinal bypass.
If the patient has recently received tetracycline hydrochloride, the use of
oxytetracycline or demeclocycline in equivalent doses may help distinguish the
new tetracycline bone labels from the old because of differences in the
fluorescent color produced by the different tetracyclines.
†Avoid all dairy products, antacids, and iron-containing medicines on days
1, 2, 3, 18, 19, and 20. Figure 1–16. Cortical bone (C) of an iliac crest biopsy
‡An interval of at least 10 days is required between the two courses of specimen undergoing remodeling. Osteoclasts within cutting
tetracycline.
cones (CC) resorb endosteal bone, resulting in cortical
§Biopsy may be performed several days later, but not sooner. cancellization (i.e., the formation of cancellous trabecular bone
from preexisting cortical bone). A reduction in cortical width
ultimately occurs. H, Normal haversian canal before activation
(trichrome stain, ×25).
surfaces containing osteoclasts, are normally absent.
Subperiosteal bone resorption is evidence of activation of
osteoclasts and is seen in states of high bone turnover or
accelerated remodeling, such as in hyperparathyroidism. defective mineralization. The amount of tetracycline
Loss of cortical bone mass is suggested when cortical fluorescence is proportional to the amount of immature
thickness is reduced. Activation of osteoclasts leads to amorphous calcium phosphate deposits in the mineraliz-
increased resorption of bone, thereby enlarging the pre- ing foci of the osteoid seam.
existing vascular canals. Resorption of bone in the longi- The location and extent of bone removal and deposi-
tudinally oriented canals results in the formation of tion determine the physical anatomy of the skeleton and
cavities termed cutting cones. The junction between cor- the physiologic status of mineral metabolism. Bone
tical and medullary trabecular bone, which is normally remodeling activity is influenced by physical forces,
sharply demarcated, is termed the endosteum. Loss of serum levels of endocrine hormones, and nutritional and
distinction between the cortex and the medullary cavity metabolic factors. Normally, bone resorption and for-
occurs with increasing cortical porosity as a result of mation are in balance. A net loss of bone tissue may occur
increased cortical osteoclastic resorptive activity, as is from excessive bone resorption, deficient bone formation,
seen in severe hyperparathyroidism. Endosteal resorption or a combination of both processes during the coupling
cavities increase in number and depth until the previously process. Bone diseases resulting from an abnormality of
solid cortical bone becomes whittled into what appears to remodeling activity are characterized by failure of the
be new, thick trabeculae, a process referred to as cancel- skeleton to provide structural support, generally second-
lization or trabecularization of cortical bone (Fig. 1–16). ary to a deficiency in skeletal mass. When the bone mass
The total amount and quality of trabecular bone located can no longer sustain normal forces, a fracture may ensue
between the two cortices reflect the weight-bearing pro- and cause pain and deformity. A metabolic bone disease
perties of the skeleton. Usually, trabecular bone occupies is defined as any generalized disorder of the skeleton,
15% to 25% of the marrow space. A trabecular bone regardless of cause; most metabolic bone diseases result
volume below 15% is histologic evidence of osteopenia. from either an imbalance in remodeling activity or a
Normally, the individual trabeculae are continuous inter- disorder of matrix mineralization.
connecting or branching bands; atrophic trabeculae appear Osteopenia refers to a generalized reduction in bone
as struts, bars, or blots (Fig. 1–17), indicating a reduction mass that, on radiographic examination, appears as an
in mean trabecular plate density. exaggerated radiolucency of the skeleton. Osteoporosis
Decalcified sections taken from the bone core should and osteomalacia are the two major causes of osteopenia.
be examined under polarized light for evidence of woven Histologically, osteoporotic diseases may be accompanied
collagen architecture. Woven bone in a transiliac crest by either increased or decreased rates of bone turnover.
specimen is an abnormal finding in an adult patient and Osteomalacic syndromes are characterized by histologic
reflects accelerated skeletal turnover. Abnormal patterns evidence of defective mineralization (Table 1–3). High
of fluorescent label deposition are the hallmark of osteo- bone turnover diseases (Table 1–4) are characterized by
malacia and represent the morphologic expression of evidence of both increased formation and increased
14 Section I Basic Science
Figure 1–17. Normal and abnormal trabecular bone architecture. A, Low-power view of an iliac crest
biopsy specimen. Cortical thickness is reduced as a result of progressive erosion by cortical cutting cones
(CC). Trabecular bone (T), however, exhibits a normal, platelike, connecting architectural pattern (trichrome
stain, ×25). B, Reduction in trabecular bone (T) volume. Not only is the volume of bone reduced, but the
architecture of trabecular bone is also abnormal because of the presence of thin, widely spaced, atrophic rods
of bone (trichrome stain, ×25).
TABLE 1–3
General Morphologic Classification of Metabolic Bone resorption of bone. States associated with reduced bone
Diseases turnover (see Table 1–4) show little evidence of either
bone formation or bone resorption. Osteomalacia is
Osteoporosis usually characterized by excessive quantities of osteoid
High remodeling: active bone turnover caused by failure of matrix calcification despite continued
Low remodeling: inactive bone turnover matrix synthesis by osteoblasts. Marked increases in the
Osteomalacia thickness of osteoid seams are characteristic, but osteo-
Low remodeling: pure osteomalacia malacia may be associated with normal or even reduced
High remodeling: mixed osteomalacia and osteitis fibrosa cystica quantities of osteoid.
TABLE 1–4
Bone Morphology Associated with Specific Metabolic Diseases
Fibrous
SUMMARY OF KEY FEATURES
Suture Skull
An understanding of the structure of joints is essential Syndesmosis Distal tibiofibular interosseous membrane
to the proper interpretation of radiographs in numerous Radioulnar interosseous membrane
diseases. Joints can be classified into three types: fibrous, Sacroiliac interosseous ligament
cartilaginous, and synovial. In addition, supporting Gomphosis Teeth
structures (tendons, aponeuroses, fasciae, and ligaments) Cartilaginous
influence the manifestation of articular disorders.
Symphysis Symphysis pubis
Intervertebral disc
Manubriosternal joint
Central mandible
INTRODUCTION Synchondrosis Physeal plate (growth plate)
Neurocentral joint
Joints have been classified according to (1) the extent of Spheno-occipital joint
joint motion and (2) the type of articular histology. The Synovial
classification of articulations based on the extent of joint
motion is as follows: Large, small joints of extremities
Sacroiliac joint
Synarthroses: fixed or rigid joints Apophyseal joint
Amphiarthroses: slightly movable joints Costovertebral joint
Sternoclavicular joint
Diarthroses: freely movable joints
The classification of joints on the basis of histology
emphasizes the type of tissue that characterizes the junc-
tional area (Table 2–1). The following categories are tribution. This obliteration usually occurs at the bregma
recognized: and subsequently extends into the sagittal, coronal, and
Fibrous articulations: apposed bony surfaces fastened lambdoid sutures, in that order. Despite the normal varia-
together by fibrous connective tissue tions in suture development and closure, their assess-
ment is important in the diagnosis of obstructive hydro-
Cartilaginous articulations: apposed bony surfaces initially
cephalus and cranial synostosis. Computed tomography
or eventually connected by cartilaginous tissue
scanning seems to be a superior technique for making
Synovial articulations: apposed bony surfaces separated this delineation.
by an articular cavity lined by synovial membrane
This second method of classification can lead to diffi-
Syndesmosis
culty, because joints that are similar histologically may
differ considerably in function and degree of allowable A syndesmosis (Fig. 2–2) is a fibrous joint in which adja-
motion; however, it is used in the following discussion. cent bony surfaces are united by an interosseous liga-
ment, as in the distal tibiofibular joint, or an interosseous
membrane, as at the diaphyses of the radius, ulna, tibia,
FIBROUS ARTICULATIONS and fibula. A syndesmosis may demonstrate minor degrees
In a fibrous articulation, apposed bony surfaces are fastened of motion related to stretching of the interosseous
together by intervening fibrous tissue. Fibrous articu- ligament or flexibility of the interosseous membrane.
lations can be subdivided into three types: sutures, syn-
desmoses, and gomphoses.
Gomphosis
A gomphosis (Fig. 2–3) is a special type of fibrous joint
Suture located between the teeth and the maxilla or mandible.
Limited to the skull, sutures (Fig. 2–1) allow no active At these sites, the articulation resembles a peg that fits
motion and exist where broad osseous surfaces are sepa- into a fossa or socket. The intervening membrane between
rated only by a zone of connective tissue. Although clas- tooth and bone is termed the periodontal ligament.
sically a suture is considered to be a fibrous joint, areas of
secondary cartilage formation may be observed during
CARTILAGINOUS ARTICULATIONS
the growth period, and in later life, sutures may undergo
bony union or synostosis. Bony obliteration of the sutures There are two types of cartilaginous joints: symphyses
is somewhat variable in its time of onset and cranial dis- and synchondroses.
16
Chapter 2 Articular Anatomy and Histology 17
Figure 2–1. Fibrous articulation: suture. Schematic drawings indicate the structure of a typical suture in
the skull. Note the interdigitations of the osseous surfaces. The specific layers that intervene between the
ends of the bones are indicated at the upper right. These include the cambial (1), capsular (2), and middle
(3) layers. A uniting (4) layer is also indicated.
(Reproduced in part from Pritchard JJ, Scott JH, Girgis FG: The structure and development of cranial
and facial sutures. J Anat 90:73, 1956. Courtesy of Cambridge University Press.)
Symphysis
In a symphysis (Fig. 2–4), adjacent bony surfaces are con-
nected by a cartilaginous disc, which arises from chon-
drification of intervening mesenchymal tissue. Eventually,
this tissue is composed of fibrocartilaginous or fibrous
connective tissue, although a thin layer of hyaline carti-
lage usually persists, covering the articular surface of the
adjacent bone. Symphyses (typified by the symphysis pubis
and intervertebral disc) allow a small amount of motion,
which occurs through compression or deformation of the
intervening connective tissue.
Some symphyses, such as the symphysis pubis and
manubriosternal joint, have a small, cleftlike central cavity
that contains fluid and may enlarge with advancing age;
this cavity may be demonstrable radiographically, owing
to the presence of gas (vacuum phenomenon). Symphy-
ses are located within the midsagittal plane of the human
body and are permanent structures, unlike synchondroses,
which are temporary joints. Infrequently, intra-articular
ankylosis or synostosis may obliterate a symphysis, such
as occurs at the manubriosternal joint.
A B Synchondrosis
Synchondroses (Fig. 2–5) are temporary joints that exist
Figure 2–2. Fibrous articulation: syndesmosis. A and B, The
interosseous membrane between the medial aspect of the radius during the growing phase of the skeleton and are com-
and the lateral aspect of the ulna originates approximately 3 cm posed of hyaline cartilage. Typical synchondroses are the
below the radial tuberosity and extends to the wrist, containing cartilaginous growth plate between the epiphysis and
apertures for various interosseous vessels. The radiograph metaphysis of a tubular bone; the neurocentral vertebral
reveals an osseous crest on apposing surfaces of bone. articulations; and the unossified cartilage in the chondro-
18 Section I Basic Science
Figure 2–3. Fibrous articulation: gomphosis. A, Diagrammatic representation of this special type of
articulation located between the teeth and the maxilla or mandible. Note the location of the periodontal
membrane. B, Radiograph reveals the radiolucent periodontal membrane (arrowhead) and the radiopaque
lamina dura (arrow).
cranium, the spheno-occipital synchondrosis. With skele- which may be reinforced by accessory ligaments. The
tal maturation, synchondroses become thinner and are inner portion of the articulating surface of the apposing
eventually obliterated by bony union or synostosis. bones is separated by a space, the articular or joint cavity.
Articular cartilage covers the ends of both bones; motion
between these cartilaginous surfaces is characterized by a
SYNOVIAL ARTICULATIONS low coefficient of friction. The inner aspect of the joint
A synovial joint is a specialized type of joint that is capsule is formed by the synovial membrane, which
located primarily in the appendicular skeleton (Fig. 2–6). secretes synovial fluid into the articular cavity. This syn-
Synovial articulations generally allow unrestricted ovial fluid acts both as a lubricant, encouraging motion,
motion. The structure of a synovial joint differs funda- and as a nutritive substance, providing nourishment to
mentally from that of fibrous and cartilaginous joints; the adjacent articular cartilage. In some synovial joints,
osseous surfaces are bound together by a fibrous capsule, an intra-articular disc of fibrocartilage partially or com-
pletely divides the joint cavity. Additional intra-articular
structures, including fat pads and labra, may be noted.
Articular Cartilage
The articulating surfaces of the bone are covered by a
layer of glistening connective tissue, the articular carti-
lage. Its unique properties include transmission and
distribution of high loads, maintenance of contact
stresses at acceptably low levels, movement with little
friction, and shock absorption. In most synovial joints,
the cartilage is hyaline in type. Articular cartilage is
devoid of lymphatic vessels, blood vessels, and nerves.
A large portion of the cartilage derives its nutrition
through diffusion of fluid from the synovial cavity. Small
blood vessels pass from the subchondral bone plate into
the deepest stratum of cartilage, providing nutrients to
this area of articular cartilage. Additionally, a vascular
Figure 2–4. Cartilaginous articulation: symphysis (symphysis ring is located within the synovial membrane at the
pubis). Note the central fibrocartilage (FC), with a thin layer of periphery of the cartilage.
hyaline cartilage (HC) adjacent to the osseous surfaces of the Articular cartilage is variable in thickness. It may be
pubis. thicker on one articulating bone than on another. Further,
Chapter 2 Articular Anatomy and Histology 19
Figure 2–5. Cartilaginous arti-
culation: synchondrosis. A, Radio-
graph of the phalanges in a grow-
ing child demonstrates a typical
epiphysis separated from the meta-
physis and diaphysis by the radio-
lucent growth plate. B, Schematic
drawing of a growth plate between
the cartilaginous epiphysis and the
ossified diaphysis of a long bone.
Note the transition from hyaline
cartilage through various carti-
laginous zones, including resting
cartilage, cell proliferation, cell
hypertrophy, cell calcification, and
bone formation.
articular cartilage is not necessarily of uniform thickness with smoothly fitted ones; and (5) in young and middle-
over the entire osseous surface. In general, it varies from aged persons compared with older people.
1 to 7 mm thick, averaging 2 or 3 mm. Cartilage is thicker
(1) in large joints than in small joints; (2) in joints or areas
Subchondral Bone Plate and Tidemark
of joints in which there is considerable functional pres-
sure or stress, such as those in the lower extremity; (3) at The bony or subchondral endplate is a layer of osseous
sites of extensive frictional or shearing force; (4) in poorly tissue of variable thickness that is located beneath the
fitted articulations (i.e., less congruent joints) compared cartilage. Immediately superficial to the subchondral bone
Articular Capsule
The articular capsule is connective tissue that envelops
the joint cavity. It is composed of a thick, tough outer
layer—the fibrous capsule—and a more delicate, thin
inner layer—the synovial membrane.
Synovial Fluid
Minute amounts of clear, colorless to pale yellow, highly
viscous fluid of slightly alkaline pH are present in healthy
joints. The exact composition, viscosity, volume, and color
vary somewhat from joint to joint. Particles, cell frag-
ments, and fibrous tissue may also be seen in the synovial
fluid as a result of wear and tear of the articular surface.
Functions of the synovial fluid are to provide nutrition to
the adjacent articular cartilage and disc and lubrication of
joint surfaces, which decreases friction and increases joint
efficiency.
articulations, the synovial membrane of the bursa may be adjacent to joints (type A) are present most frequently on
continuous with that of the joint cavity, producing com- the palmar aspect of the metacarpophalangeal joints,
municating bursae. This occurs normally about the hip particularly the first. In this location, two sesamoids are
(iliopsoas bursa) and knee (gastrocnemiosemimembranous found in the tendons of the adductor pollicis and flexor
bursa) and abnormally about the glenohumeral joint (sub- pollicis brevis, articulating with facets on the palmar surface
acromial bursa) owing to defects in the rotator cuff. of the metacarpal head. Additional sesamoids are most
Distention of communicating bursae may serve to lower frequent in the second and fifth metacarpophalangeal
intra-articular pressure in cases of joint effusion. At cer- joints and adjacent to the interphalangeal joint of the
tain sites where skin is subject to pressure and lateral thumb. This distribution of sesamoids in the hand is not
displacement, adventitious bursae may appear, allowing constant. Sesamoid bones unassociated with synovial joints
increased freedom of motion. Examples of adventitious (type B) are more frequent in the lower extremity than in
bursae include those that may develop over a hallux valgus the upper extremity. In the foot, sesamoids of this type
deformity, those occurring about prominent spinous pro- are noted in the tendon of the peroneus longus muscle
cesses, and bursae located adjacent to exostoses. adjacent to a facet on the tuberosity of the cuboid bone and
in the tendon of the tibialis anterior muscle in contact
with the medial surface of the medial cuneiform bone.
Sesamoid Bones
Sesamoids generally are small ovoid nodules embedded
in tendons (Fig. 2–10). They are found in two specific SUPPORTING STRUCTURES
situations in the skeleton.
Tendons
Type A. In type A, the sesamoid is located adjacent to a Tendons represent a portion of a muscle and are of con-
joint, and its tendon is incorporated into the joint cap- stant length, consisting of collagen fibers that transmit
sule. The sesamoid nodule and adjacent bone form an muscle tension to a mobile part of the body. They are
extension of the articulation. Examples of this type are flexible cords that can be angulated about bony protu-
the patella and the hallucis and pollicis sesamoids. berances, changing the direction of pull of the muscle.
Synovial sheaths may surround portions of the tendon. In
Type B. In type B, the sesamoid is located at sites where some locations, such as the flexor tendons about the
tendons are angled about bony surfaces. They are sepa- ankle, fluid is normally observed in these synovial sheaths.
rated from the underlying bone by a synovium-lined bursa. The attachment sites of tendons are termed entheses.
An example of this type of sesamoid is the sesamoid of
the peroneus longus.
In both type A and type B situations, the arrangement Aponeuroses
of the sesamoid nodule and surrounding tissue resembles Aponeuroses consist of several flat layers or sheets of
that of a synovial joint. In the hand, sesamoid nodules dense collagen fibers associated with the attachment of a
muscle. The fasciculi within one layer of an aponeurosis
are parallel, and they differ in direction from fasciculi of
an adjacent layer.
Fasciae
Fascia is a general term used to describe a focal collection
of connective tissue. Superficial fascia consists of a layer
of loose areolar tissue of variable thickness beneath the
dermis. It is most distinct over the lower abdomen,
perineum, and limbs. Deep fascia resembles an aponeu-
rosis, consisting of regularly arranged, compact collagen
fibers. Parallel fibers of one layer are angled with respect
to the fibers of an adjacent layer. Deep fascia is particu-
larly prominent in the extremities, and in these sites,
muscle may arise from the inner aspect of the deep fascia.
At sites where deep fascia contacts bone, the fascia fuses
with the periosteum. It is well suited to transmit the pull
of adjacent musculature. Intermuscular septa extend from
Figure 2–10. Sesamoid bones. There are two types of deep fascia between groups of muscles, producing func-
sesamoids: type A (A), in which the sesamoid is located adjacent tional compartments. These compartments are important
to an articulation, and type B (B), in which the sesamoid is
with regard to patterns of spread of infection and tumor.
separated from the underlying bone by a bursa. In both types,
the sesamoid is intimately associated with a synovial lining and Retinacula are transverse thickenings in the deep fascia
articular cartilage (hatched areas). that are attached to bony protuberances, creating tunnels
(From Resnick D, Niwayama G, Feingold ML: The through which tendons can pass. An example is the dorsal
sesamoid bones of the hands and feet: Participators in arthritis. retinaculum of the wrist, under which extend the exten-
Radiology 123:57, 1977.) sor tendons and their synovial sheaths.
Chapter 2 Articular Anatomy and Histology 23
Ligaments FURTHER READING
Ligaments represent fibrous bands that unite bones. They Barnett CH, Davies DV, MacConaill MA: Snyovial Joints:
do not transmit muscle action directly but are essential Their Structure and Mechanics. Springfield, Ill, Charles C
in the control of posture and the maintenance of joint Thomas, 1961.
stability. Histologically and biomechanically, ligaments Canoso JJ: Bursae, tendons and ligaments. Clin Rheum Dis
resemble tendons, and their sites of osseous attachment 7:189, 1981.
Davies DV: The structure and functions of the synovial mem-
(entheses) are similar to those of tendons. brane. BMJ 1:92, 1950.
Jaffe HL: Metabolic, Degenerative and Inflammatory Diseases
VASCULAR, LYMPHATIC, AND NERVE of Bones and Joints. Philadelphia, Lea & Febiger, 1972, p 80.
SUPPLY Resnick D, Niwayama G: Entheses and enthesopathy: Anatom-
ical, pathological, and radiological correlation. Radiology
The blood supply of joints arises from periarticular arte- 146:1, 1983.
rial plexuses that pierce the capsule, break up in the syn- Resnick D, Niwayama G, Feingold ML: The sesamoid bones
ovial membrane, and form a rich and intricate network of of the hands and feet: Participators in arthritis. Radiology
capillaries. A circle of vessels (circulus articularis vascu- 123:57, 1977.
losus) within the synovial membrane is adjacent to the Shepherd DET, Seedhom BB: Thickness of human articular
cartilage in joints of the lower limb. Ann Rheum Dis 58:27,
peripheral margin of articular cartilage. 1999.
The lymphatics form a plexus in the subintima of the Simkin PA: Friction and lubrication in synovial joints.
synovial membrane. Efferent vessels pass toward the J Rheumatol 27:567, 2000.
flexor aspect of the joint and then along blood vessels to Walmsley R: Joints. In Romanes GJ (ed): Cunningham’s Text-
regional deep lymph nodes. The nerve supply of movable book of Anatomy, 11th ed. London, Oxford University
joints generally arises from the same nerves that supply Press, 1972, p 207.
the adjacent musculature. The fibrous capsule and, to a Wyke B: The neurology of joints: A review of general prin-
lesser extent, the synovial membrane are both supplied ciples. Clin Rheum Dis 7:223, 1981.
by nerves.
3
CHAPTER 3
Anatomy of Individual Joints The complexity of wrist motion has led to differing
concepts of functional osseous anatomy. Some regard the
wrist as composed of carpal bones arranged in two rows
(proximal and distal), with the scaphoid bridging the two.
SUMMARY OF KEY FEATURES Others describe the joint as a vertical arrangement con-
Anatomic features related to articular and periarticular sisting of three columns. A mobile lateral column contains
soft tissue and osseous structures govern the manner in the scaphoid, trapezium, and trapezoid; osteoarthritis
which disease processes become evident on radiographs. occurs here most frequently. The central column, con-
This chapter summarizes the basic osseous and soft taining the lunate and capitate, is concerned with flexion
tissue anatomy of individual joints in the body. The and extension and is primarily implicated in most varieties
tendinous and ligamentous anatomy is reviewed in of carpal instability. The medial column is composed of
greater detail in Chapter 5. the triquetrum and hamate, and, on the axis of this column,
the rotation of the forearm is extended into the wrist. A
third concept considers the wrist as a dynamic ring, with
a fixed distal half and a mobile proximal half. Distortion
WRIST or rupture of the mobile segment with respect to the
rigid part explains both instability and dislocation.
Osseous Anatomy
The osseous structures about the wrist are the distal por- Soft Tissue Anatomy
tions of the radius and ulna, the proximal and distal rows
of carpal bones, and the metacarpals. The distal aspects The wrist is not a single joint. Rather, it consists of a
of the radius and ulna articulate with the proximal row of series of articulations or compartments (Fig. 3–2).
carpal bones. The articular surface of the radius is divided
into an ulnar and a radial portion by a faint central ridge Radiocarpal Compartment. The radiocarpal compartment
of bone. The ulnar portion articulates with the lunate, and (Fig. 3–3) is formed proximally by the distal surface of
the radial portion articulates with the scaphoid. The medial the radius and the triangular fibrocartilage complex and
surface of the distal end of the radius contains the con- distally by the proximal row of carpal bones exclusive of
cave ulnar notch, which articulates with the distal end of the pisiform. The triangular fibrocartilage prevents com-
the ulna. The proximal row of carpal bones consists of the munication of the radiocarpal and inferior radioulnar
scaphoid, lunate, and triquetrum, as well as the pisiform compartments, whereas a meniscus may attach to the tri-
bone within the tendon of the flexor carpi ulnaris muscle. quetrum, preventing communication of the radiocarpal
The distal row of carpal bones contains the trapezium, and pisiform-triquetral compartments. The triangular
trapezoid, capitate, and hamate bones. A strong fibrous fibrocartilage, the meniscus, the dorsal and volar radio-
retinaculum attaches to the palmar surface of the carpus, ulnar ligaments, the ulnar collateral ligament, the ulno-
converting the carpal groove into a carpal tunnel, through carpal ligaments, and (sometimes) the sheath of the
which pass the median nerve and flexor tendons. extensor carpi ulnaris tendon are the components of the
Ulnar variance relates to the length of the ulna com- triangular fibrocartilage complex of the wrist and repre-
pared with that of the radius. A positive ulnar variance sent important stabilizers about the inferior radioulnar
(i.e., ulnar plus) means a relatively long ulna in which joint (see Chapter 59). Synovial diverticula, or recesses,
the articular surface of the ulna projects distal to that of are common and vary in number and size.
the radius; this variance is associated with the ulnocarpal
Inferior Radioulnar Compartment. The inferior radioulnar
abutment, or impaction, syndrome. A negative ulnar
compartment (see Fig. 3–2) is an L-shaped joint whose
variance implies a relatively short ulna and is associated
proximal border is the cartilage-covered head of the ulna
with Kienböck’s disease.
and ulnar notch of the radius. Its distal limit is the
When the wrist is in the neutral position without
triangular fibrocartilage.
dorsal or palmar flexion, the distal end of the radius
articulates with the scaphoid and approximately 50% of Midcarpal Compartment. The midcarpal compartment
the lunate. The degree of radial deviation of the radio- (see Fig. 3–2) extends between the proximal and distal
carpal compartment can be measured on a posteroante- carpal rows. On the radial aspect of the midcarpal com-
rior radiograph with the wrist in this neutral attitude. A partment, the trapezium and trapezoid articulate with the
line is drawn through the longitudinal axis of the second distal aspect of the scaphoid. The radial side of this
metacarpal at its radial cortex. On a lateral radiograph of compartment is called the trapezioscaphoid space.
a normal wrist in the neutral position without palmar
flexion or dorsiflexion, a continuous line can be drawn Pisiform-Triquetral Compartment. The pisiform-triquetral
through the longitudinal axes of the radius, lunate, capi- compartment (Fig. 3–4) exists between the palmar sur-
tate, and third metacarpal. The alignment of the bones in face of the triquetrum and the dorsal surface of the pisi-
the wrist joints varies with wrist position (Fig. 3–1). form. A large proximal synovial recess can be noted.
24
Figure 3-1. Hand and wrist: normal and abnormal alignment. A and B, Frontal projection. The angle of
intersection of lines Aand B, measuring radial deviation of the radiocarpal compartment, normally averages
112 degrees (A) and is increased in rheumatoid arthritis (B). Lines C and D measure ulnar deviation a t the
metacarpophalangeal joints. C, Lateral projecdon. Line drawings of longitudinal axes of the third metacarpal,
navidar 0or scaphoid, h a t e (L),capitate (C), and radius (R) in dorsiflexioninstability (upper), in a normal
situation (middle), and in palmar flexion instability (lower). When the wrist is normal, a continuous line can
he drawn through the longitudinal axes of the capitate, lunate, and radius, and this line intersects a second line
through the longitudinal axis of the scaphoid, creating an angle of 30 to 60 degrees. In dorsiflexion insta-
bility, the h a t e is flexed toward the back of the hand and the scaphoid is displaced vertically The angle of
intersection between the two longitudinal axes is greater than 60 degrees. In palmar flexion instability, the
h a t e is flexed toward the palm and the angle between the two longitudinal axes is less than 30 degrees.
(Aand B, From Resuick D: Rheumatoid arthritis of the wrist: The comparunental approach. Med Radiogr
P h o t o g 52:50, 1976. C, From Linscheid RL, Dohyns JH, Beahout JW, Bryan RS: Traumatic instability of
the wrist. Diagnosis, classification, and pathomecbanics. J Bone Joint Snrg Am 541612, 1972.)
26 Section I Basic Science
Common Carpometacarpal Compartment. This compart- Communication between Compartments. Although the
ment exists between the base of each of the four medial wrist compartments are distinct structures, direct com-
metacarpals and the distal row of carpal bones (see munication between compartments has been well docu-
Fig. 3–2). Occasionally, the articulation between the mented. Direct communication between the radiocarpal
hamate and the fourth and fifth metacarpals is a separate and inferior radioulnar joint has been noted in 7% of the
synovial cavity, produced by a ligamentous attachment radiocarpal compartment arthrograms of living persons.
between the hamate and fourth metacarpal. This communication results from a full-thickness defect
of the triangular fibrocartilage, a finding seen more fre-
First Carpometacarpal Compartment. The carpometacarpal quently in elderly persons, which relates to cartilaginous
compartment of the thumb is a separate saddle-shaped degeneration.
cavity between the trapezium and base of the first meta- Communications have also been demonstrated between
carpal (see Fig. 3–2). the radiocarpal and midcarpal compartments (resulting
from a full-thickness defect of the interosseous ligaments
Intermetacarpal Compartments. Three intermetacarpal that extend between the bones of the proximal carpal row),
compartments extend between the bases of the second radiocarpal and pisiform-triquetral compartments, and mid-
and third, the third and fourth, and the fourth and fifth carpal, carpometacarpal, and intermetacarpal compartments.
metacarpals. These compartments usually communicate The frequency of these communications is not known.
with one another and with the common carpometacarpal Extensor tendons traverse the dorsum of the wrist, sur-
compartment. rounded by synovial sheaths (Fig. 3–5). The attachment
Chapter 3 Anatomy of Individual Joints 27
METACARPOPHALANGEAL JOINTS
Figure 3–4. Articulations of the wrist: specific compartments. Osseous Anatomy
Pisiform-triquetral compartment (coronal section). This com-
partment (PTQ) exists between the triquetrum (triq.) and At the metacarpophalangeal joints, the metacarpal heads
pisiform (pis.). The radiocarpal (1) and inferior radioulnar (2) articulate with the proximal phalanges (Fig. 3–8). The
compartments are also indicated. medial four metacarpal bones lie side by side; the first
28 Section I Basic Science
Figure 3–8. Metacarpals and phalanges: osseous anatomy. A and B, Dorsal (A) and ventral (B) aspects of
the third metacarpal and phalanges. Note the more extensive articular surface on the volar aspect of the
metacarpal head and phalanges (arrowheads). C, Drawings of the palmar and medial aspects of the metacar-
pophalangeal and interphalangeal joints of the fourth digit reveal the deep transverse metacarpal ligament
(arrowhead) with its central groove for the flexor tendons (arrow) and the capsule of the interphalangeal
joints.
with a mass or fluid may produce a positive fat pad sign, a slightly constricted area that encircles the bone,
characterized by elevation and displacement of anterior separating the head from the tuberosities. The anatomic
and posterior fat pads (Fig. 3–11). neck is the site of attachment of the capsular ligament of
the glenohumeral joint. The greater tuberosity is located
on the lateral aspect of the proximal end of the humerus.
GLENOHUMERAL JOINT
The tendons of the supraspinatus and infraspinatus mus-
The glenohumeral joint lies between the roughly hemi- cles insert on its superior portion, whereas the tendon of
spheric head of the humerus and the shallow cavity of the the teres minor muscle inserts on its posterior aspect.
glenoid region of the scapula. Stability of this articulation The lesser tuberosity is located on the anterior portion of
is limited for two major reasons: the scapular “socket” the proximal humerus, immediately below the anatomic
is small compared with the size of the adjacent humeral neck. The subscapularis tendon attaches to the medial
head, so that apposing osseous surfaces provide little inher- aspect of this structure, as well as to the humeral neck
ent stability; and the joint capsule is quite redundant, below the lesser tuberosity. Between the greater and
providing little additional support. lesser tuberosities is located the intertubercular sulcus
or groove (bicipital groove), through which passes the
tendon of the long head of the biceps brachii muscle.
Osseous Anatomy The shallow glenoid cavity is located on the lateral
The upper end of the humerus consists of the head and margin of the scapula (Fig. 3–13). Although there is
the greater and lesser tuberosities (tubercles) (Fig. 3–12). variation in the osseous depth of the glenoid region, a
Beneath the head is the anatomic neck of the humerus, fibrocartilaginous labrum encircles and slightly deepens
Chapter 3 Anatomy of Individual Joints 31
Figure 3–10. Elbow joint: normal anatomy. Drawings of coronal (A) and sagittal (B) sections. Observe the
synovium (s), articular cartilage (c), fibrous capsule (fc), anterior and posterior fat pads (f), and olecranon
bursa (ob). Note the extension of the elbow joint between the radius and ulna as the superior radioulnar joint
(arrow).
f
f
ACROMIOCLAVICULAR JOINT
The acromioclavicular joint is a synovial articulation
between the lateral aspect of the clavicle and the medial
aspect of the acromion.
Figure 3–13. Scapula: osseous anatomy—anterior (costal)
aspect. The scapula is viewed in the position it maintains when
an anteroposterior radiograph of the shoulder is obtained. Osseous Anatomy
Observe the acromion (a), clavicular facet (cf), coracoid process
(c), glenoid cavity (g), and infraglenoid tubercle (t). Note that The lateral or acromial end of the clavicle articulates
the glenoid cavity is not seen tangentially. with the acromial facet of the scapula and is the site of
attachment of the joint capsule of the acromioclavicular
joint. The inferior surface of the acromial end of the
clavicle possesses a rough osseous ridge, termed the trape-
the glenoid cavity. The glenoid contour may be almost zoid line. A conoid tubercle is located at the posterior
flat or slightly curved, or it may possess a deep, socket- aspect of the lateral clavicle. The trapezoid line and conoid
like appearance. A supraglenoid tubercle is located above tubercle are the sites of attachment of the trapezoid and
the glenoid cavity, to which is attached a tendinous conoid parts of the coracoclavicular ligament. The
portion of the long head of the biceps muscle. Below the acromion is a forward protuberance of the lateral aspect
cavity is a thickened ridge of bone, the infraglenoid of the scapula. The inferior surface of the acromion
tubercle, which is a site of tendinous attachment for the varies in its slope and configuration, factors that may be
long head of the triceps muscle. important in the pathogenesis of one type of shoulder
impingement syndrome and rotator cuff pathology (see
Chapter 59).
Soft Tissue Anatomy
A loose fibrous capsule arises medially from the circum-
ference of the glenoid labrum or, anteriorly, from the Soft Tissue Anatomy
neck of the scapula. It inserts distally into the anatomic The articular surfaces about the acromioclavicular joint
neck of the humerus and periosteum of the humeral are covered with fibrocartilage. In the central portion of
diaphysis. In certain areas, the fibrous capsule is strength- the joint is an articular margin that partially or, more rarely,
ened by its intimate association with surrounding tendons completely divides the joint cavity. The fibrous capsule
and ligaments. The tendons of the supraspinatus, infra- surrounds the articular margin and is reinforced on its
spinatus, teres minor, and subscapularis muscles form superior and inferior surfaces. Surrounding ligaments
a cuff—the rotator cuff—which blends with and rein- include the acromioclavicular and coracoclavicular liga-
forces the fibrous capsule. The coracohumeral ligament ments. The former ligament, which is located at the supe-
strengthens the upper part of the capsule and is located rior portion of the joint, extends between the clavicle and
(along with the tendon of the long head of the biceps acromion. The coracoclavicular ligament, which attaches
muscle and superior glenohumeral ligament) in a space to the coracoid process of the scapula and clavicle, is com-
between the supraspinatus and subscapularis tendon, posed of a trapezoid (medial) part and a conoid (lateral)
designated the rotator interval. Anteriorly, the capsule part. The trapezoid and conoid parts of the coraco-
may thicken to form the superior, middle, and inferior clavicular ligament may be separated by fat or a bursa.
glenohumeral ligaments. Portions of the last of these
ligaments are referred to collectively as the inferior
glenohumeral ligament complex (see Chapter 59). STERNOCLAVICULAR JOINT
Several synovium-lined recesses or bursae are present At the sternoclavicular articulation, the medial end of
about the glenohumeral joint. The subscapular recess lies the clavicle articulates with the clavicular notch of the
Chapter 3 Anatomy of Individual Joints 33
manubrium sterni and with the cartilage of the first rib vertebral discs are symphyses; those between vertebral
(Fig. 3–14). bodies that consist of anterior and posterior longitudinal
ligaments are syndesmoses (Fig. 3–15).
Osseous Anatomy Intervertebral Discs. Intervertebral discs separate the ver-
The enlarged medial or sternal end of the clavicle projects tebral bodies from the axis to the sacrum. The attach-
above the upper margin of the manubrium. The articular ments of the discs include the anterior and posterior
surface is smooth except on its superior portion, where a longitudinal ligaments and the intra-articular ligaments.
roughened area allows attachment of an articular disc. Discs are thickest in the lumbar region and thinnest in
The inferior portion of the articular surface is extended the upper thoracic area. The intervertebral discs between
to allow articulation with the first costal cartilage. Below the cervical vertebrae do not extend to the lateral edges
each clavicular notch is a rough projection, which receives of the vertebral bodies (Fig. 3–16). In this area, articular
the first costal cartilage. modifications are found on both sides of the interver-
tebral discs as cleftlike cavities between the superior
Soft Tissue Anatomy surface of the uncinate process of one vertebra and the
lateral lips of the inferior articulating surface of the
The articular end of the clavicle is covered with a layer vertebra above. These modifications are called the joints
of fibrocartilage that is thicker than the cartilage on the of Luschka.
sternum. A flat, circular disc is located between the Each disc consists of an inner portion, the nucleus
articulating surfaces of the clavicle and sternum; it pulposus, surrounded by a peripheral portion, the anulus
divides the joint into two articular cavities. The disc acts fibrosus. The nucleus pulposus is soft and gelatinous in
as a checkrein against medial displacement of the inner young persons, but it is gradually replaced by fibrocar-
clavicle. Perforations in the disc are frequent in older tilage with increasing age. The anulus fibrosus encircles
persons. A fibrous capsule surrounds the joint. The the nucleus pulposus and unites the vertebral bodies
sternoclavicular joint is freely mobile. It participates in firmly. The lamellae of the anulus fibrosus are thinner
movements of the upper extremity, including elevation, and more closely packed between the nucleus and the
depression, protraction, retraction, and circumduction. dorsal aspect of the intervertebral disc. Anteriorly, the
lamellae are stronger and more distinct. The direction in
JOINTS OF THE VERTEBRAL BODIES which they are oriented varies with their relative position
in the intervertebral disc. More peripherally, the bands of
Osseous and Soft Tissue Anatomy fibers are vertical. At the extreme periphery of the inter-
Joints separating vertebral bodies are of two types: articu- vertebral disc, the lamellae may become curved, with their
lations between vertebral bodies that consist of inter- convexity facing the periphery of the intervertebral disc.
Figure 3–14. Sternoclavicular joint: normal anatomy. A, Diagram of the anterior aspect of the upper
sternum and medial clavicles. On the right-hand side, the superficial bone has been removed, exposing the
sternoclavicular, manubriosternal, and second sternocostal joints. Identified structures are the anterior
sternoclavicular ligament (arrow), costoclavicular ligament (arrowhead on left), interclavicular ligament
(arrowhead in center), and articular disc (d). Note the first (1) and second (2) costal cartilages. B, Photographs
of a coronal section through the sternoclavicular joint in a cadaver reveal characteristics of the intra-articular
disc (d).
(A, From Warwick R, Williams P: Gray’s Anatomy, 35th Br ed. Philadelphia, WB Saunders, 1973.)
34 Section I Basic Science
Osseous Anatomy
The vertebral arch contains two pedicles and two laminae,
the latter joining posteriorly to become the spinous pro-
cess. The transverse process and two articular facets
originate from a mass of bone at the junction of lamina
and pedicle. One articular facet passes superiorly and the
second one inferiorly, to articulate with a facet from the
vertebrae above and below, respectively. The orientation
TABLE 3–1
Articulations of the Spine
A B
Figure 3–19. Atlas and axis: osseous anatomy. A, Posterior view. Observe the posterior arch (pa), posterior
tubercle (pt), superior articular process (sp), inferior articular process (ip), transverse process (tp), and
foramen transversarium (ft) of the atlas, and the odontoid process (op) and superior articular process
(arrowhead) of the axis. B, Frontal radiographs of the atlas and axis with the same structures indicated, as well
as the anterior arch (aa).
Figure 3–20. Atlantoaxial and atlanto-occipital joints. Drawing of coronal (A) and sagittal (B) sections of
the base of the skull and upper cervical spine. Note the lateral atlantoaxial joints (laa), atlanto-occipital
synovial joints (ao), anterior median joint (arrowhead) between the odontoid process and anterior arch of the
atlas, and posterior median joint (arrow) between the odontoid process and transverse ligament (tl) of the
atlas. Additional structures are the anterior longitudinal ligament (all), posterior longitudinal ligament (pll),
membrana tectoria (mt), anterior atlanto-occipital membrane (am), posterior atlanto-occipital membrane
(pm), apical ligament of the odontoid process (al), alar ligament (a), and ligamentum flavum (lf).
Chapter 3 Anatomy of Individual Joints 37
axis, contains a superior peg of bone, the dens or odon- ATLANTO-OCCIPITAL JOINTS
toid process, which possesses a small oval facet for articu-
Osseous Anatomy
lation with a facet on the posterior surface of the anterior
arch of the atlas. On the posterior surface of the odontoid The superior surface of each lateral mass of the atlas has a
process is a groove for the transverse ligament of the atlas. concave, kidney-shaped facet that articulates with the corre-
On lateral radiographs of the cervical spine, the normal sponding occipital condyle. This atlantal facet is oriented
space between the anterior arch of the atlas and the odon- medially and superiorly. The occipital condyles are oval,
toid process of the axis should be no greater than 2.5 mm located on the anterolateral aspect of the foramen magnum.
in adults. In children, the average distance between the
anterior arch of the atlas and the odontoid process is 2
Soft Tissue Anatomy
to 2.5 mm in extension and 2 to 3 mm in flexion. This
distance should not exceed 4.5 mm in children. The atlanto-occipital joints consist of a pair of synovial joints,
between the articular facets of the atlas and the condyles
of the occiput, and syndesmoses formed by the anterior
Soft Tissue Anatomy and posterior atlanto-occipital membranes (see Fig. 3–20).
Four synovial articulations occur between the atlas and The atlanto-occipital synovial joints are the reciprocally
axis: two lateral atlantoaxial joints, one on each side, curved superior articular facets separating the lateral masses
between the inferior facet of the lateral mass of the atlas of the atlas and condyles of the occipital bone. They are
and the superior facet of the axis; and two median syn- surrounded by a fibrous capsule that is particularly thick on
ovial joints, one between the anterior arch of the atlas its posterior and lateral surfaces but deficient medially.
and the odontoid process of the axis and the second The anterior atlanto-occipital membrane is attached above
between the odontoid process and the transverse liga- to the anterior margin of the foramen magnum and below
ment of the atlas (Fig. 3–20). In addition to these synovial to the anterior arch of the atlas. Its central portion contains
articulations, syndesmoses between the atlas and axis fibers continuous with those of the anterior longitudinal
include continuations of the anterior longitudinal liga- ligament. The posterior atlanto-occipital membrane is
ment anteriorly and the ligamenta flava posteriorly. attached above to the posterior margin of the foramen
magnum and below to the posterior arch of the atlas.
Figure 3–21. Cervicobasilar junction: normal osseous relationships. A, Chamberlain’s line is drawn from
the posterior margin of the hard palate to the posterior border of the foramen magnum. The odontoid
process normally does not extend more than 5 mm above this line. B, The bimastoid line (lower line),
connecting the tips of the mastoids, is normally within 2 mm of the odontoid tip. The digastric line (upper
line), connecting the digastric muscle fossae, is normally located above the odontoid process. C, The basilar
angle, which normally exceeds 140 degrees, is formed by the angle of intersection of two lines—one drawn
from the nasion to the tuberculum sellae, and the second drawn from the tuberculum sellae to the anterior
edge of the foramen magnum. D, The atlanto-occipital joint angle, constructed on frontal tomograms by the
intersection of two lines drawn along the axes of these articulations, is normally not greater than 150 degrees.
38 Section I Basic Science
OSSEOUS RELATIONSHIPS OF
CERVICOBASILAR JUNCTION
The osseous relationships at the cervicobasilar junction
have received considerable attention. Chamberlain’s line
can be drawn on a lateral radiograph from the posterior
margin of the hard palate to the posterior border of the
foramen magnum. In normal situations, the odontoid
process should not extend more than 5 mm above this
line. A modification of this line, McGregor’s line, uses
the inferior surface of the occiput rather than the margin
of the foramen magnum. In normal persons, the odon-
toid tip does not extend more than 7 mm above this line.
On frontal radiographs, a line connecting the mastoid
tips is within 2 mm of the tip of the odontoid, whereas a
line connecting the digastric muscle fossae is located at
the approximate level of the foramen magnum and above
the odontoid process (Fig. 3–21).
The basilar angle is the angle of intersection of two
lines—one drawn from the nasion to the tuberculum
sellae, and the second drawn from the tuberculum sellae
to the anterior edge of the foramen magnum. This angle
normally does not exceed 140 degrees. An atlanto-
occipital joint angle is constructed on frontal tomograms
by drawing a line along this articulation on either side.
The angle formed by the intersection of these two lines
normally should not exceed 150 degrees.
Radiographic examination of normal osseous structures neck are useful measurements, particularly in the young
about the hip has received much attention, and various skeleton. The center-edge (CE) angle of Wiberg is an
measurements have been determined. The acetabular indication of acetabular depth (Fig. 3–26). It is the angle
angle, iliac angle, and angle of anteversion of the femoral formed by a perpendicular line through the midportion
of the femoral head and a line from the femoral head
center to the upper outer acetabular margin. The pelvic
radiograph also is useful in outlining certain normal lines
and structures (Fig. 3–27). The ilioischial line is formed
by that portion of the quadrilateral surface of the ilium
that is tangent to the x-ray beam; the iliopubic line is
simply the inner margin of the ilium, which forms a
continuous line with the inner superior aspect of the
pubis. Two columns of bone produce an arch, with the
acetabulum located in the concavity of the arch. The
ilioischial, or posterior, column is a thick structure that
includes a portion of the ilium and extends to the ischial
tuberosity. The iliopubic, or anterior, column consists of
a portion of ilium and pubis and extends superolaterally
to the anterior inferior iliac spine.
The “teardrop” is a U-shaped shadow medial to the
hip joint that has been used to detect abnormalities of
acetabular depth, thereby establishing a diagnosis of
acetabular protrusion (Fig. 3–28). The lateral aspect of
the teardrop is the wall of the acetabular fossa, and the
medial aspect is the anteroinferior margin of the quadri-
lateral surface. The configuration of the teardrop varies
Figure 3–24. Pelvic-vertebral ligaments: posterior aspect. in normal persons, and it is affected significantly by posi-
Observe the iliolumbar ligament (ill), short and long dorsal
sacroiliac ligaments (dsl), sacrotuberous ligament (stl), sacrospinous tioning. Differentiation of normal acetabular depth and
ligament (ssl), and greater (arrowhead) and lesser (arrow) sciatic acetabular protrusion (protrusio acetabuli) can be accom-
foramina. plished by careful analysis of conventional radiographs in
(From Warwick R, Williams P: Gray’s Anatomy, 35th Br ed. adults. Protrusio acetabuli is diagnosed when the acetabu-
Philadelphia, WB Saunders, 1973.) lar line projects medial to the ilioischial line by 3 mm or
40 Section I Basic Science
Figure 3–26. Hip: radiographic indices. A, Center-edge (CE) angle of Wiberg. A horizontal line (C2–C1)
is drawn, connecting the center of each femoral head. A perpendicular line is drawn superiorly from the
center of the femoral head. The edge (E) of the acetabulum is determined, and a line (C1–E) is constructed.
In this case, the value of 26 degrees is considered normal. B, “Horizontal toit externe” (HTE) angle of
Wiberg. A horizontal line is drawn parallel to the C2–C1 line through the most medial portion of the
weight-bearing aspect of the acetabulum (T). A line is then constructed as shown through E. The angle
created between the two lines is normally 10 degrees or less. C, Acetabular index of depth (d) to width (w).
The two lines are constructed as illustrated. The ratio (d/w) × 100 is calculated. This ratio normally meas-
ures greater than 38. D, Percentage of the femoral head covered by the acetabulum. This represents the
relative width of the weight-bearing surface of the acetabulum (A), represented by line 1–2, and that of the
femoral head, represented by line 1–3. Normal acetabular coverage is 75% or above when the ratio of
1–2:1–3 is determined.
(Modified from Delaunay S, Dussault RG, Kaplan PA, et al: Radiographic measurements of dysplastic
adult hips. Skeletal Radiol 26:75, 1997.)
more in men or by 6 mm or more in women. In children, the femoral neck; in front, it is attached to the trochanteric
1 mm in boys and 3 mm in girls are the corresponding line at the junction of the femoral neck and shaft; above
values (Fig. 3–29). and below, it is attached to the femoral neck close to the
junction with the trochanters; behind, the capsule extends
over the medial two-thirds of the neck. Because of these
Soft Tissue Anatomy capsular attachments, the physeal plate of the femur is
The femoral head is covered with articular cartilage, intracapsular, and the physeal plates of the trochanters are
although a small area exists on its surface that is devoid of extracapsular. The fibers of the fibrous capsule, although
cartilage, to which attaches the ligament of the head of oriented longitudinally from pelvis to femur, also consist
the femur, or ligamentum teres. A fibrous capsule encircles of a deeply situated circular group of fibers termed the zona
the joint and much of the femoral neck. The capsule orbicularis. The fibrous capsule is strengthened by surround-
attaches proximally to the acetabulum, labrum, and trans- ing ligaments, including the iliofemoral, pubofemoral,
verse ligament of the acetabulum. Distally, it surrounds and ischiofemoral ligaments. The external surface of the
Chapter 3 Anatomy of Individual Joints 41
Figure 3–27. Normal osseous landmarks of the pelvis: antero- Figure 3–29. Acetabular protrusion. A protrusio acetabuli
posterior view. The posterior acetabular rim (pa) is more lateral deformity is present when the acetabular line (al) projects
than the anterior acetabular rim (aa). The ilioischial line (iil) is medial to the ilioischial line (iil) by 3 mm or more in men and
formed by that portion of the quadrilateral surface of the ilium 6 mm or more in women.
that is tangent to the x-ray beam. The iliopubic line (ipl) is the (From Armbuster TG, Guerra J Jr, Resnick D, et al: The
inner margin of the illum, which forms a continuous line with the adult hip: An anatomic study. Part I. The bony landmarks.
inner superior surface of the pubis. The “teardrop” (t) is labeled. Radiology 128:1, 1978.)
capsule is covered by musculature and separated anteriorly muscle (lateral) and the gluteus minimus muscle (medial);
from the psoas major and iliacus by a bursa. In this area, and fat plane 4 has been termed the “capsular” fat plane,
the joint may communicate with the subtendinous iliac although more recent evidence suggests that this fat pad
bursa (iliopsoas bursa) (Fig. 3–30). is not related to the joint capsule but lies between the
A number of periarticular fat planes have been des- rectus femoris and tensor fasciae latae muscles.
cribed that can be recognized on radiographs and, when The iliopsoas bursa represents the largest and most
disturbed, may indicate significant intra-articular disease. important bursa about the hip. It is present in 98% of
Four fatty layers can be identified on anteroposterior hips and is located anterior to the joint capsule. It may
radiographs (Fig. 3–31): fat plane 1 is medial to the extend proximally and communicates with the joint space
obturator internus muscle; fat plane 2 is medial to the in approximately 15% of normal hips. Extension of hip
iliopsoas muscle; fat plane 3 is between the gluteus medius disease into this bursa has been recognized in a variety of
articular diseases. Bursae about the gluteus muscles also
may be demonstrated anatomically and radiographically.
The bursa deep to the gluteus medius is larger than that
deep to the gluteus minimus. Both bursae are intimate
with the greater trochanter, and bursitis can lead to pain
and soft tissue calcifications in this region.
KNEE
In this joint, three functional spaces exist: the medial
femorotibial space, the lateral femorotibial space, and the
patellofemoral space.
TIBIOFIBULAR JOINTS
Osseous and Soft Tissue Anatomy
Articulations uniting the tibia and the fibula are the prox-
imal (superior) tibiofibular joint (synovial), the crural
interosseous membrane (syndesmosis), and the distal
tibiofibular joint (syndesmosis). These joints allow limited
movement of the fibula with respect to the tibia.
D
Figure 3-33. Patellar position. A , The ratio of patellar tendon length to the greatest diagonal length of
the patella can be used to diagnose patella alta. B, The ratio of the distance between the lower articular
surface of the patella and the tibia1 plateau line to the length of the patellar articular surface has also been
used for this purpose. C, Merchant and coworkers (T Bone Joint Surg Am 56:1391, 1974) suggested that on
an axial radiograph, the line connecting the median ridge of the patella (r) and trochlear depth (2) should
fall medial or slightly lateral to a line ( 0 ) bisecting angle 1-2-3. Here, the first line lies medial to line 0 , a
normal finding. D, Laurin and coworkers proposed other measurements that might be appropriate. The
upper two diagrams reveal the normal situation; the lower two diagrams indicate the abnormal situation. On
axial radiographs, an angle formed between a line connecting the anterior aspect of the femoral condyles (1)
and a second line along the lateral facet of the patella (2) normally opens laterally. In patients with sub-
luxation or abnormal tilting of the patella, these lines are parallel, or the angle of intersection opens medially.
(D, From Laurin CA, Levesque HP, Dussault R, et al: The abnormal lateral patellofemoral angle: A
diagnostic roentgenographic sign of recurrent patellar subluxation. J Bone Joint Surg Am 6055, 1978.)
Chapter 3 Anatomy of Individual Joints 45
the adjacent fibular notch of the tibia. Additionally, the
anterior and posterior tibiofibular ligaments reinforce
this articulation. Below this ligamentous joint, an upward
prolongation of the synovial membrane of the ankle
(talocrural joint) can extend 3 to 5 mm. This synovial
recess may be associated with cartilaginous surfaces on
the tibia and fibula.
Osseous Anatomy
The distal end of the tibia contains the medial malleolus
and articular surface. The broad malleolus has an articu-
lar facet on its lateral surface, which is comma-shaped.
On the posterior surface of the distal end of the tibia is
a groove, just lateral to the medial malleolus, related to
the tendon of the tibialis posterior muscle. The inferior
surface of the tibia represents the articular area for the
talus. It is smooth and wider anteriorly than posteriorly.
Figure 3–34. Diagnosis of a synovial effusion in the knee. In The triangular fibular notch is on the lateral side of the
normal situations, the collapsed suprapatellar pouch creates a tibia. This notch represents the site of attachment of
radiodense area (arrows) that is generally less than 5 mm wide various ligaments that connect the distal portions of the
but may be between 5 and 10 mm wide. tibia and fibula. The distal end of the fibula contains the
Figure 3–35. Ankle joint anatomy. A, Drawing of a coronal section through the distal ends of the tibia,
fibula, and talus, outlining the ankle joint (large arrowheads), interosseous ligament (il) of the tibiofibular
syndesmosis, interosseous talocalcaneal ligament (tcl), portions of the deltoid ligament (dl), posterior
talofibular ligament (tfl), calcaneofibular ligament (cfl), surrounding tendons, subtalar joint (small arrow-
head), and talocalcaneonavicular joint (arrow). B, Some of the same structures in (A) can be identified in a
drawing of a sagittal section of the ankle. Additional articulations are the calcaneocuboid (cc), cuneo-
navicular (cun), and tarsometatarsal (tmt) joints.
46 Section I Basic Science
lateral malleolus. This structure projects more inferiorly Laterally, the common sheath of the peroneus longus and
than the medial malleolus and contains a triangular facet peroneus brevis tendons is seen.
on its medial surface for articulation with the talus and Important tendons, aponeuroses, and bursae are located
an irregular surface above this facet for the interosseous about the calcaneus. The plantar aponeurosis contains
ligament. Posterior to the convex articular facet is a strong fibers that adhere to the posteroinferior surface of
depression, the malleolar fossa. the bone. It has central, medial, and lateral components.
Assessment of alignment of the ankle on radiographs The Achilles tendon, which is the thickest and strongest
is important in the post-trauma evaluation of this joint. human tendon, attaches to the posterior surface of the
Small degrees of lateral displacement of the talus on the calcaneus approximately 2 cm below the upper surface of
tibia may result in the rapid development of secondary the bone. The plantaris tendon is located medial to the
degenerative arthritis. It has been shown that even 1 mm Achilles tendon. The retrocalcaneal bursa exists between
of lateral displacement of the talus reduces the tibiotalar the Achilles tendon and the posterosuperior surface of
contact area by 42%. Incomplete ligament tears may the calcaneus.
result in relatively small amounts of displacement, which On normal radiographs, the Achilles tendon has a
may be difficult to detect radiographically. thickness of 4 to 8 mm at the level of the calcaneus or
In adults, the coronal plane of the ankle is oriented in 1 to 2 cm above the top of the calcaneus (Fig. 3–36). A
about 15 to 20 degrees of external rotation with reference vertical radiolucent area, the retrocalcaneal recess, meas-
to the coronal plane of the knee; therefore, the lateral ures 2 mm or more in length and extends from the pos-
malleolus is slightly posterior to the medial malleolus. To terior aspect of the calcaneus behind the posterior portion
obtain a true anteroposterior film of the tibiotalar articu- of the bone, reflecting fat adjacent to the normal retro-
lation, the ankle must be positioned with the medial and calcaneal bursa (Fig. 3–37). The appearance of the
lateral malleoli parallel to the tabletop—that is, in about retrocalcaneal recess can be influenced by severe dorsi-
15 to 20 degrees of internal rotation, or the mortise flexion or plantar flexion of the foot.
view. This positioning places the medial articular surface
tangent to the x-ray beam, and the short concave line
representing the posteromedial surface of the talus falls
slightly lateral to the medial articular surface.
The axial relationships of the ankle have been des-
cribed. The longitudinal axis of the tibia is perpendi-
cular to the horizontal plane of the ankle joint and is
continuous with the longitudinal axis of the talus. The
tibial angle, the angle formed by the intersection of
one line drawn tangentially to the articular surface of
the medial malleolus and a second line drawn along
the articular surface of the talus, averages 53 degrees
(range, 45 to 61 degrees). The fibular angle, drawn in a
corresponding way using the lateral malleolus rather than
the medial malleolus, averages 52 degrees (range, 45 to
63 degrees).
Figure 4–2. Schematic diagram of the collagen fibril orientation within articular cartilage. The fibrils are
tightly packed near the articular surface in a tangential layer that has been termed the armor plate layer.
Fibrils in the deeper layers become progressively larger as they approach the subchondral bone layer. The
fibrils also are more widely spaced in the deeper layers of cartilage. The change in orientation from
tangential to perpendicular in the deeper layers creates a pattern that has been termed an arcade. The
collagen fibrils anchor into the subchondral bone layer after traversing the calcified cartilage, which is
demarcated by a change in staining properties termed the tidemark line. The anchoring of these fibrils into
bone is analogous to the continuation of ligamentous attachments into bone, termed Sharpey’s fibers.
TABLE 4–1
Structurally and Genetically Distinct Collagens
MACITs: collagens
with transmembrane
domains
XIII A new class of cell adhesion [α1 (XIII)]3 Cell surface molecule with multiple
molecule; widely expressed extracellular triple helix domains and a
in human tissue, including transmembrane segment
cartilage and bone
XVII A new class of cell adhesion [α1 (XVII)]3 Cell surface molecule with multiple
molecule; a hemidesmosomal extracellular triple helix domains and a
component with extracellular transmembrane segment
domains connecting with
basement membrane
Other collagens
VI Intervertebral disc, skin, vessels α1 (VI) α2 (VI) NF; 100-nm beaded microfibrils; disulfide
Most interstitial tissues α1 (VI) bonds between tetramers; arginine–
glycine–aspartic acid sequences
VII Dermoepidermal junction [α1 (VII)]3 NF; long chain
anchoring fibrils produced
by keratinocytes
cytes. Cartilage nutrition derives almost entirely from 1000 amino acids. Therefore, the messenger RNA is
synovial fluid. The pumping action of fluid into and out necessarily one of the largest in the cellular protein
of cartilage during loading and unloading appears to be a synthetic apparatus.
key to this process. Joints that are immobilized suffer
relatively quickly in a number of important respects. The
metabolic activity of cells appears to be affected, as a loss ENZYMES OF ARTICULAR CARTILAGE
of proteoglycan and an increase in water content are soon The continuation of normal function of articular carti-
observed. The normal white, glistening appearance of the lage depends on the special properties of the constituents
cartilage changes to a dull bluish color, and the cartilage of cartilage matrix and the maintenance of their normal
thickness is reduced. How much of this process is due concentration within the matrix. If the normal rate of
to nutritional deficiency and how much is due to an upset degradation is increased or if synthesis of new matrix is
in the stress-dependent metabolic homeostasis is not interfered with, the concentration of the various articular
yet clear. cartilage components will change, and this is manifested
by a particular disease process. The production, release,
and actions of the various enzymes affecting articular
METABOLISM OF ARTICULAR CARTILAGE
cartilage are of primary importance in most articular
It should come as no surprise that articular cartilage—a cartilage diseases. Naturally occurring inhibitors exist in
highly specialized, functionally complex tissue—should the synovial fluid or serum that can modify the actions of
be active metabolically. The specialized functional struc- the matrix-degrading enzymes. Most of these inhibitors,
tures of cartilage—the collagen fabric and the proteoglycan however, are relatively large molecules that cannot reach
pump—require constant maintenance and renewal. By the enzymes in the matrix unless the latter diffuse out of
most measures of cellular activity, cartilage cells are nearly the matrix or until degradation of the matrix causes an
as active metabolically as are other cell types from rela- increase in its normal impermeability to large molecules.
tively avascular tissue sources. The enzymes active on articular cartilage matrix can be
Chondrocytes use anaerobic pathways for the most divided into endogenous enzymes peculiar to the carti-
part, a choice well suited to the relative isolation of the lage itself and exogenous enzymes arising in the syn-
cells, their lack of cell-to-cell contact, and their remote- ovium, polymorphonuclear leukocytes, macrophages, or
ness from capillary beds. Proteoglycan aggregation and blood serum.
collagen crosslinking both occur outside the cell. In this
respect, the chondrocyte is able to avoid the troublesome
problem of exporting and properly locating molecules Endogenous Enzymes
that are much too large to diffuse through cartilage Lysosomes are intracytoplasmic vacuoles enclosed by a
matrix. As it is, proteoglycan and procollagen are among lipoprotein membrane; they contain a number of enzymes
the largest molecules that mammalian cells are called on responsible for digestion of the proteoglycan and collagen
to synthesize. Each chain of procollagen has more than molecules of articular cartilage matrix. Chondrocytes may
54 Section I Basic Science
be stimulated to release the lysosomal enzymes to the cells. Enzymes in this category include protease, colla-
exterior of the cell, where initial degradation of the macro- genase, and hyaluronidase. In addition, in pathologic
molecules occurs. Subsequently, diffusion of products situations, when polymorphonucleocytes and macrophages
from the matrix for intracellular digestion in the second- reach the joint cavity, they may secrete a collagenase,
ary or digestive lysosomes occurs. In this group of enzymes lysozyme, or neutral protease, the last being distinguished
are the cathepsins, which have maximal activity at acid from the proteases occurring in the synovial membrane.
pH and little or no activity at the neutral pH of the Collagenases have been implicated in several types of
cartilage matrix. arthritis and have been detected in the synovium in patients
with rheumatoid arthritis and nonspecific inflammatory
synovial conditions. All synovial tissues have the capacity
Exogenous Enzymes to produce collagenase; however, it is likely that only in
The exogenous enzymes affecting articular cartilage states of chronic proliferative synovitis and inflammation
matrix may arise from the synovium of the joint and be is collagenase produced in sufficient quantities to bring
secreted into the synovial fluid from lysosomes of lining about the destruction of articular tissue. Although type II
Chapter 4 Articular Cartilage: Morphology, Physiology, and Function 55
although adult joint cartilage has some potential for repair,
a given insult usually results in a degenerative lesion
mediated by endogenous or exogenous enzymes.
Osteoarthritis
The cause of articular cartilage destruction in osteoarthri-
tis may be mechanical, but part of the matrix degradation
is likely due to enzymatic action. An increase in the
activity of enzymes involved in the normal turnover of
the matrix may occur, or the degradation may be due to
the production and release of special enzymes. The fact
that secondary lysosomes are rare in normal articular
cartilage but are found readily in the chondrocytes of
osteoarthritic cartilage adds weight to this theory. The
degradation process is mainly extracellular, but it may be
completed within the cell. Several endogenous enzymes
have been implicated in osteoarthritis. Although a colla-
genase capable of splitting the collagen molecule has
not been found in normal cartilage, it is present in
osteoarthritic human cartilage and is believed to be
extralysosomal in origin. It probably is present in minute
quantities, and its demonstration indicates prior removal
of an inhibitor. Collagenase does occur in the synovial
tissue of joints affected by osteoarthritis and has been
produced by synovial explants from joints affected by
osteoarthritis, but in lesser amounts than in rheumatoid
arthritis. In severe osteoarthritis, however, total loss of
articular cartilage occurs. Lysozyme also has been impli-
cated in osteoarthritic lesions by the finding of significant
elevations of the enzyme in hip and knee joint cartilage
affected by osteoarthritis. Various cytokines likewise are
important in the development of osteoarthritis.
One additional aspect of the effect of enzymes in the
osteoarthritic process is the nature of the matrix synthesis
Figure 4–7. Disaccharide configuration of the principal gly- response of chondrocytes to lysosomal enzymes. Although
cosaminoglycans of the proteoglycan constituents of articular the collagen content of osteoarthritic cartilage does not
cartilage. A, Molecular configuration of chondroitin 4-sulfate.
change, the cartilage synthesizes type I collagen in addi-
This differs from chondroitin 6-sulfate (B) only in the location
of the sulfate group on the hexosamine molecule. Both con- tion to the usual type II collagen. Thus, the enzymes alter
tain alternating glucuronic acid and galactosamine sugars. B, the function of chondrocytes, causing them to synthesize
Chondroitin 6-sulfate. C, Hyaluronan contains alternating nonspecific collagen molecules, which might lead to
molecules of glucosamine and glucuronic acid but lacks a weakening of the mechanical structure of the cartilage
sulfate group. D, Keratan sulfate contains galactose rather than and its subsequent mechanical destruction.
a uronic acid moiety in the disaccharide. The hexosamine is
glucosamine, which is sulfated in the C6 position.
Rheumatoid Arthritis
Rheumatoid arthritis is characterized by the destruction
of articular cartilage and comprises three interrelated
collagen is a poorer substrate for collagenase than is processes: inflammation, synovial proliferation, and tissue
interstitial type I collagen, cleavage of the triple helix of destruction. An autoimmune stimulus to the synovial
collagen can be augmented significantly by raising the lining cells is involved in this disease and causes inflam-
temperature above the normal range of 33°C to 36°C. mation, hyperplasia, and hypertrophy of the cells. The
Thus, in an inflamed joint affected by rheumatoid arthritis resulting inflammatory rheumatoid tissue, or pannus,
in which the joint temperature is raised, the rate of car- growing over and under the articular cartilage is thought
tilage degradation by collagenase would be increased. to release greatly increased amounts of intracellular
lysosomal enzymes, which irreversibly destroy proteo-
glycan and collagen to produce the erosive focal lesion
ENZYMES IN ARTICULAR CARTILAGE
in the cartilage. Other cellular sources for lysosomal
DISEASE PROCESSES
enzyme release are the polymorphonuclear leukocytes
The avascular nature of adult cartilage has a bearing on and lymphocyte clusters that are present in large num-
its limited capacity to respond to various insults, whether bers in the inflamed synovial cavity and in the synovium,
inflammatory, mechanical, or biomechanical. Thus, respectively, in rheumatoid arthritis. In addition, the levels
56 Section I Basic Science
of lysozyme in serum and synovial fluid are increased Brandt KD, Doherty M, Lohmander LS: Osteoarthritis. Oxford,
in rheumatoid arthritis and may accompany the loss of Oxford University Press, 1998.
cartilage glycosaminoglycans seen in this disease. In Curtiss PH Jr, Klein L: Destruction of articular cartilage in
contradistinction, in the arthritis of systemic lupus erythe- septic arthritis. J Bone Joint Surg Am 45:797, 1963.
Dingle JT: The role of lysosomal enzymes in skeletal tissues.
matosus, in which usually no cartilage destruction occurs,
J Bone Joint Surg Br 55:87, 1973.
normal serum lysozyme levels are seen. The fact that Eyre DR, Wu JJ, Woods P: Cartilage-specific collagens: Struc-
continued inflammation hastens cartilage lysis implies tural studies. In Kuettner K, et al (eds): Articular Cartilage
that treatment should involve the suppression of inflam- and Osteoarthritis. New York, Raven Press, 1992, p 119.
mation to reduce lysosomal protease accumulation and Feldmann M, Brennan FM, Maini RN: Role of cytokines in
synovial collagenase production. rheumatoid arthritis. Annu Rev Immunol 14:397, 1996.
Glimcher MJ, Krane SM: The organization and structure of
bone, and the mechanism of calcification. In Gould BS (ed):
Pyogenic Arthritis Treatise on Collagen. Vol 2, Biology of Collagen. New York,
The result of untreated pyogenic arthritis is a severe and Academic Press, 1968, p 67.
Keller K, Shortkroff S, Sledge CB, et al: Effects of rheumatoid
rapid destruction of joint cartilage, which invariably evolves
synovial cells on cartilage degradation in vitro. J Orthop Res
into an osteoarthritic process and possibly leads to fibrous 8:345, 1990.
or bony ankylosis. The extensive proteoglycan degrada- Lazarus GS, Daniels JR, Brown RS, et al: Degradation of
tion of cartilage is due to the action of several proteinases, collagen by a human granulocyte collagenolytic system.
which may be derived from neutrophil leukocytes. The J Clin Invest 47:2622, 1968.
leukocytes can release their lysosomal enzymes on Lotke PA: Diffusion in cartilage. In Simon WH (ed): The
encountering bacteria or after the eventual death of the Human Joint in Health and Disease. Philadelphia, Univer-
leukocytes. The proteinases incriminated are elastase, sity of Pennsylvania Press, 1978.
cathepsin G, and collagenase, all of which are active at Sajdera SW, Hascall VC: Protein-polysaccharide complex from
neutral pH. In the body, various inhibitors are secreted bovine nasal cartilage: A comparison of low and high shear
extraction procedures. J Biol Chem 244:77, 1969.
into the synovial fluid that can prevent the extracellular
Seyer J, Kang A: Connective tissues of the subendothelium. In
activity of the above-mentioned enzymes. It seems Creager M, Dzag V (eds): Vascular Medicine. Boston, Little,
logical, however, that in the treatment of septic joints, Brown, 1996, p 39.
removal of polymorphonuclear leukocytes by operation Sternlicht MD, Werb Z: ECM proteinases. In Kreis T, Vale R
or aspiration is essential to minimize cartilage destruction. (eds): Extracellular Matrix, Anchor and Adhesion Proteins,
2nd ed. Oxford, Oxford University Press, 1999, p 505.
Vuorio E, deCrombruggle B: The family of collagen genes.
Annu Rev Biochem 59:837, 1990.
FURTHER READING Weiss C, Rosenberg L, Helfet AJ: An ultrastructural study of
normal young adult human articular cartilage. J Bone Joint
Arend WP, Dayer JM: Inhibition of the production and effects Surg Am 50:663, 1968.
of interleukin-1 and tumor necrosis factor alpha in Weissmann G: Lysosomal mechanisms of tissue injury in
rheumatoid arthritis. Arthritis Rheum 38:151, 1995. arthritis. Semin Med Beth Israel Hosp 286:141, 1972.
Barrett AJ: The possible role of neutrophil proteinases in Weissmann G, Spilberg I: Breakdown of cartilage protein
damage to articular cartilage. Agents Actions 8:11, 1978. polysaccharide by lysosomes. Arthritis Rheum 11:162, 1968.
Benninghoff A: Form und Bau der Gelenkknorpel in ihren Ziff M, Gribetz HJ, Lospalluto J: Effect of leukocyte and
Beziehungen zur Funktion. Z Anat Entwicklungsgesch synovial membrane extracts on cartilage mucoprotein. J Clin
76:43, 1925. Invest 39:405, 1960.
SECTION
II
Diagnostic Techniques
CHAPTER 5
Arthrography, Tenography, TABLE 5–1
Evaluation of:
Presence and extent of synovial inflammation
SUMMARY OF KEY FEATURES Injuries to the triangular fibrocartilage, interosseous
ligaments, and joint capsule
Although contrast opacification of joint cavities Soft tissue masses
(arthrography), tendon sheaths (tenography), and bursae
(bursography) has been used less frequently in recent
years, owing to increased reliance on magnetic carpal compartment is the site of injection, although
resonance (MR) imaging, such opacification still has other compartments of the wrist may be studied (see later
important clinical applications. discussion). The needle is guided under the radial lip,
Arthrographic procedures generally are simple to entering the radiocarpal compartment between the
perform, and the information they provide may be scaphoid and the radius. Approximately 2 mL of contrast
essential for proper diagnosis and treatment. The agent is administered. Fluoroscopic monitoring com-
technique is used alone or in combination with other bined with sequential spot filming, videotaping, or digital
technologic methods, such as fluoroscopy, conventional technique during the injection of contrast material allows
and computed tomography (arthrotomography), and more precise delineation of sites of abnormal compart-
digital radiography. Normal and abnormal arthrographic ment communication. Selective injection of the midcarpal
findings at specific locations in the body are described. compartment, rather than the radiocarpal compartment,
Arthrographic abnormalities in patients with joint has been advocated as a superior method for analysis of
prostheses are described in Chapter 14. the scapholunate and lunotriquetral ligaments. Indeed,
some authorities recommend sequential injections into
the radiocarpal, midcarpal, and inferior radioulnar joints
as ideal.
INTRODUCTION
Arthrography is applicable in the evaluation of many Normal Wrist Arthrogram
different joints and can be coupled with fluoroscopy, con- Contrast opacification of the radiocarpal compartment
ventional and computed tomography, digital radiography, reveals a concave sac with smooth synovial surfaces
and MR imaging. Various radiopaque and radiolucent extending between the distal end of the radius and
(air or carbon dioxide) contrast media exist that can be proximal carpal row (Fig. 5–1). The prestyloid recess
used separately as a single-contrast technique or com- appears as a finger-like projection that approaches the
bined as a double-contrast technique. The administration ulnar styloid process. Volar radial recesses are located
of epinephrine also increases the quality of arthrograms beneath the distal end of the radius. If the midcarpal joint
by reducing simultaneously the egress of contrast mate- is injected, contrast material normally extends proximally
rial from the joint and the influx of body fluid through between the scaphoid and lunate and between the lunate
the synovial membrane into the joint. Application of stress and triquetrum to the level of the scapholunate and
is a useful technique in the arthrographic evaluation of lunotriquetral interosseous ligaments. It extends distally
ligamentous injuries of the ankle, wrist, and first metacar- into the common carpometacarpal and intermetacarpal
pophalangeal joint. compartments. If the distal radioulnar joint is injected,
As performed currently, arthrography is a safe and contrast material sits like a cap on the articular surface
well-tolerated procedure. Hypersensitivity reaction to of the ulna. Communication between the radiocarpal
iodinated contrast agent is rarely observed. compartment and other compartments in the wrist
during arthrography may be observed in normal persons.
The prevalence of such communication increases in
WRIST
older persons, and it may be seen in as many as 50% of
Several indications exist for wrist arthrography (Table 5–1). asymptomatic persons.
Rheumatoid Arthritis
Synovial inflammation with hypertrophy and villous
transformation accounts for an irregular outline of con-
trast material that may be seen in rheumatoid arthritis
(Fig. 5–6). Lymphatic visualization is common, and
capsular distention, sacculation, and cystic swelling may
be observed.
Nodular filling defects within the contrast-filled elbow
joint may represent hypertrophied synovium, as in rheu-
matoid arthritis, or synovial masses associated with pig-
mented villonodular synovitis and idiopathic synovial
(osteo)chondromatosis (Fig. 5–7).
Trauma
Opacification of the traumatized elbow joint, particularly
after the introduction of air or air and contrast material,
may reveal cartilaginous and osseous defects associated
with osteochondritis dissecans. Contrast medium may
dissect beneath the adjacent osseous fragment or reveal
loose or embedded bodies elsewhere in the joint cavity.
Figure 5–3. Wrist arthrography: injury of the triangular Computed arthrotomography also may be used (Fig. 5–8).
fibrocartilage. This young man had pain over the distal end of
the ulna after an injury. A radiocarpal joint arthrogram reveals
communication between the radiocarpal compartment (1) and
SHOULDER
inferior radioulnar compartment (2). The midcarpal (6) and Arthrography of the glenohumeral joint (Table 5–3) is an
common carpometacarpal (7) compartments are also opacified. aid to the diagnosis of rotator cuff tear, adhesive capsulitis,
Small contrast-filled diverticula near the proximal aspect of the previous dislocation, articular disease, and bicipital tendon
inferior radioulnar joint (arrowhead) may indicate a capsular abnormalities.
tear.
Technique
Both single-contrast and double-contrast examinations
have been advocated for glenohumeral joint arthrography.
Normal Elbow Arthrogram
Conventional and computed arthrotomography may be
On frontal radiographs, a thin layer of contrast material necessary in certain situations.
or air is observed between the humerus, radius, and ulna.
A periradial recess is apparent about the proximal portion Single-Contrast Examination. Ten to 15 mL of iodinated
of the radius, which is indented where the annular liga- contrast material is injected into the glenohumeral joint
ment surrounds the bone. Proximal extension of contrast from an anterior approach. Anteroposterior radiographs
material along the anterior surface of the humerus may are obtained in internal and external rotation, and
resemble the ears of a rabbit, the “Bugs Bunny” sign axillary and tangential bicipital groove radiographs are
(Fig. 5–5). On a lateral radiograph, the periradial or also obtained. This series of radiographs is repeated after
annular recess again is apparent. In addition, coronoid moderate exercise of the shoulder.
TABLE 5–3
TABLE 5–2
Indications for Glenohumeral Joint Arthrography
Indications for Elbow Arthrography
Evaluation of:
Evaluation of: Rotator cuff tears
Presence and extent of synovial inflammation Adhesive capsulitis
Intra-articular cartilaginous and osseous bodies Bicipital tendon abnormalities
Soft tissue masses Previous dislocations
Trauma in children Presence and extent of synovial inflammation
60 Section II Diagnostic Techniques
A B
Figure 5–4. Wrist arthrography: injuries of the scaphoid and interosseous ligaments of the proximal
carpal row. A, Scapholunate dissociation with disruption of the scapholunate ligament. After radiocarpal
joint arthrography, contrast material has flowed from the radiocarpal compartment (1) into the midcarpal
(6) and common carpometacarpal (7) compartments. Contrast material overlies the scapholunate space.
B, Scapholunate dissociation with disruption of the scapholunate ligament, lunotriquetral ligament, triangular
fibrocartilage, and ulnar capsule in a different patient. Computed tomography following radiocarpal joint
arthrography shows that contrast material has flowed through the scapholunate ligament (1), lunotriquetral
ligament (2), and triangular fibrocartilage (3). Contrast also extends through the ulnar capsule (4).
Figure 5–5. Elbow arthrography: normal arthrogram. A, Anteroposterior radiograph. Observe the thin
layer of contrast material between the humerus and ulna; the proximal extension of material in front of the
humerus, resembling the ears of a rabbit (arrowheads); and the periradial, or annular, recess (arrow). B, Lateral
radiograph. Note the periradial, or annular, recess (arrow); the coronoid, or anterior, recess (open arrow); and
the olecranon, or posterior, recess (arrowhead).
Chapter 5 Arthrography, Tenography, and Bursography 61
Figure 5–6. Elbow arthrography: rheumatoid arthritis. Figure 5–7. Elbow arthrography: idiopathic synovial (osteo)-
Arthrogram from a 50-year-old man with rheumatoid arthritis chondromatosis. A lateral view after arthrography delineates
and a periarticular mass caused by a synovial cyst. The arthro- irregular nodular filling defects (arrowheads) that represent
gram outlines distal cystic dilatation of the articular cavity with cartilaginous foci resulting from synovial metaplasia.
irregular synovium (arrows).
(From Ehrlich GE: Antecubital cysts in rheumatoid
arthritis—a corollary to popliteal [Baker’s] cysts. J Bone Joint
Surg Am 54:165, 1972.) rial should not overlie the surgical neck of the humerus.
The tangential view of the bicipital groove demonstrates
an oval filling defect within the contrast-filled sheath,
representing the biceps tendon.
Double-Contrast Examination. Double-contrast shoulder
arthrography employs 1 to 4 mL of iodinated contrast
agent and 10 to 15 mL of air. The patient then sits Complete and Incomplete Tears of the
upright with a 2.3-kg (5-lb) sandbag in his or her hand. Rotator Cuff
Radiographs in internal and external rotation are Tears in the rotator cuff musculature may involve the
obtained with or without a spot film device. The patient entire thickness of the cuff (complete tear) or a portion of
returns to the supine position, and internal rotation, the cuff (incomplete or partial tear).
external rotation, axillary, and bicipital groove films are
made. These radiographs can be repeated after mild Complete Tear. Abnormal communication exists between
exercise of the shoulder. With this technique, the width the glenohumeral joint cavity and the subacromial (sub-
of the rotator cuff tear and the integrity of cuff tendons deltoid) bursa (Fig. 5–11). Contrast material can be iden-
can be assessed. In addition, the internal structures of the tified within the bursa as a large collection superior and
joint, including the glenoid labrum, are better identified. lateral to the greater tuberosity and adjacent to the under-
surface of the acromion. Using double-contrast shoulder
Normal Glenohumeral Joint Arthrogram arthrography, the degree of degeneration of the torn
rotator cuff can be recognized. Further, the width of the
Contrast material is identified between the humeral head tear itself is identified.
and the glenoid (Figs. 5–9 and 5–10). On radiographs
taken in external rotation, the contrast substance ends Incomplete Tear. A partial tear may involve the deep sur-
abruptly laterally at the anatomic neck of the humerus. In face of the rotator cuff, the superficial surface, or the
this view, an axillary pouch may be opacified on the interior substance of the tendon (Fig. 5–12). Tears within
undersurface of the humeral head. In internal rotation, a the substance of the cuff or involving the superior surface
prominent subscapular recess is observed overlying the of the cuff are not demonstrated on glenohumeral joint
glenoid and lateral scapular region. The tendon of the arthrography. Tears on the inferior surface of the rotator
long head of the biceps is visible as a radiolucent filling cuff can be diagnosed on arthrography as an irregular
defect within the articular cavity and can be traced for a circular or linear collection of contrast material above the
variable distance within the contrast-filled tendon sheath opacified joint cavity.
into the bicipital groove and along the metaphysis of the
humerus. In the axillary view, contrast material is iden-
Adhesive Capsulitis
tified between the glenoid cavity and humeral head, ante-
rior to the scapula (subscapular recess), and within the Glenohumeral joint arthrography has been used in the
bicipital tendon sheath. In this projection, contrast mate- diagnosis and treatment of adhesive capsulitis. The main
62 Section II Diagnostic Techniques
A B
arthrographic abnormality in this disorder is a joint of dislocation of the tendon and sheath from their normal
low capacity evidenced by increased resistance to injec- positions in the intertubercular groove can be suspected
tion and a “tight” feel. Only a small amount of fluid (5 to when the positions of these structures do not change on
8 mL) can be injected successfully. The subscapular and the internal and external rotation radiographs.
axillary recesses are small or absent, and lymphatic filling
may be evident (Fig. 5–13).
Joint distention during arthrography, the “brisement” Abnormalities Occurring after Previous
procedure, may aid in the treatment of this condition. Dislocations
This technique requires slow, intermittent injection of Anterior dislocations of the glenohumeral joint are asso-
larger and larger volumes of contrast material (mixed ciated with soft tissue damage. As the dislocating humeral
with saline solution, corticosteroids, and lidocaine). head moves anteriorly, it lifts the articular capsule from
the glenoid and neck of the scapula, producing an abnormal
recess between the subscapular and axillary recesses. On
Abnormalities of the Bicipital Tendon
arthrography, the abnormal recess fills with contrast mate-
Considering the wide variation in the arthrographic rial, a finding that is more evident on radiographs taken
appearance of the bicipital tendon and sheath in normal in internal rotation (Fig. 5–14). Additional findings rela-
persons, the radiologist must not rely too heavily on the ted to anterior dislocation are injuries of cartilage and bone.
arthrogram in establishing the existence of a significant The Bankart deformity involves an avulsion or compres-
abnormality. The arthrographic diagnosis of complete sion defect of the anteroinferior rim of the glenoid and
rupture of the bicipital tendon is based on failure to may be purely cartilaginous. The arthrogram may outline
identify the tendon in the opacified sheath. Incomplete the cartilaginous abnormalities about the glenoid labrum.
tears of the bicipital tendon produce increased width of A Hill-Sachs compression deformity on the postero-
the tendon and distortion of the synovial sheath. Medial lateral aspect of the humeral head also may be detected.
Chapter 5 Arthrography, Tenography, and Bursography 63
A B
TABLE 5–4
Indications for Hip Arthrography
Evaluation of:
Developmental dysplasia of the hip
Septic arthritis and osteomyelitis with epiphyseal separation
Epiphyseal dysplasia and osteonecrosis
Certain synovial disorders
Soft tissue masses
Trauma
Legg-Calvé-Perthes Disease
Figure 5–10. Glenohumeral joint arthrography: normal
double-contrast arthrogram (upright projections). A, Normal Hip arthrography may be of benefit in evaluating patients
arthrogram: external rotation. Visualized structures include the with Legg-Calvé-Perthes disease. This method allows
axillary pouch (1), bicipital tendon (3), glenoid fibrocartilage identification of the true position of the cartilaginous
(arrow), and articular cartilage of the humeral head. The dis- head; this information may allow the surgeon to deter-
tended articular cavity (arrowhead) above the bicipital tendon mine which position of the hip will be best during treat-
should not be misinterpreted as filling of the subacromial ment of the condition (Fig. 5–17). MR imaging also can
(subdeltoid) bursa. B, Normal arthrogram: internal rotation. be used in the assessment of hip involvement in Legg-
Visualized structures include the subscapular recess (2), axillary
Calvé-Perthes disease.
pouch (1), bicipital tendon (3), glenoid fibrocartilage (arrow),
and articular cartilage of the humeral head.
Trauma
In patients with dislocations of the femoral head, hip
athrography alone or in combination with CT may out-
line distortion of or defects in the joint capsule, tears of the
ligamentum teres, and intra-articular osteocartilaginous
HIP bodies. Capsular constriction (adhesive capsulitis) may
Hip arthrography may be employed in patients with con- be seen following injury, although it may be idiopathic.
genital, traumatic, and articular disorders (Table 5–4). Accurate diagnosis is accomplished with arthrography, during
which a low capacity of the joint cavity is demonstrated.
Tears and deformities of the acetabular labrum have
Technique been recorded after trauma. Ganglion cyst formation in
Many techniques exist for puncturing the hip joint. The the labrum also has been identified. Routine radiography
author uses an anterior approach in performing hip may reveal gas-containing soft tissue masses, and during
arthrography, with the patient placed supine on the table. arthrography, contrast material may extend from the joint
In infants and children, an anterolateral subphyseal plate into the labral tear and ganglion.
Chapter 5 Arthrography, Tenography, and Bursography 65
Figure 5–11. Glenohumeral joint arthrography: complete rotator
cuff tear. A, Arthrographic findings of a complete tear of the rotator
cuff. Contrast material extends from the glenohumeral joint through
the rotator cuff into the subacromial (subdeltoid) bursa. The inset
reveals contrast material extending from the glenohumeral joint
through the rotator cuff into the subacromial bursa and from there
into the acromioclavicular joint. B and C, Double-contrast arthro-
graphy. The external rotation view (B) demonstrates that contrast
material has extended from the glenohumeral joint into the sub-
acromial (subdeltoid) bursa (thin arrows). The width of the tear of the
rotator cuff can be seen (between heavy arrows). In another patient
with a rotator cuff tear, an axillary view (C) reveals a “saddlebag”
configuration, with contrast material overlying the surgical neck of
the humerus (arrowheads).
(B, Courtesy of J. Mink, MD, Los Angeles, California.)
B C
Articular Disorders
In idiopathic synovial (osteo)chondromatosis or pig- iliopsoas bursa may accompany intra-articular diseases
mented villonodular synovitis, the extent of synovial such as osteoarthritis, rheumatoid arthritis, pigmented
and capsular abnormality can be determined with villonodular synovitis, infection, calcium pyrophosphate
arthrography (Fig. 5–18). In patients with septic arthritis, dihydrate crystal deposition disease, and idiopathic
hip arthrography provides a technique for aspiration and synovial (osteo)chondromatosis, producing a mass in the
subsequent culture, as well as a means of evaluating carti- ilioinguinal region that may stimulate a hernia and cause
laginous, osseous, and snyovial abnormalities. obstruction of the femoral vein.
B
Figure 5–12. Glenohumeral joint arthrography. A, Incom-
plete rotator cuff tear. Internal rotation view shows contrast
material (arrowhead) extending into the undersurface of the
rotator cuff in this single-contrast arthrogram. B, Reformatted
oblique coronal computed tomography image in a different
patient shows a thickened supraspinatus tendon with contrast
extending into the substance of the tendon (short black arrow),
compatible with an incomplete tear. Note contrast extending
through the distal supraspinatus at its distal attachment (short
white arrow), compatible with a small full-thickness tear. Contrast
is seen in the subacromial (subdeltoid) bursa (long black arrow).
Note also contrast extending into the superior labrum (long
white arrow), compatible with a superior labral tear.
Technique
After puncture of the joint and aspiration of joint con-
tents, 2 to 5 mL of contrast material and 30 mL of air
are injected. The patient then exercises moderately and is
placed beneath the fluoroscopic unit. Nine to 18 expo-
sures are made of each meniscus, using slight changes in
position and appropriate leg traction. After fluoroscopy, Figure 5–14. Glenohumeral joint arthrography. In a patient
overhead or spot films are taken to evaluate the articular with previous anterior dislocations of the glenohumeral joint,
cartilage and cruciate ligaments and to determine whether arthrography demonstrates an abnormal recess (arrow) between
a popliteal cyst is present. The key to the success of the the axillary pouch and subscapular recess and an intra-articular
fluoroscopic technique of knee arthrography is the exam- body (arrowhead).
Chapter 5 Arthrography, Tenography, and Bursography 67
A B
TABLE 5–5 ination of all parts of both menisci. The addition of intra-
articular epinephrine (0.2 mL of 1:1000 solution) may
Indications for Knee Arthrography enhance meniscal visualization by causing vasoconstric-
tion of synovial vessels, decreasing both the amount of
Evaluation of: contrast material absorbed from the joint cavity and the
Meniscal tears, cysts, and ossicles amount of intra-articular fluid formed.
Discoid menisci
Postmeniscectomy syndromes
Ligamentous injuries Normal Knee Arthrogram
Transchondral fractures
Chondromalacia patellae The medial meniscus is identified as a sharply defined
Degenerative joint disease soft tissue triangular shadow (Fig. 5–20). Its posterior
Intra-articular osseous and cartilaginous bodies horn is usually large, the midportion is somewhat smaller,
Synovial disorders and the anterior horn is usually the smallest portion. The
Blount’s disease peripheral surface of the medial meniscus is firmly attached
to the medial collateral ligament. A superior recess fre-
68 Section II Diagnostic Techniques
Figure 5–17. Hip arthrography: Legg-Calvé-Perthes disease. A, On the initial film, epiphyseal frag-
mentation and metaphyseal irregularity are apparent. B, An arthrographic image in hip abduction indicates
a relatively smooth radiolucent cartilaginous head (arrowhead) that is well covered by the acetabulum.
Ligamentous Injury
Collateral Ligament Tears. Recent tears of the collateral
ligaments may be documented by arthrography. Contrast
material introduced into the joint space extravasates into
the adjacent soft tissues. This finding is more readily
apparent on the medial aspect of the knee (Fig. 5–25).
Synovial Cysts
Synovial cysts about the knee are most frequent in the
popliteal region, where communication between the joint
and normal posterior bursae can be identified. The bursa
involved most commonly is the gastrocnemiosemimem-
branosus bursa, located posterior to the medial femoral
condyle between the tendons of the gastrocnemius and
TABLE 5–6
Causes of Multiple Filling Defects on Knee Arthrograms
Rheumatoid arthritis
Pigmented villonodular synovitis
Idiopathic synovial (osteo)chondromatosis
Hemangioma, angioma
Figure 5–28. Knee arthrography: suprapatellar plica Lipoma arborescens
(arrows).
Chapter 5 Arthrography, Tenography, and Bursography 73
A B
Figure 5–30. Knee arthrography: degenerative joint disease. A, In the medial compartment, findings
include severe denudation of articular cartilage on both the femur and the tibia (arrowheads). The medial
meniscus is swollen, with an incomplete vertical tear (arrow) and an irregular inner contour. B, Reformatted
coronal computed tomography image in a different patient after arthrography shows a flap tear of the medial
femoral condyle (arrow).
Figure 5–31. Knee arthrography: rheumatoid arthritis. Nodular Figure 5–32. Knee arthrography: diffuse pigmented villo-
and linear irregularity and pooling of contrast material reflect nodular synovitis. Observe the irregular distribution and
synovial hypertrophy. appearance of contrast material (arrows) in the suprapatellar
pouch.
(From Dalinka MK, Coren GS, Wershba M: Knee
semimembranosus muscles. Swelling of this posterior arthroscopy. CRC Crit Rev Radiol Nucl Med 4:1, 1973.)
bursa is termed a Baker’s cyst. Rupture of such a cyst is
associated with soft tissue extravasation of fluid contents.
Ruptures occurring posteriorly can simulate a compart- also recommended sonographic or isotopic examination
ment syndrome or thrombophlebitis. in this clinical situation. MR imaging of such cysts may
Arthrography of the knee is an accurate method of provide the most detailed information regarding the distri-
diagnosing synovial cysts, although some investigators bution and extent of the process and the degree of synovial
74 Section II Diagnostic Techniques
Figure 5–35. Knee arthrography: normal arthrogram. Anteroposterior (A) and lateral (B) views. The
tibiotalar joint has been opacified. Note the normal recesses: anterior recess (1), posterior recess (2), and
syndesmotic recess (3). Filling of the medial tendon sheaths (T) and posterior subtalar joint (arrowhead) is a
normal finding.
(Fig. 5–35). The posterior subtalar joint is opacified in of the specific ligament. Further, stress radiography may
approximately 10% of patients. indicate ligament weakening by revealing abnormal
widening of the joint. Evidence also indicates a potential
role for MR imaging in the identification of sites of
Ligamentous Injuries ligamentous (and tendinous) injury about the ankle (see
Anterior Talofibular Ligament Injury. The anterior talofibu- Chapter 59).
lar ligament extends from the anterior surface of the
distal portion of the fibula to the talar neck. With tears, Other Traumatic Disorders
contrast material is seen both inferior and lateral to the
distal end of the fibula (Fig. 5–36). Transchondral Fracture. Osteochondral fractures (osteo-
chondritis dissecans) of the talar dome are not infrequent.
Calcaneofibular Ligament Injury. The calcaneofibular Arthrography in this situation outlines the integrity of
ligament originates from the posterior aspect of the distal the overlying cartilage and the presence of intra-articular
portion of the fibula and inserts on the superior aspect of cartilaginous bodies.
the calcaneus. When this strong ligament is torn, contrast
material fills the peroneal tendon sheaths as the inner Adhesive Capsulitis. Post-traumatic adhesive capsulitis
aspect of the sheaths is also torn (see Fig. 5–36). in the ankle is associated with restricted motion. Arthrogra-
phy delineates a decrease in the articular capacity, with
Distal Anterior Tibiofibular Ligament Injury. This structure resistance to injection of contrast material, obliteration
extends from the anterior and lateral aspects of the distal of normal anterior and posterior recesses or tibiofibular
portion of the tibia to the adjacent anterior portion of the syndesmosis, opacification of lymphatic vessels, and
distal fibular end. After injury to this ligament, extrava- extravasation of contrast material along the needle track.
sation of contrast material occurs between distal tibia and
fibula, beyond the syndesmotic recess. APOPHYSEAL JOINTS
Deltoid Ligament Injury. The deltoid ligament originates The injection of contrast material, anesthetic agent, cor-
from the medial malleolus and extends to the talus and ticosteroid preparation, or any combination of the three
calcaneus. With tears of the deltoid ligament, contrast into the apophyseal joints of the lumbar spine has been
material extravasates beyond the medial confines of the advocated in the diagnosis and treatment of the facet
joint. syndrome. This syndrome leads to pain, which is exacer-
Any of these ankle injuries may be associated with bated with rotary motion, in the lower back, thighs,
abnormalities on plain films, including soft tissue swelling buttocks, and legs and to focal tenderness over the affect-
and avulsion fractures at the osseous sites of attachment ed joint. The intrinsically curved lumbar apophyseal joints
76 Section II Diagnostic Techniques
Figure 5–36. Ankle arthrography: ligamentous injuries. A, Anterior talofibular ligament injury. Contrast
material is located inferior and lateral to the tip of the fibula (arrowheads). On a lateral view (not shown), the
contrast material would be anterior to the distal portion of the fibula. B, Anterior talofibular and
calcaneofibular ligament injuries. In addition to extravasation of contrast material lateral to the distal end of
the fibula, the peroneal tendon sheaths are visualized (arrowhead). Normal filling of the medial tendon
sheaths is noted (arrows).
FURTHER READING
78
Chapter 6 Diagnostic Ultrasonography 79
up to 59% of cases, although involvement of only one
side may be evident clinically. Popliteal artery aneurysms
are commonly associated with thromboembolic compli-
cations, including thrombosis, venous occlusion, ulcera-
tion, gangrene, and peripheral embolization. The ultra-
sonographic diagnosis of a popliteal artery aneurysm is
relatively straightforward, as long as continuity of the
mass with a proximal and a distal vessel can be ascertained.
Angiography, the traditional method for diagnosing and
evaluating popliteal artery aneurysms, is not necessary. In
addition, arteriography has several limitations that are
overcome by ultrasonography. For example, thrombus
Figure 6–1. Synovial (popliteal) cyst with rupture. A longi- within an aneurysm is easily documented by sonography
tudinal view of the posterior aspect of the knee shows a popliteal
but is not well seen with arteriography, which opacifies
cyst (straight black arrow) with internal septations (arrowheads)
and an irregular inferior tail (curved arrow), the last finding only the patent lumen carrying flowing blood. Thrombus
consistent with a recent rupture. is considered by some clinicians to be an indication for
surgery. Clinical diagnosis is not always straightforward,
however, such as when pulsation is absent because of a
thrombosed aneurysm.
in persons who lack anatomic communication between
the joint space and the gastrocnemiosemimembranosus
Knee
bursa; when rupture occurs, a pseudocapsule forms to
contain fluid. Most often, these collections are located The role of ultrasonography in evaluating the knee has
between the gastrocnemius and soleus muscles. been diminished by MR imaging. However, sonography
Not every swelling in this area is the result of a may be helpful in evaluating specific problems. For exam-
popliteal cyst, and sonography can conveniently exclude ple, joint fluid, or an effusion, in the knee is easily detect-
other causes, such as a popliteal artery aneurysm (Fig. 6–2), ed by ultrasonography. Anechoic or hypoechoic fluid is
soft tissue tumor, or abscess. Aneurysms of the popliteal identified in the suprapatellar recess, deep to the quadri-
artery are the most common of the peripheral arterial ceps musculature, and in the medial and lateral joint
aneurysms. Atheromatous disease is causative in the vast recesses as well. Intra-articular cartilaginous or osteocar-
majority of cases, and a significant number of patients tilaginous bodies appear as echogenic foci.
have coexisting cardiovascular disease, including an Investigations have centered on the sonographic eval-
abdominal aortic aneurysm. The lesions are bilateral in uation of cartilage (Fig. 6–3). In a series of patients with
osteoarthritis, ultrasonography was shown to be capable
of measuring the thickness of articular cartilage and assess-
Figure 6–4. Menisci of the knee. A longitudinal scan of the Figure 6–6. Collateral ligaments of the knee. A longitudinal
lateral meniscus shows the lateral femoral condyle (F) and the image along the medial joint line of the knee demonstrates a
lateral tibial plateau (T), as well as the overlying iliotibial band normal, homogeneously hyperechoic medial collateral ligament
(ITB). The normal lateral meniscus is seen as triangular (arrows). Note the medial femoral condyle (F) and the medial
homogeneously hyperechoic tissue (black arrow). tibial plateau (T).
Chapter 6 Diagnostic Ultrasonography 81
When scanned longitudinally, the normal patellar tendons in two orthogonal planes is required whenever
tendon is visualized as a linear structure of medium possible. Criteria for the diagnosis of rotator cuff tears
echogenicity, approximately 3 to 6 mm thick, located can be categorized into four groups: (1) nonvisualization
deep to more echogenic subcutaneous fat and the hypo- of the cuff, (2) localized absence or focal nonvisualiza-
echoic serous pretibial bursa. Deep to the tendon, a second tion, (3) discontinuity, and (4) focal abnormal echogenicity.
anechoic bursa may be seen. The internal architecture is No cuff tendon is visualized in patients with large or
similar to that of other tendons, with parallel echogenic massive rotator cuff tears.
fibers and homogeneous density (Fig. 6–7). Seventy-seven Joint and bursal effusions commonly accompany large
percent to 90% of abnormalities in patellar tendinosis are tears and are seen along the biceps tendon and lateral to
visualized at the proximal attachment of the tendon. In the greater tuberosity, respectively. Large tears of the
early stages of disease, the tendon appears enlarged as a supraspinatus tendon may extend posteriorly to involve
result of edema. If the degenerative process continues, the infraspinatus tendon or anteriorly to involve the
the tendon becomes heterogeneous in its internal archi- biceps tendon and the subscapularis tendon. Smaller full-
tecture, with areas of central hypoechogenicity being the thickness tears (Fig. 6–8) appear as a localized absence of
most common sonographic finding, although hyperechoic the cuff with the subdeltoid bursa touching the humeral
foci may occasionally be seen. surface. The most common location of such smaller tears
is the anterolateral portion of the supraspinatus tendon
in the “critical zone.” Even smaller tears appear as a
Hip
discontinuity of the cuff filled with hypoechoic joint fluid
Ultrasonography can be used as a screening procedure to or hyperechoic reactive tissue highlighting the defect
document the presence of intra-articular fluid. In both (Fig. 6–9).
children and adults, distention of the joint capsule is Abnormalities of cuff echogenicity may be diffuse or
identified most readily in scans oriented along the long focal. Diffuse abnormality of cuff echogenicity is an
axis of the femoral neck. Ultrasonography can be used to unreliable sonographic sign for cuff tear but may indicate
characterize the joint effusion as simple or as containing diffuse cuff degeneration or fibrosis. Although diffuse
particulate debris; the latter is suggestive of inflammation, abnormality of cuff echogenicity is unreliable in the
infection, or hemorrhage. Abscess, hematoma, cellulitis, detection of full-thickness tears, significant disparity of
thrombophlebitis, aneurysm, and lymphadenopathy can cuff thickness suggests cuff attrition. Significant degrees
manifest in nonspecific and overlapping ways. Sonography of cuff attrition are associated with partial-thickness tears
can often differentiate among these conditions and, if and also suggest that progressive cuff changes are present.
necessary, is a convenient method of guiding percutaneous In addition to the major criteria just described, several
aspiration or biopsy of possibly abnormal areas. Ultra- minor sonographic findings may prove helpful; subdeltoid
sonography can also identify fluid collections within the bursal effusion, concavity of the subdeltoid bursal contour,
iliopsoas bursa, which are often associated with the elevation of the humeral head relative to the acromion,
synovitis that accompanies many arthritides. In addition, and obliteration of the space normally occupied by the
ultrasonography has an important role in the diagnosis of supraspinatus tendon are all associated with defects in
developmental dysplasia of the hip (see Chapter 73). the cuff.
Sonography also plays an important role in the evalu-
ation of patients who have had acromioplasty, and espe-
Shoulder
cially those who have had surgical repair of a full-thickness
Numerous reports using high-resolution linear array real-
time ultrasonography have demonstrated that sonography
is an alternative means of examining the rotator cuff.
Meticulous technique is critical, and examination of the
Foreign Bodies
Sonographic detection of foreign bodies depends on
differences in acoustic impedance between adjacent
tissues and the foreign body (Fig. 6–13). Because all
reported foreign bodies are hyperechoic, they are best
visualized in slightly hypoechoic tissues such as subcu-
taneous fat, muscle, or inflammatory tissue. The foreign
body itself is usually echogenic and, if embedded in a
tendon, can be difficult to visualize. The foreign body,
however, often incites an adjacent inflammatory reaction
with vascular granulation tissue, which forms a hypoechoic
rim about the hyperechoic foreign body, increasing its
conspicuity. Some degradable foreign bodies (e.g., wood)
may decrease in echogenicity over time and may disappear
within an inflammatory collection.
Figure 7–1. Comparison of resolution. Whole body sweep (A) does not show the increased uptake in the
right tibia (B, arrow), due to a stress reaction, as well as the spot scans of the lower legs do.
The metaphyseal regions of long bones show higher uptake renal pelvis can accumulate considerable radionuclide and
than do the diaphyses, where only mild uptake is present. give a false appearance of hydronephrosis. Bladder diver-
This is due to the higher bone turnover rate in the ticula or the lateral aspects of a normal bladder may over-
metaphyses. The areas of highest uptake in the normal lie the pubic bones, giving a false appearance of lesions.
bone scan are the sternum and the sacroiliac joints. The Urine leakage may occur, producing hot spots that over-
anterior wings of the iliac bones and the coracoid processes lie the skeleton and simulate lesions. Unbound 99mTc
normally show considerable uptake of the radionuclide. is taken up in the salivary glands, thyroid, and stomach.
The patellae may show higher uptake than the remainder Thyroid and laryngeal cartilage uptake can be differen-
of the bones about the knees, which may be due to their tiated from abnormalities in the cervical spine, because
greater vascularity. they will be hotter on the anterior scan than on the pos-
Uptake in calcification of the costal cartilage may be terior scan, whereas the reverse is true for cervical spine
seen as areas of radionuclide accumulation on the ante- lesions. The tips of the scapulae may appear hot and
rior scan of the chest. Uptake may normally be present in overlie the ribs, simulating rib lesions. Views done with
the soft tissues of the breast. Hyperostosis frontalis interna the scapulae raised and the arms brought across the chest
causes increased uptake in both frontal bones and can be may displace the scapulae from the ribs, eliminating this
recognized by its characteristic pattern on anterior and potential diagnostic problem.
lateral views. With scoliosis, the concave side of the spine The age of the patient may affect the appearance of
appears hotter than the convex side does. Thoracic kyphosis the bone scan. Children have high, diffuse bone uptake
and lumbar lordosis may cause some areas of the spine to and prominent uptake around the growth plates, owing
be farther from the camera, and they will not appear as to new bone formation in the growing skeleton. Elderly
hot as areas that are closer to the spine. patients tend to have poor-quality bone scans. Heavy
The radionuclide is excreted through the kidneys, which patients have poor-quality bone scans owing to the
normally should be visible. If no uptake is seen in the greater amount of soft tissue, which causes more scatter
kidneys, either there is renal failure or absent kidneys or and higher attenuation of the photons in the soft tissues
a superscan is present, characterized by such high uptake because of the greater distance of the detectors from the
in bone that little radioactivity is excreted by the kidneys. skeleton. Poor hydration prevents optimal clearance of
Hydronephrosis may be detected by the appearance of a the radionuclide from the soft tissues. Renal failure also
dilated ureter and renal collecting system. An accessory prevents good soft tissue clearance.
Chapter 7 Radionuclide Techniques 89
Figure 7–2. Stress fractures of the right and left pars of L5. On a posterior planar scan (A), there is a
suggestion of faint increased uptake in the posterior elements of L5, but the scan is not diagnostic. On a
SPECT scan with coronal reconstructions (B), hot spots are seen in the right and left pars of L5 (arrows).
90 Section II Diagnostic Techniques
METABOLIC BONE DISEASE or soft tissue tumor that initiates biochemical change,
High Bone Turnover Conditions causing osteomalacia. Most of these tumors are benign,
fibrous, and highly vascular and include giant cell granu-
A number of conditions, including osteomalacia, hyper- lomas, cavernous hemangiomas, fibroangiomas, nonossi-
parathyroidism, and renal osteodystrophy, are frequently fying fibromas, and, rarely, malignant tumors.
associated with increased bone turnover. Increased bone Secondary amyloidosis occurs in chronic renal failure,
resorption by itself does not cause increased uptake on a particularly when treated with hemodialysis. There is
radionuclide bone scan; however, such increased resorp- deposition of β2-microglobulin amyloid in and around
tion is often accompanied by increased formation of bone, bones and joints, as well as in other locations. Increased
which does cause increased uptake. Multiple hot spots in uptake of 99mTc phosphonates may occur in the articular
adjacent ribs on both sides of the body in a patient with and periarticular regions in some (but not all) cases of
no history of trauma are suggestive of osteomalacia, as are amyloid deposition, and this uptake may precede
hot spots in the inferior tips of the scapulae (Fig. 7–3). radiographic changes. Uptake of 67Ga and 201Tl may also
Insufficiency fractures in metabolic bone disease may also be seen in the articular regions of patients with such
be seen in the pubic rami, femoral necks, femoral condyles, amyloid deposition. Increased uptake of Tc phosphonates
metaphyses of the proximal and distal portions of the is present in the brown tumors of hyperparathyroidism
tibia, sacrum, and calcaneus. and may mimic metastatic disease. 99mTc sestamibi is
Bone scanning for detection of osteomalacia, renal often used to localize parathyroid adenomas and also
osteodystrophy, and, to a lesser degree, primary hyper- shows increased uptake in brown tumors.
parathyroidism has been shown to be more sensitive than
radiography. Bone scans in patients with osteoporosis do
not show these metabolic features, except when accom- Generalized Osteoporosis
panied by fractures. A flare response on the bone scan has If all other conditions are equal, a greater quantity of
been reported after treatment for osteomalacia. This bone in any region shows proportionately more uptake of
response is most likely caused by increased osteoblastic Tc phosphonates than does a smaller quantity. However,
activity due to bone healing and should not be mistaken the uptake in a volume of bone is determined not only by
for progressive disease. In patients with osteomalacia the quantity of bone but also by the rate of bone turnover
whose symptoms are not improved after 6 months or and bone vascularity. Thus, a bone scan cannot be
more of therapy, the possibility of oncogenic osteomalacia used to measure bone density. Bone scanning in patients
should be considered. The cause of this condition is with osteoporosis is useful for the evaluation of
thought to be production of a substance by an osseous compression fractures of the spine, insufficiency frac-
tures at any site, and other causes of bone and joint pain.
The typical appearance of a compression fracture is a
bandlike area of increased uptake in the vertebral body
(Fig. 7–4). The bone scan may be positive before
vertebral collapse is seen radiographically. If the bone
scan is normal in the presence of a compression fracture
that is visible on conventional radiographs, the fracture is
not new. Bone scans following fracture return to normal
in 59% of patients by 1 year, 90% by 2 years, and 97% by
3 years.
Insufficiency fractures associated with osteoporosis may
occur in the sacrum, pubis, femoral neck, metaphyses of
the proximal and distal portions of the tibia, calcaneus,
and ribs, especially the anterior aspect of the ribs. In osteo-
porosis, there is not the same tendency toward bilateral
and symmetrical fractures that is seen in osteomalacia.
Disuse Osteoporosis
Disuse can cause increased bone turnover and increased
uptake on a bone scan. In patients with fractures of the
shafts of long bones, diffuse increased uptake is often
seen about the joint distal to the fracture. In patients who
have bone scans for suspicion of fracture and who are
using crutches, the bone scan sometimes shows decreased
vascularity and uptake in the limb because of decreased
blood flow due to immobilization. In a study of patients
Figure 7–3. Osteomalacia. Hot spots in the inferior scapulae with hemiplegia from cerebrovascular accidents, it was
bilaterally (arrows), in a patient who presented with a clavicle found that 65% showed decreased uptake of the radionu-
fracture, suggested the diagnosis of osteomalacia, which was clide in the blood flow and blood pool images and normal
confirmed by biochemical tests and bone biopsy. uptake on delayed images in the affected hand as a result
Chapter 7 Radionuclide Techniques 91
Figure 7–4. Vertebral compression fracture. Bandlike high uptake in the T9 vertebral body (arrow) on the
posterior scan (A) was caused by a compression fracture that was also seen on a radiograph. Twelve months
later (B), there is only minimal increased uptake in T9 (arrow).
of paralysis or disuse; 25% had diffuse increased uptake In RSD, the scintigraphic abnormalities are diffuse,
on delayed images, which was thought to represent reflex not focal, with widespread increased uptake on delayed
sympathetic dystrophy. images and prominent periarticular uptake (Fig. 7–5). In
some cases, other joints of the ipsilateral extremity may
also show diffuse increased uptake. Abnormalities other
Reflex Sympathetic Dystrophy than RSD, however, can cause similar scintigraphic find-
Reflex sympathetic dystrophy (RSD) has also been desig- ings. These include synovitis and arthritis, cellulitis, soft
nated complex regional pain syndrome, sympathetically tissue trauma, and disuse osteoporosis. The bone scan
mediated pain syndrome, algodystrophy, Sudeck’s atrophy, may also be helpful in patients with suspected RSD by
and causalgia. Diagnostic criteria for RSD include pain showing a focal abnormality that may be due to trauma
and tenderness in an extremity, soft tissue swelling, dys- or neoplasm. The scintigraphic diagnosis of RSD is most
trophic skin changes, vasomotor instability, functional accurate in the early stages of the disorder, with a dura-
impairment, and patchy osteoporosis. RSD may demon- tion of less than 6 months.
strate three scintigraphic patterns, which roughly corre-
late with three clinical stages.
Transient Osteoporosis
Stage I (Inflammatory). Pain, swelling, and erythema are Transient osteoporosis of the hip, also called transient
present. Increased vascularity is seen on the blood flow bone marrow edema syndrome, is characterized by
and blood pool images, and there is increased uptake on markedly increased uptake in the entire femoral head and
the delayed static scans. increased uptake extending down the femoral neck to the
intertrochanteric region or proximal shaft of the femur
Stage II (Dystrophic). This stage is characterized by burn- (Fig. 7–6). Increased activity on blood flow and blood
ing and throbbing pain, a reduced range of joint motion, pool scans is characteristic. The hypothesis for the cause
and skin thickening. The blood flow and blood pool of transient osteoporosis is an episode of ischemia of the
images are normal, and there is increased uptake in the femoral head that does not lead to osteonecrosis. Reactive
delayed images. hyperemia and repair of the ischemic bone occur, account-
ing for the scintigraphic findings. In transient osteoporosis,
Stage III (Atrophic). The skin temperature is decreased, intense and homogeneous uptake of the radionuclide
joint function is decreased, and contractures are present. agent with femoral head and femoral neck involvement
Severe osteoporosis may be evident on radiographs. Bone is typical. Insufficiency fractures involving the femoral
scanning shows decreased uptake on blood flow and blood neck, subchondral portion of the femoral head, or both,
pool images and normal or decreased uptake on delayed may have a scintigraphic appearance similar to that of
static images. transient osteoporosis.
92 Section II Diagnostic Techniques
Figure 7–5. Reflex sympathetic dystrophy of the left hand. A, Mildly increased activity in the blood flow (left)
and blood pool (right) phases are noted in the left hand. B, Diffuse increased uptake is seen in the left hand
and wrist, with prominent periarticular uptake. C, Radiograph shows osteoporosis in the left hand and wrist.
Figure 7–6. Transient osteoporosis of the hip. A, Increased radionuclide uptake in the right femoral head,
extending into the femoral neck, is seen. B, Coronal T2-weighted MR image shows diffuse increased signal
in the right femoral head. C, Radiograph of the pelvis shows osteoporosis in the right femoral head.
Malignant transformation is a complication of Paget’s except for the assessment of multiple myeloma. The bone
disease. In such transformation, the most common scinti- scan provides information about the presence, location,
graphic finding is a cold area within the region of high extent, and response to therapy of bony metastases; allows
uptake. In some cases, there may be no cold area, and the the evaluation of equivocal radiographic findings; and
presence of bone destruction related to the sarcoma may provides guidance regarding the site of biopsy. Abnorma-
be obscured by the high uptake of the adjacent pagetic lities on bone scans alone, however, should not alter therapy
bone. Uptake in the soft tissues adjacent to a pagetic unless metastatic disease is confirmed by other methods.
lesion is another sign that is suspicious for malignant Bone scintigraphy or another imaging method is indi-
transformation. cated in patients with cancer who have musculoskeletal
pain. Although metastatic disease should be the first
NEOPLASTIC DISEASE consideration, in the majority of cancer patients without
known metastatic disease, the cause of musculoskeletal
Metastatic Disease pain is not malignancy. Bone scans detect metastatic
Radionuclide bone scanning is the procedure of choice disease that is not seen radiographically. Another issue is
for the evaluation of metastatic disease to the skeleton, the use of serial bone scans to follow patients with malig-
94 Section II Diagnostic Techniques
Figure 7–7. Paget’s disease. High uptake is seen in the cervical, thoracic, and lumbar vertebrae; sacrum;
pelvic bones; skull; and a left rib.
nant tumors. Early diagnosis of asymptomatic skeletal permeability. Scintigraphic abnormalities that are usually
metastases does not necessarily lead to increased survival related to benign causes include a pattern of focal round
or benefit to the patient. areas of increased uptake in adjacent ribs at the same
Metastatic lesions to the skeleton occur through the location, which is typical of fracture; hot spots in the
venous, arterial, or, less commonly, lymphatic circula- anterior ribs near the costochondral junctions and at the
tion. The vertebral venous plexus of Batson has an impor- posterior costovertebral margins, which are often asso-
tant role in the spread of metastases to areas in the axial
skeleton. In patients with osseous metastases, the ver-
tebrae and thorax are involved in up to 50% of cases, the
pelvis in 38%, the extremities in 34%, and the skull in
22%.
Blastic, mixed, and lytic lesions usually show increased
uptake on a bone scan. In blastic lesions, this is related to
the tumor stimulating the osteoblasts. With lytic lesions,
there is usually an osteoblastic repair response to the
osteoclastic resorption caused by the tumor. If there is no
repair response, a cold, or photopenic, lesion will occur
(Fig. 7–8). Although the exact incidence of cold metasta-
tic lesions is not certain, because they may be difficult to
detect, they are relatively uncommon. Most multifocal or
metastatic tumors, except for multiple myeloma and some
highly aggressive anaplastic tumors, are associated with
a repair response. Cold lesions are sometimes seen in
metastatic lesions arising from bronchogenic carcinoma.
Multiple areas of increased uptake in a random pattern,
predominantly in the axial and the proximal appendicular
skeleton, are typical of metastatic disease. Increased vas- Figure 7–8. Metastatic lesions. A large lytic lesion in the
cularity is often present on early-phase images, owing to sacrum from metastatic melanoma shows decreased uptake, or
tumor-induced neovascularity with increased capillary photopenia (arrows), on a posterior image of the pelvis.
Chapter 7 Radionuclide Techniques 95
ciated with osteoporosis; and uptake in the periarticular bone scan. In leukemia, bone scans are usually normal. In
regions, especially involving both sides of the joint, multiple myeloma, bone scanning shows only about 50%
usually due to arthritic disease. Isolated uptake in the of the lesions that are evident on radiography and under-
peripheral appendicular skeleton is rarely caused by metas- estimates the extent of the disease, compared with a
tases. Occasionally, lung cancer metastasizes to the hands radiographic skeletal survey. Radiographic skeletal surveys
or feet. rather than bone scans are used to follow the course of
Metastatic disease in the spine typically involves the multiple myeloma.
vertebral body or the vertebral body and the pedicle. The In following the course of treatment of metastatic
pedicle is rarely involved alone. SPECT scanning is more disease with serial bone scans, the flare response may be
sensitive than planar imaging in detecting vertebral metas- seen. This is characterized by an increase in the intensity
tases and helps in differentiating benign from malignant and number of focal areas of increased uptake on bone
disease. Using SPECT, focal uptake confined to the ver- scans performed within the first 3 months after treatment,
tebral body is typically benign, as is diffuse uptake con- followed by decreased uptake on later scans. This finding
fined to the vertebral body. In contradistinction, when is believed to predict successful therapy and is thought
there is uptake in both the vertebral body and the pedicle, to be due to an osteoblastic response. Radiographs may
the responsible tumor is typically malignant. show osteosclerosis developing in the bony lesions.
In lymphoma, bone scans show abnormal uptake, but Magnetic resonance (MR) imaging has higher sensi-
the pattern may differ from that of metastatic disease. tivity and specificity than does planar bone scanning for
There may be multiple subtle and often asymptomatic the evaluation of metastatic lesions. Whole body MR
lesions, with frequent involvement of the bones of the imaging with fast spin echo and fast short tau inversion
extremity (Fig. 7–9). Diffuse, patchy uptake may reflect recovery (STIR) sequences, covering the whole skeleton
marrow involvement. Some lesions involving the bone or only the axial skeleton, takes less than 1 hour and has
marrow, without cortical extent, may not be positive on a been shown to be an effective method that demonstrates
B
96 Section II Diagnostic Techniques
more metastatic lesions than does bone scanning. At this radioactive iodine cannot be detected with 131I scanning
time, however, bone scintigraphy remains the initial but usually can be detected with bone scans.
99m
procedure used most frequently to screen for skeletal Tc sestamibi (MIBI) localizes in bone marrow that
metastases. MR imaging is usually used as a comple- is involved with malignancy. This method has been used
mentary technique when the scintigraphic findings are to detect lesions in multiple myeloma, leukemia, lymphoma,
not diagnostic. and skeletal metastases. It may be most useful in detect-
PET scanning with 18F has been used to evaluate ing and showing the extent of conditions with diffuse
skeletal metastases and other benign and malignant marrow involvement that may not be seen with bone
osseous lesions. In comparison to radionuclide bone scanning. 201Tl acts in a manner similar to MIBI. Malig-
scanning, PET has been found to be more sensitive. The nant bone marrow lesions reveal increased uptake.
67
sensitivity of 18F PET is similar to that of MR imaging. Ga scanning is similar to bone scanning with regard
PET scanning with FDG has been used for the detection to sensitivity in detecting bony lesions in lymphoma, and
of skeletal as well as soft tissue metastases (Fig. 7–10). it allows the detection of extraosseous involvement. For
With FDG, uptake occurs in the metabolically active bone lesions related to lymphoma, 67Ga scanning can be
zones of the tumor because of increased glucose used to monitor the response to therapeutic intervention.
metabolism, whereas with scanning using bone-seeking
radionuclides, uptake is due to new bone formation. A
Primary Malignant Bone and Soft Tissue
recent study found that FDG-PET was superior to Tc
Tumors
MDP bone scanning in the detection of osteolytic metas-
tases but was inferior in the detection of osteoblastic Almost all primary malignant bone tumors reveal increased
metastases. The osteoblastic lesions apparently had lower uptake on a bone scan, owing to tumor neovascularity,
metabolic activity and so were less detectable with FDG. increased capillary permeability, and increased bone turn-
FDG-PET, when compared with bone scanning, had a over from bone destruction and repair. This finding can-
lower sensitivity for the detection of osseous metastases not be used to differentiate between benign and malignant
in prostate cancer. FDG-PET was found to be superior lesions with certainty. However, primary malignant bone
to bone scanning in the detection of skeletal lesions tumors tend to be associated with characteristic scinti-
in lymphoma. graphic patterns, although overlap occurs and there is
Iodine-131 (131I) is used to detect metastases, includ- no unique pattern of features (Fig. 7–11). Soft tissue
ing those to bone, from differentiated thyroid carcinoma. sarcomas have increased blood flow, as seen on early-
The image quality and ability to localize abnormalities phase images of a bone scan (Fig. 7–12). Delayed images
are not as good as with bone scanning. Metastases from often show mild increased uptake in soft tissue lesions.
more aggressive thyroid cancers that do not accumulate Higher uptake of the radionuclide on delayed images
Figure 7–10. An FDG-PET scan, with sagittal and coronal tomographic images, shows increased uptake
(arrows) in a bony metastasis in L5 from colon cancer, as well as multiple soft tissue metastases in lymph
nodes. (Courtesy of R. Katz, MD, New York, NY.)
Chapter 7 Radionuclide Techniques 97
Figure 7–11. Osteosarcoma of the left femur. A, Bone scan of an osteosarcoma of the left distal femur
shows patchy, inhomogeneous high uptake and lower uptake in the center. B, On the radiograph, a lytic
lesion is present, with cortical destruction and extension into the soft tissues.
may be present if calcification or ossification is present Benign Bone Tumors and Tumor-Like
within the tumor. Conditions
High-grade malignant lesions tend to have high uptake In benign bone tumors, the uptake of 99mTc phosphate
of radionuclides, and benign tumors tend to have lower bone scanning agents varies with the type of tumor;
uptake. Low-grade malignant tumors cannot always be normal, mildly increased, markedly increased, and even
differentiated from benign tumors, however. decreased uptake may be present. The vascularity on
Figure 7–12. Soft tissue sarcoma. A, Increased activity (arrows) is noted in the left thigh on the blood pool
scan. B, STIR MR image shows high signal in the soft tissues of the thigh.
98 Section II Diagnostic Techniques
early-phase imaging may also vary. Bone scanning is very Most cases of enchondroma are “warm” or “hot”
helpful in the diagnosis of osteoid osteomas, especially on bone scans; however, bone scanning cannot differ-
when radiographs are normal or nondiagnostic. This entiate enchondroma from chondrosarcoma. In Ollier’s
often occurs when the lesion is in the spine, pelvis, or hip disease (enchondromatosis), the sites of multiple enchon-
(Fig. 7–13). The characteristic scintigraphic appearance dromas can be detected. In giant cell tumors, bone scan-
is a small, round, focal area of high uptake. There may be ning shows high uptake on both the early and delayed
increased vascularity on early-phase scans, but this is images, most intensely in the periphery of the lesion.
often not present. A computed tomography (CT) scan is A “donut” appearance is frequent, with a rim of high
necessary to confirm the diagnosis of osteoid osteoma uptake surrounding a central area of lower uptake
and to localize it for removal, either by open surgery or (Fig. 7–14). A similar appearance can be seen in other
percutaneously under CT guidance. tumors, however.
C D
Chapter 7 Radionuclide Techniques 99
Figure 7–14. Giant cell tumor. A, High uptake is seen in the right lateral tibial plateau. Lighter anterior
and right lateral images show a rim of high uptake surrounding an area of lower uptake. B, A lytic lesion is
present in the lateral tibial plateau on the radiograph. The fat-suppressed intermediate-weighted spin echo
MR image shows mixed high signal in the lesion.
Increased uptake on bone scanning is present in which case high uptake is present. The scintigraphic
aneurysmal bone cysts, with most revealing a rim of appearance of hemangiomas of bone varies, depending
increased uptake around the periphery of the cyst and on the biologic activity of the tumor, its site, and the
lower uptake in its center. The intensity of the uptake resolution of the equipment.
may vary; however, some aneurysmal bone cysts may Fibrous dysplasia is a condition in which there is
show only mild or, rarely, normal uptake. Early-phase replacement of normal bone by abnormal fibro-osseous
scans may show increased radionuclide uptake, reflect- tissue with rapid bone turnover. The bone scan in fibrous
ing marked hyperemia in the center of the lesion. dysplasia shows areas of high uptake. The role of bone
Unicameral bone cysts have normal or sometimes scanning in patients with Langerhans’ cell histiocytosis is
decreased uptake, except when they are fractured, in controversial, with variable sensitivity. Most bone islands
100 Section II Diagnostic Techniques
have normal uptake on a bone scan. Growing bone islands Stress Fractures
may also have normal uptake. However, increased uptake Stress fractures are caused by focal areas of bone resorp-
has been reported in large bone islands. Bone scans show tion occurring as a response to stress, with ultimate weak-
normal findings in osteopoikilosis and osteopathia striata ening of the bone that may lead to fracture. Bone scans
but show increased uptake in melorheostosis. are almost always abnormal in stress fractures at the time
of onset of pain, whereas radiographs are often normal at
TRAUMA this time. The scintigraphic appearance of stress frac-
tures depends on the location, type of bone, and chronicity.
Traumatic Fractures Stress fractures can be divided into three types: (1) fatigue
Bone scanning may be used to detect fractures when fractures due to excessive stress on normal bone, usually
radiographs are normal or nondiagnostic, especially in found in athletes; (2) insufficiency fractures, due to stress
areas that are difficult to evaluate radiographically. Frac- on weakened bone, found in metabolic bone disease; and
tures can be detected by bone scan within 24 hours in (3) pathologic fractures caused by neoplasms.
95% of patients younger than 65 years. By 72 hours, 95% In the early stages of a stress fracture, radiographs may
of fractures in all patients are abnormal on bone scan. be normal; subsequently, periostitis is observed, and even-
Increased blood flow, growth of new blood vessels, and tually a fracture line is evident. With scintigraphy, the
callus formation by osteoblasts occur within hours after blood flow and blood pool scans are abnormal in recent
the fracture. There are differences in the magnitude of stress fractures, with first the blood flow and then the
uptake in fractures at different sites. Fractures in peri- blood pool scans returning to normal by about 2 months.
articular locations reveal high uptake within the first day The delayed images return to normal more slowly and
after trauma. Fractures in the axial skeleton and shafts of may still show mild increased uptake 8 to 10 months after
the long bones reveal a slower increase in the intensity of the time of clinical presentation. Focal areas of increased
uptake, with some requiring 12 days to appear positive on uptake are often found in the feet in symptomatic or
a bone scan. Skull fractures are usually not detected on a asymptomatic athletes and should be regarded as stress
bone scan. After the traumatic episode, all fractures have changes. A focal area of markedly increased uptake in
increasing uptake of the radionuclide bone scanning vascular and delayed phases of the bone scan should be
agent, with peak accumulation at 2 to 5 weeks. By 1 year considered a stress fracture in a patient with the sudden
following trauma, the bone scans in 79% of rib fractures, onset of foot pain.
64% of long bone fractures, and 59% of vertebral Femoral neck stress fractures are seen as focal increased
fractures return to normal. By 2 years, bone scans in 90% uptake in the medial or lateral aspects of the femoral neck
of all fractures are normal. or as a bandlike area through the entire femoral neck
The diagnosis of a fracture of the femoral neck in (Fig. 7–16). Recent articles have also described insuffi-
patients with normal or nondiagnostic radiographs can ciency fractures of the subchondral portion of the femoral
be made by radionuclide bone scanning or by MR imaging. head. These may simulate osteonecrosis or transient osteo-
Recent evidence suggests that MR imaging has greater porosis on clinical assessment and imaging studies. Sacral
sensitivity and specificity for the diagnosis of hip frac- fractures are a frequent cause of low back pain in osteo-
tures. Bone scanning is often used in the diagnosis of porotic patients. Such fractures may involve both sacral
occult scaphoid fractures (Fig. 7–15). alae and the body of the sacrum, giving a characteristic
Figure 7–15. Scaphoid fracture. The blood pool (A) and delayed 3-hour bone scan (B) images show
increased activity in the scaphoid (arrows), consistent with a fracture, in a patient who had wrist trauma and
normal radiographs.
Chapter 7 Radionuclide Techniques 101
A B
Figure 7–16. Stress fracture of the femoral neck. A, Focal area of increased uptake is present in the medial
aspect of the left femoral neck on the bone scan (arrow). B, Radiograph taken 2 weeks after the bone scan
shows an area of sclerosis in the medial aspect of the femoral neck (arrow), confirming the presence of a
stress fracture.
H appearance on the bone scan, which is diagnostic caused by a variety of abnormalities. The medial tibial
(Fig. 7–17). They may also involve only one sacral ala, stress syndrome is the most frequent type of exertional
giving the appearance of high uptake in the region of the shin pain in athletes. Pain in the posteromedial aspect of
sacroiliac joint, which must be differentiated from sacroili- the tibia may be caused by tibial periostitis, tibial stress
itis. High-dose radiation therapy that includes the bones fracture or stress reaction, or distal deep chronic poste-
of the pelvis may cause sacral and pubic bone insuffi- rior compartment syndrome. Blood flow and blood pool
ciency fractures. scans are normal in cases of shin splints. The location of
Stress fractures of the pars interarticularis (i.e., the uptake suggests that it is related to the attachment
spondylolysis) may be a cause of back pain in athletes. site of the soleus muscle. The posterior and anterior tibialis
SPECT scanning is more sensitive than planar imaging muscles may also be involved in shin splints (Fig. 7–18).
for the detection of spondylolysis and also provides better The cause of the pain is thought to be periostitis occur-
localization of the defect. Radiographs are often normal. ring along the tibia caused by muscular and tendinous
CT scanning may help visualize the fracture, which may strain associated with inflammation.
be unilateral or bilateral. Increased uptake at the site of
spondylolysis suggests that it is the cause of the patient’s
symptoms of back pain. Soft Tissue Injuries
The location of the stress fracture depends on the The scintigraphic appearance of exertional compartment
sport or activity and the type of stress that is present. The syndrome is an area of decreased uptake in the tibial shaft
most frequent site of a stress fracture in runners is the with increased uptake superiorly and inferiorly. The
tibial shaft, followed by the metatarsal shaft. Other sites decreased uptake is most likely due to decreased blood
include the calcaneus, femoral shaft, femoral neck, pubic flow from increased pressure in the compartment. 201Tl
bone, fibula, tarsal navicular bone, posterior elements chloride is reported to be effective for the diagnosis of
of the spine, and sacrum. Gymnasts, weight lifters, and exertional compartment syndrome in the lower legs.
tennis players tend to get stress fractures in the posterior Bone scans may show evidence of muscle injury. Long-
elements of the spine because of the hyperextension of distance runners show increased uptake in the muscles on
the vertebral column that accompanies participation in bone scans obtained within 48 hours of a race, with return
these activities. to normal by 1 week. The muscles that show increased
Shin splints is a term that has been used to describe uptake are the ones used most during the exercise, due to
chronic exertional shin pain in athletes, which can be exercise-induced rhabdomyolysis.
102 Section II Diagnostic Techniques
Combined 99mTc phosphate bone scanning and 67Ga the detection of femoral component loosening in patients
citrate scanning has been used for diagnosing prosthetic with hip prostheses, but it does not adequately evaluate
infection. Normal scans are accurate in ruling out infec- the acetabular component.
tion. The patterns of spatial mismatch of bone and gallium
scans and the higher uptake of gallium compared with
that of bone scan are specific for infection but are not ARTHRITIS AND PAIN AROUND JOINTS
sensitive. Radiolabeled leukocyte scans have a high sensi-
tivity for the diagnosis of infected prostheses. Specificity Arthritis
is increased by comparison with bone scans, to determine Synovitis is demonstrated by abnormalities in the early
mismatch, or with bone marrow scans, which increases phases of a 99mTc MDP bone scan that indicate diffuse
the specificity even more. increased blood flow around a joint. In cases of arthritis,
Radionuclide arthrography (i.e., arthroscintigraphy) uptake on the delayed (3-hour) scans is related to increased
has been used to determine loosening of hip and knee blood flow to the juxta-articular bone that results from
prostheses. Radionuclide arthrography is done by inject- synovitis. Uptake is also related to bony changes asso-
ing radiolabeled colloid into the joint at the time of a ciated with arthritis, including erosions, sclerosis, osteo-
joint aspiration and radiographic contrast arthrogram. A phytes, and subchondral cysts. A number of new scanning
scan is obtained about 2 hours later to determine leakage agents that target inflammation are being used for the
of the radionuclide into the prosthetic interface, which evaluation of arthritis. One approach is the use of radio-
would indicate loosening. Radionuclide arthrography per- labeled monoclonal antibodies to antigens within the
forms better than radiographic contrast arthrography for arthritic joint.
Chapter 7 Radionuclide Techniques 105
B C
Figure 7–20. Osteomyelitis. A, Blood pool and delayed 3-hour images of a bone scan show increased
activity in the metaphyseal region of the medial aspect of the left distal tibia (arrows). B, Radiograph shows
a lytic lesion in the medial metaphyseal region of the distal tibia (white arrow). C, STIR MR image
demonstrates high signal in the lesion in the medial aspect of the distal tibia and shows that the lesion crosses
the growth plate from the metaphysis into the epiphysis and extends into the soft tissues (arrows).
In patients with polyarthralgia, a normal bone scan Osteoarthritis tends to have focal uptake in a joint rather
excludes inflammatory joint disease, whereas an abnormal than diffuse uptake. These patterns on the bone scan
bone scan suggests that inflammatory disease is present, underscore the well-known morphologic findings of various
which may affect treatment. The appearance of inflam- articular disorders, but differences in severity of involve-
matory arthritis on a bone scan includes abnormal early ment may be shown on bone scans versus radiographs.
and late phases of the examination (Fig. 7–25). Any
monarticular process must be considered septic arthritis
Other Causes of Pain around Joints
until proved otherwise. Polyarthritis with involvement of
the hands and feet is consistent with rheumatoid arthritis Occult malignancy, even in patients with no history of
(Fig. 7–26). Uptake at sites of tendon insertion in the cancer, may be a cause of pain around joints. The yield
feet suggests psoriatic arthritis or Reiter’s syndrome. for identification of occult malignancy on bone scans in
106 Section II Diagnostic Techniques
patients aged 50 years or older is 9%. In patients with a plantar aspect of the calcaneus and are not necessarily
history of cancer, metastatic disease should be ruled out symptomatic.
as a cause of periarticular pain through the use of a bone Focal increased uptake in the patella on a bone scan
scan, before the patient undergoes an extensive workup. may be present in some cases of chondromalacia patellae
Bone scans are often ordered to evaluate foot pain, (Fig. 7–28). Focal uptake in the patella is not specific for
because the cause of pain may not be clear clinically or on chondromalacia and could be due to arthritis or osteo-
radiographic examination. Common abnormalities that chondral injury.
cause foot pain include plantar fasciitis, stress fractures, Bone scans show abnormalities in both secondary and
degenerative and inflammatory arthritis, sesamoid abnor- primary hypertrophic osteoarthropathy. The typical scinti-
malities, osteoid osteoma, and tendinosis and tenosyn- graphic pattern is circumferential uptake around the bones
ovitis. Coregistration of the abnormalities delineated on of the wrists, knees, and ankles (Fig. 7–29). After treatment,
the bone scan with those found on radiographs has been improvement can be seen in the scan’s appearance. Clin-
suggested when it is difficult to localize the site of ical improvement precedes improvement seen scintigraph-
radionuclide uptake anatomically, as may occur in the ically, which precedes radiographic signs of improvement.
tarsal bones. Plantar fasciitis may cause a focal area of
increased uptake on the plantar aspect of the calcaneus at
OSTEONECROSIS
the medial calcaneal tubercle, due to periostitis at the site
of insertion of the plantar fascia (Fig. 7–27). Heel “spurs” The scintigraphic appearance of osteonecrosis depends
due to enthesopathic changes at the insertion of the short on the cause and stage of the disease, as well as on the
flexor tendons may also cause increased uptake in the scintigraphic equipment and technique used. Osteonecrosis
Chapter 7 Radionuclide Techniques 107
Figure 7–22. Infection and neuropathic osteoarthropathy in a diabetic patient. A, Bone scan shows
increased uptake in the right first toe and in the left midfoot. B, Simultaneous 24-hour 111In-labeled
leukocyte scan (left) and 1-hour 99mTc sulfur colloid bone marrow scan (right) show increased leukocyte
uptake in the right first toe (arrow) that is not matched by bone marrow uptake, indicating infection. There
was matched leukocyte and bone marrow uptake in the left midfoot, consistent with neuropathic
osteoarthropathy.
108 Section II Diagnostic Techniques
Figure 7–23. Bone scan in a case of mechanical loosening of the femoral component of a total hip
prosthesis. The 3-hour image shows increased uptake around the tip of the femoral stem and around the
greater and lesser trochanters, consistent with mechanical loosening. Increased uptake in the right femoral
head was due to osteonecrosis. The radiograph shows a prominent lucency at the cement-bone interface
around the entire femoral component, consistent with loosening.
can be divided into septic and aseptic and traumatic and graphy for the detection of osteonecrosis. The charac-
nontraumatic types. teristic scintigraphic appearance of nontraumatic osteo-
Nontraumatic osteonecrosis is usually associated with necrosis in the femoral head is the cold-in-hot lesion
one or more risk factors. The most frequent risk factors (Fig. 7–30). This is a photopenic area caused by the
are use of corticosteroids and increased alcohol intake. necrotic bone, surrounded by a rim of increased uptake
Others include sickle cell anemia and dysbarism. Bone caused by the apposition of viable bone on the periphery
scanning has been shown to be more sensitive than radio- of the dead bone, which occurs during the revasculariza-
Figure 7–24. Bone scan in a patient with an infected total hip prosthesis. A, Blood pool scan shows
increased activity around the left total hip prosthesis (arrows). B, The 3-hour image shows diffuse increased
uptake around the femoral and acetabular components, consistent with infection.
Chapter 7 Radionuclide Techniques 109
Figure 7–25. Gouty arthritis of the first toe. The patient presented with pain and swelling of the first toe
but had normal radiographs. The scan was ordered to rule out infection. Blood flow (A) and blood pool (B)
scans show increased activity in the left first toe. A 3-hour plantar bone scan (C) shows increased uptake
around the left first interphalangeal joint. The scintigraphic findings are consistent with inflammation and
arthritis of the joint, which could be infectious or noninfectious. On aspiration of the interphalangeal joint,
urate crystals were obtained, indicating gout.
tion and healing phases of the process. The scintigraphic the bone scan may become normal; however, this is not
appearance may change as the stages of osteonecrosis commonly seen. Small areas of osteonecrosis may not be
progress to bone collapse and secondary osteoarthritis. detected on scintigraphy. MR imaging is more sensitive
New areas of increased uptake result from femoral head than scintigraphy in detecting osteonecrosis.
fragmentation and collapse. With secondary osteoarthritis, In traumatic osteonecrosis, which may result from
increased uptake is present on both sides of the joint. If femoral neck fracture, hip dislocation, or scaphoid or
bone collapse and secondary osteoarthritis do not occur, talar fracture, there is disruption of the blood supply at
110 Section II Diagnostic Techniques
Figure 7–27. Plantar fasciitis. The blood pool (A) and delayed (B) images of a bone scan show focally
increased activity in the plantar aspect of the right calcaneus.
112 Section II Diagnostic Techniques
Figure 7–28. Chondromalacia patellae. A, Bone scan shows a focal area of increased uptake in the medial
aspect of the left patellofemoral joint (arrows). B, Intermediate-weighted spin echo MR image shows
abnormal signal and erosion in the medial aspect of the patellar cartilage (arrows).
Chapter 7 Radionuclide Techniques 113
Figure 7–30. Osteonecrosis of the femoral head. A, A rim of high uptake surrounding an area of lower
uptake in the left femoral head (arrow) demonstrates the typical “donut” pattern of osteonecrosis. B, Scan from
a different patient shows increased uptake in the right femoral head with only a small area of decreased uptake.
114 Section II Diagnostic Techniques
Figure 7–32. Legg-Calvé-Perthes disease. A, Bone scan shows decreased uptake in virtually the entire
right femoral head. There is slight uptake in the lateral column (arrowhead). On the left, there is increased
uptake in the metaphysis of the proximal femur (arrow) and decreased uptake in the femoral head, with no
lateral column uptake. B, Radiograph of the pelvis shows a normal contour of the right femoral head. There
is collapse of the left femoral head.
without high risk of recurrence when there is normal those of open and arthroscopic synovectomy. It is used in
uptake on a bone scan. However, increased uptake may the treatment of hemophilia, rheumatoid arthritis and
be present for years. Serial bone scans are needed to other inflammatory arthritides, and pigmented villonodu-
determine the maturity of heterotopic ossification. lar synovitis. Beta radiation–producing radionuclides are
injected into a joint to destroy the synovial lining, which
RADIONUCLIDES FOR THERAPY causes the inflammation to decrease, or, in hemophilia, to
decrease the bleeding into the joint. The radionuclides
Radiation Synovectomy used for synovectomy are listed in Table 7–1.
Radiation synovectomy is a nonsurgical method of treat- These radionuclides have a soft tissue penetrance of
ing chronic synovitis whose results are comparable to less than 1 cm, which leads to effective radiation of the
Chapter 7 Radionuclide Techniques 115
TABLE 7–1
Radionuclides Used for Synovectomy
Figure 7–34. Calcific tendinitis of the thigh. A bone scan was done because of pain in the left proximal
thigh. The posterior bone scan (A) shows a focal area of increased uptake overlying the proximal femur
(arrow). A CT scan (B) shows calcification in the soft tissues of the thigh in the vastus lateralis or gluteus
maximus tendon (arrow).
116 Section II Diagnostic Techniques
shortly after treatment. The treatment is not useful for Gaucher A, Colomb JN, Naoun A, et al: The diagnostic value
relieving pain from pathologic fracture or nerve or spinal of 99m Tc-diphosphonate bone imaging in transient osteo-
cord involvement. The radioisotopes that have been porosis of the hip. J Rheumatol 6:574, 1979.
approved for this purpose by the U.S. Food and Drug Glithero PR, Grigoris P, Harding LK, et al: White cell scans
and infected joint replacements: Failure to detect chronic
Administration are phosphorus-32, strontium-89, and
infection. J Bone Joint Surg Br 75:371, 1993.
samarium-153. Graham GD, Lundy MM, Frederick RJ, et al: Scintigraphic
detection of osteomyelitis with Tc-99m MDP and
Ga-67 citrate: Concise communication. J Nucl Med
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methylene diphosphonate scintigraphy. AJR Am J Roentgenol 71:641, 1989.
158:599, 1992. Hawkins RA, Choi Y, Huang SC, et al: Quantitating tumor
Alazraki NP: Radionuclide imaging in the evaluation of glucose metabolism with FDG and PET [editorial; com-
infections and inflammatory disease. Radiol Clin North Am ment]. J Nucl Med 33:339, 1992.
31:783, 1993. Holder LE, Schwarz C, Wernicke PG, Michael RH: Radionu-
Algra PR, Bloem JL, Tissing H, et al: Detection of vertebral clide bone imaging in the early detection of fractures of the
metastases: Comparison between MR imaging and bone proximal femur (hip): Multifactorial analysis. Radiology
scintigraphy. Radiographics 11:219, 1991. 174:509, 1990.
Aliabadi P, Tumeh SS, Weissman BN, et al: Cemented total hip Jacobson AF: Bone scanning in metastatic disease. In Collier
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Radiology 173:203, 1989. cine. St Louis, Mosby, 1996, p 87.
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Chapter 7 Radionuclide Techniques 117
Van Nostrand D, Abreu SH, Callaghan JJ, et al: In-111-labeled Radionuclide Scintigraphy in Orthopaedics. New York,
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8
CHAPTER 8
imaging is by using local coils. Ideally, the coil surrounds
Magnetic Resonance Imaging: the entire limb, which is possible for the knee, ankle,
Practical Considerations wrist, and elbow, but not for the hip or shoulder.
Imaging artifacts arise from many sources, including
imperfections in the instrumentation and magnetic fields,
David A. Rubin inherent properties of the mathematics used to recon-
struct the images, and tissue interfaces and foreign bodies.
The most readily preventable source of artifacts, however,
is involuntary patient motion. Carefully positioning
SUMMARY OF KEY FEATURES the patient to ensure comfort prevents motion artifacts
In its relatively short existence, magnetic resonance better than forcibly restraining the examined body
(MR) imaging has emerged as the most powerful part.
imaging technique for the noninvasive diagnosis of
a host of traumatic, degenerative, rheumatologic,
BONE MARROW
infectious, and neoplastic conditions in the
musculoskeletal system. The gross and histologic MR imaging is the most sensitive, noninvasive test for
anatomy of different tissues predicts their MR imaging identifying pathologic states within marrow, whether they
appearances in both healthy and diseased states. be infectious, ischemic, traumatic, or neoplastic.
An understanding of the available pulse sequences
and imaging options enhances a radiologist’s ability
Normal Appearance and Technique
to distinguish normal from abnormal tissues. The true
utility of MR imaging, however, stems from its Hematopoietic or red marrow consists of approximately
depiction of fine anatomic detail. An appreciation of 40% fat, 40% water, and 20% protein, whereas fatty or
these findings, in turn, helps guide the clinical yellow marrow is composed of approximately 80% fat,
management of musculoskeletal conditions. 15% water, and 5% protein. MR pulse sequences can
exploit these differences. Because yellow marrow contains
a higher percentage of fat than red marrow does, yellow
marrow has a shorter T1 and appears brighter on T1-
weighted images. Normally, yellow marrow is isointense
INTRODUCTION with subcutaneous fat on T1-weighted images, whereas
In the past decade, MR imaging has been widely applied the signal intensity of normal red marrow falls between
clinically and has become the study of choice for the that of subcutaneous fat and skeletal muscle (Fig. 8–1A).
diagnosis of many bone, joint, and soft tissue disorders. On T2-weighted images, both yellow marrow and red
The modality provides the radiologist with an unparal- marrow have similar signal intensities and appear hyper-
leled, noninvasive look at the detailed anatomy and subtle intense in comparison to muscle, but less intense than
pathology in the living body, without the use of ionizing water. Yellow and red marrow (of which fat is still a major
radiation. MR imaging can also be cost-effective for constituent) both appear darker when fat suppression is
applications ranging from diagnosing radiographically applied (see Fig. 8–1B). T2-weighted sequences performed
occult hip fractures to reducing the number of unneces- with spectral fat suppression are sensitive to small increases
sary diagnostic knee arthroscopies, staging musculo- in water content and thus easily detect processes that
skeletal infections, and guiding biopsies in patients with replace normal marrow. Short tau inversion recovery
inflammatory myopathies. Its superior accuracy in com- (STIR) sequences, which null the signal from fat based
parison to clinical evaluation has also made MR imaging on its T1 value, are even more sensitive to subtle changes
the optimal technique for the diagnosis of conditions as in marrow composition. Gradient recalled sequences are
diverse as meniscal tears in the knee and disc disease in less useful for examining marrow.
the spine. In the neonate, most of the body’s marrow is hematopoi-
etic (Fig. 8–2). As the child grows and develops, red
marrow in the appendicular skeleton is converted to fatty
TECHNICAL CONSIDERATIONS marrow in an orderly progression, from distal to prox-
The use of MR imaging for the pathologic diagnosis of imal. The conversion begins relatively early in infancy.
musculoskeletal conditions relies on signal intensity and In children, marrow contains a mixture of fat cells and
morphologic changes in the tissues being studied. hematopoietic cells and thus exhibits heterogeneous,
Detecting subtle alterations in these features requires patchy signal intensity on T1-weighted images. Beyond
both high contrast resolution (different signal intensities adolescence, only portions of the axial skeleton and the
in normal and abnormal structures) and high spatial proximal ends of the femora and humeri contain signif-
resolution. To meet these goals, the signal-to-noise ratio icant amounts of red marrow. Even in these regions,
(SNR) of the images must be as high as possible. The though, steady replacement of the hematopoietic elements
best way to increase the SNR in musculoskeletal MR by fat occurs throughout adulthood.
118
Chapter 8 Magnetic Resonance Imaging: Practical Considerations 119
Trauma
Bone marrow can be injured either by a single blunt force
or by repetitive, cumulative insults. A direct blow to a
bone may produce a marrow contusion, or bone “bruise.”
These radiographically occult injuries probably reflect
microfractures of the trabeculae, with resultant marrow
edema and hemorrhage. Common examples include bruises
in the lateral knee compartment experienced after a tear
of the anterior cruciate ligament (ACL) and bruises in the
anterolateral femoral condyle and medial patella suffered
after a patellar dislocation-relocation injury (Fig. 8–5).
On MR images, bone contusions appear as geographic
or reticulated areas of low signal intensity within the fatty
marrow on T1-weighted images. The lesions have high
signal intensity on fat-suppressed, T2-weighted or STIR
images. These findings typically resolve over a period of
6 weeks to 4 months. In addition to bone bruises (which
are essentially microfractures), nondisplaced macroscopic
fractures can be radiographically occult. MR imaging is
exquisitely sensitive to these injuries—more so than bone
scintigraphy—and it has the added benefit of directly
demonstrating the number, completeness, and position
of the fracture plane or planes. Fracture lines appear as
linear areas of very low signal intensity in the marrow
(Fig. 8–6), typically within an area of more general-
Figure 8–4. Effect of radiation therapy. Coronal T1- ized edema. Both T1-weighted images and STIR or fat-
weighted (TR/TE, 572/12) spin echo MR image. After treat- suppressed T2-weighted images can demonstrate fractures.
ment for Ewing’s sarcoma, fatty replacement of the marrow (F) Marrow changes also occur in patients with chronic
has developed in the right side of the sacrum and pelvis. repetitive trauma. Stress fractures, both the fatigue type
Observe the characteristic sharp demarcation (arrows) between and the insufficiency type, demonstrate a pattern of marrow
normal and fatty marrow, which corresponds to the boundary edema surrounding a fracture line. Stress fracture lines
of the radiation port. are usually linear and have low signal intensity on all
Chapter 8 Magnetic Resonance Imaging: Practical Considerations 121
Active osteomyelitis produces decreased signal intensity
on T1-weighted images and increased signal intensity
on T2-weighted or STIR images. However, these signal
characteristics also occur in traumatic, ischemic, and
neoplastic conditions. In some cases, the signal intensity
changes may simply represent marrow hyperemia and
edema in response to an adjacent soft tissue infection or
septic arthritis. Conversely, the lack of marrow signal
changes has a very high negative predictive value. Cortical
destruction, a sinus tract, or an adjacent abscess or ulcer
increases the likelihood that signal changes within the
bone marrow represent active infection. The use of intra-
venous contrast in cases of bone and soft tissue infection
can help stage the process and direct treatment. Within
the infected bone, identifying a nonenhancing, nonviable
region (whether an intraosseous abscess in an acute case
or a sequestrum in a chronic one) may direct treatment
Figure 8–5. Bone bruise from patellar dislocation-relocation toward early surgical débridement in addition to antibiotic
injury. Transverse fat-suppressed intermediate-weighted administration. Similarly, mapping out deep soft tissue
(TR/TEeff, 3500/12) fast spin echo MR image. A high-signal- abscesses for drainage results in faster and more cost-
intensity contusion (arrow) is apparent in the lateral femoral effective treatment of the infection.
condyle. Also note the torn medial patellofemoral ligament For primary bone tumors, radiographs are more useful
(arrowheads). than MR images for lesion characterization. Frequently,
MR imaging cannot distinguish benign from malignant
causes of marrow replacement. Complex, dynamic
gadolinium-enhanced MR techniques have been described
to assess very early enhancement (which favors malignant
over benign lesions), but overlap between the enhance-
ment features of various processes means that the pattern
of enhancement cannot obviate biopsy.
Two other uses of MR imaging for primary bone tumors
are local staging and assessing response to chemotherapy.
For staging, MR imaging accurately demonstrates intra-
osseous tumor extent, soft tissue involvement, and prox-
imity to neurovascular bundles, all of which affect surgical
management. Early investigators suggested that MR imag-
ing could stage primary tumors more accurately than was
possible with computed tomography (CT), but a recent
multi-institutional study found that the two modalities
are comparable for local staging. For gauging response to
neoadjuvant chemotherapy in osteosarcoma and Ewing’s
tumor, standard MR examination is not useful. However,
Figure 8–6. Occult proximal femoral fracture. Coronal T1- assessment of dynamic enhancement patterns may accu-
weighted (TR/TE, 500/12) spin echo MR image. Nondisplaced, rately predict tumor response to neoadjuvant chemotherapy.
low-signal-intensity fracture lines (arrowheads) are visible in the MR imaging is more sensitive than radiography and
left intertrochanteric region of the femur and greater more specific than bone scintigraphy for the detection of
trochanter. The fractures were not visible radiographically. An
intramedullary rod, placed retrograde to treat a previous distal
osseous metastatic disease (Fig. 8–7). Additionally, MR
femoral fracture, produces signal loss in the midfemoral shaft imaging may be more sensitive than bone scintigraphy
(arrow). in a defined anatomic location or in cases of diffuse
metastatic marrow replacement in children. Scintigraphy
is a better screening test for metastases. MR imaging has
pulse sequences. A spectrum of stress injuries exists. One also been used to evaluate the bone marrow of patients
extreme is a stress fracture that has become a complete, with multiple myeloma, lymphoma, and leukemia (see
displaced fracture. At the other extreme are ill-defined Chapters 49 and 51).
regions of marrow edema without visible fracture lines,
which represent radiographically occult “stress responses”
or “stress reactions.” CORTICAL BONE, PERIOSTEUM, AND
ARTICULAR CARTILAGE
Modalities such as radiography and CT, which differen-
Marrow Replacement
tiate tissues on the basis of attenuation differences, are
Alterations in marrow MR signal intensity are sensitive ideal for the imaging of cortical bone. Although periosteal
indicators of osteomyelitis, but they are not specific. new bone formation is also visible radiographically, earlier
122 Section II Diagnostic Techniques
stages of periosteal reaction (before mineralization is facts and not to the underlying ultrastructural composi-
apparent) may be seen with MR imaging. MR imaging is tion of the cartilage. On T1- and proton density-weighted
emerging as the dominant noninvasive modality to assess spin echo images, articular cartilage has intermediate
articular cartilage disorders, whether they result from signal intensity, between that of muscle and fat. On T2-
traumatic, inflammatory, or degenerative causes. weighted images, cartilage has low signal intensity, which
contrasts with the high signal intensity of joint fluid.
Within cartilage, the T2 progressively increases when
Normal Appearance and Technique
moving from the deep to the superficial layers. T2 changes
Cortical (compact) bone has few mobile protons, which reflect variations in water concentration, under the influ-
results in low signal intensity on all pulse sequences. At ence of the surrounding collagen fiber matrix, and, to a
bone–soft tissue interfaces, cortical bone appears artifac- lesser extent, variations in proteoglycan concentration.
tually thicker when using techniques such as gradient On gradient recalled MR images, articular cartilage has
recalled sequences. In the cervical spine, “blooming” of intermediate signal intensity, which becomes progressively
bone size can cause overestimation of the amount of darker close to the subchondral plate. When fat suppres-
foraminal stenosis. Using the shortest possible TE and sion is added to a T1-weighted sequence, the cartilage
higher matrix sizes minimizes the effect. Chemical shift appears bright when compared with the intermediate
misregistration affects the apparent cortical thickness in signal intensity of joint fluid, the dark subchondral plate,
the frequency-encoding direction, and the cortex appears and the suppressed marrow fat.
artifactually thickened on one side and thinner on the
opposite side. This artifact can be reduced by swapping
the phase and frequency encoding, decreasing the field of Traumatic Disorders
view, or increasing the sampling bandwidth. The use of MR imaging can also play a role in fractures that are
fat suppression eliminates chemical shift artifacts without visible radiographically. For example, in tibial plateau frac-
necessitating an increase in bandwidth (which decreases tures, the treating physician often needs to visualize the
the SNR). The periosteum is a thin, fibrous membrane number and orientation of fracture lines, the size and posi-
closely apposed to the nonarticular surfaces of bones. tion of articular fragments, and the status of the articular
Normally, it is not visible on MR images. surface in multiple planes. Although this task can be accom-
The imaging characteristics of normal cartilage reflect plished equally well with reformatted CT examination,
its chemical composition and three-dimensional histologic MR imaging has the advantage of also showing associated
organization. Currently, there is no consensus on the soft tissue injuries such as meniscal tears and ligament
single best articular cartilage sequence. In high-resolution injuries (Fig. 8–8), which have a direct impact on patient
clinical images, a trilaminar appearance may be seen, with management.
the central layer being darker than the superficial and A visible fracture line, periosteal new bone formation,
deep ones. However, in these cases, the layering is mostly or both, distinguish a stress fracture from other stress
due to truncation, magic angle, and chemical shift arti- injuries. The fracture line may be seen in the cancellous
Chapter 8 Magnetic Resonance Imaging: Practical Considerations 123
Inflammatory Lesions
In inflammatory arthritides such as rheumatoid arthritis,
MR imaging is able to show more erosions than can be
seen on standard radiographs. Erosions appear as defects
in the subchondral plate and overlying articular cartilage
and contain high-signal-intensity pannus or joint fluid on
T2-weighted images. Because it can also quantify synovial
inflammation, MR imaging with intravenous contrast
(Fig. 8–10) is an evolving tool for the early diagnosis and
long-term prognostication of patients with inflammatory
arthritis.
Synovial Inflammation
Synovitis is caused by infection, trauma, seropositive or
seronegative arthritis, or miscellaneous conditions such
as hemophilia. The inflamed synovial membrane (called
pannus in inflammatory arthritides) is thicker than normal.
It may be nodular or masslike and, especially in chronic
cases, may shed hypertrophied particles and form rice
bodies (Fig. 8–12). Pannus has variable signal character-
istics, depending on the stage of the disease. In chronic or
Figure 8–11. Baker’s cyst. Transverse T2-weighted (TR/TE,
2500/80) spin echo MR image of the knee. Fluid distends the burned-out cases, the synovium demonstrates low signal
semimembranosus-gastrocnemius recess (B). The neck of the intensity on both T1- and T2-weighted images. With
popliteal cyst is located between the tendons of the medial active inflammation, the MR signal of synovium approx-
gastrocnemius (curved arrow) and semimembranosus (straight imates that of joint fluid on all pulse sequences. Most
arrow) tendons. important, however, is that unlike simple joint fluid,
Chapter 8 Magnetic Resonance Imaging: Practical Considerations 125
strands, joined by a few perpendicular radial tie fibers, to
provide the hoop stress necessary to withstand the force
of the femoral condyle on the tibial plateau. Vessels are
located only in the outer 10% to 30% of the meniscal
periphery.
Normal fibrocartilage has low signal intensity on most
MR imaging pulse sequences. Some important exceptions
exist, however. First, in regions where large numbers of
nearly parallel fibers are oriented obliquely to the main
magnetic field, the magic angle phenomenon may arti-
factually increase the internal signal intensity. Second,
vascularized regions of fibrocartilage may be relatively
hyperintense on MR images. Such regions include the
periphery of the knee menisci and the ulnar attachments
of the triangular fibrocartilage in the wrist.
Several MR pulse sequences can be used for examin-
ing fibrocartilage, and each has unique advantages and
Figure 8–12. Erosive rheumatoid arthritis. Coronal T2- disadvantages. For the knee menisci, proton density-
weighted (TR/TE, 2500/80) spin echo MR image of the wrist. weighted images are the most useful. Spin echo technique
Pannus with heterogeneous signal intensity is present within an is preferred to fast spin echo because of the inherent
erosion of the ulnar head (arrow). Also note the synovitis with blurring that occurs when fast spin echo images are
rice bodies (arrowheads) in the midcarpal and radiocarpal acquired with a short effective TE. Reducing the interecho
compartments. spacing, increasing the matrix size, and decreasing the
echo train length (turbo factor) can make fast spin echo
imaging nearly as sensitive as spin echo imaging for
synovitis enhances rapidly after intravenous contrast meniscal abnormalities while still maintaining a slight
administration. MR imaging can also be used to evaluate advantage in acquisition time. Regardless of the sequence
tenosynovitis in inflammatory arthritides or infections. chosen, sagittal and coronal images should be obtained
In wrists affected by rheumatoid arthritis, MR imaging with slices 3 to 4 mm thick and a field of view of no more
demonstrates more tendon sheath involvement than than 16 cm.
physical examination does and can be used to establish or T1-weighted MR imaging after arthrography with a
confirm a diagnosis of extensor tendon rupture. gadolinium-based contrast agent is most often used in
knees that have undergone meniscal surgery. MR arthro-
graphy is the most sensitive study for examining the labra
Synovial Neoplasm
in the shoulders and hips and probably for the wrist
Proliferative, noninflammatory disorders of the synovium triangular fibrocartilage as well.
can also occur. These include primary synovial (osteo)-
chondromatosis, pigmented villonodular synovitis, and
diffuse synovial lipoma (lipoma arborescens). These lesions Traumatic Disorders
can have a characteristic appearance on MR imaging (see In the knee, two MR imaging criteria are used for diag-
Chapter 71). nosing meniscal tears. The first is the presence of intra-
meniscal signal on a short TE image that unequivocally
extends to one or both articular surfaces of the meniscus
FIBROCARTILAGE
(Fig. 8–13). Hyperintense signal that is contained entirely
Several specialized supporting structures within joints within the meniscus or possibly extends to the surface
are composed of fibrocartilage. Between two articulating should not be interpreted as a tear. Hyperintense signal
bones, they are called discs if they completely separate on T2-weighted images peripheral to the meniscal outer
two joint compartments, and they are called menisci if margin was once thought to represent meniscocapsular
they partly separate compartments. Examples of discs separation, but this finding correlates poorly with true
include the triangular fibrocartilage in the wrists and the pathology seen at arthroscopy. The second criterion is
articular discs in the temporomandibular, acromioclav- abnormal meniscal shape: a tear is present if any alteration
icular, and sternoclavicular joints. Menisci occur in the of the usual cross-sectional triangular or bow-tie shapes
knees and elbows. is noted. These rules do not apply after meniscal surgery.
In a postoperative knee, a recurrent meniscal tear can
be diagnosed confidently only if an abnormality occurs in
Normal Appearance and Technique
a region separate from the initial meniscal tear, if frag-
Fibrocartilage is composed of mostly type I collagen mentation or displaced fragments are seen, or if a fluid-
interspersed with small amounts of elastin, proteoglycans, containing cleft is present within the substance of the
and vascular tissue. The articulating surfaces of the knee meniscus. Intra-articular contrast seen tracking into the
meniscus, for example, are covered by a thin layer of substance of the postoperative meniscus on an MR arthro-
parallel fibers that permit smooth gliding. Conversely, gram is the most specific finding indicating a new menis-
the bulk of the meniscus is composed of circumferential cal tear or a repaired tear that has not healed (Fig. 8–14).
126 Section II Diagnostic Techniques
Nontraumatic Disorders
A discoid meniscus is a meniscus that is larger than normal.
A complete discoid meniscus separates a joint into two
compartments (like a joint disc); in the case of a partial
discoid meniscus, there is still a central opening. Discoid
menisci in the knee are more common laterally than
medially. When compared with normal menisci, they
have an increased propensity to tear. In the elbow, a
Figure 8–13. Full-thickness longitudinal tear of the medial discoid meniscus can impinge between the posterior of
meniscus. Sagittal intermediate-weighted (TR/TE, 2300/20) the capitellum and the radial head, blocking extension.
spin echo MR image. Vertically oriented intrameniscal signal On MR imaging examinations, discoid menisci appear
(arrow) unequivocally contacts the superior and inferior larger than normal and often lack the usual triangular
meniscal surfaces. cross-sectional shape.
With aging, the fibrocartilage in the body can degen-
erate. On MR images, degenerated menisci, labra, and
An acetabular or glenoid labrum that is absent, discs often contain increased internal signal intensity that
detached from the underlying bone (except in the may be globular or linear. Degenerative signal extending
anterosuperior quadrant of the shoulder joint), or frag- to the surface of the structure represents a degenerative
mented represents a torn structure. Tears of the acetab- tear that can be visualized arthroscopically. Chondrocal-
cinosis can also occur within fibrocartilage with aging,
and occasionally, the calcification may be high in signal
intensity on short TE images. In cases in which the calci-
fication extends to the articular surface of a meniscus
(and presumably in a labrum or disc), the MR appearance
may mimic that of a tear.
A para-articular cyst forms when a fibrocartilage tear
(either traumatic or degenerative) creates a channel
through which fluid can travel to the joint periphery.
A one-way valve mechanism allows the cyst to grow.
In the knee, meniscal cysts become symptomatic because
of their size or from entrapment or compression of
surrounding structures. In the shoulder, labral cysts
tend to extend into the spinoglenoid or suprascapular
notches, where they can compress the suprascapular
nerve. On MR imaging studies, cysts may be unilocular
or multilocular and should have high signal intensity on
T2-weighted images. Because the cysts often contain
gelatinous or fibrous material, the contents may be
hyperintense in relation to fluid on T1-weighted images.
The diagnosis of meniscal cyst should be made only if
the cyst abuts a meniscal tear or the site of a healed tear
(Fig. 8–15).
TENDONS
Tendons are highly specialized tissues that provide the
mechanical linkage between muscle and bone. As such,
Figure 8–14. Recurrent meniscal tear after partial medial they are subject to large stresses when a muscle contracts
meniscectomy. Sagittal fat-suppressed T1-weighted (TR/TE,
800/15) spin echo MR image after a knee arthrogram violently. More important, repetitive, submaximal mus-
performed with a dilute gadolinium mixture. Injected contrast cle contractions produce cumulative microscopic tendon
enters the substance of a new meniscal tear (arrow) in the damage that results in a spectrum of tendon abnormali-
remnant of the posterior horn. Also note the degenerative ties ranging from chronic degeneration (tendonopathy)
cartilage loss along the medial femoral surface (arrowheads). to complete tendon rupture.
Chapter 8 Magnetic Resonance Imaging: Practical Considerations 127
angle. Common examples include the supraspinatus
tendon in the shoulder, the flexor and extensor tendons
of the hand, the patellar tendon in the knee, and the
medial, lateral, and anterior tendons of the ankle. The
peroneus brevis tendon in the ankle seems to be the most
frequently affected. To avoid mistaking the increased
signal intensity for pathology, abnormal signal intensity
should be confirmed on T2-weighted images, which are
not affected by the artifact. If the tendon in question is
dark on T2-weighted images, a tendon disorder should
be diagnosed only when morphologic changes in tendon
size or contour accompany increased signal intensity on
the short TE images.
Short-axis cross sections (typically in the transverse
plane) are used to evaluate tendon size, contour, and internal
signal, as well as the surrounding sheath or paratenon.
Longitudinal images provide an overview of the tendon
from the myotendinous junction to the osseous insertion.
These long-axis projections show focal changes in tendon
size, and any retraction of the myotendinous origin is due
to stretching of the tendon. In cases of complete tendon
rupture, longitudinal images demonstrate the size of the
tendon gap, the position of the separated fragments, and
the quality of the torn tendon ends. Gradient echo images
obtained as a three-dimensional acquisition are occasionally
Figure 8–15. Lateral meniscal cyst and recurrent meniscal useful for evaluating tendons that follow an oblique or
tear after partial meniscectomy. Coronal fat-suppressed curved course, such as the finger flexors.
intermediate-weighted (TR/TEeff, 3500/16) fast spin echo MR On MR images, normal tendons are sharply marginated
image after the intra-articular injection of a dilute gadolinium
mixture. A meniscal cyst (C) communicates with a recurrent
and are typically round, oval, or flat in cross section. The
horizontal tear of the meniscus, through which gadolinium radiologist should be familiar with the normal size range
tracks (arrow). The inner tip of the meniscus is absent because of each tendon, which is often determined by comparing
of a partial meniscectomy. tendons in the same part of the body. For example, the
tendon of the normal posterior tibialis is roughly twice as
large as the flexor digitorum longus tendon, whose size
approximately equals that of the flexor hallucis longus
tendon. Throughout its course, a normal tendon should
not change in caliber, except at its insertion, where it may
Normal Appearance and Technique widen into a broad osseous attachment. At the insertion,
Type I collagen forms the bulk of a tendon and is arranged the signal intensity may become heterogeneous as well,
in tightly wound parallel fibers to provide tensile strength. representing an admixture of tendon, fibrocartilage, and
Normal tendon is relatively avascular, and vessels do not ossification.
penetrate the individual tendon fascicles. Tendons that
change course by turning around a bony prominence or
Tendon Degeneration
under a retinaculum or pulley are surrounded by a synovial
sheath. Tendons without a sheath are surrounded by a Tendon degeneration is the main risk factor for tendon
thin, vascular membrane—the paratenon—that provides rupture. At least one third of asymptomatic subjects older
nutrients and allows the tendon to glide. than 35 years show some histologic evidence of degen-
Normal tendons have a very short T2 and thus appear eration. Probably more important than the changes of
uniformly dark on all clinical imaging sequences. There normal aging, however, are the cumulative effects of
is one exception: the magic angle phenomenon can arti- chronic repetitive injury and inefficient repair. Although
factually increase the internal signal intensity of normal the term tendinitis may be applied clinically, the condition
tendons. When a tendon is oriented at approximately is not inflammatory, so the terms tendinopathy or tendinosis
55 degrees with respect to the static magnetic field, T2 are preferred. In clinical practice, the tendons that degen-
decay is greatly retarded, so the intratendinous signal erate most frequently are the rotator cuff, long head of
dramatically increases on short TE (T1-weighted, proton the biceps, extensor carpi radialis brevis, gluteus medius,
density-weighted, and gradient recalled) images. The patellar, posterior tibialis, and Achilles tendons.
critical TE above which there is no contribution from the On MR images, tendon degeneration is recognized by
magic angle phenomenon is 37 msec on both 1.0- and changes in tendon size, contour, internal signal intensity,
1.5-T scanners. For practical purposes, however, the effect or any combination of these factors. The most common
is so slight with TEs of greater than 20 msec that it is not finding is focal or diffuse tendon hypertrophy, which
noticeable. Tendons that travel a curved course in the probably reflects the disordered collagen fiber arrange-
body are the ones most likely to pass through the magic ment. Tendinopathy of the Achilles and patellar tendons
128 Section II Diagnostic Techniques
is typically hypertrophic (Fig. 8–16). Less commonly, as occurred, MR imaging can be used to identify the loca-
degeneration progresses, the tendon loses its viscoelas- tion of the retracted proximal and distal tendon ends
ticity and elongates under the influence of its contracting for surgical planning. After repair, MR imaging may be
muscle. The result is an atrophic tendon that has been needed to evaluate complications, including tendon rerup-
stretched like taffy. This form of degeneration may be ture, and peritendinous scar formation. A normally healed
seen in the posterior tibial tendon (Fig. 8–17). Indistinct- tendon should have a uniform caliber throughout. Nodules
ness of the tendon contour is a second finding in tendino- at the repair site appear as focal bulbous swellings. Recur-
pathy. For example, loss of the usual sharp contour between rent tears are recognized by a gap between the tendon
the posterior fibers of the patellar tendon and the fibers. Once it has matured, peritendinous scar has low
infrapatellar fat in Hoffa’s pad signals early degeneration signal intensity on all sequences and typically obliterates
(see Fig. 8–16). the normal surrounding fat planes.
In tendinopathy, the increased signal on T2-weighted MR imaging is commonly used to diagnose tendon
images should be less intense than that of fluid. Signal ruptures not caused by penetrating trauma. In the lower
that is isointense to fluid or signal that is less intense extremities, these injuries most frequently involve the
but reaches the outer tendon surface probably represents distal quadriceps, proximal hamstring, patellar, and Achilles
macroscopic tearing. Remember, however, that a tendons; in the upper limb, the rotator cuff and biceps
degenerated tendon that is not visibly torn still contains long head are most commonly affected. Whether these
microscopic fiber separation and may be just as tears are preceded by a recognized acute injury or occur
dysfunctional as a ruptured tendon, especially if it has lost spontaneously, one fact is clear: normal tendons do not
its springiness. Further, a degenerated but intact tendon tear. Analysis of nearly 900 “spontaneous” tendon rup-
can still produce pain. To emphasize this, the phrase tures showed that all occurred in histologically diseased
“tendinopathy without a macroscopic tear” is used by tendons, even though two thirds of the patients never
some authors. had previous symptoms. Because tears occur in diseased
tendons, on MR imaging, the signs of a tendon tear are
typically superimposed on those of hypertrophic or atrophic
Tendon Tears
tendinopathy (Fig. 8–18). The signal intensity and mor-
Tendon tears occur after penetrating injury, from stretch- phologic changes that distinguish a low-grade, partial-
ing insults, or spontaneously. Penetrating wounds, typically thickness tear from severe tendinopathy may be subtle,
from glass or knives, produce tendon lacerations that are because the two conditions are points on a spectrum.
usually clinically apparent. The wrists, hands, and fingers Microscopic splitting of collagen strands coalesces into
are most vulnerable. When complete transection has macroscopically recognizable fiber disruption. At this
point, the MR signal within the tendon approaches that
Figure 8–16. Proximal patellar tendinopathy in a profes- Figure 8–17. Atrophic posterior tibial tendinopathy.
sional baseball player. Sagittal intermediate-weighted (TR/TE, Transaxial intermediate-weighted (TR/TE, 2500/20) spin echo
2300/14) spin echo MR image of the knee. The proximal MR image through the ankle. The posterior tibial tendon
patellar tendon is thickened, with increased internal signal (arrow) has a smaller diameter than the flexor digitorum longus
intensity. Note the indistinctness of the posterior margin (arrow) tendon (arrowhead). Normally, the posterior tibial tendon is
when compared with the normally sharp interface seen in the approximately twice as large as the other tendon. F, fibula; T,
lower tendon (arrowheads). talus.
Chapter 8 Magnetic Resonance Imaging: Practical Considerations 129
Figure 8–18. Partial Achilles
tendon tear superimposed on
severe tendinopathy. A, Sagittal
intermediate-weighted (TR/TE,
1000/23) spin echo MR image
of the ankle. The Achilles tendon
(A) is markedly thickened.
B, Transverse fat-suppressed T2-
weighted (TR/TEeff, 3000/63)
fast spin echo MR image. Signal
intensity that is nearly isointense
with fluid (arrow) is seen within
the hypertrophied tendon.
of fluid on a T2-weighted image (see Fig. 8–18). A tendon are at risk for dislocation and subluxation. These injuries
that has split along its long axis appears fragmented commonly involve the long head of the biceps brachii,
in cross section (Fig. 8–19). An abrupt decrease in although dislocation of the peroneal tendons, posterior
tendon diameter is another finding indicating a partial tibial tendon, and extensor carpi ulnaris tendon can occur
tendon tear.
In complete or full-thickness tears, MR images demon-
strate a total loss in continuity of the tendon fibers. T2-
weighted images show a high-signal-intensity defect if
the resultant gap is fluid-filled. However, if scar or granu-
lation tissue fills the gap (which is especially common
after tendon repair), the defect will not necessarily contain
high-signal-intensity material. This appearance is common
in long-standing, massive rotator cuff tears, in which
complete absence of any visible tendon is the pre-
dominant MR imaging finding. MR images also provide
important ancillary information that affects treatment
decisions. Assessment of the quality of the torn tendon
ends is important, because if they are severely degen-
erated, they will probably not hold sutures (Fig. 8–20).
Severe fatty atrophy of muscle associated with a complete
tendon tear indicates chronicity and suggests that although
repair or reconstruction of the tendon may improve pain
or function, it will probably not improve strength.
MR arthrography has a role in the diagnosis of tears in
tendons that form part of the joint capsule, such as the
rotator cuff. Performing MR arthrography increases
sensitivity for the diagnosis of partial-thickness tears of
the tendon surface facing the injected joint. Specificity is
also increased for the diagnosis of full-thickness tears,
which allow injected contrast to extravasate through the
tendon defect. For repaired tendons, MR arthrography Figure 8–19. Sagittal split of the peroneus longus tendon.
differentiates healing or healed repairs from recurrent Transverse fat-suppressed T2-weighted (TR/TEeff, 3000/42)
partial- or full-thickness tears. fast spin echo MR image. The normally oval tendon has lost its
In addition to rupture, tendons that course around a usual smooth contour because of a high-signal-intensity
bony protuberance or run within a fibro-osseous groove longitudinal tear (arrowhead).
130 Section II Diagnostic Techniques
GENERAL CONSIDERATIONS
Although the treatment of tumors of bone and soft tissue
has changed dramatically in recent years because of the
increasing use of limb salvage procedures as an option for
malignant neoplasms, establishing an accurate diagnosis
before instituting therapy remains an important consid-
eration. Whether the procedure is performed directly as
an open biopsy by an experienced orthopedic surgeon or
indirectly as a percutaneous procedure by a qualified radio-
logist, it must be done correctly. A biopsy needle or a scalpel
creates a pathway through which tumor cells can dissem-
inate. Tissues exposed at the time of an incisional biopsy
or during a percutaneous procedure must be removed
later during definitive operative excision of a malignant Figure 9–1. Biopsy with CT guidance in a patient with a
tumor. Clearly, the shortest route to a lesion may not be small lytic lesion in the ilium (arrow). Although a path through
the best one, and care must be exercised in choosing an the gluteal muscles would be more direct, such an approach
appropriate entrance point for either needle aspiration or would violate the tissue planes required for definitive surgery
trephine biopsy. As a general rule for image-guided pro- for a primary lesion.
133
134 Section II Diagnostic Techniques
Figure 9–2. Transpedicular biopsy of the T7 vertebral body. Figure 9–3. Technique of spinal biopsy. A, Lower thoracic
In this 62-year-old man with newly diagnosed prostate and lumbar vertebrae. The needle is inserted 6.5 cm from the
carcinoma, scintigraphy showed a single focus of increased spinous process at a 145-degree angle from the horizontal.
tracer accumulation in the spine. CT demonstrates the tip of B, Upper thoracic vertebrae. The needle is inserted approxi-
the biopsy needle within a small sclerotic lesion (arrows), which mately 4 cm from the midline, over the rib, at a 125-degree
documented the presence of metastasis. angle from the horizontal.
136 Section II Diagnostic Techniques
the instillation of nonbacteriostatic, sterile saline solution complications include paresis, pneumothorax, sinus tract,
should be followed by reaspiration of the joint contents. footdrop, pneumonia, pneumoretroperitoneum, meningitis,
Blood that is aspirated from an osseous lesion should and even death, but these complications are extremely
not be discarded, because it frequently provides an accurate rare. Because of the potential for local or systemic spread
diagnosis. The blood is allowed to clot in a syringe or of tumor during or after the biopsy, the procedure should
plastic cap and then sent to the laboratory as a tissue be accomplished in such a way that the needle tract can
specimen in formalin and processed separately from the be resected along with the specimen.
removed tissue. Smears can also be made from small drops
of blood. If electron microscopy is being considered as an
adjunct to routine histologic analysis, a portion of the FURTHER READING
tissue should be placed in glutaraldehyde, not formalde-
hyde. Less commonly used tissue examinations include Carces J, Hidalgo G: Lateral access for CT-guided percuta-
cytologic studies, tissue imprints, and immunofluorescence. neous biopsy of the lumbar spine. AJR Am J Roentgenol
174:425, 2000.
Craig FS: Vertebral body biopsy. J Bone Joint Surg [Am] 38:93,
COMPLICATIONS 1956.
Image-guided percutaneous biopsy is usually performed Debnam JW, Staple TW: Needle biopsy of bone. Radiol Clin
North Am 13:157, 1975.
without significant complications. Mild pain and discom- Debnam JW, Staple TW: Trephine bone biopsy by radiologists.
fort are common. Hemorrhage can result when biopsy Results of 73 procedures. Radiology 116:607, 1975.
specimens are obtained from patients with vascular tumors Kattapuram SV, Rosenthal DI: Percutaneous biopsy of the
or when a venous or arterial structure is injured, particu- cervical spine using CT guidance. AJR 149:539, 1987.
larly in biopsies of the spine (Fig. 9–4). Other reported Murphy WA, Destouet JM, Gilula LA: Percutaneous skeletal
biopsy 1981: A procedure for radiologists—results, review,
and recommendations. Radiology 139:545, 1981.
Ottolenghi CE: Aspiration biopsy of the spine. J Bone Joint
Surg [Am] 51:1531, 1969.
Pierot L, Boulin A: Percutaneous biopsy of the thoracic and
lumbar spine: Transpedicular approach under fluoroscopic
guidance. AJNR 20:23, 1999.
Robertson WW Jr, Janssen HF, Pugh JL: The spread of tumor-
cell-sized particles after bone biopsy. J Bone Joint Surg [Am]
66:1243, 1984.
Simon MA, Biermann JS: Biopsy of bone and soft-tissue
lesions. J Bone Joint Surg [Am] 75:616, 1993.
Stoker DJ, Cobb JP, Pringle JAS: Needle biopsy of musculo-
skeletal lesions. A review of 208 procedures. J Bone Joint
Surg [Br] 73:498, 1991.
Stoker DJ, Kissin CM: Percutaneous vertebral biopsy: A review
of 135 cases. Clin Radiol 36:569, 1985.
Tehranzadeh J, Freiberger RH, Ghelman B: Closed skeletal
needle biopsy: Review of 120 cases. AJR 140:113, 1983.
III
Imaging and Interventional Procedures
of the Spine
CHAPTER 10
Spinal Imaging routine radiographs to document solid spinal fusion and
3 years to confirm complete remodeling at the fusion site.
Jeffrey S. Ross Pseudarthrosis or fibrous union is a common complica-
tion of spinal fusion. Reabsorption of bone can occur
around screws and under implants where there is direct
contact with bone, and this is usually associated with
SUMMARY OF KEY FEATURES vertebral motion.
A variety of diseases affects the osseous and soft tissue
structures of the spine. Accurate diagnosis is often Myelography
challenging, although a number of imaging methods
are available for this purpose. Of these, magnetic The diagnosis of extradural neural compression by
resonance (MR) imaging is currently receiving the myelography is inferred by changes in the contour of the
greatest attention. normal contrast-filled thecal sac and root sleeves rather
than by direct visualization of the lesion (Figs. 10–1 to
10–3). The current nonionic water-soluble agents used in
myelography are associated with only mild side effects;
significant adverse reactions, such as hallucinations,
INTRODUCTION confusion, or seizures, occur rarely. The major disadvan-
tages of myelography are its invasive nature and lack of
A revolution in the diagnosis and management of spinal diagnostic specificity. The technique of myelography
disorders has occurred in the past 10 to 15 years. Power- involves instillation of a contrast agent through either
ful imaging methods can be applied to delineate the com- lumbar puncture or lateral C1–C2 puncture. Adequate
plexities of pathologic conditions in the spine. MR imaging visualization of spinal structures depends on pooling of a
has quickly emerged as the method of choice for the sufficient amount of contrast agent in the region of
assessment of virtually all disorders of the spine, with interest.
computed tomography (CT) scanning continuing to play Accuracy rates for water-soluble nonionic cervical
a key ancillary role. This chapter reviews the basic imaging myelography in the diagnosis of nerve root compression
approaches to the spine and their usefulness in specific range between 67% and 92%. Because the diagnosis of
disease entities. extradural neural compression is indirectly inferred by
changes in the contour of the contrast-filled subarachnoid
IMAGING TECHNIQUES space, the exact nature of the compressing lesion may be
uncertain. Central indentation of the contrast agent column
Routine Radiography at the level of the disc space may be due to compression
Routine films are universally available and relatively either by the disc itself or by marginal osteophytes.
inexpensive, but they are limited by an inability to allow Similarly, incomplete filling of a nerve root sleeve may be
direct visualization of neural structures and neural com- due to either lateral disc herniation or bony foraminal
pression. The presence of degenerative changes within the narrowing; the distinction is sometimes difficult to make
cervical and lumbar spine has been shown to be related to without the use of ancillary diagnostic methods such as
age, and such changes can be found in both asymptomatic postmyelographic CT.
and symptomatic persons. Twenty-five percent of
asymptomatic patients have degenerative changes in the
Computed Tomography
intervertebral disc spaces by the fifth decade of life. By
the seventh decade of life, 75% have such degenerative CT scanning permits direct visualization of potential
changes. neural-compressing structures and provides better
The goal of spinal fusion is to produce a solid arthrodesis visualization of lateral pathologic entities, such as foraminal
to correct or prevent spinal instability. Bone grafts can be stenosis. CT can also distinguish neural compression by
placed posterior to, posterolateral to, or within the disc soft tissue from compression by bone. CT scanning is
space for anterior interbody fusion or can be used to fill still relatively limited, compared with MR imaging, for
cages to promote such anterior fusion. Instrumentation visualizing the neural structures below a complete myelo-
is used to increase the success rate of bone fusion by graphic block. Disadvantages of CT scanning include the
providing purchase to an individual level and rigid link- effects of partial volume averaging, streak artifacts caused
age to other levels. It usually takes 6 to 9 months for by the dense bone of the shoulder girdle, and changes in
137
138 Section III Imaging and Interventional Procedures of the Spine
reasonable baseline standard of comparison, as long as it Cerebrospinal Fluid Myelogram Effect. Evaluation of the
is acknowledged that these sequences were developed to neural foramina for osteophytes or lateral disc herniation
detect disc herniations and generally perform relatively by two-dimensional imaging is best accomplished in the
poorly in detecting intramedullary cord lesions. The advan- transverse plane. Sequences that lead to high signal
tages of these sequences over T2-weighted spin echo scans intensity of cerebrospinal fluid are generally preferred,
when used for transverse imaging are speed, resolution, because visualizing low-signal-intensity ligaments or
slice coverage, and suppression of cerebrospinal fluid flow osteophytes on T1-weighted imaging in which such fluid
and other motion artifacts. Three-dimensional versions is of low signal intensity creates a diagnostic problem.
of GE scans are available that have both “myelographic” The partial flip angle GE technique for the detection of
and T1-weighted image contrast; they produce favorable cervical disc disease uses a small flip angle (3 to 8 degrees)
image quality, sampling speed, resolution, and multiplanar and provides the best contrast among disc, cord, and
reconstruction. cerebrospinal fluid.
Fast Spin Echo. In routine spin echo imaging, image con- Artifacts. Stainless steel implants are known to generate
trast is controlled by adjustments in TR and TE. New substantial metal artifact with MR and CT techniques.
parameters were added with FSE, such as echo train On CT, metal causes severe x-ray attenuation (missing
length and echo spacing, which can be manipulated to data) in selected planes. These missing data, or hollow
alter image contrast. New artifacts and appearances are projections, cause classic “starburst” or streak artifacts
also created by these techniques, such as T2 filtering during image reconstruction (Fig. 10–6). Materials with
(imaging blurring), bright (i.e., high-signal-intensity) fat, lower x-ray attenuation coefficients (plastic < titanium <
and diminished sensitivity to susceptibility effects. tantalum < stainless steel < cobalt chrome) produce fewer
Because multiple echoes are obtained at different TEs in artifactual distortions. Software strategies for artifact reduc-
the FSE sequence, the overall image has not one true TE tion exist, and thin-section spiral imaging has shown
but an effective TE. improvement over conventional discrete slices.
MR imaging in the presence of stainless steel, though
Fluid-Attenuated Inversion Recovery. FSE is the gold safe, leads to severe artifacts, especially if the steel has a
standard for sagittal T2-weighted and spin density-weighted low nickel content. When metal is placed in an imaging
spinal imaging, driven primarily by the overall image quality field, artifacts are produced by differences in the magnetic
and time savings associated with the technique. Other susceptibility of the metal and adjacent tissue. Magnetic
sequences are available, however, with unique image contrast susceptibility is the phenomenon by which material becomes
that can be useful for intramedullary (cord) disease. For partially magnetized in the presence of an applied external
cord disease, the primary sequence goal is contrast, with magnetic field, and nonferromagnetic metals may produce
resolution assuming much less importance. Fluid- local electrical currents induced by the changing magnetic
attenuated inversion recovery (FLAIR) is a spin echo field of the scanner. When tissues with greatly different
sequence that uses a long inversion time to suppress the magnetic susceptibility are placed within a uniform mag-
signal from cerebrospinal fluid. This pulse, when coupled
with a long TR/TE, gives the benefits of a T2-weighted
image without the interference of high signal from cere-
brospinal fluid. FLAIR has been used with considerable
success for the evaluation of a wide variety of intracranial
disorders. The application of FLAIR in the assessment of
the spinal cord has not been as straightforward as its
application in the assessment of the brain, and a conservative
interpretation of relevant reports suggests that FLAIR
should not be used to evaluate spinal cord disease.
Short Tau Inversion Recovery. Short tau inversion
recovery (STIR) methods have shown high sensitivity for
musculoskeletal disease as a result of the synergistic effects
of prolonged T1 and T2 times in abnormal tissue, coupled
with the improved contrast-to-noise ratio with fat sup-
pression. STIR (especially fast STIR) images may also be
used for the diagnosis of intramedullary disease. Fast STIR
sequences may be best for detecting multiple sclerosis
lesions and may demonstrate lesions missed with other
more routine techniques, such as FSE. Overall image
quality tends to be rather noisy, but an advantage is its high Figure 10–6. Metal artifact with CT scanning. Transverse
contrast-to-noise ratio. This technique appears to be very scan at the L3 level shows a mild amount of streak artifact from
useful for cervical cord disease, but it is prone to motion the right-sided posterior rod, as well as from the tips of the
artifact and has shortcomings in the evaluation of thoracic pedicle screws within the vertebral bodies. Nevertheless, the
cord disease. thecal sac is well defined, as are the roots of the cauda equina
after intrathecal contrast administration.
Chapter 10 Spinal Imaging 141
netic field, the difference in susceptibility causes distor- imaging and CT. Spinal angiography is extremely useful
tion in the magnetic field, which results in distortion on in evaluating spinal vascular malformations, both for
the MR image. A number of strategies can be used to delineation of vascular supply and for therapeutic treat-
reduce susceptibility artifacts on MR imaging, including ment (Fig. 10–8). Spinal angiography is also used in the
the use of spin echo techniques, especially FSE and its pretherapeutic workup of suspected vascular neoplasms
variants, over GE; larger fields of view; higher readout involving the vertebral bodies, posterior elements, and
bandwidths; smaller voxel sizes; and appropriate geo- spinal canal and is coupled with preoperative or palliative
metric orientation of the frequency-encoded direction in embolization.
relation to metallic objects. Imaging at lower field strength
also reduces susceptibility artifact but is not an operator-
Discography
selectable parameter in routine practice. Geometric orien-
tation of the metallic object with respect to the magnetic Discography was originally conceived as a technique for the
field is especially important in the case of pedicle screws study of disc herniation. Today, it is considered a useful
(Fig. 10–7). Less apparent widening of the short axis of but limited test that relies on pain provocation to determine
screws is seen when the direction of the frequency- the anatomic source of a patient’s symptoms. By one defi-
encoded gradient is parallel as opposed to perpendicular nition, discography is a physiologic evaluation of the disc
to the long axis of the screw. that consists of volumetric, manometric, and radiographic
studies plus a pain provocation challenge. This procedure
remains quite controversial and has generated volumi-
Spinal Angiography
nous literature. Discography is an invasive procedure and
Spinal angiography is no longer used for the initial assess- is not performed as a screening technique. The main
ment of spinal column disease, given the power of MR complication associated with this technique is disc space
infection. The risk of discitis is on the order of 0.1% to
0.2%; therefore, a prophylactic broad-spectrum antibiotic
is often administered.
Figure 10–9. Type I vertebral endplates. Sagittal T1-weighted spin echo MR image (A) of the lumbar
spine shows regions of low signal within the adjacent endplates at the L4–L5 level. These regions have
increased signal intensity on a sagittal T2-weighted image (B). The L4–L5 intervertebral disc is
degenerated, with a decrease in disc space height and loss of disc signal in (B). Note the recurrent disc
herniation at this level.
144 Section III Imaging and Interventional Procedures of the Spine
Figure 10–10. Type II vertebral endplates. Sagittal T1-weighted (A) and T2-weighted (B) spin echo MR
images of the lumbar spine show signal intensity changes at the L4–L5 level that are typical of a type II
endplate. The signal intensity of subchondral bone at this level is identical to that of fat. There is also
evidence of degeneration of the intervertebral disc at this level, with a decrease in disc space height and loss
of disc signal on the T2-weighted image.
anulus. The extrusion remains attached to the parent nuclear Spinal Stenosis
material. A reasonable imaging definition of extrusion is Spinal stenosis refers to narrowing of the central spinal
a situation in which the diameter of the disc material canal, neural foramina, or lateral recesses. Most commonly
beyond the interspace is wider than the bridge, if any, it is acquired secondary to degenerative disease of the
that connects it to the disc of origin. A free (sequestered) intervertebral disc, articular facets, or both structures,
disc fragment is an extrusion that is no longer contiguous although developmentally shortened pedicles are an impor-
with the parent disc. A free fragment may be located at tant component of symptomatic spinal stenosis in patients
the disc level or may migrate superiorly or inferiorly. with otherwise mild degenerative changes (Figs. 10–12
Chapter 10 Spinal Imaging 145
and 10–13). MR imaging accurately depicts the degree
and cause of thecal sac narrowing in patients with central
canal stenosis. Such narrowing is most commonly related
to bony and ligamentous hypertrophy.
In addition to central canal stenosis, stenosis of the
lateral recess is an important cause of lower extremity pain
and paresthesias. The lateral recess is bordered anteriorly
by the posterior aspect of the vertebral body and disc,
laterally by the pedicle, and posteriorly by the superior
articular facet. The root sleeve within the lateral recess is
often compressed by bony hypertrophy of the superior facet,
frequently in combination with disc bulging and osteo-
phytes along the anterior border of the lateral recess.
Facet Disease
Degenerative disease of the facet joints typically occurs in
combination with degenerative disc disease, although
facet disease alone may be responsible for symptoms of
pain and radiculopathy. Because of the richly innervated
synovium and joint capsule, these changes alone can be a
source of pain; alternatively, they can contribute to nerve
root impingement by causing spinal stenosis or foraminal
compromise. On MR images, degenerated facets appear
hypertrophied, sclerotic, and irregular. Enlarged ligamenta
flava are commonly present. Facet degeneration can lead
to the formation of synovial cysts, which can compress the
thecal sac and roots from a posterior direction. Synovial
cysts are best depicted on transverse MR images and
appear as posterolateral epidural masses adjacent to a
B
Figure 10–13. Lumbar canal stenosis. A, Sagittal T2-
weighted fast spin echo MR image of the lumbar spine shows
severe central canal stenosis at the L3–L4 and L4–L5 levels.
This stenosis is related to a combination of bulging of the
Figure 10–12. Severe bony central canal stenosis. Transverse anulus fibrosus anteriorly and degenerative facet and liga-
CT image at the L3–L4 level demonstrates marked central mentous changes posteriorly. Note the tortuous nerve roots
canal stenosis related primarily to hypertrophic degenerative and intradural vessels superior to the site of canal stenosis. B,
changes of the facet joints and their mass effect on the posterior Transverse T2-weighted spin echo MR image confirms the
margin of the thecal sac. Severe degeneration of the disc is severe nature of the canal stenosis, with nearly no recognizable
manifested as a vacuum phenomenon. thecal sac.
146 Section III Imaging and Interventional Procedures of the Spine
years after surgery, so the amount of time that has elapsed subsequent disc destruction causes a loss of disc space
since surgery should not dissuade one from using intra- height. As the infection spreads to the adjacent vertebral
venous contrast material in the postoperative state. Disc endplates, routine radiographs show characteristic irregu-
material does not enhance on early postinjection images larity of bone. Hematogenous spread also occurs in adults,
because such material is avascular (Fig. 10–18). The even though the disc has lost a great deal of its vascularity.
addition of fat suppression to gadolinium-enhanced T1- The seeding of microorganisms occurs in the vascularized
weighted images improves the visualization of enhancing endplate, with the disc and opposite endplate becoming
scar; this method aids in differentiating scar from infected secondarily.
recurrent herniated disc material and more clearly shows Because radiographic abnormalities are usually delayed
the relationship of scar to the nerve roots and thecal sac. for days to weeks, radionuclide studies have been the tra-
ditional imaging method for the early diagnosis of vertebral
osteomyelitis. The radionuclides most commonly used to
Spinal Stenosis
detect inflammatory changes of the spine are technetium-
Spinal stenosis related to osseous abnormalities has been 99m–labeled phosphonates, gallium citrate, and indium-
implicated as a cause of failed back surgery in up to 60% 111–labeled white blood cells. Indium-111 has several
of cases. Various mechanisms can lead to stenotic changes advantages over the other radionuclides, including higher
in the canal or foramina (Table 10–3). Many of these target-to-background ratios, better image quality (com-
stenoses are not symptomatic. pared with gallium), and more intense uptake by abscesses.
Its main disadvantage is its accumulation within any
inflammatory lesion, whether infectious or not. MR imaging
Arachnoiditis
appears to have a sensitivity that exceeds that of radiographs
Arachnoiditis can be classified into three categories, or and CT and approaches or equals that of radionuclide
patterns, and this method of classification can be applied studies. T1-weighted spin echo images can detect the
to MR imaging, CT, and myelography. The first pattern increased water content or marrow fluid seen with inflam-
is one of central adhesion of the nerve roots within the matory exudates or edema. Like most pathologic processes,
thecal sac, leading to a central “clump” of soft tissue signal. disc space infection or vertebral osteomyelitis results in
This pattern is most easily identified on transverse images. increased signal intensity on T2-weighted images. The
The second pattern is adhesion of the nerve roots to the diagnostic specificity of MR imaging is provided by the
meninges, which gives rise to an “empty” thecal sac. In signal intensity changes on T1- and T2-weighted images,
the third pattern, which can be considered the end stage as well as by the anatomic pattern of disease involvement
of the inflammatory response, arachnoiditis appears as an and the appropriate clinical data.
inflammatory mass that fills the thecal sac. On myelogra-
phy, this type of arachnoiditis gives rise to a spinal block
with an irregular “candle-dripping” appearance. MR imag- Pyogenic Disc Space Infection
ing shows a nonspecific-appearing soft tissue mass, as
On T2-weighted images, the normal intervertebral disc
does CT-myelography (Figs. 10–19 and 10–20).
usually shows increased signal intensity within its central
portion, that is bisected by a thin horizontal line of decreased
INFECTION signal, termed the intranuclear cleft. After age 30 years,
the cleft of normal intervertebral discs is seen almost uni-
Epidemiology
versally. On T1-weighted MR images, disc space infections
Sources of the bacterial seeding that causes vertebral typically produce decreased signal intensity of the adjacent
osteomyelitis include genitourinary, dermal, and respira- vertebral bodies and the involved intervertebral disc space.
tory infections. In children, the bacteria (or other microor- A well-defined vertebral endplate cannot be defined. T2-
ganisms) find their way to the vascularized disc, and weighted images show increased signal intensity in the
Chapter 10 Spinal Imaging 149
Figure 10–18. Recurrent lumbar disc herniation. This patient had undergone laminectomy at the L3–L4
and L4–L5 levels. Sagittal T1-weighted spin echo MR images obtained before (A) and after (B) intravenous
gadolinium administration and a sagittal T2-weighted fast spin echo MR image (C) all demonstrate a large
soft tissue mass within the anterior epidural space at the L4–L5 level contiguous with the disc space. This
mass shows peripheral low signal intensity in (C) and patchy enhancement in (B). These findings indicate
the presence of a recurrent disc herniation associated with a large amount of granulation tissue.
Figure 10–21. Disc space infection. Sagittal T1-weighted spin echo MR image (A), sagittal T2-weighted
fast spin echo MR image (B), and sagittal T1-weighted spin echo MR image obtained after intravenous
gadolinium administration (C) demonstrate loss of the endplate margins at the L3–L4 level, associated with
high signal within the adjacent vertebral bodies in (B) and enhancement of signal intensity within the disc
space itself in (C). These findings are consistent with a pyogenic disc space infection. An epidural phlegmon
is seen as a mass of homogeneously enhancing tissue posterior to the L3 vertebral body that is effacing the
thecal sac.
The patterns of Gd-DTPA enhancement of an epidural intramedullary tumor in adults. Astrocytomas produce a
abscess include (1) diffuse and homogeneous enhancement, focal enlargement and, occasionally, an exophytic growth
(2) heterogeneous enhancement, and (3) thin peripheral involving the cord. Imaging demonstrates fusiform
enhancement. Gd-DTPA–enhanced imaging is a useful enlargement of the cord that extends over several seg-
adjunct for identifying the extent of a lesion when the non- ments, and T2-weighted MR images show increased signal
contrast MR images provide equivocal results regarding intensity, reflecting both tumor and edematous cord.
the activity of an infection. Leptomeningeal infections in Although ependymomas may involve any portion of the
the lumbar spine are often secondary to spread from an cord, they most frequently involve the conus medullaris
intracranial source. Intravenous gadolinium administration and filum terminale. A typical appearance is that of an
is essential in suspected cases and shows irregular or intradural extramedullary mass involving the filum ter-
linear intradural enhancement. minale and cauda equina, although the tumor can appear
as fusiform enlargement of the cord itself. Cervical
intramedullary tumors may be seen in patients with neu-
NEOPLASMS rofibromatosis type 2. These tumors typically enhance
Neoplasms involving the lumbar spine are classified by following intravenous gadolinium administration and
the precise site of involvement into one of three may have intratumoral cysts.
categories: intramedullary, intradural extramedullary, or
extradural compartment. Inflammatory Myelopathy. The various causes of inflam-
matory myelopathies are legion and include multiple
sclerosis, postviral demyelination, viral infection, pyogenic
Intramedullary Lesions infection, and granulomatous disease. The archetypal
Tumors. The most common intramedullary neoplasms inflammatory lesion is multiple sclerosis. In this disease,
are the gliomas, principally astrocytomas and ependy- abnormalities in the spinal cord lead to severe motor dis-
momas. Ependymomas are cited as the most frequent ability, yet imaging of the spinal cord has always been
152 Section III Imaging and Interventional Procedures of the Spine
Figure 10–22. Epidural abscess. A, Sagittal T1-weighted spin echo MR image is remarkable for subtle
but abnormal signal located diffusely within the thecal sac. B, Sagittal T2-weighted fast spin echo MR image
shows a lobulated mass of high signal intensity along the posterior epidural space and extending from the
S1 to the T12 levels. C, These findings are confirmed on a sagittal T1-weighted spin echo MR image obtained
after intravenous gadolinium administration by the demonstration of patchy and irregular enhancement
within the extensive posterior epidural abscess.
subordinate to imaging of the brain in clinical investigations mately 55% of all primary spinal tumors. The great
of multiple sclerosis. Most focal plaques are less than two majority of these tumors are benign, with nerve sheath
vertebral body lengths in size, occupy less than half the tumors and meningiomas being the most common lesions.
cross-sectional diameter of the cord, and are characteris- Nerve sheath tumors are the most frequent intraspinal
tically peripherally located. Approximately 60% to 75% tumors and are histologically divided into two types:
of spinal cord lesions in multiple sclerosis are present in schwannomas (also known as neuromas, neurinomas, and
the cervical region, and more than half of patients with neurilemomas) and neurofibromas. Most intraspinal
multiple sclerosis and cord plaques have multiple plaques. nerve sheath tumors are solitary schwannomas, whereas
Of patients with cord plaques, 90% have intracranial neurofibromas are almost always associated with neurofi-
plaques as well. bromatosis type 1. Patients with neurofibromatosis type
2, however, more commonly have multiple schwannomas
Vascular Abnormalities. The main arteries that supply the rather than neurofibromas. Nerve sheath tumors are
spinal cord include the single anterior spinal artery and the easily recognized on MR imaging as typically isolated,
paired posterior spinal arteries. These arteries are contiguous well-circumscribed, solid masses of intermediate signal
along the longitudinal length of the cord. Contributions intensity on T1-weighted images, surrounded by low-
from radiculomedullary arteries occur at multiple levels, with signal cerebrospinal fluid. On T2-weighted images, they
the best known and largest being the artery of Adamkiewicz. have variable signal intensity. Schwannomas are more
Cord infarction can be caused by a variety of abnormali- vascular and undergo cystic degeneration, necrosis, and
ties, but it often relates to embolic disease such as that hemorrhage more commonly than do neurofibromas. A
occurring after aortic aneurysm repair. MR findings are variety of local osseous changes, consisting mainly of
nonspecific in many of these vascular abnormalities but smooth bony remodeling or foraminal enlargement, is
include high signal intensity on T2-weighted images. Cord common. Enhancement following intravenous gadolinium
enlargement can sometimes be seen on T1-weighted administration is almost always present, but the pattern is
images. Focal atrophy of the cord is a late finding. variable.
Meningiomas most commonly occur in the thoracic
spine. As is the case intracranially, these tumors have a
Intradural Extramedullary Lesions female sex predilection, and the lesions occur in a slightly
Intradural extramedullary neoplasms are the largest single older age group compared with nerve sheath tumors. The
group of primary spinal neoplasms and account for approxi- great majority are entirely intradural and are typically
Chapter 10 Spinal Imaging 153
isointense to the neural elements on T1- and T2-weighted Thoracolumbar Spine
images. Meningiomas enhance intensely after Gd-DTPA The main area of involvement in the injured lumbar spine
administration, which may allow demonstration of the is the thoracolumbar junction, which acts as a fulcrum for
typical broad dural base. spine motion and is susceptible to unstable traumatic
The last category of intradural extramedullary lesions injuries. The thick, sagittally oriented facets minimize rota-
is the so-called leptomeningeal pattern, which includes tional injury, but injuries related to flexion and transverse
leptomeningeal metastatic disease and benign granulo- loading often occur. The forces may combine to produce
matous processes such as sarcoid and tuberculosis. The flexion-compression injuries or the so-called burst
litany of tumors that may seed cerebrospinal fluid is long, fracture. CT remains the method of choice for detecting
but the main offenders are cranial ependymomas, glioblas- retropulsed bony fragments and for demonstrating
tomas, and medulloblastomas (especially in the pediatric fractures of the posterior elements. A hyperflexion injury
population). It must be remembered that the overall sen- occurring in the lumbar spine—the seat-belt or Chance
sitivity of MR examination is low in patients with proven fracture—is associated with motor vehicle accidents
histologic evidence of neoplastic seeding, so aspiration and involving rapid deceleration. This type of injury is charac-
study of the cerebrospinal fluid remain the gold standard. terized by a horizontal fracture through both the anterior
and posterior elements.
Although CT is more sensitive than MR imaging in
Extradural Lesions
detecting post-traumatic bony abnormalities, the latter is
Primary and secondary tumors in the extradural space are often superior for evaluating injured soft tissue structures.
well evaluated by both MR imaging and CT. Metastatic In particular, the disrupted spinal ligaments show focal dis-
disease to the spine is by far the most common type of continuity on T1-weighted images and areas of increased
extradural tumor. Because of its high contrast sensitivity signal intensity on T2-weighted images. The anterior and
and spatial resolution, MR imaging is the examination of posterior longitudinal, flaval, interspinous, and supraspinous
choice for the detection of osseous metastases. Tumor ligaments can all be evaluated, often most favorably in the
replacement of fatty marrow is depicted on T1-weighted sagittal plane. Sagittal and transverse images allow the detec-
images as focal or diffuse areas of low signal intensity, and tion of retropulsed bony fragments and narrowing of the
such depiction occurs much earlier than can be achieved spinal canal. MR imaging excels in the detection of cord com-
with plain radiography or bone scintigraphy. Because pression resulting from bony fragments (see Chapter 58).
many metastatic tumors enhance, the routine use of
contrast-enhanced studies alone (i.e., without fat
suppression) is not recommended; the resulting signal Epidural and Subdural Hemorrhage
intensities of metastases and normal marrow fat are Epidural spinal hematomas occur most frequently in the
similar, occasionally to the point of masking even large elderly, but they can occur at any age. Clinically, sudden
metastatic lesions. back or neck pain develops that may be radicular in nature.
Signs of cord compression can develop immediately or
within days. Epidural spinal hematomas are broadly classi-
TRAUMA fied into two groups: nonspontaneous and spontaneous.
Nonspontaneous epidural spinal hematomas may result
Cervical Spine
from spinal taps, spinal anesthesia, trauma, pregnancy,
Radiographs remain the initial study for screening or bleeding diathesis, anticoagulant therapy, spinal heman-
“clearing” the cervical spine in cases of trauma. The gioma, vascular malformation, hypertension, and neoplasm.
number of views considered adequate for a trauma series The history is often revealing, but it may consist of
varies between three and five (anteroposterior, lateral, merely an episode of sneezing, bending, voiding, turning
and open-mouth odontoid views, with or without supine in bed, or mild trauma. Epidural spinal hematomas can
right and left oblique views). A single lateral view is inade- be localized or can spread anywhere along the spinal
quate, and all seven cervical vertebrae must be visualized. column (Fig. 10–23).
Radiographs are also of primary importance in defining Subdural hemorrhage is capable of producing severe
spinal instability in patients with persistent pain or soft tissue and irreversible neurologic deficits, and acute surgical
swelling but without a definite fracture on the initial plain intervention may be needed. Subdural spinal hematomas
film evaluation. often have a typical configuration. As opposed to epidural
CT is indicated in the evaluation of the traumatized hematomas, which tend to be capped by fat, subdural
cervical spine to further assess definite or possible fractures hematomas are located within the thecal sac and are sepa-
revealed on plain films and to evaluate areas inadequately rate from the adjacent extradural fat and the posterior
seen on plain films. Some studies recommend CT as the elements of the vertebral bodies (Fig. 10–24). Transverse
primary screening method in patients with multiple areas images are useful in defining the epidural fat surrounding
of spinal trauma. MR imaging allows direct visualization the thecal sac and in delineating the blood in the interior
of post-traumatic cord abnormalities that cannot be of the sac in subdural hematomas. Subdural hematomas
defined by any other imaging method, and it can define should not extend into the neural foramina; such extension
intramedullary hematoma, intramedullary edema and con- is more typical of an epidural hematoma. Acute hemorrhage
tusion, disc herniation, ligamentous injury, and epidural can be fairly isointense on T1-weighted images but, over
hemorrhage. Traumatic injuries are discussed in detail in a few days, shows high signal on T1-weighted images.
Chapter 58. T2-weighted spin echo or GE images are important in
154 Section III Imaging and Interventional Procedures of the Spine
Chiari Malformation
The Chiari type I lesion consists of caudal descent of the
cerebellar tonsils. The Chiari type II malformation consists
of caudal descent of the cerebellar vermis, fourth ventricle,
and lower part of the brain stem and is almost always
associated with a myelomeningocele. Type III Chiari malfor-
mation is an occipital encephalocele of the cerebellum,
whereas a type IV lesion is cerebellar hypoplasia (and not
herniation). Clinical symptoms in the type II lesion are
varied, but brain stem symptoms develop in one third of
patients with a myelomeningocele by 5 years of age.
Syrinx
Hydrosyringomyelia is a term used to describe cavitation
of the spinal cord. Syringes are seen in approximately
50% to 75% of Chiari I malformations and 50% to 90%
Figure 11–2. Synovial cyst. A, Postero-oblique view of an opacified facet joint demonstrates a
posteroinferior recess of moderate size and a diverticular extension posteromedially. B, In a transverse CT
image obtained after the intravenous injection of a contrast agent, note the peridiscal enhancement and
peripheral enhancement of bilateral synovial cysts about the facet joints that have caused central spinal
stenosis. Osteoarthrosis is present in both facet joints.
between a transitional lumbar vertebra and the sacrum midsubstance of the piriformis muscle, with soft tissue
can also be confirmed with anesthetic injections. extravasation and perisciatic neural leakage, is nonselective
and of limited value. Anecdotal evidence suggests that
selective anesthetic blockade, combined with local steroid
Piriformis Muscle Injection injection at the superficial and deep margins of the distal
The sciatic nerve may be compressed as it leaves the pelvis end of the tendon at its trochanteric attachment site, can
through the greater sciatic foramen by the piriformis resolve symptoms associated with overuse.
muscle, and the resultant pain may be increased by muscular
contraction, palpation, or prolonged sitting. Diagnostic
Epidural Injection
testing can be used to differentiate piriformis syndrome
from other causes of sciatica, lower extremity weakness, Epidural injections used to treat back and limb pain are
and pain. Piriformis muscle injections are performed under generally accomplished through a posterior interlaminar,
CT guidance. Without such guidance, injection into the caudal, or transforaminal route. All three techniques are
Chapter 11 Interventional Spinal Procedures 161
capable of delivering steroid preparations to the epidural position of 30 to 40 degrees, a straight needle can be
space. The interlaminar and caudal methods are imprecise, directed under fluoroscopic control to the base of the
require substantial volumes of injectant with larger doses pedicle at its junction with the vertebral body beneath the
of steroids, and may necessitate a series of injections that transverse process. With the patient prone, manipulation
are expensive and more likely to lead to complications. of the needle tip around the facet and pedicle requires a
With the caudal injection technique, the injected solution needle in which the last centimeter is curved away from
dissipates mainly in a cephalad direction and seldom crosses the bevel (Fig. 11–4). To reduce the chance of inadvertent
the L5–S1 disc space (Fig. 11–3A). The transforaminal dural puncture, the needle tip should not extend beyond
route provides precise delivery of a smaller dose of corti- the midline of the pedicle. In the upper lumbar levels, the
costeroids under fluoroscopic guidance. The medication perineural arachnoid sleeve or cysts may extend more
is injected predominantly in the anterior epidural space laterally (Fig. 11–5).
(see Fig. 11–3B). Access to the S1 nerve root can be obtained through
Epidural injection of corticosteroids can be used to treat the small posterior sacral foramina; alternatively, a straight
patients with certain radicular pain syndromes of the lower needle can be passed directly through the thin posterior
extremity of intermediate duration (2 weeks to 3 months). cortical bone overlying the S1 nerve root canal just below
The short- and long-term benefits of repeated epidural the S1 pedicle. The patient frequently feels some radicular
or perineural steroid injections have been evaluated in pain when the needle approaches the nerve. When the
patients with persistent radicular or low back pain for needle tip is in the proper position, or when the patient
more than 6 weeks, and long-term improvement has been indicates that typical symptoms have been reproduced,
reported in more than half of patients. A highly variable contrast medium is injected. Antegrade flow of the contrast
success rate (23% to 84%) has been reported for epidural material outlines the exiting nerve root. Retrograde flow
injection. of the contrast agent extends in a subpedicular direction
to the ventral epidural space and to the targeted extrathecal
Selective Lumbosacral Nerve Root Block with Focal disc space. Fluoroscopy with the patient in a lateral or
Transforaminal Injection. After sterile preparation and oblique position ensures accurate placement of the needle.
anesthesia of the skin, a 22-gauge short bevel spinal needle Enough contrast agent should be injected for the operator
is inserted at a point just lateral to the pedicle and below to recognize intravascular and intrathecal flow. When
the transverse process. With the patient in a prone oblique correct needle position is confirmed and the desired flow
Figure 11–4. Epidural injection. A, High L5 transforaminal needle placement has resulted in antegrade
and retrograde flow of contrast material outlining nerve root divisions and the foraminal floor, with midline
flow of contrast agent to the L4–L5 disc margin. B, Lateral radiograph confirms the location of the tip of
the curved needle, along with retrograde ventral epidural flow of contrast material to the craniad disc space.
Figure 11–8. Lumbar discography. A, Lateral lumbar spine with discographic needle entry low in the
posterior disc margin. Note the normal unilocular appearance of the nucleogram. B, Normal bilocular
appearance of the nucleogram. The anterior arrows identify anterior vacuum phenomena in the anulus
fibrosus, consistent with peripheral annular tears that were asymptomatic at discography.
Percutaneous Discectomy
Percutaneous discectomy has gained some popularity as a
treatment method for an extruded lumbar nucleus pul-
posus. The reason why percutaneous discectomy is suc-
cessful is unclear, and no pathophysiologically based
explanation is available. There does not appear to be a
correlation between the quantity of disc removed and the
therapeutic result. Infection is an infrequent compli-
cation of percutaneous discectomy, with a prevalence of
less than 1%. Morbidity and mortality rates related to
this procedure are low. Success rates in carefully chosen
patients are between 53% and 87%, compared with 90%
Figure 11–9. Lumbar discography. Lateral examination shows in appropriately selected patients undergoing surgical
a normal L3–L4 bilocular nucleogram. The L4–L5 discogram microdiscectomy.
shows a posterolateral incomplete radial tear with annular
enhancement.
Laser Disc Therapy
Laser disc therapy entails vaporization of nuclear tissue
to relieve or reduce the mechanical deformation effect on
Chemonucleolysis the disc contour. The success of this therapy depends on
The role of chemonucleolysis in the management of leg proper patient selection and correct needle placement
pain related to disc extrusion is controversial. Nucleolysis with appropriate radiologic monitoring. The needle tip
is produced by the injection of a proteolytic or chon- must be placed just inside the anulus fibrosus, and the
drolytic enzyme, such as chymopapain or collagenase, needle itself must be parallel to the axis of the disc and
into the substance of a deranged intervertebral disc. Initial preferably halfway between the superior and inferior
success rates were between 75% and 85%. Although endplates.
Chapter 11 Interventional Spinal Procedures 165
Thermocoagulation of the Intervertebral The technique involves percutaneous puncture of the
Disc involved vertebrae via a transpedicular approach, followed
Intradiscal electrothermal annuloplasty is a relatively new by injection of polymethylmethacrylate (average injection
procedure to relieve discogenic pain. It involves heating amount, 7.1 mL) into the vertebral body (Fig. 11–10).
the intervertebral disc using a specialized flexible catheter Significant pain relief immediately after treatment has
with a 6-cm thermoactive tip. Using normal discographic been reported in 90% of patients, with a complication rate
technique, the catheter is inserted via a posterolateral route of about 6%. Careful control of the volume of injected
into the anulus fibrosus or nucleus pulposus with a 17- polymethylmethacrylate seems to be the most critical
gauge introducer. The active tip is typically advanced cir- factor for avoiding complications. Injection of opacified
cumferentially around the nuclear tissue and directed polymethylmethacrylate during vertebroplasty may cause
circuitously to return posterolaterally, ideally achieving compression of adjacent structures and necessitate emer-
full 360-degree penetration. After appropriate catheter gency decompressive surgery; thus, the procedure should
positioning, electrothermal heat is generated at the active be performed only in a surgical center. A multidiscipli-
tip. Approximately one third of patients feel the same or nary team decision to perform vertebroplasty is based on
worse after undergoing electrothermal annuloplasty. a number of factors.
Many questions remain regarding the efficacy of this
procedure.
FURTHER READING
Percutaneous Vertebroplasty
Boas RA: Nerve blocks in the diagnosis of low back pain.
Osteoporosis is a disease of low bone mass that often mani- Neurosurg Clin N Am 2:807, 1991.
fests as a fracture. Hip fractures are the most devastating, Boden P, Davis DO, Dina TS, et al: Abnormal magnetic-resonance
but vertebral fractures are the most common and occur scans of the lumbar spine in asymptomatic subjects: A prospec-
in 25% of women older than 50 years and in 40% of those tive investigation. J Bone Joint Surg Am 72:403, 1990.
80 to 85 years of age. Sixty percent of vertebral fractures Burton AK, Clarke RD, McClune TD, et al: The natural
are clinically silent but are associated with loss in height history of low back pain in adolescents. Spine 21:2323, 1996.
Bush K, Cowan N, Katz DE, et al: The natural history of
and spinal deformity. In patients in whom pain manage- sciatica associated with disc pathology: A prospective study
ment with local and systemic analgesia has failed, verte- with clinical and independent radiologic follow-up. Spine
broplasty can be effective in controlling pain, obtaining 17:1205, 1992.
stability of the spine, improving function, and decreasing Carette S, Marcoux S, Truchon R, et al: Controlled trial of
the likelihood of recurrence, progressive deformity, and corticosteroid injections into facet joints for chronic low
disability. back pain. N Engl J Med 325:1002, 1991.
Cotten A, Boutry N, Cortet B, et al: Percutaneous vertebro- Kinard RE: Diagnostic spinal injection procedures. Neurosurg
plasty: State of the art. Radiographics 18:311, 1998. Clin N Am 7:151, 1996.
El-Khoury GY, Renfrew DL: Percutaneous procedures for the Kirkaldy-Willis WH, Burton CV (eds): Managing Low Back
diagnosis and treatment of lower back pain: Diskography, Pain, 3rd ed. New York, Churchill Livingstone, 1992, p 63.
facet-joint injection, and epidural injection. AJR Am J Kraemer J, Ludwig J, Bickert U, et al: Lumbar epidural perineural
Roentgenol 157:685, 1991. injection: A new technique. Eur Spine J 6:357, 1997.
Jensen MC, Brant-Zawadzki MN, Ohuchowski N, et al: Magnetic Parziale JR, Hudgins TH, Fishman LM: The piriformis syn-
resonance imaging of the lumbar spine in people without drome. Am J Orthop 25:819, 1996.
back pain. N Engl J Med 331:69, 1994. Porzelius J: Memory for pain after nerve-block injections. Clin
Jensen ME, Evans AJ, Mathis JM, et al: Percutaneous poly- J Pain 11:112, 1995.
methylmethacrylate vertebroplasty in the treatment of osteo- Saifuddin A, Braithwaite I, White J, et al: The value of lumbar
porotic vertebral body compression fractures: Technical aspects. spine magnetic resonance imaging in the demonstration of
AJNR Am J Neuroradiol 18:1897, 1997. annular tears. Spine 23:453, 1998.
Johansson A, Sjolund B: Nerve blocks with local anesthetics and Weinstein JN, Walsh TR, Spratt KF, et al: Lumbar discography:
corticosteroids in chronic pain: A clinical follow-up study. A controlled prospective study of normal volunteers to
J Pain Symptom Manage 11:181, 1996. determine the false positive rate. Proceedings of the Inter-
Kaplan PA, Dussault RG: Image guided selective nerve national Intradiscal Therapy Society, 1989, Orlando, Fla.
blocks in the spine. Semin Musculoskel Radiol 1:231, Weinstein SM, Herring SA, Derby R: Contemporary concepts in
1997. spine care: Epidural steroid injections. Spine 20:1842, 1995.
12
CHAPTER 12
Imaging after Spinal Surgery Thoracic and Lumbar Spine
Laminotomy and laminectomy are resectional tech-
Mini N. Pathria and Steven R. Garfin niques typically used for the removal of an extruded disc
in the lumbar spine. Most disc extrusions are postero-
lateral, and disc material can be easily removed through a
unilateral defect known as a laminotomy or hemil-
SUMMARY OF KEY FEATURES aminectomy. In a laminotomy, resection is often limited
Spinal surgery is a rapidly evolving field, with new to the margins of the lamina, whereas the entire lamina is
surgical techniques and hardware being developed resected during laminectomy. During laminotomy, the
continually. Familiarity with the concepts and design spinous process and interspinous ligaments are pre-
principles of surgical instrumentation facilitates served. A laminotomy defect can usually be recognized
interpretation of the placement of and complications on the anteroposterior radiograph by noting unilateral
associated with spinal hardware. Diagnostic widening of the interlaminar space, but it is often
imaging plays an important role in determining the difficult to identify on transaxial CT scans and MR
adequacy of surgical intervention and in detecting images because of the limited nature of the bone
complications associated with operations on the resection. Even if the osseous defect is not apparent with
spine. Advances in computed tomography (CT) cross-sectional imaging, loss of portions of the
scanning and magnetic resonance (MR) imaging ligamentum flavum underlying the resection and
have enhanced the ability to monitor both the distortion of the overlying soft tissues may be recognized.
osseous and soft tissue changes associated with spinal Hemilaminectomy is readily apparent with conven-
intervention. tional radiography, CT scanning, and MR imaging
because of the presence of a unilateral defect in the
laminar bone.
Total laminectomy with foraminotomy or facetectomy
is a more extensive resection than that required for
simple disc excision. This procedure is commonly used
INTRODUCTION
for the treatment of symptomatic lumbar stenosis. It is
The surgical treatment of low back pain has followed often bilateral and includes resection of the spinous
three major trends in the past century. Originally, process, which results in unroofing of the spinal canal
degenerative or extruded discs were considered the most and removal of the ligamentum flavum. Foraminotomies
important cause of low back pain, leading to the typically involve removal of the medial half of the facet,
development of laminectomy and discectomy. The next with preservation of the interfacet joint and the pars
major trend was the addition of spinal fusion, because interarticularis. Foraminotomy results in loss of the normal
spinal instability was thought to be the cause when a width and obliquity of the inner margin of the facet. The
patient continued to have back pain after disc excision. inferior tip of the facet may be truncated, or the facet
The latest trend is directed toward the treatment of may be completely absent (facetectomy) when extensive
radiculopathy rather than back pain, deformity, or resection has been performed (Fig. 12–1). The facet joints
instability alone. and posterior intervertebral ligaments are the spine’s
Currently, numerous surgical techniques are used, principal posterior stabilizers; complete resection of the
including percutaneous interventional procedures, facet leads to an increased likelihood of instability if an
osseous resection or decompression, realignment, and arthrodesis is not carried out simultaneously. Patients with
fusion. A vast array of spinal hardware systems has been underlying degenerative spondylolisthesis or degenera-
developed to address the full spectrum of spinal tive scoliosis appear to be at highest risk for progressive
disorders. instability after decompressive surgery.
Thinning of the inferior articular facet at the site of
facetectomy predisposes this structure to the develop-
DISCECTOMY AND DECOMPRESSION
ment of a postoperative stress fracture. It has been
Resection of bone is performed most commonly for suggested that excessive removal of bone immediately
decompression of spinal stenosis, for decompression of cephalad to the flare of the inferior articular process leads
traumatic lesions, and to provide access to the disc to this type of fracture. The most common locations for
for discectomy. Only very small amounts of bone postfacetectomy fractures are the pars interarticularis and
need to be removed to perform discectomy in patients the inferior tip of the facet. Postoperative fractures are
without central or foraminal stenosis. Substantial seen in 15% of patients with the postsurgical failed back
resection, however, may be necessary in patients with syndrome. Fractures following facetectomy are difficult
severe spinal canal impingement at multiple levels, which to visualize radiographically and may be confused with
typically results from extensive degenerative spinal each other on CT scans unless careful attention is paid to
stenosis. the exact site and orientation of the fracture line. The
167
168 Section III Imaging and Interventional Procedures of the Spine
CT diagnosis of both pars and facet fractures is facilitated decompressive surgery. In adults, risk factors for
with reformatted images. postoperative instability include underlying degenerative
In patients with single-level stenosis, adequate surgical spondylolisthesis, advanced age, a primary neural disorder,
treatment includes removal of sufficient portions of the rheumatoid arthritis, or recurrent trauma. Underlying
laminae, and often the medial aspects of the facet joints, to degenerative spondylolisthesis appears to be the most
cause decompression. Laminotomies of the superior and significant risk factor. Patients deemed to be at high risk
inferior vertebrae may be adequate; in selected cases, a for instability after decompressive lumbar laminectomy
complete laminectomy can be avoided. Multilevel stenosis typically undergo fusion with posterolateral or intertrans-
necessitates more extensive surgery, with resection of all verse bone grafting at the time of surgery. Numerous
laminar and facet bone in the areas of stenosis. Most internal fixation devices have been designed to prevent
patients with lumbar stenosis severe enough to warrant further subluxation while the bone fusion is consolidating.
surgery require multilevel decompression. Postoperative Despite high rates of pseudarthrosis following fusion, long-
vertebral subluxation is the most common complication term follow-up shows significantly better overall results,
encountered in patients after extensive decompressive with less leg pain and less progression of anterolisthesis,
surgery of the posterior thoracic or lumbar region. In skele- in patients undergoing fusion.
tally immature individuals, there is a 50% rate of occurrence Tears of the dura are not uncommon during resec-
of cervicothoracic or thoracic kyphosis after posterior tional surgery and occur more frequently during revision
Chapter 12 Imaging after Spinal Surgery 169
surgery because of the presence of scar adherent to the performed. Laminectomy is typically reserved for patients
dura. Dural tears are generally detected and repaired at requiring decompression for cervical spinal stenosis and is
the time of surgery; small undetected dural leaks typically usually performed at multiple levels. Cervical spondylitic
heal spontaneously, but large leaks may persist and form myelopathy is multifactorial, in that it is due to a com-
prominent paraspinal fluid collections (Fig. 12–2). bination of progressive degenerative cervical spondylosis,
Postoperative pseudomeningocele is an uncommon com- direct spinal cord compression, cord ischemia, and, often,
plication resulting from a chronic unrecognized or incom- an underlying congenitally narrow spinal canal.
pletely repaired dural tear. With CT, a pseudomeningocele Extensive cervical laminectomy for decompression of
appears as a rounded mass of low attenuation posterior to myelopathy without adequate fusion can lead to progres-
the thecal sac; the margin typically consists of a well- sive subluxation and kyphosis and produce a “swan-neck”
defined rim of higher attenuation that can be attributed deformity (Fig. 12–3). This deformity is rarely seen after
to pseudocapsule formation. With MR imaging, the a single-level laminectomy or after multiple unilateral
signal intensity of the collection corresponds to that of hemilaminectomies. Presumably, it is a result of the loss
cerebrospinal fluid, although increased signal intensity of posterior ligamentous and osseous stability, aggravated
may be seen in cases complicated by hemorrhage. by concomitant muscle weakness. Laminoplasty was
Decompression is also used occasionally for the man- developed to prevent this postoperative complication; this
agement of fractures and dislocations leading to spinal technique widens the canal and theoretically maintains
canal compromise. Realignment alone may obviate the stability. Anterior fusion is typically necessary for
need for decompressive surgery in most cases. Con- correction of symptomatic unstable kyphosis. Unlike
siderable controversy exists over the relative merits of posterior arthrodesis, anterior fusions are not subject to
anterior versus posterior approaches and over the benefits of significant tensile forces during spinal flexion and are
concomitant fusion and instrumentation if decom- therefore more likely to successfully correct the
pression is necessary. Nowhere is this controversy more deformity.
evident than in the debate over optimal management of a Severe cervical spondyloarthropathy can also be treated
burst fracture with neurologic deficit caused by by anterior decompression and fusion. Anterior decom-
retropulsed bone. pression allows direct removal of the compressive abnor-
malities, with stabilization provided by concomitant
anterior arthrodesis. Anterior decompression is also used
Cervical Spine for resection of the ossific masses present in patients with
Unlike in the lumbar region, access to extruded cervical ossification of the posterior longitudinal ligament
discs via the posterior approach is significantly limited (OPLL). Surgical resection of the ectopic ossification is
because of the presence of the spinal cord. The anterior frequently complicated by its adherence to the dura,
approach is preferred for the management of cervical disc leading to the development of dural tears during decom-
disease when concomitant discectomy and fusion are pression. Anterior cervical decompression with fusion is
Figure 12–2. Dural tear. Transaxial CT scan obtained after Figure 12–3. Postlaminectomy kyphosis. Severe cervical
the administration of intrathecal contrast agent demonstrates a kyphosis has occurred after laminectomies at C5 and C6 for
leak of cerebrospinal fluid into the paraspinal soft tissues degenerative spondylosis. Disc space loss is seen at multiple
(arrows). Immature bone graft is present from a recent cervical levels.
laminectomy and fusion procedure.
170 Section III Imaging and Interventional Procedures of the Spine
advocated for the management of patients with neuro- its material properties are unaffected by radiation and
logic deficits after traumatic injury to the cervical region chemotherapy, both of which are known to inhibit bone
that results in spinal cord compression by anteriorly healing. The use of PMMA alone or, more typically, in
located bone or disc fragments. conjunction with metallic internal fixation and bone
grafting facilitates early mobilization of patients with
malignant lesions.
SPINAL FUSION WITHOUT
The major disadvantages of PMMA include increased
INSTRUMENTATION
rates of infection and mechanical loosening. Mechanical
The rationale for spinal arthrodesis is based on the expe- loosening is common because the material interdigitates
rience with fusion of other articulations of the body, in only with native bone and never undergoes incorpora-
which arthrodesis has been used to eradicate pain by tion. Gradual resorption at the bone-cement interface,
eliminating motion. Indications for spinal arthrodesis leading to loss of fixation and subsequent extrusion of the
include (1) prevention of progressive spinal deformity, (2) construct, is the most frequent complication and accounts
maintenance of corrected deformity, (3) reestablishment for more than 80% of PMMA failures. The substance
of spinal stability, and (4) elimination of pain caused by appears homogeneous and has a density similar to that of
motion between spinal segments. Spinal fusion may be soft tissue on conventional radiographs. Radiopaque
carried out alone or combined with decompressive material, typically barium, is added to the PMMA during
surgery, the application of spinal hardware, or both. manufacture to make it easier to visualize radiographi-
cally. PMMA exhibits inhomogeneity on CT scans, and
entrapped air bubbles may be apparent in intact nonin-
Graft Materials
fected grafts; on MR images, PMMA exhibits signal void
The graft material used most commonly for achieving with all MR imaging sequences.
spinal fusion is bone, whether it be an autograft or an Bone graft substitutes consisting of porous hydroxya-
allograft. An autograft may be obtained from numerous patite derived from the calcium carbonate exoskeleton of
anatomic regions, with the iliac crest being the most certain varieties of sea coral are commercially available.
common site. The ilium offers a variety of osseous con- Although these implants lack the osteoprogenitor cells
tours and is a source for cortical, corticocancellous, and and bone-producing factors found in viable autologous
cancellous graft harvest. The main advantages of an bone, they serve as an effective nonantigenic scaffolding
autograft are histocompatibility, availability, and high for bone ingrowth and incorporation. Some of these
osteogenic potential because of the presence of viable constructs have little inherent mechanical stability, which
osteoprogenitor cells. Banked allograft bone is used precludes their use in situations in which they must bear
when the patient’s own bone stock is of limited quantity large structural loads. On initial radiographs, the implants
or poor quality, usually as a consequence of metabolic appear much denser than normal bone and do not exhibit
bone disease or previous surgical harvesting. The use of the organized, linear trabecular pattern of bone-based
allograft decreases operative time and eliminates the risk graft materials.
of donor site complications. The major disadvantages of
allografts are the potential for disease transmission, a
slower rate of vascularization, failure of incorporation Thoracic and Lumbar Spine
(nonunion), and higher rates of collapse. The posterior approach remains the standard method of
Cortical grafts, though they are stronger mechanically, obtaining fusion in the thoracic and lumbar regions, but
are revascularized more slowly than cancellous bone. the site of fusion has been modified because of an
Solid union of cortical grafts may take up to 2 years to unacceptably high rate of pseudarthrosis with midline
develop, and nonunion rates for rib or fibular strut grafts fusion. Currently, most lumbar arthrodeses are performed
range from 8% to 37%. Major complications related to posterolaterally, adjacent to the posterior elements rather
harvest sites for autologous grafts include fracture of the than in the midline. Posterolateral fusions are performed
underlying bone when a large amount of bone has been either posterior to the transverse processes (intertrans-
harvested, infection, and damage to the overlying soft tissue verse fusion) or overlying the posterolateral surfaces of
structures. Removal of a large bicortical bone graft, particu- the facet joint and lamina (interlaminar fusion); in some
larly when associated with splitting of the inner and outer cases, both fusions are done. In a patient with significant
tables of the ilium, predisposes the bone to fracture. spondylolisthesis, fusion is often performed in situ, because
Nonosseous sources of graft material include poly- reduction of the slippage results in an increased risk of
methylmethacrylate (PMMA), which is widely used for neurologic deficit.
reconstruction after débridement or resection of spinal Anterior interbody fusions are performed less fre-
neoplasms; bioactive ceramic spacers; and manufactured quently than the standard posterolateral arthrodesis. The
hydroxyapatite bone graft substitutes. PMMA is a bone graft is located closer to the center of motion for
resinous acrylic material formed by mixing its liquid and the vertebral segment, theoretically producing increased
powder components at the time of surgery. PMMA is stiffness when compared with that derived from posterior
used most commonly after corpectomy and débridement fusion alone. Smaller amounts of bone graft are used, and
of spinal neoplasms, particularly metastatic disease. It is restoration of vertebral body height is achieved with this
strongest in compression but does not resist tension, so approach. Interbody grafting of the lumbar spine via a
this material is frequently used in the anterior spinal posterior approach, known as a posterior lumbar inter-
regions. PMMA can be molded to any desired shape, and body fusion (PLIF), was developed to provide more stable
Chapter 12 Imaging after Spinal Surgery 171
fixation than could be achieved by a posterior graft alone. with loss of the initial neuroforaminal distraction. Cortical
The posterior approach to the intervertebral disc avoids strut grafts are used when multilevel vertebral body
complications associated with the anterior transpleural, resection is performed. Because of the high mechanical
transperitoneal, and retroperitoneal approaches to the stresses in the anterior aspect of the spinal column in the
spine. thoracic and lumbar regions, all types of strut grafts used
The most common material used for PLIF is autolo- there have been reported to fracture or collapse.
gous bone in the form of tricortical or cortical cylindrical Metallic and carbon fiber implants filled with non-
dowels, usually cut from the ilium. Strut grafts may also structural cancellous bone, referred to as interbody fusion
be used to obtain more stable immediate fixation. cages, have been found to provide structural support
Complications associated with the use of fibular grafts for immediately after implantation and to provide a biologic
the fixation of symptomatic high-grade spondylolysis scaffold and substrate that promote fusion. The current
include pseudarthrosis, neurologic deficit, and progres- generation of metallic cages can be inserted via either an
sion of the grade or angle of the slip. More recently, anterior or a posterior approach to the spine with either
PLIF using metallic fixation devices placed at the level of open or laparoscopic technique. Fusion cages have rapidly
the disc space has been described (Fig. 12–4). Anterior gained popularity because of the high fusion rates they
interbody fusions via the anterior approach are used afford, without the necessity of performing combined
when anterior decompression is necessary or when the anterior and posterior fusion (circumferential arthrodesis)
posterior elements are grossly insufficient. to achieve spinal stability. Reliable radiographic indi-
Single-level intervertebral fusions are typically per- cators of fusion include bridging trabecular bone anterior
formed by placement of a cancellous or corticocancellous to or surrounding the fusion cage, in conjunction with
interbody graft. Anterior interbody grafts tend to show the absence of motion on flexion-extension radiographs.
some settling before fusion; this settling may be neces- It is very difficult to identify fusion after interbody cage
sary for successful incorporation. The interbody graft placement if the bridging trabecular bone is limited to
should appear rectangular, because wedge-shaped grafts the inside of the cage device.
that are taller anteriorly tend to become extruded upon Anterior thoracic and lumbar approaches are asso-
spinal hyperextension (Fig. 12–5). Minor degrees of graft ciated with a variety of complications. The rate of major
protrusion anterior to the vertebral margin tend to be complications associated with such procedures has been
resorbed spontaneously. Although immediate postopera- reported to range from 1% to 11%. Along with failure of
tive radiographs show restoration of disc height, at long- the graft itself, complications related to the adjacent
term follow-up, disc height is typically diminished, along vascular, neural, and visceral structures may be encoun-
tered. Vascular complications are rare but are most likely
to occur in patients with preexisting vascular abnormali-
ties such as aneurysm, pseudoaneurysm, underlying vas-
cular anomalies, or extensive calcific atherosclerosis.
Cervical Spine The strut grafts are typically derived from the iliac crest,
In contrast to the lumbar region, in the cervical spine, rib, or fibula. Fibular grafts are stronger but become
simple bone grafting is used more commonly in an ante- incorporated slowly; typically, they are reserved for
rior interbody approach rather than a posterior one. patients undergoing vertebrectomy at more than three
Grafting alone is rarely sufficient for posterior stabiliza- levels. Initial incorporation takes place at the ends of the
tion, so posterior grafting is typically combined with the graft, with slowly progressive ingrowth of bone into the
use of some form of metallic hardware to provide stability medullary portions of the strut graft.
until the graft is incorporated. The anterior approach is Anterior cervical arthrodesis failure rates of 3% to
used for removal of degenerated discs and interbody fusion 20% have been reported. Mechanical causes of failure of
with concomitant removal of symptomatic osteophytes. anterior cervical grafts include fracture, collapse, resorp-
It can also be used for traumatic lesions, cervical stenosis, tion, or frank extrusion of the graft (Fig. 12–6). Previous
inflammatory arthropathies, ligamentous ossification, posterior decompression places the anterior graft under
and many other pathologic cervical conditions requiring increased mechanical stress and significantly increases
anterior decompression. the failure rate of the anterior fusion. Graft extrusion is a
Some controversy exists over whether single-level dis- common complication that occurs in 1% to 13% of
cectomy requires interbody fusion, although many sur- patients as a result of inadequate compression across the
geons routinely use graft material for such cases. Anterior graft, failure of the graft, or failure of its interface with
intervertebral grafting is considered essential to restore the adjacent vertebrae. Extrusion of the graft typically
spinal stability and function after anterior multilevel occurs in an anterior or anterolateral direction, with
decompression. In patients with multiple levels of disc resultant kyphosis (Fig. 12–7).
degeneration, interbody grafts can be used at all operated It is extremely difficult to determine whether successful
levels, or strut grafting may be performed. Anterior cer- interbody fusion for cervical spondylosis accelerates
vical fusion is typically carried out by placing cortico- degenerative disease at adjacent disc levels because of
cancellous autologous bone grafts in the region of the diminished motion at the fusion site and increased com-
intervertebral disc after removal of a degenerated disc pensatory motion at adjacent levels. Although many
and any significant osteophytes. The graft functions as a surgeons think that the risk of accelerated spondylosis is
mechanical spacer that maintains or increases disc height; increased after spinal fusion, an adequate study that
it also distracts the neural foramina and thereby decreases supports this opinion is not available.
the compressive effect of foraminal osteophytes.
Cervical cortical strut grafts are used after corpectomy
SPINAL INSTRUMENTATION
and allow for the replacement of one or more vertebral
bodies. Anterior strut grafting or augmentation with Hardware was originally developed to allow internal
posterior fusion is often necessary when multilevel correction of scoliotic deformities and early mobilization
decompression is performed in myelopathic patients. of patients. Its applications have since been expanded to
rather than plates results in more active correction of management of scoliosis. The correction achieved is not
deformity. Rigid rod-screw systems have also been used as anatomic as initially believed, but it is still better than
successfully to treat defects of the pars interarticularis that obtained with the previous hardware systems. The
(i.e., spondylolysis) that produce spondylolisthesis; they CD instrument consists of a pair of 7-mm stainless steel
result in high fusion rates, even in older patients. rods that have their entire surface serrated with
More modular systems of pedicle screw–rod fixation diamond-shaped irregularities (Fig. 12–11). The rods are
attempt to provide flexibility in the construct to permit connected by slender threaded crosslinks. Hooks, pedicle
some motion, thereby decreasing the degree of stress screws, or both can be applied anywhere along the length
shielding of the bone graft. The newest development in of the rods and can face in any direction. Typically, each
spinal hardware is the introduction of derotational rod is attached to four or more laminar hooks or pedicle
systems that can be used to apply rigid segmental fixation screws. Rotational correction is accomplished by rotating
while also allowing for multidirectional control and the contoured rod after its insertion into hooks placed at
correction of deformity in all three planes. These dero- critical points in the spine. Hooks on the same rod can be
tational systems, represented by the Cotrel-Dubousset placed to act in compression or in tension.
(CD) and Texas Scottish Rite Hospital (TSRH) systems, The TSRH system has the same fundamental function
are adaptable modular systems that can be used for as CD hardware, but its design modifications allow for
multiple indications. These systems are biomechanically easier implantation and, more important, greater ease of
complex, technically demanding to insert, bulky, and removal or revision once the instrument is in place. The
expensive, but they are a major advance in spinal system has evolved from the initial develop-ment of a
hardware. crosslinkage plate to a complete hardware system con-
CD hardware is designed to provide bilateral seg- sisting of rods, hooks, pedicle screws, and crosslinks
mental spinal fixation and selective distraction and com- (Fig. 12–12). TSRH rods are roughened rather than ser-
pression at different levels, as well as improve alignment rated and are available in three levels of stiffness. The
in all three planes. The system applies mechanical TSRH system allows for the placement of both standard
correction primarily by rotation of the coronal deformity pedicle screws (perpendicular to the rod) and pedicle
into the sagittal plane, thereby converting scoliotic screws at variable angles relative to the rod. The hooks
deformity into a more physiologic kyphosis or lordosis. come in a wide array of configurations and sizes. Anterior
The introduction of a rotational maneuver to correct fixation in the thoracic and lumbar spine is used far less
spinal deformity was a major evolutionary change in the frequently than is posterior fixation hardware.
176 Section III Imaging and Interventional Procedures of the Spine
diagnose nonunion of the fusion mass, there is consider- and its role remains controversial; at this time, electrical
able disparity in the reported rate of pseudarthrosis after stimulation is considered to be an adjunct to bone grafting
bone grafting. The prevalence of pseudarthrosis after in patients with established previous pseudarthrosis or in
attempted fusion ranges from 3% to 30%. Higher rates those patients deemed at high risk for pseudarthrosis.
of pseudarthrosis are encountered in patients undergoing Numerous imaging methods are used for the detec-
simultaneous multilevel fusion. tion of pseudarthrosis; these methods can be divided into
The rate of nonunion for anterior cervical fusion those that assess the morphologic structure of the grafted
ranges from 0% to 26%. Anterior interbody fusions have material and those that assess the graft functionally.
a higher rate of nonunion than do posterior or pos- Structural integrity is evaluated by conventional radi-
terolateral fusions. Of the various posterolateral fusions ography and CT scanning. Functional integrity is assessed
used, intertransverse fusion is reportedly less likely to with stress views (typically, lateral flexion and extension
undergo nonunion. In general, the prevalence of views).
pseudarthrosis after posterolateral spinal fusion without The initial diagnostic test for the evaluation of spinal
instrumentation increases with the number of motion fusion consists of conventional radiographs. Solid fusion
segments included in the arthrodesis. Iliac strut grafts results in a continuous, organized trabecular pattern
become incorporated more frequently and more rapidly across the entire grafted segment (Fig. 12–16). Typically,
than do fibular struts, which show nonincorporation in as 6 to 9 months is required for the graft material to appear
many as 50% of cases. Hardware systems are designed to solid and continuous radiographically. During the early
immobilize the spine to enhance osseous fusion, but they postoperative period, pseudarthrosis is difficult to detect
are not always successful. unless gross graft or hardware failure develops. Persistent
In some studies, direct current electrical stimulation halos or lucent areas around the graft or hardware
has been demonstrated to increase the rate of osseous suggest failure of incorporation and should be further
spinal fusion. Implantable direct current stimulators have evaluated with flexion-extension views. The accuracy of
been shown by some investigators to enhance bone fusion radiography for assessing spinal fusion has been ques-
radiographically and histologically in primary lumbar tioned in several studies that demonstrate considerable
fusions. Externally applied pulsed electromagnetic coils discrepancy between the radiographic appearance of the
are also available and appear to be as effective at stimu- graft and the surgical findings. The complex anatomy of
lating bone growth as implantable units. The need for the bone graft and the overlying osseous structures
routine electrical stimulation has not been established, frequently make it difficult to assess the graft material,
Chapter 12 Imaging after Spinal Surgery 179
average prevalence of infection of 6% or more. The The MR imaging findings of postoperative discitis
most common organism implicated is Staphylococcus overlap with changes related to uncomplicated surgery.
aureus, which accounts for approximately 50% of all The most reliable finding in postoperative infective
postoperative spinal infections, although infection with a discitis on MR images is decreased signal intensity in the
variety of other organisms (single or multiple) may be vertebral marrow of both adjacent endplates in T1-
encountered. weighted images, with enhancement of these areas after
Early conventional radiographic findings of disc space the intravenous administration of a gadolinium-
loss, osteopenia, and vertebral endplate erosion may be containing contrast agent.
difficult to distinguish from some postoperative changes. Postoperative spinal epidural empyema is a rare
With progressive infection, vertebral destruction, complication encountered in less than 1% of patients
osseous fragmentation, and reactive sclerosis may occur undergoing elective spinal surgery. Patients frequently
(Fig. 12–18). Loss of fixation and loosening of spinal have a paucity of clinical symptoms or signs to suggest
hardware are seen if the infection cannot be controlled the diagnosis of infection. The frequent presence of an
adequately with débridement, irrigation, and antibiotics. associated laminectomy allows the abscess to dissect
Similarly, in the early stages of infection, CT is not posteriorly through the split retrospinal muscles, thereby
reliable. Scintigraphy is nonspecific, particularly in the minimizing compression on adjacent neural structures.
early postoperative period. Bone scans using technetium- The CT appearance of the infected collection is very
99m diphosphonate show mild to moderately increased similar to that of a post-traumatic pseudomeningocele,
activity within the first 3 weeks after surgery in approxi- although the presence of gas bubbles, loss of definition of
mately 50% of patients undergoing laminectomy and the fat planes between the collection and the dural sac or
discectomy. The gallium scan also becomes abnormal in the paraspinal muscles, and irregular enhancement of
89% of patients postoperatively. Increased activity on thickened walls are all suggestive of infection.
both bone and gallium scans is related to postoperative
alterations in bone and soft tissue and typically persists
for several months; the bone scan may remain abnormal
up to 1 year after surgery. The normally increased activity FURTHER READING
seen on scintigraphic studies limits their usefulness in An HS: Surgical exposure and fusion techniques of the spine. In
detecting postoperative spinal infection. An HS, Cotler JM (eds): Spinal Instrumentation. Baltimore,
Williams & Wilkins, 1992, p 1.
Berchuck M, Garfin SR, Bauman T, Abitbol JJ: Complications
of anterior intervertebral grafting. Clin Orthop 284:54,
1992.
Boden SD, Davis DO, Dina TS, et al: Postoperative diskitis:
Distinguishing early MR imaging findings from normal
postoperative disk space changes. Radiology 184:765, 1992.
Bohlman HH, Emery SE: The pathophysiology of cervical
spondylosis and myelopathy. Spine 13:843, 1988.
Brown MD, Malinin TI, Davis PB: A roentgenographic evalua-
tion of frozen allografts versus autografts in anterior cervical
spine fusions. Clin Orthop 119:231, 1976.
Cleveland M, Bosworth DM, Thompson FR: Pseudarthrosis
in the lumbosacral spine. J Bone Joint Surg Am 30:302,
1948.
Cotler JM, Simpson JM, An HS: Principles, indications, and
complications of spinal instrumentation: A summary chapter.
In An HS, Cotler JM (eds): Spinal Instrumentation.
Baltimore, Williams & Wilkins, 1992, p 435.
Djukic S, Lang P, Morris J, et al: The postoperative spine: Magnetic
resonance imaging. Orthop Clin North Am 21:603, 1990.
Edwards CC: Edwards instrumentation: A modular spinal
system. In An HS, Cotler JM (eds): Spinal Instrumentation.
Baltimore, Williams & Wilkins, 1992, p 303.
Garfin SR, Glover M, Booth RE, et al: Laminectomy: A review
of the Pennsylvania Hospital experience. J Spinal Disord
1:116, 1988.
Garfin SR, Herkowitz HN, Mirkovic S: Spinal stenosis. J Bone
Joint Surg Am 81:572, 1999.
Grabias S: The treatment of spinal stenosis. J Bone Joint Surg
Am 62:308, 1980.
Johnsson K, Willner S, Johnsson K: Postoperative instability
Figure 12–18. Postoperative infection. Deep infection of the after decompression for lumbar spinal stenosis. Spine
L1–L2 disc and adjacent vertebrae has occurred after TSRH 11:107, 1986.
rodding for a burst fracture of L2. The endplates are eroded, Kaneda K: Kaneda anterior spinal instrumentation for the tho-
with kyphosis and sclerosis resulting from chronic pyogenic racic and lumbar spine. In An HS, Cotler JM (eds): Spinal
infection. Instrumentation. Baltimore, Williams & Wilkins, 1992, p 413.
Chapter 12 Imaging after Spinal Surgery 181
Lenke LG, Bridwell KH, Blanke K, et al: Radiographic results Sartoris DJ, Gershuni DH, Akeson WH, et al: Coralline hydrox-
of arthrodesis with Cotrel-Dubousset instrumentation for yapatite bone graft substitutes: Preliminary report of radi-
the treatment of adolescent idiopathic scoliosis. J Bone Joint ographic evaluation. Radiology 159:133, 1986.
Surg Am 80:807, 1998. Slone RM, Heare MM, Griend RAV, et al: Orthopedic fixation
Lonstein JE: Post-laminectomy kyphosis. Clin Orthop 128:93, devices. Radiographics 11:823, 1991.
1977. Slone RM, MacMillan M, Montgomery WJ: Spinal fixation.
Marshall LF: Complications of surgery for degenerative Part 1. Principles, basic hardware, and fixation techniques
cervical and lumbar disc disease. In Garfin SR (ed): Com- for the cervical spine. Radiographics 13:341, 1993.
plications of Spine Surgery. Baltimore, Williams & Wilkins, Slone RM, MacMillan M, Montgomery WJ, Heare M: Spinal
1989, p 75. fixation. Part 2. Fixation techniques and hardware for the
McAfee PC: Interbody fusion cages in reconstructive opera- thoracic and lumbosacral spine. Radiographics 13:521, 1993.
tions on the spine. J Bone Joint Surg Am 81:859, 1999. Slone RM, MacMillan M, Montgomery WJ: Spinal fixation.
McAfee PC, Yuan HA, Lasda NA: The unstable burst fracture. Part 3. Complications of spinal instrumentation. Radiographics
Spine 7:365, 1982. 13:797, 1993.
Meyer PR Jr, Rusin JJ, Haak MH: Anterior instrumentation of Spivak JM: Degenerative lumbar spinal stenosis. J Bone Joint
the cervical spine. In An HS, Cotler JM (eds): Spinal Instru- Surg Am 80:1953, 1998.
mentation. Baltimore, Williams & Wilkins, 1992, p 49. Steinmann JC, Herkowitz HN: Pseudarthrosis of the spine.
Puno RM, Byrd JA III: The Puno-Byrd (PWB) spinal system Clin Orthop 284:80, 1992.
for transpedicular fixation of the lumbar spine. In An HS, Teplick JG, Haskin ME: Computed tomography of the
Cotler JM (eds): Spinal Instrumentation. Baltimore, Williams postoperative lumbar spine. AJR Am J Roentgenol 141:865,
& Wilkins, 1992, p 281. 1983.
Rothman SLG, Glenn WV, Kerber CW: Postoperative fractures Wang JC, Bohlman HH, Riew KD: Dural tears secondary to
of lumbar articular facets: Occult cause of radiculopathy. operations on the lumbar spine. J Bone Joint Surg Am
AJR Am J Roentgenol 145:779, 1985. 80:1728, 1998.
SECTION
IV
Imaging of the Postoperative Spine
CHAPTER 13
Imaging after Surgery in Kirschner wires; both may be smooth or threaded.
Kirschner wires may also serve as guides over which
Extraspinal Sites cannulated screws are placed.
Wires and Pins. Wires, pins, nails, and rods are generally Screws. Cortical screws are threaded over their length.
distinguished on the basis of size. Wires are the thinnest. They are used primarily to secure plate devices to bone,
Kirschner wires have spatulate ends and are thin (0.028 and they should penetrate both the near and the far
to 0.062 inch). The soft tissue end of the wire may be cut cortices. Cancellous lag screws are threaded distally and
off beneath the skin, or its protruding end may be bent or have smooth proximal shafts (Fig. 13–3). The threads are
capped to prevent injury. Steinmann pins are thicker than wider than those of cortical screws, so they hold better in
183
184 Section IV Imaging of the Postoperative Spine
Plates. A plate can be used to provide one or more of and reconstruction plates. Dynamic compression plates
the following functions: static or dynamic compression, are recognizable on radiographs by their oval holes.
neutralization, and buttressing (Fig.13–6). Static com- Tubular plates have a concave inner surface to conform
pression refers to the production of axial compression to the curvature of the underlying bone. Reconstruction
along fractures. Compression can be achieved either by plates can be bent to conform to the shape of the under-
attaching a tension device to a plate at the time of lying bone (Fig. 13–7).
internal fixation or by using a particular type of plate, the
dynamic compression plate. A dynamic compression plate
External Fixation Devices
has screw holes with sloped sides that correspond to the
slope of the undersurface of the screw. As the screws External fixation provides immobilization of fractures while
are tightened, the screw head glides down the incline maintaining the potential to adjust the fracture position.
of the screw hole, toward the center of the plate, thus In addition, external fixators may be used for compression
producing compression at the fracture line. A plate that at sites of attempted fusion (Fig. 13–8) and for distraction
is used for neutralization bridges a comminuted frac- during attempted limb lengthening. Several devices are
ture and transmits bending or torsional forces from the available that consist of one or more frames anchored to
proximal to the distal fragment, thereby protecting the the bone with pins. Some indications for external fixation
intervening fracture fragments from these forces. When of fractures include open fractures in which comminution
applied to a cortical surface that is concave, the plate is present, especially in cases of segmental bone loss and
is bent slightly so that its central portion is not in con- soft tissue damage; the presence of major vascular damage
tact with the bone. Plates used for buttressing support an requiring repair; severe osteoporosis; and extensive epi-
area of thin cortex or cancellous bone graft and prevent physeal and metaphyseal comminution. External fixators
collapse. These plates are under compression (rather are also used in the treatment of infected, nonunited frac-
than tension) and are used near joints, such as the tibial tures and during healing of free vascularized bone grafts.
metaphysis, to prevent deformity after fracture fixation Complications associated with the use of external fixation
and grafting. include pin tract infections, soft tissue contracture, and
Plates are categorized into straight plates and plates delayed union or nonunion.
with special shapes. Straight plates include those with The Ilizarov frame consists of circular rings that sur-
round holes, dynamic compression plates, tubular plates, round the limb and are connected by a series of longitu-
186 Section IV Imaging of the Postoperative Spine
vide structural stability. When corticocancellous bone Bone Formation after Grafting
grafts are used, they are positioned so that the cancellous Cancellous bone grafts have a greater capacity to induce
surface abuts the soft tissues, to facilitate vascular new bone formation than cortical grafts do. If the can-
ingrowth. cellous graft is immobile, a process of “creeping sub-
Grafts may also be described according to their compo- stitution” takes place in which new bone is deposited on
sition, position, and shape. Onlay grafts consist of cortical the scaffold of dead trabeculae. Allografts are generally
bone that is placed across a bony defect (such as a less satisfactory than autografts, in that new bone forma-
nonunion) and held by screws to a surgically denuded or tion is slower, vascular penetration is slower and less dense,
drilled surface of the host bone. A sliding inlay graft and bone replacement tends to be superficial. Rejection
consists of bone cut from the proximal fragment and slid of allografts because of sensitization of the host by
distally across the bony defect. A dowel graft consists of antigens in the graft is a major reason for their inferiority
a core of cancellous bone that is inserted into a surgically to autografts.
created channel to stimulate osteogenesis. Strut grafts,
often composed of a rib or fibula, provide stability and
stimulate osteogenesis; these grafts are used most often Vascularized Bone Grafts
in the spine (Fig. 13–11). Microsurgical techniques allow bone grafts and their asso-
ciated vasculature to be transplanted so that the grafts
remain viable. Currently, the success rate of this procedure
Indications
is greater than 90% (Fig. 13–12). This procedure is
Bone grafts are generally used to promote healing, pro- usually reserved for cases of massive bone loss (>12 cm),
vide stability, or both. Possible situations in which bone patients in whom more conventional techniques have
grafting may be used include filling of bony defects or failed, or those in whom an inadequate soft tissue bed is
cavities; bridging of joints for arthrodesis; bridging of present.
large defects in a long bone; promoting healing in cases
of nonunion; and promoting union or filling defects
in cases of delayed union, malunion, fresh fracture, or Massive Cadaver Allografts
osteotomy. Massive cadaver allografts are used for limb salvage in the
treatment of primary bone tumors and for reconstruction
in patients with severe bone loss after failed joint replace-
ment. In selected cases, such as after resection of aggressive
benign bone tumors or bone sarcomas, reconstruction is
performed by inserting a massive bone graft consisting of
bone, articular cartilage, and accompanying tendons or
TABLE 13–1
ligaments. Bone formation (by creeping substitution) occurs
Bone Graft Types by Origin at the host-graft junction and is presumed to require
many years before remodeling of the entire graft takes
Type of Graft Description place. The graft therefore functions largely as a spacer.
Autograft Transplanted bone derived from the same Failure of massive allografts is most often due to infection
person or fracture.
Allograft Transplanted material derived from another
person of the same species
Xenograft Transplanted material derived from a Donor Sites
member of a different species The most frequently used donor sites for bone grafting
are the iliac crest, tibia, fibula, greater trochanter, distal
TABLE 13–2
Comparison of Cancellous and Cortical Bone Grafts
portion of the radius, and posterior elements of the spine. older than 30 years. Graft healing is generally docu-
The ilium is a good source of cancellous bone grafts. mented by the loss of sharp margins between the graft
When the patient is supine, cancellous bone is removed and the host bone, eventually leading to osseous union,
from the area below the anterior superior iliac spine. The with bone continuity across the host-graft junction.
inner table of bone is left intact to avoid the development Fibrous union is suggested by the persistence of a thin
of a muscular hernia. If more cancellous bone is needed, residual radiolucent area between the graft and the host
the dissection is extended posteriorly. Fibular grafts are bone. The time required to achieve union depends on
obtained from the middle third to half of the bone, because the size and type of graft, the local conditions, and the
this area can be excised without adverse effects. The distal site of surgery.
quarter as well as the proximal segment of the fibula With regard to vascularized bone grafts, accumulation
should remain intact to ensure stability at the ankle and of the bone scanning agent in the area of the graft within
knee. Complications at donor graft sites appear to be few. the first week after transplantation indicates both intact
Nonetheless, fracture may occur after cortical graft vasculature and metabolically viable bone; conversely, the
removal, and intraoperative bleeding and postoperative absence of uptake of the bone scanning agent on serial
pain may follow iliac crest biopsy. Stress fractures, hernias radionuclide examinations suggests segmental nonviability
through an iliac donor site, and gait problems have also (Fig. 13–13). Accumulation of the bone-seeking radionu-
been reported. clide after this 1-week period, however, may be due to the
deposition of new bone on the surface of dead trabeculae
and does not indicate either vascular patency or the pres-
Radiologic Examination
ence of viable graft. Blood perfusion and blood pool scans
Healing of iliac donor sites is evidenced by sclerosis at are helpful in confirming vascular patency in the first
the margins of the osseous defects. Bony excrescences 2 weeks after surgery.
from the donor site may appear and be accompanied by Magnetic resonance (MR) imaging has been used to
pain. Radiographic evaluation of rib and iliac donor sites evaluate arterial anatomy before vascularized fibular graft-
shows regeneration occurring in children and in adults ing, as well as to determine the length of scaphoid bone grafts
younger than 30 years, but this is not observed in those needed to correct humpback deformity of the scaphoid.
190 Section IV Imaging of the Postoperative Spine
Girdlestone Arthroplasty
At the same time, the vascularity of the scaphoid and the
integrity of the adjacent ligaments can be assessed. The term Girdlestone arthroplasty is currently applied in a
general sense to any hip joint resection. It is used as a
salvage procedure, performed when an infected total hip
Complications prosthesis is removed. It is also used in some instances of
Delayed union in cortical grafts is defined as the absence infectious arthritis in preference to hip fusion. The
of healing 12 months after surgery (Fig. 13–14). Graft indications for Girdlestone arthroplasty are listed in
failure may be associated with progressive bone resorp- Table 13–3. The results of Girdlestone arthroplasty after
tion, leading to a decrease in the size and density of the removal of an infected hip prosthesis have not been as
graft and ultimately resulting in its disappearance. Similar dismal as might be expected, and pain relief and control
graft resorption may be due to recurrent tumor or of infection occur in more than 80% of patients.
infection. Shortening of the affected extremity, a Trendelenburg
Stress fractures within cortical grafts are not uncom- gait, and joint instability are invariable sequelae that
mon. Stress fractures usually occur only after union of make walking difficult and tiring. Radiographs confirm
the graft and host bone is seen, and they may fail to heal complete removal of the femoral neck, acetabular rim,
without operative treatment. and PMMA cement (Fig. 13–15). Optimally, all cement is
removed; however, residual cement does not necessarily
result in continued infection.
RESECTION ARTHROPLASTY
Resection arthroplasty consists of the removal of one or
ARTHRODESIS (JOINT FUSION)
both articular surfaces of a joint. Currently, this technique
is used primarily as a salvage procedure after failed total Arthrodesis refers to the surgical stiffening of a joint.
joint replacement surgery. Such surgery is usually performed to provide stability or
Chapter 13 Imaging after Surgery in Extraspinal Sites 191
adduction improves congruency. A wedge of bone is with trabecular continuity normally achieved about 4
removed that is wider laterally. The lower margin of the months after surgery. Evidence of bone resorption adja-
removed segment is at the level of the lesser trochanter cent to the orthopedic appliances may be due to abnormal
and perpendicular to the femoral shaft. The femoral head motion or infection (Fig. 13–20). Nonunion is indicated
is rotated laterally with some overcorrection so that the by the development of bone sclerosis and irregular bone
cartilage space is about 2 mm wider at the lateral acetabular resorption along the osteotomy surfaces.
margin than centrally. The distal fragment is displaced
laterally.
PROCEDURES FOR ISCHEMIC NECROSIS OF
THE FEMORAL HEAD
Radiologic Examination
Treatment strategies for ischemic necrosis of the femoral
In patients with eccentric loading (such as those with head are used before osseous collapse and include core
acetabular dysplasia), focal areas of subchondral sclerosis decompression, bone grafting, rotational osteotomy, and
develop (Fig. 13–19). With a marked increase in stress, bone angular osteotomy. Core decompression is a procedure in
resorption and cyst formation occur. Thus, radiographs which a core of bone is removed, beginning in the lateral
document uneven stress distribution, and if loading is cortex of the femur just distal to the greater trochanter and
improved postoperatively, these abnormal findings should terminating in the anterolateral segment of the femoral
regress. Postoperative radiographs document the degree head. The rationale for core decompression is the finding
of correction provided by the osteotomy, healing of the of elevated bone marrow pressure in patients suspected
osteotomy site, and any regression of the preoperative of having ischemic necrosis, even when radiographs are
osteoarthritic changes. The osteotomy site heals gradually, normal.
194 Section IV Imaging of the Postoperative Spine
Bone grafting is a procedure in which a core of bone Bowerman JW, Hughes JL: Radiology of bone grafts. Radiol
is removed (as in the core decompression procedure), and Clin North Am 13:467, 1975.
the defect is filled with bone graft. Vascularized iliac or Browner BD, Mast J, Mendes M: Principles of internal fixation.
fibular grafts may be used in an attempt to revascularize In Browner BD, Jupiter JB, Levine AM, et al (eds): Skeletal
Trauma. Philadelphia, WB Saunders, 1992, p 243.
a necrotic femoral head. The femoral head is reamed out
Buchholz HW, Elson RA, Heiner K: Antibiotic-loaded
and packed with cancellous bone graft. A fibular graft is acrylic cement: Current concepts. Clin Orthop 190:96, 1984.
then placed as far as possible into a tunnel drilled in the Chandler HP, Reineck FT, Wixson RL, et al: Total hip replace-
femoral head and neck for decompression and curettage. ment in patients younger than thirty years. J Bone Joint Surg
The vascularized fibular graft, its vascular supply, and a sur- Am 63:1426, 1981.
rounding muscle cuff are positioned within the tunnel; Edmonson AS: Surgical techniques. In Edmonson AS, Crenshaw
thus, the graft alone does not entirely fill the tunnel. AH (eds): Campbell’s Operative Orthopedics. St Louis,
Follow-up radiographs should show incorporation of the CV Mosby, 1980, p 19.
proximal fibular graft, as evidenced by blurring of its Enneking WF, Eady JL, Burchardt H: Autogenous cortical bone
margins, usually an average of 6 months after surgery. grafts in the reconstruction of segmental skeletal defects.
J Bone Joint Surg Am 62:1039, 1980.
Girdlestone GR: Acute pyogenic arthritis of the hip: An opera-
tion giving free access and effective drainage. Clin Orthop
FURTHER READING 170:4, 1982.
Harrington KD, Johnston JO, Turner RH, et al: The use of
Barr JS, Record EE: Arthrodesis of the ankle joint: Indications, methylmethacrylate as an adjunct in the internal fixation of
operative technique and clinical experience. N Engl J Med malignant neoplastic fractures. J Bone Joint Surg Am
248:53, 1953. 54:1665, 1972.
Bassett CAL: The development and application of pulsed Harrington KD, Sim FH, Enis JE, et al: Methylmethacrylate as
electromagnetic fields (PEMFs) for ununited fractures and an adjunct in internal fixation of pathological fractures. J Bone
arthrodeses. Orthop Clin North Am 15:61, 1984. Joint Surg Am 58:1047, 1976.
Berrey BH, Lord CF, Gebhardt MC, et al: Fractures of allo- Hungerford DS, Zizic TM: Pathogenesis of ischemic necrosis
grafts: Frequency, treatment and end results. J Bone Joint of the femoral head. Hip 249, 1983.
Surg Am 72:825, 1990. Insall JN, Joseph DM, Msika C: High tibial osteotomy for varus
Chapter 13 Imaging after Surgery in Extraspinal Sites 195
gonarthrosis: A long-term follow-up study. J Bone Joint Surg Murray WR: Use of antibiotic-containing bone cement. Clin
Am 66:1040, 1984. Orthop 190:89, 1984.
Kandel RA, Pritzker KPH, Langer F, et al: The pathologic fea- Sisk TD: Fractures. In Edmonson AS, Crenshaw AH (eds):
tures of massive osseous grafts. Hum Pathol 15:141, 1984. Campbell’s Operative Orthopedics. St Louis, CV Mosby,
Laros GS, Moore JF: Complications of fixation in intertrochanteric 1980, p 509.
fractures. Clin Orthop 101:110, 1974. Slone RM, Heare MM, Vander Griend RA, et al: Orthopedic
Manaster BJ, Coleman DA, Bell DA: Pre- and postoperative fixation devices. Radiographics 11:823, 1991.
imaging of vascularized fibular grafts. Radiology 176:161, 1990. Stewart M: Arthrodesis. In Edmonson AS, Crenshaw AH (eds):
Mankin HJ, Doppelt S, Tomford W: Clinical experience with Campbell’s Operative Orthopedics. St Louis, CV Mosby,
allograft implantation: The first ten years. Clin Orthop 1980, p 1113.
174:69, 1983. Wagner H: Principles of corrective osteotomies in osteoarthrosis
Masri BA, Duncan CP, Beauchamp CP: Long-term elution of of the knee. In Weil UH (ed): Progress in Orthopedic
antibiotics from bone-cement: An in vivo study using the Surgery, vol 4. Joint Preserving Procedures of the Lower
prosthesis of antibiotic-loaded acrylic cement (PROSTALAC) Extremities. New York, Springer-Verlag, 1980, p 75.
system. J Arthroplasty 13:331, 1998. Weissman BN, Reilly DT: Diagnostic imaging evaluation of
Mears DC: External Skeletal Fixation. Baltimore, Williams & the postoperative patient following musculoskeletal trauma.
Wilkins, 1983. Radiol Clin North Am 27:1035, 1989.
Müller ME, Allgower M, Schneider R, et al: Manual of Internal Young JWR, Kovelman H, Resnik CS, et al: Radiologic
Fixation: Technique Recommended by the AO Group. New assessment of bones after Ilizarov procedures. Radiology
York, Springer-Verlag, 1991. 177:89, 1990.
14
CHAPTER 14
Imaging of Joint Replacement 2. Measurement of the position of the center of the hip
is determined as the perpendicular distance from the
center of the femoral head to a line connecting the
Barbara N. Weissman teardrop shadows of both hips (interteardrop line; see
Fig. 14–1B).
3. The distance along the interteardrop line from the
SUMMARY OF KEY FEATURES intersection with a perpendicular line from the center
of the femoral head to the teardrop allows comparison
As advances and improvements are made in joint of the mediolateral position of the hip over time (see
replacement technology and surgical technique, imaging Fig. 14–1B). Changes of more than 2 mm are consid-
remains an important means of monitoring the patient’s ered significant with 10% magnification.
postoperative progress and identifying complications.
Radiographs provide a permanent record of the 4. Anteversion is usually determined from the true
procedure and can document success or failure of the lateral view of the hip. A line parallel to the table or
operation. This chapter presents the information film edge provides the baseline in the coronal plane.
required for accurate interpretation of radiographs after The angle between a perpendicular to the baseline and
various fixation devices and cement materials have been the axis of the acetabular marker wire is the angle of
used. It also reviews the expected findings, both normal anteversion (Fig. 14–2).
and abnormal, of total hip replacement, as well as 5. The tip of the femoral stem should appear on the
replacement of the knee and shoulder. Complications of frontal radiograph to be centered in the medullary
each operation are discussed, as are the uses of various canal or to be directed medially (valgus).
imaging methods. 6. Leg length is compared on the two sides by noting
the distances between the proximal edge of the lesser
trochanters and a baseline through the ischial
tuberosity on each side.
INTRODUCTION
The modern era of total joint replacement began in the Component Fixation
early 1960s with the introduction of metal-to-plastic hip Four major methods of component fixation to bone
prostheses embedded in methylmethacrylate cement. are currently used: (1) press fitting, (2) fixation with
Changes over the years have included metal backing of polymethylmethacrylate (PMMA) cement; (3) bone
the acetabular component, the development of modular ingrowth into porous surfaces, and (4) hydroxyapatite
design components and the bipolar prosthesis, the coating.
introduction of improved cement fixation, and the
development of noncemented fixation.
Press Fitting
TOTAL HIP REPLACEMENT With press fitting of components, the surgically created
bed for the prosthesis is made smaller than the prosthesis
Radiographic Technique itself, which is then forced into the defect.
Radiographs are critical for monitoring patients with total
joint replacements. The standard radiographic series
Polymethylmethacrylate Cement Fixation
consists of an anteroposterior view of the pelvis centered
at the pubis and an anteroposterior and frog-leg lateral Cement provides immediate stable fixation and functions
view of the affected hip. Enough of the femoral shaft to distribute load more evenly to the bone. Improve-
must be included to allow assessment of the size of the ments in cementing techniques have lowered the rates of
medullary canal preoperatively and the entire femoral loosening on the femoral side. On the acetabular side,
component and adjacent bone postoperatively. however, a nearly linear increase in acetabular loosening
has been reported, and improvements in cement tech-
nique are less applicable to the acetabular component.
Component Position Currently, porous-coated acetabular implants are often
1. Acetabular inclination indicates the tilt of the acetabu- matched with cemented femoral components for older,
lar component in relation to a horizontal baseline less active patients.
(Fig. 14–1A). An angle of inclination of 40 to 50 degrees
has been classified as neutral, with lower measure- Radiologic Evaluation
ments indicating a relatively horizontal orientation
and greater measurements indicating a more vertical Various radiologic methods are used to evaluate PMMA
one. cement fixation.
196
Chapter 14 Imaging of Joint Replacement 197
D t
T
Figure 14–1. Component position. A, Angle of acetabular inclination. The angle between the baseline
and the acetabular orifice (curved arrow) describes the tilt of the acetabular component in relation to the
horizontal. B, Vertical and mediolateral position. The vertical position of the hip is measured as the
perpendicular distance (D) from the center of the femoral head to the interteardrop line (T). The
mediolateral position of the hip (between arrows) is measured as the distance along the interteardrop line
from the teardrop (t) to the intersection with a perpendicular from the center of the femoral head.
TABLE 14–3
Arthrographic Criteria for Femoral Component
Loosening
A B
Figure 14–9. Aggressive granulomatous disease. A, Loosening of the cemented femoral stem is present,
as shown by wide metal-cement lucent zones. Well-defined focal areas of bone destruction (arrows) indicate
aggressive granulomatous disease. B, In another patient, a coronal reformatted computed tomography image
shows extensive, aggressive granulomatous disease adjacent to the acetabular component. Note thinning of
the polyethylene liner in the weight-bearing portion of the joint, indicative of polyethylene wear. (A, From
Weissman BN: Current topics in the radiology of joint replacement surgery. Radiol Clin North Am 28:1111,
1990.)
TABLE 14–4
Grading of Heterotopic Ossification about the Hip
From Brooker AF, Bowerman JW, Robinson RA, Riley LH: Ectopic ossification
following total hip replacement. Incidence and a method of classification.
J Bone Joint Surg Am 55:1629, 1973
Bipolar Prostheses
Bipolar hip prostheses were introduced as alternatives to
hemiarthroplasty (e.g., Moore and Thompson pros-
theses). Theoretically, the increased motion at the articu-
lation between the acetabular component and the femoral
head allows less motion at the prosthesis-acetabular articu-
lation and thus decreases acetabular wear. Other theo-
retical advantages of bipolar prostheses include a lower
frequency of prosthetic dislocation and the ability to
revise the acetabular component of a total hip replace-
ment if the need arises. Complications of bipolar pros-
theses include fracture of the ipsilateral femur, component
fracture and separation, abnormal wear of the acetabu-
lum, and wear of the acetabular liner (Fig. 14–13).
The unipolar prosthesis has replaced the bipolar pros-
thesis in some cases. A unipolar prosthesis functions as a
hemiarthroplasty but has modular components to allow
adjustment of neck length.
Hydroxyapatite-Coated Prostheses
Hydroxyapatite coating of prostheses allows chemical
bonds to form with the adjacent bone and promotes bone
apposition. It allows direct bone apposition without inter-
position of a fibrous membrane; therefore, radiographs
can be expected to show bone in direct apposition to the
prosthetic surface without an intervening lucent line. Figure 14–13. Bipolar prosthesis with acetabular protrusion.
Follow-up evaluation 2 years or longer after the insertion The bipolar prosthesis has migrated superomedially.
204 Section IV Imaging of the Postoperative Spine
Unicompartmental (unicondylar) prostheses resurface nent, in about two thirds of cases after total knee arthro-
both the femoral and the tibial sides of a damaged com- plasty. This rarefaction represents bone loss in response
partment (Fig. 14–14). They are used only in cases of to stress shielding and is not evidence of prosthetic
osteoarthritis in which damage is limited to a single loosening (Fig. 14–15).
compartment and the ligaments are intact.
Total knee arthroplasty is an exacting operation that
requires attention to the details of component position- Complications
ing and the balance of soft tissue structures. The final
tibiofemoral angulation of the knee should be 7 to 9 Patellar Complications. Revision surgery after total knee
degrees of valgus, which should re-create the normal arthroplasty is most commonly necessitated by patellar
mechanical axis of the leg (determined by constructing a complications. Stress fracture of the patella (Fig. 14–16),
line from the center of the femoral head through the loosening of the patellar component, and dislocation or
center of the knee and the center of the ankle). subluxation of the patellar component are the most
common problems encountered.
Postoperative Appearance Prosthetic Loosening. The incidence of prosthetic loosen-
Anteroposterior views of the knee taken in the supine and ing seems to be less in the knee than in the hip. The tibial
standing positions, along with lateral and tangential component is involved most often, and loosening develops
patellar views, are routinely obtained for the follow-up of most frequently at the cement-bone interface. A wide
patients with total knee replacements. Fluoroscopy may (≥2 mm) or enlarging lucent zone, collapse of subjacent
be a useful adjunct for viewing the cement-bone interface trabecular bone, fragmentation of underlying cement,
in profile. In the normal situation, anteroposterior views change in component position, development of a metal-
show femorotibial alignment of 7 to 9 degrees of valgus. cement lucent region, and change in the angulation of
If standing views of the knee are obtained, the center of the knee on weight-bearing radiographs are indications
the knee should lie on the mechanical axis of the leg. The of loosening of cemented components (Fig. 14–17). In
tibial component should be perpendicular in relation to addition, continued shedding of beads from the surface
the tibial shaft or in up to 2 degrees of varus alignment. of bone ingrowth components after the first 3 to 6 months
Varus position of the tibial component refers to relative postoperatively suggests prosthetic loosening. Arthrogra-
tilting of the component medially. On the lateral view, phy may confirm loosening of components by showing
evaluation often includes assessment of the distance of contrast agent insinuation within the cement-bone inter-
the tibial component from the anterior and posterior face (Fig. 14–18).
tibial margins, the tilt of the tibial and femoral compo-
nents in relation to the respective shafts of the tibia and
femur, the distance from the center of the tibial
component to the center of the tibia, and the height of
the joint line. The position of the patellar component is
judged on lateral and tangential patellar views.
A normal area of bone rarefaction occurs in the ante-
rior portion of the femur, beneath the femoral compo-
Figure 14–20. Silicone synovitis. A Silastic implant was used to replace a fractured scaphoid bone.
A, Coronal T1-weighted (TR/TE, 733/20) spin echo magnetic resonance (MR) image shows the signal void
of the scaphoid implant. Note the regions of intermediate signal intensity in multiple carpal bones,
corresponding to fluid within cystic lesions. The lunate-capitate joint space is narrowed. B, Coronal short
tau inversion recovery (STIR) MR image (TR/TE, 2000/30; inversion time, 140 msec) reveals the implant
of low signal intensity and carpal cysts and joint fluid of high signal intensity. Small particles of Silastic are
evident in the radial aspect of the wrist. (Courtesy of S. Eilenberg, MD, San Diego, Calif.)
Chapter 14 Imaging of Joint Replacement 207
FURTHER READING Kattapuram SV, Lodwick GS, Chandler H, et al: Porous-coated
anatomic total hip prostheses: Radiographic analysis and
Barrack RL, Harris WH: The value of aspiration of the hip clinical correlation. Radiology 174:861, 1990.
joint before revision total hip arthroplasty. J Bone Joint Surg Lieberman JR, Huo MH, Schneider R, et al: Evaluation of
Am 75:66, 1993. painful hip arthroplasties. J Bone Joint Surg Br 75:475, 1993.
Barrack RL, Tanzer M, Kattapuram SV, et al: The value of contrast Lindstrand A, Stenström A: Polyethylene wear of the PCA
arthrography in assessing loosening of symptomatic unce- unicompartmental knee. Acta Orthop Scand 63:260, 1992.
mented total hip components. Skeletal Radiol 23:37, 1994. Maus TP, Berquist TH, Bender CE, et al: Arthrographic study
Bergstrom B, Lidgren L, Lindberg L: Radiographic abnormali- of painful total hip arthroplasty: Refined criteria. Radiology
ties caused by postoperative infection following total hip 162:721, 1987.
arthroplasty. Clin Orthop 99:95, 1974. Mian SW, Truchly G, Pflum FA: Computed tomography
Charnley J: Arthroplasty of the hip: A new operation. Lancet measurement of acetabular cup anteversion and retroversion
1:1129, 1961. in total hip arthroplasty. Clin Orthop 276:206, 1992.
Chew FS, Lev MH: Polyethylene osteolysis. AJR Am J O’Neill DA, Harris WH: Failed total hip replacement: Assess-
Roentgenol 159:1254, 1992. ment by plain radiographs, arthrograms, and aspiration of
Dussault RG, Goldman AB, Ghelman B: Radiologic diagnosis the hip joint. J Bone Joint Surg Am 66:540, 1984.
of loosening and infection in hip prostheses. J Can Assoc Oswald SG, Van Nostrand D, Savory CG, et al: Three-phase
Radiol 28:119, 1977. bone scan and indium white blood cell scintigraphy following
Fackler CD, Poss R: Dislocation in total hip arthroplasties. porous coated hip arthroplasty: A prospective study of the
Clin Orthop 151:169, 1980. prosthetic tip. J Nucl Med 30:1321, 1989.
Gruen MS, McNeice GM, Amstutz HC: “Modes of failure” of Resnick D, Kerr R, André M, et al: Digital arthrography in the
cemented stem-type femoral components. Clin Orthop evaluation of painful joint prostheses. Invest Radiol 19:432,
141:17, 1979. 1984.
Harris WH, Barrack RL: Contemporary algorithms for evaluation Thomas BJ: Heterotopic bone formation. In Harlan CA (ed):
of the painful total hip replacement. Orthop Rev 22:531, Hip Arthroplasty. New York, Churchill Livingstone, 1991,
1993. p 405.
Harris WH, Barrack RL: Developments in diagnosis of the Tigges S, Stiles RG, Meli RJ, et al: Hip aspiration: A cost-
painful total hip replacement. Orthop Rev 22:439, 1993. effective and accurate method of evaluating the potentially
Insall JN: Presidential address to the Knee Society: Choices and infected hip prosthesis. Radiology 189:485, 1993.
compromises in total knee arthroplasty. Clin Orthop 226:43, Weissman BN: Current topics in the radiology of joint
1988. replacement surgery. Radiol Clin North Am 28:1111,
Jaffe WL, Scott DF: Total hip arthroplasty with hydroxyapatite- 1990.
coated prostheses. J Bone Joint Surg Am 78:1918, 1996. Weissman BN: Radiographic evaluation of total joint replace-
Johnston RC, Fitzgerald RH, Harris WH, et al: Clinical and ment. In Kelley WN, Harris ED, Ruddy S, Sledge CB (eds):
radiographic evaluation of total hip replacement. J Bone Textbook of Rheumatology, 4th ed. Philadelphia, WB Saunders,
Joint Surg Am 72:161, 1990. 1993, p 1881.
SECTION
V
Rheumatoid Arthritis and Related Diseases
CHAPTER 15
Rheumatoid Arthritis and the wrist, the knee, the elbow, and the glenohumeral and
acromioclavicular joints. The synovial articulations of the
Seronegative Spondyloarthropathies: axial skeleton may also be affected, especially the apophy-
seal and atlantoaxial joints of the cervical spine. In most
Radiographic and Pathologic of these synovium-lined cavities, changes are distributed
Concepts symmetrically on the right and left sides of the body and
consist of fusiform soft tissue swelling, regional osteo-
porosis, diffuse loss of joint space, marginal and central
erosions, and fibrous ankylosis. The synovium of bursae
SUMMARY OF KEY FEATURES and tendon sheaths is also affected. Abnormalities of carti-
The pathologic and radiographic abnormalities associated laginous articulations and entheses are less frequent and
with articular involvement in rheumatoid arthritis and extensive, with the exception of the discovertebral joints
the seronegative spondyloarthropathies (ankylosing of the cervical spine.
spondylitis, psoriatic arthritis, Reiter’s syndrome) are
similar in many respects. Involvement of synovial and Synovial Joints
cartilaginous joints, bursae, tendon sheaths, entheses,
General Enzymatic Factors. The fundamental target area
tendons, ligaments, soft tissues, and bones can be
encountered in any of these disorders. The distribution in rheumatoid arthritis is the synovium, which lines the
and extent of abnormalities differ among these diseases, articulation. Rheumatoid synovitis is the result of a cellular
however. In rheumatoid arthritis, alterations in synovium- immune response to an antigen present in the synovial
lined articulations, bursae, and tendon sheaths frequently membrane. The precise identity of this antigen is not clear;
overshadow those in cartilaginous joints and sites of however, the response to it includes the production of a
tendon and ligament attachment to bone. In ankylosing number of factors that lead to the formation of an erosive
spondylitis, psoriatic arthritis, and Reiter’s syndrome, pannus and inflammatory mediators that induce joint
abnormalities can be severe at cartilaginous articulations, destruction (Table 15–1).
including the discovertebral and manubriosternal joints Synovial Membrane. The earliest recognizable pathologic
and symphysis pubis. In addition, in those three condi- abnormality in rheumatoid arthritis is acute synovitis,
tions, a peculiar enthesopathy produces bone erosion which is associated with congestion and edema of the
and proliferation at tendo-osseous junctions. synovial membrane (Fig. 15–1). Capillary proliferation
TABLE 15–1
INTRODUCTION Abnormalities of Synovial Joints in Rheumatoid
Arthritis
Rheumatoid arthritis and the seronegative spondy-
loarthropathies (ankylosing spondylitis, psoriatic arthritis,
Pathologic Radiologic
and Reiter’s syndrome) have many radiographic and patho-
logic characteristics in common. They affect synovium- Synovial inflammation and Soft tissue swelling and
lined joints, bursae, and tendon sheaths; cartilaginous production of fluid widening of joint
articulations; entheses, or sites of ligamentous and tendi- space
nous attachment to bone; soft tissues; and bones. They lead Hyperemia Osteoporosis
to inflammation in a variety of tissues, and although the Pannus destruction of cartilage Narrowing of joint space
distribution and extent of abnormalities at specific target Pannus destruction of Marginal bony erosions
areas in the body vary among the disorders, the muscu- “unprotected” bone at
loskeletal effects of this inflammation are fundamentally margin of joint
similar. Pannus destruction of Bony erosions and
subchondral bone formation of
subchondral cysts
RHEUMATOID ARTHRITIS Fibrous and bony ankylosis Bony ankylosis
Overview Laxity of capsule and ligaments Deformity, subluxation,
and muscular contraction dislocation, fracture,
The major abnormalities of rheumatoid arthritis appear and spasm fragmentation, and
in synovial articulations of the appendicular skeleton, sclerosis
particularly the small joints of the hand and foot, the
209
210 Section V Rheumatoid Arthritis and Related Diseases
Figure 15–1. Synovial joint abnormalities in rheumatoid arthritis: pathologic overview. In a normal joint
(1), observe the articular cartilage and synovial membrane. At the edges of the articulation (arrowheads), syn-
ovium abuts on bone that does not possess protective cartilage. The very early abnormalities of rheumatoid
arthritis (2) consist of synovial proliferation (open arrows), soft tissue edema (solid arrows), and osteoporosis.
At a slightly later stage (3), the inflamed synovial tissue or pannus (open arrow) has extended across the
cartilaginous surface and is leading to chondral erosion. Capsular distention, soft tissue edema, and
osteoporosis are seen. Small osseous erosions at the margins of the joint are appearing (arrowheads). In more
advanced stages (4, 5), large marginal and central erosions and “cysts” are noted (arrowheads). In advanced
rheumatoid arthritis (6), fibrous ankylosis of the joint is typical.
and abnormal permeability are accompanied by exuda- early radiographic sign of rheumatoid arthritis—can be
tion of plasma and fibrin precipitation. Accumulation of demonstrated. This finding, which is not invariable, pro-
erythrocytes results from altered capillary permeability, duces thinning and small areas of discontinuity or gaps
and phagocytosis of these cells leads to hemosiderin depo- (dot-dash pattern) in the subchondral bone plate.
sition. These microscopic abnormalities result in a macro-
scopically evident thickened and injected synovial Articular Cartilage. After an acute inflammatory episode,
membrane. Continued proliferation may produce a bulky, the inflamed synovium soon spills from the peripheral
hypervascular synovial tissue that superficially resembles portion and marginal pockets of the joint and grows toward
a tumor. This tissue projects into the joint lumen as villous the cartilaginous tissue. The synovial tissue applied to the
fronds containing many cells that maintain contact with articular cartilaginous surface causes morphologic changes.
the synovial fluid. These changes result from enzymatic destruction of car-
The pathologic findings described occur in the “syn- tilage, interference with proper cartilaginous nutrition, or
ovial” stage of rheumatoid arthritis and are accompanied both. Cartilage destruction may ultimately become wide-
by characteristic radiographic abnormalities (Fig. 15–2). spread and severe; when the cartilaginous surface on two
Accumulation of synovial inflammatory tissue within the apposing bones is significantly compromised, granulation
joint, an increase in intra-articular fluid, capsular disten- tissue may bridge the articular cavity.
tion, and surrounding soft tissue edema lead to one early The radiographic counterpart of this “cartilaginous”
radiographic finding of the disease—soft tissue swelling. stage of rheumatoid arthritis is loss of the articular space
The periarticular soft tissue prominence is generally (see Fig. 15–2). Classically, joint space diminution is a rela-
distributed symmetrically or is fusiform in configuration. tively early finding in the disease, and it may appear within
In response to the hyperemia provoked by synovial inflam- a period of weeks or months in some patients. Along with
mation, regional or periarticular osteoporosis—the second soft tissue swelling and periarticular osteoporosis, joint
Chapter 15 Rheumatoid Arthritis and the Seronegative Spondyloarthropathies: Radiographic and Pathologic Concepts 211
A B
space narrowing is one of three early radiographic charac- The initial erosions in rheumatoid arthritis occur at the
teristics of rheumatoid arthritis. Loss of the articular space margins or unprotected areas of the articulation, where
is usually diffuse or widespread because of the generalized pannus is intimate with osseous tissue that does not possess
nature of the cartilaginous destruction. This pattern of protective cartilage. Symmetrically distributed defects
articular space diminution differs from the focal, seg- appear on both the proximal and distal bones that consti-
mental, or asymmetrical type of joint space loss that occurs tute the articulation; they are usually, but not invariably,
in osteoarthritis. accompanied by soft tissue swelling, periarticular osteo-
porosis, and joint space narrowing. Such erosions may be
Subchondral Bone. Two alterations in subchondral bone a very early manifestation of the disease and occur within
that occur in the early stages of rheumatoid arthritis are 1 or 2 years of the onset of joint symptoms.
osteoporosis and marginal erosion. Osteoporosis, which The radiographic appearance of multiple subchondral
can progress with time and eventually extend along the lucent areas throughout the articulation in patients with
adjacent diaphyses, is related to a combination of disuse of early or more advanced rheumatoid arthritis is well recog-
the articulation, osteoclastic resorption of the subchon- nized. These lesions may develop in several different
dral spongy trabeculae, and steroid-induced bone changes. fashions, including intraosseous extension of pannus, nutri-
212 Section V Rheumatoid Arthritis and Related Diseases
TABLE 15–2
Figure 15–5. Synovial joint abnormalities in rheumatoid
arthritis: productive changes in bone. On a radiograph of a Abnormalities of Bursae and Tendon Sheaths in
coronal section of the knee, observe the abnormalities related Rheumatoid Arthritis
to bone production in the form of osteophytes (solid arrow) and
subchondral sclerosis (open arrow). These findings should not Pathologic Radiologic
be regarded as evidence of a diagnosis other than rheumatoid Synovial inflammation and Soft tissue swelling
arthritis. In this case, involvement of both the medial femorotibial production of fluid
space and lateral femorotibial space, with predominant
involvement of the latter area, aids in the accurate diagnosis of Hyperemia Osteoporosis
rheumatoid arthritis. Fragmentation of the articular surface, Pannus destruction of subjacent Surface resorption of
with the formation of a large intra-articular body (arrowhead), is bone bone
seen.
A B
214 Section V Rheumatoid Arthritis and Related Diseases
itself may be affected and become disposed to a variety of the frequency and severity of such involvement are far
complications, including weakening, subluxation, entrap- less striking than in the seronegative spondyloarthropathies.
ment, and rupture. Of the cartilaginous joints, the alterations of the discover-
The most common radiographic finding associated with tebral junction in rheumatoid arthritis receive the most
tenosynovitis and bursitis is soft tissue swelling. Erosion attention (Table 15–3). Such changes predominate in the
of subjacent bone can be observed, particularly in the cervical region, although the pathogenesis of these alter-
posterosuperior aspect of the calcaneus (in relation to ations is unclear. Various schools of thought have
retrocalcaneal bursitis), the olecranon process (in relation advanced the following suggestions (Fig. 15–9): (1) dis-
to olecranon bursitis), and the inferior surface of the
acromion and distal end of the clavicle (in relation to
subacromial bursitis). In addition, surface resorption of TABLE 15–3
bone beneath inflamed tendon sheaths is characteristic, Abnormalities of Discovertebral Cartilaginous Joints in
particularly in the outer aspect of the distal portion of the Rheumatoid Arthritis
ulna (extensor carpi ulnaris tenosynovitis) (see Fig. 15–7).
Arthrography, bursography, tenography, ultrasonography, Potential Mechanism Pathogenesis
and MR imaging (Fig. 15–8) can be used to directly
Synovial inflammation “Pannus” is derived from joints
delineate the nature of the soft tissue swelling and the of Luschka (cervical region)
extent of synovial inflammation. and costovertebral articulations
(thoracic region)
Cartilaginous Joints and Entheses Trauma Apophyseal joint instability leads
to traumatic disruption of the
Although involvement of cartilaginous joints, such as the discovertebral junction with
symphysis pubis and manubriosternal and discovertebral cartilaginous node formation
articulations, and of entheses, such as those related to the Enthesopathy Inflammation at ligamentous and
spinous processes of the vertebrae, the inferior surface of capsular attachments leads to
the calcanei, the iliac wings, the ischial tuberosities, and the adjacent osseous erosion
femoral trochanters, is observed in rheumatoid arthritis,
Chapter 15 Rheumatoid Arthritis and the Seronegative Spondyloarthropathies: Radiographic and Pathologic Concepts 215
A B C
Figure 15–9. Abnormalities of the discovertebral junctions of the cervical spine in rheumatoid arthritis.
A, The normal situation. B, Proponents of the synovial school of thought suggest that inflammatory tissue
in the neurocentral joints of Luschka (solid arrow) spreads across the discovertebral junction, a process
leading to osseous erosion (open arrows). C, Proponents of the traumatic school of thought believe that
instability of the apophyseal joints resulting from synovial inflammation (arrowhead) produces recurrent
discovertebral trauma, which leads to cartilaginous nodes with surrounding sclerosis (open arrows).
A B
A B
joint space narrowing or osteoporosis. Serologic tests for Although rheumatoid synovial cysts are observed most
rheumatoid factor are commonly positive in these persons, frequently in the popliteal region, they have been described
and biopsy of the nodules and synovium reveals typical at other sites, including the calf, knee, ankle, plantar aspect
histologic changes of rheumatoid arthritis. of the foot, hip, hand and wrist, elbow, and shoulder.
Presumably, an extra-articular synovial mass can arise
Calcification. Soft tissue calcification is rare in rheumatoid around any involved articulation in this disease.
arthritis. Calcific deposits within rheumatoid nodules
and within periarticular subcutaneous tissue have occa- Sinus Tracts. Cutaneous-articular sinus tracts are recog-
sionally been described in persons with this disease. In all nized as an occasional feature of rheumatoid arthritis.
such cases, the possibility of mixed connective tissue Classic “fistulous” rheumatism is related to the
disease or collagen vascular overlap syndromes must be appearance of chronic skin sinuses near affected joints in
considered. patients with rheumatoid arthritis. In these patients,
erythematous periarticular nodules erupt and form
Synovial Cysts. Synovial cysts are a well-known manifes- draining sinuses, often multiple, in the vicinity of the
tation of rheumatoid arthritis (Fig. 15–14). Although syn- hands and feet. Synovial cyst formation with sinus tract
ovial cysts may arise as a result of rupture of the joint formation is a rare complication of fistulous rheumatism.
capsule, with extravasation of fluid and secondary encap- Although spontaneous cutaneous drainage of popliteal
sulation, or as a result of herniation of the synovial mem- cysts has been reported in patients with rheumatoid
brane, most authorities now believe that these lesions arthritis, this complication is more frequent after direct
represent abnormal distention of various bursae that com- aspiration.
municate with the adjacent articulation. These communi-
cating channels have been well documented in the knee
and frequently possess a valvular mechanism, allowing Muscles
the flow of synovial fluid to proceed in only one direction In rheumatoid arthritis, generalized muscle weakness is
(from the articulation to the cyst, not from the cyst to the common, although other symptoms and signs related to
articulation). Clinically, it may be difficult to differentiate muscle involvement are rare. Possible causes of muscle
signs and symptoms related to synovial cysts about the involvement in this disease include muscle atrophy, steroid
knee from those caused by thrombophlebitis. Rupture of myopathy, peripheral neuropathy, and acute or chronic
a synovial cyst is accompanied by swelling and edema that myositis. Toxic myopathy precipitated by various thera-
may lead to stasis, inflammation of the wall of the vein, peutic agents is also well recognized.
and thrombophlebitis; alternatively, the soft tissue swelling
accompanying thrombophlebitis may produce an increase
in pressure within a synovial cyst and predispose it to Digital Vessels
rupture. Necrotizing arteritis in mesenteric, renal, and cerebral
locations; subacute arteritis in skeletal muscles; and
fibromuscular hyperplasia in peripheral vessels are all
recognized in rheumatoid arthritis. Vasculitis com-
plicated by digital gangrene and acro-osteolysis can be
seen in rheumatoid arthritis as well as in other collagen
vascular disorders. In patients with rheumatoid arthri-
tis, such acro-osteolysis is almost invariably associated
with severe erosive arthritis and is usually associated
with peripheral neuropathy. Vasculitis in rheumatoid
arthritis has been suggested as a potential cause of
ischemic necrosis.
Bones
Generalized or periarticular osteoporosis is a common
manifestation in patients with rheumatoid arthritis. The
pathogenesis of periarticular osteoporosis appears to be
related to the adjacent synovial inflammation with hyper-
emia; however, the cause of the generalized osteoporosis
in this disease is not entirely clear.
Osteoporosis producing bone weakening contributes
to the fractures that are not uncommonly encountered in
patients with rheumatoid arthritis (Table 15–4; Fig. 15–15).
These fractures may occur after minimal trauma or spon-
Figure 15–14. Synovial cysts in rheumatoid arthritis: MR taneously in either spinal sites (compression fractures of
imaging. This sagittal, fat-suppressed, fast spin echo (TR/TE, vertebral bodies) or extraspinal locations. Typical loca-
2900/34) MR image reveals a synovial cyst (arrow) with extrava- tions include the femur, tibia, fibula, sacrum, and para-
sation of fluid (arrowhead), indicative of rupture. acetabular and symphyseal regions. Accurate diagnosis of
Chapter 15 Rheumatoid Arthritis and the Seronegative Spondyloarthropathies: Radiographic and Pathologic Concepts 219
TABLE 15–4 insufficiency fractures can be difficult and requires a high
index of clinical suspicion, quality radiographs, and even
Mechanisms and Sites of Pathologic Fractures in scintigraphy, computed tomography, or MR imaging
Rheumatoid Arthritis
(Fig. 15–16).
Erosive abnormality of bone related to the rheuma-
Mechanism Typical Sites
toid process itself can also lead to fracture and deformity.
Synovial inflammation Odontoid process, carpal Thus, pathologic fractures through the odontoid process
with erosion of bone scaphoid bone, distal and olecranon process and acetabular weakening and frac-
portion of ulna ture leading to protrusion deformity are observed. Other
Mechanical erosion of Ribs, articular surfaces of sites include the scaphoid and humeral neck (Fig. 15–17),
bone small bones in hands and as well as the phalanges of the hand.
feet, medial aspect of
humeral neck
Intraosseous cystic lesions Proximal portion of ulna,
femoral neck, femur and SERONEGATIVE SPONDYLOARTHROPATHIES
tibia about the knee Overview
Bone deformation Acetabulum
The three major seronegative spondyloarthropathies—
Generalized osteopenia Insufficiency fractures of
ankylosing spondylitis, psoriatic arthritis, and Reiter’s
vertebral bodies, pelvis,
tubular bones of lower syndrome—share many radiologic and pathologic features
extremity, small bones of with rheumatoid arthritis. They, too, involve synovial joints
foot and are associated with considerable inflammation of the
Ischemic necrosis Femoral head, vertebral synovial membrane. Fundamental differences exist between
bodies the spondyloarthropathies and rheumatoid arthritis,
Osteomyelitis Variable however, especially in the distribution and morphology
of osteoarticular lesions (Table 15–5).
A B
TABLE 15–5
Rheumatoid Arthritis versus the Seronegative
Spondyloarthropathies
TABLE 15–6
Abnormalities of Synovial Joints in the Seronegative Spondyloarthropathies
Pathologic Radiologic
Synovial inflammation and production of fluid Soft tissue swelling and widening of the joint space
Mild to moderate hyperemia Variable osteoporosis
Pannus destruction of cartilage Narrowing of the joint space
Pannus destruction of “unprotected” bone at the margin of a joint Marginal bony erosions
Pannus destruction of subchondral bone Bony erosions and formation of subchondral cysts
Fibroplasia, cartilaginous metaplasia, chondro- Bony ankylosis
ossification, capsular ossification
Bony proliferation in response to damage Marginal “whiskering,” periostitis, subchondral sclerosis
Noninflammatory proliferation of the periosteum Cortical atrophy, osteolysis
222 Section V Rheumatoid Arthritis and Related Diseases
calcaneal surface (Fig. 15–20). In some patients, prolifera- Cartilaginous Joints and Entheses
tion about the bony erosions can create poorly defined The tendency for ankylosing spondylitis, psoriatic
margins. Tenosynovitis, a frequently encountered mani- arthritis, and Reiter’s syndrome to affect cartilaginous
festation of rheumatoid arthritis, may occasionally be a articulations and the sites of tendon and ligament
prominent feature in the seronegative spondy- attachment to bone is well known (Table 15–7). At the
loarthropathies. Considerable soft tissue swelling in this manubriosternal joint and symphysis pubis, progressive
clinical situation can produce a sausage-shaped finger or fibrosis and bone formation lead to bone trabecular
toe (Fig. 15–21). thickening and intra-articular ossification. Similar abnor-
Chapter 15 Rheumatoid Arthritis and the Seronegative Spondyloarthropathies: Radiographic and Pathologic Concepts 223
malities in the discovertebral joint produce “osteitis” of
the anterior vertebral margin, which is accompanied by
erosion and sclerosis of bone and the progressive discal
ossification associated with syndesmophyte formation
(Fig. 15–22). At each of the cartilaginous articulations
(manubriosternal joint, symphysis pubis, discovertebral
junction), the changes may relate not only to true chondro-
osseous inflammation but also to an enthesopathy at the
peripheral capsular attachments.
The pathogenesis of destructive lesions at single or
multiple discovertebral junctions during the course of
ankylosing spondylitis is not clear, and several mecha-
nisms probably contribute to these lesions. These include
an increasing enthesopathy, pressure destruction of the
intervertebral disc related to kyphosis, cartilage node
formation, and pseudarthrosis about a fracture site. The
last-mentioned mechanism is expected in long-standing
disease in which a rigid vertebral column, caused by
widespread bony ankylosis, is predisposed to fracture in a
manner similar to that of a long tubular bone. Continued
motion at the fracture site leads to improper fracture
Figure 15–19. Synovial joint abnormalities in seronegative healing, often designated a pseudarthrosis, in which callus
spondyloarthropathies: radiographic changes of bony prolifera- formation, hemorrhage, fibrous proliferation, and mild
tion, or “whiskering.” In a cadaver with ankylosing spondylitis, inflammatory changes are seen.
observe the irregular osseous outline caused by superficial osseous An enthesopathy occurring at other tendinous and liga-
erosion with adjacent bony proliferation (arrows). Osteoporosis mentous connections to bone in patients with seronega-
is not apparent.
TABLE 15–7
Abnormalities of Cartilaginous Joints and Entheses in
the Seronegative Spondyloarthropathies
Pathologic Radiologic
Inflammation of subchondral Bony erosion and sclerosis
bone
Bony proliferation Bony ankylosis
Inflammation of capsular, Bony erosion and sclerosis
ligamentous, and tendinous
attachments
A B
Chapter 15 Rheumatoid Arthritis and the Seronegative Spondyloarthropathies: Radiographic and Pathologic Concepts 225
Goupille P, Roulot B, Akoka S, et al: Magnetic resonance imaging:
A valuable method for the detection of synovial inflam-
mation in rheumatoid arthritis. J Rheumatol 28:35, 2001.
Jaffe HL: Metabolic, Degenerative and Inflammatory Diseases of
Bones and Joints. Philadelphia, Lea & Febiger, 1972, p 779.
Kaye BR, Kaye RL, Bobrove A: Rheumatoid nodules: Review
of the spectrum of associated conditions and proposal of a
new classification, with a report of four seronegative cases.
Am J Med 76:279, 1984.
Martel W: Pathogenesis of cervical discovertebral destruction
in rheumatoid arthritis. Arthritis Rheum 20:1217, 1977.
Martel W, Hayes JT, Duff IF: The pattern of bone erosion in the
hand and wrist in rheumatoid arthritis. Radiology 84:204,
1965.
Rapoport AS, Sosman JL, Weissman BN: Lesions resembling
gout in patients with rheumatoid arthritis. AJR Am J
Roentgenol 126:41, 1976.
Rauschning W: Popliteal cysts and their relation to the gastroc-
nemiosemimembranosus bursa: Studies on the surgical and
functional anatomy. Acta Orthop Scand 179(Suppl):9, 1979.
Resnick D: Inflammatory disorders of the vertebral column:
Seronegative spondyloarthropathies, adult-onset rheumatoid
arthritis, and juvenile chronic arthritis. Clin Imaging 13:253,
1989.
Resnick D: Radiology of seronegative spondyloarthropathies.
Clin Orthop 143:38, 1979.
Figure 15–23. Abnormalities of entheses in seronegative Resnick D, Cone R: Pathological fractures in rheumatoid
spondyloarthropathies. Observe the poorly defined erosion and arthritis: Sites and mechanisms. Radiographics 4:549, 1984.
reactive bone formation in the ischial tuberosity in a patient Resnick D, Feingold ML, Curd J, et al: Calcaneal abnormalities
with ankylosing spondylitis. (Courtesy of V. Vint, MD, San Diego, in articular disorders: Rheumatoid arthritis, ankylosing
Calif.) spondylitis, psoriatic arthritis and Reiter’s syndrome.
Radiology 125:355, 1977.
Resnick D, Niwayama G: On the nature and significance of
bony proliferation in “rheumatoid variant” disorders. AJR
Am J Roentgenol 129:275, 1977.
FURTHER READING Resnick D, Niwayama G, Coutts R: Subchondral cysts (geodes)
in arthritic disorders: Pathologic and radiographic appear-
Ball J: Enthesopathy of rheumatoid and ankylosing spondylitis. ance of the hip joint. AJR Am J Roentgenol 128:799, 1977.
Ann Rheum Dis 30:213, 1971. Schils JP, Resnick D, Haghighi PN, et al: Pathogenesis of
Bywaters EGL: Pathology of the spondyloarthropathies. In discovertebral and manubriosternal joint abnormalities in
Calin A (ed): Spondyloarthropathies. New York, Grune & rheumatoid arthritis: A cadaveric study. J Rheumatol 16:291,
Stratton, 1984. 1989.
Bywaters EGL: Rheumatoid and other diseases of the cervical Schneider R, Kaye JJ: Insufficiency and stress fractures of the
interspinous bursae and changes in the spinous processes. long bones occurring in patients with rheumatoid arthritis.
Ann Rheum Dis 41:360, 1982. Radiology 116:595, 1975.
Cruickshank B: Pathology of ankylosing spondylitis. Clin Orthop Sokoloff L: The pathology of rheumatoid arthritis and allied
74:43, 1971. disorders. In Hollander JL, McCarty DJ Jr (eds): Arthritis
Fassbender HG: Pathology of Rheumatic Diseases. New York, and Allied Conditions, 8th ed. Philadelphia, Lea & Febiger,
Springer-Verlag, 1975, p 79. 1972, p 309.
Gardner DL: Pathology of rheumatoid arthritis. In Copeman’s Sugimoto H, Takeda A, Hyodoh K: Early stage rheumatoid
Textbook of the Rheumatic Diseases, 5th ed. Edinburgh, arthritis: Prospective study of the effectiveness of MR
Churchill Livingstone, 1978, p 273. imaging for diagnosis. Radiology 216:569, 2000.
Ginsberg MH, Genant HK, Yu TF, et al: Rheumatoid nodu- Wu PC, Fang D, Ho EKW, et al: The pathogenesis of extensive
losis: An unusual variant of rheumatoid disease. Arthritis discovertebral destruction in ankylosing spondylitis. Clin
Rheum 18:49, 1975. Orthop 230:154, 1988.
16
CHAPTER 16
Rheumatoid Arthritis 2. Soft tissue swelling (arthritis) of three or more joint
areas observed by a physician
3. Swelling (arthritis) of the proximal interphalangeal,
metacarpophalangeal, or wrist joints
SUMMARY OF KEY FEATURES
4. Symmetrical swelling (arthritis)
Rheumatoid arthritis is a common articular disorder
5. Rheumatoid nodules
with a characteristic radiographic picture. A symmetrical
polyarticular disease of the synovial joints of the 6. Presence of rheumatoid factor
appendicular skeleton is apparent, with prominent 7. Radiographic erosions, periarticular osteopenia, or
abnormalities of the proximal interphalangeal and both, in the hand, wrist, or both joints
metacarpophalangeal joints of the hand, the wrist, the Criteria 1 through 4 must have been present for at least
metatarsophalangeal joints of the foot, the posterior and 6 weeks. Rheumatoid arthritis is defined by the presence
plantar aspects of the calcaneus, the knee, the elbow, the of four or more criteria, and no further qualification (e.g.,
glenohumeral and acromioclavicular joints, the ankle, classic, definite, probable) or list of exclusions is required.
and the hip; these abnormalities are commonly combined
with changes in the cervical spine. This distribution of
synovial joint involvement generally allows an accurate CLINICAL ABNORMALITIES
diagnosis, especially when the involvement is characterized General Features
by fusiform soft tissue swelling, regional or periarticular
osteoporosis, marginal and central osseous erosions and Although rheumatoid arthritis can occur in persons of all
cysts, and diffuse loss of interosseous space. ages, it predominates in those between the ages of 25 and
55 years. Women are affected more commonly than men,
with the female-to-male ratio being approximately 2:1 to
3:1. Various prodromal symptoms have been noted in
rheumatoid arthritis, including fatigue, anorexia, weight
INTRODUCTION loss, malaise, and muscular pain and stiffness. These find-
Rheumatoid arthritis is an “everyday” disease whose ings can be obscured by prominent articular complaints,
general clinical, pathologic, and radiologic features are which are frequently an early manifestation of the disease.
well known to most physicians. An in-depth inspection Joint pain and stiffness may initially involve a single joint
of the radiographic and pathologic characteristics of rheu- for a period of weeks or months before more generalized
matoid arthritis provides a standard by which the other articular findings become evident. In these instances of
rheumatologic conditions can be measured. Such an monoarthritis, clinical diagnosis can be extremely diffi-
inspection must initially consider the basic pathology and cult. With the appearance of polyarticular disease, the
radiology of rheumatoid involvement of musculoskeletal correct diagnosis becomes more apparent. The eventual
tissue. This essential background information was pre- outcome of the joint disease is variable. Some patients
sented in Chapter 15. This chapter analyzes the changes demonstrate mild clinical manifestations for a long time,
produced by rheumatoid arthritis in specific locations of whereas others rapidly develop severe, disabling arthritis.
the body.
Articular Involvement
DIAGNOSTIC CRITERIA Articular involvement manifests as pain that is aggravated
Accurate diagnosis of rheumatoid arthritis is made with- by motion, swelling, stiffness, and limitation of move-
out difficulty in a patient with a generalized symmetrical, ment. Periarticular soft tissue swelling is fusiform or
peripherally located polyarthritis associated with (1) clini- spindle shaped, as a result of increased intra-articular
cally detectable severe morning stiffness, synovial inflam- fluid, synovial hypertrophy, and thickening of adjacent
mation, and subcutaneous nodules; (2) radiologic evidence soft tissues.
of an erosive articular process; (3) laboratory parameters The most typically affected joints are the proximal
of the disease, including a positive serologic test for rheu- interphalangeal and metacarpophalangeal joints of the
matoid factor and an elevated erythrocyte sedimentation hand, the wrist, the metatarsophalangeal joints of the
rate; and (4) pathologic documentation of typical rheuma- foot, the knee, the joints of the shoulder, the ankle, and,
toid lesions of the synovium and soft tissues. More trouble- to a lesser extent, the hip; any joint can be involved, how-
some, however, is establishing the presence of this disease ever. Although symmetry is the hallmark of joint involve-
in a person who has atypical clinical and radiologic fea- ment, some exceptions exist:
tures. Because of this difficulty, diagnostic criteria have 1. Symmetrical abnormalities of groups of joints (e.g.,
been proposed: metacarpophalangeal and metatarsophalangeal articu-
1. Morning stiffness in and around joints lasting at least lations) may be seen, but the same digits may not be
1 hour before maximal improvement affected on both sides of the body.
226
Chapter 16 Rheumatoid Arthritis 227
2. Initially, monoarticular or pauciarticular abnormalities phadenopathy, chronic leg ulceration, and abnormal skin
may be noted that do not obey the rule of symmetry. pigmentation. Women are affected more frequently than
3. A markedly asymmetrical or unilateral distribution may men, and the disease is rare in black persons. The articu-
be seen in patients with neurologic deficits. Unilateral lar manifestations of Felty’s syndrome are similar to those
muscle weakening or paralysis protects the ipsilateral of severe rheumatoid arthritis.
side from the effects of the articular disease.
Metabolic Bone Disorders. Osteopenia is a well-recognized
4. Differences in the distribution of rheumatoid arthritis manifestation of rheumatoid arthritis. In almost all patients,
are occasionally related to the sex of the patient. A such osteopenia relates to osteoporosis. In addition, osteo-
symmetrical distribution in the small peripheral joints malacia may contribute to osteopenia in some patients.
of the extremities is more typical in women than in
men. Sjögren’s Syndrome. Sjögren’s syndrome is a triad con-
5. Atypical cases of “rheumatoid arthritis” (those that do sisting of keratoconjunctivitis sicca, xerostomia, and con-
not meet all the necessary criteria for the disease) are nective tissue disease. The last feature is evident in 50%
often characterized by asymmetrical joint alterations. to 70% of patients with Sjögren’s syndrome and may be
identical to rheumatoid arthritis.
Continued Abnormalities
Figure 16–1. General distribution of disease in rheumatoid With further destruction of cartilage and bone, the artic-
arthritis. Rheumatoid arthritis is characteristically a ular space may be obliterated completely. Erosion of cen-
symmetrical arthritis of the small joints of the hands (proximal tral portions of the joint can lead to apparently enclosed
interphalangeal and metacarpophalangeal joints), feet radiolucent defects. In some patients, particularly those
(metatarsophalangeal joints and interphalangeal joint of the with rheumatoid nodulosis, prominent cystic lesions of the
great toe), wrists (all compartments), knees, ankles, elbows, phalanges may be unaccompanied by joint space narrow-
glenohumeral and acromioclavicular joints, and hips (arrows).
ing or significant synovitis.
In the axial skeleton, the articulations of the cervical spine are
typically affected (arrow). Less consistent involvement occurs in Although fibrous ankylosis is characteristically the ulti-
the thoracolumbar spine, sacroiliac and temporomandibular mate fate of severe arthritis of the metacarpophalangeal
joints, symphysis pubis, and manubriosternal joint (arrowheads). and proximal interphalangeal joints, occasional examples
of intra-articular osseous fusion can be seen in these loca-
tions. In almost all such instances, the proximal inter-
Spontaneous rupture of the extensor tendons of the digits
phalangeal joint is ankylosed; bony ankylosis of the
is a well-recognized complication of this disease.
metacarpophalangeal articulation is exceedingly unusual,
with the exception of that in the thumb.
Radiographic-Pathologic Correlation
Early Abnormalities Finger Deviations and Deformities
Radiographic abnormalities of the interphalangeal and Deviation and deformity of the fingers are common com-
metacarpophalangeal joints in rheumatoid arthritis are plications of rheumatoid arthritis.
well described. The second and third metacarpophalangeal
joints and the third proximal interphalangeal joint may Mallet Finger. Loosening or disruption of the distal attach-
reveal the earliest abnormalities (Figs. 16–2 to 16–4). ment of the extensor tendon to the terminal phalanx may
Fusiform soft tissue swelling, periarticular osteoporosis, result in the development of a typical mallet or drop
concentric loss of articular space, and marginal erosions finger. With progressive loosening of the collateral liga-
become evident at many or all of the proximal inter- ments, combined with the detrimental effect of the intra-
phalangeal and metacarpophalangeal joints. articular inflamed synovial tissue on cartilage and bone,
Marginal erosions about the distal interphalangeal instability of the distal interphalangeal joint can appear.
joints are generally small in comparison to those at the This is relatively uncommon.
more proximal digital joints. In addition to marginal
erosions, two other types of bone erosion have been Boutonnière Deformity. In normal situations, the balanced
noted in the hands of patients with rheumatoid arthritis: tendon mechanism and ligamentous restriction prevent
compressive (pressure) erosions and superficial surface collapse deformity of the digits, but in rheumatoid arthri-
resorption (Table 16–1). tis, this vulnerable balance is compromised by the direct
Chapter 16 Rheumatoid Arthritis 229
A B C
Figure 16–3. Metacarpophalangeal joint abnormalities: early changes. A, Initially, the bones appear normal,
with preservation of the subchondral bone plate on the radial aspect of the metacarpal head. B, Subsequently,
small radiolucent areas appear beneath the metacarpal head and are related to thinning of the bone plate
with focal discontinuity or gaps (arrowheads). Tiny erosions and surface irregularity of the proximal phalanx
are also evident (arrow). C, At a later stage, obvious osseous defects are seen (arrowheads). Note the mild
periosteal proliferation (arrow).
TABLE 16-1
Types of Bony Erosion in the Hand and Wrist in Rheumatoid Arthritis
or pain of the interphalangeal, metacarpophalangeal, and ulnar aspect of the wrist. Subsequent clinical features in
carpometacarpal joints. Boutonnière deformity (Z-shaped rheumatoid arthritis relate to dorsal subluxation of the
deformity, or hitchhiker’s thumb) is the most common distal portion of the ulna; carpal tunnel syndrome, attrib-
collapse deformity of the thumb in patients with rheuma- utable to synovitis in the carpal tunnel, with dysesthesias
toid arthritis. along the course of the median nerve; and rupture of one
or more extensor tendons. Further, synovial cysts arising
during the disease can create local soft tissue masses.
Wrist
Clinical Abnormalities
Radiographic-Pathologic Correlation
Although generally accompanied by abnormalities in the
Early Abnormalities
fingers, clinical (and radiologic) involvement of the wrist
may initially appear in the absence of significant digital The early radiographic and pathologic findings of rheuma-
alterations. Clinical findings may be related to synovitis toid wrist disease have been well delineated.
in any of the compartments of the wrist, adjacent tenosyn-
ovitis, and attenuation or injury of several soft tissue,
tendinous, and ligamentous structures. Extensor carpi
ulnaris tenosynovitis creates a painless swelling on the
A B
Figure 16–7. Abnormalities of the distal end and styloid
process of the ulna: sites of early soft tissue swelling and osseous
erosion. A, Soft tissue swelling about the distal end of the ulna
(U) may appear as distention of the prestyloid recess of the Figure 16–9. Abnormalities of the radial styloid process and
radiocarpal compartment (arrowhead), the inferior radioulnar scaphoid. Radiograph of a rheumatoid wrist shows erosion of
compartment (solid arrow), or the extensor carpi ulnaris tendon the radial styloid process (arrowhead) and lateral midportion of
sheath (open arrow). B, Early osseous erosions appear at three the scaphoid bone (arrow), characteristic of rheumatoid arthri-
distinct areas in the distal portion of the ulna. tis. Widespread abnormalities are present throughout the wrist.
232 Section V Rheumatoid Arthritis and Related Diseases
B
Figure 16–11. Pancompartmental abnormalities of a rheuma-
toid wrist. A, Some of the typical sites of osseous erosion in
rheumatoid arthritis are indicated. Note that all the compart-
ments in the wrist are soon involved. B, Radiograph demon-
strates typical pancompartmental osseous erosions in rheuma-
toid arthritis.
Elbow
Clinical Abnormalities
The elbow is frequently involved in rheumatoid arthritis.
ciated with ulnar deviation at the metacarpophalangeal Involvement is commonly bilateral but may be more
joints, producing a zigzag deformity of the hand. marked in the dominant extremity, and it is usually asso-
ciated with polyarthritis. Clinical symptoms and signs are
Intercarpal Malalignment. Both palmar flexion instability variable but can lead to considerable disability as a result
(palmar, or volar, intercalated segment instability) and of limitation of both flexion and extension of the joint.
dorsiflexion instability (dorsal intercalated segment insta- Additional clinical manifestations include mild to mod-
bility) occur in the wrist in rheumatoid arthritis, although erate pain and tenderness, swelling over the lateral aspect
the latter is more frequent. Palmar flexion instability is of the joint between the radial head and the olecranon,
manifested by medial migration of the proximal row of antecubital soft tissue masses related to synovial cysts
carpal bones, with resulting palmar flexion of the lunate with compression of adjacent nerves, and paraolecranon
and scaphoid bones. Dorsiflexion instability is sometimes nodules, synovial cysts, or bursitis. Olecranon bursitis is
related to abnormality of the distal attachment of the seen not only in rheumatoid arthritis but also in gout and
palmar radiocarpal ligaments and disruption of the scapho- in association with trauma or infection.
lunate ligament.
Radiographic-Pathologic Correlation
Inferior Radioulnar and Distal Ulnar Malalignment. Rheu-
matoid arthritis deformities on the ulnar aspect of the Synovial inflammation in the elbow with progressive
wrist include distal and dorsal subluxation of the ulna destruction of cartilage and bone produces soft tissue
and diastasis of the inferior radioulnar compartment swelling with a positive “fat pad” sign, periarticular osteo-
(Fig. 16–13). The caput ulnae syndrome consists of pain, porosis, joint space narrowing, and bone erosions. Erosion
limited motion, and dorsal prominence of the distal end and deformity of the radial head, the coronoid process
of the ulna. The abnormally located, eroded head of of the ulna, and the distal portion of the humerus are
the ulna projects into the compartments of the extensor most typical. More severe changes are characterized by
tendons on the dorsum of the wrist and produces fraying extensive osteolysis of large portions of the humerus,
of the tendon surfaces. Mechanical attrition in associa- radius, and ulna. Prominent cystic lesions of the olecra-
tion with tenosynovitis may lead to weakening of these non process can fracture spontaneously or after minor
tendons and rupture. trauma (Fig. 16–14).
234 Section V Rheumatoid Arthritis and Related Diseases
A B
Figure 16–14. Abnormalities of the elbow: articular destruction. A, Radiograph of a sagittal section of
a rheumatoid elbow joint indicates considerable narrowing and bony eburnation of the humeroulnar space.
B, Lateral radiographs of the elbow in a different patient with rheumatoid arthritis demonstrate a fracture
of the distal portion of the humerus that occurred after minor trauma. The fragment and the radius and ulna
are displaced, predominantly in a proximal, radial, and posterior direction.
A B
Figure 16–15. Abnormalities of the glenohumeral joint: articular destruction and rotator cuff tear and
atrophy. A, Radiograph of a coronal section of the articulation illustrates joint space narrowing, bony
eburnation, and erosive and cystic changes, predominantly on the lateral aspect of the humeral head (arrows).
B, Radiograph of a coronal section of a rheumatoid glenohumeral joint indicates the presence of severe
articular changes and rotator cuff atrophy. Note the joint space narrowing and sclerosis, erosion of the
superolateral aspect of the humeral head, elevation of the humeral head with respect to the glenoid, and
narrowing of the acromiohumeral head distance (arrow). The rotator cuff is atrophic.
porosis, slight irregularity of the osseous surfaces, ebur- The earliest alterations appear at the metatarsopha-
nation, decreased height of the intervening fibrocartilage, langeal joints, particularly the fifth. Changes predom-
and bony ankylosis, but these changes are less frequent inate on the medial aspect of the metatarsal head, with
and less severe than in ankylosing spondylitis. the exception of the fifth digit, where soft tissue swelling
and subjacent osseous erosion on the lateral aspect of
Forefoot the bone can be very early and important findings
(Fig. 16–18).
Clinical Abnormalities
Early radiographic alterations at the metatarsopha-
Clinical abnormalities of the forefoot are especially com- langeal joints consist of soft tissue swelling, periarticular
mon in rheumatoid arthritis (80% to 90% of patients) osteoporosis, concentric joint space narrowing, and mar-
and may be the initial manifestation of the disease (10% ginal and central osseous defects. In the great toe, changes
to 20% of patients). The metatarsophalangeal joints of the in the metatarsal head and the proximal phalanx about
lateral digits are affected most frequently. Intermittent or the metatarsophalangeal joint are accompanied by osteo-
constant pain, tenderness, and soft tissue swelling can be porosis, joint space loss, and erosion of the adjacent
prominent findings. Characteristic deformities include sesamoids. The frequency of hallux valgus deformity rises
spreading of the metatarsal bones, hallux valgus, lateral with increasing duration of the disease. Especially charac-
deviation of the toes at the metatarsophalangeal joints, teristic is an elongated surface irregularity that appears
hammer toe, and “cock-up” toe. Additional findings in on the medial margin of the proximal phalanx adjacent to
rheumatoid arthritis include insufficiency (stress) fractures, the interphalangeal joint; this irregularity may be asso-
peripheral neuropathy, tendon injury and rupture, wide- ciated with smaller erosions on the medial aspect of the
spread edema, rheumatoid nodules, and hallux rigidus. adjacent distal phalanx. The radiographic characteristics
of a deformed forefoot in rheumatoid arthritis include
fibular deviation of the toes (with the exception of the
Radiographic-Pathologic Correlation
fifth digit) and dorsiflexion and lateral subluxation or dis-
Radiologic abnormalities of the forefoot are also frequent location of the proximal phalanges at the metatarsopha-
and are commonly the initial manifestation of the disease. langeal articulations (Fig. 16–19).
A B
Figure 16–18. Abnormalities of the forefoot: target areas. A, Early osseous erosions of rheumatoid
arthritis appear on the medial aspect of the first to fourth metatarsal bones, the medial and lateral aspects of
the fifth metatarsal bone, and the medial aspect of the distal portion of the proximal phalanx of the great
toe. B, Radiograph reveals early erosions. All the metatarsal heads are involved, particularly the medial
aspects. Note the prominent changes at the interphalangeal joint of the great toe and the relative absence of
findings in other interphalangeal articulations.
Chapter 16 Rheumatoid Arthritis 237
A B
Hip
Clinical Abnormalities
Abnormalities of the hip are far less frequent than those
of the knee and increase with the duration and severity of
rheumatoid arthritis. Pain, tenderness, shortening of the
limb, gait abnormalities, and decreased range of motion,
particularly internal rotation, extension, and abduction,
are observed. Soft tissue swelling on the anterior aspect
of the joint and over the greater trochanter (secondary to
bursitis) may be evident.
Radiographic-Pathologic Correlation
Radiographic abnormalities of the hip are generally bilat-
eral and symmetrical. The most typical early abnormality
is loss of joint space (Fig. 16–24). In almost all patients,
diminution of articular space is concentric and reflects Figure 16–25. Abnormalities of the hip: late changes with
thinning and loss of the cartilaginous coat over much of acetabular protrusion. Protrusion deformity with acetabular
the femoral and acetabular surfaces. The femoral head fragmentation is shown. Observe the small size of the femoral
moves centrally along the axis of the femoral neck (axial head.
migration). Much less frequently, greater loss of joint
space appears on the superior aspect of the joint and pro-
duces upward or superior migration of the femoral head
with respect to the acetabulum, similar to that accom- line so that the distance between this line and the later-
panying osteoarthritis. Eventually, this space may be com- ally located ilioischial line is 3 mm or more in men and
pletely obliterated, and the femoral head and acetabulum 6 mm or more in women—is particularly characteristic of
protrude into the pelvis (Fig. 16–25). Acetabular pro- rheumatoid arthritis. The deformity is commonly
trusion—defined as inward movement of the acetabular bilateral and is associated with subchondral cystic lesions,
osseous collapse of the acetabular roof and femoral head,
and osteoporosis.
Radiographically, the earliest lucent zones appear at
the chondro-osseous margin of the femoral head near the
femoral neck. Additional lesions are observed as surface
irregularities throughout the femoral head and, to a lesser
degree, the acetabulum. Some degree of sclerosis may
appear about the hip after a considerable length of time.
The eventual outcome of rheumatoid hip disease is often
complete obliteration of the articular space and fibrous
ankylosis. Bony ankylosis is exceedingly rare.
Osteonecrosis of the femoral head is not uncommon
and generally occurs in patients with rheumatoid arthritis
who are being treated with corticosteroids. The radio-
graphic appearance of osteonecrosis of the femoral head
in a relatively normal joint with osseous collapse, cyst
formation, and sclerosis without joint space loss is iden-
tical to that of osteonecrosis occurring in patients with-
out rheumatoid disease.
Sacroiliac Joint
Figure 16–24. Abnormalities of the hip: early changes. As opposed to the frequent and severe clinical and radio-
Radiograph of a coronal section of a rheumatoid cadaveric hip logic abnormalities that characterize sacroiliac joint
indicates diffuse loss of articular space, with migration of the disease in ankylosing spondylitis, changes in this location
femoral head axially along the axis of the femoral neck. are relatively infrequent and mild in rheumatoid arthritis.
240 Section V Rheumatoid Arthritis and Related Diseases
Symphysis Pubis
The symphysis pubis, which is frequently affected in
the seronegative spondyloarthropathies, is not com-
monly altered in rheumatoid arthritis. Occasionally,
radiographic abnormalities are detected in patients with
chronic disease, but they are generally clinically silent.
These abnormalities include subchondral erosion, mild
eburnation, and narrowing of the interosseous space.
Parasymphyseal insufficiency fractures of the os pubis
have been described in patients with rheumatoid
arthritis, as well as in those with postmenopausal Figure 16–26. Abnormalities of the thoracolumbar spine:
osteoporosis. discovertebral lesions. Lateral conventional tomogram of the
lumbar spine in a rheumatoid arthritis patient shows disc space
narrowing, poorly defined osseous erosion, and sclerosis, which
Ribs resemble the findings in infection and intervertebral
osteochondrosis.
Abnormalities of the thoracic cage in patients with
rheumatoid arthritis can include erosions on the supe-
rior margin of the posterior aspect of the upper ribs.
They are identical to those encountered in other col- Cervical Spine
lagen vascular disorders, neurologic processes, and
Clinical Abnormalities
chronic obstructive lung disease. Rib erosions in
rheumatoid arthritis, as well as in some other pro- Cervical spine involvement is common in rheumatoid
cesses, are probably due to a pressure effect from the arthritis and can lead to severe pain and disability, as well
scapula. as a variety of neurologic manifestations, although some
patients with significant radiographic evidence of disease
may be entirely asymptomatic. Initial or predominant
Thoracic and Lumbar Spine involvement of the cervical spine can occur without
When compared with the distinctive alterations of the obvious clinical (or radiographic) abnormalities at other
cervical spine, which represent a common and well- locations.
known manifestation of rheumatoid arthritis, abnormal- Symptoms and signs related to cervical spine abnor-
ities of the thoracic and lumbar spine are relatively malities develop in approximately 60% to 80% of rheu-
uncommon. Rheumatoid arthritic changes in the apophy- matoid arthritis patients at some time during their illness.
seal joints of the thoracic and lumbar spine are reported Pain is the most common clinical manifestation. Weak-
only infrequently. Alterations at the discovertebral junc- ness and abnormal mobility may also be evident. Neuro-
tion of the thoracic and lumbar spine are also infrequent. logic manifestations include paresthesias, paresis, and
Intervertebral disc space narrowing, irregularity of the muscle wasting; in some instances, quadriplegia and death
subchondral margins of the vertebral bodies, “erosion,” can occur.
and sclerosis may be evident on radiography of these sites
(Fig. 16–26).
Radiographic-Pathologic Correlation
Patients with rheumatoid arthritis who are receiving
corticosteroid medication are predisposed to ischemic General Features. The entire cervical spine is affected by
necrosis of bone. Although the femoral head is the typi- the rheumatoid process; changes may be evident as far
cal site of this complication, vertebral bodies in the cephalad as the base of the occiput and as far caudad as
thoracic and lumbar segments of the spine can be the C7–T1 junction (Table 16–2). Further, synovial and
affected. Radiographic abnormalities include vertebral cartilaginous articulations, the joints of Luschka, tendi-
collapse and fragmentation. Radiolucent fracture lines, nous and ligamentous attachments, and soft tissues of
which may accumulate gas from surrounding tissues the cervical region can exhibit significant abnormalities
(vacuum vertebral body), are an important clue that in this disease. In patients who can undergo complete
allows a correct diagnosis. radiographic evaluation, the frequency of radiographic
Chapter 16 Rheumatoid Arthritis 241
TABLE 16-2
Cervical Spine Abnormalities in Rheumatoid Arthritis
Occipitoatlantoaxial Articulations
Atlantoaxial subluxation
Odontoid erosion and fracture
Apophyseal joint erosion, sclerosis, and fusion
Subaxial Articulations
Subaxial subluxation
Apophyseal joint erosion, sclerosis, and fusion
Intervertebral disc space narrowing
Erosion and sclerosis of vertebral body margins
Spinous process erosion
Osteoporosis
changes of the cervical spine is consistently high and may Figure 16–27. Abnormalities of the cervical spine: directions
of atlantoaxial subluxation. Varying types of subluxation may
reach 85%. Radiographic abnormalities in the cervical
occur at the atlantoaxial articulations in patients with
spine have been reported to correlate with those in the rheumatoid arthritis. Most typically, anterior movement of the
peripheral joints and with the presence of serum rheuma- atlas with respect to the axis (1) is seen. Vertical translocation of
toid factor and subcutaneous nodules. Careful radio- the odontoid process, or cranial settling (2), can also occur.
graphic technique is important; in an apparently normal Lateral subluxation (3) can be recorded on frontal radiographs
cervical spine, considerable subluxation at the C1–C2 as asymmetry becomes apparent between the odontoid process
junction can be revealed with the addition of a lateral and the lateral masses of the atlas. In addition, the anterior arch
view of the flexed neck. When all radiographic para- of the atlas can move inferiorly (4) with respect to the odontoid
meters are used, demonstration of abnormalities through- process, a finding associated with cranial settling. Finally, in the
out the cervical spine is frequent, although they usually presence of severe erosion of the odontoid process, the anterior
arch can move posteriorly against the eroded bone or, rarely,
predominate in the upper cervical region.
behind the eroded odontoid process.
Occipitoatlantoaxial Articulations. Various types of malalign-
ment in the atlantoaxial region have been described in
rheumatoid arthritis (Fig. 16–27). The major types include arteries, and hydrocephalus. Atlantoaxial impaction is
anterior atlantoaxial subluxation, vertical subluxation (also diagnosed by applying one or more radiographic measure-
known as cranial settling and atlantoaxial impaction), lateral ments that assess the relationship of the tip of the odon-
subluxation, and posterior subluxation. Subluxations of toid process to landmarks at the base of the skull. When
all types have been noted in 40% to 85% of patients with severe, such settling is easily diagnosed on radiographs
rheumatoid arthritis. obtained in a lateral projection or on conventional tomo-
Abnormal separation between the anterior arch of the grams or computed tomography (CT) scans. Cranial
atlas and the odontoid process (dens) of the axis, desig- settling has been observed in 5% to 22% of patients with
nated anterior atlantoaxial subluxation, is a characteristic rheumatoid arthritis. In general, vertical translocation
finding in rheumatoid arthritis that may be evident at an of the dens results from disruption and collapse of the
early stage of the disease, when other cervical spine abnor- osseous and articular structures that exist between the
malities, including odontoid erosion, are not apparent. occiput and the atlas and between the atlas and the axis.
Generally, the interosseous distance between the poste- Magnetic resonance (MR) imaging (Fig. 16–29) is an
rior aspect of the anterior arch of the atlas and the ante- effective method for the further evaluation of such sub-
rior aspect of the odontoid process does not exceed luxation, as well as its effects on the spinal cord.
2.5 mm in adults. Measurement of the inferior aspect of Lateral subluxation of the atlantoaxial joints has also
this articulation is used most often. Anterior atlantoaxial been observed in patients with rheumatoid arthritis. In
subluxation occurs in 20% to 25% of patients with rheu- these patients, asymmetry is recorded between the odon-
matoid arthritis (Fig. 16–28). The pathogenesis of ante- toid process (and body of the axis) and the atlas. This
rior atlantoaxial subluxation relates to the presence of complication is diagnosed when the lateral masses of the
transverse ligament laxity caused by synovial inflamma- atlas are displaced more than 2 mm with respect to those
tion and hyperemia of the adjacent articulations. of the axis, and it is observed in 10% to 20% of patients
Vertical subluxation at C1–C2 can also be observed in with rheumatoid arthritis (Fig. 16–30). The sequence of
patients with rheumatoid arthritis and, when extensive, events that leads to lateral subluxation of the atlantoaxial
can be fatal (see Fig. 16–28). This complication has been joints includes articular space narrowing, bone erosion,
associated with neurologic symptoms and signs related to disruption of the articular capsules, and, in severe cases,
entrapment of the first and second cervical nerve roots, collapse of the lateral masses of the axis. Bone erosion
impairment of cranial nerves, bulbomedullary compres- may be the most important factor in the development of
sion, occlusion of the branches of the spinal and vertebral this deformity.
242 Section V Rheumatoid Arthritis and Related Diseases
A B
Figure 16–28. Abnormalities of the cervical spine: anterior atlantoaxial subluxation and vertical
translocation of the odontoid process (cranial settling). A and B, Lateral radiographs of the cervical spine in
a patient with rheumatoid arthritis obtained 9 years apart. In the earlier study (A), anterior atlantoaxial
subluxation (arrowheads) is evident. In the later study (B), cranial settling has occurred, with the anterior arch
of the atlas (arrow) having moved inferiorly with respect to the axis. Note that the apparent absence of
anterior atlantoaxial subluxation in (B) suggests improvement in the radiographic findings, but this is
definitely not the case.
A B
Figure 16–31. Abnormalities of the cervical spine: odontoid process erosions. A, Odontoid process
erosions occur in areas that are intimate with the synovial articulations between the anterior arch of the atlas
and the dens (1) and between the dens and the transverse ligament of the atlas (2), and in areas that are
intimate with the ligamentous attachments (3) at the tip of the odontoid process. B, Sagittal MPGR
(TR/TE, 300/9; flip angle, 15 degrees) MR image reveals prominent synovial inflammation in the posterior
median atlantoaxial joint (arrowhead), along with erosion of the odontoid process (arrow) and compression
of the spinal cord. (Courtesy of S. Moreland, MD, San Diego, Calif.)
244 Section V Rheumatoid Arthritis and Related Diseases
disease. The presence or absence of bone erosions appears replaced by MR imaging. Arthrography can delineate the
to have profound implications for treatment and prog- presence and extent of synovial hypertrophy and inflam-
nosis. Many patients have such erosions when first exam- mation, showing an irregular or corrugated appearance
ined, and in most patients who eventually develop bone of the contrast material and lymphatic filling. Rotator
erosions, they occur during the first 2 years of clinically cuff disruption, synovial cysts (Fig. 16–35), and sinus tracts
evident disease. As many as 25% of patients with rheu- are also definitively delineated during arthrographic
matoid arthritis, however, have no bone erosions even examination.
when closely monitored for 5 years or more. Whether
these latter patients would have had bone erosions with-
out early and aggressive therapy is not clear. Because of Scintigraphy
the clinical importance of the early detection of bone As in other articular disorders, radionuclide examination
erosions, most experts recommend that serial radiographs using bone- or joint-seeking pharmaceutical agents is
of classic target sites in patients with early polyarthritis a sensitive method for evaluating disease activity in
be an integral part of their treatment. Accurate radio- rheumatoid arthritis. Technetium pertechnetate and
graphic diagnosis of rheumatoid arthritis lies in analysis technetium phosphate scans outline joint inflammation
of the hands, wrists, and feet. that may antedate clinical activity, and these studies can
Because osteoporosis, joint space narrowing, and bone be used to monitor response to therapy. A typical abnor-
erosion are considered characteristic radiographic features mal radionuclide pattern in rheumatoid arthritis consists
of rheumatoid arthritis, most attempts at grading the of a symmetrically distributed peripheral joint process
severity of articular involvement emphasize one or more most prominent in the wrist, the metacarpophalangeal
of these features. In some cases, problems arise as a result and proximal interphalangeal (and occasionally distal inter-
of the independence of one radiographic feature with phalangeal) joints of the hand, the metatarsophalangeal
respect to another (e.g., progression of bone erosion with and interphalangeal joints of the foot, the knee, and the
lack of progression of joint space loss) or the independ- elbow (Fig. 16–36). Enhanced uptake has been observed
ence of one joint with respect to another (e.g., progres- in the cervical spine, atlantoaxial region, and temporo-
sion of disease in the second metacarpophalangeal joint mandibular joint.
and absence of progression in the third metacarpopha-
langeal joint), and as a result of the difficulty in defining
the changing size of an osseous erosion or the extent of
joint space loss, especially in the presence of effusion,
subluxation, or deformity.
In general, radiographically evident bone erosions show
little change in patients with significant clinical improve-
ment; in these persons, the lack of progression of such
erosions on radiographs may be a more reliable indicator
of therapeutic success. Although radiographic evidence
of healing of bone erosions, characterized by decreased
size and a sclerotic margin, has been recorded, it is infre-
quent and requires a long period of observation—usually
years. Therefore, when planning a radiologic protocol that
is appropriate in patients receiving treatment for rheuma-
toid arthritis, physicians should use longer intervals
between examinations and standardize the radiographic
technique. Further investigation is required to delineate
the ideal use of conventional radiographs as well as
other diagnostic methods, particularly scintigraphy and
MR imaging.
Figure 16–36. Scintigraphy in rheumatoid arthritis. A, Radionuclide scan of the right hand demonstrates
uniform wrist activity and greater proximal than distal joint involvement. Note the deformities of the first
(curved arrow) and fifth (arrow) digits. B, Radiograph of same hand shows typical deformities. Areas of
greatest radionuclide activity correlate well with radiographic changes.
(From Weissberg D, Resnick D, Taylor A, et al: Rheumatoid arthritis and its variants: Analysis of scinti-
photographic, radiographic, and clinical examinations. AJR Am J Roentgenol 131:665, 1978.)
Computed Tomography
of the flexor tendons of the hand detectable with ultra-
CT is generally not required when assessing the distri- sonography include loss of normal texture, tenosynovial
bution and extent of joint involvement in this disease. cysts, tenosynovitis, and tendon (i.e., rheumatoid) nodules.
Acetabular protrusion and ischemic necrosis of the
femoral head are two examples of problems seen in
patients with rheumatoid arthritis that can be evaluated Magnetic Resonance Imaging
with CT. It is clear, however, that ischemic necrosis of General applications of MR imaging in the evaluation of
the femoral head cannot be diagnosed at an earlier stage rheumatoid arthritis include detection of articular
with CT than with MR imaging. CT is well suited to the disease, assessment of such disease activity, determination
evaluation of para-articular masses, which in rheumatoid of the nature of some complications, and analysis of the
arthritis are commonly related to synovial cyst formation. extent of articular and para-articular changes in specific
CT has been advocated as a useful noninvasive locations.
method in the diagnosis and management of cervical MR imaging is a very sensitive imaging method for
spine abnormalities in rheumatoid arthritis patients. The the detection of articular diseases and has been used to
craniocervical junction and atlantoaxial region are well assess the activity of rheumatoid arthritis, as well as its
displayed in the transaxial and reformatted images obtain- response to a variety of therapeutic regimens. It should
able with CT. be noted, however, that differentiating synovial fluid and
inflammatory synovial tissue, or pannus, in a rheumatoid
joint on standard spin echo and gradient echo sequences
Ultrasonography
may be difficult (but possible) and that modification of
With regard to rheumatoid arthritis, ultrasonography has the MR imaging technique may be required. Imaging
proved capable in the analysis of para-articular masses following intravenous administration of gadolinium-
such as synovial cysts and tendon sheath and joint involve- containing contrast agents is useful in differentiating syn-
ment (especially in the hands and wrists). Abnormalities ovial inflammatory tissue and fluid.
248 Section V Rheumatoid Arthritis and Related Diseases
On routine spin echo studies without such injection, rupture, which may affect the hand, foot, knee, and
low-intensity signal is derived from both fluid and pannus shoulder in patients with rheumatoid arthritis, are best
on T1-weighted images, whereas high-intensity signal assessed with MR imaging.
characterizes both fluid and pannus on T2-weighted Insufficiency fractures, an important complication of
images. Immediately after the intravenous injection of rheumatoid arthritis that occurs in the sacrum, femoral
a gadolinium-containing agent, the effusion remains neck, parasymphyseal bone, tubular bones of the lower
of low signal intensity on T1-weighted spin echo images, extremity, and elsewhere, reveal characteristic signal
and the synovium demonstrates enhancement with intensity abnormalities with MR imaging. With standard
increased signal intensity on these images (Fig. 16–37). spin echo techniques, such fractures demonstrate serpen-
Synovial enhancement is greater in regions of hyper- tine linear shadows of low signal intensity surrounded by
vascular proliferative pannus and less in areas of fibrotic larger regions of intermediate signal intensity on T1-
synovial tissue. Delayed imaging after intravenous weighted images. The areas of intermediate signal, pre-
injection of the contrast medium is characterized by sumably representing surrounding edematous foci, become
seepage of contrast agent across the inflammatory brighter on T2-weighted images (Fig. 16–39).
pannus, with an increase in the signal intensity of joint Evaluation of rheumatoid spines with MR imaging
fluid on T1-weighted spin echo images. The enhance- deserves special emphasis (Fig. 16–40). Although this
ment of joint fluid occurs within minutes, reaches a technique can be used to assess any spinal segment, most
plateau in about 30 minutes, and persists for at least reports have emphasized changes in the cervical region,
60 minutes. especially the craniocervical junction. MR imaging allows
A variety of musculoskeletal complications of rheu- assessment of the relationship of the occiput, atlas, and
matoid arthritis can be well evaluated with MR imaging. axis and is therefore useful in delineating the extent of
These include ischemic necrosis of bone, bursitis, syn- subluxation in this spinal segment. MR imaging allows
ovial cyst formation, rheumatoid nodules, carpal tunnel direct visualization of the spinal cord so that sites of
syndrome and other entrapment neuropathies, tendon physical distortion of the cord by displaced vertebrae,
injury and disruption, and insufficiency fractures. Fluid inflammatory masses, or both can be detected. The severity
collections and abnormal synovium within the iliopsoas, of myelopathy correlates better with MR imaging
subdeltoid-subacromial, retrocalcaneal, olecranon, and findings than with findings on conventional radiography.
prepatellar bursae are easily demonstrated with this tech- Medullary compression by the tip of the odontoid pro-
nique (Fig. 16–38). Similarly, synovial cysts around any cess in instances of cranial settling is far better appre-
involved joint can be delineated with MR imaging. In ciated on MR images than on standard radiographs. In
superficial articulations such as the knee, ultrasonography instances of cord compression, areas of abnormal signal
is equally effective in the assessment of synovial cysts, intensity within the spinal cord itself, perhaps reflecting
although in deeper locations, including the hip and spine, edema, have been detected in rheumatoid arthritis patients
MR imaging has distinct advantages. Tendon injury and by MR imaging.
A B
Figure 16–37. MR imaging in rheumatoid arthritis: differentiation of effusion and pannus with an
intravenous gadolinium contrast agent. A, Initial intermediate-weighted (TR/TE, 1200/30) parasagittal spin
echo MR image before the intravenous injection of a gadolinium compound reveals relatively low signal
intensity of fluid, pannus, or both in the knee joint and within a synovial cyst (open arrows). B, Immediately
after intravenous injection of the gadolinium agent, an intermediate-weighted (TR/TE, 1600/30)
parasagittal spin echo MR image obtained with fat suppression reveals enhancement of the inflamed synovial
membrane in the joint and bursa (open arrows). The fluid (arrowhead) remains of low signal intensity.
Chapter 16 Rheumatoid Arthritis 249
DIFFERENTIAL DIAGNOSIS and marginal and central erosions affecting the proximal
interphalangeal and metacarpophalangeal joints of the
General Abnormalities hands, the wrists, the metatarsophalangeal joints of the
The diagnosis of rheumatoid arthritis is obvious when feet, the knees, and the elbows. When radiographs disclose
radiographic examination reveals a symmetrically distrib- some degree of asymmetry, mild or absent osteoporosis,
uted articular disorder characterized by osteoporosis, or preservation of joint space, an accurate appraisal is
fusiform soft tissue swelling, concentric joint space loss, much more difficult.
A B
Seronegative “Rheumatoid” Arthritis. Many patients with spondylitis, psoriatic arthritis, and Reiter’s syndrome can
polyarticular inflammatory disease are serologically nega- produce significant and extensive abnormalities of the
tive (seronegative) for rheumatoid factor, and the precise cartilaginous joints and entheses; these sites are much less
nature of their joint abnormality is variable and often frequently and severely affected in rheumatoid arthritis.
unclear. Patients who have seronegative “rheumatoid” Widespread spondylitis and sacroiliitis are also charac-
arthritis can generally be divided into two major groups: teristic of the seronegative spondyloarthropathies.
those with otherwise typical rheumatoid arthritis who are
serologically negative for rheumatoid factor, and those Gout. Gouty arthritis is associated with a form of asym-
with some other form of arthritis. In the former group, metrical articular disease of the appendicular skeleton that
some patients will become seropositive, and some have is characterized by soft tissue masses, asymmetric osseous
become seronegative as a result of therapy. In the latter erosions, bony proliferation, preservation of joint space,
group, certain patients share clinical and radiologic fea- and absence of osteoporosis. Generally, the features are
tures that will eventually allow their placement into a easily differentiated from those of rheumatoid arthritis.
more homogeneous disease category. With respect to
radiologic abnormalities, many persons with seronega- Collagen Vascular Disorders. The arthropathy of systemic
tive “rheumatoid” arthritis have atypical features, and in lupus erythematosus is a deforming, nonerosive process
fact, an observer can often predict such seronegativity that is initially reversible. Cartilaginous destruction caused
when reviewing the radiographs. One such feature is the by pressure erosion at areas of contact between malposi-
asymmetry of articular involvement. tioned osseous structures, as well as periarticular bony
The presence of one or more radiographic features “hook” erosions caused by capsular and ligamentous
that are otherwise atypical for rheumatoid arthritis should changes, can be observed in systemic lupus erythematosus,
alert the observer that the patient is probably seronega- but marginal and central osseous defects and diffuse joint
tive for rheumatoid factor. The greater the number of space narrowing are unusual in this disease unless coexis-
such features, the greater the likelihood of seronega- tent rheumatoid arthritis is present. In contrast, the
tivity. The disease in such patients is best regarded as deformities in rheumatoid arthritis are almost universally
“rheumatoid-like” arthritis, an indication that the radio- associated with joint space narrowing and bone erosion.
graphic abnormalities are explained by a pathologic pro- In scleroderma, articular changes may be especially
cess resembling that of true rheumatoid arthritis. prominent in the distal interphalangeal joints of the fingers
and the first carpometacarpal and inferior radioulnar
Remitting Seronegative Symmetrical Synovitis with Pitting compartments of the wrist. They are generally associated
Edema (RS3PE Syndrome). Patients with RS3PE syndrome with tuft resorption and soft tissue and capsular calci-
are generally elderly with an acute onset of bilateral and fication. Mixed connective tissue disease and overlap syn-
symmetrical synovitis involving predominantly the hands dromes can lead to radiographic abnormalities common
and wrists, along with evidence of tenosynovitis and pit- to more than one collagen vascular disorder. Thus, detec-
ting edema of the dorsum of the hands. Patients are typi- tion of rheumatoid arthritis–like alterations as well as
cally seronegative for rheumatoid factor, and most possess those of scleroderma or systemic lupus erythematosus can
the HLA-B27 antigen. Other clinical features include the provide a clue to the mixed nature of the collagen disease.
involvement of large joints and flexion contractures of
the wrists, fingers, and elbows. Although the remitting Calcium Pyrophosphate Dihydrate Crystal Deposition
course and absence of joint destruction are clearly dif- Disease. This articular disorder is radiographically mani-
ferent from the characteristics of rheumatoid arthritis, fested as articular and periarticular calcification and
many similarities exist between RS3PE syndrome and pyrophosphate arthropathy. The calcification involves
polymyalgia rheumatica. Clearly, the final word regard- various structures, including cartilage (chondrocalcinosis),
ing RS3PE syndrome has yet to be written. and is most prominent in the knees, wrists, metacar-
pophalangeal joints, and symphysis pubis. Pyrophosphate
Spondyloarthropathies. Rheumatoid arthritis character- arthropathy leads to joint space narrowing, eburnation,
istically has articular lesions that differ in distribution and cyst formation, collapse, and fragmentation. The result-
morphology from those of the seronegative spondylo- ing radiographic picture does not resemble that of rheu-
arthropathies. Ankylosing spondylitis (which shows a matoid arthritis. The arthropathy of hemochromatosis is
predilection for the axial skeleton, although it may also very similar to that of idiopathic CPPD crystal deposi-
produce appendicular articular disease), psoriatic arthritis tion disease.
(which may affect the axial and appendicular skeleton
as well as the distal interphalangeal articulations), and
Reiter’s syndrome (which leads to asymmetrical arthritis Abnormalities at Specific Sites
of the lower extremity, with or without sacroiliac and Hand and Wrist. Symmetrical changes at the metacarpo-
spinal alterations) may each be associated with prominent phalangeal and proximal interphalangeal joints are com-
findings in synovial and cartilaginous joints and entheses. mon in rheumatoid arthritis (Table 16–4). Psoriatic arthritis
In the synovial joints, the absence of osteoporosis and the leads to significant abnormalities of the distal interpha-
presence of bony proliferation and intra-articular osseous langeal joints, as well as the more proximal articulations.
fusion are commonly encountered in the seronegative Osteoarthritis (inflammatory or noninflammatory) most
spondyloarthropathies, findings that differ from the typically affects the distal interphalangeal, proximal inter-
features of rheumatoid arthritis. Similarly, ankylosing phalangeal, and metacarpophalangeal joints. Gouty arthritis
Chapter 16 Rheumatoid Arthritis 251
TABLE 16-4
Compartmental Analysis of Hand and Wrist Disease*
Common First
Inferior Pisiform- Carpo- Carpo-
DIP PIP MCP Radiocarpal Radioulnar Midcarpal Triquetral metacarpal metacarpal
Joints Joints Joints Joint Joint Joint Joint Joint Joint
Rheumatoid arthritis + + + + + + + +
Osteoarthritis + + + +† +
Inflammatory + + ± +† +
osteoarthritis
Calcium pyrophosphate + + +† +
dihydrate crystal
deposition disease
Gouty arthritis + + + + + + +‡ +
Scleroderma§ + + + +
can involve any joint of the hand, including the distal Acromioclavicular Joint. Resorption of the distal end of
interphalangeal joints. CPPD crystal deposition disease the clavicle and widening of the acromioclavicular joint
has a predilection for the metacarpophalangeal articu- are observed in rheumatoid arthritis, ankylosing spondyli-
lations. tis, infection, other collagen vascular disorders, and hyper-
Rheumatoid arthritis can manifest initially as soft tissue parathyroidism, as well as after trauma. When the changes
swelling, joint space narrowing, and osseous erosions in are secondary to an articular process, prominent acromial
one or two locations of the wrist. Soon, however, pancom- abnormalities may accompany the clavicular alterations.
partmental alterations become evident. In patients with- In hyperparathyroidism, subchondral resorption of bone
out a significant history of accidental or occupational is evident on both the acromial and the clavicular aspects
trauma, osteoarthritis leads to articular abnormalities that of the articulation, but the acromial alterations are rela-
are invariably confined to the first carpometacarpal com- tively mild. Post-traumatic osteolysis of the distal end of
partment, the trapezioscaphoid space of the midcarpal the clavicle is accompanied by a pertinent clinical history
compartment, or both. CPPD crystal deposition disease of acute or chronic injury.
favors the radiocarpal compartment; scleroderma may
selectively involve the first carpometacarpal and inferior Coracoclavicular Joint. Resorption of the undersurface of
radioulnar compartments; and gout produces pancom- the distal portion of the clavicle is seen in rheumatoid
partmental disease, with predominant involvement of the arthritis, hyperparathyroidism, and ankylosing spondylitis.
common carpometacarpal compartment. In ankylosing spondylitis, bone proliferation is an asso-
ciated radiographic manifestation.
Elbow. Radiographic abnormalities of the elbow in rheu-
matoid arthritis are sufficiently characteristic in most Sternoclavicular and Manubriosternal Joints. Changes in
instances to allow an accurate diagnosis. Joint space the sternoclavicular and manubriosternal joints are not
narrowing, osseous erosions, and cysts are frequently specific for rheumatoid arthritis. Similar abnormalities
accompanied by soft tissue prominence related to rheu- accompany the seronegative spondyloarthropathies.
matoid nodules and olecranon bursitis. The elbow is
involved in other polyarticular disorders, but differences Forefoot. The most common sites of articular disease of
in radiographic findings between these diseases and rheu- the forefoot in rheumatoid arthritis are the metatarso-
matoid arthritis are usually obvious. phalangeal joints and the interphalangeal joint of the
great toe. Although these same articulations are involved
Glenohumeral Joint. Marginal erosions of the humeral in psoriatic arthritis, Reiter’s syndrome, and gout, exten-
head are seen in rheumatoid arthritis, ankylosing spondyli- sive abnormalities of other interphalangeal joints in
tis, and infection, as well as in other disorders. In anky- one or more digits should be evident in these disorders
losing spondylitis, the size of the defect may be (Table 16–5). Further, forefoot abnormalities are usually
considerably larger than that in rheumatoid arthritis. symmetrical in distribution in rheumatoid arthritis and
Septic arthritis of the glenohumeral joint is usually asymmetrical in psoriatic arthritis, Reiter’s syndrome,
monoarticular. and gouty arthritis.
252 Section V Rheumatoid Arthritis and Related Diseases
Midfoot. Diffuse changes with a predilection for the Knee. In rheumatoid arthritis, symmetrical involvement
talonavicular portion of the talocalcaneonavicular joint of the medial and lateral femorotibial compartments is
characterize midfoot involvement in rheumatoid arthritis. seen, with or without patellofemoral compartment changes.
The talonavicular space is also affected in CPPD crystal In osteoarthritis, asymmetrical alterations of the medial
deposition disease and neuropathic osteoarthropathy, but and lateral femorotibial compartments (the medial side is
both of these disorders are associated with bony sclerosis usually the dominant side of involvement, especially in
and fragmentation. In gout, abnormalities often predom- men) can be combined with patellofemoral compartment
inate at the tarsometatarsal joints. disease. In CPPD crystal deposition disease, patellofemoral
abnormalities, occurring alone or in combination with
Heel. Retrocalcaneal bursitis producing soft tissue swelling asymmetrical medial and lateral femorotibial alterations,
and subjacent osseous erosion occurs not only in rheu- are typical. A varus deformity is especially characteristic
matoid arthritis but also in ankylosing spondylitis, pso- of osteoarthritis, whereas valgus deformity is not uncom-
riatic arthritis, and Reiter’s syndrome (Table 16–6). The mon in rheumatoid arthritis and CPPD crystal deposi-
changes are virtually indistinguishable in these four dis- tion disease. Marginal erosions of the femur and tibia are
orders, and tendon thickening can be seen in any one of evident in rheumatoid arthritis, the seronegative spondy-
them. Nodular prominence of the Achilles tendon can loarthropathies, gout, and infection, especially tubercu-
also be encountered in gout (as a result of tophi) and losis. In gout and tuberculosis, such erosions may be
hyperlipoproteinemia (secondary to xanthoma). unaccompanied by joint space narrowing.
TABLE 16-6
Abnormalities of the Heel
Retrocalcaneal
Bursitis with Enthesophyte at
Posterosuperior Achilles Posterior Attachment Well-Defined Plantar Poorly Defined Plantar
Calcaneal Erosion Tendinosis of Achilles Tendon Calcaneal Enthesophyte Calcaneal Enthesophyte
Rheumatoid arthritis + + + +
Ankylosing spondylitis + + + +*
Psoriatic arthritis + + + +*
Reiter’s syndrome + + +*
Gouty arthritis +† +‡
Xanthoma +† +‡
Park WM, O’Neill M, McCall IW: The radiology of rheuma- Sharp JT, Young DY, Bluhm GR, et al: How many joints in the
toid involvement of the cervical spine. Skeletal Radiol 4:1, hands and wrists should be included in a score of radiologic
1979. abnormalities used to assess rheumatoid arthritis? Arthritis
Resnick D: Patterns of migration of the femoral head in osteo- Rheum 28:1326, 1985.
arthritis of the hip: Roentgenographic-pathologic correla- Stiskal MA, Neuhold A, Szolar DH, et al: Rheumatoid arthritis
tion and comparison with rheumatoid arthritis. AJR Am J of the craniocervical region by MR imaging: Detection and
Roentgenol 124:62, 1975. characterization. AJR Am J Roentgenol 165:585, 1995.
Resnick D: Rheumatoid arthritis of the wrist: The compart- Sugimoto H, Takeda A, Hyodoh K: Early-stage rheumatoid
mental approach. Med Radiogr Photogr 52:50, 1976. arthritis: Prospective study of the effectiveness of MR imag-
Resnick D, Feingold ML, Curd J, et al: Calcaneal abnormalities ing for diagnosis. Radiology 216:569, 2000.
in articular disorders: Rheumatoid arthritis, ankylosing Swanson AB, Swanson GG: Pathogenesis and pathomechanics
spondylitis, psoriatic arthritis and Reiter’s syndrome. of rheumatoid deformities in the hand and wrist. Orthop
Radiology 125:355, 1977. Clin North Am 4:1039, 1973.
Ropes MW, Bennett GA, Cobb S, et al: Diagnostic criteria for Weissberg D, Resnick D, Taylor A, et al: Rheumatoid arthritis
rheumatoid arthritis, 1958 revision. Ann Rheum Dis 18:49, and its variants: Analysis of scintiphotographic, radiographic,
1959. and clinical examinations. AJR Am J Roentgenol 131:665,
Schils JP, Resnick D, Haghighi PN, et al: Pathogenesis of 1978.
discovertebral and manubriosternal joint abnormalities in Weissman BNW, Alibadi P, Weinfeld MS, et al: Prognostic
rheumatoid arthritis: A cadaveric study. J Rheumatol 16:291, features of atlanto-axial subluxation in rheumatoid arthritis.
1989. Radiology 144:745, 1982.
Scott JT (ed): Copeman’s Textbook of the Rheumatic Diseases, Wolfe BK, O’Keeffe D, Mitchell DM, et al: Rheumatoid arthritis
5th ed. Edinburgh, Churchill Livingstone, 1978. of the cervical spine: Early and progressive radiographic
Sharp JT: An overview of radiographic analysis of joint damage features. Radiology 165:145, 1987.
in rheumatoid arthritis and its use in metaanalysis. J
Rheumatol 27:254, 2000.
17
CHAPTER 17
Juvenile Chronic Arthritis arthritides (Tables 17–1 and 17–2). At the time of the initial
examination, however, it is often impossible to classify
childhood arthritis into one specific subgroup of juvenile
chronic arthritis.
SUMMARY OF KEY FEATURES
Juvenile chronic arthritis is a disorder encompassing a Juvenile-Onset Adult-Type (Seropositive)
variety of conditions that affect articular structures in Rheumatoid Arthritis
children. Although the specific radiographic features of
An articular disease that resembles and behaves like its
juvenile chronic arthritis depend on the subgroup of
adult counterpart has been noted in 10% of children with
patients being evaluated, certain characteristics are
juvenile chronic arthritis (Fig. 17–1A). This subtype is
sufficiently common in most patients to allow differen-
more common in girls than in boys and is more common
tiation of juvenile chronic arthritis from various adult
after the age of 10 years. The clinical onset is usually
diseases. Loss of articular space and osseous erosions are
polyarticular (involvement of five or more joints), with
relatively late manifestations of juvenile disease. Metaphyseal
early involvement of the interphalangeal and metacar-
radiolucency, periostitis, intra-articular bony ankylosis,
pophalangeal joints of the hand, the wrist, the knee, and
epiphyseal compression fractures, subluxation or
the metatarsophalangeal and interphalangeal joints of the
dislocation, and growth disturbances are common.
foot. Subcutaneous nodules, particularly about the elbow,
Although changes may be observed in many different
can be detected in approximately 10% to 20% of children.
skeletal sites, abnormalities of the hand, wrist, foot, knee,
Iridocyclitis is not present.
hip, cervical spine, mandible, and temporomandibular
Radiologic changes in juvenile-onset adult-type
joint are especially characteristic.
rheumatoid arthritis include soft tissue swelling, periar-
The differential diagnosis of juvenile chronic arthritis
ticular osteoporosis, and periostitis in the hands and feet.
includes hemophilia, idiopathic multicentric osteolysis,
Periosteal bone formation can involve large segments of
mucopolysaccharidoses, epiphyseal dysplasias, multicentric
the metaphyses of the phalanges, metacarpals, and
reticulohistiocytosis, and infection.
metatarsals. The frequency and severity of periostitis
account for the fundamental difference between juvenile-
onset and adult-onset disease. Significant osseous erosions
are also encountered in children with this subtype of disease,
INTRODUCTION unaccompanied by loss of the interosseous space. The
appearance of significant erosive abnormality in the absence
In 1897, Still, an English pediatrician, described in detail of joint space loss is an important diagnostic sign of juvenile-
an articular condition in 22 children that appeared to be onset rheumatoid arthritis. The prognosis of polyarthritis
distinct from the adult type of rheumatoid arthritis because in children who become seropositive for rheumatoid factor
of its predilection for large joints rather than small ones, is generally poor.
its propensity for producing joint contractures and muscle
wasting, and its association with significant extra-articular
manifestations such as splenomegaly, lymphadenopathy, Seronegative Chronic Arthritis (Still’s Disease)
anemia, fever, pleuritis, and pericarditis. Since that report, In this most common (approximately 70%) subtype of
the term Still’s disease has frequently been used to describe juvenile chronic arthritis, systemic or articular (or both)
rheumatoid arthritis in children. It is now recognized
that a number of separate disorders can lead to chronic
arthritis in children and that in many patients, scrutiny of
TABLE 17–1
the clinical and radiographic features allows a more
precise diagnosis. Juvenile Chronic Arthritis: Classification
TABLE 17–2
Juvenile Chronic Arthritis: Clinical and Radiographic Features
symptoms and signs develop in the absence of positive accompanied by arthritis. Affected children have toxic
serologic test results for rheumatoid factor. Within this symptoms along with irritability, listlessness, anorexia, and
subgroup are certain clinical varieties, such as classic weight loss. A rash accompanies the fever in approximately
systemic disease, polyarticular disease, and pauciarticular 80% to 90% of children. Generalized lymphadenopathy
or monoarticular disease. and hepatosplenomegaly can simulate findings in leukemia
or lymphoma. Pericarditis and myocarditis represent serious
Classic Systemic Disease. This pattern, which is usually manifestations of Still’s disease. Although joint manifes-
seen in boys and girls younger than 5 years and repre- tations are common, they are generally mild. Myalgia and
sents approximately 20% of cases of juvenile chronic arthralgia may precede actual arthritis. Radiologic findings
arthritis, is associated with severe extra-articular clinical are unusual, although chronic and disabling articular
manifestations. An acute febrile onset may or may not be changes occasionally become evident.
Chapter 17 Juvenile Chronic Arthritis 257
A B
Figure 17–1. Juvenile chronic arthritis: subtypes of disease. A, Juvenile-onset adult-type (seropositive)
rheumatoid arthritis in an 18-year-old girl with seropositive rheumatoid arthritis for approximately 6 years.
Observe that the radiographic abnormalities are similar to those seen in adult-onset disease. Involvement
occurs in all the compartments of the wrist and in the metacarpophalangeal and proximal interphalangeal
joints, with less striking changes in the distal interphalangeal joints. Radiographic changes include soft tissue
swelling, periarticular osteoporosis, joint space narrowing, and marginal erosions. At some metacarpopha-
langeal joints, considerable erosive alterations are not accompanied by severe loss of joint space (arrows).
Also note the intra-articular osseous fusion at several proximal interphalangeal joints and periostitis of the
phalangeal shafts (arrowheads). B, Seronegative chronic arthritis (Still’s disease): polyarticular disease. An 11-
year-old girl, seronegative for rheumatoid factor, had symmetrical articular disease of the hands, wrists,
knees, feet, and cervical spine. The radiograph outlines considerable generalized osteoporosis, periarticular
soft tissue swelling, superficial erosions of carpal bones and metacarpal heads with shape irregularities, joint
space narrowing, epiphyseal collapse, and enlargement of the epiphyses, particularly those of the distal end
of the radius and distal portion of the ulna (arrows). The crenated or “crinkled” appearance of the carpus and
metacarpal heads is distinctive. Flexion contractures of several digits are evident.
Polyarticular Disease. Polyarticular arthritis (see Fig. 17–1B) sequent intra-articular bony ankylosis. Synovitis of the flexor
may occur at the onset of Still’s disease or as a later com- tendon sheaths may produce periostitis of the diaphyses and
plication in a child with systemic manifestations. This metaphyses of the phalanges, metacarpals, and metatarsals.
pattern is evident in approximately 20% of patients with Premature fusion of the epiphyses of the bones is not
juvenile chronic arthritis. Boys and girls are affected in uncommon and accounts for the characteristic growth
equal numbers. Symmetrical or asymmetrical involvement defects. The epiphyses may appear enlarged or ballooned
of the metacarpophalangeal and proximal interphalangeal in relation to the diaphyses, and a decrease in bone length
joints of the hands, the wrists, the knees, the ankles, and is characteristic. Osseous erosion is unusual.
the intertarsal, metatarsophalangeal, and interphalangeal In larger articulations such as the knee, osteoporosis and
joints of the feet is typical. The cervical spine is fre- epiphyseal overgrowth are more typical. In the hip, enlarge-
quently a site of early abnormality and is characteristi- ment and osteoporosis of the femoral capital epiphysis,
cally the only region of the vertebral column affected. failure of growth and premature fusion of the femoral neck,
In the initial stages of polyarthritis, radiographic findings coxa valga deformity, hypoplasia of the iliac bones, and
are soft tissue swelling, osteoporosis, and advanced skeletal protrusio acetabuli are seen. Apophyseal joint erosions,
maturation. In the hands and feet, abnormalities in the narrowing, and bony ankylosis predominate in the upper
shape (squaring) of the carpal and tarsal bones are fre- cervical region. Associated hypoplasia of the vertebral
quently combined with initial loss of joint space and sub- bodies and intervertebral discs is characteristic.
258 Section V Rheumatoid Arthritis and Related Diseases
A B
Figure 17–2. Juvenile chronic arthritis: general radiographic abnormalities. A, Periostitis. Prominent
periosteal proliferation of numerous phalanges (arrows) is associated with osteoporosis and periarticular soft
tissue swelling. Joint space diminution and erosions are not significant features in this child. B, Epiphyseal
compression fractures. The irregular outline of the metacarpal heads (arrows) is produced by compression
of weakened osteoporotic bones.
260 Section V Rheumatoid Arthritis and Related Diseases
(resulting from muscle spasm and joint subluxation) acting appear, including boutonnière deformity (characterized by
on weakened osteoporotic bone. Flattening and deformity flexion at the proximal interphalangeal joint and hyper-
of the epiphyseal ossification centers and formation of dense extension at the distal interphalangeal joint), flexion defor-
intraosseous foci as a result of trabecular compression are mity (characterized by flexion at both the proximal
evident. interphalangeal and distal interphalangeal joints), and,
less frequently, swan-neck deformity (characterized by
Joint Subluxation. Subluxation and dislocation can be hyperextension at the proximal interphalangeal joint and
observed in any articulation but are most common in the flexion at the distal interphalangeal joint).
hip. These complications result from large intra-articular
effusions or, more importantly, from ligamentous destruc- Wrist. Abnormalities of the wrist are extremely common
tion and muscle foreshortening secondary to fibrosis. (Fig. 17–4). Soft tissue prominence, osteoporosis, and
irregular carpal ossification centers are seen. Intra-articular
Soft Tissue Calcification. Periarticular soft tissue calcific osseous ankylosis of the compartments of the wrist may
deposits may be recorded in this disease. They appear to be prominent. Growth disturbances and articular destruc-
be located in the joint capsule, ligament, or muscle. The tion can lead to significant wrist deformities. The ulna
exact cause of periarticular calcification in juvenile chronic may be relatively short in comparison to the radius and
arthritis is not known, although an association with intra- produce ulnar deviation at the wrist.
articular corticosteroid therapy or other forms of intra-
articular treatment has been reported. Knee. Radiographic abnormalities of the knee in juvenile
chronic arthritis include osteoporosis, soft tissue swelling,
enlargement with ballooning of the distal femoral and
Abnormalities in Specific Locations proximal tibial epiphyses, flattening of the femoral condyles,
A comparison of the radiographic abnormalities of chronic widening of the intercondylar notch, joint space narrow-
juvenile arthritis and those of similar diseases is presented ing, and marginal or central osseous erosions (Fig. 17–5).
in Table 17–4. These findings are virtually identical to those in hemophilia.
Alterations in patellar shape consist of flattening of the
Hand. Any articulation of the hand may be affected inferior pole of the patella, which results in squaring of
(Fig. 17–3). Asymmetrical abnormalities of both hands the bone.
are most typical. Swelling and regional osteoporosis can
develop about the distal interphalangeal, proximal inter- Hip. Involvement of the hip is common and is radiographi-
phalangeal, and metacarpophalangeal joints. Periostitis of cally evident in approximately 35% to 45% of patients
the metacarpal and phalangeal shafts, preservation of the (Fig. 17–6). In younger patients, impairment of iliac bone
joint space, and absence of significant erosions are most development and coxa valga deformity are not uncommon.
common. Epiphyseal collapse and deformity are also charac- The femoral capital epiphysis may be enlarged and irregular
teristic. A variety of finger deformities may eventually in outline, and premature fusion of the growth plate may
TABLE 17–4
Comparison of Radiographic Abnormalities
A B
A B
Figure 17–4. Abnormalities of the wrist. A, In this young child, erosions of multiple carpal bones have led
to crenated osseous contours. Joint space narrowing and acceleration of bone maturation are seen. B, In this
patient, bony ankylosis of the radiocarpal and midcarpal compartments is prominent (arrows). Joint space
narrowing and partial ankylosis at the common carpometacarpal joint are also apparent (arrowheads).
be evident. Articular space narrowing and osseous sophalangeal and interphalangeal joint alterations consist
erosion may also develop as a joint deteriorates, and of osteoporosis, epiphyseal enlargement, brachydactyly,
osteophytosis may appear. Protrusio acetabuli is more and periostitis (Fig. 17–7). Late deformities include clawing
frequent in older children. of the toes, hammer toes, hindfoot varus or valgus, pes
cavus, and hallux valgus.
Foot and Ankle. The radiographic abnormalities of the
tarsus are similar to those in the carpus. Enlargement and Other Articulations of the Appendicular Skeleton. Alter-
irregularity of the tarsal bones, joint space narrowing, ations of the humeral head parallel those of the femoral
and intra-articular bony ankylosis can be seen. Metatar- head, including osteoporosis, bone enlargement, joint
262 Section V Rheumatoid Arthritis and Related Diseases
A B
Figure 17–8. Abnormalities of the cervical spine: apophyseal joint bony ankylosis. A and B, In two
children with Still’s disease, apophyseal joint ankylosis is evident. The process is usually first apparent in the
upper cervical region and progresses to the lower vertebrae. Hypoplasia of the vertebral bodies and
intervertebral discs is prominent in both cases. Atlantoaxial subluxation is also evident in (A).
spine. Growth disturbances consist of decreased vertical 20% of patients, and frequently occurs in association with
and anteroposterior diameters of the vertebral bodies at temporomandibular joint abnormalities. Radiographic
the levels of apophyseal joint ankylosis. The adjacent abnormalities include shortening of the body and vertical
intervertebral discs are also diminished in height (or rami of the mandible, with widening of the mandibular
completely obliterated) and may contain calcification. notches. Both mandibular condyles are frequently flattened
The resulting radiographic appearance in juvenile and poorly differentiated, although the temporomandibular
chronic arthritis is distinctive, with dwarflike alterations joints themselves may appear normal. Articular space
of both the vertebral bodies and the intervertebral discs narrowing, bone erosion, and abnormal joint motion may
and apophyseal joint ankylosis. Although apophyseal joint be encountered in some persons.
ankylosis can be seen in ankylosing spondylitis, the ver- Antegonial notching of the mandible has been empha-
tebral bodies are not significantly diminished in size in sized as an additional radiographic manifestation of juve-
that disease, nor are the intervertebral discs diminutive, nile chronic arthritis. This notching represents a concavity
because disease onset generally occurs at a more advanced on the undersurface of the mandibular body just anterior
age. Further, in ankylosing spondylitis, syndesmophytes to the angular process (gonion) (Fig. 17–9).
are characteristic. Congenital fusion of vertebral bodies
(Klippel-Feil deformity) may simulate the changes in
juvenile chronic arthritis, although in the former disorder, OTHER DIAGNOSTIC TECHNIQUES
the spinous processes of several vertebrae may also be
incorporated into a single ossific mass. Fibrodysplasia Scintigraphy
ossificans progressiva can be accompanied by vertebral The major application of bone scintigraphy in persons
alterations, but additional abnormalities, such as soft with juvenile chronic arthritis relates to the determina-
tissue ossification, ensure an accurate diagnosis of that tion of disease distribution. This method allows simulta-
condition. neous assessment of all major articulations of the body,
and monoarticular, oligoarticular, or polyarticular patterns
Thoracic and Lumbar Spine. The thoracolumbar spine is of disease can be identified.
not commonly affected (see Table 17–4). Compression
fractures of vertebral bodies may be observed, particu-
Computed Tomography
larly in patients who have received steroid medication.
Scoliosis, which may appear rapidly, has also been noted. Computed tomography scanning is best applied in regions
with complex anatomy, such as the hip, shoulder, spine,
Mandible, Temporomandibular Joint, and Other Facial and temporomandibular joint. The value of computed
Structures. Underdevelopment of the jaw (micrognathia) tomography in the evaluation of children with juvenile
is not uncommon, occurring in approximately 10% to chronic arthritis is limited.
264 Section V Rheumatoid Arthritis and Related Diseases
Ultrasonography
Ultrasonography has been used to detect joint effusions
in children with juvenile chronic arthritis. This modality
is particularly helpful in the assessment of periarticular
swelling in this disease, which may relate to bursitis,
tenosynovitis, or synovial cysts.
B C
Chapter 17 Juvenile Chronic Arthritis 265
Additional applications of MR imaging in children DIFFERENTIAL DIAGNOSIS
with juvenile chronic arthritis include analysis of the
Hemophilia
temporomandibular and sacroiliac joints and assessment
of such complications as ischemic necrosis of bone and Differentiating juvenile chronic arthritis and hemophilia
growth disturbances. The technique is also useful in the can be difficult. Soft tissue swelling, osteoporosis, subchon-
evaluation of the spine. dral osseous irregularity, interosseous space diminution,
and growth disturbances may be evident in both disorders.
In the knee, ankle, and elbow, the resulting radiographic
PATHOLOGIC ABNORMALITIES picture may be identical in the two conditions. Polyarticular
Synovial inflammation in juvenile chronic arthritis resem- disease and significant involvement of the small joints of
bles that in adult-onset rheumatoid arthritis. Macro- the hand and wrist are less frequent in hemophilia than in
scopically evident villous hypertrophy of the synovial juvenile chronic arthritis, whereas radiodense joint effu-
membrane may be associated with varying degrees of sions and multiple subchondral cystic lesions are some-
fibrin accumulation in the lining and subintimal layers, what more common in hemophilic arthropathy. Squaring
cellular hyperplasia, and an inflammatory cellular response. or flattening of the inferior pole of the patella may be
Subcutaneous nodules are identified in 10% to 20% of more characteristic of juvenile chronic arthritis.
patients with juvenile chronic arthritis.
Idiopathic Multicentric Osteolysis
SPECIAL TYPE OF JUVENILE CHRONIC Idiopathic multicentric osteolysis is a disorder characterized
ARTHRITIS: ADULT-ONSET STILL’S DISEASE by multifocal articular destruction beginning in infancy
Some adults may develop a disorder that is indistinguish- or childhood (see Chapter 83). Although the clinical and
able from Still’s disease, designated adult-onset Still’s radiographic features of idiopathic multicentric osteolysis
disease. Pauciarticular abnormalities predominate, with a are somewhat variable, pain and swelling of the hands,
predilection for the knees, fingers, and wrists. Radiographic wrists, feet, ankles, and elbows are typical. Because of a
alterations develop in only a few patients. A distinctive remarkable predilection for the carpal and tarsal bones, the
radiographic pattern of articular disease of the wrist has disorder is also referred to as carpal and tarsal osteolysis
been noted: narrowing of portions of the common car- or disappearing carpal bones. Radiographic characteristics
pometacarpal and midcarpal compartments without include osteoporosis, progressive osteolysis, and defor-
osseous erosions, which may culminate in bony ankylosis mity, findings that may simulate the changes in juvenile
(Fig. 17–11). Similar patterns of ankylosis of the carpal chronic arthritis.
(and tarsal) bones can be observed in children with
juvenile chronic arthritis.
Mucopolysaccharidoses and Related
Disorders
Articular abnormalities accompanying mucopolysaccha-
ridoses may simulate those in juvenile chronic arthritis,
although characteristic findings are noted on skeletal sur-
veys in patients with mucopolysaccharidoses (see Chapter
75). In many of these syndromes, periarticular involve-
ment is apparent and leads to alterations in ligamentous
and tendinous structures.
Other Disorders
Figure 17–11. Adult-onset Still’s disease: wrist involvement.
This 44-year-old woman has had Still’s disease since age 25 years. Multiple epiphyseal dysplasia (and spondyloepiphyseal
A frontal radiograph shows bony ankylosis of the midcarpal, dysplasia) can lead to irregularity of many epiphyses and
common carpometacarpal, and intermetacarpal compartments secondary degenerative joint abnormalities that could be
of the wrist. The radiocarpal compartment is moderately narrowed. confused with the changes of juvenile chronic arthritis
(Courtesy of J. Esdaile, MD, Montreal, Quebec, Canada.) (see Chapter 74). Progressive pseudorheumatoid arthritis
266 Section V Rheumatoid Arthritis and Related Diseases
of childhood (i.e., progressive pseudorheumatoid chon- Martel W, Holt JF, Cassidy JT: Roentgenologic manifestations
drodysplasia) is a term applied to a hereditary arthropa- of juvenile rheumatoid arthritis. AJR Am J Roentgenol
thy affecting the major and minor joints; findings include 88:400, 1962.
restricted articular motion, swelling about the interpha- Martinez-Lavin M, Buendia A, Delgado E, et al: A familial syn-
drome of pericarditis, arthritis, and camptodactyly. N Engl J
langeal articulations, platyspondyly, and irregularities of
Med 309:224, 1983.
vertebral bodies. Infection or synovial hemangiomas can Medsger TA Jr, Christy WC: Carpal arthritis with ankylosis in
produce abnormalities of single articulations, which also late onset Still’s disease. Arthritis Rheum 19:232, 1976.
simulate the findings of juvenile chronic arthritis. Similarly, Parke WW, Rothman RH, Brown MD: The pharyngovertebral
neuromuscular disorders may lead to skeletal abnor- veins: An anatomical rationale for Grisel’s syndrome. J Bone
malities of certain articulations that are indistinguishable Joint Surg Am 66:568, 1984.
from those of juvenile chronic arthritis. Other entities to Poznanski AK, Hernandez RJ, Guire KE, et al: Carpal length
be considered in the differential diagnosis of juvenile in children—a useful measurement in the diagnosis of
chronic arthritis include multicentric reticulohistio- rheumatoid arthritis and some congenital malformation
cytosis (see Chapter 50). syndromes. Radiology 129:661, 1978.
Richardson ML, Helms CA, Vogler JB III, et al: Skeletal changes
in neuromuscular disorders mimicking juvenile rheumatoid
arthritis and hemophilia. AJR Am J Roentgenol 143:893,
FURTHER READING 1984.
Schaller J, Wedgwood RJ: Juvenile rheumatoid arthritis: A review.
Ansell BM: Chronic arthritis in childhood. Ann Rheum Dis Pediatrics 50:940, 1972.
37:107, 1978. Schnedl WJ, Lipp RW, Trinker M, et al: Bone scintigraphy and
Ansell BM, Kent PA: Radiological changes in juvenile chronic magnetic resonance imaging in adult-onset Still’s disease.
polyarthritis. Skeletal Radiol 1:129, 1977. Scand J Rheumatol 28:257, 1999.
Brill PW, Kim HJ, Beratis NG, et al: Skeletal abnormalities in Senac MO Jr, Deutsch D, Bernstein BH, et al: MR imaging in
the Kniest syndrome with mucopolysacchariduria. AJR Am juvenile rheumatoid arthritis. AJR Am J Roentgenol 150:873,
J Roentgenol 125:731, 1975. 1988.
Bywaters EGL: Still’s disease in the adult. Ann Rheum Dis Spranger J, Albert C, Schilling F, et al: Progressive pseudorheuma-
30:121, 1971. toid arthropathy of childhood (PPAC): A hereditary disorder
Cassidy JT, Levinson JE, Bass JC, et al: A study of classification simulating juvenile rheumatoid arthritis. Am J Med Genet
criteria for a diagnosis of juvenile rheumatoid arthritis. 14:399, 1983.
Arthritis Rheum 29:274, 1986. Stabrun AE, Larheim TA, Höyeraal HM, et al: Reduced mandibu-
Chlosta EM, Kuhns LR, Holt JF: The “patellar ratio” in lar dimensions and asymmetry in juvenile rheumatoid arthritis:
hemophilia and juvenile rheumatoid arthritis. Radiology Pathogenetic factors. Arthritis Rheum 31:602, 1988.
116: 137, 1975. Still GF: On a form of chronic joint disease in children. Med Chir
Cohen PA, Job-Deslandre CH, Lalande G, et al: Overview of the Trans 80:47, 1897.
radiology of juvenile idiopathic arthritis (JIA). Eur J Radiol Tyler T, Rosenbaum HD: Idiopathic multicentric osteolysis.
33:94, 2000. AJR Am J Roentgenol 126:23, 1976.
Hervé-Somma CMP, Sebag GH, Prieur A-M, et al: Juvenile Winchester P, Grossman H, Lim WN, et al: A new acid
rheumatoid arthritis of the knee: MR evaluation with Gd- mucopolysaccharidosis with skeletal deformities simulating
DOTA. Radiology 182:93, 1992. rheumatoid arthritis. AJR Am J Roentgenol 106:121, 1969.
18
CHAPTER 18
Ankylosing Spondylitis peripheral joints (10% to 20% of patients) or in the chest.
Sciatic pain may be the initial symptom in 5% to 10% of
patients. Constitutional findings include anorexia, weight
loss, and low-grade fever. The consensus is that in less than
SUMMARY OF KEY FEATURES 20% of patients with adult-onset ankylosing spondylitis does
Ankylosing spondylitis is a disease with widespread the disease progress to a condition of significant disability.
musculoskeletal manifestations. Abnormalities are
detected at the synovial and cartilaginous joints and at
sites of tendinous and ligamentous attachment to bone Axial Skeletal Symptoms and Signs
in spinal and extraspinal locations. The hallmark of the Clinical manifestations related to the spine and sacroiliac
disorder is sacroiliitis, which is typically bilateral and joints are characteristic of ankylosing spondylitis. Initially,
symmetrical in distribution. Spondylitis leads to transient aching pain and stiffness of variable intensity
significant abnormalities at the discovertebral junction, are observed in the low back region, but these symptoms
apophyseal and costovertebral joints, and posterior may subsequently become persistent. With evolution of
ligamentous attachments. Accurate differentiation of the disease, spread to higher levels of the vertebral column
ankylosing spondylitis from rheumatoid arthritis is not is frequent.
difficult, but distinguishing the articular abnormalities Local pain and tenderness over the sacroiliac joints
of ankylosing spondylitis from those of psoriatic can be prominent in the early phases of the disease. With
arthritis and Reiter’s syndrome is more troublesome. ankylosis of these joints, the clinical manifestations may
become milder or disappear completely. Pain radiating into
the lower extremities, not unlike sciatica, can be observed
in approximately 50% of patients at some stage of the
disorder. In the lumbar spine, paravertebral muscle spasm,
INTRODUCTION straightening of the vertebral column, tenderness to per-
Ankylosing spondylitis is a chronic inflammatory disorder cussion, and muscle atrophy are observed; in the thoracic
of unknown cause that principally affects the axial skeleton, spine, similar abnormalities may be accompanied by
although the appendicular skeleton may also be signifi- diminished chest expansion and exaggeration of the normal
cantly involved. Alterations occur in synovial and cartilagi- kyphotic curvature. Slight, moderate, or marked limitation
nous joints and in sites of tendon and ligament attachment of movement may be evident in the cervical spine. The
to bone. Although ankylosing spondylitis is now the accepted head and neck protrude forward, and the patient may
name for this disease, many synonyms and eponyms have eventually be forced to constantly gaze at the floor.
been used in the past, including Marie-Strümpell disease, The cauda equina syndrome can be observed in patients
von Bechterew’s syndrome, and rheumatoid spondylitis. with ankylosing spondylitis. Although its pathogenesis in
It is difficult to determine the true prevalence; however, this disease is not clear, the occurrence of loss of cutaneous
ankylosing spondylitis is a common cause of back pain sensation in the sacral and lower lumbar dermatomes,
and disability, especially in young men. The prevalence muscle weakness, and disturbed sphincter function may be
of this disease in the general population is more difficult related to arachnoiditis with subsequent loss of meningeal
to determine, although a figure of approximately 0.1% is elasticity.
commonly quoted. The precise ratio of the disease in men
versus women is not known, with reports varying from
Peripheral Skeletal Symptoms and Signs
4:1 to 10:1. The true prevalence of ankylosing spondylitis
in women may be much higher than these reports indi- Peripheral articular manifestations are initially apparent
cate, as the disease may be more subtle and difficult to in approximately 10% to 20% of patients and eventually
diagnose in female patients. occur in as many as 50%. In most persons, the manifestations
are mild and transient, and asymmetrical involvement of a
few joints is typical. Asymmetry is more frequent, and residual
CLINICAL ABNORMALITIES destruction and deformity are less frequent, in ankylosing
spondylitis than in rheumatoid arthritis. Involvement of the
General Features proximal or “root” joints (hips and shoulders) is particularly
The onset of ankylosing spondylitis generally occurs characteristic and may lead to severe clinical disability.
between the ages of 15 and 35 years (average age of onset, Typical findings include regional pain, limitation of motion,
26 to 27 years) in both men and women, although an earlier muscle atrophy, and flexion contractures.
onset has been noted in some female patients. An insidious
onset of disease, which occurs in 75% to 80% of patients,
Extraskeletal Symptoms and Signs
can lead to considerable delay in accurate diagnosis. Early
clinical manifestations are generally noted in the back (70% Iritis occurs in 20% of patients with ankylosing spondylitis
to 80% of patients), although they may appear in the and may be the initial feature of the disease. Spondylitic
267
268 Section V Rheumatoid Arthritis and Related Diseases
Laboratory Findings
Many patients with ankylosing spondylitis possess the
histocompatibility antigen HLA-B27. The erythrocyte
sedimentation rate is frequently elevated during the active
phase and may become normal in later phases.
RADIOGRAPHIC-PATHOLOGIC CORRELATION
General Distribution
Ankylosing spondylitis affects synovial and cartilaginous
joints, as well as sites of tendon and ligament attachment
to bone (entheses). An overwhelming predilection exists for
involvement of the axial skeleton, especially the sacroiliac,
apophyseal, discovertebral, and costovertebral articulations
(Fig. 18–1).
Classically, changes are noted initially in the sacroiliac
joints and next appear at the thoracolumbar and lum-
bosacral junctions; with disease chronicity, the midlumbar,
upper thoracic, and cervical vertebrae may become involved.
Radiographic abnormalities of the sacroiliac joint with- Figure 18–1. Ankylosing spondylitis: distribution of articular
out vertebral changes are unusual. The combination of disease. Initial abnormalities are most frequent in the sacroiliac
sacroiliac joint and cervical spine abnormalities without joint and the thoracolumbar and lumbosacral junctions (arrow-
significant thoracic or lumbar spine changes appears to be heads). Subsequent abnormalities are common in the entire ver-
tebral column, tendinous insertions in the pelvis and proximal
more common in female patients than in male patients.
end of the femur, sternal joints, symphysis pubis, hips, and
Spinal alterations without sacroiliac joint changes are glenohumeral joints (arrows).
unusual in classic ankylosing spondylitis in either men or
women.
The frequency of radiographically evident abnormali-
ties in peripheral locations in cases of long-standing
ankylosing spondylitis is greater than 50% if all articula- metaplasia with chondral ossification. Intra-articular bony
tions are included. Radiographic changes predominate in ankylosis may be evident. This ankylotic process in the
the hips and glenohumeral joints, followed in descending small articulations of the axial skeleton can also result from
order of frequency by the knees, hands, wrists, and feet, ossification of the joint capsule.
including the calcaneus. Bilateral abnormalities are com- The basic similarity of the pathologic alterations in
mon, although the degree of symmetry is less striking synovial joints in ankylosing spondylitis and rheumatoid
than in cases of rheumatoid arthritis. arthritis accounts for the overlap in their radiographic fea-
Radiographic abnormalities can be encountered in the tures. Both cause some degree of osteoporosis, joint space
symphysis pubis and in the manubriosternal, acromio- narrowing, and osseous erosion. Certain findings are more
clavicular, sternoclavicular and temporomandibular joints. characteristic of ankylosing spondylitis than of rheumatoid
Changes are also evident at tendinous and ligamentous arthritis, however. Prominent periarticular osteoporosis is
attachments to bone. not common in ankylosing spondylitis. In fact, subchon-
dral eburnation is typical. Periostitis is also observed and
results in irregular or shaggy periarticular osseous surfaces.
General Radiographic and Pathologic Extensive and diffuse joint space diminution is more
Abnormalities frequent in rheumatoid arthritis than in ankylosing
Synovial Articulations. In general, the inflammatory process spondylitis. In ankylosing spondylitis, intra-articular bony
in ankylosing spondylitis is similar to that in rheumatoid ankylosis is common in the synovial joints of both the
arthritis; however, it is more discrete and of lower inten- axial skeleton (sacroiliac, costovertebral, and apophyseal
sity. Marked fibroplasia may be followed by cartilaginous joints) and the extra-axial skeleton (hip, wrist, and articu-
Chapter 18 Ankylosing Spondylitis 269
lations of the midfoot). In adult-onset rheumatoid arthritis, Radiographic and Pathologic Abnormalities
fibrous ankylosis is more characteristic, except in the carpal at Specific Sites
and tarsal areas.
Sacroiliac Joint
Cartilaginous Articulations. In the cartilaginous joints of Sacroiliitis is the hallmark of ankylosing spondylitis. It
the axial skeleton (discovertebral junction, symphysis pubis, occurs early in the course of the disease. Although an
and manubriosternal articulation), the fundamental process asymmetrical or unilateral distribution may be evident on
appears to be inflammatory in nature. At the junction of the initial radiographic examination, radiographic changes
the intervertebral disc and the vertebral edge, chondro- at later stages of the disease are almost invariably bilateral
cytes undergo calcification and become vascularized and and symmetrical (Fig. 18–4).
subsequently ossified, with production of syndesmophytes Changes in the sacroiliac joint occur in both the syn-
that extend from one vertebral body to another (Fig. 18–2). ovial and ligamentous (superior and posterior) portions.
Eventually, extensive bony ankylosis of the entire articu- They predominate in the ilium. Preferential involvement
lation may be evident. These alterations in cartilaginous of the ilium likely results from mechanical or anatomic
joints are typical of the seronegative spondyloarthropathies features. These include thinner iliac cartilage (compared
and are not characteristic of the skeletal involvement in with the sacrum) and the presence of degenerative clefts
rheumatoid arthritis. through which inflammatory synovial tissue may gain
access to the subchondral bone of the ilium. Initial changes
Entheses. Abnormalities in ligamentous attachments consist of patchy periarticular osteoporosis, loss of defi-
(enthesopathy) are a prominent feature of ankylosing nition, superficial erosion, and focal sclerosis of subchon-
spondylitis and the other seronegative spondyloarthropathies. dral bone. A poorly defined subchondral bone plate is an
Inflammation with cellular infiltration is associated with important radiographic sign of sacroiliitis that is not
erosion and eburnation of the subligamentous bone. On observed in degenerative sacroiliac joint disease. Further
radiographs, poorly defined erosive abnormalities with erosive changes lead to considerable fraying of the osseous
surrounding sclerosis are observed. As the lesions heal, surface and widening of the interosseous space. As pro-
the sclerosis decreases, the osseous surface becomes less liferative bony changes in the sacroiliac joint become more
irregular, and well-defined bony excrescences appear prominent, irregular bony bridges traverse the articular
(Fig. 18–3). cavity. This process of osseous fusion is initially incomplete.
A B
270 Section V Rheumatoid Arthritis and Related Diseases
A B
C D
Figure 18–4. Abnormalities of the sacroiliac joint: radiographic stages (four different patients). A, Initial
abnormalities consist of superficial bony erosion and eburnation, predominantly in the ilium. B, At a slightly
later stage, note the larger erosions (arrows), progressive sclerosis, and focal narrowing of the articular space.
C, At a more advanced stage, bilateral symmetrical changes consist of extensive sclerosis and focal ankylosis.
D, Eventually, complete ankylosis of the synovial and ligamentous portions of the sacroiliac space on both
sides is evident. The sclerosis has diminished.
clinically. Hyperextension injuries lead to spinal fractures Discal Calcification. Central or eccentric circular or linear
that begin anteriorly in the vertebral body or, more com- calcific collections may appear within the intervertebral
monly, the intervertebral disc; hyperflexion injuries disc at single or multiple sites (Fig. 18–11). Calcifications
produce spinal fractures that commence in the posterior are accentuated by osteoporosis of the surrounding ver-
osseous elements of the vertebra. Many of the resulting tebral bodies. Similar deposits accompany other conditions
fractures become displaced. With continued movement of the vertebral column that are characterized by ankylosis,
at the fracture site, however, fibrous union can occur and such as diffuse idiopathic skeletal hyperostosis (DISH)
result in what has been labeled a pseudarthrosis. and juvenile chronic arthritis.
272 Section V Rheumatoid Arthritis and Related Diseases
TABLE 18–1
Terminology Commonly Applied to Spinal Abnormalities in Ankylosing Spondylitis
Term Definition
Osteitis Enthesopathy occurring at the discovertebral junction associated with erosion, sclerosis, and
syndesmophytosis
“Shiny corner” sign Increased radiodensity of the corners of the vertebral body related to “osteitis”
Squaring Straightened or convex anterior margin of the vertebral body related to erosion
Syndesmophyte Ossification within the anulus fibrosus leading to thin, vertical, radiodense areas
Bamboo spine Undulating vertebral contour caused by extensive syndesmophytosis
Discitis “Erosive” abnormalities of the discovertebral junction related to several mechanisms
Discal ballooning Biconvex shape of the intervertebral disc related to osteoporotic deformity of the vertebral body
Trolley-track sign Three vertical radiodense lines on frontal radiographs related to ossification of the supraspinous and
interspinous ligaments and apophyseal joint capsules
Dagger sign Single central radiodense line on frontal radiographs related to ossification of the supraspinous and
interspinous ligaments
A B C
Figure 18–5. Osteitis: radiographic abnormalities (three different patients). A, Osseous erosion and sclerosis
have produced whitening of the corners and margins along the anterior surfaces of the vertebrae (arrows).
Note the straightening of the vertebral surface (arrowhead). B, Considerable straightening (arrowheads) and
bone formation (arrows) along the anterior vertebral surface are observed. C, Convex anterior margin
(arrowheads) of the vertebral bodies is associated with eburnation.
A B
joint involvement and the size of the syndesmophytes at are readily apparent on lateral radiographs. In any segment
the corresponding spinal level. of the spine, apophyseal joint space narrowing is common
as the disease process continues.
Erosion and Sclerosis. Poorly defined erosions of apophy-
seal joints in the lumbar, thoracic, and cervical segments Bony Ankylosis. Apophyseal joint osseous fusion and cap-
of the spine are accompanied by reactive subchondral sular ossification are frequent in the lumbar, thoracic, and
bone formation. In the cervical spine, such abnormalities cervical spine in this disease (Fig. 18–12). These findings
274 Section V Rheumatoid Arthritis and Related Diseases
Costovertebral Joints
The costovertebral joints may demonstrate erosion, scle-
rosis, and ankylosis (Fig. 18–14). Indistinctness and ero-
sion of subchondral bone, sclerosis, and partial or complete
B C osseous fusion are more easily defined with computed
A
tomography (CT) scanning.
Figure 18–7. Discovertebral erosion and destruction: types of
lesions. A, Localized central discovertebral lesions. Defects
with surrounding eburnation in the central portion of the Posterior Ligamentous Attachments
discovertebral junction may reflect intraosseous displacement Lesions affecting the posterior ligamentous attachments
of disc material (cartilaginous nodes). B, Localized peripheral include calcification, ossification, and subligamentous
discovertebral lesions. Defects may occur on the anterior or erosion (Fig. 18–15).
posterior aspect of the discovertebral junction. Their cause is
obscure; they may be related to kyphosis with discal injury,
cartilaginous node formation, or enthesopathy. C, Extensive Atlantoaxial Articulations
central and peripheral discovertebral lesions. Destruction of the
entire discovertebral junction is frequently the result of Inflammatory changes of the synovial and adjacent liga-
improper fracture healing. mentous structures can lead to erosion of the dens. Similar
abnormalities may be evident in rheumatoid arthritis.
Although atlantoaxial subluxation can be observed in
patients with ankylosing spondylitis, the frequency of this
are less common in psoriatic spondylitis and Reiter’s syn- complication appears to be less than that in rheumatoid
drome. Apophyseal joint ankylosis can be very striking in arthritis. When present, atlantoaxial subluxation is
the cervical spine (Fig. 18–13). Complete obliteration of generally observed in the later stages of the disease.
the articular spaces between the posterior elements of the
second through seventh vertebrae results in a true column,
or pillar, of bone. The appearance is reminiscent of that Complications of Spinal Involvement
Neurospinal complications, including spinal cord com-
pression and even death, are a recognized though infre-
quent manifestation of ankylosing spondylitis. Vertebral
fractures, especially in the cervical region, are associated
with significant morbidity and mortality related to defor-
mity of the cord produced by osseous surfaces, as well as
hematomas. Atlantoaxial instability, in either a horizontal
or a vertical direction, can produce neurologic deficit and
may be fatal. Spondylodiscitis has been associated with
compression of the cord or nerve roots, especially in the
lumbar segment.
Although the mechanism is not clear, spinal stenosis is
being recognized with increasing frequency in patients
with ankylosing spondylitis. The cauda equina syndrome
is also observed in some patients with ankylosing spondyli-
tis. This syndrome occurs late in the course of the disease.
Typical clinical findings include cutaneous sensory impair-
ment of the lower limbs and perineum with sphincter
disturbances. Widening of the neural canal in the lumbar
segment, dilatation of the dural sac, and thecal diverticula
may also be seen (Fig. 18–16).
Symphysis Pubis
Figure 18–8. Discovertebral erosion and destruction. Local- Alterations of the symphysis pubis (Fig. 18–17) have been
ized central and peripheral defects with bony sclerosis (arrow- described in 16% to 23% of patients with ankylosing
heads) are observed on a lateral conventional tomogram of the spondylitis. Erosion and blurring of the subchondral bone
thoracolumbar junction. Syndesmophytosis (arrow) is also seen. on both sides of the joint are combined with adjacent ebur-
The apophyseal joint at this level is only partially ossified. nation. Articular space narrowing and bony ankylosis may
(Courtesy of V. Vint, MD, San Diego, Calif.) be noted.
A B
C D
Figure 18–9. Discovertebral erosion and destruction: central and peripheral lesions—improper fracture
healing or pseudarthrosis. A, Lateral radiographs reveal a typical pseudarthrosis of the lower thoracic spine
characterized by extensive osseous resorption and sclerosis (arrows). The appearance simulates that of an
infection. B, Sagittal reformatted CT scan shows the fracture to better advantage. C and D, Corresponding
sagittal T1-weighted (TR/TE, 500/14) spin echo MR image (C) and fast T2-weighted (TR/TE, 4000/108)
MR image (D) show nonspecific abnormal signal intensity adjacent to the pseudarthrosis (arrow).
276 Section V Rheumatoid Arthritis and Related Diseases
Manubriosternal Joint
Involvement of the manubriosternal joint generally
occurs in patients with long-standing disease and severe
sacroiliitis. Bony proliferation leads to sclerosis and
intra-articular ankylosis, and soft tissue masses about the
affected joint have been described.
Sternoclavicular Joint
Bilateral or, less commonly, unilateral abnormalities of the
sternoclavicular joint in ankylosing spondylitis consist of
erosion and sclerosis of the sternum and the medial aspect
of the clavicle, findings mimicking those in rheumatoid
arthritis or infection. Osseous fusion may eventually occur.
A B
Figure 18–18. Abnormalities of the hip: osteophytosis. A, Observe the bone formation on the lateral
margin of the femoral head (arrow), which has resulted in a bumpy contour. B, The osteophytes have formed
a collar about the femoral neck (arrows). Extensive concentric joint space narrowing has occurred.
(From Dwosh IL, Resnick D, Becker MA: Hip involvement in ankylosing spondylitis. Arthritis Rheum
19:683, 1976.)
effusions, osteoporosis, and joint space narrowing. Addi- a predilection for the metatarsophalangeal and first tar-
tional manifestations are marginal erosions, subchondral sometatarsal joints and for the interphalangeal joint of the
cysts, and juxta-articular periostitis. great toe. Soft tissue swelling, diffuse joint space narrowing,
erosions with adjacent bony proliferation predominantly
on the medial aspect of the metatarsal heads, periostitis of
Forefoot and Midfoot the phalangeal and metatarsal shafts, and intra-articular
Bilateral symmetrical or asymmetrical abnormalities of bony ankylosis can be detected. Subluxation at the metatar-
the feet, which may be evident in approximately 15% of sophalangeal joints consisting of fibular deviation of the toes
patients with long-standing ankylosing spondylitis, show is less frequent and severe than in rheumatoid arthritis.
280 Section V Rheumatoid Arthritis and Related Diseases
Calcaneus
Although clinically manifested heel abnormalities are
infrequent in ankylosing spondylitis, radiographic changes
of the calcaneus are common (Fig. 18–21). Bilateral abnor-
malities predominate. Well-defined plantar or posterior
calcaneal enthesophytes are a common manifestation but
are similar in appearance to those in a “normal” population.
Retrocalcaneal swelling (related to bursitis), posterior
calcaneal erosion, and Achilles tendon thickening are also
frequent findings. Bony erosion and proliferation resulting
in poorly defined enthesophytes at the site of ligamen-
tous attachment to bone on the inferior surface of the
calcaneus are identical to the findings of psoriatic arthritis
and Reiter’s syndrome.
Temporomandibular Joint
Clinical and radiographic manifestations result from tem-
poromandibular joint arthritis in patients with ankylosing
spondylitis. Asymmetrical or unilateral involvement is fre-
quent. Erosions predominate on the mandibular condyle.
A B
Figure 18–22. Scintigraphic abnormalities in ankylosing spondylitis. A, In this patient with typical
spondylitic changes in the sacroliac joints, 99mTc pyrophosphate radionuclide scintigraphy shows marked
increased focal uptake. Uptake in the hip corresponds to entheses. B, In another patient with long-standing
ankylosing spondylitis who had been injured in a fall, a focal area of markedly increased activity on bone scan
corresponds to the site of a fracture and pseudarthrosis. The sacroiliac joints are ankylosed and are normal
on scintigraphy.
282 Section V Rheumatoid Arthritis and Related Diseases
A B
C D
Figure 18–25. MR imaging in ankylosing spondylitis: sacroiliitis. A and B, In this 17-year-old patient with
suspected sacroiliitis, conventional radiography and CT failed to reveal any abnormalities. Transaxial T1-
weighted (TR/TE, 500/22) spin echo MR image (A) shows regions of low signal intensity about both
sacroiliac joints (arrows). In a transaxial intermediate-weighted (TR/TE, 1500/37) spin echo MR image (B),
the signal intensity in the periarticular bone marrow is increased (arrows). C and D, In a different patient
with confirmed ankylosing spondylitis, a transaxial T1-weighted (TR/TE, 500/22) spin echo MR image
(C) reveals low signal intensity within periarticular bone marrow (arrows). A similar T2-weighted (TR/TE,
2000/90) MR image (D) shows persistent low signal intensity in these regions (arrows).
(From Ahlström H, Feltelius N, Nyman R, Hallgren R: Magnetic resonance imaging of sacroiliac joint
inflammation. Arthritis Rheum 33:1763, 1990.)
A B
Figure 18–26. MR imaging in ankylosing spondylitis: sacroiliitis. As shown in these transaxial T1-
weighted (TR/TE, 600/20) spin echo (A) and fat-suppressed T2-weighted (TR/TE, 4000/104) fast spin
echo (B) MR images, abnormalities of subchondral bone may be the most prominent finding of active
sacroiliitis. The subchondral bone in the sacrum and ilium is of low signal intensity in (A) and high signal
intensity in (B). (Courtesy of S. Lee, MD, Orange, Calif.)
284 Section V Rheumatoid Arthritis and Related Diseases
Patterns of enhancement of signal intensity after contrast erative joint disease, although in the last two conditions,
agent administration may provide information with regard an asymmetrical or unilateral distribution is not uncom-
to disease activity. The specificity of MR imaging in the mon. Unilateral abnormalities are most typical in infection.
differentiation of inflammatory and degenerative condi- Poorly defined erosive abnormality with adjacent
tions of the sacroiliac joint is not yet clear, however. sclerosis (particularly in the ilium), associated joint space
narrowing, intra-articular osseous fusion, and ligamentous
ossification is the characteristic appearance of sacroiliac
CAUSE AND PATHOGENESIS
joint disease in classic ankylosing spondylitis and in the
A strikingly high frequency of the HLA-B27 antigen has sacroiliitis of inflammatory bowel disease. In psoriatic
been documented in patients with ankylosing spondylitis. arthritis and Reiter’s syndrome, extensive bony eburnation
In most reports, more than 90% of white spondylitic patients may be unaccompanied by intra-articular osseous fusion.
possess the B27 gene; the frequency of this gene in whites Sacroiliac joint involvement in rheumatoid arthritis is
who do not have ankylosing spondylitis is approximately usually manifested as superficial erosions, minimal sclerosis,
6% to 8%. To date, this finding is the most significant and absence of significant bony ankylosis. Subchondral
association between a readily detectable HLA antigen and resorption of bone, predominantly in the ilium, in con-
a well-defined disease, and it underscores the importance junction with primary or secondary hyperparathyroidisms
of genetics in the causation of ankylosing spondylitis. leads to irregularity of the osseous surface, adjacent scle-
rosis, and widening of the interosseous joint space. In
osteitis condensans ilii, a triangular segment of bony
DIFFERENTIAL DIAGNOSIS
sclerosis is evident in the inferior aspect of the ilium. The
Sacroiliitis joint surface is well defined, and the articular space is not
The sacroiliac joint abnormalities in ankylosing spondylitis diminished. In chronic tophaceous gouty arthritis, large,
must be differentiated from those accompanying other gouged-out defects with surrounding sclerosis are observed,
disorders (Table 18–3). This can be accomplished by whereas in degenerative arthritis, joint space narrowing,
analyzing both the distribution and the morphology of the bony sclerosis, and anterior osteophytes are associated
articular changes. Classically, a bilateral and symmetrical with a noneroded, smooth subchondral bony margin.
distribution is observed in ankylosing spondylitis. Although Calcification and ossification of the interosseous ligament
a similar pattern may be evident in other seronegative above the synovial sacroiliac joint can be encountered in
spondyloarthropathies, such as psoriatic arthritis and degenerative joint disease and DISH. Cartilage atrophy
Reiter’s syndrome, asymmetrical or, infrequently, uni- accompanying paralysis or disuse produces diffuse loss of
lateral alterations may accompany these latter disorders. articular space with surrounding osteoporosis.
A bilateral and symmetrical distribution, identical in every
regard to that of classic ankylosing spondylitis, is also
associated with the sacroiliitis of inflammatory bowel Spondylitis
disease (ulcerative colitis, Crohn’s disease, Whipple’s disease). Spinal abnormalities in classic ankylosing spondylitis ini-
In rheumatoid arthritis, minor sacroiliac articular abnor- tially appear in the thoracolumbar and lumbosacral junc-
malities are commonly bilateral but may be asymmetrical tions and may subsequently extend throughout the thoracic
in appearance. Bilateral and symmetrical alterations are and lumbar spine and into the cervical region. An iden-
also noted in hyperparathyroidism (and renal osteodys- tical distribution is encountered in the spondylitis of inflam-
trophy), osteitis condensans ilii, gouty arthritis, and degen- matory bowel disease. Although the entire vertebral column
TABLE 18–3
TABLE 18–4
Bony Outgrowths of the Spine
A B C D
E F
TABLE 18–5
Abnormalities of Synovial Articulations
TABLE 19-2
Characteristics of Psoriatic Arthritis
one phalanx may produce a small, blunt osseous surface neck deformities are not as common in psoriatic arthritis
that projects into the expanded base of a neighboring as in rheumatoid arthritis.
phalanx, reminiscent of a pencil-and-cup or cup-and-
saucer arrangement. Similar changes are encountered at
Radiographic Abnormalities at Specific Sites
metacarpophalangeal and metatarsophalangeal locations.
In some patients with psoriatic arthritis, complete disso- Hand. The destructive arthritis of the distal interphalangeal
lution of large segments of apposing bones, fragmenta- joints of the hand (Figs. 19–2 and 19–3) is the best-known
tion with osseous debris, and disorganization of the joint manifestation of psoriatic arthritis. At these sites, bilateral
resemble the changes of neuropathic osteoarthropathy. symmetrical or asymmetrical changes or unilateral changes
are observed. Initial erosions occur at the margins of the
Bone Proliferation. As in the other seronegative spondylo- articulation and proceed centrally. The resulting irregular
arthropathies, proliferation of bone is a striking feature osseous surfaces may become separated from each other
of psoriatic arthritis. Irregular excrescences create a spicu- as a consequence of the extensive nature of the erosive
lated, frayed, or “paintbrush” appearance. Bone prolif- process. This lack of apposition of adjacent bony margins
eration probably relates to an exaggerated healing response distinguishes the radiographic picture of psoriatic arthritis
of the injured bone. The osseous erosion in rheumatoid from that of osteoarthritis, in which closely applied undu-
arthritis is generally not associated with adjacent bony lating osseous surfaces are the rule. The adjacent proximal
deposition. Although bone proliferation may accompany interphalangeal joints are frequently affected. The metacar-
erosions in gouty arthritis, the resulting excrescences are pophalangeal joints may be relatively spared.
generally well defined. At any altered interphalangeal site, radiographic find-
Periostitis in the metaphyses and diaphyses of bones is ings may include separated, eroded, well-demarcated bone
not uncommon in psoriatic arthritis, particularly in the margins; protrusion of a blunted and distorted osseous
hands and feet. In these locations, periosteal bone forma- surface into an adjacent expanded one (pencil-and-cup
tion, probably related to tenosynovitis, may lead to signif- appearance); irregular periosteal bone proliferation
icant “cloaking” of an entire phalanx or a portion of a (whiskering); and intra-articular osseous fusion. Tuft
metacarpal or metatarsal bone. This change may appear resorption may be evident in one or more terminal
early in the disease course, in association with soft tissue phalanges. Both phalangeal resorption and destructive
swelling, before significant abnormalities occur in the arthritis of the distal interphalangeal joints are generally
adjacent articulations. Condensation of bone on the associated with significant nail changes in the same digit.
periosteal and endosteal surfaces of the cortex and tra-
becular thickening in the spongiosa can cause an entire Wrist. Abnormalities in the wrist in psoriatic arthritis
phalanx to appear radiodense. This latter appearance is (Fig. 19–4) are not as frequent as those in the fingers and
termed the ivory phalanx and is most common in the are rarely encountered without more typical distal changes.
terminal phalanges of the toes.
Intra-articular osseous fusion is another manifestation Other Upper Extremity Sites. Psoriatic arthritis can lead
of bone proliferation in psoriatic arthritis, and it is to changes in the elbow and glenohumeral, acromio-
particularly prominent in the hands and feet. Although clavicular, and sternoclavicular joints.
intra-articular osseous fusion is also observed in inflam-
matory (erosive) osteoarthritis, septic arthritis, and even Forefoot. The forefoot is commonly affected in psoriatic
rheumatoid arthritis (carpal and tarsal areas), it is an arthritis (Fig. 19–5). Bilateral asymmetrical changes pre-
important radiographic sign of the seronegative spondylo- dominate in the interphalangeal and metatarsophalangeal
arthropathies. joints and are characterized by marginal erosions, bony
Bone proliferation occurs at sites where tendons and proliferation, alterations in joint space (narrow or wide),
ligaments insert on bones. These locations include the and lack of osteoporosis. Extensive destruction of the
posterior and inferior surfaces of the calcaneus, femoral interphalangeal joint of the great toe is more charac-
trochanters, ischial tuberosities, medial and lateral malleoli, teristic of this articular disorder than of any other disease.
ulnar olecranon, and anterior surface of the patella. Osteolysis of tufts and the phalangeal and metatarsal
shafts may be encountered. In the terminal phalanges,
Tuft Resorption. Resorption of the tufts of the distal extensive new bone formation may lead to increased
phalanges of the hands and feet is characteristic of osseous density of the entire bone (ivory phalanx).
psoriatic arthritis. Soft tissue swelling and adjacent inter-
phalangeal joint abnormalities are frequent. The nail of Calcaneus. As in the other seronegative spondyloarthro-
the involved digit is almost always affected. pathies, erosion and proliferation of the posterior or infe-
rior surface of the calcaneus, or both, may be prominent
Malalignment and Subluxation. Deformities of the hands in psoriatic arthritis (Fig. 19–6). Retrocalcaneal bursitis
and feet can be encountered in some patients with psoriatic creates a radiodense area adjacent to the posterosuperior
arthritis. Telescoping of one bone on its neighbor may aspect of the bone that may extend into the pre-Achilles
lead to the “opera-glass hand.” In this situation, excess fat pad. Subjacent erosion of the calcaneus is associated
skin may be folded over the involved joints and produce with surrounding bony proliferation.
a concertina-like appearance. Ulnar deviation at the
metacarpophalangeal joints, fibular deviation at the Other Lower Extremity Sites. Articular involvement in
metatarsophalangeal joints, and boutonnière and swan- psoriatic arthritis may be apparent in the midfoot or
Chapter 19 Psoriatic Arthritis 291
A B C
Figure 19–2. General radiographic abnormalities. A to C, Classic radiographic changes are depicted
about the distal interphalangeal joints in three patients with psoriatic arthritis. These changes include soft
tissue swelling, lack of osteoporosis, joint space narrowing, osseous erosions with accompanying proliferation
(solid arrows), osteolysis with a pencil-and-cup appearance (open arrow), and tuft resorption (arrowheads).
Figure 19–3. Radiographic abnormalities of the hand. hindfoot, ankle, or knee. Abnormality of the hip is rela-
Interphalangeal joint changes consist of articular space tively unusual.
narrowing, intra-articular bony ankylosis, marginal and central
erosions, flexion contractures, and osteolysis of the phalangeal Sacroiliac Joint. Approximately 10% to 25% of patients
tufts. Metacarpophalangeal joint abnormalities, although less with moderate or severe psoriatic skin disease have
marked, include joint space narrowing, marginal erosions, and sacroiliac joint changes on radiographic examination. It
bony proliferation. also appears that such changes develop in approximately
292 Section V Rheumatoid Arthritis and Related Diseases
B C
PATHOLOGIC ABNORMALITIES
Synovial Articulations
Although the pathologic changes of psoriatic arthritis are
basically similar to those of rheumatoid arthritis, some
pathologic characteristics of psoriatic arthritis are distinc-
tive: (1) synovial inflammation is encountered, but the
degree of cellular infiltration with lymphocytes and plasma
cells is much less marked than in rheumatoid arthritis; (2)
inflammatory synovial tissue, or pannus, is prominent only
on the surface of the cartilage, whereas in rheumatoid
arthritis, hyperplastic synovium is seen in both the super-
ficial and the deep layers of cartilage; (3) bone prolifer-
ation is evident in the periarticular regions; (4) fibrous
ankylosis of the articulation may be noted, as in rheu-
matoid arthritis, but in psoriatic arthritis, bony ankylosis
is also prominent.
Figure 19–10. Scintigraphic abnormalities. Radionuclide
imaging using a bone-seeking pharmaceutical preparation in a
patient with psoriatic arthritis outlines uptake in the Discovertebral Junction
metacarpophalangeal and interphalangeal joints of the hand Details of the histologic findings related to paravertebral
(arrows). Although the other side was involved, the distribution
ossification in psoriatic arthritis (and Reiter’s syndrome)
of abnormalities was asymmetrical.
(From Weissberg D, Resnick D, Taylor A, et al: Rheumatoid are lacking. An inflammatory process in the paravertebral
arthritis and its variants: Analysis of scintiphotographic, connective tissue or a periosteal reaction at the site of
radiographic, and clinical examinations. AJR Am J Roentgenol osseous attachment of the ligaments and tendons may be
131:665, 1978.) significant in this regard.
A B
Figure 19–11. MR imaging abnormalities. Marrow and soft tissue edema are seen in this patient with
psoriatic arthritis and a swollen, hot foot. A, Sagittal T1-weighted (TR/TE, 506/12) spin echo MR image
shows marrow edema of low signal intensity principally involving the navicular, cuneiform, and metatarsal
bones. Dorsal soft tissue edema is also seen. B, Sagittal short tau inversion recovery (STIR) MR image
(TR/TE, 7268/60; inversion time, 150 msec) reveals high signal intensity at these same sites, as well as an
ankle effusion, increased signal intensity in the sinus tarsi, and subtle calcaneal edema. Biopsy of the bone
excluded the presence of infection. (Courtesy of T. Learch, MD, Los Angeles, Calif.)
Chapter 19 Psoriatic Arthritis 295
Osteolysis molysis bullosa, erythema elevatum diutinum, and
Tapering and dissolution of the terminal phalanges are ichthyosiform erythroderma.
characteristic alterations in psoriatic arthritis. Initially,
thinning or loss of the cortex is related to irregular rapid
removal and synthesis of bone. The surrounding periosteal DIFFERENTIAL DIAGNOSIS
membrane demonstrates noninflammatory cellular prolifer-
Other Seronegative Spondyloarthropathies
ation, although the periosteal process ceases with time.
(Ankylosing Spondylitis, Reiter’s Syndrome)
The radiographic findings in psoriatic arthritis are funda-
CAUSE AND PATHOGENESIS mentally similar to those in the other two seronegative
Hereditary factors appear to be important in the patho- spondyloarthropathies, ankylosing spondylitis and Reiter’s
genesis of uncomplicated psoriatic arthritis, but the exact syndrome (Table 19–3). In all three disorders, synovial
mode of inheritance is not known. The role of heredity joint involvement is characterized by the absence of
in the articular manifestations of this disease has also osteoporosis and the presence of soft tissue swelling,
been emphasized. Histocompatibility typing in patients joint space abnormality, osseous erosion, and bony pro-
with psoriatic arthritis has revealed a high frequency liferation. In psoriatic arthritis and ankylosing spondylitis,
(approximately 25% to 60%) of HLA-B27 antigen, intra-articular bony ankylosis is not uncommon; in Reiter’s
particularly in patients with sacroiliitis or spondylitis. syndrome, such ankylosis is less frequent. In psoriatic
arthritis, the extent of osteolysis of juxta-articular bone
is greater than in Reiter’s syndrome and ankylosing
ADDITIONAL DISEASES OF SKIN spondylitis.
AND JOINTS In each of these seronegative spondyloarthropathies,
Certain cutaneous disorders are associated with clinical abnormalities of the cartilaginous joints, consisting of
and radiographic findings of arthritis, which in some cases erosion and bony proliferation, may be observed.
simulate those of psoriatic arthritis. In acne fulminans, Similarly, each of these diseases may be associated with
arthritis typically occurs in male adolescents. Musculo- abnormalities at tendon and ligament attachments to
skeletal symptoms typically begin at the same time as skin bone. In psoriatic arthritis, osteolysis of the terminal
abnormalities. Findings may include sacroiliitis and syn- phalanges is characteristic.
ovitis in the peripheral joints, associated with osteopenia The distribution of articular abnormalities differs
and periostitis. Osteolytic lesions of the cervical vertebral among psoriatic arthritis, Reiter’s syndrome, and anky-
bodies and intervertebral discs may be multiple in about losing spondylitis. In psoriatic arthritis, an asymmetrical
50% of patients and are commonly accompanied by polyarticular disorder involving the upper and lower
periosteal reaction. The clavicle, sternum, and tubular extremities, with a predilection for the interphalangeal
bones are frequent sites of involvement. joints of the hands and the metatarsophalangeal and
In acne conglobata and hidradenitis suppurativa, interphalangeal joints of the feet, is observed. In Reiter’s
articular disease is seen in adults. Bone erosion about the syndrome, asymmetrical disease of the articulations of
small joints of the hand, wrist, and foot; periostitis; soft the lower extremity is most characteristic, whereas in
tissue swelling; and osteoporosis are noted. In the axial ankylosing spondylitis, appendicular skeletal involvement
skeleton, unilateral or, less commonly, bilateral sacroiliitis is less prominent than axial skeletal involvement.
and syndesmophytosis, particularly in an asymmetrical Spinal and sacroiliac joint alterations occur in psoriatic
distribution in the lumbar and thoracic segments, are the arthritis, Reiter’s syndrome, and ankylosing spondylitis.
reported manifestations. In the first two disorders, symmetrical or asymmetrical
Pyoderma gangrenosum is characterized by painful abnormalities of the sacroiliac joints and large, broad
skin ulcers and occurs as an isolated event or in com- excrescences of the spine may be seen; however, the
bination with ulcerative colitis, Crohn’s disease, myelo- prevalence and severity of spinal changes, particularly in
proliferative disorders, and paraproteinemias. Articular the cervical spine, are less in Reiter’s syndrome than in
manifestations develop in approximately 30% of patients psoriatic arthritis. In ankylosing spondylitis (as well as in
with this skin disease. Although the joint alterations are the sacroiliitis and spondylitis of inflammatory bowel
variable, pyoderma gangrenosum may be accompanied disease), bilateral symmetrical sacroiliac joint abnor-
by a seronegative polyarthritis that is similar or identical malities are almost universal, and spinal changes typically
to rheumatoid arthritis. consist of thin, linear, and symmetrically distributed
Pustular lesions of the skin in the hand and foot outgrowths.
(pustulosis palmaris et plantaris) are observed in some
persons with hyperostosis of the clavicles, upper ribs, and
Rheumatoid Arthritis
sternum. This syndrome, which is termed sternocosto-
clavicular hyperostosis, and related syndromes, including In some patients with psoriatic arthritis, the distribution
chronic recurrent multifocal osteomyelitis (CRMO) and of articular abnormalities in the appendicular skeleton is
synovitis, acne, pustulosis, hyperostosis, and osteitis similar to that in rheumatoid arthritis, whereas in others,
(SAPHO) syndrome, are discussed in Chapter 82. asymmetry and extensive distal interphalangeal articular
Acro-osteolysis may accompany a variety of derma- alterations facilitate differentiation from rheumatoid
tologic conditions in addition to psoriatic arthritis; they arthritis. In the latter disease, osteoporosis, diffuse joint
include mycosis fungoides, pityriasis rubra pilaris, epider- space narrowing, marginal erosions, and fibrous anky-
296 Section V Rheumatoid Arthritis and Related Diseases
TABLE 19-3
Differential Diagnosis of Psoriatic Arthritis
*Symphyses and entheses are less commonly and less extensively involved in rheumatoid arthritis than in psoriatic arthritis or Reiter’s syndrome.
†
–, absent; +, occasionally present; ++, commonly present.
‡
Less frequent than in psoriatic arthritis.
§
Occassionally seen in male patients with rheumatoid arthritis and in those with both rheumatoid arthritis and diffuse idiopathic skeletal hyperostosis.
RADIOGRAPHIC ABNORMALITIES
The radiographic features of Reiter’s syndrome have
received considerable emphasis in the literature
(Table 20–1).
A B
pathies and is the most helpful radiographic feature in Tendinous Calcification and Ossification. Tendinous calcifi-
distinguishing these conditions from rheumatoid arthritis. cation and ossification are frequent about the knee, where
Linear or fluffy periosteal bony proliferation is not the findings can resemble Pellegrini-Stieda syndrome (post-
uncommon in Reiter’s syndrome, especially in the traumatic calcification of the medial collateral ligament).
metacarpal, metatarsal, and phalangeal shafts; the
malleolar region; and the knee. At sites of tendon and
Specific Sites of Abnormality
ligament attachment to bone (e.g., plantar aspect of
calcaneus, ischial tuberosity), osseous surfaces frequently Forefoot. Radiographs of the feet frequently reveal asym-
appear poorly defined or frayed. The eroded bony sur- metrical involvement of the metatarsophalangeal and
faces appear irregular, fuzzy, or blurred in outline, and interphalangeal joints (Fig. 20–3); the reported preva-
the articular bone may be enlarged. Subchondral sclerosis lence of these findings varies from 40% to 55%. At any
and eburnation and adjacent periostitis are associated location in the foot, osteoporosis, joint space loss, and
radiographic findings. Intra-articular bony ankylosis has marginal erosions with adjacent proliferation can be
been recorded in the small joints of the hands and feet, observed. The sesamoid bones can undergo significant
but this complication is far less frequent. erosion and proliferation. Subluxation and deformity of
Chapter 20 Reiter’s Syndrome 301
A B
Figure 20–3. Abnormalities of the forefoot. A, Magnification radiograph of the third metatarsophalangeal
joint outlines erosions of the metatarsal head (arrowheads) and adjacent bony proliferation (arrows). The joint
space is not narrowed. B, Radiograph of the forefoot reveals soft tissue swelling of the second digit
(arrowheads), destruction of the distal interphalangeal joint, and intra-articular bony ankylosis of the
proximal interphalangeal joint. Note the absence of osteoporosis.
the metatarsophalangeal articulations may be evident, an Hand and Wrist. Severe and widespread radiographic
appearance that has been termed Launois’ deformity. abnormalities of the upper extremity are distinctly
unusual in Reiter’s syndrome. In 10% to 30% of patients,
Calcaneus. Calcaneal alterations are characteristic of however, one or more fingers of one or both hands reveal
Reiter’s syndrome (25% to 50% of patients). Both the significant radiographic changes (Fig. 20–5). Proximal
posterior (Fig. 20–4) and the plantar (see Fig. 20–2C) interphalangeal joint abnormalities are more frequent
aspects of the bone are affected. Bilateral changes are than metacarpophalangeal or distal interphalangeal joint
frequent. Retrocalcaneal bursitis with fluid accumulation alterations. Fusiform or sausage-like soft tissue swelling,
creates a radiodense shadow that, on lateral radiographs, regional or periarticular osteoporosis, and joint space
obliterates the normal lucent area between the top of the narrowing may be evident. The erosive changes are accom-
calcaneus and the adjacent Achilles tendon and projects panied by fluffy new bone formation. Wrist involvement
into the pre-Achilles fat pad. Subsequently, poorly defined is usually asymmetrical (Fig. 20–6).
calcaneal erosions appear on the posterior and postero-
superior aspects of the bone. The Achilles tendon is Manubriosternal Joint and Symphysis Pubis. Osseous ero-
frequently thickened. Posterior calcaneal enthesophytes sion and adjacent bony proliferation at the manubrioster-
at the site of attachment of the Achilles tendon to the nal articulation are not rare in Reiter’s disease. Similar
calcaneus are rare in Reiter’s syndrome. abnormalities occur at the symphysis pubis, and at this
location, apposing margins of the pubic bones may appear
Ankle and Tarsal Areas. In approximately 25% of patients, eroded and sclerotic.
erosive and proliferative changes are evident, with irregular
or fluffy exuberant new bone formation in response to Sacroiliac Joint. Sacroiliitis is common in Reiter’s syn-
erosion. Radiographic abnormalities about one or both drome (Fig. 20–7). Initially, abnormalities may be detect-
ankles can be recognized in 30% to 50% of patients, ed in only 5% to 10% of patients, but after several years,
including soft tissue swelling, linear or fluffy periostitis, sacroiliac joint alterations may affect 40% to 60%.
articular space loss, and, less frequently, marginal erosions. Bilateral symmetrical or asymmetrical changes are typical;
302 Section V Rheumatoid Arthritis and Related Diseases
A B C
Figure 20–5. Abnormalities of the hand. A, Soft tissue swelling (arrowheads), erosion, and bony
proliferation (arrow) are the radiographic findings. B, Note joint space narrowing and irregular excrescences,
or “whiskers” (arrows), at the margins of the distal interphalangeal joint. C, In another patient, intra-articular
bony ankylosis is evident.
Chapter 20 Reiter’s Syndrome 303
DIFFERENTIAL DIAGNOSIS
Other Seronegative Spondyloarthropathies
Although its general features resemble those of the other
two seronegative spondyloarthropathies (ankylosing
spondylitis and psoriatic arthritis), Reiter’s syndrome has
a sufficiently characteristic articular distribution to allow
accurate diagnosis. This syndrome is associated with an
asymmetrical arthritis of the lower extremity, sacroiliitis,
and, less commonly, spondylitis. Ankylosing spondylitis
has a similar axial skeletal appearance, but significant
peripheral articular changes are less frequent. Psoriatic
arthritis may lead to considerable alterations in the articu-
lations of both the appendicular and the axial skeleton.
In psoriatic arthritis, however, widespread involvement
of the upper extremity may be apparent. In all three spondy-
loarthropathies, the presence of soft tissue swelling, joint
space narrowing, erosions, and bony proliferation in syn-
Figure 20–9. Radionuclide abnormalities. Asymmetrical
sacroiliitis, greater on the right side, is evident with scinti- ovial articulations is typical. In Reiter’s syndrome, the
graphy (arrow). frequency of osteoporosis in the acute phase of the disease
appears to be greater than in psoriatic arthritis and anky-
losing spondylitis, and the frequency of intra-articular
bony ankylosis is less than in the other two diseases.
The sacroiliac and spinal changes of Reiter’s syndrome
are virtually identical to those of psoriatic arthritis,
although the prevalence and severity of these abnor-
malities and the tendency to involve the cervical spine are
greater in psoriatic arthritis. Symmetrical, asymmetrical,
or unilateral sacroiliac articular changes and broad
asymmetrical spinal outgrowths occur in both Reiter’s
syndrome and psoriatic arthritis. In classic ankylosing
spondylitis, symmetrical sacroiliac joint changes and
symmetrical slender bony outgrowths of the spine are
typical. Further, in ankylosing spondylitis, vertebral body
osteitis, apophyseal joint ankylosis, and intra-articular
osseous fusion of the sacroiliac joint are more frequent
than in Reiter’s syndrome or psoriatic arthritis. The
spondylitis and sacroiliitis of inflammatory bowel disease
are identical to those of classic ankylosing spondylitis.
Figure 20–10. Magnetic resonance (MR) imaging abnormal-
ities: sacroiliitis. Oblique, transverse, fat-suppressed T1-weighted
spin echo MR image obtained after intravenous gadolinium Rheumatoid Arthritis
administration reveals high signal intensity in the left sacrum The radiographic features of rheumatoid arthritis differ
and ilium, indicating sacroiliitis. (Courtesy of G. Sandrini De
considerably from those of Reiter’s syndrome. Rheuma-
Toni, MD, São Paulo, Brazil.)
toid arthritis is associated with bilateral and symmetrical
alterations. Osteoporosis, early joint space narrowing,
and marginal erosions are observed. Bony proliferation is
difficulty has been encountered in attempting to isolate a unusual, and the irregular, proliferative, erosive changes
specific infective agent in Reiter’s syndrome. of the spondyloarthropathies are not seen in rheumatoid
An alternative explanation for the association of arthritis. Widespread and severe sacroiliac and thora-
Reiter’s syndrome and bowel or genitourinary infection is columbar spinal changes are very uncommon in rheu-
that Reiter’s syndrome is related not to purulent arthritis matoid arthritis.
but to the reaction of a joint (or other target site) to
infection elsewhere in the body. The concept of reactive
arthritis is discussed in Chapters 21 and 53, with rheu- Septic Arthritis and Osteomyelitis
matic fever, initiated by a beta-hemolytic streptococcal The early localized abnormalities of Reiter’s syndrome
infection in the throat, being a classic example. may resemble the findings of osseous and articular infec-
The increased frequency (up to 96%) of the histo- tion. Soft tissue swelling, osteoporosis, bony and carti-
compatibility antigen HLA-B27 in this disorder is well laginous destruction, and periostitis are evident in both
recognized. Possession of this antigen may predispose Reiter’s syndrome and infectious disease. Eventually, the
patients to Reiter’s syndrome after exposure to an infec- polyarticular nature of Reiter’s syndrome allows its
tious agent. accurate differentiation from infectious disease.
Chapter 20 Reiter’s Syndrome 305
FURTHER READING McEwen C, Di Tata D, Lingg C, et al: Ankylosing spondylitis and
spondylitis accompanying ulcerative colitis, regional enteritis,
El-Khoury GY, Kathol MH, Brandser EA: Seronegative psoriasis and Reiter’s disease. Arthritis Rheum 14:291, 1971.
spondyloarthropathies. Radiol Clin North Am 34:343, 1996. Peterson CC Jr, Silbiger ML: Reiter’s syndrome and psoriatic
Fan PT, Yu DTY: Reiter’s syndrome. In Kelley WN, Harris ED arthritis: Their roentgen spectra and some interesting simi-
Jr, Ruddy S, et al (eds): Textbook of Rheumatology, 5th ed. larities. AJR Am J Roentgenol 101:860, 1967.
Philadelphia, WB Saunders, 1997, p 983. Resnick D, Feingold ML, Curd J, et al: Calcaneal abnormalities
Helliwell PS, Hickling P, Wright V: Do the radiological changes in articular disorders: Rheumatoid arthritis, ankylosing
of classic ankylosing spondylitis differ from the changes spondylitis, psoriatic arthritis and Reiter’s syndrome. Radio-
found in the spondylitis associated with inflammatory bowel logy 125:355, 1977.
disease, psoriasis, and reactive arthritis? Ann Rheum Dis Sholkoff SD, Glickman MG, Steinback HL: Roentgenology of
57:135, 1998. Reiter’s syndrome. Radiology 97:497, 1970.
Keat A: Reiter’s syndrome and reactive arthritis in perspective. Sundaram M, Patton JT: Paravertebral ossification in psoriasis
N Engl J Med 309:1606, 1983. and Reiter’s disease. Br J Radiol 48:628, 1975.
Kransdorf MJ, Wehrle PA, Moser RP Jr: Atlantoaxial sub- Weinberger HW, Ropes MW, Kulka JP, et al: Reiter’s syn-
luxation in Reiter’s syndrome: A report of three cases and drome: Clinical and pathological observations—a long term
review of the literature. Spine 13:12, 1988. study of 16 cases. Medicine 41:35, 1962.
Martel W, Braunstein EM, Borlaza G, et al: Radiologic features Weldon WV, Scalettar R: Roentgen changes in Reiter’s syn-
of Reiter’s disease. Radiology 132:1, 1979. drome. AJR Am J Roentgenol 86:344, 1961.
21
CHAPTER 21
Enteropathic Arthropathies ULCERATIVE COLITIS
General Abnormalities
+, present; r, rare.
range from 2% to 18%. Spondylitis in ulcerative colitis is tuberosities and iliac crests. In the spine, vertebral body
poorly correlated with activity of the bowel disease. Further, erosions with alterations in vertebral shape (squaring) are
spinal abnormalities may manifest before, at the same time seen. Eventually, a bamboo spine may be evident. In the
as, or after the onset of intestinal changes. The clinical fea- apophyseal joints, articular space narrowing, erosion,
tures of ankylosing spondylitis in patients with ulcerative sclerosis, and bony ankylosis are identical to changes in
colitis are identical to those of classic ankylosing spondyli- classic ankylosing spondylitis.
tis, although the male predilection may be less striking. The sacroiliitis and spondylitis of classic ankylosing
Laboratory evaluation reveals an increased frequency of spondylitis and ulcerative colitis (as well as Crohn’s disease)
the histocompatibility antigen HLA-B27, although not are readily differentiated from the sacroiliitis and spondyli-
as great as in classic ankylosing spondylitis. This antigen tis of psoriatic arthritis and Reiter’s syndrome. Bilateral,
is not commonly detected in patients with inflammatory symmetrical sacroiliac joint changes with eventual intra-
bowel disease. articular bony ankylosis are more common in classic anky-
On radiographic examination, the spinal and sacroiliac losing spondylitis and colitic disease than in psoriatic
joint abnormalities in ulcerative colitis are virtually indis- arthritis and Reiter’s syndrome. In the spine, vertical syn-
tinguishable from those in classic ankylosing spondylitis. desmophytes, significant apophyseal joint abnormalities,
Sacroiliac joint involvement is usually bilateral and sym- and vertebral squaring are also more typical of classic
metrical in distribution (Fig. 21–2). Osseous erosion and ankylosing spondylitis and colitic disorders.
sclerosis are evident, as well as joint space alterations.
Initially, the articular space may appear widened, although
Miscellaneous Abnormalities
joint space narrowing and bony ankylosis are common in
later stages of the disease. Additional pelvic abnormalities Clubbing of the fingers is a recognized complication of
include bone erosion and sclerosis about the symphysis ulcerative colitis. On rare occasion, hypertrophic
pubis and bone erosion and proliferation at the ischial osteoarthropathy leading to periosteal bone formation in
308 Section V Rheumatoid Arthritis and Related Diseases
CROHN’S DISEASE
General Abnormalities
Figure 21–2. Ulcerative colitis and ankylosing spondylitis:
sacroiliac joint abnormalities. Bilateral, symmetrical sacroiliac Crohn’s disease is a chronic and recurrent granulomatous
articular abnormalities are characterized by erosion and process of unknown cause that involves principally the ter-
sclerosis, predominantly in the ilium. minal ileum and proximal portion of the colon. Crohn’s
disease is associated with a variety of clinical findings,
including abdominal pain, tenderness, diarrhea, weight
the tibia, fibula, radius, and ulna may also be apparent loss, and fever. Complications include intestinal hemor-
(Fig. 21–3). rhage, obstruction, draining sinuses, and fistulas. Crohn’s
disease is most frequent in young adults, although patients
of all ages can be affected. Musculoskeletal manifestations
Differential Diagnosis
in Crohn’s disease can take several forms: peripheral joint
Radiographic features of peripheral joint arthritis in arthralgia and arthritis, sacroiliitis and spondylitis, and
patients with ulcerative colitis are not specific. Although miscellaneous abnormalities.
Chapter 21 Enteropathic Arthropathies 309
Peripheral Joint Arthralgia and Arthritis conversely, a high frequency of Crohn’s disease occurs in
The reported frequency of enteropathic arthritis in patients with ankylosing spondylitis. Men and women are
patients with Crohn’s disease varies from less than 1% to affected with equal frequency. Symptoms and signs may
22%. Peripheral joint abnormalities in Crohn’s disease antedate or follow the onset of bowel disease. Exacer-
are equally frequent in men and women. The pattern of bation of these findings does not appear to be related to
articular involvement is typically a mild, migratory syn- activity of the bowel disease, nor does treatment of the
ovitis of one or, less commonly, several joints that involves intestinal disorder influence the progress of the arthritis.
the lower extremity more frequently than the upper Serologic testing for the presence of HLA-B27 antigen is
extremity. The knee is the most common site of abnor- positive in approximately 50% of patients with Crohn’s
mality, followed by the ankle, shoulder, wrist, elbow, and disease and sacroiliitis or spondylitis.
small joints of the hands and feet. Arthritis, which may be The radiographic features of the spinal and sacroiliac
associated with erythema nodosum, can occur simulta- articular abnormalities in Crohn’s disease are identical to
neously with the onset of bowel disease or at any time those of classic ankylosing spondylitis. Bilateral sacroiliac
during its course. Infrequently, it may precede intestinal joint narrowing and erosion and sclerosis of the ilium and
alterations. The recrudescence of arthritis is commonly sacrum are evident (Figs. 21–4 and 21–5). Spinal involve-
associated with an exacerbation of intestinal disease. ment can include syndesmophytosis; vertebral erosion,
Radiographic abnormalities are nondistinctive. Soft sclerosis, and squaring; and apophyseal joint erosion,
tissue swelling and regional osteoporosis may be observed. sclerosis, and narrowing. Progressive alterations can lead
Permanent cartilaginous and osseous changes are present to a bamboo spine.
infrequently. The cause of the enteropathic arthritis accom-
panying Crohn’s disease is not known.
Miscellaneous Abnormalities
Digital clubbing has been detected in as many as 40% of
Sacroiliitis and Spondylitis patients with Crohn’s disease. Bilateral, symmetrical perios-
Sacroiliac joint and spinal changes develop in a significant titis, which is a manifestation of hypertrophic osteoarthropa-
number of patients with Crohn’s disease (3% to 16%); thy, is an extremely rare complication of Crohn’s disease.
A B
310 Section V Rheumatoid Arthritis and Related Diseases
Granulomatous and infectious processes of bone have been the onset of gastrointestinal findings, the pattern of arthritis
reported in association with Crohn’s disease. Although an generally remains unaltered, although occasionally, joint
abscess of the psoas muscle is an infrequent complication symptoms and signs may decrease in intensity. Articular
of Crohn’s disease, with a prevalence of approximately abnormalities are usually transient and last hours to days.
3% to 5%, some investigators regard Crohn’s disease as The findings generally subside and subsequently recur after
the leading cause of psoas abscesses. periods of remission of variable length. Residual joint defor-
mities are rare. Characteristically, the ankles, knees, shoulders,
and wrists are affected. Polyarthritis is more frequent than
Differential Diagnosis
monoarthritis. Clinical findings include mild to severe
The radiographic abnormalities in the peripheral joints of joint pain, swelling, warmth, and restrictive motion. Stiff-
patients with Crohn’s disease lack specificity. Soft tissue ness is usually not a prominent manifestation of the disease.
swelling and osteoporosis are observed, but permanent Radiographic examination of involved peripheral joints
cartilaginous and osseous changes are unusual. Thus, the may yield entirely normal results. Soft tissue swelling,
presence of joint space narrowing and erosions virtually osteoporosis, and joint space narrowing are occasionally
eliminates the diagnosis of enteropathic arthritis. evident (Fig. 21–6). These findings are most common in
The axial skeletal abnormalities in this disease cannot the metacarpophalangeal and metatarsophalangeal joints,
be differentiated from those of classic ankylosing spondyli- wrist, ankle, hip, and knee.
tis or the spondylitis of ulcerative colitis. The presence of
bilateral and symmetrical sacroiliac joint changes and syn-
desmophytosis of the spine is typical of these disorders.
WHIPPLE’S DISEASE
General Abnormalities
Whipple’s disease (intestinal lipodystrophy) is a rare pro-
gressive disorder that affects predominantly men in the
fourth and fifth decades of life and leads to fever, weight
loss, lymphadenopathy, peripheral edema, hypotension,
brown pigmentation of the skin, polyserositis, and poly-
arthritis. Pathologically, the major feature is the accu-
mulation of periodic acid-Schiff–positive inclusions in
macrophages of the lamina propria of the small intestine,
lymph nodes, and other tissues. The cause of the disease
appears to be related to a bacterial organism, inasmuch as
antibiotic therapy can effect a cure.
Musculoskeletal manifestations are an important
feature of the disease. These manifestations can be divided
into peripheral joint arthralgia and arthritis, sacroiliitis
and spondylitis, and miscellaneous abnormalities.
Figure 21–6. Whipple’s disease: peripheral joint abnormali-
Peripheral Joint Arthralgia and Arthritis ties. Observe the hip joint space narrowing and osseous cysts.
Similar changes were seen in the contralateral hip.
Acute migratory episodic arthralgia and arthritis are apparent (From Chevallier PL, Vallat JP, Luthier F, et al: Bilateral
in 60% to 90% of patients with Whipple’s disease. These (coxopathy revealing Whipple disease. Apropos of a case.
findings may antedate other changes of the disease. With Article in French.) Rev Rhum Mal Osteoartic 43:663, 1976.)
Chapter 21 Enteropathic Arthropathies 311
Sacroiliitis and Spondylitis Four possible mechanisms are postulated by which an
Sacroiliitis and spondylitis have been described in patients enteric infectious agent may initiate or perpetuate articular
with Whipple’s disease. The exact frequency of these inflammation: (1) altered bowel anatomy (e.g., the intes-
abnormalities is not known. In most cases, the spinal and tinal bypass syndrome), (2) autoimmunity as a result of
sacroiliac joint alterations resemble those of classic anky- molecular mimicry (e.g., postdysenteric reactive arthritis
losing spondylitis. Approximately one quarter to one third and ankylosing spondylitis), (3) altered bowel permeability
of patients with Whipple’s disease have been reported to (e.g., inflammatory bowel disorders, milk allergy, and
possess the HLA-B27 antigen. celiac disease), and (4) toxin-mediated synovitis (e.g.,
pseudomembranous enterocolitis). Recent investigations
support the third of these possible mechanisms: altered
Miscellaneous Abnormalities bowel permeability.
Subcutaneous nodules, particularly on extensor surfaces General clinical characteristics of reactive arthritis
of the extremities, are evident in some patients with joint include a symptom-free interval between the initiation of
symptoms. Additional manifestations include clubbing of infection and the rheumatic reaction; a self-limited clinical
the fingers, hypertrophic osteoarthropathy, reflex sympa- course, usually associated with the acute onset of a migra-
thetic dystrophy, and myalgias. tory polyarthritis and fever; a tendency in some patients
toward involvement of the heart; and a negative serologic
test for rheumatoid factor. Although the location of the
Differential Diagnosis inciting infection varies, three portals of entry are most
The peripheral joint abnormalities in Whipple’s disease common: the oronasopharynx (e.g., tonsillitis, dental
are entirely nonspecific on radiographic evaluation. In the infection), the urogenital tract (e.g., urethritis), and the
axial skeleton, sacroiliac joint and spinal changes, when intestinal tract.
present, are identical to those found in classic ankylosing Seronegative sterile arthritis may follow intestinal infec-
spondylitis and the spondylitis of ulcerative colitis and tions related to Salmonella, Shigella, Yersinia, or Campy-
Crohn’s disease. Although clinical features permit the lobacter organisms. The frequency of arthritis in patients
separation of these disorders, the radiographic changes after Salmonella or Shigella infection is 1% to 2%; that
do not allow an accurate differential diagnosis. occurring after Yersinia infection may be as high as 30%.
The various forms of sterile arthritis can be associated
with soft tissue swelling and joint effusions, but other
INTESTINAL INFECTIONS AND REACTIVE radiographic features are generally lacking. Infrequently,
ARTHRITIS erosive bone changes are seen. After bowel disease caused
A variety of infectious agents can initiate or trigger a syn- by Yersinia, sacroiliitis and spondylitis have been noted.
ovial reaction at one or more sites distant to the area of In this regard, it is significant that HLA-B27 antigen has
infection. The phenomenon, which is unrelated to direct been detected in approximately 90% of patients in whom
contamination of the joint, is referred to as reactive sterile arthritis develops after bowel infection with Salmonella,
arthritis (Table 21–2). The diagnostic criteria for reactive Shigella, or Yersinia organisms. A true septic arthritis is a
arthritis are not agreed on, although emphasis has been definite and serious complication of Salmonella infection,
placed on evidence of a preceding infection and asymmet- particularly in children. Yersinia enterocolitica infection has
rical articular disease of the lower limbs. Certain genetic also been associated with septic arthritis, as well as
factors, such as presence of the HLA-B27 antigen, appear osteomyelitis and psoas muscle abscess.
to impart susceptibility to reactive arthritis in some persons.
ARTHROPATHY AFTER INTESTINAL BYPASS
SURGERY
Intestinal bypass surgery has been performed for many
years for the treatment of intractable obesity. Rheumato-
TABLE 21–2 logic manifestations in the postoperative period predomi-
Mechanisms of Infection-Induced arthritis nate in women and include polymyalgia, polyarthralgia,
acute or subacute arthritis, and tenosynovitis. The preva-
Mechanism Example lence of these manifestations in patients undergoing such
surgery has decreased in recent years as a result of improved
Direct penetration and subsequent Bacterial arthritis
multiplication of viable organisms technology and professional awareness. The symptoms
and signs are usually transient, although they occasionally
“Reactive” arthritis as a response to a Rheumatic fever
distant infection persist. They generally develop within 2 years after sur-
gical intervention and resolve within 1 to 2 years. Sym-
Immune-mediated response to an Hepatitis
antigen in the joint or blood metrical polyarthritis is the rule. Articular involvement is
most frequent in the knees, ankles, fingers, and wrists.
Direct toxin released by the infectious None known
agent Radiographic evaluation demonstrates soft tissue swelling
and osteoporosis. On rare occasion, osseous erosions are
evident. Sacroiliitis and spondylitis have also been observed
Modified from Goldenberg DL: “Postinfectious” arthritis: New look at an old
concept with particular attention to disseminated gonococcal infection. Am J in patients undergoing jejunocolic-type bypass proce-
Med 74:625, 1983. dures (Fig. 21–7). The cause and pathogenesis of the
312 Section V Rheumatoid Arthritis and Related Diseases
Figure 21–7. Intestinal bypass surgery: peripheral joint disease and sacroiliitis.
Fourteen years after a jejunocolic bypass, peripheral joint disease is noted. It is
characterized by osteopenia; soft tissue swelling, especially about the proximal
interphalangeal and metacarpophalangeal joints; and joint space narrowing in the
wrist, particularly the radiocarpal and ulnocarpal regions (A). Bilateral sacroiliitis is
also evident (B).
arthropathy after intestinal bypass procedures have not to be fundamental to its pathogenesis. Clinical findings
been clearly delineated. Apparently, the type of bowel sur- include the insidious onset of jaundice, pruritus,
gery influences the pattern and distribution of arthritis. hepatomegaly, and abnormal liver function. The diag-
Additional radiographic findings seen after intestinal bypass nosis is confirmed by the presence of antimitochon-
surgery include gout, osteomalacia, and osteoporosis. drial antibodies and characteristic findings on liver
biopsy.
Musculoskeletal complaints can relate to osteomalacia
LAËNNEC’S CIRRHOSIS
and osteoporosis, arthralgia or arthritis, hypertrophic
Inflammatory polyarthritis can occur in patients with osteoarthropathy, or the coexistence of a connective tissue
Laënnec’s cirrhosis during the recovery period, when liver disease such as scleroderma or Sjögren’s syndrome. The
function tests are showing improvement. Most commonly osteomalacia and osteoporosis in this disorder are proba-
the shoulders, elbows, and knees are affected. Clinical bly due to a combination of steatorrhea, cholestasis, and
findings include pain, swelling, tenderness, and limitation hepatic dysfunction. Hypercholesterolemia can lead to
of motion. Radiographs reveal osteoporosis and a possible xanthoma formation in soft tissues and in subperiosteal
proclivity toward the development of calcific periarthritis. and intramedullary sites. Associated radiographic findings
The condition appears to be self-limited, with improve- include nodular soft tissue masses, subperiosteal erosion
ment usually occurring within 1 year. The pathogenesis of bone, and cystic lesions. Of particular interest is the
of these arthritic alterations is unknown. appearance of erosive arthritis of the hands and wrists in
patients with primary biliary cirrhosis Erosions are well
defined, of varying size, and marginal in location, and they
PRIMARY BILIARY CIRRHOSIS
are distributed predominantly in an asymmetrical fashion
Primary biliary cirrhosis is a rare disorder that affects in the distal interphalangeal and proximal interphalangeal
women almost exclusively. Immune mechanisms appear joints (Fig. 21–8).
Chapter 21 Enteropathic Arthropathies 313
or it can represent an isolated phenomenon. Osteolytic
lesions with moth-eaten bone destruction and periostitis
of the tubular bones of the extremities resemble the
findings in osteomyelitis or osteonecrosis (Fig. 21–9).
These changes occur in the long bones of the extremities,
in the small bones of the hands and feet, or in both loca-
tions. In the bones of the hands and feet, cystic defects and
a coarsened trabecular pattern may be apparent, and the
epiphyses may be unaffected. Magnetic resonance (MR)
imaging is sensitive to the early changes of intraosseous
fat necrosis and shows multiple foci of abnormal signal
(Fig. 21–10).
Although the exact pathogenesis of the articular and
osseous findings associated with pancreatic disorders has
not been delineated accurately, it appears probable that
widespread fat necrosis is responsible for these abnormali-
ties. Obstruction of pancreatic ducts by edema, calculi, or
tumor or hormonal hypersecretion by acinar cell carcino-
mas and functioning metastases can lead to the release of
Figure 21–8. Primary biliary cirrhosis: peripheral joint abnor- excess circulating lipase into the bloodstream, which results
malities. Note the small marginal erosions in the proximal in autodigestion of fat deposits at distant sites.
interphalangeal and distal interphalangeal joints (arrows).
(From O’Connell DJ, Marx WJ: Hand changes in primary
biliary cirrhosis. Radiology 129:31, 1978.) Osteonecrosis
Osteonecrosis is a recognized manifestation of pancreatic
disease. This complication is most frequently associated
with chronic or inactive pancreatitis. Abnormalities of the
VIRAL HEPATITIS
Articular manifestations can occur during the course of
hepatitis B (serum hepatitis) and, less commonly, hepa-
titis A (infectious hepatitis) and hepatitis C. A symmet-
rical, polyarticular pattern of joint disease is observed and
most commonly involves the hands, wrists, elbows, knees,
and ankles. Clinical findings include pain and stiffness,
which may persist for a few days to more than 1 month
and may decrease as jaundice develops. Joint destruction
and deformity are not seen.
PANCREATIC DISEASE
Fat Necrosis
Pancreatic disorders can be complicated by fat necrosis at
multiple distant sites, with subsequent subcutaneous nodular
skin lesions, polyarthritis, and medullary fat necrosis. These
manifestations appear most frequently in older men in
association with carcinoma of the pancreas, although they
can also occur with acute pancreatitis caused by either
abdominal trauma or alcohol abuse, pancreatic pseudo-
cysts, and pancreatic duct calculi. In many patients, joint
pain and nodules precede abdominal pain. Articular abnor-
malities are characterized by a symmetrical or asymmet-
rical polyarthritis, which can be associated with pain,
swelling, tenderness, warmth, and effusion. A predilection
for the ankles, elbows, knees, wrists, and small joints of
the hands and feet is seen.
Radiographic findings related to joint disease are absent Figure 21–9. Pancreatic disease: fat necrosis. Radiographic
or minimal; osteoporosis and soft tissue swelling may be abnormalities include lytic defects of the metacarpals, pha-
seen, but joint space narrowing and osseous erosion are langes, and metatarsals, with associated periostitis. Soft tissue
reported infrequently. Bone involvement can occur simul- swelling is also evident. (Courtesy of A. Brower, MD, Norfolk,
taneously with subcutaneous nodules and polyarthritis, Va.)
314 Section V Rheumatoid Arthritis and Related Diseases
A B
316
Chapter 22 Periodic, Relapsing, and Recurrent Disorders 317
TABLE 22-1
Sacroiliac Joint Abnormalities
Distribution
Bilateral Bilateral
Disorder Symmetrical Asymmetrical Unilateral
Familial Mediterranean fever x x* x
Relapsing polychondritis x x* x
Behçet’s syndrome† x x* x
ing and osseous erosion can simulate the findings of tract involvement, nasal chondritis, and ocular involve-
arthritides associated with synovial inflammation, such as ment are evident at the outset of the disease. Auricular
rheumatoid arthritis and septic arthritis. The sacroiliitis chondritis leads to painful erythematous swelling of the
in familial Mediterranean fever simulates that of anky- cartilaginous portion of one or both external ears, lasting
losing spondylitis and the other seronegative spondylo- from 5 to 10 days. Arthralgia and arthritis occur in as
arthropathies. many as 80% of patients and generally affect more than
one joint, including the hips, knees, manubriosternal and
sternoclavicular articulations, costochondral junctions,
RELAPSING POLYCHONDRITIS and small and large joints of the upper extremity.
Respiratory tract involvement is a potentially serious
Clinical Abnormalities
manifestation of the disease that may require a tra-
Relapsing polychondritis is an uncommon disorder cheostomy. Nasal chondritis may be sudden in onset and
characterized by episodic inflammation of cartilaginous painful, and chronic involvement can lead to a charac-
tissue and special sense organs; abnormalities are espe- teristic saddle nose deformity. Additional manifestations
cially prominent in the external ear, nose, trachea, larynx, include back pain as a result of spinal alterations, chest
sclera, ribs, and articular cartilage. Relapsing polychon- pain related to costochondritis, hearing loss attributable
dritis appears in all age groups, with a maximal frequency to obstruction of the external auditory meatus, fever,
in the fourth decade of life. Men and women are affected anorexia and weight loss, and cardiovascular abnormal-
in equal numbers. The initial clinical findings are usually ities. Clinical manifestations, in their order of frequency,
auricular chondritis and arthritis; less typically, respiratory include auricular chondritis (89%), polyarthritis (81%),
nasal chondritis (72%), ocular inflammation (65%), respi-
ratory tract chondritis (56%), audiovestibular damage
(46%), cardiovascular involvement (24%), and cutaneous
lesions (17%).
Radiographic Abnormalities
In most cases, the radiographic features of joint involve-
ment in relapsing polychondritis are not striking,
although extra-articular findings may be present, such as
tracheal narrowing or stenosis, calcification of the auric-
ular cartilage, and aortic alterations after repeated attacks.
Periarticular osteoporosis may or may not be evident.
Typically, a nonerosive, nondeforming arthropathy
appears. The presence of joint space narrowing in the
absence of bone erosions is reported to be characteristic
of relapsing polychondritis (Fig. 22–3).
Sacroiliitis may be evident in some patients with
relapsing polychondritis, although the prevalence is
Figure 22–2. Familial Mediterranean fever: sacroiliitis. probably not high (see Table 22–1). Unilateral or
Bilateral, asymmetrical sacroiliac joint disease is characterized
by osseous erosions and reactive sclerosis, predominantly in the bilateral abnormalities characterized by the presence of
ilium. joint space loss, erosion, and eburnation and by the
(From Brodey PA, Wolff SM: Radiographic changes in the absence of spinal alterations predominate (Fig. 22–4).
sacroiliac joints in familial Mediterranean fever. Radiology HLA-B27 antigen does not appear to be associated with
114:331, 1975.) the arthropathy of relapsing polychondritis.
318 Section V Rheumatoid Arthritis and Related Diseases
VI
Connective Tissue Disease
CHAPTER 23
Systemic Lupus Erythematosus lupus erythematosus. Additional clinical abnormalities
may involve the gastrointestinal system (peritonitis, pan-
creatitis, perihepatitis, intestinal arteritis), the reticulo-
endothelial system (splenomegaly, lymphadenopathy), and
SUMMARY OF KEY FEATURES the peripheral vasculature (gangrene, thrombophlebitis).
Musculoskeletal abnormalities represent a significant Infections may develop in patients with systemic lupus
part of the clinical and radiographic picture of systemic erythematosus, perhaps related to decreased complement
lupus erythematosus. These abnormalities include levels, impairment of delayed hypersensitivity, and
myositis, symmetrical polyarthritis, deforming defective phagocytosis.
nonerosive arthropathy, spontaneous rupture of tendons,
osteonecrosis, soft tissue calcification, osteomyelitis and MUSCULOSKELETAL ABNORMALITIES
septic arthritis, and terminal phalangeal sclerosis and
erosion. Many of these abnormalities resemble the Characteristic and significant musculoskeletal abnormal-
changes that occur in other collagen vascular disorders ities are encountered in patients with systemic lupus
and rheumatoid arthritis. In addition, findings of erythematosus (Table 23–1). These abnormalities may
systemic lupus erythematosus may be combined with include myositis, symmetrical polyarthritis, deforming
those of other collagen vascular diseases in various nonerosive arthropathy, subchondral cysts, spontaneous
overlap syndromes and in mixed connective tissue tendon weakening and rupture, osteonecrosis, soft tissue
disease, and they may also be evident in the calcification, osteomyelitis and septic arthritis, and other
antiphospholipid syndrome. abnormalities.
Myositis
Muscle involvement has been observed in 30% to 50%
INTRODUCTION of persons with systemic lupus erythematosus. Possible
Systemic lupus erythematosus is a relatively common causes include pain referred to muscles from adjacent
connective tissue disorder characterized by significant involved joints, corticosteroid-induced myopathy, and
immunologic abnormalities and involvement of multiple myositis. Myositis, which has been reported in 4% of
organ systems. The musculoskeletal system is frequently patients with systemic lupus erythematosus, may be
affected, and a number of clinical, pathologic, and radio- associated with diffuse muscular tenderness and weak-
graphic findings have been noted. ness, with or without elevated serum levels of muscle
enzymes.
GENERAL CLINICAL ABNORMALITIES
Systemic lupus erythematosus is much more common in
women than in men and is more common in blacks than
in whites. Its familial nature is also well recognized.
Although disease onset can occur at any age, it is most
frequent in women during the childbearing years. The TABLE 23–1
disease is rare in persons older than 45 years. Symptoms
and signs are variable and depend on the distribution and Musculoskeletal Manifestations of Systemic Lupus
extent of systemic alterations. Initial clinical manifesta- Erythematosus
tions most frequently include constitutional symptoms
and signs (malaise, weakness, fever, anorexia, and weight Myositis
loss) and articular (polyarthritis) and cutaneous (skin rash) Polyarthritis
findings. Subsequently, major clinical abnormalities may Deforming nonerosive arthropathy
Subchondral cysts
involve the musculoskeletal, cutaneous, neurologic, renal, Tendon weakening and rupture
pulmonary, and cardiac systems. Osteonecrosis
Skin manifestations are variable and include a typical Soft tissue calcification
butterfly eruption on the face; erythematous rashes on Osteomyelitis and septic arthritis
the eyelids, forehead, and neck; subungual erythema; Acrosclerosis
urticaria; vasculitis with livedo reticularis; and alopecia. Tuft resorption
Skin nodules resembling those of rheumatoid arthritis Insufficiency fracture
may be observed in 5% to 10% of patients with systemic
321
322 Section VI Connective Tissue Disease
Osteonecrosis
Osteonecrosis is observed in approximately 5% of patients
with systemic lupus erythematosus, with some estimates
as high as 40%. Although the femoral head is the most
common site of abnormality, involvement of other and
multiple sites has been confirmed; osteonecrosis is not
uncommon in the humeral head, femoral condyle, tibial
plateau, and talus. The pathogenesis of osteonecrosis in
this disease has not been fully delineated. Although it is
Figure 23–3. Lupus arthropathy: severe manifestations.
Findings include radial deviation at the radiocarpal joint, ulnar
deviation and subluxation at multiple metacarpophalangeal
joints, and boutonnière and swan-neck deformities. Observe
the joint space narrowing in the radiocarpal compartment
(arrow) and hook erosions of the metacarpal heads (arrowheads).
Subchondral Cysts
Osteoporosis and cyst formation within subchondral Figure 23–4. Tendon weakening and rupture. Sagittal
bone can occur in systemic lupus erythematosus. The intermediate-weighted (TR/TE, 1600/30) spin echo MR images
most common sites of involvement are the bones in the show complete rupture of the patellar tendon, surrounding
hand, wrist, and foot, particularly the carpus and edema, and an elevated patella.
324 Section VI Connective Tissue Disease
usually attributable to steroid administration, the develop- Osteomyelitis and Septic Arthritis
ment of osteonecrosis may be multifactorial, related to An unusually high frequency of bacterial and mycotic
both the basic disease process and the previous adminis- infections is found in patients with systemic lupus erythe-
tration of steroids. matosus. Two major factors contribute to this suscep-
The radiographic and pathologic features of osteonecro- tibility to infection: steroid administration and renal
sis in patients with systemic lupus erythematosus are disease. The respiratory tract, urinary tract, skin, and soft
identical to those in patients who do not have this disease tissues are the sites commonly infected. The occurrence
(see Chapter 67). The occurrence of bone necrosis in of bone and joint infection in systemic lupus erythe-
unusual sites, such as the metacarpal heads, carpal bones, matosus is less frequent. Monoarticular infection of the
tarsus, and metatarsal heads, however, should suggest knee, hip, and ankle appears to be most typical. Impli-
systemic lupus erythematosus as a potential diagnosis cated organisms include Neisseria gonorrhoeae, Neisseria
(Figs. 23–5 and 23–6). Detection of osteonecrosis at any meningitidis, Staphylococcus aureus, gram-negative bacilli,
site is accomplished with more sensitivity and specificity atypical mycobacteria, Mycobacterium tuberculosis, and
with magnetic resonance (MR) imaging than with routine Salmonella species. Radiographic findings of a large or
radiography. increasing joint effusion and progressive cartilaginous
and osseous destruction should raise the possibility of
articular infection.
Soft Tissue Calcification
Soft tissue calcification is occasionally observed in systemic
lupus erythematosus. Several patterns of calcification are Sacroiliitis
reported: diffuse linear, streaky, or nodular calcification Rarely in patients with systemic lupus erythematosus,
in the subcutaneous and deeper tissues, particularly in the imaging studies reveal alterations about the sacroiliac
lower extremities; focal or localized plaquelike calcifica- joints. Radiographic changes include joint space narrow-
tion; periarticular calcification; and arterial calcification. ing, bone erosion, and reactive sclerosis in a unilateral or
Diffuse or focal calcific deposits may be associated with bilateral distribution. These changes are similar to those
adjacent skin ulceration, inflammation, and necrosis. seen in osteoarthritis, so caution is required in assuming
Juxta-articular calcifications appear as single or multiple that they are signs of sacroiliitis.
deposits of varying size, with or without adjacent joint
disease (Fig. 23–7).
Miscellaneous Abnormalities
Osteopenia, presumably representing osteoporosis, is
observed in some patients with systemic lupus erythe-
matosus. Potential causes include debilitation and corti-
A B
Figure 23–5. Osteonecrosis: multiple sites. In a young female patient with systemic lupus erythematosus,
osteonecrosis developed at multiple sites after corticosteroid therapy. A, In the shoulder, typical changes are
seen, including fragmentation of the humeral head with depression of the subchondral bone (arrowhead).
B, Changes in this wrist related to osteonecrosis include fragmentation and collapse of the proximal portion
of the scaphoid (arrowhead) and intra-articular debris. (B, Courtesy of I. Dwosh, MD, Toronto, Ontario,
Canada.)
Chapter 23 Systemic Lupus Erythematosus 325
A B
Figure 23–6. Osteonecrosis: ankle and foot. This young man with systemic lupus erythematosus developed
vasculitis, which necessitated amputation of the leg. He had been treated with corticosteroids. A, Lateral
radiograph reveals evidence of ischemic necrosis of bone, manifested as serpentine calcification in the tibia
and calcaneus. Increased radiodensity of the proximal and distal articular surfaces of the talus, navicular, and
cuboid is evident. B, Radiograph of a sagittal section of the hindfoot documents regions of bone infarction
in the talus, calcaneus, and cuboid (arrows) that are associated with peripheral calcification.
A B
326 Section VI Connective Tissue Disease
DIFFERENTIAL DIAGNOSIS
The symmetrical polyarthritis associated with systemic
lupus erythematosus produces nonspecific radiographic
findings, including soft tissue swelling and osteoporosis.
The deforming nonerosive arthropathy of the hands and
wrists (and, less commonly, the feet) in systemic lupus
erythematosus is similar to Jaccoud’s arthropathy, which
usually follows rheumatic fever (Table 23–2). In the classic
Figure 23–8. Insufficiency fractures. In this 48-year-old descriptions of Jaccoud’s arthropathy, involvement of the
woman with systemic lupus erythematosus, insufficiency
ulnar digits (fourth and fifth fingers) is stressed, although
fractures occurred in both femora. A coronal T1-weighted
(TR/TE, 500/20) spin echo MR image reveals these fractures as a more extensive distribution may be encountered; in the
linear areas of low signal intensity (arrows). More diffuse arthropathy of systemic lupus erythematosus, often all
regions of decreased signal intensity about the fractures the digits, including the thumb, are affected. A deform-
represent foci of bone marrow edema. (Courtesy of A. Motta, ing arthropathy without significant erosive abnormality
MD, Cleveland, Ohio.) may also be seen in such rare disorders as Ehlers-Danlos
Chapter 23 Systemic Lupus Erythematosus 327
TABLE 23–2
Differential Diagnosis of Radiographic Abnormalities in the Hand
A B C
Figure 24–1. Bone and soft tissue abnormalities: digits of the hand. A, Observe the atrophy of the soft
tissue (arrow) and hyperostosis of the phalangeal tuft (arrowhead). B, In this digit, findings include resorption
of the tuft (arrowhead) and adjacent calcification. C, Soft tissue swelling, tuft resorption, and calcification are
seen. Note the deformity of the nail.
330 Section VI Connective Tissue Disease
Articular Involvement
In addition to the “articular” abnormalities that result from
primary osseous resorption in scleroderma, several other
articular manifestations occur (Table 24–2):
Figure 24–2. Bone and soft tissue abnormalities: mandible.
Observe the exaggerated radiolucent area between the teeth Interphalangeal Joints. Alterations at the distal interpha-
and the mandible (arrows), corresponding to the location of a langeal joints are usually confined to regional or periar-
thickened periodontal membrane. ticular osteoporosis and swelling and thickening of
adjacent soft tissue, without evidence of joint space
narrowing or osseous erosion. Occasionally, however, joint
manifestations in scleroderma closely resemble those of
20% to 33%. The enlarged membrane creates an exag- rheumatoid arthritis, with osteoporosis, joint space
gerated radiolucent area between the tooth and the narrowing, and osseous erosion in typical “target” areas.
mandibular bone that is best detected on dental films. In some patients with scleroderma, alterations occur at
the distal interphalangeal joints, articulations that are not
Ribs. Symmetrically distributed erosions of the superior commonly involved in rheumatoid arthritis (Fig. 24–6).
aspect of multiple ribs are seen (Fig. 24–3). The changes
predominate along the posterior aspects of the third to First Carpometacarpal Joints. Selective involvement of the
sixth ribs and may occur in the absence of bone resorption first carpometacarpal joint of the wrist may be apparent
at other sites. Evidence suggests that the rib resorption in in scleroderma. Distinctive bilateral resorption of the
scleroderma is related to intercostal muscle atrophy, with trapezium and adjacent metacarpal bone is observed, with
resultant loss of mechanical stress on the cortical bone at varying degrees of radial subluxation of the metacarpal
the muscle insertions, leading to osseous resorption. base. Intra-articular calcification may be seen (Fig. 24–7).
Preferential involvement of the first carpometacarpal joint
Spine. Paraspinal calcification has been emphasized as an is the distinctive feature of this arthropathy. Its patho-
additional manifestation of scleroderma (Fig. 24–4). Such genesis is unknown, but a similar arthropathy may be
TABLE 24-2
Figure 24–4. Bone and soft tissue abnormalities: cervical Sites of Erosive Articular Disease in Scleroderma
spine. Lateral radiograph shows calcific masses about the
apophyseal joints (arrows) between the third and sixth cervical Metacarpophalangeal joints
levels. (Courtesy of W. Peck, MD, Orange, Calif.)
Proximal and distal interphalangeal joints
First carpometacarpal joint
apparent in other disorders that alter muscle and tendon Inferior radioulnar joint
balance, such as systemic lupus erythematosus, Jaccoud’s Metatarsophalangeal joints
arthropathy, dermatomyositis, and Ehlers-Danlos
syndrome.
Other Sites and Manifestations. In addition to soft tissue scleroderma (Fig. 24–8). Radiographs reveal cloudlike
and periarticular calcific deposition, intra-articular (free radiodense regions conforming to a portion of the joint
or intrasynovial) calcification may become evident in or the entire articulation (see Fig. 24–7). Intra-articular
calcification is most frequent in the elbow, the inferior
radioulnar and first carpometacarpal joints of the wrist,
Vascular Involvement
Arteriography confirms the presence of vascular abnormal-
ities in patients with Raynaud’s phenomenon. Vasospasm,
narrowing, and obstructive lesions of the digital arteries
are observed, although the extent and severity of the
occlusive lesions do not always correlate with the clinical
severity of digital ischemia, the duration of the disease,
or the presence of Raynaud’s phenomenon. Additional
arteriographic findings include incomplete, poorly formed,
or absent palmar arterial arches and ulnar artery.
PATHOLOGIC ABNORMALITIES
Figure 24–7. Articular involvement: wrist. Selective involve- The pathologic changes in the skin of patients with
ment of the first carpometacarpal joint in scleroderma. Observe scleroderma consist of a low-grade inflammatory reac-
the scalloped erosions of the trapezium and base of the metacarpal tion characterized by perivascular cellular infiltration.
(open arrows), radial and proximal subluxation of the metacarpal Subsequently, a progressive increase in compact collagen
base (solid arrow), and intra-articular calcification (arrowhead). fibers in the dermis, thinning of the overlying epidermis,
(From Resnick D, Greenway G, Vint VC, et al: Selective
involvement of the first carpometacarpal joint in scleroderma.
and atrophy of dermal appendages are evident, as well as
AJR Am J Roentgenol 131:283, 1978.) thickening of the intima, medial fibrosis, and thrombotic
occlusion of arterioles. Abnormalities of the synovial
membrane consist of inflammatory changes characterized
by hyperemia, cellular infiltration, vascular sclerosis, and
Figure 24–8. Sites of calcification in scleroderma. Deposits Figure 24–9. Articular involvement: juxta-articular calcifica-
may occur in the soft tissues (1), tendons and tendon sheaths tion. Intra-articular calcification is particularly evident at the
(2), and capsule (3), as well as within the joint (4). first carpometacarpal and inferior radioulnar joints (arrows).
Chapter 24 Scleroderma (Progressive Systemic Sclerosis) 333
matic disorders occurring after breast augmentation with
silicone, a true association has not been firmly estab-
lished. The observed disorders often lack specificity,
although scleroderma-like clinical manifestations have
been noted in 10% to 25% of cases. At this time, a proven
relationship between silicone implants and rheumatic
disease does not exist.
Eosinophilic Fasciitis
Eosinophilic fasciitis, also known as Shulman’s syndrome,
is seen predominantly in adults. It is associated with
inflammation and induration of the skin and subcutaneous
tissues of the hands, forearms, feet, and legs, usually after
an episode of physical exertion. Polyarthralgia, polyarthri-
tis, muscle atrophy, and carpal tunnel syndrome have
been observed. Arthritis is common in the joints of the
hands and wrists and in the knees, and clinical findings
resemble those of rheumatoid arthritis. Joint contrac-
tures, related to induration and sclerosis of subcutaneous
tissue, are seen especially in the elbows, wrists, ankles,
knees, and hands. The disorder is associated with
peripheral blood eosinophilia, especially during the early
stages; hypergammaglobulinemia (IgG); and an elevated
erythrocyte sedimentation rate. Raynaud’s phenomenon
and visceral manifestations of progressive systemic
sclerosis are conspicuously absent. Improvement in
clinical and laboratory parameters follows the systemic
administration of corticosteroids.
Figure 24–10. Articular involvement: tendon and bursal calci- Because the pathologic alterations involve the lower
fication. Striking example of diffuse calcification about the subcutaneous tissues and the fascia, a deep wedge biopsy
Achilles tendon (arrows).
that extends through these tissues, as well as through
muscle, is required for diagnosis. Radiographic abnor-
malities are generally confined to osteopenia, although
surface fibrin deposition. Subsequently, intense fibrosis additional findings may include bone erosions and
of the superficial or all layers of the synovium becomes periostitis. Magnetic resonance (MR) imaging has been
evident. used to study the soft tissue abnormalities of eosinophilic
fasciitis. Findings include thickening of the fascia and
OTHER SYNDROMES AND CONDITIONS increased signal intensity in the fascia, subcutaneous tissue,
ASSOCIATED WITH SCLERODERMA and superficial muscle fibers on fluid-sensitive sequences,
as well as contrast enhancement (Fig. 24–11). Increased
Chemically Induced Scleroderma-Like attenuation values of the superficial and deep fasciae have
Conditions been evident with computed tomography.
Certain chemicals, including vinyl chloride, pentazocine, The cause of eosinophilic fasciitis is unknown. It most
and bleomycin, can induce cutaneous abnormalities resembles scleroderma, and some investigators believe
simulating those of scleroderma. Raynaud’s phenomenon that it may not be a separate disorder at all; however, it is
and pulmonary fibrosis, along with a distinctive pattern generally self-limited and without systemic manifesta-
of acro-osteolysis, are evident in workers who clean tions. Eosinophilia, raised serum IgG levels, and the
reactor vessels and are exposed to the polymerizing agent identification of immunoglobulin in the inflamed fascia
vinyl chloride (see Chapter 83). Scleroderma-like changes suggest that a humoral or cellular immune mechanism is
are also observed with other chemical agents, including involved in eosinophilic fasciitis.
solvents, paraffin and silicone implants, and cocaine. A
toxic epidemic syndrome (toxic oil syndrome) has been
Eosinophilia-Myalgia Syndrome
associated with the consumption of adulterated rapeseed
oil, marketed illegally and sold as a cheap cooking oil. The eosinophilia-myalgia syndrome has been related to
ingestion of the amino acid L-tryptophan and bears a
close resemblance to eosinophilic fasciitis. Initial clinical
Rheumatic Diseases Associated with abnormalities include fatigue, fever, and diffuse myalgia
Silicone Breast Implants of the proximal muscles. The disease continues to unfold
The precise relationship between silicone breast implants over a period of several weeks or months, and eventually,
and rheumatic disease is controversial. Despite numerous an impressive list of organs may be involved. Arthralgia
reports (some better documented than others) of rheu- (10% to 50% of cases) or, less commonly, arthritis (10%
334 Section VI Connective Tissue Disease
A B
to 20% of cases) is seen, usually involving the large joints. as antigenically foreign, and the host must be unable to
Progressive contracture of the knees and elbows may reject the graft effectively.
develop, and limited mobility of the ankles and wrists has Graft-versus-host disease occurs in either an acute or
been evident in some cases. a chronic form, the former appearing before 100 days after
The natural history of this syndrome is variable. the transplant and the latter generally after 100 days. It is
Corticosteroid therapy is not effective in most patients, the chronic form of graft-versus-host disease that resem-
and the mortality rate varies from 2% to 6%. The rela- bles a collagen vascular disease, specifically, scleroderma.
tionship of the eosinophilia-myalgia syndrome to Chronic graft-versus-host disease develops in 10% to
eosinophilic fasciitis has been the subject of speculation; 30% of patients undergoing bone marrow transplanta-
evidence suggests that the two diseases are not identical tion. In untreated cases, tightly bound skin, contractures,
and that the eosinophilia-myalgia syndrome is a more and tissue wasting are observed. Laboratory aberrations
severe disorder. include anemia, leukopenia, thrombocytopenia, eosinophilia,
elevated serum levels of immunoglobulins, and the pres-
ence of circulating autoantibodies.
Graft-versus-Host Disease
Graft-versus-host disease occurs when immunologically
competent cells engrafted onto a foreign host attack the DIFFERENTIAL DIAGNOSIS
tissues of that host. In current practice, this disease usually
Bony Abnormalities
appears after allogeneic bone marrow transplantation.
The specific prerequisites for identifying graft-versus- Generalized resorption of the terminal phalanges of the
host disease are the following: the graft must contain hand, foot, or both in scleroderma is characterized by
immunocompetent cells, the host must be sufficiently “penciling” of the tuft (Fig. 24–12). A similar finding can
genetically different from the graft for it to be perceived be seen in other disorders, such as Raynaud’s disease
Chapter 24 Scleroderma (Progressive Systemic Sclerosis) 335
Tuft resorption in scleroderma should not be confused
with congenital disorders associated with hypoplasia of
the terminal phalanges. In addition, the type of bone
resorption in scleroderma is usually easily differentiated
from disorders characterized by bandlike resorption of
the distal phalangeal shafts (polyvinyl chloride–associated
acro-osteolysis, familial acro-osteolysis, and hyperpara-
thyroidism) and from those characterized by intraosseous
or eccentric destruction of the terminal phalanx (inclu-
sion cyst, glomus tumor, enchondroma, osteomyelitis, and
skeletal metastasis). The combination of tuft resorption
and erosive arthritis of the distal interphalangeal joints
may be apparent in scleroderma as well as in hyper-
parathyroidism, psoriatic arthritis, thermal injuries, and
Figure 24–12. Phalangeal resorption: differential diagnosis. multicentric reticulohistiocytosis.
The normal situation is depicted in diagram 1. Resorption of
the tuft (2) can be seen in scleroderma, other collagen vascular
disorders, thermal injuries, hyperparathyroidism, psoriatic arthri-
Soft Tissue Abnormalities
tis, and epidermolysis bullosa. Bandlike resorption of the terminal Atrophy of the distal phalangeal soft tissue is a finding
phalanx (3) is seen in familial and occupational acro-osteolysis, in scleroderma that can be observed in other collagen
collagen vascular disorders, and hyperparathyroidism. Erosions vascular diseases, thermal injuries, vascular disorders,
about the distal interphalangeal joint (4) can be noted in psori- Raynaud’s disease, and epidermolysis bullosa, as well as
atic arthritis, multicentric reticulohistiocytosis, gout, thermal
injuries, scleroderma, and hyperparathyroidism.
other conditions. Scleroderma-like skin changes can also
be evident in graft-versus-host disease after allogeneic
bone marrow transplantation, in eosinophilic fasciitis,
and after exposure to certain chemicals.
without scleroderma, thermal injuries (burn, frostbite, Calcification of the phalangeal soft tissues may be
electrical), trauma, other collagen vascular diseases (der- apparent in other collagen vascular diseases. More diffuse
matomyositis), neuropathic diseases (congenital indiffer- calcification in muscles and subcutaneous tissue is observed
ence to pain, leprosy, diabetes mellitus, meningomye- in many collagen vascular disorders, renal osteodystrophy,
locele), articular disorders (psoriatic arthritis, Lesch- hypoparathyroidism, pseudohypoparathyroidism, fat
Nyhan syndrome), hyperparathyroidism, progeria, and necrosis, hypervitaminosis D, idiopathic hypercalcemia,
epidermolysis bullosa. In most of these diseases, the milk-alkali syndrome, parasitic infections, Ehlers-Danlos
distribution of changes and the presence of additional syndrome, and idiopathic tumoral calcinosis. In many of
abnormalities usually allow an accurate diagnosis (Table these disorders, the pattern of calcification is distinctive
24–3). Further, the association of tuft resorption, skin and differs from the punctate or linear deposits observed
atrophy, and soft tissue calcification in scleroderma is of in scleroderma. Scleroderma may be associated with
great diagnostic significance. “tumoral” periarticular calcifications, an appearance that
TABLE 24-3
Characteristic Sites of Distal Phalangeal Resorption in Various Disorders*
Site
Midportion
Disorder Tuft or Waist Periarticular
Scleroderma + +
Hyperparathyroidism + + +
Thermal injury + +
Psoriatic arthritis + +
Epidermolysis bullosa +
Polyvinyl chloride acro-osteolysis +
Multicentric reticulohistiocytosis + +
Inflammatory (erosive) osteoarthritis +
Lesch-Nyhan syndrome +
Progeria +
*Only the characteristic sites of bone resorption are indicated, although in any single disease,
considerable variability in these sites may exist.
336 Section VI Connective Tissue Disease
may also be apparent in other collagen vascular diseases, Keats TE: Rib erosions in scleroderma. AJR Am J Roentgenol
renal osteodystrophy, milk-alkali syndrome, hypervita- 100:530, 1967.
minosis D, calcium hydroxyapatite crystal deposition Lakhanpal S, Ginsburg WW, Michet CJ, et al: Eosinophilic
disease, and sarcoidosis. fasciitis: Clinical spectrum and therapeutic response in 52
cases. Semin Arthritis Rheum 17:221, 1988.
Martin L: Silicone breast implants: The saga continues. J
Articular Abnormalities Rheumatol 26:1020, 1999.
Monsees B, Murphy WA: Distal phalangeal erosive lesions.
An erosive arthritis of the distal interphalangeal joints Arthritis Rheum 27:449, 1984.
accompanies scleroderma, inflammatory (erosive) osteo- Moore TL, Zuckner J: Eosinophilic fasciitis. Semin Arthritis
arthritis, psoriatic arthritis, multicentric reticulohistio- Rheum 9:228, 1980.
cytosis, gout, and thermal injuries. In most patients with Rabinowitz JG, Twersky J, Guttadauria M: Similar bone mani-
scleroderma, erosions of these joints are mild, although festations of scleroderma and rheumatoid arthritis. AJR Am
uncommonly, larger erosions with extensive phalangeal J Roentgenol 121:35, 1974.
destruction and intra-articular bony ankylosis may be Resnick D, Greenway G, Vint VC, et al: Selective involvement
of the first carpometacarpal joint in scleroderma. AJR Am J
indistinguishable from psoriasis or inflammatory (erosive)
Roentgenol 131:283, 1978.
osteoarthritis. Associated radiographic findings, includ- Resnick D, Scavulli JF, Goergen TG, et al: Intra-articular calci-
ing intra-articular calcification, allow an accurate diagnosis fication in scleroderma. Radiology 124:685, 1977.
of scleroderma in some cases. Rocco VK, Hurd ER: Scleroderma and scleroderma-like dis-
The intra-articular cloudlike calcification in scleroderma orders. Semin Arthritis Rheum 16:22, 1986.
differs in radiographic appearance from that of calcium Roubenoff R, Coté T, Watson R, et al: Eosinophilia-myalgia
pyrophosphate dihydrate crystal deposition disease, cal- syndrome due to L-tryptophan ingestion. Arthritis Rheum
cium hydroxyapatite crystal deposition disease, idiopathic 33:930, 1990.
synovial osteochondromatosis, intra-articular osseous Rush PJ, Bell MJ, Fam AG: Toxic oil syndrome (Spanish oil
bodies, gout, and synovioma or hemangioma. disease) and chemically induced scleroderma-like conditions.
J Rheumatol 11:262, 1984.
Schweitzer ME, Cervilla V, Manaster BJ, et al: Cervical para-
spinal calcification in collagen vascular diseases. AJR Am J
FURTHER READING Roentgenol 157:523, 1991.
Spielvogel RL, Goltz RW, Kersey JH: Scleroderma-like changes
Barnett AJ: Scleroderma (Progressive Systemic Sclerosis). in chronic graft vs host disease. Arch Dermatol 113:1424,
Springfield, Ill, Charles C Thomas, 1974. 1977.
Bassett LW, Blocka KLN, Furst DE, et al: Skeletal findings in Vasey FB, Seleznick MJ: Epidemiology versus outcome: The
progressive systemic sclerosis (scleroderma). AJR Am J silicone breast implant controversy. J Rheumatol 26:1018,
Roentgenol 136:1121, 1981. 1999.
Edworthy SM, Martin L, Barr S, et al: A clinical study of the Ward M, Curé J, Schabel S, et al: Symptomatic spinal calcinosis
relationship between silicone breast implants and connective in systemic sclerosis (scleroderma). Arthritis Rheum
tissue disease. J Rheumatol 25:254, 1998. 40:1892, 1997.
Ishakawa O, Akimoto S, Sato M, et al: Multiple bursitis in
systemic sclerosis. J Rheumatol 24:1189, 1997.
25
CHAPTER 25
Dermatomyositis, Polymyositis, TABLE 25-1
ciated tumors arise from the lungs, prostate, female pelvic damage is unusual (see discussion later in this chapter).
organs, breast, and gastrointestinal tract. The prognosis Clinical manifestations of visceral involvement in dermato-
in patients with type III dermatomyositis is guarded. myositis and polymyositis include cardiac (arrhythmia,
pericarditis), pulmonary (pneumonitis, fibrosis), gastro-
intestinal (dysphagia, abdominal pain, constipation),
Type IV: Childhood Dermatomyositis
renal, neurologic, and ocular abnormalities.
Dermatomyositis or, less commonly, polymyositis affects
children or adolescents in approximately 20% of cases.
Girls are affected more commonly than boys, most often RADIOGRAPHIC ABNORMALITIES
between the ages of 5 and 10 years. Clinical findings Radiographic abnormalities of the musculoskeletal system
include proximal muscle weakness, extensive edema and in dermatomyositis and polymyositis can be divided into
calcification of the skin and subcutaneous tissue, vasculi- soft tissue and articular abnormalities.
tis, lipodystrophy, and joint contractures. Although the
mortality in this disease is approximately the same in
children and adults, the interval between the onset of Soft Tissue Abnormalities
myositis and death is shorter and the degree of soft tissue Although soft tissue abnormalities occur in both children
calcification is more extensive in childhood dermato- and adults, findings in children occur with greater fre-
myositis. With regard to prognosis, two types of der- quency and severity. The initial soft tissue manifestation
matomyositis occurring in children have been identified: is edema of the subcutaneous tissue and muscle, which
an acute fulminant pattern (Banker type) associated with produces increased muscular bulk and radiodensity,
vasculitis, little calcinosis, and a poor prognosis, and a thickening of subcutaneous septa, and poor delineation
slowly progressive pattern (Brunsting type) associated of the subcutaneous tissue–muscle interface (Fig. 25–1).
with calcinosis without vasculitis and a good prognosis. The changes are more prominent in the proximal muscu-
lature, axilla, chest wall, forearms, thighs, and calves.
After effective treatment, tissue edema may decrease or
Type V: Acute Myolysis
disappear entirely, although in many patients, fibrosis and
Type V disease, which is present in approximately 3% of muscle atrophy and contracture become apparent in the
patients, is characterized by the sudden onset of myolysis, later stages of the disease. In these stages, decreased soft
particularly in patients in the first or second decade of tissue and muscular bulk, increased soft tissue lucency,
life. Clinical manifestations include diffuse muscle weak- contractures, and associated osteoporosis are evident.
ness involving the facial, proximal, and distal muscula-
ture. A fatal outcome is not infrequent.
CLINICAL ABNORMALITIES
The most constant clinical finding is muscle weakness,
which eventually appears in virtually all persons with the
disease; this manifestation is the initial symptom in
approximately 50% of patients. Symmetrical involvement
of proximal muscles is most characteristic. Fatigue,
inability to walk or climb stairs, and aching are frequent.
Typical or atypical skin rashes eventually occur in 40% to
60% of patients and are an initial manifestation of the
disease in 20% to 25%. Raynaud’s phenomenon occurs in
about one third of patients with polymyositis. It appears
more commonly in persons who exhibit clinical features
of scleroderma or acrosclerosis, and it is generally mild Figure 25–1. Dermatomyositis and polymyositis: early soft
in nature. tissue edema. Observe the extensive edema of the subcutaneous
Arthralgias and arthritis are present in 20% to 50% of tissue septa. (From Ozonoff MB, Flynn FJ Jr: Roentgenologic
patients. Typically, the wrists, knees, and small joints of features of dermatomyositis of childhood. AJR Am J Roentgenol
the fingers are affected symmetrically. Permanent joint 118:206, 1973.)
Chapter 25 Dermatomyositis, Polymyositis, and Other Inflammatory Myopathies 339
The most characteristic soft tissue abnormality in der-
matomyositis and polymyositis is calcification. The fre-
quency of this finding in children is high. Calcification
may occur within the first year of illness. The extent of
calcification, particularly within musculature, appears to
increase with the severity of the disease. Small or large
calcareous intermuscular fascial plane calcification is dis-
tinctive (Fig. 25–2), although it may not be as common as
subcutaneous calcification (Fig. 25–3). The favorite sites
of intermuscular calcification are the large muscles in the
proximal portion of the limbs. The appearance and distri-
bution of subcutaneous calcification in dermatomyositis
and polymyositis simulate those in scleroderma. Finger-
tip calcification can be associated with terminal phalangeal
erosion, which further complicates the radiographic
differentiation of these two disorders.
Four distinct patterns of soft tissue calcification are
described in childhood dermatomyositis: deep calcareous
masses; superficial calcareous masses; deep linear deposits;
and lacy, reticular, subcutaneous collections encasing the
torso. Of these, the first and third patterns, evident in
deeper tissues, are more common; the fourth, though
infrequent, appears to identify a subgroup of children in
whom the disorder has a severe clinical course. Figure 25–3. Dermatomyositis and polymyositis: subcutaneous
calcification. In this child, diffuse linear subcutaneous calcinosis
is evident.
Articular Abnormalities
The arthralgia and arthritis of dermatomyositis and poly-
myositis are usually unaccompanied by radiographic abnormalities or are associated with transient radio-
graphic features, including soft tissue swelling and
periarticular osteoporosis. Destructive joint changes have
occasionally been noted. Reported radiographic changes
include soft tissue swelling (particularly in the metacar-
pophalangeal and interphalangeal joints), periosteal and
soft tissue calcification in the form of flecks or small
clumps in these same joints, bone erosion (inferior radio-
ulnar, metacarpophalangeal, proximal interphalangeal,
and distal interphalangeal joints, as well as the ulnar
styloid), and alignment abnormalities (flexion deformities
of the metacarpophalangeal joints, swan-neck deformities)
(Fig. 25–4). Particularly characteristic is radial sublux-
ation or dislocation at the interphalangeal joint of the
thumb (“floppy thumb” sign).
SCINTIGRAPHIC ABNORMALITIES
Technetium polyphosphate and similar agents may accu-
mulate in abnormal muscle in patients with dermato-
myositis and polymyositis. This abnormal accumulation
demonstrates some correlation with the severity of mus-
cle weakness and may improve after corticosteroid therapy.
Gallium may also be used in this clinical situation.
Figure 25–2. Dermatomyositis and polymyositis: intermus- MR imaging is well suited to the analysis of a variety of
cular fascial plane calcification. Large calcareous muscular masses muscle disorders, including dermatomyositis and poly-
have produced nodular deformity of the overlying skin. Note myositis. Muscle atrophy, fatty replacement of muscle,
the “tumoral” nature of the calcifications. (From Ozonoff MB, and intramuscular regions of abnormal signal intensity
Flynn FJ Jr: Roentgenologic features of dermatomyositis of that correlate with fibrosis and inflammation are noted.
childhood. AJR Am J Roentgenol 118:206, 1973.) At the outset of the disease, a significant increase in
340 Section VI Connective Tissue Disease
PATHOLOGIC ABNORMALITIES
The microscopic aberrations in involved musculature
include focal or extensive degeneration of muscle fibers,
regenerative activity, infiltration with chronic inflam-
matory cells, interstitial fibrosis, phagocytosis of necrotic
fibers, and variation in the cross-sectional diameter of
adjacent muscle fibers. These histologic aberrations vary
Figure 25–4. Dermatomyositis and polymyositis: destructive little among the various subgroups of the disease.
articular abnormalities. In this 50-year-old woman with well-
documented polymyositis, note the bilateral articular abnor- CAUSE AND PATHOGENESIS
malities consisting of erosions of multiple distal interphalangeal
joints, periarticular calcifications, and subluxation of the inter- Although the cause of dermatomyositis and polymyositis
phalangeal joint of the right thumb. is unknown, evidence indicates that a cell-mediated immune
mechanism is responsible for this damage by affecting
either the muscle or the adjacent blood vessels. A genetic
predisposition to such immunologic mechanisms has been
signal intensity in T2-weighted MR images occurs in the suggested, as has an infectious cause. The association of
involved muscle groups. With clinical improvement, this dermatomyositis and neoplasm has raised the additional
signal intensity returns to normal, and with the recur- possibility that tumor may precipitate inflammatory
rence of clinically evident disease activity, increased signal muscle disease.
A B
Figure 25–5. Dermatomyositis and polymyositis: MR imaging abnormalities. This 36-year-old woman
with dermatomyositis had been symptomatic for 2 years with anterior thigh pain. Transaxial T2-weighted
(TR/TE, 3700/96) spin echo (A) and short tau inversion recovery (STIR) (TR/TE, 5320/30; inversion time,
150 msec) (B) MR images reveal signal intensity alterations in the anterior musculature of both thighs. The
changes, characterized by increased signal intensity, are more prominent in (B). (Courtesy of J. Hodler, MD,
Zurich, Switzerland.)
Chapter 25 Dermatomyositis, Polymyositis, and Other Inflammatory Myopathies 341
A B
Figure 25–6. Dermatomyositis and polymyositis: MR imaging abnormalities. Long-standing disease in a
67-year-old woman. Transaxial T1-weighted (TR/TE, 616/17) spin echo (A) and STIR (TR/TE, 4000/55;
inversion time, 150 msec) (B) MR images reveal signal intensity alterations in the anterior musculature of
both thighs compatible with atrophy, fatty infiltration, fibrosis, and inflammation. Based on imaging, a
biopsy specimen was taken from the area of high signal in the vastus lateralis. Note marked involvement
of the vastus lateralis, vastus intermedius, and vastus medialis muscles, with relative sparing of the rectus
femoris.
A B
Figure 25–7. Focal myositis. In a 13-year-old girl, a transaxial T1-weighted (TR/TE, 577/11) spin echo
MR image (A) shows enlargement of the anterior tibialis muscle. After the intravenous administration of a
gadolinium-containing agent, a transaxial fat-suppressed T1-weighted (TR/TE, 500/8) spin echo MR image
(B) shows enhancement of signal intensity in the intramuscular mass. (Courtesy of J. Jacobson, MD, Ann
Arbor, Mich.)
CHURG-STRAUSS SYNDROME
Churg-Strauss syndrome, also called allergic granulo-
matosis and angiitis, is characterized by the combina-
tion of asthma, eosinophilia, vasculitis, and extravascular
granulomas. It is a rare disease that is more frequent
in men than in women and affects patients of different
ages. The cause of the syndrome is not clear, although
immunoglobulins and complement have been found in
the walls of vessels. The basic pathologic lesion is a necro-
tizing vasculitis involving the small arteries and veins that
results in granulomas. Regardless of which organs are
involved, the two essential findings in this syndrome are
angiitis and extravascular necrotizing granulomas, usu-
ally with eosinophilic infiltrates. Subcutaneous nodules
may be observed. Polyarthralgias are uncommon, and
arthritis is rare. Diagnostic criteria emphasize six find-
ings: asthma, eosinophilia, mononeuropathy or poly-
neuropathy, pulmonary infiltrates, abnormalities of the Figure 26–3. Wegener’s granulomatosis: articular abnormal-
paranasal sinuses, and a biopsy specimen containing a ities. In this patient with well-established disease, osteoporosis
blood vessel with extravascular eosinophils. and subtle periarticular bone erosions are present about the
interphalangeal and metacarpophalangeal joints. The nature
and significance of these findings are unknown. (Courtesy of A.
WEGENER’S GRANULOMATOSIS Brower, MD, Norfolk, Va.)
Wegener’s granulomatosis is associated with necrotizing
granulomatous lesions of the respiratory tract, vasculitis,
and glomerulonephritis. Patient age at disease onset GIANT CELL (TEMPORAL) ARTERITIS AND
ranges from very young to aged; the disease is slightly POLYMYALGIA RHEUMATICA
more common in men than in women. Clinical features Giant cell (temporal) arteritis is characterized by granu-
include acute or chronic sinusitis, chronic rhinitis, nasal lomatous inflammation of large arteries, particularly the
ulceration, anorexia, weight loss, and fever. Arthralgias internal and external carotid, occipital, temporal, and
and arthritis are evident in approximately half the cases; ophthalmic arteries. Typically, patients older than 50 years
they are generally not associated with significant radio- are affected. The arteritis is more frequent in women
graphic changes. Infrequently, bone erosions are seen, than in men, with a ratio of approximately 2:1. Clinical
although they may be related to chronic renal disease findings include painful swelling of the temporal arteries,
(Fig. 26–3). The precise cause of Wegener’s granulo- headaches, visual disturbances, and peripheral neuropathy.
matosis is unknown, but hypersensitivity is a leading can- The diagnosis is supported by characteristic findings on
didate. Diagnostic criteria, as proposed by the American temporal artery biopsy or angiography. Diagnostic criteria
College of Rheumatology, include abnormal urinary sedi- recommended by the American College of Rheuma-
ment, abnormal findings on chest radiography (nodules, tology include disease onset at age 50 years or older, new
cavities, or fixed infiltrates), oral ulcers or nasal dis- onset of localized headache, tenderness of the temporal
charge, and granulomatous inflammation on biopsy. artery or decreased temporal artery pulse, elevation of
the erythrocyte sedimentation rate, and a biopsy sample
of an artery showing necrotizing arteritis.
MIDLINE GRANULOMA
Synovitis is detected in approximately 15% of biopsy-
Midline granuloma is a disease associated with destructive proven cases of giant cell arteritis. Clinical findings related
lesions of the nose, midface, and upper airway. Pulmonary to articular involvement are usually confined to soft tissue
and renal involvement is typically lacking. Soft tissues swelling, although an erosive, seronegative polyarthritis
and adjacent bones are affected by a pathologic process may appear before or after the onset of arteritis. Coexistent
that is associated with dense cellular infiltrates consisting rheumatoid arthritis has also been documented.
of lymphocytes, plasma cells, histiocytes, and immunoblasts An association has been suggested between giant cell
in an angiocentric distribution. Symptoms and signs are arteritis and polymyalgia rheumatica. The latter condi-
related to abnormalities in the mouth, nose, and upper tion is encountered most frequently in elderly patients,
airway. particularly women, and is associated with progressive
Chapter 26 Polyarteritis Nodosa and Other Vasculitides 347
pain in the back, thighs, neck, and shoulders. Transient
inflammatory synovitis may develop in the knee, shoulder,
hip, wrist, and, infrequently, small joints of the fingers.
Chronic inflammatory changes are evident when the
synovial membrane is examined. Significant cartilaginous
and bony abnormalities are generally not encountered. In
some cases, involvement of the axial skeleton, the sterno-
clavicular and sternomanubrial joints, and the shoulder
region is consistent with a “central” synovitis in this
disease. Approximately 50% of patients with giant cell
arteritis experience a prodromal phase with features of
polymyalgia rheumatica, and approximately 30% of
patients with polymyalgia rheumatica have symptoms
and signs of giant cell arteritis.
HENOCH-SCHÖNLEIN (ANAPHYLACTOID)
PURPURA
This syndrome, which consists of nonthrombocytopenic
purpura, arthralgia or arthritis, abdominal pain, and renal
disease, is related to a generalized leukocytoclastic or
necrotizing angiitis involving the arterioles and capil-
laries. Increased vascular permeability is associated with
edema and hemorrhage. The cause of this condition is
unknown, although an allergic phenomenon has been
suggested. The disease is more frequent in children than
in adults, with a peak incidence at age 3 to 10 years.
Diagnostic criteria proposed by the American College
Figure 26–4. Cryoglobulinemia: articular abnormalities. In
of Rheumatology are disease onset at age 20 years or this 55-year-old man, small discrete osteolytic foci (arrows) are
younger, palpable purpura, acute abdominal pain, and a seen in the phalanges. Bone proliferation involves the proximal
biopsy specimen showing granulocytes in the walls of phalanx of the fourth finger. (Courtesy of V. Vint, MD, San
small arterioles or venules. Diego, Calif.)
The classic triad of findings, observed in 70% to 80%
of cases, consists of purpura, abdominal pain, and arthritis.
Articular manifestations consisting of pain, tenderness,
and swelling develop in approximately 60% to 70% of and the elastic pulmonary arteries. It is observed most
patients with Henoch-Schönlein purpura. The joints commonly in young women between the ages of 15 and
involved most commonly are the knees, ankles, hips, wrists, 45 years and is most prevalent in Asia. Initial symptoms
and small joints of the hand. Periarticular soft tissue are not specific and include weight loss, fever, arthralgia,
swelling, the only radiographic abnormality, is related to myalgia, and anemia. The disorder progresses at a vari-
synovial effusion. Complete resolution of clinical and able rate, with gradual obliteration of the involved vessels.
radiographic joint abnormalities is characteristic. Aortic arch syndrome, pulseless disease, renovascular
hypertension, and claudication are among the later mani-
festations. Diagnostic criteria include disease onset at age
CRYOGLOBULINEMIA
40 years or younger, claudication of an extremity, decreased
Essential cryoglobulinemia is a distinct type of disease pulse in the brachial artery, a greater than 10 mm Hg
that manifests as arthralgia or arthritis; the skin, lungs, difference in systolic pressure between arms, a bruit over
kidneys, nervous system, and gastrointestinal tract are the subclavian arteries or the aorta, and arteriographic
involved. The disorder may follow a chronic course, with evidence of narrowing or occlusion of the entire aorta, its
minimal symptoms, or death may result from renal primary branches, or large arteries in the proximal portion
abnormalities. Intermittent arthralgia is observed in 20% of the upper or lower extremities. Although arthralgias
to 75% of patients, whereas arthritis is evident in only may be observed in more than 50% of patients, radio-
5% to 25% of cases. Most commonly, joints in the hands, graphic findings of joint disease are lacking.
knees, ankles, and elbows are involved. Although the
radiographic features in these locations are not dramatic
(Fig. 26–4), well-defined subchondral cystic lesions have BUERGER’S DISEASE
been identified. Buerger’s disease, or thromboangiitis obliterans, is a
segmental, inflammatory, occlusive, nonatherosclerotic
vascular disease. It is more common in men than in
TAKAYASU’S ARTERITIS
women and typically occurs in adult smokers. Buerger’s
Takayasu’s arteritis is a chronic, nonarteriosclerotic disease always affects two or more limbs, beginning
inflammatory disease of the aorta and its main branches distally with proximal progression. The lower extremities
348 Section VI Connective Tissue Disease
are involved more frequently than the upper extremities, Albert DA, Rimon D, Silverstein MD: The diagnosis of
but both the lower and upper extremities may be affected polyarteritis nodosa. Arthritis Rheum 31:1117, 1988.
together. Claudication of the calf or ankle is a common Fauchald P, Rygvold O, Oystese B: Temporal arteritis and
initial feature. Small and medium-sized arteries and veins polymyalgia rheumatica—clinical and biopsy findings. Ann
Intern Med 77:845, 1972.
are affected more often than large vessels. Buerger’s
Gonzalez-GA Ma, Garcia-Porrura C, Vazquez-Caruncho M:
disease may progress rapidly unless the use of tobacco Polymyalgia rheumatica in biopsy proven giant cell arteritis
(smoking or chewing) is eliminated. Angiography reveals does not constitute a different subset but differs from
multiple, bilateral focal segments of arterial stenosis or isolated olymyalgia rheumatica. J Rheumatol 25:1750, 1998.
occlusion, particularly in the digital, palmar, plantar, ulnar, Guillevin L, Cohen P, Gayraud M, et al: Churg-Strauss
radial, tibial, and peroneal vessels, without evidence of syndrome: Clinical study and long-term follow-up of 96
atherosclerosis. Intermittent arthralgias, especially in the patients. Medicine (Baltimore) 78:26, 1999.
large joints, have been reported. Hamidou MA: Periosteal new bone formation in Wegener’s
granulomatosis. J Rheumatol 24:814, 1977.
Lightfoot RW Jr, Michel BA, Bloch DA, et al: The American
ERYTHEMA NODOSUM College of Rheumatology 1990 criteria for the classification
of polyarteritis nodosa. Arthritis Rheum 33:1088, 1990.
Erythema nodosum is a disorder characterized by red, Moncada R, Baker D, Rubinstein H, et al: Selective temporal
tender, warm nodular cutaneous lesions (particularly in arteriography and biopsy in giant cell arteritis: Polymyalgia
the lower legs) that on biopsy demonstrate vasculitis and rheumatica. AJR 122:580, 1974.
cellular infiltration. The disorder may be an allergic or Salvarani C, Hunder GG: Musculoskeletal manifestations in a
hypersensitivity response to a number of precipitating population-based cohort of patients with giant cell arteritis.
factors, such as infection or drugs. Erythema nodosum Arthritis Rheum 42:1259, 1999.
occurs in some patients with ulcerative colitis, Crohn’s Truelove LH: Articular manifestations of erythema nodosum.
Ann Rheum Dis 19:174, 1960.
disease, sarcoidosis, and Behçet’s syndrome.
Valente RM, Hall S, O’Duffy JD, et al: Vasculitis and related
Approximately three fourths of patients with erythema disorders. In Kelley WN, Harris Jr, ED, Ruddy S, Sledge
nodosum experience recurrent episodes of arthralgia and CB (eds): Textbook of Rheumatology, 5th ed. Philadelphia,
arthritis. Joint findings are usually symmetrical in WB Saunders, 1997, p 1079.
distribution, with a predilection for the knees, ankles, Weinberger A, Berliner S, Pinkhas J: Articular manifestations
elbows, wrists, and small joints of the hands. Soft tissue of essential cryoglobulinemia. Semin Arthritis Rheum
swelling related to joint effusion is the only prominent 10:224, 1981.
radiographic abnormality. Woodward AH, Andreini PH: Periosteal new bone formation
in polyarteritis nodosa. A syndrome involving the lower
extremities. Arthritis Rheum 17:1017, 1974.
Zvaifler NJ: Vasculitides: Classification and pathogenesis. Aust
FURTHER READING N Z J Med 8(Suppl 1):134, 1978.
A B
Figure 27–1. Mixed connective tissue disease: deforming nonerosive arthropathy. A 32-year-old woman
had progressive deformities of the hand. A, The initial radiograph outlines periarticular osteoporosis, capsular
calcification (arrowhead), and a minor degree of ulnar deviation at the metacarpophalangeal joints. B, Nine
years later, the joint deformities have progressed. Observe the flexion at the first metacarpophalangeal joint
and hyperextension of the interphalangeal joint of the same digit. Also note the radial deviation at the wrist,
ulnar deviation and flexion at the metacarpophalangeal joints, and flexion at the proximal interphalangeal
joints. The width of the joint spaces is difficult to evaluate because of the associated deformities. (Courtesy
of M. Dalinka, MD, Philadelphia, Pa.)
Osteoporosis. Diffuse or periarticular osteoporosis is Changes in Phalangeal Tips. Soft tissue atrophy, soft tissue
common (10% to 100% of patients). In this regard, the calcification, and resorption of the terminal tufts of the
findings simulate those of rheumatoid arthritis. phalanges (detected in 25% to 70% of patients) simulate
the findings of scleroderma. Osseous resorption leads to
Soft Tissue Swelling. Symmetrical soft tissue swelling is typical “penciling” of the phalanges.
common about the involved articulations (65% to 75%
of patients). Diffuse swelling of the hand related to wide- Joint Subluxation. Joint subluxation (present in 12% to
spread edema is also apparent. 100% of patients) is identical to that in rheumatoid
arthritis or systemic lupus erythematosus.
Joint Space Narrowing. Diffuse narrowing of the artic- Although clinical involvement of other joints is not
ular space is also common (10% to 100% of patients). uncommon, radiographic changes in these locations are
Intra-articular bony ankylosis has also been noted. recorded infrequently. Periarticular osteoporosis and,
uncommonly, calcification may be seen about the knee,
Bone Erosions. The osseous erosions in MCTD (found in elbow, or hip (Fig. 27–4). The erosive arthritis of MCTD
25% to 75% of patients) are similar to those in rheuma- is generally indistinguishable from rheumatoid arthritis.
toid arthritis. Erosions of the distal interphalangeal joints In patients with MCTD who demonstrate erosive arthri-
of the fingers may be observed in MCTD. In addition, tis, serologic testing for rheumatoid factor may yield
in an occasional patient with MCTD, severe destructive positive or negative results. The soft tissue calcifications
arthritis can simulate the appearance of psoriatic arthritis. detected in patients with MCTD are generally indistin-
Chapter 27 Mixed Connective Tissue Disease and Collagen Vascular Overlap Syndromes 351
TABLE 27-1
Radiographic Features of Mixed Connective Tissue Disease
FURTHER READING
A B
Figure 28–1. Rheumatic fever: Jaccoud’s arthropathy. A, On a posteroanterior radiograph with the hand
pressed firmly against the cassette, the only striking deformity is ulnar deviation of the fifth finger at the
metacarpophalangeal joint. Mild periarticular osteoporosis is seen. No evidence of osseous erosion is
apparent. B, On the oblique radiograph, the hand has been lifted from the cassette. Boutonnière and swan-
neck deformities of all the digits can be seen.
ings of joint space narrowing and osseous erosion. The TABLE 28-2
deforming nonerosive arthropathy of rheumatic fever is
similar to that of systemic lupus erythematosus and, more Radiographic Characteristics of Jaccoud’s Arthropathy
infrequently, other collagen vascular diseases and vasculi-
tides (Table 28–3). Although classically the ulnar digits Flexion and ulnar deviation of the metacarpophalangeal joints,
are more commonly affected in rheumatic fever, and all particularly in the fourth and fifth digits
the digits (including the thumb) are affected in systemic Flexion and fibular deviation of the metatarsophalangeal joints
Periarticular osteoporosis
Joint space narrowing (rare)
Hook erosions on the radial and palmar aspects of the
metacarpal heads (rare)
TABLE 28-1
Clinical Manifestations Necessary for a Diagnosis of
Jaccoud’s Arthropathy
TABLE 28-3
History of recurrent attacks of acute rheumatic fever Diseases That May Lead to Deforming Nonerosive
Delayed recovery after joint inflammation with subsequent Arthropathy
deformity, particularly the MCP joints
Characteristic articular deformity consisting of flexion and Rheumatic fever
ulnar deviation at the MCP, particularly in the fourth and Collagen vascular disorders, particularly systemic lupus
fifth digits erythematosus
Elicitation of tendon crepitus Rheumatoid arthritis (rare)
Joint disease that is generally asymptomatic Agammaglobulinemia (rare)
Ehlers-Danlos syndrome (rare)
MCP, metacarpophalangel.
Chapter 28 Rheumatic Fever 355
VII
Degenerative Diseases
CHAPTER 29
Degenerative Diseases of TERMINOLOGY AND CLASSIFICATION
Extraspinal Locations Degenerative joint disease is the best general phrase to
describe degenerative alterations in any type of articula-
tion. These alterations may appear in fibrous, cartilagi-
SUMMARY OF KEY FEATURES nous, or synovial articulations. Their pathologic and
radiographic appearances vary from one location to
Degenerative joint disease is widespread and common. another, but at any site, the abnormalities appear to be
In synovial joints, the process is termed osteoarthritis. related to degeneration of some articular structure. The
At these sites, abnormalities predominate in the terms osteoarthrosis and osteoarthritis are reserved for
cartilaginous and osseous tissues, whereas alterations degenerative disease of synovial joints. Although inflam-
in the synovial membrane generally are mild. Typical matory changes are not pronounced in most of these
findings include joint space loss, eburnation, cyst joints (making the suffix “-osis” more appropriate than
formation, and osteophytosis. Subluxation, malalignment, “-itis”), the term osteoarthritis is used throughout this
fibrous ankylosis, and intra-articular osseous and chapter, as this designation is widely accepted in the
cartilaginous bodies may complicate osteoarthritis. United States. Furthermore, when significant synovial
The most common sites of extraspinal osteoarthritis inflammatory abnormalities do accompany degenerative
are the interphalangeal and metacarpophalangeal joints joint disease, osteoarthritis is the more accurate descrip-
of the hand, first carpometacarpal and trapezioscaphoid tion of the disorder.
areas of the wrist, acromioclavicular and sternoclavicular Degeneration may also affect fibrous joints or entheses.
articulations, hip, knee, and tarsometatarsal and An enthesis is a site of attachment of a tendon, ligament,
metatarsophalangeal joints of the great toe. At each of or joint capsule to bone. An enthesopathy is an abnor-
these sites, radiographic features of the disease are mality at this site, and a degenerative enthesopathy is the
sufficiently characteristic to allow their differentiation proper term for degeneration at this site. Degeneration
from the features of other articular disorders. of a cartilaginous joint is also recognized, especially at
Two special varieties of osteoarthritis have been the discovertebral junction, where it is termed interver-
described, although their existence as discrete entities tebral osteochondrosis and spondylosis deformans (see
is not universally accepted. Generalized osteoarthritis Chapter 30).
may be a particular form of degenerative joint disease Traditionally, degenerative joint disease was classified
in which multiple articulations are affected. Inflammatory into primary (idiopathic) and secondary types. Primary
(erosive) osteoarthritis is associated with clinical and degenerative joint disease was regarded as a process in
pathologic features of joint inflammation. Although which articular degeneration occurs in the absence of
its distribution is virtually identical to that of any obvious underlying abnormality, whereas secondary
noninflammatory digital osteoarthritis, certain degenerative joint disease was regarded as articular degen-
radiographic features, such as erosions and intra- eration produced by alterations from a preexisting afflic-
articular bony ankylosis, appear to be more typical tion. This classification into primary and secondary
of inflammatory osteoarthritis. degenerative joint disease is misleading. Careful evalua-
tion of many examples of so-called primary degenerative
joint disease discloses some mechanical deviation in the
involved articulation that has led to secondary degener-
ation of the joint. It appears likely, therefore, that primary
INTRODUCTION
degenerative joint disease does not exist, and use of such
Degenerative joint disease is the most frequent articular a designation only underscores our limited diagnostic
affliction. Despite its common occurrence, degenerative capabilities.
arthritis did not receive a great deal of attention in the Articular degeneration may result from either an
past, because many considered the disorder to be the dull abnormal concentration of force across a joint with a
and inevitable accompaniment of advancing age. More normal articular cartilage matrix or a normal concen-
recently, a new curiosity and an enthusiasm to learn tration of force across an abnormal joint (one with carti-
about degenerative arthritis have stimulated the consid- laginous or subchondral osseous alterations) (Table 29–1).
erable investigation that this common and significant Eventually, abnormalities of force and articular structure
condition deserves. appear together.
357
358 Section VII Degenerative Diseases
TABLE 29–2
Main Causative Factors According to Location of Osteoarthritis
PATHOGENESIS
Figure 29–1. Occupation-induced degenerative joint disease.
In this pneumatic driller with 10 years of professional The previous discussion makes it clear that numerous
experience, cystic lesions are seen in the lunate and scaphoid. factors can initiate the pathologic and radiologic features
(Courtesy of S. Sintzoff, MD, Brussels, Belgium.) of degenerative joint disease. One way or another, these
360 Section VII Degenerative Diseases
factors create a situation in which the intra-articular surface are pathologically evident, and vascular invasion,
structures can no longer resist the physical forces being infarction, and necrosis of the subchondral trabeculae
applied to the joint. In some instances, it is the force itself account for joint space loss, bony sclerosis, and cyst for-
that is abnormal, whereas in other instances, weakened mation, which are apparent on radiographs; in the non-
cartilage or subchondral bone is unable to combat the pressure segment, pathologically evident hypervascularity
normal forces acting across the articulation. of marrow and cartilage leads to radiographically detect-
Traditionally, degenerative alterations are thought to able osteophytosis. These findings emphasize the simul-
begin in the articular cartilage. Physical forces apparently taneous occurrence of both destructive and reparative
disrupt the cartilage matrix and adversely affect the processes in this disease (Table 29–3).
chondrocytes. The alterations in the matrix are almost
certainly related to enzymatic destruction. An alternative
theory emphasizes the initial role of subchondral bony Cartilaginous Abnormalities
abnormalities in the pathogenesis of degenerative joint Characteristic changes in articular cartilage accompany
disease. According to this theory, overload produces micro- degenerative joint disease (Fig. 29–2). Involved cartilage
fractures in the subchondral bony trabeculae. Repair of appears discolored (brown-gray or yellow-gray) and
these fractures leads to increased stiffness of the bone, a thinned. Irregular crevices or cracks and larger areas of
reduction in its shock-absorbing efficiency, and exposure
of overlying cartilage to increased force. This theory
gains support from the clinical observations that some TABLE 29–3
patients with osteoarthritis demonstrate increased bone
density when radiography, computed tomography (CT), Degenerative Joint Disease of Synovial Articulations
or photon absorption techniques are used; that persons (Osteoarthritis): Radiographic-Pathologic Correlation
with disorders characterized by increased bone density
(Paget’s disease, osteopetrosis) may have degeneration Pathologic Abnormality Radiographic Abnormality
of neighboring articulations; and that patients with Cartilaginous fibrillation Localized loss of joint space
osteoporosis of the femoral head may have a lower rate of and erosion
occurrence of osteoarthritis of the hip. Increased cellularity and Bony eburnation
There is no convincing evidence that alterations in the hypervascularity of
synovial membrane and joint lubrication play important subchondral bone
roles in the initiation of degenerative joint disease. After Synovial fluid intrusion or Subchondral cysts
the appearance of degeneration in the cartilage or sub- bony contusion
chondral bone, a vicious circle of events occurs that Revascularization of Osteophytes
aggravates the articular insult, resulting in progression of remaining cartilage and
disease. Cartilaginous destruction increases joint insta- capsular traction
bility, provoking further stress on cartilage and bone. Periosteal and synovial Osteophytes and buttressing
In synovium-lined joints, damage confined to cartilage membrane stimulation
generally shows a poor reparative response, whereas that Compression of weakened Bony collapse
extending to subchondral bone is accompanied by the and deformed trabeculae
appearance of cartilaginous tissue formed by vascular Fragmentation of Intra-articular osseous
invasion. This tissue is not as sound mechanically as the osteochondral surface bodies
original hyaline surface; it resembles fibrocartilage and Disruption and distortion of Deformity and
accounts for such events as restoration of joint space, capsular and ligamentous malalignment
disappearance of bone eburnation and cysts, and improved structures
joint congruity.
RADIOGRAPHIC-PATHOLOGIC CORRELATION
Synovial Joints
The concept that excessive wear and tear initiates
articular degeneration in the stressed (pressure) areas of a
joint is a valuable one that has been emphasized repeat-
edly in the literature. It is also important to recognize
that changes are common in the nonstressed (nonpres-
sure) segments of the joint. In general, the type and
severity of cartilaginous and osseous abnormalities are
different in the pressure and nonpressure segments of the
articulation. In fact, the segmental nature of the alter-
ations is an important characteristic of degenerative joint
disease, allowing its pathologic and radiographic differ- Figure 29–2. Cartilaginous abnormalities: pathologic find-
entiation from other disorders. In the pressure segment, ings. Drawing depicts the irregular cracks or crevices (arrow-
progressive thinning and denudation of the cartilaginous heads) that may appear in the chondral surface.
Chapter 29 Degenerative Diseases of Extraspinal Locations 361
erosion develop, and ulcers of variable depth are seen,
some of which are deep enough to expose the subchon-
dral bone. Denuded areas appear, uncovering the sub-
chondral bone. Eventually, loss of entire segments of the
cartilaginous coat may be evident.
It is this progressive loss of cartilage, evident on
pathologic examination, that accounts for one funda-
mental radiographic sign of degenerative joint disease:
diminution of the articular space. Characteristically, the
loss of joint space is localized predominantly in the area
of the joint that has been subject to excessive pressure.
Thus, joint space narrowing is apparent in the supero-
lateral aspect of an osteoarthritic hip and in the medial
femorotibial space of an osteoarthritic knee. In certain
sites, articular space diminution may be more diffuse
(osteoarthritis of the interphalangeal or metacarpopha- Figure 29–3. Subchondral bone cysts: pathologic findings.
langeal joints), and joint space loss may involve the entire Drawing reveals the typical appearance of multiple subchondral
articulation. With these few exceptions, however, it is the cysts of varying size in areas of cartilaginous degeneration or
focal nature of the cartilaginous destruction and resulting disappearance. They are surrounded by sclerotic bone.
loss of the interosseous space that allow the differentia-
tion of osteoarthritis from processes, such as rheumatoid
arthritis, that lead to diffuse chondral alterations. nation using the term cyst is not entirely accurate, in that
a cyst implies a cavitary lesion with an epithelial lining.
Subchondral cysts are not surrounded by such a lining,
Subchondral Bony Abnormalities
nor are they uniformly cavitary. The term geode, which is
Subchondral bony abnormalities accompany degenera- popular in Europe, is a geologic term and is likewise not
tive joint disease and can be divided into a destructive very appropriate; geodes are small, hollow rocks lined
phase (regressive remodeling) and a productive phase (pro- with crystals. Here, the designation subchondral cyst is
gressive remodeling). Characteristics of the destructive used despite its obvious inadequacies.
phase are bony eburnation, cyst formation, flattening, and Within the pressure segment of the subchondral bone
deformity, which predominate in the pressure segment in osteoarthritis, cystic spaces appear between thickened
of the joint; characteristics of the productive phase are trabeculae. These cysts commonly are multiple, of vari-
osteophytes, which predominate in the nonpressure able size, and piriform in shape. On pathologic exami-
segment of the joint. nation, some cysts are noncavitary, containing myxoid
and adipose tissue mixed with loose fibrous elements;
others possess central cavitation containing proteinaceous
Eburnation
material, are surrounded by fibrous tissue, and are well
After cartilage loss, bony eburnation becomes evident in demarcated by adjacent eburnated bone. On radiographic
the closely applied osseous surfaces, apparently related to examination, cysts appear in association with joint space
deposition of new bone on preexisting trabeculae and to loss and bony eburnation. Communication with the ar-
trabecular compression and fracture with callus forma- ticular space may or may not be identifiable; when present,
tion. The weakened hyperemic subchondral bone in the such communication may allow gas to pass from the joint
pressure segment of the joint is vulnerable to further into the cyst, creating a pneumatocyst.
deformity. The loss of resilient cartilage and the restric- The pathogenesis of cystic lesions in degenerative joint
tion of joint motion heighten and localize the abnormal disease has received a great deal of attention. Although
stress on the bone, leading to trabecular fracture, flatten- all reports emphasize the importance of concentrated
ing, and collapse. pressure or stress on the articular cartilage and sub-
On radiographic examination, sclerosis and joint space chondral bone in the development of these lesions, two
diminution are closely related. Generally, radiographic fundamental theories of pathogenesis have been proposed:
evidence of loss of articular space is present before signi- synovial fluid intrusion and bony contusion (Fig. 29–4).
ficant eburnation becomes apparent. With progressive Recent evidence indicates that both elements are opera-
obliteration of this space, sclerosis becomes more ting together in the pathogenesis of cystic lesions.
prominent, extending vertically into deeper regions of Although the pathogenesis of subchondral cysts is con-
the subchondral bone and horizontally into adjacent tested, their occurrence in degenerative joint disease is
osseous segments. not. In this disease, the cystic lesions create multiple
radiolucent areas of varying size on apposing surfaces of
bone in the pressure segment of the joint, which must be
Cyst Formation
differentiated from the subchondral lucent lesions that
Cysts are an important and prominent finding in may accompany other disorders (Table 29–4).
osteoarthritis (as well as in other articular disorders) In rheumatoid arthritis, cysts occur initially at
(Fig. 29–3). These lesions have been termed subchondral chondro-osseous junctions as a result of erosion of
cysts, subarticular pseudocysts, and geodes. Any desig- cartilage-free bone by inflammatory synovial tissue or
362 Section VII Degenerative Diseases
Figure 29–4. Pathogenesis of subchondral cysts. Two fundamental theories are illustrated. The theory of
synovial intrusion (1) states that abnormal stress on cartilage (arrow) leads to cartilaginous degeneration.
Synovial fluid is driven into the subchondral bone through gaps in the chondral surface and bone plate
(arrowhead), producing cysts that initially communicate with the joint and subsequently may become
occluded with fibrous tissue. The theory of bony contusion (2) also holds that cartilage loss occurs due to
abnormal stress (arrow). Subsequently, fracture and vascular insufficiency of the bone itself (arrowhead)
produce cysts, which may communicate with the joint secondarily.
pannus and are accompanied by early loss of articular tence of this lesion as an entity that can be separated from
space. They frequently are multiple, lack sclerotic margins, post-traumatic and degenerative cysts of bone. This lesion,
and subsequently extend over large segments of the joint which is commonly encountered in middle-aged adults,
surface. In calcium pyrophosphate dihydrate crystal deposi- is characterized by mild, localized pain and the absence of
tion disease, multiple, large, and widespread cystic lesions a significant history of trauma. The ganglion is generally
are characteristic. They resemble the cysts in degener- solitary and is located in the epiphysis of a long bone
ative joint disease, except that they are larger, more (particularly the medial malleolus and femoral head), a
numerous, and more frequently associated with disrup- carpal bone, or a subarticular region of a flat bone (par-
tion, collapse, and fragmentation of the subchondral bone ticularly the acetabulum). It is apparent that the radio-
plate. In osteonecrosis, cysts appear within the pressure graphic and pathologic characteristics of intraosseous
segment of the articulation, related to osteoclastic ganglia and degenerative cysts are similar, although some
resorption of necrotic trabeculae, with fibrous replace- differences may be encountered. Characteristics of an
ment of bone. Collapse of the subchondral bone plate intraosseous ganglion that may be distinctive include its
and preservation of the joint space are additional charac- occurrence in nonpressure areas of a joint, its association
teristic features of osteonecrosis. with a relatively normal adjacent articulation, its lack of
Subchondral cyst formation is occasionally a sequela communication with the nearby joint, and its relatively
of bone injury, although its precise mechanism is not large size. In fact, some investigators emphasize that these
known. A radiolucent lesion of variable size becomes ganglionic cysts do not originate from an articular dis-
evident over a period of months after the traumatic order; degenerative or inflammatory changes are usually
episode. The lesion, which is observed most commonly absent in the adjacent articulation.
about the ankle, knee, or hip, is well marginated by a rim Subchondral cysts in degenerative joint disease must
of bony sclerosis and may communicate with the articular also be distinguished from a variety of primary and
cavity (Fig. 29–5). It resembles a degenerative cyst, secondary neoplasms of bone. These include chondro-
although its larger size and the relatively normal appear- blastoma (10- to 30-year-old age group, solitary lesion),
ance of the joint itself are useful diagnostic features. giant cell tumor (20- to 40-year-old age group, solitary
An intraosseous ganglion is another subchondral eccentric trabeculated lesion), and skeletal metastasis (older
radiolucent lesion that may simulate a degenerative cyst patients, single or multiple lesions of variable appear-
(Fig. 29–6), although there is debate regarding the exis- ance). In general, this differentiation is not difficult.
Chapter 29 Degenerative Diseases of Extraspinal Locations 363
TABLE 29–4
Differential Diagnosis of Subchondral Cystic Lesions
Features of degenerative cysts such as multiplicity, The excrescences frequently predominate on one side of
segmental distribution, surrounding sclerosis, and adja- the joint. Marginal osteophytes develop initially in areas
cent abnormal joint are of great diagnostic aid. of relatively normal joint space and usually are unasso-
ciated with significant adjacent sclerosis or cyst forma-
tion; articular space loss, eburnation, and subchondral
Osteophytosis cysts are findings that are characteristic of pressure
Osteophytes are considered by many physicians to be segments of the joint.
the most characteristic abnormality of degenerative joint
disease. Osteophytes develop in areas of a joint that are Central (Interior) Osteophytes. The presence of osteophytic
subjected to low stress; they may be marginal (peripheral), outgrowths in the central or interior portions of the
although they may become apparent at additional loca- articular space is a less well recognized manifestation
tions (Table 29–5). of osteoarthritis (Fig. 29–8). In central areas where
remnants of articular cartilage still exist, hypervascularity
Marginal Osteophytes. At the peripheral portions of of subchondral bone stimulates endochondral ossifica-
involved joints, characteristic outgrowths develop in tion. The resulting excrescences (which are most promi-
degenerative joint disease (Fig. 29–7). Vascularization of nent in the hip and knee) are button-like or flat and are
the subchondral bone marrow in this region produces often demarcated at their bases by remnants of the original
calcification of the adjacent cartilage and stimulates calcified cartilage.
endochondral ossification. As it grows, it leaves behind Central osteophytes frequently lead to a bumpy ar-
remnants of the original calcified cartilage (and sub- ticular contour on radiographic examination. The small
chondral bone plate) as a telltale indicator of the location excrescences can be misinterpreted as evidence of intra-
of the original joint surface, which can be identified articular osseous (loose) bodies (Fig. 29–9) or cartilage
histologically and radiographically. calcification (chondrocalcinosis). The presence of conti-
Radiographically, marginal osteophytes appear as lips nuity between the osteophyte and the underlying bone
of new bone around the edges of the joint. They may be and of ossification rather than calcification should lead to
smooth, pitted, or undulating and are of variable size. the correct analysis of the radiographs.
364 Section VII Degenerative Diseases
TABLE 29–5
Types of Osteophytes
Osteonecrosis
The frequency and significance of osteonecrosis (Fig.
29–12) in osteoarthritis have not been fully delineated.
Bone necrosis may be apparent on histologic examination
of the eburnated surface in the pressure segment of the
joint. The changes of necrosis become more exaggerated
as the disease process becomes more advanced; however, C
bone necrosis is generally a localized microscopic process
in osteoarthritis and usually cannot be detected on radio- Figure 29–8. Central osteophytes: pathologic findings. A,
graphic examination. Further, flattening and collapse Reduplication of cartilage and bone. In the central portions of
may be apparent in osteoarthritis without indicating the the articulation, hypervascularity of subchondral bone can lead
presence of significant osteonecrosis. The radiographic to stimulation of remnants of cartilage, producing endochondral
differentiation of osteoarthritis (with or without osteo- ossification. The flat outgrowths that develop (arrows) are often
necrosis) from osteonecrosis with secondary joint demarcated by the original zone of calcified cartilage (arrow-
heads). B, Shifting of cartilage and bone border. In this situation,
degeneration can be extremely difficult, however.
the osteophyte develops (arrows) without leaving behind a zone
of calcified cartilage. C, On a photograph of a macerated coronal
Synovial Membrane Abnormalities section of the femoral head in a patient with osteoarthritis, the
osteophytes (solid arrows) are developing by reduplication and
Synovial membrane alterations are not prominent in most shifting of the cartilage-bone junction. In places, a calcified
cases of osteoarthritis. With increasing severity of carti- zone of cartilage is identified (arrowheads). A marginal osteo-
laginous and osseous alterations, the changes in the synovial phyte is also apparent (open arrow).
membrane may become more prominent. Cartilaginous
and osseous debris that originated from the articular
surfaces may become embedded in the synovial mem-
brane, acting as a local irritant and producing prolifer- matory (erosive) osteoarthritis (see the later discussion in
ative changes. this chapter). Small synovial effusions may be encoun-
Infrequently, synovial abnormalities in osteoarthritis tered in osteoarthritis. Sizable joint effusions that occur
may become so severe that they resemble the changes of in the absence of trauma or osseous collapse should
rheumatoid arthritis. In the interphalangeal joints of the stimulate an investigation to exclude a superimposed arti-
fingers, such synovial inflammation is present in inflam- cular process, such as infection or crystal deposition disease.
366 Section VII Degenerative Diseases
Differential Diagnosis
Osteoarthritis is associated with joint space loss, bony
sclerosis, and cyst formation in the pressure (or stressed)
segment of the joint and with osteophytes in the non-
pressure segment (Table 29–6). Osteoporosis, osseous
erosion, and sizable joint effusions are not typical of this
disease. Figure 29–10. Periosteal and synovial osteophytes:
In rheumatoid arthritis, joint effusion, osteoporosis, buttressing. Note the bone formation along the medial aspect
and uniform joint space loss are characteristic. In the of the femoral neck (arrow) in addition to other changes of
seronegative spondyloarthropathies (ankylosing spondylitis, osteoarthritis, including joint space narrowing, sclerosis, and
psoriatic arthritis, and Reiter’s syndrome), bone forma- cyst formation.
tion may be seen, but it does not resemble the well-
defined subchondral sclerosis or osteophytosis of
osteoarthritis. Rather, in the seronegative spondylo-
arthropathies, irregular, poorly defined proliferation and former disorder are intra-articular and extra-articular
intra-articular bony ankylosis may be noted. calcification, involvement of unusual joints, large tumor-
In gouty arthritis, bulky asymmetrical masses, eccentric like cystic lesions, progressive abnormalities with fragmen-
well-circumscribed osseous erosions, and preservation of tation and collapse of bone, and absence of osteophytosis.
the articular space are common. In calcium pyrophos- Osteonecrosis may produce considerable collapse of
phate dihydrate crystal deposition disease (idiopathic or subchondral bone while the joint space is maintained.
that associated with hemochromatosis), findings are very This combination is not evident in osteoarthritis. The
similar to those of osteoarthritis. Diagnostic aids in the subsequent appearance of secondary degenerative joint
Chapter 29 Degenerative Diseases of Extraspinal Locations 367
A B
Figure 29–11. Capsular osteophytes: pathologic and radiographic findings. A, Drawing of a section
through an osteoarthritic distal interphalangeal joint reveals capsular osteophytes (arrows) arising from the
distal and middle phalanges. Note that the outgrowths extend proximally and are associated with small
ossicles (arrowhead). Additional findings include cartilaginous destruction and subchondral sclerosis.
B, Radiograph shows the capsular osteophyte (arrow) arising from the distal phalanx. Joint space narrowing
and sclerosis are also seen.
A B
Figure 29–12. Osteoarthritis versus osteonecrosis. A, Osteoarthritis with collapse and fragmentation. In
this patient with long-standing disease, considerable collapse of the weight-bearing surface of the femoral
head is evident. Note superior or upward migration of the femoral head with respect to the acetabulum, a
large medial osteophyte (arrow), acetabular osteophytes, and buttressing. The changes are those of
osteoarthritis; the extent of bony collapse, though perhaps indicating secondary osteonecrosis, should not
lead to an erroneous diagnosis of primary osteonecrosis. B, Osteonecrosis with secondary cartilaginous
destruction. In this patient, segmental collapse of the femoral head (arrows) is evident. Note diffuse loss of
joint space, related either to secondary osteoarthritis or to chondrolysis. Although sclerosis and buttressing
are evident, the findings do not resemble those of primary osteoarthritis.
368
TABLE 29–6
Differential Diagnosis of Osteoarthritic Changes
Intra-articular Osseous
Osteo- Joint Space Osseous Osseous Bone Osseous Fragmentation,
porosis Narrowing Erosions Cysts Sclerosis Osteophytosis “Whiskering” Fusion Collapse Typical Location
Osteoarthritis – Focal – + + + – – In large joints DIP, PIP, MCP joints of
hand; first CMC and
trapezioscaphoid joints of
wrist; first MTP, TMT
joints of foot; knee; hip;
apophyseal joints of spine
Inflammatory – Focal + + + + – DIP, PIP – DIP, PIP, MCP joints of
osteoarthritis joints hand; first CMC and
trapezioscaphoid joints
of wrist
Rheumatoid + Diffuse + + – – – Carpal, tarsal Rare PIP, MCP joints of hand;
arthritis areas wrist; MTP joints of
foot; knee; elbow;
glenohumeral joint;
cervical spine
Gouty arthritis – May be + + + + – Rare ± MTP, IP joints of foot;
absent DIP, PIP, MCP joints of
hand; wrist; elbow; knee
Pyrophosphate – Diffuse – + + ± – – + MCP joints of hand;
arthropathy radiocarpal joint of wrist;
knee
Seronegative ± Diffuse + + + – + + – Spine; sacroiliac joint;
spondylo- various articulations of
arthropathies the appendicular skeleton
Osteonecrosis – Absent – + + – – – + Hip; glenohumeral joint;
until late knee; sites of trauma
Neuropathic – Variable – ± + ± – – + Sites depend on underlying
osteoarthropathy disorder
CMC, carpometacarpal; DIP, distal interphalangeal; IP, interphalangeal; MCP, metacarpophalangeal; MTP, metatarsophalangeal; PIP, proximal interphalangeal; TMT, tarsometatarsal.
Chapter 29 Degenerative Diseases of Extraspinal Locations 369
disease in osteonecrosis can lead to difficulty in making a
diagnosis. Neuropathic osteoarthropathy is characterized
by severe fragmentation and collapse of the articular
surfaces, extensive bony sclerosis, multiple cartilaginous
and osseous intra-articular bodies, large joint effusions,
subluxation, and malalignment.
Cartilaginous Joints
Two major extraspinal symphyses are the symphysis pubis
and the manubriosternal joint. In the symphysis pubis,
cleftlike cavities appear in the central portion of the joint,
usually after the age of 10 to 20 years (Fig. 29–13). They
subsequently widen and become more irregular and may
contain gas, leading to a vacuum phenomenon. Age-
related degenerative changes in the manubriosternal
joint are similar.
Figure 29–14. Degenerative enthesopathy: calcaneus.
Syndesmoses and Entheses Radiograph depicts the irregular osseous proliferation that can
occur at sites of tendon and ligament attachment to bone. One
At syndesmoses (e.g., tibiofibular and radioulnar inter- such site is the plantar aspect of the calcaneus (arrow).
osseous membranes and ligaments), fibrous degeneration
and bone proliferation may become apparent. At osseous
sites of tendon attachment, a degenerative enthesopathy
COMPLICATIONS OF DEGENERATIVE JOINT
becomes apparent (Fig. 29–14). The degree of bone pro-
DISEASE
liferation may be considerable, resulting in radiographi-
cally detectable excrescences (e.g., ischial tuberosity, Although there are numerous local complications of
trochanters) and enthesophytes (e.g., calcaneus, ulnar degenerative joint disease, several complications are
olecranon, patella). Tendon and ligament calcification particularly well known and deserve special emphasis.
may also be noted (e.g., sacrospinous and sacrotuberous
ligaments). These changes are accelerated in the pres-
Malalignment and Subluxation
ence of trauma or chronic stress and may be generalized
in diffuse idiopathic skeletal hyperostosis. Inflammatory Angular deformity in osteoarthritis is not unexpected in
enthesopathies are common in ankylosing spondylitis view of the nonuniform nature of the joint involvement.
and other spondyloarthropathies. Focal loss of articular space is characteristic and may
produce, for example, varus (and, less commonly, valgus)
deformity of the knee (Fig. 29–15). Changes in bone,
capsule, and supporting tissue allow progressive sublux-
ation, examples of which are lateral displacement of the
tibia on the femur, lateral displacement of the femoral
head in the acetabulum, and radial and proximal displace-
ment of the first metacarpal base on the trapezium.
variety of disease processes, including osteoarthritis. unchanged. Osteochondral bodies commonly migrate to
Cartilaginous and osseous debris may remain on the joint and become lodged in saclike articular regions, such as
surface or become dislodged or loose in the joint cavity. the acetabular fossa of the hip, olecranon fossa of the
Debris subsequently may become embedded at a distant elbow, or subscapular recess and axillary pouch of the
synovial site, eliciting a local inflammatory response. glenohumeral joint (Fig. 29–17). They must be differen-
On radiographs, the osteocartilaginous bodies can tiated from the osteophytes and fibrocartilaginous
increase or decrease in size, disappear, or remain (meniscal) calcification and ossification that may accom-
A B
Chapter 29 Degenerative Diseases of Extraspinal Locations 371
pany other disease processes, such as idiopathic synovial
osteochondromatosis, transchondral fractures, or osteo-
chondritis dissecans. Differentiation is usually not diffi-
cult when osseous or cartilaginous dense lesions are seen
in joints that do not reveal any manifestations of osteo-
arthritis (as in idiopathic synovial osteochondromatosis
or osteochondritis dissecans). Furthermore, idiopathic
synovial osteochondromatosis is commonly associated
with a large number of osteocartilaginous bodies of
approximately equal size, whereas secondary synovial
osteochondromatosis (as is evident in osteoarthritis) leads
to fewer bodies (generally <10) of varying size. Distin-
guishing intra-articular osseous and cartilaginous bodies
from osteophytes or meniscal calcification (or ossifica-
tion) can be difficult, however.
Radiographic-Pathologic Correlation
lesions, or ossicles, are apparent overlying the joint capsule;
Distal and proximal interphalangeal joints are frequently they resemble intra-articular osseous bodies.
affected simultaneously; however, extensive alterations at
distal interphalangeal joints may occur in the absence of
Differential Diagnosis
proximal interphalangeal joint abnormalities (Fig. 29–19).
Some degree of symmetry in the location of alterations in Occasionally, the undulating, irregularly enlarged sub-
the two hands is the rule. chondral or marginal bone may contain small pockets
Radiographs reveal prominent osteophytes and joint that can be misinterpreted as osseous erosion (Fig. 29–20),
space narrowing, providing close apposition of adjacent leading to an erroneous diagnosis of inflammatory (ero-
enlarged osseous surfaces. It is the closely applied, undu- sive) osteoarthritis. Erosive abnormalities of distal (and
lating articular surfaces that produce the diagnostic proximal) interphalangeal joints accompany various other
radiographic appearance of the disease, allowing its disorders, including psoriatic arthritis, gout, multicentric
differentiation from erosive disorders, which cause reticulohistiocytosis, thermal injuries, hyperparathyroidism,
separation of the involved bones. In osteoarthritis, the and rheumatoid arthritis.
wavy contour of the base of the distal phalanx resembles
the wings of a bird, the seagull sign. On frontal radio-
Metacarpophalangeal Joints
graphs, the involved digits frequently reveal mild to
moderate radial and ulnar subluxation at distal or proxi- Metacarpophalangeal joint involvement in osteoarthritis
mal interphalangeal joints, producing a zigzag contour. is almost invariably associated with more prominent
At the margins of the affected joint, focal radiodense abnormalities at distal and proximal interphalangeal joints.
372 Section VII Degenerative Diseases
Wrist
The radial distribution of osteoarthritis of the wrist is
well known. In the absence of significant accidental or
occupational trauma, changes are usually confined to the
Figure 29–19. Osteoarthritis of the interphalangeal joints of trapeziometacarpal (first carpometacarpal) joint and
the hand: typical distribution. Findings are apparent in distal
trapezioscaphoid space of the midcarpal joint.
interphalangeal, proximal interphalangeal, and, to a lesser extent,
metacarpophalangeal joints. The interphalangeal joint of the
thumb is also affected. Trapeziometacarpal (First Carpometacarpal) Joint. Involve-
ment of the trapeziometacarpal joint can lead to promi-
nent clinical abnormalities. These include pain that is
Uniform narrowing of one or more metacarpophalangeal aggravated by twisting and gripping motions, restricted
interosseous spaces is evident in 90% to 95% of patients movement, crepitus, and instability. The radiographic
with interphalangeal joint osteoarthritis (see Fig. 29–19). features of degenerative trapeziometacarpal joint disease
Cystic lesions and osteophytes may also be apparent. include increasing radial subluxation of the metacarpal
Nonuniform loss of interosseous space is unusual, and base, narrowing of the interosseous space, sclerosis and
erosions are absent. It is the combination of uniform loss cystic changes in the subchondral bone, osteophytosis,
of joint space and absence of marginal erosions that and bony fragmentation. Bony excrescences extend from
allows differentiation of osteoarthritis from rheumatoid the trapezium in a distal direction between the first and
arthritis at these articulations. Osteophytosis is an addi- second metacarpal bones (Fig. 29–21).
tional manifestation of metacarpophalangeal joint degen-
eration. The outgrowths generally are small and may be Trapezioscaphoid Space. Osteoarthritis in the trapezio-
distributed on either the metacarpal head or the phalanx. scaphoid space of the midcarpal compartment of the
wrist is frequently combined with degenerative changes
at the trapeziometacarpal joint (see Fig. 29–21). Isolated
abnormalities at this space are also reported, but pre-
dominant and severe changes resembling those of
degenerative joint disease at this articulation can indicate
the presence of calcium pyrophosphate dihydrate crystal
deposition disease. Typical radiographic features of osteo-
arthritis are apparent in a unilateral or bilateral distri-
bution, including joint space narrowing and sclerosis of
apposing surfaces of the trapezium, trapezoid, and
scaphoid.
A B
Figure 29–24. Osteoarthritis of the glenohumeral joint: subchondral sclerosis in the humeral head. A,
Radiograph demonstrates focal eburnation involving the mid-superior portion of the humeral head, with
radiographic evidence of bony sclerosis (arrowheads). B, Coronal oblique T1-weighted (TR/TE, 650/20)
spin echo MR image after the intra-articular injection of gadolinium compound reveals sclerosis, with low
signal intensity, in the humeral head (arrow). The rotator cuff is intact, there is sclerosis in the glenoid, and
debris (arrowhead) is present in the axillary pouch.
Chapter 29 Degenerative Diseases of Extraspinal Locations 375
Sternoclavicular Joint
Osteoarthritis of the sternoclavicular joint is not uncom-
mon. Its clinical manifestations include pain, tenderness,
and palpable enlargement of the articulating bone.
Radiographic findings include unilateral or bilateral joint
space narrowing, sclerosis, and osteophytosis.
Sacroiliac Joint
Degenerative changes in the sacroiliac joint can be
demonstrated pathologically in middle-aged and elderly
persons. The pathologic abnormalities become promi-
nent, particularly in the ilium, and include cartilage
Figure 29–26. Shoulder impingement syndrome: subacro- fibrillation and erosion, denudation of cartilaginous
mial enthesophyte. Radiograph reveals a prominent subacromial surfaces, and partial or complete fibrous ankylosis of the
enthesophyte (arrow), sclerosis, and cyst formation (arrowhead) joint. Subchondral eburnation and osteophytes become
in the greater tuberosity, with progressive elevation of the apparent. Changes may be unilateral or bilateral in distri-
humeral head, indicative of disruption of the rotator cuff. bution and may be associated with significant clinical
findings, including pain and tenderness.
Radiographic manifestations include loss of articular
Acromioclavicular Joint space and well-defined subchondral bony sclerosis
Degenerative changes in this synovial articulation are (Fig. 29–28). Although erosion of subchondral bone is
almost universal in elderly persons. Diffuse discomfort infrequent in degenerative disease of the sacroiliac joint,
in the shoulder region, radiating to the upper arm and osteophytes are common. Osteophytes can occur at any
aggravated by motion, is suggestive of osteoarthritis in level in the joint but are most characteristic at the antero-
the acromioclavicular joint. Radiographic examination of superior and anteroinferior limits of the articular cavity.
the degenerating acromioclavicular joint reveals joint They may produce prominent focal radiopaque lesions,
space diminution, sclerosis of apposing osseous surfaces, which can simulate the appearance of osteoblastic skeletal
marginal osteophytes, hypertrophy and inferior subluxa- metastasis (Fig. 29–29). On frontal radiographs, these
tion of the acromial end of the clavicle, and osseous anterior osteophytes are superimposed on the inter-
proliferation on the superior surface of the acromion. It osseous space, simulating intra-articular bony ankylosis.
376 Section VII Degenerative Diseases
Hip
Clinical Abnormalities
Osteoarthritis of the hip may produce significant clinical
symptoms confined to the hip region or referred to other
sites, such as the knee, buttock, thigh, groin, or greater
trochanter. Restriction of motion, particularly rotation
and extension, is also common. Flexion deformities are
frequent in many patients with long-standing osteoarthritis
Figure 29–28. Degenerative disease of the sacroiliac joint: of the hip.
joint space narrowing and bony sclerosis. The joint space is
uniformly narrowed (arrow), with linear condensation of bone
Radiographic-Pathologic Correlation
in the ilium (arrowhead). Note the sharp definition of the
subchondral bone. A vacuum phenomenon is apparent within Degenerative hip disease may demonstrate several distinct
the inferior aspect of the joint. patterns on frontal radiographs, based on the relative
TABLE 29–7
Differential Diagnosis of Degenerative Disease of the Sacroiliac Joint
Osteitis
Osteoarthritis Ankylosing Spondylitis Condensans Ilii
Age Older patients Younger patients Younger patients
Sex Men and women Men > women Women > men
Distribution Bilateral or unilateral Bilateral, symmetrical Bilateral, symmetrical
Sclerosis Iliac Iliac Iliac
Mild, focal May be extensive Triangular in shape
Erosions Absent Common Absent
Intra-articular bony ankylosis Rare Common Absent
Para-articular osteophytosis Common Rare Rare
Ligamentous ossification Less common Common Absent
Chapter 29 Degenerative Diseases of Extraspinal Locations 377
A B
Figure 29–29. Degenerative disease of the sacroiliac joint: osteophytosis. A, Frontal radiograph demon-
strates a radiodense lesion (arrows) overlying the superior aspect of the synovium-lined portion of the
sacroiliac joint. B, With CT, which provided a transaxial section through this lesion, it is apparent that bony
sclerosis and a bridging osteophyte (arrow) are the cause of the abnormal density seen in the plain film.
(B, Courtesy of J. Scavulli, MD, San Diego, Calif.)
A B
Figure 29–31. Degenerative disease of the sacroiliac joint: pneumatocyst. A, Transaxial CT scan shows
the well-defined gas-filled iliac cyst. B, Corresponding axial T2-weighted (TR/TE, 5710/120) fast spin echo
MR image shows a prominent signal void. Although the location is atypical, a bone island could exhibit a
similar signal void.
nate on the outer aspect of the femoral head and the Superomedial migration (tilt deformity) of the femoral
acetabulum. In some instances, the acetabular cysts head, which may be detected in 35% to 50% of patients
become large, contain gas, and are associated with tears with osteoarthritis, is more frequent in men than in
and mucinous degeneration of the labrum (Fig. 29–34). women and is commonly bilateral (Fig. 29–35). Patients
with this pattern of disease may become symptomatic at
a relatively young age. Its cause is unknown; however, in
most patients, it is unrelated to previous epiphysiolysis.
Early radiographic abnormalities consist of superior
displacement of the femoral head, associated with joint
space narrowing in the outer one third of the articular
cavity. Progressive joint space loss results in close apposi-
tion of the superior and middle surfaces of the femoral
head and acetabulum. With continued deformity along
the outer one third of the femoral head, osteophytosis on
its medial and inferior surface is seen. These osteophytes
begin as broad-based shallow outgrowths that fill in the
free space on the medial aspect of the joint cavity. With
further growth along its inferomedial aspect and resorp-
tion along its superolateral aspect, the femoral head
assumes an altered relationship with the femoral neck.
Careful examination of radiographs often reveals the
presence of a curvilinear radiodense area on the inner
aspect of the femoral head. This represents the original
calcified zone of articular cartilage, allowing accurate
appraisal of the position of the femoral head before its
Figure 29–32. Patterns of migration of the femoral head remodeling.
with respect to the acetabulum for the right hip. Three direc- This description of the tilt deformity (superomedial
tions can be identified. When superior joint space loss predom- pattern of migration) emphasizes its similarity to the
inates, the femoral head moves in an upward, or superior,
superolateral pattern of femoral head migration. In both
direction with respect to the acetabulum. When joint space loss
is confined to the inner one third of the joint, a medial migra- situations, the femoral head is displaced laterally until
tion pattern is evident. With diffuse loss of articular space, the restraining osteophytes are formed on the lateral aspect
femoral head migrates axially along the axis of the femoral neck. of the acetabulum, at which time subluxation ceases.
(Redrawn from Graham J, Harris WH: Paget’s disease Osteophyte formation is then noted on the inferomedial
involving the hip joint. J Bone Joint Surg Br 53:651, 1971.) aspect of the femoral head. With the tilt deformity, the
Chapter 29 Degenerative Diseases of Extraspinal Locations 379
A B
Figure 29–33. Osteoarthritis of the hip: superolateral migration pattern. A, Progressive narrowing of the
outer one third of the joint space results in upward migration of the femoral head with respect to the
acetabulum. Associated findings include eburnation, lateral acetabular osteophytes, and medial femoral
buttressing. B, In this patient, joint space narrowing and sclerosis (solid straight arrows) along the superior
articulating surface are associated with subchondral collapse of the adjacent femoral head. A lateral
osteophyte (open arrow), widened inferomedial joint space (W), and medial femoral neck buttressing (curved
arrow) are noted.
acetabular depth is normal grossly, and although minor Radiographic analysis indicates medial displacement
degrees of lateral movement occur, acetabular outgrowths of the femoral head, with narrowing of the medial joint
develop, which resist considerable lateral subluxation of space and associated widening of the lateral joint space.
the head. Intermediate varieties of superior migration Mild to moderate protrusio acetabuli deformities may
exist (10% to 15% of patients) in which the degree of become evident with increased bone density of the
lateral subluxation is less than that associated with central and inferior aspects of the acetabulum.
superolateral migration but greater than that associated Osteophyte formation occurs on both the acetabulum
with superomedial migration. and the femur.
The tilt deformity resulting from remodeling in the
osteoarthritic hip can be differentiated from a similar Axial Migration Pattern. Axial migration of the femoral
deformity accompanying epiphysiolysis with secondary head due to diffuse or concentric loss of articular space
degeneration. In the latter case, because of true slip- is infrequent. When radiographs reveal a degenerative-
ping of the femoral head in relation to the femoral type articular disease associated with diffuse loss of joint
neck, the original zone of calcified cartilage, along space, other primary diagnoses must be considered before
with the remainder of the head, becomes displaced attributing the abnormalities to osteoarthritis. When
posteromedially. axial migration is present in degenerative joint disease,
the pattern of joint space loss is similar to that in rheuma-
Medial Migration Pattern. In 10% to 35% of patients with toid arthritis. The presence of osteophytes and sclerosis
osteoarthritis of the hip, a pattern of medial migration of and the absence of erosions, osteoporosis, and severe
the femoral head is evident (Fig. 29–36). This pattern acetabular protrusion, however, allow the differentiation
is commonly bilateral and symmetrical and is more of osteoarthritis and rheumatoid arthritis.
frequent in women. It is interesting to speculate that
specific idiosyncrasies in the acetabular structure produce Miscellaneous Migration Patterns. The previously described
stress in what would otherwise be a medial nonpressure patterns of migration are based on data derived from
area, leading to degenerative alterations. frontal radiographs. CT images used to study osteo-
380 Section VII Degenerative Diseases
A B
Figure 29–36. Osteoarthritis of the hip: medial migration
pattern. A, As the femoral head migrates medially, joint space
narrowing and sclerosis of the inferomedial aspect of the
articular surface result, and the superior joint space widens.
B, Medial joint space loss (solid arrows) and a widened lateral
joint space (W) have resulted from this pattern of migration.
A lateral femoral osteophyte (open arrow) and medial femoral
neck buttressing (curved arrow) are evident. Mild protrusion
deformity is seen.
joint space loss with axial migration often indicates another disease, and alkaptonuria. Further, secondary degenera-
disease process, such as rheumatoid arthritis, infection, tive joint disease superimposed on another primary process
or cartilage atrophy due to disuse or immobilization. If can lead to symmetrical loss of joint space, osteophytosis,
axial migration of the femoral head is accompanied by and eburnation; these processes include trauma with
sclerosis and osteophyte formation, additional diseases acetabular fracture, synovial disorders such as rheuma-
should be considered, including ankylosing spondylitis, toid arthritis, Paget’s disease, chondrolysis, epiphyseal
calcium pyrophosphate dihydrate crystal deposition dysplasia, and osteonecrosis.
A B
Figure 29–38. Osteoarthritis of the hip: posteromedial migration pattern. A, Frontal radiograph reveals
mild medial migration of the femoral head and slight acetabular protrusion. Osteophytes are seen.
B, Transaxial CT scan shows that the femoral head is closely applied to the posterior surface of the
acetabulum and possesses a prominent posterior osteophyte (arrow). (B, Courtesy of D. Haselwood, MD,
Sacramento, Calif.)
A B
Figure 29–39. Osteoarthritis of the hip: rapidly progressive disease. A and B, Radiographs obtained
several months apart indicate progressive compression and deformity of the femoral head, presumably
related to the collapse of subchondral cysts. The severity, rapidity, and morphology of the changes are
distinctly unusual for uncomplicated osteoarthritis. A thorough investigation for other causes of the articular
disease, such as crystal- or steroid-induced arthropathy and infection, was unrewarding in this patient.
Chapter 29 Degenerative Diseases of Extraspinal Locations 383
TABLE 29–8
Differential Diagnosis of Patterns of Femoral Head
Migration
+, common.
resting. Stiffness, tenderness, swelling, and warmth may Femorotibial Compartments. Bilateral or unilateral altera-
also be recorded. Symptoms and signs of synovitis are tions may be evident. Radiographic findings are usually
generally less striking in osteoarthritis than in rheuma- more prominent on the medial aspect of the joint (see
toid arthritis, although patients occasionally have large Fig. 29–42). Unicompartmental (medial or, less frequently,
joint effusions and masses related to synovial rupture and lateral femorotibial compartment) or bicompartmental
popliteal cysts. Angular deformity (usually varus), insta- (medial femorotibial and patellofemoral compartments
bility, and soft tissue atrophy are late manifestations of or, less often, lateral femorotibial and patellofemoral
the disease. compartments) involvement is more typical than tricom-
partmental involvement in patients with osteoarthritis.
The discovery of alterations confined to or predominantly
Radiographic-Pathologic Correlation in the medial femorotibial compartment is most consis-
Compartmental Distribution. When interpreting the tent with osteoarthritis. Similar abnormalities in the lateral
radiographic and pathologic changes in an osteoarthritic femorotibial compartment are compatible with this disease,
knee, it is useful to regard the joint as consisting of three especially in women, and may even be characteristic after
compartments or spaces: medial femorotibial, lateral certain injuries, such as those resulting in laxity of the
femorotibial, and patellofemoral (Fig. 29–41). Radio- anterior cruciate ligament; however, this pattern requires
graphic abnormalities usually predominate in one or two that rheumatoid arthritis and calcium pyrophosphate
of the three compartments, although pathologic aberra- dihydrate crystal deposition disease be given careful con-
tions are evident in all three areas. Typical pathologic sideration as alternative diagnoses. Symmetrical changes
changes include cartilage fibrillation and denudation in both the medial and lateral femorotibial compartments
of the osseous surfaces, eburnation, subchondral cystic generally indicate a disorder other than osteoarthritis.
lesions, and osteophytosis. Joint space narrowing, which corresponds to cartilagi-
The ability of radiographs to detect pathologic abnor- nous erosion, varies from mild to severe, with complete
malities depends on the method of examination. Routine obliteration of the articular space (Fig. 29–43). Sclerosis
techniques are limited in their sensitivity to delineate of subchondral bone is more frequent in the tibia;
early alterations, although some degree of joint space isolated sclerosis of the femur is unusual. Sclerotic bone
narrowing, sclerosis, cysts, and osteophytes are usually is found consistently in compartments with articular space
detected in the more involved weight-bearing compart- narrowing. Most commonly it is localized to the imme-
ment (medial or lateral femorotibial compartment). diate subchondral regions. Subchondral cysts are less
“Tunnel” projections obtained during knee flexion may frequent in the knee than in the hip. Osteophytes are
reveal cartilaginous and osseous lesions that are not frequent and predominate at the margins of the articu-
evident on routine radiographs. Radiographs obtained lation in both the femur and the tibia. Central, or inte-
during stress or weight bearing provide a better assess- rior, osteophytes simulating intra-articular bodies may
ment of cartilage loss as the joint space collapses under also be observed, especially on the femoral condyles.
the body weight (Fig. 29–42). They also allow more Marginal or central osteophytes contribute to intra-
accurate delineation of subluxation of the femur and tibia articular surface irregularity, as well as to sharpening of
and of varus or valgus angulation. Patellofemoral com- the tibial spines.
partment analysis requires special radiographic projec-
tions, including tangential and oblique views. Patellofemoral Compartment. The patellofemoral com-
partment is frequently affected in osteoarthritis. Abnor-
malities in this area are best detected on axial projections.
The ideal projection, particularly for the evaluation of
possible patellar subluxation, is obtained with only a mod-
erate amount of knee flexion. In osteoarthritis, cartilagi-
nous fibrillation and erosion and subchondral bony
eburnation are detected. In the patella, these changes
predominate in the lateral facet, probably related to the
large articular surface and the lateral vector of forces
resulting from physiologic valgus.
On radiographs, patellofemoral compartment altera-
tions are usually combined with abnormalities of the
femorotibial compartments. Most typically, the medial
femorotibial compartment is altered, although occasion-
ally, lateral femorotibial and patellofemoral compartment
changes occur together. It is unusual to see extensive
changes in the patellofemoral compartment without sig-
nificant abnormality of one of the femorotibial compart-
Figure 29–41. Compartmental analysis of knee disease. ments. Thus, the finding of abnormalities resembling
Evaluation of the medial femorotibial (M), lateral femorotibial degenerative joint disease isolated to the patellofemoral
(L), and patellofemoral (PF) compartments should be accom- area should lead to a search for another disease process,
plished on all knee radiographs. A fourth area, the proximal such as calcium pyrophosphate dihydrate crystal deposi-
tibiofibular joint (arrowhead), may also be affected. tion disease, Wilson’s disease, or hyperparathyroidism.
Chapter 29 Degenerative Diseases of Extraspinal Locations 385
A B
C D
Figure 29–42. Osteoarthritis of the knee: supplementary views. A, Standard non–weight-bearing antero-
posterior radiograph shows mild medial femorotibial compartment narrowing. B, Standard non–weight-
bearing “tunnel” radiograph shows moderate joint space loss in the medial femorotibial compartment.
C, Standard weight-bearing radiograph of the fully extended knee reveals moderate to severe joint space loss
in the medial femorotibial compartment. D, Weight-bearing radiograph of the slightly flexed knee
(“standing tunnel projection”) shows severe joint space loss in the medial femorotibial compartment.
Radiographic features of patellofemoral osteoarthritis Angulation and Subluxation. Angulation and subluxation
include joint space narrowing, sclerosis, and osteophytes, at the knee joint may accompany the ligamentous changes
particularly on the patellar side of the space. Associated evident in severe osteoarthritis. Varus angulation is more
scalloped defects of the anterior cortex of the femur may frequent than valgus angulation. Translation or sublux-
become prominent in osteoarthritis (Fig. 29–44). This ation of the tibia on the femur laterally with varus
finding appears to arise from pressure erosion of the angulation and medially with valgus angulation is typical,
femoral cortex, generally by the adjacent patella; the although the magnitude of displacement is generally less
femoral defect is located at the level that the patella than 10 mm.
assumes in full extension of the knee. Abnormal tilting
and posterior angulation of the superior pole of the Synovitis, Synovial Cyst Formation, and Intra-articular
patella due to quadriceps dysfunction probably contrib- Osteocartilaginous Bodies. Joint effusions are generally
ute to the lesion. small in osteoarthritis of the knee. Localized cartilagi-
An additional degenerative phenomenon that occurs nous and osseous debris arises from the disintegrating
on the anterior surface of the patella consists of bony articular surfaces of the femur, tibia, and patella. Such
proliferation at the site of osseous attachment of the debris may exist as loose bodies, or “joint mice,” before
quadriceps apparatus. This is not a manifestation of osteo- its incorporation into the synovial membrane (Fig. 29–46).
arthritis but rather is an enthesopathic alteration prob- Arthrography, CT scanning, MR imaging or CT or MR
ably related to abnormal stress on the ligamentous arthrography can also be used in the assessment of intra-
connection to the bone (Fig. 29–45). It produces articular bodies in the knee (Fig. 29–47).
irregular excrescences that are detected radiographically
as hyperostosis or “whitening” of the anterior patellar Sesamoid Involvement. In osteoarthritis of the knee, carti-
surface, termed the “tooth” sign or patellar “whiskering.” laginous fibrillation and erosion and osseous prolifera-
386 Section VII Degenerative Diseases
A B C
Figure 29–45. Degenerative disease of the knee: enthesopathy and differential diagnosis. Patellar
excrescences and radiodense lesions may have several causes. A, Enthesopathy. Typical osseous excrescences
are developing at the quadriceps attachment to the anterior surface of the patella (arrows). This is not a
manifestation of osteoarthritis. B, Osteoarthritis. Osteophytes are evident (arrow) at the superior articular
surface of the patella. Note patellofemoral compartment narrowing and an effusion (arrowhead). C, Calcific
tendinitis. Linear radiodense areas (arrows) in the tendon are related to calcific tendinitis. Calcium
hydroxyapatite or calcium pyrophosphate dihydrate crystal deposition can create this appearance.
Isolated patellofemoral compartment abnormalities are Sclerosis, Cyst Formation, and Osteophytosis. Crystal-
encountered in calcium pyrophosphate dihydrate crystal induced arthropathies (gout or calcium pyrophosphate
deposition disease, Wilson’s disease, and hyperparathy- dihydrate crystal deposition disease) can also be asso-
roidism. Uncommonly, such changes are noted in ciated with sclerosis, cysts, and osteophytes; however, the
osteoarthritis. presence of osseous erosions and the absence of articular
TABLE 29–9
Compartmental Analysis of Knee Disease
*Subchondral resorption can produce collapse of bone in the medial or lateral femorotibial compartment, or both.
388 Section VII Degenerative Diseases
Ankle
In the absence of significant trauma, osteoarthritis of the
ankle is infrequent. It may occur after fracture of the
neighboring bones or ligamentous injury, particularly
when the ankle mortise is disrupted (Fig. 29–49).
Capsular traction can produce a talar beak on the dorsal
aspect of the bone, which, although reminiscent of the
beak that accompanies tarsal coalition, is distinctive in
appearance.
Tarsal Articulations
Significant degenerative changes may develop at the first
tarsometatarsal joint (Fig. 29–50). In this location, joint
space narrowing and sclerosis related to osteoarthritis
simulate the findings of gouty arthritis. Alterations at
additional tarsal locations may develop after trauma.
Figure 29–47. Osteoarthritis of the knee: intra-articular Persistent hindfoot pain occurring after a calcaneal frac-
osteocartilaginous bodies. Transaxial T2-weighted (TR/TE, ture is a potential complication of osteoarthritis in one or
1800/90) spin echo MR image reveals a joint effusion, medial both subtalar joints. Radiographs are generally inadequate
meniscal degeneration, cartilage loss, and a large synovial cyst for delineating the degenerative abnormalities; these are
containing ossified bodies (arrows). seen to better advantage on CT scans (Fig. 29–51).
Chapter 29 Degenerative Diseases of Extraspinal Locations 389
Clinical Abnormalities
The onset of the disease may be abrupt. Painful nodules
of the distal and proximal interphalangeal joints of the
fingers are associated with edema and redness of the
overlying skin, as well as tenderness and restricted motion.
Figure 29–52. Osteoarthritis of the first metatarsophalangeal Interphalangeal joints are affected most commonly,
joint: hallux rigidus. Frontal radiograph reveals considerable although abnormalities of the metacarpophalangeal and
joint space narrowing, sclerosis, and osteophytosis (arrowheads) carpometacarpal joints, trapezioscaphoid area of the mid-
about the first metatarsophalangeal joint. Observe the flatten- carpal joints, knees, and hips may become apparent. The
ing of the metatarsal head and a large metatarsal cyst (arrow).
course of the disease is variable. In some patients, the
inflammatory signs subside with conservative therapeutic
of the digits. Four types of deformity of the toes have measures after a period of months to years. In others, the
been defined: claw toe, characterized by hyperextension inflammatory signs progress and the clinical manifesta-
of the metatarsophalangeal joint and flexion of the proxi- tions resemble or become identical to those of rheuma-
mal and distal interphalangeal joints; hammer toe, in toid arthritis (see the later discussion in this chapter).
which there is hyperextension of the metatarsophalangeal
joint, flexion of the proximal interphalangeal joint, and
hyperextension of the distal interphalangeal joint; mallet Radiographic Abnormalities
toe, with flexion of the distal interphalangeal joint; and The radiographic changes are often characterized by a
curly toe, in which the metatarsophalangeal joint is in a combination of bone proliferation and erosion. Typically,
neutral position and there is flexion of the proximal and osteophytosis resembles that in noninflammatory osteo-
distal interphalangeal joints. arthritis, predominating at the articular margins of the
distal and proximal interphalangeal joints. At these sites,
SPECIAL TYPES OF DEGENERATIVE JOINT joint space narrowing is common, with associated sub-
DISEASE chondral sclerosis. Interosseous space diminution also
becomes evident at the interphalangeal joint of the
Generalized Osteoarthritis thumb, the metacarpophalangeal and first carpometa-
The concept of generalized or polyarticular osteoarthritis carpal joints, and the trapezioscaphoid space of the mid-
is not universally accepted. When radiographs reveal carpal articulation.
evidence of degenerative changes in multiple sites, diag- In some involved joints, erosions may become promi-
noses other than generalized osteoarthritis must be con- nent. They are particularly frequent in interphalangeal
sidered. Multiple epiphyseal dysplasia, spondyloepiphyseal articulations (Fig. 29–53). The erosions commonly begin
dysplasia, osteonecrosis, alkaptonuria, Paget’s disease, at the central portion of the joint in the form of sharply
acromegaly, occupation-induced articular disorders, marginated, etched defects. This central location is charac-
calcium pyrophosphate dihydrate or calcium hydroxyapa- teristic, differing from the marginal location of erosions
tite crystal deposition disease, gout, hemophilia, and in rheumatoid arthritis, psoriatic arthritis, and related
inflammatory arthritides all may lead to similar changes disorders. Although the exact pathogenesis of erosions in
at multiple articular locations. In some of these disorders inflammatory osteoarthritis is not clear, they may be
(osteonecrosis, gout, hemophilia, inflammatory arthrit- related to collapse or pressure atrophy of subchondral
ides), secondary degenerative abnormalities related to bone rather than to synovial inflammation. Intra-articular
altered joint mechanics can obscure the more diagnostic bony ankylosis is also evident in many patients with
radiographic features; in others (calcium pyrophosphate inflammatory osteoarthritis. This finding is virtually
dihydrate crystal deposition disease, alkaptonuria), abnor- confined to one or several interphalangeal joints; after
malities resembling those of degenerative joint disease ankylosis, osteophytosis and sclerosis about the involved
are part of the basic disease process. joint may disappear. Radiographic abnormalities in ar-
Chapter 29 Degenerative Diseases of Extraspinal Locations 391
Figure 29–53. Inflammatory
(erosive) osteoarthritis: interpha-
langeal joint abnormalities. A, Draw-
ing depicts the usual appearance of
inflammatory osteoarthritis of the
interphalangeal joints. Findings
resemble those of noninflammatory
osteoarthritis, including traction
osteophytes (solid arrows), ossicles
(arrowhead), and eburnation. Erosive
changes are also apparent, particu-
larly in the central aspect of the joint
(open arrows) (compare with Fig.
29–11). B, In a more advanced case,
disruption of the entire central
aspect of the joint (arrows) is typical.
Note the changes in the proximal
interphalangeal joint, which are
identical to those of noninflam-
matory osteoarthritis. C, The even-
tual result may be intra-articular
osseous fusion, as seen in the distal
interphalangeal joint.
A
B C
bution of affected joints is almost identical in both hydroxyapatite, monosodium urate, and even calcium
diseases. The major differences between inflammatory pyrophosphate dihydrate crystals, should be considered.
and noninflammatory osteoarthritis are the clinical signs
of joint inflammation and the radiographic findings of
bone erosion and ankylosis in the former disorder.
Inflammatory osteoarthritis may represent one extreme FURTHER READING
in the spectrum of degenerative joint disease, however,
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cloudlike radiodense lesions, may be seen. This pattern Boegård T, Jonsson K: Radiography in osteoarthritis of the
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radiographic alterations, although in some cases, it arises Boegård T, Rudling O, Dahlstrom J, et al: Bone scintigraphy
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Gout. The deposition of monosodium urate crystals in radiographically diagnosed osteophytes and magnetic reso-
osteoarthritic joints of the fingers, leading to articular nance detected cartilage defects in the tibiofemoral joint.
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30
CHAPTER 30
Degenerative Disease of the Spine The central portion of the intervertebral disc, the
nucleus pulposus, is soft and gelatinous at birth and con-
sists mainly of mucoid material. Gradually, the mucoid
substance is replaced by fibrocartilage. The anulus
SUMMARY OF KEY FEATURES fibrosus surrounds the nucleus pulposus and contains an
A variety of degenerative processes involve the spine, outer zone of collagenous fibers and an inner zone of
each of which is characterized by distinctive radiographic fibrocartilage. Anteriorly, the anulus fibrosus is anchored
manifestations. Intervertebral (osteo)chondrosis is to the cartilaginous endplate, vertebral rim, and perios-
associated with vacuum phenomena, disc space teum of the vertebral body; the last site of attachment con-
narrowing, and reactive sclerosis of the vertebral body; sists of strong fibers termed Sharpey’s fibers (Fig. 30–1).
spondylosis deformans is associated with osteophytosis; Cartilaginous endplates are closely applied to the central
osteoarthritis leads to joint space narrowing, bone depressions present on the superior and inferior surfaces
sclerosis and hypertrophy, and, infrequently, osseous of the vertebral body.
fusion; and ligamentous degeneration can be associated Apophyseal articulations are situated between the ar-
with calcification and ossification. Although any one ticular facets of adjacent vertebrae. They possess a synovial
degenerative process may predominate in a patient, membrane, meniscus-like structures, and thin and loose
frequently, all occur together. articular capsules.
Several complications may appear during the course The anterior longitudinal ligament, which extends
of degenerative disorders of the spine, including along the anterior surface of the vertebral column, is
abnormalities of vertebral alignment (segmental particularly adherent to the edge of the vertebral body
instability, degenerative anterior spondylolisthesis, adjacent to the discovertebral junction. The posterior
degenerative retrolisthesis, senile thoracic kyphosis, longitudinal ligament extends along the inside of the
and degenerative lumbar scoliosis and kyphosis), vertebral column and is intimate with the posterior sur-
intervertebral disc displacement (cartilaginous nodes, faces of the vertebral bodies and intervertebral discs.
intraspinal displacement), disc calcification and
ossification, and spinal stenosis. Plain-film radiographic
examination in patients with such complications may
be supplemented with conventional tomography,
myelography, discography, computed tomography,
and magnetic resonance imaging.
INTRODUCTION
The convenient label “degenerative disease” is carelessly
applied to a variety of distinct processes of the spinal
joints. Because the joints of the spine have fundamental
anatomic differences, it is incorrect to assume that they Figure 30–1. Anatomy of the discovertebral junction.
deteriorate in an identical fashion. Rather, the synovial Drawing of a sagittal section of a normal discovertebral joint
indicates its major constituents. Observe the nucleus pulposus,
joints, the symphyses, the syndesmoses, and the entheses located slightly posterior to the midportion of the intervertebral
(sites of ligamentous attachment to bone) each degenerate disc. It is surrounded by the concentric fibers of the anulus
in a specific fashion that is associated with characteristic fibrosus. Each vertebral body possesses elevated cortical rims,
clinical, pathologic, and imaging aberrations. related to fusion of the marginal apophyseal (epiphyseal) rings,
and a central depression that is covered by a cartilaginous end-
plate. Beneath the endplate is a subchondral bone plate (solid
MAJOR TYPES OF SPINAL ARTICULATIONS arrows). The anulus fibrosus is attached to the bony rims through
Below the level of the second cervical vertebra, the verte- calcified cartilage connected to osseous tissue (open arrows). The
brae are united principally by a series of cartilaginous outer fibers of the anulus fibrosus, occasionally termed periver-
joints (intervertebral discs) between the vertebral bodies tebral ligaments, anchor to the cortex of the vertebral body by
strong Sharpey’s fibers (arrowheads). The anterior longitudinal
and a series of synovial joints (apophyseal joints) between ligament attaches to the anterior surface of the vertebral body
the vertebral arches. In addition, ligamentous connec- and is less adherent to the intervertebral disc. The posterior
tions exist both anteriorly (anterior longitudinal liga- longitudinal ligament is applied to the back of the intervertebral
ment, posterior longitudinal ligament) and posteriorly discs and vertebral bodies.
(ligamenta flava and interspinous, supraspinous, and (From Resnick D: Degenerative diseases of the vertebral
intertransverse ligaments). column. Radiology 156:3, 1985.)
394
Chapter 30 Degenerative Disease of the Spine 395
Additional ligaments are the ligamenta flava (connecting from the marrow of the vertebral bodies across the sub-
the laminae of adjacent vertebrae), supraspinous liga- chondral bone and cartilaginous endplate or through the
ments (connecting the apices of the spinous processes), anulus fibrosus from the surrounding blood vessels.
interspinous ligaments (connecting adjoining spinous Magnetic resonance (MR) imaging is a noninvasive
processes), intertransverse ligaments (connecting trans- means of determining the integrity of the intervertebral
verse processes), and ligamentum nuchae (connecting the disc. Although considerable data indicate that the signal
occipital protuberance and external occipital crest to the characteristics of a healthy disc differ from those of a
spine of the seventh cervical vertebra). diseased one, it is not entirely clear which components of
the intervertebral disc contribute most significantly to
these signal characteristics. Because degenerative changes
MAJOR TYPES OF DEGENERATIVE
of the disc are accompanied by alterations in both the
DISEASES OF THE SPINE
water and proteoglycan content of the tissue, either or both
Tables 30–1 and 30–2 list degenerative diseases of the spine. could be responsible for alterations in signal intensity. MR
imaging can be used to provide qualitative and quantitative
data that depend primarily on the water content of the
Cartilaginous Joints
intervertebral disc; therefore, it can monitor the bio-
The major components of the cartilaginous joints, the chemical aberrations that accompany the major degenera-
nucleus pulposus, anulus fibrosus, and cartilaginous tive diseases of the intervertebral disc.
endplates (see Fig. 30–1), are intimately related both
anatomically and physiologically. A vascular network that Intervertebral (Osteo)Chondrosis. Aging results in dehy-
supplies blood to the cartilaginous endplate and inter- dration and loss of tissue resiliency in the intervertebral
vertebral disc in infants and young children soon atrophies disc, particularly in the nucleus pulposus. This process
and may disappear by the age of 8 to 12 years. The has been termed intervertebral chondrosis or interver-
metabolism of the intervertebral disc becomes primarily tebral (osteo)chondrosis (Fig. 30–2). The cause of inter-
anaerobic and is dependent on diffusion of fluid either vertebral (osteo)chondrosis is not known. Changes are
observed in the nucleus pulposus, anulus fibrosus, and
hyaline cartilage endplate, although the findings in the
nucleus pulposus are most striking. The nucleus pulposus
TABLE 30–1 appears desiccated and friable, related to loss of water
Major Types of Degenerative Diseases of the Spine and proteoglycans. Clefts or crevices appear and extend
in an oblique or transverse fashion from the nucleus into
Cartilaginous joints (discovertebral junction)
the anulus fibrosus.
Intervertebral (osteo)chondrosis Even in the early stages of intervertebral (osteo)-
Spondylosis deformans chondrosis, radiographic findings of linear or circular
Uncovertebral (neurocentral) joints radiolucent collections—vacuum phenomena—appear
Arthrosis within the intervertebral discs. This finding is accen-
Synovial joints tuated in extension and may disappear in flexion. Distrac-
Apophyseal joint osteoarthritis tion of the intervertebral disc (which occurs in extension)
Costovertebral joint osteoarthritis increases the disc space and attracts gas from the
Fibrous joints and entheses surrounding extracellular fluid. In flexion, the space is
Diffuse idiopathic skeletal hyperostosis obliterated and the gas is resorbed. Vacuum phenomena
Ossification of the posterior longitudinal ligament are a reliable indicator of disc degeneration; they are very
Degeneration of the ligamenta flava and the supraspinous, rare in the presence of disc infection.
interspinous, and iliolumbar ligaments As the process of intervertebral (osteo)chondrosis pro-
gresses, the clefts or cavities enlarge and involve both the
TABLE 30–2
Degenerative Disorders of the Spine
With disc displacement through the endplate, fissures Fracture and fragmentation of vertebrae with intra-
may appear within the affected disc. These fissures course osseous vacuum phenomena have been described in cases
horizontally through the disc and extend vertically into the of ischemic necrosis, particularly in patients taking steroid
osseous defect. Gas collections within these clefts demon- medications. Visualization of the crescent sign, a radio-
strate a branching pattern, with both horizontal and ver- lucent region at the site of a linear or semicircular frac-
tical components, and gas may extend into the vertebral ture through the subchondral bone, is suggestive of bone
body at the site of displacement in a linear or arclike necrosis (Fig. 30–4) and is reminiscent of that accom-
form. Detection of the branching nature of the vacuum panying ischemic necrosis of the femoral or humeral head.
phenomenon and its vertical component suggests the Intraosseous gas collections in collapsed vertebral bodies,
presence of intervertebral disc displacement even when however, may occur in the absence of bone necrosis.
the osseous defect itself is not apparent. Displacement of Radiolucent areas representing gas in the apophyseal
portions of a degenerating intervertebral disc into the spinal joints of the spine, especially in the lumbar region, are
canal can be associated with gas formation in both the occasionally present in patients with osteoarthritis in this
disc and the canal, a finding that is readily apparent with location (Fig. 30–5). Similar areas have been noted in other
the use of computed tomography (CT) or MR imaging. degenerative joints, such as the sacroiliac articulations.
The observation of gas overlying the spinal canal adjacent Vacuum phenomena are a frequent finding in the ver-
to a degenerative intervertebral disc strongly supports tebral column and can be localized to the intervertebral
the diagnosis of intraspinal disc displacement. disc or, less commonly, the spinal canal, apophyseal joints,
398 Section VII Degenerative Diseases
TABLE 30–4
Types of Spinal Vacuum Phenomena
A B
Spondylosis Deformans
The most obvious pathologic and radiographic degener-
ative disease of the spine is associated with bone pro-
Figure 30–7. Normal and abnormal intervertebral disc: duction, particularly along the anterior and lateral aspects
sagittal T2-weighted (TR/TE, 3400/96) spin echo MR imaging of the vertebral column. The outgrowths are termed
technique. In discs that are relatively normal (L1–L2, L3–L4, osteophytes, although spondylosis deformans is currently
and L4–L5), a central portion of high signal intensity con- the most popular and accurate designation. Spinal osteo-
taining a horizontal line of low signal intensity is evident. In the
phytosis is extremely common. By the age of 50 years,
disc (L2–L3) with mild intervertebral (osteo)chondrosis, mini-
mal loss of signal intensity is seen, particularly in its anterior approximately 60% of women and 80% of men have such
third. With severe intervertebral (osteo)chondrosis (L5–S1), excrescences. These bony outgrowths are more frequent
the disc is of low signal intensity and diminished in height. A in men than in women and are more common in the
large posterior extruded disc (arrow) with low signal intensity is older population. Any segment of the vertebral column
also evident. can be affected; in the thoracic spine, right-sided out-
400 Section VII Degenerative Diseases
A B
Figure 30–8. Abnormal intervertebral disc: Modic type I change. A, Sagittal T1-weighted (TR/TE, 650/15)
spin echo MR image shows decreased signal intensity (arrows). B, Sagittal T2-weighted (TR/TE, 4400/108)
spin echo MR image shows increased signal intensity (arrows).
A B
Figure 30–9. Abnormal intervertebral disc: Modic type II change. A, Sagittal T1-weighted (TR/TE,
663/12) spin echo MR image shows increased signal intensity (arrows). B, Sagittal T2-weighted (TR/TE,
4630/138) spin echo MR image shows isointense or slightly increased signal intensity (arrows).
growths predominate, presumably related to pulsations initiating factor in this disorder. Once the attachment
of the descending aorta on the left side. between the anulus fibrosus and the vertebral rim is
Schmorl’s concept of the pathogenesis of spondylosis disrupted, even though this attachment is related to the
deformans, with some modification, is generally accepted presence of calcified cartilage, minor degrees of anterior
today (Fig. 30–11). This concept emphasizes abnormali- and anterolateral disc displacement are possible and may
ties in the peripheral fibers of the anulus fibrosus as the lead to traction at the site of osseous attachment of the
Chapter 30 Degenerative Disease of the Spine 401
A B
outermost fibers of the anulus fibrosus or short peri- chondrosis. In this condition, enlarging tears of the nucleus
vertebral ligaments to the vertebral surfaces. Osteophytes pulposus may extend into the anulus fibrosus and even-
develop at this location, several millimeters from the tually lead to the disruption of annular attachments to bone.
discovertebral junction (Fig. 30–12). The type and severity The changes of spondylosis deformans are then superim-
of clinical manifestations related to spinal osteophytes posed on those of intervertebral (osteo)chondrosis.
depend on the size and location of the outgrowths and Additional findings of intervertebral (osteo)chondrosis
their relationship to adjacent soft tissue and neurologic are seen, including loss of disc integrity with diminution
structures. Dysphagia, stiffness, restricted motion, and of intervertebral disc height, approximation of adjacent
even pain can probably be attributed to spinal osteophytes osseous surfaces, and reactive sclerosis and bone forma-
in some patients. Neurologic deficits produced by bony tion. Diffuse idiopathic skeletal hyperostosis is associated
impingement on the spinal cord are unusual. with extensive ossification along the anterior and lateral
Spinal osteophytes accompanying spondylosis defor- aspects of the vertebral column, resembling the features
mans must be differentiated from other bony outgrowths of severe spondylosis deformans. Ankylosing spondylitis
of the vertebral column (Fig. 30–13). Small triangular is characterized by ossification within the outer portion
osteophytes are encountered in intervertebral (osteo)- of the anulus fibrosus, resulting in outgrowths (syndesmo-
402 Section VII Degenerative Diseases
Figure 30–11. Spondylosis deformans: progressive stages. The normal situation is depicted in (1). The
anulus fibrosus is attached to the vertebral rim by calcified cartilage (double-headed arrows) and to the anterior
vertebral surface by Sharpey’s fibers (arrowheads). The anterior longitudinal ligament (open arrows) is
connected to the anterior vertebral surface. In (2), breakdown in sites of attachment of the anulus fibrosus
to the vertebral rim is evident (arrows), although Sharpey’s fibers (arrowheads) are intact. Mild anterior disc
displacement is seen. With progression of disease, as shown in (3), osteophytes (arrowheads) develop at the
site of attachment of Sharpey’s fibers to the anterior vertebral surface. The anterior longitudinal ligament
(open arrows) is stretched by the displaced intervertebral disc. With still further progression, as in (4a), an
osteophyte may bridge the intervertebral disc (arrowheads). Alternatively, as in (4b), continued traction on
the anterior longitudinal ligament may lead to proliferative enthesopathy, with the production of new bone
(open arrows) at its site of attachment to the anterior vertebral surface, combined with more typical
osteophytes (arrowheads).
(3, From Resnick D: Degenerative diseases of the vertebral column. Radiology 156:3, 1985.)
phytes) that are typically thin and vertically oriented as diagnosis. Additional causes of bony excrescences of the
they extend from one vertebral body to the next. Bony spine that may resemble or are identical to the osteo-
outgrowths in psoriasis and Reiter’s syndrome may resem- phytes of spondylosis deformans are hypoparathyroidism,
ble the typical syndesmophytes of ankylosing spondylitis sternocostoclavicular hyperostosis, acne conglobata,
or appear as distinct paravertebral ossifications. In the paralysis, and certain medications such as vitamin A
latter circumstance, they are separated from the vertebral derivatives.
surface and, initially, possess a poorly defined or irregular
contour.
Uncovertebral (Neurocentral) Joint Arthrosis
Osseous excrescences of the spine accompanying neu-
ropathic osteoarthropathy and ochronosis do not resemble The five lowest cervical vertebral bodies (C3 to C7) con-
the outgrowths of spondylosis deformans. Spinal infec- tain bony ridges extending from each side—the uncinate,
tion or trauma can produce outgrowths that are identical or lunate, processes. Between the superior process of the
to those of spondylosis deformans; however, they are usually lower vertebral body and the inferior process of the
localized to one (or two) sites. Fluorosis leads to osteo- upper vertebral body is a joint termed the uncovertebral
phytosis identical to that in spondylosis deformans, but or neurocentral joint, or joint of Luschka.
additional radiographic findings (e.g., increased osseous With increasing intervertebral disc degeneration,
density and ligamentous ossification) allow an accurate progressive loss of disc space height occurs, and the bony
Chapter 30 Degenerative Disease of the Spine 403
A B C
Figure 30–14. Uncovertebral (neurocentral) joint arthrosis: radiographic abnormalities. Frontal
(A), oblique (B), and lateral (C) radiographs show arthrosis of the uncovertebral joints, particularly at the
C5–C6 level, that has led to narrowing of the corresponding intervertebral foramen (B) and a radiolucent
line (arrow in C) simulating a fracture.
A B
Figure 30–15. Apophyseal joint osteoarthritis: lumbar spine. A, Lateral radiograph of a cadaveric spine
shows considerable alterations of osteoarthritis involving the apophyseal joints between the fourth and fifth
lumbar vertebrae and between the fifth lumbar vertebra and the sacrum. Joint space narrowing, bone
sclerosis, and osteophytes are apparent. B, Radiograph of a transverse section at the approximate level
indicated by the double-headed arrow in (A) reveals the osteoarthritic changes to better advantage. Findings
include joint space narrowing, vacuum phenomenon (arrow), and bone fragmentation in the articular
processes. Although the neural foramina are narrowed, there is no impingement on the nerve roots
(arrowheads), which have passed through the foramina at a higher level.
(B, From Resnick D: Degenerative diseases of the vertebral column. Radiology 156:3, 1985.)
the relationship of bone proliferation to the central spinal presumably related to stress-induced marrow edema.
canal, lateral recesses, and neural foramina can be defined. Prominent clinical symptoms and signs may accompany
The presence of a vacuum phenomenon in the osteo- osteoarthritis of the apophyseal joints. The synovial
arthritic apophyseal joint leads to an arclike radiolucent membrane, capsules, and ligaments of these articulations
collection between the articular processes. In some patients are richly supplied with nerves, which may explain the
with osteoarthritis of the apophyseal joints, MR imaging common but controversial occurrence of pain in patients
reveals altered signal intensity in the adjacent pedicle, with degenerative joint disease at these sites.
Chapter 30 Degenerative Disease of the Spine 405
Costovertebral Joint Osteoarthritis (Osteoarthrosis). The tered. Newly formed articulations may exist between the
costovertebral joints are located between the heads of the enlarged transverse process of the involved vertebra and
ribs and the vertebral bodies and between the necks and the wings of the sacrum (or, infrequently, the ilium). These
tubercles of the ribs and the transverse processes of the articulations have a bilateral or unilateral distribution.
vertebrae (costotransverse joints). Degenerative changes Osteoarthritis can result from abnormal stress and move-
predominate in the articulations of the 11th and 12th ribs. ment at the various joints. Articular space narrowing, scle-
Joint space narrowing, bony eburnation, and osteophy- rosis, and osteophytosis are evident. Bony ankylosis may
tosis are the expected alterations (Fig. 30–16), although eventually occur. The relationship of back pain to
radiographic demonstration of these changes is difficult congenital transitional vertebrae at the lumbosacral junc-
because of the adjacent osseous shadows of the vertebral tion is not clear. The relationship between transitional
bodies and ribs. Osteoarthritis of the costovertebral joints lumbosacral joints and posteriorly extruded discs is also
may lead to partial or complete bone bridging between debated.
the vertebrae and ribs This finding is associated with
pagetoid, or Paget’s disease–like, cortical thickening and Osteoarthritis (Osteoarthrosis) of Median and Lateral
hyperostosis of the posterior portion of the affected ribs. Atlantoaxial Joints. Degenerative abnormalities in the
The pathogenesis of these bone changes appears to be anterior median atlantoaxial joint (Fig. 30–17) are reported
stress exerted by muscles affixed to ribs made hypomobile infrequently. They are generally regarded as incidental
or immobile by the exuberant osteophytosis. findings. On radiographs and conventional tomograms in
the lateral projection, narrowing of the interosseous space
Osteoarthritis (Osteoarthrosis) of Transitional Lumbosacral is observed between the anterior arch of the atlas and the
Joints. Congenital variations at the lumbosacral junction odontoid process, frequently combined with cortical
are frequent, and many transitional patterns are encoun- thickening and osteophytosis. CT findings of osteoarthritis
of the anterior median atlantoaxial joint include osteo-
phytes of the median facet of the atlas, small ossicles, and
calcification of the transverse ligament. Such ligament calci-
fication may also be a manifestation of calcium pyrophos-
phate dihydrate crystal deposition disease.
B
Figure 30–16. Costovertebral joint osteoarthritis: hyperos-
tosis of adjacent ribs. A, Transaxial CT scan reveals a bridge of
bone extending from the rib to the anterior surface of the ver-
tebral body. Note the cortical thickening of the right rib. B, In Figure 30–17. Osteoarthritis of the atlantoaxial joints.
a different patient, transaxial CT scan shows severe disease of Reformatted coronal CT image reveals a minor offset between
both costotransverse joints, with bone ankylosis on the left and the atlas and the axis and considerable joint space narrowing
costovertebral bone proliferation on the right. (A, Courtesy of and bone sclerosis in the left lateral atlantoaxial joint (arrow).
J. Haller, MD, Vienna, Austria.) (Courtesy of L. Kaseff, MD, Burlingame, Calif.)
406 Section VII Degenerative Diseases
Thoracic Spine
Degenerative diseases of the lumbar and cervical spine
have received more attention than those of the thoracic
spine. Spondylosis deformans predominates in the
middle and lower thoracic region, whereas intervertebral
(osteo)chondrosis has a predilection for the midthoracic
area. Obviously, costovertebral osteoarthritis is confined
to the thoracic region.
Lumbar Spine
Degenerative changes in the lumbar spine are frequent.
Intervertebral (osteo)chondrosis, spondylosis deformans,
or osteoarthritis of the apophyseal joints is detected on
Figure 30–18. Baastrup’s disease. Massive enlargement and pathologic examination in 60% to 80% of men and
prominent sclerosis of the spinous processes are evident. women by the sixth decade of life and in about 100% of
(Courtesy of C. Boles, MD, Hillside, Ill.) subjects by age 70 years. These changes predominate in
Chapter 30 Degenerative Disease of the Spine 407
the lower lumbar region, particularly between the fourth asymmetry in the ability to bend in both directions, loss
lumbar and first sacral segments. Disc space narrowing is of normal vertebral rotation and tilt, abnormal degree of
very common in these areas, although assessment of disc disc closure or opening, malalignment of spinous processes
space loss at the L5–S1 level is difficult because of the and pedicles, and lateral translation of one vertebra on
common occurrence of anatomic variations at this another because of an abnormal degree of rotation. The
segment and discrepancies in the height of the anterior traction osteophyte is an important sign of spinal insta-
and posterior portions of the L5–S1 disc (posterior disc bility (Fig. 30–21). This osseous excrescence arises on the
height is a more reliable indicator of the status of the anterior surface of the vertebral body, several millimeters
disc). The clinical significance of radiographic evidence from the discovertebral junction, and extends in a hori-
of degenerative disease in the lumbar spine is debated. zontal direction, developing at the site of attachment of
the strong outermost fibers of the anulus fibrosus to the
vertebral surface.
COMPLICATIONS OF DEGENERATIVE
DISEASES OF THE SPINE Degenerative Anterior Spondylolisthesis. The term
spondylolysis refers to an interruption of the pars inter-
Alignment Abnormalities
articularis of the vertebra. Although the cause of spondy-
Segmental Instability. Assessment of the degree and lolysis (Table 30–5) has been debated, most investigators
pattern of motion in the lumbar spine is possible with now believe that it is an acquired abnormality characterized
appropriate radiographs. In general, lateral radiographs by a mechanical failure of bone related to abnormal verte-
with the patient in the neutral position and during spinal bral stress. The term spondylolisthesis refers to displacement
flexion and extension are obtained. Some investigators of one vertebra on another. Although spondylolisthesis
suggest the addition of frontal radiographs in the neutral often occurs with spondylolysis (Fig. 30–22), it may occur
attitude and with the patient bent to either side. General without it, often in association with degenerative disease.
radiographic findings suggestive of instability include the Degenerative anterior spondylolisthesis occurs in
presence of gas within the intervertebral disc, osteophytes approximately 4% of elderly patients and predominates
on adjacent vertebral bodies below the rims of the end- at the interspace between the fourth and fifth lumbar
plate (traction osteophytes), and evidence of a radial fissure vertebrae in older women. The facets at the L4–L5 level
in the intervertebral disc during discography. Lateral are oriented more sagittally than those at the L5–S1 level
radiographs obtained in flexion and extension should be and are therefore more capable of allowing anterior move-
regarded as positive when they reveal forward or back- ment. In the presence of degenerative joint changes, this
ward displacement of one vertebra with respect to another sagittal orientation may become even more striking because
(Fig. 30–20), an abrupt change in the length of the pedi- of bone remodeling about the degenerative apophyseal
cles, narrowing of the intervertebral foramina, and loss of joint. The check of one facet lying behind the other
height of an intervertebral disc. On frontal radiographs becomes deficient, and the inferior facets of the fourth
obtained with the patient bending first in one direction lumbar vertebra gradually erode between the superior
and then in the other, additional abnormalities include facets of the fifth lumbar vertebra and produce forward
A B
408 Section VII Degenerative Diseases
TABLE 30–5
Spondylolisthesis
A B
Figure 30–22. Spondylolisthesis with spondylolysis. A, Bilateral defects through the pars interarticularis
allow anterior displacement of the vertebral body with respect to its neighbor. The alignment of the
apophyseal joints is normal. B, Observe the anterior spondylolisthesis of the fifth lumbar vertebral body on
the sacrum. The pars defects are not well delineated. Note the considerable intervertebral disc space
narrowing, sclerosis, and fragmentation of the osseous surfaces.
kyphosis, the failure occurs in the weakened vertebral loss of height of the anterior portion of the lumbar
bodies. It is the wedgelike vertebral deformities of osteo- vertebral bodies. The initiating event in this condition is
porotic kyphosis that prevent the development of disc not clear.
deterioration. Both senile and osteoporotic thoracic
kyphosis can occur in the same patient.
Intervertebral Disc Displacement
Degenerative Lumbar Scoliosis. Scoliosis may occur in
the lumbar spine of elderly persons. Degenerative lumbar Normally, the intervertebral disc is a load-bearing struc-
scoliosis (Fig. 30–25) in elderly patients may progress, ture with hydrostatic properties related to its high water
generally at a slow rate, although it occasionally develops content. The nucleus pulposus consists of about 85%
in a rapid fashion. Its relationship to significant symptoms water, and the anulus fibrosus consists of 78% water; these
and signs, as well as to idiopathic scoliosis of adolescence, percentages decrease to approximately 70% with degenera-
is debated. Typically, degenerative diseases of the spine tion. The anatomic arrangement of a centrally located
do not lead to the development of scoliosis, although nucleus pulposus surrounded by concentric lamellae of
they may appear during the course of scoliosis. the anulus fibrosus allows the conversion of axial loading
forces to tensile strain on the annular fibers and cartilagi-
Degenerative Lumbar Kyphosis. Although the occurrence nous endplates. Disc pressure becomes elevated in the
of increased thoracic kyphosis and decreased lumbar presence of abnormal externally applied loads. As the
lordosis in older persons is well recognized, marked loss nucleus is subjected to elevated pressure, it attempts to
of lordosis or even reversal of lordosis (i.e., kyphosis) in prolapse from its confined space. The direction of this
the lumbar spine as a degenerative phenomenon has been prolapse is variable; the intervertebral disc may be dis-
emphasized only recently. Affected persons walk in a placed anteriorly and laterally (spondylosis deformans),
forward-bending posture and complain of low back pain. posteriorly or posterolaterally (intraspinal or intraforami-
Radiographs reveal a marked loss of sacral inclination, nal displacement), or superiorly and inferiorly (cartilagi-
marked narrowing of the lumbar intervertebral discs, and nous nodes) (Table 30–7; Fig. 30–26).
410 Section VII Degenerative Diseases
A B
Figure 30–24. Spondylolisthesis without spondylolysis: degenerative retrolisthesis. A, As the interver-
tebral disc space narrows because of intervertebral (osteo)chondrosis, telescoping of the apophyseal joints
allows backward displacement of the upper lumbar vertebra with respect to the lower one. B, Retrolisthesis
of L2 in relation to L3 (arrowhead) is related to severe intervertebral (osteo)chondrosis in the intervening
intervertebral disc.
(B, From Resnick D: Degenerative diseases of the vertebral column. Radiology 156:3, 1985.)
TABLE 30–6
Senile versus Osteoporotic Kyphosis
Disc protrusion: The displaced nucleus pulposus extends The features of disc herniation have been well
through some of the fibers of the anulus fibrosus but described. Detection of abnormalities depends on central
is still confined by the intact outermost fibers. or lateral localization, as well as the extent of posterior
Disc extrusion: The displaced nucleus pulposus penetrates displacement. Centrally located herniated discs that lie
all the fibers of the anulus fibrosus and lies under the beneath the posterior longitudinal ligament (i.e., disc
posterior longitudinal ligament. extrusion) produce a smooth, focal, radiodense area that
deforms or displaces the dural sac. When nuclear mate-
Disc sequestration: The displaced nucleus pulposus pene- rial penetrates this ligament or extends around it (i.e.,
trates or extends around the posterior longitudinal liga- disc sequestration), a soft tissue mass is evident within
ment and lies within the epidural space, or the displaced the epidural fat. Inferior migration of the sequestered
nucleus pulposus, although not extending through this fragment is slightly more common than superior
ligament, migrates for a considerable distance in a migration is.
cephalad or caudad direction as a fragment that is sepa- Although extension of central disc displacement into a
rate from the remaining portion of the intervertebral neural foramen is unusual, extreme lateral displacement,
disc. which represents approximately 12% of all protrusions
If disc herniation is defined as a situation in which the and extrusions and is most frequent at the L4–L5 spinal
nucleus pulposus extends through some or all of the fibers level, presents a diagnostic challenge. MR imaging is
of the anulus fibrosus, disc prolapse, extrusion, and seques- ideal to define the type or extent of disc displacement
tration would all be forms of herniation (Fig. 30–32). (prolapse, extrusion, or sequestration). Disc protrusions
412 Section VII Degenerative Diseases
TABLE 30–8
Conditions Associated with Cartilaginous (Schmorl’s)
Nodes
Intervertebral (osteo)chondrosis
Scheuermann’s disease (juvenile kyphosis)
Trauma
Hyperparathyroidism
Osteoporosis
Infection
Neoplasm
TABLE 30–7
Disc Displacement
B C D
A B
A B C
A B C
Figure 30–32. Posterior disc extrusion. Extrusion of disc
material can occur centrally (A), eccentrically (B), or laterally
(C). The precise direction of displacement influences the
clinical manifestations by affecting different portions of the
spinal cord and nerve roots. With lateral extrusions, CT or MR
D E F G imaging is diagnostically superior to myelography.
Figure 30–31. Posterior disc displacement. A to C, Annular
bulge in three different patterns. D, Disc protrusion. The dis-
placed nucleus pulposus is still confined by the outermost
annular fibers. E, Disc extrusion. The displaced nucleus
pulposus has violated the outer fibers of the anulus fibrosus but
is still confined by the posterior longitudinal ligament. F, Disc
sequestration. The displaced nucleus pulposus has penetrated
pathologic findings is far from perfect. Currently, the
the posterior longitudinal ligament and lies within the epidural
space. A nearby disc fragment is seen. G, Disc sequestration. A consensus is that imaging examinations are most useful in
fragment of the displaced disc has migrated beneath the patients who are completely disabled from pain, have
posterior longitudinal ligament in a cephalad or caudad objective neurologic findings, or have symptoms that merit
direction and is separated from the remaining portion of the consideration of surgery or hospital admission. They are
intervertebral disc. less useful in patients with stable back pain only.
Chapter 30 Degenerative Disease of the Spine 415
location and extends cephalad to the intervertebral disc
space.
CT provides far more diagnostic information and
typically shows a soft tissue mass adjacent to a degen-
erative apophyseal joint; the mass may contain gas or may
have a partially or completely calcified rim (Fig. 30–37).
CT scans obtained after the injection of contrast material
into the adjacent apophyseal joint may show opacifica-
tion of the cystic lesion. Indeed, in cases in which the
apophyseal joint and synovial cyst communicate freely,
injection of corticosteroid medication into the joint may
provide effective therapy by passing into the cyst itself.
The MR imaging findings of intraspinal synovial cysts
include a mass lesion with variable signal intensity, signal
void related to the presence of gas, and degenerative
changes with fluid or gas collections in the apophyseal
joints. Enhancement of signal intensity in the rim of the
cyst may be seen after the intravenous injection of a
gadolinium-based contrast agent.
A B
C D
Figure 30–34. Posterior disc displacement: MR imaging findings. A and B, Sagittal (A) and transaxial
(B) T1-weighted (TR/TE, 517/20 and 650/20, respectively) spin echo MR images reveal a large sequestered
disc at the L3–L4 spinal level. The displaced disc (arrows), still continuous with the parent disc, has migrated
inferiorly and is located behind the fourth lumbar vertebral body. Its signal intensity is similar to that of the
parent disc in these images. Degeneration of multiple lumbar intervertebral discs is seen. C and D, Sagittal
intermediate-weighted (TR/TE, 2000/20) (left image in C) and T2-weighted (TR/TE, 2000/80) (right
image in C) spin echo MR images and transaxial intermediate-weighted (TR/TE, 2000/20) (top image in D)
and T2-weighted (TR/TE, 2000/80) (bottom image in D) spin echo MR images reveal massive disc
displacement (arrows) at the L4–L5 spinal level. Note that the signal intensity of the displaced disc is similar
to that of the parent disc on the intermediate-weighted images and is lower than that of cerebrospinal fluid
on the T2-weighted images. (C and D, Courtesy of J. Kirkham, MD, Minneapolis, Minn.)
A B
may also appear after surgical fusion or spontaneous weeks after only symptomatic treatment. Further, the
ankylosis. calcification usually disappears within a few weeks to
Calcification localized to one or two disc levels is months.
encountered in two broad clinical situations. In adults, It is evident that the clinical and radiographic charac-
chronic degenerative calcific deposits may occur in the teristics of localized disc calcification depend on the age
anulus fibrosus, nucleus pulposus, or cartilaginous end- of the patient. In adults, calcification predominates in the
plates, particularly in older men and in the midthoracic anulus fibrosus of the thoracic and lumbar intervertebral
and upper lumbar regions of the spine. In an autopsy discs and is usually asymptomatic and permanent; in
study, the reported frequency of such calcification in the children, calcification predominates in the nucleus pul-
anulus fibrosus was 71%; in the nucleus pulposus, it was posus of the cervical spine and is usually symptomatic
6.5%. and transient. The temporary nature of this phenomenon
The second situation associated with disc calcification in children may relate to calcific resorption through vas-
is observed in children (Fig. 30–40). Such calcification is cular channels, which are normally present in the carti-
most common between 6 and 10 years of age, although it laginous plates in children.
may occur at any age from birth to early adulthood. It
affects boys and girls with equal frequency. The cervical Fibrosis, Vascularization, and Ossification. Disc degener-
spine is affected most commonly. Symptoms, which may ation or trauma may be followed by proliferation of
be evident in as many as 75% of children with disc fibrous tissue and blood. Hypervascularity may stimulate
calcification, include pain, stiffness, limitation of motion, ossification (Fig. 30–42) and produce radiodense lesions
and torticollis. Fever, leukocytosis, and elevation of the possessing trabeculae that overlie the intervertebral disc.
erythrocyte sedimentation rate may be apparent. On These ossifications, which appear within the nucleus
radiographs, the calcification involves predominantly the pulposus or the anulus fibrosus, may be displaced poste-
nucleus pulposus and produces single or multiple, oval or riorly into the spinal canal or extend from the adjacent
flat, dense areas. Rupture of these calcific collections vertebral body, similar to osteophytes (spondylosis
into the vertebral bodies (cartilaginous nodes), interver- deformans).
tebral foramina, spinal canal, or adjacent soft tissues may
be evident. Anterior displacement of the calcified disc
may lead to dysphagia, and posterior and posterolateral Spinal Stenosis
displacement may lead to neurologic findings. The A narrow spinal canal may be present at birth. This
displaced and calcified discs may diminish in size with stenosis can later be accentuated by developmental
time. narrowing produced by postnatal growth abnormalities
MR imaging reveals expansion of affected discs and and by acquired narrowing associated with various
inhomogeneity of disc signal intensity, with areas of signal disease processes. Degenerative disorders of the vertebral
void in regions of calcification. Of interest, disc expan- column (osteoarthritis) lead to hypertrophic alterations
sion may also be evident at other spinal levels that do not about involved joints that may compromise the spinal
show radiographically evident calcification (Fig. 30–41). contents.
The prognosis of disc calcification in children is excel- Narrowing of the spinal canal may occur in the absence
lent. In most cases, pain resolves within a few days to of significant extraspinal alterations in the lumbar, tho-
418 Section VII Degenerative Diseases
A B
C D
Figure 30–36. Annular tears: discographic, MR imaging, and MR discographic findings. A, In a cadaveric
spine, a sagittal fat-suppressed T2-weighted (TR/TE, 3000/108) fast spin echo MR image of the L1–L2
level reveals linear regions of high signal intensity in the nucleus pulposus near each vertebral endplate.
B, Conventional discography reveals contrast material collecting in these annular tears. C, MR discography
using a sagittal fat-suppressed T1-weighted (TR/TE, 500/8) spin echo MR image obtained after the
intradiscal injection of a gadolinium-containing contrast agent reveals the full extent of the annular tears.
D, Photograph of a sagittal section of the spine confirms the presence of these annular tears. (Courtesy of
Y. Kakitsubata, MD, Miyazaki, Japan, and D. Theodorou, MD, Ioannina, Greece.)
racic, or cervical segments of the vertebral column in both (e.g., central, lateral recess, or foraminal degenerative
adults and children. The classification of spinal stenosis spondylolisthesis), iatrogenic stenosis (e.g., following
takes into account its various causes and includes cate- laminectomy, discectomy, or arthrodesis), traumatic steno-
gories of congenital or developmental stenosis (e.g., idio- sis, and miscellaneous causes of stenosis (e.g., acromegaly,
pathic, achondroplasia, osteopetrosis) and acquired stenosis. Paget’s disease, fluorosis, or ankylosing spondylitis)
The latter is further divided into degenerative stenosis (Fig. 30–43).
Chapter 30 Degenerative Disease of the Spine 419
is 13 mm or more. Measurement between these osseous
landmarks on radiographs does not always provide an
accurate appraisal of the width of the spinal cord, how-
ever. Further, magnification errors may be introduced
during routine radiography. These errors can be elimi-
nated by using a ratio calculated by dividing the antero-
posterior diameter of the spinal canal by the anteroposterior
diameter of the vertebral body; normally, this ratio is
about 1, with a value of 0.8 or less being indicative of
developmental canal stenosis. Cervical spinal stenosis can
be related to many congenital factors (anomalies, dys-
plasias, malformations, subluxations) and can be acquired
in traumatic, infectious, neoplastic, and metabolic dis-
orders. Cervical spinal stenosis also develops in degen-
erative articular diseases, such as spondylosis deformans
and osteoarthritis, as well as in additional degenerative
processes, such as ossification of the posterior longitu-
Figure 30–37. Intraspinal synovial cyst: lumbar spine. On a
CT scan, observe a mass adjacent to the apophyseal joint. It dinal ligament (see Chapter 32).
contains gas (arrow).
Thoracic Spinal Stenosis. Significant spinal stenosis in
the thoracic segment related to degenerative articular
processes is much less frequent than is stenosis in the
cervical or lumbar segments. Occasionally, osteophytes
developing at the apophyseal joint or discovertebral
junction and thickening of the laminae may lead to
Although symptoms and signs are occasionally narrowing of the thoracic spinal canal and encroachment
encountered in the young, they are far more frequent in of neural tissue.
middle-aged and elderly persons, in whom degenerative
abnormalities may further compromise a congenitally Lumbar Spinal Stenosis. As in the cervical spine, congenital
narrowed spinal canal. Clinical manifestations of spinal and developmental factors may lead to a narrowed lumbar
stenosis likewise vary according to the vertebral segment spinal canal (see Fig. 30–43), which can be compromised
affected. further by acquired alterations related to many disease
processes. With developmental stenosis, characteristic
Cervical Spinal Stenosis. Radiographic measurement of alterations include narrowing of the anteroposterior and
the sagittal diameters of the spine (measured from the interpediculate diameters of the lower two or three
posterior surface of the vertebral body to the spinolaminar lumbar vertebrae, vertical orientation of the laminae, and
line) may be used to detect stenosis of the cervical canal; extreme diminution of the interlaminar space. These
cord compression may occur in adults if this diameter is osseous abnormalities can be detected radiographically
10 mm or less and is unlikely to occur if this diameter by measuring the anteroposterior (midsagittal) diameter
A B
420 Section VII Degenerative Diseases
A B C
D E
Figure 30–39. Patterns of sclerosis of the vertebral body. A, Sandwich vertebral body: osteopetrosis tarda.
A bone-within-bone appearance is created by well-defined radiodense bands. B, Rugger-jersey vertebral
body: renal osteodystrophy. Bands of increased radiodensity are evident at the top and bottom of each
vertebral body. C, Ivory vertebral body: skeletal metastasis (carcinoma of the prostate). The entire vertebral
body is radiodense and, on pathologic inspection, contains cartilaginous nodes. D, Picture-frame vertebral
body: Paget’s disease. The peripheral portions of the vertebral body are radiodense in comparison with the
central portion. E, Marginal condensation: intervertebral (osteo)chondrosis. Triangular region of sclerosis
borders on a narrowed intervertebral disc. (A, Courtesy of P. Kaplan, MD, Boston, Mass.)
(the distance from the posterior surface of the vertebral intervertebral disc material, hypertrophy and subluxation
body to the base of the superior portion of the spinous of the articular facets, enlarged laminae, and hyperplasia
process) and the transverse diameter (the distance between or ossification of the ligamenta flava lead to further
the inner aspect of the pedicles) of the spinal canal. The encroachment on the canal and may produce clinical
lower limit of normal for the midsagittal diameter of the symptoms and signs in a patient whose spine has already
five lumbar vertebrae in men and women is 15 mm; for been compromised by developmental stenosis. Although
the transverse diameter, it is 20 mm. Measurements less these alterations are usually apparent on radiographs,
than these values indicate the presence of lumbar spinal their exact location and extent can be delineated more
canal stenosis. Most investigators regard an anteropos- fully by CT or MR imaging. Using these techniques,
terior spinal diameter less than 12 mm as unequivocally spinal stenosis in the lumbar segment can be further
pathologic. On CT scans, anteroposterior diameters less divided into three groups on the basis of its anatomic
than 11.5 mm, interpediculate distances less than 16 mm, location: stenosis of the central canal; stenosis of the
and canal cross-sectional areas less than 1.45 cm2 are subarticular, or lateral, recesses; and stenosis of the
considered small. Although these CT measurements of neural foramina.
the central canal are useful as guidelines, they should not The central canal is normally round or slightly oval
be applied too rigorously. in cross section. CT findings of central canal stenosis
Degenerative processes contribute to stenosis of the include distortion of its normal configuration, com-
lumbar spinal canal. Ventral osteophytes, prolapse of pression of the thecal sac in an anteroposterior direction,
Chapter 30 Degenerative Disease of the Spine 421
A B
C
Figure 30–43. Patterns of lumbar spinal stenosis. The normal situation is indicated in (1). Observe the
stenosis caused by laminar thickening (2) and by both anterior and posterior bony overgrowth (3), a situation
resulting in the trefoil, or fleur-de-lis, appearance.
Chapter 30 Degenerative Disease of the Spine 423
Figure 30–44. Central spinal stenosis. Axial T1-weighted Figure 30–45. Lateral recess stenosis. Axial TSE T2-
(TR/TE, 520/20) spin echo MR image demonstrates narrowing weighted (TR/TE, 4200/110) spin echo MR image shows
of the anteroposterior and interpediculate diameters, with marked lateral recess stenosis.
hypertrophy of the ligamentum flavum, facet hypertrophy, and
disc bulging.
FURTHER READING Maldague BE, Noel HM, Malghem JJ: The intravertebral
vacuum cleft: A sign of ischemic vertebral collapse. Radiology
Adams MS, McNally DS, Dolan P: “Stress” distributions inside 129:23, 1978.
intervertebral discs: The effects of age and degeneration. J Marcelis S, Seragini FC, Taylor JAM, et al: Cervical spine:
Bone Joint Surg Br 78:965, 1996. Comparison of 45 and 55 anteroposterior oblique radio-
Bick EM: Vertebral osteophytosis: Pathologic basis of its graphic projections. Radiology 188:253, 1993.
roentgenology. AJR Am J Roentgenol 73:979, 1955. Martel W, Seeger JF, Wicks JD, et al: Traumatic lesions of
De Maeseneer M, Lenchik L, Everaert H, et al: Evaluation the discovertebral junction in the lumbar spine. AJR Am J
of lower back pain with bone scintigraphy and SPECT. Roentgenol 127:457, 1976.
Radiographics 19:901, 1999. McCullough JA: Chemonucleolysis: Experience with 2000
DePalma A, Rothman R: The Intervertebral Disc. Philadelphia, cases. Clin Orthop 145:138, 1980.
WB Saunders, 1970. Modic MT, Masaryk T, Boumphrey F, et al: Lumbar herniated
Dorwart RH, Vogler JB, Helms CA: Computed tomography of disk disease and canal stenosis: Prospective evaluation by
spinal stenosis. Radiol Clin North Am 21:301, 1983. surface coil MR, CT, and myelography. AJR Am J Roentgenol
Edelman RR, Shoukimas GM, Stark DD, et al: High-resolution 147:757, 1986.
surface-coil imaging of lumbar disk disease. AJR Am J Modic MT, Masaryk TJ, Ross JS, et al: Imaging of degenerative
Roentgenol 144:1123, 1985. disc disease. Radiology 168:177, 1988.
Epstein JA, Epstein BS, Jones MD: Symptomatic lumbar Modic MT, Steinberg PM, Ross JS, et al: Degenerative disk
scoliosis with degenerative changes in the elderly. Spine disease: Assessment of changes in vertebral body marrow
4:542, 1979. with MR imaging. Radiology 166:193, 1988.
Francois RJ: Ligament insertions into the human lumbar verte- Morrison JL, Kaplan PA, Dussault RG, et al: Pedicle marrow
bral body. Acta Anat 91:467, 1975. signal intensity changes in the lumbar spine: A manifestation
Hadley LA: Anatomico-Roentgenographic Studies of the Spine, of facet degenerative joint disease. Skeletal Radiol 29:703,
2nd ed. Springfield, Ill, Charles C Thomas, 1973, p 447. 2000.
Hemminghytt S, Daniels DL, Williams AL, et al: Intraspinal Muhle C, Metzner J, Weinert D, et al: Classification system
synovial cysts: Natural history and diagnosis by CT. Radiology based on kinematic MR imaging in cervical spondylitic
145:375, 1982. myelopathy. AJNR Am J Neuroradiol 19:1763, 1998.
Inoue H, Ohmori K, Miyasaka K, et al: Radiographic evalua- Pech P, Haughton VM: Lumbar intervertebral disk: Correlative
tion of the lumbosacral disc height. Skeletal Radiol 28:638, MR and anatomic study. Radiology 156:699, 1985.
1999. Resnick D, Niwayama G: Intervertebral disc herniations: Carti-
Jacobson HG, Tausend ME, Shapiro JH, et al: The “swayback” laginous (Schmorl’s) nodes. Radiology 126:57, 1978.
syndrome. AJR Am J Roentgenol 79:677, 1958. Resnick D, Niwayama G, Guerra Jr, et al: Spinal vacuum
Jones RAC, Thomson JLG: The narrow lumbar canal: A clinical phenomena: Anatomical study and review. Radiology
and radiological review. J Bone Joint Surg Br 50:595, 1968. 139:341, 1981.
Kirkaldy-Willis WH, Wedge JH, Yong-Hing K, et al: Pathology Schmorl G, Junghanns H: The Human Spine in Health and
and pathogenesis of lumbar spondylosis and stenosis. Spine Disease, 2nd ed. Trans EF Besemann. New York, Grune &
3:319, 1978. Stratton, 1971, p 138.
Knutsson F: The vacuum phenomenon in the intervertebral Schweitzer ME, El-Noueam KI: Vacuum disc: Frequency of
discs. Acta Radiol 23:173, 1942. high signal intensity on T2-weighted MR images. Skeletal
Love TW, Fagan AB, Fraser RD: Degenerative spondylolis- Radiol 27:83, 1998.
thesis: Developmental or acquired? J Bone Joint Surg Br Seidenwurm D, Litt AW: The natural history of lumbar spine
81:670, 1999. disease. Radiology 195:323, 1995.
Macnab I: Spondylolisthesis with an intact neural arch—the so- Silverman FN: Calcification of intervertebral disks in child-
called pseudospondylolisthesis. J Bone Joint Surg Br 32:325, hood. Radiology 62:801, 1954.
1950. Williams AL, Haughton VM, Daniels DL, et al: Differential
Macnab I: The traction spur: An indicator of segmental instability. CT diagnosis of extruded nucleus pulposus. Radiology
J Bone Joint Surg Am 53:663, 1971. 148:141, 1983.
31
CHAPTER 31
Diffuse Idiopathic Skeletal TABLE 31–1
RADIOGRAPHIC ABNORMALITIES
Distinctive radiographic abnormalities in spinal and
extraspinal sites occur in DISH (Table 31–3). cences on the superior and inferior margins of the vertebra,
and (2) a more anterior position of the deposited bone at
the level of the intervertebral disc. Radiolucent areas
Spinal Abnormalities within the ossified mass at the level of the intervertebral
Thoracic Spine. The radiographic abnormalities of DISH discs correspond to anterolateral extension of disc mate-
are encountered most commonly in the thoracic spine rial. An additional radiolucent lesion in the form of a
(Fig. 31–1). Such abnormalities are most frequently linear defect is present between the newly deposited bone
apparent between the 7th and 11th thoracic vertebrae. and the subjacent vertebral body. This radiolucent area
Laminated calcification and ossification appear along the may not be apparent at each thoracic level but is usually
anterolateral aspect of the vertebral bodies and continue observed at some level. This linear defect ends abruptly
across the intervertebral disc spaces. Such calcification at the superior and inferior margins of the vertebral body,
and ossification are most apparent on lateral radiographs where horizontal struts of new bone are deposited. In
of the thoracic spine. The deposited bone varies con- some persons, an exaggerated concavity along the ante-
siderably in thickness; when broad, the deposited bone rior aspect of the vertebral body produces a semicircular,
appears as a radiodense shield in front of the vertebral rather than a linear, radiolucent lesion. Thoracic disc
column. Although ossification may extend to involve space narrowing is generally mild or absent.
both the right and the left lateral aspects of the vertebral
column, it is more common and exuberant on the right Cervical Spine. Cervical spine alterations (Fig. 31–2) are
side of the thoracic spine, presumably related to an also frequent in DISH. Abnormalities are more common
inhibiting effect by a left-sided pulsating, descending in the lower cervical region (between the fourth and
thoracic aorta. The contour of the involved thoracic spine seventh cervical vertebral bodies). The initial finding is
is generally irregular and bumpy; occasionally, examples hyperostosis of the cortex along the anterior surface of
of a smooth “pseudospondylitic” pattern of ossification the vertebral body. Gradually, elongated bony outgrowths
may be seen. The bumpy spinal contour is particularly appear at the anterior margin of the vertebra and extend
prominent at the level of the intervertebral discs as a across the intervertebral disc space. These outgrowths
result of two processes: (1) increased deposition of bone are observed most commonly at the inferior lip of the
at the disc space, frequently merging with bony excres- vertebral body and extend downward. Progressive bony
deposition can be either smooth and homogeneous or
bumpy and irregular. A flowing pattern of ossification
may result, but this ossification is frequently interrupted
TABLE 31–2 by radiolucent disc extensions at the level of the inter-
Clinical Features of Diffuse Idiopathic Skeletal
vertebral disc.
Hyperostosis The predilection for anterior deposition of bone in
the cervical spine follows the general distribution of ossi-
Recurrent Achilles tendinosis
fication throughout the vertebral column. In the cervical
region, however, posterior vertebral abnormalities are
Recurrent “tennis elbow”
not infrequent and include hyperostosis of the posterior
Progressive restriction of range of motion aspect of the vertebra, posterior spinal osteophytosis, and
Palpable calcaneal enthesophytes posterior longitudinal ligament calcification and ossifi-
Palpable olecranon enthesophytes cation. Elongation of the styloid process at the base of
Dysphagia the skull or calcification or ossification of the stylohyoid
Restricted motion after total joint replacement ligament, or both, may be evident in patients with DISH
(Fig. 31–3).
Chapter 31 Diffuse Idiopathic Skeletal Hyperostosis 427
A B C
Figure 31–1. Thoracic spine abnormalities in DISH. A, Findings include flowing anterior ossification
(arrowhead) with a bumpy spinal contour, radiolucent disc extensions (d), and a radiolucent area between the
deposited bone and underlying vertebral bodes (lu). B, Note the anterior spinal ossification (arrowheads),
radiolucent disc extensions (d), radiolucent areas between deposited bone and subjacent vertebrae (lu),
exaggerated anterior vertebral concavity (c), and horizontal bony struts (s). C, The bumpy spinal contour
(arrowheads) reflects anterior hyperostosis with radiolucent disc extensions (d), linear radiolucent areas (lu),
and horizontal struts of bone (s).
(A and B, From Resnick D, Shapiro RF, Wiesner KB, et al: Diffuse idiopathic skeletal hyperostosis
(DISH) [ankylosing hyperostosis of Forestier and Rotes-Querol]. Semin Arthritis Rheum 7:153, 1978. C,
From Resnick D, Niwayama G: Radiographic and pathologic features of spinal involvement in diffuse
idiopathic skeletal hyperostosis (DISH). Radiology 119:559, 1976.)
Lumbar Spine. Lumbar spine abnormalities in DISH are reflects the nature of the hyperostosis and associated
almost as frequent as thoracic spine abnormalities (Fig. calcification and ossification, ranging from low to an
31–4). These findings are usually observed in the upper intensity similar to that of marrow fat (Fig. 31–5).
lumbar region. The lumbar changes resemble alterations
in the cervical spine. Initially, hyperostosis is observed
Extraspinal Abnormalities
along the anterior aspect of the vertebral body. With
progression, cloudlike increased bone density and pointed The extraspinal radiographic manifestations of DISH
bony excrescences develop. Additional findings include are not only frequent but also distinctive (Table 31–4).
radiolucent anterior disc extension, occasional radiolu- Although extraspinal radiographic features of DISH can
cent areas between the deposited bone and the subjacent occur at virtually any skeletal site, they are most charac-
vertebral body, and the rare occurrence of posterior out- teristic in certain locations. Typically, they have a
growths. The predilection for right-sided involvement in bilateral and symmetrical distribution.
the thoracic spine is not apparent in the lumbar region.
Pelvis. Abnormalities of the pelvic bones are common in
Miscellaneous Spinal Abnormalities. Osteoporosis is not a DISH and consist of bony proliferation or “whiskering,”
feature of this disorder, and skeletal radiodensity may ligament calcification and ossification, and para-articular
appear excessive in view of the patient’s advanced age. osteophytes (Fig. 31–6). Proliferation (whiskering) is
Additionally, osseous ankylosis is commonly observed in seen at sites of ligament and tendon attachment to bone,
the thoracic spine and less frequently in the cervical and particularly on the iliac crest, ischial tuberosity, and
lumbar spine. trochanters. Ligament calcification and ossification occur
in the iliolumbar and sacrotuberous ligaments. Para-
Magnetic Resonance Imaging. DISH is a frequent inci- articular osteophytes are noted along the inferior aspect
dental finding on spinal magnetic resonance (MR) of the sacroiliac joint, lateral aspect of the acetabulum,
imaging. The signal intensity of the involved region and superior pubic margins, where they produce para-
428 Section VII Degenerative Diseases
A B
A B
Shoulder and Humerus. Findings in this location may The osteophytes develop close to the discovertebral
include prominence and exaggerated bony irregularity junction and appear identical to those of spondylosis
along the deltoid tuberosity and greater tuberosity, medial deformans; however, the other abnormalities are not
aspect of the humeral shaft, inferior glenoid, inferior prominent in typical spondylosis deformans, although this
distal end of the clavicle, and osseous attachments of the difference does not indicate with certainty that DISH
coracoclavicular ligament. and spondylosis deformans are separate entities.
A B
C D
Chapter 31 Diffuse Idiopathic Skeletal Hyperostosis 431
B
Figure 31–11. Pathologic abnormalities of the thoracic spine
in DISH. A, Bone formation occurs in the middle of the
anterior surface of the vertebral body (arrowheads), where the
anterior longitudinal ligament (solid arrow) is attached to the
vertebral surface. Open arrows indicate Sharpey’s fibers. B,
Osseous proliferation (arrowhead) is seen on the anterior surface
of the vertebral body. Mild sclerosis (arrow) in the vertebral
body is also apparent.
Rheumatoid Arthritis
Although rheumatoid arthritis and DISH do not appear
to be causally related, they are both common disorders
and can coexist in the same patient. The radiographic
abnormalities of rheumatoid arthritis may be modified in
Figure 31–10. Pathologic abnormalities of the thoracic spine the presence of DISH. Atypical radiographic features of
in DISH. Anterior longitudinal ligament calcification and rheumatoid arthritis include the absence of osteoporosis
ossification (arrowheads) are observed on the anterior (A) and the presence of bony sclerosis and proliferation about
surface of the vertebrae, with subjacent radiolucent areas (lu). erosions, osteophytes, and intra-articular bony ankylosis.
Chapter 31 Diffuse Idiopathic Skeletal Hyperostosis 433
tum flavum or bony proliferation about the apophyseal
joints.
Fracture
Ankylosing diseases of the spine theoretically predispose
the vertebral column to acute fracture and possible
pseudarthrosis. These complications are well recognized
in ankylosing spondylitis and, increasingly, in DISH (Fig.
31–13). Fractures occur in moderate to severe cases of
DISH in which osseous fusion of long segments of the
spine is present. The ankylosed region is vulnerable to
fracture, even with relatively minor trauma; transverse
fractures are typical. The cervical and thoracic segments
appear to be more susceptible than the lumbar segment.
Significant neurologic complications, including quadri-
plegia, are related in part to spinal instability and to delay
in both diagnosis and treatment.
Infective Spondylitis
Although the hyperostotic spine in DISH is apparently
not associated with a predisposition to infective spondyli-
tis, the two conditions may occasionally coexist. In such
cases, accurate diagnosis of spinal infection may be diffi-
Figure 31–12. Heterotopic ossification in DISH. After total cult because of the possibility that the bridging vertebral
hip arthroplasty, extensive ossification is seen lateral to the outgrowths prevent disc space collapse, which is a funda-
femoral prosthesis (arrow).
mental radiographic sign of pyogenic spondylitis.
(From Resnick D, Shapiro RF, Wiesner KB, et al: Diffuse
idiopathic skeletal hyperostosis (DISH) [ankylosing hyper-
ostosis of Forestier and Rotes-Querol]. Semin Arthritis Rheum CAUSE
7:153, 1978.)
The cause of DISH is unknown. One common factor in
patients with DISH is advanced age. Some patients relate
a history of spinal trauma or occupational stress; others have
no history of significant accidental or occupational trauma.
Ankylosing Spondylitis Although the occurrence of spinal and extraspinal
A meaningful association between ankylosing spondylitis hyperostosis resembling that of DISH has been reported
and DISH has not been established. In some patients, in various diseases, as well as with the administration of
spinal abnormalities of both disorders are observed; in vitamin A derivatives (e.g., 13-cis-retinoic acid) to patients
others, the spinal abnormalities of DISH are combined with cutaneous disorders, there is no definite evidence
with the sacroiliac alterations of ankylosing spondylitis. It connecting the hyperostosis in DISH with any of these
should be recognized, however, that diagnostic difficulties other disorders. Because of the apparent similarity between
arise because of the spondylitis-type changes that may DISH and ankylosing spondylitis, the prevalence of the
occur in some persons with DISH. HLA-B27 antigen has been studied in patients with
DISH, but published studies have failed to confirm an
increased prevalence of HLA-B27 in patients with DISH.
Ossification of the Posterior Longitudinal
Ligament
Ossification of the posterior longitudinal ligament, which
is discussed in detail in Chapter 32, occurs with increased
frequency in patients with DISH (see Fig. 31–3). In almost
all instances, the cervical spine is affected. Approximately
50% of patients with DISH have radiographic evidence
of calcification or ossification of the posterior longitu-
dinal ligament.
A B
Spinal Stenosis
Figure 31–13. Spinal fractures and DISH. In this 56-year-
Compromise of the cord in the cervical region can relate old man with DISH, note a transverse fracture of high signal
to hyperostosis or ossification of the spinal ligaments, intensity on sagittal intermediate-weighted (TR/TE, 2200/30)
whereas such compromise in the thoracic and lumbar (A) and T2-weighted (TR/TE, 2200/70) (B) spin echo MR
regions may be secondary to hypertrophy of the ligamen- images. (Courtesy of C. Gundry, MD, Minneapolis, Minn.)
434 Section VII Degenerative Diseases
TABLE 31–6
Differential Diagnosis of Radiographic Findings in Diffuse Idiopathic Skeletal Hyperostosis (DISH), Ankylosing
Spondylitis, and Intervertebral (Osteo)Chondrosis
abnormalities consisting of massive new bone formation DISH. One of its radiographic features, tendon calci-
are occasionally identified. In sternocostoclavicular fication, superficially resembles the findings of DISH.
hyperostosis, ossification also affects the medial aspect of Other findings of this crystal deposition disease,
the clavicles, the upper ribs, and the sternum, and pustular including chondrocalcinosis and pyrophosphate arthrop-
skin lesions are common on the palms and soles. athy, are characteristic and allow an accurate diagnosis.
TABLE 32–1
Calcification and Ossification of Posterior Spinal Ligaments and Tissues
438
Chapter 32 Calcification and Ossification of the Posterior Spinal Ligaments and Tissues 439
Space between
ligamenta flava
Pedicle
Posterior
longitudinal
ligament
Ligamentum
Intervertebral flavum
disc
Pedicle of
vertebra
Lamina
A B
Figure 32–1. Posterior spinal ligaments: anatomic considerations. A, Posterior longitudinal ligament:
lumbar spine. Drawing of the posterior aspect of the vertebral bodies and intervertebral discs shows the
position and appearance of the posterior longitudinal ligament. Note that it is narrow at the level of the
vertebral bodies and broad at the level of the intervertebral discs. B, Ligamenta flava: lumbar spine. Drawing
of the anterior aspect of the vertebral arches reveals the position of the paired ligamenta flava. At the bases
of the spinous processes, spaces between the ligaments allow passage of veins.
(From Williams PL, Warwick R: Gray’s Anatomy, 36th Br ed. Philadelphia, WB Saunders, 1980, p 44.)
the ligament expands to form the ligamentum nuchae, causes no obvious neurologic deficits and is associated
which extends cephalad to the external occipital protu- with pain in the neck, shoulder, and arm. Neurologic
berance. The intertransverse ligaments are located manifestations are more frequent in patients in whom
between the transverse processes of the vertebrae. the thickness of the ligament is greater than 30% to 60%
of the sagittal diameter of the cervical spinal canal as
measured by radiographs.
OSSIFICATION OF THE POSTERIOR The choice of therapy is made difficult by some incon-
LONGITUDINAL LIGAMENT sistency in the relationship between the neurologic mani-
festations and the degree of ligamentous ossification and
Clinical Abnormalities
by reports of postoperative progression of such ossification.
Ossification of the posterior longitudinal ligament
(OPLL) is more frequent in men than women, with a
ratio of approximately 2:1. Diagnosis of the disorder is Radiographic Abnormalities
usually established in the fifth to seventh decades of life. The diagnosis of OPLL is established by its characteristic
Although persons with OPLL may be entirely asymp- radiographic appearance (Fig. 32–2). In the cervical spine
tomatic, a variety of symptoms and signs have been (and, infrequently, in the thoracic and lumbar spine), a
associated with this disorder. Neurologic symptoms may dense, ossified strip or plaque of variable thickness (1 to
be divided into three groups: cord signs, which are most 5 mm) is evident along the posterior margins of the ver-
common, manifested by dominant motor and sensory tebral bodies and the intervertebral discs. It is most com-
disturbances in the lower extremity; segmental signs, mon in the midcervical region (C3 to C5), although any
manifested by dominant motor and sensory disturbances cervical level may be affected. The ossified ligament may
in the upper extremity; and cervicobrachialgia, which be confined to one or several vertebral bodies, without
440 Section VII Degenerative Diseases
involvement of the intervening intervertebral discs (seg- ing to that of fat, can be observed in more than half
mental distribution), or it may extend in an uninterrupted of patients with the continuous type of OPLL and occa-
fashion for several vertebrae (continuous distribution). sionally with segmental OPLL. The degree of spinal
Often, OPLL is separated from the vertebral body by a thin cord compression is generally more severe in cases of
radiolucent zone. The intervertebral discs in the involved continuous OPLL, and disc degeneration is a frequently
area are generally well preserved, without evidence of disc associated feature (see Fig. 32–4).
space narrowing; however, anterior vertebral osteophytes The differential diagnosis of a lesion around the spinal
are frequently identifiable (30% to 50% of patients). cord, when characterized by low signal intensity on both
OPLL is not confined to the cervical region and may T1- and T2-weighted images, includes OPLL, a hyper-
involve the thoracic and lumbar segments as well. In the trophied ligament, hemosiderin deposition, flowing blood
thoracic spine, OPLL is most common at the fourth to or cerebrospinal fluid, and calcified meningioma. When high
seventh thoracic vertebral levels (Fig. 32–3). Involvement signal intensity is evident as well, OPLL, osteophyte for-
in the lumbar spine can be apparent at any level, although mation, and disc displacement are diagnostic considerations.
changes predominate in the L1 to L2 region. The extent
of OPLL and the degree of spinal encroachment have
Pathologic Abnormalities
been further delineated by means of conventional
tomography, axial transverse tomography, computed The bony overgrowth appears as cortical bone composed
tomography (CT), myelography, and magnetic resonance of lamellar bone with well-developed haversian canals
(MR) imaging (Fig. 32–4). and poorly developed marrow cavities. In places, the
ossified mass is firmly attached to the posterior aspect of
the vertebral body and intervertebral disc; elsewhere, it is
Magnetic Resonance Imaging Abnormalities separated from these structures by intervening connec-
MR imaging is well suited to the evaluation of patients tive tissue. Closer inspection of the ossification allows the
with OPLL. OPLL is generally seen as a band of low identification of calcific areas within the posterior longi-
signal intensity between the bone marrow of the verte- tudinal ligament, which suggests that the ossification
bral body and the dural sac on T1- and T2-weighted spin involves the ligament itself but need not be complete.
echo MR images. Within the area of ossification, an area The exact mechanism leading to mineral deposition in
of increased or intermediate signal intensity, correspond- the posterior longitudinal ligament is not clear.
Chapter 32 Calcification and Ossification of the Posterior Spinal Ligaments and Tissues 441
Figure 32–4. Ossification of the
posterior longitudinal ligament:
MR imaging and CT abnormalities
of the thoracic spine. The patient is
a 47-year-old woman with back pain
and leg weakness. A, Radiograph
shows a dense ossified strip along
the posterior margin of the verte-
bral bodies. Note continuous
involvement superiorly and relative
preservation of intervertebral disc
spaces. B and C, Corresponding
sagittal T1-weighted (TR/TE,
500/9) (B) and turbo T2-weighted
(TR/TE, 4000/100) (C) MR images
show that the signal intensity within
the posterior longitudinal ligament
is equal to or less than that of the
adjacent fatty marrow, with the
more densely ossified areas show-
ing decreased signal. D, Sagittal
reformatted noncontrast CT scan
A B shows the ossification to better
advantage. Note mineralization of
the ligamentum flavum and inter-
spinous and supraspinous ligaments.
E, Midthoracic axial CT scan at the
pedicular level shows extensive
ossification of the posterior longi-
tudinal ligament, with significant
compromise of the spinal canal. F,
Axial CT scan at the level of the
facet joints shows ligamentum
flavum and apophyseal joint capsule
mineralization.
C D
E F
442 Section VII Degenerative Diseases
A B
Figure 32–5. Arachnoiditis ossificans. A, In this transaxial CT scan, observe the extensive ossification
(open arrows) in the lumbosacral region of the spinal canal. The unossified nerve roots are identifiable. B, On
a transaxial gradient echo (MPGR) MR image (TR/TE, 1000/15; flip angle, 50 degrees), the calcified areas
are of low signal intensity (open arrows). (Courtesy of S. Eilenberg, MD, San Diego, Calif.)
Chapter 32 Calcification and Ossification of the Posterior Spinal Ligaments and Tissues 443
In the thoracic and upper lumbar spine, ossification Ehara S, Shimamura T, Nakamura R, et al: Paravertebral
(rather than calcification) of the ligamentum flavum ligamentous ossification: DISH, OPLL and OLF. Eur J
predominates. Both men and women are affected, Radiol 27:96, 1998.
typically in the fifth and sixth decades of life. Neurologic Griffiths ID, Fitzjohn TP: Cervical myelopathy, ossification of
the posterior longitudinal ligament, and diffuse idiopathic
manifestations include decreased sensation, paresis, and
skeletal hyperostosis: Problems in investigation. Ann Rheum
even neurogenic bladder. Ossification of the ligamentum Dis 46:166, 1987.
flavum appears to be an accentuation of the bone spicu- Kudo S, Ono M, Russell WJ: Ossification of thoracic ligamenta
lation that is observed anatomically at the sites of osseous flava. AJR 141:117, 1983.
attachment of the ligamenta flava. Minagi H, Gronner AT: Calcification of the posterior
CPPD crystal deposition disease is a recognized cause longitudinal ligament: A cause of cervical myelopathy. AJR
of calcification in the ligamentum flavum, as well as in 105:365, 1969.
other posterior spinal structures such as the posterior Ono K, Ota H, Tada K, et al: Ossified posterior longitudinal
longitudinal ligament, interspinous and supraspinous ligament. Spine 2:126, 1977.
ligaments, and apophyseal joints. Linear or nodular Ono K, Yonenobu K, Miyamoto S, et al: Pathology of
ossification of the posterior longitudinal ligament and
deposits related to CPPD crystal accumulation in the
ligamentum flavum. Clin Orthop 359: 18, 1999.
thoracic spine are typical. Ono M, Russell WJ, Kudo S, et al: Ossification of the thoracic
posterior longitudinal ligament in a fixed population.
CALCIFICATION OR OSSIFICATION OF Radiological and neurological manifestations. Radiology
143:469, 1982.
OTHER POSTERIOR SPINAL LIGAMENTS
Resnick D, Pineda C: Vertebral involvement in calcium
Hyperostosis at the sites of attachment of the supra- pyrophosphate dihydrate crystal deposition disease.
spinous ligament and calcification and ossification within Radiographic-pathologic correlation. Radiology 153:55,
this ligament occur as isolated abnormalities or in asso- 1984.
ciation with DISH. Calcification within the interspinous Resnick D, Guerra J Jr, Robinson CA, et al: Association of
diffuse idiopathic skeletal hyperostosis (DISH) and
ligament occurs in CPPD crystal deposition disease.
calcification and ossification of the posterior longitudinal
Ossification in the ligamentum nuchae is an inciden- ligament. AJR 131:1049, 1987.
tal and frequent radiographic finding that is also seen Revilla TY, Ramos A, Gonzalez P, et al: Arachnoiditis
in DISH. ossificans: Diagnosis with helical computed tomography.
Clin Imaging 23:1, 1999.
Williams DM, Gabrielsen TO, Latack JT, et al: Ossification in
FURTHER READING the cephalic attachment of the ligamentum flavum. An
anatomical and CT study. Radiology 150:423, 1984.
Barthelemy CR: Arachnoiditis ossificans. J. Comput Assist
Tomogr 6:809, 1982.
SECTION
VIII
Crystal-Induced and Related Diseases
CHAPTER 33
Gouty Arthritis TABLE 33–1
Classification of Hyperuricemia and Gout
Bone Erosions
Bone erosions in gout are produced by tophaceous
deposits and may be intra-articular, para-articular, or
located a considerable distance from the joint (Fig. 33–3).
Intra-articular erosions usually commence in the
marginal areas of the joint and proceed centrally; para-
articular erosions are eccentric in location, frequently
beneath soft tissue nodules. Gouty erosions may be
surrounded by a sclerotic border and produce a “punched
out” appearance.
In about 40% of patients with gouty erosions of
bone, an elevated bony margin, or lip, extends outward
in the soft tissues, apparently covering the tophaceous
nodule (see Fig. 33–3). The overhanging edge may relate
to bone resorption beneath the gradually enlarging
Figure 33–1. Radiographic features of gout: soft tissue abnor- tophus, with periosteal bony apposition at the outer
malities. Partial peripheral calcification (arrow) of an eccentric aspect of the involved cortex. Although the appearance is
mass (representing a tophus) is evident on the radial aspect of not pathognomonic, it is strongly suggestive of gouty
the wrist. arthritis.
448 Section VIII Crystal-Induced and Related Diseases
A B
B
Figure 33–6. Forefoot abnormalities in gout. A, Early
involvement of the first metatarsophalangeal joint. Erosions
predominate at the dorsomedial aspect of the metatarsal head
(arrow) and are associated with soft tissue swelling, articular
space narrowing (arrowhead), and osteophyte formation. B,
Severe forefoot abnormalities. Findings include extensive bony
destruction with overhanging margins and pathologic fractures
(arrow).
(From Resnick D: The radiographic manifestations of gouty
arthritis. CRC Crit Rev Diagn Imaging 9:265, 1977.)
Figure 33–12. Patellar abnormalities in gout. Observe a joints, particularly within the menisci of the knee, sym-
prepatellar tophus (arrow), partially calcified, that is producing physis pubis, and triangular fibrocartilage of the wrist.
erosion of the anterior surface of the patella. An additional soft Hyaline cartilage calcification or widespread chondro-
tissue deposit with subjacent bone erosion can be seen
calcinosis is rare in gouty arthritis. Both monosodium
(arrowhead).
(From Resnick D: The radiographic manifestations of gouty urate and CPPD crystals may be obtained from the same
arthritis. CRC Crit Rev Diagn Imaging 9:265, 1977.) joint. Crystals are frequently found in asymptomatic joints.
Calcium-containing crystals are found in a large array
of clinical situations, and examination of deposits often
reasons. Based on anecdotal experience with gout, it demon- reveals a mixture of crystalline material.
strates variable, multiple hypermetabolic foci, reflecting
the inflamed synovium associated with tophi (Fig. 33–15).
Osteoarthritis
COEXISTENT ARTICULAR DISORDERS In patients with osteoarthritis of the interphalangeal
joints of the hand (nodal osteoarthritis) in whom inflam-
Calcium Pyrophosphate Dihydrate and mation and radiographically evident erosions develop,
Hydroxyapatite Crystal Deposition diagnostic possibilities include secondary gout, calcium
Chondrocalcinosis can occur in patients with gout. These hydroxyapatite crystal deposition, CPPD crystal deposi-
calcific deposits are usually localized in one or two sets of tion, and inflammatory (erosive) osteoarthritis.
A B
C D
Figure 33–14. MR imaging abnormalities in gout. A, Sagittal T1-weighted (TR/TE, 537/17) spin echo
MR image of the foot shows multiple soft tissue and intra-articular masses of intermediate signal intensity,
with extensive osseous involvement. B, Corresponding fat-suppressed T1-weighted (TR/TE, 400/17) spin
echo MR image following contrast shows intense heterogeneous enhancement, with no enhancement in the
areas of calcification and amorphous urate deposits and variably intense enhancement in the inflamed
synovium. C, Coronal T2-weighted (TR/TE, 2930/80) spin echo MR image shows the tophi to have a
heterogeneous intermediate signal intensity. Note subcutaneous edema around the ankle. D, Tophi show
hyperintense signal on sagittal fat-suppressed T2-weighted (TR/TE, 6000/62) fast spin echo MR image.
Figure 33–15. PET imaging abnormalities in a 52-year-old Lesch-Nyhan and Kelley-Seegmiller Syndromes. Lesch-
woman with gout. Anteroposterior image of the lower Nyhan syndrome includes spasticity, choreoathetosis,
extremities shows multiple hypermetabolic foci in the feet, mental retardation, and compulsive self-mutilation mani-
corresponding to multiple tophi. fested as finger and lip biting. Boys are affected, and the
A B
Figure 33–16. Early-onset gouty arthritis. Progressive abnormalities occurred over a 2-year period in this
23-year-old man with gout. A, Initial film reveals soft tissue swelling and bony destruction of the proximal
phalanx (arrow). B, Subsequent radiograph demonstrates increased severity of the bony and soft tissue
changes.
(From Resnick D: The radiographic manifestations of gouty arthritis. CRC Crit Rev Diagn Imaging
9:265, 1977.)
Chapter 33 Gouty Arthritis 455
disorder is X-linked (Fig. 33–17). Complete or virtually Gout Associated with Other Clinical
complete deficiency of hypoxanthine-guanine phospho- Disorders (Secondary Gout)
ribosyltransferase (HPRT) is associated with Lesch- A number of other disorders may result in hyperuricemia
Nyhan syndrome. Radiographic abnormalities of and secondary gout. Myeloproliferative disorders, which
Lesch-Nyhan syndrome reflect self-mutilation, with are apparent in approximately 5% to 10% of patients with
amputation of the soft tissues and osseous structures of gout, include polycythemia vera, leukemia, lymphoblas-
the hands. Gouty erosions, retarded skeletal maturation, toma, myeloid metaplasia, hemolytic anemia, sickle cell
coxa valga deformities with subluxation of the hips, and anemia, pernicious anemia, thalassemia, multiple myeloma,
soft tissue tophi have been described in this disorder. secondary polycythemia, infectious mononucleosis, and
Additional findings include traumatic changes occurring Waldenström’s hyperproteinemia, as well as some
after seizures, cerebral atrophy, and uric acid calculi in carcinomas.
the urinary tract. Hyperuricemia may be associated with a variety of
In Kelley-Seegmiller syndrome, the deficiency of endocrine disorders, including hyperparathyroidism,
HPRT is incomplete, and patients may have adolescent- hypoparathyroidism, myxedema, and hypoadrenal states.
or adult-onset gouty arthritis and occasional mild neuro- Additional causes of hyperuricemia and gout are obesity,
logic disease. Radiographic abnormalities are generally idiopathic hypercalciuria, psoriasis, myocardial infarction
limited to gouty arthritis, because most patients are and vascular disease, renal disease, and near-starvation
normal neurologically. states. Drug-induced gout has been noted in association
with diuretic, pyrazinamide, and salicylate therapy.
The radiographic manifestations of secondary and
Saturnine Gout
idiopathic gout are generally indistinguishable.
The accidental contamination of alcoholic beverages with
lead has been recognized for centuries. In addition, lead
was sometimes intentionally added to wine to improve its DIFFERENTIAL DIAGNOSIS
flavor or prevent spoiling, a practice that was subse- Because radiographic abnormalities in gouty joints appear
quently ruled illegal. Gout occurring as a complication late in the course of the disease, the diagnosis is usually
of chronic lead intoxication is called saturnine gout. well established clinically, and the radiologist need only
Currently, this form of gout is principally associated with document its extent and severity. Occasionally, the disease
the ingestion of illegally manufactured alcohol (moon- is not suspected before radiographic examination.
shine). It is caused in large part by decreased urate clear-
ance by the kidneys as a result of lead nephropathy.
Saturnine gout accounts for only a small number of cases Rheumatoid Arthritis
of gout. Patients are typically male, black, aged 45 to Rheumatoid arthritis produces radiographic alterations
55 years, often azotemic and anemic, but otherwise that differ from those of gout, including symmetrical joint
asymptomatic except for joint disease. Radiographic find- involvement, fusiform soft tissue swelling, and regional
ings resemble those of idiopathic gout. osteoporosis (Table 33–2; Fig. 33–18). Early diffuse joint
space loss accompanies marginal erosive changes in the
bone.
Psoriatic Arthritis
Psoriatic arthritis may produce radiographic changes that
resemble those of gout, and the presence of elevated
serum uric acid levels may lead to further diagnostic
difficulty. Articular manifestations of psoriatic arthritis
include progressive destruction of the peripheral joints
of the extremities, periosteal proliferation at the margins
of the joint, paravertebral ossification, and sacroiliac
disease. Osteoporosis is generally not apparent.
Sarcoidosis
Sarcoidosis may produce skeletal abnormalities, particu-
larly in the hand. These abnormalities include widening
of the medullary portion of the bone, a honeycomb con-
figuration, focal lucent osseous defects, and sclerosis.
Although granulomatous involvement of the synovium
and juxta-articular bone occurs in the chronic arthritis
of sarcoidosis, articular destruction and deformity are
unusual.
Amyloidosis
Amyloidosis, which occurs in a primary form or as a
secondary manifestation of another disease, can result in
bone or joint lesions. Amyloid infiltration of the articular
structures may cause soft tissue masses and focal erosive
osseous lesions indistinguishable from those of gout.
Xanthomatosis
Tendinous xanthomas are particularly frequent on the
Figure 33–18. Rheumatoid arthritis versus gout. In rheu- extensor surface of the hand and foot and in the patellar
matoid arthritis (left), findings include diffuse loss of articular and Achilles tendon regions. Tuberous xanthomas occur
space and marginal erosions. In gout (right), note the preser- in the subcutaneous tissues of the elbows and knees.
vation of joint space, eccentric erosions, and an “overhanging
edge.”
Radiographic changes consist of eccentric soft tissue
(From Resnick D: The radiographic manifestations of gouty nodular masses with subjacent bone erosion, findings
arthritis. CRC Crit Rev Diagn Imaging 9:265, 1977.) simulating gouty tophi (Fig. 33–20). Hypercholes-
terolemia in patients with xanthomatosis is an important
laboratory abnormality.
calcinosis in gout is usually localized to one or two joints
and involves fibrocartilage alone. The structural joint
abnormalities of CPPD crystal deposition disease (termed Inflammatory (Erosive) Osteoarthritis and
pyrophosphate arthropathy) demonstrate an unusual Multicentric Reticulohistiocytosis
predilection for the wrist, metacarpophalangeal joints, Destructive articular abnormalities of the interpha-
and knee; they include joint space narrowing, subchon- langeal joints, which are noted in both gout and psoriatic
dral sclerosis and cyst formation, bone fragmentation and arthritis, are also apparent in inflammatory (erosive)
collapse, and variable osteophyte formation. Although the osteoarthritis and multicentric reticulohistiocytosis. The
radiographic manifestations of pyrophosphate arthrop- former disease affects middle-aged and elderly women
athy may resemble those of gout, the presence of lobu- and produces symmetrical joint changes, usually confined
lated soft tissue masses, intact joint spaces, and osseous to the interphalangeal joints of the fingers, the first
erosions in the latter disease usually permits differen- carpometacarpal joint, and the trapezioscaphoid articu-
tiation of the two disorders (Fig. 33–19). lation. The erosions in inflammatory osteoarthritis
TABLE 33–2
Radiographic Features of Gouty and Rheumatoid Arthritis
A B
Figure 33–19. Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease versus gout. A, In
CPPD crystal deposition disease, changes at the first metatarsophalangeal joint may consist of soft tissue
calcification (arrow). B, In gout, typical marginal and central erosions are observed (arrowheads).
FURTHER READING
Martel W: The overhanging margin of bone: A roentgenologic Ruiz ME, Erickson SJ, Carrera GF, et al: Monoarticular gout
manifestation of gout. Radiology 91:755, 1968. following trauma: MR appearance. J Comput Assist Tomogr
Martin DJ, Merenda G, McDonald DJ, et al: Recurrent 17:151, 1993.
hemarthrosis associated with gout. Clin Orthop 277:262, Seegmiller JE: Human aberrations of purine metabolism and
1992. their significance for rheumatology. Ann Rheum Dis 39:103,
Resnick D: The radiographic manifestations of gouty arthritis. 1980.
CRC Crit Rev Diagn Imaging 9:265, 1977. Watt I, Middlemiss H: The radiology of gout. Clin Radiol
Resnick D, Broderick TW: Intraosseous calcifications in topha- 26:27, 1975.
ceous gout. AJR Am J Roentgenol 137:1157, 1981.
Resnick D, Reinke RT, Taketa RM: Early-onset gouty arthritis.
Radiology 114:67, 1975.
34
CHAPTER 34
Calcium Pyrophosphate Dihydrate identified as calcium pyrophosphate dihydrate (CPPD)
by their x-ray diffraction powder pattern. When clinical
Crystal Deposition Disease and radiographic findings in these patients were analyzed,
it was recognized that the same disease had been des-
cribed as chondrocalcinosis polyarticularis, reflecting the
calcific deposits within cartilage. It thus became clear
SUMMARY OF KEY FEATURES that patients with a distinctive goutlike pattern of arthritis
Until recently, calcium pyrophosphate dihydrate (CPPD) (pseudogout syndrome) had crystal accumulation within
crystal deposition disease was a commonly overlooked joints (CPPD deposition) that could cause cartilage calci-
condition. In the past 3 decades, considerable attention fication (chondrocalcinosis). Subsequently, other crystals
to its various clinical and radiographic manifestations were found to produce calcific collections in the knee
has increased physicians’ awareness of this disorder. (Table 34–1).
Chondrocalcinosis can no longer be considered the
only significant radiographic feature of CPPD crystal
TERMINOLOGY
deposition disease. An equally characteristic
radiographic finding is the presence of other intra- The variety of names linked to this condition has led
articular and periarticular calcifications and structural to considerable confusion in nomenclature. Articular
joint damage. The pattern and distribution of structural chondrocalcinosis and pseudogout are but two of these
joint damage (pyrophosphate arthropathy) are especially names, and at times they have been used interchangeably.
distinctive and can be differentiated from the features The following related terms are defined in accordance
of degenerative joint disease to allow an accurate with current usage:
diagnosis of CPPD crystal deposition disease even in
the absence of chondrocalcinosis. Calcium pyrophosphate dihydrate (CPPD) crystal deposition
disease: General term for a disorder characterized by
the presence of Ca2P2O7 • 2H2O (CPPD) crystals in
or around joints.
INTRODUCTION
Pseudogout: Term applied to one of the clinical patterns
In 1961 and 1962, McCarty and others discovered nonurate that may be associated with crystal deposition. This
crystals in the joint fluid of patients experiencing goutlike pattern, characterized by intermittent acute attacks of
attacks of arthritis. These crystals were subsequently arthritis, simulates that of gout.
TABLE 34–1
Reported Prevalence in Early Investigations of Chondrocalcinosis in the Knee
Number Incidence of
Author of Cases Age of Patients Selection of Material Method of Study Chondrocalcinosis
Tobler (1929) 1400 Elderly Autopsy and surgical Histologic examination 30%
specimens
Wolke (1935) 12,268 Wide range Retrospective Conventional radiograph 0.42%
Bennett et al (1942) 63 Elderly Cadavers Histologic study 4.1%
McCarty and Haskin 215 71 yr (mean) Cadavers Specimen radiograph, 7% total
(1963) histologic study, and 3.3% CPPD
crystal analysis 2.3% DCPD
1.4% HA
Bocher et al (1965) 455 80 yr (mean) Retrospective and Conventional radiograph 7%
prospective surgery
Lagier and Baud (1968) 320 Elderly Cadavers Histologic study and 6.8% CPPD
crystal analysis
Schmied et al (1971) 97 64 yr (mean) Prospective control Conventional radiograph 4.2%
and diabetic survey
Ellman and Levin (1975) 58 83 yr (mean) Survey of patients in Industrial film 28%
home for elderly radiograph
CPPD, calcium pyrophosphate dihydrate; DCPD, dicalcium phosphate dihydrate; HA, calcium hydroxyapatite.
After Genant HK: Roentgenographic aspects of calcium pyrophosphate dihydrate crystal deposition disease (pseudogout). Arthritis Rheum 19(Suppl)3:307, 1976.
459
460 Section VIII Crystal-Induced and Related Diseases
A B
Synovial Calcification. Calcification within the synovial Soft Tissue and Vascular Calcification. In some patients,
membrane is a common feature of CPPD crystal deposi- poorly defined calcific deposits are seen in the soft tissues
464 Section VIII Crystal-Induced and Related Diseases
A B
C D
Figure 34–4. Radiographic abnormalities: chondrocalcinosis. A–C, Chondrocalcinosis of fibrocartilage
(F) is apparent in the menisci of the knee, triangular cartilage of the wrist, anulus fibrosus of the inter-
vertebral discs, and meniscus of the acromioclavicular joint. It is thick and shaggy in character. Hyaline
cartilage calcification (H), which is seen in the knee and wrist, is thin and parallels the osseous surface.
Possible ligamentous calcification (L) is also noted in the wrist. D, Hyaline cartilage (H) calcification in the
elbow.
(A, From Resnick D, Niwayama G, Goergen TG, et al: Clinical, radiographic and pathologic abnormal-
ities in calcium pyrophosphate dihydrate deposition disease [CPPD]: Pseudogout. Radiology 122:1, 1977.)
A B
Figure 34–10. Characteristics of pyrophosphate arthropathy: unusual intra-articular distribution. A, Observe
the selective involvement of the radiocarpal compartment of the wrist (arrow). B, Predilection for the
patellofemoral compartment of the knee is apparent on this lateral radiograph.
Chapter 34 Calcium Pyrophosphate Dihydrate Crystal Deposition Disease 467
A B
Figure 34–11. Characteristics of pyrophosphate arthropathy: destructive bone changes that are severe and
progressive. Radiographs of the glenohumeral joint obtained 16 months apart outline the rapidity of joint
destruction in this disease (arrows).
(From Resnick D, Niwayama G, Goergen TG, et al: Clinical, radiographic and pathologic abnormalities
in calcium pyrophosphate dihydrate deposition disease [CPPD]: Pseudogout. Radiology 122:1, 1977.)
RADIOGRAPHIC FEATURES IN SPECIFIC particularly between the scaphoid and lunate and between
ARTICULATIONS the lunate and triquetrum. Carpal malalignment with
separation of the scaphoid and lunate may be observed,
Knee with disruption of the intervening interosseous ligament.
The knee is the joint involved most commonly (Fig. 34–13; The wrist arthropathy of CPPD crystal deposition
Table 34–3). Chondrocalcinosis and synovial calcification disease demonstrates an unusual predilection for the
may be combined with tendinous and ligamentous deposits radiocarpal compartment. Joint space narrowing, sclerosis,
in the quadriceps muscle, gastrocnemius muscle, and col- and discrete subchondral radiolucent lesions are observed
lateral and cruciate ligaments. Intra-articular osseous and between the distal end of the radius and the proximal
calcific bodies are frequently observed. carpal row. The scaphoid moves proximally, and at the
Pyrophosphate arthropathy most commonly involves same time, the lunate may move distally and approach
the medial femorotibial compartment; the patellofemoral
compartment is the second most commonly involved
area. More important, the distribution of knee compart- TABLE 34–3
mental alterations in CPPD crystal deposition disease Most Frequent Sites of Clinical and Radiographic
differs from that of degenerative joint disease. Isolated Abnormalities in CPPD Crystal Deposition Disease
or severe patellofemoral compartment changes have been
stressed as a manifestation of this crystal deposition disease. Clinical
Isolated lateral compartment alterations (especially in Manifestations Calcification Arthropathy
men), severe flattening of either the medial or lateral Hand + +
tibial condyles, and significant varus or valgus angulation Wrist + + +
are additional characteristics of knee involvement in this Elbow +
disease.
Shoulder +
Hip + +
Wrist Knee + + +
Calcification in the wrist (Fig. 34–14) is observed most Ankle +
commonly in the triangular fibrocartilage; hyaline carti- Spine +
lage of the radiocarpal, midcarpal, and common carpo- Pelvis +
metacarpal joints; synovium; and ligamentous structures,
468 Section VIII Crystal-Induced and Related Diseases
A B
Figure 34–13. Knee abnormalities in CPPD crystal deposi-
Figure 34–12. Characteristics of pyrophosphate arthropathy: tion disease. Considerable collapse and fragmentation of the
variable osteophyte formation. A, In some persons, large osteo- medial femorotibial compartment are visualized (arrows).
phytes (arrowhead) accompany joint space narrowing (arrow). (From Resnick D, Niwayama G, Goergen TG, et al: Clinical,
B, In other persons, joint space narrowing (arrow) occurs with- radiographic and pathologic abnormalities in calcium pyro-
out osteophyte formation. Intra-articular bodies are apparent phosphate dihydrate deposition disease [CPPD]: Pseudogout.
(arrowhead). Radiology 122:1, 1977.)
(From Resnick D, Niwayama G, Goergen TG, et al: Clinical,
radiographic and pathologic abnormalities in calcium pyro-
phosphate dihydrate deposition disease [CPPD]: Pseudogout.
Radiology 122:1, 1977.)
Metacarpophalangeal Joints
Radiographic abnormalities in this location include carti-
laginous, capsular, and synovial calcifications and arthrop-
athy (Fig. 34–15). The structural joint changes show a
predilection for the second and third metacarpopha-
langeal joints and are characterized by joint space narrow-
ing, sclerosis, cyst formation, and bony collapse, particularly
of the metacarpal head. Although the second and third Figure 34–14. Wrist abnormalities in CPPD crystal deposi-
tion disease. In addition to chondrocalcinosis and synovial and
metacarpophalangeal joints are also involved in hemo- ligamentous calcification (arrowheads), observe the considerable
chromatosis, changes in the fourth and fifth digits are not narrowing of the radiocarpal (arrow) and midcarpal compart-
infrequent in that disease. Further, the absence of ero- ments, with cyst formation and sclerosis. Note the “stepladder”
sions of the metacarpal heads in CPPD crystal deposition appearance, with proximal migration of the scaphoid and distal
disease differs from the findings of rheumatoid arthritis. migration of the lunate.
Chapter 34 Calcium Pyrophosphate Dihydrate Crystal Deposition Disease 469
Elbow
Radiographic findings in this joint include chondrocal-
cinosis, capsular and synovial calcification, triceps tendon
deposits, and arthropathy. Osseous resorption in the
proximal portions of the radius and ulna, joint space
narrowing, extensive sclerosis, cyst formation, and bone
fragmentation are features of arthropathy of the elbow.
Hip
Both fibrocartilaginous calcification of the acetabular labra
and hyaline cartilage calcification may be seen (Fig. 34–16).
Joint space narrowing may involve the entire joint or be
confined to its superolateral aspect. In the latter situa-
tion, the changes resemble those of osteoarthritis, whereas
B
with diffuse loss of joint space, the findings are similar to Figure 34–16. Hip abnormalities in CPPD crystal deposi-
those of rheumatoid arthritis. Additional manifestations tion disease. A, Fibrocartilage (F) calcification of the acetabular
of hip involvement in CPPD crystal deposition disease limbus is seen adjacent to a small subchondral cystic lesion
are rapid and extensive destruction of the femoral head (arrow). B, Considerable flattening and deformity of the femoral
and acetabulum, fragmentation, protrusio acetabuli defor- head are associated with an elongated lateral acetabular osteo-
mity, tendon calcification, and large tumor-like lesions phyte and new bone formation on the medial aspect of the
(i.e., tophaceous pseudogout). femoral neck. The articular space is obliterated, and a large
subchondral cyst (arrow) is evident.
(From Resnick D, Niwayama G, Goergen TG, et al: Clinical,
Shoulder radiographic and pathologic abnormalities in calcium pyrophos-
phate dihydrate deposition disease [CPPD]: Pseudogout.
Abnormalities in this location include chondrocalcinosis; Radiology 122:1, 1977.)
capsular, tendinous, and bursal deposits; rotator cuff
tears; joint space narrowing; bony eburnation; and cysts.
Acromioclavicular joint destruction and masses above Ankle, Hindfoot, and Midfoot
this joint are also well-known features of CPPD crystal Although articular and periarticular calcification can be
deposition disease. seen about the ankle, arthropathy in this site is unusual.
470 Section VIII Crystal-Induced and Related Diseases
Forefoot
Capsular calcification may be apparent about any meta-
tarsophalangeal joint. In unusual circumstances, carti-
laginous and synovial calcification and soft tissue swelling
at the first metatarsophalangeal joint resemble the
changes of gout.
Spine
In the spine, CPPD crystal deposition disease frequently
demonstrates intervertebral disc calcification, with deposits
initially appearing in the outer fibers of the anulus fibrosus Figure 34–17. Spine abnormalities in CPPD crystal deposi-
(Figs. 34–17 and 34–18). The nucleus pulposus is gener- tion disease: alterations of the intervertebral disc. In association
ally not involved. CPPD deposits are detected in other with widespread disc calcification, disc space loss and bone
spinal tissues as well, including the ligamentum flavum, eburnation are seen in this sagittal section of the lumbosacral
posterior longitudinal ligament, interspinous and supra- junction.
spinous ligaments, and interspinous bursae.
Disc space narrowing is a common finding in CPPD tion. Unilateral or bilateral subchondral erosions, sclerosis,
crystal deposition disease. The narrowing may be exten- and cyst formation may be accompanied by joint space
sive, widespread, and associated with considerable verte- narrowing and bridging osteophytosis.
bral sclerosis. In unusual circumstances, severe osseous
destruction of the adjacent vertebral bodies is seen. CPPD
crystal deposition in the spine is widespread, and destruc- CLINICAL AND RADIOGRAPHIC
tive abnormalities of the cervical spine are occasionally CORRELATIONS
apparent. In the atlantoaxial joint, tumor-like masses may Although calcification and arthropathy frequently coexist
compress the spinal cord, and the anterior longitudinal in the same joint, either radiographic finding can be iden-
ligament may become calcified as well. Complications of tified in the absence of the other. More commonly, calci-
involvement include atlantoaxial subluxation and sponta- fication precedes arthropathy, with calcification beginning
neous fractures of the odontoid process. in the fibrocartilage and subsequently involving the hyaline
cartilage of large joints and eventually the hyaline carti-
lage of the small joints of the hands and feet. In some
Sacroiliac Joint
instances, however, arthropathy develops in patients who
Abnormalities in the sacroiliac joint have been observed demonstrate neither local (in the same joint) nor distant
in 45% to 50% of cases. Calcification of articular carti- (in any joint) calcification, and it is unusual to document
lage is infrequently identified on radiographic examina- the initial appearance of calcification after arthropathy.
A B
Chapter 34 Calcium Pyrophosphate Dihydrate Crystal Deposition Disease 471
MAGNETIC RESONANCE IMAGING
of the underlying articular disorder are usually apparent.
ABNORMALITIES
Diagnostic difficulty arises in certain cases of CPPD
CPPD crystal accumulation typically leads to masses with crystal deposition disease in which homogeneous, tumoral,
predominantly low signal intensity, although these masses intra-articular or periarticular calcifications (tophaceous
may show enhancement of signal intensity after the intra- pseudogout), or osteochondral fragments are seen or in
venous administration of a gadolinium-containing con- cases with both CPPD and calcium hydroxyapatite accu-
trast agent. Small collections of CPPD crystals in the mulation (mixed crystal deposition disease).
menisci of the knee and the triangular fibrocartilage of
the wrist are easily overlooked during magnetic resonance
(MR) imaging examinations, as are those within the Periarticular Calcification
intervertebral disc. Low signal intensity is characteristic Periarticular radiodense deposits may be associated with
of such calcific collections. In the menisci of the knee, metastatic calcification, dystrophic calcification, or calci-
however, regions of intermediate signal intensity may nosis. Such calcification may be seen in renal osteodys-
simulate meniscal tears. Similarly, in hyaline cartilage, trophy, idiopathic tumoral calcinosis, collagen vascular
CPPD crystal deposits appear as hypointense foci. Synovial diseases, milk-alkali syndrome, and hypervitaminosis D.
calcifications and intra-articular debris (often composed Calcific periarthritis or peritendinitis may be asso-
of both CPPD and calcium hydroxyapatite crystals) are ciated with the deposition of calcium hydroxyapatite
also apparent on MR imaging. crystals in tendons and bursae, particularly about the
shoulder. Tendinous calcification in CPPD crystal deposi-
DIFFERENTIAL DIAGNOSIS tion disease produces similar abnormalities, but the
deposits may be more elongated. Differentiation between
Intra-articular Calcification
these two disorders is aided by the absence of chondro-
Chondrocalcinosis, particularly of the menisci of the knee, calcinosis in patients with calcific periarthritis.
may relate not only to the presence of CPPD crystals but
also to the deposition of other crystalline material, such
as dicalcium phosphate dihydrate and calcium hydroxy- Pyrophosphate Arthropathy
apatite. Chondrocalcinosis of more than one set of joints The arthropathy of CPPD crystal deposition disease
usually indicates CPPD crystal deposition. closely resembles degenerative joint disease but, as indi-
Additional causes of intra-articular radiodense lesions cated previously, involves unusual joints and compart-
that may simulate CPPD crystal deposition generally ments and is associated with extensive sclerosis, multiple
produce abnormalities of a single joint and include cysts, bone fragmentation, osseous debris, and variable
meniscal ossicles of the knee; osteochondritis dissecans of osteophyte formation (Table 34–4). The degree of bone
the knee, ankle, or elbow; osteonecrosis; and idiopathic fragmentation, sclerosis, and collapse encountered in
synovial osteochondromatosis. Disintegration of articular patients with CPPD crystal deposition disease is reminis-
surfaces with the production of single or multiple calcific cent of the changes accompanying other disorders such
and ossific dense areas may be seen in a variety of other as neuropathic osteoarthropathy, steroid-induced arthrop-
articular disorders, including neuropathic osteoarthrop- athy, osteonecrosis, and infection. The absence of clinical
athy, steroid-induced arthropathy, infection, gout, and features typical of these other disorders usually ensures
rheumatoid arthritis. In these cases, characteristic signs an accurate diagnosis of CPPD crystal deposition disease.
TABLE 34–4
Differential Diagnosis of Pyrophosphate Arthropathy
Bone erosion is not a feature of CPPD crystal deposi- phalanges in CPPD crystal deposition disease allows its
tion disease. The absence of erosive change usually aids differentiation from rheumatoid arthritis.
in distinguishing this condition from rheumatoid arthritis The arthropathies of idiopathic CPPD crystal deposi-
and related synovial disorders such as psoriatic arthritis tion disease and hemochromatosis are very similar, but
and ankylosing spondylitis. The appearance of joint space subtle differences have been defined. Although both dis-
narrowing, bony eburnation, osteophytosis, and sclerosis orders involve the second and third metacarpophalangeal
in CPPD crystal deposition disease may resemble the joints, changes in the fourth and fifth digits are more
features of gout. Differentiation between gout and CPPD prevalent in hemochromatosis. Peculiar hooklike osteo-
crystal deposition disease is further complicated by the phytes on the radial aspect of the metacarpal heads are
occasional presence of periarticular calcification and also more characteristic of hemochromatosis than idio-
chondrocalcinosis in gouty arthritis. In gouty arthritis, pathic CPPD crystal deposition disease.
however, focal soft tissue swelling and bone erosion—
findings not generally seen in CPPD crystal deposition Hip. Focal loss of joint space in the superolateral aspect
disease—may be noted. of this joint may occur in patients with CPPD crystal
deposition disease and mimic the findings of degenera-
Knee. Although the distribution of compartmental tive joint disease. Diffuse joint space loss may also be
abnormalities in the knee in CPPD crystal deposition seen, simulating the pattern of joint space loss in rheu-
disease may be identical to that of degenerative joint matoid arthritis and ankylosing spondylitis. The degree
disease, isolated patellofemoral compartment changes are of bony sclerosis and osteophytosis in CPPD crystal
an important diagnostic clue suggesting the presence of deposition disease allows its differentiation from rheuma-
pyrophosphate arthropathy. In degenerative joint disease, toid arthritis, but the hip involvement of pyrophosphate
patellofemoral changes are frequent, but in most cases arthropathy may resemble that of ankylosing spondylitis.
they are combined with alterations in the medial femoro- The presence of bone fragmentation and collapse and the
tibial compartment. Symmetrical loss of joint space in the absence of sacroiliac joint abnormalities in CPPD crystal
medial and lateral femorotibial spaces or tricompartmen- deposition disease are findings that are not apparent in
tal involvement is common in rheumatoid arthritis and ankylosing spondylitis.
infection. Patellofemoral compartment erosions may be A rapidly destructive pattern of hip disease has been
noted in patients with primary hyperparathyroidism or described, termed rapidly destructive osteoarthritis. Rapid
renal osteodystrophy, but additional skeletal findings allow progression of hip pain and disability is a consistent clin-
the diagnosis of these conditions. ical feature. Radiographs reveal flattening of the femoral
head, joint space loss, subchondral cysts, sclerosis on both
Wrist. Selective involvement of the radiocarpal compart- sides of the joint, and lateral subluxation of the femoral
ment of the wrist is most characteristic of CPPD crystal head. Acetabular protrusion may or may not be present.
deposition disease. Degenerative joint disease in the wrist The resulting imaging features resemble those of neuro-
usually produces changes in the first carpometacarpal pathic osteoarthropathy or infection. The features also
and trapezioscaphoid areas rather than in the radiocarpal resemble those of osteonecrosis. A similar pattern of hip
compartment. After injury, however, osteoarthritis can destruction may occur in patients with calcium hydroxy-
appear in any region of the wrist, including the radio- apatite or pyrophosphate crystal deposition disease.
scaphoid, radiolunate, and lunate-capitate spaces. Radio-
carpal compartment changes may occasionally be seen in Foot. Selective involvement of the talonavicular portion
patients with gout or occupation-related degenerative of the talocalcaneonavicular joint is observed in some
disease. Rheumatoid arthritis also involves this joint, but patients with pyrophosphate arthropathy. This distribu-
additional changes in other compartments of the wrist tion of abnormalities is also observed in the neuropathic
are generally apparent in this disease. In rheumatoid osteoarthropathy accompanying diabetes mellitus, and
arthritis, the inferior radioulnar compartment demon- differentiation of the midfoot changes in these two dis-
strates extensive abnormalities; this site is not commonly orders may be difficult. The absence of significant erosion
involved in pyrophosphate arthropathy. of the first metatarsophalangeal joint distinguishes
The SLAC pattern of osteoarthritis, occurring either pyrophosphate arthropathy from gouty arthritis.
spontaneously or after trauma, is characterized by
narrowing of both the radioscaphoid and capitolunate Spine. Disc calcification related to CPPD crystal deposi-
spaces. A similar pattern of arthropathy is characteristic tion initially occurs in the outer fibers of the anulus
of CPPD crystal deposition disease and, in the presence fibrosus. With progressive calcification, widespread disc
of chondrocalcinosis and the absence of traumatic injury, deposits resemble the changes of alkaptonuria. The
strongly suggests the diagnosis of CPPD crystal deposi- resemblance between these two disorders is accentuated
tion disease. by the possible occurrence of both peripheral arthropathy
and chondrocalcinosis in patients with alkaptonuria. The
Metacarpophalangeal Joints. The metacarpophalangeal disc space narrowing in the thoracic and lumbar spine in
joints are frequently altered in CPPD crystal deposition CPPD crystal deposition disease is identical to that seen
disease. The changes in this location predominate over in intervertebral osteochondrosis (degenerative disease
changes at the interphalangeal joints, whereas the con- of the nucleus pulposus). Vertebral body sclerosis may
verse is true in degenerative joint disease. The absence of be observed in both disorders, but the degree of bony
osseous erosion of the metacarpal heads and proximal eburnation and the possible occurrence of vertebral body
Chapter 34 Calcium Pyrophosphate Dihydrate Crystal Deposition Disease 473
destruction in CPPD crystal deposition disease allow an Martel W, McCarter DK, Solsky MA, et al: Further observa-
accurate diagnosis. tions on the arthropathy of calcium pyrophosphate crystal
Cervical spine subluxation, particularly at the atlanto- deposition disease. Radiology 141:1, 1981.
axial junction, in CPPD crystal deposition disease resem- McCarty DJ, Hollander JL: Identification of urate crystals in
gouty synovial fluid. Ann Intern Med 54:452, 1961.
bles the findings of rheumatoid arthritis. The absence
McCarty DJ Jr, Kohn NN, Faires JS: The significance of calcium
of discovertebral and apophyseal joint erosions in pyro- phosphate crystals in the synovial fluid of arthritis patients:
phosphate arthropathy distinguishes it from rheumatoid The “pseudogout” syndrome. Ann Intern Med 56:711, 1962.
arthritis. McCarty DJ, Silcox DC, Coe F, et al: Diseases associated with
calcium pyrophosphate dihydrate crystal deposition. Am J
Med 56:704, 1974.
FURTHER READING Resnick D, Pineda C: Vertebral involvement in calcium pyro-
phosphate dihydrate crystal deposition disease. Radiographic-
Adamson TC III, Resnik CS, Guerra J Jr, et al: Hand and wrist pathologic correlation. Radiology 158:55, 1984.
arthropathies of hemochromatosis and calcium pyrophos- Resnick D, Niwayama G, Goergen TG, et al: Clinical, radio-
phate deposition disease: Distinct radiographic features. graphic and pathologic abnormalities in calcium pyrophos-
Radiology 147:377, 1983. phate dihydrate deposition disease (CPPD): Pseudogout.
Burke BJ, Escobedo EM, Wilson AJ, et al: Chondrocalcinosis Radiology 122:1, 1977.
mimicking a meniscal tear on MR imaging. AJR 170:69, Rosenberg ZS, Shankman S, Steiner GC, et al: Rapid destruc-
1998. tive osteoarthritis: Clinical, radiographic, and pathologic fea-
Chen C, Chandnani VP, Kang HS, et al: Scapholunate advanced tures. Radiology 182:213, 1992.
collapse: A common wrist abnormality in calcium pyrophos- Rynes RI, Merzig EG: Calcium pyrophosphate crystal deposi-
phate dihydrate crystal deposition disease. Radiology 177: tion disease and hyperparathyroidism: A controlled, prospec-
459, 1990. tive study. J Rheumatol 5:460, 1978.
Dieppe PA, Alexander GJM, Jones HE, et al: Pyrophosphate Utsinger PD, Zvaifler NJ, Resnick D: Calcium pyrophosphate
arthropathy: A clinical and radiological study of 105 cases. dihydrate deposition disease without chondrocalcinosis. J
Ann Rheum Dis 41:371, 1982. Rheumatol 2:258, 1975.
Leisen J: Calcium pyrophosphate dihydrate deposition disease: Watt I, Dieppe PA: Medial femoral condyle necrosis and chon-
Tumorous form. AJR 138:962, 1982. drocalcinosis: A causal relationship? Br J Radiol 56:7, 1983.
Ling D, Murphy WA, Kyriakos M: Tophaceous pseudogout. Zitbrenan D, Sitaj S: Chondrocalcinosis articularis. Section I.
AJR 138:162, 1982. Clinical and radiological study. Ann Rheum Dis 22:142,
Martel W, Champion CK, Thompson GR, et al: A roentgeno- 1963.
logically distinctive arthropathy in some patients with the
pseudogout syndrome. AJR 109:587, 1970.
35
CHAPTER 35
Calcium Hydroxyapatite Crystal PERIARTICULAR CRYSTAL DEPOSITION
Clinical Features
Deposition Disease
Recurrent painful periarticular calcific deposits in tendons
and soft tissues have been described by a variety of names,
including calcific tendinitis, peritendinitis and bursitis, and
SUMMARY OF KEY FEATURES hydroxyapatite rheumatism. Although inflammation about
Calcium hydroxyapatite (HA) crystal deposition can lead a calcified tendon (peritendinitis) may be noted, degenera-
to periarticular accumulations that are associated with tion (tendinosis or tendinopathy) rather than inflamma-
typical clinical and radiographic findings. The radiographic tion (tendinitis) is evident in the involved tendon. These
manifestations are observed most frequently in the deposits are usually monoarticular in distribution, although
shoulder, although similar abnormalities may be apparent they can be polyarticular. The most frequent site of involve-
at other articular sites. Intra-articular alterations have ment is the shoulder, although other sites such as the wrist,
also been documented as a consequence of calcium HA hand, foot, elbow, hip, neck, and lumbar spine may be
crystal deposition disease, and the combination of involved. The disease affects both men and women and is
calcium HA and calcium pyrophosphate dihydrate particularly common between 40 and 70 years of age.
(CPPD) crystal accumulation (mixed calcium phosphate Acute symptoms include pain, tenderness on pressure,
crystal deposition disease) is being identified with local edema or swelling, restricted motion, and mild fever.
greater frequency. Chronic symptoms and signs may also be present. In
many patients, deposits are detected radiographically in
persons who are entirely asymptomatic.
474
Chapter 35 Calcium Hydroxyapatite Crystal Deposition Disease 475
was assumed that such collections resulted from the Teres Minor Tendon Calcification. The teres minor tendon
deposition of calcium in degenerated, injured, or necrotic attaches to the posterior aspect of the greater tuberosity
tissue, perhaps related to a change in local pH values as a below the site of attachment of the infraspinatus tendon.
result of increased alkalinity or decreased carbon dioxide Calcification within this tendon is projected over the
tension. More recently, documentation of familial cases, humeral head in external rotation and moves laterally
polyarticular involvement, and an increased frequency in internal rotation, where it may be seen in profile (see
of certain histocompatibility antigens in these patients Fig. 35–4).
suggest that multiple local, systemic, metabolic, and
genetic factors may be involved. Although the precise Subscapularis Tendon Calcification. The subscapularis
pathogenesis of calcium HA crystal deposition may tendon attaches mainly to the lesser tuberosity of the
vary among these many causes, tissue degeneration is humerus. Subscapularis tendon calcification is projected
a common theme. Thus, calcium HA crystal deposi- over the humeral head in external rotation because of the
tion occurring in tendons is more accurately termed anterior position of the lesser tuberosity (see Fig. 35–4).
calcific tendinosis rather than calcific tendinitis, and This calcification moves medially on internal rotation
the former designation is used for the remainder of and can be seen near the inner surface of the humeral
this chapter. head in this position. Calcification is evident tangentially
In the past, many techniques were used to treat calcific adjacent to the lesser tuberosity in an axillary projection.
tendinosis and bursitis of the shoulder, with varying
success. These techniques included heat and cold therapy, Bicipital Tendon Calcification. The long head of the biceps
ultrasonic and diathermic procedures, needling and tendon attaches to the superior aspect of the glenoid
aspiration, steroid injections, surgery, and radiotherapy. fossa after passing through the bicipital groove, and calcific
In certain instances, calcific collections may diminish in tendinosis of this structure appears as a radiodense area
size and even disappear; at other times, the calcification that does not move appreciably on external or internal
may become more extensive or the deposits may remain rotation. The short head of the biceps attaches to the
unchanged in size. coracoid process. Calcification in this tendon is apparent
adjacent to the coracoid tip. Calcification in the long
head of the biceps is also seen along the shaft of the
Calcific Tendinosis and Bursitis at Specific humerus. In this location, calcification occurs at the junc-
Sites tion of the tendon and muscle (Fig. 35–5). Its appearance
simulates that of an osseous body trapped in the sheath of
Shoulder
the tendon.
The capsular, tendinous, ligamentous, and bursal tissues
about the shoulder are the most common sites of calcific Other Tendon Calcification. Other sites of tendon calcifi-
deposits. These deposits occur in about 3% of adults, are cation about the shoulder are recorded infrequently.
bilateral in almost 50% of persons, and are most fre- These sites include the pectoralis major tendon, the
quently in the supraspinatus tendon. Clinical findings are tendon of the long head of the triceps muscle (with
apparent in about one third of patients. calcification adjacent to the inferior margin of the
The radiographic appearance of shoulder calcification glenoid cavity), the deltoid attachment to the acromion,
depends on the exact location of the abnormal deposits; the trapezius insertion (with calcification adjacent to the
they are commonly encountered in the tendons of the acromioclavicular joint), and the subacromial bursa.
rotator cuff, adjacent tendons, and bursae. The precise Subacromial bursa calcification appears as a teardrop-
appearance and position of the calcification depend on shaped, ulcerated, or “skullcap” radiodense area adjacent
the disease and the specific tendon in which the deposits to the superolateral aspect of the joint capsule and pos-
are located (Figs. 35–1 and 35–2). sibly extending under the greater tuberosity (Fig. 35–6).
A B
C D
Figure 35–1. Periarticular crystal deposition in the shoulder: phases of the disease. A, Silent phase.
Subclinical deposition of calcium occurs in the substance of the rotator cuff tendons. B, Mechanical phase—
elevation of bursal floor. As the deposits increase in size, the floor of the subdeltoid (subacromial) bursa is
raised. C, Mechanical phase–sub-bursal rupture. Observe that rupture of the calcific deposits has occurred
beneath the floor of the bursa. D, Mechanical phase–intrabursal rupture. The entire deposit is being
expelled into the subdeltoid bursa.
of calcification appears to be within the flexor carpi any location. Calcifications may relate to other tendinous
ulnaris tendon (Fig. 35–7). In the hand, deposits show a structures, such as the piriformis, rectus femoris, vastus
predilection for the regions of the metacarpophalangeal lateralis, adductor magnus, and biceps femoris.
joints and fingers.
Knee
Hip and Pelvis Calcific tendinosis and bursitis about the knee have been
Calcific deposits are frequent in the gluteal insertions described, particularly adjacent to the femoral condyles,
into the greater trochanter and in the surrounding bursae, fibular head, and prepatellar region.
where they appear as single or multiple cloudlike linear,
triangular, or circular radiodense areas. Calcification in
Ankle, Foot, and Heel
the femoral insertion of the gluteus maximus muscle
(Fig. 35–8) is particularly characteristic and leads to a Calcific deposits may occasionally be seen about the
radiodense shadow adjacent to the proximal posterior ankle, foot, and heel. Involvement may include the tendons
portion of the femur, best observed in the frog-leg view. of the flexor hallucis brevis, flexor hallucis longus, and
Adjacent erosion of the femoral cortex is not unusual at peroneus muscles.
Chapter 35 Calcium Hydroxyapatite Crystal Deposition Disease 477
E F
G
Figure 35–1.—Continued E, Adhesive periarthritis. Note the adduction of the shoulder with adhesive
bursitis. F, Intraosseous loculation. The calcific deposit has extended into the bone. G, Dumbbell loculation.
Rarely, a biloculated deposit may be seen as a result of pressure from the adjacent coracoacromial ligament.
(Redrawn from Moseley HF: Shoulder Lesions, 3rd ed. Edinburgh, E&S Livingstone, 1969.)
Neck
magnetic resonance (MR) imaging findings of this
Calcific tendinosis of the neck may appear within the disorder (Fig. 35–10), and the calcification may be diffi-
longus colli muscle and tendon, the principal flexor of the cult to appreciate on many MR imaging sequences (except
cervical spine (Fig. 35–9). Tendinosis and peritendinitis for fast spin echo or gradient echo sequences) unless
in this region may result in acute neck and occipital pain, it is large. The calcification is better delineated with
rigidity, and dysphagia. Radiographic findings include routine radiography, although computed tomography
prevertebral soft tissue swelling, particularly in the upper (CT) scanning is especially useful in areas where the
cervical region, as well as amorphous calcification, which osseous anatomy is complex. Inflamed and edematous
is usually seen anterior to the second cervical vertebra, soft tissues and muscles appear as regions of high signal
just inferior to the body of the atlas. Resorption of calci- intensity on T2-weighted spin echo and other fluid-
fication is common and may be complete in 1 to 2 weeks, sensitive sequences, and signal enhancement after the
with disappearance of the soft tissue swelling. intravenous administration of gadolinium compounds is
to be expected. Bone erosion beneath calcified tendons is
well shown by CT and, to a lesser extent, MR imaging,
Magnetic Resonance Imaging and Computed and marrow signal intensity may be altered because of
Tomography Features intraosseous edema. The calcified collections are of low
The occurrence of pain and swelling at an involved site, signal intensity on all MR imaging sequences, and they
indicative of inflammation about calcific tissue, is well may be more apparent as a result of the surrounding
documented. This inflammatory response dominates the inflammatory response.
478 Section VIII Crystal-Induced and Related Diseases
A B
Figure 35–2. Periarticular crystal deposition in the shoulder: sites of tendon calcification. A, In external
rotation, calcification in the supraspinatus (SS) tendon is observed adjacent to the greater tuberosity,
whereas that in the infraspinatus (IS) and teres minor (TM) tendons overlies the greater tuberosity.
Calcification in the subscapularis (SSc) tendon overlies the lesser tuberosity. B, In internal rotation,
calcification in the infraspinatus (IS) and teres minor (TM) tendons is seen lateral to the greater tuberosity,
calcification in the supraspinatus (SS) tendon is projected over the greater tuberosity, and calcification in the
subscapularis (SSc) tendon rotates medially, adjacent to the inner margin of the humeral head.
A B
Figure 35–3. Periarticular crystal deposition: supraspinatus tendon calcification. A, In external rotation,
calcification is apparent above the greater tuberosity (arrow). B, In internal rotation, the calcification moves
medially (arrow).
Chapter 35 Calcium Hydroxyapatite Crystal Deposition Disease 479
Differential Diagnosis
Tendinosis with peritendinitis and bursitis may produce
soft tissue swelling without radiographically recognizable
calcification. Stenosing tenosynovitis of the abductor
pollicis longus and extensor pollicis brevis muscles (de
Quervain’s disease) is well known, and similar involve-
ment is recognized at other sites, such as the flexor
and extensor digital tendons, flexor carpi radialis tendon,
tibialis posterior tendon, common peroneal tendon sheath,
and flexor tendons of the wrist within the carpal tunnel.
An example of noncalcific bursitis is Haglund’s syndrome,
characterized by painful soft tissue swelling at the level of
the Achilles tendon insertion in the calcaneus. A localized
Figure 35–5. Periarticular crystal deposition. Calcification in area of soft tissue prominence termed a “pump bump”
the biceps tendon (arrows) is shown in external rotation. may be encountered. The condition appears to be
480 Section VIII Crystal-Induced and Related Diseases
A B
C D
E F
Figure 35–9. Periarticular crystal deposition in the longus colli in a 36-year-old woman. A, Drawing of a
sagittal section of the neck indicates the course of the longus colli muscle (arrow), which attaches to the
anterior tubercle of the atlas and the anterior surface of the second through fourth cervical vertebrae.
B, Lateral radiograph shows soft tissue swelling with calcification immediately below the anterior arch of
C1. C and D, Corresponding reformatted sagittal (C) and axial (D) CT scans of the spine show the
calcification (arrows) to better advantage. E and F, Corresponding sagittal T1-weighted (TR/TE, 500/15)
(E) and conventional T2-weighted (TR/TE, 2000/90) (F) spin echo MR images show the calcification as an
area of low signal intensity (arrow). Note the surrounding high signal intensity, on T2-weighted image (F)
representing inflammatory changes as well as marrow edema.
482 Section VIII Crystal-Induced and Related Diseases
A B
Figure 35–10. Periarticular crystal deposition in the subdeltoid bursa: MR imaging abnormalities.
Coronal oblique T1-weighted (TR/TE, 700/20) (A) and T2-weighted (TR/TE, 1800/80) (B) spin echo MR
images show areas of low signal intensity, reflecting calcification (arrows), which in (B) is associated with
surrounding inflammatory tissue and fluid of high signal intensity.
TABLE 35–1
Conditions Associated with Periarticular Calcification
numerous patients. When the crystals occur together, it Certain radiographic signs should raise the possibility
is difficult to precisely identify their relative roles in the of mixed calcium phosphate crystal deposition disease.
production of joint damage. The radiographic diagnosis An association appears to exist between intra-articular
of such mixed crystal disease is also difficult. A diagnostic accumulation of CPPD crystals (in the form of chondro-
clue is provided by cartilage calcification (chondrocal- calcinosis) and periarticular accumulation of calcium HA
cinosis), which, when widespread, is much more charac- crystals (in the form of capsular and tendinous calcifica-
teristic of CPPD crystal deposition than calcium HA tion). The presence of both disorders should be suspect-
crystal accumulation. Conversely, in calcium HA crystal ed if radiographs reveal extensive cartilage calcification
deposition, diffuse amorphous calcification within the (indicative of CPPD crystal deposition) and diffuse
joint is more typical. capsular calcification or dense homogeneous calcific
A B
Chapter 35 Calcium Hydroxyapatite Crystal Deposition Disease 485
Figure 35–17. Mixed calcium phosphate
crystal deposition disease. This 83-year-old
man had widespread chondrocalcinosis,
presumably related to calcium pyrophosphate
dihydrate crystal deposition. Intra-articular
bodies in the knee were removed and found to
be composed of calcium hydroxyapatite
crystals. A, Routine radiography shows diffuse
calcification in the suprapatellar bursa of the
knee. B, Transaxial gradient echo MR image
(TR/TE, 450/15; flip angle, 60 degrees)
reveals joint fluid of relatively high signal
intensity containing foci of low signal inten-
sity. Calcification within the synovial mem-
brane is also evident.
A B
collections at tendinous insertions (indicative of calcium Hayes CW, Conway WF: Calcium hydroxyapatite deposition
HA crystal deposition) (Fig. 35–17). disease. Radiographics 10:1031, 1990.
The number of cases of mixed calcium phosphate Hayes CW, Rosenthal DI, Plata MJ, et al: Calcific tendinitis in
crystal deposition disease is expected to rise as awareness unusual locations associated with cortical bone erosion. AJR
of the condition increases and diagnostic methods become 149:967, 1987.
more readily available. It is evident that the factors Kraemer EJ, El-Khoury GY: Atypical calcific tendinitis with
necessary for deposition of HA and CPPD crystals are cortical erosions. Skeletal Radiol 29:690, 2000.
not mutually exclusive. Indeed, it is possible that cases in Martin JF, Brogdon BG: Peritendinitis calcarea of the hand and
which both crystals are present are more numerous than wrist. AJR 78:74, 1957.
those in which one or the other is present alone. McCarty DJ, Gatter RA: Recurrent acute inflammation
associated with focal apatite crystal deposition. Arthritis
Rheum 9:804, 1966.
McCarty DJ, Halverson PB, Carrera GF, et al: “Milwaukee
FURTHER READING shoulder” — association of microspheroids containing
hydroxyapatite crystals, active collagenase, and neutral
Bonavita JA, Dalinka MK, Schumacher HR Jr: Hydroxyapatite protease with rotator cuff defects. I. Clinical aspects.
deposition disease. Radiology 134:621, 1980. Arthritis Rheum 24:464, 1981.
Codman EA: The Shoulder. Boston, Thomas-Todd Co, 1934. Neer CS II, Craig EV, Fukuda H: Cuff-tear arthropathy. J.
Dieppe PA, Doherty M, MacFarlane DG, et al: Apatite Bone Joint Surg [Am] 65:1232, 1983.
associated destructive arthritis. Br J Rheumatol 23:84, 1984. Newmark H III, Forrester DM, Brown JC, et al: Calcific
Fritz P, Bardin T, Laredo J-D, et al: Paradiaphyseal calcific tendinitis of the neck. Radiology 128:355, 1978.
tendinitis with cortical bone erosion. Arthritis Rheum Pavlov H, Heneghan MA, Hersh A,et al: The Haglund
37:718, 1994. syndrome: Initial and differential diagnosis. Radiology
Goldman AB: Calcific tendinitis of the long head of the biceps 144:83, 1982.
brachii distal to the glenohumeral joint: Plain film radio- Rosenberg ZS, Shankman S, Steiner GC, et al: Rapid destruc-
graphic findings. AJR 153:1011, 1989. tive osteoarthritis: Clinical, radiographic, and pathologic fea-
Halverson PB, McCarty DJ: Clinical aspects of basic calcium tures. Radiology 182:213, 1992.
phosphate crystal deposition. Rheum Dis Clin North Am Schumacher HR, Somlyo AP, Tse RL, et al: Arthritis associated
14:427, 1988. with apatite crystals. Ann Intern Med 87:411, 1977.
Halverson PB, McCarty DJ: Patterns of radiographic abnor- Uhthoff HK, Sarkar K, Maynard JA: Calcifying tendinitis. A
malities associated with basic calcium phosphate and new concept of its pathogenesis. Clin Orthop 118:164, 1976.
calcium pyrophosphate dihydrate crystal deposition in the ViGario DG, Keats TE: Localization of calcific deposits in the
knee. Ann Rheum Dis 45:603, 1986. shoulder. AJR 108:806, 1970.
Halverson PB, McCarty DJ, Cheung HS, et al: Milwaukee
shoulder syndrome: Eleven additional cases with involve-
ment of the knee in seven (basic calcium phosphate crystal
deposition disease). Semin Arthritis Rheum 14:36, 1984.
36
CHAPTER 36
Hemochromatosis and Wilson’s Clinical Features
Disease Most patients with primary hemochromatosis become
symptomatic between the ages of 40 and 60 years. The
disorder is 8 to 20 times more frequent in men than in
SUMMARY OF KEY FEATURES women, and women with this disorder frequently report
absent or scanty menses. Initial clinical manifestations
The skeletal manifestations of hemochromatosis include relate to the classic triad of the disease: cirrhosis, skin
osteoporosis, chondrocalcinosis, and a distinctive pigmentation, and diabetes. Subsequent complaints relate
arthropathy characterized by joint space narrowing, to ascites and cardiac failure.
subchondral cyst formation, and osteophyte formation. The arthropathy associated with hemochromatosis
These abnormalities resemble the findings of calcium is more frequent in genetic hemochromatosis than in
pyrophosphate dihydrate (CPPD) crystal deposition secondary hemochromatosis and is manifested as a
disease but are associated with more uniform loss of noninflammatory condition initially involving the small
joint space and a less progressive course. joints of the hands, particularly the second and third
Wilson’s disease is associated with distinctive skeletal metacarpophalangeal joints, and eventually involving
abnormalities, including osteopenia, bone fragmentation large articulations.
and cyst formation, ossicles, poorly defined subchondral
bone, and osteochondritis dissecans. These findings
superficially resemble the changes in hemochromatosis Pathologic Features
and idiopathic CPPD crystal deposition disease. Articular abnormalities consist of two major features:
abnormal amounts of hemosiderin granules, and CPPD
crystal deposition disease. Both iron and CPPD crystals
may accumulate in the same joint.
HEMOCHROMATOSIS
Disorders of iron storage can be conveniently divided Synovial Membrane Abnormalities. Hemosiderin granules
into those that are primary or idiopathic, and those that are seen in the synovial membrane. Increased synovial
are secondary to iron overload states. Such overload states tissue deposition of iron is not limited to hemochro-
are associated with an increase in the availability of iron matosis but may be observed in a variety of disorders,
related to parenteral or oral intake, ineffective erythro- including rheumatoid arthritis, degenerative disease,
poiesis, or inherited disease. pigmented villonodular synovitis, hemophilia, and
Primary hemochromatosis, a rare autosomal recessive hemarthrosis. In these situations, light microscopy has
disease, is believed to be a consequence of a genetically revealed that the iron occurs predominantly in deep
determined error of metabolism characterized by an macrophages, in contrast to its localization in synovial
unexplained increased absorption of iron from the gastro- lining cells in patients with hemochromatosis. CPPD
intestinal tract. The clinical findings include a classic triad crystals may be seen in the synovium, particularly in the
of abnormalities: bronze pigmentation of the skin, superficial layers of the membrane.
cirrhosis, and diabetes. Specific clinical manifestations
relate to tissue damage at the site of abnormal iron accu- Cartilage Abnormalities. Chondrocalcinosis occurs in as
mulation: iron within the parenchymal cells of the liver is many as 30% of patients with hemochromatosis, either as
associated with hypertrophy and cirrhosis, iron deposits an isolated phenomenon or in association with structural
in the pancreas result in diabetes, iron and melanin accu- joint damage (Fig. 36–1). Calcification related to CPPD
mulations in the skin produce abnormal pigmentation, crystal deposition involves predominantly the fibrocar-
and cardiac deposition of iron results in heart failure. tilage and hyaline cartilage of the knee but is also fre-
Secondary hemochromatosis is associated with an quent in the symphysis pubis, wrist, and intervertebral
increased intake and accumulation of iron of known cause, disc. Cartilaginous fibrillation and erosion with partial or
such as alcoholic cirrhosis, multiple blood transfusions, complete denudation may be observed.
refractory anemia, and chronic excess oral iron ingestion.
In both primary and secondary hemochromatosis, signi- Bone Abnormalities. The osseous abnormalities in hemo-
ficant iron overload occurs only after many years, so the chromatosis are similar to those noted in idiopathic
onset of disease is generally between 40 and 60 years of CPPD crystal deposition disease. Specifically, the presence
age. Hemochromatosis is less frequent in women, pre- of bony eburnation and cysts and the absence of sig-
sumably because of menstrual blood loss. The disease is nificant osteophyte formation are findings of pyrophos-
diagnosed by detection of an elevated serum iron concen- phate arthropathy. Because CPPD crystal deposition is
tration and increased saturation of the plasma iron- a documented feature of hemochromatosis, the bone
binding protein transferrin, combined with a typical changes in this disease may be related, at least in part, to
histologic appearance on liver biopsy. the presence of these crystals.
486
Chapter 36 Hemochromatosis and Wilson’s Disease 487
Figure 36–1. Radiographic findings in hemochromatosis:
chondrocalcinosis. A, Magnification radiography outlines
calcium deposits (arrowhead) in cartilage in the wrist. B, Diffuse
chondrocalcinosis of both fibrocartilage and hyaline cartilage is
present in the knee. C, In the symphysis pubis, chondrocalcinosis
(arrowhead) of fibrocartilage is apparent.
B C
space narrowing, sclerosis, and osteophytosis—definite cysts, osteophytes on the medial aspect of the
characteristics in its distribution and appearance enable metacarpal head, and flattening or mild collapse of the
it to be recognized on radiographic examination. The osseous surface. Although both CPPD crystal deposi-
arthropathy in hemochromatosis is almost identical to tion disease and hemochromatosis produce structural
the arthropathy in idiopathic CPPD crystal deposition alterations in the metacarpophalangeal joints, more
disease, and they share the following features: prevalent narrowing in these articulations (including
1. Involvement of unusual articular sites. Arthropathy may those in the fourth and fifth digits) and peculiar hook-
produce abnormalities at joints that are not commonly like osteophytes on the radial aspect of the metacarpal
involved in degenerative joint disease, such as the heads are characteristic of hemochromatosis.
metacarpophalangeal joints, midcarpal and radiocarpal 2. Widespread abnormalities of the wrist. Involvement
compartments of the wrists, elbows, and glenohumeral of the carpal bones may be noted in 30% to 50% of
articulations. patients (Fig. 36–3). Findings include joint space
2. Formation of large subchondral cystic lesions. Ar- narrowing, sclerosis, and cyst formation. Although the
thropathy may be associated with multiple cysts in the radiocarpal compartment may be affected, as in idio-
subchondral bone, which can occasionally attain a pathic CPPD crystal deposition disease, this compart-
large size. ment is occasionally unaffected in the arthropathy of
3. Uniform loss of articular space. Arthropathy may be hemochromatosis.
characterized by diffuse loss of articular space, an 3. Slowly progressive alterations. The arthropathy of
unusual finding in degenerative joint disease. The hemochromatosis may be slowly progressive. More
joint space loss is associated with subchondral bony rapid change, with fragmentation of bone, is more
eburnation and cyst formation. characteristic of idiopathic CPPD crystal deposition
Although it is difficult to differentiate the arthropathy disease.
of hemochromatosis and that of idiopathic CPPD crystal 4. Unusual pattern of osteophytosis. Beaklike osteophytes
deposition disease, several subtle findings appear to be of the metacarpal heads are particularly characteristic
more typical of hemochromatosis: (Fig. 36–4). Similarly, osteophytes may be apparent
1. Predilection for the metacarpophalangeal joints. These about other involved articulations, such as the hip and
joints are the most characteristic sites of involvement glenohumeral joint.
in hemochromatosis (Fig. 36–2). Abnormalities are The arthropathy of hemochromatosis may be wide-
particularly frequent in the second and third metacar- spread and occur throughout the skeleton, including
pophalangeal joints. The first, fourth, and fifth involvement of the hand and wrist, elbow, glenohumeral
metacarpophalangeal joints and the interphalangeal joint, hip, knee, ankle, foot, and spine. Radiographic
joints are less commonly abnormal. Involvement of abnormalities typically include joint space narrowing,
the metacarpophalangeal joints is associated with diffuse bony eburnation, subchondral cyst formation, and osteo-
or focal loss of articular space, well-defined subchon- phytosis. Chondrocalcinosis may or may not be apparent
dral bony eburnation, sharply marginated 1- to 3-mm in the involved joint.
Pathologic Features
The pathologic features of the osseous and articular
involvement in Wilson’s disease have not been well
delineated. Synovial biopsy findings include microvillus
formation, with mild lining cell hyperplasia, inflamma-
tory changes, and vasculitis. Copper accumulation is not
apparent in these synovial specimens, nor can evidence of
CPPD crystal deposition be found. Copper deposition in
articular cartilage, however, has been reported.
Radiographic Features
The radiographic features of the skeleton in Wilson’s
disease can be divided into osteopenia, chondrocalcinosis,
A arthropathy, and miscellaneous abnormalities.
Idiopathic CPPD
Crystal Deposition
Hemochromatosis Wilson’s Disease Disease
Osteopenia + + –
Chondrocalcinosis +* ?† +
Additional calcification ± ?† +
Joint space narrowing + + +
Subchondral cysts + + +
Rapid progression ± – +
Involvement of unusual +‡ + +
articular sites
*Hemochromatosis may be associated with more prominent hyaline cartilage calcification than noted in
idiopathic CPPD crystal deposition disease.
†Bone fragmentation in Wilson’s disease may resemble intra-articular and periarticular calcification.
‡Wrist involvement in hemochromatosis may be more diffuse and metacarpophalangeal joint involvement may
be more widespread than in idiopathic CPPD crystal deposition disease.
CPPD, calcium pyrophosphate dihydrate.
Differential Diagnosis
Additional radiographic characteristics of the arthrop-
athy in Wilson’s disease are small joint effusions; peculiar Although reports emphasize that the articular abnormal-
tonguelike osteophytes at bony prominences, such as ities in Wilson’s disease may be confused with degenera-
those about the elbow and ankle; and fluffy periostitis of tive joint disease, close inspection of the radiographs
the trochanters and inferior surface of the calcaneus (Fig. allows the differentiation of these two disorders. The
36–9). These latter changes may reflect periosteal bone distribution of articular abnormality in Wilson’s disease
formation at osseous sites of tendon and ligament includes a predilection for the small joints of the hands
attachment and resemble the alterations apparent in such and wrists, particularly the metacarpophalangeal articula-
rheumatoid variant disorders as ankylosing spondylitis, tions; this distribution is not observed in osteoarthritis.
psoriatic arthritis, and Reiter’s syndrome. The bone fragmentation and irregular osseous surfaces in
A B
492 Section VIII Crystal-Induced and Related Diseases
FURTHER READING
Adamson TC III, Resnik CS, Guerra J Jr, et al: Hand and wrist
arthropathies of hemochromatosis and calcium pyrophos-
phate deposition disease: Distinct radiographic features.
Radiology 147:377, 1983.
Atkins CJ, McIvor J, Smith PM, et al: Chondrocalcinosis and
arthropathy: Studies in haemochromatosis and in idiopathic
chondrocalcinosis. Q J Med 39:71, 1970.
Felitti VJ, Beutler E: New developments in hereditary hemo-
chromatosis. Am J Med Sci 318:257, 1999.
Finby N, Bearn AG: Roentgenographic abnormalities of the
skeletal system in Wilson’s disease (hepatolenticular degen-
eration). AJR Am J Roentgenol 79:603, 1958.
Hamilton E, Williams R, Barlow KA, et al: The arthropathy of
idiopathic haemochromatosis. Q J Med 37:171, 1968.
Hirsch JH, Killien C, Troupin RH: The arthropathy of hemo-
chromatosis. Radiology 118:591, 1976.
Mindelzun R, Elkin M, Scheinberg IH, et al: Skeletal changes
in Wilson’s disease: A radiological study. Radiology 94:127,
1970.
Moore EA, Vennart W, Jacoby RK, et al: Magnetic resonance
Figure 36–9. Radiographic findings in Wilson’s disease: bony manifestations of idiopathic hemochromatosis in the wrist.
proliferation. Observe the fluffy bone production on the lesser Br J Rheumatol 32:917, 1993.
trochanter (arrowhead). (From Golding DN, Walshe JM: Narvaez J, Alegre-Sancho JJ, Juanola X, et al: Arthropathy of
Arthropathy of Wilson’s disease. Study of clinical and radio- Wilson’s disease presenting as noninflammatory polyarthritis.
logical features in 32 patients. Ann Rheum Dis 36:99, 1977.) J Rheumatol 24:2494, 1997.
Chapter 36 Hemochromatosis and Wilson’s Disease 493
Schumacher HR Jr: Hemochromatosis and arthritis. Arthritis Yarze JC, Martin P, Muoz SJ, et al: Wilson’s disease: Current
Rheum 7:41, 1964. status. Am J Med 92:643, 1992.
Sinigaglia L, Fargion S, Ludovica A, et al: Bone and joint Yu-zhang X, Xue-zhe Z, Xian-hao X, et al: Radiologic study of
involvement in genetic hemochromatosis: Role of cirrhosis 42 cases of Wilson’s disease. Skeletal Radiol 13:114, 1985.
and iron overload. J Rheumatol 24:1809, 1997.
37
CHAPTER 37
Alkaptonuria Alkaptonuria: General name of a disease characterized
by the absence of homogentisate 1,2-dioxygenase and the
accumulation of homogentisic acid in the urine.
Ochronosis: The abnormal pigmentation, brown-black
SUMMARY OF KEY FEATURES in color, that may be observed in various connective
Alkaptonuria is a rare hereditary disorder resulting tissues in patients with alkaptonuria.
from an inability to metabolize homogentisic acid. Ochronotic arthropathy: Structural damage that results
Clinical and radiographic findings relate to from the pigmented deposits in the joints of the appen-
homogentisic aciduria and ochronosis. The latter dicular and axial skeletons.
feature is accompanied by typical radiographic and
pathologic findings in both spinal and extraspinal sites.
The most characteristic manifestations of ochronosis are GENERAL CLINICAL FEATURES
widespread disc calcification, with loss of intervertebral Alkaptonuria has a worldwide distribution and affects
disc height, and a distinctive arthropathy of the axial both men and women. Patterns of inheritance have not
and extra-axial articulations. Careful evaluation of the been consistent in previous descriptions of the disease.
radiographs usually allows an accurate diagnosis by In general, alkaptonuria is asymptomatic until adult life,
differentiating this condition from degenerative joint although in children, discoloration of urine may be
disease and ankylosing spondylitis, the two disorders detected. Alkaptonuria almost inevitably progresses to
with which it is most likely to be confused. ochronosis and arthropathy.
Ochronotic pigmentation is observed infrequently
before the age of 20 or 30 years, initially appearing as
mild pigmentation of the ears or sclerae, although more
widespread ocular abnormality may be apparent. In the
INTRODUCTION
ear, cartilage may appear thickened, with slate blue or
Alkaptonuria is a rare hereditary metabolic disorder gray discoloration. Discoloration of skin leading to stain-
characterized by absence of the enzyme homogentisic ing of clothes is caused by perspiration in the axillary and
acid oxidase. As a result of this defect, homogentisic acid, genital areas.
produced during the metabolism of phenylalanine and Ochronotic arthropathy is a manifestation of long-
tyrosine, accumulates and is excreted in the urine. When standing alkaptonuria. Symptoms and signs usually appear
the urine of affected persons is allowed to stand, the in the fourth decade of life. Initial clinical manifestations
homogentisic acid is oxidized to a melanin-like product, such as pain and limitation of motion may be seen in the
which causes the urine to gradually turn dark. The term hip, knee, and shoulder. Acute exacerbations of arthritis
ochronosis describes the bluish black pigmentation of may resemble rheumatoid arthritis. Joint effusions result
connective tissue that may be apparent at such sites as from fragmentation of friable cartilage, with subsequent
the skin, sclera, and ear in patients with alkaptonuria. irritation of the synovial membrane. Stiffness and low back
Ochronotic arthropathy results from pigment deposition pain, obliteration of the normal lumbar curve, thoracic
in the joints of the appendicular and axial skeletons of kyphosis, and restriction of motion are spinal manifesta-
affected individuals. tions of the disease, and an elderly person with alkap-
tonuria may be completely disabled. Symptoms and signs
of alkaptonuria may relate to ochronotic deposition in
PATHOGENESIS
other organs, including the cardiovascular and genitouri-
Under normal conditions, the enzyme homogentisate nary systems and upper respiratory tract.
1,2-dioxygenase metabolizes the homogentisic acid
formed from phenylalanine and tyrosine, and the acid is
not detectable in either urine or plasma. In alkaptonuria, GENERAL PATHOLOGIC FEATURES
no enzyme activity is present in either liver or kidney Abnormal pigmentation of the connective tissue may
tissue. As a result, homogentisic acid is not metabolized be observed in the sclera; cornea; laryngeal, tracheal,
and appears in the urine and plasma. Homogentisic acid bronchial, and costal cartilage; tympanic membrane; aortic
can be oxidized when alkali and oxygen are present, such intima; heart valves; kidney; and prostate. Pigment may
as occurs in the urine. An ochronotic pigment is formed also involve articular cartilage, tendons, and ligaments. It
that has a high affinity for cartilage and connective tissue appears coal black in some areas, and the chemical charac-
macromolecules. teristics of the pigment resemble those of melanin. In the
large diarthrodial joints, the pigment is located in the
deeper layers of cartilage. Pigmented necrotic cartilage
TERMINOLOGY
may become embedded within the marrow, and displaced
For clarity, it is necessary to define several terms related pieces of cartilage and bone may become lodged in the
to this disorder. synovial membrane, where they may stimulate metaplasia
494
Chapter 37 Alkaptonuria 495
of synovial lining cells into chondrocytes. Foreign body TABLE 37–1
reaction, synovial polyp formation, and osteochondral
bodies are observed. Inflammatory changes in the syn- Diagnostic Features of Ochronotic Arthropathy
ovial membrane are absent or minimal.
In the spine, ochronotic disc deterioration is related to Spinal Abnormalities
cartilage brittleness, similar to that observed in the carti- Osteoporosis of vertebral bodies
lage of diseased peripheral joints. Disc fragmentation Calcification and ossification of intervertebral discs
provokes an adjacent connective tissue reaction that is Disc space narrowing with vacuum phenomenon
identical to the reaction noted in the synovial membrane Small or absent osteophytes
about embedded cartilaginous and osseous debris. Loss of lumbar lordosis
Extraspinal Abnormalities
GENERAL RADIOGRAPHIC FEATURES Involvement of sacroiliac joints, symphysis pubis, and large
peripheral joints
The radiographic manifestations of skeletal involvement
Articular space narrowing
in alkaptonuria have been well outlined. These findings
can be divided into spinal and extraspinal abnormalities Bony sclerosis
(Table 37–1). Collapse and fragmentation with intra-articular osseous
bodies
Small or absent osteophytes
Spinal Abnormalities Tendinous calcification, ossification, and rupture
Disc calcification is the most characteristic abnormality Unusual involvement of hand, wrist, foot, elbow, and ankle
of the spine (Fig. 37–1). The calcium deposits are found
predominantly in the inner fibers of the anulus fibrosus,
although they may be distributed diffusely throughout
the intervertebral disc in a wafer-like configuration. They
consist of apatite crystals and are considered dystrophic
in nature. Calcification may appear in any segment of the tiple levels, is also suggestive of this diagnosis (Fig. 37–2).
vertebral column but has a predilection for the interver- Progressive ossification of the discs may be seen, with the
tebral discs of the lumbar spine; cervical spine alterations formation of marginal intervertebral bridges and obliter-
are less frequent. ation of the intervertebral space (Fig. 37–3). These bridges
Narrowing of the intervertebral disc space is also a resemble the syndesmophytes of ankylosing spondylitis.
characteristic manifestation of alkaptonuria. The so-called Narrowing of the intervertebral disc is associated with
vacuum phenomenon, with linear or circular radiolucent bony eburnation of apposing vertebral bodies and small
collections of gas overlying the intervertebral disc at mul- marginal osteophytes.
A B
496 Section VIII Crystal-Induced and Related Diseases
A B
Extraspinal Abnormalities
At the symphysis pubis, articular space narrowing, calci-
fication, bony eburnation, and fragmentation may be seen.
Similarly, joint space narrowing, sclerosis, and osteo-
Figure 37–3. Radiographic features of alkaptonuria: disc
calcification and ossification. Frontal radiograph of the lumbar phytosis may be apparent at the sacroiliac articulations
spine shows osteoporosis, disc calcification, and, at the (Fig. 37–4).
periphery of the intervertebral disc, ossification simulating the The knee is the most common site of peripheral
syndesmophytes of ankylosing spondylitis. (Courtesy of J. abnormality (Fig. 37–5). Findings in this location simu-
Loewy, MD, Saskatoon, Saskatchewan, Canada.) late those of uncomplicated degenerative joint disease
Chapter 37 Alkaptonuria 497
A B
Figure 37–4. Radiographic features of alkaptonuria: symphysis pubis and sacroiliac joint. A, Symphysis
pubis. The findings are extreme narrowing of the joint space and extensive bony sclerosis. Beaklike
osteophytes are seen. B, Sacroiliac joint. Diffuse narrowing of the articular space is associated with
irregularity of subchondral bone and sclerosis. The articular margins are more sharply defined than would
be expected in ankylosing spondylitis.
and consist of effusion, articular space narrowing, and collapse and fragmentation with multiple radiopaque intra-
bony sclerosis. Differences between these two diseases articular bodies, meager osteophytosis, and tendinous
may include isolated involvement of the lateral femorotibial calcification in alkaptonuria.
compartment, relatively symmetrical involvement of both Radiographic findings in the hip may be identical to
the medial and lateral femorotibial compartments, bony the changes of degenerative joint disease, with articular
space narrowing and sclerosis (Fig. 37–6). In some patients
with alkaptonuria, diffuse loss of joint space, severe
destruction with fragmentation and formation of intra-
articular cartilaginous and osseous bodies, and tendinous
calcification and ossification permit differentiation from
typical degenerative alterations. Involvement of the
glenohumeral joint, small joints of the hands and feet,
elbow, and ankle is not common.
DIFFERENTIAL DIAGNOSIS
Spinal Manifestations
Disc calcification in alkaptonuria may be confused with
that accompanying other disorders (Table 37–2). Dys-
trophic calcification of the nucleus pulposus is not infre-
quent. In these instances, the radiodense collections are
generally globular and confined to the central portion of
the intervertebral disc. This pattern of central disc calcifi-
cation is generally not widespread and can be readily
differentiated from the diffuse disc calcification at mul-
tiple levels of the spine in patients with alkaptonuria.
Calcification of the intervertebral disc may be seen as
a secondary phenomenon in patients with primary dis-
orders that lead to spinal ankylosis. Thus, disc calcifica-
tion can be noted in patients with ankylosing spondylitis,
Figure 37–5. Radiographic features of alkaptonuria: knee. In diffuse idiopathic skeletal hyperostosis, juvenile-onset
this patient, abnormalities include joint space narrowing in the rheumatoid arthritis, Klippel-Feil deformities, and sur-
lateral femorotibial and patellofemoral compartments, asso- gical fusion of the spine.
ciated with bony sclerosis, small osteophytes, and multiple Disc calcification is also observed in calcium pyro-
intra-articular osseous bodies (arrows). phosphate dihydrate (CPPD) crystal deposition disease.
498 Section VIII Crystal-Induced and Related Diseases
Extraspinal Manifestations
Involvement of extraspinal sites in patients with alkap-
tonuria may lead to a radiographic appearance reminis-
cent of degenerative joint disease. Certain features of
ochronotic arthropathy usually permit its identification
in these persons (Table 37–3):
1. Involvement of unusual articular sites. Arthropathy
of the glenohumeral joint resembling that of severe
degenerative articular disease should suggest the diag-
nosis of alkaptonuria, because considerable findings of
Figure 37–6. Radiographic features of alkaptonuria: hip. degenerative joint disease are unusual in this location
Extensive resorption and flattening of the femoral head are without a history of significant trauma. Similarly, severe
associated with a bizarre radiographic appearance. Findings changes in the sacroiliac or symphyseal joints may be
include joint space narrowing, mild acetabular protrusion, and a clue to the presence of ochronotic arthropathy.
bone fragmentation (arrow). 2. Unusual patterns of joint space loss. Ochronotic
arthropathy of the knee may lead to isolated lateral
femorotibial compartment changes or to diffuse loss
In this disorder, the deposits predominate in the outer of articular space in both the medial and lateral
fibers of the anulus fibrosus, and wafer-like collections femorotibial compartments. These patterns are
are not seen. Disc space loss, vacuum phenomenon, and unusual in degenerative joint disease.
vertebral body marginal sclerosis accompany interver- 3. Severe abnormalities with extreme sclerosis, fragmen-
tebral osteochondrosis. The findings are generally not tation, and intra-articular cartilaginous and osseous
as pronounced or widespread as in alkaptonuria, and they bodies. The severity of the changes accompanying
frequently occur in elderly patients. Nonetheless, the ochronotic arthropathy may be greater than those
usually seen in degenerative joint disease. The
production of multiple intra-articular bodies is
particularly characteristic.
TABLE 37–2 The peculiar pattern of “degenerative” arthropathy
Disc Calcification that is characteristic of alkaptonuria may be simulated by
Site of Nature of
Diagnosis Calcification Calcification TABLE 37–3
Ochronosis AF, NP HA Ochronotic Arthropathy versus Degenerative Joint
CPPD crystal deposition Disease
disease
Sporadic AF CPPD Ochronotic Arthropathy Degenerative Joint Disease
Familial AF, NP ?
Involvement of hip, shoulder, Involvement of hip, knee,
Hemochromatosis AF, NP CPPD knee, sacroiliac joint, and and hand
Hyperparathyroidism AF, NP CPPD symphysis pubis
Acromegaly AF, NP HA Focal or diffuse joint space loss Focal joint space loss
Poliomyelitis AF, NP ? Absent or meager osteophytosis Prominent osteophytosis
Amyloidosis AF, NP ? Small cystic lesions Small or large cystic
Spinal fusion NP ? lesions
Collapse, fragmentation, and No collapse or
intra-articular osseous bodies fragmentation
AF, anulus fibrosus; CPPD, calcium pyrophosphate dihydrate; HA,
hydroxyapatite; NP, nucleus pulposus. Tendon abnormalities No tendon abnormalities
From Weinberger A, Myers AR: Semin Arthritis Rheum 18:69, 1978.
Chapter 37 Alkaptonuria 499
other disorders, such as CPPD crystal deposition disease, Lagier R, Sitaj S: Vertebral changes in ochronois: Anatomical
calcium hydroxyapatite crystal deposition disease, and radiological study of one case. Ann Rheum Dis 33:86,
acromegaly, and epiphyseal and spondyloepiphyseal 1974.
dysplasias. Lasker RH, Sargison KD: Ochronotic arthropathy: A review
with four case reports. J Bone Joint Surg Br 52:653, 1970.
Millea TP, Segal LS, Liss RG, et al: Spine fracture in ochrono-
sis: Report of a case. Clin Orthop 281:208, 1992.
FURTHER READING Pomeranx MM, Friedman LJ, Tunick IS: Roentgen findings in
alkaptonuric ochronosis. Radiology 37:295, 1941.
Dom K, Pittevils T: Ochronotic arthropathy: The black hip. Selvi E, Manganelli S, Benucci M, et al: Chronic ochronotic
Case report and review of the literature. Acta Orthop Belg arthritis: Clinical, arthroscopic, and pathologic findings. J
63:122, 1997. Rheumatol 27:2272, 2000.
Hamdi N, Cooke TD, Hassan B: Ochronotic arthropathy: Thompson MM Jr: Ochronosis. AJR Am J Roentgenol 78:46,
Case report and review of the literature. Int Orthop 23:122, 1957.
1999.
Justesen P, Andersen PE Jr: Radiologic manifestations in
alcaptonuria. Skeletal Radiol 11:204, 1984.
38
CHAPTER 38
Other Crystal-Induced Diseases time and be misinterpreted as monosodium urate or
CPPD during polarizing microscopy. The use of intra-
synovial corticosteroid therapy has been followed
inconstantly by local exacerbations of symptoms in the
SUMMARY OF KEY FEATURES treated joints. This phenomenon, termed a postinjection
Other crystals besides monosodium urate, calcium flare, usually commences within 1 to 3 days after the
pyrophosphate dihydrate (CPPD), and calcium injection and persists for several days.
hydroxyapatite crystals can lead to soft tissue, articular,
and osseous abnormalities. Cholesterol crystals are
identified in patients with rheumatoid arthritis, as well CHARCOT-LEYDEN CRYSTALS
as osteoarthritis; they appear to reflect local rather than Charcot-Leyden crystals have been identified in a variety
systemic alterations and may be responsible for low- of conditions associated with eosinophilia, including
grade synovial inflammation. When corticosteroid allergy, asthma, parasitic disorders, and granulocytic
preparations are injected into joints, there is a mild leukemia. They have been noted in synovial fluid, where
synovial inflammatory response. Accumulation of they demonstrate weakly positive birefringence and are
calcium oxalate crystals is seen in both primary and accompanied by turbid joint effusions. It is not certain
secondary oxalosis, the latter most typically occurring whether the crystals contribute to acute inflammation.
as a complication of chronic renal disease. Destructive
lesions of the metaphyseal regions of tubular bones,
discovertebral regions, and sometimes joints become CALCIUM OXALATE (OXALOSIS)
apparent. Cystine, hemoglobin, and Charcot-Leyden Deposition of crystals of calcium oxalate in tissue occurs
crystals can also accumulate in bones or joints, although in two main situations: as a rare primary hereditary process
the precise relationship between such accumulation and or, more commonly, as a secondary or acquired process,
structural abnormality is not known. Xanthine and usually in association with chronic renal disease.
hypoxanthine crystals may be deposited in muscles.
Primary Oxalosis
Inherited as an autosomal recessive trait, primary oxalosis
INTRODUCTION is divided into two types: type 1, glycolic aciduria caused
When synovial fluid is examined with polarizing light by the absence of α-ketoglutarate–glyoxylate carboxylase
microscopy, a variety of crystals can be identified; this may (or glyoxylate aminotransferase) activity; and type 2,
provide an immediate clue to the precise cause of articular 1-glyceric aciduria caused by a defect in D-glycerate
symptoms and signs. Previous chapters discussed three dehydrogenase–glyoxylate reductase. Overproduction of
basic crystal-induced arthropathies: monosodium urate oxalate related to these enzyme defects is accompanied
crystal deposition disease (gout), CPPD crystal deposition by its accumulation in various tissues. Damage to the
disease (pseudogout and other clinical manifestations), and kidneys in the form of calcium oxalate nephrolithiasis
calcium hydroxyapatite crystal deposition disease. This and nephrocalcinosis produces progressive renal failure
chapter describes several additional crystals, as well as their and uremia. Extrarenal accumulation of calcium oxalate
possible relationship to articular manifestations. occurs in the small arteries, eyes, soft tissues, and bones.
Both boys and girls are affected. Clinical manifesta-
tions generally become apparent before the age of 5 years
CHOLESTEROL CRYSTALS and are mainly the result of renal accumulation of calcium
Crystals of cholesterol are frequently identified in the oxalate crystals. Calculi and pyelonephritis are observed.
joint effusions of patients with rheumatoid arthritis and, Radiographic examination of the genitourinary system
less commonly, in the effusions of those with osteo- may reveal small, contracted kidneys with parenchymal
arthritis, chronic tophaceous gout, ankylosing spondylitis, calcification (Fig. 38–1).
and systemic lupus erythematosus. The exact origin of The skeletal abnormalities associated with primary
cholesterol crystals within the joint is unknown, although oxalosis include irregular transverse sclerotic bands in the
local rather than systemic factors appear to play a more metaphyseal segments of tubular bones, including the
important role in their cause. In certain situations, these femur, humerus, tibia, fibula, metacarpals, metatarsals,
crystals may be responsible for low-grade synovial inflam- and phalanges. These bands extend into the epiphyses,
mation and the production of articular abnormalities. and narrow translucent zones, which may replace the
radiodense lines, are seen at the level of the physis
between the epiphyseal and metaphyseal components
CORTICOSTEROIDS (Fig. 38–2A). Similar radiodense regions can appear in
Corticosteroid preparations for intra-articular injection subchondral areas in the humeri and femora and
are suspensions of microcrystals that may persist for some resemble the findings of ischemic necrosis. Alternating
500
Chapter 38 Other Crystal-Induced Diseases 501
figuration of the metacarpal bones and patchy sclerosis
in the clavicles. Eventually, chronic renal failure can lead
to the widespread skeletal abnormalities of renal osteo-
dystrophy.
Histologic investigation of the skeletal abnormalities
in both primary and secondary oxalosis indicates that the
sclerotic regions in the tubular bones represent, in large
part, deposition of calcium oxalate crystals in the marrow.
A foreign body giant cell reaction results and stimulates
new bone formation. Massive deposits of calcium oxalate
are associated with resorption and disappearance of tra-
beculae, cystic lesions, fracture, and deformities such as
acetabular protrusion.
Secondary Oxalosis
Figure 38–1. Primary oxalosis: radiographic abnormal- Oxalosis is a recognized complication of other diseases,
ities. In a 27-year-old woman with primary oxalosis, a especially renal disorders; secondary forms of oxalosis are
preliminary film from an intravenous pyelogram, before also related to (1) ingestion of substances that either con-
the injection of contrast material, reveals bilateral diffuse tain the oxalate ion or are metabolized to oxalate (rhubarb,
calcification within contracted kidneys. (Courtesy of L. ethylene glycol), (2) thiamine and pyridoxine deficiencies
Cooperstein, Pittsburgh, Pa.) that inhibit glyoxylate metabolism and increase the pro-
duction of oxalate, (3) formation of oxalate by Aspergillus
niger, and (4) bowel disorders that cause malabsorption
radiolucent and radiodense bands have been identified in and steatorrhea. Because oxalic acid is excreted by the
the ilium and sternum, whereas in the spine, sclerotic zones kidneys, plasma oxalate levels generally rise in proportion
at the top and bottom of the vertebral bodies simulate the to blood urea nitrogen in end-stage renal failure. Deposi-
rugger-jersey appearance of renal osteodystrophy (see tion of calcium oxalate subsequently occurs in the body’s
Fig. 38–2B). Diffuse osteosclerosis of the vertebrae may tissues, primarily in the kidney itself; more than 75%
also occur, and oxalate crystal deposition in adjacent liga- of patients with end-stage renal disease of longer than
ments and soft tissues may contribute to spinal stenosis. 2 months’ duration have significant accumulations of
Additional skeletal alterations include a drumstick con- calcium oxalate in the kidney. Other organs that are
involved include the myocardium, thyroid gland, spleen, similar aberration. Sclerosis in the metaphyseal segments
liver, lymph nodes, brain, salivary glands, dentin, dental of tubular bones is a finding of secondary (as well as
pulp, arteries and veins, and musculoskeletal tissues. primary) oxalosis, although it can apparently be seen in
Trabecular condensation or disarray, or both, accounts chronic renal disease without oxalate deposition.
for the resulting radiodensity and cystic appearance on
radiographs. Articular manifestations, which may include
CYSTINOSIS
effusions, pain, and stiffness, have been reported in mul-
tiple locations, including the knee, wrist, and metacarpo- Cystinosis is a rare familial disorder characterized by the
phalangeal and interphalangeal joints. In addition to widespread deposition of cystine crystals in body tissues.
juxta-articular osteoporosis, radiographs may reveal cap- Although the cause of this deposition is unknown, cystine
sular and cartilage calcification (chondrocalcinosis), the accumulates within lysosomes, most prominently in the
latter unrelated to CPPD crystal deposition (Fig. 38–3). reticuloendothelial cells of the liver, spleen, lymph nodes,
The radiographic characteristics of musculoskeletal and bone marrow; in peripheral leukocytes; in the medullary,
involvement in secondary oxalosis present diagnostic cortical, and glomerular mesangium of the kidney; in the
difficulties. Chondrocalcinosis, as well as calcification in rectal mucosa; and in the uvea and conjunctiva of the eye.
the joint capsule and tendons, may be related to CPPD, The renal deposition is responsible for the secondary
calcium hydroxyapatite, or calcium oxalate crystal deposi- tissue damage that results in renal insufficiency and failure
tion (or any combination of the three) in patients with in certain forms of the disease.
chronic renal disease. Bone erosions may be related to Three varieties of the disease have been identified:
any of these crystals or to secondary hyperparathyroidism. infantile cystinosis, of autosomal recessive inheritance, in
Discovertebral destruction, a recognized complication of which renal insufficiency becomes apparent in the first
renal dialysis, may represent an additional manifestation decade of life and may lead to early death; juvenile or
of calcium oxalate crystal accumulation, inasmuch as such intermediate cystinosis, in which renal disease becomes
crystals have been observed in areas of disc degeneration; evident in the second or third decade of life; and adult or
however, subchondral resorption resulting from second- benign cystinosis, which is manifested as corneal rather
ary hyperparathyroidism, calcium hydroxyapatite or CPPD than renal deposition of cystine and is compatible with
crystal deposition, or amyloid deposition can lead to a long survival. In the first two forms of the disease, damage
to the tubules and, subsequently, to the glomeruli of the
kidneys leads to electrolyte imbalance, anemia, cardiac
failure, retinopathy, and rickets. In general, the amount
of cystine deposited in the tissues is directly related to the
clinical severity of the disorder.
In the infantile variety, polyuria, dehydration, chronic
acidosis, vitamin D–resistant rickets, and growth distur-
bance may antedate progressive uremia and death, all of
which may occur before the age of 10 years. In adult
cystinosis, ocular manifestations predominate and include
burning and itching of the eyes, photophobia, and
headache. In the juvenile variety, clinical abnormalities
are intermediate between the infantile and adult types
and consist of both ocular and renal alterations. Although
accumulation of cystine crystals in the bone marrow is a
recognized sequela of the disease, osseous and articular
manifestations, other than those related to rickets, have
not received a great deal of attention. Radiolucent cystic
lesions in bone and articular calcification have been
reported, but their precise relationship to cystine deposi-
tion is not clear.
FURTHER READING
Brancaccio D, Poggi A, Ciccarelli C, et al: Bone changes in
end-stage oxalosis. AJR Am J Roentgenol 136:935, 1981.
Danpure CJ: The molecular basis of alanine glyoxylate amino-
transferase mistargeting: The most common single cause of
primary hyperoxaluria type 1. J Nephrol 11:8, 1998.
Day DL, Scheinman JI, Mahan J: Radiological aspects of primary
Figure 38–3. Secondary oxalosis: radiographic abnormali- hyperoxaluria. AJR Am J Roentgenol 146:395, 1986.
ties. Radiograph reveals periarticular and intra-articular Elmstahl B, Rausing A: A case of hyperoxaluria: Radiological
calcification. The latter is within the capsule, the synovium, aspects. Acta Radiol 38:1031, 1997.
and, possibly, the cartilage. Minimal subperiosteal bone resorp- Fam AG, Sugai M, Gertner E, et al: Cholesterol “tophus.”
tion is suggested. Arthritis Rheum 26:1525, 1983.
Chapter 38 Other Crystal-Induced Diseases 503
Freiberg AA, Fish DN, Louis DS: Hand manifestations of Milgram JW, Salyer WR: Secondary oxalosis of bone in chronic
oxalosis. J Hand Surg [Am] 18:140, 1993. renal failure: A histopathological study of three cases. J Bone
Kemper MJ, Conrad S, Muller-Wiefel DE: Primary hyper- Joint Surg Am 56:387, 1974.
oxaluria type 2. Eur J Pediatr 156:509, 1997. Reginato AF, Falasca GF, Usmani Q: Do we really need to pay
Martijn A, Thijn CJP: Radiologic findings in primary hyper- attention to the less common crystals? Curr Opin Rheumatol
oxaluria. Skeletal Radiol 8:21, 1982. 11:446, 1999.
McCarty DJ Jr, Hogan JM: Inflammatory reaction after intra- Zuckner J, Uddin J, Gantner GE Jr, et al: Cholesterol crystals
synovial injection of microcrystalline adrenocorticosteroid in synovial fluid. Ann Intern Med 60:436, 1964.
esters. Arthritis Rheum 7:359, 1964.
SECTION
IX
Temporomandibular Manifestations of
Articular Disease
CHAPTER 39
Temporomandibular Joint Major osseous landmarks in Towne’s projection are
portions of the condyle, including its neck and medial
William A. Murphy Jr., Phoebe A. Kaplan, and lateral poles (Fig. 39–2). The mandibular fossa is
limited by an osseous ridge medially, which is a barrier to
and Donald Resnick medial dislocation of the condyle. In both lateral
(sagittal) and frontal (coronal or Towne’s) perspectives,
the joint surfaces are smooth, and the joint space is of
SUMMARY OF KEY FEATURES nearly uniform thickness along the articulating surfaces.
The condyle rests symmetrically within the fossa. The
Temporomandibular joint (TMJ) pain and dysfunction joint surfaces are covered by fibrocartilage rather than
are important clinical problems. Many of these disorders hyaline cartilage, presumably because of the great forces
have long been misunderstood or ignored by physicians, to which they are subjected.
who for years had little interest in or opportunity to
learn about them. In the past 15 or 20 years, however,
radiologists have had a major role in the diagnosis of Soft Tissue Anatomy
TMJ disorders because of the explosion of new imaging The major soft tissue structure of the TMJ is an articular
methods. Foremost among these methods are disc (meniscus) that is interposed between the adjacent
arthrography, computed tomography (CT), and, more osseous elements (Fig. 39–3). The disc separates the joint
recently, magnetic resonance (MR) imaging. The impact into two synovial articulations—one superior, between
of these imaging techniques on the diagnosis of TMJ the temporal bone and the disc, and the other inferior,
disorders is undeniable. between the disc and the condylar head. The disc is thin
centrally (the intermediate zone) and ridged or thickened
peripherally. It is attached to capsular, ligamentous, and
other soft tissue structures about its entire periphery.
INTRODUCTION The disc is a biconcave fibrocartilage. In the sagittal
The TMJ is afflicted by many osseous and soft tissue perspective, the anterior and posterior ridges of the disc
conditions, as are other joints. Because of its particular are prominent and are termed the anterior and posterior
anatomy, however, the TMJ is subject to a unique bio- bands; their mediolateral length is much greater than
mechanical environment and set of pathologic conditions. their anteroposterior length. The anterior band of the
disc is smaller than the posterior band and attaches to
the anterior margin of the articular eminence and to the
ANATOMY anteroinferior aspect of the articular margin of the
The condylar and temporal bone components of the condyle. The posterior band of the disc blends with
TMJ are maintained in apposition by muscles, ligaments, highly vascular and innervated areolar tissue, termed the
and the joint capsule. The capsule attaches about the bilaminar zone or posterior attachment. These anterior
joint margins and is reinforced laterally by a strong and posterior attachments are continuous medially and
temporomandibular ligament and medially by two laterally and bind the disc to the condyle and the fossa.
weaker ligaments. Branches of the mandibular division With the jaw closed, the disc is positioned between the
of the trigeminal nerve innervate the joint. Similarly, condyle and the fossa such that the posterior band is
branches of the superficial temporal and maxillary located between the apex of the condyle and the depth of
arteries provide the blood supply. the fossa, sometimes termed the 12 o’clock position. As
the jaw opens, the disc and condyle move forward in a
complex coordinated fashion. The result is that the thin
Osseous Anatomy central portion of the disc maintains a position between
Important osseous landmarks in the lateral projection the juxtaposed articular surfaces of the condyle and
are the cortical and trabecular elements of the condyle, eminence.
mandibular fossa, temporal eminence, and auditory canal
(Fig. 39–1). The long axis of the mandibular condyle IMAGING METHODS
is perpendicular to the mandibular ramus. The sagittal
Radiography
surface of the eminence is gently convex, and the com-
bined mandibular fossa and eminence describe a smooth Conventional radiography has long been considered the
sigmoid curve. initial imaging method that is best suited for displaying
505
506 Section IX Temporomandibular Manifestations of Articular Disease
A B
Figure 39–1. Lateral perspective of normal adult osseous anatomy. A, Surface photograph of the skull.
B, Magnified lateral projection (closed-mouth position) in a 22-year-old woman. The following anatomic
landmarks can be identified: AT, anterior glenoid tubercle (articular eminence); CH, condylar head; CP,
coronoid process; EAM, external auditory meatus; EP, ear plug; GF, glenoid (mandibular) fossa; MN,
mandibular or sigmoid notch; MP, mastoid process; PT, posterior glenoid tubercle; STF, squamotympanic
fissure; TH, threaded hole in the radiographic positioning device; TP, tympanic process; VP, vaginal
process; Z, zygoma.
A B
Figure 39–2. Frontal (Towne’s) perspective of normal adult osseous anatomy. A, Surface photograph of
the skull. B, Magnified Towne’s projection (closed-mouth position) in a 23-year-old woman. The following
anatomic landmarks can be identified: AT, anterior glenoid tubercle; CC, carotid canal; CH, condylar head;
L, lingula; MF, mandibular foramen; MP, mastoid process; SMF, stylomastoid foramen; SP, styloid process;
TP, tympanic process; VP, vaginal process; Z, zygoma.
Chapter 39 Temporomandibular Joint 507
A
Figure 39–3. Normal soft tissue anatomy of the temporomandibular
joint. A, Sagittal section shows disc or meniscus (m) separating the
articular eminence (AE) from the condylar head (CH). Anterior (AA) and
posterior (PA) meniscal attachments are broadly based in the adjacent soft
tissues. B, Coronal section shows the meniscus (m) separating the temporal B
fossa (TF) from the condylar head (CH). Meniscal attachments to the
condylar head are thin medially (MA) and thick laterally (LA).
the osseous anatomy and the general positional relation- scanners have markedly improved the ability to obtain
ship of the condyle with respect to the mandibular fossa; reformatted images (Fig. 39–5). A greater emphasis, how-
however, because of the small size of the joint, its com- ever, has been placed on the detection of internal derange-
plex anatomic structure, and its location among the other ment of the disc, and MR imaging has surpassed CT as
bones of the skull, obtaining optimal images is techni- the noninvasive imaging method used in most institutions.
cally demanding and not always successful.
The panoramic radiographic projection provides an
Magnetic Resonance Imaging
excellent survey of the mandible and dentition. It images
both TMJs simultaneously and may reveal disease For TMJ imaging, the sagittal plane has been empha-
processes of these joints, the mandible, or the teeth sized. Images obtained in the coronal plane, however,
(Fig. 39–4). It provides a relatively lateral projection of may provide greater accuracy than sagittal MR images
both joints and is particularly useful for evaluating sym- alone for diagnosing discs that are displaced mainly medial
metry. Panoramic radiography is also an effective method or lateral to the condyle—an unusual occurrence.
for studying mandibular fractures and tumors. Documenting the position of the disc and the status of
the osseous structures of the TMJ can be done well with
T1-weighted spin echo MR images alone. Identifying
Radionuclide Imaging fluid or masses within the joint or in the muscles of
Two general methods of imaging are used: planar and mastication about the joint requires the use of some type
tomographic. Planar images are generally obtained with of T2 weighting, especially in the setting of trauma as
a gamma camera having a large field of view and fitted opposed to routine internal derangements. Fast gradient
with a high-resolution collimator. Single photon emission echo imaging is useful for demonstrating the TMJ in
computed tomography (SPECT) scans require a special different phases of mouth opening. For the evaluation
rotating gamma camera device with an appropriate colli- of routine internal derangements of the TMJ, two sets of
mator. Planar radionuclide imaging of the TMJ has not sagittal T1-weighted images (one with the mouth closed
had any impact on the detection or management of TMJ and the other with the mouth completely open) consti-
disorders. SPECT is more sensitive than the planar tute a standard approach. In the final analysis, however,
method, but it is not more specific. optimized MR imaging is the leading noninvasive
method for determining disc location (Fig. 39–6).
Computed Tomography
DISORDERS
CT is especially useful for the evaluation of osseous
anatomy, including the detection and staging of tumors, Disorders of the TMJ region can be divided into two
arthritic changes, and fractures. Spiral and multidetector general categories: extracapsular and intracapsular (Table
508 Section IX Temporomandibular Manifestations of Articular Disease
A B
Figure 39–5. CT scan of the temporomandibular joint (TMJ). A, Reformatted sagittal image of the TMJ
displayed at bone window shows the normal anatomy. B, Corresponding surface-rendering three-
dimensional CT image.
39–1). Both groups of conditions can produce similar ever, is more likely to reveal one or more abnormalities,
symptoms, including pain, the sensation of various noises, and the imaging workup should disclose a structural or
and decreased or uncoordinated range of motion. functional abnormality. The combination of symptoms,
Extracapsular disorders are seen frequently by clinicians signs, and imaging findings is usually sufficiently charac-
and are often classified as myofascial pain-dysfunction teristic to allow a specific diagnosis.
syndrome. Affected patients often complain of some
combination of neck and masticatory muscle pain, head-
Congenital or Developmental Disorders
ache, joint noise, tinnitus, malocclusion, deviation of the
mandible, loss of motion, and stress or anxiety. In these Congenital TMJ disturbances generally relate to develop-
extracapsular conditions, the intraoral structures and mental abnormalities of the first branchial arch and result
masticatory muscles may be abnormal on physical exami- in agenesis, hypoplasia, or hyperplasia of the mandible.
nation, but examination of the TMJ is unremarkable. The defects may be focal, diffuse, unilateral, or bilateral.
Likewise, the results of radiography, arthrography, and Conventional or panoramic radiographic imaging usually
sectional imaging are normal. allows the identification of these congenital or develop-
Intracapsular disorders may also exhibit similar com- mental conditions and demonstrates features useful for
binations of symptoms. The physical examination, how- their classification.
Chapter 39 Temporomandibular Joint 509
A B
C D
Figure 39–6. MR imaging of the temporomandibular joint (TMJ). A, T1-weighted sagittal spin echo MR
image of a normal TMJ. View with the mouth closed shows high signal intensity from the condylar marrow
(C) and articular eminence (E). Surrounding cortical bone is devoid of signal. The disc, of low signal
intensity, is interposed between the condyle and the fossa; the intermediate zone articulates with the condyle
and eminence where they are most closely apposed. The solid arrow points to the anterior band and the open
arrow to the posterior band of the disc. B, Sagittal gradient echo MR image used for fast (pseudodynamic)
scanning shows a normal position of the disc with the mouth closed. Marrow becomes low in signal intensity
with this sequence, and fluid in the inferior joint space becomes bright (arrows); the disc remains low in
signal intensity. C, condyle; E, eminence. C, Sagittal gradient image of a normal TMJ with the mouth open.
The intermediate zone of the disc maintains its position between the condyle (C) and the eminence (E),
whereas the posterior band slides posterior to the condyle (arrow). D, T1-weighted sagittal spin echo MR
image in a patient with clicking and pain demonstrates internal derangement, with both the anterior (solid
arrow) and posterior (open arrow) bands of the disc displaced anteriorly relative to the condyle (C).
Tumors and Tumor-Like Disorders of the effect of trauma to this region must include an
Tumors of the TMJ are rare, although many types of bone analysis of the mandible, the dental occlusion, and the
or joint neoplasms have been described in this location. TMJ. Because of the complex anatomy of the mandible
Most often, the joint is affected secondarily by extension and its articulations, CT scanning is an important diag-
of a mandibular neoplasm, such as osteosarcoma or metas- nostic method for the analysis of mandibular and TMJ
tasis. Intra-articular tumor-like processes include osteo- trauma. It shows both soft tissue and osseous anatomy
cartilaginous bodies, synovial (osteo)chondromatosis and can display complicated fractures and the location of
(Fig. 39–7), and pigmented villonodular synovitis. fragments better than other studies can. The mandible is
a ringlike structure; therefore, when one fracture is dis-
covered, another mandibular fracture should be sought.
Traumatic Disorders A common combination is a condylar fracture associated
Injury to the mandible or a direct blow to the TMJ is a with a body or ramus fracture. Multiple fractures (more
common cause of TMJ pain and dysfunction. The trauma than two) are often associated with facial fractures. When
may result in soft tissue injury, fracture, or some com- force is applied directly to the chin and the mandible does
bination of soft tissue, bone, and joint injury. Evaluation not fracture, the energy may be dissipated into the TMJ.
510 Section IX Temporomandibular Manifestations of Articular Disease
A B
Fractures of the mandible are classified according to position of the condylar head with respect to the mandibu-
their anatomic location, which includes the condylar head, lar fossa of the temporal bone (Table 39–2). Intracapsular
condylar neck, coronoid process, and mandibular ramus, fractures of the condylar head are infrequent. Extra-
angle, body, and symphysis (Fig. 39–8). Condylar fractures capsular condylar neck fractures may be nondisplaced or
are subdivided according to their intracapsular or extra- displaced, and the condylar head may remain in the
capsular location, displacement at the fracture site, and mandibular fossa or be dislocated (Fig. 39–9).
Chapter 39 Temporomandibular Joint 511
TABLE 39–1 they develop as a direct extension of oral infections or
after TMJ surgery. The radiographic findings are similar
Alderman’s Classification of Temporomandibular Joint to those of rheumatoid arthritis, but the clinical course is
Disorders
much more rapid (Fig. 39–10). The history and physical
examination should suggest the diagnosis.
Extracapsular Disorders
Psychophysiologic: tension, anxiety, oral habits
Iatrogenic: misdirected mandibular nerve block, excessive Articular Disorders
depression of the mandible during anesthesia
or oral procedures
TMJ arthritis is a common problem. Many patients have
osteoarthritis, usually secondary to internal derangement
Traumatic: blow to face not involving fracture
of the disc. Although it is very rare, calcium pyrophos-
Dental: occlusal abnormalities; periapical or periodontal phate dihydrate crystal deposition or gout may occur in
lesions; mobile, sensitive, or damaged teeth;
ulcerations
the TMJ.
Rheumatoid arthritis, juvenile chronic arthritis, psori-
Infectious: secondary, outside the joint
atic arthritis, ankylosing spondylitis, and systemic lupus
Otologic: otitis media or external ear infection erythematosus may all affect the TMJ. All may show a
Neoplastic: parotid gland, nasopharyngeal tumor spectrum of radiographic changes, including osteopenia,
Intracapsular Disorders joint space narrowing, and bone erosion or production
Congenital: agenesis, hyperplastic or hypoplastic condyle (Fig. 39–11).
Infectious: primary bacterial infection within the joint Osteoarthritis of the TMJ is generally similar in
Arthritic: rheumatoid arthritis, osteoarthritis, psoriatic radiographic appearance to osteoarthritis of other joints.
arthritis, juvenile chronic arthritis Joint space narrowing, bone erosion, osteosclerosis,
Traumatic: fracture, disc tear osteophytosis, and remodeling are seen (Figs. 39–12 and
Functional: subluxation, dislocation, disc derangement, 39–13). Cartilage loss may be the predominant feature.
hypermobility, ankylosis Osteophytes may vary in size from small to large, and
Neoplastic: benign or malignant tumor they typically develop at the margins of the articular
surface. The degree of bone sclerosis and remodeling can
be very pronounced. Even with advanced osteoarthritis, a
remarkable range of joint mobility may be retained.
Complications of mandibular fractures include loss of It is now well recognized that TMJ osteoarthritis often
teeth, malunion or nonunion, displacement with defor- follows the development of an internal derangement. At
mity, and infection. Secondary complications of trauma least 20% of patients with internal derangement have
are often more severe. The two major secondary com- osteoarthritis at the time of the initial clinical exami-
plications are malocclusion and TMJ internal derange- nation. The frequency of osteoarthritis seems to increase
ment, both of which result in pain and malfunction. with increasing duration of internal derangement. Osteo-
arthritis is closely associated with disc dislocation, joint
locking, disc perforation, and disc fragmentation.
Infectious Disorders
Pyogenic or granulomatous infections of the TMJ are
uncommon. They may occur as a result of hematogenous Internal Derangement
seeding from a distant infection, but more commonly Internal derangement is the most frequent disorder of
the TMJ. It is defined as an abnormal relationship of the
disc relative to the condyle, fossa, and articular eminence.
The disc is usually displaced anteriorly, but infrequently,
it may be displaced lateral, medial, or posterior to the
TABLE 39–2
Fractures of the Mandibular Condyle
B C
D E
Figure 39–9. Multiple mandibular fractures in a 23-year-old woman following a fall. A, Panoramic
radiograph shows fracture of the symphysis (black arrow) and right coronoid process (white arrow). Fractures
of both condylar necks with dislocation of both condylar heads are not well seen. B, Anteroposterior
radiograph shows symphysis and right coronoid fractures. Neck fractures are not as visible, C, Reformatted
coronal CT scan through the anterior mandible shows the symphysis fracture to better advantage. D,
Reformatted coronal CT scan through the condyles shows fractures of both condyles, with medial
displacement of the displaced fragments (white arrows). Remaining condylar heads are dislocated laterally
(black arrows). E, Reformatted three-dimensional image shows the fractures of the symphysis (black
arrowhead) and coronoid (black arrow) to better advantage. The lateral displacement of the condylar heads
(white arrows) is also well seen.
Chapter 39 Temporomandibular Joint 513
A B
Figure 39–13. Osteoarthritis compared in a radiograph (A) and photograph (B) of a sagittally sectioned
specimen. The joint space is narrow, and the disc is dislocated anteriorly, with thinning and fraying of the
meniscal (m) posterior attachment or bilaminar zone. The condylar head cortex is thickened, with small
osteophytes (arrows). The mandibular fossa is sclerotic and remodeled, and only a shallow concavity is seen
where the articular eminence once was.
(Fig. 39–17). During mouth opening, the posterior band Locking is the result of a dislocated disc that will not
moves posteriorly instantaneously, thereby producing reduce. An acute lock is generally painful and associated
an opening click and reducing the dislocation. During with very limited function (Fig. 39–18). With time, the
closure, the posterior band moves anteriorly, thereby bilaminar zone (retrodiscal tissue) usually stretches, such
producing a closing click instantaneously and resulting that pain diminishes and considerable range of motion is
in redislocation of the disc. The phenomenon of regained (Fig. 39–19).
combined opening and closing clicks is termed reciprocal The value of radiographs for diagnosing internal
clicking. derangement is limited. The abnormalities that may be
A B
Figure 39–14. Functional anatomy of the temporomandibular joint. A, Sagittal section of a fresh
specimen in a slightly open-mouth position shows posterior sulci (arrows) beginning to open. Articular
cartilage (arrowheads) covers the articular eminence. Note that the posterior band of the disc is at the
12 o’clock position. B, In the intermediate open-mouth position, the posterior and anterior superior sulci
are open. Note that the thin region of the disc stays between the articular surfaces and that changes in joint
shape are primarily a result of changes in the soft tissues about the sulci. Also note that translation is related
primarily to motion of the disc and condyle with respect to the eminence.
Chapter 39 Temporomandibular Joint 515
A B
Figure 39–15. MR imaging of the normal temporomandibular joint (TMJ). A, T1-weighted sagittal spin
echo MR image of a normal TMJ. View with the mouth closed shows high signal intensity from the condylar
marrow and articular eminence. Surrounding cortical bone is devoid of signal. The disc, of low signal
intensity, is interposed between the condyle and the fossa; the intermediate zone articulates with the condyle
and eminence where they are most closely apposed. B, Sagittal T1-weighted image shows a normal position
of the disc with the mouth open. The intermediate zone of the disc maintains its position between the
condyle and the eminence, whereas the posterior band slides posterior to the condyle. Note translation of
the condyle to the apex of the eminence.
seen are often nonspecific and occur late in the disease demonstrating marrow abnormalities. The sagittal plane
process. High-resolution CT with reformatted sagittal is most useful in this assessment, although coronal MR
and coronal images is the ideal method for evaluating the images are often included. Imaging is performed with the
morphologic osseous abnormalities. Owing to its non- mouth both open and closed. Disagreement exists, how-
invasive nature, MR imaging is the preferred modality ever, about other specifics of MR imaging methodology,
for diagnosing internal derangements, and it is ideal for including the choice of spin echo or gradient echo
A B
Figure 39–17. Anteriorly displaced disc. A, Bicompartment arthrogram shows a posterior band anterior
to the condyle, which is creating a concave defect in the anterior recess. B, Sagittal T1-weighted spin echo
MR image in a different patient with a biconcave disc dislocated anterior to the condyle with the mouth
closed. Note the normal high signal intensity in the center of the anterior and posterior bands.
A B
Figure 39–18. Acute temporomandibular joint lock from a nonreducing displaced disc. A, T1-weighted
sagittal spin echo MR image with the mouth closed shows the dislocated disc (arrow) anterior to the condyle.
B, With attempted mouth opening, no appreciable anterior translation of the condyle occurs, but the disc
folds on itself in the thin intermediate zone because of increased pressure from the condyle. The normal
biconcave configuration of the disc and the normal intradiscal signal intensity are maintained (arrow).
sequences, or both, and whether intravenous contrast up adhesions, and disc removal with placement of either
agents should be used. an alloplastic disc implant or some type of autograft. One
A number of different techniques have been devised complication of such TMJ surgery was a foreign body
for the treatment of internal derangements, including giant cell reaction to the alloplastic disc implants that
splint therapy, arthroplasty with plication and reposition- were previously in use (Fig. 39–20).
ing of the disc, arthroscopy to distend the joint and break
Chapter 39 Temporomandibular Joint 517
A B
Figure 39–19. Nonreducing anteriorly displaced disc. A, T1-weighted sagittal spin echo MR image with
the mouth closed shows a dislocated disc (arrow) anterior to the condyle. B, With mouth opening, the disc
folds on itself and becomes crumbled. Anterior translation is somewhat reduced. Mild degenerative changes
are noted with osteoarthritis.
A B
Figure 39–20. Foreign body reaction to an allograft disc implant. A, T1-weighted sagittal spin echo MR
image of the temporomandibular joint in a patient with a thin, Proplast-Teflon disc implant of low signal
intensity (arrow). The condyle is not seen in the fossa, but a mass of granulation tissue of intermediate signal
intensity surrounds the implant (arrowheads). B, In the adjacent image, the implant is fragmented (arrows),
and the fossa is filled with a mass of granulation tissue (arrowheads) that has eroded the condyle. The
remnant of the mandible is of low signal intensity where marrow fat (of high signal intensity) is normally
seen.
518 Section IX Temporomandibular Manifestations of Articular Disease
X
Target Approach to Articular Disease
CHAPTER 40
Target Area Approach to Articular tissue swelling, regional osteoporosis, diffuse loss of
interosseous space, and marginal and central bony ero-
Disorders: A Synopsis sions are the observed findings.
HAND
The joints of the hand consist of the distal interpha-
langeal, proximal interphalangeal, and metacarpophalangeal
joints of the second to fifth digits and the metacarpopha-
langeal and interphalangeal joints of the thumb.
Figure 40–6. Systemic lupus erythematosus. Figure 40–7. Scleroderma and polymyositis.
522 Section X Target Approach to Articular Disease
WRIST
The major joints or compartments of the wrist are
summarized in Table 40–1 and Figure 40–10.
TABLE 40–1
Major Compartments of the Wrist
Compartment Location
Radiocarpal Between the distal end of the
radius and the proximal carpal
row
Midcarpal Between the distal and the
Figure 40–9. Calcium pyrophosphate dihydrate crystal proximal carpal rows
deposition disease.
Common Between the distal carpal row and
carpometacarpal the bases of the four ulnar
metacarpals
First carpometacarpal Between the trapezium and the
Other Diseases base of the first metacarpal
Inferior radioulnar Between the distal ends of the
Multicentric reticulohistiocytosis can lead to significant abnor- radius and ulna, separated from
malities of both hands, which usually are most striking in the radiocarpal compartment by
the distal interphalangeal and, to a lesser extent, the proxi- the triangular fibrocartilage of
mal interphalangeal joints. Thermal injuries, including the wrist
frostbite and burns, may produce alterations that also Pisiform-triquetral Between the pisiform and
predominate in distal locations. In some cases, the joints triquetrum
of the thumb are spared in frostbite. Hyperparathyroidism
Chapter 40 Target Area Approach to Articular Disorders: A Synopsis 523
joint space loss in the midcarpal and common car-
pometacarpal compartments. In fact, eventual osseous
fusion of the proximal and distal carpal rows and bases of
the four ulnar metacarpals may be seen, with relative
sparing of the first carpometacarpal and radiocarpal com-
partments. A similar pattern of disease may be apparent
in some patients with adult-onset Still’s disease.
Figure 40–14. Degenerative joint disease (osteoarthritis) and Figure 40–16. Gouty arthritis. CCMC, common car-
inflammatory (erosive) osteoarthritis. CCMC, common car- pometacarpal compartment; CMC, first carpometacarpal
pometacarpal compartment; CMC, first carpometacarpal com- compartment; IRU, inferior radioulnar compartment; MC,
partment; IRU, inferior radioulnar compartment; MC, midcarpal compartment; RC, radiocarpal compartment.
midcarpal compartment; RC, radiocarpal compartment.
noninflammatory osteoarthritis. At these sites, joint space compartmental distribution, similar to that in rheumatoid
narrowing and eburnation predominate, although rarely, arthritis, may be apparent. Of diagnostic significance, the
erosive abnormalities may be detected. common carpometacarpal compartment may be the site
of the most extensive abnormality in this disease. At this
site, scalloped erosions of the bases of one or more of the
Scleroderma (Fig. 40–15) four ulnar metacarpals are seen. Additional findings, such
Selective involvement of one or both first carpometacarpal as the absence of osteoporosis and the presence of eccen-
compartments may be noted in some patients with scle- tric erosions with sclerotic margins, lobulated soft tissue
roderma. The changes consist of scalloped erosions of the masses, and preservation of joint space, also aid in the dif-
base of the metacarpal and adjacent trapezium. Similar ferentiation of gouty arthritis from rheumatoid arthritis.
alterations of the first carpometacarpal joint may be
encountered in systemic lupus erythematosus, polymyositis,
and Ehlers-Danlos syndrome. In scleroderma, intra- Calcium Pyrophosphate Dihydrate Crystal
articular and periarticular calcification frequently can be Deposition Disease (Fig. 40–17)
observed about the altered joint. CPPD crystal deposition disease leads to bilateral sym-
metrical or asymmetrical changes that reveal a distinct
predilection for the radiocarpal compartment of the wrist.
Gouty Arthritis (Fig. 40–16)
At this site, extensive narrowing of the space between the
In long-standing gouty arthritis, bilateral symmetrical or distal portion of the radius and scaphoid may be seen.
asymmetrical changes can be observed in the wrist. A pan- Elsewhere in the wrist, the trapezioscaphoid or lunate-
FOREFOOT
The joints of the forefoot include the distal interpha-
langeal, proximal interphalangeal, and metatarsophalangeal
joints of the second to fifth digits and the interphalangeal
and metatarsophalangeal joints of the great toe.
Figure 40–18. Rheumatoid arthritis. Figure 40–20. Psoriatic arthritis and Reiter’s syndrome.
526 Section X Target Approach to Articular Disease
Other Diseases
Infrequently, metatarsophalangeal joint abnormalities
may be seen in CPPD crystal deposition disease, scleroderma,
systemic lupus erythematosus, and Jaccoud’s arthropathy. Other
findings usually ensure an accurate diagnosis in these cases.
Similarly, infectious processes of the foot may follow diabetes
Figure 40–21. Degenerative joint disease (osteoarthritis). mellitus and local puncture wounds.
Chapter 40 Target Area Approach to Articular Disorders: A Synopsis 527
MIDFOOT AND HINDFOOT sometatarsal joints. A bilateral symmetrical or asym-
The major joints of the midfoot and hindfoot are shown metrical process is most frequent. Prominent osseous
in Figure 40–24. erosions of the bases of one or more metatarsal bones are
especially characteristic.
Rheumatoid Arthritis (Fig. 40–25)
Calcium Pyrophosphate Dihydrate Crystal
Rheumatoid arthritis frequently affects all the joints of
Deposition Disease (Fig. 40–28)
the midfoot, commonly in association with changes at
the metatarsophalangeal joints. Bilateral and symmetrical Considerable osseous fragmentation in a bilateral distri-
abnormalities predominate. The most typical sites of bution about the talonavicular aspect of the talocal-
involvement are the talonavicular portion of the talocal- caneonavicular joint represents an infrequent but
caneonavicular joint, the tarsometatarsal joints, and the distinctive pattern in this disorder. The appearance and
“posterior” subtalar joint. distribution are difficult to distinguish from those
accompanying the neuropathic osteoarthropathy of
diabetes mellitus.
Juvenile Chronic Arthritis (see Fig. 40–25)
Any of the joints of the midfoot can be affected in juvenile
Neuropathic Osteoarthropathy
chronic arthritis. Bony ankylosis of the tarsal bones and
bases of the metatarsal bones of both feet may eventually The midfoot or hindfoot is not an infrequent site of
occur. involvement in diabetes mellitus. Tarsal disintegration
with extension to the bases of the metatarsal bones may
be combined with changes at one or more metatarso-
Degenerative Joint Disease (Osteoarthritis) phalangeal joints. In some cases, fragmentation and sub-
(Fig. 40–26) luxation at the tarsometatarsal joints simulate the
Abnormalities of the first tarsometatarsal joint in one or appearance of a Lisfranc’s fracture-dislocation.
both feet represent the most typical pattern of degenera-
tive joint disease in the midfoot. The findings, consisting
CALCANEUS
of joint space narrowing, sclerosis, and osteophytosis, can
simulate those of gout. Five potential target areas exist on the calcaneus: (1) supe-
rior surface; (2) posterior surface above the attachment of
the Achilles tendon; (3) posterior surface at the site of
Gouty Arthritis (Fig. 40–27) attachment of the Achilles tendon; (4) plantar surface at
Although any of the compartments of the midfoot can be the site of attachment of the plantar aponeurosis; and
affected, gouty arthritis shows a predilection for the tar- (5) plantar surface anterior to the attachment of the aponeu-
Figure 40–24. Joints of the midfoot and hindfoot. CC, calcaneocuboid; CN,
cuneonavicular; ST, posterior subtalar; TCN, talocalcaneonavicular; TMT,
A tarsometatarsal.
528 Section X Target Approach to Articular Disease
B
Figure 40–27. Gouty arthritis.
CC, calcaneocuboid; CN, cuneo-
navicular; ST, posterior subtalar;
TCN, talocalcaneonavicular;
A TMT, tarsometatarsal.
B
Figure 40–28. Calcium pyrophosphate dihydrate
crystal deposition disease. CC, calcaneocuboid;
CN, cuneonavicular; ST, posterior subtalar; TCN,
A talocalcaneonavicular; TMT, tarsometatarsal.
plantar (site 4) calcaneal enthesophytes also are typical. Retrocalcaneal bursitis leads to osseous erosions that pre-
Plantar osseous erosions of considerable size are dis- dominate at site 2. The changes, which occasionally may
tinctly unusual. Achilles tendinitis producing an enlarged be combined with alterations at site 1, resemble the findings
or poorly defined tendinous outline can be seen. in rheumatoid arthritis, although reactive bone forma-
tion may be more prominent. Well-defined calcaneal
enthesophytes at the site of attachment of the Achilles
Ankylosing Spondylitis and Psoriatic tendon (site 3) may be observed, a nonspecific finding.
Arthritis (Fig. 40–31) On the plantar aspect of the bone, at sites 4 and 5, poorly
Similar abnormalities occur in both ankylosing spondylitis marginated erosions, reactive sclerosis, and poorly defined
and psoriatic arthritis, frequently in a bilateral distribution. enthesophytes may be detected. The outgrowths are more
530 Section X Target Approach to Articular Disease
Figure 40–29. Target areas of the calcaneus (see text for Figure 40–32. Reiter’s syndrome.
identification of numbered areas).
Other Diseases
Hyperparathyroidism can lead to subligamentous erosion
at site 4, as well as subtle defects elsewhere in the calca-
neus, including site 1 (Fig. 40–35). Haglund’s syndrome is
characterized by retrocalcaneal bursitis with a soft tissue Figure 40–36. Rheumatoid arthritis. L, lateral; M, medial;
PF, patellofemoral.
mass at sites 1 and 2.
KNEE
It is useful to analyze separately three major areas or Involvement of the patellofemoral space is often com-
spaces of the knee: the medial femorotibial space, the bined with involvement of the other two compartments
lateral femorotibial space, and the patellofemoral space. in the knee in rheumatoid arthritis. Although not invari-
Anteroposterior radiographs allow analysis of the medial ably present, tricompartmental abnormalities that are of
and lateral femorotibial compartments, analysis that can equal severity are most suggestive of rheumatoid arthritis.
be improved by obtaining radiographs with the patient
standing. Lateral and axial radiographs allow evaluation
of the patellofemoral compartment. Ankylosing Spondylitis, Psoriatic Arthritis,
and Reiter’s Syndrome (Fig. 40–37)
Any of these three disorders can affect one or both knees.
Rheumatoid Arthritis (Fig. 40–36)
A tricompartmental distribution may be encountered,
Rheumatoid arthritis usually leads to alterations that are although the degree of joint space narrowing, osteoporosis,
bilateral and symmetrical and affect both medial and lateral and osseous erosion is less than in rheumatoid arthritis,
femorotibial compartments to an equal degree. Diffuse and the extent of periosteal proliferation or “whiskering”
loss of interosseous space in the medial and lateral com- may be pronounced.
partments may be combined with osteoporosis, marginal
or central osseous erosions, and subchondral sclerosis.
Crumbling of the osteoporotic bone of the tibia in
combination with ligamentous abnormalities may create
varus or, more characteristically, valgus angulation of the
knee.
Degenerative Joint Disease (Osteoarthritis) malities, also can be encountered in this disease and, in
(Fig. 40–38) some instances, may lead to valgus deformity of the knee.
A unilateral or bilateral distribution can be seen. Asymmet- Further, findings isolated to the patellofemoral compart-
rical involvement of the medial and lateral femorotibial ment are observed in some patients. In fact, a “degenerative”-
compartments predominates, frequently in combination like arthropathy of the patellofemoral compartment,
with significant patellofemoral compartment disease. appearing in the absence of significant medial or lateral
Thus, bicompartmental rather than tricompartmental femorotibial space alterations, raises the possibility that
findings are evident on radiographs. In most instances, it this disease is present.
is the medial femorotibial compartment that is more
severely affected, and the asymmetrical nature of the
Other Diseases
process may lead to varus deformity. Severe alterations in
the lateral femorotibial compartment and valgus deformity In addition to subperiosteal resorption of bone along the
are less common on radiographs of the osteoarthritic medial aspect of the tibia, hyperparathyroidism can pro-
knee and, when present, are usually seen in women. duce distinctive types of articular abnormality on knee
Isolated abnormalities of the patellofemoral compart- radiographs. Subchondral resorption of bone may be
ment are relatively unusual in degenerative joint disease evident in any compartment. The changes, consisting of
of the knee. The detection of joint space narrowing, poorly defined “erosion” and sclerosis, may be especially
sclerosis, and osteophytosis in this location in the absence marked in the patellofemoral areas (Fig. 40–40).
of similar changes in either the medial or lateral Septic arthritis affecting the knee can become evident
femorotibial space should initiate a search for CPPD initially in any compartment. As in rheumatoid arthritis,
crystal deposition disease. the alterations can spread to all areas of the joint, pro-
ducing tricompartmental disease. A unilateral distribution
is most frequent.
Calcium Pyrophosphate Dihydrate Crystal Neuropathic osteoarthropathy accompanying tabes dor-
Deposition Disease (Fig. 40–39) salis or, more rarely, other diseases with neurologic deficit
The distribution of abnormalities of the knee in patients can lead to involvement of one or both knees with scle-
with CPPD crystal deposition disease is somewhat variable. rosis, fragmentation, subluxation, and disorganization of
Usually both knees are affected. The medial femorotibial the joint.
and patellofemoral compartments are commonly affected Hydroxyapatite crystal deposition disease or mixed calcium
simultaneously, a distribution that is identical to that in phosphate crystal deposition disease has a distinctive appear-
osteoarthritis. In these cases, the greater extent of osseous ance in the knee. Involvement predominates in the lateral
destruction and fragmentation may allow accurate femorotibial compartment, leading to osseous collapse
differentiation of CPPD crystal deposition disease from and fragmentation, and may be combined with changes
osteoarthritis. Lateral femorotibial compartment changes, in the shoulder.
with or without medial femorotibial compartment abnor- Ischemic necrosis commonly involves the distal portion
of the femur or, to a lesser extent, the proximal part of the
tibia. It may relate to an underlying condition, such as
corticosteroid medication, or occur on an idiopathic basis.
The medial compartment is typically affected in spon-
taneous osteonecrosis about the knee.
Wilson’s disease is associated with abnormality of the
patellofemoral compartment (see Fig. 40–40). In alkap-
tonuria, changes resemble those of osteoarthritis, although
isolated involvement of the lateral femorotibial compart-
ment and considerable bony collapse and fragmentation
are encountered.
HIP
In evaluating articular disorders that affect the hip, it is
useful to define the nature or location of any accom-
panying joint space loss. With diminution of the articular
space, the femoral head migrates in one of three basic
directions with respect to the acetabulum. If the loss is
confined to the superior aspect of the joint, the femoral
head moves in an upward or superior direction; if the loss
is confined to the inner third of the joint, the femoral
head migrates in a medial direction; and if the joint space
loss involves the entire joint, the femoral head migrates
in an axial direction along the axis of the femoral neck
Figure 40–38. Degenerative joint disease (osteoarthritis). L, (Fig. 40–41). Certain disorders are associated with
lateral; M, medial; PF, patellofemoral. characteristic patterns of femoral head migration.
Chapter 40 Target Area Approach to Articular Disorders: A Synopsis 533
Figure 40–40. Hyperparathyroidism and Wilson’s disease. Figure 40–41. Patterns of migration of the femoral head.
L, lateral; M, medial; PF, patellofemoral. A, axial migration; M, medial migration; S, superior migration.
534 Section X Target Approach to Articular Disease
SHOULDER
In the shoulder region, three potential target areas can be
affected in various articular disorders: the glenohumeral
joint, the acromioclavicular joint, and the undersurface of
the distal end of the clavicle at the site of attachment of
the coracoclavicular ligament (Fig. 40–45).
Other Diseases
Alkaptonuria (ochronosis) can lead to an arthropathy resem-
bling degenerative joint disease of the glenohumeral joint
in one or both shoulders. Similar, in acromegaly, osteo-
phytosis may be evident in this site, especially on the
inferior aspect of the humeral head. Hyperparathyroidism
leads to osseous resorption of the distal end of the clavicle
and adjacent acromion, as well as of the inferior aspect of
the clavicle at the site of attachment of the coracoclavicular
ligament. Post-traumatic changes include osteolysis of the
distal end of the clavicle, and degenerative joint disease may
be seen in association with recurrent posterior dislocation
Figure 40–47. Rotator cuff degeneration. AC, acromioclav- of the glenohumeral joint.
icular joint; CC, coracoclavicular ligament; GH, glenohumeral
joint; RC, rotator cuff.
SACROILIAC JOINT
The most important aspect in the differential diagnosis
Calcium Pyrophosphate Dihydrate Crystal
of diseases that affect the sacroiliac joint is the distri-
Deposition Disease (Fig. 40–48)
bution of the abnormalities. Findings can be bilateral and
Both the glenohumeral and the acromioclavicular joints symmetrical, bilateral and asymmetrical, or unilateral
can be affected in this disease. Fibrocartilage or hyaline (Fig. 40–49). In nearly every instance, the synovial portion
cartilage calcification, joint space narrowing, sclerosis, of the joint (the lower one half or two thirds of the
and osteophytosis can appear at either shoulder location. interosseous space between sacrum and ilium) is affected
to a greater degree than is the ligamentous portion (the
area above the synovium-lined space).
A B
C
Figure 40–49. Distribution of sacroiliac joint changes.
A, Symmetrical; B, Asymmetrical; C, Unilateral.
Psoriatic Arthritis and Reiter’s Syndrome junction with osteoarthritis of the contralateral hip may
(see Fig. 40–49A–C) be encountered. Findings include joint space narrowing,
The distribution of abnormalities is variable in psoriatic sclerosis, and osteophytosis. Erosions are not prominent,
arthritis and Reiter’s syndrome. Changes may be bilateral and para-articular rather than intra-articular ankylosis
and symmetrical, bilateral and asymmetrical, or unilateral. predominates.
Osseous erosion and bony sclerosis are similar to the
findings in ankylosing spondylitis, although joint space
Gouty Arthritis (see Fig. 40–49A–C)
narrowing and bony ankylosis occur with decreased
frequency. Proliferation of bone in the ilium and sacrum Abnormalities of the sacroiliac joint can be seen in patients
above the synovial aspect of the joint may be prominent, with long-standing tophaceous gout. Bilateral symmetrical,
particularly in Reiter’s syndrome. bilateral asymmetrical, or unilateral alterations consisting
of large erosions and reactive sclerosis are found.
Degenerative Joint Disease (Osteoarthritis)
(see Fig. 40–49A–C) Other Diseases
Osteoarthritis of the sacroiliac joint can be unilateral or Sacroiliitis accompanying inflammatory bowel diseases is
bilateral. Unilateral abnormalities of this joint in con- bilateral and symmetrical and cannot be differentiated from
Chapter 40 Target Area Approach to Articular Disorders: A Synopsis 539
that seen in ankylosing spondylitis (see Fig. 40–49A). In Osteitis condensans ilii produces bilateral and symmetrical
hyperparathyroidism, subchondral resorption of bone, espe- alterations in young women consisting of well-defined
cially in the ilium, produces bilateral and symmetrical triangular sclerosis of the inferior aspect of the ilium (see
changes consisting of joint space widening and reactive Fig. 40–49A). Sacroiliac joint involvement also may
sclerosis (see Fig. 40–49A). Unilateral abnormalities are accompany familial Mediterranean fever, relapsing poly-
typical of sacroiliac joint infection (see Fig. 40–49C). Such chondritis, Behçet’s syndrome, alkaptonuria, immobilization,
infection can be related to bacterial, mycobacterial, or and disuse.
fungal agents and is not infrequent in drug abusers.
SECTION
XI
Metabolic Diseases
CHAPTER 41
Osteoporosis computed tomography (CT) scanning, dual energy
radiographic absorptiometry (DEXA), and ultrasonog-
raphy, which are discussed elsewhere in this book.
SUMMARY OF KEY FEATURES
TERMINOLOGY
Osteoporosis is an extremely common metabolic disorder
that can accompany a variety of disease processes. It can Osteoporosis is characterized by qualitatively normal
be conveniently divided into generalized, regional, and (i.e., a normal mineral-to-matrix ratio) but quantitatively
localized types. Localized osteoporosis is commonly deficient bone. Radiographs in patients with osteoporosis
associated with focal skeletal lesions, such as neoplasm reveal increased radiolucency of bone, a finding that is
and infection. Generalized osteoporosis accompanies best termed osteopenia, meaning “poverty of bone.”
senile and postmenopausal states; endocrine disorders Osteopenia is a generic term, and it can have multiple
such as acromegaly, hyperthyroidism, hyperparathyroidism, causes. Other terms, such as demineralization and under-
and Cushing’s disease; pregnancy; heparin administration; mineralization, are not accurate descriptions of this
and alcoholism. This type of osteoporosis, which must radiographic finding. Demineralization implies a specific
be distinguished from other metabolic disorders such as loss of mineral without a concomitant loss of the organic
osteomalacia and osteitis fibrosa cystica, predominates component of bone, a phenomenon called halisteresis,
in the axial skeleton, with major effects on the vertebrae; which is not prominent in osseous tissue. Undermineral-
abnormalities of the appendicular skeleton are mild and ization might be more accurately applied to osteomalacia,
consist of uniform loss of osseous density (osteopenia). a condition characterized by inadequate secretion of mineral
In the vertebral bodies, characteristic changes in into osteoid matrix. The term deossification implies
radiolucency, trabecular pattern, and osseous contour abnormal loss of bone occurring as a result of accelerated
are encountered. bone resorption. In fact, some types of osteoporosis are
Regional osteoporosis accompanies disuse or associated with increased rates of resorption; others are
immobilization, reflex sympathetic dystrophy, and characterized by normal rates of resorption. Thus, osteo-
transient regional osteoporosis. Changes predominate porosis and deossification are not equivalent terms.
in the appendicular skeleton. A more aggressive type of The World Health Organization has used bone density
bone resorption in these conditions can lead to cortical measurements to classify categories of osteoporosis. These
bone changes at the endosteal, intracortical, and categories include terms such as osteopenia, osteoporosis,
subperiosteal bony envelopes and to spongy bone and established osteoporosis, which are dependent on the
changes at subchondral and metaphyseal locations. severity of bone loss and the presence or absence of frac-
Additional manifestations of osteoporosis include tures. At present, however, osteopenia is the most suit-
acute and insufficiency stress fractures and bone bars able term to describe increased radiolucency of bone.
(reinforcement lines). Magnetic resonance (MR) Osteopenia occurs when bone resorption exceeds bone
imaging shows promise in the assessment of some forms formation, no matter what the specific pathogenesis
of regional osteoporosis and in the detection or further (Table 41–1). Diffuse osteopenia is found in osteoporosis,
evaluation of complications associated with generalized osteomalacia, hyperparathyroidism, neoplasm, and a variety
osteoporosis. of other conditions. Accurate diagnosis of osteoporosis
rests on the radiographic findings of osteopenia, coupled
with typical clinical and histologic features.
Osteoporosis is established when the decrease in bone
mass is greater than that expected for a person of a given
INTRODUCTION age, sex, and race and when it results in structural bone
Osteoporosis is the most frequent metabolic bone failure manifested by fractures. These fractures are most
disease. It relates to a generalized decrease in bone mass.
The remaining bone is structurally normal, as deter-
mined by histologic and chemical analysis. Routine
radiographic procedures are not very helpful in the early TABLE 41–1
detection of this metabolic condition. It has been esti-
mated that 30% to 50% of skeletal calcium must be lost Major Causes of Diffuse Osteopenia
before a change appears on radiographs. Because of this
inadequacy, newer diagnostic techniques have been used Osteoporosis Hyperparathyroidism
for the earlier detection and quantitation of osteoporosis, Osteomalacia Neoplasm
including photon absorptiometry, transaxial (quantitative)
541
542 Section XI Metabolic Diseases
CAUSE
Osteoporosis can be classified as generalized (involving
the major portion of the skeleton, particularly its axial
component), regional (involving one segment of the
skeleton), or localized (single or multiple focal areas of
osteoporosis). The generalized, regional, and localized
forms of osteoporosis have many causes. Some of the
diseases associated with generalized or regional osteo-
porosis are discussed here. Localized osteoporosis may
accompany focal skeletal lesions such as arthritis,
infection, and neoplasm, and it is often overshadowed by
the radiographic features of the primary process itself.
Generalized Osteoporosis
The osseous manifestations of conditions leading to
generalized osteoporosis predominate in the axial
skeleton and proximal portions of the long bones of the
appendicular skeleton. Abnormalities of the spine are
particularly prominent and lead not only to osteopenia
but also to changes in vertebral contour, characterized
by biconcave vertebral bodies (“fish vertebrae”) and
collapse. Changes in the pelvis include osteopenia and
a coarsened trabecular pattern. Trabecular resorption
occurs in certain areas of the appendicular skeleton, such
as the femoral necks. In both the axial and the appen-
dicular skeleton, the decrease in radiodensity is usually Figure 41–1. Senile and postmenopausal osteoporosis. In
this elderly woman, a lateral radiograph of the lumbar spine
uniform, particularly in conditions with slowly progres- outlines increased lucency and biconcave deformity (fish
sive and long-standing osteoporosis. Although some differ- vertebrae) of multiple vertebral bodies.
ences are apparent in the distribution and appearance of
osteopenia among the various diseases that produce
generalized osteoporosis (Table 41–2), the previously
mentioned characteristics are most typical.
Hyperparathyroidism
Osteoporosis may occur during the course of hyperpara-
thyroidism. The characteristic skeletal changes in this
disease relate to increased rates of both bone resorption Figure 41–2. Steroid-induced osteoporosis. Lateral radiograph
and bone formation. Superimposed on osteoporosis are of the lumbar spine outlines osteoporosis and compressed
the typical findings of osteitis fibrosa cystica. vertebral bodies with peripheral condensation of bone (arrows).
This latter feature, which leads to radiodense superior and
inferior vertebral margins, is characteristic of exogenous or
Steroid-Induced Osteoporosis endogenous hypercortisolism.
Osteoporosis occurring during the course of either
Cushing’s syndrome or exogenous (iatrogenic) hyper-
cortisolism (Fig. 41–2) is well known. Histologic studies
have revealed decreased bone formation and increased
Pregnancy and Related Conditions
bone resorption. Radiographic evaluation discloses the
usual findings of osteoporosis, although peculiar conden- Though uncommon, osteoporosis may be observed in
sation of bone at the margins of the vertebral bodies and childbearing women. Its cause in this clinical setting has
involvement of extraspinal sites may be encountered. not been documented. Osteoporosis appearing in asso-
Acute fractures may be evident, especially at sites that ciation with pregnancy or lactation is accompanied by
contain predominantly trabecular bone (such as the ribs normal results on histologic examination and serum and
and spine). Insufficiency fractures in the axial (pelvis) urinary laboratory analysis.
and appendicular skeleton are characteristic and easily
overlooked.
Heparin-Induced Osteoporosis
The development of osteoporosis has been documented
Hyperthyroidism in patients receiving large doses of heparin (>15,000
Osteoporosis in hyperthyroidism is associated with an units/day). The changes may be reversible with cessation
increase in both bone resorption and bone formation. of therapy, but the mechanism of osteoporosis in these
Radiographic findings are typical of osteoporosis, although patients is not known. Typical radiographic findings in
rapid progression before treatment and rapid improve- the spine include osteopenia and vertebral compression.
ment after therapy may be evident.
Alcoholism
Acromegaly Alcoholic patients have reduced bone mass in comparison
Osteoporosis can occur in acromegaly in association with to controls and increased bone fragility, manifested as a
other forms of endocrine dysfunction or in acromegaly high rate of occurrence of fractures. The cause of this
unaccompanied by such dysfunction. loss in bone mineral is not known.
544 Section XI Metabolic Diseases
A B
C
Chapter 41 Osteoporosis 545
complication. Although transverse and oblique fractures deficiency states, and chronic liver disease. In some of
may occur in the bones of the peripheral skeleton, a more these disorders (plasma cell myeloma, storage diseases),
typical feature appears to be metaphyseal injury, espe- the primary disease process involves the bone directly; in
cially about the knees and ankles. This finding is less others (sickle cell anemia, neoplasm), the bone is altered
common in osteogenesis imperfecta, the disease most likely as a complication of the primary disease.
to be confused with idiopathic juvenile osteoporosis.
The major problem in the differential diagnosis is
distinguishing idiopathic juvenile osteoporosis from Differential Diagnosis
osteogenesis imperfecta (Fig. 41–4). Osteogenesis imper- Differentiation between generalized osteoporosis and
fecta congenita appears in early infancy and has distinc- osteomalacia, especially in adults, may be extremely diffi-
tive features. Osteogenesis imperfecta tarda may not cult on the basis of radiographic abnormalities and may
produce abnormalities until late childhood or adoles- require histologic evaluation. In infants and children, the
cence. Osteogenesis imperfecta may be associated with presence of rickets, with its characteristic metaphyseal
blue sclerae and progressive cranial, facial, and pelvic changes, is a valuable diagnostic clue. An accurate diag-
deformities, findings not evident in idiopathic juvenile nosis is especially important in infants, particularly those
osteoporosis. In osteogenesis imperfecta, thin diaphyseal receiving long-term intravenous hyperalimentation; in
cortices lead to characteristic fractures of the shaft. Other these patients, the development of rickets may lead to
childhood disorders, such as leukemia, homocystinuria, multiple fractures that contribute to an erroneous diag-
Cushing’s disease, and juvenile chronic arthritis, are usually nosis of osteogenesis imperfecta, the child abuse syn-
easily differentiated from idiopathic juvenile osteoporosis. drome, or copper deficiency (Fig. 41–5).
In adults with osteomalacia, diffuse osteopenia simu-
lates generalized osteoporosis. In osteomalacia, however,
Other Disorders
trabeculae may appear indistinct, and the interface of
Generalized osteoporosis may accompany plasma cell cortical and medullary bone may be obscured. Osseous
myeloma, Gaucher’s disease, glycogen storage disease, deformity (such as acetabular protrusion and a bell-shaped
anemia, nutritional deficiency, diabetes mellitus, immuno- thorax) and insufficiency fractures and pseudofractures
(which are most frequent in the pubic rami, medial portion
of the femoral neck, axillary margins of the ribs, scapula,
and, occasionally, tubular bones) may also be seen
(Fig. 41–6). In some forms of osteomalacia and rickets,
additional distinctive alterations are apparent. X-linked
hypophosphatemic osteomalacia (or rickets) is associated
with a generalized enthesopathy in which bone prolifera-
tion at sites of tendon and ligament attachment and tendi-
TABLE 41–3
Major Causes of Regional Osteoporosis
A B
Figure 41–7. Osteoporosis of immobilization and disuse. Radiographs obtained immediately before wrist
ligament reconstruction (A) and 2 months later (B) are shown. Observe in (B) the extent of the osteopenia.
infectious states, calcific tendinosis, vasculitis, electrical which produces excessive striation or tunneling in cortices;
injuries, and neoplasm. (4) endosteal bone resorption, which causes scalloping of
The pathogenesis of RSD is not entirely clear. The the endosteal surface, with subsequent uniform remodel-
most widely held theory is that of the “internuncial ing of the endosteum and widening of the medullary
pool,” in which it is assumed that an injury or lesion canal; and (5) subchondral and juxta-articular erosion,
produces painful impulses that travel via afferent path- which may lead to small periarticular erosions and intra-
ways to the spinal cord, where a series of reflexes are articular gaps in the subchondral bone. Because of the
initiated that spread by means of the interconnecting widespread nature and severity of the bone resorption in
pool of neurons. These latter reflexes stimulate the lateral RSD, radiographs may reveal rapid and severe osteopenia,
and anterior tracts and thereby provoke efferent path- particularly in the periarticular regions, that simulates
ways that travel to the peripheral nerves and produce the the appearance of primary articular disorders. The absence
local findings of RSD. of significant intra-articular erosions and joint space loss
Clinical symptoms and signs are variable. They may usually allows the accurate differentiation of RSD from
be evident in any involved site, although the characteristic these various arthritides. The preservation of joint space
distribution is the shoulder and hand. Rarely, multiple cannot be overemphasized as a characteristic finding in
extremities are affected. Initially, stiffness, pain, tender- this syndrome.
ness, and weakness may be associated with swelling, vaso- Bone and joint scintigraphy demonstrates typical
motor changes, hyperesthesia, and disability. The duration abnormalities in RSD, which may antedate the clinical
of RSD varies, and in some cases, findings may persist for and radiographic changes. It also reveals increased
years and become irreversible. technetium-99m pertechnetate radionuclide accumu-
Soft tissue swelling and regional osteoporosis are the lation in the articular regions. This finding appears to be
most important radiographic findings (Fig. 41–8). Five related to increased vascularity of the synovial mem-
types of bone resorption can be detected: (1) resorption brane, and bone-seeking agents likewise reveal increased
in the metaphyseal region, which leads to bandlike, patchy, accumulation in the involved bones in RSD, also related
or periarticular osteoporosis; (2) subperiosteal bone to increased blood flow. With three-phase bone scan-
resorption, which is similar to that occurring in cases of ning, the delayed images delineate this pattern most fre-
hyperparathyroidism; (3) intracortical bone resorption, quently, although scintigraphic abnormalities also occur
548 Section XI Metabolic Diseases
A B
Figure 41–8. Reflex sympathetic dystrophy: radiographic abnormalities. Pain and swelling developed in
a 65-year-old man after a minor injury to the hand. A, The initial film outlines mild periarticular
osteoporosis and soft tissue swelling. B, Six weeks later, the osteoporosis is much more exaggerated. The
periarticular osteoporosis simulates the appearance of rheumatoid arthritis.
during the angiographic and blood pool stages of the into this category: transient osteoporosis of the hip and
study. In these earlier stages of the study, soft tissue regional migratory osteoporosis.
uptake may be prominent. Bone scans obtained at differ-
ent times in a single patient may reveal migration of the Transient Osteoporosis of the Hip. Originally described in
scintigraphic abnormality from one side of a joint to the third trimester of pregnancy, transient osteoporosis is
another. Rarely, decreased or normal accumulation of the now known to typically occur in young and middle-aged
radionuclide is observed on bone scans, a finding that adults, particularly men. In male patients, either hip may
may be more frequent in children. be involved, whereas in female patients, the left hip is
Radiographic, scintigraphic, and quantitative bone affected almost exclusively. Hip pain begins sponta-
mineral analyses all reveal that RSD is often a bilateral neously and usually progresses within a few weeks and
process; the abnormalities are much more marked on one becomes severe enough to produce a limp. The clinical
side than the other, however. Almost universally, an entire findings regress in 2 to 6 months without permanent
extremity distal to an affected site is altered. Rarely, a sequelae.
segmental pattern affecting only a portion of an extrem- Radiographic findings are characteristic. Progressive
ity may be encountered. This localized form of RSD may and marked osteoporosis of the femoral head, which
lead to the involvement of one joint in an extremity, one begins several weeks after the onset of clinical abnormal-
or several digits of the hand or foot, or a portion of an ities, is associated with less extensive involvement of the
articular surface (e.g., patella, femoral condyle). femoral neck and acetabulum (Fig. 41–9). The joint space
is not diminished in size. Incomplete or complete frac-
tures of the femoral neck may occur. This complication
Transient Regional Osteoporosis has been emphasized in pregnancy or in the immediate
Transient regional osteoporosis is a term applied to postpartum period. Restoration of normal radiographic
conditions that share certain features: rapidly developing density of the bone takes place rapidly. Radionuclide
osteoporosis affecting periarticular bone, self-limited and studies using bone-seeking agents reveal abnormal accu-
reversible nature, and the absence of clear-cut evidence mulation of isotope before radiographically demonstrable
of inciting events such as trauma and immobilization. osteoporosis. MR imaging may show altered signal inten-
Two important diseases (which are probably related) fall sity of the bone marrow in affected sites and joint effusions.
Chapter 41 Osteoporosis 549
A B
C D
Figure 41–9. Transient osteoporosis of the hip in a 66-year-old man. A, Radiograph, collimated for
comparison of both hip joints, shows dramatic osteopenia of the right femoral head and neck. B and
C, Coronal T1-weighted (TR/TE, 800/15) spin echo (B) and STIR (TR/TE, 4000/15; inversion time,
150 msec) (C) MR images show the typical features of marrow edema. Note the sparing of the medial aspect
of the femoral head and joint effusion. D, Corresponding fat-suppressed T1-weighted (TR/TE, 467/15)
spin echo MR image following intravenous gadolinium contrast administration shows extensive
enhancement of the edema, as well as synovial enhancement.
The cause of this condition is unknown. Although it pattern is not invariable. Radiographic evidence of osteo-
has been regarded as a self-limited ischemic necrosis of porosis becomes apparent within weeks or months of the
bone, its similarity to RSD suggests a related neurogenic onset of clinical findings (Fig. 41–10). It also progresses
pathogenesis. Because transient osteoporosis of the hip is rapidly, subsequently diminishes, and appears at other
occasionally bilateral in distribution or may involve other sites. In extreme cases, periarticular osteoporosis extends
joints, a relationship with regional migratory osteoporosis for a considerable distance into the adjacent bone. The
is possible. joint space is not narrowed, nor can evidence of intra-
articular erosion be found. MR imaging shows signal
Regional Migratory Osteoporosis. The second form of intensity alterations in the bone marrow (see subsequent
transient osteoporosis is migratory in nature. Abnormal- discussion).
ities of the hip are less frequent than abnormalities in The migratory nature of the syndrome is the major
other areas, particularly the knee, ankle, and foot. It feature that differentiates this condition from transient
occurs in men more frequently than women and usually osteoporosis of the hip. Usually, the joint nearest the
becomes evident in the fourth or fifth decade of life. The diseased one is the next to be involved. The clinical
disorder is characterized by local pain and swelling, features may overlap, so more than one joint can be
particularly in the lower extremity, that develop rapidly, symptomatic at a given time. The pathogenesis of this
last up to 9 months, and then diminish and disappear. condition is not known, although regional migratory
Subsequent involvement occurs in other regions of the osteoporosis may be closely related to RSD and transient
same or opposite extremity, although this migratory osteoporosis of the hip.
550 Section XI Metabolic Diseases
Differential Diagnosis
Accurate diagnosis of regional osteoporosis associated
with disuse is not difficult. The osteoporosis associated
with RSD, transient osteoporosis of the hip, and regional
migratory osteoporosis may simulate other conditions.
Septic arthritis can lead to regional osteoporosis (Fig.
41–11); however, joint space narrowing and osseous
erosion are eventually observed in infection, whereas in
regional osteoporosis, these findings are not apparent.
Similarly, in rheumatoid arthritis, intense synovial inflam-
mation can produce significant cartilaginous and osseous
destruction. In addition, symmetrical involvement of
multiple joints is characteristic of rheumatoid arthritis.
In monoarticular processes such as pigmented villo-
nodular synovitis and idiopathic synovial osteochondro-
matosis, osteoporosis is not a dominant feature and,
when present, occurs late in the course of the articular Figure 41–12. Osteonecrosis. The diagnosis is established
disease. Monoarticular involvement of the hip or other by detecting a depression in the articular surface of the femoral
joints is not infrequent in osteonecrosis (Fig. 41–12). head (arrowhead); this finding is associated with patchy lucent
In osteonecrosis, the presence of osteoporosis on radio- areas and sclerosis.
graphic examination, the presence of increased radionuclide
accumulation on scintigraphic examination, and the
absence of articular space narrowing are findings that are
identical to those in regional osteoporosis. Further, the RADIOGRAPHIC-PATHOLOGIC CORRELATION
MR imaging features of certain types of regional osteo-
porosis overlap with those of ischemic necrosis of bone. General Distribution of Abnormalities
Although one condition can certainly occur without the Generalized osteoporosis is most prominent in the axial
other, a meaningful association of the two may exist (see skeleton, particularly the vertebral column, pelvis, ribs, and
discussion later in this chapter). sternum (Fig. 41–13). Eventually, less extensive changes
Chapter 41 Osteoporosis 551
TABLE 41–4
Abnormalities in Vertebral Body Shape
B C
A
Figure 41–18. Fish vertebrae: differential diagnosis of osteoporosis and osteomalacia. A, Normal vertebra
(a) becomes biconcave in outline in both osteomalacia (b) and osteoporosis (c). In osteomalacia, however, the
indentation of the osseous surface is smooth, and both the superior and inferior borders are involved to
approximately the same degree. In osteoporosis, the depression is more angular or irregular, and the supe-
rior and inferior surfaces of the vertebral body are frequently involved to different degrees. B, Osteomalacia.
On a lateral radiograph of the lumbar spine, observe the smooth biconcave deformities (fish vertebrae) of
the vertebral bodies and the poorly defined trabeculae. Vertical trabeculae are not accentuated. C, Osteoporosis.
On a radiograph of a coronal section of an involved spine, osteopenia and biconcave deformities are evident
in most of the vertebrae. The subchondral bone plates are distinct, and the superior and inferior surfaces of
the vertebral bodies are not involved to the same extent.
formation is based on the presence of a break in the portion of the femoral head in slightly curved radial
subchondral bone plate (corresponding to the site of disc lines. It contains the thickest and most densely packed
displacement), a lucent area of varying size bordering on trabeculae in the region.
the intervertebral disc (corresponding to the site and
variable amount of displaced disc material), and a small 2. Secondary compressive group. Trabeculae that arise from
degree of surrounding bone sclerosis (corresponding to the medial cortex of the shaft below the principal com-
trabecular condensation and thickening). pressive group form this group. They curve upward
and slightly laterally toward the greater trochanter
Proximal Portion of the Femur and the upper portion of the femoral neck. These
trabeculae are thin and widely spaced.
Analysis of the trabecular pattern of the upper end of the
femur has been used as an index of osteoporosis (Fig. 3. Principal tensile group. Trabeculae that arise from
41–21). In this region, four major anatomic groups of the lateral cortex below the greater trochanter and
trabeculae can be identified: extend in a curvilinear fashion superiorly and medially
1. Principal compressive group. This group comprises the across the femoral neck, ending in the inferior portion
uppermost compression trabeculae, which extend from of the femoral head, form the thickest tensile
the medial cortex of the femoral neck to the upper trabeculae.
Chapter 41 Osteoporosis 555
Patterns of trabecular loss may correlate with increas-
ing severity of osteoporosis (see Fig. 41–21). With early
trabecular resorption, accentuation of the principal com-
pressive and principal tensile groups is seen, and Ward’s
triangle is more prominent. With an increased degree of
trabecular resorption, tensile trabeculae are reduced in
number. With a further increase in trabecular resorption,
the outer portion of the principal tensile trabeculae oppo-
site the greater trochanter disappears. As the osteoporosis
increases in severity, all trabecular groups are resorbed,
with the exception of bony trabeculae in the principal
compressive group. With severe osteoporosis, even these
latter trabeculae may be partially or completely obliter-
ated. The value of this trabecular analysis (termed the
Singh index) is debated and has fallen into disuse with the
advent of more quantitative methods for assessing bone
mineral content.
Figure 41–19. Cupid’s bow contour. Frontal radiograph of a Cortex of the Tubular Bones
lower lumbar vertebral body reveals a normal variation in
vertebral outline, the Cupid’s bow contour. Observe the smooth Osseous resorption of bone cortices may become
parasagittal concavities on the inferior surface of the vertebral apparent in three specific sites (Table 41–5; Fig. 41–22):
body (arrows). (1) a cellular, vascularized membrane covering the
endosteal surface of the cortex, designated the endosteal
envelope; (2) an intracortical (haversian) envelope that
constitutes the surfaces within the cortical bone (haver-
4. Secondary tensile group. These trabeculae arise from the sian and Volkmann’s canals); and (3) a periosteal envelope
lateral cortex below the principal tensile group. They that covers the surface of the cortex. The response to
extend superiorly and medially and terminate after stimuli induced by various endocrine and metabolic dis-
crossing the middle of the femoral neck. orders is not always identical in these three bone envelopes.
In the femoral neck, a triangular area—Ward’s Endosteal resorption produces scalloped concavities
triangle—contains thin and loosely arranged trabeculae. This on the inner margin of the cortex that enlarge the marrow
area is enclosed by trabeculae from the principal compres- cavity. Intracortical resorption is characterized by the
sive, secondary compressive, and principal tensile groups. appearance of prominent longitudinal striations within
A B
Figure 41–20. Osteoporosis: cartilaginous (Schmorl’s) nodes. A, Schmorl’s nodes occur when a portion of
the intervertebral disc protrudes into the vertebral body (arrow) through a gap in the cartilaginous endplate
(1) and subchondral bone plate (2). B, Radiograph of a coronal section reveals depression of the subchondral
bone (arrowheads). A cartilaginous node (arrow) has resulted from disruption of the cartilaginous and bony
plates, creating a radiolucent defect within the bones with a small rim of sclerosis. A small cartilaginous node
is noted on the opposite side of the vertebral body.
(B, From Resnick D, Niwayama G: Intravertebral disk herniations: Cartilaginous [Schmorl’s] nodes.
Radiology 126:57, 1978.)
556 Section XI Metabolic Diseases
TABLE 41–5
Patterns of Osseous Resorption in Tubular Bones
Site Pattern
Cortex
Endosteal Diffuse cortical thinning or scalloped
erosions
Intracortical Cortical radiolucent areas or striations
Periosteal Subperiosteal erosions
Spongiosa
Subchondral Linear, bandlike, or spotty radiolucent areas
Metaphyseal Bandlike radiolucent areas
A Diffuse Homogeneous or spotty radiolucent areas
B C
558 Section XI Metabolic Diseases
A B C
Figure 41–23. Spongiosa of tubular bones: sites of osseous resorption. A, Patterns of bone loss include
bandlike radiolucent areas in the metaphysis or subchondral bone (top drawing) and homogeneous
periarticular radiolucent areas (bottom drawing). B, Bandlike resorption. Metaphyseal and subchondral
linear radiolucent areas are evident. C, Spotty resorption. A cystic pattern is apparent.
bone, findings that simulate a malignant tumor. Those of Vertebral Body Fractures
the sacrum are associated with hip, back, or buttock pain The common occurrence of both osteoporosis and frac-
and with a characteristic scintigraphic pattern in which tures of the vertebral body in elderly patients presents an
vertical and horizontal regions of increased accumulation immediate diagnostic challenge, particularly in those with
of the bone-seeking radiopharmaceutical agent produce a known extraosseous malignant neoplasms. Is the verte-
configuration referred to as the H pattern. Typical MR bral collapse related to osteoporosis alone, or does it indi-
imaging abnormalities also occur (see later discussion). cate that the vertebral body contains metastatic foci?
Insufficiency fractures of the supra-acetabular bone pro- Although routine radiography may be helpful in certain
duce poorly defined linear or punctate zones of increased cases, MR imaging is especially useful in this clinical
radiodensity of variable size. setting. The potential value of this technique relies on
alterations in the signal intensity of bone marrow in the
Reinforcement Lines (Bone Bars) involved vertebral body that allow the differentiation of a
tumorous process from one accompanying osteoporosis
In patients with chronic osteopenia, radiographs of the alone. In theory, this method seems attractive, but in
tubular bones, particularly the femur and tibia, com- practice, difficulties arise because of variations in the
monly reveal strands of trabeculae of variable thickness signal intensity of normal bone marrow, as well as of
that extend partially or completely across the marrow bone marrow that has been injured.
cavity (Fig. 41–26). These strands, which are referred to The signal intensity of normal vertebral marrow is
as reinforcement lines or bone bars, are evident in both generally dominated by its fatty content. Therefore, with
adults and children. The precise pathogenesis of bone a standard spin echo technique, high signal intensity is
bars is not clear. expected on T1-weighted images and intermediate signal
intensity is expected on T2-weighted images in the
MAGNETIC RESONANCE IMAGING normal marrow of the vertebral body. In the presence of
ABNORMALITIES tumor or any other condition in which replacement of
marrow fat has occurred, decreased signal intensity is
Generalized Osteoporosis
seen in the marrow on T1-weighted images, and T2-
Potential applications of MR imaging in patients with weighted images show either low or high signal, depend-
generalized osteoporosis include assessment of vertebral ing on the specific pathologic process present. With most
body fractures, analysis of extraspinal sites of insufficiency tumors, foci of increased signal intensity are noted on
fractures, and evaluation of bone mass and strength. T2-weighted spin echo images.
Chapter 41 Osteoporosis 559
Recent reports have emphasized that most vertebral
fractures resulting from metastatic involvement are asso-
ciated with bone marrow replacement on T1-weighted
images (seen as foci of decreased signal intensity), and
replacement is total in the vast majority of cases. It is
postulated that in cases of tumor, vertebral compression
occurs only when the entire bone marrow in the vertebral
body is replaced. Other features suggesting tumor include
an associated soft tissue or epidural mass and extension of
marrow abnormalities into the posterior elements. Con-
cavity to the posterior vertebral body cortex and focal
increased or heterogeneous signal on fluid-sensitive or
A non–fat-suppressed T1-weighted images following con-
trast enhancement also suggest tumor involvement. MR
imaging may also identify other lesions in noncom-
pressed vertebrae.
Although the initial experience with the use of MR
imaging to assess vertebral compression fractures has
been encouraging, a number of diagnostic difficulties can
be predicted. The effects of irradiation and chemother-
apy on the signal intensity of vertebral marrow in cases of
tumor may complicate the differential diagnosis. The
possibility also exists that tumorous involvement of a
vertebral body in a patient with preexisting osteoporosis
could lead to vertebral collapse before the entire verte-
bral body is infiltrated with neoplasm. The presence of
plasma cell myeloma rather than carcinoma in the col-
lapsed vertebral body produces patterns on MR imaging
B that closely simulate those of osteoporotic collapse.
Figure 41–24. Osteoporosis: insufficiency fracture of the Heterogeneous (rather than homogeneous) signal inten-
symphysis pubis. A, This 72-year-old woman with rheumatoid sity of marrow may be encountered in myeloma. Further,
arthritis received corticosteroid therapy. Radiograph of the the signal intensity of marrow during the treatment of
pubis shows considerable bone fragmentation and subluxation myeloma is somewhat variable.
(arrow). The findings are diagnostic of an insufficiency fracture,
although they are easily misinterpreted as evidence of a
malignant neoplasm. B, This 79-year-old woman has bilateral Insufficiency Fractures of Extraspinal Sites
insufficiency fractures of the ischium, pubis, and sacrum. The In comparison to conventional radiographs, MR imaging
distribution is quite characteristic and suggests the diagnosis. has superior sensitivity and specificity in the analysis of
Sacral fractures (arrows) are subtle.
insufficiency fractures of the pelvic bones. The sensitivity
of MR examination in this situation relates to the occur-
rence of bone marrow edema as an early manifestation of
the fracture. In common with edema from other causes
In general, pathologic fractures of the vertebral body and in other processes in which bone marrow is replaced
(defined as fractures related to skeletal metastasis) result or modified, the MR imaging characteristics of traumatic
in homogeneous replacement of vertebral marrow, with edema include decreased signal intensity on T1-weighted
low signal intensity demonstrated on T1-weighted spin spin echo images and increased signal intensity on T2-
echo images and high signal intensity on T2-weighted weighted spin echo and other fluid-sensitive sequences.
sequences. The MR imaging findings of a so-called benign The specificity of the MR examination relies, in large
fracture, in common with scintigraphic abnormalities, are part, on the morphology and distribution of the abnormal
dependent in part on the age of the fracture. In persons foci. Involvement of the portion of the sacrum located
with long-standing or chronic benign fractures, isoin- just medial to the sacroiliac joint in a unilateral or bilat-
tense marrow signal comparable with that of normal eral distribution, the portion of the symphysis pubis that
vertebral bodies is evident on all the MR sequences used. abuts on the fibrocartilaginous disc, the inner contour of
In those with more acute benign fractures, high signal the ilium, and the supra-acetabular bone is most charac-
intensity in the bone marrow of the affected vertebral teristic of insufficiency fractures of the pelvis (Fig. 41–27).
body is evident on fluid-sensitive sequences and is typi- Some diagnostic pitfalls are encountered when MR imag-
cally characterized by abnormal signal bands that parallel ing is used for the analysis of pelvic insufficiency frac-
the endplates; these may return to normal on non–fat- tures. First, the pelvis is also a frequent site of skeletal
suppressed T1-weighted images following contrast metastasis, and the resulting altered signal intensity is
enhancement. The MR imaging characteristics of the similar to that seen in cases of fracture. Second, the corti-
bone marrow in cases of acute compression fractures usu- cal disruption and fragmentation accompanying insuffi-
ally revert to a normal pattern in 1 to 3 months. ciency fractures may resemble the findings of an aggressive
560 Section XI Metabolic Diseases
A B
C D
Figure 41–25. Osteoporosis: bilateral sacral insufficiency fracture. This 81-year-old woman presented
with low back pain. Axial T1-weighted (TR/TE, 600/12) (A) and fast T2-weighted (TR/TE, 4000/102)
(B) spin echo MR images show extensive edema through the sacral ala bilaterally. The fracture line on the
left is vaguely seen. Axial (C) and reformatted oblique coronal (D) noncontrast CT scans show sacral
fractures and subtle bone sclerosis. Fractures are seen to better advantage on the reformatted oblique
coronal image (D).
Regional Osteoporosis
tumor. Discrete soft tissue masses, however, do not gen-
erally occur in cases of insufficiency fracture. Third, the Transient Bone Marrow Edema
conversion of hematopoietic marrow to fatty marrow in Transient bone marrow edema is the term used to describe
normal persons is not as complete in the pelvis as it is in the abnormality in patients with clinical manifestations
other extraspinal locations. identical to those of transient osteoporosis of the hip but
MR imaging can also be useful in the diagnosis of with normal radiographs. The abnormalities in the process
insufficiency fractures of the femoral neck and intertro- relate to the presence of edema in the femoral head and
chanteric region. Insufficiency fractures tend to course neck. Decreased signal intensity in this area on T1-
horizontally, across the femoral neck, at angles almost weighted sequences and increased signal intensity in
perpendicular to those of some of the trabecular groups the corresponding region on T2-weighted sequences are
(Fig. 41–28). typically observed (Fig. 41–29). Associated joint effusions
Chapter 41 Osteoporosis 561
FURTHER READING
usually abrupt and marked by sphericity and progres- Osteomalacia (Fig. 42-5) is characterized by abnormal
sive enlargement. Cells nearing the metaphyseal side quantities of osteoid (inadequately mineralized bone
of the growth plate become quite large and vacuolated, matrix) coating the surfaces of trabeculae and lining the
with the last cells in the column becoming nonviable. haversian canals in the cortex (“osteoid seams”). Tra-
The upper portions of this zone are active metaboli- beculae become thin and decrease in number. In the
cally, and calcification of intervening cartilage matrix cortex, the haversian systems become irregular, and large
occurs. channels develop. Osteoid seams are not pathognomonic
4. The zones of primary and secondary spongiosa are located for osteomalacia and may be found in other states of high
in the metaphysis immediately subjacent to the growth bone turnover. In osteomalacia, however, these osteoid
plate. Cartilage bars are partially or completely calci- seams increase in both number and width. Looser’s lines
fied and become ensheathed with osteoblasts, which or Milkman’s pseudofractures, which strongly suggest the
produce layers of osteoid. Bone is produced by endo- radiographic diagnosis of osteomalacia, are composed of
chondral ossification. focal accumulations of osteoid. Osteitis fibrosa cystica
frequently is superimposed on the lesions of rickets and
The rachitic lesion displays disorganization in the
osteomalacia, reflecting hyperparathyroidism. This feature
growth plate and subjacent metaphysis (Fig. 42-4). The
is particularly prominent in renal osteodystrophy.
resting and proliferative zones of the cartilaginous growth
plate appear normal. The zone of maturation reveals a
disorganized increase in the number of cells and a loss of Radiographic Diagnosis of Rickets
normal columnar pattern. This cell mass results in an and Osteomalacia
increase in length and width of the growth plate. Concomi- Rickets. Rachitic changes are more obvious in regions of the
tantly, vascular intrusion from the metaphysis and subse- most active growth; the sites of highest radiographic yield
quent calcification of the intervening cartilaginous bars are the costochondral junctions of the middle ribs, the distal
are decreased and grossly disordered. Defective mineraliza- part of the femur, the proximal portion of the humerus,
tion in the zone of primary spongiosa and a lack of proper both ends of the tibia, and the distal ends of ulna and radius.
formation of bone lamellae and haversian systems occur.
Vitamin D Deficiency
Classic vitamin D–deficiency rickets is encountered
uncommonly in the United States today, owing to the
widespread addition of synthetic vitamin D2 to foods,
notably dairy products and bread. The natural source of
vitamin D for humans is not dietary, however. Rather,
humans depend mainly on the ultraviolet rays of the sun
for endogenous conversion of 7-dehydrocholesterol in
the skin to the prohormone vitamin D3 (cholecalciferol).
A reasonable degree of exposure to the sun should prevent
the development of rickets and osteomalacia in a normal
person whose diet contains adequate amounts of calcium
and phosphorus.
From a worldwide perspective, deficiency rickets and
osteomalacia are still health problems. Although decreased
Figure 42–7. Pseudofracture (arrow) in adult patient with exposure to the sun appears to be important, deficiency
X-linked hypophosphatemic osteomalacia occurring in a
rickets and osteomalacia have been reported even in sun-
characteristic location in the proximal portion of the ulna. Note
bowing of the ulna. (From Pitt MJ, Haussler MR, Davis J, et al: rich areas. Further, in developed countries, osteomalacia
Current concepts of vitamin D metabolism: Correlation with occurs in the elderly. The elderly segment of the popu-
clinical syndromes. Crit Rev Radiol Sci 10:145, 1977.) lation may constitute the largest group subject to the
development of osteomalacia, particularly those patients
who are house-bound or institutionalized for long periods.
Although decreased exposure to the sun is a prime cause,
other postulated factors include deficient intake of oral
CLINICAL SYNDROMES
vitamin D2, intestinal malabsorption in the elderly, and
An etiologic approach to the rachitic and osteomalacic lower vitamin D hydroxylase activity in the liver and
disorders, as shown in Figure 42-8, includes the kidney. Although “deficiency” rickets (and osteomalacia)
following: usually connotes a lack of vitamin D, insufficiencies of
1. Abnormalities of vitamin D metabolism: prohormone calcium, phosphorus, or both, should also be considered.
vitamin D, 25-OH-D, or 1,25(OH)2D. Rickets secondary to phosphorus deficiency is usually a
result of congenital or acquired renal disease. Proximal
2. Rachitic and osteomalacic syndromes resulting prima-
renal tubular dysfunction with defective absorption of
rily from renal tubular phosphate loss.
phosphorus is seen in X-linked hypophosphatemia and
3. Rachitic and osteomalacic syndromes that do not show Fanconi’s syndromes. Phosphate loss also may complicate
abnormalities of vitamin D metabolism or aberrations hemodialysis, cadaveric renal transplantation, or excessive
of calcium or phosphorus metabolism. ingestion of aluminum hydroxide, which binds phosphate
Table 42-1 lists useful points in diagnosing specific in the gut. Rickets secondary to insufficient calcium in
syndromes. the diet is rare.
Gastrointestinal Malabsorption
Disorders of the small bowel, hepatobiliary system, and
pancreas associated with intestinal malabsorption are the
most common causes of vitamin D deficiency in the Figure 42–9. Rickets in a 4-month-old Mexican infant with
United States. Rickets and osteomalacia may develop in celiac disease. The metaphysis is demineralized and disorganized.
many small bowel malabsorptive states, including sprue, Metaphyseal widening reflects the enlarged growth plate.
570 Section XI Metabolic Diseases
requirements for calcium, phosphorus, and vitamin D are Secondary Hyperparathyroidism. Secondary hyperparathy-
greater than those of an infant born at term. This increased roidism is noted consistently in untreated uremia occur-
need may not be provided for in the diet. Human milk ring early in the course of the disease. Parathyroid
and standard infant formulas, which are adequate for term hormone levels may be increased significantly and fre-
babies, have insufficient amounts of vitamin D, phosphorus, quently are higher than the levels reached in primary
and probably calcium for premature infants. Rickets and hyperparathyroidism. The secondary hyperparathyroidism
osteomalacia in these premature infants lead to signifi- is provoked by hypocalcemia, which results from several
cant morbidity from fractures, respiratory distress, and different mechanisms; phosphate retention is the major
skeletal deformity. factor.
of aluminum toxicity include bone pain, muscle weak- typically are short, bowlegged, and stocky. The develop-
ness, dementia, microcytic anemia, and hypercalcemia. ment and severity of rickets may differ among patients
Advanced complications may include pathologic fractures, with the classic syndrome.
seizures, encephalopathy, and death. Radiographic features may allow for the specific diag-
Radiographs may be helpful in predicting aluminum nosis of this syndrome. In children, rachitic changes at
toxicity without resorting to biopsy. Diagnostic findings the growth plates, in themselves nonspecific, may be only
include the following: (1) an increased frequency of frac- mild in degree. Osteopenia is not prominent. Bowing of
tures, (2) a lack of osteosclerosis, (3) a relative decrease in long bones, particularly of the lower extremities, may
subperiosteal resorption (hyperparathyroidism) compared occur, but deformity frequently is minimal. With increas-
with patients without aluminum toxicity, and (4) a signi- ing age, the trabecular pattern becomes coarsened. Looser’s
ficant increase in osteonecrosis after transplantation. zones are more prevalent and can be complicated by
complete fractures. By adulthood, a generalized increase
in bone density, especially in the axial skeleton, is charac-
Hereditary Vitamin D–Dependent Rickets
teristic. Enthesopathic calcification and ossification
Hereditary vitamin D–dependent rickets, also termed develop in the paravertebral ligaments, anulus fibrosus,
pseudovitamin D–deficiency rickets, is a rare autosomal and capsules of apophyseal and appendicular joints (Figs.
recessive disorder characterized by the clinical, radio- 42-12 and 42-13). The spinal changes may resemble those
graphic, and biochemical features of vitamin D deficiency. of ankylosing spondylitis or diffuse idiopathic skeletal
Vitamin D intake is normal, however, and no evidence hyperostosis. In contrast to ankylosing spondylitis, how-
exists of other disease states, such as intestinal malabsorp- ever, the sacroiliac joints in X-linked hypophosphatemia
tion and liver or kidney disorders, that would account for show no bone erosions. Narrowing of the spinal canal,
derangement in vitamin D metabolism. Symptoms may which may relate to ossification of the ligamentum flavum,
be present as early as 3 months of age (in contrast to the is common. In the pelvis, multiple sites of calcification
later onset of nutritional rickets), with most patients being may involve the acetabulum, iliolumbar ligaments, and
symptomatic by the age of 1 year. Rachitic bone changes sacroiliac ligaments (see Fig. 42-12B). The appendicular
may be severe and rapidly progressive, with pathologic skeleton shows multiple sites of new bone formation at
fractures. Although hypophosphatemia is present, the various muscle and ligament attachments. Separate small
primary rachitogenic factor is hypocalcemia, which results ossicles may develop around various joints, particularly
from a decrease in intestinal calcium absorption. This those in the carpus (see Fig. 42-13).
condition is often apparent soon after birth, and second- X-linked hypophosphatemia and the various Fanconi’s
ary hyperparathyroidism follows. syndromes produce rickets and osteomalacia principally
by renal tubular phosphate loss. Two separate renal tubular
mechanisms for phosphate resorption have been identi-
Rickets and Osteomalacia Secondary
fied: a parathyroid hormone–sensitive component, which
to Phosphate Loss
is responsible for about two thirds of the total net resorp-
A number of rachitic and osteomalacic syndromes have tion, and an additional system, which is responsive to the
been identified that, although differing in genetic and serum calcium level. The parathyroid hormone–sensitive
clinical features, share one or several renal tubular abnor- component is completely absent in male patients with X-
malities: renal phosphate loss with secondary hypophos- linked hypophosphatemia and is partially absent in female
phatemia, glycosuria, aminoaciduria, renal tubular acidosis, patients. Abnormalities in vitamin D metabolism also
hypokalemia, and vasopressin-resistant polyuria. These have been demonstrated in both X-linked hypophos-
diseases are collectively designated Fanconi’s syndrome. phatemia and Fanconi’s syndromes.
The most common is cystinosis; tyrosinemia and the
oculocerebrorenal syndrome (Lowe’s syndrome) are
Tumor-Associated Rickets and Osteomalacia
other, less common examples. In addition to congenital
diseases, acquired renal tubular disorders with similar Hypophosphatemic vitamin D–refractory rickets and
clinical and biochemical features may be secondary to osteomalacia in association with various neoplasms are
drug toxicity, heavy metal poisoning, paraproteinemias, being recognized with increasing frequency. The asso-
and tumors. ciated neoplasms occur in children and adults, are located
in soft tissues or bone, and vary in size. The lesions typi-
X-Linked Hypophosphatemia. X-linked hypophosphatemia cally are vascular and often show foci of new bone forma-
(also known as familial vitamin D–resistant rickets) is the tion; the most frequent histologic diagnosis is hemangio-
most common form of renal tubular rickets and osteo- pericytoma. Bone lesions include nonossifying fibroma,
malacia. The classic syndrome is transmitted genetically giant cell tumor, osteoblastoma, and non-neoplastic
as an X-linked dominant trait. Men are affected to a diseases such as fibrous dysplasia and neurofibromatosis.
greater degree than women. The syndrome is character- Radiographic changes of rickets and osteomalacia may
ized by lifelong hypophosphatemia that is secondary to be advanced. Hypophosphatemia is the predominant bio-
renal tubular phosphate loss, decreased intestinal absorp- chemical feature, related to failure of renal tubular
tion of calcium, and normal serum levels of calcium. reabsorption of phosphate. Circumstantial evidence
Rickets generally appears between 12 and 18 months of suggests the existence of a tumor-elaborated humoral
age. Remission usually follows growth plate closure, but substance that affects renal phosphate absorption in the
recurrence of symptoms is common later in life. Patients proximal tubule directly.
Chapter 42 Rickets and Osteomalacia 573
Hypophosphatasia
Hypophosphatasia is a rare disorder transmitted geneti-
Figure 42–13. X-linked hypophosphatemia. Small ossicles cally in an autosomal recessive pattern. It is characterized
are noted at the radial aspect of the wrist, and ossification of the by defective skeletal mineralization resembling that of
triangular fibrocartilage complex is present. Capsular ossifica- rickets and osteomalacia, low serum alkaline phosphatase
tion is noted at multiple interphalangeal and metacarpopha- levels, and abnormal amounts of phosphoethanolamine
langeal joints. in the urine and blood. Although most cases are diag-
574 Section XI Metabolic Diseases
Figure 43–1. Osteolytic stage of Paget’s disease. A, Cranial vault (osteoporosis circum-
scripta). The osteolytic bone usually commences in the frontal or occipital area of the skull.
Its advancing edge (arrowheads) is well demarcated from adjacent normal bone. Focal B
radiodense areas (arrow) are apparent within the areas of osteolysis. In some locations,
involvement of a portion of the cranial vault creates beveled margins (open arrow).
B, Tubular bones. An advancing wedge-shaped radiolucent edge (arrowhead) is observed in
the femur.
A B
Figure 43–3. General stages of Paget’s disease: osteosclerotic stage. A, Long-standing Paget’s disease of
the pelvis with diffuse osteosclerosis is evident. Note the degenerative joint disease in both hips. B, Axial CT
scan at the level of the femoral heads shows dramatic involvement of the pelvis with sparing of the femora.
Note the extensive osteosclerosis, coarse trabeculae, and cortical thickening. It is difficult to appreciate the
increased size of the involved bone owing to the bilateral involvement.
ment of the vertebral posterior elements. In the lumbar spastic quadriplegia. Pagetic abnormalities of the verte-
spine, Paget’s disease can lead to compression of the cauda bral bodies at any level can result in collapse.
equina and encroachment on the foramina. In the thoracic The vertebral bodies and posterior elements may be
spine, cord compression and intervertebral foraminal altered, with the changes being more apparent in the
impingement may be seen. With cervical involvement, vertebral bodies. Enlarged, coarsened trabeculae are
Paget’s disease can lead to cord compression and even observed, and condensation of bone may be especially
prominent along the contours of the vertebral body. In
this situation, the highlighted contour of the involved
vertebra resembles a picture frame. In some patients, a
uniform increase in osseous density produces an ivory
vertebra, and differentiation of Paget’s disease from other
causes of ivory vertebra, such as metastasis and lymphoma,
may be difficult. In this situation, if the vertebra is enlarged,
the diagnosis of Paget’s disease is usually ensured.
Biconcave deformities termed “fish vertebrae” are
identical to those occurring in metabolic disorders such
as osteoporosis, osteomalacia, and hyperparathyroidism.
These deformities are related to compression of affected along the inner contour of the pelvis. The iliopubic and
vertebrae by the adjacent intervertebral disc. Pagetic ilioischial lines may be prominent, with little other
changes in the posterior elements may occur in conjunc- osseous abnormality of the pelvis. Thickening in these
tion with vertebral body abnormalities or as an isolated areas must be distinguished from irregularity of the
spinal manifestation of the disease. With pediculate involve- iliopubic contour, which is frequent in normal elderly
ment, increased radiodensity may simulate osteoblastic persons, and from calcification of Cooper’s ligament.
metastasis. Sacral alterations are usually associated with The periphery of the iliac bone may appear radiodense,
involvement of additional areas of the pelvis. Osteolytic with a relatively lucent central portion. Iliac sclerosis
features commonly predominate in the sacrum, and in such adjacent to the sacroiliac joint can simulate the changes
instances, a missed diagnosis is not infrequent (Fig. 43–6). of osteitis condensans ilii. Involvement of the pubis and
ischium is often associated with enlarged osseous con-
Pelvis. Manifestations of Paget’s disease in the pelvis (see tours. Para-acetabular alterations can lead to acetabular
Fig. 43–3) usually include both bone resorption and bone protrusion. Diffuse abnormalities of the pelvis are not
formation. Trabecular thickening may initially be evident infrequent and are commonly asymmetrical.
A B
C D
Figure 43–6. Radiographic abnormalities in Paget’s disease: sacrum in a 76-year-old man. A, Radiograph
shows few trabeculae, and the entire bone is osteopenic. The remaining trabecular pattern is coarsened,
diagnostic of Paget’s disease. B, Marked focal accumulation is seen throughout the entire sacrum, a pattern
virtually diagnostic of Paget’s disease. C, Oblique coronal T1-weighted (TR/TE, 500/20) spin echo MR
image shows preservation of normal fatty marrow. The coarse trabeculae are not well appreciated. D, Axial
CT scan viewed at a bone window demonstrates increased fatty marrow, with coarse trabeculae and
thickened cortex.
Chapter 43 Paget’s Disease 581
Additional Sites. Paget’s disease can affect any part of the OTHER DIAGNOSTIC METHODS
skeleton (Fig. 43–7). Involvement of the tubular bones of
Scintigraphy
the extremities is most common in the femur, tibia, and
humerus. As noted previously, such involvement is Bone scintigraphy has been used in the evaluation of
characterized by an epiphyseal predilection, an advancing patients with Paget’s disease (Fig. 43–8). As would be
wedge of radiolucency, periosteal apposition of new bone expected, areas of skeletal disease are depicted as sites of
leading to widening of the osseous surface, and deformity increased uptake of bone-seeking radiotracers and, in
and fracture. Cortical thickening with encroachment appropriate locations, as sites of marrow replacement.
on the medullary canal is evident. In rare instances, the Scintigraphic abnormalities may precede radiographically
involved bone reveals narrowing or contraction. Diaphy- detectable changes and can document the extent of the
seal changes without epiphyseal abnormality are extremely initial lytic phase of Paget’s disease. In this phase, increased
unusual but may be observed, particularly in the tibia, radionuclide activity may be particularly prominent at
where pagetic involvement may begin in the mid- the advancing edge of bone. Serial bone scintigraphs may
diaphysis. A more characteristic site of initial tibial provide objective evidence of the effect of various
involvement in cases of “diaphyseal” Paget’s disease is the therapeutic agents.
anterior tibial tubercle. Peculiarities in the distribution of
stress in the lower extremity lead to typical patterns of
Computed Tomography
deformity; most characteristically, exaggerated lateral
curvature of the femur and anterior curvature of the tibia Computed tomography (CT) scanning is generally not
become apparent. Lateral bowing of the humerus is also required in the evaluation of uncomplicated Paget’s
seen. The fibula is frequently spared. Paget’s disease of disease. When used, this technique reveals the coarsened
the bones of the feet and hands is less common. In the trabecular pattern, thickened cortex, increased size of the
foot, changes show a predilection for the calcaneus. bone, and decreased size of the medullary cavity that are
A B C
Figure 43–7. Radiographic abnormalities in Paget’s disease: additional sites. A, Femur. Alterations extend
from the femoral head to the mid-diaphyseal region. The cortex is thickened, with cortical encroachment
on the medullary canal. Observe the coarse trabecular pattern. B, Humerus and ulna. Coarsely trabeculated
bone is associated with radiolucent regions of varying size. The osseous outlines are enlarged. C, Hand. The
characteristic findings of Paget’s disease are observed throughout an entire metacarpal bone.
582 Section XI Metabolic Diseases
A B
Figure 43–9. MR imaging abnormalities in Paget’s disease: inactive disease in the spine. A, Routine
radiograph shows classic pagetic abnormalities with a “picture-frame” appearance. The vertebral body is
mildly compressed, and osteophytes are seen. Note the involvement of the posterior osseous elements. B,
On a sagittal T1-weighted (TR/TE, 600/20) spin echo MR image, coarse trabeculation appears as regions
of low signal intensity (arrows).
Chapter 43 Paget’s Disease 583
TABLE 43–2 indefinitely in some patients, or progressing and extend-
ing across the entire bone in others. Pathologic fractures
Musculoskeletal Complications of Paget’s Disease in Paget’s disease are the most common orthopedic com-
plication and are typically seen in the femur, humerus,
Neurologic abnormalities tibia, spine, and pelvis. Because the risk of sarcoma is
Osseous deformities substantial in cases of pathologic fracture in Paget’s
Fractures disease, some authors recommend a biopsy whenever
Neoplasms such a fracture develops.
Soft tissue masses
Osteomyelitis Neoplasm
Extramedullary hematopoiesis
Crystal deposition Neoplastic involvement in Paget’s disease includes sarco-
Monosodium urate
matous degeneration, giant cell tumor, and superimposi-
tion of another tumorous condition such as metastatic
Calcium pyrophosphate dihydrate*
disease, plasma cell myeloma, and lymphoma. It is gener-
Calcific periarthritis ally believed that malignant changes develop in approxi-
Degenerative joint disease mately 1% of patients with Paget’s disease. The tumor
Rheumatoid arthritis and its variants* is apparent in areas of pagetic involvement (Figs. 43–11
and 43–12).
*Questionable association. Patients with sarcomatous degeneration in Paget’s
disease are usually between 55 and 80 years of age. Men
are affected slightly more frequently than women, and at
lower extremity, particularly in the femur and tibia. They a younger age. Clinical findings include pain and
appear as single or multiple horizontal radiolucent areas, swelling. The bones most commonly affected are the
with a predilection for the convex aspect of the bone femur, pelvis, and humerus. Except for the higher fre-
(lateral aspect of the femoral neck and shaft, anterior quency of neoplastic changes in the humerus and the
aspect of the tibia). Although these linear radiolucent lower frequency in the skull and vertebrae, the distri-
areas that partially traverse the bone resemble Looser’s bution of sarcomas in Paget’s disease is similar to that of
zones, which are apparent in osteomalacia, the involve- the disorder itself. Predominance of certain cells most
ment of convex rather than concave surfaces is distinc- commonly leads to a diagnosis of osteosarcoma (50% to
tive. These stress fractures appear most frequently in 60%) or fibrosarcoma (20% to 25%), although additional
patients with long-standing (inactive) disease and have diagnoses such as chondrosarcoma (10%) may be enter-
a variable course, healing or remaining unchanged tained. No matter which cell type predominates, the
A B
584 Section XI Metabolic Diseases
A B
prognosis for the patient is ominous. Osteolysis rather tissue lesion that may contain new bone (Fig. 43–14) and
than osteosclerosis is the major radiographic character- may also simulate neoplastic degeneration.
istic of the sarcoma. In addition to bone destruction,
findings include a soft tissue mass, cortical disruption, ARTICULAR ABNORMALITIES
bony spiculation, and a persistent fracture without evi-
dence of healing. Crystal Deposition
Giant cell tumors associated with Paget’s disease are Hyperuricemia has been reported in 40% of patients
uncommon but well described; they often occur in the with symptomatic Paget’s disease, related to overproduc-
skull, facial bones, pelvis, and clavicle. The tumors invari- tion of urate, and patients may have typical clinical and
ably affect regions of the skeleton involved in the pagetic radiographic features of gout. Coexistence of calcium
process; however, the pagetic involvement may be incon- pyrophosphate dihydrate crystal deposition and Paget’s
spicuous radiographically. A lytic lesion causing a disease has also been noted, although the frequency of
remodeled, expansile osseous contour without radiologic crystal deposition in patients with Paget’s disease may not
matrix is observed. The prognosis is usually good. In differ significantly from that in controls.
some cases of giant cell tumor complicating Paget’s disease,
familial and geographic clustering is evident.
Metastatic disease and Paget’s disease may coexist, and Degenerative Joint Disease
it is interesting to speculate that increased local blood Clinical and radiographic features of degenerative joint
flow in the latter disorder may make the bone more disease may be apparent in patients with Paget’s disease.
susceptible to metastasis. Typical sites of the primary This complication is reported most frequently in the hip
tumor are the breast, lung, kidney, prostate, and colon. and knee. In the hip, the pattern of narrowing of the
Of interest, metastasis to uninvolved skeletal sites has articular space depends on whether the acetabulum or
been recorded in patients with Paget’s disease. the femur is involved. The pattern of narrowing in Paget’s
The diagnosis of neoplasm complicating Paget’s disease can generally be differentiated from that seen in
disease is usually not difficult. Clinically, increased pain primary osteoarthritis (Fig. 43–15). Acetabular protrusion
and a soft tissue mass are observed. On radiographs, soft may complicate Paget’s disease of the acetabulum. Osteo-
tissue swelling and a lytic lesion may be seen, and in some phyte formation is usually a mild feature of the disease.
tumors, the initial sign may be a pathologic fracture. Any The pathogenesis of the arthritic changes associated
enlarging lytic lesion within pagetic bone should be care- with Paget’s disease is not clear. Hypervascularity and
fully evaluated. These lytic neoplasms must be differen- rapid bone turnover in pagetic subchondral bone may
tiated from the osteolytic phase of Paget’s disease and lead to accelerated endochondral bone formation at the
from lucent areas representing fat-filled marrow spaces expense of articular cartilage. Superimposed on this inter-
(Fig. 43–13). Periosteal bone proliferation in uncom- ference with dynamics at the chondro-osseous junction
plicated Paget’s disease can produce a radiodense soft are mechanical abnormalities about the articulation.
Chapter 43 Paget’s Disease 585
Figure 43–12. Complications
of Paget’s disease: osteosarcoma.
A, Radiograph of the humerus
in a 60-year-old man shows
pagetic involvement of the
distal portion of the bone and a
bone-forming malignant tumor
in the diaphysis, along with soft
tissue ossification (arrow). B,
Bone scan shows intense uptake
of radionuclide in the distal two
thirds of the humerus and a
relative photopenic region in
the diaphysis (arrow) that
corresponds to the site of the
tumor. This illustration is
oriented to correspond to that
of the radiograph. C, Sagittal
T1-weighted (TR/TE, 700/12)
spin echo MR image shows that
the tumor has low signal inten-
sity in the shaft of the humerus,
along with a soft tissue mass
(arrows). D, Coronal STIR
(TR/TE, 4000/39; inversion
time, 150 msec) MR image
demonstrates high signal inten-
sity in the tumor.
A B
C D
DIFFERENTIAL DIAGNOSIS
General Features
Although the initial lytic phase of Paget’s disease may be
difficult for an inexperienced observer to identify, its
radiographic features are diagnostic. Involvement of the
end of a long bone, sharply demarcated bone lysis, and an
advancing wedge of radiolucency allow an accurate diag-
nosis. Widespread sclerosis, which can be apparent in
Paget’s disease of long duration, also has specific charac-
teristics, such as bony enlargement and a coarsened
trabecular pattern, which can be distinguished from find-
ings associated with other diseases. Diffuse increased
skeletal radiodensity may be observed in bony metastasis
(particularly from prostatic carcinoma; Fig. 43–17),
myelofibrosis, fluorosis, mastocytosis (urticaria pigmen-
tosa), renal osteodystrophy, fibrous dysplasia, and tuberous
Figure 43–14. Paget’s disease: soft tissue extension. Note the sclerosis. Additional findings in these other disorders
extraosseous extension of Paget’s disease at three sites (arrow- ensure their recognition. For example, hepatosplenomegaly
heads) in the humerus characterized by ill-defined periosteal (myelofibrosis, mastocytosis), ligamentous ossification
new bone formation. (fluorosis), focal radiodensity (mastocytosis and tuberous
Chapter 43 Paget’s Disease 587
A B
Figure 43–16. Treatment of Paget’s disease: etidronate disodium (EHDP). Radiographs obtained 1 year
after initial manifestation of the disease (A) and 4 years 6 months after discontinuation of treatment with
EHDP (B) reveal improvement in the abnormalities of the second lumbar vertebra on the later examination.
(From Nicholas JJ, Helfrich DJ, Cooperstein L, Goodman L: Clinical and radiographic improvement of
bone of the second lumbar vertebra in Paget’s disease following therapy with etidronate disodium: A case
report. Arthritis Rheum 32:776, 1989.)
A B
Figure 43–17. Skeletal metastasis from prostate carcinoma compared with Paget’s disease A, Patient with
metastatic prostate carcinoma demonstrates patchy osteosclerosis. B, Corresponding image from a patient
with Paget’s disease shows a coarsened trabecular pattern, cortical thickening, and osseous enlargement.
FURTHER READING
XII
Endocrine Diseases
CHAPTER 44
Pituitary Disorders Patients with acromegaly demonstrate distinctive symp-
toms and signs that relate to the morphologic, biochem-
ical, and metabolic effects of growth hormone excess
(Table 44–1).
SUMMARY OF KEY FEATURES
The osseous manifestations of acromegaly are the result General Clinical Features
of the effects of elevated levels of serum growth hormone
on the adult skeleton. Reactivation of endochondral Typically, the symptoms and signs of acromegaly begin
bone formation and stimulation of periosteal bone in the third or fourth decade of life; however, older and
formation in association with connective tissue younger patients can be affected, and acromegaly (as
proliferation are apparent, leading to characteristic distinct from gigantism) has been recorded in children.
radiographic findings, including increased soft tissue Although the physical appearance of a patient with hyper-
thickness and bony overgrowth. Joint abnormalities pituitary gigantism is essentially that of a tall, normally
result from chondrocyte proliferation in articular proportioned person, the features of a patient with acro-
cartilage. The excessively stimulated cartilage is megaly may be dramatic. Facial characteristics include
vulnerable to fissuring, fragmentation, and ulceration, a large mandible, producing a “lantern jaw” appearance;
which are followed by a brisk reparative response. poor dental occlusion, with separation of the teeth;
These histologic alterations have their radiographic coarsening of facial features related to overgrowth of
counterparts. Initial joint space widening is followed by bone and soft tissue; prominence of the forehead, pro-
joint space narrowing, bony sclerosis, cyst formation, duced by calvarial thickening and enlargement of the
and osteophytosis. The latter radiographic abnormalities frontal sinuses; deepening of the voice; and prominence
simulate those of osteoarthritis. In contradistinction, of the tongue. The hands appear broad and spadelike,
articular abnormalities in hypopituitarism are infrequent; and the fingers are separated and blunted. The entire
the dominant osseous abnormalities related to skeletal frame enlarges as a result of overgrowth of bone. Hyper-
development are manifested by a delay in the appearance secretion of growth hormone also affects other organ
and growth of ossification centers and a similar delay in systems, producing thickening and coarsening of the skin
their fusion and disappearance and enlargement of the kidneys, liver, pancreas, spleen,
thyroid gland, and heart. Associated endocrine altera-
tions may be observed, including diabetes mellitus (12%
to 25%), persistent lactation (4%), increased secretion of
cortisol by the adrenal cortex, and increased frequency of
ACROMEGALY AND GIGANTISM parathyroid and pancreatic islet cell adenomas.
Growth hormone (somatotropin) hypersecretion can be The clinical manifestations of acromegaly may stabilize
associated with acidophilic or chromophobic adenomas after the disease has progressed to a certain degree. This
of the anterior lobe of the pituitary gland. Less frequently, fact, in addition to the insidious nature of the disease, may
such hypersecretion is associated with diffuse hyperplasia lead to considerable delay before an acromegalic patient
of the acidophilic cells or no histologic abnormality at all. seeks medical attention. Headaches and visual distur-
In the immature skeleton, in which the growth plates are bances, which are related to the pressure exerted by the
still open, growth hormone hypersecretion leads to exces- enlarging pituitary gland, may eventually lead the patient
sive proportional growth of bone (i.e., in both length and to a physician.
width). This results from direct hormonal stimulation of Rheumatic complaints are common in patients with
endochondral bone formation at the physeal growth acromegaly. The fundamental morphologic abnormality
plates. The resulting syndrome is termed hyperpituitary
gigantism and is characterized by extreme height. In the
mature skeleton, in which the growth plates have closed TABLE 44–1
and endochondral bone formation has ceased, growth
Rheumatic Complaints in Acromegaly
hormone hypersecretion may reactivate endochondral
bone formation at various existing cartilage-bone junc-
Backache
tions (such as the costochondral junctions) and induce
periosteal bone formation, leading to widening of osseous Limb arthropathy
structures. This overgrowth of bone in association with Compression neuropathy
enlargement of soft tissue is particularly prominent in the Neuromuscular symptoms
acral parts (hands, feet, lower jaw) of the skeleton, lead- Raynaud’s phenomenon
ing to use of the term acromegaly for this condition.
589
590 Section XII Endocrine Diseases
of the ungual tufts. Bone enlargement can be observed Abnormalities of the Foot. Radiographic changes in the
about the wrist as well. foot resemble those in the hand (Fig. 44–4). Thickening
To determine the sesamoid index, the size of the medial of the soft tissues of the toes, enlargement of the sesamoid
sesamoid is measured at the first metacarpophalangeal bones and articular spaces (particularly at the metatarso-
joint. On a nonmagnified radiograph of the hand exposed phalangeal joints), and prominence of the metatarsal
at a 36-inch focus-film distance, the greatest diameter of
this bone is multiplied by the greatest diameter of the
same sesamoid image that is perpendicular to the first
measurement. Although the reliability of this index has
been challenged, a sesamoid index greater than 40 in men
and greater than 32 in women is suggestive of acromegaly,
whereas a sesamoid index below 30 militates against but
does not exclude the diagnosis of acromegaly. Other
measurements used in the diagnosis of acromegaly are
listed in Table 44–3.
TABLE 44–3
Bone and Soft Tissue Measurements Suggestive
of Acromegaly
Differential Diagnosis
The combination of radiographic findings in acromegaly
is sufficiently characteristic that accurate diagnosis is not
difficult, particularly in advanced cases. Individual radio-
graphic signs that are apparent in this disease, such as
increased soft tissue width, tuft prominence, and vertebral
scalloping, may also be noted in other disorders, however.
The early radiographic diagnosis of acromegaly relies on
a variety of measurements, especially in the hands and feet.
Figure 44–7. Acromegaly: cartilage hypertrophy. Joint space Enlargement of Phalangeal Tufts. Widening and promi-
widening is apparent in the elbow. nence of the tufts of the distal phalanges of the hand (and
Chapter 44 Pituitary Disorders 595
TABLE 44–4
Causes of Scalloped Vertebral Bodies
From Mitchell GE, Lourie H, Berne AS: The various causes of scalloped
vertebrae with notes on their pathogenesis. Radiology 89:67, 1967.
TABLE 44–5
Causes of Short Stature
Endocrine Disorders
Hypopituitarism
Hypothyroidism
Diabetes mellitus
Hypercortisolism
Congenital adrenal hyperplasia
Deficient somatomedin production (Laron dwarfism)
Chronic Disorders of Major Organ Systems
Chronic renal disease
Congenital heart disease
Juvenile chronic arthritis
Sickle cell anemia
Malabsorption syndromes
Skeletal Disorders
Achondroplasia
Osteochondrodysplasias
Pseudohypoparathyroidism and pseudo-
pseudo-hypoparathyroidism
Rickets
Chromosomal Aberrations
Gonadal dysgenesis
Trisomy conditions
Miscellaneous Disorders
Malnutrition
Familial short stature
Figure 44–10. Hypopituitarism: delayed skeletal maturation. Inborn errors of metabolism
In this 23-year-old woman, a marked reduction in the rate Intrauterine infections
of skeletal maturation is confirmed by the absence of closure Systemic inflammatory diseases
of the physes of the distal portions of the radius and ulna. Renal tubular disorders
Osteopenia is evident. Psychosocial dwarfism
Neurologic disorders
THYROID ACROPACHY
General Characteristics
Thyroid acropachy is an unusual manifestation of thyroid
disease that may be seen in approximately 0.5% to 1%
of patients with thyrotoxicosis. Although the cause is
unknown, this condition is usually observed after treat-
ment of hyperthyroidism, at which time the patient may
be euthyroid or hypothyroid. Men and women are affected
equally. Clinical findings in thyroid acropachy include
exophthalmos, which may be severe and progressive;
painless symmetrical or asymmetrical soft tissue swelling
of the fingers and toes, which may be erythematous and
fluctuant; pretibial myxedema; and clubbing of the
fingers (Table 45–2).
A B
Figure 45–2. Thyroid acropachy. A, Thick, shaggy periostitis, asymmetrical in distribution, has a
predilection for the radial aspect of the proximal phalanx and metacarpal of the thumb (arrows). B, In a
different patient, observe soft tissue swelling and spiculated or feathery periostitis in the proximal phalanx
of the index finger. There are less extensive changes in the middle finger.
Chapter 45 Thyroid Disorders 599
of the metacarpals, metatarsals, and proximal and middle HYPOTHYROIDISM
phalanges, although this change may be visualized occa-
General Characteristics
sionally at other sites, including the long bones. Perios-
titis is asymmetrical in distribution and more prominent Hypothyroidism and myxedema are terms describing a
on the radial aspect of the bone; it tends to be dense and clinical state of thyroxine and triiodothyronine deficiency.
solid in appearance, with a feathery contour. Soft tissue The deficiency can be divided into a primary form, in
swelling in the hands and feet, phalangeal tufts, and which the thyroid gland itself is involved, and a
anterior tibial region is observed. The osseous abnor- secondary form, characterized by a deficiency in thyroid-
malities generally are not progressive, and correcting stimulating hormone. Hypothyroidism can have many
thyroid function has little or no effect on the acropachy. causes, including atrophy; thyroid gland destruction after
Increased radionuclide activity in areas of bony alteration radioactive iodine therapy or surgery; thyroiditis, which
has been observed. may be acute or chronic (Hashimoto’s disease); infiltra-
tive disorders such as lymphoma, cystinosis, amyloidosis,
and metastasis; deficiency in iodine or iodine metabolism;
Differential Diagnosis use of certain medications; and a variety of pituitary
Thyroid acropachy must be differentiated from other disorders.
disorders associated with periosteal bone formation In infants, thyroid deficiency results in cretinism; in
(Table 45–3). Hypertrophic osteoarthropathy, which is children, it produces juvenile myxedema, with mental
commonly associated with thoracic neoplasm, is charac- retardation and developmental abnormalities. Symptoms
terized by bony proliferation and soft tissue swelling of and signs include lethargy, constipation, enlarged tongue,
the hands and tufts, but the distribution of osseous abdominal distention, hypotonia, dry hair and skin, and
abnormality differs from that of thyroid acropachy. In delayed dentition. In adults, the disease is more frequent
hypertrophic osteoarthropathy, periosteal bone formation in women and can be associated with dry, coarse skin and
is observed most commonly in the tibia, fibula, radius, hair, fatigue, lethargy, edema, hoarseness, constipation,
and ulna. Changes limited to the hands and feet are paresthesias, and bradycardia, as well as other symptoms
unusual in this condition. Further, the feathery pattern and signs (Table 45–4).
of bony proliferation seen in thyroid acropachy is not In adult-onset hypothyroidism, there are altered kinetics
typical of hypertrophic osteoarthropathy. in bone but no diagnostic radiographic abnormalities. In
Pachydermoperiostosis is associated with typical facies, cretinism and juvenile myxedema, skeletal manifestations
soft tissue prominence of the hands, and periostitis. are more marked. Delayed bony maturation is most
Periosteal bone formation generally is apparent in a characteristic, and in infants, absence of the distal femoral
symmetrical pattern in the tibia, fibula, radius, and ulna, and proximal tibial epiphyses is an important radio-
although the hands may also be affected. Periostitis in graphic clue. In older children, abnormal epiphyseal
this disorder is not limited to the diaphysis but may be maturation leads to distinctive radiographic findings,
exuberant at the metaphyseal and epiphyseal areas.
Hypervitaminosis A and venous stasis may produce
periosteal bone proliferation, but clinical and radiographic TABLE 45–4
features allow an accurate diagnosis of these conditions.
Fluorosis is associated with more extensive abnormality Symptoms and Signs of Hypothyroidism
of the axial skeleton and long bones. Leukemic acropachy
is particularly prominent in the terminal phalanges. Percentage of
Periosteal bone formation in acromegaly and vasculitides Cases
such as periarteritis nodosa is readily distinguished from Dry, coarse skin and hair 70–97
the changes of thyroid acropachy. Infectious and traumatic Fatigue, lethargy, mental or physical slowness 70–91
disorders cause alterations in addition to periostitis. Edema; puffy hands, face, or eyes 67–95
Pallor 50–59
Cold intolerance 58–95
TABLE 45–3 Decreased sweating 68–89
Conditions Associated with Periostitis of Multiple Hoarseness 48–74
Bones Constipation 36–61
Weight gain 48–76
Hypertrophic osteoarthropathy Loss or thinning of hair 32–57
Pachydermoperiostosis Paresthesias 56
Thyroid acropachy
Hypervitaminosis A Enlarged tongue 19
Venous stasis Bradycardia 14
Fluorosis Menstrual disturbance 16–30
Leukemia Decreased hearing 6
Vascular insufficiency
Infection
From Robbins J, et al: The thyroid and iodine metabolism. In Bondy PK,
Trauma Rosenberg LE (eds): Metabolic Control and Disease, 8th ed. Philadelphia,
WB Saunders, 1980, p 1390.
600 Section XII Endocrine Diseases
Vertebral abnormalities are also common in hypothy- Figure 45–7. Hypothyroidism: spinal abnormalities. Note
roidism and are particularly prominent at the thora- bullet-shaped vertebrae and beaklike anterior surfaces of the
columbar junction, where short, bullet-shaped 12th vertebral bodies.
thoracic and 1st lumbar vertebral bodies may be visual-
ized (Fig. 45–7). A gibbous deformity may result.
bone sclerosis may be seen in association with soft tissue
calcific deposits. Increased bony eburnation in the peri-
Abnormal Calcification
orbital region is termed the lunette sign. Parotid calci-
In hypothyroidism, abnormal calcium metabolism and fication and premature atherosclerosis are two examples
excretion are present. Retention of calcium with increased of abnormal calcific deposition in hypothyroidism.
Additional Rheumatologic Manifestations Borg SA, Fitzer PM, Young LW: Roentgenologic aspects of
adult cretinism: Two case reports and review of the
An entrapment neuropathy, including the carpal tunnel literature. AJR Am J Roentgenol 123:820, 1975.
syndrome, is seen in approximately 7% of patients with Hernandez RJ, Poznanski AK: Distinctive appearance of the
hypothyroidism. The carpal tunnel syndrome, which is distal phalanges in children with primary hypothyroidism.
produced by nerve impingement by the myxedematous Radiology 132:83, 1979.
tissue and accompanying tenosynovitis, may be persistent, Hernandez RJ, Poznanski AW, Hopwood NJ: Size and skeletal
showing little response to hydrocortisone injections, maturation of the hand in children with hypothyroidism and
although it may improve rapidly after thyroid hormone hypopituitarism. AJR Am J Roentgenol 133:405, 1979.
replacement. Jaffe HL: Metabolic, Degenerative and Inflammatory Diseases
Muscle cramps and stiffness are common in hypothy- of Bones and Joints. Philadelphia, Lea & Febiger, 1972, p 346.
Lauwers A, Alexandre C: Bone manifestations of untreated
roidism, with a predilection for the calves, thighs, and hypothyroidism in adults. Rev Rhum Engl Ed 63:612, 1996.
shoulders. Symptoms and signs include pain and tender- Lintermans JP, Seyhnaeve V: Hypothyroidism and vertebral
ness that increase after exercise. Prominent soft tissues anomalies: A new syndrome? AJR Am J Roentgenol
are due to myxomatous infiltration of soft tissues, and 109:294, 1970.
tendon reflexes are characteristically slow, with delayed McLean RM, Podell DN: Bone and joint manifestations of
relaxation. hypothyroidism. Semin Arthritis Rheum 24:282, 1995.
Meunier PJ, Bianchi GG, Edouard CM, et al: Bony mani-
festations of thyrotoxicosis. Orthop Clin North Am 3:745,
Slipped Capital Femoral Epiphysis 1972.
Slipped capital femoral epiphyses may occur in either Moorefield WG, Urbaniak JR, Ogden WS, et al: Acquired
hypothyroidism and slipped capital femoral epiphysis: Report
treated or untreated patients with hypothyroidism. of three cases. J Bone Joint Surg Am 58:705, 1976.
Although usually seen in prepubescent subjects, the Scanlon GT, Clemett AR: Thyroid acropachy. Radiology
complication is occasionally apparent in more mature 83:1039, 1964.
patients. A bilateral or unilateral distribution is evident Vanhoenacker FM, Pelckmans MC, De Beuckeleer LH, et al:
(Fig. 45–8). The cause of this complication of hypothy- Thyroid acropachy: Correlation of imaging and pathology.
roidism is not known. Whatever the cause, slipped capital Eur Radiol 11:1058, 2001.
femoral epiphyses are an important manifestation of Wietersen FK, Balow RM: The radiologic aspects of thyroid
hypothyroidism and may be the presenting feature of the disease. Radiol Clin North Am 5:255, 1967.
disease.
FURTHER READING
A B C
Figure 46–1. Hyperparathyroidism: subperiosteal bone resorption in the phalanges and phalangeal tufts.
A and B, Phalanges. Mild (A) and severe (B) subperiosteal resorption. Early findings include lacelike bone
resorption, particularly on the radial aspect of the middle phalanges. This pattern may progress to severe
cortical destruction, with loss of definition between cortex and spongiosa. C, Phalangeal tufts. Severe tuftal
resorption and osteolysis of the phalangeal waist (arrows) are seen.
TABLE 46–2
Articular Manifestations of Hyperparathyroidism
Subligamentous and Subtendinous Bone Resorption. Figure 46–8. Hyperparathyroidism: subligamentous and
Osseous resorption occurs at sites of tendon and liga- subtendinous bone resorption in the ischial tuberosity. Erosion
ment attachment to bone (Fig. 46–8). This is particularly of bone has produced a poorly defined, irregular osseous surface.
608 Section XII Endocrine Diseases
Bone Sclerosis
Bone sclerosis is observed far more frequently in patients
with renal osteodystrophy and secondary hyperparathy-
roidism, although it may be apparent in primary hyper-
parathyroidism as well. In patients with primary
hyperparathyroidism, bone sclerosis may be localized or
patchy, apparent in the metaphyseal regions of the long
bones, the skull, or the vertebral endplates. In the spine,
bone deposition in the subchondral areas of the vertebral
bodies results in the appearance of radiodense bands
across the superior and inferior margins (rugger-jersey
spine). This manifestation, too, is much more frequent in
secondary than in primary hyperparathyroidism. Diffuse
bone sclerosis is rarely noted in primary hyperparathy- Figure 46–10. Rheumatic manifestations of hyperparathy-
roidism. The mechanism of bone sclerosis in hyperpara- roidism (and renal osteodystrophy). Findings may be related to
thyroidism has not been clearly defined. subperiosteal resorption at the margins of the joint (1); sub-
chondral resorption leading to cartilage and bone disintegra-
tion and fragmentation (2); subligamentous and subtendinous
Chondrocalcinosis (Calcium Pyrophosphate resorption (3); intra-articular crystal (calcium pyrophosphate
Dihydrate Crystal Deposition) dihydrate, monosodium urate) deposition in cartilage, synovium,
and capsule (4); tendinous and ligamentous injury and rupture
The association of primary hyperparathyroidism and (5); and periarticular crystal (monosodium urate, calcium
calcium pyrophosphate dihydrate (CPPD) crystal deposi- hydroxyapatite) deposition in soft tissues (6).
Chapter 46 Parathyroid Disorders and Renal Osteodystrophy 609
Hyperparathyroidism in Infants and Children of life. Clinical manifestations are variable but include
Primary hyperparathyroidism may be evident in infants those related to renal calculi. Hypercalcemia usually is
and children. Congenital primary hyperparathyroidism is not severe.
a rare disorder that occurs in infants and demonstrates Familial MEN type IIA, also called Sipple’s syndrome,
autosomal recessive inheritance. Symptoms and signs is inherited as an autosomal dominant trait with very high
include hypotonicity, respiratory distress, fever, dehydra- penetrance for medullary thyroid carcinoma in adults.
tion, constipation, anorexia, lethargy, vomiting, dysphagia, Abnormalities of other endocrine glands, particularly
craniotabes, and hepatosplenomegaly. Hypercalcemia is primary parathyroid hyperplasia and pheochromocytoma,
generally but not universally present. Radiographs may occur in approximately one third of cases, but malignant
reveal severe bone disease with extensive bone resorp- change related to these other endocrine aberrations is
tion. The degree of periostitis may be so severe as to rare.
simulate the findings of syphilis. Unless parathyroidec- Familial hypocalciuric hypercalcemia, also called
tomy is performed, the infants usually die. familial benign hypercalcemia, is a rare disorder of
A second disorder, transient hyperparathyroidism of unknown cause characterized by autosomal dominant
the neonate, occurs secondary to hypoparathyroidism in inheritance with high penetrance for expression of
the mother. The radiographic changes are similar to those hypercalcemia at all ages. The syndrome is associated
of congenital primary hyperparathyroidism, although they with lifelong hypercalcemia, low urinary calcium excre-
resolve rapidly. tion, the absence of prominent symptoms and signs, and
In older children, skeletal involvement in hyperpara- the failure of subtotal parathyroidectomy to decrease
thyroidism is characterized by osteopenia, genu valgum, plasma calcium levels. Superficially, familial hypocalciuric
fractures, cystic lesions of bone, and clubbing of the hypercalcemia resembles asymptomatic primary hyper-
fingers. Renal involvement and rickets-like changes with parathyroidism; in the former condition, however, there
metaphyseal irregularity may be observed. is no significant reduction of bone mass in the axial or
appendicular skeleton, and the prevalence of fractures is
not increased.
Familial Hypercalcemia
Specific and distinct syndromes of familial hypercalcemia
Differential Diagnosis
have been identified, including several types of disorders
accompanied by tumors of multiple endocrine glands and Subperiosteal bone resorption is the most helpful diag-
familial hypocalciuric hypercalcemia (Table 46–3). nostic clue of hyperparathyroidism on radiographs.
Familial multiple endocrine neoplasia (MEN) syn- Widespread subperiosteal resorption is confined to
drome type I, which is also referred to as multiple hyperparathyroid bone disease. Focal areas of sub-
endocrine adenomatosis and Wermer’s syndrome, is an periosteal bone resorption may be seen in cases of tumor,
autosomal dominant disease associated with primary infection, and even articular disorders, but the resulting
hyperparathyroidism (95% of cases) and, less frequently, contour defects in these cases are much better defined
with excessive secretion of gastrin (20% to 40% of cases), than the defects in hyperparathyroidism. Intracortical
insulin (2% to 10% of cases), other pancreatic islet bone resorption may be noted in disease states charac-
peptides (rare), and anterior pituitary peptides (rare). terized by rapid bone turnover, such as hyperthyroidism
Nonsecretory neoplastic masses, including lipomas, and acromegaly, whereas endosteal bone resorption can
pituitary chromophobe adenomas (30% to 65% of cases), be produced by osteoporosis or marrow-containing dis-
carcinoid tumors, and adrenal and thyroid adenomas, orders, such as multiple myeloma. The severely depressed
also occur but rarely metastasize. MEN syndrome type and fragmented subchondral bone surface that may
I generally manifests in the third, fourth, or fifth decade accompany hyperparathyroidism can simulate the find-
TABLE 46–3
Major Features in Syndromes of Familial Hypercalcemia
ings of septic arthritis, osteonecrosis, or crystal-induced metabolism of vitamin D, renal diseases can lead to
arthropathy. rickets and osteomalacia.
In the interphalangeal joints of the hand, the findings Renal osteodystrophy is a term applied to the bone
may resemble inflammatory osteoarthritis, psoriasis, or disease that is apparent in patients with chronic renal
even rheumatoid arthritis; in the sacroiliac articulations failure. Osseous abnormalities of renal osteodystrophy
and symphysis pubis, the differential diagnosis includes have been well outlined and can be divided into hyper-
ankylosing spondylitis and other seronegative spondylo- parathyroidism, rickets and osteomalacia, osteoporosis,
arthropathies; in the acromioclavicular and sternoclavicular fractures, soft tissue and vascular calcification, and
joints, hyperparathyroid subchondral bone resorption miscellaneous alterations.
may simulate the changes of rheumatoid arthritis or
ankylosing spondylitis; in the patellofemoral joints, the
appearance is almost identical to that in Wilson’s disease Hyperparathyroidism
and CPPD crystal deposition disease; and at the disco- The abnormalities associated with hyperparathyroidism
vertebral junction, hyperparathyroidism with subchondral may become manifest in patients with renal osteodys-
resorption resembles infection, neuropathic osteoarthrop- trophy, including subperiosteal, intracortical, endosteal,
athy, or degenerative disc disease. Subligamentous bone trabecular, subchondral, and subligamentous and sub-
resorption in hyperparathyroidism may produce a radio- tendinous bone resorption; brown tumors; bone sclerosis;
graphic appearance that simulates the changes seen in and chondrocalcinosis. The frequency of some of these
ankylosing spondylitis and other seronegative spondylo- findings is different in renal osteodystrophy with second-
arthropathies. Localized areas of osseous resorption ary hyperparathyroidism than in primary hyperparathy-
(brown tumors) in hyperparathyroidism resemble a variety roidism (Table 46–4). Modern techniques allow the
of neoplastic or neoplastic-like diseases, particularly giant diagnosis of primary hyperparathyroidism at an early
cell tumor and fibrous dysplasia. stage, when bone abnormalities are not extensive; there-
The rugger-jersey spine is a relatively specific finding fore, many of the osseous changes originally believed to
of secondary hyperparathyroidism, although a similar be more frequent in primary than in secondary hyper-
vertebral sclerosis may occur in Paget’s disease (leading parathyroidism are now found to be more typical of
to the “picture frame” vertebral body), osteopetrosis, the latter.
osteomesopyknosis, and osteoporosis. Diffuse bone The reported frequency of brown tumors in associa-
sclerosis, which is also more frequent in secondary hyper- tion with renal osteodystrophy and secondary hyper-
parathyroidism, is seen in many other conditions, includ- parathyroidism is lower than that in primary hyper-
ing myelofibrosis, fluorosis, mastocytosis, anemia parathyroidism. Osteosclerosis is a well-known feature of
(particularly sickle cell anemia), neoplasm (especially renal osteodystrophy (Fig. 46–11). It predominates in the
metastasis), irradiation, hypoparathyroidism, sarcoidosis, axial skeleton, with involvement of the pelvis, ribs, and
and Paget’s disease. superior and inferior portions of the vertebral bodies
The CPPD crystal deposition in primary and (less (rugger-jersey spine), although the appendicular skeleton
commonly) secondary hyperparathyroidism is identical (Fig. 46–12) may also be involved, particularly the
to that occurring in idiopathic CPPD crystal deposition metaphyseal regions of long bones. Chondrocalcinosis
disease and hemochromatosis. Any patient with chondro- due to CPPD crystal deposition is much more frequent
calcinosis should be evaluated for the presence of hyper- in primary hyperparathyroidism than in renal osteo-
parathyroidism. Monosodium urate crystal deposition in dystrophy.
hyperparathyroidism leads to secondary gout, which Periosteal neostosis is a term applied to periosteal
resembles primary gout. bone formation in patients with renal osteodystrophy
In infants and children, periostitis and extensive bone (Fig. 46–13). Its frequency is estimated to be 8% to 25%
resorption in hyperparathyroidism may simulate the find- in such patients, and it is more frequent in those with
ings of syphilis or leukemia, whereas metaphyseal destruc- severe skeletal abnormalities. Periosteal neostosis is
tion in the long bones in hyperparathyroidism produces
a radiographic picture identical to that of rickets.
TABLE 46–4
A B
observed most commonly in the metatarsals, femur, and Looser’s zones are most common in the pubic rami, ilia,
pelvis, particularly the pubic rami, although it may be ribs, femoral necks, scapulae, and long bones and are
visualized elsewhere. observed in 1% of patients with chronic renal disease.
Radiographic features consistent with rickets include
osteopenia, irregularity and widening of the growth plate,
Rickets and Osteomalacia
and poor definition of the epiphysis (Fig. 46–15).
The cause of the osteomalacia seen in chronic renal Slipped epiphyses have been described in approxi-
disease before dialysis treatment is probably complex. mately 10% of children with chronic renal disease. The
The kidney is essential in the further hydroxylation of capital femoral epiphysis is the most common site of
25-hydroxycholecalciferol to 1,25-dihydroxycholecalciferol, involvement by far, although similar changes may be seen
which is an active metabolite of vitamin D, and the at other skeletal locations, including the proximal end of
absence of this metabolite in patients with chronic renal the humerus; the distal portion of the radius, ulna, or
disease can lead to abnormality of bone. Further, patients femur; and, rarely, the small bones of the hands and feet.
with renal failure have malabsorption of calcium from Bilateral involvement is frequent. Three radiographic
the gut, which can also contribute to bone disease. signs may precede slipping of the capital femoral epi-
Osteomalacia may also be related to vitamin D deficiency physis in renal osteodystrophy: bilateral subperiosteal
as well as resistance, with the deficiency occurring as a erosion on the medial aspect of the femoral neck,
result of anorexia and inadequate diet, the increased increase in width of the cartilaginous growth plate, and
requirement of hyperparathyroid bone, and abnormality bilateral coxa vara (see Fig. 46–15).
of the hepatic enzyme system.
The radiographic features of osteomalacia in chronic
renal disease include osteopenia, bone deformities, and Osteoporosis
Looser’s zones. The latter abnormalities are narrow A diminution of bone volume occurs in renal osteodys-
radiolucent bands, frequently symmetrical and oriented trophy. On radiographs, decreased bone density, or
perpendicular to the osseous surface (Fig. 46–14). osteopenia, is evident, although the diminished radio-
612 Section XII Endocrine Diseases
Fractures
Pathologic fractures are a recognized complication of
renal osteodystrophy. Potential causes of such fractures
include hyperparathyroidism itself, osteomalacia, osteo-
Figure 46–14. Renal osteodystrophy: osteomalacia. Observe
porosis, and aluminum intoxication. In the spine, the bony deformities of the pelvis. Acetabular protrusion, coxa
vertebral compression in association with a rugger-jersey vara, and bowing of the radius, ulna, tibia, and fibula are seen.
appearance is commonly encountered. Fractures in Trabecular detail is virtually absent.
regions of brown tumors are also characteristic. Insuffi-
ciency fractures, which in some cases represent Looser’s
zones of osteomalacia, are seen in patients with renal Soft Tissue and Vascular Calcification
osteodystrophy. The medial aspect of the femoral neck is Although calcification may be observed in the soft tissues
an important and common site of such insufficiency and vessels of patients with primary hyperparathy-
fractures, with bilateral involvement reported in as many roidism, it is much more frequent in patients with renal
as 50% of patients. osteodystrophy. In patients with chronic renal failure,
Chapter 46 Parathyroid Disorders and Renal Osteodystrophy 613
soft tissue calcification occurs when multiplication of the Figure 46–16. Renal osteodystrophy: soft tissue calcification.
Extensive “tumoral” calcification is apparent in the periarticular
respective concentrations (in mg/dL) of plasma calcium regions of the elbow.
and plasma phosphorus produces a value greater than 70.
Soft tissue deposits can occur in multiple sites, including
corneal and conjunctival tissue, viscera, vasculature, and the bone changes of renal osteodystrophy resolve, provid-
subcutaneous and periarticular tissue (Fig. 46–16). The ed that the hemodialysis is of adequate quality and duration
deposits may occur in patients with chronic uremia who (Table 46–5; Fig. 46–17). In poorly managed patients on
have not been treated, although they appear to be more dialysis, increasing osteopenia may be observed in
frequent and extensive in uremic patients who have association with spontaneous fractures. These fractures
undergone hemodialysis or renal transplantation. Peri- are most frequent in the ribs, although they may occur at
articular deposits may reach considerable size and pro- other sites, including the femoral necks, vertebrae, pubic
duce striking patchy or tumoral radiodense areas, rami, tibias, and metatarsals.
particularly in the hips, knees, shoulders, and wrists.
Bilateral symmetrical deposits about multiple joints are Aluminum Intoxication. It is generally believed that the
not uncommon. primary cause of the progression of skeletal abnormal-
Vascular collections involve not only the heart and ities in patients on chronic hemodialysis is osteomalacia
great vessels but also the peripheral arteries, especially due to aluminum intoxication (Fig. 46–18). Clinical
the dorsalis pedis artery of the foot. characteristics of this syndrome include bone pain,
myopathy, fracture, and dialysis encephalopathy accom-
panied by dementia, speech disorders, seizures, and
Miscellaneous Abnormalities dyspraxia. Fractures of the ribs and proximal portion of
Patients with chronic renal insufficiency have hyper- the femora are characteristic, although the spine and
uricemia and may develop secondary gout. Radiographic other tubular bones are also affected. High aluminum
features resemble those of primary gout, although in levels in tissues, including the brain and bone as well as
secondary gout, atypical articular sites may be affected. various articular structures, support the speculation that
Oxalosis of bone may develop as a rare secondary aluminum toxicity is involved in this syndrome. The
manifestation of chronic renal failure. source of the aluminum is related to the contents of
phosphate-binding gels or the ambient water or dialysate.
Aluminum-induced changes, however, have been docu-
Musculoskeletal Abnormalities after mented in patients with chronic renal disease who have
Hemodialysis not undergone dialysis, and in such cases, considerable
In the vast majority of patients with chronic renal failure evidence exists that aluminum is absorbed from the orally
who are placed on maintenance hemodialysis, many of administered aluminum-containing phosphate-binding
614 Section XII Endocrine Diseases
A B
Figure 46–17. Renal osteodystrophy: resolution of skeletal abnormalities during hemodialysis. In this
38-year-old man on maintenance hemodialysis, radiographs obtained at presentation (A) and after 4 years
(B) show significant improvement in osseous abnormalities following treatment.
A B
medications prescribed for patients with renal failure. Progression of such calcification, however, is a more
Reversal of the findings follows removal of the source of typical finding.
aluminum contamination.
Musculoskeletal Infection. Septicemia, osteomyelitis, and
Soft Tissue and Vascular Calcification. Soft tissue and septic arthritis are well-recognized complications of
vascular calcification is frequent in patients undergoing hemodialysis. External or internal arteriovenous fistulas
hemodialysis. Reversal of periarticular and, less com- provide an ideal site of entry for infectious organisms. In
monly, vascular calcification may be seen during dialysis. addition, impaired host resistance related to the presence
Chapter 46 Parathyroid Disorders and Renal Osteodystrophy 615
of a chronic debilitating illness and the use of immuno- spinal abnormalities has been debated, accumulating data
suppressive agents and adrenal steroids increase the risk underscore the importance of amyloid deposition in
of infection in these persons. Osteomyelitis and septic regions of spinal destruction. Amyloid masses, composed
arthritis may occur at any site. of beta2-microglobulins, have been documented repeat-
edly, not only in the vertebral column but also in other
Osteonecrosis. Ischemic necrosis of bone may complicate skeletal and extraskeletal sites in patients undergoing
the administration of steroids in patients with chronic chronic hemodialysis.
renal disease undergoing dialysis. This same complica-
tion is well recognized after renal transplantation. Carpal Tunnel Syndrome. Carpal tunnel syndrome is well
Affected sites include the femoral and humeral heads and recognized in patients receiving hemodialysis and is
the talus. usually attributed to alterations in vascular hemodynamics
at the access site, resulting in edema, venous distention,
Destructive Spondyloarthropathy. A peculiar pattern of and secondary compression of the median nerve within
spondyloarthropathy has been identified in 5% to 23% the carpal canal. The rate of occurrence of these abnor-
of patients with chronic renal disease who are under- malities is directly related to the length of dialysis.
going hemodialysis. Middle-aged or elderly patients who Currently, amyloid is also considered an important cause
have been hemodialyzed for several years are affected; it of carpal tunnel syndrome in hemodialyzed patients.
is extremely rare in persons whose dialysis treatment has Amyloid accumulation also occurs in the accompanying
been ongoing for less than 2 or 3 years. The most com- small cystic lesions in the carpal bones (Fig. 46–20).
mon site of involvement is the cervical spine (approxi-
mately 70% of cases), followed by the lumbar spine Amyloid Deposition. Amyloid accumulation in osseous and
(20%) and thoracic spine (10%). Single or multiple spinal articular structures is a prominent feature of hemodialysis
levels reveal rapidly progressive radiographic abnormal- in patients with chronic renal disease. Periarticular soft
ities characterized by loss of intervertebral disc space, tissue prominence, intraosseous and subchondral bone
erosion of subchondral bone in the neighboring vertebral destruction, pathologic fracture, and carpal tunnel syn-
bodies, and new bone formation (Fig. 46–19). The find- drome are characteristic clinical and radiographic fea-
ings resemble infection, neuropathic osteoarthropathy, tures of amyloid accumulation. The variety of amyloid
severe intervertebral (osteo)chondrosis, or CPPD crystal in this clinical setting is a beta2-microglobulin. Such
deposition disease. Although the precise cause of the amyloid deposition must be considered one important
factor, in addition to hyperparathyroidism, aluminum
intoxication, and crystalline and iron accumulation, in
the pathogenesis of the musculoskeletal manifestations
of hemodialysis.
The accumulation of beta2-microglobulin in patients soft tissue changes are less frequent and less severe with
undergoing hemodialysis appears to affect predominantly this therapeutic technique than with hemodialysis, but
the musculoskeletal system, and the prevalence of such others have not found this to be true. Deposition of
amyloid deposition increases progressively with the dura- beta2-microglobulin amyloid has been documented in
tion of dialysis therapy. Amyloid deposition may lead to patients with chronic renal disease receiving long-term
arthralgias and arthropathies, especially in the shoulder, peritoneal dialysis. Although there are theoretical reasons
but also in the hip, wrist, and other joints; amyloidomas why this form of dialysis might lead to such deposition
in periarticular bone may produce radiolucent lesions of less frequently than hemodialysis does, the known occur-
variable size that may fracture, particularly in the femoral rence of arthralgias, destructive arthropathy and spondylo-
neck and vertebra, but in additional sites as well; amyloid arthropathy, intraosseous lytic lesions, and carpal tunnel
accumulation in soft tissues may produce neurovascular syndrome in association with chronic peritoneal dialysis
compromise, most evident about the wrist; and masses appears to underscore the importance of amyloidosis as a
of amyloid may extend from affected vertebral bodies and complication of this form of therapy as well.
intervertebral discs into the spinal canal, leading to signif-
icant neurologic compromise. Amyloid deposition in soft
Musculoskeletal Abnormalities after
tissue, joint capsule, and synovium may lead to prominent
Renal Transplantation
periarticular masses about the shoulder, sternoclavicular
joint, knee, hip, wrist, and other sites (Fig. 46–21). After transplantation, normal renal function is restored;
Magnetic resonance (MR) imaging has been used to the nature and progress of bone disease depend on the
investigate dialysis-related arthropathy and generally reveals state of the bones at the time of transplantation. The
that subchondral intraosseous collections of amyloid occurrence of osteonecrosis after renal transplantation is
communicate with the articular surface. Reported signal well known (Fig. 46-22). Osteonecrosis generally becomes
characteristics of dialysis-related arthropathy are not uni- evident 4 to 36 months after surgery. It is generally
form; however, regions of low signal intensity correspon- assumed that osteonecrosis in the post-transplant period
ding to sites of amyloid accumulation on both T1- and is due to steroid medications; the reported frequency
T2-weighted spin echo MR images are most typical. is 1.4% to 40%. The occurrence of clinically occult
ischemic bone necrosis, detected only by MR imaging, in
renal transplant patients makes analysis of the prevalence
Musculoskeletal Abnormalities after of osteonecrosis difficult. The most common site of
Peritoneal Dialysis
osteonecrosis after renal transplantation is the femoral
Bone disease and soft tissue calcification have also been head; additional sites are the distal end of the femur,
observed in uremic patients undergoing peritoneal humeral head, talus, humeral condyles, cuboid, and
dialysis. Some investigators suggest that the osseous and carpal bones.
Radiographic manifestations of osteonecrosis are
characteristic but are seen late in the disease process. A
linear subchondral radiolucent shadow represents a frac-
ture beneath the cartilaginous surface. Focal areas of
sclerosis are observed about this lucent area, as well as
TABLE 46–5
Musculoskeletal Abnormalities Following Dialysis
and Renal Transplantation
Hyperparathyroidism
Osteomalacia and rickets
Osteosclerosis
Fractures
Soft tissue and vascular calcification
Osteomyelitis and septic arthritis
Osteonecrosis
Crystal deposition
Destructive spondyloarthropathy
Amyloidosis
Carpal tunnel syndrome
Figure 46–21. Renal osteodystrophy: amyloid deposition Digital clubbing
during hemodialysis leading to pathologic fracture. Note the Aluminium toxicity
displaced fracture of the femoral neck, with osteolytic
destruction of the femoral head and neck. Additional Dialysis cysts
destructive lesions of the acetabulum and pubic ramus are Olecranon bursitis
present. (Courtesy of A. Newberg, MD, Boston, Mass.)
Chapter 46 Parathyroid Disorders and Renal Osteodystrophy 617
identical to those accompanying rickets related to dietary
deficiencies and chronic renal tubular disorders. The
presence of slipped epiphyses and genu valgum defor-
mities may be more common in the rickets of renal
osteodystrophy.
Periarticular calcification is found not only in renal
osteodystrophy but also in various collagen vascular
diseases, hypervitaminosis D, milk-alkali syndrome, idio-
pathic tumoral calcinosis, and idiopathic calcium hy-
droxyapatite crystal deposition disease. The periarticular,
soft tissue, and vascular calcifications of renal osteodys-
trophy are usually accompanied by other radiographic
changes of the disorder.
HYPOPARATHYROIDISM
Background and General Features
Figure 46–22. Renal osteodystrophy: complications of renal
transplantation. The radiographic features of osteonecrosis are Hypoparathyroidism is characterized by hypocalcemia
characteristic, including patchy radiolucency and sclerosis and and its neuromuscular symptoms and signs. The disease
collapse of the femoral head. may result from a deficiency in parathyroid hormone
production or an end-organ resistance to the action of
the hormone. Deficiency of parathyroid hormone most
bony eburnation. Spontaneous fractures are not infre- commonly results from excision of or trauma to the
quent after renal transplantation and have been observed parathyroid glands during thyroid surgery, although it
in 5% to 25% of patients. Although the cause is not can also occur as idiopathic hypoparathyroidism, in
known, a reduction in bone mass clearly contributes to which the parathyroid glands are usually absent or
spontaneous complete fractures, as well as insufficiency- atrophied. End-organ unresponsiveness to parathyroid
type stress fractures. Bone and joint infection may also hormone is seen in pseudohypoparathyroidism and
be noted. pseudo-pseudohypoparathyroidism.
Tendinosis and spontaneous tendon ruptures have been Postsurgical hypoparathyroidism occurs in less than
identified in some patients who have had renal trans- 13% of thyroidectomies, may be unrecognized for years,
plants. An increased rate of malignant change may be and becomes evident clinically during pregnancy and
evident in patients with renal transplants. Possible lactation. Idiopathic hypoparathyroidism usually occurs
explanations for this increased risk of neoplasm are the in childhood, with girls being affected more commonly
presence of an underlying autoimmune renal disease, than boys. The disease is rare in blacks. A familial occur-
transplantation of malignancy, and immunosuppressive rence is noted. The disorder may be characterized by the
therapy. presence of circulating antibodies to the parathyroid,
adrenal, and thyroid glands, supporting the concept that
idiopathic hypoparathyroidism may be part of a gener-
Differential Diagnosis
alized autoimmune disease.
In renal osteodystrophy, the radiographic features of General radiographic abnormalities of this condition
hyperparathyroidism must be distinguished from those include thickening of the cranial vault and facial bones;
accompanying primary hyperparathyroidism. In second- increased intracranial pressure with sutural diastasis;
ary hyperparathyroidism, diagnostic features include an calcification of the basal ganglia and, rarely, the choroid
increased frequency of vascular and soft tissue calcifi- plexus and cerebellum; ventricular dilatation; dental
cation, more common and widespread bone sclerosis, and abnormalities such as hypoplasia of enamel and dentin,
a decreased frequency of chondrocalcinosis. Diffuse delay or failure of eruption, blunting of the roots, and
osteosclerosis in this condition may be particularly promi- thickening of the lamina dura; and gastrointestinal
nent and, although usually accompanied by other hypersecretion and spasm.
radiographic changes of renal osteodystrophy, must be
differentiated from the increased radiodensity associated
with various other endocrine, metabolic, and neoplastic Skeletal Abnormalities
diseases. Periosteal neostosis in renal osteodystrophy Osteosclerosis, which may be generalized or localized, is
produces diffuse periosteal bone formation, a finding the most common skeletal abnormality of hypoparathy-
that can also occur with hypertrophic osteoarthropathy, roidism (Table 46–6). Localized osteosclerosis is more
neoplasm, and infection. common, reported in 23% of patients; generalized
Osteomalacia in chronic renal disease produces osteosclerosis is seen in 9%. In addition to osteosclerosis,
decreased radiodensity of bone, a finding that lacks typical radiographic findings of hypoparathyroidism (and
specificity. Occasionally, Looser’s zones appear identical pseudohypoparathyroidism) include calvarial thickening
to those accompanying other types of osteomalacia. and hypoplastic dentition (Fig. 46–23). Also noteworthy
Rachitic changes of renal osteodystrophy are almost are peculiar bandlike areas of increased radiodensity in
618 Section XII Endocrine Diseases
TABLE 46–6
Radiographic Features of the Skeleton in
Hypoparathyroidism
Osteosclerosis
Calvarial thickening
Hypoplastic dentition
Subcutaneous calcification
Basal ganglion calcification
Premature physeal fusion
Spinal ossification
A B
Chapter 46 Parathyroid Disorders and Renal Osteodystrophy 619
Differential Diagnosis teristic somatotype, which includes short stature, obesity,
Widespread osteosclerosis, particularly of the axial round face, and brachydactyly. Additional clinical find-
skeleton, may be identified in hypoparathyroidism, but it ings of this disease are abnormal dentition, mental retar-
is also seen in certain other disorders, including osteo- dation, strabismus, dermatoglyphic abnormalities, and
blastic metastasis, myelofibrosis, Paget’s disease, fluorosis, impaired taste and olfaction. Typical radiographic
renal osteodystrophy, sickle cell anemia, and mastocy- features of PHP are short metacarpals, metatarsals, and
tosis. In these diseases, other radiographic features are phalanges; exostoses; cone epiphyses; and wide bones. In
usually apparent, allowing an accurate diagnosis. In some cases, changes of secondary hyperparathyroidism
hypoparathyroidism (and pseudohypoparathyroidism), are seen.
additional findings such as calvarial thickening and hypo- PHP is more frequent in women than in men and
plastic dentition are helpful clues, although other causes appears to be transmitted as an X-linked dominant trait.
of generalized sclerosis, such as Paget’s disease, sickle cell PHP is usually diagnosed in the second decade of life.
anemia, and even metastasis, may produce increased Affected persons reveal increased levels of serum phos-
thickness and sclerosis of the cranial vault. Hypoplastic phorus, decreased levels of serum calcium, and dimin-
dentition is also seen in a variety of congenital syn- ished phosphaturia. In addition, because of similarities in
dromes, including cleidocranial dysostosis and pyknodys- somatotypic findings, some investigators suggest that
ostosis, and in a number of other endocrine disorders, PHP may be related to other hereditary syndromes, such
such as hypopituitarism and hypothyroidism. Sclerosis of as multiple epiphyseal dysplasia, basal cell nevus syn-
the metaphyseal region of the long bones, which is seen drome, and Turner’s syndrome.
in some patients with hypoparathyroidism, is not specific. PHP results from end organs’ inability to respond to
A similar finding may be noted in systemic illnesses in parathyroid hormone rather than from deficient secre-
infancy and childhood, leading to growth recovery lines, tion of the hormone or the presence of a biologically
leukemia during treatment, heavy metal poisoning, ineffective hormone. Excessive secretion of parathyroid
hypothyroidism, healing scurvy, and hypervitaminoses, hormone has been demonstrated in patients with PHP. In
although additional radiographic and clinical features fact, reports indicate an apparently rare variation of PHP
usually allow easy differentiation among these disorders. characterized by renal unresponsiveness to parathyroid
Basal ganglion calcification is particularly characteristic hormone but a normal osseous response to the hormone.
of hypoparathyroidism and pseudohypoparathyroidism. This condition, termed pseudohypohyperparathyroidism,
It is also seen in infectious disorders such as toxoplas- is associated with histologic findings similar to those of
mosis and cytomegalic inclusion disease, in Fahr’s syn- renal osteodystrophy. Abnormal somatic features may
drome (ferrocalcinosis), after radiation therapy and or may not be present. Radiographic abnormalities are
exposure to toxic substances such as carbon monoxide, those of hyperparathyroidism, including subperiosteal
and rarely in certain other diseases. Also, subcutaneous bone resorption, brown tumors, osteosclerosis, periosteal
calcification, seen in hypoparathyroidism and pseudo- neostosis, and slipped capital femoral epiphyses.
hypoparathyroidism, is not a specific finding, being Pseudo-pseudohypoparathyroidism (PPHP) is the
observed in collagen vascular disease, hypervitaminosis normocalcemic form of PHP. Patients with PPHP possess
D, milk-alkali syndrome, and renal osteodystrophy. the same somatic abnormalities as those with PHP. Both
The “pseudospondylitic” manifestations of hypopara- PHP and PPHP may occur in the same family, suggest-
thyroidism are of particular interest. Descriptions of ing a close association of these disorders. Both conditions
these manifestations bear a striking resemblance to those have been grouped under the term Albright’s hereditary
of diffuse idiopathic skeletal hyperostosis, including the osteodystrophy. Patients with PHP frequently come to
tendency toward spinal and extraspinal ligament ossifi- medical attention with tetany and occasionally with con-
cation, osteophytosis, and bony excrescences at sites of vulsions in late childhood or adolescence. Additionally,
tendon and ligament attachment to bone. Similar spinal they reveal hyperexcitability, cramping of the extremities,
alterations may be seen in fluorosis and ankylosing and stridor.
spondylitis, although, unlike in the latter disorder, thin
vertical syndesmophytes, erosion, and intra-articular Skeletal Abnormalities
ankylosis of the sacroiliac joints are not characteristic of
hypoparathyroidism. Skeletal abnormalities are an integral part of PHP and
PPHP (Table 46–7). Radiographic findings include
shortening of all the metacarpals, with premature fusion
PSEUDOHYPOPARATHYROIDISM AND of the growth plates; broad and short phalanges, with
PSEUDO-PSEUDOHYPOPARATHYROIDISM pseudoepiphyses; and soft tissue calcification. The calci-
fication is plaquelike in appearance, asymmetrically
Background and General Features distributed, and located beneath the skin surface (Fig.
Pseudohypoparathyroidism (PHP) is a heritable disorder 46–25). Metacarpal shortening shows a predilection for
that shares many features with idiopathic hypoparathy- the first and fourth digits (Fig. 46–26), and it is rare to
roidism, including hypocalcemia, hyperphosphatemia, observe metatarsal shortening as an isolated skeletal
and basal ganglion and soft tissue calcification. PHP abnormality. The first metacarpal bone may reveal exces-
differs from idiopathic hypoparathyroidism in several sive width and curvature, and the phalanges are short and
respects; it involves end-organ resistance to the action of wide, with cone-shaped epiphyses. Additional findings
parathyroid hormone and is associated with a charac- include basal ganglion calcification, calvarial thickening,
620 Section XII Endocrine Diseases
TABLE 46–7
Radiographic Features of the Skeleton in PHP and PPHP
A B
Figure 46–26. Pseudohypoparathyroidism: metacarpal and metatarsal shortening. A, Shortening of all the
metacarpal bones is evident, although the most severe abnormality is in the third digit. Note irregularity of the
distal end of the ulna and carpal bones, with joint space narrowing and sclerosis. B, Shortening of all the metatarsal
bones, particularly the fourth, is associated with soft tissue ossification on the medial aspect of the first digit.
Chapter 46 Parathyroid Disorders and Renal Osteodystrophy 621
FURTHER READING
Bywaters EGL, Dixon ASJ, Scott JT: Joint lesions of hyper-
parathyroidism. Ann Rheum Dis 22:171, 1963.
Cardinal E, Buckwalter KA, Braunstein EM, et al: Amyloidosis
of the shoulder in patients on chronic hemodialysis:
Sonographic findings. AJR Am J Roentgenol 166:153, 1996.
Chary-Valckenaere I, Kessler M, Mainard D, et al: Amyloid and
non-amyloid carpal tunnel syndrome in patients receiving
chronic renal dialysis. J Rheumatol 25:1164, 1998.
Dodds WJ, Steinbach HL: Primary hyperparathyroidism and
articular cartilage calcification. AJR Am J Roentgenol
104:884, 1968.
Doppman JL: Multiple endocrine syndromes—a nightmare
for the endocrinologic radiologist. Semin Roentgenol 20:7,
1985.
Elmstedt E: Avascular bone necrosis in the renal transplant
patient: A discriminant analysis of 144 cases. Clin Orthop
158:149, 1981.
Falbo SE, Sundaram M, Ballal S, et al: Clinical significance
of erosive azotemic osteodystrophy: A prospective masked
study. Skeletal Radiol 28:86, 1999.
Fenves AZ, Emmett M, White MG, et al: Carpal tunnel syn-
drome with cystic bone lesions secondary to amyloidosis in
chronic hemodialysis patients. Am J Kidney Dis 7:130, 1986.
Flipo RM, Cotton A, Chastanet P, et al: Evaluation of destruc-
tive spondyloarthropathies in hemodialysis by computerized
tomographic scan and magnetic resonance imaging. J
Rheumatol 23:869, 1996.
Franco M, Van Elslande L, Passeron C, et al: Tumoral calci-
A B nosis in hemodialysis patients: A review of three cases. Rev
Figure 46–27. Pseudohypoparathyroidism and pseudo- Rhum Engl Ed 64:59, 1997.
pseudohypoparathyroidism: positive metacarpal sign. Normally, Garrett P, McWade M, O’Callaghan J: Radiological assessment
a line drawn tangential to the heads of the fourth and fifth of aluminum-related bone disease. Clin Radiol 37:63, 1986.
metacarpal bones does not intersect the end of the third meta- Genant HK, Baron JM, Strauss FH II, et al: Osteosclerosis in
carpal bone or just contacts its articular surface (A). A positive primary hyperparathyroidism. Am J Med 59:104, 1975.
metacarpal sign is present when such a line intersects the third Genant HK, Heck LL, Lanzl LH, et al: Primary hyperpara-
metacarpal bone (B). thyroidism: A comprehensive study of clinical, biochemical,
and radiographic manifestations. Radiology 109:513, 1973.
Goldman AB, Lane JM, Salvati E: Slipped capital femoral
epiphyses complicating renal osteodystrophy: A report of
three cases. Radiology 126:333, 1978.
Greenfield GB: Roentgen appearance of bone and soft tissue
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dysplasia, and Beckwith-Wiedemann syndrome, as well 116:749, 1972.
as in acquired conditions, such as juvenile chronic Griffiths HJ, Ennis JT, Bailey G: Skeletal changes following
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neonatal hyperthyroidism. Further, this sign may be Kaplan P, Resnick D, Murphey M, et al: Destructive non-
unreliable in diagnosing PHP and PPHP because the infectious spondyloarthropathy in hemodialysis patients: A
third metacarpal bone may also be short in these condi- report of four cases. Radiology 162:241, 1986.
Kriegshauser JS, Swee RG, McCarthy JT, et al: Aluminum
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be seen in some patients with PHP and PPHP. ings and prediction of bone biopsy results. Radiology
164:399, 1987.
Differential Diagnosis Kuntz D, Naveau B, Bardin T, et al: Destructive spondylo-
arthropathy in hemodialyzed patients: A new syndrome.
Radiographic abnormalities of the hand in PHP and Arthritis Rheum 27:369, 1984.
PPHP are almost identical. These hand abnormalities Mankin HJ: Metabolic bone disease. J Bone Joint Surg Am
resemble the findings in acrodysostosis (except for the 76:760, 1994.
much smaller size of the bones in the latter condition), Meema HE, Oreopoulos DG, Rabinovich S, et al: Periosteal
Turner’s syndrome (except that the changes in Turner’s new bone formation (periosteal neostosis) in renal osteo-
dystrophy: Relationship to osteosclerosis, osteitis fibrosa,
syndrome may be less severe than those in PHP and and osteoid excess. Radiology 110:513, 1974.
PPHP, and drumstick phalanges and thin bones may Milgram JW, Salyer WR: Secondary oxalosis of bone in chronic
be apparent in Turner’s syndrome), and brachydactyly renal failure. J Bone Joint Surg Am 56:387, 1974.
E and D. Some radiographic features of PHP and PPHP Orzincolo C, Bedani PL, Scutellari PN, et al: Destructive
resemble the findings of myositis (fibrodysplasia) ossifi- spondyloarthropathy and radiographic follow-up in hemo-
cans progressiva and multiple hereditary exostoses. dialysis patients. Skeletal Radiol 19:483, 1990.
622 Section XII Endocrine Diseases
Poznanski AK, Werder EA, Giedion A, et al: The pattern of Steinbach HL, Rudhe U, Jonsson M, et al: Evolution of skeletal
shortening of the bones of the hand in PHP and PPHP—a lesions in pseudohypoparathyroidism. Radiology 85:670,
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Pugh DG: Subperiosteal resorption of bone: A roentgenologic hypoparathyroidism (PH) and pseudo-pseudohypoparathy-
manifestation of primary hyperparathyroidism and renal roidism (PPH): Differentiation from other syndromes
osteodystrophy. AJR Am J Roentgenol 66:577, 1951. associated with short metacarpals, metatarsals and phalanges.
Resnick D: Abnormalities of bone and soft tissue following AJR Am J Roentgenol 97:49, 1966.
renal transplantation. Semin Roentgenol 13:329, 1978. Steinberg H, Waldron BR: Idiopathic hypoparathyroidism:
Resnick D, Niwayama G: Subchondral resorption of bone in Analysis of 52 cases, including report of new case. Medicine
renal osteodystrophy. Radiology 118:315, 1976. 31:133, 1952.
Ross LV, Ross GJ, Mesgarzadeh M, et al: Hemodialysis-related Sundaram M, Phillipp SR, Wolverson MK, et al: Ungual tufts
amyloidomas of bone. Radiology 178:263, 1991. in the follow-up of patients on maintenance dialysis. Skeletal
Slavotinek JP, Coates PTH, McDonald SP, et al: Shoulder Radiol 5:247, 1980.
appearances at MR imaging in long-term dialysis recipients. Tervonen O, Mueller DM, Matteson EL, et al: Clinically occult
Radiology 217:539, 2000. avascular necrosis of the hip: Prevalence in an asymptomatic
Steinbach HL, Gordan GS, Eisenberg E, et al: Primary hyper- population at risk. Radiology 182:845, 1992.
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47
CHAPTER 47
Disorders of Other Endocrine strual abnormalities may herald the onset of the disease.
Generalized obesity, muscle weakness, emotional distur-
Glands and of Pregnancy bance, and backache are common symptoms. On physical
examination, patients may demonstrate moon face
(increased fullness of the face and cheeks), buffalo hump
(fatty deposition over the dorsal spine), increased trans-
SUMMARY OF KEY FEATURES parency of the skin, abdominal and axillary purple striae,
Various musculoskeletal manifestations may accompany abnormal distribution of hair with hirsutism, hyper-
endocrine disorders. Further, certain complications are tension, and bone tenderness. In children, growth arrest
apparent in women during and after pregnancy. and short stature are observed.
In Cushing’s disease, osteoporosis is most typical, and
although osteonecrosis is seen occasionally, it is not as
Osteoporosis
frequent as in cases of exogenous hypercortisolism.
The list of disorders that may be associated with Reduced bone substance in Cushing’s syndrome is
diabetes mellitus includes crystal-induced arthropathy frequently severe, reflecting decreased bony deposition
(gout and calcium pyrophosphate dihydrate crystal associated with increased bony resorption. Radiographic
deposition disease), osteoarthritis, diffuse idiopathic examination of patients with Cushing’s syndrome reveals
skeletal hyperostosis, soft tissue contractures, typical findings of osteoporosis. The changes predomi-
osteomyelitis, septic arthritis, and neuropathic nate in the spine, where diminished bone density, bicon-
osteoarthropathy. Musculoskeletal anomalies are cave deformities of vertebral bodies (fish vertebrae),
identified in some infants born to diabetic mothers. compression fractures, and exaggerated kyphosis are
After a normal pregnancy, osteitis condensans ilii and apparent (Fig. 47–1). The appearance is not unlike that in
osteitis pubis may be apparent, and a similar-appearing other disorders associated with osteoporosis, although
abnormality of the clavicle has been described.
Other conditions associated with pregnancy include
osteonecrosis, transient osteoporosis of the hip,
disseminated intravascular coagulation, symphyseal
diastasis and rupture, and stress fractures.
INTRODUCTION
Several endocrine disorders, other than those covered in
the preceding chapters, may be associated with significant
abnormalities of the skeleton. In addition, musculoskeletal
manifestations accompanying or occurring after preg-
nancy and one miscellaneous disease, osteitis condensans
of the clavicle, are discussed.
CUSHING’S DISEASE
General Features
Cushing’s syndrome is caused by the presence of exces-
sive amounts of adrenocortical glucocorticoid steroids in
the body. This excess may be induced by hyperplasia or
hyperfunctioning tumors of the adrenal cortex (endoge-
nous Cushing’s disease) or by excessive administration of
corticosteroid medication (exogenous Cushing’s disease).
Endogenous Cushing’s disease usually results from adrenal
hyperplasia (approximately 75% of cases), less commonly
from adenoma or carcinoma of the adrenal gland, and
rarely from hormone-producing tumors. The disease is
most frequent in women between the ages of 20 and Figure 47–1. Endogenous and exogenous Cushing’s disease:
60 years. Its onset is variable. Easy bruising and the vertebral osteoporosis. Anteroposterior radiograph outlines osteo-
development of purpura, especially on the hands and porosis of the vertebrae, collapse of multiple vertebral bodies,
forearms, may be early manifestations. In women, men- and condensation of bone (arrows) at the vertebral margins.
623
624 Section XII Endocrine Diseases
increased radiodensity of the superior and inferior margins initially, but premature physeal closure may eventually
of compressed or collapsed vertebral bodies allows a lead to short stature. Additional musculoskeletal manifes-
specific diagnosis in some patients with Cushing’s disease. tations include accelerated dental maturation, prominent
Further, as opposed to the characteristics of postmeno- musculature, and premature calcification of the costal
pausal and senile osteoporosis, the osteoporosis of Cushing’s cartilage.
syndrome may involve the skull, creating peculiar patchy Tumors arising from the adrenal cortex are rare in
radiolucent areas in the cranial vault. The ribs are also infants and children. These may be benign (adenomas) or
involved, and rib fractures, which are frequent in Cushing’s malignant (carcinomas). Because histologic differentiation
syndrome, may heal with abundant callus formation. of the benignancy or malignancy of the tumor is often
Osteoporosis of the pelvis may be associated with protrusio difficult, these tumors are considered together as adreno-
acetabuli. Osteopenia in the appendicular skeleton gen- cortical neoplasms. Such adrenocortical neoplasms are
erally is not attributed to Cushing’s syndrome. Osteo- far less common than neuroblastomas but more common
porosis, vertebral compression, and kyphosis may also than pheochromocytomas. Adrenocortical neoplasms
occur with exogenous corticosteroid therapy. typically occur before the age of 5 years and are more
common in girls than in boys. Clinical findings involve
endocrine abnormalities, including those of Cushing’s
Osteonecrosis syndrome (often manifested as generalized obesity) and
Osteonecrosis is a well-recognized complication of exoge- virilization. A characteristic finding is precocious puberty,
nous hypercortisolism. In endogenous hypercortisolism, in which secondary sex characteristics appear before the
osteonecrosis is less well reported and most frequently age of 8 years in girls and before the age of 9 years in boys.
involves the femoral head, although changes in the humeral Adrenocortical neoplasms may be associated with
head have also been noted. Considering that similar factors hemihypertrophy ipsilateral or contralateral to the side
may be operational in both exogenous and endogenous of the tumor. They also may be part of the Beckwith-
steroid excess, it is surprising that osteonecrosis is not Wiedemann syndrome. This syndrome, which is some-
reported more frequently during the course of endoge- times referred to as exomphalos-macroglossia-gigantism
nous Cushing’s syndrome. When osteonecrosis occurs in syndrome, is accompanied by benign and malignant
this syndrome, typical radiographic features are evident, tumors of multiple organs. The most common asso-
with subchondral curvilinear radiolucent shadows, osteo- ciated neoplasm is nephroblastoma (Wilms’ tumor),
porosis, osteosclerosis, bony collapse and fragmentation, followed in frequency by adrenocortical carcinoma and
and a relatively normal articular space. hepatoblastoma.
NEUROBLASTOMA
Tumors of the sympathetic nervous system are generally
classified as neuroblastoma, ganglioneuroblastoma, and
ganglioneuroma. Of these, neuroblastoma is most
frequent and usually arises in the adrenal medulla. In
common with pheochromocytoma, the tumor may be
accompanied by excessive production of catecholamines
and catecholamine metabolites; however, neuroblastoma
is a very aggressive tumor characterized by rapid growth
and widespread metastases. Approximately 80% of patients
are children younger than 5 years, and most of these are
younger than 2 years. Boys and girls are affected in equal
numbers. Skeletal metastases are frequent, often demon-
strating bilateral and symmetrical osseous lesions, espe-
cially in the metaphyseal segments (Fig. 47–3). Osteolytic
lesions with permeative bone destruction predominate,
particularly in the femur and tibia; sclerosis is less com-
mon and is a late manifestation of the disease, but it may
become extensive. In the skull, increased intracranial
pressure (due to leptomeningeal involvement and leading
to sutural diastasis), vertical osseous striations extending
Figure 47–3. Neuroblastoma: skeletal metastasis. Note
from the outer table, and soft tissue swelling are seen. permeative bone destruction and periostitis in the metaphysis
Additional manifestations of the skeletal metastases of the tibia. Growth recovery lines are also evident.
include “floating” teeth related to mandibular involve-
ment, vertebral collapse, rib alterations with extrapleural
extension, and pelvic bone destruction with soft tissue
masses compressing nearby viscera, such as the bladder. DIABETES MELLITUS
The differential diagnosis includes Ewing’s sarcoma,
General Features
lymphomas, leukemias, and metastases from other tumors,
such as rhabdomyosarcoma, medulloblastoma, retinoblas- Although numerous musculoskeletal disorders have been
toma, and Wilms’ tumor. described in conjunction with diabetes mellitus (Table
47–1), the association between them has not been well
documented. Much of the difficulty in associating diabetes
and additional rheumatic conditions is related to its
extreme frequency; thus, the occurrence of diabetes in
combination with another disease process may be no
more than the coincidental appearance of two diseases in
the same patient.
Gouty Arthritis
The relationship between diabetes mellitus and gouty
arthritis is not well defined. When patients with gout are
compared with age- and weight-matched control subjects,
no significant difference in glucose metabolism is found
between the two groups. This evidence suggests that the
prevalence of diabetes in patients with gout is related to
the high frequency of obesity in hyperuricemic subjects.
Radiographic Findings
The most striking radiographic finding is a decrease or
absence of body fat, manifested as loss of soft tissue planes.
A markedly advanced bone age may be seen in children
with this syndrome. Thickening of the diaphyseal cortices,
metaphyseal sclerosis, and hypertrophy of the epiphyses
have been described in the long bones. Small cystic lesions
may appear in the metaphysis. Changes in the skull may
include dolichocephaly, brachycephaly, calcification of
the falx cerebri, thickening of the calvaria, and advanced
dentition. Evidence of organomegaly may be seen in the
Figure 47–6. Diabetes mellitus: association with neuropathic chest and abdomen.
osteoarthropathy. In a patient with diabetes, neurologic deficit, Cystic and sclerotic foci are particularly common in
and soft tissue infection, note the degree of sclerosis and frag- periarticular regions, and the findings may resemble
mentation of the tarsometatarsal joint (arrow), a common site those of osteonecrosis or osteopoikilosis (Fig. 47–7). The
of occurrence of neuropathic osteoarthropathy in diabetic regions of increased density may extend into the me-
patients. Lateral displacement of the metatarsal bases is also taphysis and involve the margins of the vertebral bodies.
seen.
It has been suggested that the radiodense foci represent
an osteoblastic reaction to loss of fat in the bone marrow.
Because there are wide variations in the radiographic
appearance of lipoatrophic diabetes, its differential diag-
Vascular Calcification nosis depends on the distribution and severity of imaging
Arterial calcifications, especially of the internal carotid findings in the particular patient. This condition should
and renal arteries, the aorta, and the arteries of the lower be differentiated from partial lipodystrophy, in which loss
extremities and pelvis, are commonly observed during of facial, trunk, and upper extremity fat is apparent in
radiography in patients with diabetes mellitus. In the leg, children between the ages of 5 and 15 years. A decrease
arterial calcifications of the media, rather than the intima, in subcutaneous fat may also be seen in hyperthyroidism,
are the typical lesions of diabetes mellitus and lead to anorexia nervosa, progeria, and various other congenital
regular, diffuse, and fine-grained collections that gener- and acquired disorders. The gigantism of lipoatrophic
ally affect the whole circumference of the vessel and accu- diabetes must be distinguished from pituitary and cere-
mulate in rings. Although common in diabetes mellitus, bral gigantism. In pituitary gigantism, increased subcu-
arterial calcifications generally do not impinge on the
lumen of the vessel; however, such calcifications are more
frequent in diabetic patients with gangrene than in those
without gangrene, suggesting that arterial calcific deposits
can lead to impaired circulation owing to the increased
rigidity of the vessel wall. The cause of the angiopathy is
not clear.
LIPOATROPHIC DIABETES
General Features
Lipoatrophic diabetes is characterized by insulin-resistant
diabetes, hepatosplenomegaly, hyperlipidemia, hyper-
metabolism, accelerated growth and maturation, muscular
overdevelopment, hirsutism, hyperpigmentation, and pro- Figure 47–7. Lipoatrophic diabetes. Note the increased
gressive loss of adipose tissue without ketosis. Additional radiodensity in the femoral necks. (From Gold RH, Steinbach
clinical features are cutaneous xanthomas, protuberant HL: Lipoatrophic diabetes mellitus (generalized lipodystrophy):
abdomen, corneal opacities, and mental retardation. Lipo- Roentgen findings in two brothers with congenital disease. AJR
atrophic diabetes may be congenital or acquired. In the Am J Roentgenol 101:884, 1967.)
Chapter 47 Disorders of Other Endocrine Glands and of Pregnancy 629
taneous fat and typical osseous changes are apparent, DISORDERS AND COMPLICATIONS
whereas in cerebral gigantism, there is excessive body fat OF PREGNANCY
and an absence of hepatosplenomegaly.
In women, there is an increase in lumbar lordosis and
angulation at the lumbosacral junction, as well as an
ANOMALIES IN INFANTS OF DIABETIC
increase in obliquity of the pelvic bones themselves.
MOTHERS
During pregnancy, certain biomechanical changes occur
Hyperinsulinemia in the fetus of a diabetic mother is that produce increased stress on the pelvic joints. As a
presumed to be the result of maternal hyperglycemia, result of increased weight during pregnancy, the degree
because insulin does not traverse the placental barrier. of lumbar lordosis increases, and the center of mass is
Elevated levels of insulin can promote abnormal growth, displaced. Sliding movement at the sacroiliac joint, which
which is manifested in the newborn as visceromegaly and is evident in normal persons, becomes accentuated owing
increased body fat. The frequency of congenital anomalies to softening and relaxation of adjacent ligaments and
in the offspring of diabetic mothers may be as high as diastasis. It is generally assumed that relaxation of the
20% but is probably far less. These include respiratory joints of the pelvis is an essential and normal accompani-
anomalies (respiratory distress syndrome, wet lung syn- ment of pregnancy, which ensures the accommodation of
drome, persistence of fetal circulation); cardiomyopathy the growing fetus and facilitates vaginal delivery. The
and congenital heart disease; hyperviscosity, thrombosis, major radiographic findings associated with physiologic
and hemorrhage; renal anomalies (hydronephrosis, dupli- and pathologic changes during pregnancy occur at the
cation, cystic kidneys, renal agenesis, pseudohermaph- sacroiliac joints and symphysis pubis. Bony eburnation
roditism, renal vein thrombosis); small left colon syn- about the sacroiliac joint is termed osteitis condensans
drome; and alterations in the musculoskeletal system. ilii, whereas that at the symphysis pubis is termed osteitis
Sacrococcygeal agenesis, or the caudal regression syn- pubis. Although either condition may be apparent in
drome, is one of the most specific anomalies in these nulliparous women or even men, both are observed most
infants. Approximately 20% of patients with this syndrome frequently in multiparous women.
are the children of diabetic mothers, and approximately
16% of children born to diabetic mothers have sacral
anomalies. Agenesis varies in severity; in some cases, only Osteitis Condensans Ilii
minor coccygeal changes are present, whereas in others, Osteitis condensans ilii is a condition of the pelvis that
aplasia of the lower thoracic and entire lumbar spine is involves predominantly the ilium adjacent to the sacroiliac
combined with absence of one or more segments of the joint. Usually, though not invariably, it is a bilateral and
sacrum (Fig. 47–8). Associated abnormalities include relatively symmetrical process in women; rarely, men are
meningocele, arthrogryposis, hip dislocation, flexion affected, and asymmetrical or unilateral changes may be
contractures of the knee and hip, foot deformities, and observed. Osteitis condensans ilii is associated with well-
urinary tract anomalies. defined triangular sclerosis on the iliac aspect of the
sacroiliac joint (Fig. 47–9). The bony eburnation involves
the inferior portion of the bone, and the apex of the
sclerosis extends into the articular portion of the ilium.
The subchondral bone is generally well defined, high-
lighted by the radiodensity of the involved osseous
surface. Significant narrowing of the sacroiliac joint and
extensive involvement of the sacrum are distinctly unusual.
The cause of osteitis condensans ilii is not clear. The
predominant theory suggests that the condition is second-
ary to mechanical stress across the sacroiliac joint coupled
with increased vascularity during pregnancy. Its occur-
rence in nulliparous women and in men is inconsistent
with this theory, although mechanical stress of a different
cause may be operational in these people. Generally, this
disorder is easily differentiated from ankylosing spondylitis
(Table 47–2).
Osteitis Pubis
Osteitis pubis is a painful condition of the symphysis pubis
that may become apparent within 1 month or longer
after delivery or other pelvic procedures or operations. In
Figure 47–8. Anomalies in children of diabetic mothers: caudal men, osteitis pubis is particularly frequent after prostate
regression syndrome. In this 27-year-old daughter of a diabetic (or bladder) surgery. Clinical findings are characterized
mother, aplasia of the lower lumbar spine and sacrum is by local pain and tenderness, muscle spasm, and unstable
associated with pelvic and acetabular deformity and dislocation gait. The disorder should be distinguished from the normal
of the right hip. (Courtesy of S. Hilton, MD, San Diego, Calif.) symphyseal separation and hypermobility that may occur
630 Section XII Endocrine Diseases
during pregnancy. In this normal situation, widening of fashion, although asymmetrical or unilateral findings are
the symphysis can be detected radiographically and is accen- occasionally encountered. The degree of resorption or
tuated by the patient’s standing on one leg at a time. The sclerosis may rarely become striking, with osteolysis of a
width of the articulation is usually less than 7 mm, and large segment of the pubic bones. Restoration of the
the symphyseal changes regress within a few months post osseous surface with disappearance of the sclerosis may
partum. In the unusual case in which the pubic changes be associated with bony ankylosis of the joint.
do not regress and are associated with bony erosion, resorp- In most cases, the cause of osteitis pubis remains
tion, and eburnation, osteitis pubis may be diagnosed. speculative. The differential diagnosis of osteitis pubis
The radiographic appearance of osteitis pubis includes includes not only trauma and infection but also a variety
mild to severe subchondral bony irregularity of the of articular disorders characterized, in part, by inflam-
symphysis pubis with resorption (Fig. 47–10). The condi- mation of the symphysis pubis. Subchondral resorption
tion usually involves both pubic bones in a symmetrical of bone in primary and secondary hyperparathyroidism
TABLE 47–2
Osteitis Condensans Ilii (OCI) versus Ankylosing Spondylitis (AS)
OCI AS
Age Young adults Young adults
Sex Women > men Men > women
Clinical symptoms, Absent or mild Mild to severe
signs
HLA-B27 Present in 8% Present in 90%
(same as controls)
Sacroliliac joint
abnormalities
Distribution Bilateral, symmetrical; Bilateral, symmetrical;
iliac iliac > sacral
Sclerosis Well defined Poorly defined
Joint space Normal Narrowed
Erosion Absent Common
Bony ankylosis Absent Common
Spinal abnormalities Absent Common
Symphysis pubis Less common More common
abnormalities
Chapter 47 Disorders of Other Endocrine Glands and of Pregnancy 631
during labor or delivery. The width of the symphysis pubis
in this physiologic condition is usually less than 7 mm. In
rare circumstances, rupture of the symphysis pubis occurs
during the final stages of pregnancy, particularly during
labor and delivery. Radiographs reveal symphyseal separa-
tions varying from 1 to 12 cm (Fig. 47–11). Nonoperative
treatment is generally sufficient, and functional recovery
should be complete.
although histologic data suggest that the decrease in bone (Fig. 47–12). The sternoclavicular joint space is not
mass is related principally to low-turnover osteoporosis. narrowed, and adjacent soft tissue and osseous structures
are not affected. Scintigraphy demonstrates increased
accumulation of bone-seeking radiopharmaceutical
Stress Fractures
agents. Computed tomography scanning and MR imag-
Stress fractures have been observed in the later stages of ing can be used to document the extent of bone involve-
pregnancy and in the postpartum period. Pelvic sites such ment. The cause of condensing osteitis of the clavicle is
as the parasymphyseal bone and sacrum are typically unknown. Its occurrence in women of childbearing age
involved. A potential cause of such fractures in the pubic suggests a common causative agent with osteitis condensans
rami in the final stages of pregnancy is the forceful descent ilii, and stress-induced changes are most likely.
of the fetal head against a pelvic ring that contains lax The differential diagnosis of this clavicular lesion is
ligaments. Stress fractures in the postpartum period may limited. The most difficult entity to exclude is ischemic
be related to increased body weight and increased levels necrosis of the medial clavicular epiphysis (Friedreich’s
of physical activity. disease). This rare disorder typically occurs in children
and adolescents and is believed to be related to direct
trauma or to an embolic event that results in obliteration
Other Complications
of the vascular supply to the medial clavicular epiphysis.
Carpal tunnel syndrome is a frequent finding during Sternocostoclavicular hyperostosis is a second disease
pregnancy, often occurring bilaterally in the second or that affects the medial end of the clavicle. Hyperostosis
third trimester. It is probably related to soft tissue of the clavicle, sternum, and upper anterior ribs, with
swelling in and around the carpal tunnel, as the syndrome ossification of intervening soft tissues, is observed. In
is associated with swelling of the fingers, generalized contrast to condensing osteitis of the clavicle, this condi-
edema, preeclampsia, and hypertension. Tenosynovitis, tion is more common in older patients and in men, is
especially De Quervain’s, is also encountered during usually bilateral, and is often accompanied by pustular
pregnancy, generally attributed to fluid retention. lesions of the palms and soles (pustulosis palmaris et
plantaris). Pyarthrosis of the sternoclavicular joint, espe-
cially the type seen in intravenous drug abusers, may
OSTEITIS CONDENSANS OF THE CLAVICLE
mimic condensing osteitis of the clavicle, although labo-
Although this relatively newly described condition is not ratory values and radiographic evidence of narrowing of
a complication of pregnancy, osteitis condensans of the the sternoclavicular joint and osseous destruction assist in
clavicle shares morphologic and radiographic features the differential diagnosis. Osteoarthritis of the sterno-
with osteitis condensans ilii and osteitis pubis. Patients clavicular joint may also resemble condensing osteitis;
are women with an average age of 40 years (range, 20 to however, joint space narrowing, osteophytosis, and sub-
50 years). A history of stress to the region of the sterno- chondral cysts are seen on both sides of the articulation.
clavicular joint, usually associated with heavy lifting or Chronic recurrent multifocal osteomyelitis, also referred
sports activity, is typical. Pain is most commonly referred to as cleidometaphyseal or plasma cell osteomyelitis, is an
to the ipsilateral shoulder and is accentuated with abduc- unusual syndrome seen in children and adolescents that
tion of the arm. No definite association with osteitis leads to symmetrical lesions of the tubular bones and
pubis or osteitis condensans ilii has been reported. clavicles. Hyperostosis is a prominent feature of clavic-
Radiographs reveal bone sclerosis and mild enlarge- ular involvement, producing bone enlargement that
ment of the inferomedial aspect of the clavicle, as well as resembles the findings in sternocostoclavicular hyper-
osteophytes in the inferior margin of the clavicular head ostosis. Chronic recurrent multifocal osteomyelitis and
A B
Figure 47–12. Condensing osteitis of the clavicle. This 40-year-old woman developed pain and tender-
ness over the medial end of the right clavicle. A, Conventional tomography reveals bone sclerosis involving
the inferomedial aspect of the bone (arrow). B, Coronal T1-weighted (TR/TE, 300/11) spin echo MR image
shows low signal intensity in this area (arrow). The diminished signal intensity persisted on T2-weighted
spin echo and gradient echo images (not shown). (Courtesy of G. Greenway, MD, Dallas, Tex.)
Chapter 47 Disorders of Other Endocrine Glands and of Pregnancy 633
potentially related disorders such as sternocostoclavicular James RE, Baker HL, Scanlon PW: The roentgenologic aspects
hyperostosis and the synovitis-acne-pustulosis-hyperostosis- of metastatic pheochromocytoma. AJR Am J Roentgenol
osteitis (SAPHO) syndrome are discussed in Chapters 53 115:783, 1972.
and 82. Jelinek JS, Murphey MD, Aboulafia AJ, et al: Muscle infarction
in patients with diabetes mellitus: MR imaging findings.
Radiology 211:241, 1999.
Johnson JP, Carey JC, Gooch WM III, et al: Femoral
FURTHER READING hypoplasia—unusual facies syndrome in infants of diabetic
mothers. J Pediatr 102:866, 1983.
Agrons GA, Lonergen GJ, Dickey GE, et al: Adrenocortical Kincaid OW, Hodgson JR, Dockerty MB: Neuroblastoma: A
neoplasms in children: Radiologic-pathologic correlation. roentgenographic and pathologic study. AJR Am J Roentgenol
Radiographics 19:989, 1999. 78:420, 1957.
Barnes WC, Malament M: Osteitis pubis. Surg Gynaecol Obstet Madell SH, Freeman LM: Avascular necrosis of bone in
117:277, 1963. Cushing’s syndrome. Radiology 83:1068, 1969.
Brower AC, Sweet DE, Keats TE: Condensing osteitis of the Mendelson EB, Fisher MR, Deschler TW, et al: Osteomyelitis
clavicle: A new entity. AJR Am J Roentgenol 121:17, 1974. in the diabetic foot: A difficult diagnostic challenge.
Cone RO, Resnick D, Goergen TG, et al: Condensing osteitis Radiographics 3:248, 1983.
of the clavicle. AJR Am J Roentgenol 141:387, 1983. Numaguchi Y: Osteitis condensans ilii, including its resolution.
Dhar S, Anderton JM: Rupture of the symphysis pubis during Radiology 98:1, 1971.
labor. Clin Orthop 283:252, 1992. Nunez-Hoyo M, Gardner CL, Motta AO, et al: Skeletal muscle
Dunn V, Nixon GW, Jaffe RB, et al: Infants of diabetic mothers: infarction in diabetes: MR findings. J Comput Assist Tomogr
Radiographic manifestations. AJR Am J Roentgenol 137: 17:986, 1993.
123, 1981. Pognowska MJ, Collins LC, Dobson HL: Diabetic osteopathy.
Gold RH, Steinbach HL: Lipoatrophic diabetes mellitus (gen- Radiology 89:265, 1967.
eralized lipodystrophy): Roentgen findings in two brothers Rand T, Schweitzer M, Rafii M, et al: Condensing osteitis of
with congenital disease. AJR Am J Roentgenol 101:884, the clavicle: MRI. J Comput Assist Tomogr 27:291, 1998.
1967. Segal G, Kellogg DS: Osteitis condensans ilii. AJR Am J
Gray RG, Gottlieb NL: Rheumatic disorders associated with Roentgenol 71:643, 1954.
diabetes mellitus: Literature review. Semin Arthritis Rheum Sinha S, Munichoodappa CS, Kozak GP: Neuro-arthropathy
6:19, 1976. (Charcot joints) in diabetes mellitus (clinical study of 101
Greenspan A, Gerscovich E, Szabo RM, et al: Condensing cases). Medicine 51:191, 1972.
osteitis of the clavicle: A rare but frequently misdiagnosed
condition. AJR Am J Roentgenol 156:1011, 1991.
SECTION
XIII
Diseases of the Hematopoietic System
CHAPTER 48
Hemoglobinopathies and The clinical manifestations of anemia are variable and
influenced by its rate of development and the status or
Other Anemias function of the cardiovascular, cerebrovascular, and renal
systems. Characteristic abnormalities include exertional
dyspnea, tachycardia, claudication, vertigo, and angina, con-
SUMMARY OF KEY FEATURES ditions indicating an inadequate supply of oxygen to the
body’s tissues. Specific types of anemia (of which there are
The hemoglobinopathies are associated with characteristic many) are sometimes associated with typical clinical clues,
abnormalities of the skeleton. In general, the abnormalities including the cholelithiasis seen in chronic hemolytic
are related to marrow hyperplasia, vascular occlusion, states and the bone pain and tenderness accompanying
and several additional problems, including fracture and osseous metastasis with compromise of the bone marrow.
infection. Although these features are apparent in The hypoxia of anemia is a stimulus for increased eryth-
almost all the hemoglobin disorders, their severity ropoiesis through the action of the hormone erythro-
varies, which in some cases allows differentiation among poietin, formed principally in the kidneys. This hormone
these disorders. The articular manifestations in the is responsible for increased cellular activity, leading to an
hemoglobinopathies can be attributed to epiphyseal augmented rate of formation of red blood cells from pre-
osteonecrosis, growth disturbances, osseous weakening, cursor cells. In the presence of bone marrow compro-
infection, crystal deposition, hemarthrosis, and synovial mise, which may be related to marrow infiltration by tumor
membrane microvascular obstruction. In some patients, or myelofibrosis, extramedullary sites such as the liver
these articular abnormalities overshadow other clinical and spleen become actively involved in hematopoiesis.
manifestations of the disease. Hepatosplenomegaly and even paravertebral masses are
radiographic findings consistent with such hematopoiesis.
associated abnormalities, which may include malaise, Radiographic and Pathologic Features
weight loss, and fever, are more likely manifestations of
the underlying disease. In patients with sickle cell trait, Marrow Hyperplasia
painful crises are not usually apparent unless the patient Hypercellularity of the bone marrow in this disease is a
is exposed to an atmosphere low in oxygen. response to anemia of long duration. In infants with
Sickle cell anemia rarely has its clinical onset in infants sickle cell anemia, red marrow extends into all the bones,
younger than 6 months because of the persistence of Hb including those of the hands and feet; in older children
F, which reduces the sickling properties of the red blood and adults, red marrow recedes from some of these small
cell. Fever, pallor, and swelling of the hands are observed bones but persists in the ankles, wrists, and shafts of the
in children with this disease, apparently related in part to long bones. Marrow hyperplasia produces widening of
infarction of the small tubular bones. This “hand-foot” the medullary cavities and intertrabecular spaces and
syndrome, or dactylitis, is most frequent between the ages rarefaction of the remaining trabeculae in the spongiosa
of 6 months and 2 years. It is the initial manifestation of and cortex. These pathologic findings are associated with
the disease in approximately 30% of cases. The clinical radiographic abnormalities, including increased radiolu-
features are self-limited, with a duration that varies from cency of osseous tissue, fewer and more accentuated bony
a few days to several weeks. trabeculae, and cortical thinning.
Other clinical manifestations that may be apparent In the skull, diffuse widening of the diploic space is
in sickle cell anemia are hepatosplenomegaly; cardiac associated with thinning of both the outer and inner
enlargement; chronic leg ulcers, particularly over the tables. The appearance is that of a coarse, granular osteo-
malleoli; osteomyelitis; septic arthritis; pulmonary abnor- porosis involving the entire cranial vault except for the
malities, including pneumonia and infarction; abdominal base of the occiput, which is relatively spared (Fig. 48–1).
pain; cholelithiasis; jaundice; peptic ulcer disease; hema- Localized areas of hyperplastic marrow (or infarcts) lead
turia; priapism; neurologic and muscular findings; and to focal radiolucent areas simulating metastasis or myeloma.
lymphadenopathy. Death may result from infection, In rare instances, focal or diffuse osteosclerosis of the
cardiac decompensation related to severe anemia, or cranium is observed, perhaps related to myelofibrosis.
thrombosis and infarction of various organs. In recent The facial bones, with the exception of the mandible, are
years there has been increased longevity, and many affect- not generally involved in sickle cell anemia. Prominence
ed persons live past the age of 50 years. of the lamina dura may be observed.
A B
Figure 48–6. Sickle cell anemia: vascular occlusion and epiphyseal infarction. A, Osteonecrosis of the
femoral head is characterized by focal areas of sclerosis and subsequent collapse, with irregularity of the
articular surface. Observe the lateral femoral osteophytes and buttressing (arrowhead) of the femoral neck.
B, The “snow-capped” appearance of the humeral head is related to patchy sclerosis. Collapse of the
articular surface (arrowhead) and subchondral fractures (arrow) are evident.
metaphyseal growth disturbances have also been empha- Osteomyelitis and Septic Arthritis. The frequency of
sized in this disease and are particularly frequent in the osteomyelitis related to a variety of organisms in patients
hands and feet, although they may occur elsewhere, with sickle cell anemia is reported to be more than 100
including the long tubular bones. Cone-shaped epiphyses times that seen in normal persons. Bone and joint infec-
and inverted V, “cup,” or “channel” deformities of the tions in sickle cell anemia are caused by salmonellae in
adjacent metaphyses are observed in sickle cell anemia,
but similar changes occur in many other disorders.
Tibiotalar deformity consists of slanting of the articu-
lar surfaces of the distal portion of the tibia and the talus.
It is an infrequent sign of sickle cell anemia and may also
be seen in hemophilia, juvenile-onset rheumatoid arthri-
tis, and multiple epiphyseal dysplasia.
Central depression of the vertebral bodies may be an
additional growth disturbance of sickle cell anemia. The
resulting deformity of the vertebra consists of squared-
off endplate depressions, the H vertebra (Fig. 48–7). This
deformity has been postulated to result from ischemia of
the central portion of the vertebral growth plate, a vul-
nerability paralleling that of the central aspect of the
growth plate in the peripheral skeleton.
Miscellaneous Findings
Fractures. Fractures in the appendicular and axial skele-
tons in patients with sickle cell anemia can occur spon-
taneously or after minor trauma. In the long tubular
bones, marrow hyperplasia produces cortical thinning,
which predisposes to this complication. In the spine,
A B
marrow hyperplasia results in diffuse weakening of the
Figure 48–7. Sickle cell anemia: growth disturbance and H
entire vertebral body. Typical deformities result from vertebrae. A and B, Two examples of H vertebrae, which are
compression of the bone by the adjacent intervertebral characterized by central indentations of the vertebral bodies.
discs (fish vertebrae) or from intraosseous displacement Initially, the abnormalities may simulate cartilaginous nodes or
of portions of the intervertebral disc (cartilaginous, or fish vertebrae (arrowheads), although eventually, typical
Schmorl’s, nodes). squared-off indentations (arrows) are observed.
640 Section XIII Diseases of the Hematopoietic System
over 50% of cases. It is suggested that these organisms generally confined to one or two joints and subsides in 2
gain entry to the bloodstream as a result of intestinal to 14 days. Radiographic examination may demonstrate
infarction produced by sickling within mesenteric vessels; osteonecrosis of adjacent bones.
typically, Salmonella bacteremia precedes osteomyelitis,
and the organisms then lodge in the bone marrow.
Radionuclide Findings
Staphylococci are a second common cause of osteomyelitis
in sickle cell anemia and, in some series, are implicated In normal adults, bone marrow is located predominantly
more frequently than salmonellae. Osteomyelitis is most in the axial skeleton and proximal portions of the femora
frequent in the long tubular bones. Infection may pro- and humeri; in patients with various anemias, the marrow
duce symmetrical involvement with diaphyseal localiza- expands symmetrically to occupy the long bones and
tion (Fig. 48–8). Osteomyelitis in these patients may be skull. During asymptomatic periods, patients with sickle
related not only to hematogenous dissemination of infec- cell anemia and related disorders may reveal focal areas
tion to bone but also to direct spread from a contiguous of decreased uptake, which can probably be attributed to
soft tissue infection. Septic arthritis complicating sickle sites of previous infarction. During a crisis, additional
cell anemia is less frequent than osteomyelitis. Radio- areas are found that are without radionuclide activity,
graphic findings are typical of septic arthritis: soft tissue surrounded by active marrow. After a crisis, the infarcted
swelling, joint space narrowing, variable osteoporosis, marrow may return to normal in 1 to 12 months or
and osseous destruction. progress to permanent fibrosis, with corresponding
changes in radionuclide activity.
Crystal Deposition. Hyperuricemia occurs in some patients Bone scanning agents such as technetium-99m (99mTc)
with sickle cell anemia. The reported frequency of this polyphosphate demonstrate uptake related to the integ-
laboratory finding in adults with the disease varies from rity of blood flow and the presence of new bone forma-
20% to 40%. The frequency of clinical attacks of gout tion. In patients with sickle cell anemia and related diseases,
and successful recovery of urate crystals from inflamed marrow expansion is associated with increased blood flow
joints in patients with sickle cell anemia is lower. and an increased accumulation of radionuclide in the
skeleton, particularly in the lower extremities. Immediately
Joint Effusions. Joint effusions in sickle cell anemia that after a crisis, an area of infarction may demonstrate
are not associated with infection, crystal deposition, or decreased or absent radionuclide activity (Fig. 48–9).
hemarthrosis are relatively common. Fluid production is The size of this defect in activity is generally smaller on
bone scans than on bone marrow scans. One to 2 weeks
after the crisis, increased activity on bone scanning is due
to reactive bone formation about the area of infarction.
This abnormal activity may persist for several months.
It has been suggested that scanning with both bone-
and bone marrow–seeking radionuclides allows differen-
tiation of osteomyelitis and osteonecrosis in patients with
sickle cell anemia. The combination of a large defect on
the bone marrow scan and a smaller defect on the bone
scan is typical of osteonecrosis. Marrow scans with 99mTc
sulfur colloid may also outline sites of extramedullary
hematopoiesis in sickle cell anemia or related disorders.
A B
for “sea,” because their patients were of Mediterranean thalassemia (thalassemia minor) is generally associated
origin. It is now known that thalassemia is not a single with mild clinical findings, including slight to moderate
disease but a group of disorders related to an inherited anemia, splenomegaly, and jaundice. It is observed
abnormality in globin production. These disorders differ throughout the world, most commonly in Mediterranean
from sickle cell anemia, which results from an inherited populations, and occurs in approximately 1% of American
structural abnormality in one of the constituent globin blacks, most of whom are entirely asymptomatic.
chains; the thalassemias result from inherited defects in
the rate of synthesis of one of the globin chains. This
abnormality leads to an imbalance in globin chain pro- Radiographic and Pathologic Features
duction, which contributes to ineffective erythropoiesis, Marrow Hyperplasia. The radiographic and pathologic
hemolysis, and a variable degree of anemia. features of beta-thalassemia are due in large part to
Thalassemia is divided into two main groups: alpha- marrow hyperplasia. Initially, both the axial and the
thalassemia is characterized by a deficiency in alpha-globin appendicular skeletons are altered, but as the patient
chain synthesis, and beta-thalassemia is characterized by reaches puberty, the appendicular skeletal changes diminish
a deficiency in beta-globin chain synthesis. Because Hb F because of normal regression of the hematopoietic marrow.
contains alpha chains, the fetus is affected by alpha- The changes in thalassemia major are much more severe
thalassemia; beta-thalassemia becomes apparent after the than those in thalassemia minor.
newborn period, when Hb A replaces Hb F. Thalassemia In the skull, the frontal bones reveal the earliest and
may exist in a homozygous form, called thalassemia most severe changes, and the inferior aspect of the
major, or a heterozygous form, termed thalassemia minor occiput is usually unaltered. Findings include granular
or minima. osteoporosis, widening of the diploic space, and thinning
of the outer table. Bony proliferation on the outer table
of the vault leads to a hair-on-end appearance. Marrow
Clinical Features hyperplasia of the skull is not confined to the cranial
Homozygous beta-thalassemia (thalassemia major), typi- vault but involves the facial bones as well (Fig. 48–15). In
cally seen in persons of Mediterranean ancestry, is charac- infancy and early childhood, osseous expansion of the
terized by severe anemia, prominent hepatosplenomegaly, nasal and temporal bones leads to obliteration of the air
and early death, often in childhood. Heterozygous beta- spaces of the paranasal sinuses. Maxillary alterations can
Chapter 48 Hemoglobinopathies and Other Anemias 645
produce lateral displacement of the orbits, leading to accentuation of vertical trabeculation, and biconcave
hypertelorism, malocclusion of the jaws, and displace- deformities (fish vertebrae) are evident. Medullary
ment of dental structures, with a resultant “rodent” hyperplasia is also evident in other bones of the axial
facies. The extent and severity of the cranial vault changes, skeleton, including the ribs, pelvis, and clavicles, as well
including the hair-on-end appearance, are much greater as the bones of the appendicular skeleton (Figs. 48–16
in thalassemia than in other anemic disorders. and 48–17). Similarly, the tubular bones reveal a widened
Osteoporosis of the vertebrae is most evident in the marrow cavity, cortical thinning, and a coarse, trabecu-
vertebral bodies. In the spine, reduction in the number of lated appearance. The contour of some of the long bones
trabeculae, thinning of the subchondral bone plates, is altered; normal concavity is lost, and the bones may
A B
Figure 48–15. Marrow hyperplasia in the skull and face. In this example, a hair-on-end appearance is well
shown on a routine radiograph (A), and overgrowth of the facial bones is delineated with transaxial CT
scanning (B). (Courtesy of S. K. Brahme, MD, San Diego, Calif.)
A B
Figure 48–16. Thalassemia major: marrow hyperplasia in the axial and appendicular skeletons. A, Femur.
An Erlenmeyer flask deformity is apparent, with loss of normal concavity and straightening or convexity of
the osseous contour, particularly along the medial aspect of the bone. B, Hands. Osteopenia, small cystic
lesions, and bone reinforcement lines are apparent. (B, Courtesy of S. K. Brahme, MD, San Diego, Calif.)
646 Section XIII Diseases of the Hematopoietic System
B
Figure 48–19. Thalassemia major: extramedullary
hematopoiesis. A, Bilateral lobulated posterior
mediastinal masses can be seen. The heart is
enlarged. B, In a different patient, a coronal T1-
weighted (TR/TE, 700/20) spin echo MR image of
the thorax reveals evidence of marrow reconversion
and heterotopia. Note the presence of hematopoietic
marrow in the vertebral bodies and ribs. The poste-
A rior portions of the ribs are expanded dramatically.
648 Section XIII Diseases of the Hematopoietic System
A B
maintain acceptable levels of hemoglobin and chelation Figure 48–21. Thalassemia major: deferoxamine-induced
to remove the excessive amounts of iron that result from bone changes. A, In an anteroposterior radiograph of the knee,
such transfusions. Although these methods have succeed- the metaphyses of the distal portion of the femur and proximal
end of the tibia are deficient circumferentially. Note the irregu-
ed in prolonging the life span of some patients with larity of the femoral metaphysis and, to a lesser extent, the tibial
various types of thalassemia, complications related to and fibular metaphyses. B, In the lumbar spine, flattened
chelation therapy have been identified. vertebral bodies are evident. (From Brill PW, Winchester P,
One of the chelation agents, deferoxamine, may lead Giardina PJ, Cunningham-Rundles S: Deferoxamine-induced
to significant skeletal abnormalities. Clinically, affected bone dysplasia in patients with thalassemia major. AJR Am J
patients have short trunks with moderate sternal protru- Roentgenol 156:561, 1991.)
sion, genu valgum, generalized joint stiffness, and peri-
articular bone deformities. Irregular flattening of the
HEREDITARY SPHEROCYTOSIS
vertebral bodies in the thoracic and lumbar spine may be
observed. More dramatic alterations affect the metaphyses This hemolytic anemia, which is inherited as an autosomal
of the tubular bones of the extremities (Fig. 48–21). The dominant disorder, is worldwide in distribution but most
bone changes are typically bilateral, and common sites of common in northern Europeans. It is characterized by abnor-
involvement include the distal femoral, proximal tibial, mally shaped red blood cells, or spherocytes, in the peripheral
and distal ulnar areas. Although the skeletal abnormal- blood that are hemolyzed in the spleen. Clinical features
ities resemble those encountered in spondylometaphy- include anemia, jaundice, and splenomegaly. Gallstones and
seal dysplasia, the findings do not precisely correspond to chronic leg ulcerations are two other potential manifes-
any of the recognized genetic dysplasias. The precise tations of this disorder. Coincident syndromes have been
relationship among the dose of deferoxamine, the age of described, including unconjugated hyperbilirubinemia,
the patient, and the severity of the skeletal alterations familial myocardiopathy, and spinal cord dysfunction.
remains to be established. As in other hemolytic diseases, compensatory hyper-
plasia of the bone marrow occurs, with extension of red
marrow into the diaphyses of long bones. In the cranial
IRON DEFICIENCY ANEMIA vault, diploic widening and thinning of the outer table are
In children, iron deficiency anemia results from insuf- evident. Because anemia in this condition may not mani-
ficient intake or excessive loss of iron during the first fest until late childhood or adolescence, significant long
6 months of life, which depletes the iron that was stored bone changes are unusual. Occasionally, extramedullary
during late prenatal life. This type of anemia is most hematopoiesis may result in paravertebral masses
frequent between 9 months and 2 years of age. Skeletal simulating infection or tumor.
abnormalities are generally mild. Marrow hyperplasia
may result in changes in the cranial vault, with thin-
HEREDITARY ELLIPTOCYTOSIS
ning of the outer table and diploic widening observed in
the frontal, parietal, or occipital bone. Radial striations In hereditary elliptocytosis, red blood cells are oval or
extending from the outer table are rare and usually mild elliptic in shape. The disorder is closely linked to
in iron deficiency anemia, allowing its distinction from hereditary spherocytosis. Hemolysis occurs in the spleen.
thalassemia. The bone abnormalities are largely undescribed.
Chapter 48 Hemoglobinopathies and Other Anemias 649
NONSPHEROCYTIC HEMOLYTIC ANEMIA sickle cell–hemoglobin C disease than in sickle cell
anemia, although this point is debated. Diaphyseal infarc-
Nonspherocytic hemolytic anemia is a designation that tion with surrounding sclerosis producing a bone-within-
can be used for hereditary hemolytic processes not bone appearance is suggestive of sickle cell anemia or
attributable to thalassemia, spherocytosis, elliptocytosis, sickle cell–hemoglobin C disease because it is rare in other
or one of the hemoglobinopathies. The two most impor- anemias. Infarction of the small bones of the hands and
tant disorders in this category are pyruvate kinase defi- feet in children with subsequent dactylitis and the hand-
ciency and glucose-6-phosphate dehydrogenase deficiency foot syndrome is almost diagnostic of sickle cell anemia.
anemias. Radiographic changes have been documented in Abnormalities in vertebral body contour consisting of
association with pyruvate kinase deficiency, including squared-off compressions of the vertebrae (H vertebrae)
marrow hyperplasia producing osteoporosis, diploic occur in sickle cell anemia, sickle cell–hemoglobin C
widening, and thinning of the cranial vault. Unlike with disease, and sickle cell–thalassemia disease, although such
thalassemia, the facial bones and sinuses are unaffected. abnormalities may be seen in thalassemia and in other
conditions such as Gaucher’s disease. Flaring of the ends
APLASTIC ANEMIA of the tubular bones, the Erlenmeyer flask appearance, is
most suggestive of thalassemia. Premature fusion of the
An uncommon condition, aplastic anemia is charac- growth plate has been described in both sickle cell anemia
terized by acellular or hypocellular bone marrow and is and thalassemia. Osteopenia, which may be found in many
accompanied by pancytopenia, including anemia, neutro- of the anemic disorders, leads to osseous weakening and
penia, and thrombocytopenia. Its causes are diverse, thereby predisposes to skeletal fractures. Pathologic frac-
including drugs (chloramphenicol, phenytoin, gold, tures have been noted in sickle cell anemia and thalassemia,
benzene), radiation, paroxysmal nocturnal hemoglo- although they are to be expected in the other anemias
binuria, Fanconi’s anemia, viral hepatitis, pregnancy, and as well. Osteomyelitis, particularly that associated with
thymoma, although many cases are idiopathic. Patients Salmonella infection, is a known complication of sickle cell
with aplastic anemia have an impaired ability to incor- anemia, although infections of bones and joints can be
porate iron into marrow erythroid precursors. Various observed in thalassemia and other hemoglobinopathies.
types of therapy, including repeated blood transfusions or
marrow transplantation, can effect partial or complete
recovery of the bone marrow. Differentiation of Anemia from Other
MR imaging may be useful in the initial diagnosis of Conditions
aplastic anemia and as a method of monitoring the thera- In anemic disorders, diffuse skeletal abnormality charac-
peutic response. MR imaging in untreated patients with terized by decreased radiographic density (osteopenia)
aplastic anemia emphasizes an augmentation of marrow is an indication of marrow hyperplasia. This finding may
signal intensity because of the presence of fat. During be simulated by changes in other disorders. Other
therapy, focal areas of low signal intensity, representing primary marrow diseases, such as Gaucher’s disease and
islands of active hematopoietic cells scattered in other- Niemann-Pick disease, are associated with similar find-
wise fatty marrow, may be evident. ings. Osteopenia may also accompany osteogenesis
imperfecta, idiopathic juvenile osteoporosis, hyperpara-
DIFFERENTIAL DIAGNOSIS thyroidism, and leukemia.
Thickening of the cranial vault is a radiographic find-
Differentiation among the Anemias ing that is apparent in many disorders. In anemias, this
(Table 48–1) thickening frequently involves the entire cranium, with
Extensive thickening of the cranial vault associated with the exception of the inferior aspect of the occipital bone
marked diploic expansion and a hair-on-end appearance and base of the skull. Fibrous dysplasia and leontiasis
is most characteristic of thalassemia. Its occurrence in ossea can lead to excessive hyperostosis of the skull, but
patients with sickle cell anemia has been overemphasized, changes usually predominate in the frontal regions and
as this finding is evident in less than 5% of patients with face. The hair-on-end appearance is characteristic of
the disease. Some thickening of the cranial vault may be thalassemia.
apparent in sickle cell–hemoglobin C disease, sickle Epiphyseal and diaphyseal bone infarction occurs not
cell–thalassemia disease, iron deficiency anemia, heredi- only in anemia but also in other diseases. Epiphyseal
tary spherocytosis, and pyruvate kinase deficiency, but ischemia, particularly of the femoral head, is noted in
widespread and severe skull alterations are rare. endogenous and exogenous corticosteroid excess, caisson
Changes of marrow hyperplasia in the peripheral disease, Gaucher’s disease, Legg-Calvé-Perthes disease,
skeleton are most common and marked in thalassemia. In collagen vascular disorders, alcoholism with pancreatitis,
iron deficiency anemia and sickle cell–thalassemia disease, and radiation therapy, as well as after trauma and in many
changes of marrow hyperplasia in the tubular bones are other processes. Diametaphyseal ischemia is seen in asso-
absent or mild. Bone infarction is particularly common in ciation with steroid administration, caisson disease,
sickle cell anemia and sickle cell–hemoglobin C disease. pancreatitis, and Gaucher’s disease.
Ischemic skeletal changes are less frequent in sickle Abnormality in vertebral body contour characterized
cell–thalassemia disease and sickle cell trait and are not by steplike depressions of the osseous surface, the H
characteristic of thalassemia or iron deficiency anemia. vertebra, is a relatively specific radiographic sign of
Osteonecrosis of the epiphyses may be more frequent in anemia. It has been noted rarely in other conditions. An
TABLE 48–1
Characteristic Skeletal Findings in the Anemias
C D
Specific Sites of Involvement. In the skull, numerous Figure 49–3. Plasma cell myeloma: diffuse osteopenia.
discrete lytic areas of uniform size are more common in Radiograph of the pelvis reveals increased radiolucency of the
plasma cell myeloma than in skeletal metastasis. Myeloma skeleton and a coarsened trabecular pattern. No evidence of
discrete lytic lesions can be seen. The pattern resembles that in
may demonstrate a predilection for mandibular involve- osteoporosis or osteomalacia.
ment, an uncommon site for skeletal metastasis. Sternal
involvement in myeloma is not infrequent and may lead
to pathologic fracture. In the spine, preferential destruc-
tion of the vertebral bodies, with sparing of the posterior
elements, has been emphasized. Paraspinal and extradural
extension of tumor is quite characteristic of myeloma. Pathologic Features
Preferential involvement of the distal end of the clavicle, Myelomatous involvement of the skeleton is variable in
acromion, glenoid, and ulnar olecranon is seen, whereas extent and predominates in regions of red marrow.
this distribution is less common in metastatic disease Extraosseous involvement is most common in the spleen,
involving the skeleton. liver, and lymph nodes. Pathologic fractures are frequent,
Chapter 49 Plasma Cell Dyscrasias and Dysgammaglobulinemias 655
Computed Tomography
Figure 49–6. Plasma cell myeloma: CT abnormalities in the
In contrast to plain film radiography, in which extensive skull. Observe the well-defined radiolucent lesions involving
osseous destruction is required before abnormalities the diploic portion and both tables of the skull.
656 Section XIII Diseases of the Hematopoietic System
A B C D
Figure 49–8. Plasma cell myeloma: MR imaging abnormalities in the spine. Sagittal T1-weighted
(TR/TE, 350/20) spin echo MR images show four patterns of marrow involvement seen in patients with
monoclonal gammopathies. A, Normal-appearing marrow (62-year-old woman). Note the absence of diffuse
or focal abnormalities of signal intensity. One possible lesion is seen in the second lumbar vertebral body,
and loss of height of the third lumbar vertebral body is evident. B, Focal lesions (54-year-old woman).
Several foci of low signal intensity are seen in the second and third lumbar vertebral bodies, and collapse of
the superior endplate of the fifth lumbar vertebral body is evident. C, Focal lesions (45-year-old man).
Numerous foci of low signal intensity are seen in every visualized vertebral body. D, Diffuse infiltration (50-
year-old man). The signal intensity of the vertebral bodies is lower than that of the intervertebral discs.
(From Lecouvet FE, Malghem J, Michaux L, et al: Vertebral compression fractures in multiple myeloma.
Part II. Assessment of fracture risk with MR imaging of spinal bone marrow. Radiology 204:201, 1997.)
Chapter 49 Plasma Cell Dyscrasias and Dysgammaglobulinemias 657
markedly when a gadolinium-based contrast agent is Clinical and Prognostic Value. Although MR imaging
administered intravenously to young children, such findings in cases of plasma cell myeloma lack specificity,
enhancement has been reported to be subtle or absent in their potential role in providing information about the
adults. In plasma cell myeloma, especially in cases of stage and prognosis of the disease has been emphasized.
more aggressive disease, the degree of enhancement of Whereas conventional radiography was used in the past
signal intensity in involved marrow tends to be greater to determine tumor mass in myeloma patients, compara-
than that of normal marrow (Fig. 49–9). Further, tive studies of MR imaging, routine radiography, and
gadolinium-related diffuse enhancement of marrow in scintigraphy in this clinical setting clearly indicate that
vertebral bodies occurs not only in myeloma but also in MR imaging results in the highest detection rate. In
lymphomas, leukemias, and myelofibrosis. general, the frequency of abnormal MR imaging findings
Short tau inversion recovery (STIR) sequences may in patients with plasma cell myeloma increases with
allow the detection of small focal collections of tumor that advancing stages of the disease. In reports of patients with
escape visualization with standard spin echo MR imaging stage III myeloma, MR imaging indicated the presence of
methods. Other fat-suppression techniques, such as marrow abnormalities in approximately 80% of cases.
chemical shift imaging, can also be used in patients with
plasma cell myeloma and may be combined with the use Vertebral Collapse. Collapse of one or more vertebral
of intravenous gadolinium-based contrast agents, although bodies is a common and well-recognized complication of
the value of these modified MR imaging methods has yet plasma cell myeloma. Although MR imaging criteria
to be proved in patients with this disease. have been developed with regard to the differentiation of
A B
nontumorous and tumorous collapse of vertebral bodies cystic expansile lesion with thickened trabeculae or a
(see Chapters 41 and 72), these criteria may be less purely osteolytic focus without expansion may be observed
valuable in cases of myeloma. Specifically, the preserva- (Figs. 49–10 and 49–11). Sclerotic lesions have also been
tion of signal intensity typical of fat in collapsed vertebral identified; in fact, solitary plasmacytoma may appear as a
bodies, generally believed to indicate a nontumorous radiodense vertebral body, the ivory vertebra (Fig.
pathogenesis, has been noted in cases of myelomatous 49–12). Calcification or even ossification in plasmacy-
infiltration. Whether specific MR imaging patterns of tomas may indicate amyloid deposition, and the resulting
vertebral involvement in plasma cell myeloma are radiographic appearance can simulate that of osteosar-
predictive of fracture risk is not clear. coma or chondrosarcoma.
Solitary plasmacytoma of the spine deserves special
Extraspinal Sites of Involvement. Even though existing emphasis. This diagnosis must be considered in any
data relate mainly to the assessment of spinal involve- middle-aged or elderly patient with a single osteolytic
ment in plasma cell myeloma, MR imaging can also be lesion of a vertebral body or, less commonly, a vertebral
applied to the evaluation of disease presence and extent arch. An involved vertebral body may collapse and
in extraspinal sites and to the analysis of solitary or disappear completely, or the lesion may extend into the
multifocal plasmacytomas. spinal canal or across the intervertebral disc and invade
the adjacent vertebral body.
Plasmacytoma
Myeloma can occur as a solitary lesion of bone. Although Rheumatologic Manifestations
laboratory analysis may reveal the same abnormalities that
occur in disseminated myelomatosis, this is not always Neurologic Findings. Sciatica and brachial neuralgia
the case. In some patients with solitary plasmacytoma, related to nerve root pressure are encountered in patients
serologic tests are negative, or abnormal patterns of serum with spinal disease. In addition, patients with sclerotic
electrophoresis disappear after excision of the tumor. In plasma cell myeloma (or, rarely, osteolytic myeloma) may
many cases of apparently solitary plasmacytoma, multiple have an associated peripheral neuropathy. It has been
lesions eventually appear if the patient is monitored long suggested that peripheral neuropathy may be related to
enough; in fact, some investigators believe that all dysglobulinemia. It occurs in less than 1% of plasma cell
patients with plasmacytoma will eventually demonstrate neoplasms.
multiple lesions.
Compared with multiple myeloma, solitary plasmacy- POEMS Syndrome. A syndrome of plasma cell dyscrasia,
toma is rare (representing <5% of plasma cell dyscrasias), chronic progressive polyneuropathy, and endocrine
affects younger patients (average age, about 50 years), is disturbances, including diabetes mellitus, has been
commonly accompanied by neurologic manifestations, recognized (Fig. 49–13). To facilitate recognition of the
and can simulate giant cell tumor on radiographic exami- most constant features of this syndrome—polyneuropathy
nation. Plasmacytoma is most common in the spine (P), organomegaly (O), endocrinopathy (E), M proteins
(especially the thoracic and lumbar segments) and pelvis, (M), and skin changes (S)—the acronym POEMS has
although it may occur at many other axial and extra-axial been suggested. M protein abnormalities, if present, are
sites. The radiographic features are variable. A multi- lambda light chains, unlike the kappa light chains evident
A B
Figure 49–10. Plasmacytoma: spine. Progressive changes in the spine in a 37-year-old man with low back
pain. A, The initial radiograph demonstrates increased radiolucency of the third lumbar vertebral body, with
slight compression of its superior surface. B, Two years later, the lesion has progressed and appears “bubbly”
or septated.
Chapter 49 Plasma Cell Dyscrasias and Dysgammaglobulinemias 659
B
elements of the spine, including the articular, transverse,
Figure 49–11. Plasmacytoma: pelvis. This 34-year-old man and spinous processes. Similar findings are observed
had left hip pain that progressed over a 2-year period. A, about the sacroiliac and costovertebral joints. The cause
Radiograph reveals an expansile trabeculated lesion of the of this syndrome is not known.
ischium. B, Transaxial CT image at the level of the ischial
tuberosity, obtained at the same time as (A), reveals the Polyarthritis and Amyloid Deposition. Plasma cell myeloma
expansile lesion, cortical thinning and perforation, and absence may manifest as a polyarthritis that superficially resembles
of a soft tissue mass. Open biopsy confirmed the diagnosis of rheumatoid arthritis. These articular manifestations can
plasmacytoma. (Courtesy of G. Greenway, MD, Dallas, Tex.) precede any overt manifestation of myelomatosis or
develop after the onset of the latter disease.
Amyloidosis occurs in approximately 15% of patients
with plasma cell myeloma. The distribution of amyloid in
in classic myeloma. Additional characteristics of this these patients resembles that in primary amyloidosis. In
syndrome are thickening and pigmentation of the skin fact, differentiation of myeloma with coexistent amyloi-
(sometimes resembling scleroderma), cutaneous angiomas, dosis from primary amyloidosis may be extremely diffi-
edema, excess perspiration, hirsutism, impotence, gyneco- cult. It appears that most patients with plasma cell
mastia or amenorrhea, thrombocytosis, and hepato- myeloma and polyarthritis have coexistent amyloidosis.
splenomegaly. Affected patients do not have associated These patients must be distinguished, however, from those
amyloidosis and rarely exhibit Bence Jones proteinuria. with plasma cell myeloma and coincidental rheumatoid
The syndrome is more frequent in men and usually has arthritis, from patients with primary amyloidosis and
its onset at a young age. Sclerotic plasmacytomas are joint manifestations, and from those with rheumatoid
evident in most cases, particularly in the spine and pelvis. arthritis and serum M components without myeloma or
The lesions may be uniformly sclerotic or appear target- amyloidosis.
like, with a peripheral margin of bone sclerosis. Ivory Rarely, a circumscribed mass of amyloid may be iden-
vertebral bodies may be evident, and areas with features tified within a plasmacytoma in the skeleton. Its presence
similar to those of bone islands may be seen. Bone can be suspected when areas of calcification or ossifica-
proliferation is apparent at sites of tendon and ligament tion are evident in the lesion. The resulting radiographic
attachment to bone. Particularly characteristic are abnormalities resemble those of osteosarcoma or chondro-
irregular bony excrescences involving the posterior sarcoma (Fig. 49–14).
660 Section XIII Diseases of the Hematopoietic System
Percutaneous Vertebroplasty
Percutaneous vertebroplasty is a recently introduced
technique for reducing pain and strengthening bone in
patients with plasma cell myeloma (or skeletal metastasis)
involving the spine, as well as in patients with osteo-
porotic vertebral collapse. This method entails the injec-
tion, usually with fluoroscopic or CT guidance, of a
polymer of methylmethacrylate through a large needle
into the weakened vertebral body. The pain relief provid-
ed by percutaneous vertebroplasty is frequent and often
dramatic; it may be related to tumor necrosis, destruction
of sensory nerve endings, stabilization of microfractures,
or a combination of these mechanisms. The degree of
pain relief is independent of the percentage of the lesion
that is filled with methylmethacrylate during the injec-
tion procedure. Certain patterns of extraosseous leakage
Figure 49–13. Syndrome of plasma cell dyscrasia, polyneu- of methylmethacrylate, such as foraminal or spinal canal
ropathy, and endocrine disturbances (POEMS syndrome). On a
leakage, may require decompressive surgery because of
lateral radiograph of the lumbar spine, observe the peculiar
proliferative changes about the apophyseal joints (arrowheads) the occurrence of neurologic symptoms and signs.
and, to a lesser extent, the discovertebral junctions (arrows).
The bones appear radiodense. Differential Diagnosis
Widespread osteolytic lesions in a middle-aged or elderly
patient should arouse suspicion of myeloma or skeletal
metastasis, and differentiation of the radiographic
features of these two disorders causes some difficulty
(Table 49–1). Certain features favor the diagnosis of
myeloma, including symmetrically distributed lesions of
equal size without adjacent sclerosis; subcortical lucent
areas with endosteal scalloping; involvement of the
mandible, shoulder girdle, and elbow region; and spinal
lesions with preservation of the pediculate shadow. None
of these characteristics is pathognomonic of myeloma,
however. Other disorders may also be associated with
widespread osteolysis, including macroglobulinemia,
leukemia, histiocytosis, Gaucher’s disease, vascular tumor,
infection, fibrous dysplasia, hyperparathyroidism, pan-
creatitis with fat necrosis, and Weber-Christian disease.
Diffuse osteosclerosis is unusual in plasma cell myeloma.
When present, the pattern simulates that associated with
myelofibrosis, osteoblastic metastasis, sickle cell anemia,
Paget’s disease, fibrous dysplasia, lymphoma, mastocytosis,
tuberous sclerosis, renal osteodystrophy, and sarcoidosis.
Single (or several) lytic foci in myeloma are difficult to
Figure 49–14. Plasmacytoma with amyloid deposition. distinguish from many other primary and secondary skele-
Biopsy of solitary lesion of the skeleton documented the tal neoplasms and infections. Expansile myelomatous
presence of a plasmacytoma with amyloid deposition. lesions can simulate skeletal metastasis (particularly from
Transaxial CT image shows the extent of bone destruction and thyroid and renal carcinoma), brown tumors of hyperpara-
the presence of calcification (arrow). (Courtesy of J. Castello, thyroidism, fibrous dysplasia, angiomatous lesions, hemo-
MD, Madrid, Spain.) philic pseudotumors, and several primary bone tumors.
Chapter 49 Plasma Cell Dyscrasias and Dysgammaglobulinemias 661
TABLE 49–1
Plasma Cell Myeloma versus Skeletal Metastasis
WALDENSTRÖM’S MACROGLOBULINEMIA
Macroglobulinemia is a disorder of middle-aged and
elderly persons. It is a disease of differentiating B
lymphocytes that is associated with the production of
monoclonal macroglobulins. Symptoms include weakness,
weight loss, bleeding diathesis, dyspnea, and personality
changes; physical findings include retinal hemorrhage,
hepatosplenomegaly, and lymphadenopathy. Laboratory
analysis reveals anemia, an elevated sedimentation rate,
increased serum viscosity, hyperglobulinemia, and increased
cerebrospinal fluid protein. Cellular infiltration (lympho-
cytes, plasma cells, histiocytes, and mast cells) into various
organs accounts for the clinical and radiographic mani-
festations of this disease.
Skeletal Manifestations
Skeletal findings in Waldenström’s macroglobulinemia
are similar to those of multiple myeloma. Osteopenia,
widening of the marrow spaces, and endosteal erosion are
evident. The remaining trabeculae may be coarsened and
result in focal osteosclerosis. Vertebral collapse is encoun-
tered. Osteolytic lesions may also be observed, with a Figure 49–15. Waldenström’s macroglobulinemia. Observe
reported frequency of 10% to 15% (Fig. 49–15). Involve- the osteolytic lesions of the para-acetabular region.
ment of the pelvis, perhaps with a predilection for the
para-acetabular regions, is not uncommon. Large lesions
in periarticular locations may result in joint destruc- synovial thickening, and osseous erosions. Secondary gouty
tion. MR imaging of spinal lesions in Waldenström’s arthritis can be observed in patients with Waldenström’s
macroglobulinemia shows diffuse or patchy patterns of macroglobulinemia.
marrow involvement, similar to those of plasma cell
myeloma. Ischemic necrosis of the femoral or humeral
AMYLOIDOSIS
heads, which could be related to the hyperviscosity of
blood, may be an additional manifestation of Waldenström’s Amyloidosis can be classified as primary, secondary,
macroglobulinemia. heredofamilial, senile, or localized amyloid tumors, based
primarily on clinical parameters. In addition, in patients
undergoing chronic hemodialysis, amyloid deposition
Rheumatologic Manifestations consists of beta2-microglobulin, which in rare circum-
As in myeloma with secondary amyloidosis, the resulting stances can accumulate in the tissues of patients without
arthropathy may simulate rheumatoid arthritis because a history of renal disease or hemodialysis. This last type
of the presence of subcutaneous nodules, symmetrical of amyloidosis is discussed in detail in Chapter 46.
662 Section XIII Diseases of the Hematopoietic System
Primary Amyloidosis. The primary form of amyloidosis Although no laboratory findings are pathognomonic
occurs without coexistent or antecedent disease. Most of amyloidosis, the diagnosis can be substantiated by
frequently it involves certain mesenchymal structures performance of the Congo red test. The gingiva, rectum,
such as the heart, muscle, tongue, synovial membrane, liver, spleen, kidney, small intestine, and skin have been
and perivascular connective tissue. In many patients, used for diagnostic biopsy, although aspiration biopsy of
primary amyloidosis is associated with multiple myeloma, the subcutaneous abdominal fat pad has become the
which is generally nearly simultaneous in onset. method of choice.
TABLE 49–2
Radiographic Manifestations of Musculoskeletal
Involvement in Amyloidosis
Osteoporosis
Lytic lesions
Pathologic fractures
Osteonecrosis
Soft tissue nodules and swelling
Subchondral cysts and erosions
Figure 49–17. Amyloidosis: soft tissue abnormalities. Joint subluxations and contractures
Radiograph shows soft tissue swelling and associated osteo- Neuropathic osteoarthropathy
porosis of underlying bones.
664 Section XIII Diseases of the Hematopoietic System
ment of leptomeningeal deposits of amyloid with the use mon disorders, particularly skeletal metastasis and plasma
of gadolinium-containing contrast agents has been cell myeloma. Bone lysis in Waldenström’s macroglobu-
noted, however (Fig. 49–18). linemia is also virtually identical to that accompanying
amyloidosis.
Localized destructive lesions of the skeleton in
Differential Diagnosis amyloidosis resemble sites of metastasis or a primary
Bone Lesions. Diffuse lytic lesions in amyloidosis are bone neoplasm. A soft tissue mass (which may contain
indistinguishable from those accompanying more com- calcifications) is common, but periostitis is rare.
A B
C D
Figure 49–18. Dialysis-related amyloidosis: soft tissue masses and joint destruction. In this patient with
renal disease who was receiving long-term hemodialysis, pain and swelling developed about multiple joints,
and a gradually enlarging soft tissue mass appeared around the shoulder. A, Anteroposterior radiograph of
the shoulder shows multiple well-defined erosions in the humeral head and acromion. B and C, Transverse
T1-weighted (TR/TE, 650/14) (B) and fat-suppressed gadolinium-enhanced T1-weighted (TR/TE,600/14)
(C) spin echo MR images show multiple enhancing soft tissue masses (asterisks). Note the associated osseous
erosions (arrows) and enhancing synovitis. D, Coronal conventional T2-weighted (TR/TE, 2000/80) spin
echo MR image shows the masses (asterisks) to have an inhomogeneous but mainly low signal intensity. Note
the massive rotator cuff tear with associated synovitis and fluid.
Chapter 49 Plasma Cell Dyscrasias and Dysgammaglobulinemias 665
Articular Lesions. Articular lesions in amyloidosis are Grossman RE, Hensley GT: Bone lesions in primary amyloi-
characterized by bulky soft tissue masses, well-defined dosis. AJR Am J Roentgenol 101:872, 1967.
erosions and cysts, and preservation of the joint space. Grover SB, Dhar A: Imaging spectrum in sclerotic myelomas:
These features can usually be distinguished from those of An experience of three cases. Eur Radiol 10:1828, 2000.
Heiser S, Schwartzman JJ: Variation in the roentgen appearance
rheumatoid arthritis, which is associated with diffuse or
of the skeletal system in myeloma. Radiology 58:178, 1952.
fusiform soft tissue swelling, early joint space loss, and Jacobson HG, Poppel MH, Shapiro JH, et al: The vertebral
marginal erosions of bone. Amyloid joint disease shares pedicle sign: A roentgen finding to differentiate metastatic
many radiographic characteristics with gouty arthritis carcinoma from multiple myeloma. AJR Am J Roentgenol
and xanthomatosis, but the clinical and laboratory 80:817, 1958.
manifestations of these latter disorders usually ensure an Katz GA, Peter JB, Pearson CM, et al: The shoulder-pad
accurate diagnosis. The arthropathy of amyloidosis also sign—a diagnostic feature of amyloid arthropathy. N Engl J
resembles pigmented villonodular synovitis; some Med 288:354, 1973.
distinguishing features of the former include multiple Lecouvet FE, Malghem J, Michaux L, et al: Vertebral compres-
sites of involvement, juxta-articular osteoporosis, and sion fractures in multiple myeloma. Part II. Assessment of
fracture risk with MR imaging of spinal bone marrow.
older patient age.
Radiology 204:201, 1997.
Libshitz HI, Malthouse SR, Cunningham D, et al: Multiple mye-
loma: Appearance at MR imaging. Radiology 182:833, 1992.
FURTHER READING Meszaros WT: The many facets of multiple myeloma. Semin
Roentgenol 9:219, 1974.
Abdelwahab IF, Miller TT, Hermann G, et al: Transarticular Murata H, Kusazaki K, Hashiguchi S, et al: Bilateral
invasion of joints by bone tumors: Hypothesis. Skeletal metachronous periosteal tibial amyloid tumors. Skeletal
Radiol 20:279, 1991. Radiol 29:346, 2000.
Bardwick PA, Zvaifler NJ, Gill GN, et al: Plasma cell dyscrasia Narvaez JA, Majos C, Narvaez J, et al: POEMS syndrome:
with polyneuropathy, organomegaly, endocrinopathy, M Unusual radiographic, scintigraphic and CT features. Eur
protein, and skin changes: The POEMS syndrome. Medicine Radiol 8:134, 1998.
59:311, 1980. Reinus WR, Kyriakos M, Gilula LA, et al: Plasma cell tumors
Barnett EV, Winkelstein A, Weinberger HJ: Agammaglobu- with calcified amyloid deposition mistaken for chondro-
linemia with polyarthritis and subcutaneous nodules. Am J sarcoma. Radiology 189:505, 1993.
Med 48:40, 1970. Renner RR, Nelson DA, Lozner EL: Roentgenologic manifes-
Bataille R, Chevalier J, Rossi M, et al: Bone scintigraphy in tations of primary macroglobulinemia (Waldenström). AJR
plasma cell myeloma: A prospective study of 70 patients. Am J Roentgenol 113:499, 1971.
Radiology 145:801, 1982. Renner RR, Smith JR: Plasma cell dyscrasias (except myeloma).
Buckley RH: Specific immunodeficiency diseases, excluding Semin Roentgenol 9:209, 1974.
acquired immunodeficiency syndrome. In Kelly WN, Harris Resnick D, Greenway GD, Bardwick PA, et al: Plasma-cell dys-
ED Jr, Ruddy S, Sledge CB (eds): Textbook of Rheuma- crasia with polyneuropathy, organomegaly, endocrinopathy,
tology, 5th ed. Philadelphia, WB Saunders, 1997, p 1282. M-protein, and skin changes: The POEMS syndrome.
Clarisse PDT, Staple TW: Diffuse bone sclerosis in multiple Radiology 140:17, 1981.
myeloma. Radiology 99:327, 1971. Subbarao K, Jacobson HG: Amyloidosis and plasma cell dys-
Fahey JL, Barth WF, Solomon A: Serum hyperviscosity crasias of the musculoskeletal system. Semin Roentgenol
syndrome. JAMA 192:464, 1965. 21:139, 1986.
Flores M, Nadarajan P, Mangham DC: Soft-tissue amyloidoma: Tong D, Griffin TW, Laramore GE, et al: Solitary plasma-
A case report. J Bone Joint Surg Br 80:654, 1998. cytoma of bone and soft tissues. Radiology 135:195, 1980.
Gootnick LT: Solitary myeloma: Review of sixty-one cases. Vermess M, Pearson KD, Einstein AB, et al: Osseous mani-
Radiology 45:385, 1945. festations of Waldenström’s macroglobulinemia. Radiology
Grayzel AI, Marcus R, Stern R, et al: Chronic polyarthritis 102:497, 1972.
associated with hypogammaglobulinemia: A study of two Weinfeld A, Stern MH, Marx LH: Amyloid lesions of bone.
patients. Arthritis Rheum 20:887, 1977. AJR Am J Roentgenol 108:799, 1970.
50
CHAPTER 50
Lipidoses, Histiocytoses, and TABLE 50–1
A B
668 Section XIII Diseases of the Hematopoietic System
mity. Spinal cord compression is rare. In the calvaria, long tubular bones is most frequent in the lower ends of
trabecular destruction and thinning of both the outer and both femoral shafts, particularly medially. It results in
inner tables can be seen. Mandibular involvement is not cortical thinning and loss of the normal concavity of the
unusual. bony outline. This appearance has been termed an
Erlenmeyer flask deformity (Fig. 50–3); it is very sug-
Fractures. Infiltration of the marrow spaces with gestive of the diagnosis of Gaucher’s disease, particularly
Gaucher cells and trabecular resorption produce osseous when associated with epiphyseal osteonecrosis.
weakening, which may result in pathologic fractures Peculiar steplike depressions of the superior and infe-
(Fig. 50–2). Most frequently, fractures appear in the rior margins of the vertebral bodies (Fig. 50–4) have been
vertebral column. Intraosseous disc displacement (carti- described in Gaucher’s disease and are identical to the
laginous or Schmorl’s nodes) and compression of the depressions typical of sickle cell anemia and other
vertebral bodies are seen. The involved vertebral body hemoglobinopathies. This deformity, which is called the
may become completely flattened (vertebra plana). This H vertebra, has been attributed to ischemic growth distur-
appearance, which is identical to that accompanying the bance at the chondro-osseous junction.
histiocytoses, may be evident at multiple levels and is
associated with paravertebral soft tissue swelling. Frac- Osteonecrosis. Osteonecrosis of the epiphyses and dia-
tures are also observed in the ribs and in the long and physes is well recognized in Gaucher’s disease (Fig. 50–5).
short tubular bones of the appendicular skeleton, parti- Risk factors may include previous splenectomy and male
cularly the femur, tibia, and humerus. These fractures gender. In the shafts of long bones, alternating radiolu-
may appear in the diaphyses or the epiphyses, although cency and sclerosis are seen, with associated periostitis;
in the latter location, their occurrence may reflect under- this bone-within-bone appearance is identical to that seen
lying osteonecrosis. in sickle cell anemia.
Epiphyseal bone necrosis can be visualized in the
Modeling Deformities. One of the most characteristic femoral head, humeral head, and tibial plateau. The result-
osseous manifestations of chronic Gaucher’s disease is ing radiographic picture of osteonecrosis is identical to
modeling deformities. Expansion of the contour of the that accompanying many other diseases.
Figure 50–2. Gaucher’s disease: fractures. Vertebral fracture Figure 50–3. Gaucher’s disease: modeling deformities.
and collapse are particularly frequent. In this case, marrow Erlenmeyer flask deformity of the distal end of the femur is
replacement has led to the dramatic collapse of multiple verte- observed. Note the expansion of the contour of the long tubular
bral bodies, some of which have been reduced to a flattened bone, with straightening and convexity of the osseous margin,
structure (vertebra plana). particularly along the medial aspect of the metaphysis.
Chapter 50 Lipidoses, Histiocytoses, and Hyperlipoproteinemias 669
show considerable variability, the T2 values of hyper- within-bone appearance, a finding that is not apparent in
plastic normal marrow are also variable, and high signal these other disorders. Similar changes, however, can be
intensity on T2-weighted spin echo MR sequences in the seen in sickle cell anemia and its variants; this similarity
bone marrow of patients with Gaucher’s disease has been is related to the occurrence of bone infarction and necrosis
encountered. in these anemias and in Gaucher’s disease. Epiphyseal
MR imaging can also be used effectively to study the osteonecrosis, particularly of the femoral head, which is
skeletal complications that occur in Gaucher’s disease. seen in both Gaucher’s disease and hemoglobinopathies,
Bone marrow ischemia and necrosis, osteomyelitis, and is also apparent in Cushing’s syndrome and exogenous
vertebral collapse are among the complications that may hypercortisolism, pancreatitis, caisson disease, collagen
be assessed with MR imaging (Fig. 50–8). vascular disorders, and numerous other diseases.
An Erlenmeyer flask appearance is seen in Gaucher’s
disease and other disorders (Table 50–2), including cer-
Differential Diagnosis
tain anemias, fibrous dysplasia, Niemann-Pick disease,
The generalized increased radiolucency (osteopenia) seen (cranio)metaphyseal dysplasia (Pyle’s disease), heavy
in Gaucher’s disease is also apparent in a variety of meta- metal poisoning, and osteopetrosis. In many of these
bolic (osteoporosis, osteomalacia, hyperparathyroidism), diseases, other findings allow an accurate diagnosis. The
hematologic (sickle cell anemia, thalassemia), and neo- diagnosis of Gaucher’s disease should be considered in any
plastic (plasma cell myeloma, leukemia) disorders. In patient with hepatosplenomegaly who has widespread
Gaucher’s disease, localized lucent areas and cystic lesions osteopenia with a coarsened trabecular pattern, focal osteo-
producing a honeycomb appearance may simulate the sclerosis, ischemic necrosis, and flaring or Erlenmeyer
findings of plasma cell myeloma, amyloidosis, and skeletal flask deformities of the distal portions of the femora.
metastasis.
Generalized or localized osteosclerosis is likewise not NIEMANN-PICK DISEASE
specific for Gaucher’s disease and may be seen in skeletal
metastasis, tuberous sclerosis, mastocytosis, myelofibrosis, General Features
and Hodgkin’s disease. In Gaucher’s disease, the sclerosis Niemann-Pick disease is a rare, genetically determined
includes a coarsened trabecular pattern and a bone- disorder characterized by the widespread accumulation
A B
Figure 50–7. Gaucher’s disease: MR imaging abnormalities. Marrow infiltration has occurred in this 30-
year-old man with type 1 disease. Corresponding coronal T1-weighted (TR/TE, 566/11) (A) and fast T2-
weighted (TR/TE, 5000/108) (B) spin echo MR images show replacement of the normal marrow with a
signal intensity similar to that of muscle on both pulse sequences.
Chapter 50 Lipidoses, Histiocytoses, and Hyperlipoproteinemias 671
Type E. This is an indeterminate form that occurs in
adults. There is visceral involvement and questionable
sphingomyelinase deficiency.
Clinical Features
The clinical manifestations vary with the specific disease
type. In general, symptoms and signs become apparent
during infancy, progress rapidly, and lead to the patient’s
demise. Findings in these infants may include jaundice,
hepatosplenomegaly, abdominal enlargement, lymphaden-
opathy, emaciation, and decreased visual acuity or blind-
ness. In some patients, clinical manifestations may not be
detected until later in childhood, and affected children
may live into adolescence.
Musculoskeletal Abnormalities
Figure 50–8. Gaucher’s disease: MR imaging abnormalities.
Marrow infiltration and osteonecrosis are evident in this 31- Lipid-containing foam cells accumulate in the bone marrow
year-old woman. Coronal T1-weighted (TR/TE, 600/20) spin and produce abnormalities in the adjacent bony trabeculae
echo MR image shows abnormally low signal intensity in the (Fig. 50–9). These changes are particularly frequent in
marrow of the femora and innominate bones. Note the children who have a type of Niemann-Pick disease that is
deformity of the right femoral head, indicative of osteonecrosis. compatible with longer life (type B). On radiographs, this
combination of spongiosa and cortical abnormalities results
in increased osseous radiolucency and medullary widen-
of lipid, particularly sphingomyelin, in the body. Niemann- ing with cortical diminution, findings similar to those of
Pick disease is not a single entity; five types have been Gaucher’s disease. Modeling deformities in Niemann-Pick
reported. disease are also similar to those of Gaucher’s disease and
consist of straightening and convexity of the distal femoral
Type A. This acute neuronopathic form, often observed contour. As in Gaucher’s disease, MR imaging is effective
in Ashkenazic Jews, is characterized by a rapidly fatal in documenting marrow infiltration in the long tubular bones.
disease of infancy, with involvement of viscera and the
nervous system.
TABLE 50–2
Conditions Characterized by Erlenmeyer Flask
Deformity
Gaucher’s disease
Niemann-Pick disease
Anemias
Fibrous dysplasia
Metaphyseal dysplasia (Pyle’s disease)
Osteopetrosis
Heavy metal poisoning Figure 50–9. Niemann-Pick disease: marrow infiltration.
The distal femoral shaft is both osteopenic and expanded.
672 Section XIII Diseases of the Hematopoietic System
TABLE 50–5
Differential Diagnosis of Multicentric Reticulohistiocytosis
Intra-
Involvement articular Soft
Symmetrical of DIP Marginal Osteo- Bony Atlantoaxial Tissue
Involvement Joints Erosions porosis Ankylosis Subluxation Nodules
Multicentric reticulohistiocytosis + + + – – + +
Rheumatoid arthritis + – + + – + +
Psoriatic arthritis ± + + – + + –
Inflammatory (erosive) osteoarthritis + + ± – + – –
Gouty arthritis ± + + – – – +
associated with osteophytosis and intra-articular bony histiocytosis is also difficult on the basis of histologic
ankylosis. These latter features are not part of the manifestations. Additionally, in all three entities, specific
radiographic picture of multicentric reticulohistiocytosis. histiocytic cells (Langerhans cells) containing cytoplas-
Distal interphalangeal joint erosions are unusual in mic inclusion bodies (Langerhans granules, or X bodies)
scleroderma and, when present, are associated with more are found. Langerhans cells are believed to be virtually
typical findings, such as tuftal resorption and soft tissue diagnostic of the histiocytoses.
calcification. Despite the presence of Langerhans cells in the three
Gouty arthritis is characterized by soft tissue nodular classic forms of histiocytosis, many observers maintain
masses, sharply marginated erosions, and lack of osteo- that the three disorders are unrelated and emphasize their
porosis, radiographic findings identical to those of multi- differing clinical manifestations. Early and diffuse organ
centric reticulohistiocytosis. Preservation of articular space involvement and rapid patient demise, characteristics
is noted in gout and may be apparent in multicentric of Letterer-Siwe disease, support its classification as a
reticulohistiocytosis, particularly in the early phases of malignant lymphoma, although the Langerhans cells,
the disease. An asymmetrical distribution, partially calci- which are unreactive with antilymphocytes, appear to be
fied soft tissue masses, and bone production with over- of other than lymphatic or myelocytic origin. On the
hanging edges are findings of gout that are not apparent other side of the spectrum, eosinophilic granuloma is
in multicentric reticulohistiocytosis. certainly benign in its clinical characteristics and resem-
bles an inflammatory process more than a neoplasm.
Hand-Schüller-Christian disease is believed by some
LANGERHANS CELL HISTIOCYTOSIS investigators to be a multifocal variety of eosinophilic
(HISTIOCYTOSIS X) granuloma. The precise cause and pathogenesis of
Langerhans cell histiocytosis remain unclear, however.
General Features
Evidence exists that the disorder is a manifestation of or
The three major conditions in the category of Langerhans is associated with immunologic aberrations.
cell histiocytosis are eosinophilic granuloma, Hand-Schüller-
Christian disease, and Letterer-Siwe disease. The term
histiocytosis X has been used as a comprehensive Eosinophilic Granuloma
designation for these three conditions, as an expression Eosinophilic granuloma is characterized by single or
of the same basic pathologic process underlying them. multiple skeletal lesions occurring predominantly in
Eosinophilic granuloma, the mildest form of histiocy- children, adolescents, or young adults, but occasionally in
tosis, manifests as single or multiple lesions of bone. older persons. It represents approximately 70% of the
Hand-Schüller-Christian disease, the most varied form, total number of cases of Langerhans cell histiocytosis; it
involves chronic dissemination of osseous lesions. is more common in men than in women and in whites
Letterer-Siwe disease, the acute form, is characterized by than in blacks. Clinical manifestations include local pain,
rapid dissemination and a poor clinical prognosis. tenderness, and swelling related to adjacent skeletal lesions.
Clear distinction among these entities on the basis of Fever and leukocytosis may also be apparent.
clinical and radiographic manifestations is not always Solitary lesions are more common than multiple lesions,
possible. Some patients initially have solitary bone lesions although it has been estimated that multifocal osseous
consistent with eosinophilic granuloma and subsequently (and extraosseous) disease eventually develops in about
develop more widespread skeletal and extraskeletal involve- 10% of patients with solitary lesions. In general, new
ment, consistent with Hand-Schüller-Christian disease. skeletal lesions occur within 1 or 2 years after the initial
Other patients have a fulminant onset consistent with solitary lesion is identified. The most common sites of
Letterer-Siwe disease that progresses to a more chronic involvement are the skull, mandible, spine, ribs, pelvis,
form. Clear separation among these three forms of and long bones, particularly the femur and humerus. In
Chapter 50 Lipidoses, Histiocytoses, and Hyperlipoproteinemias 675
older patients, changes in the flat bones are more com- such as Ewing’s sarcoma and lymphoma. In the ribs,
mon than changes in the tubular bones. Spinal involve- single or multiple lesions can lead to pathologic fractures
ment predominates in the thoracic and lumbar regions. (Fig. 50–12). In the skull and pelvis, the lytic areas may
Vertebral body localization is most typical. Involvement be particularly well defined, with or without surrounding
of the sternum and clavicles is also encountered. Epiphyseal sclerosis. A radiodense focus within a lytic cranial lesion
lesions may occur in children, and these lesions may cross (or, rarely, in another location) has been termed a “button”
the open physeal plate. sequestrum (Fig. 50–13). Skull lesions are especially
The radiographic characteristics of eosinophilic granu- prevalent in the frontal and parietal bones. Nonuniform
loma vary with its skeletal location. In long and short growth of the lesion leads to beveled bone margins and
tubular bones, the lesions appear as relatively well defined unequal destruction of the inner and outer tables of the
radiolucent areas, particularly in the medullary cavity vault (Fig. 50–14). Flat bone lesions may demonstrate
(Fig. 50–11). With further growth, they encroach on the a “hole within a hole” appearance (Fig. 50–15). In the
cortical bone, with endosteal erosion of the cortex and mandible, radiolucent lesions about the teeth may lead to
periosteal new bone formation. The appearance can loss of supporting bone and a “floating teeth” appearance.
simulate that of osteomyelitis or malignant neoplasm, Vertebral destruction can lead to a flattened vertebral
body, termed vertebra plana, a finding that is much more
frequent in children than in adults (Fig. 50–16). Thoracic
and lumbar spine involvement predominates. The height
of the intervening intervertebral disc is usually normal. A
paraspinal mass may be evident, simulating a soft tissue
abscess related to vertebral osteomyelitis. Rarely, neuro-
logic manifestations ensue.
Hand-Schüller-Christian Disease
The classic triad of Hand-Schüller-Christian disease is
diabetes insipidus, unilateral or bilateral exophthalmos,
and single or multiple areas of bone destruction. The
finding of diabetes insipidus is attributable to extensive
B
Figure 50–11. Eosinophilic granuloma: radiographic abnor-
malities in the tubular bones of the appendicular skeleton. A, In
this 33-year-old man, thigh pain developed that was worse at
night and relieved by aspirin. Radiograph reveals an eccentric Figure 50–12. Eosinophilic granuloma: radiographic abnor-
osteolytic lesion (arrow) with a sharp zone of transition between malities in the ribs. This 44-year-old woman had pleuritic chest
the abnormal and normal bone. Biopsy confirmed the presence pain of 1 month’s duration. Radiograph reveals a poorly defined
of an eosinophilic granuloma. B, In another patient, a lesion has osteolytic lesion. A pathologic fracture (arrowhead) extends across
violated the growth plate, with involvement of both the epiphy- the lesion. An eosinophilic granuloma was found on exami-
sis and the metaphysis. (A, Courtesy of P. Ellenbogen, MD, nation of the resected rib. (Courtesy of G. Greenway, MD,
Dallas, Tex. B, Courtesy of M. Murphey, MD, Washington, D.C.) Dallas, Tex.)
676 Section XIII Diseases of the Hematopoietic System
B C
Figure 50–13. Eosinophilic granuloma: radiographic abnormalities in the skull. A, Widespread lytic
lesions of variable size are observed (arrows). Most of them are sharply defined. B, Lytic lesion with a central
radiodense area, the “button” sequestrum. C, Computed tomography scan of the skull in a different patient
shows an osteolytic lesion (arrow) with a radiodense center representing the “button” sequestrum (arrow-
head). Note adjacent soft tissue prominence.
destruction of bone at the base of the skull and is not general, the disease runs a protracted course, sometimes
universally present in this disease. In fact, the classic triad extending over a period of 10 to 20 years.
may be apparent in less than 10% of patients. Hand- The skeletal lesions of Hand-Schüller-Christian
Schüller-Christian disease predominates in children disease may be widely disseminated (Fig. 50–17). The
(approximately two thirds of patients are younger than radiographic manifestations of individual lesions are
5 years at the initial clinical examination). similar to those of eosinophilic granuloma. In the skull,
Clinical characteristics are otitis media; cutaneous which is involved in more than 90% of patients,
involvement consisting of eczema, xanthomatosis, and confluent areas of destruction may isolate islands of bone
soft tissue nodules; ulceration of the gums; lymphad- and create a geographic, or maplike, skull. With
enopathy; and hepatosplenomegaly. Visceral involvement mandibular involvement, osteolytic lesions can surround
of the lungs, kidneys, brain, liver, and spleen is observed. the teeth, giving a “floating teeth” appearance. A
Anemia, when present, is a grave prognostic sign. In diagnosis of Hand-Schüller-Christian disease or a related
Chapter 50 Lipidoses, Histiocytoses, and Hyperlipoproteinemias 677
Figure 50–14. Eosinophilic granuloma: radiographic abnor- Figure 50–16. Eosinophilic granuloma: vertebra plana.
malities. Unequal involvement of the tables of the skull gives Observe the flattened vertebral body with preserved adjacent
the margins of the lesion a “beveled edge” appearance. disc spaces.
multiple organ systems, and the disease is fatal in 10% to prognosis. The typical healing response of spinal lesions
30% of cases. that have produced vertebra plana is partial reconstitu-
tion of vertebral height. The degree of recovery of verte-
bral body height is potentially greater when the patient
Letterer-Siwe Disease
is young.
Letterer-Siwe disease is a relatively acute syndrome that Intralesional injection of methylprednisolone sodium
is most frequent in children younger than 3 years, although succinate after bone biopsy has become a popular method
occasional cases are encountered in late childhood or of treatment of the histiocytoses. Progressive healing of
young adulthood. This disorder is characterized by histio- the osseous lesions generally becomes detectable radio-
cytic proliferation in multiple visceral organs. It accounts graphically after 2 to 4 months, as evidenced by a decrease
for approximately 10% of all cases of Langerhans cell in size, sclerosis of marginal bone, and increasing tra-
histiocytosis. beculation. Clinical improvement, manifested as diminu-
Clinical manifestations include febrile episodes, cachexia, tion of pain, accompanies the radiographic changes. The
hepatosplenomegaly, lymphadenopathy, purpuric skin mechanism of action of the corticosteroid preparation
eruption, hyperplasia of the gums, and progressive anemia. after intralesional administration is not clear, and the
The course is generally rapidly progressive; most patients possibility that the lesions will resolve spontaneously or
die within 1 or 2 years, although occasionally a more pro- after biopsy alone complicates the interpretation of the
longed clinical course, with features of Hand-Schüller- benefits of such a therapeutic procedure.
Christian disease, develops.
Single or multiple areas of bone destruction are
observed, particularly in the calvaria, base of the skull, Scintigraphy
and mandible. More diffuse skeletal involvement can be In general, in the evaluation of patients with Langerhans
encountered, although the hands and feet are usually cell histiocytosis, bone scintigraphy has proved to be less
spared. During treatment, extensive bone sclerosis may sensitive than radiography in the detection of osseous
be observed. Histologically and radiographically, the bone lesions, with falsely negative radionuclide studies com-
lesions simulate those in eosinophilic granuloma. monly reported. The purely lytic nature of the lesions
Osteolytic lesions, which are relatively well defined and is cited by some investigators as the reason for the
lack significant surrounding bony eburnation, represent disappointing results of bone scintigraphy. When bone
sites of histiocytic proliferation. scan findings are present, osseous lesions in histiocytosis
demonstrate a spectrum of radionuclide abnormalities,
ranging from “cold” areas with decreased or absent
Disease Prognosis and Evolution of Osseous
accumulation of the radionuclide to areas of augmented
Abnormalities
activity.
In general, the prognosis of any of the types of histio-
cytosis is related to the location and extent of organ
involvement, so the clinical, radiographic, and laboratory Magnetic Resonance Imaging
evaluation must include an examination of all potential Spin echo imaging of the skeleton in patients with histio-
target areas of disease. The greater the number of tissues cytosis reveals one or more foci of decreased signal inten-
or systems affected, the poorer the prognosis. Patients sity on T1-weighted images and increased signal intensity
with polyostotic disease tend to fare worse than those with on T2-weighted images (Fig. 50–18). Lesion enhance-
monostotic disease and are more likely to have visceral ment after the intravenous injection of a gadolinium con-
involvement. The prognosis of the histiocytoses is also trast agent may be seen. The MR imaging abnormalities
related to the age of the patient at the onset of clinical of the musculoskeletal system in Langerhans cell histio-
abnormalities; generally, the younger the patient, the cytosis include features that simulate those of aggressive
poorer the prognosis. tumors and infection. Cortical perforation, periostitis,
Healing of skeletal lesions, whether initiated through and soft tissue extension are known radiographic mani-
aggressive intervention or complete neglect, is accom- festations of eosinophilic granuloma and the other histio-
panied by characteristic radiographic changes. Bone scle- cytoses. The large size, poor definition, and signal
rosis typically develops in osteolytic lesions in extraspinal inhomogeneity of the soft tissue component accom-
locations during resolution, and the lesions may divide panying skeletal involvement in the histiocytoses must
into smaller isolated areas as a result of septation. Addi- be emphasized.
tional findings at these sites during the healing process The value of MR imaging of the musculoskeletal
include periosteal reaction, cortical thickening, and patho- system in patients with Langerhans cell histiocytosis lies
logic fracture, and the changes may resemble those of in its sensitivity rather than specificity. Although MR
Paget’s disease. Because sclerosis appears to represent the imaging is not a practical screening method for skeletal
hallmark of the healing response, its appearance in the involvement in these diseases, its usefulness in defining
form of a partial or complete rim of increased radio- the extent of involvement at one or a few skeletal sites,
density or patchy or diffuse bone reaction in untreated such as the spine, has been established. Problems exist,
lesions may indicate spontaneous resolution and should however, even in this regard, because it is difficult to
be regarded as a favorable prognostic sign. Conversely, determine the degree of cortical violation (a task better
enlargement of foci of osseous destruction or the appear- accomplished with computed tomography) and to distin-
ance of new areas of lysis is indicative of an unfavorable guish between histiocytic tissue and surrounding edema.
Chapter 50 Lipidoses, Histiocytoses, and Hyperlipoproteinemias 679
Figure 50–18. Eosinophilic granuloma: MR imaging abnormalities in the
femur. This 4-year-old girl experienced pain and swelling in the thigh. A,
Routine radiograph shows an osteolytic lesion with moth-eaten bone
destruction and thick periosteal reaction in the diaphysis of the femur. B,
Coronal T1-weighted (TR/TE, 500/20) spin echo MR image reveals low
signal intensity in the diaphyseal and distal metaphyseal portions of the
femur. Periosteal new bone also exhibits low signal intensity. C, With T2
weighting (TR/TE, 2000/90), the femoral lesion is inhomogeneous but
generally of high signal intensity. Note the elevated periosteum with new
bone formation and the irregular and extensive soft tissue component with
very high signal intensity. Differentiation of the lesion itself and adjacent
edema is impossible. (Courtesy of M. Pathria, MD, San Diego, Calif.)
B C
A B
TABLE 50–6
Hyperlipoproteinemias
Arthralgia Hyper-
Type Lipoprotein Abnormality Primary Form Secondary Forms Xanthomas or Arthritis uricemia
I Chylomicrons Autosomal recessive Diabetes mellitus Skin
Pancreatitis
Alcoholism
II Beta lipoprotein Autosomal dominant Hypothyroidism Skin +
Myeloma Tendinous
Macroglobulinemia Tuberous
Obstructive hepatic Subperiosteal
disease
III Beta or pre-beta lipoprotein Autosomal recessive (?) Diabetes mellitus Skin +
Hypothyroidism Tendinous
Tuberous
Subperiosteal
IV Pre-beta lipoprotein Autosomal recessive Diabetes mellitus Skin + +
Pancreatitis Tuberous
Alcoholism Intraosseous
Hypothyroidism
Glycogen storage
disease
Gaucher’s disease
Gout
Hypercalcemia
V Pre-beta lipoprotein, Autosomal dominant (?) Skin +
chylomicrons
Type II (Familial Hypercholesterolemia). Type II, the most both beta and pre-beta lipoproteins. In its primary form,
common type, is related to an increase in the plasma type II hyperlipoproteinemia is an autosomal dominant
concentration of low-density lipoproteins or beta lipo- hereditary abnormality. It may also be secondary to many
proteins. Type II is further divided into two subgroups: disorders, including hypothyroidism, plasma cell myeloma,
types IIA and IIB. The former is characterized by an macroglobulinemia, and obstructive liver disease. The
excess of beta lipoproteins, and the latter by an excess of diagnosis of primary type II hyperlipoproteinemia is
682 Section XIII Diseases of the Hematopoietic System
usually established in early childhood. Findings include endosteal erosion of the cortex, and even pathologic frac-
xanthomas and premature coronary, cerebral, and periph- ture or osteonecrosis from vascular occlusion.
eral vascular disease. In type I hyperlipoproteinemia, xanthomas are of the
eruptive type; they may appear at times of severe hyper-
Type III (“Broad-Beta” Disease). Type III, an uncommon lipidemia and disappear when the triglyceride level is
type, is associated with beta or pre-beta lipoprotein reduced with a low-fat intake.
abnormality. In its primary form, type III hyperlipopro- In type II hyperlipoproteinemia, xanthomas may occur
teinemia may be an autosomal recessive disorder. Rarely, in as many as 80% of patients before death. In persons
it is secondary to severe insulinopenic diabetes and hypo- who are homozygous with respect to the abnormal gene,
thyroidism. Primary type III hyperlipoproteinemia may xanthomatosis occurs in childhood and may be extensive.
not be detected until the third, fourth, or fifth decade of In heterozygotes, tendinous xanthomas do not usually
life, or even later. It is rarely observed in patients younger appear until age 30 years. In type III hyperlipoproteinemia,
than 25 years. Xanthomas and premature peripheral vas- tuberous, tendinous, and subperiosteal xanthomas may
cular disease are apparent. occur. In addition, lipid deposition (planar xanthoma)
may produce yellowish elevation on the palmar surface of
Type IV. Type IV is characterized by the presence of the hands and fingers, a distinctive clinical manifestation
increased low-density lipoproteins or pre-beta lipopro- that may also be seen in homozygous type II and possibly
teins without chylomicronemia. In its primary form, the type IV hyperlipoproteinemia. The frequency of planar
disease appears to be an autosomal recessive disorder. xanthomas in type III patients may reach 65% to 70%.
Type IV hyperlipoproteinemia may be secondary to many Tuberous and tendinous xanthomas produce nodular
diseases, including diabetes mellitus, pancreatitis, alco- masses in soft tissue and tendons (Fig. 50–21). These
holism, hypothyroidism, glycogen storage disease, Gaucher’s rarely calcify. Subtle thickening of the tendon may be the
disease, gout, and hypercalcemia. In its primary form, the only radiographic abnormality. Subperiosteal xanthomas
disease is rarely detected in those younger than 20 years. are associated with scalloping of the external cortical
Xanthomas, hyperuricemia, and coronary vascular disease surface. Intramedullary lipid deposition leads to lytic
may be evident. defects, endosteal erosion, subchondral collapse, juxta-
articular erosive changes, and pathologic fractures (Fig.
Type V. Type V is similar to type IV, with an increase in 50–22). In general, the osseous defects are well defined,
low-density lipoproteins and chylomicrons. In its primary with a sharp zone of transition between abnormal and
form, it is probably an autosomal dominant hereditary normal bone.
disease that is rarely clinically manifested in childhood. The diagnosis of soft tissue xanthomas, particularly
Xanthomas, hyperuricemia, abdominal pain, hepato- those of the Achilles tendon, can be accomplished with
splenomegaly, paresthesias, and lipemia retinalis may be ultrasonography and MR imaging. With ultrasonography,
detected. xanthomas appear as focal or confluent hypoechoic regions.
Musculoskeletal Abnormalities
Xanthoma
Xanthomas may be apparent in all five types of hyper-
lipoproteinemia and in primary and secondary forms of
the disease. Xanthomas can be classified as eruptive,
tendinous, tuberous, or subperiosteal and osseous.
Acute Acute
Donald Resnick and Parviz Haghighi Childhood Adult Chronic
Leukemia Leukemia Leukemia
Osteopenia ++ ++ +
Metaphyseal abnormalities ++ + –
SUMMARY OF KEY FEATURES
Osteolysis ++ ++ +
Musculoskeletal abnormalities accompany a variety Periostitis ++ + +
of myeloproliferative disorders. In leukemias, such Osteosclerosis + + +
abnormalities are particularly frequent in children. Articular abnormalities ++ + +
Osseous involvement from the primary disease leads
to local symptoms and signs. Synovial involvement
++, common; +, uncommon; –, absent.
from leukemic infiltration and hemorrhage can result
in articular findings. Hyperuricemia and secondary
gout can complicate this disease. Bone destruction and
secondary gout may also be observed in the lymphomas.
Sjögren’s syndrome is associated with an arthritis that undergo initial differentiation in the bone marrow. Thus,
usually appears identical to rheumatoid arthritis. leukemias can be classified not only on the basis of their
In systemic mastocytosis, characteristic skeletal acute or chronic nature but also according to their pre-
abnormalities include focal or diffuse lytic or sclerotic dominant cellular morphology and their T-cell or B-cell
lesions. In polycythemia vera, osteonecrosis, osteopenia, constituency.
and hyperuricemia may appear. Myelofibrosis may be Acute leukemia can affect both children and adults. In
accompanied by osteosclerosis, particularly in the axial children, acute leukemia is almost always lymphoblastic
skeleton. In addition, hyperuricemia and secondary gout in cell origin, and the survival time is limited to approxi-
are relatively common in this disorder. mately 1 year; in adults, acute leukemia is frequently
myeloid in cell origin. Chronic leukemia may be granu-
locytic or lymphocytic. The chronic types of leukemia
have a peak age of onset of 35 to 55 years and an average
survival time of approximately 3 years. The general clinical
INTRODUCTION manifestations of the leukemias are a consequence of the
Certain lymphoproliferative and myeloproliferative (i.e., distribution of abnormal cells in the various organs and
hematoproliferative) disorders are associated with definite tissues of the body.
skeletal manifestations, which may be an initial or domi-
nant part of the entire clinical picture. In some instances,
Acute Childhood Leukemia
musculoskeletal symptoms and signs related to the
primary disease process are responsible for these clinical Acute leukemia, the most common form of childhood
findings; in other instances, metabolic consequences of malignancy, is a disease of the first few years of life, with
the primary disease are responsible. the peak prevalence occurring between 2 and 5 years of
age. In some children, acute leukemia has a paucity of
LEUKEMIAS clinical signs, but this lack of signs is more typical of
acute myeloblastic leukemia than acute lymphoblastic
General Features leukemia, in which numerous clinical findings may be
It is convenient to divide the leukemias into acute and encountered, including lymphadenopathy and spleno-
chronic forms (Table 51–1). Acute leukemias are accom- megaly. Bone and joint pain, tenderness, and swelling,
panied by the accumulation of immature, or blast, cells as which are common findings in children with leukemia,
a result of a defect in the production of mature hemic may be confused with rheumatic fever, juvenile chronic
cells; chronic leukemias are accompanied by massive polyarthritis, or osteomyelitis. Arthralgias and arthritis
overgrowth of mature cells. Determination of the pheno- are attributable to hemorrhage or to leukemic masses in
types of lymphoid and myeloid cells can be accomplished the metaphyseal periosteum, subarticular bone, and syn-
by using monoclonal antibodies that are generally direct- ovium. Several radiographic findings may be observed.
ed against surface membrane proteins. Two broad popu-
lations of lymphocytes involved in the immune response Diffuse Osteopenia (15% to 100%). Osteopenia, with
can be identified: thymus-derived lymphocytes (T cells), medullary widening and cortical thinning in tubular bones,
which require the thymus for normal differentiation, and and vertebral compression are encountered. Osteopenia
bone marrow–derived lymphocytes (B cells), which may progress slowly without treatment or improve with
685
686 Section XIII Diseases of the Hematopoietic System
therapy. The occurrence of unexplained generalized osteo- Prominent symmetrical periosteal new bone deposition
penia in a child should initiate a search for clinical and in the tubular bones simulates the appearance of sec-
laboratory manifestations of leukemia. ondary hypertrophic osteoarthropathy, syphilis, and
prostaglandin-induced periostitis.
Radiolucent and Radiodense Metaphyseal Bands (10% to
55%). Symmetrical metaphyseal bandlike radiolucent Osteosclerosis (5% to 10%). Osteosclerosis is a relatively
areas are observed in leukemia and other chronic child- infrequent finding in leukemia. When apparent, it is
hood illnesses (Fig. 51–1). This nonspecific finding prob- particularly prominent in the metaphyses of long bones.
ably reflects a nutritional deficit that interferes with
proper osteogenesis. It is most commonly seen at sites of Other Skeletal Abnormalities. Sutural diastasis is common
rapid bone growth, including the distal femoral, proximal in infants and children with leukemia (Fig. 51–3). It is
tibial, proximal humeral, and distal radial metaphyses. produced by an increase in intracranial pressure result-
After the age of 2 years, radiolucent metaphyseal bands ing from leukemic cell infiltration of the meninges or
are more characteristic of leukemia than of other condi- cerebrum or from intracerebral hemorrhage.
tions. Radiodense metaphyseal bands may be noted adja-
cent to the areas of increased radiolucency. Parallel Course of the Skeletal Lesions. The extent of the bone
radiodense growth recovery lines (of Harris) are presum- lesions correlates poorly with the progress of leukemia.
ably related to alternating periods of arrest and acceler- During treatment, resolution of the lytic defects is observed.
ation of bone growth. They may be observed in 50% of The disappearance of lucent metaphyseal bands during
children with leukemia. Transverse radiolucent bands remission has also been noted and may be associated with
may also be observed under the vertebral endplates. transient metaphyseal sclerosis. Skeletal lesions may or
may not reappear during relapse.
Osteolytic Lesions (30% to 50%). Multiple (or solitary)
radiolucent lesions related to bone destruction are Articular Abnormalities. The joint manifestations of acute
encountered in tubular and flat bones (Figs. 51–1 and leukemia that can lead to significant clinical findings
51–2). In the long bones of the extremities, the radio- are due to intra-articular leukemic cell infiltration and
lucent lesions of a metaphysis can extend into the di- hemorrhage and, more commonly, to periarticular bone
aphysis. Similar lesions are seen in the cranial vault, lesions. Pauciarticular and asymmetrical involvement
pelvis, ribs, and shoulder girdle. The medial cortex of the appears to be most characteristic. Severe pain is a com-
proximal portion of the humerus is a characteristic site of mon clinical finding. Soft tissue swelling, effusion, and
involvement. juxta-articular osteoporosis are occasionally seen.
Epiphyseal osteonecrosis may occur in some patients,
Periostitis (10% to 35%). Periosteal bone formation can be particularly those treated with steroid medications. It has
associated with lytic lesions (see Fig. 51–2). Proliferating also been documented in leukemic patients before such
leukemic cells in the marrow invade the cortex via haver- therapy and has led to the early onset of symptoms. In
sian canals and extend to subperiosteal locations, where such cases, potential mechanisms include leukemic infil-
they cause elevation of the periosteal membrane. Sub- tration in the bone marrow or walls of the blood vessels
periosteal hemorrhage may be an associated finding. and hyperviscosity of the blood. Osteonecrosis occurring
Periostitis is particularly prominent in the long bones. in association with leukemia typically affects the femoral
A B
Chapter 51 Lymphoproliferative and Myeloproliferative Disorders 687
epiphyses and condyles and the proximal portion of the
humerus. Septic arthritis and osteomyelitis can compli-
cate the acute leukemias in both children and adults.
Metaphyseal radiolucent bands are not as frequent as in invariably, related to steroid administration. Secondary
children with acute leukemia. Rare radiographic findings gout is a well-known complication of chronic leukemia.
are large destructive lesions, periostitis, acro-osteolysis, Septic arthritis and osteomyelitis may also be evident
discrete subperiosteal erosions, and focal or diffuse (Fig. 51–5).
osteosclerosis.
Special Types of Leukemia
Chronic Leukemia
Hairy Cell Leukemia. Hairy cell leukemia, which is also
The osseous and articular manifestations of chronic termed leukemic reticuloendotheliosis, is responsible for
leukemia are less common and less severe than those of approximately 2% of all cases of leukemia. Its name
acute leukemia. Marrow hyperplasia in some patients originates from the numerous short villi, resembling hairs,
may become evident as nonspecific diffuse osteopenia, about the membranes of lymphocytes. The major clinical
particularly in the axial skeleton. Discrete osteolytic lesions consequences of the disease are related to depressed bone
are observed in less than 3% of persons, particularly in marrow function and hypersplenism.
the femur and humerus (Fig. 51–4). Occasionally, larger Hairy cell leukemia typically develops in adults. Its
and more aggressive lesions may be encountered. Rarely, insidious onset is characterized by fatigue, weakness,
widespread or multifocal bone sclerosis is evident, perhaps infectious episodes, abdominal pain (or, more rarely,
related to diffuse marrow fibrosis. Soft tissue accumu- hemorrhage), splenic rupture, and pathologic fracture of
lation of masses of leukemic cells (chloromas) can pro- bone. Splenomegaly, hepatomegaly, and lymphadenopa-
duce subjacent osseous erosion. thy are present, in order of decreasing frequency. The
In adults (and in children), leukemic involvement of disease is slowly progressive, but most patients die in the
the small bones of the hand may be associated with soft
tissue edema, clubbing, and bone destruction. This com-
bination of findings is termed leukemic acropachy. Clinical
findings are usually a late manifestation of the disease and
show a predilection for the knees, shoulders, and ankles.
Radiographic findings are limited; osteopenia and soft
tissue swelling may be evident, however. Epiphyseal
osteonecrosis has been described and is usually, but not
A B
Figure 51–5. Chronic leukemia: osteomyelitis. In this 34-year-
old woman, osteomyelitis from Salmonella infection occurred in
the humerus. As depicted in these radiographs obtained 2 weeks
apart, findings include poorly defined osteolysis in the diaphysis
associated with periostitis, abnormalities seen in uncomplicated
Figure 51–4. Chronic leukemia: osteolytic lesions. A 27-year- leukemia as well. A biopsy of bone undertaken in the interval
old woman with chronic leukemia demonstrates small, focal between the two studies (A, initial radiograph; B, 2 week follow-
radiolucent lesions in the proximal portions of the humerus and up) explains the circular osteolytic defects in the later radiograph.
femur. Salmonella organisms were recovered from the biopsy material.
Chapter 51 Lymphoproliferative and Myeloproliferative Disorders 689
first 5 years. Bone involvement is an infrequent feature of with leukemia include determination of the extent of
hairy cell leukemia. When present, such involvement can bone marrow alterations before the initiation of therapy,
lead to solitary or, less commonly, multiple (usually two) assessment of the response of the disease to such therapy,
osteolytic lesions, with a predilection for the spine and detection of some of the skeletal complications of the
proximal portions of the femora (head and neck). Bone disease or the therapeutic regimen, and investigation of
sclerosis is rare. Spontaneous fracture is a recognized some special forms of leukemia, such as hairy cell
complication of such lesions. Radiation therapy and leukemia and granulocytic sarcoma.
chemotherapy can lead to a decrease in symptoms and The MR imaging abnormalities accompanying
regression of the osteolytic process. leukemic infiltration of bone marrow are similar or iden-
tical to those in a number of other infiltrative processes
Acute Megakaryoblastic Leukemia. This rare disorder, (e.g., plasma cell myeloma, lymphoma, metastasis) (Fig.
also termed malignant myelofibrosis, affects children and 51–7). Most commonly, leukemic marrow demonstrates
adults and demonstrates a progressive course character- prolongation of the T1 relaxation value, which leads to
ized by anemia, pancytopenia, and diffuse marrow fibrosis. diminution of marrow signal intensity on T1-weighted
Splenomegaly is absent or mild. The proliferating cells spin echo MR images. Diffuse or focal abnormalities are
in the bone marrow are variable in type, immature, and evident, with the latter pattern being especially evident
dominated by megakaryocytes. in acute myelogenous leukemia. Because many of the
In children, radiolucency in the metaphyseal regions leukemias affect children, the resulting decrease in signal
of tubular bones and osteolytic lesions are seen. The intensity is sometimes difficult to differentiate from the
degree of periosteal bone formation can be profound. In signal intensity of normal hematopoietic marrow. T2
adults, focal or diffuse bone sclerosis is observed. changes are more variable and less dramatic. Quantitative
chemical shift MR imaging has been used in patients with
Granulocytic Sarcoma (Chloroma). Granulocytic sarcoma leukemia to determine the stage of the disease. This tech-
is a localized tumor mass composed of immature cells of nique can help distinguish the individual contributions
the granulocyte series (Fig 51–6). Its designation as of fat and water to the total signal intensity, thus render-
chloroma stems from the greenish color of the tumor. ing a more quantitative assessment of the bone marrow
Granulocytic sarcoma is most commonly associated with than can be accomplished with conventional MR imaging
acute leukemia of the myeloid type, especially in children, techniques.
although it is also observed in adults and in patients with The role of other MR imaging techniques, such as
other varieties of leukemia, with other myeloproliferative gadolinium enhancement and short tau inversion recovery
disorders, or without obvious bone marrow dysfunction. (STIR) and in-phase and out-of-phase gradient echo
Granulocytic sarcomas are more common in children imaging, in the evaluation of the therapeutic response of
than in adults and may be single or multiple. Frequent patients with leukemia (and other disseminated malig-
sites of involvement are bone, periosteum, soft tissue, nant disorders of bone marrow) is still to be determined.
orbits, lymph nodes, and skin. Lytic lesions characterize Although the value of MR imaging as a technique for
intraosseous involvement and are especially prominent monitoring disease activity requires further study, its
in the skull, spine, ribs, long tubular bones, and sternum. value in defining local complications of musculoskeletal
Soft tissue tumors can lead to masses, as in a paraspinal involvement is well established. Soft tissue extension of
location, that may subsequently erode the neighbor- intraosseous lesions and compromise of the spinal cord
ing bone. accompanying vertebral involvement, especially in focal
lesions associated with hairy cell leukemia and granulo-
cytic sarcoma, are well shown with standard MR imaging
Magnetic Resonance Imaging
techniques. Further, assessment of complications result-
Broad applications of magnetic resonance (MR) imaging ing from chemotherapy, such as ischemic necrosis of
to the analysis of musculoskeletal involvement in patients bone, can be accomplished with these techniques.
A B
690 Section XIII Diseases of the Hematopoietic System
A B
C
Figure 51–7. Leukemia: MR imaging abnormalities—spin echo and gadolinium-enhanced techniques.
This adult patient has a pathologic fracture of the femur. A, Standard coronal T1-weighted (TR/TE,
525/25) spin echo MR image reveals diffuse low signal intensity in the marrow of the diaphyses and
metaphyses of the femora and the innominate bones. The distribution of this signal intensity is too
widespread to represent normal sites of hematopoietic marrow in an adult patient. Some fatty marrow with
high signal intensity is seen in the femoral epiphyses and, on the left side, in the femoral metaphysis and
diaphysis. B, With T2 weighting (TR/TE, 2200/90), regions of very high signal intensity are evident in the
femoral necks, especially on the right side, and joint effusions are apparent in both hips. The remaining
portions of the bone marrow are of moderately low signal intensity. C, Coronal T1-weighted (TR/TE,
525/25) spin echo MR image obtained after the intravenous administration of a gadolinium contrast agent
shows enhancement of signal intensity in the marrow, particularly in both femoral necks. The distribution
of the abnormal signal changes in the femora is similar to that in (B). (Courtesy of J. Kramer, MD, Vienna,
Austria.)
A B
Figure 51–8. Non-Hodgkin’s lymphoma: histocytic type with disseminated disease. A, Extensive
destructive lesion of the scapula in a 58-year-old man is characterized by a soft tissue mass, osteolysis of the
glenoid process and adjacent bone, and osteosclerosis. The humeral head appears to be involved, and the
shaft reveals osteoporosis. B, In this 47-year-old man, multiple organ systems are affected. Observe an
eccentric diaphyseal lesion of the femur, with moth-eaten bone destruction and cortical violation.
A B
Figure 51–9. Non-Hodgkin’s lymphoma: histocytic type with primary bone involvement. A, Patchy
osteolysis in the distal end of the femur is evident on the radiograph. B, Sagittal T1-weighted (TR/TE,
700/15) spin echo MR image reveals the extent of the tumor, which is of low signal intensity. No
enhancement of the lesion was evident on MR images obtained after the intravenous administration of a
gadolinium contrast agent (not shown). (Courtesy of J. Kramer, MD, Vienna, Austria.)
metastasis, and Paget’s disease (Fig. 51–10). Widespread Burkitt’s Lymphoma. Burkitt’s lymphoma is a stem cell
osteosclerosis may result from an osseous response to lymphoma that is seen predominantly in children. This
extensive bone marrow involvement or bone marrow type of lymphoma is strongly associated with the
fibrosis rather than from frank involvement of the bone Epstein-Barr virus. It is the most common malignant
itself. Osteolytic lesions are poorly defined and associated disease of children in tropical Africa. Involvement of the
with periostitis in approximately one third of cases. facial bones is particularly characteristic. Early radio-
Similar periosteal bone formation may indicate graphic changes include loss of the lamina dura, particularly
associated hypertrophic osteoarthropathy. around the molar teeth, and diminution and obscuration
Chapter 51 Lymphoproliferative and Myeloproliferative Disorders 693
A B
of trabeculae in cancellous bone. With disruption of the Mycosis Fungoides. Mycosis fungoides is considered an
cortex, a soft tissue mass may extend into the buccal unusual form of malignant (T-cell) lymphoma with
cavity or maxillary antrum. primary involvement of the skin. It is uncommon but not
Lesions in the tubular bones and pelvis have been rare. Its onset is in the fourth or fifth decade of life.
described but are less frequent. The femur and tibia are Initially, mycosis fungoides may appear as nonspecific
especially vulnerable. Osteolytic foci develop in the skin ulcerations. Cutaneous lesions may be associated
medullary portion of the bone, coalesce, penetrate the with localized or generalized lymphadenopathy. In the
cortex, produce periostitis, and lead to a soft tissue mass. tumor stage, extracutaneous manifestations with visceral
694 Section XIII Diseases of the Hematopoietic System
involvement are evident. After the tumor stage, death puted tomography, a mass effect is often seen, although
commonly occurs within a few years, frequently as a the involved tissue may have attenuation values similar to
result of septicemia. those of normal muscle. On MR images, signal intensity
Bone marrow involvement is rare. When present, similar to that of normal muscle is typical on T1-weighted
bone lesions occur in the appendicular skeleton, with sequences, with relatively high signal intensity on T2-
discrete or poorly defined medullary defects, cortical weighted sequences (Fig. 51–11). The adjacent fat is
destruction, periostitis, and soft tissue swelling. Although often infiltrated, and multiple muscle compartments and
the skeletal abnormalities are similar to those accom- a long segment of an extremity may be involved. The
panying other aggressive lesions, such as metastasis and differential diagnosis of muscle involvement in lymphoma
plasma cell myeloma, involvement of the peripheral includes various types of myositis, muscle infarction,
skeleton, including the hand, may be a helpful clue in the denervation changes in muscle, and sarcoidosis.
diagnosis of mycosis fungoides.
Effects of Therapy
Muscle Abnormalities
The association between ischemic necrosis of bone and
Enlargement of one or more muscles is a recognized treatment of lymphoma with combination chemotherapy
manifestation of the lymphomas. Such enlargement can regimens that include intermittent corticosteroids has
be painful and may affect the musculature of the axial been reported. The frequency of this complication in
or the appendicular skeleton, or both. Lymphomatous treated patients is approximately 1% to 3% in Hodgkin’s
infiltration of the muscle related to either contiguous disease and somewhat lower in non-Hodgkin’s lymphoma,
tumor mass or lymph node represents one mechanism typically occurring 1 to 3 years after the initiation of
for enlargement of the muscle. Similar enlargement, therapy. The femoral head and, less commonly, the
however, can occur without histologic evidence of humeral head are preferred sites of involvement.
lymphomatous infiltration, although the pathogenesis A second complication of therapy in leukemia and, less
of the finding in this situation is not clear. typically, lymphoma is related to the administration of
With ultrasonography, muscle involvement is charac- methotrexate. Skeletal changes, termed methotrexate
terized by an ill-defined hypoechoic mass. With com- osteopathy, usually occur 6 to 18 months after institution
A B
Figure 51–11. Non-Hodgkin’s lymphoma: muscle involvement. In a 68-year-old man, oblique coronal
T1-weighted (TR/TE, 400/16) spin echo (A) and fat-suppressed fast spin echo (TR/TE, 3500/39) (B) MR
images show fusiform enlargement of a long segment of the involved muscle (arrows), characterized by signal
intensity identical to that of normal muscle in (A) and high signal intensity in (B). Note the associated
lymphadenopathy. (Courtesy of C. Chen, MD, Kaohsiung, Taiwan.)
Chapter 51 Lymphoproliferative and Myeloproliferative Disorders 695
of the therapy and are characterized by pain, osteopenia, nant lymphoblastic lymphoma. Articular involvement
growth recovery lines, dense metaphyseal bands, and occurs in approximately 10% of cases. Joint effusions,
insufficiency or complete fractures. Periostitis is either soft tissue swelling, and, rarely, periarticular osteoporosis,
absent or localized. The cause of methotrexate osteopa- joint space narrowing, and osseous erosions have been
thy is not known. encountered.
A B
Figure 51–12. Non-Hodgkin’s lymphoma: disseminated disease. Spin echo and STIR MR imaging tech-
niques. A, Coronal T1-weighted (TR/TE, 800/20) spin echo MR image reveals bilateral femoral involve-
ment, greater on the left side. Focal areas of low signal intensity are evident in the bone marrow. B, With
STIR imaging (TR/TE, 2000/30; inversion time, 160 msec), the lesions are of high signal intensity.
696 Section XIII Diseases of the Hematopoietic System
A B
Figure 51–13. Mastocytosis: radiographic abnormalities in extraspinal sites. A, Observe the multiple
focal, well-defined osteosclerotic lesions in the hemipelvis. B, In a different patient, diffuse osteosclerosis of
the entire pelvis and proximal ends of the femora is evident.
A B
Figure 51–14. Mastocytosis: MR imaging abnormalities. Coronal T1-weighted (TR/TE, 550/25) (A) and
T2-weighted (TR/TE, 2200/90) (B) spin echo MR images show marrow infiltration in the spine and osseous
pelvis, as well as the proximal portions of the femora, manifested as low signal intensity in (A) and
intermediate signal intensity (similar to that of fat) in (B). (Courtesy of J. Kramer, MD, Vienna, Austria.)
TABLE 51–3
Differential Diagnosis of Osteosclerosis
Differential Diagnosis
The radiographic diagnosis of myelofibrosis should be
suggested when axial skeleton osteosclerosis is combined
with splenomegaly in a middle-aged or elderly patient
(see Table 51–3). Although lymphoma and leukemia can
lead to splenomegaly, the extent of the bone sclerosis is
less in these conditions than in myelofibrosis. Systemic
mastocytosis can produce diffuse or focal osteosclerosis
and hepatosplenomegaly, and it may be difficult to distin-
guish this condition from myelofibrosis.
Increased radiodensity of bone without splenic enlarge-
ment may be seen in some patients with myelofibrosis
and is apparent in patients with this disorder who have
had splenectomies. This combination of findings may
be evident in other processes as well, including skeletal
Figure 51–16. Myelofibrosis: MR imaging abnormalities in metastasis, fluorosis, Paget’s disease, axial osteomalacia,
the axial skeleton. On a coronal T1-weighted (TR/TE, 650/30) and renal osteodystrophy. Differentiation of myelofibrosis
spin echo MR image of the pelvis, the marrow of the vertebral and skeletal metastasis can be difficult. In most cases, the
bodies, pelvic bones, and proximal ends of the femora is pre-
dominantly low in signal intensity. This pattern is consistent
sclerosis observed in metastatic disease of the bone is less
with the presence of hematopoietic marrow or fibrosis. Schmorl’s generalized, less symmetrical, and more frequently asso-
nodes are evident, and the spleen is enlarged. (Courtesy of T. ciated with osteolytic lesions. In fluorosis, spinal osteo-
Mattsson, MD, Riyadh, Saudi Arabia.) phytosis, ligament calcification and ossification, and
periostitis may be noted. In renal osteodystrophy, other
changes are evident, including those of hyperparathy-
roidism. In Paget’s disease, a characteristic coarsened
trabecular pattern is present.
Chapter 51 Lymphoproliferative and Myeloproliferative Disorders 701
BONE MARROW TRANSPLANTATION opposite situation is apparent, with high signal intensity
Bone marrow transplantation is a technique for replen- at the superior and inferior vertebral margins and low
ishing the bone marrow with normal pluripotential signal intensity centrally. At histologic examination, the
stem cells. Normal marrow cells from a histocompatible marginal, or peripheral, regions of the vertebral body
allogeneic donor are used to repopulate the diseased are found to contain a concentrated collection of repopu-
marrow, and such transplantation is frequently combined lating cells, and the central region contains predomi-
with intensive chemotherapy or immunosuppressive nantly marrow fat.
therapy. The marrow is usually obtained from the donor’s
iliac crest.
Infections developing immediately before or soon FURTHER READING
after bone marrow transplantation relate to the effects
of chemotherapy and radiation therapy, as well as the Amano Y, Onda M, Amano M, et al: Magnetic resonance
transplantation itself. Bacterial and fungal infections imaging of myelofibrosis: STIR and gadolinium-enhanced
dominate in the pretransplantation period, and viral MR images. Clin Imaging 21:264, 1997.
infections may also be encountered. During the first 2 or Avila NA, Ling A, Metcalfe D, et al: Mastocytosis: Magnetic
resonance imaging pattern of marrow disease. Skeletal
3 weeks after transplantation, severe granulocytopenia, Radiol 27:119, 1998.
fever, and, in about 50% of patients, at least one episode Bloch KJ, Buchanan WW, Whol MJ, et al: Sjögren’s syndrome:
of bacteremia are seen. Neutropenia at this time predis- A clinical, pathological and serological study of sixty-two
poses to infections caused by opportunistic organisms. cases. Medicine 44:187, 1965.
Delayed post-transplantation infections are usually Braunstein EM, White SJ: Non-Hodgkin’s lymphoma of bone.
related to varicella-zoster virus or recurrent bacterial or Radiology 135:59, 1980.
fungal infections. Chew FS, Schellingerhout D, Keel SB: Primary lymphoma of
Graft-versus-host disease, which is discussed in detail skeletal muscle. AJR Am J Roentgenol 172:1370, 1999.
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cells that were transfused with the graft or that developed lymphoma. Radiology 50:458, 1948.
Dalinka MK: Primary lymphoma of bone: Radiographic
from its reaction to targets of the genetically different appearance and prognosis. Radiology 147:288, 1983.
host. An immunologic attack on the recipient’s tissues Demanes DJ, Lane N, Beckstead JH: Bone involvement in
develops. Acute graft-versus host disease occurs within hairy-cell leukemia. Cancer 49:1697, 1982.
the first 3 months after transplantation and is associated Ferris RA, Hakkai HG, Cigtay OS: Radiologic manifestations
with lesions of the skin, liver, and gastrointestinal tract. of North American Burkitt’s lymphoma. AJR Am J
Specific clinical findings include an erythematous macu- Roentgenol 123:614, 1975.
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Chronic graft-versus-host disease affects 20% to 40% of Harris NL, Jaffe ES, Diebold J, et al: World Health Organi-
patients who survive more than 6 months after transplan- zation classification of neoplastic diseases of the hemato-
poietic and lymphoid tissues: Report of the clinical advisory
tation. It involves the skin, oral mucosa, serosal surfaces, committee meeting−Airlie House, Virginia, November
skeletal muscle, gastrointestinal tract, liver, and lung, and 1997. J Clin Oncol 17:3835, 1999.
its manifestations resemble those of a collagen vascular Kaplan KR, Mitchell DG, Steiner RM, et al: Polycythemia vera
disorder, particularly scleroderma. and myelofibrosis: Correlation of MR imaging, clinical, and
Chemotherapy and radiotherapy administered in the laboratory findings. Radiology 183:329, 1992.
pretransplantation period are designed not only to induce Lecouvet FE, Dechambre S, Malghem J, et al: Bone marrow
immunologic suppression in the recipient but also to transplantation in patients with multiple myeloma: Prog-
eliminate any residual malignant cell populations. The nostic significance of MR imaging. AJR Am J Roentgenol
initial response of the bone marrow to such therapy 176:91, 2001.
is congestion and edema. Subsequently, hematopoietic Leigh TF, Corley CC Jr, Huguley CM Jr, et al: Myelofibrosis:
The general and radiologic findings in 25 proven cases. AJR
tissue disappears, and the marrow becomes predominantly Am J Roentgenol 82:183, 1959.
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spin echo MR images, and intermediate signal intensity is 200:226, 1985.
seen on T2-weighted spin echo images, signal character- Metzler JP, Fleckenstein JL, Vuitch F, et al: Skeletal muscle
istics that are typical of adipose tissue. During transplan- lymphoma: MRI evaluation. Magn Reson Imaging 10:491,
tation, bone marrow cells are infused intravenously. After 1992.
transient residence in the lungs and spleen, the infused Neiman RS, Barcos M, Berard C, et al: Granulocytic sarcoma:
stem cells migrate to the bone marrow. Hematologic A clinicopathologic study of 61 biopsied cases. Cancer
engrafting typically takes 3 to 4 weeks and is heralded by 48:1426, 1981.
a peripheral rise in granulocytes. Repopulation of the Nixon GW, Gwinn JL: The roentgen manifestations of
leukemia in infancy. Radiology 107:603, 1973.
bone marrow in the spine after transplantation can be Olson DO, Schields AF, Scheurich CJ, et al: Magnetic reso-
studied with MR imaging. On T1-weighted spin echo nance imaging of the bone marrow in patients with leukemia,
MR images, the superior and inferior margins of the aplastic anemia, and lymphoma. Invest Radiol 21:540,
vertebral bodies reveal low signal intensity, whereas the 1986.
central portion of the vertebral bodies show high signal O’Reilly GV, Clark TM, Crum CP: Skeletal involvement in
intensity, consistent with fat. With STIR sequences, the mycosis fungoides. AJR Am J Roentgenol 129:741, 1977.
702 Section XIII Diseases of the Hematopoietic System
Parker BR, Marglin S, Castellino RA: Skeletal manifestations of Schabel SI, Tyminski L, Holland RD, et al: The skeletal mani-
leukemia, Hodgkin disease and non-Hodgkin lymphoma. festations of chronic myelogenous leukemia. Skeletal Radiol
Semin Roentgenol 15:302, 1980. 5:145, 1980.
Patzik SB, Smith C, Kubicka RA, et al: Bone marrow trans- Schwartz AM, Leonidas JC: Methotrexate osteopathy. Skeletal
plantation: Clinical and radiologic aspects. Radiographics Radiol 11:13, 1984.
11:601, 1991. Silbiger ML, Peterson CC Jr: Sjögren’s syndrome: Its
Pettigrew JD, Ward HP: Correlation of radiologic, histologic roentgenographic features. AJR Am J Roentgenol 100:554,
and clinical findings in agnogenic myeloid metaplasia. 1967.
Radiology 93:541, 1969. Simmons CR, Harle TS, Singleton EB: The osseous manifes-
Phillips WC, Kattapuram SV, Doseretz DE, et al: Primary tations of leukemia in children. Radiol Clin North Am
lymphoma of bone: Relationship of radiographic appearance 6:115, 1968.
and prognosis. Radiology 144:285, 1982. Talbott JH: Gout and blood dyscrasias. Medicine 38:173,
Poppel MH, Gruber WF, Silber R, et al: The roentgen mani- 1959.
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Rafii M, Firooznia H, Golimbu C, et al: Pathologic fracture in White LM, Schweitzer ME, Khalili K, et al: MR imaging of
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52
CHAPTER 52
Bleeding Disorders bleeding may be apparent only during surgery. With
moderate or severe forms, bleeding episodes may also
occur spontaneously or after minor or significant trauma.
The diagnosis is established by performing appropriate
SUMMARY OF KEY FEATURES laboratory tests to detect defects in blood coagulation.
The skeletal abnormalities associated with hemophilia Hemarthrosis is a particularly characteristic abnor-
and other bleeding diatheses are characteristic. mality, occurring in approximately 75% to 90% of patients.
They result from hemorrhage in soft tissue, muscle, It may begin in the early years of life, and young children
and subperiosteal, intraosseous, and intra-articular and adolescents demonstrate more frequent episodes of
locations. In involved joints, typical findings are joint bleeding than adults do. The joints altered most
radiodense effusions, regional or periarticular commonly are the knee, elbow, ankle, hip, and gleno-
osteoporosis, subchondral bony erosions and cysts, humeral joint, in descending order of frequency. Joints
and joint space narrowing. Hyperemia may lead to such as the knee, whose stability depends on adjacent soft
epiphyseal overgrowth in a child affected by these tissue structures rather than intrinsic factors, are particu-
disorders. Tumor-like lesions are occasionally larly vulnerable. Usually a single joint is involved in each
encountered that are due to massive subperiosteal, episode, although eventually multiple joints are affected.
osseous, or soft tissue hemorrhage, with erosion and Joint involvement may be markedly asymmetrical or even
distortion of adjacent bone. Hemosiderin deposition in unilateral.
any of these disorders leads to characteristic findings on Clinical manifestations of hemophilic arthropathy can
magnetic resonance imaging. The differential diagnosis be divided into three types, although they are not rigidly
is usually not difficult when both clinical and imaging defined:
features are studied. 1. Acute hemarthrosis. Joint bleeding may occur rapidly,
producing a tense, swollen, red, and tender articula-
tion that is painful and stiff. Associated muscle spasm
leads to flexion of the extremity and restricted motion.
INTRODUCTION Symptoms decrease quickly after administration of the
appropriate clotting factor.
Hemophilia is a term applied to a group of disorders
characterized by an anomaly of blood coagulation caused 2. Subacute hemarthrosis. After multiple acute episodes,
by a deficiency in a specific plasma clotting factor. This complete recovery of the joint is not evident. Joint
anomaly leads to easy bruising and prolonged and exces- motion is restricted, a finding that appears to best
sive bleeding. Of these disorders, two are associated most correlate with the degree of cartilaginous destruction,
commonly with intraosseous and intra-articular bleeding: and contractures and muscle atrophy become evident.
classic hemophilia (hemophilia A), characterized by a func- 3. Chronic hemarthrosis. After 6 months to 1 year, a chronic
tional deficiency of antihemophilic factor (factor VIII), stage may develop. More severe and persistent con-
and Christmas disease (hemophilia B), marked by a func- tractures are found, particularly in the elbow and knee.
tional deficiency of plasma thromboplastin component The final stage is a fibrotic, contracted, and destroyed
(factor IX). These two types of hemophilia are X-linked joint. Approximately 50% of hemophilic patients
recessive disorders that are clinically manifested in men develop permanent changes in the peripheral joints.
and carried by women. Rarely, other disorders of blood Articular bleeding may be accompanied by hemor-
coagulation may manifest as bone and joint abnormalities. rhage into muscles, fascial planes, and bones. Soft tissue
One such disorder is von Willebrand’s disease, a rare familial bleeding can lead to fixed joint deformities and soft tissue
disease of both men and women that apparently is attrib- necrosis. Compartment syndromes may complicate
utable to a dominant autosomal mutant gene; in this disease, intramuscular bleeding in the upper or lower extremities.
both factor VIII and functional platelet abnormalities occur. The most common of these in hemophilia are Volkmann’s
contractures, related to massive hemorrhage into the
HEMOPHILIA volar muscles of the forearm. Hemorrhage in and around
the spinal cord can lead to neurologic abnormalities. Sub-
Clinical Abnormalities periosteal and intraosseous bleeding can induce trabecu-
Classic hemophilia occurs in approximately 1 of every lar distortion and destruction, and large expansile lesions,
10,000 men and boys in the United States. Christmas particularly of the femur and ilium (hemophilic pseudo-
disease occurs about one tenth as often as classic hemo- tumors), may simulate neoplasm.
philia. Although both forms are confined almost exclu-
sively to male subjects, reports exist of significant clinical
Pathologic Abnormalities
and radiographic abnormalities in female patients. The
severity of the clinical manifestations in either form of After an acute episode of intra-articular bleeding, blackish
hemophilia varies. In mild forms of disease, excessive fluid containing clots is apparent. With each recurring
703
704 Section XIII Diseases of the Hematopoietic System
Radiographic Abnormalities
General Features
The findings of hemophilia have been divided into five
stages on the basis of a variety of radiographic abnormal-
ities: (1) soft tissue swelling, (2) osteoporosis, (3) osseous
subchondral cystic lesions, (4) narrowing of the interosseous
space with cartilage destruction, and (5) joint disorgani-
zation with severe cartilaginous and osseous abnormal-
ities. The division of the radiographic changes into stages
E
Figure 52–1. Hemophilia: pathologic abnormalities—intra-
articular findings. A, Acute episodes of bleeding lead to accu-
TABLE 52–1 mulation of blood in the articular cavity and periarticular soft
Hemophilia: Radiographic-Pathologic Correlation tissues. B, After numerous bleeding episodes, absorption of
blood is incomplete from the articular cavity and soft tissues.
Brownish discoloration of the synovial membrane is associated
Pathology Radiology
with hypertrophy and hyperemia. Synovial inflammatory tissue,
Recurrent intra-articular Radiodense joint or pannus, appears at the margins of the articular cartilage
hemorrhage with hemosiderin- effusions (arrows). C, At a later stage, periarticular osteoporosis and focal
laden hypertrophied synovial areas of cartilage and osseous destruction become apparent.
membrane Cystic lesions are evident, which generally communicate with
Synovial inflammation and Osteoporosis; epiphyseal the joint cavity. Note areas of relatively normal cartilage and
pannus formation; hyperemia overgrowth; accelerated bone. D, Continued destruction of cartilage and bone leads to
skeletal maturation enlarging cystic lesions, surface irregularities, osteoporosis, and
Cartilaginous erosion; Bony erosions and cysts joint space narrowing. E, In late stages of the disease, fibrous
subchondral trabecular adhesions extend across the articular space. New episodes of
resorption and collapse bleeding occur.
Cartilaginous denudation Joint space narrowing
Bony proliferation Sclerosis and
osteophytosis (see Fig. 52–1) does not imply that all cases follow this
Soft tissue, superiosteal, and Pseudotumors sequence of events. Further, radiographic abnormalities
intraosseous hemorrhage do not always develop in a systematic fashion. Although
bone change usually precedes cartilage loss, this is not
Chapter 52 Bleeding Disorders 705
Distribution of Abnormalities
The knee, ankle, and elbow are the joints involved most
frequently. Bilateral involvement is common, but the
changes need not be symmetrical.
Knee. The knee is affected most often (Fig. 52–3). Dense
joint effusions are common. Periarticular osteoporosis
creates lucent epiphyses of both the distal end of the
femur and the proximal part of the tibia. Irregularity of
the articular surface of the femoral condyles, the tibial
plateaus, and the posterior surface of the patella may
become apparent. Multiple subchondral cysts are fre-
quent and sometimes grow to a large size.
Overgrowth of the distal femoral and proximal tibial
epiphyses may result. The distal condylar surface may
appear flattened, and the intercondylar notch of the femur
is commonly widened. Squaring of the inferior pole of
the patella has been detected in as many as 20% to 30%
of patients with hemophilia. However, squaring of the
patella is not specific for hemophilia, being a recognized
manifestation of juvenile chronic arthritis. Differentia-
tion between these two disorders on the basis of knee
A B radiographs is very difficult.
Figure 52–2. Hemophilia: pathologic abnormalities— Ankle. The radiographic findings in the ankle are similar
intraosseous (A) and subperiosteal (B) hemorrhage. These types
of hemorrhage can lead to hemophilic pseudotumors, with
to those in other involved joints, including soft tissue
destruction and deformity of bone. swelling, osteoporosis, marginal and central osseous ero-
sions, and joint space narrowing (Fig. 52–4). Tibiotalar
slanting may be observed, creating an angular joint sur-
always the case. In some patients, articular involvement face, a finding observed in epiphyseal dysplasias, juvenile
never progresses beyond the first or second stage; in chronic arthritis, and perhaps sickle cell anemia. Bony
other patients, despite appropriate medical or surgical ankylosis of the subtalar joints has also been described in
therapy, severe arthropathy develops. hemophilia. Osteonecrosis of the talus may be observed.
A B
Figure 52–3. Hemophilia: knee joint abnormalities. Frontal (A) and lateral (B) radiographs demonstrate
characteristic findings of hemophilia. The bones are mildly osteoporotic, and the epiphyses are enlarged.
Note the well-defined or “etched” erosions of subchondral bone (arrowheads), the radiodense joint effusion
(arrow), and the widened intercondylar notch of the femur.
706 Section XIII Diseases of the Hematopoietic System
Additional Abnormalities
Several other abnormalities may be associated with
hemophilia.
Scintigraphy
Increased sensitivity of the isotopic examination com-
pared with clinical and radiographic evaluation is not
unexpected, particularly at sites of acute arthropathy.
The radionuclide examination lacks specificity, however,
and it is less effective in evaluating joints with chronic
arthropathy.
Computed Tomography
Because hemophilic pseudotumors commonly arise in
or extend to periosseous soft tissues, they are often well
Figure 52–7. Hemophilia: ectopic ossification. A large band characterized by computed tomography. Soft tissue
of ossification extends from the lateral aspect of the ilium to the ossification in hemophilia is also well shown with this
proximal portion of the femur. (Courtesy of M. Dalinka, MD, technique (Fig. 52–9).
Philadelphia, Pa.)
Magnetic Resonance Imaging
Fractures. In hemophilia, fractures may occur sponta- Spin echo magnetic resonance (MR) imaging sequences
neously or after minor trauma. Fracture healing in hemo- typically reveal regions in the joint with low to inter-
philic patients proceeds normally, although pseudotumors mediate signal intensity on T1- and T2-weighted images,
may develop at the site of fracture. with foci of increased signal intensity on T2-weighted
images. Persistent low signal intensity in both types of
Hemophilic Pseudotumor. Hemophilic pseudotumor is a images is consistent with the presence of synovial fibrosis,
relatively uncommon manifestation of the disease, prob- hemosiderin deposition, or both (Fig. 52–10). The foci of
ably occurring in less than 2% of cases. The bones that high signal intensity on T2-weighted images are consis-
are implicated most frequently, in descending order of tent with areas of synovial inflammation or fluid. Owing
frequency, are the femur, the components of the osseous to the changing signal characteristics of resolving hemor-
pelvis, the tibia, and the small bones of the hands. rhage, it may be difficult to distinguish between viscous
Pseudotumors may be intraosseous or subperiosteal, or joint fluid and fresh blood with MR imaging in this
they may occur within the soft tissues. It is probable that disease. The intravenous administration of paramagnetic
pseudotumors arise from hemorrhage. contrast agents, such as those containing gadolinium, may
Medullary bone destruction may produce small or large be useful in distinguishing among synovial inflammation,
central or eccentric radiolucent lesions that are fairly well hemorrhage, and joint effusion. The MR imaging charac-
demarcated. Trabeculae can extend across the lesions, teristics of hemosiderin deposition in this disease are
and the surrounding bone is frequently sclerotic. Cortical similar to those seen in other disorders accompanied by
violation and periosteal bone formation may reach consid- recurrent episodes of intra-articular bleeding. Such pro-
erable proportions. A large soft tissue mass may be encoun- cesses include pigmented villonodular synovitis, neoplasms
tered. Mild, moderate, or massive bleeding may occur in such as synovial hemangiomas, neuropathic osteoar-
subperiosteal locations. In the immature skeleton, the thropathy, and chronic renal disease. Hemosiderin
periosteum is lifted easily by the accumulation of blood. collections lead to low signal intensity on all spin echo
708 Section XIII Diseases of the Hematopoietic System
A B
Figure 52–8. Hemophilia: pseudotumors. A, Radiograph of the forearm reveal a pseudotumor involving
the distal portion of the radius. Note new bone formation extending into the soft tissues, with destruction
and deformity of the underlying bone. B, Striking bone and soft tissue abnormalities may accompany
bleeding in hemophilia. The deformed femur has a “cystic” appearance, and in places, its contour has been
obliterated completely. The hip is also abnormal. (A, Courtesy of A. Brower, MD, Norfolk, Va.)
A B
Figure 52–10. Hemophilia: MR imaging. A, Sagittal T1-weighted (TR/TE, 500/16) spin echo MR image
shows anterior extension of the joint (arrow). The intra-articular contents are of low signal intensity.
Irregularity of the subchondral bone of the tibia is evident (arrowhead). B, Sagittal T2-weighted (TR/TE,
2000/80) spin echo MR image shows persistent low signal intensity anteriorly, with peripheral regions of
higher signal intensity (arrow). Abnormal signal intensity is also seen in the tibia (arrowhead). The articular
findings indicate synovial fibrosis or hemosiderin deposition (low signal intensity) and inflammation (high
signal intensity).
sequences and, to a greater degree, on all gradient echo In the synovial membrane, hypertrophy and inflammation,
sequences (Fig. 52–11). subsynovial fibrosis, and hemosiderin deposition are known
Subchondral cystic lesions, a prominent feature of responses to experimental hemarthrosis and resemble the
hemophilic arthropathy, can be evaluated with MR imag- findings in other articular disorders, particularly pigment-
ing. The signal characteristics of these cysts, however, are ed villonodular synovitis. Cartilaginous abnormalities
dependent on the precise imaging sequence used and the occurring after hemarthrosis are less constant. The role
contents of the lesions. Fluid, fibrotic material, hemor- of intra-articular iron deposits in the pathogenesis of
rhage, or hemosiderin, in various combinations, may be hemophilic arthropathy is not clear. The osseous abnor-
present in the subchondral cysts, leading to inhomogeneity malities in hemophilic joints may result from certain
in signal intensity in some cases. Cysts with high signal toxic and chemical effects on bone and elevation of intra-
intensity on both T1- and T2-weighted spin echo MR articular pressure (due to hemarthrosis) and intramarrow
images are related to recent hemorrhage; those with low pressure (due to focal destruction of weight-bearing sur-
signal intensity on T1-weighted and high signal intensity faces). Hyperemia may be responsible for epiphyseal over-
on T2-weighted spin echo MR images contain non- growth in hemophilia. Osteoporosis may relate to increased
hemorrhagic fluid; and those with low signal intensity in blood flow in capsular and epiphyseal blood vessels, as
both types of MR images relate to the presence of fibrous well as to disuse and immobilization.
tissue and hemosiderin (see Fig. 52–11).
The signal behavior of these pseudotumors is com-
plex, reflecting the effects of remote and recurrent bleed- Differential Diagnosis
ing and clot organization. A peripheral margin of low Hemarthrosis is not confined to hemophilic arthropathy.
signal intensity on T1- and T2-weighted spin echo MR It is frequent after trauma, in other articular and nonar-
sequences is consistent with the presence of fibrous tissue ticular disorders such as scurvy or myeloproliferative
or hemosiderin, or both, in the wall of the pseudotumor. disease, and after excessive administration of anticoagulant
Less uniform, however, are the signal characteristics of medication. In these other conditions, permanent
the interior portions of the pseudotumor, which may cartilaginous and osseous findings resembling those of
reveal regions of either high or low signal intensity on hemophilia are not encountered.
one or both of these sequences. Intramuscular pseudo- Articular abnormalities of hemophilia most resemble
tumors (as well as those in other locations) may reveal the changes of juvenile chronic arthritis (Table 52–2). It
mural nodules (Fig. 52–12). is often impossible to distinguish between these two dis-
orders on the basis of radiographic abnormalities in a
single joint. Rather, it is the distribution of articular
Pathogenesis of Hemophilic Arthropathy
abnormalities in hemophilia and juvenile-onset rheuma-
Generally, it is assumed that arthropathy in hemophilia toid arthritis (and other forms of juvenile chronic arthri-
results from intra-articular and periarticular hemorrhage. tis) that permits an accurate radiographic diagnosis. In
A B
Figure 52–11. Hemophilia: MR imaging—hemosiderin deposition. A, Sagittal intermediate-weighted
(TR/TE, 2100/20) spin echo MR image demonstrates synovial proliferation of low signal intensity extend-
ing into and distorting Hoffa’s fat pad (arrows). B, Sagittal T2-weighted three-dimensional (TR/TE, 30/10;
flip angle, 40 degrees) gradient echo MR image demonstrates “blooming” of the hemosiderin within the
synovium (arrows) and a posterior tibial cyst containing fluid or hemorrhage (curved arrow).
A B
Figure 52–12. Hemophilia: MR imaging—pseudotumors. Sagittal T1-weighted (TR/TE, 600/20) (A)
and T2-weighted (TR/TE, 1500/80) (B) spin echo MR images reveal a pseudotumor in the gastrocnemius
muscle with an elliptic shape. Multiple various-sized lobulated nodules are seen attached to the capsule (arrows),
exhibiting heterogeneous signal intensity compatible with blood clots in various stages of organization.
(From Jaovisidha S, Ryu KN, Hodler J, et al: Hemophilic pseudotumor: Spectrum of MR findings. Skeletal
Radiol 26:468, 1997.)
710
Chapter 52 Bleeding Disorders 711
TABLE 52–2 accompanying neuromuscular diseases such as cerebral
palsy, muscular dystrophy, and poliomyelitis may also
Hemophilia versus Juvenile-Onset Rheumatoid Arthritis resemble those of hemophilia. On rare occasions, intra-
(JRA)
articular bleeding in association with certain hemorrhagic
diatheses may lead to an arthropathy identical to that of
Hemophilia JRA
hemophilia (Table 52–3).
Common articular sites Knee, wrist, elbow Knee, ankle,
wrist, hand
Soft tissue swelling + + BLEEDING DIATHESES AND HEMANGIOMAS
Osteoporosis + +
General Features
Joint space narrowing ± ±
Bony ankylosis – + Hemangiomas are vascular tumors, most often located
Epiphyseal overgrowth + + in the skin, that appear in the early postnatal period.
Growth inhibition – + Hemangiomas may be associated with unusual syndromes,
Epiphyseal collapse or + + some of which produce hematologic abnormalities.
osteonecrosis The association of varicose veins, soft tissue and bony
Periostitis ± + hypertrophy, and cutaneous hemangiomas is known as
Klippel-Trénaunay syndrome. An underlying vascular
Pseudotumors + –
abnormality consisting of atresia and hypoplasia or
Spondylitis – +
obstruction of the deep venous system is noted. When
these findings are associated with an arteriovenous fistula,
+, present; –, absent. the condition is commonly called Parke-Weber syndrome.
Additional variations of Klippel-Trénaunay syndrome
include cutaneous lymphangiomas and facial hemihyper-
hemophilia, the knee, ankle, and elbow are altered most trophy. Klippel-Trénaunay syndrome affects both sexes.
commonly; in juvenile-onset rheumatoid arthritis, the The nevus is usually present at birth, and varices appear,
articulations of the hands and wrists, as well as the larger sometimes at birth but typically in the first few years
joints and the spine, may be affected. of life. Osseous and soft tissue hypertrophy is also evident
In some joints, the findings of hemophilia may simu- in early life but becomes more obvious during the
late those of pigmented villonodular synovitis or infec- adolescent growth spurt. Usually only one lower limb
tion. These latter disorders are characteristically mono- is involved. The natural history of Klippel-Trénaunay
articular, whereas joint involvement in hemophilia is syndrome is variable, although worsening of venous
usually polyarticular. Articular and skeletal alterations insufficiency is the rule.
TABLE 52–3
Heritable Disorders of Blood Coagulation
Hemorrhagic
Disorder Hereditary Tendency Hemarthrosis
Classic hemophilia (factor VIII deficiency) X-linked Mild to severe Common
Christmas disease (factor IX deficiency) X-linked Mild to severe Common
von Willebrand’s disease (factor VIII
deficiency, platelet abnormalities) Autosomal dominant Mild to severe Uncommon
Plasma thromboplastin antecedent (PTA, factor IX) Autosomal recessive Mild Rare
deficiency
Hageman trait (deficiency of Hageman factor, factor XII) Autosomal recessive None to mild Usually absent
Fletcher trait (deficiency of plasma prekallikrein) Autosomal recessive None Absent
Fitzgerald trait (deficiency of high-molecular-weight Autosomal recessive None Absent
kininogen)
Parahemophilia (factor V deficiency) Autosomal recessive Moderate Rare
Stuart factor deficiency (factor X deficiency) Autosomal recessive Severe Variable
Factor VII deficiency Autosomal recessive Mild to moderate Variable
Hereditary hypoprothrombinemia (prothrombin deficiency) Autosomal recessive Mild to severe Variable
Congenital deficiency of fibrinogen Autosomal recessive Severe Variable
Congenital dysfibrinogenemia (structural abnormality Autosomal dominant None to mild Variable
of fibrinogen)
Congenital deficiency of fibrin-stabilizing factor (factor XIII Unknown Severe Rare
deficiency)
712 Section XIII Diseases of the Hematopoietic System
Kasabach-Merritt syndrome consists of papillary suffer joint stiffness and “degenerative” changes. Rarely,
hemangiomas and extensive purpura. Hematologic abnor- an arthropathy of the knee resembling hemophilia may
malities associated with Kasabach-Merritt syndrome be evident (Fig. 52–13).
include thrombocytopenia; deficiencies of factors V, VII,
VIII, and IX; prothrombin depression; hypofibrinogene-
mia; and microangiopathic hemolytic anemia. A consump- FURTHER READING
tion coagulopathy caused by intravascular coagulation
within the hemangioma makes these patients (usually Arnold WD, Hilgartner MW: Hemophilic arthropathy:
infants) susceptible to hemorrhage. Current concepts of pathogenesis and management. J Bone
Joint Surg Am 59:287, 1977.
Brant EE, Jordan HH: Radiologic aspects of hemophilic
Articular Abnormalities pseudotumors in bone. AJR Am J Roentgenol 115:525,
Arthropathies are not commonly reported in either 1972.
deValderrama JAF, Matthews JM: The haemophilic pseudo-
Klippel-Trénaunay syndrome or Kasabach-Merritt syn- tumor or haemophilic subperiosteal haematoma. J Bone
drome, although, in the latter condition, patients may Joint Surg Br 47:256, 1965.
Gaary E, Gorlin JB, Jaramillo D: Pseudotumor and arthropathy
in the knees of a hemophiliac. Skeletal Radiol 25:85, 1996.
Greene WB, Yankaskas BC, Guilford WB: Roentgenographic
classifications of hemophilic arthropathy. J Bone Joint Surg
Am 71:237, 1989.
Hermann G, Gilbert MS, Abdelwahab IF: Hemophilia: Evalu-
ation of musculoskeletal involvement with CT, sonography,
and MR imaging. AJR Am J Roentgenol 158:119, 1992.
Johnson JB, Davis TW, Bullock WH: Bone and joint changes
in hemophilia. Radiology 63:64, 1954.
Jordan HH: Hemophilic Arthropathies. Springfield, Ill, Charles
C Thomas, 1958.
Kasabach HH, Merritt KK: Capillary hemangioma with exten-
sive purpura: Report of a case. Am J Dis Child 59:1063,
1940.
Kontras SB: The Klippel-Trenaunay-Weber syndrome. Birth
Defects 10:177, 1974.
Pettersson H, Ahlberg S, Nilsson IM: A radiologic classifica-
tion of hemophilic arthropathy. Clin Orthop 149:153, 1980.
Pettersson H, Gilbert MS: Diagnostic Imaging in Hemophilia.
Berlin, Springer-Verlag, 1985.
Phillips GN, Gordon DH, Martin EC, et al: The Klippel-
Trenaunay syndrome: Clinical and radiological aspects.
Radiology 128:429, 1978.
Plazanet FR, du Boullay CH, De Faux F, et al: Open synovec-
tomy for the prevention of recurrent hemarthrosis of the
ankle in patients with hemophilia: A report of five cases with
magnetic resonance imaging documentation. Rev Rhum
Engl Ed 64:166, 1997.
Resnick D, Oliphant M: Hemophilia-like arthropathy of the
knee associated with cutaneous and synovial hemangiomas:
Report of 3 cases and review of the literature. Radiology
114:323, 1975.
Richardson ML, Helms CA, Vogler JB III, et al: Skeletal changes
Figure 52–13. Arthropathy in association with hemangiomas in neuromuscular disorders mimicking juvenile rheumatoid
and bleeding diatheses. This 11-year-old had recurrent bilateral arthritis and hemophilia. AJR Am J Roentgenol 143:893,
knee pain and abdominal discomfort. At the age of 6 weeks, 1984.
multiple hemangiomas of the lower extremities and abdomen Roebuck DJ: Klippel-Trenaunay and Parke-Weber syndromes.
were noted. Extensive clinical and laboratory evaluation con- AJR Am J Roentgenol 169:311, 1997.
firmed the diagnosis of multiple giant hemangiomas and vari- Roosendaal G, Vianen ME, Wenting MJG, et al: Iron deposits
cosities (Klippel-Trénaunay syndrome), Kasabach-Merritt and catabolic properties of synovial tissue from patients with
syndrome, and a consumption coagulopathy. On this radiograph haemophilia. J Bone Joint Surg Br 80:540, 1998.
of the knee, a soft tissue phlebolith (arrow) is apparent. The Vaz W, Cockshott WP, Martin RF, et al: Myositis ossificans in
distal femoral and proximal tibial epiphyses are enlarged, with hemophilia. Skeletal Radiol 7:27, 1981.
irregularities of the subchondral bone and a widened inter- Weber FP: Hemangiectatic hypertrophy of limbs—congenital
condylar notch (open arrows). (From Resnick D, Oliphant M: phlebacteriectasis and so-called congenital varicose veins. Br
Hemophilia-like arthropathy of the knee associated with cuta- J Child Dis 15:13, 1918.
neous and synovial hemangiomas: Report of 3 cases and review Wilson DA, Prince JR: MR imaging of hemophilic pseudo-
of the literature. Radiology 114:323, 1975.) tumors. AJR Am J Roentgenol 150:349, 1988.
SECTION
XIV
Infectious Diseases
CHAPTER 53
Osteomyelitis, Septic Arthritis, and Articular infection implies a septic process of the joint
itself. Septic arthritis can occur as an isolated condition
Soft Tissue Infection: Mechanisms that may soon spread to the neighboring bone or as a
and Situations complication of adjacent osteomyelitis or soft tissue
infection.
A sequestrum represents a segment of necrotic bone
that is separated from living bone by granulation tissue.
SUMMARY OF KEY FEATURES Sequestra may reside in the marrow for protracted periods,
A thorough understanding of regional anatomy is harboring living organisms that have the capability of
fundamental to the accurate interpretation of clinical, evoking an acute flare-up of the infection.
radiographic, and pathologic characteristics of infections An involucrum denotes a layer of living bone that has
of bone, joint, and soft tissue. In most persons with such formed about the dead bone. It can surround and even-
infections, a specific mechanism of contamination can tually merge with the parent bone.
be recognized; infection may be derived from Cloaca is an opening in the involucrum through which
hematogenous seeding, spread from a contiguous granulation tissue and sequestra can be discharged.
source, direct implantation, or operative contamination. Sinuses are tracts leading to the skin surface from the
The radiographic findings of osteomyelitis (including bone.
abscess, involucrum, and sequestration), septic arthritis A bone abscess (Brodie’s abscess) is a sharply delineated
(including joint space loss and marginal and central focus of infection. It is of variable size, can occur at single
osseous erosions), and soft tissue suppuration (including or multiple locations, and represents a site of active infec-
swelling, radiolucent streaks, and periostitis) are generally tion. It is lined by granulation tissue and frequently is
delayed for a variable period after the clinical onset of surrounded by eburnated bone.
infection. Other diagnostic techniques, including Garré’s sclerosing osteomyelitis is a sclerotic, nonpurulent
scintigraphy and magnetic resonance imaging, allow an form of osteomyelitis. Although this term is applied care-
accurate diagnosis at an earlier stage of the process. lessly to any form of osteomyelitis with severe osseous
eburnation, it should be reserved for those cases in which
intense proliferation of the periosteum leads to bony
deposition and in which no necrosis or purulent exudate
and little granulation tissue are present.
INTRODUCTION The clinical stages of osteomyelitis are frequently desig-
Infection of bone, joint, and soft tissue is a common and nated acute, subacute, and chronic. This does not imply
disturbing problem that often represents a diagnostic and that definitive divisions exist between one stage and
therapeutic challenge. Early diagnosis is imperative because another, nor does it signify that all cases of osteomyelitis
it allows prompt treatment, which can prevent many of progress through each of these phases. The relatively
the dreaded complications. abrupt onset of clinical symptoms and signs during the
initial stage of infection is a clear indication of the acute
osteomyelitic phase; if this acute phase passes without
TERMINOLOGY complete elimination of infection, subacute or chronic
Ostemyelitis implies an infection of bone and marrow. It most osteomyelitis can become apparent. The transition from
commonly results from bacterial infections, although fungi, acute to subacute and chronic osteomyelitis may indicate
parasites, and viruses can infect the bone and the marrow. that therapeutic measures have been inadequate.
Infective (suppurative) osteitis indicates contamination
of the bone cortex. Infective osteitis can occur as an iso-
OSTEOMYELITIS
lated phenomenon or, more frequently, as a concomitant
to osteomyelitis. Routes of Contamination
Infective (suppurative) periostitis implies contamination Osseous (and articular) structures can be contaminated
of the periosteal cloak that surrounds the bone. In this by four principal routes:
situation, a subperiosteal accumulation of organisms fre-
quently leads to infective osteitis and osteomyelitis. 1. Hematogenous spread of infection. Infection can reach
Soft tissue infection indicates contamination of cutaneous, the bone (or joint) via the bloodstream.
subcutaneous, muscular, fascial, tendinous, ligamentous, 2. Spread from a contiguous source of infection. Infection can
or bursal structures. This may be seen as an isolated condi- extend into the bone (or joint) from an adjacent con-
tion or as a complication of periosteal, osseous, marrow, taminated site. Cutaneous, sinus, and dental infections
or articular infection. are three important sources of extraskeletal infective foci.
713
714 Section XIV Infectious Diseases
3. Direct implantation. Direct implantation of infectious younger age group, the long tubular bones of the extrem-
material into the bone (or joint) may occur following ities are especially vulnerable; in adults, hematogenous
puncture or penetrating injuries. osteomyelitis is encountered more frequently in the axial
4. Postoperative infection. Postoperative infection may skeleton.
occur via direct implantation, spread from a con-
tiguous septic focus, or hematogenous contamination Vascular Anatomy
of the bone (or joint).
The vascular supply of a tubular bone is derived from
several points of arterial inflow, which become compli-
Hematogenous Infection cated sinusoidal networks within the bone (Fig. 53–1).
One or two diaphyseal nutrient arteries pierce the cortex
Bacteremia
and divide into ascending and descending branches. As
Bacteria usually enter the blood vessels (or the lym- they extend to the ends of the bones, they branch repeat-
phatics and then the blood vessels) by direct extension edly, becoming finer channels, and are joined by the
from extravascular sites of infection, which include the terminals of metaphyseal and epiphyseal arteries. The
genitourinary, gastrointestinal, biliary, and respiratory metaphyseal arteries originate from neighboring systemic
systems; the skin and soft tissue; and other structures. In vessels, whereas the epiphyseal arteries arise from peri-
some instances, no primary source of infection is iden- articular vascular arcades. The arteries within the bone
tifiable. Bacteremia is often transient and totally asymp- marrow form a series of cortical branches that connect
tomatic; however, in some cases, prominent clinical with the fenestrated capillaries of the haversian systems.
manifestations may occur. At the bony surface, the cortical capillaries form con-
A single pathogenic organism is usually responsible nections with overlying periosteal plexuses, which them-
for hematogenous osteomyelitis. In neonates and infants, selves are derived from the arteries of the neighboring
Staphylococcus aureus, group B streptococcus, and Escherichia muscles and soft tissues. The cortices of the tubular
coli are the bone isolates recovered most frequently. In bones derive nutrition from both the periosteal and the
children older than 1 year of age, S. aureus, Streptococcus medullary circulatory systems. The central arterioles
pyogenes, and Haemophilus influenzae are responsible for drain into a thin-walled venous sinus, which subsequently
most cases of hematogenous osteomyelitis. In those older unites with veins that retrace the course of the nutrient
than 4 years, staphylococci are the major pathogens in arteries, piercing the cortex at various points and joining
this disease, as the prevalence of osteomyelitis related to larger and larger venous channels.
H. influenzae decreases. Gram-negative organisms assume Joints receive blood vessels from periarterial plexuses
importance as pathogens in bone and joint infections in that pierce the capsule to form a vascular plexus in the
adults and in intravenous drug abusers. A recent surgical deeper part of the synovial membrane. The blood vessels
procedure or concurrent soft tissue infection is frequently of the synovial membrane terminate at the articular
associated with staphylococcal septicemia and osteomye- margins as looped anastomoses (circulus articularis vascu-
litis; disorders of the gastrointestinal or genitourinary tract losus). The epiphysis and the adjacent synovium share a
may initiate a gram-negative septicemia; and an acute or common blood supply.
chronic respiratory infection is important in the patho- The radiographic and pathologic features of osteo-
genesis of tuberculous, fungal, and pneumococcal osteo- myelitis differ in children, infants, and adults, related in
myelitis. Blood cultures are positive in approximately large part to peculiarities of the vascular anatomy of the
50% of patients with acute hematogenous osteomyelitis. tubular bones in each age group (Fig. 53–2; Table 53–1).
A B C
Figure 53–2. Normal vascular patterns of tubular bone, based
on age. A, In the child, the capillaries of the metaphysis turn
sharply, without violating the open growth plate. B, In the infant,
some metaphyseal vessels may penetrate or extend around the
open growth plate, ramifying in the epiphysis. C, In the adult,
with closure of the growth plate, a vascular connection between
the metaphysis and epiphysis can be recognized.
Age-Related Patterns
The development of hematogenous osteomyelitis varies
according to age-related characteristics of the bones (Fig.
53–3; Table 53–2).
TABLE 53–2
Hematogenous Osteomyelitis of Tubular Bones
B
718 Section XIV Infectious Diseases
growth plate by a tortuous channel. Radiographic detec- One or more areas of osseous necrosis are commonly
tion of this channel is important; identification of a meta- situated in the medullary aspect of a tubular bone
physeal defect connected to the growth plate by such a (sequestration is less prominent in flat bones), where they
tract ensures the diagnosis of osteomyelitis. In the di- create radiodense bony spicules (Fig. 53–9). The seques-
aphysis, the radiolucent abscess cavity may be located in trum frequently is marginated sharply, surrounded by
central or subcortical areas of the spongiosa or in the granulation tissue. Sequestra may extrude through cor-
cortex itself and may contain a central sequestrum. In an tical breaks, extending into the adjacent soft tissues, where
epiphysis, a circular, well-defined osteolytic lesion is seen, they eventually may be discharged through draining sinuses.
which, in the immature skeleton, may border on the
chondro-osseous junction or on the physis, where it may Sclerosing Osteomyelitis. In the subacute and chronic
extend into the metaphysis. When an abscess is located in stages of osteomyelitis, considerable periosteal bone
the cortex, its radiographic appearance, consisting of a formation can surround the altered cortex, and an
lucent lesion with surrounding sclerosis and periostitis, increased number and size of spongy trabeculae can
simulates that of an osteoid osteoma or stress fracture. A reappear in the affected marrow, leading to considerable
rounded radiolucent lesion without calcification is radiodensity and contour irregularity of the affected bone
characteristic of a cortical abscess; a circular lucent area (Fig. 53–10). Cystic changes may occur within the scle-
with or without calcification that is smaller than 2 cm is rotic area, but sequestra are uncommon. At any site, the
more typical of an osteoid osteoma; and a linear lucent radiographic findings of sclerosing osteomyelitis resem-
shadow without calcification is characteristic of a stress ble those of osteoid osteoma, fibrous dysplasia, and Ewing’s
fracture. In any skeletal location, computed tomography sarcoma.
(CT) or magnetic resonance (MR) imaging can be used
to better assess the extent of the abscess and any signs of Infection from a Contiguous Source
its reactivation (Fig. 53–8).
General Clinical Features
Sequestration. During the course of hematogenous In most cases of osteomyelitis and septic arthritis arising
osteomyelitis, cortical sequestration may become evident. from such a contiguous source, soft tissue infections are
720 Section XIV Infectious Diseases
Specific Locations
metacarpophalangeal joints are altered less commonly.
Hand. Three distinct routes are available to organisms Such tenosynovitis causes exquisite tenderness over the
that become lodged in the soft tissues of the hand; infec- course of the sheath, a semiflexed position of the finger,
tion may disseminate via tendon sheaths, fascial planes, severe pain on extension of the finger, and fusiform
or lymphatics (Fig. 53–12). Infective digital tenosynovitis swelling of the digit.
can result from a puncture wound, particularly in a flexor Infections in the fascial planes of the hand are numerous
crease of the finger, where skin and sheath are intimately but result in joint or bone alterations less frequently than
related. A sheath infection may perforate into an adjacent do those in the synovial sheaths. Lymphangitis may result
bone or joint in the finger; the most characteristic site of from superficial injuries. In intense cases, complications
such extension is the proximal interphalangeal articula- may include tenosynovitis, septicemia, osteomyelitis, and
tions and adjacent middle phalanx (Fig. 53–13). The septic arthritis.
Chapter 53 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Mechanisms and Situations 721
Foot. The plantar aspect of the foot is especially vulner-
able to soft tissue infection. Foreign bodies, puncture
wounds, or skin ulceration from weight bearing can
represent the portal of entry for various organisms. In a
diabetic patient, soft tissue breakdown over certain pres-
sure points (e.g., metatarsal heads, calcaneus) leads to
infection that is combined with vascular and neurologic
abnormalities.
Puncture wounds of the plantar aspect of the foot can
lead to osteomyelitis and septic arthritis (Fig. 53–16). The
infective organisms can vary, but gram-negative agents
such as Pseudomonas aeruginosa are frequently implicated;
this is not surprising, because these organisms are usually
found in the soil and may be normal inhabitants of skin.
Typically, local pain and swelling appear within days after
a puncture wound, although radiographs usually are
normal at this time. After a delay of 1 to 3 weeks, the
radiographs reveal typical abnormalities of osteomyelitis
or septic arthritis. Osteomyelitis of the os calcis is a
recognized complication of repeated heel punctures in
neonates.
The clinical, radiographic, and pathologic character-
istics of osteomyelitis (and septic arthritis) complicating
foot infections in diabetic patients are modified by the
associated problems of these persons, including vascular
insufficiency and neurologic deficit. The radiographic
picture usually reveals significant soft tissue swelling and
mottled osteolysis (Fig. 53–17). Osteosclerosis, fragmen-
tation, periostitis, and soft tissue gas may be seen. In
some diabetic patients with foot infections complicated
Figure 53–10. Chronic sclerosing osteomyelitis. Chronic by osteomyelitis, findings can simulate those of diabetic
osteomyelitis can be associated with considerable new bone neuropathic osteoarthropathy, and differentiation of
formation. In this patient, a cortical abscess contains a neurologic and infectious processes can be difficult. In
sequestrum (arrow) and is surrounded by sclerosis (arrowheads). fact, both infection and neuropathic osteoarthropathy of
The appearance is reminiscent of that of an osteoid osteoma. the midfoot and forefoot frequently coexist in diabetic
patients.
TABLE 53–4
Osteomyelitis Due to Spread from a Contiguous Source
of Infection: Radiographic-Pathologic Correlation
Figure 53–11. Diagrammatic representation of the sequen-
tial steps of osteomyelitis resulting from a contiguous contami-
nated source. 1, Initially, a soft tissue focus of infection is Pathologic Abnormality Radiographic Abnormality
apparent. Occasionally, such a focus can irritate the underlying Soft tissue contamination and Soft tissue swelling, mass
bone, producing periostitis without definite invasion of the cortex. abscess formation formation, obliteration
2, The infection subsequently invades the cortex, spreading via of tissue planes
haversian and Volkmann’s canals. 3, Finally, the medullary bone Infectious invasion of the Periostitis
and marrow spaces are affected. periosteum with lifting of the
membrane and bone formation
Subperiosteal abscess formation Cortical erosion
A felon results from infection in the terminal pulp and cortical invasion
space. Bone involvement is not infrequent in neglected Infection in haversian and Cortical lucency and
cases because of the close proximity of the terminal phalanx Volkmann’s canals of cortex destruction
(Fig. 53–14). Subcuticular abscesses of the nail fold are Contamination and spread in Bone lysis
termed paronychia. On rare occasions, osseous destruc- marrow
tion of a terminal phalanx may be evident (Fig. 53–15).
722 Section XIV Infectious Diseases
Figure 53–12. Spread of infection in the hand: available anatomic pathways A, Drawing demonstrates the
relationships of the tendon sheaths, bursae, and fascial planes (thenar space, midpalmar space). B, Drawing
of a section through the metacarpal bones outlines two spaces—the midpalmar and thenar spaces—
separated by a septum and located above the digital flexor tendon sheaths. Note the close relationship
between the sheath of the index finger and the thenar space and between the sheaths of the third, fourth,
and fifth fingers and the midpalmar space. (From Resnick D: Osteomyelitis and septic arthritis complicating
hand injuries and infections: Pathogenesis of roentgenographic abnormalities. J Can Assoc Radiol 27:21,
1976.)
especially near the sacrum, ischial tuberosities, trochan- innominate bones and proximal portions of the femora,
teric regions, and buttocks. Local soft tissue infection areas subjacent to sites of skin breakdown, and is related
and bacteremia are commonly associated with decubitus to spread from a contiguous contaminated source.
ulcers. Osteomyelitis is observed most commonly in the The accurate diagnosis of osteomyelitis complicating
pressure sores is difficult, owing to a number of other
conditions that may become evident in immobilized or
paralyzed patients. Pressure-related changes in bone are
not infrequent, leading to flattening and sclerosis of bony
Human Bites
The most common cause of human bite injury is a fist
blow to the mouth that results in laceration of the
dorsum of the metacarpophalangeal joint. Joint infection
is more common than bone infection in these cases. S.
aureus or Streptococcus species are the usual implicated
organisms. The radiographic findings, which are particu-
larly well shown on steep oblique and lateral radiographs,
include peculiar bony defects and fractures, tooth frag-
ments, and osseous and articular destruction (Fig. 53–18).
Animal Bites
Superficial animal bites or scratches can inoculate local
soft tissues, leading to infection of underlying bones and
joints. Deep animal bites can introduce organisms
directly into osseous and articular structures. Dog bites
account for approximately 90% of these injuries and cat
bites for about 10%. Approximately 5% of dog bites and
20% to 50% of cat bites become infected significantly.
The infecting organisms vary, but Pasteurella multocida is
commonly implicated, especially in cat bites. Any anatomic
site can be affected, although animal bites are seen pre-
dominantly in the hand, arm, and leg (Fig. 53–19).
A B
Figure 53–19. Infection due to direct implantation: animal
bites. A, After a cat bite, this patient developed Pasteurella
osteomyelitis and septic arthritis. Observe soft tissue swelling,
osseous destruction of the proximal and middle phalanges, and
joint space narrowing and flexion at the proximal interpha-
langeal joint. B, In a different patient who developed Pasteurella
osteomyelitis and septic arthritis after a cat bite, a coronal T1-
weighted (TR/TE, 800/20) spin echo MR image obtained with
fat saturation technique and intravenous gadolinium enhance-
ment shows high signal intensity in the third metacarpo-
phalangeal joint and adjacent bone and soft tissue.
Postoperative Infection
Postoperative infections occur as a result of contamina-
Figure 53–18. Infection due to direct implantation: human tion of bones and joints from adjacent infected soft
bites. Destruction of the third metacarpal head (solid arrow) and
tissues, direct inoculation of osseous and articular tissue
a narrowed metacarpophalangeal joint (open arrow) resulted
from infection after a fistfight in which the patient’s fist struck at the time of surgery, or, less frequently, hematogenous
the opponent’s teeth. (From Resnick D: Osteomyelitis and spread to an operative site from a distant location.
septic arthritis complicating hand injuries and infections: Particularly troublesome are instances of infection that
Pathogenesis of roentgenographic abnormalities. J Can Assoc occur after internal fixation of fractures, intervertebral
Radiol 27:21, 1976.) disc surgery, median sternotomy, and various types of
Chapter 53 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Mechanisms and Situations 725
reconstructive procedures and arthroplasty. One or more of neoplasm is variable, although a time span of 20 to
organisms may be implicated; S. aureus is the most com- 30 years is typical. Neoplasm most frequently arises adja-
mon pathogen. cent to the femur and the tibia and is clinically evident
One special type of postoperative infection relates to as pain, increasing drainage, hemorrhage, onset of a foul
the transcutaneous insertion of pins into bone. The odor from the sinus tract, a mass, and lymphadenopathy.
causative organisms vary, but infections caused by gram- Radiographically, there is progressive destruction of bone
negative bacteria are common. The mechanisms of (Fig. 53–22). The prognosis is guarded.
contamination are also variable; in some cases, the pins
are inserted into bones that are already the site of Amyloidosis. Secondary amyloidosis can complicate
osteomyelitis, whereas in others, osseous infection occurs chronic osteomyelitis. This complication has become less
at the time of or after pin insertion. Radiographs reveal frequent, however, owing to improvement in the chemo-
progressive osteolysis about the metal or, after removal of therapy of infection. It is seen in less than 5% of cases of
the pin, a ring sequestrum (Fig. 53–20). In the latter chronic osteomyelitis.
instance, the central circular radiolucent area created by
the pin itself is surrounded by a ring of bone, which, in
turn, is surrounded by an area of osteolysis. Modifications and Diagnostic Difficulties
Antibiotic-Modified Osteomyelitis. The previous discus-
sion was concerned with the radiographic and pathologic
Complications
findings of untreated osteomyelitis. During the early
Severe Osteolysis. If osteomyelitis is not treated ade- healing phase of osteomyelitis, bone resorption continues
quately or early enough, severe osteolysis may ensue. as damaged osseous tissue is removed. Therefore,
Large foci of destruction eventually can lead to disap- radiographically evident increased destruction can occur
pearance of long segments of tubular or flat bones. at a time when the clinical picture is improving.
Epiphyseal Growth Disturbance. Injury to the cartilage cells Active and Inactive Chronic Osteomyelitis. Differentiation
on the epiphyseal side of the growth plate is irreparable, of active and inactive chronic osteomyelitis by imaging
and subsequent growth disturbances are to be expected. techniques can be extremely difficult, but certain indica-
Even with severe epiphyseal disintegration, however, tions on the radiograph may be helpful (Table 53–5).
some regeneration of the epiphysis can occur after
eradication of the infection (Fig. 53–21). It is difficult to
predict the occurrence and extent of epiphyseal recovery
after injury.
TABLE 53–5
Radiographic Signs of Activity in Chronic Osteomyelitis
A B
C D
Figure 53–23. Septic arthritis: potential routes of contamination. A, Hematogenous spread of infection
to a joint can result from direct lodgment of organisms in the synovial membrane (1). Spread into the joint
from a contiguous source can occur from a metaphyseal focus that extends into the epiphysis and from there
into the joint (2), from a metaphyseal focus with extension into the joint when the growth plate is intra-
articular (3), or from a contiguous soft tissue infection (4). Direct implantation after a penetrating wound
(5) can also lead to septic arthritis. B, Hematogenous spread of infection to a joint can occur owing to
vascular continuity between the epiphysis and the synovial membrane. The vessels shown include arterioles
(1), venules (2), and capillaries (3) of the capsule; periosteal vessels (4); the nutrient artery (5); and
metaphyseal-epiphyseal anastomoses (6). C, Sequence of events by which the synovial membrane can
become infected from an osseous focus before the joint fluid is contaminated. D, Spread from a contiguous
osseous surface can result from penetration of the cartilage (1) or pathologic fracture with articular
contamination (2). In this situation, synovial fluid may become infected before the synovial membrane.
Chapter 53 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Mechanisms and Situations 729
TABLE 53–6
Septic Arthritis: Radiographic-Pathologic Correlation
B
Figure 53–24. Septic arthritis: pathologic abnormalities. 1,
Normal synovial joint. 2, An edematous, swollen, and hyper- Figure 53–25. Septic arthritis: hematogenous spread of
trophic synovial membrane becomes evident. 3 and 4, Accumu- infection. A, Radiograph reveals joint space narrowing and
lating inflammatory pannus leads to chondral destruction and osseous erosions, which predominate at the margins of the talus
to marginal and central osseous erosions. 5, Bony ankylosis may (arrows). B, In this patient with acquired immunodeficiency
eventually result. syndrome, a sagittal STIR (TR/TE, 3200/18; inversion time,
150 msec) MR image show classic features of septic arthritis of
the ankle, with cartilage and bone erosion and marrow edema
in the tibia and talus.
Radiographic-Pathologic Correlation
Usually, radiographic evidence exists that the infective
process originates outside the articulation. This evidence
may include soft tissue deficit, swelling, or gas forma-
tion; osteomyelitis with typical epiphyseal or metaphy-
seal destruction; and diverticulitis or cystitis with
fistulization. In certain situations, however, joint effusion
and cartilaginous and subchondral osseous destruction are
the first radiographic clues to infection. Once the articu-
lation has been violated, the radiographic and pathologic
abnormalities of the infection are virtually identical to Figure 53–26. Septic arthritis of the hip: infancy. This infant
those associated with hematogenously derived suppura- developed septic arthritis of the right hip and osteomyelitis.
tive joint disease. Note displacement of the “capsular” and obturator fat planes
(solid arrows), obliteration of the iliopsoas fat plane (arrowhead),
and a metaphyseal focus of infection (open arrow). The
Specific Entities femoral head is displaced laterally and is slightly enlarged.
Neonatal septic arthritis affects the hip joint most fre- The soft tissue findings indicative of intra-articular fluid
quently, and S. aureus is the organism most commonly may be of more diagnostic help in this age group than in
implicated. In this age group, infection can reach the hip adults. (Courtesy of J. Weston, MD, Lower Hutt, New
via spread from a metaphyseal focus of osteomyelitis either Zealand.)
directly into the joint (the growth plate is intra-articular)
or to the epiphysis by way of vascular channels that cross
the growth plate. Clinically, infants with septic arthritis
of the hip may manifest irritability, loss of appetite, and
fever. Initial radiographs of the hip are frequently unre-
markable. With accumulation of intra-articular fluid, child (or infant) is an important complication of the
pathologic subluxation or dislocation of the femoral head disease (Fig. 53–28). Osteonecrosis of the epiphysis is
can occur, although the lack of ossification in most of usually not recognized until 6 to 8 weeks after the onset
the proximal capital femoral epiphysis makes this sign of infection. The epiphysis can reveal a generalized
difficult to apply (Fig. 53–26). A helpful finding, how- increase in radiodensity, followed by fragmentation and,
ever, is radiographically detectable osteomyelitis of the less commonly, collapse.
femoral metaphysis manifested as osteolysis, osteoscle-
rosis, or periostitis.
The radiographic findings of hip infection in infants Direct Implantation of Infection
can simulate those of other conditions; therefore, Arthrocentesis for the evaluation of synovial contents
aspiration of the joint is mandatory to firmly establish the or for arthrography can introduce gram-positive or
diagnosis of septic arthritis and to provide guidelines for gram-negative bacteria. Similarly, penetrating injuries,
adequate therapy. Only as the child develops and such as those that occur in a fistfight or from a bullet,
ossification of the immature skeleton proceeds will the knife, nail, or other sharp object, can lead to septic
degree of residual deformity from destruction of the arthritis.
cartilaginous femoral head and acetabulum become
apparent (Fig. 53–27).
Postoperative Infection
Septic arthritis of the hip is also frequent in children,
although the overall frequency of this problem and the Articular surgery in the form of arthroscopy, arthrotomy,
extent of its devastating effects on local cartilage and arthrodesis, arthroplasty, or another procedure can be
bone are less than in infants. It may be associated with complicated by joint infection in the postoperative
the acute onset of fever, pain, swelling, and limping, as period. Infections occurring soon after such procedures
well as a dramatic leukocytosis. On radiographs, are usually related to direct inoculation of the joint
accumulation of intra-articular fluid may produce soft during the operation or to intra-articular spread from an
tissue swelling, capsular distention, and subtle lateral adjacent contaminated focus (e.g., soft tissue abscess).
displacement of the ossified epiphysis. The prognosis of Joint infection occurring long after surgery is frequently
septic arthritis of the hip for a child is far better than that associated with obvious preceding sepsis elsewhere in the
for an infant. Osteonecrosis of the femoral head body and may relate to hematogenous spread to the joint
occurring after metaphyseal or epiphyseal infection in a from this distant process.
Chapter 53 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Mechanisms and Situations 731
A B
Figure 53–27. Septic arthritis of the hip: infancy. A, This neonate developed septic arthritis of the right
hip. The initial radiograph reveals soft tissue swelling and periosteal reaction along the femur (arrowhead).
B, At age 13 years, the ossification centers of the greater and lesser trochanters are apparent. Femoral
dislocation, acetabular shallowness, and absence of epiphyseal ossification are evident. (From Freiberger
RH, Ghelman B, Kaye JJ, and Spragge JW: Hip disease of infancy and childhood. In Moseley RD Jr, et al
[eds]: Current Problems in Radiology. Chicago, Year Book Medical Publishers, 1973.)
A B
A B
Figure 53–34. Infective myositis: soleus muscle. Staphylococcus aureus infection in a 34-year-old patient
with acquired immunodeficiency syndrome. A, On a transaxial T1-weighted (TR/TE, 500/30) spin echo
MR image, a rim of increased signal intensity (arrow) is evident. B, With T2 weighting (TR/TE, 2000/90),
a similar transaxial image shows a central area of marked hyperintensity surrounded by a hypointense band
(arrow), which is itself surrounded by a more diffuse region of hyperintensity. The MR imaging findings are
those of an abscess. (From Fleckenstein JL, Burn DK, Murphy FK, et al: Differential diagnosis of bacterial
myositis in AIDS: Evaluation with MR imaging. Radiology 179:653, 1991.)
OTHER DIAGNOSTIC TECHNIQUES Figure 53–38. Acute osteomyelitis: CT. In this 54-year-old
Computed Tomography man with Enterobacter osteomyelitis of the hip and femur,
transaxial CT scan at the level of the proximal portion of the
The primary applications of CT to the evaluation of femur reveals intramedullary gas collections (arrow). (Courtesy
musculoskeletal infections are the delineation of the of V. Vint, MD, San Diego, Calif.)
Chapter 53 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Mechanisms and Situations 737
medullary canal or in the adjacent joint are also reported
in osteomyelitis and septic arthritis. CT evaluation in
patients with subacute or chronic osteomyelitis may reveal
cortical sequestration, cloacae, and bone and soft tissue
abscesses (Fig. 53–39).
Sinography
Retrograde injection of contrast material defines the
course and extent of the sinus tract and its possible com-
munication with an underlying bone or joint. Sinography
may be combined with CT scanning or MR imaging for
better delineation of the sinus tracts.
Arthrography
The principal reason for performing a joint puncture in
the clinical setting of infection is to obtain fluid for Figure 53–39. Chronic osteomyelitis: CT. Transverse CT
bacteriologic examination. After removal of the joint scan reveals bone destruction and a sequestrum in the calcaneus
that resulted from a pin tract infection.
contents, however, contrast opacification of the joint can
be used to outline the extent of the synovial inflammation
and the presence of capsular, tendinous, and soft tissue
injury. a photo-deficient area (“cold” spot), a finding that is
related to fulminant infection with thrombosis or vas-
cular compression; within a few days, however, increased
Ultrasonography accumulation of the radioisotope (“hot” spot) is typical
Ultrasonography is a useful technique for detecting effu- (Fig. 53–40). The bone scan can also be used to monitor
sions in the hip in children with transient synovitis, septic the disease course and response to treatment, although
arthritis, or Legg-Calvé-Perthes disease. The absence of several weeks may be required before the scan returns to
joint fluid in this joint on sonographic examination normal, and the correlation between clinical and scinti-
excludes the diagnosis of septic arthritis, though it does graphic improvement is not uniformly good. Occasional
not eliminate the possibility of osteomyelitis. Further, difficulty in interpreting the bone scan in younger patients
sonography can be used to monitor aspiration of the arises from an inability to differentiate between normal
effusion. Ultrasonography can also be employed in the and abnormal activity in the metaphyseal region.
detection of joint fluid in adults with septic arthritis of A gallium scan can be performed in conjunction with
the hip and, in a similar fashion, to assess the presence a technetium scan in the same patient, and the resulting
and extent of infected fluid in superficially located joints, information may be more useful than that obtained by
synovial cysts, bursae, and tendon sheaths in both children either examination alone (Table 53–7; see Fig. 53–40).
and adults. Gallium scans may reveal abnormal accumulation in
patients with active osteomyelitis when technetium scans
reveal decreased activity (cold lesions) or perhaps normal
Radionuclide Examination activity (transition period between cold and hot lesions).
Although the use of scintigraphy in the evaluation of It should be remembered that gallium is a bone scanning
musculoskeletal infections is discussed in Chapter 7, a agent that accumulates in regions of increased bone
few comments are appropriate here. Technetium phos- remodeling, such as occur in osteomyelitis. Therefore, its
phate bone scans become abnormal within hours to days accumulation in osseous sites that are also positive on
after the onset of bone infection and days to weeks before technetium phosphate scans is not unexpected, and such
the disease becomes manifest on conventional radiographs. accumulation by itself does not increase the specificity of
The scintigraphic abnormality initially may be evident as the radionuclide examination. Rather, when both tech-
TABLE 53–7
Radionuclide Evaluation of Osseous and Soft Tissue Infection
A B C
Figure 53–40. Soft tissue infection: imaging findings. This 37-year-old man, with a history of chronic
inactive osteomyelitis of the proximal portion of the radius, developed a staphylococcal soft tissue infection.
A, Radiograph shows soft tissue swelling and osseous deformity of the proximal portion of the radius and
ulna. B, Technetium phosphate bone scan reveals accentuated uptake of the radiopharmaceutical agent
(arrows) in the humerus, radius, and ulna about the elbow. C, Gallium scan indicates abnormality of soft
tissue alone (arrow). (Courtesy of V. Vint, MD, San Diego, Calif.)
netium phosphate and gallium scanning are used, it is phase to the scintigraphic examination, representing a
important to compare the degree and extent of radio- static image obtained 24 hours after injection of the bone-
nuclide uptake on the two examinations. Disparate distri- seeking radiopharmaceutical agent, may help in this
bution of uptake or increased intensity of uptake on the differentiation. Advantages of the 24-hour image relate
gallium study is an important sign of osteomyelitis. Such to continued accumulation of the technetium phosphate
infection is unlikely when both technetium phosphate and
gallium scanning are negative or when the distribution of
both tracers is spatially congruent and the relative inten-
sity of gallium uptake is less than that of the bone tracer.
Although a negative delayed bone image appears to be
specific in excluding infection, a positive finding during
the delayed static phase of the examination lacks speci-
ficity for infection and has stimulated considerable interest
in “three-phase” examinations in patients with musculo- A C
skeletal infection. This encompasses serial images obtained
during the first minute after a bolus injection of a tech-
netium compound (angiographic phase), a postinjection
image obtained at the end of the first minute or after
several minutes (blood pool phase), and additional images
obtained 2 or 3 hours later (delayed phase). If increased
accumulation of radionuclide within bone is observed in
all three phases, the diagnosis of osteomyelitis is highly
likely. Conversely, if such an increase is present only
on the delayed image, an alternative diagnosis should be
considered. Soft tissue infections are characterized by B D
delayed images that either are normal or reveal mini-
mally increased tracer accumulation within the bone, Figure 53–41. Septic arthritis: bone scanning. A to C, Three-
phase technetium phosphate study documents increased flow
presumably because of regional hyperemia. Septic arthri- (arrow) in the angiographic phase (A), diffuse hyperemia about
tis is usually accompanied by increased uptake of the the hip (arrow) in the blood pool stage (B), and increased uptake
radiopharmaceutical agent in juxta-articular bone in the of the radiopharmaceutical agent (arrow) in the delayed image
delayed images, moderate and diffuse blood pool hyperemia, (C). These findings indicate septic arthritis. D, Gallium scan is
and, on the radionuclide angiogram, increased flow to also abnormal, with increased scintigraphic activity about the
the joint space (Fig. 53–41). The addition of a fourth hip (arrow). (Courtesy of G. Greenway, MD, Dallas, Tex.)
Chapter 53 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Mechanisms and Situations 739
radionuclide in the abnormal woven bone about foci of
infection.
The accumulation of leukocytes at sites of abscess for-
mation has led to the use of indium-labeled autologous
leukocytes for the evaluation of inflammatory processes.
In general, 111In-labeled leukocyte scintigraphy is less
sensitive in detecting bone infections than soft tissue
infections and leads to difficulty in differentiating osteo-
myelitis and septic arthritis. It can demonstrate soft tissue
extension from an area of bone infection. Positive leuko-
cyte images are encountered in musculoskeletal condi-
tions other than infection. Rheumatoid arthritis and other
synovial inflammatory disorders can lead to findings simu-
lating those of septic arthritis, and primary or secondary
tumors in the soft tissue or bone can produce positive
leukocyte images similar to those accompanying infec-
tion. Compared with bone imaging with 99mTc com-
pounds, 111In scintigraphy has increased sensitivity in the A
detection of early osteomyelitis.
A B C
Figure 53–45. Diabetic foot infection: MR imaging. This 63-year-old man required amputation of the
third toe at the level of the metatarsophalangeal joint for control of infection. He later developed clinical
manifestations of recurrent infection. A, Transverse T1-weighted (TR/TE, 700/15) spin echo MR image
shows abnormally low signal intensity in the third and fourth metatarsal bones (arrows) and in the adjacent
soft tissues. The head of the second metatarsal bone also appears to be involved. B, Transverse STIR
(TR/TE, 1800/25; inversion time, 160 msec) image reveals high signal intensity in these metatarsal bones
(arrows) and soft tissues. C, Transverse T1-weighted (TR/TE, 900/13) fat-suppressed image obtained in
conjunction with intravenous administration of gadolinium contrast agent provides information similar to
that in (B). A third and fourth ray resection confirmed the presence of osteomyelitis.
740
Chapter 53 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Mechanisms and Situations 741
4. In soft tissue infections, differentiating infective perios-
titis, osteitis, or osteomyelitis from bone marrow edema.
The assessment of infection in the feet of diabetic
patients provides unique challenges (Fig. 53–45). Although
reported data indicate the value of scintigraphic methods,
particularly 111In-labeled leukocyte imaging with or with-
out bone scintigraphy, the day-to-day clinical experience
of many physicians suggests otherwise. A normal bone
scan virtually excludes the presence of osteomyelitis,
neuropathic osteoarthropathy, or both. The hyperemia
associated with either process can lead to positive results
with three-phase bone scintigraphy. Decreased blood flow
and possible impaired leukocyte responsiveness limit the
sensitivity achievable with 111In-labeled leukocyte scin-
tigraphy in diabetic foot infections; however, reports
indicate that the finding of definite increased uptake on
leukocyte scans has a high positive predictive value, and
the absence of increased leukocyte uptake in or near bone
makes the diagnosis of osteomyelitis very unlikely.
Although high signal intensity in the bone marrow on
T2-weighted spin echo and STIR images and on T1-
weighted spin echo MR images (with or without fat
saturation) after the intravenous administration of gado-
linium contrast agent is compatible with the diagnosis of
Figure 53–46. Diabetic neuropathic osteoarthropathy and
osteomyelitis, it is not a specific finding (see Fig. 53–43). osteomyelitis: MR imaging. Transverse STIR (TR/TE, 1800/20;
Neuropathic osteoarthropathy in the absence of coexis- inversion time, 125 msec) MR image shows high signal inten-
tent infection is accompanied by persistent low signal sity in the marrow of the second and third metatarsal bones, in
intensity in the bone marrow on T2-weighted spin echo the intermediate and lateral cuneiform bones, and in the soft
MR images, although this is not a constant finding. In tissues. Note the neuropathic changes about the first tarso-
rapidly developing neuropathic osteoarthropathy, acute metatarsal joint (arrow) and a Lisfranc pattern of subluxation
fragmentation of bone is associated with marrow edema, (arrowhead). In such cases, it is difficult to differentiate sites of
resulting in high signal intensity on T2-weighted spin osteomyelitis from neuropathic disease with marrow edema.
echo and STIR images and those images obtained after
the intravenous administration of a gadolinium-containing
contrast agent (Fig. 53–46). With application of the last tions are related to osteomyelitis rather than to neuro-
of these methods, the presence of soft tissue or osseous pathic osteoarthropathy. Some guidelines for this choice
abscesses suggests that the accompanying marrow altera- in cases of osteomyelitis, septic arthritis, and soft tissue
infections are provided in Table 53–8.
TABLE 53–8
Some Useful MR Imaging Protocols in Assessment of FURTHER READING
Musculoskeletal Infections
Azouz EM: Computed tomography in bone and joint infec-
tions. J Can Assoc Radiol 32:102, 1981.
Condition Suggested Protocols Bonakdar-pour A, Gaines VD: The radiology of osteomyelitis.
Osteomyelitis in red T2-weighted spin echo Orthop Clin North Am 14:21, 1983.
marrow T1-weighted spin echo with Boutin RD, Resnick D: The SAPHO syndrome: An evolving
gadolinium contrast enhancement concept for unifying several idiopathic disorders of bone and
STIR skin. AJR Am J Roentgenol 170:585, 1998.
Osteomyelitis in yellow T1-weighted spin echo Bressler EL, Conway JJ, Weiss SC: Neonatal osteomyelitis
marrow T1-weighted spin echo with examined by bone scintigraphy. Radiology 152:685, 1984.
gadolinium contrast enhancement Butt WP: The radiology of infection. Clin Orthop 96:20, 1973.
and fat suppression Butt WP: Radiology of the infected joint. Clin Orthop 96:136,
STIR 1973.
Septic arthritis T1-weighted spin echo with Capitanio MA, Kirkpatrick JA: Early roentgen observations in
gadolinium contrast enhancement acute osteomyelitis. AJR Am J Roentgenol 108:488, 1970.
with or without fat suppression Curtiss PH Jr: The pathophysiology of joint infections. Clin
Orthop 96:129, 1973.
Soft tissue infection T1-weighted spin echo with Davis LA: Antibiotic modified osteomyelitis. AJR Am J
gadolinium contrast enhancement Roentgenol 103:608, 1968.
with or without fat suppression Erdman WA, Tamburro F, Jayson HT, et al: Osteomyelitis:
Characteristics and pitfalls of diagnosis with MR imaging.
STIR, short tau inversion recovery. Radiology 180:533, 1991.
742 Section XIV Infectious Diseases
Fitzgerald RH, Brewer NS, Dahlin DC: Squamous-cell niques versus gadolinium-enhanced T1-weighted images.
carcinoma complicating chronic osteomyelitis. J Bone Joint Skeletal Radiol 26:654, 1997.
Surg Am 58:1146, 1976. Miller WB, Murphy WA, Gilula LA: Brodie abscess: Reap-
Fletcher BD, Scoles PV, Nelson AD: Osteomyelitis in children: praisal. Radiology 132:15, 1979.
Detection by magnetic resonance. Work in progress. Radiol- Mok PM, Reilly BJ, Ash JM: Osteomyelitis in the neonate:
ogy 150:57, 1984. Clinical aspects and the role of radiography and scintigraphy
Gilday DL, Paul DJ, Paterson J: Diagnosis of osteomyelitis in in diagnosis and management. Radiology 145:677, 1982.
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Goldberg JS, London WL, Nagel DM: Tropical pyomyositis: A Pennington WT, Mott MP, Thometz JG, et al: Photopenic
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nonseptic inflamed joint: MRI characteristics. Skeletal Rasool MN: Primary subacute haematogenous osteomyelitis in
Radiol 28:616, 1999. children. J Bone Joint Surg Br 83:93, 2001.
Green NE, Beauchamp RD, Griffin PP: Primary subacute epi- Resnick D: Osteomyelitis and septic arthritis complicating hand
physeal osteomyelitis. J Bone Joint Surg Am 63:107, 1981. injuries and infections: Pathogenesis of roentgenographic
Grey AC, Davies AM, Mangham DC, et al: The “penumbra abnormalities. J Can Assoc Radiol 27:21, 1976.
sign” on T1-weighted MR imaging in subacute osteomyelitis: Resnick D, Pineda CJ, Weisman MH, et al: Osteomyelitis and
Frequency, cause and significance. Clin Radiol 53:587, 1998. septic arthritis of the hand following human bites. Skeletal
Handmaker H, Leonards R: The bone scan in inflammatory Radiol 14:263, 1985.
osseous disease. Semin Nucl Med 6:95, 1976. Rosenbaum DM, Blumhagen JD: Acute epiphyseal osteo-
Hofer P: Gallium and infection. J Nucl Med 21:484, 1980. myelitis in children. Radiology 156:89, 1985.
Kahn M-F, Chamot A-M: SAPHO syndrome. Rheum Dis Clin Solheim LF, Paus B, Liverud K, et al: Chronic recurrent multi-
North Am 18:225, 1992. focal osteomyelitis. Acta Orthop Scand 51:37, 1980.
Kaye JJ: Bacterial infections of the hips in infancy and child- Tehranzadeh J, Wang F, Mesqarzadeh M: Magnetic resonance
hood. Curr Probl Radiol 3:17, 1973. imaging of osteomyelitis. CRC Crit Rev Diagn Imaging
Kemp HBS, Lloyd-Roberts GC: Avascular necrosis of the capital 33:495, 1992.
epiphysis following osteomyelitis of the proximal femoral Trueta J: Studies of the Development and Decay of the Human
metaphysis. J Bone Joint Surg Br 56:688, 1974. Frame. Philadelphia, WB Saunders, 1968, p 254.
Kido D, Bryan D, Halpern M: Hematogenous osteomyelitis in Unger E, Moldofsky P, Gatenby R, et al: Diagnosis of osteo-
drug addicts. AJR Am J Roentgenol 118:356, 1973. myelitis by MR imaging. AJR Am J Roentgenol 150:605,
McAfee JG, Subramanian G, Gagne G: Technique of leukocyte 1988.
harvesting and labeling: Problems and perspective. Semin Waldvogel FA, Vasey H: Osteomyelitis: The past decade. N
Nucl Med 14:83, 1984. Engl J Med 303:360, 1980.
Mendelson EB, Fisher MR, Deschler TW, et al: Osteomyelitis Wolfson JJ, Kane WJ, Laxdal SD, et al: Bone findings in chronic
in the diabetic foot: A difficult diagnostic challenge. Radio- granulomatous disease of childhood: A genetic abnormality
graphics 3:248, 1983. of leukocyte function. J Bone Joint Surg Am 51:1573, 1969.
Miller TT, Randolph DA Jr, Staron RB, et al: Fat-suppressed Wood BP: The vanishing epiphyseal ossification center: A sequel
MRI of musculoskeletal infection: Fast T2-weighted tech- to septic arthritis of childhood. Radiology 134:387, 1980.
54
CHAPTER 54
Osteomyelitis, Septic Arthritis, recognized pelvic precursors of vertebral osteomyelitis.
The common localization of early foci of osteomyelitis in
and Soft Tissue Infection: the subchondral region of the vertebral body, an area
Axial Skeleton richly supplied by nutrient arterioles, emphasizes that
arterial rather than venous pathways may be more impor-
tant in hematogenous osteomyelitis of the spine.
The role of hematogenous spread of infection directly
SUMMARY OF KEY FEATURES into the intervertebral disc has stimulated great interest.
The routes of contamination of the spine, the sacroiliac It has been suggested that in children and adolescents
joint, and other axial skeletal sites are identical to those younger than 19 or 20 years, vascular channels perforate
of the appendicular skeleton. In the spine, early loss the vertebral endplate and allow organisms in the blood-
of intervertebral disc space is characteristic of pyogenic stream to have direct access to the intervertebral disc.
infection and is associated with lysis and sclerosis of This concept has led to the application of such popular
neighboring bone. These findings can simulate those terms as “discitis.” A similar occurrence in adults has
of other disorders, such as rheumatoid arthritis, been attributed to a persistent disc blood supply or to a
intervertebral (osteo)chondrosis, and conditions supply that has been reinstated by vascular invasion of
complicated by cartilaginous node formation. Sacroiliac degenerating disc tissue.
joint infection is typically unilateral in distribution,
a feature that allows its differentiation from many Spread from a Contiguous Source of Infection. Vertebral or
other articular processes. Additional locations in the intervertebral disc infection can result from contamina-
axial skeleton are not uncommonly infected in tion by an adjacent soft tissue suppurative focus. Even in
intravenous drug abusers and in patients after trauma, cases in which bone or cartilage involvement follows soft
surgery, or diagnostic and therapeutic procedures. tissue infection, it is extremely difficult to eliminate the
possibility that osteomyelitis or disc infection is a result
of hematogenous or lymphatic seeding. Tuberculous and
fungal infection, however, can extend from the spine to
the neighboring tissue, dissect along the subligamentous
INTRODUCTION areas for a considerable distance, and then reenter the
The distribution of osteomyelitis and septic arthritis is vertebral body or intervertebral disc. Further, cases of
dramatically influenced by the age of the patient, the osteomyelitis and infective discitis have been reported as
specific causative organism, and the presence or absence a complication of colonic, hypopharyngeal, and esophageal
of any underlying disorder or situation. In children and perforation or instrumentation. In all these examples,
infants, frequent involvement of the bones and joints of however, the role of bacteremia with subsequent hema-
the appendicular skeleton is evident, whereas in adults, togenous seeding of the spine must be considered.
localization of infection to the osseous and articular A specific entity that may be related to this mechanism
structures of the vertebral column is common. Spinal of infection is Grisel’s syndrome, in which spontaneous
infection is also frequent in intravenous drug abusers and atlantoaxial subluxation accompanies inflammation of
in patients with tuberculosis. neighboring soft tissues, mainly in children (Fig. 54–2).
Proposed causes of this phenomenon include muscle
SPINAL INFECTIONS spasm, ligamentous laxity, and synovial effusion; the direct
continuity of the periodontoidal venous plexus and the
Routes of Contamination suboccipital epidural sinuses with the pharyngovertebral
Hematogenous Spread of Infection. Hematogenous spread veins suggests the existence of a hematogenous route for
through arterial and venous routes (Batson’s paravertebral the transport of peripharyngeal septic exudates to the
venous system) can result in lodgment of organisms in upper cervical spine structures.
the bone marrow of the vertebrae. This vascular arrange-
ment allows two direct routes for the hematogenous Direct Implantation. Organisms can be directly implanted
spread of infection: via the nutrient arteries and via the into the intervertebral disc (and, far less commonly, the
paravertebral venous system. Although the contribution vertebra) during attempted puncture of the spinal canal,
of each system to cases of spinal osteomyelitis is a matter intervertebral disc, paravertebral and peridural tissues, or
of debate, it is attractive to implicate the valveless venous aorta, or in penetrating injuries. Usually, the interver-
plexus—whose direction and extent of flow are dramati- tebral disc is the initial site of infection, especially in cases
cally influenced by changes in abdominal pressure—in of misguided puncture, and the vertebra becomes con-
the frequent spread of infection (and neoplasm) to the taminated as a secondary event.
spine from pelvic sources (Fig. 54–1). Urinary tract infec-
tions or surgery, rectosigmoid disease and enteric fistulas, Postoperative Infection. The more frequent and aggres-
and septic abortion or postpartum infection are well- sive spinal operations that are currently being undertaken
743
744 Section XIV Infectious Diseases
Clinical Abnormalities
The reported frequency of osteomyelitis and disc space
infection (together termed infective spondylitis) has risen
dramatically. Initially, infective spondylitis was thought
to represent less than 1% of all cases of osteomyelitis;
now it appears that 2% to 4% is a more accurate esti-
mate. Men are affected more commonly than women.
The highest frequency of septic spondylitis occurs in the
fifth and sixth decades of life. The lumbar spine is the
most typical site of involvement, followed by the thoracic
spine; sacral and cervical abnormalities occur with about
equal frequency. The usual location of infection in the
vertebra is the vertebral body.
A history of recent primary infection, instrumentation,
or a diagnostic, therapeutic, or surgical procedure is com-
mon. The most frequently encountered (55% to 90%)
pyogenic organism is Staphylococcus aureus or, less com-
monly, Staphylococcus epidermidis, although other gram-
positive and, less typically, gram-negative agents may be
implicated. Clinical manifestations vary with the virulence
Figure 54–1. Anatomic considerations: Batson’s paraverte- of the organism and the nature of the host’s resistance.
bral venous system. This valveless, plexiform set of veins lies General findings include fever, malaise, anorexia, and
outside the thoracoabdominal cavity and anastomoses with the weight loss. Back pain is a common initial local manifes-
cavitary veins at each segmental level. Thus, communication tation and may be intermittent or constant, exacerbated
exists between the pelvic and vertebral venous system, femoral by motion, and throbbing at rest. With accompanying
and iliac veins, inferior and superior venae cavae, and other soft tissue abscess formation, hip contracture can occur
important venous structures. 1, paravertebral venous plexus; 2, (psoas muscle irritation). Appropriate culture of the
inferior vena cava; 3, inferior mesenteric vein; 4, internal iliac
vein; 5, pelvic plexus. (Modified from Vider M, Maruyama Y,
blood can identify the causative organism in some cases,
Narvaez R: Significance of the vertebral venous (Batson’s) plexus although more drastic methods, such as needle biopsy or
in metastatic spread in colorectal carcinoma. Cancer 40:67, aspiration, may be necessary.
1977.)
A B C D
Figure 54–3. Spinal infection: sequential stages. A, An anterior subchondral focus in the vertebral body
is typical. B, Infection may then perforate the vertebral surface and reach the intervertebral disc space. C,
With further spread of infection, contamination of the adjacent vertebral body and narrowing of the
intervertebral disc space are recognizable. D, With continued dissemination, infection may spread in a
subligamentous fashion and erode the anterior surface of the vertebral body (arrowhead) or perforate the
anterior ligamentous structures (arrow).
A B
Figure 54–4. Spinal infection: early and later radiographic abnormalities. A, Observe the loss of definition
of the superior aspect of a lumbar vertebral body (arrowheads), with narrowing of the adjacent intervertebral
disc space. This appearance (in a middle-aged man with pyogenic infection) conforms to the stage in Figure
54–3B. B, In this young child, a staphylococcal infection has led to destruction of two adjacent vertebral
bodies (arrowheads) and narrowing of the intervening intervertebral disc. A soft tissue mass is apparent. This
appearance corresponds to the stage in Figure 54–3D.
746 Section XIV Infectious Diseases
variable in severity and has been used in the past as a (upper respiratory tract, urinary tract, or ear infection) is
helpful sign in differentiating pyogenic from tuberculous usually apparent. Manifestations are generally mild and
infection. Although such sclerosis is indeed common in include back pain, abdominal pain, hip irritability, and
pyogenic (nontuberculous) spondylitis, it may also be evi- altered gait. Low-grade fever, irritability, malaise, eleva-
dent in tuberculosis. More helpful in this differentiation tion of the erythrocyte sedimentation rate, and, on occa-
is a combination of findings that strongly indicates tuber- sion, leukocytosis are noted in many cases. When positive,
culous spondylitis, including the presence of a slowly blood or bone biopsy culture most typically reveals S.
progressive vertebral process with preservation of inter- aureus. Negative culture results are reported in 50% to
vertebral discs, subligamentous spread of infection with 90% of cases.
erosion of the anterior vertebral margins, large and calci- Scintigraphy may reveal increased accumulation of
fied soft tissue abscesses, and the absence of severe bony bone-seeking pharmaceuticals at a relatively early stage.
eburnation (Fig. 54–5). Intervertebral disc space narrowing is later accompanied
In the lumbar spine, such extension can lead to oblit- by erosion of the subchondral bone plate and osseous
eration or displacement of the psoas margin; in the eburnation (Fig. 54–7). Magnetic resonance (MR) imag-
thoracic spine, a paraspinal mass can be encountered; and ing in cases of childhood discitis reveals findings similar
in the cervical spine, retropharyngeal swelling can lead to to those in adults with infective spondylitis.
displacement and obliteration of adjacent prevertebral fat
planes. With early and proper treatment, reconstitution
can result, with production of a radiodense (ivory) ver- Other Diagnostic Techniques
tebra, a relatively intact or ankylosed intervertebral disc, The role of radionuclide studies in establishing the pres-
and surrounding osteophytosis (Fig. 54–6). ence of spinal infection at a stage when radiographs are
entirely normal is well documented. Technetium-,
gallium-, and indium-labeled radiopharmaceutical agents
Special Types of Spinal Infection
can be used in this regard. The application of pinhole
Most infections of the intervertebral disc occur as an scintigraphy and single photon emission computed
extension of vertebral osteomyelitis or direct inoculation tomography (SPECT) has further increased the
during diagnostic or surgical procedures. In children, sensitivity of bone scanning in the diagnosis of infective
however, a hematogenous route to the disc still exists, spondylitis.
and hematogenous contamination of disc tissue is pos- Computed tomography (CT) scanning allows defini-
sible. Clinical symptoms and signs may become evident tion of the extent of osseous and disc destruction and
between 1 and 16 years of age (average age, approxi- paravertebral and intraspinal involvement (Fig. 54–8).
mately 5 years), and a preexisting infectious condition The intravenous injection of contrast material aids in the
A B
Chapter 54 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Axial Skeleton 747
A B
Figure 54–6. Spinal infection: residual deformity. Klebsiella spondylitis developed in the cervical region in
this 41-year-old man. A, Three weeks after the onset of infectious spondylitis, note the collapse and
fragmentation of the superior aspect of the fifth cervical vertebral body and lysis of the interior aspect of the
fourth cervical vertebral body (arrowheads). Soft tissue swelling is evident. B, Two weeks later, angulation and
subluxation are apparent. Soft tissue swelling is again seen.
sity on T1-weighted spin echo MR images and increased epidural and paraspinal extension, posterior disc protru-
signal intensity on T2-weighted spin echo images (Fig. sion, vertebral collapse, and spinal deformity.
54–9). The conspicuity of the bone marrow infection on Infected marrow usually enhances diffusely after the
these MR images depends on the extent of red and yellow administration of gadolinium. In some instances, how-
marrow in the vertebral body and is more variable on ever, this technique may produce a decrease rather than
T2-weighted spin echo MR images. Imaging with long an increase in the contrast between normal and infected
repetition and echo times (as well as fat suppression) is vertebral bodies (Fig. 54–10). The combination of con-
beneficial in demonstrating high signal intensity within trast enhancement and fat suppression in cases of pyo-
the marrow. genic spondylitis may eliminate this problem. Vertebral
With MR imaging, irregularity of the vertebral end- marrow involvement in cases of infection (or tumor) may
plates and narrowing of the intervertebral disc may be also be effectively demonstrated by using short tau inver-
evident. On T2-weighted spin echo MR sequences, the sion recovery (STIR) sequences.
infected disc reveals increased signal intensity with absence
of the normal nuclear cleft (i.e., the normal anatomic
Differential Diagnosis
structure that, on T2-weighted images, appears as an area
of signal void in the center of lumbar discs in subjects The radiographic hallmark of infective spondylitis is inter-
30 years or older). The high signal intensity of the inter- vertebral disc space narrowing, frequently accompanied
vertebral disc in cases of pyogenic spondylitis is an impor- by lysis or sclerosis of adjacent vertebrae (Table 54–1).
tant diagnostic sign. Additional MR imaging findings of A similar radiographic pattern can be encountered in
pyogenic spondylitis are less frequent but include various articular disorders, such as rheumatoid arthritis,
A B
Discovertebral Vacuum
Disorder Margin Sclerosis Phenomena Osteophytosis Other Findings
†
Infection Poorly defined Variable* Rare Absent Vertebral lysis, soft tissue mass
Intervertebral Well defined Prominent Present Variable Cartilaginous nodes
osteochondrosis
Rheumatoid arthritis Poorly or well defined Variable Absent Absent or mild Apophyseal joint
with “erosions” abnormalities, subluxation
Calcium pyrophosphate Poorly or well Prominent Variable Variable Fragmentation, subluxation
dihydrate crystal defined
deposition disease
Neuropathic Well defined Prominent Variable Prominent Fragmentation, subluxation,
osteoarthropathy disorganization
Trauma Well defined Prominent Variable Variable Fracture, soft tissue mass
Sarcoidosis Poorly or well Variable, Absent Absent Soft tissue mass
defined may be
prominent
the seronegative spondyloarthropathies, calcium pyrophos- infection. Rarely, infections by gas-forming bacteria may
phate dihydrate crystal deposition disease, alkaptonuria, lead to a vacuum phenomenon–like appearance.
and neuropathic osteoarthropathy; in each of these dis- In general, primary or metastatic tumor in the spine
orders, however, clinical and additional radiographic fea- does not lead to significant loss of intervertebral disc
tures usually ensure an accurate differential diagnosis. space; the combination of widespread lysis or sclerosis of
Sarcoidosis can occasionally be associated with disc a vertebral body and an intact adjacent intervertebral disc
space narrowing and bone eburnation at one or more is much more characteristic of tumor than of infection.
levels of the spine. Diminution of intervertebral disc Certain neoplasms such as plasma cell myeloma, chordoma,
height and bony sclerosis are associated with cartilagi- and even skeletal metastasis can extend across or around
nous node formation (Schmorl’s nodes). In general, the the intervertebral disc to involve the neighboring vertebra,
poor definition of the subchondral bone plate is less in however. Paraspinal masses occur in infective spondylitis
cases of cartilaginous nodes than in cases of infection. and traumatic and neoplastic disorders. Infection is likely
MR imaging can also be applied to the differentiation of if such masses contain gas.
cartilage node formation and infection, although diag- Accurate radiographic differentiation of pyogenic infec-
nostic difficulties are encountered. The widespread carti- tive spondylitis from granulomatous infections (tuber-
laginous nodes detected in Scheuermann’s disease (juvenile culosis and fungal disorders) can be difficult. Rapid loss
kyphosis) create an appearance that is not generally con- of intervertebral disc height, extensive sclerosis, and the
fused with that of infection. absence of calcified paraspinal masses are findings that
Intervertebral (osteo)chondrosis also produces inter- are more typical of pyogenic infection.
vertebral disc space narrowing and reactive sclerosis of
the neighboring bone (see Chapter 30). The resulting SACROILIAC JOINT INFECTIONS
radiographic picture can resemble that of infective
Routes of Contamination
spondylitis. In intervertebral (osteo)chondrosis, the ver-
tebral endplates are usually smooth and well defined, The sacroiliac joint may become infected by the hema-
although focal defects can represent sites of interver- togenous route, by contamination from a contiguous
tebral disc displacement (cartilaginous nodes). Of partic- suppurative focus, by direct implantation, or after surgery.
ular diagnostic significance is the presence of one or Hematogenous involvement of this joint likely begins in
more radiolucent collections overlying the intervertebral the subchondral bone of the ilium.
disc in intervertebral (osteo)chondrosis. These vacuum Contamination of the sacroiliac joint or neighboring
phenomena represent gaseous collections (nitrogen) bone can occur from an adjacent infection. Pelvic abscesses
within the nucleus pulposus and are a reliable sign of disc can disrupt the anterior articular capsule or the perios-
degeneration. They are exceedingly rare in cases of disc teum and cortex of the ilium or sacrum. Thus, vaginal,
infection, and their detection makes the diagnosis of uterine, ovarian, bladder, and intestinal processes can lead
infection very unlikely. Similarly, the presence of gas in to iliac or sacral osteomyelitis and sacroiliac joint suppu-
the vertebral body virtually eliminates the diagnosis of ration by contiguous contamination (as well as by
750 Section XIV Infectious Diseases
Clinical Abnormalities
Pyogenic infection of the sacroiliac joint may develop in
patients of all ages. Unilateral alterations predominate.
Fever, local pain and tenderness, and a limp may be
evident. Accurate diagnosis is often delayed in cases of
septic sacroiliitis, which increases the frequency of such
extra-articular contamination. Elevation of the erythro-
cyte sedimentation rate and leukocytosis are common but
variable laboratory findings. Identification of the causa-
tive organisms from blood culture or joint aspiration can
be difficult. Gram-negative bacterial agents are especially
common in pyogenic arthritis of the sacroiliac joint in
intravenous drug abusers. Figure 54–11. Sacroiliac joint infection: early abnormalities.
Pseudomonas osteomyelitis and septic arthritis developed in a
35-year-old male heroin addict. Radiograph reveals changes in
Radiographic-Pathologic Correlation
the right sacroiliac joint consisting of subchondral osseous
In almost all cases of sacroiliac joint infection, a unilateral erosion, poorly defined articular margins, and widening of the
distribution is encountered. In pyogenic arthritis, joint space (arrows).
radiographic findings generally occur in 2 or 3 weeks and
are characterized by blurring and indistinctness of the Differential Diagnosis
subchondral osseous line and narrowing or widening of The unilateral nature of infective sacroiliac joint disease
the interosseous space. Although these two alterations
frequently coexist, their time of appearance is dictated by
the initial site of contamination: if osteomyelitis precedes
septic arthritis, bony abnormalities may antedate the
articular changes; if the joint is affected initially, carti-
laginous and osseous alterations may coexist. In both situa-
tions, the most extensive findings are commonly evident
about the inferoanterior aspect of the joint (Fig. 54–11).
Surrounding condensation of bone is variable in fre-
quency and degree, and it is influenced by the type and
virulence of the infecting microorganism.
B C
Figure 54–13. Sacroiliac joint infection: MR imaging.
Staphylococcal infection of the left sacroiliac joint developed in Figure 54–14. Sternal infection. Group B streptococcal sep-
this 48-year-old woman. A, The infectious process in the ticemia resulting in infections in the hip, spine, and sternum
sacrum and ilium is not well visualized on this transaxial T1- developed in this 47-year-old man. Transaxial CT scan shows a
weighted (TR/TE, 650/11) spin echo MR image because of the destroyed sternum and an anterior soft tissue mass, both con-
similar signal intensity of the inflammatory response and the taining gas (arrows), and mediastinal adenopathy. Mediastinitis
hematopoietic bone marrow. The soft tissue extension of infec- was confirmed at surgery.
tion is not evident either. B, Transaxial fat-suppressed T2-
weighted (TR/TE, 6000/102) fast spin echo MR image reveals
high signal intensity in the sacrum and ilium, as well as in the FURTHER READING
anterior and posterior soft tissues and musculature (arrows). C,
Transaxial T1-weighted (TR/TE, 500/11) spin echo MR image Allen EH, Cosgrove D, Millard FJC: The radiological changes
obtained with fat saturation technique after the intravenous in infections of the spine and their diagnostic value. Clin
injection of a gadolinium contrast agent reveals the inflamma- Radiol 29:31, 1978.
tory reaction, with high signal intensity in the bone and about Batson OV: The vertebral vein system. AJR Am J Roentgenol
the anterior and posterior abscesses. Note the low signal inten- 78:195, 1957.
sity of the fluid in the joint and in the soft tissues and muscu- Brant-Zawadzki M, Burke VD, Jeffrey RB: CT in the evalua-
lature. (Courtesy of M. Schweitzer, MD, Philadelphia, Pa.) tion of spine infection. Spine 8:358, 1983.
Dagirmanjian A, Schils J, McHenry M, et al: MR imaging of
vertebral osteomyelitis revisited. AJR Am J Roentgenol
167:1539, 1996.
is its most useful diagnostic feature. Bilateral symmetrical Fernandez-Ulloa M, Vasavada PJ, Hanslits ML, et al: Diagnosis
or asymmetrical articular changes are characteristic of of vertebral osteomyelitis: Clinical radiological and scinti-
ankylosing spondylitis, psoriasis, Reiter’s syndrome, osteitis graphic features. Orthopedics 8:1141, 1985.
condensans ilii, and hyperparathyroidism. Unilateral Goldin RH, Chow A, Edwards JE Jr, et al: Sternoarticular
changes can be encountered in rheumatoid arthritis, septic arthritis in heroin users. N Engl J Med 289:616, 1973.
gout, Reiter’s syndrome, psoriasis, and paralysis (because Jamison RC, Heimlich EM, Miethke JC, et al: Non-specific
of chondral atrophy). spondylitis of infants and children. Radiology 77:355, 1961.
Larde D, Mathieu D, Frija J, et al: Vertebral osteomyelitis: Disk
hypodensity on CT. AJR Am J Roentgenol 139:963, 1982.
INFECTION AT OTHER AXIAL SITES Lewkonia RM, Kinsella TD: Pyogenic sacroiliitis: Diagnosis
and significance. J Rheumatol 8:153, 1981.
In intravenous drug abusers, osteomyelitis and septic Modic MT, Weinstein MA, Pavlicek W, et al: Magnetic reso-
arthritis of the sternoclavicular and acromioclavicular nance imaging of the cervical spine: Technical and clinical
joints, in addition to the spine and sacroiliac joint, may be observations. AJR Am J Roentgenol 141:1129, 1983.
752 Section XIV Infectious Diseases
Murphy KJ, Brunberg JA, Quint DJ, et al: Spinal cord infec- Sandrasegaran K, Saifuddin A, Coral A, et al: Magnetic reso-
tion: Myelitis and abscess formation. AJNR Am J Neuro- nance imaging of septic sacroiliitis. Skeletal Radiol 23:289,
radiol 19:341, 1998. 1994.
Numaguchi Y, Rigamonti D, Rothman MI, et al: Spinal epidural Sartoris DJ, Moskowitz PS, Kaufman RA, et al: Childhood
abscess: Evaluation with gadolinium-enhanced MR imaging. diskitis: Computed tomographic findings. Radiology 149:701,
Radiographics 13:545, 1993. 1983.
Pinckney LE, Currarino G, Higgenboten CL: Osteomyelitis Sharif HS: Role of MR imaging in the management
of the cervical spine following dental extraction. Radiology of spinal infections. AJR Am J Roentgenol 158:1333,
135:335, 1980. 1992.
Rosenberg D, Baskies AM, Deckers PJ, et al: Pyogenic sacroili- Wiley AM, Trueta J: The vascular anatomy of the spine and its
itis: An absolute indication for computerized tomographic relationship to pyogenic vertebral osteomyelitis. J Bone
scanning. Clin Orthop 184:128, 1984. Joint Surg Br 41:796, 1959.
55
CHAPTER 55
Osteomyelitis, Septic Arthritis, Streptococcal Infection
and Soft Tissue Infection: Streptococci are gram-positive cocci that can be classified
on the basis of the type of hemolysis they produce on
Organisms sheep blood agar plates (e.g., beta-hemolytic strepto-
cocci). In infants, hemolytic streptococcal agents are
an important causative factor in neonatal or infantile
SUMMARY OF KEY FEATURES osteomyelitis. The clinical manifestations of strepto-
coccal bone infection may be mild, even in the presence
Osseous, articular, and soft tissue structures may of significant radiographic alterations. Infection of a
become involved in many infectious disorders. single bone is most frequent, and a predilection for the
Bacteria, mycobacteria, spirochetes, fungi, viruses, humerus has been noted (Fig. 55–2). Lytic lesions with
rickettsiae, protozoa, and worms are all capable of mild or absent sclerosis and periostitis may be seen. The
affecting the musculoskeletal system. In many instances, joints may be infected by streptococcal organisms either
imaging features, though typical of an infection, do not by extension from a neighboring site of osteomyelitis or
allow the diagnosis of a specific causative agent; in some cellulitis or directly.
cases, the distribution and morphology of the lesions
are sufficiently characteristic to suggest a single
infectious process. In all cases, the imaging studies Pneumococcal Infection
must be interpreted in conjunction with the clinical Formerly termed Diplococcus pneumoniae, the pneumo-
and pathologic manifestations. coccus is now referred to as Streptococcus pneumoniae.
Pulmonary infections and those of the upper respiratory
tract predominate. Pneumococcal arthritis is not fre-
quent. Pneumococcal osteomyelitis is rare and generally
confined to periarticular bone or the spine. Sickle cell
INTRODUCTION anemia may be an underlying problem.
Although the skeleton can react in only a limited number
of ways, certain characteristics of its response to a par- Gram-Negative Cocci
ticular infectious agent may differ, at least subtly, from
Meningococcal Infection
the changes encountered in the presence of another
agent. Thus, certain organisms produce rapid and destruc- Meningococcal infection caused by Neisseria meningitidis
tive osseous or articular disease, whereas others are occurs almost exclusively in persons who have no meas-
associated with a more indolent process. Further, some urable antimeningococcal antibody. It varies remarkably
agents show a predilection for certain anatomic regions in severity, from benign and asymptomatic to a fulminant
of the skeleton. and fatal disorder. Septicemia may lead to contamination
of many sites, but the microorganisms commonly lodge
in the central nervous system, skin, adrenal glands, and
BACTERIAL INFECTION serosal surfaces. Meningococcemia leads to the rapid
Gram-Positive Cocci development of fever, shaking chills, skin eruption,
petechiae, myalgias, and a variety of neurologic mani-
Staphylococcal Infection festations. In fulminant cases (Waterhouse-Friderichsen
Staphylococci are responsible for most cases of acute syndrome), hypotension, confusion, tachypnea, periph-
osteomyelitis and nongonococcal infectious arthritis. The eral cyanosis, and consumptive coagulopathy develop. In
two major pathogens are Staphylococcus aureus (coagulase children, characteristic skeletal abnormalities have been
positive) and Staphylococcus epidermidis (coagulase nega- described in which localized premature fusion of part of
tive). S. aureus is most typical in pyogenic osteomyelitis. several physes is seen, usually in a bilateral and relatively
Localization of the infection to the metaphysis of the symmetrical distribution (Fig. 55–3). Commonly, the
tubular bones in children is typical, and Brodie’s abscesses central aspect of the physis is affected, and a cupped or
may be seen (Fig. 55–1). Staphylococci are also respon- cone-shaped metaphysis results. Subsequently, epiphy-
sible for many of the deep infections that occur after bone seal disintegration and bowing and angular deformities
or joint surgery; the foot infections in diabetic patients; appear, especially in the legs, and lead to limb shortening.
cases of osteomyelitis and septic arthritis in hemodialysis
patients with infected shunts, intravenous drug addicts,
Gonococcal Infection
and patients with rheumatoid arthritis; and the osseous,
articular, and soft tissue suppurative processes that follow Gonorrhea is produced by the microorganism Neisseria
penetrating or open wounds. S. aureus is implicated in gonorrhoeae, which infects the mucous membranes of the
most cases of pyomyositis. urethra, cervix, rectum, and pharynx. The frequency of
753
754 Section XIV Infectious Diseases
B
Figure 55–3. Meningococcemia with skeletal deformities.
This 5 year-old-boy demonstrates the skeletal deformities that
can follow meningococcemia with intravascular coagulation. A,
Findings in the knee include metaphyseal sclerosis, epiphyseal
irregularity and deformity, subluxation, and, in one tibia, a
previous fracture. B, Observe the metaphyseal cupping and
irregularity in the phalanges, metacarpal bones, and radii, with
amputation of some of the digits. (Courtesy of M. Dalinka,
MD, Philadelphia, Pa.)
Figure 55–2. Streptococcal osteomyelitis and septic arthritis. last occurring in approximately 75% of disseminated
In this infant, radiography shows an osteolytic lesion of the
metaphysis of the humerus. The proximal epiphysis is not
cases. The articular disease may have an insidious onset
ossified. (Courtesy of J. Tomanek, MD, Johnson City, Tenn.) with fleeting arthralgias or a sudden onset with fever and
red, hot, swollen, and tender joints. Polyarticular findings
are frequent, but the infection tends to localize in one or
two joints. The affected articulations, in decreasing order
gonococcal arthritis is rising, possibly owing to an increasing of frequency, are the knee, ankle, wrist, and joints of the
resistance to antibiotics. The disease is transmitted shoulder, foot, and spine. In approximately 50% to 70%
almost exclusively through sexual contact with persons of cases, acute asymmetrical tenosynovitis or periarthritis
who have asymptomatic or ignored symptomatic infec- is evident, particularly in the dorsal aspect of the fingers,
tion. It is also encountered during pregnancy and after hand, or wrist or in the ankle.
gonococcal vulvovaginitis in children and neonates. Only If appropriate treatment is delayed, more prominent
a minority of gonococcal infections eventually disseminate. radiographic findings are encountered, including joint
Gonococcemia leads to skin rash, fever, and arthritis, the space narrowing, marginal and central osseous erosions,
Chapter 55 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Organisms 755
lytic destruction of adjacent metaphyses and epiphyses, Proteus Infection
and periostitis (Fig. 55–4). These features are identical to Proteus mirabilis infection of a joint is rarely observed.
those accompanying other pyogenic infections, although Monoarticular involvement of the knee or another joint
abnormalities at multiple joints and tenosynovitis are is typical. Osteomyelitis related to this microorganism is
helpful clues to a specific diagnosis of gonococcal infec- also rare.
tion. Differentiating the radiographic features of gono-
coccal pyarthrosis and Reiter’s syndrome can be extremely
difficult; clinical differentiation is complicated by the Pseudomonas Infection
presence of skin rash, urethral discharge, and articular Premature infants, children with congenital anomalies,
abnormalities in both conditions. intravenous drug abusers, patients with myeloprolifera-
tive disorders or those receiving immunosuppressive
Enteric Gram-Negative Bacilli agents, and geriatric patients with debilitating diseases
are susceptible to osteomyelitis or septic arthritis from
Although the terminology related to this group of bacteria Pseudomonas aeruginosa. Hematogenous spread of infec-
is not constant, two major families of microorganisms are tion is common in intravenous drug abusers; Pseudomonas
identified: Enterobacteriaceae and Pseudomonadaceae. infection commonly localizes in the axial skeleton and
In general, these gram-negative bacilli are responsible for affects the spine and the sacroiliac, sternoclavicular, and
as many as 25% of skeletal infections. acromioclavicular joints. Pseudomonas osteomyelitis is a
recognized complication of puncture wounds. No specific
Coliform Bacterial Infection radiographic features characterize skeletal involvement
with this organism.
The coliform bacteria are gram-negative bacilli that
normally inhabit the human intestinal tract. The best-
known organisms in this group are Escherichia coli and Klebsiella Infection
Enterobacter (Aerobacter) aerogenes. Articular and osseous Rarely, Klebsiella pneumoniae results in osteomyelitis and
infections with these agents are rare except in intra- septic arthritis in a host with diminished resistance.
venous drug abusers, those with preexisting joint disease, Emphysematous septic arthritis may also be seen.
and patients with chronic debilitating disorders. No
specific radiographic features are evident.
Salmonella Infection
Salmonella typhi produces a systemic infection, typhoid
fever. Bone infection in such cases is rare. Involvement
can occur in spinal locations, with a radiographic picture
resembling that of tuberculosis. An association exists
between Salmonella infection and sickle cell anemia and
other hemoglobinopathies, as well as leukemia, lymphoma,
bartonellosis, cirrhosis of the liver, and systemic lupus
erythematosus. It has been postulated that multiple bowel
infarcts allow the organisms to leave the colon and enter
the bloodstream and that Salmonella organisms are well
suited for survival in areas of medullary bone infarction.
In fact, Salmonella osteomyelitis frequently originates in
the medullary cavity of a tubular bone. Salmonella infec-
tion may be characterized by a symmetrical distribu-
tion, a combination of lysis and sclerosis, and periostitis,
findings that are difficult to differentiate from those of
infarction alone.
Shigella Infection
Two to 3 weeks after an episode of acute bacillary dysen-
tery, a noninfectious polyarthritis showing a predilection
for the knees, elbows, wrists, or fingers may be evident.
It simulates rheumatic fever.
Yersinia Infection
Figure 55–4. Gonococcal arthritis: knee. Observe the joint Two types of bone or joint affliction can occur in asso-
space loss, poorly defined marginal and central osseous ero- ciation with infection caused by Yersinia enterocolitica. A
sions, and soft tissue swelling. The lack of osteoporosis is nonsuppurative, self-limited polyarthritis, especially of
impressive. Bone proliferation is evident along the distal medial the knees and ankles, can appear approximately 3 weeks
portion of the femur. after the onset of illness. This articular manifestation
756 Section XIV Infectious Diseases
may be complicated by sacroiliitis and presence of the In fact, this microorganism appears to be the leading
HLA-B27 antigen. The second type of affliction relates cause of pyarthrosis in the first 2 years of life. Hematoge-
to the presence of Y. enterocolitica septicemia, particularly nous spread is the usual mechanism of joint infection.
in patients with underlying abnormalities. Septic arthritis Single or, less commonly, multiple joints may be affected,
or osteomyelitis may appear in this setting. with the knee and ankle being the most frequent sites of
involvement.
Serratia Infection
Serratia marcescens can cause infection of the musculo- Brucella Infection
skeletal system, especially in persons with underlying Brucellosis (undulant fever) is endemic in Saudi Arabia,
disorders such as diabetes mellitus, systemic lupus South America, Spain, Italy, and the midwestern United
erythematosus, neutrophil dysfunction syndromes, and States. It is transmitted to humans from animals such as
rheumatoid arthritis. It can also occur after trauma; goats, cows, and hogs through the ingestion of milk or
placement of intravenous, arterial, or urinary catheters; milk products containing viable bacteria or through skin
ischemic necrosis of bone; or intravenous drug abuse. contact with infected tissues or secretions. The disease
The usual pathway for articular and osseous infection with is rarely transmitted from one human to another. The
this organism is the bloodstream. Radiographic features invading organisms localize in tissues of the reticulo-
are entirely nonspecific. endothelial system, such as the liver, spleen, lymph nodes,
and bone marrow.
Other Gram-Negative Bacilli Involvement of joints, bones, and bursae is relatively
uncommon, although an inflammatory process in any
Haemophilus Infection
one of these sites in a farmer or meat handler should
Acute septic arthritis caused by Haemophilus influenzae is arouse suspicion of brucellosis. The arthritis is usually
more frequent in children, particularly those between the monoarticular or pauciarticular, with the hip and knee
ages of 7 months and 4 years, than in adults (Fig. 55–5). being involved most frequently. Alterations in bursae
may be especially characteristic, with inflammation com-
mon in the prepatellar region.
Osteomyelitis of long, short, or flat bones may be
encountered. Brucellar spondylitis, which appears to be
the most common form of musculoskeletal disease, typi-
cally affects the lumbar spine and is associated with an
acute clinical onset and rapid progression of radiographic
findings (Fig. 55–6). Abnormalities include destruction
of vertebrae and intervertebral discs, sclerosis, paraver-
tebral abscess formation, and healing with intraosseous
fusion and osteophytosis. A large, parrot beak–like osteo-
phyte has been reported as a characteristic feature of
spinal brucellosis, although this radiographic abnormality
resembles that in other types of pyogenic or tuberculous
spondylitis.
Aeromonas Infection
Aeromonas hydrophila is an aerobic gram-negative rod
found in fresh water, tap water, swimming pools, and soil,
as well as in the stools of some persons. Although rarely
a pathogen, it can cause infection in patients with neo-
plasm or chronic liver disease. A history of exposure to
water in a pool or lake, especially when combined with
trauma, is characteristic. Necrotizing lesions can lead to
gas formation. After initiation of septic arthritis resulting
from hematogenous spread of infection, patients respond
poorly to therapy.
Pasteurella Infection
Figure 55–5. Haemophilus osteomyelitis and septic arthritis. Typical pathogens in animals, Pasteurella organisms can
Osteomyelitis of the proximal metaphysis and diaphysis of the also produce human infection. Cutaneous and subcuta-
humerus, with glenohumeral joint involvement, developed in neous abscesses, cellulitis, and lymphangitis are local and
this infant secondary to Haemophilus infection. Observe the regional manifestations of Pasteurella infection and can
metaphyseal erosion (arrow), permeative bone destruction, and be followed by septicemia and involvement of distant
periostitis (arrowhead). sites. Localization of infection in the knee is common,
Chapter 55 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Organisms 757
Other Bacteria
Clostridial Infection
Wounds that are contaminated by gas gangrene may con-
tain a mixture of clostridial organisms. These organisms
are anaerobic and are capable of producing extensive tissue
destruction with gas formation at the site of invasion.
Soft tissue contamination with gas gangrene develops in
devitalized tissues in which the arterial blood supply has
been compromised. War wounds, vehicular trauma, sur-
gery, burns, and decubitus ulcers are some predisposing
factors. Clinical manifestations of clostridial myonecrosis
may become evident within 6 to 8 hours of injury and
include severe pain and an edematous, pulseless, and
gangrenous limb. Crepitation with detection of gas in the
soft tissues is apparent in later stages. The clinical mani-
festations of clostridial cellulitis are less striking.
On radiographs, radiolucent collections may appear
within subcutaneous or muscular tissue (Fig. 55–7). In
the former location, they produce linear or netlike lucent
areas. Gas in muscular tissue may produce circular collec-
tions of varying size. It should be emphasized, however,
A that soft tissue gas is not specific for clostridial infection,
because it is evident in some cases of infection with E.
coli, other coliform bacteria, streptococci, and Bacteroides
species. Clostridial organisms can be introduced into
a joint by contamination from a penetrating injury or,
rarely, by hematogenous spread from its normal site
of residence in the gastrointestinal tract. Monoarticular
disease, particularly of the knee, is typical. Synovial edema
and inflammation and cartilaginous destruction may be
evident, perhaps related to the effect of the highly toxic
enzymes produced by these bacteria. In addition to joint
space narrowing and osseous defects, radiographs may
delineate gas in the adjacent soft tissues or in the articu-
lation itself.
B
Figure 55–6. Brucellar spondylitis: lumbar spine. A, Involve-
ment in this case is characterized by irregular destruction of the
osseous surfaces of two adjacent vertebrae with reactive sclerosis.
Note the parrot beak–like osteophytes. B, In another patient,
findings include disc space loss at two levels (T12–L1, L1–L2)
with osteophyte formation. A sagittal fat-suppressed T1-weighted
(TR/TE, 600/20) spin echo MR image obtained after the intra-
venous administration of a gadolinium contrast agent reveals
high signal intensity in the affected vertebral bodies and inter-
vertebral discs, as well as in the anterior soft tissues and intra-
spinal regions. The spinal cord is displaced and encased by the
infection.
Bacteroides and Related Anaerobic Infection culous arthritis can lead to pain, swelling, weakness,
In comparison to clostridia, which are spore-forming muscle wasting, a draining sinus, and other manifesta-
obligate anaerobic bacteria, Bacteroides and several other tions, which may be present for 1 to 2 years before
anaerobic bacteria are non–spore-forming obligate diagnosis. Tuberculous spondylitis first manifests clini-
anaerobes. Many of these organisms exist as part of the cally as an insidious onset of back pain, stiffness, local
normal microflora on the skin and adjacent mucous tenderness, and possibly fever. Tuberculous dactylitis
membranes, and clinically evident infections result when usually appears as painless swelling of the hand or foot.
breaks in the mucosa or skin allow the microflora to Tuberculous tenosynovitis and bursitis can produce soft
become displaced into deeper tissues and reach the blood- tissue swelling and tenderness in the ulnar or radial
stream. Characteristics of such infections include local- bursa, fingers, and toes.
ization to a site normally inhabited by anaerobic bacteria, A positive skin test for tuberculosis is of little help in
traumatic disruption of the skin or mucous membrane, diagnosing this disease (older patients who have never
and a history of diabetes mellitus or other chronic debili- exhibited any clinical manifestations of tuberculosis have
tating disease. In the musculoskeletal system, crepitant a high frequency of positive skin tests), although a nega-
cellulitis, necrotizing fasciitis, and myonecrosis are typical tive skin test usually (but not invariably) excludes the
expressions. Septic arthritis, infective spondylitis, and diagnosis. A negative chest radiograph in an adult patient
osteomyelitis are rare and may be manifestations of does not exclude the possibility of skeletal tuberculosis.
hematogenous dissemination. Gas formation in the soft In a child, such a radiograph makes tuberculosis an unlikely
tissues or, rarely, the articular cavity or intervertebral disc cause of bony abnormalities.
aids in the precise radiographic diagnosis.
Pathogenesis
Mycobacteria It is generally accepted that skeletal involvement in
tuberculosis occurs mainly by the hematogenous route.
Tuberculous Infection
Hematogenous seeding of the skeleton may arise from
The frequency of tuberculosis has changed dramatically a primary infection of the lung, particularly in children,
since the advent of appropriate chemotherapy for this or, at a later date, from a quiescent primary site or an
disease; however, even when pulmonary tuberculosis was extraosseous focus. Pulmonary involvement may be
common, musculoskeletal involvement was not frequent. evident in 50% of cases, is more frequent in children, and
Currently, the rate of pulmonary tuberculosis is on the on radiographs may appear either active or inactive.
rise, and the frequency of musculoskeletal tuberculosis Urogenital lesions may coexist with skeletal involvement
has also increased. Contributing factors to this increase in 20% to 45% of cases.
in tuberculosis cases include a greater number of people
who have suppressed immune systems, the development
of drug-resistant strains of mycobacteria, an aging popu- General Pathologic Considerations
lation, and an increase in the number of health care The typical response of tissue is the formation of tubercles
workers exposed to the disease. Further, the use of modern that are sharply demarcated from surrounding tissue.
therapeutic techniques, including bacille Calmette-Guérin Around a central zone are clusters of epithelioid cells. In
(BCG) vaccination, has produced examples of iatrogenic the central part of the tubercle are multinucleated giant
infection (see the discussion later in this chapter). cells, whereas at the periphery of the tubercle is a mantle
Additionally, the pattern of osteoarticular tuberculosis of lymphocytes. Central caseating necrosis is characteristic
has changed over the years. Initially, the disease was usu- of these tubercles and is incited by the tuberculin pro-
ally encountered in children and young adults; currently, duced by the bacilli.
patients of all ages are affected. Persons with underlying It should be emphasized that granulomatous disease,
disorders, those receiving corticosteroid medication, including that of the bone marrow, is a nonspecific
alcoholic patients, intravenous drug abusers, persons who response to a persistent antigenic stimulus and has been
harbor human immunodeficiency virus (HIV), and immi- identified in a wide range of illnesses (Table 55–1).
grants may be hosts for this disease. Tuberculous
spondylitis is the most typical form of the disease, with
the spine being involved in 25% to 50% of cases of skeletal Tuberculous Spondylitis
tuberculosis. In recent years, however, articular changes The vertebral column is affected in 25% to 60% of cases
in extraspinal sites, such as the hip, knee, wrist, and elbow, of skeletal tuberculosis. The first lumbar vertebra is most
have become more prominent. Tuberculous dactylitis, commonly affected, and the frequency of involvement
multiple sites of involvement, and tendon sheath abnor- decreases equally as one proceeds in either direction
malities are also commonly encountered. from this level. Tuberculous infection of the upper cervi-
cal and sacroiliac joint is not rare. More than one ver-
tebra is typically affected, and the vertebral body is
Clinical Abnormalities
involved more commonly than the posterior elements
Skeletal tuberculosis can affect persons of all ages. Extra- (Fig. 55–8). In the vertebral body, an anterior predilec-
pulmonary tuberculosis is more common in children tion is striking. Tuberculous spondylitis is generally
than in adults. The vertebral column, pelvis and hip, and thought to result from hematogenous spread of infection.
knee are the most frequent sites of involvement. Tuber- Whether the primary vascular pathway is supplied by the
Chapter 55 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Organisms 759
TABLE 55–1 chondral bone plate (Fig. 55–9). Infection may spread to
the adjacent intervertebral discs. Such spread may occur
Diseases Associated with Bone Marrow Granuloma if the bacilli extend beneath the anterior or posterior
longitudinal ligament to violate the peripheral disc tissue
Infectious Diseases
Bacterial infection and exposures
Mycobacterial disease
Tuberculosis
BCG vaccination
Leprosy
Brucellosis
Tularemia
Glanders
Fungal infection (disseminated)
Histoplasmosis
Cryptococcosis
Paracoccidioidomycosis
Saccharomyces cerevisiae infection
Viral infection
Infectious mononuclesosis
Cytomegalovirus infection
Viral hepatitis
Parasitic infection
Toxoplasmosis
Leishmaniasis
Other
Rocky Mountain spotted fever
Q fever
Mycoplasma pneumoniae infection Figure 55–8. Tuberculous spondylitis: sites of involvement.
Malignant Diseases Tuberculous lesions can localize in the vertebral body (1) or,
Hodgkin’s disease more rarely, the posterior osseous or ligamentous structures (2).
Non-Hodgkin’s lymphoma Extension to the intervertebral disc (3) or prevertebral tissues
Metastatic carcinoma (4) is not infrequent. Subligamentous spread (5) can lead to
Acute lymphocytic leukemia erosion of the anterior vertebral surface.
Drugs
Chlorpropamide
Phenylbutazone (oxyphenbutazone)
Allopurinol
Procainamide
Ibuprofen
Phenytoin
Autoimmune or Allergic Diseases
Rheumatoid arthritis (Felty’s syndrome)
Systemic lupus erythematosus
Primary biliary cirrhosis
Farmer’s lung
Miscellaneous
Syndrome of marrow and lymph node granuloma, uveitis, and
reversible renal failure
Berylliosis
Sarcoidosis
B
Figure 55–12. Tuberculous spondylitis: psoas abscess. A, Large,
noncalcified left psoas abscess (arrows). B, Diffusely calcified
psoas abscesses are noted in association with spinal abnormalities.
A B
A B
Chapter 55 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Organisms 763
A B
Figure 55–18. Tuberculous dactylitis. Radiographic findings
Figure 55–17. Cystic tuberculosis. A, Note the well-defined in this child include soft tissue swelling of multiple digits, lytic
lytic lesions of the medullary and cortical areas of the meta- lesions of several middle and proximal phalanges and metacarpals,
physis and diaphysis of the humerus. The proximal epiphysis is and exuberant periostitis and enlargement of the proximal
also affected. Sclerosis is absent, although periostitis can be phalanx of the third finger.
seen. B, Similar lesions are present in the tibia and fibula. Some
of these lesions are central, whereas others are eccentric or
peripheral.
lesions occur secondary to adjacent osteomyelitis. Most
patients are middle-aged or elderly, and many have under-
especially frequent in young children, uncommon after lying disorders or have received intra-articular injections
the age of 5 years, and rare after the age of 10 years. of steroids. Tuberculous joint disease may persist and
Although involvement of one bone of the hand or foot is cause chronic pain and only minimal signs of inflamma-
common, multiple osseous foci can be identified in 25% tion. Delay in diagnosis is frequent, and correct diagnosis
to 35% of cases and are especially characteristic of child- requires the use of synovial fluid and tissue for culture
hood dactylitis. Soft tissue swelling is usually the initial and histologic studies.
manifestation. Mild or exuberant periostitis of the pha- A triad of radiographic findings (Phemister’s triad) is
langes, metacarpals, or metatarsals may be evident (Fig. characteristic of tuberculous arthritis: juxta-articular
55–18). Cystlike expansion of the bone is termed spina osteoporosis, peripherally located osseous erosions, and
ventosa. Infection with a pyogenic or fungal origin may gradual narrowing of the interosseous space (Fig. 55–19).
have similar radiographic manifestations. Syphilitic Marginal erosions are especially characteristic of tuber-
dactylitis in infants and children produces bilateral and culosis in “tight” or weight-bearing articulations such as
symmetrical involvement; in that disease, periostitis is the hip, knee, and ankle. They produce corner defects
more exuberant and soft tissue swelling is less prominent simulating the erosions of other synovial processes, such
than in tuberculous dactylitis. Fibrous dysplasia, hyper- as rheumatoid arthritis. The combination of regional
parathyroidism, leukemia, sarcoidosis, and sickle cell anemia osteoporosis, marginal erosions, and relative preservation
may produce phalangeal, metacarpal, and metatarsal of joint space is highly suggestive of tuberculous arthritis.
changes, although characteristic radiographic alterations In rheumatoid arthritis, early loss of articular space is
at other sites allow an accurate diagnosis in many cases. more typical. The rapidity of joint space loss in tuber-
culosis is highly variable. Bony proliferation is generally
not as exuberant in tuberculous arthritis as it is in pyogenic
Tuberculous Arthritis
arthritis. The eventual result in tuberculous arthritis is
Tuberculous arthritis typically affects large joints such as usually fibrous ankylosis of the joint. Bony ankylosis is
the knee and hip, although any articular site can be occasionally seen, but this sequela is more frequent in
involved. Monoarticular disease is the rule. Most joint pyogenic arthritis.
764 Section XIV Infectious Diseases
A B
Figure 55–19. Tuberculous arthritis. A, Knee. On a conventional tomogram, typical marginal and central
osseous erosions (arrows) accompany tuberculous arthritis. Osteoporosis is not prominent. B, Hip. In a
different patient, osseous erosions on both sides of the joint, diffuse loss of interosseous space, and osteo-
porosis are evident. (B, Courtesy of J. Kaye, MD, Nashville, Tenn.)
The diagnosis of tuberculous arthritis is not difficult tained. In idiopathic chondrolysis, osteoporosis and early
when classic radiographic features appear in typical loca- joint space loss are evident, especially in the hip, although
tions, such as the knee, hip, wrist, or elbow. The appear- they occasionally occur in other locations as well.
ance of periarticular osteoporosis, marginal erosions, and
absent or mild joint space narrowing is most helpful in
Tuberculous Bursitis and Tenosynovitis
the accurate diagnosis of this disease (Table 55–2). In
rheumatoid arthritis, osteoporosis and marginal erosions The synovial membrane of bursae and tendon sheaths
are accompanied by early and significant loss of articular and the tendons themselves may be involved in tuber-
space. In gout, osteoporosis is mild or absent, although culosis (Fig. 55–20). Typical sites include the radial and
marginal erosions and preservation of interosseous space ulnar bursae of the hand, the flexor tendon sheaths of the
can be observed. In regional osteoporosis, marginal osseous fingers, the bursae about the ischial tuberosities, and the
defects are not apparent, and the joint space is main- subacromial (subdeltoid) and subgluteal bursae. Rarely,
other tendon sheaths are affected. Osseous destruction
can be encountered in the region of the greater trochanter,
and the hip joint may become infected secondarily. In
TABLE 55–2 any bursal location, dystrophic calcification may appear,
Comparison of Tuberculous and Pyogenic Arthritis a finding that is especially characteristic about the hip
and elbow.
Tuberculous Pyogenic
Arthritis Arthritis
BCG Vaccination–Induced Infection
Soft tissue swelling + +
Osteoporosis + ±
BCG is a vaccine of an attenuated bovine tubercle bacillus
that has been used for immunization against tuberculosis.
Joint space loss Late Early
Although complications are unusual, generalized BCG
Marginal erosions + + infection and bone and joint infection have been identi-
Bony proliferation (sclerosis, ± + fied after vaccination. The former complication is almost
periostitis) invariably fatal and is especially common in patients with
Bony ankylosis ± + immunologic deficiency. The latter complication results
Slow progression + – from hematogenous spread of BCG infection to the
skeleton, is not usually associated with immunologic dis-
+, common; ±, infrequent; –, rare or absent. orders, and has a favorable prognosis. The interval between
Chapter 55 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Organisms 765
Musculoskeletal Abnormalities
Musculoskeletal abnormalities include (1) those directly
related to the presence of the bacilli, in which granulo-
matous lesions appear in the osseous tissue, and (2) those
that involve the skeleton indirectly as a result of neural
abnormalities.
A B
Figure 55–23. Leprosy: neuropathic lesions. A, Examples of concentric bone atrophy in the foot illustrate
the tapered osseous surfaces (arrows) and phalangeal osteolysis. B, Fragmentation and collapse of the talar,
tibial, and calcaneal surfaces can be seen. The appearance is similar to that in tabes dorsalis. (A, Courtesy of
W. Coleman, MD, Carville, La.)
Infection is usually spread during sexual contact. Children in children older than 10 years. Such children may have
may acquire the disease by sharing a bed with an infected the hutchinsonian triad, which consists of Hutchinson’s
person. Such cases are termed acquired syphilis. In addi- teeth, interstitial keratitis, and nerve deafness. Additional
tion, the fetus may be infected by transmission of the manifestations include fissuring about the mouth and
organism through the placenta, termed congenital
syphilis.
Approximately 3 to 6 weeks after the organism has
entered the body, a primary lesion, called a chancre,
develops at the site of inoculation. This skin ulceration
heals spontaneously. About 6 weeks later, a generalized
skin eruption known as secondary syphilis develops. After
healing of both primary and secondary manifestations,
the patient may be without symptoms and signs for a pro-
tracted period—a stage termed latent syphilis—although
progressive inflammatory alterations may be slowly occur-
ring in many of the organ systems. Cardiovascular syphilis
or neurosyphilis may manifest 10 to 30 years later; how-
ever, tertiary manifestations of syphilis never develop in
approximately 50% of cases. In patients with significant
later alterations, large destructive lesions, or gummas, may
be evident in almost any organ of the body, particularly
the skin and the bones.
Congenital Syphilis
The disorder originates from transplacental migration
of the treponema and invasion of the perichondrium,
periosteum, cartilage, bone marrow, and sites of active
endochondral ossification, especially in the metaphyseal
regions of tubular bones. The spirochetes inhibit osteo- Figure 55–24. Leprosy: calcification of nerves. The linear
genesis. A fetus that is heavily infiltrated with spirochetes radiodense regions (arrows) represent calcification of nerves.
may be aborted or die shortly after birth. Others survive This finding, though rare, suggests the diagnosis of leprosy but
and develop the stigmata of congenital syphilis. Approxi- must be distinguished from vascular calcification. (Courtesy of
mately 75% of cases of congenital syphilis are diagnosed M. Dalinka, MD, Philadelphia, Pa.)
768 Section XIV Infectious Diseases
anus (rhagades), anterior bowing of the lower part of the chondritis generally heal quickly with specific therapy;
leg (saber shin), collapse of the nasal bones (saddle nose), healing is evident within 2 weeks and may be complete
and perforation of the palate. within 2 months.
Diaphyseal osteomyelitis (osteitis) can appear in infants
Early Osseous Lesions. In fetuses, neonates, and very young with congenital syphilis who have not received therapy or
infants, bony abnormalities include osteochondritis, dia- in whom treatment has been inadequate. Granulation
physeal osteomyelitis (osteitis), periostitis, and miscella- tissue in the metaphysis may extend into the diaphysis
neous changes. and induce infective foci of variable size (Fig. 55–26).
Syphilitic osteochondritis usually results in symmet- Osteolytic lesions with surrounding bony eburnation and
rical involvement of sites of endochondral ossification. overlying periostitis may be seen on radiographs of
The epiphyseal-metaphyseal junction of tubular bones, involved tubular bones.
the costochondral regions, and, in severe cases, the flat Periostitis is a less frequent manifestation of congenital
and short tubular bones and the centers of ossification syphilis than is osteochondritis. The long tubular bones
of the sternum and vertebrae are affected. In the grow- and, less commonly, the flat bones are affected. Reparative
ing metaphyses of the long bones, particularly about (reactive) periostitis is a second type of periosteal response
the knee, widening of the provisional calcification zone, that may be noted about healing foci of osteochondritis
serrations, and adjacent osseous irregularity are seen, or after epiphyseal slipping.
which on histologic evaluation are found to result from
a disturbance in endochondral ossification. Radiographs Late Osseous Lesions. The early manifestations of con-
outline broad horizontal radiolucent bands reminiscent genital syphilis generally regress or disappear in the first
of those identified in leukemia or metastasis from neuro- few years of life, even in the absence of adequate therapy.
blastoma. If the process continues, metaphyseal irregu- Exacerbation of disease may appear in a young child or
larities appear (Fig. 55–25). On radiographs, irregular adolescent (5 to 20 years of age), however. Although the
erosive lesions appear along the contour of the bone at evolving skeletal lesions that occur late in the course of
the metaphyseal–growth plate junction. Epiphyseal sepa- congenital syphilis may rarely resemble those of early
ration may result. The medial surface of the proximal congenital syphilis (osteochondritis, osteomyelitis, perios-
tibial shaft is a particularly characteristic site of erosion, a titis), they more typically resemble the changes observed
finding termed Wimberger’s sign. The lesions of osteo- in acquired syphilis (see later discussion). Osteomyelitis
A B
Figure 55–25. Congenital syphilis: osteochondritis. A, In this 3-week-old infant, the lucent band in the
metaphysis (arrow) is caused by a disturbance in endochondral ossification. The appearance is similar to that
in leukemia or neuroblastoma. B, In another infant with syphilis, a “celery stalk” appearance, with alter-
nating longitudinal lucent and sclerotic bands caused by an abnormality in endochondral ossification,
resembles the changes seen in rubella. (B, Courtesy of D. Edwards, MD, San Diego, Calif.)
Chapter 55 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Organisms 769
and periostitis can involve the tubular bones, the flat
bones, and even the cranium.
Gummatous or nongummatous osteomyelitis or perios-
titis results in diffuse hyperostosis of the involved bone.
Endosteal bony proliferation produces encroachment on
the medullary cavity, whereas periosteal bony prolifera-
tion creates an enlarged, undulating, and dense osseous
contour. In the tibia, a typical saber shin may be encoun-
tered, with anterior bending of the bone. Its radiographic
appearance may resemble that of Paget’s disease, although
the syphilitic hyperostosis may not extend to the epiphy-
sis (Fig. 55–27). Lucent defects within areas of hyperos-
tosis may represent gummas. Abnormalities of the skull
and mandible include destruction of the nasal bones,
calvarial gumma, and Hutchinson’s teeth (characterized
by peg-shaped, notched, and hypoplastic dental struc-
tures). Bilateral painless effusions, especially of the knee,
have been termed Clutton’s joints.
Acquired Syphilis
The osseus and articular manifestations of acquired
syphilis usually appear in the latent or tertiary phase of
the disease. The frequency of osseous lesions varies from
rare to as high as 8% to 20%.
Figure 55–26. Congenital syphilis: osteochondritis and Early Acquired Syphilis. Spirochetemia appearing 1 to
osteomyelitis. Note tibial erosion with periostitis (arrows). The 3 months after documentation of a primary lesion can
predilection for the medial tibial metaphysis is noteworthy. lead to dissemination of organisms throughout the body.
A B C
770 Section XIV Infectious Diseases
The spirochetes can reach the bone, with development Articular Involvement
of osteochondritis, periostitis, osteitis, or osteomyelitis. The frequency of articular involvement in syphilis is low.
Proliferative periostitis is the most common osseous Joint abnormalities may occur in either the congenital or
lesion in early acquired syphilis. It may be especially the acquired form of the disease. In congenital syphilis,
prominent in the tibia, skull, ribs, and sternum, although articular changes predominate in the late phases of the
other bones, such as the clavicle, femur, fibula, and osseous disorder; in acquired syphilis, joint manifestations appear
structures of the hand and foot, can also be affected. in the tertiary or, less frequently, the secondary stage. Joint
Destructive bone lesions occur much less commonly than effusions associated with pain and tenderness, which may
periostitis in early syphilis. These lesions are attributable be infectious or noninfectious in origin, are commonly
to osteomyelitis and infective osteitis (as well as septic bilateral and most typically affect the knee. Infectious
arthritis) (Fig. 55–28). Involvement of the skull is syphilitic arthritis can occur in any axial or extra-axial
particularly characteristic and has been noted in nearly site. Syphilitic infectious arthritis is associated with an
9% of patients with secondary syphilis. effusion and capsular distention. The synovial membrane
may become hypertrophied, with cellular infiltration.
Late Acquired Syphilis. Osseous lesions occurring during Synovial inflammation with pannus can lead to cartilagi-
the later stages of acquired syphilis can be related to nous and osseous destruction. On radiographs, osteo-
gummatous or nongummatous inflammation. A gumma porosis, joint space narrowing, bony destruction, sclerosis,
is a discrete or confluent area of variable size that con- and intra-articular osseous fusion may be seen, findings
tains caseous necrotic material and is related to the effects similar to those occurring in other infectious arthritides.
of the toxic products of spirochete degeneration, although
the organisms themselves are not usually demonstrable
within the lesions. Yaws
The radiographic features are characterized by lytic Yaws is an infectious disorder caused by Treponema pertenue.
and sclerotic areas of bone, which may reach considerable Yaws occurs in tropical climates and is prevalent in Africa,
size. Involvement of the cranial vault, nasal bones, maxilla, South America, the South Pacific islands, and the West
mandible, tubular bones of the appendicular skeleton, Indies. It is generally acquired before puberty during
spine, and pelvis has been noted. The degree of periosteal contact with open lesions containing the spirochetes.
proliferation can become extreme and, in tubular bones, Transmission of the disease is rarely associated with sexual
can lead to gross enlargement of osseous tissue. The contact. Within a period of weeks after inoculation, a
resultant radiographic and pathologic features resemble granulomatous primary lesion appears, referred to as the
those in the late stages of congenital syphilis, including the mother yaw. Approximately 1 to 3 months later, a gener-
saber shin deformity. Dactylitis, which is not infrequent alized papular skin eruption occurs on the extremities,
in congenital syphilis, is less typical of acquired disease. buttocks, neck, and face. Involvement of the soles, called
A B
Figure 55–28. Acquired syphilis: osteitis, osteomyelitis, and periostitis. A, Lytic lesions of the frontal
region of the skull (arrows) are accompanied by reactive sclerosis. B, Note the osteolysis of the metatarsal
bones and phalanges, associated with soft tissue swelling, periostitis, pathologic fracture, and articular
involvement.
Chapter 55 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Organisms 771
crab yaws, may make walking painful. After several years, osteomas (Fig. 55–30). Approximately 25% of cases show
late destructive lesions may become evident in cutaneous malignant degeneration leading to epidermoid carcino-
and osseous tissue. mas of the involved skin, which can cause destruction
The tubular bones of the extremities, including those of subjacent cortical and medullary tissue. The cause of
of the hands and feet, the pelvis, the skull, and the facial tropical ulcer appears to be multifactorial. Trauma is
bones may become the sites of periostitis or osteitis. common, and cultures of the lesion frequently isolate
Lucent lesions in the cortex or spongiosa are accom- Vincent’s types of fusiform bacilli and spirochetes.
panied by florid periosteal bone formation (Fig. 55–29).
Saber shin deformities (as in syphilis), dactylitis, and nasal
destruction may be encountered. Localized expansile Lyme Disease
lesions in the epiphyses of tubular bones may simulate First observed in 1975, Lyme disease is an inflammatory
neoplasms, and destructive changes in the fingers and articular condition named for the Connecticut town where
toes can resemble leprosy or psoriatic arthritis. In yaws, it was initially encountered. In the United States, most
however, the distal phalanges are usually spared. The cases of Lyme disease have occurred in the Northeast,
radiographic changes in the skeleton in patients with Upper Midwest, and Far West. Both children and adults
yaws are similar to those of syphilis. are affected. Clinical manifestations generally appear
within 7 days of a tick bite. The illness characteristically
begins in the summer in the form of a distinctive skin
Tropical Ulcer
lesion, erythema chronicum migrans, in the area of a
Tropical ulcers are seen in patients of all ages from previous tick bite. The rash disappears over a period of
Central and East Africa. The initial lesions are painful, weeks. Although the cutaneous manifestations are an
tense swellings with a serosanguineous discharge that important clue to the correct diagnosis, they are not
appear on the anterolateral aspect of the distal portions constant. Approximately 2 to 6 months later, joint mani-
of the lower limbs and spread rapidly. Because the ulcer festations appear and are characterized by a monoarticu-
erodes muscles and tendons, it may reach the underlying
bone. The favorite target area is the middle third of the
tibia; fibular involvement is less frequent and, when
present, is most common in the distal third of the shaft.
Periostitis leads to broad-based excrescences resembling
A B
Figure 55–30. Tropical ulcer. A, The anterior surface of the
tibia reveals a broad-based excrescence and an ivory osteoma.
The bone is bowed, and the thickened trabeculae in the
medullary bone indicate a response to altered stress. B, In a
Figure 55–29. Yaws. Dactylitis is characterized by lytic different patient, malignant degeneration at the site of skin
lesions surrounded by florid periosteal proliferation. Note the ulceration has led to destruction of the underlying bone. Note
enlarged and sclerotic osseous contours. (Courtesy of W. P. the irregularity of the involved soft tissues. (Courtesy of S.
Cockshott, MD, Hamilton, Ontario, Canada.) Bohrer, MD, Winston-Salem, N.C.)
772 Section XIV Infectious Diseases
lar, oligoarticular, or polyarticular process that is sudden and neck, which is probably explained by the prevalence
in onset, short in duration, and associated with recurrence of these organisms within the oral and nasal cavities.
and, sometimes, migration from one location to another. Trauma is important in the introduction of organisms
Sites of involvement, in order of decreasing frequency, into tissues. From infective foci in the face, lung, or
are the knee, shoulder, and elbow. bowel, hematogenous dissemination of organisms can
The radiographic characteristics of joint involvement lead to contamination of subcutaneous tissue, liver, spleen,
are soft tissue swelling and effusions. Infrequently, a kidneys, brain, bones, and joints.
chronic oligoarthritis develops, especially in the knees, Typically, the skeleton becomes contaminated from
that is associated with persistent and prominent joint an adjacent infected soft tissue focus; less commonly,
swelling. This finding may occur in as many as 10% of hematogenous seeding of osseous or articular tissue
untreated patients. In such cases, juxta-articular osteo- occurs. The mandible, the flat bones of the axial skeleton
porosis, cartilage loss, and marginal bone erosions may (pelvis, ribs, spine), and the major joints of the appen-
appear (Fig. 55–31). Lyme disease is transmitted by Ixodes dicular skeleton are most commonly affected. Mandibu-
dammini or related ixodid ticks. The disease itself is lar and maxillary bone involvement may follow trauma
caused by a recently recognized spirochete. The clinical or extraction of a tooth. Osseous involvement is charac-
and radiographic features of Lyme disease resemble those terized by a combination of lysis and sclerosis (Fig. 55–32).
of juvenile chronic arthritis, Reiter’s syndrome, and granu- In the ribs, the degree of bony proliferation may be
lomatous infections such as tuberculosis. extensive, and the combination of severe osseous ebur-
nation, cutaneous sinus tracts, and pleuritis is suggestive
of actinomycosis. In the vertebral column, infection can
FUNGAL AND HIGHER BACTERIAL
originate from adjacent mediastinal or retroperitoneal
INFECTION
foci. Several vertebrae are commonly affected and demon-
A variety of pathogenic fungi can produce human disease. strate lytic defects with surrounding sclerosis, and the inter-
Although healthy persons may become hosts for fungal vening intervertebral discs may be spared. Paravertebral
diseases, these pathogens become more virulent in subjects abscesses may appear, but they are usually smaller than
with depressed immunologic function, in whom wide- the abscesses in tuberculosis and do not calcify.
spread and sometimes fatal abnormalities may occur.
Cryptococcosis (Torulosis)
Actinomycosis
Cryptococcosis, a serious disease of worldwide distribu-
Actinomycosis is a noncontagious suppurative infection tion, is caused by Cryptococcus neoformans, an organism
caused by anaerobic organisms that are normally found that demonstrates an unusual predilection for the central
in the mouth. These organisms are higher bacteria that nervous system. The disease is generally acquired by the
resemble mycobacteria and are frequently misclassified as respiratory route through the inhalation of aerosolized
fungi. The infections are especially frequent in the face spores. Once they reach the body and proliferate, crypto-
cocci can be detected in the brain, meninges, lungs, other
viscera, and bones and joints. Neurologic manifestations
of the disease predominate, and many patients die within
Figure 55–31. Lyme disease. The glenohumeral joint space Figure 55–32. Actinomycosis has led to erosion and sclerosis
is narrowed, and erosions and osteophytes in the humeral head of a segment of the mandible (arrows). (Courtesy of R. Taketa,
are seen. (Courtesy of J. Lawson, MD, New Haven, Conn.) MD, Long Beach, Calif.)
Chapter 55 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Organisms 773
a few months. Cryptococcus infection may be seen in asso- some cases, with infection commonly following cuta-
ciation with leukemia, lymphoma, Hodgkin’s disease, sar- neous injuries. The respiratory tract may be a second site
coidosis, tuberculosis, and diabetes mellitus, as well as in of entry. In the skin, cutaneous abscesses develop beneath
persons with AIDS, those receiving steroid medications, the epidermis and are surrounded by a granulomatous
and those who have undergone renal transplantation. reaction. Similar lesions may be encountered in the lungs.
Osseous involvement is a manifestation of disseminated The bones may be altered in as many as 50% of patients
cryptococcosis and appears in 5% to 10% of such cases. with disseminated disease. Skeletal changes can occur from
Adults are affected far more frequently than children. hematogenous seeding or by direct extension from over-
The most commonly involved skeletal sites are the spine, lying cutaneous lesions. One or several osseous sites can
pelvis, ribs, skull, tibia, and bones about the knees, in be affected, especially the vertebra, rib, tibia, and carpus
descending order of frequency. Bony prominences may and tarsus (Fig. 55–34). No portion of the skeleton is
be affected, a peculiarity that is also evident in other fungal immune. The radiographic features of blastomycotic
disorders such as coccidioidomycosis. Single or multiple osteomyelitis are not specific. Moth-eaten bony destruc-
osseous foci are associated with soft tissue swelling and tion may be seen in association with osteoporosis and
pain. The radiographic features of bony involvement are periostitis. In the tubular bones of the extremities, eccen-
not specific. Osteolytic lesions predominate, with discrete tric saucer-shaped erosions may be detected beneath cuta-
margins, mild surrounding sclerosis, and little or no neous abscesses, or areas of focal or diffuse osteomyelitis
periosteal reaction (Fig. 55–33). Arthritis related to crypto- may be encountered in the subchondral regions of the
coccosis is very uncommon and almost invariably is the epiphysis or in the metaphysis. The lesions frequently
result of intra-articular extension of organisms from an possess sclerotic margins and are surrounded by perios-
adjacent osseous focus. titis. Extension from the infected foci to soft tissues or
joints is not unusual. Draining sinuses and cortical seques-
tration may appear in neglected cases. In the spine, blasto-
North American Blastomycosis
mycosis resembles tuberculosis. Articular involvement
This fungal disease is produced by Blastomyces dermatitidis. is usually related to extension from an adjacent site of
In the United States, its frequency is highest in the Ohio osteomyelitis.
and Mississippi River valleys and in the Middle Atlantic
states. The skin appears to be the portal of entry in
South American Blastomycosis and joints develop in only 10% to 20% of patients. Bone
(Paracoccidioidomycosis) alterations relate to hematogenous spread, although cuta-
The fungal disorder called South American blastomyco- neous infection can lead to contamination of subjacent
sis, caused by the organism Blastomyces (Paracoccidioides) bones (and joints). Osseous involvement can be confined
brasiliensis, occurs only in South America and in areas of to a single bone, but multiple, symmetrical osseous lesions
Mexico and Central America. The infective agents invade may be seen. Radiographs frequently reveal multiple
the pharynx, presumably after inhalation, and from there osseous lesions in the metaphyses of long tubular bones
spread locally or are disseminated throughout the body. and in bony prominences (patella, tibial tuberosity, calca-
Nasopharyngeal ulceration and local lymphadenopathy neus, ulnar olecranon) (Fig. 55–35). Well-demarcated lytic
may antedate the clinical findings in other locations. foci of the spongiosa are typical. Periostitis may be seen,
Hematogenous spread of infection to the lungs, spleen, but bone sclerosis and sequestration are unusual. Lesions
other abdominal viscera, and bones can occur. In general, involving the ribs are typically marginal in location and
the features of musculoskeletal involvement are similar can be associated with prominent extrapleural masses
to those in North American blastomycosis. Solitary or (Fig. 55–36). In the spine, abnormalities of one or more
multiple lytic lesions, geographic or well-defined bone vertebral bodies, with paraspinal masses and contiguous
destruction, marginal sclerosis, and periostitis are observed rib changes, are typical (Fig. 55–37). Joint involvement is
radiographic findings. most common in the ankle and knee (Fig. 55–38). In
general, articular changes result from extension of an
osteomyelitic focus. Monoarticular involvement is most
Coccidioidomycosis typical, with radiographs showing osteoporosis, effusion,
Coccidioidomycosis results from inhalation of the fungus joint space narrowing, and bony destruction.
Coccidioides immitis in endemic areas in the southwestern
United States, Mexico, and some regions of South America.
Histoplasmosis
After inhalation, the organisms lodge in the terminal bron-
chioles and alveoli of the lungs, where an inflammatory Histoplasmosis is caused by the dimorphic fungus Histo-
reaction may ensue. In some persons, disseminated disease plasma capsulatum, which is present in many areas of the
may develop, with spread of infection to the liver, spleen, United States, particularly the Mississippi River valley in
lymph nodes, skin, kidneys, meninges, pericardium, and the central portion of the country. Histoplasmosis, which
bones. Clinical manifestations vary in accordance with the is the most common systemic fungal infection in the
distribution of the lesions, and in cases of wide dissemi- United States, results from exposure to soil containing
nation, the mortality rate is high. the spores of this fungus. The portal of entry is usually
Although an acute, self-limited arthritis (desert rheuma- the respiratory tract, although the gastrointestinal system
tism) may develop in approximately 33% of cases of may be an additional portal in some persons. Diffuse
coccidioidomycosis, granulomatous lesions in the bones disease can result, and the fungus proliferates most exten-
A B
Chapter 55 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Organisms 775
A B
Figure 55–38. Coccidioidomycosis: septic arthritis. A, Radiograph reveals soft tissue swelling, marginal
osseous erosions (arrows), and flattening with sclerosis of the medial femoral condyle (arrowhead). The last-
mentioned findings resemble those in spontaneous osteonecrosis of the knee. B, In a different patient, a
coronal T1-weighted (TR/TE, 500/16) spin echo MR image shows similar marginal erosions, especially in
the tibia, along with marrow edema and a joint effusion.
A B
Figure 55–39. Histoplasma capsulatum var. duboisii osteomyelitis. A, Radiograph of the hand reveals cystic
lesions of the metacarpal bones and phalanges. In many areas, they are well marginated and surrounded by
reactive bone formation and mature periostitis. B, Extensive lesions of the tibial epiphysis and metaphysis
have produced collapse of the articular surface, with sclerosis and mild periostitis. Extension across the
growth cartilage is not unusual in fungal infections. (A, From Cockshott WP, Lucas AO: Histoplasmosis
duboisii. Q J Med 33:223, 1964.)
Chapter 55 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Organisms 777
orders, or pigmented villonodular synovitis. Involvement
of the small joints of the hands and feet appears to be
more characteristic of this fungal disease than the others.
Candidiasis (Moniliasis)
Of the various Candida species, Candida albicans is most
commonly associated with human disease. Candida
organisms normally reside on the mucous membranes.
Abnormal proliferation on these membranes may occur
in otherwise normal persons but is especially character-
istic in debilitated children or adults, in patients receiving
broad-spectrum antibiotics, in those with diabetes mellitus,
and in patients with intravenous or Foley catheters. In
the mouth, a mucocutaneous infection can be found, con-
sisting of white patches (thrush) with subjacent inflam-
mation of the buccal mucosa. Rarely, widespread infection
may develop.
Candida infection of the musculoskeletal system occurs
when host resistance is depressed. Intravenous drug addicts
may be affected, and a distinctive syndrome leading to
systemic candidiasis with costochondral involvement has
been recognized in approximately one third of heroin
addicts. Bone involvement in cases of disseminated can-
Figure 55–40. Sporotrichosis: septic arthritis. Note soft tissue
swelling, joint space loss, and irregularity and poor definition of didiasis is relatively rare, evident in less than 1% to 2%
subchondral bone, with marginal and central osseous erosions. of cases. When present, such osseous involvement can
The changes are identical to those in other forms of septic result from direct hematogenous seeding or extension
arthritis. Joint involvement is not infrequent in this disorder, from an overlying soft tissue abscess. Osteomyelitis in
and osteoporosis may or may not be present. (Courtesy of A. any age group can occur in one or more sites, including
Brower, MD, Norfolk, Va.) the tubular bones of the extremities, flat bones, and
spine. Common patterns of distribution are involvement
of a single long bone, involvement of the sternum, and
involvement of two consecutive vertebral bodies (Fig.
55–42). Septic arthritis is also observed in candidiasis.
Typically, infection predominates in large weight-bearing
joints; the knee is the most common site of involvement.
The pathogenesis of the articular infection can relate to
Aspergillosis
Aspergillus is normally a harmless inhabitant of the upper
respiratory tract. Uncommonly, in patients with low
resistance or in those who have received an
overwhelming inoculum, a chronic localized pulmonary
infection may result. The usual organism is Aspergillus
fumigatus. The musculoskeletal system is rarely involved
in aspergillosis. Two potential mechanisms of infection
have been emphasized: hematogenous infection, which
reportedly predominates in adults, and spread from a Figure 55–44. Aspergillosis: osteomyelitis. Transaxial CT
scan of the thorax in a 5-year-old boy with X-linked chronic
pulmonary or cutaneous infective site, a mechanism that granulomatous disease shows consolidation in the right apical
may predominate in children. Indeed, pulmonary portion of the lung, soft tissue swelling, loss of fat planes, and
aspergillosis leading to chest wall involvement has been osteolytic lesions (arrow) in the posteromedial aspect of the
emphasized as a finding of chronic granulomatous right third rib. (From Kawashima A, Kuhlman JE, Fishman
disease in children (Fig. 55–44). EK, et al: Pulmonary Aspergillus chest wall involvement in
chronic granulomatous disease: CT and MRI findings. Skeletal
Radiol 20:487, 1991.)
Maduromycosis (Mycetoma)
Maduromycosis, a chronic granulomatous fungal disease,
affects the feet (Madura foot). It can be observed Infection of the foot (and, less commonly, other sites)
throughout the world but is especially prevalent in India; results from post-traumatic soft tissue invasion by
in fact, the town of Madura in India is the source of its organisms that are normal inhabitants of soil. After soft
name. In the United States, a variety of organisms may tissue contamination, the organisms may penetrate the
cause Madura foot. underlying structures. Sinus tracts arising from the infect-
ed osseous tissues are common. The course of maduromy-
cosis is usually progressive. Initially, soft tissue swelling is
seen. Over a period of months to years, the foot becomes
swollen, deformed, and necrotic. The radiographic find-
ings vary with the virulence of the invading organism. In
some cases, single or multiple localized osseous defects
are evident; in others, extensive soft tissue and bony disrup-
tion occurs, with associated periostitis and sclerosis. Intra-
articular osseous fusion may occur and lead to an
appearance that is termed “melting snow” (Fig. 55–45).
VIRAL INFECTION
Rubella (German Measles)
Rubella is a contagious viral disease. Although it is gener-
ally a benign disorder in adults, maternal infection in the
first half of pregnancy can lead to serious skeletal and
nonskeletal alterations in the fetus.
Rheumatologic Disorders
Many of the rheumatologic manifestations of HIV infec-
tion fall within the spectrum of differentiated and undif-
ferentiated forms of spondyloarthropathy. In patients with
Figure 55–47. Cytomegalic inclusion disease. Metaphyseal HIV infection, the classic triad of clinical abnormalities
changes consist of irregularity of the growth plate and osseous in Reiter’s syndrome is commonly lacking because of the
fragmentation, most evident in the distal ends of the femora. infrequent occurrence of conjunctivitis. Fasciitis and
(Courtesy of F. N. Silverman, MD, Palo Alto, Calif.) enthesopathy occur, especially in the feet (Fig. 55–49),
A B
Chapter 55 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Organisms 781
been reported to be present in 15% to 20% of patients
with HIV infection. In these patients, Kaposi’s sarcoma is
aggressive in behavior, with multiorgan involvement.
Bacillary Angiomatosis
Bacillary angiomatosis is a disorder characterized by
histologic evidence of vascular proliferation in affected
tissues such as the skin, bone, lymph nodes, and brain and
by the presence of numerous bacillary organisms. Recent
biologic and microbiologic investigations have confirmed
that at least two organisms, Rochalimaea henselae and
Rochalimaea quintana, can cause this disease. Bacillary
angiomatosis can occur in patients infected with HIV, but
it is not restricted to such patients; it has also been
observed in immunocompetent patients and in recipients
of organ transplants. The typical clinical manifestation is
that of a cutaneous disorder with multiple friable angioma-
Figure 55–49. Human immunodeficiency virus infection: tous papules closely resembling pyogenic granulomas, as
reactive arthritis with enthesopathy and bursitis. Note enthesitis, well as the skin lesions of Kaposi’s sarcoma. Fever, chills,
with bone proliferation in the plantar aspect of the calcaneus, weight loss, night sweats, cellulitis, and subcutaneous
and retrocalcaneal bursitis, with erosion of the posterosuperior nodules are additional clinical features. Bone lesions may
portion of the calcaneus. also be seen, sometimes as an initial manifestation of the
disease. Tubular bones of the extremities are typically
affected (Fig. 55–50), with the flat and irregular bones
and are accompanied by marked muscle wasting. These affected less commonly. Osteolysis is the dominant
characteristics, which are nearly diagnostic, have been radiographic feature, and the osteolytic focus or foci may
referred to as the AIDS foot. As with Reiter’s syndrome,
psoriasis-like joint involvement may be seen in patients
with HIV infection.
Infectious Disorders
Osteomyelitis, septic arthritis, pyomyositis, and septic
bursitis are among the most frequent complications asso-
ciated with HIV infection. Although the knee is the most
common site of involvement, joints of the upper extremity
and the acromioclavicular, sternoclavicular, and sacroiliac
joints may also be affected. S. aureus or S. pneumoniae
is implicated most often, but opportunistic pathogens
represent additional potential causative agents.
Pyomyositis is a bacterial infection of muscle that is
generally, but not exclusively, caused by S. aureus and is
endemic in tropical regions of Africa, Southeast Asia, and
South America. It commonly affects patients who are
immunologically compromised or who have underlying
chronic disorders such as HIV infection. Almost 95% of
reported cases of pyomyositis associated with HIV infec-
tion involve localization in the lower extremity. Multiple
abscesses are identified in about 50% of cases. The most
typical pattern of disease, however, appears to be a solitary
abscess in the quadriceps musculature. Clinical findings
include fever and local muscle pain, redness, and swelling;
if untreated, this may be accompanied by marked edema
and septicemia.
Figure 55–50. Human immunodeficiency virus infection:
bacillary angiomatosis. This 34-year-old homosexual man had
Kaposi’s Sarcoma pain and swelling in the calf for 6 weeks. A poorly defined
osteolytic lesion of the fibula is evident. (From Conrad SE,
Among the malignant tumors encountered with increased Jacobs D, Gee J, et al: Pseudoneoplastic infection of bone in
frequency in patients with HIV infection are lymphomas, acquired immunodeficiency syndrome. A case report involving
Hodgkin’s disease, and Kaposi’s sarcoma. The last of these the cat-scratch disease bacillus. J Bone Joint Surg Am 73:774,
tumors arises from lymphatic endothelial cells, and it has 1991.)
782 Section XIV Infectious Diseases
be either well or poorly defined. Cortical or medullary the diagnosis of CSD (Fig. 55–51). Surrounding edema
destruction, or both, is seen, and an adjacent soft tissue is common, and enhancement of signal intensity in the
mass is an associated feature. Periostitis is often identi- peripheral portion of the mass may be noted after intra-
fied, and this finding combined with skin lesions in a venous gadolinium administration. Osseous lesions,
patient with AIDS is considered strong evidence of bacil- probably related to hematogenous dissemination and
lary angiomatosis. spread from a contiguous contaminated source, such as
a lymph node, are encountered uncommonly. These
lesions are not specific when studied by conventional
RICKETTSIAL INFECTION radiography because they resemble other processes such
as eosinophilic granuloma.
Cat-Scratch Disease
Cat-scratch disease (CSD) has been linked to two soil-borne
proteobacteria, Bartonella henselae and, occasionally, Afipia PROTOZOAN INFECTION
felia. CSD is often manifested as a local lymphadenitis
with lymphadenopathy occurring within 1 or 2 weeks Toxoplasmosis
after being scratched by a cat. Such lymphadenopathy is Toxoplasmosis is an infectious disorder caused by an
generally preceded by an erythematous pustule or papule intracellular protozoan parasite, Toxoplasma gondii. Human
at the site of inoculation that resolves spontaneously infections with Toxoplasma may be either congenital or
within several weeks to months. Involvement of the central acquired. The congenital variety of toxoplasmosis can be
nervous system, lung, liver, spleen, bone, and skin may severe. An infant may be stillborn at term or be born
complicate CSD in as many as 2% of cases. A serologic prematurely with active infection characterized by fever,
test (indirect fluorescent antibody) for R. henselae is rash, hepatosplenomegaly, mental retardation, chorio-
currently considered a useful adjunct for obtaining the retinitis, and convulsions, which may lead to death in
correct diagnosis. Affected persons are between the ages 10% to 20% of cases. Osseous lesions are unusual,
of 5 and 21 years, and CSD is probably the leading cause although metaphyseal alterations in tubular bones may
of chronic benign lymphadenopathy. More than 90% of simulate those of rubella, cytomegalic inclusion disease,
patients with CSD have a history of exposure to cats. or syphilis. Cerebral calcification may be evident. The
The initial clinical manifestations are cutaneous and acquired variety of the disease can occur at any age and
consist of skin eruptions at the site of inoculation. Because may display variable manifestations, including rash, lym-
the hands and forearms are the most frequent sites of phadenopathy, ocular changes, and widespread vascular
inoculation, adenopathy often develops around the elbow, alterations. Myalgias and myositis can accompany acquired
axilla, and head and neck; specific areas of adenopathy, toxoplasmosis. Radiographically evident osteoporosis,
in decreasing order of frequency, are the axillary and soft tissue swelling, and osseous cystic lesions have been
epitrochlear nodes, the cervical and submandibular nodes, described.
the groin, and the preauricular, postauricular, and supra-
clavicular chains. Conventional radiographs reveal soft
tissue edema, masses, or both. Although ultrasonography HELMINTHIC INFECTION
can also be used to study the soft tissue and systemic A variety of helminths (worms) can cause infection
manifestations of CSD, MR imaging may be more sensi- (Table 55–3).
tive and is often diagnostic. An ill-defined mass with
heterogeneous low signal intensity on T1-weighted spin
echo MR images and intermediate to high signal inten- Hookworm Disease
sity on T2-weighted spin echo MR images in the region Hookworm disease is produced by Ancylostoma duodenale
of the epitrochlear nodes about the elbow should suggest or Necator americanus. Anemia and its complications are
TABLE 55–3
Major Helminthic Infections of Humans
From Korzeniowdki OM: Diseases due to helminths. In Stein JH (ed): Internal Medicine. Boston, Little, Brown, 1983, p 1455.
the major clinical manifestations of this disorder. Muscu- be greatly enlarged, with soft tissue thickening, blurring
loskeletal abnormalities are rare. of subcutaneous fat planes, and a linear striated pattern.
Soft tissue calcification has been noted in W. bancrofti
infestation; elongated radiodense shadows in the subcu-
Loiasis taneous tissue represent calcified, dead, encysted filariae.
Loiasis is prevalent in West and Central Africa and is
produced by the filaria Loa loa (African eyeworm).
Infective larvae are deposited in the victim’s skin after a
bite of the mango fly. The larvae burrow into the deeper
subcutaneous tissue, where they mature to adult worms
over a period of 6 months or longer. The dead worms
cause abscesses or undergo calcification, or both. Calcific
deposits in subcutaneous tissue may be fine, coiled,
lacelike, and filamentous (calcification of the worm), or
they may be thicker, beadlike, and lobulated (calcification
of the fibrous capsule surrounding the worm) (Fig.
55–52; Table 55–4). Polyarthritis has been associated
with loiasis.
Onchocerciasis
Onchocerciasis is a form of filariasis produced by
Onchocerca volvulus and transmitted by flies. It is prevalent
in Africa and Central and South America. Soft tissue
calcifications, similar to those in loiasis, may be detected,
although radiographic demonstration of the calcified
areas is extremely difficult because of their small size.
Filariasis
Filariasis is produced by adult worms of the species
Wuchereria bancrofti or Brugia malayi, which localize in
the lymphatic and soft tissues of the human body. The
disease is predominant in tropical areas of Asia, Africa,
South America, Australia, and the South Pacific islands.
After prolonged and repeated attacks, filariasis can lead Figure 55–52. Loiasis (African eyeworm disease). Soft tissue
to massive lymphedema or elephantiasis, especially of the calcifications (arrowheads) are evident in the hand of this 29-
legs and scrotum. Radiographs show an affected limb to year-old man. (Courtesy of M. Dalinka, MD, Philadelphia, Pa.)
784 Section XIV Infectious Diseases
TABLE 55–4
Helminths (Worms) Associated with Calcification
Frequency of
Radiographic
Helminth (Disease) Calcification Typical Location of Calcification Typical Appearance of Calcification
Loa loa (loiasis) Common Widespread; subcutaneous tissues Extended or coiled, linear or
beaded, variable in size
Onchocerca volvulus (river blindness) Rare Legs, trunk, head; subcutaneous Extended or coiled, linear or
nodules beaded, small
Wuchereria bancrofti, Brugia malayi Rare Thighs, legs, scrotum; Straight or coiled, small
(filariasis) subcutaneous tissues
Dracunculus medinensis (guinea Common Extremities Extended or coiled, long
worm disease)
Taenia solium (cysticercosis) Common Widespread; muscular tissues Numerous, linear or oval, variable
in size, lie in plane of muscle
Echinococcus granulosus Common Liver, lungs, other organs Curvilinear, cystic, eggshell
(echinococcosis)
Sarcocystis lindemanni Common Extremities; muscular and Numerous, linear or oval, variable
(sarcosporidiosis) subcutaneous tissues in size and orientation
Armillifer armillatus, Porocephalida, Variable Abdomen, thorax Multiple, crescent shaped or oval
Pentastomida (porocephaliasis)
Schistosoma haematobium Variable Bladder, urinary tract Linear, nodular
(schistosomiasis)
Trichinosis
After ingestion of infected pork, humans may contract
trichinosis from the intestinal nematode Trichinella spiralis.
Calcification of the cysts of the parasite is commonly
detected on microscopic examination but is rarely, if
ever, noted on radiographic evaluation. Clinical features
resemble those of dermatomyositis. Figure 55–53. Dracunculiasis (guinea worm disease). Observe
the long linear calcification (arrows) adjacent to the lower part
of the tibia caused by the presence of a dying female worm.
Chapter 55 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Organisms 785
Cysticercosis the intestinal mucosa, and are disseminated via venous
The relationship between humans and the pork tape- and lymphatic channels. Cysts may develop in various
worm Taenia solium is twofold: humans are the only viscera, particularly the liver and the lungs. They may
definitive host of the adult tapeworm (the parasite inhabits calcify and produce irregular curvilinear radiodense areas.
the intestine), and humans may serve as an intermediate Bone lesions are reported in 1% to 2% of cases of
host (the usual intermediate host is the hog) and harbor echinococcosis. Osseous involvement is almost invariably
the larval stage, Cysticercus cellulosae. In the latter case, related to primary infection and is not the result of exten-
deposits of the larval form of the tapeworm may appear sion from a neighboring soft tissue lesion. Although
in subcutaneous and muscular tissue and in a variety of hematogenous seeding of the skeleton can conceivably
viscera, including the heart, brain, lung, liver, and eye. occur at any site, one bone, a few adjacent bones, or one
When the larvae die, a foreign body reaction may ensue, skeletal region is usually affected. The vertebral column,
followed over a period of years by caseation and calcifi- pelvis, long bones, and skull are most commonly involved.
cation. On radiographs, linear or oval elongated calcifica- The spine is involved in about 50% of cases.
tions appear in the soft tissues and musculature and may Radiographs can reveal single or multiple expansile,
reach 23 mm in length. The long axis of the calcified cystic, osteolytic lesions containing trabeculae (Fig. 55–55).
cysts lies in the plane of the surrounding muscle bundles These lesions may be associated with cortical violation
(Fig. 55–54). and soft tissue mass formation, with subsequent calcifi-
cation. The radiographic characteristics are similar to
those of fibrous dysplasia, plasmacytoma, giant cell tumor,
Echinococcosis cartilaginous neoplasm, skeletal metastasis (especially from
Echinococcosis is produced principally by the larval stage a tumor of the kidney or thyroid), brown tumor of hyper-
of Echinococcus granulosus and is most prevalent in sheep- parathyroidism, angiosarcoma, or hemophilic pseudo-
and cattle-raising areas of North and South Africa, South tumor. CT features of echinococcosis include a soft tissue
America, Central Europe, Australia, and Canada; less mass adjacent to sites of bone involvement; the center of
commonly, Echinococcus multilocularis is the causative the mass contains fluid with water attenuation values.
agent, especially in Alaska and Eurasia. In humans, E. Cystic lesions within the bone and adjacent soft tissue are
granulosus is contracted by ingestion of the eggs, which also identified with MR imaging; the signal character-
are contained in the feces of dogs (sheep or cattle dogs). istics of the cyst are variable (dependent on the type of
After ingestion, the embryos escape from the eggs, traverse cyst present and whether it is intact, ruptured, infected,
A B
Tietze’s Syndrome
Tietze’s syndrome and costochondritis are terms used to
describe pain, tenderness, and swelling at the costosternal Figure 55–57. Ainhum. Note the soft tissue groove (arrow)
joints. This condition is common and may occur in as and osseous resorption, especially on the medial aspect of the
many as 10% of patients with chest pain. Tietze’s proximal and middle phalanges of the fifth toe. Periostitis is
syndrome is benign and self-limited. Typically, painful absent.
swelling and tenderness to local palpation of one or
more costosternal junctions are observed. Radiographs
are seldom revealing, although rarely, soft tissue swelling, FURTHER READING
calcification, osteophytosis, and periostitis are encoun- Alexander GH, Mansuy MM: Disseminated bone tuberculosis
tered. Increased activity may be demonstrated on bone (so-called multiple cystic tuberculosis). Radiology 55:839,
scans. CT scanning may reveal sclerosis of the sternal 1950.
manubrium, partial calcification of costal cartilage, and Allen JH Jr: Bone involvement with disseminated histoplas-
soft tissue swelling. The cause is unknown. mosis. AJR Am J Roentgenol 82:250, 1959.
Chapter 55 Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Organisms 787
Baron AL, Steinbach LS, LeBoit PE, et al: Osteolytic lesions Harisinghani MG, McLoud TG, Shepard JO, et al: Tuber-
and bacillary angiomatosis in HIV infection: Radiographic culosis from head to toe. Radiographics 20:449, 2000.
differentiation from AIDS-related Kaposi sarcoma. Radiol- Harverson G, Warren AG: Tarsal bone disintegration in leprosy.
ogy 177:77, 1990. Clin Radiol 30:317, 1979.
Barre PS, Thompson GH, Morrison SC: Late skeletal defor- Haygood TM, Williamson SL: Radiographic findings of extrem-
mities following meningococcal sepsis and disseminated ity tuberculosis in childhood: Back to the future? Radio-
intravascular coagulation. J Pediatr Orthop 5:584, 1985. graphics 14:561, 1994.
Beggs I: The radiology of hydatid disease. AJR Am J Hong SH, Kim SM, Ahn JM, et al: Tuberculous versus pyogenic
Roentgenol 145:639, 1985. arthritis: MR imaging evaluation. Radiology 218:848, 2001.
Bertcher RW: Osteomyelitis variolosa. AJR Am J Roentgenol Hook EW, Campbell CG, Weens HS, et al: Salmonella
76:1149, 1956. osteomyelitis in patients with sickle cell anemia. N Engl J
Bonakdarpour A, Zadeh YFA, Maghssoudi H, et al: Costal Med 257:403, 1957.
echinococcosis: Report of six cases and review of the litera- Jacobs P: Osteo-articular tuberculosis in coloured immigrants:
ture. AJR Am J Roentgenol 118:371, 1973. A radiological study. Clin Radiol 15:59, 1964.
Braithwaite PA, Lees RF: Vertebral hydatid disease: Radio- Jaffe HL: Metabolic, Degenerative and Inflammatory Diseases
logical assessment. Radiology 140:763, 1981. of Bones and Joints. Philadelphia, Lea & Febiger, 1972.
Brown JS, Middlemiss JH: Bone changes in tropical ulcer. Br J Keats TE: Cysticercosis: A demonstration of its roentgen mani-
Radiol 29:213, 1956. festations. Mo Med 58:457, 1961.
Casado E, Olivé A, Holgado S, et al: Musculoskeletal mani- Kelly PJ, Martin WJ, Schirger A, et al: Brucellosis of the bones
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2001. Kumar R, Gulati MS, Nag HL: MR appearances in a case of
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Comstock C, Wolson AH: Roentgenology of sporotrichosis. Lewis R, Gorbach S, Altner P: Spinal Pseudomonas chondro-
AJR Am J Roentgenol 125:651, 1975. osteomyelitis in heroin users. N Engl J Med 286:1303, 1972.
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788 Section XIV Infectious Diseases
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culous spondylitis: Comparative imaging features. Radiology culosis of the adult spine. Skeletal Radiol 12:178,
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SECTION
XV
Traumatic Diseases
CHAPTER 56
Physical Injury: Concepts and DIAGNOSTIC TECHNIQUES
The sensitivity as well as the availability of conventional
Terminology radiography has led to its routine use in the delineation
of skeletal injuries. In the evaluation of trauma in children,
Donald Resnick and Thomas G. Goergen comparison views provide important information in less
than 5% of cases and should be obtained selectively.
They are most useful in the evaluation of Salter-Harris
type I growth plate injuries and in the assessment of
SUMMARY OF KEY FEATURES bowing fractures and injuries about the elbow. In certain
In addition to such complications as reflex sympathetic situations, radiographs obtained during the application of
dystrophy, osteolysis, osteonecrosis, many of the manual stress (i.e., stress radiography) can be effective in
osteochondroses, neuropathic osteoarthropathy, uncovering an articular injury that is not apparent on
heterotopic bone formation, and infection, physical initial radiographs (Fig. 56–1). This technique is used most
trauma may lead to a variety of radiographic commonly for injuries to the acromioclavicular joint,
abnormalities. The radiographic characteristics of knee, and ankle.
fractures in the various skeletal sites are explained on The role of computed tomography (CT) in the diag-
the basis of biomechanical principles. Special types of nosis of skeletal trauma is prominent. In general, this
injuries include pathologic, stress, greenstick, torus, technique is able to define the presence and extent of
bowing, and transchondral fractures; trabecular certain fractures or dislocations; detect intra-articular
microfractures; and osseous infractions accompanying abnormalities, including cartilage damage and osteocarti-
subluxations and dislocations. The precise cause of laginous bodies; and assess nearby soft tissues. Its appli-
osteochondritis dissecans is not clear, but trauma is cation to traumatic abnormalities in regions of complicated
suspected. Trauma to synovial joints may lead to anatomy, such as the spine, the bones in the face and
synovitis, hemarthrosis, and lipohemarthrosis; trauma pelvis, the glenohumeral and sternoclavicular joints, and
to symphyses may result in intraosseous cartilaginous the midfoot and hindfoot, is especially noteworthy (Fig.
displacement (cartilaginous nodes); trauma to 56–2). Recent advances in CT scanning, such as helical
synchondroses may cause variable patterns of acute scanning and three-dimensional reconstruction of image
or chronic growth plate injury; and trauma to supporting data, ensure rapid examination and vivid display of abnor-
structures, syndesmoses, and entheses may lead to malities, respectively.
tendinous and ligamentous laceration and disruption, After trauma to the musculoskeletal system, arteriogra-
avulsion, and diastasis. A variety of skeletal muscle phy may be useful for the identification and possible treat-
alterations are related to physiologic stress and acute ment of vascular abnormalities, including disruption and
or chronic injury. Characteristic skeletal abnormalities occlusion of major vessels, arteriovenous fistulas, and
also appear in abused children. aneurysms. Peripheral pulses may be palpable even with
complete transection of an artery, however, and absent
distal pulses may occur secondary to hypovolemia, pre-
existing atherosclerosis, or vasospasm. The vessels that
are injured most commonly are in close proximity to a
INTRODUCTION
bone and held in a relatively fixed position by fascial or
Physical injury contributes to a wide variety of alterations muscular attachment; for example, the subclavian artery
in bones, joints, and soft tissues. In addition to fractures, may be injured by the distal fragment of a clavicular frac-
dislocations, subluxations, and capsular, tendinous, mus- ture, or the axillary artery may be damaged in gleno-
cular, and ligamentous tears, trauma can affect the growth humeral joint dislocations. Mechanisms leading to
plate of immature skeletons, as well as the hyaline carti- vascular injury include a tear from the presence of a sharp
laginous and fibrocartilaginous joint structures. Further bone fragment, compression related to hematoma or
complications of trauma, covered in other chapters, include swelling within a tight fascial compartment, a shearing
reflex sympathetic dystrophy, osteolysis, osteonecrosis, type of injury, and entrapment in the fracture fragments
many of the osteochondroses, neuropathic osteoar- with angulation and occlusion. Magnetic resonance (MR)
thropathy, infection, and heterotopic bone formation. angiography can be used as a substitute for arteriography
Trauma has also been implicated in the development in some patients.
of certain neoplasms, such as aneurysmal bone cysts. Scintigraphy is useful in the evaluation of patients with
Nonmechanical trauma to the musculoskeletal system skeletal trauma, particularly in the diagnosis of stress
can result from thermal and electrical injury, irradiation, fractures. It also may be helpful in detecting subtle acute
and chemical substances. fractures when radiographs are normal or in excluding
789
790 Section XV Traumatic Diseases
FRACTURES
Epidemiology
The likelihood, location, and configuration of a fracture
after an injury depend on a number of factors, including
Figure 56–1. Physical injury: stress radiography. Injuries the age and sex of the person, the type and mechanism of
involving the ligaments about the ankle may not be detected on the injury, and the presence of any predisposing factors
routine radiographs, but the application of manual stress may that might alter the bones or soft tissues of the muscu-
allow their detection. In this case, radiographs obtained during loskeletal system. Birth-related trauma in a newborn,
varus stress of the plantar-flexed foot clearly demonstrate sports-related activities in an adolescent or young adult,
displacement of the talus with respect to the tibia, indicative of occupation-related stresses in a mature adult, and normal
an injury to the anterior talofibular ligament.
activities in the elderly are typical situations leading to
skeletal injury.
A B
Figure 56–2. Physical injury: osseous pelvis. Double-headed arrow indicates the approximate level of the
transaxial CT scan. Although the initial radiograph (A) shows symphyseal diastasis and a fracture of the
sacrum (arrow), the latter is better demonstrated with CT (B), which also reveals diastasis of the contralateral
sacroiliac joint with a vacuum phenomenon (arrowhead).
Chapter 56 Physical Injury: Concepts and Terminology 791
A B
Figure 56–3. Physical injury: proximal portion of the tibia. A, Initial radiograph reveals minimal sclerosis
beneath the tibial eminences but was interpreted as normal. B, Coronal T1-weighted (TR/TE, 750/20) spin
echo MR image vividly displays the fracture.
involves a cartilaginous surface. If the cartilage alone is be amplified by the use of terms denoting the direction of
involved, the term chondral fracture is used; a fracture the fracture line with reference to the shaft (long bones)
involving cartilage and subjacent bone is termed an osteo- or cortex (irregular bones). Four basic types of linear
chondral fracture. fractures involving the shaft of a tubular bone are recog-
In a closed (simple) fracture, the skin is intact and thus nized: transverse, oblique, oblique-transverse, and spiral.
prevents communication between the fracture and the A comminuted fracture is one with more than two frac-
outside environment. An open fracture allows communica- ture fragments, regardless of the total number of such
tion between the fracture and the outside environment fragments. Comminuted fractures may result from a
because of the disruption of skin. Bone fragments may or variety of different mechanisms, but in general, the greater
may not protrude through the cutaneous defect. Although the applied force and the more rapid its application, the
closed and open fractures are clinically distinguishable, greater the energy absorption by the bone and the more
specific findings accompanying an open fracture may be severe the comminution. Crush fractures result in severe
apparent on radiographs (Table 56–1). Open fractures comminution and associated soft tissue injury.
have a higher rate of disturbances in healing, in part Certain distinctive subtypes of comminuted fractures
related to an increased frequency of infection. exist. A butterfly fragment (Fig. 56–4) is a wedge-shaped
A complete fracture occurs when the entire circum- fragment arising from the shaft of a long bone at the apex
ference of a tubular bone or both cortical surfaces of a flat of the force input. A segmental fracture (Fig. 56–5) is one
bone have been disrupted. In an incomplete fracture, a in which the fracture lines isolate a segment of the shaft
break in the cortex does not extend completely through of a tubular bone. Segmental fractures have special impli-
the bone. When angular deformity is present, the intact cations in terms of the adequacy of the blood supply and
cortex may prevent satisfactory realignment. Incomplete the healing rate.
fractures occur in the resilient elastic bones of children The alignment of a fracture refers to the longitudinal
and young adults. They may be further classified into relationship of one fragment to another. If no significant
various types, including bowing, greenstick, and torus angulation is seen, the fracture is said to be in anatomic
fractures. The descriptive nomenclature of fractures can or near anatomic alignment. By convention, angulation
of the distal fragment is described in relation to the
proximal one. Such angulation may be medial or lateral,
dorsal or ventral, or, in the forearm, radial or ulnar. Varus
TABLE 56–1 refers to angulation of the distal fracture fragment toward
the midline of the body, and valgus refers to its angulation
Radiographic Signs of Open Fractures
away from the midline (Fig. 56–6). Anterior angulation at
the fracture site means that the apex of the fracture is
Soft tissue defect
directed anteriorly (ventrally). Conversely, posterior angu-
Bone protruding beyond soft tissues lation at the fracture site indicates that the apex of the
Subcutaneous or intra-articular gas fracture site is directed posteriorly (dorsally).
Foreign material beneath skin Fracture position describes the relationship of the frac-
Absent pieces of bone ture fragments, exclusive of angulation, to the normal
anatomic situation. Deviation from anatomic position is
792 Section XV Traumatic Diseases
A B
Figure 56–6. Varus and valgus angulation. A, Varus angula-
tion. The distal fragment is angulated toward the midline.
B, Valgus angulation. The distal fragment is angulated away
from the midline.
Fracture Healing
After a fracture, a remarkable series of events takes place
that leads to osseous healing in most cases (Fig. 56–12).
Three indistinctly separated phases of healing are recog- Figure 56–9. Avulsion fracture. The triceps tendon is attached
nized: an inflammatory phase (representing approximately to the large fracture fragment. A joint effusion is noted (arrow-
10% of the entire healing time), a reparative phase (about heads).
40%), and a remodeling phase (the longest phase, account-
ing for 50% to as much as 70% of healing time). Initially,
bleeding takes place from the damaged ends of the bones
and from the neighboring soft tissues. Formation of a
hematoma within the medullary canal, between the frac-
A B C
Figure 56–12. Normal fracture healing. A, After the injury,
bleeding is related to osseous and soft tissue damage. A hematoma,
followed by clot formation, develops within the medullary canal
between the fracture ends and beneath the periosteal mem-
brane, which may have been torn. B, Callus formation takes
place and consists of external bridging callus at the periosteal Figure 56–13. Abnormal fracture healing: nonunion in the
surface, intramedullary callus, and primary callus at the ends of tibia. Classic hypertrophic nonunion is evident. Prominent
the fracture fragments. C, Callus envelops the bone ends rapidly external callus and irregular bone ends are seen. The adjacent
and produces increasing stability at the fracture site. fibular fracture has healed.
Chapter 56 Physical Injury: Concepts and Terminology 795
process that weakens the osseous tissue structurally can
result in a pathologic fracture. Of the tumorous causes
of pathologic fracture, skeletal metastasis predominates.
Radiographic distinction between a pathologic and a
nonpathologic fracture is not always easy. Such differen-
tiation is not difficult, however, when a fracture line
traverses a large area of osseous destruction or when the
adjacent or distant bones are riddled with additional
lesions (Fig. 56–14). When a smaller lesion is present, the
fracture itself may obscure the area of lysis or sclerosis,
especially in the presence of displacement at the fracture
site. The absence of a history of trauma or fracture pain
and the presence of symptoms and signs of a preexisting
abnormality, such as angular deformity, painless swelling,
or generalized bone pain, are clinical aids to the diagnosis
of a pathologic fracture. Diagnostic difficulty may be
encountered in a patient who has a nonpathologic frac-
ture that is days to weeks old, because resorption, osteolysis,
or rotation about the fracture site may create the illusion
of an underlying lesion.
A B
Figure 56–15. Trabecular microfracture (bone bruise). Sagittal intermediate-weighted (TR/TE, 2200/30)
(A) and T2-weighted (TR/TE, 2200/80) (B) spin echo MR images show the characteristic finding of a bone
bruise (arrows), which in this case involves mainly the posterior portion of the medial tibial plateau. The
lesion is of high signal intensity in (B). Note the joint effusion.
796 Section XV Traumatic Diseases
Stress Fractures
Stress fractures can occur in normal or abnormal bone
that is subjected to repeated cyclic loading with a load
less than that which causes acute fracture of bone. Two
types of stress fracture are recognized: fatigue fracture,
which results from the application of abnormal stress or
torque on a bone with normal elastic resistance, and
Figure 56–16. Insufficiency fracture: rheumatoid arthritis
insufficiency fracture, which occurs when normal stress is and osteoporosis. In parasymphyseal bone, such fractures
placed on a bone with deficient elastic resistance. (arrows) are accompanied by osteolysis, osteosclerosis, and bone
Fatigue fractures frequently share the following fragmentation.
features: the activity is new or different for the person,
the activity is strenuous, and the activity is repeated with
a frequency that ultimately produces symptoms and is apparent) and is consistent with the belief that shin
signs. Fatigue fractures are relatively common in athletes splints represent periosteal disruptions of varying length,
and military recruits undergoing basic training; they are possibly caused by the rupture of Sharpey’s fibers that
especially frequent in runners and ballet dancers, and extend from the muscle through the periosteum into the
their sites of involvement can often be predicted by an cortical structure of bone.
analysis of the specific sporting activity. Typical examples MR imaging has comparable sensitivity and superior
are the fatigue fractures that occur in the metatarsal specificity with respect to bone scintigraphy in the assess-
bones of military recruits (“march” fractures) and in the ment of stress fractures. Stress fractures typically appear
lower extremities in athletes, joggers, and dancers. as a linear zone of low signal intensity surrounded by a
The causes of insufficiency fractures are diverse and broader, poorly defined area of slightly higher (though
include rheumatoid arthritis, osteoporosis, Paget’s disease, still low) signal intensity on T1-weighted spin echo MR
osteomalacia or rickets, hyperparathyroidism, renal osteo- images and as a linear area of low signal intensity
dystrophy, osteogenesis imperfecta, osteopetrosis, fibrous surrounded by a broader region of high signal intensity
dysplasia, and irradiation. Of these causes, rheumatoid on T2-weighted spin echo images (Fig. 56–19). High
arthritis and osteoporosis are most common (Fig. 56–16). signal intensity on STIR images and after the intravenous
Fatigue and insufficiency fractures are not infrequent after administration of a gadolinium contrast agent may also
certain surgical procedures that result in altered stress or be observed. The fracture line may or may not be evi-
an imbalance of muscular force on normal or abnormal dent. Soft tissue edema may be an associated feature.
bones. Common examples are noted in the metatarsal The clinical findings of stress fractures are character-
bones after bunion surgery and in the lower extremities istic. Activity-related pain that is relieved by rest is typical.
after arthrodesis or arthroplasty. Localized tenderness and soft tissue swelling may also be
Although routine radiography plays an essential role observed. The specific site of involvement is influenced
in the diagnosis of stress fractures, radionuclide examina- by the type of physical activity engaged in. The bones in
tion is one means of early detection (Fig. 56–17). The the lower extremity are affected more frequently than
finding of abnormal scintigraphic patterns in athletic those in the upper extremity. More than one site can be
individuals in whom stress fractures do not subsequently involved simultaneously, more than one stress fracture
develop has led to a redefinition of some osseous stress can occur in a single bone, and symmetrical changes are
injuries. A shin splint is one of these conditions. In not unusual. The radiographic abnormalities are influ-
patients with shin splints, radionuclide angiograms and enced by the location of the fracture and the interval
blood pool images are normal, whereas on delayed images, between the time of injury and the radiographic exami-
a longitudinally oriented area of increased radionuclide nation. In a diaphysis, a linear cortical radiolucent area is
accumulation is seen in the posteromedial cortex of the frequently associated with periosteal and endosteal cor-
tibia (Fig. 56–18). This pattern of abnormality differs tical thickening (Fig. 56–20). In some cases, multiple
from that typically seen in an acute stress fracture (in radiolucent striations that extend partially or completely
which all phases of the radionuclide examination are across the cortex are seen. In an epiphyseal or metaphy-
abnormal and a more fusiform area of augmented activity seal location or in a cancellous area, focal sclerosis repre-
Chapter 56 Physical Injury: Concepts and Terminology 797
A B
Figure 56–17. Stress fracture. This athletic young woman complained of persistent hip pain aggravated
by activity. A, Radionuclide examination reveals a focal, sharply marginated area of increased activity in the
femoral neck (arrow). B, Radiograph of the hip delineates a minimal amount of indistinct new bone
formation along the medial aspect of the femoral neck (arrow).
senting condensation of trabeculae is the typical finding, Specific examples of activities that cause stress
and periostitis is not prominent (Fig. 56–21). With heal- fractures are listed in Table 56–2.
ing at either site, the sclerosis may become more diffuse,
and eventually, it and the fracture line can disappear. Calcaneal or Other Tarsal Stress Fracture. Fatigue fractures
of the calcaneus are not uncommon in military recruits,
and insufficiency fractures in this site can accompany
rheumatoid arthritis, neurologic disorders, and other
diseases (Fig. 56–22). Stress fractures in other tarsal
bones are less common. Those in the tarsal navicular
bone have been observed in physically active persons,
especially basketball players and runners (Fig. 56–23).
A B
Figure 56–19. Stress fracture: fatigue fracture of the femur in a runner. Coronal T1-weighted (TR/TE,
634/16) spin echo (A) and STIR (TR/TE, 3940/72; inversion time, 150 msec) (B) images show a linear focus
of decreased signal (arrows), representing sclerosis, with surrounding edema.
and becomes displaced in some situations; the compres- Metatarsal Stress Fracture. The metatarsal bones are fre-
sion type is more common in younger patients, appears quent sites of stress fracture, which may be caused by
as a haze of callus in the inferior aspect of the neck, and marching, ballet dancing, prolonged standing, foot
is stable in most cases (Fig. 56–25). deformities, and surgical resection of adjacent metatarsal
bones. The middle and distal portions of the shafts of the
second and third metatarsal bones are affected most often,
but any metatarsal bone can be involved. In the first
metatarsal, the base of the bone is altered, and periostitis,
a frequent finding at other metatarsal sites, is relatively
uncommon (Fig. 56–26). Occasionally, stress fractures
involve the metatarsal heads and lead to sclerosis and
flattening of subchondral bone, findings resemble those
of Freiberg’s infraction.
Modified from Daffner RH: Stress fractures: Current concept. Skeletal Radiol
2:221, 1978.
A
Figure 56–24. Tibial stress fracture. In this 12-year-old boy,
observe a band of sclerosis across the proximal metadiaphysis of
the bone, along with periostitis. (Courtesy of K. Van Lom, MD,
San Diego, Calif.)
A C
Chapter 56 Physical Injury: Concepts and Terminology 801
tennis players, golfers, and baseball players; the olecranon
process in baseball pitchers and javelin throwers; the
phalangeal tufts in guitar players; the phalanges of the
hand in bowlers; and the inferior edge of the glenoid
fossa in baseball pitchers.
A B
Figure 56–29. Spondylolysis in the lumbar spine: L5. Lateral (A) and left posterior oblique (B) projec-
tions reveal a defect through the pars interarticularis (arrowheads). The spine has a “Scottie dog” appearance
on oblique views. The resulting lucent lesion has produced a break in the “neck” of the “Scottie dog” in the
oblique projection. Grade I spondylolisthesis of L5 on S1 can be noted on the lateral radiograph.
A B
Figure 56–30. Unilateral spondylolysis with contralateral bony hypertrophy and reactive sclerosis. The
predominant abnormality on a frontal radiograph of the lumbar spine (A) is osteosclerosis in the region of
the right pedicle of the fifth lumbar vertebra (arrow). The left transverse process of this vertebra is smaller
than that on the right. A transaxial CT scan (B) of this vertebra reveals spondylolysis (arrowheads) and
contralateral bone hypertrophy (arrow). (Courtesy of J. A. Amberg, MD, San Diego, Calif.)
Greenstick, Torus, and Bowing Fractures young tree branch that, when broken, is disrupted on its
In an immature skeleton, fractures that do not completely outer surface but remains intact on its inner surface.
penetrate the entire shaft of a bone are not infrequent. A Typical locations of greenstick fractures are the proximal
greenstick fracture is one that perforates one cortex and metaphysis or diaphysis of the tibia and the middle third
ramifies within the medullary bone (Fig. 56–32). The of the radius and ulna. A torus (buckling) fracture results
name is derived from this fracture’s resemblance to a from an injury insufficient in force to create a complete
Chapter 56 Physical Injury: Concepts and Terminology 803
Toddler’s Fractures
Infants and toddlers frequently develop an acute-onset
limp without a clear history of specific injury. The classic
toddler’s fracture is a nondisplaced, oblique fracture of
the distal diaphysis of the tibia (Fig. 56–36). Although
this fracture is the most common pattern of injury, other Figure 56–36. Toddler’s fracture: tibia. Radiograph reveals a
causes of these clinical manifestations are occult fractures nondisplaced oblique fracture of the distal tibia.
of the fibula, femur, metatarsal bones (particularly the
first), and, less commonly, calcaneus. Most toddlers’ frac-
tures occur between the ages of 1 and 3 years; the
Acute Chondral and Osteochondral Fractures
remainder occur before the age of 1 year.
Shearing, rotational, or tangentially aligned impaction
forces generated by abnormal joint motion may produce
fractures of one or both of the two apposing joint surfaces.
Acute injuries can produce fragments consisting of carti-
lage alone (chondral fractures) or cartilage and underlying
bone (osteochondral fractures). A purely cartilaginous
fragment creates no direct radiographic abnormalities,
whereas one containing calcified cartilage and bone
becomes apparent because of a varying degree of radio-
density (Fig. 56–37). Secondary radiographic signs con-
sisting of soft tissue swelling and joint effusion can be
apparent with either chondral or chondro-osseous frag-
ments. After the injury, the detached portion of the artic-
ular surface can remain in situ, be slightly displaced, or
become loose, or free, within the joint cavity (Fig. 56–38).
Figure 56–38. Acute chondral and osteochondral fractures: fate of fragments. Chondral or osteochondral
fragments can remain in situ (arrowhead), be slightly displaced or loose in the articular cavity (open arrow),
or become embedded at a distant synovial site and evoke a local inflammatory reaction (solid arrow).
Radiographic identification of loose osteocartilaginous zone of high signal intensity at the interface of the frag-
bodies or those attached to the synovial lining requires a ment and the parent bone is a reliable sign of lesion
careful search of the recesses and dependent portions of stability. Unstable lesions are characterized by (1) the
the joint. Such bodies are common in the olecranon, presence of a line of high signal intensity at the interface
coronoid, and radial fossae in the elbow; the axillary and between the osseous fragment and the adjacent bone, (2)
subscapular recesses in the glenohumeral joint; the an articular fracture indicated by joint fluid of high signal
acetabular fossa and recesses about the zona orbicularis intensity passing through the subchondral bone plate, (3)
of the hip; and the posterior regions and recesses in the a focal osteochondral defect filled with joint fluid, and (4)
knee. Detection of osteocartilaginous bodies should stimu- a 5 mm or larger fluid-filled cyst deep to the lesion. MR
late a search for their site of origin. Osteochondral injuries arthrography may also be useful in the evaluation osteo-
are a well-recognized component of a variety of momen- chondritis dissecans and in establishing fragment stability.
tary or persistent subluxations and dislocations. Classic
examples include injuries to the glenoid region of the Femoral Condyle. One of the most common locations of
scapula and humeral head with dislocation of the gleno- osteochondritis dissecans is the condylar surfaces of the
humeral joint, injuries to the patella and lateral femoral distal portion of the femur, accounting for as many as
condyle with dislocation of the patella, and injuries to the 75% of cases. With regard to femoral involvement, men
femoral head with dislocation of the hip (Fig. 56–39). are affected more frequently than women, and the average
age at the onset of symptoms and signs is 15 to 20 years,
although the age range is highly variable. Unilateral
Osteochondritis Dissecans changes predominate over bilateral changes by a ratio of
Osteochondritis dissecans is characterized by fragmen-
tation and possible separation of a portion of the articular
surface. The age at onset varies from childhood to middle
age, but an onset in adolescence is most common. Patients
may be entirely asymptomatic; however, pain aggravated
by movement, limitation of motion, clicking, locking, and
swelling may be apparent. Single or multiple sites can
be affected. The condition is generally believed to be the
eventual result of an osteochondral fracture that was
initially caused by shearing, rotatory, or tangentially
aligned impaction forces.
Considerable interest has developed in determining
the stability of the osteochondral fragment. MR imaging
is most useful in this regard, with high signal intensity on
T2-weighted images adjacent to the osteochondral frag-
ment indicative of fluid or granulation tissue, which is
strong but not infallible evidence of an unstable lesion; Figure 56–39. Acute chondral and osteochondral fractures:
the presence of fluid encircling the fragment or focal lateral patellar dislocation. Note the fracture of the medial
cystic areas beneath the fragment are the best indicators margin of the patella (arrow) and lateral subluxation of the
of instability (Fig. 56–40). Similarly, the absence of a patella on the axial radiograph.
806 Section XV Traumatic Diseases
A B
Figure 56–40. Osteochondritis dissecans of the lateral femoral condyle. Sagittal intermediate-weighted
(TR/TE, 2200/30) (A) and T2-weighted (TR/TE, 2200/80) (B) spin echo MR images reveal the lesion
involving the anterior surface of the lateral condyle. The fragment is ossified, and the junction between it
and the parent bone (arrows) demonstrates intermediate signal intensity in (A) and high signal intensity in
(B). The abnormalities are consistent with granulation tissue or fluid in this junctional area. Although the
overlying cartilage is not seen well, the MR imaging findings suggest the presence of an unstable lesion.
(Courtesy of J. Blassinghame, MD, San Diego, Calif.)
A B
Figure 56–44. Osteochondritis dissecans of the talus: medial lesion. A, Note the lucent lesion of the
medial talar dome (arrow), the site of an osteochondral fragment. B, Corresponding coronal, volume
gradient (TR/TE, 28/7; flip angle, 25 degrees) MR image shows the nondisplaced fragment.
FRACTURE TYPE
TABLE 56–3
Biomechanics of Fractures in Long Tubular Bones
Energy
Fracture Pattern Mechanism of Injury Location of Soft Tissue Hinge Load Common Sites
Transverse Bending Concavity Low Diaphyses
Oblique Compression, bending, and Concavity (often destroyed) Moderate Radius, ulna, tibia, fibula
torsion
Oblique-transverse Compression and bending Concavity or side of butterfly Moderate Femur, tibia, humerus
fragment
Spiral Torsion Vertical segment Low Tibia, humerus
Diaphyseal impaction Compression Variable Variable Humerus, femur, tibia
Comminuted Variable Destroyed High Variable
From Gonza ER, Harrington IJ: Biomechanics of Musculoskeletal Injury. Baltimore, Williams & Wilkins, 1982, p 2.
810 Section XV Traumatic Diseases
Figure 56–48. Biomechanics of fractures in long tubular bones. A and B, Application of longitudinal
compression force (axial compression). Loading of a long bone in this manner may cause an incomplete
(bowing or torus) fracture in a child or impaction of the diaphysis into the metaphysis in an adult (e.g.,
intercondylar fracture of the distal portion of the humerus or femur). C and D, Application of angular force
(bending). Angular force may result in a greenstick fracture in a child or a transverse fracture in an adult.
Cortical bone is stronger in compression than in tension, so the bone fails initially on the tension side
(opposite the input force) and the fracture propagates transversely to the long axis of the bone. The
periosteum may be preserved on the side of the input force. E, Traction force (tension) at the site of a tendon
or ligament insertion may result in a transverse (avulsion) fracture. F, An oblique fracture configuration
results from a combination of multiple forces, including longitudinal compression and angular and rotational
forces. G, An oblique-transverse fracture results from a combination of angular and axial compression forces.
The direction of the fracture line is determined by the relative magnitude of the two forces: when axial
compression force predominates, the oblique component is larger; conversely, a predominantly angular
force results in a more transverse fracture configuration. The oblique fragment may separate in a butterfly
configuration. H, A spiral fracture results from rotational forces. The fracture line approximates an angle of
40 to 45 degrees, and the direction of the spiral denotes the direction of the rotational forces.
cially; differentiation between the two is important, tion of forces can lead to several different types of
however, because oblique fractures have a higher fre- fracture: if the compression forces are larger than the
quency of nonunion, whereas spiral fractures usually bending forces, an oblique fracture is produced; if the
heal uneventfully. In an oblique fracture, the ends of bending forces are sufficiently large, a purely trans-
the bones are short and blunt, a vertical segment is not verse fracture is seen; and if both the compression and
identified, and a clear space may be seen; in a spiral the bending forces are of sufficient magnitude, an
fracture, long, sharp, pointed ends and a vertical segment oblique-transverse fracture results (see Fig. 56–49). A
are characteristic, and unless the fracture is distracted, butterfly fragment may be an added component of the
no clear space is evident (Fig. 56–49). oblique-transverse fracture pattern.
Oblique-transverse fracture (mechanism: combination of Spiral fracture (mechanism: torsion force). Spiral fractures
axial compression and bending forces). This combina- are relatively uncommon and result from twisting or
Chapter 56 Physical Injury: Concepts and Terminology 811
rotational forces, perhaps combined with axial com-
pression. They are usually observed in the humerus
and the tibia (see Fig. 56–49).
Diaphyseal impaction fracture (mechanism: axial compres-
sion force). In certain locations, such as the humerus,
femur, and tibia, an axially applied load drives the
diaphyseal bone, with its thick and rigid cortex, into
the thin metaphyseal bone. Examples of this injury are
a supracondylar fracture of the femur and a com-
minuted fracture of the tibial plateaus.
Comminuted fracture (mechanism: variable). Indirect or
direct application of force, usually of high energy, leads
to multiple osseous fragments of varying size.
DISLOCATIONS
Terminology
Dislocation is complete loss of contact between two osseous
surfaces that normally articulate. Subluxation is a partial
loss of this contact. Closed subluxation or dislocation
exists when the skin and soft tissues remain intact over
the injured joint; open dislocation or subluxation exists
if associated soft tissue injury exposes the joint to the
outside environment (Fig. 56–50). Subluxations and dislo-
cations are usually caused by physical trauma, although
they can also occur when congenital or acquired condi-
tions produce muscle imbalance. Many dislocations and Figure 56–50. Open dislocation. Dorsal dislocation of the
subluxations related to trauma are associated with frac- terminal phalanx at the interphalangeal joint of the thumb is
ture of a neighboring bone. Typical examples of such associated with a soft tissue defect in the volar surface of the
fractures are the Hill-Sachs lesion of the humeral head finger and soft tissue air (arrow).
A B C
Figure 56–49. Long bone fracture. A, Oblique (tapping) fracture of the midshaft of the ulna resulting from
a direct blow. B, Spiral fracture of the femoral diaphysis. C, Oblique-transverse fracture with mild comminution.
812 Section XV Traumatic Diseases
Lipohemarthrosis
Bloody synovial fluid containing fat droplets and bone
marrow spicules is reliable evidence of an intra-articular
fracture. Frequently, however, a hemorrhagic effusion
containing fat may be observed in patients without frac-
ture, probably related to significant cartilaginous or liga-
mentous injury.
Radiographic examination using a horizontal beam
technique may demonstrate a fat-blood fluid level after
injury to the joint (Fig. 56–54). Most commonly, this B
finding is seen in a knee or shoulder, although it may also
be noted in other joints. In the knee, subtle tibial plateau Figure 56–55. Lipohemarthrosis. A, On a coronal T1-weighted
fractures may be the source of the fat. Lipohemarthroses (TR/TE, 500/20) spin echo MR image of the knee, globules of
can also be detected with CT or MR imaging (Fig. 56–55). fat (arrow) in the suprapatellar pouch, above the patella, are of
high signal intensity. The bloody joint effusion is of intermediate
With MR imaging, the appearance of a lipohemarthrosis signal intensity. B, Sagittal T1-weighted (TR/TE, 900/30) spin
is more complex. The most superior zone contains float- echo MR image shows a dominant fluid level (arrow) at the
ing fat, a central zone contains serum, and an inferior interface of fat (above) and serum (below). The image has been
zone contains dependent red blood cells. A signal void, rotated 90 degrees to simulate the orientation of a cross-table
representing a chemical shift artifact, may be visible at radiograph. (A, Courtesy of R. Reinke, MD, Long Beach, Calif.
the interface of fat and serum. B, Courtesy of R. Stiles, MD, Atlanta, Ga.)
814 Section XV Traumatic Diseases
TRAUMA TO SYMPHYSES
Traumatic insult to symphyses, including the symphysis
pubis, manubriosternal joint, and intervertebral disc, is
not infrequent. Subluxation (i.e., diastasis) or dislocation
of the symphysis pubis leads to a single break in the
pelvic ring and is commonly combined with a second
injury causing pelvic disruption, such as fracture of the
ilium or sacrum or diastasis of the sacroiliac joint. Sub-
luxation or dislocation of the manubriosternal joint usu-
ally indicates significant trauma and may be seen after
automobile accidents in which the chest strikes the steer-
ing wheel. Similar displacement at this site occasionally
occurs spontaneously owing to the exaggerated thoracic
kyphosis associated with generalized osteoporosis, osteo-
malacia, renal osteodystrophy, or plasma cell myeloma.
Violation of the intervertebral disc may be combined with
fractures of the vertebral bodies and posterior elements, A B
leading to spinal instability. One injury that can lead to
disruption of both the intervertebral disc (plus surround- Figure 56–57. Cartilaginous nodes. A, Transaxial CT scan
ing bone) and the posterior spinal structures is the seat- reveals the typical appearance of a cartilaginous node. It is
belt fracture (Fig. 56–56). During an automobile accident, generally well defined, circular or lobulated, close to the
the trunk flexes over the seat belt. A horizontal fracture discovertebral junction, and accompanied by a rim of bone
sclerosis. B, Sagittal T1-weighted (TR/TE, 600/20) spin echo
of the vertebral body (Chance fracture) or tearing of the MR image shows the location and appearance of cartilaginous
intervertebral disc may be combined with laminar and nodes in the lumbar spine (arrows). (B, Courtesy of M. Solomon,
spinous process fractures or ligamentous tear. MD, San Jose, Calif.)
Trauma to the discovertebral junction can result from
obvious or occult injury. In either situation, violation of
the cartilaginous endplate and subchondral bone plate of
the vertebral body may allow intraosseous displacement cranial and caudal discs may be involved. The intraver-
of disc material (cartilaginous or Schmorl’s nodes). These tebral disc material may be associated with surrounding
nodes can result in an obvious compression fracture of osseous compression and reactive bone formation.
the vertebral body or subtle injury at the discovertebral Radiographs reveal one or more radiolucent areas, with
junction. Fracture of the cancellous bone of the vertebral bony sclerosis in the vertebral body that may be
body and disruption of the cartilaginous endplate allow combined with intervertebral disc space loss. CT and
disc material to enter the vertebral body. Typically, the MR imaging may clarify the nature of the abnormalities
cranial disc protrudes into the vertebra, although both (Fig. 56–57).
Another type of injury of the discovertebral junction
occurs at the site of attachment of the anulus fibrosus to
the rim of the vertebral body. In a developing skeleton,
this union is far more solid than that between the carti-
lage in the vertebral rim and the ossified portion of the
vertebral body. Thus, in a young patient, injury with
prolapse of the contiguous intervertebral disc can lead to
displacement of the ossified portion of the vertebral rim
as a result of separation of the osteocartilaginous junction
between the rim and the remaining vertebral body. This
injury may occur either anteriorly or posteriorly; the
latter is associated with displacement of a bony ridge into
the spinal canal (Fig. 56–58).
TRAUMA TO SYNCHONDROSES
(GROWTH PLATES)
Mechanism and Classification
The growth plate of an immature skeleton is especially
vulnerable to injury; approximately 6% to 15% of
fractures of the tubular bones of children younger than
Figure 56–56. Seat-belt injury. After an automobile accident, 16 years involve the growth plate and neighboring bone.
a transverse fracture of an upper lumbar vertebra is sometimes Forces that produce ligamentous tear or joint dislocation
associated with a transverse fracture through the pedicles and in adults may lead to growth plate injury in children and
laminae (arrows). adolescents because the joint capsule and ligamentous
Chapter 56 Physical Injury: Concepts and Terminology 815
Figure 56–58. Cartilaginous
nodes: anterior and posterior
prolapse. A, In this young child,
the radiograph shows a radio-
lucent area in the vertebral body
(arrow) resulting from a cartilagi-
nous node. This finding, in com-
bination with loss of height of
the intervertebral disc, simulates
infection. B, In a different patient,
transaxial CT shows the charac-
teristics of a limbus vertebra. A
radiolucent area is accompanied
by bone spicules anteriorly, which
represent a portion of the ossified
vertebral rim, as well as bone
A B sclerosis posteriorly (arrows). C
and D, Posterior prolapse leading
to intraspinal bone displacement
in an 18-year-old weight-lifter
with an acute onset of back and
leg pain. Lateral radiograph (C)
shows displacement of a portion
of the ring apophysis (arrow) into
the spinal canal. Transaxial CT
(D) reveals the location of the
displaced bone (arrow). (A,
Courtesy of A. D’Abreu, MD,
Porto Alegre, Brazil. B, Courtesy
of R. Yagan, MD, Cleveland,
Ohio. C and D, Courtesy of G.
Greenway, MD, Dallas, Tex.)
C D
structures are approximately two to five times stronger striations and growth recovery lines that are modified
than the cartilaginous plate. Four types of stress may in appearance according to the sites of arrested physeal
produce growth plate injury: shearing or avulsive forces growth. Large physeal bars, or bridges, may also be detect-
account for approximately 80% of injuries, and splitting ed (Fig. 56–59).
or compressive stresses account for the remainder. Sites Although several classification systems of growth plate
most typically affected are the phalanges; the distal tibial, injuries have been proposed, that of Salter and Harris is
fibular, ulnar, and radial growth plates; and the proximal accepted most widely. This original system separated the
humeral growth plate. Growth plate injuries may occur
acutely as a result of a single episode of trauma or chroni-
cally as a consequence of prolonged stress, particularly
that associated with athletics (e.g., gymnastics).
Metaphyseal failure is more common in sites where
the metaphysis is not protected from compression stress;
one example is the vertebral endplate, where a compres-
sion fracture can produce a cartilaginous node. Metaphy-
seal fragility is also accentuated by any condition asso-
ciated with osseous weakening, whether related to the
osteoclastosis of hyperparathyroidism or the hyper-
vascularity of the normal growth spurt. Avulsion injury
to the growth plate is commonly observed at sites of
apophyses. Examples include the lesser trochanter and
the medial epicondyle of the distal portion of the humerus.
The vulnerability of any specific apophysis to avulsion
injury is governed by its development and maturation Figure 56–59. Growth plate injury: physeal bar. An ossific
and depends on the time of appearance and fusion of the bridge (arrows) involving the medial aspect of the distal tibial
apophysis. Radiographically evident residua of growth physis resulted from a previous Salter-Harris type II injury to
disturbances related to previous physeal injury may be this physis (which was associated with a displaced distal fibular
observed. Such residua include transphyseal linear ossific fracture).
816 Section XV Traumatic Diseases
A B
C D
Figure 56–61. Growth plate injury: fracture types. A, Type I. Note the widening (arrows) of the growth
plate of the distal end of the femur. B, Type II. Observe the widening of the growth plate (arrow) and the
metaphyseal fracture (arrowhead). C, Type III. The epiphyseal fracture line (arrowhead) and growth plate
violation (arrow) can be recognized. D, Type IV. Observe the fracture line extending vertically through the
epiphysis and metaphysis (arrows).
mation external to the growth plate. The resultant involvement of the growth plate or metaphysis. Trans-
osseous bridge may act as a barrier to growth of the chondral fractures and osteochondritis dissecans are
adjacent portion of the plate, and progressive osseous examples of type VII injuries.
angulation may occur. Type VIII. This injury affects metaphyseal growth and
Type VII. This relatively common type of injury is asso- remodeling mechanisms in an immature skeleton,
ciated with epiphyseal alterations in the absence of primarily as a result of effects on the blood supply.
818 Section XV Traumatic Diseases
A B
Type IX. An injury to the periosteum of the diaphysis may, than girls, and black patients are affected more frequently
in rare circumstances, result in disruption of normal than whites; overweight children have an especially high
diaphyseal growth and remodeling. Segmental com- occurrence. About 20% to 35% of patients with slipped
minuted fractures, wringer injuries, and severe burns capital femoral epiphysis have bilateral involvement,
are examples of the type of trauma that can lead to this which is more frequent in girls. A variety of contributing
kind of injury. factors have been emphasized in the pathogenesis of this
Some degree of growth deformity develops in injury:
approximately 25% to 30% of patients with growth plate • Trauma. Although trauma is an important precipitating
injuries, and in 10% of patients, this deformity is signifi- event in infants and young children, it appears to be
cant. The prognosis is related to the age of the patient, only a minor factor in older persons. Less than 50% of
the anatomy of the vascular supply to the region, the type patients have a history of significant injury.
of injury, and the immediacy and adequacy of the reduc- • Adolescent growth spurt. The association between slipped
tion. In general, the younger the patient at the time of capital femoral epiphysis and the adolescent growth
injury, the poorer the prognosis for residual deformity. spurt involves the relatively wide physis during this
Type I, II, and III injuries have a relatively good prog- period and its change in configuration from a horizontal
nosis, whereas type IV injuries carry a guarded prognosis to an oblique plane, with increased shearing stress.
and type V and VI injuries have a poor prognosis. Late • Hormonal influences. The list of endocrine diseases asso-
sequelae include growth impairment, premature growth ciated with this femoral disorder includes hypothy-
plate fusion, epiphyseal malposition and rotation, and roidism, hypoestrogenic states, acromegaly, gigantism,
osteonecrosis. cryptorchidism, and pituitary and parathyroid tumors.
Premature partial arrest of growth is produced by a Despite these observations, no clear evidence exists
bridge of bone, or bone bar, that extends from the meta- that levels of growth hormone are abnormal in patients
physis to the epiphysis across a portion of the physis. with slipped capital femoral epiphysis.
Continued growth of the remaining portion of the physis
results in increasing angular deformity. Type IV injuries • Weight and activity. One of the most striking charac-
characteristically lead to closure of a portion of the physis; teristics of patients with slipped capital femoral epi-
type V injuries may result in premature closure of the physis is a tendency to be overweight. Obesity
entire growth plate. MR imaging allows accurate assess- increases the shearing stress on the growth plate and
ment of the extent of the initial injury (Fig. 56–62), as can lead to slippage even during usual activity. The
well as the extent of physeal arrest (Fig. 56–63). propensity for epiphyseal slippage appears to be greater
in physically active adolescents than in those who are
Specific Injuries less active.
The femoral head seated in the acetabulum is located
Slipped Capital Femoral Epiphysis
in a posterior and medial direction with respect to the
Slippage of the capital femoral epiphysis is typically remainder of the femur. By convention, reference is
observed between the ages of 10 and 17 years in boys and made to the movement of the femoral head with respect
8 and 15 years in girls. Boys are affected more frequently to the shaft in cases of slipped capital femoral epiphysis.
Chapter 56 Physical Injury: Concepts and Terminology 819
A B
Figure 56–63. Growth plate injury. A, Routine radiograph shows widening of the medial portion of the
proximal tibial physis, suggestive of a Salter-Harris type I injury. B, Coronal T2-weighted (TR/TE,
2000/80) spin echo MR image reveals high signal intensity (arrows) in the tibia, consistent with a Salter-
Harris type IV injury; in a bone bruise in the lateral portion of the distal femoral epiphysis; and in and about
the medial collateral ligament, indicative of ligamentous disruption and soft tissue edema. An effusion is
present. (Courtesy of A. Newberg, MD, Boston, Mass.)
Although posteromedioinferior slippage of the capital tosis of apposing osseous margins. Some joint space
femoral epiphysis is typical, other directions can be seen. recovery may be seen after a period of months in approxi-
Radiographic analysis remains essential to the diagnosis mately one third of patients. Its cause is unknown.
of slipped capital femoral epiphysis. Both anteroposterior
and frog-leg or lateral projections are mandatory; abnor-
Growth Plate Injuries about the Knee
malities on the frontal projection alone may be subtle,
even in the presence of significant epiphyseal displace- Growth plate trauma in the distal portion of the femur
ment. Comparison radiographs of the opposite side can may be related, in many cases, to birth or to athletic or
be very useful. On the anteroposterior view, osteoporosis automobile injuries. Examples include the wagon-wheel
of both the femoral head and the femoral neck is com- fracture resulting when children catch their legs between
mon (Fig. 56–64). The margin of the metaphysis may the spokes of wagon or bicycle wheels and the clipping
seem blurred or indistinct, and the growth plate may injury of adolescent football players. Salter-Harris type II
appear widened. A tangential line along the lateral border and III injuries are especially common. Injury to the
of the femoral neck may fail to intersect any part of the proximal tibial physis is relatively rare.
epiphysis or may cross only a small portion of it. CT
scanning and MR imaging appear to be more sensitive
Growth Plate Injuries about the Ankle
for the early diagnosis of this condition at a time when
routine (and specialized) radiographs are normal. In Injuries to the growth plate in the distal portion of the
chronic stages of slipped capital femoral epiphysis, tibia are common. Type II injury is most frequent,
routine radiography shows reactive bone formation along followed in order of decreasing frequency by type III, IV,
the medial and posterior portions of the femoral neck, a and I lesions. Ten percent to 12% of physeal injuries at
buttressing phenomenon similar to that which occurs in this site are followed by growth disturbance.
degenerative joint disease. Triplane fractures of the distal portion of the tibia
Sequelae of slipped capital femoral epiphysis include represent approximately 5% to 10% of all injuries in this
severe varus deformity, shortening and broadening of the location. The primary mechanism of injury appears to be
femoral neck, osteonecrosis, chondrolysis, and degenera- external rotation of the foot, although plantar flexion has
tive joint disease. Osteonecrosis has been described in also been suggested. The resulting injury has several vari-
6% to 15% of patients with this disorder. Chondrolysis ations, including a two-plane fracture pattern (Tillaux or
may be observed in as many as 40% of patients with epi- Kleiger fracture) that involves only the epiphysis, and a
physeal slippage and is more frequent in black than white three-plane fracture pattern in which an additional meta-
patients, in women than men, and in persons with severe physeal fracture is present (Fig. 56–65). Radiographi-
slippage. It usually occurs within 1 year of the slippage cally, a triplane fracture resembles two different types of
and may be evident in untreated or treated persons. Salter-Harris injury (a type III lesion on the antero-
Radiographs outline osteoporosis, concentric narrowing posterior radiograph and a type II lesion on the lateral
of the interosseous space, and eburnation and osteophy- radiograph), although in reality, it is a variation of a type
820 Section XV Traumatic Diseases
A B
C
Figure 56–64. Slipped capital femoral epiphysis: radiographic abnormalities. A, Anteroposterior radiograph
shows subtle findings, including mild osteoporosis of the proximal portion of the femur and an indistinct
metaphyseal margin. B, In the frog-leg view, the degree of posterior slippage is readily apparent. Note the
widened growth plate. C, Transaxial CT scan in a different patient reveals that the femoral head is displaced
posteriorly with respect to the femoral neck. D, In a third patient, coronal intermediate-weighted (TR/TE,
2000/20) spin echo MR images reveal posteromedial displacement of the right proximal femoral epiphysis.
A T2-weighted image confirmed the presence of a joint effusion and periphyseal marrow edema. (C, From
Busch MT, Morrissy RT: Slipped capital femoral epiphysis. Orthop Clin North Am 18:637, 1987. D,
Courtesy of A. Vieira, MD, Porto, Portugal.)
IV injury pattern (Fig. 56–66). Two, three, or four frag- lateral extension of the fracture through the open physis
ments may result, with two fragments being most com- (Fig. 56–67).
mon. Because of the complexity of the injury, CT is an
excellent technique for delineating the site and extent of
Growth Plate Injuries about the Shoulder
involvement.
An isolated vertical fracture of the distal tibial epiphy- Disruption of the epiphysis and physis in the proximal
sis occurs in the adolescent age group. This injury, often portion of the humerus is relatively infrequent. Its occur-
designated the juvenile fracture of Tillaux, classically rence in adolescent baseball pitchers as an epiphysi-
involves the lateral portion of the distal tibial epiphysis olysis is termed the Little League shoulder syndrome.
and conforms to a Salter-Harris type III injury because of Separation of the proximal humeral epiphysis may result
Chapter 56 Physical Injury: Concepts and Terminology 821
from an injury at birth, particularly during a difficult
delivery.
The epiphysis of the inner margin of the clavicle
ossifies at approximately 18 to 20 years of age and, with
closure of the growth plate, merges with the shaft of the
clavicle at approximately 25 years of age. Injury to the
medial end of the clavicle in an immature skeleton can
produce an epiphyseal separation that may be misdiag-
nosed as sternoclavicular joint dislocation. Physeal injuries
about the shoulder may account for pseudodislocations
A B of the acromioclavicular joint in children. Such injuries
include displacement of the distal portion of the clavicle
from its periosteal sleeve and avulsion of the epiphysis of
the coracoid process.
A B
822 Section XV Traumatic Diseases
A B
the middle third of the capitular ossification center. In placed on the flexor pronator tendon that attaches to this
the presence of supracondylar fractures of the humerus site; this represents approximately 10% of all elbow
(the most common fracture about the elbow in children), injuries. In some instances, the epicondyle may become
posterior displacement or angulation of the distal frag- entrapped within the joint (Fig. 56–69). In this situation,
ment allows the anterior humeral line to pass through the displaced epicondylar ossification center can simulate
the anterior third of the ossification center or even ante- a normal trochlear center; however, the appearance of a
rior to the capitulum (Fig. 56–68). Because of absent trochlear center without a medial epicondylar center is
or incomplete ossification of the developing centers inconsistent with the normal sequence of ossification
about the elbow in infants and children, complete assess- about the distal portion of the humerus.
ment of injuries may not be possible with conventional Separation, with or without fracture, of the entire
radiography. distal humeral epiphysis may be mistaken for fracture of
Fracture of the lateral condyle is frequent and repre- the lateral humeral condyle or dislocation of the elbow
sents a Salter-Harris type IV injury. The fracture line (Fig. 56–70). In most cases, a Salter-Harris type I or II
splits the epiphysis and separates a portion of the adja- injury is present. Radiographs usually reveal normal align-
cent metaphysis and the capitulum. Separation of the ment of the radial shaft and capitellar ossification center,
medial epicondyle ossification center is a result of stress normal alignment of the radius and ulna, and malalign-
ment of these bones with the humerus.
A B
Figure 56–70. Growth plate injury of the distal portion of the humerus. Although an anteroposterior
radiograph (A) appears to delineate dislocation of the elbow, with medial displacement of the ulna and
radius, a lateral radiograph (B) identifies the metaphyseal ossific flake (arrow) and normal alignment of the
radius and capitulum, findings indicating that separation of the distal humeral growth plate has occurred.
TABLE 56–5 portions of the radius and ulna, proximal portion of the
humerus, distal aspect of the femur, and distal end of the
Relative Frequency of Physeal Injuries tibia are affected most commonly. The general radio-
graphic abnormalities accompanying the chronic stress
Typical Age are similar in each of these locations. Part or all of the
Frequency Age* Range
(%) (Yr) (Yr) physis appears widened and irregular, with varying degrees
of accompanying sclerosis in the adjacent metaphysis
Distal portion of radius 49.9 9–14 (Fig. 56–71). Superficially, these radiographic abnormalities
Distal portion of humerus† 16.7 Birth–5‡ resemble those occurring in rickets, hypophosphatasia,
6§ 3–10 or metaphyseal dysplasia. In some cases, a unilateral or
Distal portion of tibia 11.0 12 8–13 asymmetrical distribution of changes provides an impor-
Distal portion of fibula 9.1 tant diagnostic clue.
Distal portion of ulna 5.7
Proximal portion of radius 4.2 9–10 8–13
TRAUMA TO SUPPORTING STRUCTURES,
Proximal portion of humerus 3.1 14–15 10–16
SYNDESMOSES, AND ENTHESES
Distal portion of femur 1.2 11–12 10–15
Proximal portion of ulna 0.7 Tendon and Ligament Injury and Healing
Proximal portion of tibia 0.5 13–15 In most regions of the body, tendons are subjected to
Proximal portion of femur 0.1 2–6 axial loading. Acute failure of a musculotendinous system
12–15 may occur at the junction between muscle and tendon or
Other 0.8 at the osseous site of tendinous attachment; the latter
sometimes results in an avulsion fracture (e.g., mallet
*Girls, because of earlier skeletal maturity and advanced skeletal age relative to finger). Failure within the tendon itself is rare unless
boys, have injuries at an average age 1 to 2 years younger than boys. preexisting tendon degeneration is present. Tendon tears
†The majority of these fractures are lateral condyle lesions. or ruptures can appear at virtually any site in the body.
‡Distal humeral fracture-separations. Typical examples are injuries to the tendons in the hands
§Lateral humeral condyle fracture-separations. and feet and to the patellar, triceps, peroneal, quadriceps,
From Shapiro F: Epiphyseal growth plate fracture-separations: A rotator cuff, and Achilles tendons. In many cases, a
pathophysiologic approach. Orthopedics 5:720, 1982.
significant traumatic event initiates the tendon injury,
although spontaneous rupture has been documented,
especially in patients with rheumatoid arthritis and
Chronic Stress Injuries systemic lupus erythematosus and in those receiving local
A variety of musculoskeletal manifestations related to the corticosteroid injections. The gold standard for the
chronic application of stress can occur in professional evaluation of tendons is MR imaging, and its role is
and recreational athletes. Growth plates in the distal discussed in detail in Chapter 59.
824 Section XV Traumatic Diseases
A B
Figure 56–72. Avulsion injury: cruciate ligament avulsion. A, Observe the posterior bony fragment
(arrow) from an old posterior cruciate ligament injury. B, Sagittal intermediate-weighted (TR/TE, 1000/40)
spin echo MR image shows a tibial avulsion fracture (arrow) related to the site of attachment of the posterior
cruciate ligament (arrowhead).
Chapter 56 Physical Injury: Concepts and Terminology 825
A B
Figure 56–73. Avulsion injury: pelvis. A, Anterior inferior iliac spine. The avulsed fragment (arrow) is
related to stress at the origin of the rectus femoris muscle. B, Symphysis pubis and inferior pubic ramus. The
osseous irregularity may result from avulsion injuries caused by stress in the adductor brevis, adductor
longus, and gracilis muscles. (B, From Schneider R, Kaye JJ, Ghelman B: Adductor avulsive injuries near the
symphysis pubis. Radiology 120:567, 1976.)
with abrupt directional change during running; and avul- of injuries that can affect skeletal muscle. Skeletal muscles
sion injuries near the symphysis pubis related to adductor do not act independently. Rather, they transmit their
muscle (adductor longus, adductor brevis, gracilis) inser- contractile forces to adjacent structures through tendi-
tion sites (see Fig. 56–73B). Follow-up radiographs may nous attachments. The junctional region between muscle
show considerable new bone formation or healing with fibers and the tendinous attachment is termed the myo-
incorporation of the fragment into the parent bone, which tendinous, or musculotendinous, junction. Many, if not
in some cases is associated with bizarre skeletal overgrowth. most, of the injuries to muscle occur at the myotendinous
junction.
Diastasis
The term diastasis implies a separation of normally joined Muscle Anatomy and Physiology
bony elements; it is frequently applied to syndesmoses Most muscles show a mixture of two types of fibers: type
and, specifically, to injuries of the ligaments that extend I, or slow, fibers, and type II, or fast, fibers. Histo-
between the lower portions of the tibia and fibula. Com- chemical differences are used to distinguish between
plete or partial diastasis can occur, depending on the these types of fibers. Type I fibers are better suited to a
extent of damage to the tibiofibular and interosseous relatively slow but repetitive type of contraction, such as
ligaments. Radiographs may reveal abnormal separation the tonic forces characteristic of postural muscles, and
of the tibia and fibula in which the space (la ligne claire) are more resistant to fatigue than type II fibers are.
between the medial cortex of the fibula and the posterior Hence, type I fibers are involved more in endurance
edge of the peroneal groove is greater than 5 to 5.5 mm activities than in those requiring speed and strength of
on an anteroposterior radiograph. Soft tissue ossification short duration. Type II fibers, in contrast, are adapted to
and even ankylosis between the tibia and the fibula may produce rapid phasic forces that are operational in large-
subsequently occur. The term diastasis is likewise applied scale movements of the human body. Type I fibers have
to separation of apposing bone surfaces about symphyses been designated slow-twitch fibers and type II as fast-
(e.g., symphysis pubis). twitch fibers.
frequent development of compartment syndromes after exercise-related, or exertional, muscle injury and show
trauma. Skeletal muscle is capable of limited regenera- several typical characteristics. First, such muscles com-
tion. With extensive muscle damage, however, such regen- monly perform eccentric actions (e.g., hamstring muscles).
eration is not possible, and connective tissue is formed to Second, the muscles commonly act across two joints
replace the injured muscle, with resultant loss of func- (e.g., biceps brachii and gastrocnemius muscles). Such
tion. In certain situations, such as after laceration, healing muscles often limit the range of joint motion because of
of skeletal muscle is associated with extensive scarring their intrinsic tightness. For example, when the knee is
and ossification. extended, the gastrocnemius muscle restricts dorsiflexion
of the ankle, and when the hip is flexed, the hamstring
muscles limit extension of the knee. Third, muscles that
Direct Muscle Injury
have type II, or fast, fibers and are involved in activities
Muscle Laceration. Muscle lacerations result from pene- requiring sudden acceleration or deceleration are typi-
trating injuries. The typical healing response results in cally affected (e.g., hip flexors and adductors, rectus
the formation of a scar composed of dense connective femoris muscle).
tissue. MR imaging in cases of lacerated muscle is charac- The precise MR imaging findings are influenced by
terized by a defect, often transverse, with interruption of the severity of the strain and its duration. Minor, or grade
the continuity of the muscle and changes in signal inten- 1, strains produce MR imaging findings similar to those
sity reflecting the presence of blood and edema. of a muscle contusion. With moderate, or grade 2, muscle
strains, a focal masslike lesion or a stellate defect, or both,
Muscle Contusion. Compression of a muscle from direct may be observed. The signal intensity of the involved
trauma leads to a contusion. The term charley horse is muscle reflects the presence of edema or blood. With
sometimes used to describe the injury. The gastrocnemius severe, or grade 3, muscle strains, discontinuity of the
and quadriceps muscles are typical sites of involvement. muscle is evident, and blood collects between the torn
Injury results in capillary rupture and interstitial hemor- edges (Fig. 56–75).
rhage (bleeding between the fibers of the damaged con- Delayed-onset muscle soreness represents another
nective tissue), followed by edema and an inflammatory response to physical exertion. Clinical findings consisting
mass. Contusions vary in severity, and the muscle is still of muscle soreness and tenderness to palpation occur
able to function, even in severe cases. within 1 to several days after muscle exertion. The sore-
There are four basic MR imaging characteristics of a ness is initially most evident at the musculotendinous
muscle contusion: (1) the affected muscle is usually slightly junction and later becomes more diffuse. MR imaging
increased in girth; (2) edema leads to an increase in signal findings resemble those of grade 1 muscle strains.
intensity on T2-weighted spin echo and STIR images;
(3) a feathery, interstitial pattern is evident that relates to
Muscle Herniation
the dispersion of inflammatory fluid within and between
the muscle fibers; and (4) no evidence of disruption of Focal herniation of muscle through a defect in its enclosing
muscle fibers is present (Fig. 56–74). fascia may occur as a complication of local blunt trauma
or as a result of muscle hypertrophy. Typically, the lower
part of the leg, especially the anterior tibial compart-
Indirect Muscle Injury
ment, is involved, and the tibialis anterior muscle is
Indirect injuries to muscle occur from overzealous use affected most frequently. MR imaging is an effective
during physical exercise. Certain muscles are prone to diagnostic technique when such herniation is suspected.
A B
Chapter 56 Physical Injury: Concepts and Terminology 827
A C
Figure 56–75. Skeletal muscle injury in three different patients. A, Grade 1 strain. Transaxial T2-weighted
(TR/TE, 2000/80) spin echo MR image reveals increased signal intensity of the gastrocnemius muscle
(arrows) and a perifascial fluid collection (arrowheads). B, Grade 2 strain. On a transaxial T2-weighted
(TR/TE, 2000/80) spin echo MR image, note that the biceps femoris muscle demonstrates edema through-
out its substance (arrowheads), with several small focal areas that may represent limited interruption of
muscle fibers (small arrow). The perifascial edema (large arrows) is striking. C, Grade 3 strain—ruptured right
pectoralis muscle. Transaxial T2-weighted (TR/TE, 2000/80) spin echo MR image shows the distortion of
muscle anatomy, with localized edema and a focal hematoma (arrow) of high signal intensity. (B, From
Deutsch AL, Mink JH, Kerr R: MRI of the Foot and Ankle. New York, Raven, 1992. C, From Fleckenstein
JL, Shellock FG: Exertional muscle injuries: Magnetic resonance imaging evaluation. Top Magn Reson
Imaging 3:50, 1991.)
A B
A B C
Figure 56–77. Abused child syndrome: radiographic abnormalities. A, Periostitis (arrow) is a delayed
radiographic sign of trauma. B, Metaphyseal irregularity and corner fractures (arrowheads) are more imme-
diate radiographic clues to child abuse. C, Rib fractures (arrows) are frequent in an abused child. (Courtesy
of D. Edwards, MD, San Diego, Calif.)
FURTHER READING John SD, Moorthy CS, Swischuk LE: Expanding the concept of
the toddler’s fracture. Radiographics 17:367, 1997.
Berquist TH: Imaging of Orthopedic Trauma, 2nd ed. New Kleinman PK, Raptopoulos VD, Brill PW: Occult nonskeletal
York, Raven, 1991. trauma in the battered-child syndrome. Radiology 141:393,
Cone RO III, Nguyen V, Flournoy JG, et al: Triplane fracture 1981.
of the distal tibial epiphysis: Radiographic and CT studies. Mammone JF, Schweitzer ME: MRI of occult sacral insufficiency
Radiology 153:763, 1984. fractures following radiotherapy. Skeletal Radiol 24:101, 1995.
De Smet AA, Fisher DR, Graf BK, et al: Osteochondritis Milgram JW: The development of loose bodies in human joints.
dissecans of the knee: Value of MR imaging in determining Clin Orthop 124:292, 1977.
lesion stability and the presence of articular cartilage defects. Mink JH, Deutsch AL: Occult cartilage and bone injuries of the
AJR 155:549, 1990. knee: Detection, classification, and assessment with MR
Deutsch AL, Mink JH: Magnetic resonance imaging of imaging. Radiology 170:823, 1989.
musculoskeletal injuries. Radiol Clin North Am 27:983, Murray RO, Duncan C: Athletic activity in adolescence as an
1989. etiological factor in degenerative hip disease. J Bone Joint
Evans GFF, Haller RG, Wyrick PS, et al: Submaximal delayed- Surg Br 53:406, 1971.
onset muscle soreness: Correlations between MR imaging Neer CS II: Displaced proximal humeral fractures. Part 1. Classi-
findings and clinical measures. Radiology 208:815, 1998. fication and evaluation. J Bone Joint Surg Am 52:1077,
Fleckenstein JL, Weatherall PT, Parkey RW, et al: Sports-related 1970.
muscle injuries: Evaluation with MR imaging. Radiology Pfirrmann CWA, Resnick D: Schmorl nodes of the thoracic
172:793, 1989. and lumbar spine: Radiographic-pathologic study of preva-
Hagglund G, Hansson LI, Ordeberg G: Epidemiology of lence, characterization, and correlation with degenerative
slipped capital femoral epiphysis in southern Sweden. Clin changes of 1,650 spinal levels in 100 cadavers. Radiology
Orthop 191:82, 1984. 219:368, 2001.
Harrington IJ: Biomechanics of joint injuries. In ER Gozna, IJ, Quinn SF, McCarthy JL: Prospective evaluation of patients
Harrington (eds): Biomechanics of Musculoskeletal Injury. with suspected hip fracture and indeterminate radiographs:
Baltimore, Williams & Wilkins, 1982, p 31. Use of T1-weighted MR images. Radiology 187:469, 1993.
Harris JH, Harris WH: The Radiology of Emergency Medicine. Resnick D, Niwayama G: Intravertebral disk herniations:
Baltimore, Williams & Wilkins, 1975. Cartilaginous (Schmorl’s) nodes. Radiology 126:57, 1978.
830 Section XV Traumatic Diseases
Rockwood CA, Green DP: Fractures in Adults, 2nd ed. Salter RB: Injuries of the ankle in children. Orthop Clin North
Philadelphia, JB Lippincott, 1984. Am 5:147, 1974.
Rogers LF, Poznanski AK: Imaging of epiphyseal injuries. Shapiro F: Epiphyseal growth plate fracture-separations: A
Radiology 191:297, 1994. pathophysiologic approach. Orthopedics 5:720, 1982.
Rogers LF: Radiology of Skeletal Trauma, 2nd ed. New York, Stevens MA, El-Khoury GY, Kathol MH, et al: Imaging
Churchill Livingstone, 1992. features of avulsion injuries. Radiographics 19:655, 1999.
Rorabeck CH: Compartment syndromes. In BD Browner, JB Steinbach LS, Fleckenstein JL, Mink JH: MR imaging of
Jupiter, AM Levine, et al (eds): Skeletal Trauma. Fractures, muscle injuries. Semin Musculoskel Radiol 1:127, 1997.
Dislocations, Ligamentous Injuries. Philadelphia, WB White PG, Mah JY, Friedman L: Magnetic resonance imaging
Saunders, 1992, p 285. in acute physeal injuries. Skeletal Radiol 23:627, 1994.
Salter RB, Harris WR: Injuries involving the epiphyseal plate.
J Bone Joint Surg Am 45:587, 1963.
Salter RB, Harris WR: Injuries of the growth plate. In M Rang
(Ed): The Growth Plate and Its Disorders. London, E & S
Livingstone, 1969, p 132.
57
CHAPTER 57
Physical Injury: Extraspinal Sites A second type of injury accompanying anterior dislo-
cation of the humeral head involves the glenoid fossa and
is called a Bankart lesion (Fig. 57–4). Although large
osseous fractures of the glenoid are occasionally apparent,
SUMMARY OF KEY FEATURES the fracture may include only the cartilaginous surface
Routine radiographic findings are emphasized in this of the bone. Conventional magnetic resonance (MR)
chapter, although reference is made to the assessment imaging or MR arthrography are the methods of choice
of such injuries with other methods such as computed for detecting alterations in the glenoid labrum (see
tomography and magnetic resonance imaging. Both Chapter 59).
these techniques are fundamental to the evaluation of A number of other osseous and nonosseous injuries
internal derangements of joints. can accompany anterior dislocations of the glenohumeral
joint. An avulsion fracture of the greater tuberosity of the
humerus occurs in 10% to 15% of cases. Disruption of
the rotator cuff may complicate anterior dislocation of
INTRODUCTION the glenohumeral joint, particularly in patients who are
older than 40 years. Injuries to the brachial plexus occur
This survey of physical injuries at extraspinal sites begins in 7% to 45% of anterior glenohumeral joint disloca-
in the upper extremity, proceeding in a proximal to distal tions; such injuries are more common in older patients
direction; then considers the lower extremity, again in a and those with large hematomas. Approximately 40% of
proximal to distal direction; and finally extends to the anterior glenohumeral joint dislocations are recurrent.
bony pelvis and thoracic cage. It is not meant to compete Recurrent dislocations are more likely in cases of sub-
with standard references but rather to provide an over- coracoid and subglenoid dislocations and in younger
view of the more important physical injuries occurring in persons. Many types of surgical procedures have been
extraspinal sites. A discussion of injuries to the cranium advocated for the treatment of patients with recurrent
and facial bones is beyond the scope of this chapter. anterior glenohumeral joint dislocations, some of which
are listed in Table 57–1.
UPPER EXTREMITY Posterior dislocation of the glenohumeral joint is rare
and accounts for approximately 2% to 4% of all shoulder
Shoulder dislocations. More than 50% of these cases are unrecog-
Injuries about the shoulder are among the most com- nized on initial evaluation, despite the presence of a history
monly encountered traumatic abnormalities, although of trauma, pain, swelling, and limitation of motion. Many
their precise patterns vary according to the person’s age. cases of posterior dislocation result from convulsions,
and in these instances, bilateral dislocations may be evi-
dent. Physical examination reveals a posteriorly displaced
Glenohumeral Joint Dislocation humeral head that is held in internal rotation. Absence of
Even under normal circumstances, the glenohumeral joint external rotation and limitation of abduction are present
is relatively unstable, and this instabilty results in a spec- in virtually all cases. Associated injuries include stretch-
trum of abnormalities. Anterior dislocation accounts for ing of the posterior capsule, fracture of the posterior
more than 95% of such injuries. Anterior dislocations are aspect of the glenoid rim, avulsion fracture of the lesser
further classified as subcoracoid (the most common type), tuberosity of the humerus, and stretched or detached
subglenoid (second in frequency), and subclavicular and subscapularis tendon.
intrathoracic (rare) (Fig. 57–1). Intrathoracic dislocations On an anteroposterior radiograph, posterior disloca-
are generally accompanied by fractures of the proximal tion of the humeral head distorts the normal elliptic radio-
portion of the humerus. Anterior dislocations are best dense area created by overlapping of the head and the
evaluated radiographically by the inclusion of a lateral glenoid fossa (Fig. 57–5). An empty, or vacant, glenoid
scapular projection, an axillary projection, or both, in addi- cavity is a second radiographic sign of dislocation in this
tion to standard frontal views of the shoulder (Fig. 57–2). projection; posterior displacement of the humeral head
Anterior dislocations are associated with a compres- may create a space between the anterior rim of the glenoid
sion fracture on the posterolateral aspect of the humeral and the humeral head that is frequently greater than 6 mm.
head that is produced by impaction of the humerus In addition, the normal parallel pattern of the articular
against the anterior rim of the glenoid fossa. This osseous surfaces of the glenoid concavity and the humeral head
defect of the humerus is called a Hill-Sachs lesion (see Fig. convexity is lost. Other radiographic signs of posterior
57–2). This lesion is observed in many cases of anterior dislocation on frontal radiographs include a fixed posi-
dislocation of the glenohumeral joint. Films obtained in tion of internal rotation of the humerus and a second
various degrees of internal rotation are mandatory because cortical line, the trough line, parallel and lateral to the
such rotation of the humerus produces a tangential view subchondral articular surface of the humeral head. This
of the osseous lesion (Fig. 57–3). line represents the margin of a troughlike impaction frac-
831
832 Section XV Traumatic Diseases
A B
A B
Figure 57–2. Glenohumeral joint: anterior dislocation. A, Tangential scapular projection reveals the
abnormal position of the humeral head, which is located in front of the glenoid cavity. B, In a different
patient, axillary radiograph reveals an anterior dislocation of the humeral head. Impaction of the anterior
glenoid process and the posterolateral aspect of the humeral head has produced a Hill-Sachs lesion
(arrowhead). Although the dislocation is well shown on this radiograph, axillary projections are difficult to
obtain while the humeral head is still displaced.
ture of the humeral head that is created when this struc- lature, as well as fracture of the acromion, clavicle, coracoid
ture contacts the posterior glenoid rim during dislocation process, or humeral tuberosities. Inferior dislocation of
(Fig. 57–6). As such, it is analogous to the Hill-Sachs the glenohumeral joint (luxatio erecta) is also rare (Fig.
lesion seen in association with anterior glenohumeral joint 57–9). A direct axial force on a fully abducted arm or a
dislocation and is diagnostic of a posterior dislocation. hyperabduction force leading to leverage of the humeral
Computed tomography (CT) is an additional imaging head across the acromion is responsible for this type of
technique that can be used to evaluate patients with acute dislocation. After this injury, the superior aspect of the
(or chronic) posterior dislocations of the glenohumeral articular surface of the humeral head is directed infe-
joint (Fig. 57–7). riorly and does not contact the inferior glenoid rim; as a
Superior dislocation of the glenohumeral joint is rare result, the arm is held over the patient’s head.
(Fig. 57–8). An extreme forward and upward force on an A special type of inferior displacement of the humeral
adducted arm can produce extensive damage to the rota- head is termed the drooping shoulder (Fig. 57–10). It can
tor cuff, capsule, biceps tendon, and surrounding muscu- be associated with uncomplicated fractures of the surgical
Chapter 57 Physical Injury: Extraspinal Sites 833
A B
Figure 57–4. Glenohumeral joint: Bankart lesion. A, In addition to a Hill-Sachs lesion (white arrow), note
the fragmentation of the glenoid rim (black arrow), representing an osseous Bankart lesion. B, Three-
dimensional CT of the shoulder with the humerus electronically removed shows the osseous Bankart lesion
(arrow); the fragment is displaced medially from the anterior inferior glenoid.
834 Section XV Traumatic Diseases
TABLE 57–1
Surgical Procedures Used for Recurrent Anterior Glenohumeral Joint Dislocation
Procedure Technique
Bankart Repair of anterior capsular mechanism with drill holes and sutures
Putti-Platt Shortening of anterior capsule and subscapularis muscle
Magnuson-Stack Transfer of subscapularis tendon from lesser tuberosity to greater tuberosity
Eden-Hybbinette Bone graft to anterior glenoid region
Oudard Bone graft to coracoid process
Trillat Osteotomy with displacement of coracoid process
Bristow-Helfet Transfer of coracoid process with its attached tendons to neck of scapula
A B
TABLE 57–2
Acromioclavicular Injuries
A B
Figure 57–14. Sternoclavicular joint: posterior dislocation. A, Frontal chest radiograph shows asymmetry
in the position of the medial margins of the clavicle, with the right clavicle (on the injured side) being located
inferior to the left clavicle. B, Transverse CT scan confirms posterior dislocation of the right sternoclavicular
joint.
Delayed union or nonunion can occur with any type portion) of the coracoclavicular ligaments is severed (Fig.
of fracture of the proximal portion of the humerus and 57–20). A characteristic medial flange of bone extending
may be accompanied by significant angulation at the from the distal clavicular fragment may be evident in type
fracture site. Osteonecrosis is reported in 7% to 50% of II fractures. Type II fractures have a poorer prognosis
cases and is most typical with displaced fractures and related to more significant displacement at the fracture
severe fractures or fracture-dislocations of the bone (four- site and to nonunion. Associated fractures of the coracoid
part fractures) (Fig. 57–18). Less commonly, injury to the process and ribs may be evident.
nearby brachial plexus and the axillary artery may be Direct trauma accounts for most fractures of the
seen. A brachial plexus injury, which results from contu- medial end of the clavicle. They have been divided into
sion or mild traction, may be self-limited. transverse fractures, which do not become displaced
because of ligamentous and musculature attachments,
and intra-articular fractures. Intra-articular fractures may
Fracture of the Clavicle
be overlooked unless conventional tomography or CT
It is convenient to divide the clavicle into three functional scanning is used.
segments: a distal, or interligamentous, segment consist- In children, clavicular fractures heal rapidly without
ing of the outer 25% to 30% of the bone—the region significant sequelae. In adults, exuberant callus affecting
about and distal to the coracoclavicular ligament; an inter- the middle segment of the clavicle can be associated with
mediate segment consisting of the middle 40% to 50% of persistent neurologic defects and circulatory disturbances
the bone; and an inner segment consisting of the medial because of compression of the adjacent structures. Non-
25% of the bone. Approximately 75% to 80% of clavic- union of a clavicular fracture is uncommon and, when
ular fractures involve the middle segment of the bone, present, is associated with lack of immobilization. Post-
15% to 20% involve the distal segment, and 5% affect traumatic pseudarthrosis of the clavicle must be differ-
the inner segment. The prognosis of clavicular fractures entiated from congenital pseudarthrosis. Congenital
depends on the precise site of involvement; for example, pseudarthrosis of the clavicle usually manifests as a pain-
nonunion is frequent in fractures distal to the coraco- less swelling overlying the middle third of the bone, more
clavicular ligament, whereas this complication is rare in frequently on the right side. These patients are infants,
fractures of the medial segment. and callus and periosteal bone formation are absent.
The middle segment, located at the junction of the
two curvatures of the clavicle, is the most common site of
fracture. In adults and children, the mechanism of injury Fracture of the Scapula
is usually a fall onto an outstretched hand or a fall on the Scapular fractures are infrequent. Although they may occur
shoulder. In most cases, the radiographic findings permit as an isolated phenomenon, as many as 95% of cases are
a prompt and accurate diagnosis (Fig. 57–19). Fractures associated with additional injuries. Scapular fractures
of the distal portion of the clavicle, related to a force may involve one or more of the following anatomic regions:
applied to the shoulder that drives the humerus and glenoid fossa and articular surface, neck, body, spinous
scapula downward, are divided into two types: type I process, acromion process, and coracoid process (Fig.
fractures, in which the coracoclavicular ligaments are 57–21). They are found most frequently in the scapular
intact, and type II fractures, in which a portion (conoid body, followed by the neck and other regions of the bone.
Chapter 57 Physical Injury: Extraspinal Sites 839
A fracture of the rim of the glenoid cavity occurs (in posterior dislocations) may be affected. Larger portions
in approximately 20% of traumatic glenohumeral joint of the glenoid fossa may be fractured when the humeral
dislocations. The fracture fragment may be cartilaginous head is driven against the glenoid cavity by a direct force.
or osteocartilaginous in nature. Either the anterior glenoid A fracture in the neck of the scapula characteristically
rim (in anterior dislocations) or the posterior glenoid rim occurs after a direct blow to the shoulder. The fracture
840 Section XV Traumatic Diseases
A B
C D
Figure 57–20. Clavicular fracture: distal segment. A, Type I injury. The coracoclavicular ligaments
remain intact. B, Type II injury. The conoid portion of the coracoclavicular ligaments is severed. C, Type I
injury. A subtle fracture (arrow) of the distal end of the clavicle in a child is unassociated with clavicular
displacement. D, Type II injury. Disruption of the conoid portion of the coracoclavicular ligament has
allowed superior migration of the proximal segment of the clavicle. Note the bone flange (arrow) extending
from the distal clavicular segment to which the trapezoid portion of the coracoclavicular ligament inserts.
boring ribs and pneumothorax. Isolated fractures of the of the coracoid process do not disrupt the coracoclav-
spinous process of the scapula are infrequent and, when icular ligaments. Anteroposterior radiographs may not
present, are a result of direct trauma. Fractures of the demonstrate the coracoid process adequately and must be
acromion process generally follow direct trauma, although supplemented with a lateral scapular view, axillary pro-
muscular traction can rarely produce a similar lesion. On jection, or both (Fig. 57–23). The tip of this process may
radiographs, a fracture line is evident, usually adjacent be displaced in an inferior and medial direction, similar
to the acromioclavicular joint, though occasionally at the to the appearance of a normal accessory ossification center
base of the acromion process. Significant neurologic (infracoracoid bone).
injury, although rare, is a recognized complication.
Fractures of the coracoid process are caused by a
Fracture of the First and Second Ribs
direct injury from a dislocating humeral head; a direct
force on the tip of the coracoid itself; or an avulsion Fractures of the first or second rib indicate major trauma
resulting from traction on the coracoclavicular ligament, to the thorax or shoulder. Associated abnormalities are
the short head of the biceps brachii muscle, or the frequent and potentially serious; they include rupture of
coracobrachialis muscle. Fractures of the anterior aspect the apex of the lung or the subclavian artery, aneurysm
842 Section XV Traumatic Diseases
Humeral Diaphysis
Fractures of the humeral diaphysis account for about 3%
Figure 57–21. Scapular fracture: sites of injury. Fractures of to 5% of all fractures (see Table 57–3). These humeral
the scapula may involve the glenoid fossa and articular surface fractures may occur as a result of either direct trauma
(1), the neck (2) or body (3) of the bone, or the spinous (4), (e.g., motor vehicle accidents, blows received during
acromion (5), or coracoid (6) process. They are most common a physical assault, gunshot wounds) or indirect forces,
in the scapular body and neck. including torsional forces that occur during athletic
endeavors. In fractures occurring above the insertion of
of the aortic arch, tracheoesophageal fistula, pleurisy, the tendon of the pectoralis major muscle (Fig. 57–24),
hemothorax, cardiac alterations, neurologic injury, and the proximal fragment is displaced into abduction and
additional fractures. Because of the serious implications external rotation as a result of the action of the rotator
of these accompanying abnormalities, detection of a frac- cuff musculature; fractures occurring in the interval
ture of the first rib requires careful evaluation of the between the insertion of the pectoralis major tendon
intrathoracic structures to exclude the presence of addi- proximally and the deltoid insertion distally result in
tional injuries. adduction of the proximal fragment and lateral displace-
ment of the distal fragment; and fractures occurring distal
to the insertion of the deltoid muscle result in abduction
of the proximal fragment and proximal displacement of
the distal fragment.
Transverse fractures of the humeral shaft are most
frequent and represent 50% to 70% of all diaphyseal
fractures of the humerus; oblique or spiral fractures, each
representing about 20% of all humeral diaphyseal frac-
tures, result from torsional forces; and segmental and
comminuted fractures constitute the other patterns of
humeral shaft fracture (Fig. 57–25). Approximately three
fourths of all such fractures involve the middle third of
the bone.
Among the complications of fracture of the humeral
diaphysis, neurologic injury is most common. Radial nerve
palsy occurs in as many as 18% of closed fractures of the
humeral shaft and is associated most often with trans-
verse fractures of the diaphysis, particularly those occur-
ring in the junction of the middle and distal thirds of the
Figure 57–22. Scapular fracture: injuries of the scapular bone. Injury to the median or ulnar nerve is rare in cases
body and neck. Horizontal fracture involving the body and of humeral diaphyseal fracture. Vascular compromise occurs
neck of the bone (arrows). in less than 5% of patients with fracture of the humeral
Chapter 57 Physical Injury: Extraspinal Sites 843
TABLE 57–3
Fractures of the Humeral Metaphyses and Shaft
Pectoralis major m.
Deltoid m.
A B C
Figure 57–24. Humeral diaphysis: muscular anatomy. Effects of muscular forces in cases of humeral shaft
fractures are illustrated. (From Browner BD, Levine AM, Jupiter JB, Trafton PG [eds]: Skeletal Trauma,
vol 2, 2nd ed. Philadelphia, WB Saunders, 1998, p 1526.)
844 Section XV Traumatic Diseases
miscellaneous. Transcondylar fractures resemble supra- and ulna in a mediolateral direction and is termed a fracture-
condylar fractures (Fig. 57–28) but are intra-articular in dislocation.
location. The fracture line traverses both condylar sur- The epicondyles, as well as other osseous structures
faces in a horizontal direction. Intercondylar fractures of throughout the elbow, are injured more frequently in
the distal portion of the humerus (Fig. 57–29) result in children or adolescents than in adults (Fig. 57–31). In a
comminuted and complex fracture lines that generally mature skeleton, the medial epicondyle is fractured more
include one component that traverses the supracondylar commonly than the lateral epicondyle, and the injury is
region of the humerus in a transverse or oblique fashion related in most cases to a direct force applied to the epi-
and a second component, vertical or oblique in nature, condyle. Injury to the adjacent ulnar nerve may also be
that enters the articular lumen. The resulting configura- apparent. Isolated fractures of the lateral epicondyle are
tion of the fracture is thus T- or Y-shaped. These frac- very rare in adults.
tures should be distinguished from those involving only
an epicondyle (medial or lateral epicondylar fracture) and
those affecting the capitulum or the trochlea. Fracture of the Olecranon and Coronoid Processes
Condylar fractures are relatively uncommon and occur of the Ulna
predominantly in children. Fractures of the lateral condyle Fractures of the olecranon process, which represent
are more frequent than those of the medial condyle (Fig. approximately 20% of all elbow injuries in adults, result
57–30). Each can be associated with significant instability from direct injury, indirect injury, or a combination of
and restriction of motion, especially if the fracture frag- the two (Fig. 57–32). The pull of the triceps muscle
ment is large. In this regard, a classification system has accounts for displacement of the fragment or fragments.
been devised on the basis of the size of the fragment and Significant posterior displacement of the olecranon frag-
the presence or absence of disruption of the lateral trochlear ment, combined with anterior movement of the remain-
ridge. This structure, which separates the trochlea and ing portion of the ulna and the radial head, is a serious
capitulum, is important in providing medial and lateral injury that is termed a fracture-dislocation of the elbow.
stability to the elbow. In type I fractures of the condyles, Complications of olecranon fractures include a decreased
the lateral trochlear ridge is not disrupted, whereas in range of elbow motion, osteoarthritis, and nonunion and
type II fractures, the larger fracture fragment contains ulnar nerve damage. Isolated fractures of the coronoid
the separated condyle and a portion of this ridge. This process of the ulna are rare and are commonly associated
latter pattern of injury allows translocation of the radius with posterior dislocation of the elbow.
846 Section XV Traumatic Diseases
Fracture of the Head and Neck of the Radius dislocation. In addition, an impaction fracture of the
Radial head fractures are common injuries in adults and radial neck, without involvement of the radial head, is
result principally from indirect trauma. Such fractures encountered frequently. The diagnostic importance of a
are classified as undisplaced fractures (Mason type I), positive fat pad sign, as well as of oblique and specialized
marginal fractures with displacement (e.g., angulation, radiographic projections, is well recognized, and
depression, impaction; Mason type II), comminuted identification of an associated capitulum fracture or
fractures (Mason type III), and, in recognition of their intra-articular osseous fragments has been emphasized
poorer prognosis, fractures associated with elbow (Fig. 57–33).
Chapter 57 Physical Injury: Extraspinal Sites 847
Complications after radial head fractures are infrequent quadratus, pronator teres, and supinator—serve to decrease
and consist of limited range of motion, osteoarthritis, and, the interosseous space. The final resting position of the
in more severe injuries, heterotopic ossification. Such radius after fracture depends on the action of these
fractures, however, may be part of a more complex or muscles and others, such as the biceps brachii muscle,
widespread injury such as elbow dislocation, fracture of and is influenced by the level of the fracture itself. These
the capitulum, subluxation of the distal radioulnar joint patterns of rotation of the proximal portion of the radius
(Essex-Lopresti injury), or other carpal bones. The Essex- are important to orthopedic surgeons, who, when reduc-
Lopresti injury consists of a comminuted and displaced ing the radial fracture, must attempt to eliminate any
radial head fracture and disruption of the distal radio- rotational malalignment. Routine radiography, often
ulnar joint. supplemented with specialized projections, can be used
to judge the degree of supination and pronation of the
proximal portion of the radius by analysis of the appear-
Radial and Ulnar Diaphyses ance of the bicipital tuberosity. The bicipital tuberosity
When a fracture occurs in one or both bones of the
forearm, the muscles that join these bones—pronator
A B
C D
Figure 57–33. Radial fracture: head of the radius. A, Magnification radiography is used to illustrate a
subtle radial head fracture (arrowhead). B, Specialized radiographic projection is used to demonstrate a radial
head fracture (arrowhead). C and D, In a different patient, reformatted CT (C) shows an impacted fracture
of the radial head (arrow), with a displaced posterior fragment (arrowhead). Surface-rendering three-
dimensional CT (D) shows to better advantage the displaced and rotated articular surface.
of the radius is located medially when the arm is positioned jection of the forearm with the arm pronated, the styloid
in full supination, laterally when the arm is positioned in process is located on the outer margin of the distal part
full pronation, and posteriorly when the arm is positioned of the ulna.
in midpronation. Radiographs of the forearm generally Injuries involving a single anatomic structure in the
include a frontal anteroposterior projection with the arm forearm are rare (see Table 57–4). In common with the
supinated. In such a position, the ulnar styloid process is pelvis, mandible, and ankle, the forearm can be considered
not a border-forming structure on the outer aspect of the a ring structure because of the ligamentous and articular
distal end of the ulna; on a frontal (posteroanterior) pro- connections of the radius and ulna. Disruption of the ring
Chapter 57 Physical Injury: Extraspinal Sites 849
at one site is usually accompanied by disruption at a
second (or even third) site. When both injuries are frac-
tures, accurate diagnosis with routine radiography is not
difficult. When a fracture and dislocation are compo-
nents of the two injuries (e.g., Monteggia’s and Galeazzi’s
fracture-dislocations, Essex-Lopresti injury), routine
radiographic diagnosis is straightforward as long as the
entire forearm (i.e., elbow to wrist) is surveyed. When
subluxation is a component of the injury pattern, how-
ever, the initial radiographic examination may be inter-
preted as showing only a single lesion, particularly if the
subluxation is transient or appears only in certain posi-
tions of the forearm. In such cases, scintigraphy, CT, or
MR imaging may provide diagnostic assistance.
TABLE 57–5
Fractures of the Distal Portions of the Radius and Ulna
reduction, articular incongruity, subluxation or disloca- fracture affects the volar rim of the distal end of the radius
tion of the distal radioulnar joint, median nerve compres- and is sometimes referred to as a reverse Barton’s fracture
sion, ulnar nerve injury, entrapment of flexor tendons, or a type of Smith’s fracture.
reflex sympathetic dystrophy, carpal malalignment, delayed A fracture of the styloid process of the radius (see Fig.
union, or nonunion. 57–35) is often referred to as Hutchinson’s fracture or,
A distal radial fracture similar in position to Colles’ because of its original occurrence when the starting crank
fracture but associated with volar (or palmar) angulation of a car engine suddenly reversed during a backfire,
or displacement of the distal fragment is known as Smith’s chauffeur’s fracture.
fracture (Fig. 57–37). Smith’s fracture is much less com-
mon than Colles’ fracture. Complications of Smith’s frac-
tures are similar to those of Colles’ fractures and may Dislocation of the Inferior Radioulnar Joint
include injury to the extensor tendons. Dislocations of the inferior radioulnar joint may occur in
Barton’s fracture involves an injury to the dorsal rim association with a fracture of the radius. This combination
of the distal portion of the radius, generally related to of findings is termed Galeazzi’s fracture-dislocation (Fig.
dorsiflexion and pronation of the forearm on a fixed wrist 57–39). Classically, the shaft of the radius is fractured.
(Fig. 57–38). Differentiation from Colles’ fracture can be Fractures of the distal end of the radius may be associated
accomplished based on findings on a lateral radiograph of with dislocation of the ulnar head. Fractures of the radial
the wrist. Barton’s fracture extends in an oblique fashion neck and head, which may be associated with dislocation
from the dorsal surface of the radius proximally to the of the inferior radioulnar joint, are not regarded as Galeazzi’s
articular surface of the radius distally. It does not violate fracture-dislocations unless the radial shaft is also fractured.
the volar surface of the radius and is accompanied by Dislocation of the ulna usually occurs in a distal, dorsal,
dorsal displacement of the carpus. A variant of Barton’s and medial direction; volar dislocation is less frequent.
Chapter 57 Physical Injury: Extraspinal Sites 851
A B
852 Section XV Traumatic Diseases
A B
C D
Figure 57–40. Lateral (static) carpal instability. A and B, Dorsal intercalary segment instability. Observe
the gap or widening in the scapholunate space (arrows) on the posteroanterior radiograph (A) and
dorsiflexion of the lunate (arrowheads) on the lateral radiograph (B). The radiocarpal and midcarpal joints
are narrowed. A small fracture of the volar surface of the lunate is seen. C and D, Volar intercalary segment
instability. Findings include palmar displacement of the distal pole of the scaphoid, resulting in a ringlike
shadow (arrow) and volar tilting of the lunate (arrowheads).
sequence of events generally occurs after trauma (Fig. Greater arc injuries represent fracture-dislocation patterns
57–44). Lesser arc injuries occur in four stages, with each as this arc passes through the scaphoid, capitate, hamate,
successive stage indicating increased carpal instability. A and triquetrum.
stage I injury represents scapholunate dissociation with
rotatory subluxation of the scaphoid; a stage II injury is
Fracture of the Carpal Bones
characterized by perilunate dislocation; a stage III injury
creates ligamentous disruption at the triquetrolunate; and Approximately 65% of all carpal bone fractures involve
a stage IV injury is associated with lunate dislocation. the scaphoid bone. Fractures of the scaphoid are classi-
854 Section XV Traumatic Diseases
A B
Figure 57–47. Carpal fracture: hamate. A, Carpal tunnel view shows occult fracture of the hook of the
hamate. Arrows identify the radial aspect of the base and a displaced fragment. B, Axial T1-weighted
(TR/TE, 564/16) spin echo MR image shows the flexor tendon of the little finger entrapped between
fracture fragments (arrows mark the ulnar aspect of the hamate hook base and the displaced fragment).
fourth metacarpal, or both; and the articular surface of followed in order of frequency by the proximal phalanges
the second metacarpal (Fig. 57–51). and the middle phalanges. Important varieties include a
Phalangeal fractures are more frequent than metacarpal mallet fracture (in which an avulsion injury at the base of
fractures and typically involve the distal phalanges, the dorsal surface of the terminal phalanx is produced by
damage to the extensor mechanism); a volar plate frac-
ture (in which dorsal dislocation of a proximal interpha-
langeal joint may be associated with an avulsion fracture
in the middle phalanx at the site of attachment of the
volar plate); a central slip attachment fracture (in which
an intra-articular fracture of the dorsal part of the middle
phalanx at the proximal interphalangeal joint accompanies
palmar dislocation of this articulation); and, in an imma-
ture skeleton, physeal separation at the base of the distal
phalanx (the nail-bed injury, in which an open skin sur-
face leads to secondary infection) (Fig. 57–52).
LOWER EXTREMITY
Hip
Hip Dislocation
Dislocation of the femoral head with or without an
acetabular fracture usually follows considerable trauma
and represents approximately 5% of all dislocations. Hip
dislocations are generally classified as anterior, posterior,
or central.
Anterior dislocation of the hip represents 5% to 10%
of all hip dislocations, and it is caused by forced abduc-
tion and external rotation of the leg. On radiographs, the
abnormal position of the femoral head is readily apparent;
A B on frontal radiographs, an anteriorly displaced femoral
head typically moves inferomedially (i.e., obturator dislo-
Figure 57–48. Metacarpophalangeal and interphalangeal joint cation) with the femur abducted and externally rotated,
dislocation. A, Dorsal dislocation of the second metacarpo- and a posteriorly displaced femoral head is usually located
phalangeal joint. B, Dorsal dislocation of the fifth proximal superolaterally with the femur adducted and internally
interphalangeal joint with a small dorsal phalangeal fracture. rotated. In anterior hip dislocations, associated fractures
Chapter 57 Physical Injury: Extraspinal Sites 857
A B
Figure 57–49. Fracture and dislocation of the base of the first metacarpal bone. A, Bennett’s fracture-
dislocation. Observe the typical oblique fracture of the volar lip of the first metacarpal bone (arrow). B,
Rolando’s fracture. A comminuted fracture of the metacarpal base is evident.
of the acetabular rim, greater trochanter, femoral neck, persistently widened hip joint may indicate abnormally
or femoral head may be observed (Fig. 57–53). placed fragments or significant acetabular injury. CT can
Posterior dislocation of the hip represents approxi- be used to identify small osseous fragments (Fig. 57–54).
mately 80% to 85% of all hip dislocations and may result
from a “dashboard injury,” in which the flexed knee strikes
the dashboard during a head-on automobile collision.
The leg is shortened, internally rotated, and adducted. A
A B
Figure 57–52. Phalangeal fracture. A and B, Avulsion fractures. Small avulsion fractures (arrows) near the
proximal interphalangeal or distal interphalangeal joint can result from traction on the volar plate (A) or
extensor tendons (B). Dislocations may also be evident.
Central acetabular fracture-dislocation usually results this injury, and hemorrhage into the pelvis may be observed.
from a force applied to the lateral side of the trochanter Secondary degenerative joint disease is not infrequent. In
and pelvis, with the stress applied through the femoral general, CT scanning is the preferred imaging method
head. Various patterns of acetabular fracture complicate (Fig. 57–55).
A
Figure 57–54. Hip dislocation: posterior dislocation. A,
Radiograph shows posterosuperior displacement of the femoral
head and a curvilinear bone fragment (arrows) in the joint. B,
Figure 57–53. Hip dislocation: anterior dislocation. Radio- Transaxial CT scan reveals the fragment (arrow), which repre-
graph reveals an inferomedial position of the femoral head and sents a portion of the femoral head. This fragment has rotated
a fracture fragment of the lateral portion of the head (arrow). approximately 180 degrees.
Chapter 57 Physical Injury: Extraspinal Sites 859
including subcapital, transcervical, basicervical, intertro-
chanteric, and subtrochanteric, are frequently used to
define the location of the fracture and can be modified to
include intracapsular fractures (those in the subcapital
and transcervical regions) and extracapsular fractures (those
in the basicervical and trochanteric regions). Subcapital
fractures occur immediately beneath the articular surface
of the femoral head, transcervical fractures pass across
the middle of the femoral neck, basicervical fractures
occur at the base of the femoral neck, intertrochanteric
fractures are located in a line between the greater and
lesser trochanters, and subtrochanteric fractures occur
subjacent to this position.
Intracapsular fractures are approximately twice as fre-
quent as those in the trochanteric region. Classification
of intracapsular fractures according to the degree of
displacement on prereduction radiographs is commonly
referred to as the Garden system. Four types of fractures
are identified: type I, incomplete or impacted; type II,
complete without osseous displacement; type III, com-
plete but with partial displacement of fracture fragments;
and type IV, complete with total displacement of fracture
fragments (Fig. 57–57). Type III and IV fractures are
Figure 57–55. Hip dislocation. In association with posterior
dislocation of the hip, a transverse CT scan shows gas (solid
associated with significant complications and technical
arrow), fat (open arrow), and a fluid level (arrowhead) between failure.
the serum above and the cellular components of blood below. Under normal circumstances, femoral neck fractures
reveal evidence of healing in the first 6 to 12 months.
Complications are not uncommon, and nonunion occurs
in approximately 5% to 25% of cases; ischemic necrosis
Fracture of the Proximal Portion of the Femur
of the femoral head occurs in 10% to 30% of cases. MR
Fractures of the proximal portion of the femur in elderly imaging and bone scintigraphy have been used not only
persons with osteopenia, particularly women with osteo- for the initial diagnosis of the fracture but also as a means
porosis, have received great attention. In this situation, of detecting osteonecrosis and other complications.
the injuries are similar to insufficiency stress fractures, Intertrochanteric fractures predominate in elderly
although a history of minor trauma is usually evident. patients, with a somewhat higher frequency in women
Nondisplaced fractures of the proximal portion of the (unlike the overwhelming female predilection observed
femur may escape detection on initial routine radiographic in intracapsular femoral neck fractures). Fracture com-
examination. Diagnostic help is provided by MR imaging minution is common and leads to multiple fragments of
(Fig. 57–56). bone, which may include the greater trochanter, lesser
No classification system for fractures of the proxi- trochanter, or both (Fig. 57–58). Radiographic analysis
mal femur is uniformly accepted. Anatomic designations, is complicated by this comminution, as well as by the
A B
860 Section XV Traumatic Diseases
A B
Figure 57–57. Fracture of the femoral neck: intracapsular fracture. A and B, Observe the bands of
increased radiodensity in each case (arrows). According to the Garden classification system, these injuries
represent a type II fracture (A) and a type III fracture (B), but accurate classification requires analysis of
lateral radiographs as well. With regard to anatomic location, these fractures are predominantly subcapital.
typical displacement and rotation that occur. No uniform In adults, isolated fractures of the greater trochanter
classification system exists for intertrochanteric fractures. are infrequent and are generally related to injury from a
MR imaging is a sensitive and specific method for the fall, particularly in elderly persons. Differentiation of a
diagnosis of intertrochanteric fractures. Incomplete rather fracture of the greater trochanter from one that also
than complete fractures are seen in some cases and appear involves the proximal portion of the femur may require
to be associated with a better prognosis (Fig. 57–59). advanced imaging techniques. Greater trochanter frac-
tures are not usually significantly displaced, and their
detection may be difficult. Fractures of the femur that
commence immediately below the trochanter are consid-
ered subtrochanteric in location. Approximately 5% to
30% of fractures of the proximal portion of the femur
occur in the subtrochanteric region. These fractures occur
in older patients with relatively minor injuries and in
younger patients with major trauma. Pathologic fractures
also occur in this region of the femur and are typical of
Paget’s disease.
TABLE 57–6
Fractures of the Femoral Shaft
rotation. Supracondylar fractures (without intra-articular plateau fractures (5% to 10%). Important imaging con-
extension) are commonly transverse or slightly oblique in siderations related to tibial fractures include the detection
configuration, with varying degrees of displacement and of lipohemarthrosis, avulsion fractures and sites of liga-
comminution of the fracture fragments. Supracondylar mentous detachment (femoral condyle, fibular head, inter-
fractures may be accompanied by a vertical fracture line condylar eminence), meniscal injuries, abnormal widening
extending into the knee that leads to intercondylar frac- of the joint space during the application of stress, and
tures. In condylar fractures, sagittal or coronal fracture disruption of the articular surface (Fig. 57–63).
lines are isolated to the region of a single condyle (Fig.
57–61).
Fractures of the tibial spine or intercondylar eminence panied by ligamentous and meniscal injury, whereas in
are indicative of possible damage to the cruciate ligaments children they may be an isolated phenomenon. Routine
of the knee. Varying degrees of osseous displacement are radiography supplemented with tunnel projections,
seen. Either the anterior tibial spine or, less commonly, radiographs obtained during the application of stress,
the posterior tibial spine is affected; rarely, both are arthrography, and MR imaging are important in the
involved. Avulsion injuries of the anterior tibial spine assessment of these injuries (Fig. 57–64).
occur more commonly in children and adolescents than An avulsion fracture at the site of insertion of the
in adults. Such fractures in adults are typically accom- lateral capsular ligament, termed a Segond fracture, occurs
with the knee in flexion because of internal rotation of
the tibia. Disruption of the anterior cruciate ligament
occurs in 75% to almost 100% of patients with a Segond
fracture; medial ligamentous damage may also be present,
and anterolateral rotatory instability of the knee is evi-
dent on clinical examination (Fig. 57–65).
Knee Dislocation
Figure 57–63. Fracture of the proximal portion of the tibia: Knee dislocation is a rare but serious injury because of
lateral plateau fracture. Valgus stress has led to a markedly the neurovascular insult that may result from popliteal
displaced fracture, as shown on a frontal radiograph. artery and peroneal nerve damage. Anterior, posterior,
864 Section XV Traumatic Diseases
Patellar Dislocation
Traumatic dislocation of the patella can be produced by a
direct blow or an exaggerated contraction of the quadri-
ceps mechanism. Abnormalities predisposing to displace-
ment may include an abnormally high patella (patella
Figure 57–64. Fracture of the proximal portion of the tibia:
intercondylar eminence fracture. Sagittal T1-weighted
(TR/TE, 886/15) spin echo MR image reveals an avulsion
fracture (arrow) of the proximal portion of the tibia and a
lipohemarthrosis (arrowhead). In this image, the anterior
cruciate ligament cannot be evaluated.
Figure 57–65. Fracture of the proximal portion of the tibia: Figure 57–66. Knee dislocation. Sagittal T1-weighted
Segond fracture. Coronal T1-weighted (TR/TE, 800/16) spin (TR/TE, 600/14) spin echo MR image in a patient with a
echo MR image shows a marginal defect (solid arrow) in the recent knee dislocation shows disruption of both the anterior
lateral margin of the tibia—the Segond fracture—and disrup- (arrow) and posterior (arrowhead) cruciate ligaments. A fracture
tion of the anterior cruciate ligament (open arrow). An osteo- of the anterior aspect of the tibia is also present. An artifact
chondral fracture of the lateral femoral condyle and bone obscures visualization of the patella. (Courtesy of Naval
bruises are also evident. Hospital, San Diego, Calif.)
Chapter 57 Physical Injury: Extraspinal Sites 865
alta), deficient height of the lateral femoral condyle, force, the more likely it is that both bones will fracture.
shallowness of the patellofemoral groove, genu valgum Isolated fractures of the shaft of the fibula are uncommon
or recurvatum, lateral insertion of the patellar tendon, and result from a direct blow. More typically, an appar-
muscle weakness, and excessive tibial torsion. Lateral ently isolated fibular fracture is actually one component
dislocation, which may be transient, predominates; conse- of a more complicated injury involving the ankle as well
quently, the patella may no longer be dislocated on initial (see later discussion). Tibial and fibular fractures occur-
radiographs. Identification of characteristic associated ring together may appear at the same level, although it is
fractures therefore assumes diagnostic importance. In common for them to occur at different levels. Additional
particular, osteochondral fractures of the medial patellar injuries are not uncommon and may be clinically occult.
facet and lateral femoral condyle are common. Axial Survey radiographs of the pelvis and ipsilateral femur
radiographs are most useful in detecting these fractures. may be appropriate when a severe injury of the tibia and
The MR imaging features of patellar dislocation include fibula has occurred.
hemarthrosis, intra-articular chondral or osteochondral Tibial shaft fractures generally heal more quickly in
bodies, osteochondral fractures or marrow edema of the children than in adults. The diagnosis of delayed union
medial aspect of the patella and the anterior aspect of the or nonunion of a tibial shaft fracture is made on the basis
lateral femoral condyle, and disruption of the medial of both clinical and radiographic parameters. Additional
patellar retinaculum (Fig. 57–67). complications of tibial (and fibular) shaft fractures include
malunion, infection, neurovascular injury, and compart-
ment syndromes. In children, distinctive fractures of the
Proximal Tibiofibular Joint Dislocation
tibial shaft include a spiral fracture in the first 3 years of
Though rare, proximal tibiofibular joint dislocation may life, designated a toddler’s fracture, and a fracture involv-
be seen in parachuting, hang-gliding, skydiving, and ing the proximal metaphysis of the bone. Fractures of the
horseback riding injuries. Anterior or, less frequently, proximal metaphyseal region of the tibia may be associated
posterior dislocation of the fibular head may be noted. with a subsequent valgus deformity at the fracture site.
Peroneal nerve injury may appear after posterior dislo-
cation of this joint. As the fibular head is displaced ante- Ankle
riorly, it also moves laterally. In cases of posterior disloca-
tion, the fibular head is displaced medially. Fractures about the Ankle
Stability of the ankle joint depends on the integrity of a
ring formed by the tibia, fibula, and talus, united by the
Tibial and Fibular Diaphyses
surrounding ligaments (Fig. 57–68). A single break in the
Of all the long tubular bones, the tibia is fractured most ring does not allow subluxation of the talus in the mortise,
commonly (Table 57–7). In general, the more severe the whereas two or more breaks in the ring, whether frac-
A B
Figure 57–67. Patellar dislocation. A, Axial radiograph reveals lateral dislocation of the patella, with
fragmentation and erosion of a portion of the patellar articular surface (arrows). B, In a different patient,
transverse fat-suppressed fast spin echo (TR/TE, 2000/19) MR image shows a joint effusion, marrow edema
(representing a bone bruise) in the anterior aspect of the lateral femoral condyle, irregularity of the medial
margin of the patella, partial disruption of the medial retinaculum (arrowhead), and a chondral defect (arrow)
in the patella.
866 Section XV Traumatic Diseases
TABLE 57–7
Fractures of the Tibial and Fibular Shafts
tures or a fracture in combination with a ruptured liga- is displaced medially without significant rotation), and
ment, allow abnormal talar motion. Displacement of the dorsiflexion (in which the talus is dorsiflexed on the tibia).
talus in the ankle joint may be evident on routine radio- Thus, with the foot in either pronation or supination, the
graphs, including the mortise view (Fig. 57–69), although talus may be subjected to one of these five vectors of force.
application of stress to the ankle during radiographic
1. Supination–external rotation (SER) injury (stages I to IV).
examination can be of considerable diagnostic help.
The SER category constitutes almost 60% of all ankle
Ankle fractures are classified, according to the mecha-
injuries (Fig. 57–70). The fracture complex is caused
nism of injury, into five major fracture complexes. Within
by external rotation of the supinated foot. External
each group are stages of injury designated by Roman
rotation of the foot forces the talus against the fibula,
numerals; the higher the number, the greater the applied
commonly rupturing the anterior tibiofibular ligament
force and resultant damage. Each group is designated by
(stage I). As the mechanism of injury continues, a short
two terms. The first term is either pronation or supina-
oblique fracture of the distal portion of the fibula
tion, which refers to the position of the foot at the time
occurs (stage II). Stage III is a fracture of the posterior
of injury. The foot is pronated when the forefoot is out-
aspect of the tibia of varying size. Stage IV is charac-
wardly rotated and everted and the hindfoot is abducted.
terized by fracture of the medial malleolus or rupture
Supination represents inward rotation and inversion of
of the deltoid ligament (Fig. 57–71).
the forefoot with adduction of the hindfoot. The second
term reflects the direction in which the talus is displaced 2. Supination-adduction (SAD) injury (stages I and II).
or rotated relative to the mortise formed by the distal The SAD category constitutes about 20% of all ankle
regions of the tibia and fibula. Five directions of talar injuries (Fig. 57–72). These injuries are produced by a
displacement are possible: external rotation (in which the medially directed force acting on a supinated foot.
talus is displaced externally, or laterally), internal rotation Supination causes tension on the lateral ligaments, and
(in which the talus is displaced internally, or medially), with adduction, either a lateral ligament rupture or a
abduction (in which the talus is displaced laterally with- transverse fracture of the distal portion of the fibula
out significant rotation), adduction (in which the talus occurs (stage I). The characteristic transverse fibular
Ankle Dislocation
Subluxations and dislocations of the ankle, particularly
those in a medial or lateral direction, are commonly asso-
B
Figure 57–78. Ankle injuries: pronation-dorsiflexion injury.
A, Initially, a fracture of the medial malleolus occurs (stage I).
Subsequent injuries include a fracture of the anterior tibial
margin (stage II), a supramalleolar fracture of the fibula (stage
III), and a transverse fracture of the posterior aspect of the tibia
Figure 57–77. Ankle injuries: pronation–external rotation that connects with the anterior tibial fracture (stage IV). B and
injury, stage III. Findings include fracture of the medial C, Stage IV injury. Routine radiograph (B) and sagittal T1-
malleolus and an oblique fracture of the fibula (arrowhead), well weighted (TR/TE, 500/12) spin echo MR image (C) show the
above the level of the ankle joint. characteristics of the distal tibial fracture (arrows).
870 Section XV Traumatic Diseases
ciated with fracture of the adjacent malleolar surfaces. beneath the tip of the lateral malleolus may result from
Medial dislocation of the talus appears to be the most severe dorsiflexion and external rotation. Fractures involv-
common type, although any type may be associated with ing the posterior process of the talus may be difficult to
a fracture of the tibial surface. A peculiar type of ankle differentiate from the os trigonum.
injury resulting from severe external rotation of the foot Fractures of the talar neck are second in frequency to
may lead to posterior displacement of the fibula, which avulsion injuries of the bone (Fig. 57–80). Dorsiflexion
becomes locked behind the tibia. This injury is some- related to a force from below or, more rarely, a direct
times referred to as a Bosworth fracture-dislocation of blow to the talus produces this fracture. Complications of
the ankle. talar neck fractures include delayed union or nonunion,
infection, osteoarthritis of adjacent articulations, and
Foot ischemic necrosis, the last occurring in a minority of
nondisplaced fractures but in as many as 80% to 90% of
Fracture and Dislocation of the Talus
displaced fractures, particularly those with dislocations.
The talus is second only to the calcaneus as a site of frac- The appearance of a linear subchondral lucent area
ture in the tarsal bones. Such fractures occur after sudden (Hawkins’ sign) in the talar dome on radiographs obtained
hyperextension of the forefoot (e.g., sudden application 1 to 3 months after a talar neck fracture relates to hyper-
of the brakes in an automobile to avoid an accident). emia and continuity of the blood supply and should not
Avulsion fractures predominate and occur in the superior be misinterpreted as a crescent sign of osteonecrosis.
surface of the talar neck and the lateral, medial, and Fractures of the body of the talus are infrequent; they
posterior aspects of the body; major injuries to the talus may involve the posterior or lateral process, the articular
are also seen. CT scanning is generally regarded as the surface, or all regions, especially in instances of fracture
best of the advanced imaging methods for detecting and comminution. Fractures of the lateral process of the talus
fully assessing these injuries. are believed to result from inversion of the ankle with the
Avulsion fractures are typically the result of a twisting foot in dorsiflexion. They are considered intra-articular
or rotational force combined with flexion or extension in nature, are related most often to a fall from a height,
stresses (Fig. 57–79). A longitudinal compression force and are a recognized injury of snowboarders.
in combination with acute plantar flexion presumably Subluxations or dislocations of the talus are generally
accounts for the avulsion fracture of the anterosuperior accompanied by fractures of the bone, although they may
surface of the talar neck. Eversion stress may lead to occur as an isolated phenomenon. Medial subtalar dislo-
osseous avulsion at the site of attachment of the deep cations are most frequent and represent approximately
fibers of the deltoid ligament to the body of the talus. 55% to 80% of all such dislocations. Lateral subtalar
The posterior process may be fractured during severe dislocations are second in frequency, followed by anterior
plantar flexion of the foot because of compression between and posterior subtalar dislocations, which are rare. Total
the posterior surface of the tibia and the calcaneus. dislocation of the talus is an extremely infrequent and
Disruption of the talar body where the bone projects serious injury.
A B
Figure 57–82. Calcaneal injuries: intra-articular fracture. A, Although routine radiography reveals an
intra-articular fracture of the calcaneus, the status of the posterior subtalar joint is difficult to determine. B,
Direct coronal CT scan shows marked lateral displacement of the lateral portion of the calcaneus, abutment
of this region of the calcaneus and the distal end of the fibula, displacement of the peroneal tendons with a
fibular avulsion fracture (arrow), and uncovering of the posterior facet of the talus.
872 Section XV Traumatic Diseases
complex forces, as detailed in Figure 57–86. In this tions, such as the anterior superior iliac spine, anterior
scheme, stability of the pelvic ring is considered to depend inferior iliac spine, and ischial tuberosity. Unilateral
primarily on the integrity of its ligamentous structures, fractures of a single ramus are common, occur in elderly
especially those located posteriorly. Instability is most patients after a fall or in the form of stress fractures in
characteristic of injuries resulting from vertical shear and athletes and after hip surgery, and are somewhat more
complex forces. In other classification systems, stable frequent in the superior ramus than the inferior ramus.
fractures are generally considered to be those that either These fractures are extremely stable but may be asso-
do not disrupt the osseous ring or disrupt it in only one ciated with subsequent osteolysis simulating that of a
place, and unstable fractures are those that disrupt the malignant tumor.
ring in two or more places (Table 57–8; Fig. 57–87). A fracture of the iliac wing, Duverney’s fracture, follows
a direct injury and is rarely displaced. An isolated trans-
Type I Injuries. Type I injuries, which do not lead to verse fracture of the sacrum is rare and can be easily over-
disruption of the pelvic ring, represent approximately looked on radiographic examination. Neurologic com-
30% of all injuries that involve the bony pelvis. Avulsion promise, related to disruption of higher or lower sacral
fractures occur at sites of muscular and tendinous inser- nerve roots, is a recognized complication of these frac-
874 Section XV Traumatic Diseases
TABLE 57–8
Injuries of the Bony Pelvis
tures. Transverse fractures of the upper or lower portion pelvic fracture patterns and are accompanied by urethral
of the sacrum should be distinguished from vertical frac- or visceral damage in about 30% to 40% of cases.
tures of the sacrum (Fig. 57–88), which may be associated The term Malgaigne’s fracture is generally applied to
with additional osseous and ligamentous injuries, and from a variety of injuries that have in common disruption of
insufficiency stress fractures, which occur in an osteo- the anterior and posterior regions of the pelvic ring.
penic skeleton and have both a vertical and a horizontal Forms of this injury include (1) a vertical fracture of both
configuration. In all instances of sacral fracture, careful pubic rami combined with either dislocation of the
radiographic analysis of the foramina and arcuate lines is sacroiliac joint or fracture of the ilium or sacrum and (2)
required. symphyseal dislocation combined with either dislocation
of the sacroiliac joint or fracture of the ilium or sacrum.
Type II Injuries. Ipsilateral fractures of both pubic rami, a Malgaigne’s fractures represent approximately 15% of all
fracture of the symphysis itself, subluxation of the sacroiliac injuries to the bony pelvis. More extensive disruptions of
joint or symphysis pubis, and a fracture near the sacroiliac the pelvis result from massive crushing injuries in which
articulation are examples of type II pelvic injuries. The the osseous ring is disrupted in multiple locations or
greater the degree of joint diastasis or the more extensive completely shattered (Fig. 57–90).
the displacement at the fracture site, the more likely the
occurrence of a second break in the pelvic ring (type III Type IV Injuries. Fractures of the acetabulum were con-
pattern). Diastasis of the symphysis pubis greater than sidered earlier in this chapter.
15 mm and symphyseal disruption with overlapping of With regard to the complications of pelvic fractures
the pubis represent anterior injuries that should raise the and dislocations, excessive bleeding may accompany many
strong possibility of disruption of the posterior aspect of of these fractures, particularly type III injuries. Injuries to
the pelvic ring as well. the urinary tract are typically associated with symphyseal
diastasis, fractures of the pubic rami, or both; urethral dam-
Type III Injuries. Examples of these injuries, which repre- age is somewhat more frequent than injury to the bladder.
sent double breaks in the pelvic ring (Fig. 57–89), include Damage to the peripheral nerves occurs in approximately
straddle fractures (characterized by vertical fractures of 10% of patients after injuries to the bony pelvis, and this
the rami or dislocation of the symphysis pubis) and frequency increases in those with sacral fractures.
Malgaigne’s fractures (characterized by simultaneous
disruption of both the anterior and posterior aspects of Thoracic Cage
the pelvic ring).
Fracture and Dislocation of the Sternum
Straddle injuries are typified by disruption of the ante-
rior portion of the pelvis in two places; bilateral vertical The usual mechanism leading to fracture or dislocation
fractures involving both pubic rami or a unilateral frac- of the sternum is direct trauma, and associated injuries
ture of both rami combined with symphyseal diastasis of the anterior portion of the ribs and costocartilage are
fulfills this criterion. These injuries represent 20% of all common. Aortic, other arterial, tracheal, cardiac, and
Chapter 57 Physical Injury: Extraspinal Sites 875
Figure 57–86. Fracture and dislocation of the pelvis: biomechanical principles. A, Normal situation,
illustrating the major ligamentous structures. These structures include the iliolumbar ligaments (1),
posterior (2) and anterior (3) sacroiliac ligaments, sacrospinous ligaments (4), and sacrotuberous ligaments
(5). B, External rotation forces. A direct blow to the posterior superior iliac spines (large arrows) leads to
opening of the symphysis pubis (small arrows). Without the addition of a shearing force, the posterior
ligamentous complex remains intact. C, External rotation forces. External rotation of the femora (curved
arrows) or direct compression against the anterior superior iliac spines also produces springing of the
symphysis pubis (small arrows). Without the addition of a shearing force, the posterior ligamentous complex
remains intact. D, Lateral compression forces. A lateral compression force against the iliac crest (large arrow)
causes the hemipelvis to rotate internally. The anterior portions of the sacrum and pubic rami (small arrows)
are injured. E, Lateral compression forces. A direct force against the greater trochanter (large arrow) leads
to similar injuries to the pubic rami and ipsilateral sacroiliac joint ligamentous complex (small arrows). F,
Lateral compression forces. A force (large arrow) directed parallel to the trabeculae about the sacroiliac joint
may produce impaction of bone posteriorly and disruption of the pubic rami (small arrows). G, Vertical
shearing forces. A shearing force (large arrow) crosses perpendicular to the main trabecular pattern and
causes both anterior and posterior injuries (small arrows). (From Tile M: Fractures of the Pelvis and
Acetabulum. Baltimore, Williams & Wilkins, 1984, p 22.)
876 Section XV Traumatic Diseases
A B
C D E
F G H
I
Figure 57–87. Fracture and dislocation of the pelvis: classification system. A, Type I injury: avulsion
fracture. This injury may involve the anterior superior iliac spine (1), anterior inferior iliac spine (2), or
ischial tuberosity (3). B, Type I injury: fracture of a single pubic ramus or iliac wing (Duverney’s fracture).
A single break in the superior or inferior pubic ramus (1 and 2), certain fractures of the ilium (3), and some
types of fractures of the sacrum (4) or coccyx (5) do not lead to disruption of the pelvic ring. C, Type II
injury: ipsilateral fractures of the pubic rami. Such fractures (open arrows) lead to a single break in the pelvic
ring. D, Type II injury: diastasis of the symphysis pubis. This injury (open arrow), or an isolated fracture of
parasymphyseal bone, also leads to a single break in the pelvic ring. E, Type II injury: subluxation of the
sacroiliac joint. This subluxation (open arrow), or an isolated fracture near the sacroiliac joint, is an additional
example of a single break in the pelvic ring. F, Type III injury: straddle fracture. Note the disruption of the
pelvis in two places as a result of bilateral vertical fractures involving both pubic rami (open arrows). G, Type
III injury: Malgaigne’s fracture. Vertical fractures of both pubic rami on one side combined with a sacral
fracture (open arrows) lead to disruption of the pelvic ring in two places. H, Type III injury: Malgaigne’s
fracture. Similar vertical fractures of both pubic rami on one side combined with dislocation of the sacroiliac
joint (open arrows) produce disruption of the pelvic ring in two places. I, Complex injury: “sprung” pelvis.
Disruptions of the pelvic ring relate to bilateral dislocations of the sacroiliac joint and diastasis of the
symphysis pubis (open arrows).
Chapter 57 Physical Injury: Extraspinal Sites 877
Figure 57–88. Fracture and dislocation of the
pelvis: type II and III fractures of the sacrum. A,
Vertical fractures of the sacrum disrupt the arcuate
lines (open arrow), as depicted on a frontal radio-
graph. B, CT is better able to define the extent of
injury (open arrows).
pulmonary injuries represent serious complications of cal segment of the spine (which may be fractured), with
direct sternal trauma. transmission of the force through the upper ribs or clavicle
Indirect mechanisms resulting in sternal fracture or to the sternum, and stress fractures or collapse (i.e.,
dislocation include a blow to the upper thoracic or cervi- buckling) of the sternum (Fig. 57–91).
Figure 57–89. Fracture and dislocation of the pelvis: type III Figure 57–90. Fracture and dislocation of the pelvis: Complex
injury. Symphyseal diastasis and a vertical fracture of the injury. “Sprung” pelvis is characterized by subluxations or dislo-
sacrum (open arrows) have led to disruption of the pelvic ring in cations of both sacroiliac joints and symphyseal diastasis (open
two locations. arrows).
878 Section XV Traumatic Diseases
879
880 Section XV Traumatic Diseases
TABLE 58–1
Classification of Injuries of the Cervical Spine
From Gehweiler JA Jr, Osborne RL Jr, Becker RF: The Radiology of Vertebral Trauma. Philadelphia, WB Saunder, 1980, p 107.
tebral soft tissues at the level of the retropharynx, ante- The lateral radiograph is the initial examination that
rior to the upper four cervical vertebrae. Unfortunately, suggests the presence of this unusual injury. Radiographi-
there is significant overlap in the measurements obtained cally, however, the diagnosis of atlanto-occipital disloca-
in injured and noninjured patients. No significant differ- tion may be difficult to establish, with up to 40% of cases
ence in the width of the retropharyngeal soft tissues is not being appreciated on the initial examination. The most
seen with flexion and extension of the cervical spine in apparent finding is retropharyngeal soft tissue swelling.
adults. Dramatic changes in the retropharyngeal measure- Typically, the cranium is subluxed anteriorly relative
ment can take place with axial rotation, lateral bending, to the cervical spine; however, superior displacement or,
and swallowing, however. The largest increase in the pre- rarely, posterior subluxation occurs in other cases. Specific
vertebral tissues is due to screaming. Soft tissue thick- signs of anterior or posterior atlanto-occipital dislocation
ness also increases with body weight and age in normal include displacement of the basion (midsagittal point of
persons. the anterior lip of the foramen magnum at the lower tip
The cervical prevertebral fat stripe is a thin radiolu- of the clivus) from its normal position superior to the
cent line located immediately anterior to the vertebrae odontoid process, malalignment between the spinolami-
and represents alveolar tissue in the retropharyngeal and nar line of C1 and the posterior margin of the foramen
retroesophageal spaces. A thin collection of air in the magnum, and failure of the clival line to intersect the
esophagus, a frequent normal finding, should not be con- odontoid process.
fused with this structure. Widening of the prevertebral A quantitative technique for evaluating anterior cranial
fat stripe due to obesity may result in an abnormal retro- subluxation is based on the ratio between two lines rather
pharyngeal and retrotracheal soft tissue thickness. than on absolute measurements. This technique, known
as the Powers ratio, is unaffected by variations in patient
size or film magnification (Fig. 58–2). The first line (BC)
OCCIPITOATLANTOAXIAL REGION is the distance between the basion (B) and the posterior
arch of C1 (C) at the spinolaminar line. The second line
General Considerations
(AO) is the measurement between the posterior margin
The occipitoatlantoaxial region, also known as the cervico- of the anterior arch (A) of C1 and the posterior lip of the
cranial junction, differs both anatomically and function- foramen magnum, known as the opisthion (O). These
ally from the remainder of the cervical spine and serves anatomic landmarks are not affected significantly during
as the transition between the cranium and the cervical flexion, extension, or rotation of the neck and can usually
spine. Injuries to the occipitoatlantoaxial region are typi- be identified on the lateral view, although the opisthion
cally the result of direct trauma to the head rather than to is often difficult to delineate.
the spine. In normal persons, the ratio of BC/AO is 0.77 ± 0.09.
The osseous anatomy of both the C1 vertebra (atlas) In patients with atlanto-occipital dislocation, the ratio is
and the C2 vertebra (axis) differs considerably from that 1.15 or greater. This method is valid only when atlanto-
of the remaining spinal vertebrae. The occipitoatlantal occipital dislocation results in anterior translation of the
joint is reinforced by a unique ligamentous arrangement cranium; it is insensitive for longitudinal or posterior
that allows moderate flexion and extension and minimal displacement (Fig. 58–3).
lateral flexion but restricts other movements markedly. In CT allows assessment of the osseous alignment in this
contradistinction, the primary motion at the C1–C2 joint region without moving the patient. Both coronal and
is rotation rather than flexion and extension, which are sagittal image reconstructions are necessary to compre-
the dominant motions in the lower cervical segment. hend the pattern of displacement fully. Sagittal recon-
struction can be used to derive the Powers ratio, because
all the necessary anatomic landmarks are well visualized
Atlanto-occipital Dislocation
in this plane. Associated findings observed in the upper
Atlanto-occipital dislocation, subluxation, and instability cervical region with CT scanning include hemorrhage
may be secondary to acute trauma or a variety of nontrau-
matic conditions, including rheumatoid arthritis, congen-
ital skeletal anomalies, Down’s syndrome, infection, and
calcium pyrophosphate dihydrate crystal deposition at the
craniovertebral junction. Although the large majority of
cases of atlanto-occipital dislocation result in death, the
injury is not invariably fatal or neurologically catastrophic.
Most survivors have some neurologic injury, but up to
20% have no deficit at the time of presentation. Cranial
nerve dysfunction, most commonly involving the sixth and
more caudal cranial nerves, is the most common neuro-
logic abnormality, presumably resulting from avulsion of
the nerve roots from the brain stem. This injury is more
common in children than in adults and typically results
from motor vehicle accidents to pedestrians. Traumatic
atlanto-occipital dislocation has also been documented in Figure 58–2. Powers ratio. This ratio can be used to identify
postmortem studies of abused children. atlanto-occipital dislocation.
Chapter 58 Physical Injury: Spine 883
A B
Figure 58–3. Atlanto-occipital dislocation: horizontal translation type. A, The basion (arrow) is displaced
anteriorly relative to its normal position above the tip of the odontoid process. B, Similar malalignment of
the basion (arrow) is seen in a different patient with atlanto-occipital dislocation. Note the extensive soft
tissue swelling in both examples. The Powers ratio is abnormal in both cases.
around the foramen magnum, thickening of the anterior it is measured at the lower aspect of the anterior arch of
tectorial membrane, and extradural hemorrhage. MR C1.
imaging has also been used to establish the diagnosis of The distance between the anterior arch of C1 and the
atlanto-occipital dislocation, and some investigators have dens can be assessed with CT, but the CT examination is
suggested that MR imaging is the best method for such typically performed in the neutral or extended position,
evaluation, owing to the ability to visualize the ligamen- limiting its sensitivity for atlantoaxial subluxation. In
tous structures directly. transaxial CT scans, the normal atlantoaxial space appears
to be less than 1 mm wide. Normal findings on a CT
examination, however, do not exclude atlantoaxial sub-
Atlantoaxial Subluxation
luxation unless the study is obtained with cervical flexion.
Isolated post-traumatic atlantoaxial subluxation is a rare MR imaging affords a more sensitive method for the
injury. Nontraumatic atlantoaxial subluxation is far more detection of atlantoaxial instability and allows concomi-
frequent, and rheumatoid arthritis is probably the most tant assessment of the degree of cord compression.
common cause of transverse ligament incompetence.
Numerous other arthritides and connective tissue dis-
orders, including the seronegative spondyloarthropathies,
have an association with atlantoaxial subluxation. Five
percent to 30% of patients with Down’s syndrome have
laxity of the transverse ligament.
Traumatic tears of the transverse ligament or avulsion
fractures at its site of insertion are sufficient to allow
displacement of C1 relative to C2 (Fig. 58–4). Almost all
cases of atlantoaxial displacement manifest anterior dislo-
cation of the atlas relative to the axis. Displacements of
up to 5 or even 10 mm can develop after isolated transverse
ligament rupture; further displacement requires insuffi-
ciency of the alar and other secondary ligaments. The
normal distance between the dens and the posterior
margin of the anterior arch of C1 is less than 3 mm in
adults and 5 mm in children. Measurements of the atlanto-
axial distance are reliable only when the odontoid process
is intact and attached to the C2 body. In many normal
persons, the width of the atlantoaxial interval is uneven,
with superior widening resulting in a V-shaped predens Figure 58–4. Atlantoaxial subluxation. Flexion view shows
space. Nine percent of adults show this configuration. A abnormal widening of the atlantoaxial space (arrow), which
normal atlantoaxial distance is seen in these patients when measures 4 mm.
884 Section XV Traumatic Diseases
A B
886 Section XV Traumatic Diseases
tebral edema is suggestive of Jefferson’s fracture. The The classification of odontoid fractures is based on
fracture is characterized more readily on the open-mouth fracture location and has both therapeutic and prognostic
odontoid view, owing to the presence of lateral displace- implications:
ment of the lateral masses of the atlas relative to those of Type 1 odontoid fractures are rare, and some authorities
the axis. Unilateral offset of the lateral masses or bilateral doubt their existence. They are unilateral oblique frac-
offset, particularly when the combined displacement is tures occurring through the tip of the dens and are
3 mm or greater, is highly specific for injuries to the probably secondary to alar ligament avulsion. The
atlantoaxial region (Fig. 58–9). True offset of the lateral fracture can be differentiated easily from the round,
masses must be differentiated from malalignment pro- corticated os terminale, a normal ossicle located at the
duced by uneven skeletal maturation of C1 and C2, head superior tip of the odontoid process.
positioning, and normal variations in the size of the lateral
Type 2 fractures are probably the most common type of
masses. Minimal degrees of displacement of the lateral
odontoid fracture, representing 31% to 65% of dens
masses of the atlas and axis may be seen in healthy persons,
fractures. This injury occurs at the junction of the dens
and offsets of 1 mm are within the normal range and can
and the body of C2 and disrupts the blood supply to
be safely discounted. Currently, CT is used most widely
the odontoid process, which enters adjacent to its base
to evaluate the integrity of the anterior arch of the atlas.
(Fig. 58–10). Approximately 50% of type 2 fractures
are displaced more than 2 mm at the time of presen-
Fracture of the Axis tation. Displacement of more than 4 mm is generally
considered evidence of instability. The rate of nonunion
The axis serves as the transitional vertebra between the
for type 2 fractures is high (26% to 36%). Most authors
cervicocranium above and the remaining portion of
believe that an os odontoideum is an acquired post-
the cervical spine below. The dens, in conjunction with
traumatic abnormality in the majority of cases. The
the anterior arch of C1 and the transverse ligament,
mechanism is believed to be post-traumatic disruption of
prevents anterior and posterior subluxation of C1 on C2.
the bone, followed by interruption of the blood supply
Fracture of the Odontoid Process. Fractures of the odon- to the base of the dens, leading to osteonecrosis and
toid process make up 7% to 13% of all cervical spine resorption of bone. This ossicle can be distinguished
injuries. They represent the most common fracture of from an acute odontoid fracture radiographically by
the axis in most series, accounting for up to 55% of C2 noting the presence of a rounded or oval ossified mass
fractures. The mechanism of injury is complex, and the with smooth, thin cortical borders and its smaller size
fracture is a result of a combination of extreme flexion, than expected for a complete odontoid process.
extension, or rotation, along with a shearing force. Most Type 3 fractures are also relatively common injuries. In
fractures of the dens are the result of a major force. The these cases, horizontal or oblique fractures adjacent to
majority of patients with odontoid fractures have no the base of the dens extend into the cancellous bone of
neurologic deficit and manifest posterior upper cervical the C2 vertebral body. The fracture line usually extends
pain, which often radiates to the occiput, and paraver- into the joints between the lateral masses of C1 and
tebral spasm. The most common neurologic deficits are C2. Although the fractures frequently are displaced,
transient mild upper extremity weakness, lower extremity fracture nonunion is unusual. Lateral tilting of the
hyperreflexia, and diminished occipital sensation. dens greater than 5 degrees may be seen.
A B
Figure 58–9. Jefferson’s fracture. A, Initial open-mouth radiograph shows an asymmetrical position of the
odontoid process between the lateral masses of the atlas. The right lateral atlantoaxial joint is obliterated,
and the position of the lateral borders of the right lateral masses of the atlas and axis is offset (arrowheads).
B, CT scanning reveals two fractures of the atlas (arrowheads). Identification of such fractures requires
analysis of multiple contiguous transaxial scans.
Chapter 58 Physical Injury: Spine 887
A B
Figure 58–10. Type 2 odontoid fracture. A, Anteroposterior open-mouth view shows a transverse fracture
(arrows) across the base of the dens at the same level as the articulation of the C1–C2 lateral masses. B,
Lateral conventional tomogram confirms the presence of a fracture (arrow) at the base of the dens.
The lateral and anteroposterior open-mouth odontoid the hangman’s fracture because of its similarity to the
views are most useful in the assessment of the dens. osseous injury produced by hanging.
Flexion and extension views should be avoided in a patient This injury is defined as bilateral avulsion of the neural
with a dens fracture, because the resultant bone displace- arches from the vertebral body, with or without sublux-
ment may be fatal. Because many odontoid fractures ation, with the odontoid process remaining intact. The
are horizontal, reformatted thin-section CT scans are fractures occur through the pars interarticularis of C2 or
preferable to routine transaxial CT images. Transaxial through the adjacent portion of the articulating facet
CT scans may fail to detect fractures at the base of the (Fig. 58–12). Extension into the posterior vertebral cortex
odontoid process. Fractures of the odontoid process are is seen in up to 18% of cases and is considered a variant
best demonstrated on the anteroposterior open-mouth or part of the spectrum of this injury. Extension into one
view. Type 1 fractures are seen only on this projection, or both vertebral artery foramina is also common. The
provided there is adequate visualization of the tip of the incidence of vertebral artery injury following fracture
odontoid process. In type 2 and 3 fractures, the fracture extension into the vascular foramen is unknown.
line at the base of the odontoid process or within the C2 The lateral view of the cervical spine demonstrates
centrum can be identified, even if the upper part of the the fracture in more than 90% of patients. Prevertebral
odontoid process is obscured. The fracture line may hematoma is found in patients with associated disc disrup-
be very subtle in cases of undisplaced fractures. Lateral tion. The fracture lines run obliquely in a superopos-
tilting of the odontoid process more than 5 degrees like-
wise has been emphasized as an important indicator of
a type 3 odontoid fracture. Displaced or angulated odon-
toid fractures are usually apparent on the lateral view.
Even if the odontoid step-off is not visualized, malalign-
ment between the spinolaminar lines of C1 and C2 may
be apparent. The lateral view may not demonstrate the
odontoid fracture in the absence of displacement or
angulation, although prevertebral soft tissue swelling
typically is present. Type 3 fractures can be distinguished
from type 2 fractures by noting disruption of the “ring
shadow” of the axis, a feature seen only in type 3 injuries
(Fig. 58–11). The ring shadow is a composite of struc-
tures, including the junction of the body of the axis with
its lateral masses, the cortex of the facet joints, and the
vertebral margin; the dens itself does not form any of the
borders of the ring.
A B
Figure 58–13. Hangman’s fracture. Sagittal (A) and transverse (B) T1-weighted (TR/TE, 600/20) spin
echo MR images of a patient with a type 2 hangman’s fracture. A, Hangman’s fracture extends into the C2
vertebral body at its posteroinferior corner. The C2 vertebral body is displaced anteriorly. B, The left
vertebral artery canal is involved in the fracture, and the normal flow void of the vertebral artery is absent.
The patient had no clinical evidence of vascular compromise.
Chapter 58 Physical Injury: Spine 889
Avulsion of the Ring Apophysis. In the immature spine,
hyperextension may produce an avulsion fracture of the
inferior ring apophysis. Its appearance is similar to that of
the avulsion fracture that occurs with hyperextension in
the adult spine. However, unlike its adult counterpart, the
apophyseal avulsion fracture characteristically is not asso-
ciated with soft tissue swelling or other osseous injury.
Follow-up examinations show that healing occurs with
the formation of a pronounced bone excrescence in the
inferior vertebral margin, related to fusion of the avulsed
apophysis with the underlying vertebral body.
Hyperflexion Injuries
One of the most common, if not the most common,
mechanisms resulting in cervical spine injury is hyper-
flexion of the cervical region. Indeed, 46% to 79% of all
cervical injuries are due to hyperflexion. Hyperflexion
injuries are usually caused by a blow to the posterior
Figure 58–15. Hyperextension injury: retrolisthesis and focal aspect of the cranium, forcing the face toward the chest.
hyperlordosis. This woman injured her spine 2 weeks before
this radiograph was taken. The initial radiograph (not shown) Hyperflexion Sprain. Hyperflexion sprain is predomi-
appeared normal. This view was obtained in active supervised nantly a ligamentous injury consisting of disruption of
extension of the neck. There is focal increased lordosis at the the ligaments of the posterior column and, in severe
C3–C4 level, and there is more than 5 mm of retrolisthesis of cases, the posterior longitudinal ligament and posterior
the C3 vertebral body relative to C4. portion of the anulus fibrosus in the middle column. The
injury typically occurs in the absence of any fracture,
although minor wedgelike compressions of the anterior
spinal stability is maintained. Distinction between a sprain portion of the vertebral body occasionally coexist. The
and a reduced dislocation may be impossible on the basis injured ligaments, in order of disruption, are the
of conventional radiographic findings. In both hyper- supraspinous ligament, interspinous ligament, ligamenta
extension sprain and hyperextension dislocation, severe flava, and capsule of the facet joint. Neurologic defect is
neurologic damage is thought to occur when the spinal usually mild and reversible. Approximately 20% to 33%
cord is caught between the posterior margin of the ver- of patients with posterior ligamentous disruption who are
tebral body or posterior osteophytes and the infolded treated conservatively develop progressive kyphosis and
ligamenta flava, particularly in a spondylitic spine. The delayed spinal instability.
characteristic neurologic defect in hyperextension sprain Radiographs of the cervical spine frequently are normal
is the central cord syndrome, related to hemorrhage in despite the presence of a clinically evident cord injury.
the central gray matter. MR imaging is the optimal method When radiographs are positive, widening of the inter-
for evaluating patients with neurologic injuries whose spinous distance or the posterior aspect of the apophyseal
radiographic findings are equivocal. joints is evident. Interspinous widening that is more than
2 mm greater than the width at both adjacent levels has
been suggested as a sensitive criterion of spinal instability.
Extension-Rotation Injuries
An interspinous distance that is more than 1.5 times
A combination of extension, compression, and rotation the distance at both adjacent levels is definitely abnormal
produces unilateral pillar or facet fractures, which repre- and indicates the presence of anterior cervical disloca-
sent 3% to 11% of all cervical spine fractures. Fractures tion. Localized kyphotic angulation of the cervical spine
of the lateral masses are more common than is generally is highly suggestive of this injury. Focally exaggerated
appreciated because they are difficult to detect on con- kyphosis is required for diagnosis of the injury, because
ventional radiographs, and their true prevalence is under- diffuse kyphosis of the cervical spine may result from
estimated. Facet fractures occur most commonly at voluntary flexion or muscle spasm. Another radiographic
C6 and C7 and result in radiculopathy in 6% to 39% feature in patients with hyperflexion injury of the cervical
of patients. spine is kyphosis at a single spinal level with persistent
Chapter 58 Physical Injury: Spine 891
lordosis in the remaining portions of the cervical spine.
If this injury is suspected, it has been suggested that super-
vised flexion and extension views of the cervical spine may
help demonstrate these radiographic findings (Fig. 58–16).
ligament, and the capsular, interspinous, and supraspinous involves the lower cervical spine, with C5 being the most
ligaments invariably is present. As the facet joints lock, common level of involvement. The vertebral body is
marked narrowing of the spinal canal and intervertebral divided into a smaller anteroinferior fragment and a larger
neural foramen results. Such narrowing leads to a neuro- posterior fragment (Fig. 58–19). The anteroinferior tear-
logic deficit in up to 75% of patients with this injury. drop fragment may be large and often involves one third
Vascular injury to the vertebral artery has been shown to to one half of the vertebral body. The teardrop fragment
be a common complication of facet joint dislocation, typically remains aligned with the vertebral body below
although the vascular lesion is typically asymptomatic or or shows minimal anterior and downward displacement.
associated with nonspecific findings. The most specific finding in this injury is posterior
Bilateral facet lock is recognized easily on lateral radi- displacement of the inferior aspect of the larger posterior
ographs, owing to the severe spinal malalignment. Ante- fragment of the fractured vertebral body relative to the
rior displacement of the superior vertebra invariably vertebra below. The most commonly associated fracture
is present and typically is greater than half the width is a sagittal vertebral body fracture.
of the vertebral body (Fig. 58–18). On transverse CT
scans and MR images, this injury produces the naked
Flexion-Rotation Injuries
facet sign bilaterally, whereby the articular surfaces of
both apophyseal joints at one spinal level are no longer in Unilateral facet lock is the only common cervical injury
apposition. produced by the combination of flexion and rotation and
represents between 4% and 16% of all cervical spine
Flexion Teardrop Fracture. Flexion teardrop fractures, like injuries. Rupture of the interspinous ligament and the
bilateral facet locks, are highly unstable injuries that capsule of the apophyseal joint allows the ipsilateral infe-
characteristically result in severe neurologic deficit. rior facet of one vertebra to pivot, dislocate, and come to
Complete quadriplegia, paraplegia, Brown-Séquard syn- rest in the neural foramen anterior to the superior facet
drome, or the anterior cord syndrome develops in 87% of the vertebra below. The spinal cord is impinged on
of patients with this injury. The characteristic neurologic by the posterosuperior aspect of the body below and by
deficit is the anterior cord syndrome, consisting of com- the lamina above. The most common levels of involve-
plete motor paralysis and partial sensory dysfunction, ment are C4–C5 and C5–C6. Although this injury is con-
with loss of pain, touch, and temperature sensations but sidered mechanically stable, the presence of neurologic
preservation of posterior column function. This injury is damage or an associated facet fracture should suggest
the result of combined flexion and axial loading, with the potential mechanical and neurologic instability.
most common inciting events being diving and motor Unilateral facet lock produces alterations of spinal
vehicle accidents. The flexion teardrop injury typically alignment on the lateral and anteroposterior projections
but is easiest to recognize on oblique views. Anterior sub-
luxation of the superior vertebra is typically present, but
the degree of subluxation is less than that seen with
bilateral facet lock, averaging one quarter of the sagittal
A B
joints. The flexion teardrop fracture is grossly unstable, widening, paraspinal hematoma, and left apical capping
whereas burst fractures typically maintain the integrity are features that can be seen in both vertebral and vascu-
of the ligaments of the posterior spinal column. On the lar injuries.
anteroposterior view, burst fractures characteristically are
associated with a sagittal fracture line that extends across
the entire height of the involved vertebral body. This Compression Fracture
sagittal fracture line is difficult to appreciate on conven- Compression fractures of the midthoracic spine are com-
tional radiographs but may be recognized on a well- mon in patients with osteoporosis. Eighteen percent of
penetrated anteroposterior view. white women older than 50 years demonstrate at least
one compression fracture, and prevalence rates approach
78% among women older than 90 years. These fractures
UPPER THORACIC SPINE typically result from flexion or axial forces, or both, pro-
ducing compression loading of the vertebral body. The
General Considerations
cancellous bone of the vertebral body is weaker in com-
If compression deformities secondary to osteoporosis are pression than in tension. The force required to fracture
excluded, fractures of the thoracic spine above the thora- the vertebral body in an osteoporotic person can be trivial.
columbar junction are uncommon. It has been estimated The distribution of acute compression fractures is bimodal,
that only 30% of all traumatic thoracic fractures occur with one peak at the T6–T7 level and the second, larger
above the T10 level. The majority of significant upper peak at the thoracolumbar junction. Typical compression
thoracic spine injuries are due to motor vehicle accidents fractures cause localized pain and kyphosis but generally
or falls. The magnitude of force necessary to overcome do not manifest any acute neurologic deficit. Paraspinal
the inherent stability of the upper thoracic spine is greater swelling, loss of vertebral height, and cortical disruption
than that required for the remainder of the spine. The are apparent (Fig. 58–23). By definition, the middle column
relatively small cross-sectional area of the spinal canal in is intact in compression fractures, so there is no loss of
the upper thoracic region, combined with the high force height of the posterior margin of the vertebral body and
necessary to create an unstable injury in this region, leads no vertebral subluxation. The anteroposterior view may
to a high rate of neurologic damage when such injury show interspinous widening in geometric proportion to
occurs. the degree of anterior vertebral wedging.
Typical compression fractures result in failure confined
to the anterior column. Because the middle column is
Imaging Studies
normal, this injury is generally considered stable. The
The initial radiographic examination of the traumatized major risk factor for early kyphosis after traumatic com-
upper thoracic spine consists of supine anteroposterior pression fractures in nonosteoporotic patients is the pres-
and lateral radiographs. The upper four thoracic vertebrae ence of multiple fractures in the upper thoracic region.
are particularly difficult to see on lateral radiographs. A Conversely, progressive kyphosis is rare with fractures
modified swimmer’s view can be helpful for evaluating at the thoracolumbar region. Anterior wedging that is
the upper vertebral bodies, but this projection requires arm greater than 50% of the height of the vertebral body
movement. In some patients, conventional tomography allows the middle column to act as a fulcrum, potentially
or CT scanning is necessary to assess a suspected injury leading to damage to the posterior ligamentous complex.
of the upper thoracic region. Less commonly, nontraumatic compression fractures
The majority of acute fractures are associated with manifest both anterior and middle column failure, result-
the development of a focal paraspinal hematoma that is ing in fractures with a configuration similar to that of
centered at the level of injury. A hemothorax is also com- burst fractures, with retropulsed fragments of bone and
mon in association with severe spinal injury. Mediastinal potential neurologic compromise.
Chapter 58 Physical Injury: Spine 895
A B
Figure 58–23. Compression fracture. A, Lateral radiograph reveals compression of the anterior and
middle portions of the superior endplate (arrow), with loss of height of the vertebral body anteriorly. B,
Central compression fracture of the seventh thoracic vertebral body produces a central deformity (arrow) of
the superior endplate, with preservation of height of the vertebral body anteriorly and posteriorly.
Middle and lower thoracic compression deformities and bulging of the posterior vertebral cortex suggest that
are encountered in elderly patients. Solitary compression the vertebral body has been replaced by tumor. Both acute
fractures related to osteoporosis are uncommon above benign and pathologic fractures show low signal intensity
the seventh thoracic vertebra. Identification of an isolated on T1-weighted MR images and high signal intensity on
compression fracture in the high thoracic region suggests T2-weighted and short tau inversion recovery (STIR)
a cause other than simple osteoporosis. Distinguishing MR images, but the pattern of increased signal intensity
benign compression fractures from pathologic fractures is less homogeneous and less intense in cases of benign
can be very difficult. Radiographs, CT scans, and MR fracture. Marrow replacement is often partial and band-
images can all be used to determine the cause of non- like in the presence of a benign fracture, whereas the entire
traumatic fractures. The typical deformity of the vertebral body is more likely to show signal abnormalities if the
endplates in osteoporotic compression fractures consists fracture is pathologic. Benign fractures tend to show a
of anterior wedging, diffuse compression, or central com- return to normal marrow signal intensity following the
pression with smooth, diffuse concavity of the upper or intravenous administration of a gadolinium-containing
lower endplate. Biconcavity caused by depression of both contrast agent, whereas pathologic fractures show increased
the upper and lower endplates has been termed “fish signal intensity within the affected vertebra on both T1-
vertebra.” Fish vertebrae typically are seen at the thora- and T2-weighted images.
columbar or lower lumbar level; the characteristic defor-
mity created by osteoporotic compression fractures in the
upper thoracic spine is kyphosis, with anterior collapse or Fracture in Children and Adolescents
wedging of the vertebral body. Upper thoracic fractures in very young children must be
The signal intensity characteristics of an acutely frac- evaluated carefully. This region is rarely traumatized in
tured vertebra on MR images are nonspecific. With MR this age group, so underlying neoplasm, seizures, or child
imaging, preservation of normal fatty marrow within the abuse must be considered. Compression fractures of the
compressed vertebral body is a reliable indicator of a vertebral bodies, vertebral subluxation, and avulsion frac-
benign fracture that is probably chronic (Fig. 58–24). tures of the spinous processes in the thoracic spine are
Incomplete replacement of the bone marrow adjacent to known to occur in abused infants. The most common
the fracture can be seen in about 25% of vertebral com- spinal abnormality in an abused child consists of a supe-
pression fractures, particularly acute fractures. Complete rior endplate compression fracture, producing notching
replacement of the bone marrow generally occurs when of the anterior aspect of the vertebral body, associated
vertebral compression is related to the presence of skeletal with mild disc space narrowing. Forceful shaking, result-
metastasis. The presence of posterior retropulsed bone is ing in repetitive flexion and extension of the infant’s trunk,
more common with benign fractures, whereas convexity is the presumed mechanism for the injuries to the verte-
896 Section XV Traumatic Diseases
A B
bral body and spinous process. Other manifestations of for Kümmell’s disease may be ischemia associated with
child abuse, such as skull and rib fractures, as well as the trauma, which leads to osteonecrosis and delayed collapse
characteristic metaphyseal fractures, are typically present. of the vertebral body. Almost all reported cases of intra-
In older children and adolescents, exaggerated verte- vertebral vacuum clefts occur in noninfectious and non-
bral wedging, vertebral endplate irregularities, Schmorl’s neoplastic conditions.
nodes, and disc space narrowing are characteristic of The typical vacuum cleft appears as a transverse radi-
Scheuermann’s disease. Although numerous theories have olucent line seen in the centrum of the collapsed verte-
been advanced to explain this common condition, the bral body or adjacent to one of its endplates. This linear
most likely cause of Scheuermann’s disease is repetitive intraosseous gas collection must be differentiated from
traumatic stress to the growing spine. Although Schmorl’s the common finding of gas within a degenerated disc.
nodes are a constant finding in Scheuermann’s disease, The vacuum cleft is exaggerated by spinal extension, par-
such lesions are more often seen as an isolated finding on ticularly in the lateral decubitus position, and diminishes
lateral spinal radiographs. A Schmorl’s node represents a with spinal flexion and prolonged supine positioning. On
site of displacement of the nucleus pulposus into the CT scans, the gas collection may appear more inhomo-
vertebral body via a defect in the vertebral endplate. It geneous and irregular than it does on plain films. On MR
appears as a well-defined rounded or hemispheric lucent images, gas is of low signal intensity on all sequences,
lesion with a thin sclerotic margin abutting a vertebral with magnetic inhomogeneity effects evident on gradient
endplate. These intraosseous extensions of the disc are echo images (Fig. 58–25). This thin, linear, horizontal
very common, found in 36% to 79% of cadaveric studies. collection of high signal intensity located centrally within
MR imaging obtained after the intravenous administra- the vertebral body is suggestive of vertebral osteonecrosis.
tion of a gadolinium-containing contrast agent shows
enhancement surrounding the Schmorl’s node, as well
as adjacent bone marrow edema, in up to 19% of such Fracture-Dislocation
lesions. Fracture-dislocation is an unstable injury usually result-
ing from a combination of hyperflexion and axial loading
of the vertebrae, combined with ligamentous disruption
Kümmell’s Disease by rotatory and shear forces. These severe injuries are
Kümmell’s disease is a form of delayed, post-traumatic associated with a very high rate of neurologic injury.
collapse of the vertebral body that occurs weeks or even Most injuries resulting in complete paraplegia occur in
months after an injury. The most widely accepted the mid-dorsal spine, with the majority located at the T3
mechanism for Kümmell’s disease is osteonecrosis. The to T8 levels (Fig. 58–26). The combination of the severe
presence of gas within the vertebral body, also known force necessary to cause vertebral injury and the rela-
as the intravertebral vacuum cleft, suggest that the basis tively small cross-sectional area of the spinal canal in the
Chapter 58 Physical Injury: Spine 897
upper thoracic spine makes this area particularly vulner- THORACOLUMBAR SPINE
able to spinal cord damage. Rarely, thoracic spine trans-
General Considerations
location occurs without the development of a neurologic
deficit. The thoracolumbar junction is one of the most com-
Unfortunately, most patients with an upper thoracic monly injured areas of the spine. This region is particu-
fracture-dislocation develop an intrinsic cord lesion or larly vulnerable because of its wide range of motion, a
severe cord compression. The typical injury produced by facet joint orientation that changes from a sagittal to a
this combination is a fracture-dislocation involving two coronal plane, and the absence of significant adjacent
adjacent vertebrae and all the intervening soft tissues. A supporting structures, such as the rib cage or psoas muscles.
predominantly osseous injury or a mixture of osseous and Most injuries in this region are secondary to pure flexion
soft tissue injuries may be evident; pure soft tissue injury or to flexion combined with compression, rotatory, or
disrupting all three columns is rare. The more superior distraction forces. Spinal flexion results in distraction
vertebral body may show a variety of fractures, with avul- forces posteriorly and compression forces anteriorly.
sion fractures of its inferior margin being most common. Major injuries of the thoracolumbar region can be
The superior vertebra is subluxed anteriorly with respect divided into four types (Table 58–2). The first type is the
to the inferior vertebra. The intervening disc and inter- compression fracture resulting from hyperflexion, with
spinous ligaments are disrupted, and the disc may be compression of only the anterior column and an intact
herniated. Rotational deformity, manifested by rotatory middle column; the majority of these fractures are asso-
malalignment of the pedicles and spinous processes above ciated with a normal posterior column as well. In severe
and below the injury, may also be seen. anterior compressive injuries, partial failure of the poste-
CT scanning and MR imaging can be used to evaluate rior column related to tension may be present. The second
these complex injuries. Typically, the malalignment and category of major injury is the burst fracture, character-
degree of displacement are assessed more readily using ized by failure of both the anterior and middle columns
MR imaging. CT scanning is superior for identifying in compression. The posterior column remains intact
fractures of the posterior elements and determining the functionally, although fractures of the lamina and splay-
extent of encroachment on the spinal canal by displaced ing of the facet joints are often present. The third major
bone fragments. MR imaging is the method of choice for
evaluation of the spinal cord, which is often injured in
association with upper thoracic injuries.
TABLE 58–2
Major Fractures and Dislocations of the Thoracolumbar Spine
Site of Injury
Type of Injury Anterior Column Middle Column Posterior Column
Compression fracture Compression None None or distraction
Burst fracture Compression Compression None
Seat-belt injury None or compression Distraction Distraction
Fracture-dislocation Compression, rotation, shear Distraction, rotation, shear Distraction, rotation, shear
From Denis F: The three column spine and its significance in the classification of acute thoracolumbar spinal injuries. Spine 8:817, 1983.
category, the seat-belt–type injury, represents failure in fracture in a nonosteoporotic person. Vertebral compres-
tension of both the middle and posterior columns as a sion that leads to loss of more than 40% of the height of
result of spinal flexion and, in some cases, additional the anterior portion of the vertebral body often requires
distraction. The anterior column may fail partially due posterior stabilization, even in the absence of neurologic
to compression, but the anterior longitudinal ligament deficit, to prevent progressive spinal deformity.
maintains its ability to act as a “hinge.” The last major In uncomplicated vertebral body fractures, the inter-
category is the fracture-dislocation. In this injury, all vertebral disc remains intact, and no disc space loss is
three columns are disrupted owing to a combination of apparent. On CT scans, compression fractures of the
compression, tension, rotation, and shear. The spine is thoracolumbar region can be recognized by the presence
free to subluxate or dislocate, although radiographs may of an arc of irregular bony fragments displaced circum-
show little malalignment in the resting position. Most ferentially from the vertebral body. The posterior verte-
patients with this injury demonstrate vertebral subluxa- bral wall remains intact, and no retropulsed fragments of
tion or dislocation at the time of initial presentation. bone are present. Fracture lines involving the vertebral
Malalignment in the sagittal, coronal, or transaxial plane body typically involve only its superior aspect, with normal
may be translational or rotatory. bone seen below the level of the pedicles.
Supine anteroposterior and cross-table lateral radi-
ographs constitute an adequate screening examination
for the thoracolumbar region in the setting of trauma. Burst Fracture
Collimated coned views centered on the thoracolumbar Axial loading, or compression, of the vertebral body, usu-
region can be very helpful, because this area is not well ally combined with flexion, produces the commonly seen
seen in the conventional thoracic and lumbar projections. burst fracture. Burst fractures represent 1.5% of all spinal
Most injuries to the thoracolumbar region produce fractures and 14% of thoracolumbar injuries. The peak
kyphosis, which is often reduced in the supine position. site of spinal burst fractures is the thoracolumbar junc-
Soft tissue alterations due to thoracolumbar injuries, tion, with T12, L1, and L2 being the most common levels
unlike injuries of the upper thoracic spine, are absent on involved. The majority of such fractures are associated
conventional radiographs. with neurologic deficit. Burst fractures can occur only in
the spinal segments with the ability to straighten—the
cervical and thoracolumbar regions.
Compression Fracture
Characteristic components of this injury are centripetal
The thoracolumbar region is the most common site of disruption, increased sagittal diameter, and moderate to
traumatic compression fractures, and it is also a major marked anterior wedging of the vertebral body; unilateral
site of fractures resulting from trivial injury in persons or bilateral laminar fractures; an increased interpedicu-
with osteoporosis. The most common sites of involve- late distance; involvement of the inferior endplate; and
ment are L1 and L2, followed in frequency by T12, T7, narrowing of the spinal canal related to retropulsion of
and L3. Most traumatic compression fractures occur the posterosuperior portion of the vertebral body. It is
between T12 and L2. Among osteoporotic compression essential to differentiate the burst fracture from the more
fractures, a large proportion involve the thoracolumbar common simple compression fracture because of the
region, although the midthoracic region shows a slightly potential for spinal instability and compromise of the
higher prevalence of compression deformities. neural canal in the former. Anterior vertebral wedging
The typical thoracolumbar compression fracture results can be pronounced in either injury. In burst fractures, the
in loss of height of the anterior vertebral body, with average degree of wedging of the anterior portion of the
preservation of the middle and posterior spinal columns. vertebral body does not differ significantly from that seen
In severe compression fractures, partial or complete with compression fractures. Careful evaluation of the
posterior ligamentous injury may be present. The usual posterior margin of the vertebral body usually allows accu-
treatment for these injuries is conservative. Progressive rate differentiation of these two injuries. The posterior
kyphosis is unusual after thoracolumbar compression aspect of the normal vertebral body is seen clearly on a
Chapter 58 Physical Injury: Spine 899
well-penetrated, nonrotated lateral view. The posterior
cortical line is continuous except in the center of the
vertebral body, where it is interrupted by the entry of the
nutrient vessels. Disruption, displacement, or rotation of
this line indicates disruption of the middle spinal column
(Fig. 58–27).
The retropulsed bone fragment in burst fractures charac-
teristically arises from the posterosuperior corner of the
fractured vertebral body superior to the basivertebral fora-
men. On lateral views, the fragment is often triangular,
with its sides formed by the posterior part of the superior
endplate, the superior part of the posterior vertebral
margin, and the fracture line itself. The retropulsed frag-
ment frequently is comminuted. Posterior element frac-
tures are seen in 50% to 100% of patients with burst
fractures. Typical fractures of the posterior elements asso-
ciated with burst fractures include vertical fractures of
one or both laminae and the spinous process. Posterior
element fractures may be difficult to identify on conven-
tional radiographs, and a higher rate of posterior element Figure 58–27. Thoracolumbar burst fracture. Lateral radi-
ograph reveals loss of height of the vertebral body, with involve-
fractures is reported in studies using CT scanning. ment of the cortex both anteriorly and posteriorly (arrow).
Normally, the interpediculate distance increases grad-
ually from the T6 spinal level caudally. In the thoracic
region, comparison of measurements made at the injured
level with those obtained at both adjacent levels is recom- and, in some cases, superimposed distraction of the spinal
mended. The mean value for the interpediculate distance column. Not all patients sustaining injuries of this type
in cases of burst fracture is increased by 25%; however, are wearing seat belts at the time of injury. Such injuries
such widening is not present in all cases. CT scanning is occur most frequently at the thoracolumbar and upper
the optimal method for identifying the presence of poste- lumbar levels, particularly L1 or L2; these levels are
rior element fractures and retropulsed bone fragments, involved in the majority of adults. In children restrained
features not present in simple compression fractures (Fig. by lap belts, the midlumbar level is injured more frequently
58–28). The failure of the posterior vertebral wall is usu- than the thoracolumbar region.
ally readily apparent. With CT, the degree of narrowing The fulcrum of flexion in lap-belt injuries is located
of the spinal canal produced by retropulsed bone frag- anterior to the vertebral body, at the level of the anterior
ments can be quantitated in the preoperative and imme- abdominal wall where it abuts on the seat belt. Hyper-
diate postoperative periods. flexion in this setting subjects the middle and posterior
vertebral columns, or all three columns, to distraction
forces. The resulting tensile force can lead to purely
Seat-Belt Injury
osseous, purely ligamentous, or combined bony and soft
The term seat-belt or lap-belt injury refers to a variety of tissue injuries. Because, in general, ligaments withstand
osseous and ligamentous injuries caused by hyperflexion tension better than bone does, fractures are typically
A B
900 Section XV Traumatic Diseases
present, particularly in the posterior elements. These fractures, with one peak at the T6–T7 level and a second
injuries, of which the horizontal Chance fracture is the best- peak at the thoracolumbar junction. The vast majority
known subtype, begin posteriorly and propagate anteriorly. of these injuries are caused by a combination of spinal
The radiographic findings of a seat-belt injury depend flexion and rotation. The posterior and middle columns
on whether the injury is predominantly osseous or soft fail in tension and rotation, whereas the anterior column
tissue. In general, distraction at the level of the posterior fails in compression and rotation. Fracture-dislocations
fracture or sites of ligament disruption, relative preserva- are associated with a very high rate of neurologic deficit,
tion of or increase in the height of the vertebral body, and with 53% to 93% of patients developing a permanent
mild focal kyphosis are seen (Fig. 58–29). Posterior liga- neurologic deficit, typically complete paraplegia, after
mentous damage results in interspinous widening; increased their injury.
height of the intervertebral foramina; and widening, The major hallmark of these severe injuries is the
superior subluxation, perching, or locking of the facet presence of intervertebral subluxation or dislocation; loss
joints. Interspinous widening is often difficult to assess in of height of a vertebral body is not a characteristic feature
this region, owing to the changing orientation of the in these injuries. In one series, anterior subluxation
spinous processes. Sufficient angulation may be present averaged one third the width of the vertebral body. Such
to give rise to an “empty” or “vacant” appearance of the subluxation is better seen on the lateral view, whereas
vertebral body as a result of cephalad displacement of the rotation and lateral translation are seen to better advan-
posterior elements of the cranial vertebra. If the posterior tage on the anteroposterior projection. Radiographic
fibers of the anulus fibrosus are disrupted, widening of signs suggestive of rotation injury include slice fractures
the posterior portion of the disc space may be apparent. of the vertebral body, multiple fractures of the ribs or
The characteristic injury seen with this mode of failure is transverse processes, dislocation of the costotransverse
a horizontal fracture extending transversely through the joints, unilateral displaced fractures of the articular facets,
vertebral body, pedicles, and other posterior elements. and rotatory malalignment between the pedicles and
Careful scrutiny of these structures on the anteroposte- spinous processes of vertebrae located above and below
rior view is recommended, because the posterior elements the injury (Fig. 58–30). The rotational slice fracture is a
are often obscured on the lateral radiograph. CT scan- horizontal fracture involving the superior endplate and a
ning is helpful in elucidating the nature of the posterior horizontal, sliver-like fracture of the vertebral body below
injury and is essential for excluding posterior element the dislocation; the upper vertebra, intervertebral disc,
fractures and facet disruption. and slice fracture fragment are displaced as a unit, which
pivots relative to the caudal vertebra. The interpediculate
distance is normal, and the posterior cortex of the ver-
Fracture-Dislocation
tebral body characteristically remains intact. Radiographic
The main characteristic of this severe injury is complete signs suggestive of shear injury include lateral vertebral
disruption of all three spinal columns, allowing vertebral translation, severely comminuted fractures of the articu-
subluxation and dislocation. The spinal distribution of lar processes, multiple fractures of the spinous processes,
fracture-dislocations is bimodal, as with compression and free-floating laminae.
A B
Figure 58–29. Thoracolumbar seat-belt injury. Frontal (A) and lateral (B) radiographs reveal a horizontal
fracture (arrows) involving the laminae, articular processes, pedicles, and posterior portion of the first lumbar
vertebral body. Note the widening of the distance between the spinous processes of the T12 and L1 vertebrae.
Chapter 58 Physical Injury: Spine 901
Fractures of the transverse processes of the vertebrae
result from lateral flexion-extension or, less commonly,
from a direct blow, or they may be seen in association
with Malgaigne’s fracture of the pelvis. These fractures
tend to occur in the absence of other vertebral fractures,
although multiple transverse processes may be involved.
The most commonly involved levels are L3 and L4.
These fractures are stable injuries, although they may be
associated with abdominal, thoracic, and genitourinary
injuries. The fractures are most evident on the antero-
posterior radiograph, in which disruption of the cortex
and displacement of the fractured tip of the transverse
process can be seen. Isolated fractures of the transverse
processes may be difficult to identify, however, owing to
the relatively thin bone in this region and overlying bowel
gas. Isolated fractures of the pedicles may be related
to trauma or may represent fatigue fractures caused by
repetitive stress.
A B
Figure 58–33. Avulsion of the posterior ring apophysis. A, Lateral radiograph shows a bone fragment
(arrow) located posterior to the L3–L4 intervertebral disc. Note the narrowing of the intervertebral disc
space and the irregularity of the posteroinferior margin of the L3 vertebral body, the source of the displaced
bone fragment. B, CT scan demonstrates the characteristic arcuate configuration of the displaced
apophyseal fragment (arrow) and the Schmorl’s node at the posteroinferior margin of the vertebral body.
904 Section XV Traumatic Diseases
Apophyseal fractures can occur throughout the spine, Denis F: The three column spine and its significance in the
but the most common levels of involvement are the infe- classification of acute thoracolumbar spinal injuries. Spine
rior ring apophyses of the lower lumbar vertebrae, parti- 8:817, 1983.
cularly the posteroinferior margin of L4. Alternatively, El-Khoury GY, Kathol MH, Daniel WW: Imaging of acute
injuries of the cervical spine: Value of plain radiography, CT,
some authors have suggested that the posterior cephalad
and MR imaging. AJR Am J Roentgenol 164:43, 1995.
rim of the sacrum is affected most frequently. This dis- Flanders AE, Schaefer DM, Doan HY, et al: Acute cervical spine
crepancy can be explained by the imaging method used; trauma: Correlation of MR imaging findings with degree of
rim avulsions of the sacrum are extremely difficult to neurologic deficit. Radiology 177:25, 1990.
visualize on conventional radiographs and are diagnosed Frankel HL, Hancock DO, Hyslop G, et al: The value of pos-
by CT scanning or MR imaging in most cases. tural reduction in the initial management of closed injuries
Conventional radiographs must be examined carefully, of the spine with paraplegia and tetraplegia. Paraplegia
because these lesions are difficult to see. Findings on the 7:179, 1969.
lateral view include mild disc space narrowing, irregu- Gehweiler JA, Duff DE, Martinez S, et al: Fractures of the atlas
larity of the posterior vertebral corner, and an irregular vertebra. Skeletal Radiol 1:97, 1976.
Kliewer MA, Gray L, Paver J, et al: Acute spinal ligament
wedge-shaped ossific defect displaced into the spinal canal.
disruption: MR imaging with anatomic correlation. J Magn
On CT scans, the osseous fracture fragment has a charac- Reson Imaging 3:855, 1993.
teristic arcuate or semilunar configuration that parallels McArdle CB, Crofford MJ, Mirfakhraee M, et al: Surface coil
the border of the posterior vertebral body (Fig. 58–33). MR of spinal trauma: Preliminary experience. AJNR Am J
The fragment may appear irregular rather than arcuate if Neuroradiol 7:885, 1986.
it contains a large fragment of the vertebral body. This Mirvis SE, Young JWR, Lim C, et al: Hangman’s fracture:
fragment must be differentiated from osteophytes, calci- Radiologic assessment in 27 cases. Radiology 163:713, 1987.
fied disc fragments, and calcification or ossification of the Orrison WW, Johansen JG, Eldevik OP, et al: Optimal
posterior longitudinal ligament. The fragment is more computed-tomographic techniques for cervical spine
difficult to characterize on MR images because it may be imaging. Radiology 144:180, 1982.
Penecot GF, Couraud D, Hardy JR, et al: Roentgenographical
hard to distinguish the osseous rim from the low signal
study of the stability of the cervical spine in children. J
intensity of the posterior longitudinal ligament. Pediatr Orthop 4:346, 1984.
Pennell RG, Maurer AH, Bonakdarpour A: Stress injuries of
the pars interarticularis: Radiologic classification and indica-
FURTHER READING tions for scintigraphy. AJR Am J Roentgenol 145:763, 1985.
Powers B, Miller MD, Kramer RS, et al: Traumatic anterior
Acheson MB, Livingston RR, Richardson ML, et al: High- atlanto-occipital dislocation. Neurosurgery 4:12, 1979.
resolution CT scanning in the evaluation of cervical spine Sherk HH, Nicholson JT: Fractures of the atlas. J Bone Joint
fractures: Comparison with plain film examinations. AJR Surg Am 52:1017, 1970.
Am J Roentgenol 148:1179, 1987. Tarr RW, Drolshagen LF, Kerner TC, et al: MR imaging of
Anderson LD, D’Alonzo RT: Fractures of the odontoid process recent spinal trauma. J Comput Assist Tomogr 11:412, 1987.
of the axis. J Bone Joint Surg Am 56:1663, 1974. Traughber PD, Havlina JM Jr: Bilateral pedicle stress fractures:
Bundschuh CV, Alley JB, Ross M, et al: Magnetic resonance SPECT and CT features. J Comput Assist Tomogr 15:338,
imaging of suspected atlanto-occipital dislocation: Two case 1991.
reports. Spine 17:245, 1992. Wiltse LL, Newman PH, Macnab I: Classification of spondy-
Daffner RH, Deeb ZL, Goldberg AL, et al: The radiologic lolisis and spondylolisthesis. Clin Orthop 117:23, 1976.
assessment of post-traumatic vertebral stability. Skeletal
Radiol 19:103, 1990.
SECTION
XVI
Internal Derangement of Joints
CHAPTER 59
Internal Derangements of Joints computed arthrotomography are addressed, their cover-
age is brief because of the prominent role of magnetic
resonance (MR) imaging. A number of conditions occur-
ring around these anatomic regions are included, even
SUMMARY OF KEY FEATURES though they do not precisely fall into the category of
internal derangement of joints.
This chapter summarizes some of the important
internal derangements and related conditions occurring
in six anatomic regions. Abnormalities of articular WRIST
cartilage, subchondral bone, synovial membrane,
Anatomy
joint capsule, ligament, tendon, muscle, and nerve are
emphasized, with particular attention to anatomy and The osseous and articular anatomy of the wrist is shown
concepts of pathology and pathophysiology. in Figure 59–1. The radiocarpal compartment is formed
These abnormalities can be delineated with a variety of proximally by the distal surface of the radius and the trian-
imaging methods, including routine radiography, gular fibrocartilage (TFC) and distally by the proximal row
arthrography, tenography, bursography, ultrasonography, of carpal bones, exclusive of the pisiform. In the coronal
bone scintigraphy, computed tomography, computed plane, the radiocarpal compartment is a C-shaped cavity
arthrotomography, and magnetic resonance imaging. with a smooth, shallow curve that is concave distally. The
Although clinical assessment is the key to correctly distal radioulnar joint is stabilized primarily by the triangular
diagnosing many of these conditions, the contributions fibrocartilage complex (TFCC) of the wrist. The
of these imaging methods are significant. components of the TFCC are not agreed on but include
the TFC itself, the dorsal and volar radioulnar ligaments,
the ulnomeniscal homologue, the ulnar collateral liga-
ment, and the sheath of the extensor carpi ulnaris tendon.
The flexor tendons of the fingers, the sublimis digi-
INTRODUCTION torum and profundus digitorum, are enveloped by digital
This chapter reviews the diagnostic imaging methods for sheaths from the line of insertion of the flexor profundus
internal derangements of joints in six anatomic regions: to a line 1 cm proximal to the proximal border of the deep
wrist, elbow, shoulder, hip, knee, and ankle and foot. transverse ligament (Fig. 59–2). This arrangement, which
Although ancillary techniques such as arthrography and is not constant, is most frequent in the index, middle, and
Intermetacarpal joint
First
carpometacarpal
joint
Pisiformtriquetral
Midcarpal joint joint
Triangular
fibrocartilage
Radiocarpal joint
Inferior
radioulnar
joint
905
906 Section XVI Internal Derangement of Joints
ring fingers. may be found enveloping the index flexor TABLE 59–1
tendons. Additionally, a small synovial sac may enclose
the tendon of the flexor carpi radialis muscle as it passes Extensor Tendon Compartments of the Wrist
under the crest of the trapezium.
Several synovial sheaths are located in the dorsum of 1. Abductor pollicis longus
the wrist beneath the dorsal carpal ligament; they extend Extensor pollicis brevis
for a short distance proximal and distal to that ligament 2. Extensor carpi radialis longus
(Fig. 59–3). Six distinct avenues are created for transport Extensor carpi radialis brevis
of the ligamentous structures (Table 59–1). 3. Extensor pollicis longus
4. Extensor digitorum communis
Extensor indicis proprius
Distal Radioulnar Joint Abnormalities
5. Extensor digiti quinti proprius
Functional Anatomy. Features that are fundamental to the 6. Extensor carpi ulnaris
optimal function of the distal radioulnar joint include the
following:
1. The articulating surface between the ulnar head and
the sigmoid notch of the radius must be intact. As the
forearm moves from supination to pronation, the
ulnar head rotates up to 150 degrees and also glides
several millimeters in a proximal to distal direction
within the sigmoid notch.
2. The soft tissue and ligamentous support system of the
distal radioulnar joint must be intact. This includes
the various components of the TFCC (consisting of
the TFC or articular disc, dorsal and volar radioulnar
ligaments, ulnar collateral ligament, meniscus homo-
logue, and sheath of the extensor carpi ulnaris tendon).
3. A proper relationship must exist between the lengths
of the radius and ulna (Fig. 59–4). Changes in the
length of the ulna relative to the length of the radius, Figure 59–4. Ulnar variance. Negative and positive ulnar
designated positive and negative ulnar variance, alter variance are illustrated.
Chapter 59 Internal Derangements of Joints 907
the distribution of compressive forces across the wrist. signal intensity. Although MR images of a normal TFC
The consequences of a short ulna, or negative ulnar generally show low signal intensity, regions of higher signal
variance, include increased force applied to the radial intensity within the TFC are regularly encountered on
side of the wrist and to the lunate bone, which may T1-weighted spin echo MR images in older individuals,
explain the association of negative ulnar variance and related to degenerative change.
Kienböck’s disease. A consequence of a long ulna, or The increased signal intensity in a degenerative TFC
positive ulnar variance, is the ulnar impaction or ulnar on T1-weighted spin echo MR images can simulate the
abutment syndrome. appearance of traumatic lesions. Detection of a less high
signal intensity in the TFC on T2-weighted spin echo MR
images is useful in differentiating TFC degeneration from
Lesions of the Triangular Fibrocartilage Complex TFC perforation, in which high signal intensity on T2-
Lesions of the TFCC are variable in extent; they may be weighted spin echo MR images is evident. Although dif-
confined to the horizontal, or flat, portion of the TFCC ferentiating between a traumatically induced communicating
(referred to as the TFC or articular disc) or involve one defect of the TFC and one related to degeneration is diffi-
or more components of the TFCC with or without insta- cult with the use of MR imaging (or standard arthrography),
bility of the distal radioulnar joint. characteristic MR imaging features frequently allow an
Degenerative lesions of the TFC are more common than accurate diagnosis of some type of communicating defect
traumatic lesions. Either type may result in full-thickness or perforation of the structure. Linear regions of high
defects, as can be documented with arthrography and MR signal intensity, identical to that of fluid, that traverse the
imaging. In young persons, a normal TFC is characterized entire thickness of the TFC on T1- and T2-weighted spin
by homogeneously low signal intensity on virtually all MR echo MR images (or gradient echo images) are strong
pulse sequences in the coronal, sagittal, and transverse evidence that a communicating defect is present (Fig. 59–6).
planes. In the coronal plane, a normal TFC usually appears Similar regions of high signal intensity extending par-
as an elongated triangle with its apex attaching to the tially through the thickness of the TFC are compatible
articular cartilage of the radius (Fig. 59–5). The triangle with the diagnosis of a noncommunicating defect.
may be thinner in persons with positive ulnar variance and Most previous investigations of the value of MR imaging
thicker in those with negative ulnar variance. The ulnar in the assessment of the TFC relied on findings derived
attachment of the TFC may appear bifurcated, with two from spin echo sequences. Gradient echo imaging
bands of low signal intensity separated by a region of higher sequences may also be valuable, however. On multiplanar
gradient recalled (MPGR) MR images in which a low flip
angle is used, fluid accumulating within communicating
defects in the TFC is of high signal intensity. When
gradient recalled imaging is combined with volumetric of the lunotriquetral interosseous ligament. MR imaging
acquisition, thin sections of the TFC can be obtained and also documents these abnormalities of the TFC and lunotri-
might accentuate the abnormalities associated with such quetral interosseous ligament, as well as chondromalacia
defects. The results of MR imaging and arthrography in and alterations in the subchondral bone of the proximal
the assessment of defects of the TFC appear to agree surfaces of the lunate and triquetrum (Fig. 59–7).
closely. At this time, state-of-the-art MR imaging and
arthrography can be considered equally accurate in this
Ulnar Impingement Syndrome
assessment.
The ulnar impingement syndrome is associated with
clinical manifestations that are similar to but often more
Ulnar Impaction Syndrome disabling than those accompanying the ulnar impaction
The ulnar impaction syndrome, also termed ulnar abut- syndrome. Pain may be aggravated during pronation and
ment syndrome, is defined as a degenerative condition supination of the forearm. Three radiographic features
characterized by ulnar-sided wrist pain, swelling, and are characteristic of the ulnar impingement syndrome:
limitation of motion related to excessive load bearing negative ulnar variance, scalloped concavity of the distal
across the ulnar aspect of the wrist. Chronic impaction of radius, and convergence of the ulna with the distal portion
the ulnar head against the TFCC and ulnar-sided carpal of the radius.
bones results in progressive deterioration of the TFCC,
chondromalacia of the lunate and head of the ulna, and
Instability of the Distal Radioulnar Joint
attrition of the lunotriquetral interosseous ligament. The
ulnar impaction syndrome is almost always associated The distal radioulnar joint is involved in pronation and
with a positive ulnar variance. supination of the forearm, during which the radius moves
Routine radiographs in patients with the ulnar with respect to a relatively fixed ulna. Clinical manifesta-
impaction syndrome may reveal alterations in the lunate, tions include pain, weakness, loss of forearm rotation, and
triquetrum, and ulnar head. Findings include bone scle- snapping. Dorsal instability predominates, and physical
rosis, cysts, and osteophytes. Arthrography often demon- examination confirms a dorsal prominence of the ulnar
strates communicating defects of the TFC and disruption head, especially in a position of forearm pronation.
A B C
Figure 59–7. Ulnar impaction syndrome. A, Coronal intermediate-weighted (TR/TE, 1700/20) spin echo
MR image shows a large communicating defect (open arrow) in the triangular fibrocartilage. Fluid (solid
arrows) is apparent in the distal radioulnar joint. The lunotriquetral interosseous ligament is not well seen.
Note the abnormal regions of low signal intensity in the lunate and scaphoid, consistent with marrow
edema. The lunate changes occurred as a consequence of the ulnar impaction syndrome. B, Coronal T2-
weighted (TR/TE, 1700/70) spin echo MR image also shows the communicating defect (open arrow) in the
triangular fibrocartilage. Fluid fills the distal radioulnar joint (solid arrows) and is seen in the midcarpal joint
(arrowheads), including the space between the lunate and triquetrum. High signal intensity in the proximal
portion of the lunate is indicative of marrow edema. C, Coronal STIR MR image (TR/TE, 1650/25;
inversion time, 160 msec) shows defects in the triangular fibrocartilage (open arrow) and lunotriquetral
interosseous ligament. Fluid fills the distal radioulnar joint (solid arrow) and portions of the radiocarpal and
midcarpal joints. Marrow edema in the scaphoid and lunate is manifested as regions of increased signal
intensity. (From Schweitzer ME, Brahme SK, Hodler J, et al: Radiology 182:205, 1992.)
Chapter 59 Internal Derangements of Joints 909
Detection of subluxation of the distal portion of the produces dorsal intercalated segmental instability , and
ulna is best evaluated with computed tomography (CT). lunotriquetral dissociation, which leads to volar inter-
Accurate appraisal of the relative position of the ulna calated segmental instability (Fig. 59–9).
with respect to the radius requires imaging of the wrist in Diagnosis of many of the static patterns of carpal insta-
supination, neutral, and pronation and the construction bility can be established on the basis of routine radiographic
of a number of lines, as shown in Figure 59–8. When the findings. Arthrography can be used for the diagnosis of
wrist is pronated, the ulna moves dorsally, and when the communicating defects of the scapholunate and lunotri-
wrist is supinated, the head moves in a volar direction. quetral interosseous ligaments. Such defects, however,
may relate not to injury but to progressive deterioration,
a process that increases in frequency with advancing age.
Carpal Abnormalities
MR imaging is useful for the identification of defects
Carpal Instability of the scapholunate and lunotriquetral interosseous liga-
Anatomically, three types of carpal instability are recog- ments. The coronal plane is most useful for delineating
nized: lateral instability, which usually occurs between
the scaphoid and the lunate; medial instability, which
occurs between the triquetrum and the lunate or between
the triquetrum and the hamate; and proximal instability,
which occurs when the abnormal carpal alignment is
secondary to an injury to the radius or massive radiocarpal
disruption. The patterns of instability that result from
ligamentous disruption in the proximal carpal row (i.e.,
between the scaphoid and lunate or between the lunate
and triquetrum) include scapholunate dissociation, which
the interosseous ligaments. Of the two interosseous or after injury). The features of osteonecrosis at these sites
ligaments, the scapholunate is visualized more consistently, are discussed in Chapter 67. MR imaging is currently
although some investigators have indicated that the lunotri- regarded as the most sensitive and specific imaging method
quetral ligament can also be identified regularly. On coronal for the detection of osteonecrosis (Fig. 59–12).
spin echo or gradient echo images, a normal scapholu-
nate interosseous ligament is typically seen as a thin or
triangular (delta shaped) structure of low signal intensity Improper Fracture Healing
traversing the space between the scaphoid and the lunate Although fractures may involve any of the carpal bones,
(Fig. 59–10). When visualized, a normal lunotriquetral fracture of the scaphoid is most common and may be
interosseous ligament has a similar appearance and extends followed by improper fracture healing (i.e., delayed union
between the lunate and the triquetrum. Variations in the and nonunion). Causes of improper fracture healing of
normal appearance of these ligaments include a linear the scaphoid bone include a delay in the initial diagnosis
shape and circular or linear regions of intermediate signal of the fracture, rotation or displacement at the fracture
intensity within them. site, inadequate immobilization, interposition of soft tissue
An abnormality of the scapholunate interosseous liga- in the fracture gap, and a tenuous blood supply. Routine
ment is suggested when it is elongated, appears incom- radiography provides some diagnostic information
plete, courses other than in a horizontal direction, or is not regarding delayed union or nonunion, although CT pro-
seen at all (Fig. 59–11). An abnormality of the lunotri- vides more definitive evidence of a persistent fracture
quetral interosseous ligament is suggested when it is line, especially if direct or reformatted coronal or sagittal
elongated, is incomplete, or extends in a direction other images are included.
than horizontal. Although successful visualization of a
lunotriquetral interosseous ligament is dependent on the
specific imaging parameters used, its nonvisualization on
MR images is not a reliable indicator of abnormality.
Volumetric gradient echo imaging provides thin con-
tiguous sections of the wrist (see Fig. 59–10). With this
method, the scapholunate and lunotriquetral interosseous
ligaments are generally well delineated. Nonvisualization
of either ligament, particularly the scapholunate interosseous
ligament, with volumetric imaging is strong evidence
that an abnormality exists.
Osteonecrosis
Osteonecrosis of the carpal bones affects three principal Figure 59–11. Intercarpal ligaments: communicating defect
sites: the lunate bone (i.e., Kienböck’s disease), the scaphoid of the scapholunate interosseous ligament. Coronal T1-weighted
bone (particularly its proximal portion after fracture of (TR/TE, 800/20) spin echo MR image shows discontinuity of
the waist of the bone), and, less commonly, the capitate the scapholunate interosseous ligament (arrow). The lunotriquetral
bone (typically its proximal portion, either idiopathically interosseous ligament is not seen.
A B
Figure 59–10. Intercarpal ligaments: three-dimensional Fourier transform (3DFT) gradient recalled MR
imaging. A, Normal scapholunate interosseous ligament. Coronal 3DFT (TR/TE, 60/11; flip angle, 10 degrees)
MR image shows the low signal intensity and linear morphology that characterize normal scapholunate
(arrow) and lunotriquetral (arrowhead) interosseous ligaments. The triangular fibrocartilage is also normal.
B, Communicating defect of the scapholunate interosseous ligament. Coronal oblique 3DFT (TR/TE,
60/10; flip angle, 30 degrees) MR image shows altered morphology (arrow) of the scapholunate interosseous
ligament. (A, Courtesy of S. K. Brahme, MD, La Jolla, Calif.)
Chapter 59 Internal Derangements of Joints 911
A B
Figure 59–12. Kienböck’s disease and nonunion of a scaphoid fracture. A, Coronal T1-weighted
(TR/TE, 800/20) spin echo MR image reveals low signal intensity throughout the lunate bone and in the
fracture gap of the scaphoid bone. B, Coronal T2-weighted (TR/TE, 2500/60) spin echo MR image shows
foci of high signal intensity (arrowhead) in the lunate bone. Fluid of high signal intensity (arrow) is evident
in a portion of the fracture gap in the scaphoid bone.
MR imaging shares many of the advantages of CT in ration between adjacent tendons in the carpal tunnel;
the diagnosis of fracture complications. High signal increased signal intensity, resulting from fluid in the
intensity in the fracture defect on T2-weighted spin echo enlarged tendon sheaths; and volar bowing, or convexity,
and certain gradient echo MR images is consistent with of the flexor retinaculum.
the presence of fluid, a sure sign of fracture nonunion
(see Fig. 59–12). Persistent low signal intensity on T1-
and T2-weighted spin echo MR images may indicate the Ulnar Tunnel Syndrome
presence of fibrosis at the fracture site. Marrow continuity Compression of the ulnar nerve as it traverses through
across the fracture line is strong evidence that fracture the ulnar tunnel, or Guyon’s canal, in the volar aspect of
healing has occurred. the wrist is well recognized (see Chapter 65). Causes of
such entrapment include masses, vascular injury, anatomic
variations, muscle hypertrophy, fracture, and hypertrophy
Carpal Tunnel Abnormalities
of the transverse carpal ligament. Although the MR imaging
Carpal Tunnel Syndrome abnormalities associated with ulnar tunnel syndrome are
As indicated in Chapter 65, carpal tunnel syndrome is a not well documented, findings similar to those of carpal
relatively common entrapment neuropathy that affects tunnel syndrome are likely.
the median nerve within the carpal tunnel in the palmar
aspect of the wrist. Clinical findings include paresthesias Abnormalities of the Extensor and Flexor
of the fingers in the distribution of the median nerve and Tendons and Tendon Sheaths
weakness and atrophy of the thenar muscles. Diagnostic
imaging plays a minor role in the assessment of carpal Tendinosis and Tenosynovitis
tunnel syndrome. Ultrasonography, CT, and MR imaging The MR imaging abnormalities of tendinosis and
have been used for this purpose. There are four consis- tenosynovitis include enlargement and increased signal
tent MR imaging findings in carpal tunnel syndrome: (1) intensity of the affected tendon and enlargement of its
swelling of the median nerve, best evaluated at the level sheath as a result of the accumulation of fluid or syn-
of the pisiform bone; (2) flattening of the median nerve, ovitis. De Quervain’s syndrome consists of tendinosis and
best evaluated at the level of the hamate bone; (3) palmar tenosynovitis of the abductor pollicis longus and extensor
bowing of the flexor retinaculum, best evaluated at the brevis tendons and sheaths in the first extensor com-
level of the hamate bone; and (4) increased signal partment. Involvement occurs at the level of or just proxi-
intensity of the median nerve on T2-weighted spin echo mal to the styloid process of the radius. Inflammation in
images (Fig. 59–13). The pattern of enlargement of the the second extensor compartment (which contains the
median nerve is variable; the nerve may be diffusely extensor carpi radialis longus and brevis tendons and
enlarged or focally enlarged, especially at the level of the sheaths) leads to the intersection syndrome, which is
pisiform bone. related to friction where the tendons in the first and second
Inflammation of the flexor tendon sheaths is considered compartments cross, or intersect. Similarly, involvement
an important and perhaps the most common cause of of the extensor carpi ulnaris tendon and sheath in the
carpal tunnel syndrome. The MR imaging abnormalities sixth extensor compartment at the level of the distal
of such tenosynovitis include enlargement of individual portion and styloid process of the ulna may be evident
tendon sheaths, as a result of effusion and increased sepa- (Fig. 59–14).
912 Section XVI Internal Derangement of Joints
A B
Figure 59–13. Carpal tunnel syndrome. A, Transaxial T2-weighted (TR/TE, 3000/66) fat-suppressed
fast spin echo MR image of the wrist at the level of the pisiform bone. Note the enlargement and increased
signal intensity in the median nerve (arrow). B, Transaxial image distal to (A) at the level of the hook of the
hamate bone. Although the signal intensity in the median nerve (arrow) is increased, the nerve is smaller at
this level than at the level of the pisiform bone.
A B
Figure 59–14. Tenosynovitis. Transaxial T1-weighted (TR/TE, 500/16) (A) and fat-suppressed, contrast-
enhanced T1-weighted (TR/TE, 550/16) (B) spin echo MR images of the wrist at the midmetacarpal level
show marked thickening and enhancement of the flexor tendons.
MR imaging can be used to identify a Stener lesion. The annular ligament is a thick band of fibrous tissue
On coronal MR images of the first metacarpophalangeal that attaches to the anterior and posterior margins of the
joint, a normal ulnar collateral ligament appears as a band radial notch of the ulna. It serves as a restraining liga-
of low signal intensity medial to the joint. The adductor ment that prevents withdrawal or inferior displacement
aponeurosis is often visible as a paper-thin band of low of the head of the radius from its socket.
signal intensity superficial to the ulnar collateral ligament It is convenient to divide the many muscles about the
and extending from the distal half of that ligament over elbow into four groups: posterior, anterior, lateral, and
the base of the proximal phalanx. A nondisplaced tear of medial. The muscles of the posterior group are the triceps
the ulnar collateral ligament appears as a discontinuity and the anconeus; those of the anterior group are the
of the ligament distally, without ligamentous retraction biceps brachii and brachialis; the lateral group of muscles
and with the adductor aponeurosis covering the distal includes the supinator and brachioradialis muscles and
end of the ligament. Displacement of the ulnar collateral the extensor muscles of the wrist and hand; and the medial
ligament, or the Stener lesion, is associated with proximal group of muscles includes the pronator teres, palmaris
retraction or folding of the ligament. The proximal margin longus, and flexors of the hand and wrist.
of the adductor aponeurosis may be seen to abut the folded Three major nerves are located in the elbow region.
ulnar collateral ligament, thereby creating a rounded The median nerve parallels the course of the brachial
region of low signal intensity that has been designated a artery, courses anterior to it in the cubital area, and
“yo-yo on a string” appearance (Fig. 59–17). descends farther between the superficial and deep heads
of the pronator teres muscle. The ulnar nerve is present
ELBOW on the posteromedial side of the elbow and passes in a
groove between the olecranon process of the ulna and the
Anatomy
medial epicondyle of the humerus. The radial nerve
The articulation about the elbow has three constituents: descends above the elbow between the brachialis and
(1) humeroradial, the area between the capitulum of the brachioradialis muscles and divides near the elbow into
humerus and the facet on the radial head; (2) humero- deep and superficial branches.
ulnar, the area between the trochlea of the humerus and
the trochlear notch of the ulna; and (3) superior
(proximal) radioulnar, the area between the head of the Tendon and Muscle Abnormalities
radius and the radial notch of the ulna and the annular Although uncommon, avulsions (and tears) of tendons
ligament. about the elbow are encountered. The tendons of the
A fibrous capsule completely invests the elbow. biceps brachii and the triceps are involved most commonly.
Lateral and medial collateral ligamentous complexes Routine radiographic features of such avulsions include
reinforce the fibrous capsule of the elbow (Fig. 59–18). joint effusion and bone fragmentation and displacement.
A B C
Figure 59–17. Gamekeeper’s thumb. A, Normal ulnar collateral ligament. Coronal T1-weighted (TR/TE,
500/20) spin echo MR image of the first metacarpophalangeal joint shows the normal ulnar collateral
ligament (arrow) as a band of low signal intensity. The adductor aponeurosis is not visible. B, Nondisplaced
tear of the ulnar collateral ligament. Coronal T1-weighted (TR/TE, 500/20) spin echo MR image of the
first metacarpophalangeal joint shows disruption of the ulnar collateral ligament (arrow) and a superficial
curvilinear structure of low signal intensity (arrowhead) representing the adductor aponeurosis. C, Displaced
tear of the ulnar collateral ligament (Stener lesion). Coronal T1-weighted (TR/TE, 500/20) spin echo MR
image of the first metacarpophalangeal joint reveals a yo-yo appearance. The adductor pollicis aponeurosis
(arrowheads) looks like a string holding a yo-yo—the balled-up, displaced ulnar collateral ligament (open
arrow). The thin region of low signal intensity (solid arrow) represents an avulsed bone fragment. (From
Spaeth HJ, Abrams RA, Bock GW, et al: Gamekeeper thumb: Differentiation of nondisplaced and displaced
tears of the ulnar collateral ligament with MR imaging. Work in progress. Radiology 188:553, 1993.)
Chapter 59 Internal Derangements of Joints 915
Figure 59–18. Elbow joint: ligamen-
tous anatomy. A, Medial aspect of the
elbow. B, Lateral aspect of the elbow.
(From Williams PL, Warwick R [eds]:
Gray’s Anatomy, 36th Br ed. Edinburgh,
Churchill Livingstone, 1980, p 431.)
Although other imaging methods, such as CT and ultra- on MR imaging sequences. A magic angle phenomenon,
sonography, may be used to delineate tendinous injuries, however, occasionally leads to regions of intermediate
MR imaging is most suited to this task. signal intensity within the tendons when MR sequences
Normal tendons about the elbow (and elsewhere) with short TE are used. Tendinous tears and avulsion are
appear as smooth, linear structures of low signal intensity associated with irregular and frayed contours and altered
916 Section XVI Internal Derangement of Joints
signal intensity of the tendons (Fig. 59–19). Complete as tennis elbow. Although the type of pathologic lesion
tears are accompanied by discontinuity and retraction of associated with chronic overuse syndromes of the elbow
the tendon, and partial tears are accompanied by some varies, partial tearing of fibers within the extensor tendons
remaining intact tendinous fibers. Alterations in signal (and, less commonly, the flexor tendons) from their epi-
intensity depend on the age of the injury, although high condylar attachments is typical. Regions of high signal
signal intensity within the tendon itself and the adjacent intensity on T2-weighted spin echo images in the tendon,
soft tissues and bone is commonly present on T2-weighted the soft tissue, and the bone may be evident (Fig. 59–20).
spin echo MR images. Tears of the tendon of the triceps
brachii muscle are uncommon, with complete tears domi-
Ligament Abnormalities
nating over partial tears. Rupture of the distal portion of
the tendon of the biceps brachii muscle constitutes less Ligamentous disruptions about the elbow can accompany
than 5% of all biceps tendon injuries. severe physical trauma (e.g., elbow dislocation) or can
Chronic overuse syndromes can also lead to tendinous occur as a response to less extensive acute trauma (e.g.,
alterations. Clinical and imaging abnormalities predominate valgus injury) or chronic stress (e.g., baseball pitching).
on the lateral side of the joint, in the region of the humeral Clinical findings include pain and tenderness in the medial
epicondyle and capitulum. This condition is often described aspect of the elbow, symptoms of ulnar nerve compression,
A B
Nerve Abnormalities
Entrapment and compression neuropathies about the elbow
may involve the ulnar, radial, or median nerve, with those
involving the ulnar nerve most common. Entrapment of
the ulnar nerve in the fibro-osseous tunnel posterior to
the medial epicondyle of the humerus results in the Figure 59–22. Entrapment neuropathy: cubital tunnel syn-
cubital tunnel syndrome. Typical causes of this syndrome drome. Transaxial T2-weighted (TR/TE, 2000/70) spin echo
are injury and progressive cubitus valgus deformity (tardy MR image, accomplished with fat suppression, shows increased
signal intensity in the ulnar nerve (arrow) within the cubital
ulnar nerve palsy); other causes include osteoarthritis, tunnel. The medial (m) and lateral (l) epicondyles of the humerus
rheumatoid arthritis, nerve subluxation, prolonged bed and the olecranon process (o) of the ulna are indicated. A joint
rest, anomalous muscles, and masses. Clinical manifes- effusion is present. (Courtesy of S. K. Brahme, MD, La Jolla,
tations include weakness of the flexor carpi ulnaris muscle, Calif.)
flexor digitorum profundus muscle of the fourth and fifth
fingers, and intrinsic hand muscles. MR imaging is the
preferred diagnostic modality, showing displacement or
shift of the ulnar nerve, soft tissue mass, and enlargement Bone Abnormalities
and increased signal intensity in the compressed nerve
(Fig. 59–22). Osteochondral Fracture and Osteochondritis
Dissecans
Osteochondritis dissecans about the elbow usually affects
the capitulum. This disorder differs from a developmental
alteration of the capitular ossification center known as
Panner’s disease. MR imaging of osteochondritis disse-
cans of the capitulum may be used to gain information
regarding the integrity of the adjacent articular cartilage,
the viability of the separated fragment, and the presence
or absence of associated intra-articular osseous and carti-
laginous bodies. The presence of joint fluid or granulation
tissue at the interface between the fragment and the
parent bone, manifested as increased signal intensity on
T2-weighted spin echo MR images, generally indicates
an unstable lesion (Fig. 59–23). MR arthrography per-
formed after the intra-articular injection of gadolinium
compounds may be advantageous. Computed arthroto-
mography provides similar information.
Chondroepiphyseal Injuries
Accurate radiographic diagnosis of elbow injuries in
young children is difficult because of the large number of
developing ossification centers, the normal irregularities
of bone in these centers, and the cartilaginous component
of these centers, which is not visible on routine radiographs.
Figure 59–21. Medial (ulnar) collateral ligament tear. Differentiation among the many Salter-Harris lesions of
Coronal fat-suppressed, fast spin echo (TR/TE, 4000/18) MR the growth plate may be impossible when standard radiogra-
image in a baseball pitcher shows disruption (arrow) of the ante- phy alone is performed. Because such differentiation has
rior band of the medial collateral ligament, with surrounding prognostic implications, a diagnostic role may exist for
edema and hemorrhage. imaging methods such as arthrography and MR imaging.
918 Section XVI Internal Derangement of Joints
Synovial Cyst
Para-articular synovial cysts that communicate with the
Figure 59–23. Osteochondritis dissecans: capitulum of the elbow joint are a recognized manifestation of rheumatoid
humerus. Coronal intermediate-weighted (TR/TE, 2000/20) spin arthritis, but theoretically they can occur with any process
echo MR image shows the osseous defect (arrow) containing an leading to elevated intra-articular pressure in the elbow.
ossified body (arrowhead). The integrity of the adjacent Arthrography, cystography, ultrasonography, CT, and MR
articular cartilage cannot be determined. (Courtesy of M.
Schweitzer, MD, Philadelphia, Pa.)
imaging are all effective in establishing the diagnosis and
determining whether the contents of the cyst have ruptured.
Synovial Abnormalities
Synovial Proliferation
Synovial proliferation in the elbow may accompany a variety
of disease processes, including rheumatoid arthritis, septic
arthritis, crystal deposition disorders, pigmented villo-
nodular synovitis, and idiopathic synovial osteochondro-
matosis. Accumulation of joint fluid leads to a characteristic
displacement of the extrasynovial fat pads of the elbow,
which can be recognized with routine radiography, CT, and
MR imaging. The finding lacks specificity and indicates
only the presence of an effusion.
Rheumatoid arthritis and other synovial inflammatory
disorders affecting the elbow lead to proliferation of the
synovial membrane and accumulation of joint fluid. The
signal intensity characteristics of the abnormal synovium
and fluid are similar on standard MR imaging sequences.
Intravenous administration of gadolinium compounds leads
to enhancement of the signal intensity of the inflamma-
tory tissue; it does not affect the signal intensity of fluid
on MR images obtained immediately after the injection.
Pigmented villonodular synovitis may involve the elbow,
and the diagnosis may be suggested by an appropriate
clinical history and typical radiographic abnormalities. Figure 59–24. Intra-articular osteocartilaginous bodies: MR
The deposition of hemosiderin in the affected synovial arthrography. Coronal T1-weighted (TR/TE, 950/13) spin
tissue produces regions of persistently low signal echo MR image obtained after the intra-articular injection of a
intensity on spin echo and, especially, gradient echo MR gadolinium compound and with fat suppression shows a body
images. This finding may also be observed in cases of (arrow) in the olecranon fossa.
Chapter 59 Internal Derangements of Joints 919
surface may be composed of cartilage alone or cartilage glenoid rim and adds an element of stability to the gleno-
and bone together. Free bodies in the elbow joint com- humeral articulation. A loose fibrous capsule arises from
monly migrate to dependent portions of the joint and to the circumference of the glenoid labrum or from the
sites of normal depressions in bone, particularly the neck of the scapula and inserts distally into the humerus.
olecranon fossa. In certain areas, the fibrous capsule is strengthened by its
Calcified bodies in the elbow joint can be detected by intimate association with the surrounding ligaments and
routine radiography; however, those that lodge in the tendons; with regard to tendons, the capsule is reinforced
olecranon fossa may be overlooked unless conventional above by the supraspinatus, below by the long head of the
tomography or CT is used. Noncalcified bodies are more triceps, anteriorly by the subscapularis, and posteriorly
difficult to detect, requiring arthrography, computed arthro- by the infraspinatus and teres minor. The tendons of the
tomography, or MR imaging. Of these techniques, com- supraspinatus, infraspinatus, teres minor, and subscapu-
puted arthrotomography is often preferred. Conventional laris form a cuff (the rotator cuff) that blends with and
MR imaging is relatively insensitive to the diagnosis of small reinforces the fibrous capsule.
calcified bodies because the signal void of these bodies is The coracohumeral ligament strengthens the upper
easily overlooked. Further, small noncalcified cartilagi- part of the capsule. It arises from the lateral edge of the
nous bodies are characterized by high signal intensity on coracoid process, extends over the humeral head, and
T2-weighted spin echo images, similar to the signal attaches to the greater tuberosity. Anteriorly, the capsule
intensity of joint fluid. MR arthrography (see Fig. 59–24) may thicken to form the superior, middle, and inferior
may be useful in the detection of small calcified or glenohumeral ligaments. These ligaments and the recesses
noncalcified bodies. formed between them are variable in configuration. Several
openings may be found in the fibrous capsule, although
they are variable in number and location. One or two ante-
SHOULDER rior perforations, or foramina, below the coracoid process
establish joint communication with the bursa behind the
Anatomy
subscapularis tendon, the subscapular “recess.” Although
Articular. In the glenohumeral joint, the articular surfaces another opening between the greater and lesser tuberosi-
of the glenoid and humerus are covered with hyaline ties has been described, allowing passage of the tendon
cartilage (Fig. 59–25). The cartilage on the humeral head and synovial sheath of the long head of the biceps brachii
is thickest at its center and thinner peripherally, whereas muscle, this tendon typically invaginates the synovial mem-
the reverse is true on the glenoid portion of the joint. A brane rather than piercing it. Another inconstant perforation
fibrocartilaginous structure, the labrum, attaches to the may exist posteriorly and allow communication of the
articular cavity and a bursa under the infraspinatus tendon.
Several bursae are located about the glenohumeral
joint; the most important of these is the subacromial
(subdeltoid) bursa, which lies between the deltoid muscle
and joint capsule (Fig. 59–26). It extends underneath the
acromion and the coracoacromial ligament. The subacro-
mial (subdeltoid) bursa is separated from the articular
cavity by the rotator cuff and does not communicate with
the joint unless the cuff has been perforated. The acromio-
clavicular joint also has a surrounding fibrous capsule.
Surrounding ligaments include the acromioclavicular
and coracoclavicular ligaments (Fig. 59–27).
A B
Figure 59–32. External subacromial impingement syndrome: MR imaging abnormalities. A, On a sagittal
oblique T1-weighted (TR/TE, 800/20) spin echo MR image, a subacromial enthesophyte (solid arrow)
containing marrow projects from the anterior surface of the acromion (a) toward the coracoid process (c).
Note its relationship to the coracoacromial ligament (open arrows) and supraspinatus tendon (arrowhead).
B, In a second patient, a coronal oblique intermediate-weighted (TR/TE, 2000/30) spin echo MR image
reveals the flattened contour and low signal intensity characteristic of a subacromial enthesophyte (arrows).
Also observe osteoarthritis of the acromioclavicular joint manifested as osteophytosis (arrowhead) and an
elevated position of the humeral head, indicative of a rotator cuff tear. The tear was demonstrated better on
other MR images (not shown).
Chapter 59 Internal Derangements of Joints 923
Internal Impingement corticosteroid injection, hypovascularity, or damage
produced by impingement.
Impingement related to coracoacromial arch and
glenohumeral joint instability has been recognized for
Clinical Abnormalities. Tears of the rotator cuff predomi-
many years. More recently, attention has been given to
nate in patients older than 40 years, in men, and in the
another form of impingement that is seen especially in
dominant arm. Shoulder pain, stiffness, and weakness are
athletes whose sports require overhead arm motion. In
common clinical manifestations, although patients may
these patients, when the arm is placed in abduction and
be entirely asymptomatic. Weakness of flexion, abduction,
external rotation (i.e., the throwing position), impinge-
and external rotation of the arm may be apparent. The
ment is found between the posterosuperior margin of the
clinical manifestations of rotator cuff disease may allow an
glenoid cavity and the undersurface of the tendinous
accurate diagnosis; however, in some cases, they simulate
insertions of the supraspinatus and infraspinatus muscles.
the findings of other disorders of the shoulder, such as
There are five characteristic sites of injury: (1) superior
adhesive capsulitis and calcific or noncalcific tendinosis.
portion of the labrum, (2) tendons of the rotator cuff, (3)
greater tuberosity of the humerus, (4) inferior gleno-
Radiographic Abnormalities. No radiographic findings are
humeral ligament complex or adjacent labrum, and (5)
superior glenoid bone. All five sites must be surveyed diagnostic of an acute rotator cuff tear that has occurred
when such patients are evaluated. Although standard in the absence of glenohumeral joint dislocation.
arthrography and CT allow analysis of some of these Radiographs obtained during active abduction of the
sites, MR imaging and MR arthrography are preferred. shoulder to 90 degrees from the horizontal or to the
maximum extent possible may reveal narrowing of the
coracoacromial distance in patients with rotator cuff dis-
ruption. With chronic tears of the rotator cuff, a number
Rotator Cuff Tear
of radiographic abnormalities may become apparent
Classification and Prevalence. A variety of methods can be (Fig. 59–33):
used to classify failure of the rotator cuff. One simple
1. Narrowing of the acromiohumeral space. This
method is to divide tears of the rotator cuff into acute and
interosseous space may measure less than the lower
chronic tears. A second method of classification relates to
limit of normal, 0.6 to 0.7 cm.
the depth of the rotator cuff tear. Full-thickness (or com-
plete) tears extend from the articular surface to the bursal 2. Reversal of the normal inferior acromial convexity. Ele-
surface of the cuff, and partial-thickness (or incomplete) vation of the humeral head leads to closer apposition
tears involve only the articular surface or the bursal
surface of the cuff. A tear within the substance of the
rotator cuff (intrasubstance tear) represents a special type
of incomplete tear. Massive tears are full-thickness tears
involving multiple tendons of the rotator cuff. Lesions of
the rotator cuff generally begin in the supraspinatus
tendon, and their extension into other components of the
rotator cuff is well established.
Partial-thickness tears of the rotator cuff are more
common than full-thickness tears, and those on the articular
side are probably slightly more common than those on
the bursal side. Intratendinous tears appear to be less fre-
quent than tears involving the articular or bursal portion
of the rotator cuff. Because no existing method allows the
detection of all intrasubstance tears, their true prevalence
is difficult to ascertain. The prevalence of rotator cuff dis-
ruption increases in the later decades of life. The inci-
dental discovery of chronic tears of the rotator cuff on chest
radiographs obtained for unrelated reasons is common.
of the humerus and the acromion and repeated trau- circular or linear collection of contrast material may be
matic insult to the acromion. Straightening and con- identified above the opacified joint cavity. The intact
cavity of the undersurface of the acromion may then superficial fibers of the rotator cuff prevent opacification
become evident. of the subacromial bursa. A false-negative arthrogram in
3. Cystic lesions and sclerosis of the acromion and humeral the presence of a partial tear of the rotator cuff may
head. Small cystic lesions surrounded by a thin rim of indicate that the tear is too small for recognition or that
sclerosis can be noted along the inferior aspect of the a fibrous nodule has occluded the defect.
acromion and within the greater tuberosity.
Ultrasonographic Abnormalities. The value of ultrasonogra-
Arthrographic Abnormalities. Standard arthrography phy in the assessment of the rotator cuff is discussed in
remains a popular technique for the diagnosis of some Chapter 6.
tears of the rotator cuff. Complete tears of the rotator cuff
are associated with abnormal communication between Magnetic Resonance Imaging Abnormalities. The normal
the glenohumeral joint cavity and the subacromial (sub- tendons of the supraspinatus, infraspinatus, teres minor,
deltoid) bursa (Fig. 59–34). Contrast material can be and subscapularis muscles usually appear as regions of low
identified within the bursa as a large collection superior signal intensity on all MR imaging sequences, in contrast
and lateral to the greater tuberosity and adjacent to the to the areas of high signal intensity on some MR imaging
undersurface of the acromion. Tears within the substance sequences that are characteristic of tears. Although these
of the cuff generally escape arthrographic detection. differences in signal intensity in normal and torn tendons
Tears involving the superior surface of the cuff are not of the rotator cuff are fundamental to the diagnosis of
demonstrated on glenohumeral joint arthrography; rarely, cuff disruption, regions of intermediate signal intensity
they may be seen with direct subacromial bursography. and inhomogeneity of signal intensity in intact tendons
Tears of the inferior surface of the rotator cuff can be of the rotator cuff are regularly encountered and create
diagnosed with arthrography. In these cases, an irregular some diagnostic difficulty. Specifically, regions of inter-
mediate and sometimes high signal intensity within the
outer portion of the supraspinatus tendon on T1- and
intermediate-weighted spin echo MR images have been
encountered. These areas relate to the magic angle phe-
nomenon, partial volume averaging, variations in normal
anatomy, and histologic findings of myxoid degeneration.
As a general rule, on T2-weighted spin echo MR images,
the signal intensity of nondisrupted tendons of the rotator
cuff, including the supraspinatus tendon, is equal to or
less than that on T1- and intermediate-weighted spin echo
MR images. When the signal intensity of the rotator cuff
tendons is higher on T2-weighted than on T1- and
intermediate-weighted spin echo MR images, tendon
disruption is usually present. Unfortunately, there are excep-
tions to this rule. Further, when fast spin echo sequences
are combined with fat suppression, a surprisingly high
signal intensity may be seen in tendons that are not torn.
The most definite feature of such tears is a tendinous
defect that is filled with fluid, granulation tissue, or both
(Fig. 59–35). These tears show high signal intensity on
short tau inversion recovery (STIR), T2-weighted spin
echo, and other fluid-sensitive MR sequences. A second
feature is retraction of the musculotendinous junction
beyond the limits of normal. When the first of these two
signs is present alone, or when both are present together,
the diagnosis of a full-thickness tear of the rotator cuff
can be made confidently. A number of other signs of a
full-thickness tear of the rotator cuff have been described
(Fig. 59–36), but none of them occurring in isolation is
diagnostic of such a tear. These signs include fluid in the
subacromial (subdeltoid) bursa or in the subcoracoid bursa,
fluid in the glenohumeral joint, loss of the peribursal fat
Figure 59–34. Full-thickness rotator cuff tear: glenohumeral
joint arthrography. Contrast material extends from the gleno-
plane, and muscle atrophy.
humeral joint through the rotator cuff into the subacromial Analysis of coronal oblique and sagittal oblique images
(subdeltoid) bursa. The inset reveals contrast material extending provides information regarding the extent of a full-thickness
from the glenohumeral joint through the rotator cuff into the rotator cuff tear. Initial tears in the supraspinatus tendon
subacromial bursa and from there into the acromioclavicular may extend anteriorly to involve the coracohumeral
joint. ligament and subscapularis tendon and extend posteriorly
Chapter 59 Internal Derangements of Joints 925
A B
A B
Figure 59–38. Partial-thickness rotator cuff tear: articular side of the supraspinatus tendon. Coronal
oblique intermediate-weighted (TR/TE, 2000/20) (A) and T2-weighted (TR/TE, 2000/70) (B) spin echo
MR images show two small regions of altered signal intensity (arrows in A) in the distal portion of the
supraspinatus tendon. One of these regions shows a further increase in signal intensity (arrowhead in B). No
fluid is present in the subacromial (subdeltoid) bursa (curved arrow in B). (From Kursunoglu-Brahme S,
Resnick D: Magnetic resonance imaging of the shoulder. Radiol Clin North Am 28:941, 1990.)
Chapter 59 Internal Derangements of Joints 927
apparent on T1- and intermediate-weighted spin echo MR
images and less evident on T2-weighted spin echo MR
images (Fig. 59–39). Regions of intense signal intensity
within the cuff tendons on T2-weighted spin echo MR
images, similar to the signal intensity of fluid, are not
compatible with the diagnosis of tendinosis or tendinopathy
and suggest the presence of a full-thickness or partial-
thickness tear.
Adhesive Capsulitis
Adhesive capsulitis is one of several terms applied to a
clinical condition characterized by severe restriction of
active and passive motion of the glenohumeral joint for
which no other cause can be documented.
Glenohumeral joint arthrography is the modality most
frequently used in the diagnosis and treatment of adhe-
sive capsulitis (Fig. 59–40). The diagnostic role of MR
imaging is not yet clear. Thickening of the joint capsule
adjacent to the medial cortex of the humerus has been
described as a finding of adhesive capsulitis. More specific,
however, is the presence of increased signal intensity in
pericapsular tissue on fluid-sensitive images and, in
particular, after intravenous gadolinium administration
(Fig. 59–41). Figure 59–40. Adhesive capsulitis: arthrography. Frontal
radiograph obtained after the injection of 5 mL of radiopaque
contrast material into the glenohumeral joint reveals a tight-
Glenohumeral Joint Instability appearing articulation with lymphatic filling (arrow). No
axillary pouch is seen.
Classification. Glenohumeral joint instability is generally
considered a symptomatic clinical situation characterized
by altered movement of the humeral head with respect to
the glenoid cavity. Methods of classification of gleno-
humeral joint instability are based on the degree and direc-
tion of instability, its chronology, and its pathogenesis.
With regard to the degree of instability, dislocations Reinforcing the capsule of this joint are the coracohumeral
or subluxations of the joint may be encountered. In dislo- ligament and the three glenohumeral ligaments (Fig. 59–43).
cation, no contact remains between the apposing surfaces Although the glenohumeral ligaments are inconstant and
of the glenoid cavity and the humeral head. With sub- variable in size, their importance in providing stability to
luxation, abnormal translation of the humeral head in the glenohumeral joint is undisputed. Of the three gleno-
relation to the glenoid cavity is evident; it is not accom- humeral ligaments, emphasis has been given to the inferior
panied by complete separation of the apposing articular glenohumeral ligament complex, particularly its anterior
surfaces, and it is usually greater than the small amount band, which is understood to be the primary static restraint
of translation of the humeral head that represents normal against anterior instability when the arm is in an abducted
range of motion. Subluxation of the glenohumeral joint and externally rotated position.
is transient and often momentary. Dislocation and sub- No single or essential lesion appears to be the basis of
luxation of the glenohumeral joint may occur in several anterior or posterior instability of the joint. Identification
different directions: anterior, posterior, superior, and of a detached portion of the glenoid labrum (Bankart lesion),
inferior. a compression fracture of the posterolateral surface of the
With regard to the chronology of instability, acute humeral head (Hill-Sachs lesion), a deficiency of one or
dislocations are defined as those seen within the first 24 more of the glenohumeral ligaments, capsular laxity,
to 48 hours, and chronic dislocations are defined as those disruption of one or more tendons of the rotator cuff, or
evaluated after this time. Dislocations and subluxations of detachment or lengthening of the subscapularis muscle as
the glenohumeral joint may also be categorized according the primary cause of shoulder instability is an oversim-
to their pathogenesis. They may be traumatic or atraumatic, plification of a complex process.
congenital or acquired, and voluntary or involuntary.
The acronym TUBS describes patients with traumatic Clinical Abnormalities. The clinical abnormalities related
instability who usually have unilateral involvement, typically to instability of the glenohumeral joint are variable and
have a Bankart lesion, and often require surgery; AMBRI influenced by its atraumatic or traumatic pathogenesis
refers to patients with atraumatic instability who have and by its acute or chronic nature. Many patients recall
multidirectional instability, often with bilateral involvement, an experience in which the humeral head felt displaced,
and may respond to a rehabilitation program or require whether initiated by trauma or not. Fixed dislocations of
inferior capsular shift (Table 59–2). the glenohumeral joint are usually identifiable during
physical examination, particularly in a patient with anterior
Cause and Pathogenesis. Although developmental factors dislocation of the glenohumeral joint. Posterior disloca-
may lead to congenital instability of the glenohumeral joint, tions of this joint are associated with more subtle but
trauma is the leading cause of subluxation and dislocation definite clinical manifestations, including fixed internal
of the glenohumeral joint. Indirect forces are more rotation of the affected arm with limited external rotation
common than direct forces as causes of glenohumeral and elevation. Complications of anterior or posterior
joint instability. Many structures, such as the components dislocations, such as neurovascular injury, may provide
of the rotator cuff, the glenoid labrum, the glenohumeral additional clinical findings. Although provocative tests
ligaments, the joint capsule and supporting musculature, may be used during physical examination to establish the
and the coracoacromial arch, contribute to glenohumeral occurrence of subluxation of the glenohumeral joint,
joint stability (Fig. 59–42). Both static (passive) and imaging examinations are fundamental to an accurate
dynamic (active) constraints are believed to contribute to diagnosis.
shoulder stability. Static constraints include the bone
configuration of the glenohumeral joint and the capsule Radiographic and Arthrographic Abnormalities. The routine
and ligamentous complex that surrounds the shoulder; radiographic abnormalities associated with dislocation of
dynamic constraints include the muscle and tendon units the glenohumeral joint are discussed in Chapter 57. In
that surround the glenohumeral joint, particularly the addition to confirming an abnormal position of the humeral
muscles and tendons of the rotator cuff. head with respect to the glenoid cavity, conventional
The capsule of the glenohumeral joint is large, loose, radiographs are useful in the identification of bone injuries
and redundant, thereby allowing great range of motion. that may occur during dislocation. Foremost among
these injuries are Hill-Sachs lesions of the humerus and
Bankart-type lesions of the anterior portion of the gle-
noid rim. Standard arthrography also has a limited role in
the assessment of glenohumeral joint instability and is
TABLE 59–2
discussed in Chapter 5.
Clinical Categories of Glenohumeral Joint Instability
Computed Arthrotomographic Abnormalities. Computed
AMBRI TUBS AIOS arthrotomography is especially useful for assessing the
Atraumatic Traumatic Acquired shoulder in patients who are unable to undergo MR
Multidirectional Unilateral Instability imaging. Abnormalities of the glenoid labrum depicted
Bilateral Bankart lesion Overstress on computed arthrotomography include foreshortening
Rehabilitation Surgery Surgery or thinning or contrast imbibition along its free margin
Inferior capsular shift (Fig. 59–44). The labrum may also be completely detached
(a true Bankart lesion). An osseous Bankart-type lesion is
Chapter 59 Internal Derangements of Joints 929
Figure 59–42. Capsular complex of the glenohumeral joint, rotator cuff, and supraspinatus outlet: normal
anatomy. Right, A right shoulder is sketched as though all the muscles have been dissected away to reveal the
glenohumeral, coracohumeral, coracoacromial, and coracoclavicular ligaments. The tendons of the long
head of the biceps brachii muscle, the subacromial (subdeltoid) bursa, and the subscapularis bursa are also
depicted. Left, The relationships of these structures in the sagittal plane are emphasized. In numerical order,
the structures are the following:
2. Coracoid process
4. Glenoid cavity
12. Supraspinatus muscle and tendon
13. Infraspinatus muscle and tendon
14. Subscapularis muscle and tendon
15. Teres minor muscle
23. Tendon of the long head of the biceps brachii muscle
27. Superior glenohumeral ligament
28. Middle glenohumeral ligament
29. Inferior glenohumeral ligament
34. Coracohumeral ligament
35. Subscapularis bursa
38. Trapezoid ligament
39. Conoid ligament
40. Coracoacromial ligament
41. Subacromial bursa
42. Subdeltoid bursa
43. Acromioclavicular joint and ligaments
53. Suprascapular notch
54. Superior transverse scapular ligament
(Courtesy of B. O. Sewell, MD, San Diego, Calif.; from Petersilge CA, Witte DH, Sewell BO, et al: Normal
regional anatomy of the shoulder. Magn Reson Imaging Clin N Am 1:1, 1993.)
typically visualized as elevation of a small sliver of bone arthrography) allows visualization of the anterior and pos-
and irregularity of the adjacent glenoid rim. A depression terior capsular insertions of the glenohumeral ligaments,
along the posterolateral aspect of the humeral head is as well as the coracohumeral ligament. In cases of anterior
indicative of a Hill-Sachs lesion. glenohumeral joint instability, the capsular reflections
Distention of the glenohumeral joint accomplished may appear lax or distorted, with either loss or thickening
during computed arthrotomography (and during MR of the anterior subscapular soft tissues (Fig. 59–45). With
930 Section XVI Internal Derangement of Joints
A B C
ciated with anterior glenohumeral joint instability occur the base of the biceps tendon and crosses the subscapu-
at the 3 to 6 o’clock positions. laris tendon to insert on the humerus. No anterosuperior
Labral Variations. The superior portion of the labrum labral tissue is present between this attachment site and
may normally have a tight (i.e., slablike) or loose (i.e., the midglenoid notch. This anomaly, which may be present
meniscus-like) attachment to the glenoid margin. If the in 2% to 5% of persons, may simulate labral detachment
attachment is loose, a potential space or recess exists (Fig. 59–48).
between it and the glenoid bone. This space is designated Osseous Abnormalities. A variety of osseous injuries
the sublabral recess, or sulcus, and it may fill with con- accompany glenohumeral joint instability and may be
trast agent during MR arthrography. This opacification detected with MR imaging. These injuries include frac-
simulates the appearance of a detached labrum, a type of tures of the anterior or posterior portion of the glenoid
SLAP lesion. This normal variant is encountered in about rim, Hill-Sachs and trough lesions, and avulsion fractures
10% of normal persons. This foramen is considered a of the lesser or greater tuberosity of the humerus
nonsignificant finding, although its imaging appearance (Fig. 59–49). The presence of a normal groove in the
may simulate that of pathologic labral conditions. It posterolateral aspect of the proximal humeral metaphysis
extends inferiorly no lower than the 3 o’clock position can lead to difficulty in the diagnosis of a Hill-Sachs lesion
(Fig. 59–47). Almost invariably, a sublabral recess is also on transaxial CT scans and MR images. This groove,
present. A relatively uncommon variation of capsulolabral however, is located lower than a Hill-Sachs lesion. The
anatomy designated the Buford complex is characterized latter is almost universally apparent on the first two or
by a cordlike middle glenohumeral ligament that origi- three transaxial images through the superior portion of
nates directly from the superior portion of the labrum at the humeral head (with a 3- or 5-mm slice thickness).
932 Section XVI Internal Derangement of Joints
A B
Figure 59–49. Posterior glenohumeral joint instability. A, Transaxial SPGR (TR/TE, 45/15; flip angle,
20 degrees) MR image shows posterior subluxation of the humeral head, irregularity of the posterior glenoid
rim (solid arrow), and a trough fracture (open arrow) involving the anterior surface of the humeral head.
B, Transaxial CT scan at a slightly lower level in the same patient confirms posterior displacement of the
humeral head, a fracture of the posterior glenoid region (solid arrow), and a trough fracture of the humeral
head (open arrow). (Courtesy of M. Schweitzer, MD, Philadelphia, Pa.)
Labroligamentous Abnormalities. Numerous labroligamen- ligament complex. Failure of this complex may occur at
tous abnormalities have been described, most often in asso- its glenoid insertion site (approximately 70% to 75% of
ciation with glenohumeral joint instability (Table 59–3). cases), at its humeral insertion site (5% to 10% of cases),
These abnormalities can be broadly divided into two or within its substance (15% to 20% of cases). Lesions
categories: those that are associated with such instability, associated with glenoid-sided failure are the Bankart lesion
and those that are generally not. In cases of anterior (cartilaginous or both bony and cartilaginous), anterior
glenohumeral joint instability, abnormalities are usually labroligamentous periosteal sleeve avulsion, and Perthes’
related to the anterior band of the inferior glenohumeral lesion (Fig. 59–50). Lesions associated with humeral
Chapter 59 Internal Derangements of Joints 933
TABLE 59–3 failure are humeral avulsion of the glenohumeral ligament
lesion and its bony counterpart. Failure of this ligament
Labroligamentous Lesions at both its glenoid and humeral insertion destabilizes both
ends of the anterior band of the inferior glenohumeral
Unstable Stable complex (floating avulsion of the inferior glenohumeral
Glenoid failure (70–75%) Labral ligament complex). Similar lesions related to the poste-
Bankart lesion Flap/bucket-handle tear rior band of this ligament complex have been associated
ALPSA lesion Labral—cartilage with posterior glenohumeral joint instability.
Perthes’ lesion GLAD lesion The Bankart lesion represents an avulsion of the
Capsular failure (15–20%) Labral—tendon labroligamentous complex from the anteroinferior portion
Tear/laxity SLAP lesion of the glenoid bone. The periosteum of the scapula is
Humeral failure (5–10%) lifted and disrupted (Fig. 59–51). The Bankart lesion is
HAGL lesion located at the 3 to 6 o’clock positions. In some cases,
BHAGL lesion
failure results in avulsion of a piece of bone, producing
Humeral and glenoid failure the bony Bankart-like lesion. This piece arises from the
(floating AIGHL)
anteroinferior aspect of the glenoid rim. CT may be
effective in showing this lesion. Recurrent unidirectional
AIGHL, alvulsion of the inferior glenohumeral ligament complex; ALPSA, anterior instability of the glenohumeral joint may be
anterior labroligamentous periosteal sleeve avulsion; BHAGL, bony humeral
avulsion of the glenohumeral ligament; GLAD, glenolabral articular disruption; associated not only with a classic Bankart lesion (avulsion
HAGL, humeral avulsion of the glenohumeral ligament; SLAP, superior labral, of the anterior labroligamentous structures from the
anterior and posterior lesion.
A B
C D E
Figure 59–50. Anterior glenohumeral joint instability: lesions associated with failure of the anterior band
of the inferior glenohumeral ligament complex at its glenoid attachment. A, Normal. Structures depicted
are the anterior band of the inferior glenohumeral complex (solid arrow), labrum (open arrow), and scapular
periosteum (arrowhead). B, Bankart lesion. This lesion represents an avulsion of the labroligamentous com-
plex, with lifting and disruption of the scapular periosteum. C, Acute anterior labroligamentous periosteal
sleeve avulsion (ALPSA) lesion. This lesion represents an avulsion of the labroligamentous complex, with
lifting but not disruption of the scapular periosteum. D, Chronic ALPSA lesion. This lesion represents an
acute ALPSA lesion that is followed by anteromedial displacement of the labrum and the formation of scar
tissue. E, Perthes’ lesion. This lesion represents a nondisplaced labroligamentous avulsion, with lifting but
not disruption of the scapular periosteum. (Courtesy of N. Lektrakul, MD, San Diego, Calif.)
934 Section XVI Internal Derangement of Joints
A B
Figure 59–51. Anterior glenohumeral joint instability: Bankart and anterior labroligamentous periosteal
sleeve avulsion (ALPSA) lesions. A, Transaxial fat-suppressed fast spin echo (TR/TE, 2500/15) MR arthro-
graphic image shows the avulsed and displaced labroligamentous complex (arrow) characteristic of ALPSA
lesion. B, In a different patient, transaxial intermediate-weighted (TR/TE, 1800/30) spin echo MR image
shows anteromedial displacement of the detached labrum (solid arrow), with lifting (open arrow) but not
disruption of the scapular periosteum. (Courtesy of C. Ho, MD, Stanford, Calif.)
anterior glenoid rim) but also with avulsion of the portion of the labrum. Type II lesions (40%) are charac-
periosteal sleeve of the anterior portion of the scapula terized by separation of the superior portion of the glenoid
(see Fig. 59–51). This lesion differs from a Bankart lesion, labrum and the tendon of the long head of the biceps
in that the anterior scapular periosteum does not rupture. brachii muscle from the glenoid rim. Type III lesions
Perthes’ lesion, another variation of a Bankart lesion, is (30%) are bucket-handle tears of the superior portion of
characterized by a labroligamentous avulsion injury the labrum without involvement of the attachment site of
without displacement of the labrum and with stripping the tendon of the long head of the biceps brachii muscle.
but not disruption of the scapular periosteum. Type IV lesions (15%) are characterized by bucket-
The labroligamentous injuries associated with poste- handle tears of the superior portion of the labrum that
rior glenohumeral joint instability have been less well extend into the biceps tendon. Additional types of SLAP
documented than those associated with anterior instability. lesions have also been described.
Posterior labral lesions are less diagnostic of posterior Detection of a SLAP lesion requires analysis of both
instability than are anterior labral lesions in the setting of coronal oblique and transaxial MR images. Type I lesions
anterior instability. Posterior labral tears and detachments, are characterized by irregularity in labral contour and a
ligamentous injuries, and capsular tears or stripping may slight increase in its signal intensity. Type II lesions are
occur, and the resulting pathologic variations may resemble associated with a globular region of high signal intensity
those seen in anterior glenohumeral joint instability. interposed between the superior part of the glenoid labrum
and the superior portion of the glenoid fossa. Type III
lesions are accompanied by superior labral tears, and type
Superior Labral, Anterior and Posterior Lesion
IV lesions are associated with diffuse high signal intensity
In athletes subjected to repetitive overhead use of the in the superior portion of the labrum and in the proximal
arm, an injury to the superior portion of the glenoid part of the biceps tendon. The coronal plane is most
labrum may result from sudden forced abduction of the sensitive in the diagnosis of SLAP lesions. MR arthrography
arm. This injury may be related to excessive traction may be more sensitive and specific (Fig. 59–52).
caused by a sudden pull of the tendon of the long head of
the biceps brachii muscle and is frequently referred to as
Perilabral Ganglion Cyst
a SLAP lesion or tear. The lesion begins posteriorly and
extends anteriorly, terminates before or at the midgle- Certain tears of the anterior or posterior portion of the
noid notch, and includes the anchor of the biceps tendon labrum are accompanied by the development of perilabral
to the glenoid labrum. cysts or ganglia. The pathogenesis of these cystic lesions
The SLAP lesion was initially divided into four types. is not certain, although similar lesions have been described
Type I lesions (approximately 10% of all SLAP lesions) in association with meniscal tears of the knee. In the
are associated with degenerative fraying of the superior shoulder, such perilabral cysts or ganglia may extend into
Chapter 59 Internal Derangements of Joints 935
Biceps Tendon Abnormalities
Anatomic Considerations. The tendon of the long head of
the biceps brachii muscle, with rare exception, arises from
the supraglenoid tubercle or the superior portion of the
glenoid labrum or from both structures, and it extends
obliquely across the top of the humeral head into the
intertubercular sulcus or groove. This groove is bounded
by the tuberosities of the humerus. The tendon emerges
from the joint at the lower portion of the groove, almost
surrounded by a sheath that communicates directly with
the joint. The subscapularis tendon inserts, in part, into
the lesser tuberosity of the humerus. Disruption of the
subscapularis tendon leaves the biceps tendon unhin-
dered to slip medially over the lesser tuberosity into the
glenohumeral joint.
A B
Figure 59–53. Perilabral cyst. A, Transaxial fast T1-weighted (TR/TE, 500/15) fat-suppressed MR
arthrographic image shows a small tear of the posterior superior labrum (large arrow). A small perilabral cyst
(small arrows) is not readily apparent. B, Coronal T2-weighted (TR/TE, 3800/105) fat-suppressed MR
image shows the perilabral cyst (small arrow), as well as its connection to the posterior labrum (large arrow).
936 Section XVI Internal Derangement of Joints
TABLE 59–4
Terms Applied to Normal Variations or Lesions of the Labroligamentous Complex and
Surrounding Structures of the Shoulder
Term Description
Hill-Sachs lesion Fracture of the posterior surface of the humeral head, indicative of previous anterior glenohumeral joint
dislocation
Trough lesion Fracture of the medial surface of the humeral head, indicative of previous posterior glenohumeral joint
dislocation
Bankart lesion Injury of the anteroinferior portion of the glenoid labrum, indicative of previous anterior glenohumeral
joint dislocation
Sublabral foramen Normal variation in which a foramen is identified between the anterosuperior portion of the glenoid
labrum and the anterior glenoid rim
Buford complex Normal variation in which a cordlike middle glenohumeral ligament is associated with absence of the
anterosuperior portion of the glenoid labrum
GLOM sign Designation for a glenoid labral ovoid mass (GLOM), indicative of an injury with avulsion of a portion of
the anterior aspect of the glenoid labrum
ALPSA lesion Designation for anterior labroligamentous periosteal sleeve avulsion (ALPSA) associated with recurrent
anterior glenohumeral joint dislocation caused by incompetence of the anterior portion of the inferior
glenohumeral ligament complex
Perthes’ lesion Designation for avulsion of the anteroinferior portion of the glenoid labrum without displacement and
with stripping of the periosteal membrane
HAGL lesion Designation for humeral avulsion of the glenohumeral ligament (HAGL); usually seen in older patients
and associated with recurrent anterior glenohumeral joint instability and a tear of the subscapularis
tendon
BHAGL lesion Designation for a bony HAGL (BHAGL) lesion that is associated with an avulsion fracture of the
humerus
GLAD lesion Designation for glenolabral articular disruption (GLAD), which is associated with a tear of the
anteroinferior portion of the labrum and erosion of the articular cartilage of the glenoid fossa and not
associated with anterior glenohumeral joint instability
SLAP lesion Designation for superior labral, anterior and posterior (SLAP) tear, often seen in athletes involved in
sports requiring repetitive overhead use of the arm and varying in severity but involving the superior
portion of the glenoid labrum and, sometimes, the biceps anchor
Bennett’s lesion Enthesophyte that arises from the posteroinferior portion of the glenoid rim, often seen in baseball
pitchers and probably arising at the site of insertion of the posterior band of the inferior glenohumeral
ligament complex
Osteochondritis A lesion of the glenoid cavity related to an impaction force.
dissecans
GARD lesion Designation for glenoid articular rim divot (GARD) indicative of a lesion involving the posterior glenoid
rim, glenoid cavity, or both and related to an impaction injury
Perilabral ganglion Ganglion cyst arising adjacent to the glenoid labrum and often associated with a labral tear
cyst
with massive tears of the rotator cuff. Medial displace- tubercular groove but is typically located medial to the
ment of the biceps tendon may also accompany isolated lesser tuberosity. On MR imaging, the dislocated tendon
tears or avulsion of the subscapularis muscle or tendon. is seen medial to the bicipital groove, particularly on
Factors predisposing to dislocation of the biceps tendon transaxial images (Fig. 59–54). The tendon may appear
include anomalies and dysplastic changes of the inter- thickened and may contain abnormal increased signal
tubercular groove, degenerative and attritional changes intensity, and fluid may be seen in the surrounding tendon
in the tendon itself, and capsular and ligamentous (i.e., sheath. Disruption of one or more components of the rotator
coracohumeral ligament) abnormalities. cuff and the coracohumeral ligament may also be evident.
Ultrasonography, arthrography, and MR imaging may Intra-articular entrapment of the tendon of the long head
be used in the assessment of medial dislocation of the biceps of the biceps brachii muscle may be documented; access
tendon. The arthrographic diagnosis of this condition is to the joint is provided by disruption of the subscapularis
established by direct visualization of a bicipital tendon tendon or by detachment of this tendon from the lesser
within its opacified sheath that is not located in the inter- tuberosity of the humerus (Fig. 59–55).
Chapter 59 Internal Derangements of Joints 937
(extracapsular portion), although intracapsular tears of
the bicipital tendon are also encountered. The diagnosis
is usually obvious on clinical examination and requires no
further diagnostic tests. Partial tears of the biceps tendon,
however, may be more subtle, and in these cases, ultra-
sonography, arthrography, and MR imaging may be useful.
Entrapment Neuropathies
Several entrapment neuropathies occurring about the
shoulder are discussed in Chapter 65, but only three of
them are summarized here because of the importance of
MR imaging in their diagnosis.
Brachial Neuritis
Acute brachial neuritis, also known as Parsonage-Turner
syndrome, is a painful neuromuscular disorder associated
with weakness of the musculature about the shoulder. Its
cause is not known, although the syndrome may be related
to a viral neuritis. Most commonly, the involved shoulder
muscles are those innervated by the suprascapular nerve,
but additional muscles may also be affected, such as those
innervated by the axillary nerve. Thus, the supraspinatus,
infraspinatus, deltoid, and teres minor muscles, in various
combinations, may be abnormal when studied with MR
imaging. Denervation edema and, later, atrophy are observed.
The major differential diagnostic considerations are the
entrapment neuropathies listed in Table 59–5.
Figure 59–56. Suprascapular nerve: normal anatomy. The
suprascapular nerve (SN) adjacent to the suprascapular artery
(SA) passes beneath the superior transverse scapular ligament HIP
(STSL) in the suprascapular notch and then runs deep to the
supraspinatus muscle (SS) and the inferior transverse scapular
Anatomy
ligament (ITSL) to pass through the spinoglenoid notch. The Articular. The femoral head is covered with articular
infraspinatus muscle (IS), teres minor muscle (TMi), teres cartilage, although a small area on its surface is devoid of
major muscle (TMa), and long head of the triceps muscle (TR) cartilage, where the ligament of the head of the femur, or
are also indicated. The quadrilateral space (QS) is shown. The ligamentum teres, attaches (Fig. 59–59). The lunate
axillary nerve and the posterior humeral circumflex artery traverse
this space. (Modified from Fritz RC, Helms CA, Steinback LS,
surface is covered with articular cartilage; the floor of the
et al: Suprascapular nerve entrapment: evaluation with MR acetabular fossa within this surface does not contain car-
imaging. Radiology 182:437, 1992.)
A B
Figure 59–57. Suprascapular nerve: entrapment related to ganglion cysts. A, Transaxial MPGR (TR/TE,
383/12; flip angle, 15 degrees) MR image reveals a ganglion cyst of high signal intensity adjacent to the
posterior margin of the glenoid cavity. Observe a tear in the adjacent labrum (arrow). B, In a different patient
with documented suprascapular nerve palsy, coronal T2-weighted (TR/TE, 2000/80) spin echo MR image
at the level of the supraspinatus muscle shows changes of denervation in the supraspinatus muscle (asterisk),
with diffuse increased signal intensity.
Chapter 59 Internal Derangements of Joints 939
tilage but has a fibroelastic fat pad covered with synovial
membrane. A fibrous capsule encircles the joint and much
of the femoral neck. The capsule attaches proximally to the
acetabulum, labrum, and transverse ligament of the acetabu-
lum. Distally, it surrounds the femoral neck (Fig. 59–60).
The fibrous capsule is strengthened by surrounding
ligaments, including the iliofemoral, pubofemoral, and
ischiofemoral ligaments. The acetabular labrum, the fibro-
cartilaginous rim about the acetabulum, is firmly attached
to the bony rim and transverse ligament, is triangular on
cross section, and has a free edge or apex that forms a
smaller circle that closely embraces the femoral head.
The iliopsoas bursa is the largest and most important
bursa about the hip. It may extend proximally and com-
municates with the joint space in approximately 15% of
normal hips through a gap between the iliofemoral and
Figure 59–58. Quadrilateral space syndrome. Coronal oblique pubofemoral ligaments.
intermediate-weighted (TR/TE, 2000/25) spin echo MR image
shows selective atrophy with fatty replacement of the teres Vessels and Nerves. The major arterial supply of the
minor muscle (arrows). The infraspinatus muscle (13), deltoid adult femoral head originates from the medial and lateral
muscle (16), teres major muscle (17), and long (24) and lateral
(25) heads of the triceps muscle are identified and are not
femoral circumflex arteries, which are branches of either
involved. The humeral diaphysis (7) is also seen. (Courtesy of the femoral artery or the deep femoral artery. The con-
W. Glenn, MD, Long Beach, Calif.) tribution to the blood supply of the femoral head pro-
vided by the artery of the ligamentum teres is variable.
TABLE 59–5
Muscle Involvement in Neuropathies of the Shoulder
+, may be involved.
–, not involved.
A B Synovial Abnormalities
A B
C
942 Section XVI Internal Derangement of Joints
MR imaging provides information regarding the extent adults who possess this aperture, or creation of a commu-
of synovial disease in the hip that is not available on nicating pathway in adults who do not, is an expected
routine radiographs (Fig. 59–64). Abnormal collections consequence of virtually any disease process of the hip
of joint fluid may be detected. As in other locations, such that leads to an elevation in intra-articular pressure
as the knee, the differentiation of synovial inflammatory (Fig. 59–65).
tissue and joint effusion by MR imaging may require the Suspected iliopsoas bursal distention should be evaluated
intravenous administration of gadolinium compounds. by ultrasonography. If a nonpulsatile fluid collection
without Doppler evidence of flow is demonstrated, this
technique can be used for diagnostic aspiration of its con-
Synovial Cyst tents. Fluid analysis should distinguish a synovial cyst or
With any process of the hip that leads to an elevation in iliopsoas bursitis from a lymphocele, abscess, or hematoma.
intra-articular pressure, escape of fluid from the joint Subsequent injection of contrast material into the bursa
through a number of anatomic pathways serves to decom- or cyst may opacify the hip joint and thus confirm the
press the articulation. Typically, the fluid passes into a diagnosis; if not, hip arthrography, CT, or MR imaging
surrounding synovial sac, and its distention can be seen may be desirable to delineate potential articular commu-
or palpated. These synovial cysts can also be assessed nication if surgery is being contemplated.
with imaging methods. Of all the potential locations of
synovial cysts about the hip, the iliopsoas bursa deserves
Iliopsoas Bursitis
special emphasis.
The synovium lining the iliopsoas bursa can be involved
Iliopsoas Bursal Distention. Communication of the hip and in a variety of processes, including rheumatoid arthritis
iliopsoas bursa in the presence of intra-articular diseases and infection. CT, MR imaging, and ultrasonography are
such as osteoarthritis, rheumatoid arthritis, pigmented the best techniques for evaluating an inflamed and dis-
villonodular synovitis, infection, calcium pyrophosphate tended iliopsoas bursa. Afflictions of other bursal cavities
dihydrate crystal deposition disease, and idiopathic syn- about the hip and pelvis can be similarly evaluated
ovial (osteo)chondromatosis may lead to bursal enlarge- (Fig. 59–66).
ment and produce a mass in the ilioinguinal region that
simulates a hernia and causes obstruction of the femoral
vein. The iliopsoas bursa communicates with the hip joint Bone Abnormalities
via an aperture ranging in diameter from 1 mm to 3 cm, Three conditions are important in any discussion of hip
and enlargement of this channel in the 15% of normal disorders: osteonecrosis of the femoral head, transient bone
A B
A B
Figure 59–68. Transient bone marrow edema. Coronal T1-weighted (TR/TE, 500/15) (A) and fat-
suppressed fast T2-weighted (TR/TE, 4000/90) (B) spin echo MR images reveal characteristic features of
transient bone marrow edema. In (A), note the low signal intensity in the marrow of the left femoral head
and neck, with normal signal intensity in the adjacent acetabulum. The opposite femur and acetabulum are
also normal. In B, note the high signal intensity in the involved marrow in the left femur and a joint effusion.
The altered signal intensity extends to the base of the femoral neck.
Miscellaneous Abnormalities
Although MR imaging is useful in the assessment of many
processes involving the hip and adjacent bones and soft
tissues, some conditions are better evaluated with other
imaging methods. Intra-articular bodies in the hip (and
in other joints) may escape detection when MR imaging
is used alone. Such bodies, which commonly localize in
the region of the acetabular fossa, are detected more
easily with routine radiography supplemented with CT
or arthrography. Osteoid osteomas are better evaluated
with CT (Fig. 59–70).
A B
Figure 59–76. Synovitis: MR imaging with and without intravenous gadolinium administration in a
patient with rheumatoid arthritis. A, Coronal T1-weighted (TR/TE, 600/20) spin echo MR image shows
distention of the knee with fluid or pannus of inhomogeneous low signal intensity (arrows). B, Identical T1-
weighted (TR/TE, 600/20) spin echo MR image obtained immediately after the intravenous administration
of a gadolinium compound reveals pannus of high signal intensity (solid arrows) and joint fluid of low signal
intensity (open arrows). (Courtesy of S. K. Brahme, MD, La Jolla, Calif.)
peripheral attachment and, posteriorly, is separated from The posterior horn of the medial meniscus inserts
the capsule by the popliteus tendon and its sheath. The directly anterior to the tibial insertion of the posterior cru-
shape of the medial meniscus is semicircular, and its width ciate ligament, and the insertion site of the anterior horn
is greater posteriorly than anteriorly. The width of its of the lateral meniscus is adjacent to the tibial insertion of
central portion is variable. Peripherally, the medial menis- the anterior cruciate ligament. Some of the tibia-inserting
cus is firmly attached to the joint capsule, particularly in fibers of the anterior cruciate ligament are intimate with the
its midportion, in the region of the medial (tibial) col- insertion site of the anterior horn of the medial meniscus.
lateral ligament. The tibial attachment of the meniscus is The meniscofemoral ligaments are accessory ligaments
often referred to as the coronary ligament. of the knee that extend from the posterior horn of the
Chapter 59 Internal Derangements of Joints 951
Figure 59–82. Intra-articular
ganglion cyst. A, Alar folds. Sagittal
MPGR (TR/TE, 550/25; flip angle,
25 degrees) MR image shows a
septated ganglion cyst located
anteriorly and extending into the
infrapatellar fat body. B, Posterior
cruciate ligament. Sagittal T2-
weighted (TR/TE, 2000/80) spin
echo MR image shows a large gan-
glion cyst (open arrows) that arose
from the posterior cruciate liga-
ment. (A, Courtesy of J. Schils,
A B MD, Cleveland, Ohio.)
lateral meniscus to the lateral aspect of the medial femoral flaps of meniscal tissue (i.e., flap tears). Degenerative tears
condyle. As they extend across the knee, they are intimate are believed to result from normal forces acting on a
with portions of the posterior cruciate ligament. The ante- degenerated structure. They are typically horizontal cleavage
rior meniscofemoral ligament, or Humphry’s ligament, lesions that occupy the posterior half of the menisci. In
passes in front of the posterior cruciate ligament, whereas young persons, especially athletes, a single traumatic episode
the posterior meniscofemoral ligament, or Wrisberg’s is responsible for most meniscal lesions. Degenerative
ligament, passes behind the posterior cruciate ligament. tears occur more commonly in older persons and in
The reported incidence of one of the two meniscofemoral association with osteoarthritis of the knee.
ligaments occurring in a single knee is 70% to 100%, and No classification system for meniscal tears has been
the reported incidence of both ligaments occurring in a uniformly accepted. Most classification systems emphasize
single knee is 6% to 80%. the direction of the tear, describing tears as longitudinal,
radial, vertical, or horizontal (or combinations of these)
and as complete, incomplete, or complex. With most (but
Meniscal Tear not all) types of tears, the medial meniscus is involved
Two categories of meniscal tears are commonly identified: more frequently than the lateral meniscus, and the pos-
traumatic and degenerative. Traumatic tears are believed terior horn of the medial meniscus and the anterior horn
to result from excessive application of force to a normal of the lateral meniscus are most commonly affected.
meniscus; they are usually vertical tears that propagate in Longitudinal tears are the most common type encoun-
a longitudinal direction. Less commonly, longitudinal tered. Such tears may occur in a vertical direction and
horizontal or oblique tears may result from this mechanism divide the meniscus into inner and outer segments, or they
of injury. Displacement of these latter tears may result in may occur in a horizontal direction and divide the meniscus
952 Section XVI Internal Derangement of Joints
A B
Figure 59–90. Meniscal tear: abnormal intrameniscal signal intensity and meniscal morphology—
posterior horn of the medial meniscus. Sagittal intermediate-weighted (TR/TE, 2200/20) spin echo MR
image (A) and sagittal three-dimensional Fourier transform SPGR (TR/TE, 58/10; flip angle, 60 degrees)
MR image obtained with fat suppression (B) show altered intrameniscal signal intensity (solid arrows), with
a further increase in signal intensity in (B) and an irregular notch (open arrows) in the superior surface of the
meniscus.
Chapter 59 Internal Derangements of Joints 955
Radial tears of the menisci may be difficult to recognize.
Such tears are oriented perpendicular to the long circum-
ferential axis of the meniscus, may be full thickness
(extending from the apex to the peripheral portion of the
meniscus) or partial thickness, and may be straight (true
radial tear) or obliquely oriented (parrot-beak tear). The
MR imaging findings vary according to the specific type
of tear present, but such findings include complete absence
of visualization of the meniscus on at least one image and
blunting of the apex of the meniscus (Fig. 59–91).
Displaced meniscal tears are of three classic types:
bucket-handle tears (displaced longitudinal tears), parrot-
beak tears (displaced radial or oblique tears), and flap
tears (displaced horizontal tears). Bucket-handle tears of
the meniscus (so named because of their appearance) are
associated with characteristic MR imaging abnormalities; Figure 59–91. Meniscal tear: abnormal meniscal contour—
radial tear of the body of the lateral meniscus. Note the blunted
the inner displaced meniscal fragment resembles a handle, inner contour (arrow) of the body of the lateral meniscus on a
and the peripheral nondisplaced portion of the meniscus sagittal intermediate-weighted spin echo MR image. (Courtesy
looks like a bucket. Bucket-handle tears are usually observed of J. Edwards, MD, Savannah, Ga.)
in the medial meniscus, and they may be associated with
a history of locking of the knee joint. MR imaging findings
include a foreshortened and blunted meniscus with central
displacement of its inner fragment. On sagittal MR images,
the displaced fragment of a bucket-handle meniscal tear Meniscal Cyst
often lies in front of, below, and parallel to the posterior Meniscal cysts are multiloculated collections of mucinous
cruciate ligament (Fig. 59–92). material of unknown cause that generally occur at the
periphery of the meniscus and therefore appear as a focal
mass or swelling at the joint line. They are observed in
Meniscocapsular Separation
approximately 1% of meniscectomies. Meniscal cysts on
Meniscocapsular separation refers to disruption of the the lateral side of the knee are three to seven times more
meniscal attachment to the joint capsule. The posterior frequent than those on the medial side, and medial menis-
horn of the medial meniscus is involved most frequently. cal cysts tend to be larger. Most meniscal cysts are asso-
The presence of fluid between the peripheral portion of ciated with myxoid degeneration and horizontal cleavage
the posterior horn of the medial meniscus and the joint tears of the adjacent meniscus that extend into the
capsule is the most important finding. parameniscal region. Fluid from the joint extending into
The differential diagnosis of meniscocapsular sepa- a horizontal tear of the meniscus and subsequently into
ration includes normal recesses that may appear above or the cystic lesion is one explanation for their occurrence.
below the peripheral portion of the meniscus, a large joint On MR images, an ovoid mass of variable size containing
effusion displacing the meniscus, a longitudinal vertical fluid is seen (Fig. 59–93). Although high signal intensity
tear through the periphery of the meniscus, and bursitis of the cyst on T2-weighted spin echo MR images is
beneath the medial collateral ligament. typical, it is not invariable. Meniscal cysts arising from
A B
Meniscal Ossicle
Meniscal ossicles represent foci of ossification within the
tears of the posterior horn of the medial meniscus may menisci. They should be differentiated from meniscal
extend centrally, become intimate with the posterior cruciate calcifications, which are frequent and relate to calcium
ligament, and simulate the appearance of a ganglion cyst pyrophosphate dihydrate crystal deposition in many
of that ligament. cases. The origin of meniscal ossicles in humans is not
known. There is an association between meniscal ossicles
and longitudinal tears of the medial meniscus. Patients
Discoid Meniscus
with knee ossicles may be asymptomatic or have local
A discoid meniscus has an altered shape with a broad and pain and swelling.
disclike appearance rather than a semilunar configura- Initial films reveal ossification of variable shape in the
tion, although intermediate varieties of discoid menisci anterior or posterior portion of either the medial or the
have been described, including the slab type (flat, circular lateral meniscus. The most common site is the posterior
meniscus), biconcave type (biconcave disc, thinner in its horn of the medial meniscus. MR imaging may also be used
central portion), wedge type (large but normally tapered to diagnose or confirm a meniscal ossicle. Because the ossicle
meniscus), anterior type (enlarged anterior horn), ring may contain bone marrow, the signal intensity charac-
type (oval meniscus with a hole in the middle), forme teristics of fat may be seen within the ossicle (Fig. 59–95).
fruste (slightly enlarged meniscus), and grossly torn type The ossicle may also have low signal intensity on all MR
(too deformed for accurate classification). A discoid lateral imaging sequences.
A B
Chapter 59 Internal Derangements of Joints 957
ligament has no meniscal attachment and slides posteriorly
over the proximal portion of the tibia during flexion of
the knee. The third layer is the capsule of the knee joint,
which attaches to the margins of the joint. The capsule is
thin anteriorly and provides little stability to the joint. It
holds the meniscal rim to the tibia (i.e., the coronary
ligament) and, to a lesser extent, to the femur (i.e., the
meniscofemoral ligament). The superficial and deep
portions of the medial collateral ligament are separated
by a bursa that allows movement between the two.
The medial supporting structures of the knee are
injured more frequently than are the lateral supporting
structures. Indeed, the medial collateral ligament is the
most commonly injured ligament about the knee. Most
Figure 59–95. Meniscal ossicles. Coronal T1-weighted
(TR/TE, 600/15) spin echo MR image shows an ossicle con-
of the major injuries to the medial capsuloligamentous
taining fat (arrow) within the posterior horn of the medial complex are the result of a valgus stress. O’Donoghue’s
meniscus. (Courtesy of A. Deutsch, MD, Los Angeles, Calif.) triad of injury consists of tears of the anterior cruciate liga-
ment, medial collateral ligament, and medial meniscus.
Tension of Semimembranosus
quadriceps
Adductor tubercle
Patella
Medial patellar
retinaculum
Ligamentum patellae
(tendon of quadriceps)
Tibial collateral
Gracilis tendon
ligament
Sartorius tendon
Semitendinosus
tendon
Figure 59–96. Medial supporting structures: anatomic considerations. (From Williams PL, Warwick R
[eds]: Gray’s Anatomy, 35th Br ed. Philadelphia, WB Saunders, 1973, p 453.)
Injuries to the medial collateral ligament vary in severity Semimembranosus Tendon Abnormalities
and include sprains, partial tears, and complete tears. Many injuries to the posteromedial corner of the knee
The MR imaging characteristics are dependent on the also involve other medial supporting structures, as well as
severity of the injury and its acute or chronic nature. the medial meniscus, and any peripheral tear of the pos-
With acute injuries, subcutaneous edema or hemorrhage terior horn of the medial meniscus usually includes a tear
and, in some cases, a joint effusion are evident; with of the posterior oblique ligament. Isolated avulsion
chronic injuries, such edema and hemorrhage are not injuries involving this corner of the knee are rare.
apparent. Sprains of the medial collateral ligament may
lead to slight contour irregularity or thickening of the
ligament, but no discontinuity of its fibers. In acute sprains, Abnormalities of the Lateral Supporting
Structures
subcutaneous edema is an associated finding, but the
signal intensity of the ligament is usually normal. Partial Anatomic Considerations. As on the medial side of the
tears of the medial collateral ligament lead to discon- knee, the anatomy of the lateral supporting structures can
tinuity of some of its fibers, which in acute injuries is be considered in terms of layers (according to the depth
associated with increased signal intensity (particularly on of the tissues) or in terms of their position in an antero-
T2-weighted spin echo and gradient echo MR images) posterior plane. Three layers of tissue can be defined:
within the substance of the ligament and in the subcu- superficial, intermediate, and deep. The superficial layer
taneous tissues. Complete tears of the medial collateral is composed of the deep fascia of the thigh and calf,
ligament are associated with frank discontinuity of all its including the condensed portion known as the iliotibial
fibers. In the acute stage, hemorrhage and edema within tract. This tract has functionally important capsular and
the ligament and subcutaneous tissues are also evident bone attachments that are sometimes referred to as the
(Fig. 59–98). anterolateral ligament of the knee; some fibers of the
Chapter 59 Internal Derangements of Joints 959
the capsule of the knee joint and the lateral (fibular) col-
lateral ligament. The lateral collateral ligament extends
from the lateral epicondyle of the femur to the proximal
lateral surface of the fibular head. The fibular collateral
ligament is taut when the knee is extended and loose when
the knee is flexed, and it serves as the primary restraint to
varus stress at the knee. The popliteus muscle originates
in the posterior surface of the proximal portion of the
tibia, attaches to the posterior horn of the lateral meniscus
and to the arcuate ligament, and terminates in the lateral
femoral condyle, distal and posterior to the attachment
site of the lateral collateral ligament. The anatomy of the
posterolateral corner of the knee (Fig. 59–99) is complex
and is not agreed on.
The lateral supporting structures restrain varus
angulation at the knee and external rotation of the tibia.
Injuries involving both the lateral collateral ligament and
these deep posterolateral structures are associated with
greater varus instability than are injuries confined to the
lateral collateral ligament. Lateral instability patterns are
less frequent than those on the medial side of the knee,
although they may eventually be more disabling.
Figure 59–97. Medial collateral ligament: normal appearance.
Coronal T1-weighted (TR/TE, 800/20) spin echo MR image
shows the course of the vertical fibers of the superficial portion Lateral Collateral Ligament Injuries
of the medial collateral ligament (solid arrows). Note that this With injury to the lateral collateral ligament of the knee,
portion of the ligament is separated (open arrow) from the
routine radiography generally does not reveal specific
medial meniscus. The medial inferior genicular vessels are seen
(arrowhead). findings. Focal soft tissue swelling may be evident in some
cases. Further, because the lateral collateral ligament, along
with the tendon of the biceps femoris muscle, attaches to
the fibular head and styloid process, avulsion fractures of
iliotibial tract are continuous with those of the vastus this portion of the fibula may be seen. The fragment is of
lateralis tendon and aponeurosis, and together they form variable size and may be large, although it is usually dis-
the lateral patellar retinaculum. The second layer consists placed only minimally. On MR imaging, the integrity of the
of the retinaculum of the patella and the patellofemoral lateral collateral ligament and the adjacent tendon of the
ligaments. The third and deepest layer is composed of biceps femoris muscle is well demonstrated (Fig. 59–100).
A B
Figure 59–98. Injuries of the medial collateral ligament: complete tear. Coronal intermediate-weighted
(TR/TE, 1500/12) (A) and T2-weighted (TR/TE, 1500/80) (B) spin echo MR images show complete
disruption (arrows) of the fibers of the medial collateral ligament. Note the increase in signal intensity in the
ligament and soft tissues in B. A joint effusion is present. Additional injuries in this patient included tears of
the lateral meniscus and anterior cruciate ligament. (Courtesy of V. Chandnani, MD, Pittsburgh, Pa.)
960 Section XVI Internal Derangement of Joints
Plantaris
Gastrocnemius Gastrocnemius
bursa
Lateral ligament
Oblique popliteal
ligament Popliteus ligament
Short lateral ligament
Arcuate popliteal
Semimembranosus ligament
Attachment of
Insertion of biceps
popliteus to capsule
Popliteus
Soleus
Gastrocnemius,
medial head Plantaris
Posterior cruciate
ligament Gastrocnemius,
lateral head
Tibial collateral
ligament Fibular collateral
ligament
Medial meniscus
Lateral meniscus
Semimembranosus
tendon
Popliteus tendon
Popliteus, medial
fibers cut away Posterior
meniscofemoral
ligament
These structures can be identified on sagittal, coronal, sity on T2-weighted spin echo MR images within or adja-
and transaxial MR images. Disruption of the lateral col- cent to these tendons (Fig. 59–101). Associated injuries to
lateral ligament, the tendon of the biceps femoris muscle, other lateral supporting structures or to the cruciate ligaments
or both is recognized on MR images as an interruption or may also be evident. In dislocations of the knee, injuries to
waviness of these tendons or regions of high signal inten- lateral, medial, and cruciate ligaments are encountered.
A B C
Figure 59–100. Lateral collateral ligament and biceps femoris muscle and tendon: normal appearanace.
A and B, Two consecutive coronal intermediate-weighted (TR/TE, 2200/20) spin echo MR images show the
normal biceps femoris muscle (arrowhead) and its tendon (solid straight arrows) and the normal lateral collateral
ligament (open arrows). In A, the more posterior image, the biceps femoris muscle and tendon are seen extending
lateral to the joint and inserting into the proximal portion of the fibula. In B, the lateral collateral ligament extends
from the fibula superiorly to insert into the femur. The popliteus tendon (curved arrows) is identified. C, Sagittal
intermediate-weighted (TR/TE, 2200/20) spin echo MR image of the lateral aspect of the knee shows the
biceps femoris muscle (arrowheads) and its tendon (solid arrow) and the lateral collateral ligament (open arrows).
Note the V-like pattern created at the junction of the biceps femoris tendon and lateral collateral ligament.
Patellar Tendinosis
Patellar tendinosis appears to be an overuse syndrome
related to sudden and repetitive extension of the knee
that may occur in persons, particularly athletes, involved
in such activities as kicking, jumping, and running. This
syndrome, often referred to as jumper’s knee, leads to
local pain, swelling, and tenderness that may ultimately
result in disruption of the patellar tendon. The major
histologic findings associated with patellar tendinosis are
not those of inflammation but rather those of fiber failure,
mucoid degeneration, and fibrinoid necrosis, so the term Figure 59–104. Patellar tendon: normal appearance. As
“tendinitis” is inappropriate. Clinical manifestations include shown on this sagittal intermediate-weighted (TR/TE, 3000/20)
activity-related pain below the patella that is initially spin echo MR image, the patellar tendon typically has low
intermittent but may become persistent. signal intensity throughout its length, although intermediate
Routine radiographs do not usually provide diagnostic signal intensity in the posterior margin of the proximal portion
information in cases of patellar tendinosis. Soft tissue of the tendon (solid arrow) and in a triangular area near its distal
swelling, thickening of the patellar tendon, and obscuration insertion (open arrow) may be evident. (Courtesy of J. Yu, MD,
of portions of the infrapatellar fat body may be evident. Columbus, Ohio.)
Bone scintigraphy shows nonspecific accumulation of
radionuclide in the infrapatellar soft tissues or patella.
Ultrasonography reveals an enlarged and hypoechoic
tendon, particularly in its proximal portion. MR images
in the sagittal plane display the entire length of the
patellar tendon (Fig. 59–104). MR imaging in patients
with patellar tendinosis shows an enlarged sagittal diameter or incomplete tears of the patellar tendon, MR imaging
of the tendon (Fig. 59–105). The proximal portion of the displays these tears more vividly (Fig. 59–106).
patellar tendon is usually thickened more than its distal Partial or complete tears of the quadriceps tendon
portion. may result from an injury involving a direct blow to the
tendon or forceful flexion of the knee, or they may occur
spontaneously in association with such chronic diseases
Tears of the Patellar and Quadriceps Tendons
as systemic lupus erythematosus, rheumatoid arthritis,
Indirect forces applied to the extensor mechanism of the gout, or renal failure or after corticosteroid therapy. Rarely,
knee can lead to failure of one or more of its components. spontaneous rupture of the quadriceps tendon occurs in
Failure of the patella resulting in fracture is common, healthy persons. The clinical diagnosis of a complete tear
especially in adults. Systemic disorders such as rheuma- of the quadriceps tendon is usually obvious from the
toid arthritis, chronic renal disease, and systemic lupus patient’s history.
erythematosus contribute to failure of the quadriceps and Routine radiographic findings associated with complete
patellar tendons, although such failure, particularly tears of the quadriceps tendon include soft tissue swelling,
failure of the patellar tendon, may occur in the absence of distortion of soft tissue planes above the patella, an inferior
these causative factors. Patellar tendon ruptures are not position of the patella (patella infra, or baja), calcification
uncommon in persons with chronic patellar tendinosis. or ossification within portions of an avulsed patellar frag-
Failure of the patellar tendon usually occurs at its junction ment, and an undulating patellar tendon. Similar but less
with the inferior pole of the patella or, less commonly, dramatic findings accompany partial tears of the quadri-
the tibial tuberosity, although intrasubstance disruption ceps tendon. Sagittal MR images display the quadriceps
of the patellar tendon may also occur. Complete tears of tendon in exquisite detail (Fig. 59–107). A laminated appear-
the patellar tendon are associated with a high position of ance with two, three, or four layers of tissue characterizes
the patella, designated patella alta. Incomplete tears are the MR imaging appearance of a normal quadriceps
not associated with this change in patellar position. tendon. Tears of the quadriceps tendon lead to partial or
Although ultrasonography can be used to verify complete complete interruption of its fibers (Fig. 59–108).
964 Section XVI Internal Derangement of Joints
Patellofemoral Instability
Patellofemoral instability is a frequent and complex prob-
lem. Clinical diagnosis of such instability is often difficult
because the resulting symptoms and signs may simulate
those of other disorders of the knee. A number of different
methods based on the position of the patella on the lateral
radiograph have been described to confirm the presence
of patella alta or baja. The Insall-Salvati ratio (Fig. 59–109)
compares the patella tendon length to the longest length
of the patella, with a normal ratio of approximately 1. Axial
radiographic views to assess the configuration of the
trochlea, the shape of the patella, the sulcus angle, and
the relationship of the patella to the femur have also been
used (Fig. 59–110).
Lateral dislocation (or subluxation) of the patella is
often a transient phenomenon with spontaneous reduc-
tion. The patella is pulled laterally from the trochlea and
across the lateral femoral condyle, with resultant
osteochondral injuries to the medial patellar facet, lateral
femoral condyle, or both structures. The medial reti-
A B naculum is injured as a result of distraction, or a small
avulsion fracture occurs at the patellar site of attachment
Figure 59–106. Patellar tendon: partial tear. Sagittal T1- of the medial retinaculum. These pathologic findings can
weighted (TR/TE, 850/25) spin echo MR image (A) and sagittal
be observed directly on MR images of the knee obtained
MPGR (TR/TE, 600/20; flip angle, 45 degrees) MR image
(B) reveal a partial tear (arrows) involving the proximal portion shortly after the injury. Typical MR imaging features of
of the patellar tendon and thickening of the entire tendon, an acute patellar dislocation include hemarthrosis, chon-
indicative of chronic patellar tendinosis. The findings of partial dral and osteochondral fractures, disruption or attenua-
patellar tendon rupture are much more pronounced in B. tion of the medial retinaculum, and intra-articular bodies
(Courtesy of S. Fernandez, MD, Mexico City, Mexico.) (Fig. 59–111).
Chapter 59 Internal Derangements of Joints 965
Figure 59–108. Quadriceps tendon:
complete tear. Sagittal intermediate-
weighted (TR/TE, 2500/30) (A) and
T2-weighted (TR/TE, 2500/80) (B) spin
echo MR images show a complete tear
(arrows) of the quadriceps tendon at
the tendo-osseous junction. Note the
high signal intensity at the site of the
tear in B. The patella is displaced
inferiorly.
A B
Chondromalacia Patellae
Chondromalacia patellae is a term applied to cartilage loss
involving one or more portions of the patella that leads
to patellofemoral pain. Fundamental to the diagnosis of
chondromalacia patellae is the presence of histologic
abnormalities affecting the articular cartilage of the patella.
The use of imaging techniques to assess patellar cartilage
loss is an area of great clinical interest. MR imaging appears
to be the best noninvasive diagnostic technique for the
assessment of patellar cartilage (see later discussion).
A B
Figure 59–110. Patellar position: methods of measuring lateral patellar displacement and patellar tilt.
A, Merchant and coworkers (J Bone Joint Surg Am 56:1391, 1974) suggested that on an axial radiograph,
the line connecting the median ridge of the patella (r) and trochlear depth (2) should fall medial or slightly
lateral to a line (O) bisecting angle 1-2-3. Here, the first line lies medial to line O, a normal finding. B, Laurin
and coworkers indicated other measurements that might be appropriate. The upper two diagrams reveal the
normal situation; the lower two diagrams indicate the abnormal situation. On axial radiographs, an angle
formed between a line connecting the anterior aspect of the femoral condyles (1) and a second line along the
lateral facet of the patella (2) normally opens laterally. In patients with abnormal tilting of the patella, these
lines are parallel, or the angle of intersection opens medially. (B, From Laurin CA, Levesque HP, Dussault
R, et al: The abnormal lateral patellofemoral angle: A diagnostic roentgenographic sign of recurrent patellar
subluxation. J Bone Joint Surg Am 60:55, 1978.)
Tibial collateral
Anterior ligament ligament
of head of fibula
Tibia
A Fibula
Lateral meniscus
Medial meniscus
60% to 75% of acute traumatic hemarthroses are asso- substance of the anterior cruciate ligament is injured
ciated with anterior cruciate ligament injury. The classic most commonly.
mechanism of injury to the anterior cruciate ligament
(which is frequently evident in skiers and American Associated Osseous Injuries. In patients with acute tears
football players) is indirect trauma leading to deceleration, of the anterior cruciate ligament, nonspecific soft tissue
hyperextension, or twisting forces, often accompanied by swelling and a joint effusion may be evident. Several
an audible pop and the rapid onset of pain, soft tissue types of fracture of the proximal portion of the tibia and
swelling, and disability. The combination of external rota- distal portion of the femur, however, are specific for or
tion of the tibia with respect to the femur, knee flexion, imply the likelihood of an injury to the anterior cruciate
and valgus stress may also produce an anterior cruciate ligament:
ligament injury, often combined with additional injuries 1. Avulsion fracture of the anterior tibial eminence.
to the medial collateral ligament and the medial menis- 2. Lateral tibial rim fracture (Segond fracture).
cus. O’Donoghue’s triad of injury consists of a tear of the
anterior cruciate ligament, complete disruption of the medial 3. Posterior fracture of the lateral or medial tibial
collateral ligament, and a tear (or capsular separation) of plateau.
the peripheral portion of the medial meniscus. The mid- 4. Osteochondral fracture of the lateral femoral condyle.
968 Section XVI Internal Derangement of Joints
A constant localized chondral or transchondral lesion in this section (or sections), the anterior cruciate ligament
occurring in the region of the condylopatellar sulcus of appears as a straight band of low signal intensity whose
the lateral femoral condyle has been observed and is course parallels or is steeper than that of the intercondylar
designated the lateral notch sign. The lateral notch sign line. The anterior cruciate ligament may not be seen in its
is considered present when the depth of the sulcus is entirety on this image (or images), but the course of the
greater than 1.5 to 2 mm (Fig. 59–113). A depth greater visible portion of the anterior cruciate ligament should
than 2 mm has a specificity and a positive predictive value still be parallel to this line of low signal intensity. On
of 100% for a complete tear of the anterior cruciate coronal and transaxial MR images, the normal anterior
ligament. cruciate ligament can again be identified. The dominant
pattern of signal intensity in a normal anterior cruciate
Magnetic Resonance Imaging. The best plane for analysis ligament is low, although it is not as low as the signal
of either cruciate ligament is the sagittal plane, but the intensity in the posterior cruciate ligament.
supplementary data supplied by coronal and transaxial
MR images are important. The sagittal images are com-
Anterior Cruciate Ligament Tears
monly obtained with the leg externally rotated 15 to
30 degrees, or they are obtained by determining the MR imaging is a sensitive (90% to 98%), specific (90%
orientation of the anterior cruciate ligament from initial to 100%), and accurate (90% to 98%) method for iden-
transaxial (or coronal) MR images and using a sagittal tifying tears of the anterior cruciate ligament. The MR
oblique imaging plane parallel to this orientation (i.e., a imaging diagnosis of injuries to the anterior cruciate liga-
15- to 30-degree internally rotated imaging plane). ment relates to the documentation of (1) abnormalities in
Identification of a normal anterior cruciate ligament begins the anterior cruciate ligament itself, (2) alterations in the
with analysis of the sagittal MR images (Fig. 59–114). In appearance of other structures (e.g., posterior cruciate
choosing a proper sagittal section in which a normal ligament and patellar tendon) related to the abnormal
anterior cruciate ligament should be visualized, it is often alignment of the tibia and femur that results from an
useful to find one that displays the intercondylar roof of the anterior cruciate ligament tear, and (3) abnormalities that
femur as a straight line of low signal intensity. Typically, result from bone impaction.
A B C
Figure 59–113. Injuries of the anterior cruciate ligament. A, Method of measuring the depth of the lateral
condylopatellar sulcus. A line (AB) drawn tangentially across the lower articular surface of the lateral aspect
of the femur forms the reference line. The depth of the sulcus is measured perpendicular to this line at the
deepest point (arrowheads). B, In a patient with a chronic tear of the anterior cruciate ligament, note the
prominent condylopatellar sulcus (arrow). C, In a different patient with a torn anterior cruciate ligament and
a deep lateral condylopatellar sulcus, a T1-weighted (TR/TE, 800/30) spin echo MR image shows alterations
in marrow signal intensity in the midportion of the lateral condyle (arrow) and in the posterolateral portion
of the tibia (arrowhead). These findings are consistent with marrow edema related to impaction fractures.
(A, From Cobby MJ, Schweitzer ME, Resnick D: The deep lateral femoral notch: An indirect sign of a torn
anterior cruciate ligament. Radiology 184:855, 1992. C, Courtesy of M. Pathria, MD, San Diego, Calif.)
Chapter 59 Internal Derangements of Joints 969
A B
A B
Figure 59–116. Injuries of the anterior cruciate ligament:
acute partial anterior cruciate ligament tear. Sagittal T2-
weighted (TR/TE, 4000/105) (A) and coronal T2-weighted
(TR/TE, 3200/108) (B) fast spin echo MR images show an
attenuated appearance of the anterior cruciate ligament, with
intraligamentous regions of high signal intensity (solid arrows).
Note that joint fluid is collecting between the anterior and
posterior cruciate ligaments (open arrow), consistent with a
tear in the synovial lining surrounding these ligaments.
A B
Tears of the posterior cruciate ligament may be are delineated with MR imaging, the detection of more
treated by primary repair, which involves suturing of the superficial cartilage lesions with this imaging method is
ligament and, perhaps, graft augmentation. The specific far more challenging. A multitude of MR pulse sequences
therapeutic strategy, however, is often dictated by the have been advocated, and MR arthrography enhanced with
pattern of injury. Acute tears isolated to the posterior intra-articular administration of gadolinium compounds
cruciate ligament may be treated nonoperatively, whereas has been shown to be effective in the detection of cartilage
those involving multiple ligaments often require surgical abnormalities. With fat-suppressed gradient echo sequences,
reconstruction. cartilage has a trilaminar appearance consisting of a super-
ficial region of high signal intensity, an intermediate region
of low signal intensity, and a deep region of high signal
Abnormalities of Multiple Ligaments
intensity (Figs. 59–122 to 59–124).
Knee dislocation is the classic cause of multiple ligamen-
tous injuries. Although the precise number of ligaments
that are torn, stretched, or avulsed during knee dislocation Abnormalities of Subchondral Bone
depends on the magnitude of the force and the specific Occult Injuries. The detection of occult injuries in the distal
type of dislocation, injury to both cruciate ligaments is portion of the femur and the proximal portion of the tibia
common. When injuries to both cruciate ligaments are with MR imaging has been emphasized. MR imaging
documented, even when no other ligaments are torn, knee may document soft tissue swelling, joint effusions, and
dislocation is likely even when the knee is reduced at the alterations in signal intensity in the bone marrow. Such
time of initial evaluation. The most common injury pattern alterations in signal intensity within subchondral marrow
of knee dislocation involves both cruciate ligaments and after injury to the knee (or other joints) led to the intro-
the medial collateral ligament. Vascular and bone injuries duction of the term bone bruise. The precise histologic corre-
are also important complications of knee dislocation. lates of a bone bruise are not clear, although microfractures
of trabeculae and hyperemia, edema, and hemorrhage in
adjacent marrow appear to contribute to the changes in
Cartilage Abnormalities signal intensity. Reticulated regions of low signal intensity
None of the standard imaging methods used to evaluate are apparent on T1-weighted spin echo MR images, and
cartilage degeneration is ideal. Routine radiographic assess- similar regions of inhomogeneous high signal intensity
ment with weight bearing, varus or valgus stress, knee are observed on T2-weighted spin echo MR images
flexion, or combinations of these techniques (e.g., weight (Fig. 59–125). STIR imaging sequences dramatically dis-
bearing and knee flexion) is adequate for the determi- play these lesions as areas of high signal intensity within
nation of cartilage loss in the more involved weight- involved marrow. The MR imaging characteristics of bone
bearing compartment. Contrast material injected into the bruises are similar to those noted in cases of transient
joint cavity allows portions of the articular cartilage to be painful bone marrow edema. Additional occult cartilage
visualized. The role of ultrasonography in the assessment and bone injuries about the knee include stress fractures
of articular cartilage is limited by the necessity of (insufficiency and fatigue fractures), osteochondral
positioning the joint in a manner that provides proximity fractures, and acute fractures of the femoral condyles and
to the articular surface for a probe placed on the skin. MR tibial plateaus that generally extend into the chondral
imaging appears to be unique in its ability to demonstrate surfaces.
not only cartilage loss but the internal structure as well.
Although severe chondral abnormalities, including full- Hematopoietic Hyperplasia. Islands of hypercellular but
thickness defects and denudation of the articular surface, otherwise normal-appearing hematopoietic marrow may
Chapter 59 Internal Derangements of Joints 973
of the femoral head. Typical sites of involvement are the Ultrasonography and MR imaging are the two most
femoral condyles and proximal portion of the tibia, effective imaging methods for the diagnosis of these con-
although involvement of the diaphyseal and metaphyseal ditions. With MR imaging, bursal fluid is of high signal
regions of the femur and tibia is encountered (see intensity on T2-weighted spin echo images, as well as on
Chapter 67). STIR images.
Peroneus
longus Peroneus brevis
Tibialis posterior Tibialis anterior
Tibialis anterior Superior extensor retinaculum
Superior extensor Extensor hallucis longus
retinaculum
Extensor digitorum longus
Inferior extensor
Peroneus tertius
retinaculum
Common sheath for extensor
digitorum longus and peroneus tertius
Tibialis anterior Inferior extensor retinaculum
Extensor hallucis Extensor hallucis brevis
longus
Peroneus
Flexor Flexor Tibialis longus Peroneus
hallucis digitorum posterior
brevis Extensor
longus longus digitorum Tuberosity
brevis of fifth metatarsal
A B bone
Figure 59–128. Tendon sheaths about the ankle: normal anatomy. A, Medial aspect of the ankle. B, Lateral
aspect of the ankle. (From Williams PL, Warwick R [eds]: Gray’s Anatomy, 36th Br ed. Edinburgh, Churchill
Livingstone, 1980, pp 611, 612.)
976 Section XVI Internal Derangement of Joints
3. Flexor retinaculum. The flexor retinaculum is located of the tendon. Inflammation and degeneration of any or
medially and encloses the tarsal tunnel. It extends all of these structures may relate to direct or indirect injury,
between the medial malleolus and the calcaneus. occupational stress and overuse syndromes, misuse syn-
dromes related to anomalies of the foot or other struc-
4 and 5. Superior and inferior peroneal retinacula. The two
tures, and local or systemic rheumatic diseases. Adhesions
peroneal retinacula are laterally located fascial thicken-
may develop between the sheath and the tendon (i.e.,
ings that extend from the lateral malleolus to the cal-
stenosing or constrictive tenosynovitis) and lead to altered
caneus. These retinacula serve to hold the tendons of
or restricted movement in a tendon sheath–tendon unit
the peroneus longus and brevis muscles firmly in place
that normally requires smooth, almost frictionless
behind the fibula.
function.
The tendons of the flexor hallucis longus, flexor digi- A tethered tendon is one whose range of movement is
torum longus, and tibialis posterior muscles are inti- limited because of its abnormal fixation to an adjacent
mately related at the levels of the medial malleolus and structure. Causes of such tethering include anatomic
below. At these levels, the tendon of the tibialis posterior anomalies, fractures with resulting deformities that lead
muscle is most anterior, the tendon of the flexor digi- to abnormal fixation or displacement of the tendon or its
torum longus muscle is in the intermediate position, and sheath, a hypertrophied os trigonum that leads to con-
the tendon of the flexor hallucis longus muscle is located striction and displacement of the flexor hallucis longus
posteriorly. A mnemonic device, “Tom, Dick, and Harry,” tendon within its sheath, fracture-dislocations of the ankle
indicates the anterior to posterior relationships of these that may lead to tethering or even incarceration of one or
three tendons: T for the tibialis posterior tendon, D for more regional tendons, and checkrein deformities resulting
the flexor digitorum longus tendon, and H for the flexor from abnormal fixation of the flexor hallucis longus tendon
hallucis longus tendon. under or just proximal to the flexor retinaculum or under
The tendons of the tibialis anterior, extensor hallucis the annular ligament.
longus, and extensor digitorum longus muscles lie along- Partial or complete tears of the tendons of the foot
side one another in the anterior portion of the lower part and ankle may relate to a laceration, especially in the sole
of the leg. The most medial of these structures is the of the foot, or more commonly, they may occur sponta-
tibialis anterior tendon, the most lateral is the extensor neously. Spontaneous rupture of these tendons usually
digitorum longus tendon, and the tendon of the extensor implies some type of intrinsic pathologic process, because
hallucis longus muscle lies in an intermediate position. normal tendons rarely rupture in this fashion. Tendon
The mnemonic device “Tom, Harry, and Dick” can be degeneration in older persons or chronic tendon overuse
used to define the medial to lateral relationships of these in younger persons, particularly athletes, may predispose
three tendons: T for tibialis anterior, H for extensor hallucis to spontaneous rupture. Although virtually any tendon of
longus, and D for extensor digitorum longus. the foot or ankle can be affected, spontaneous disruptions
of the tibialis posterior tendon and the Achilles tendon
are encountered most frequently.
Tendon Abnormalities
Anatomic Considerations. With the exception of the Achilles Imaging Considerations. MR imaging is generally con-
tendon, all the foot tendons change from a vertical orien- sidered the best method for the detection of tendinosis,
tation in the lower leg to a horizontal orientation at or near tenosynovitis, and partial and complete tendon rupture.
the level of the ankle, a modification in direction that is CT is superior to MR imaging in the delineation of tendon
accomplished by means of a pulley system consisting of calcification and retinacular avulsion of bone, and CT
either bone (i.e., the malleoli) or retinacula. Where ten- and MR imaging are of approximately equal value when
dons are applied to the surface of a bone, a groove or applied to the analysis of tendon dislocation.
sulcus is present to promote the angular deviations in their Routine radiographic findings associated with abnor-
course that occur about the ankle. The groove behind the malities of the tendons and tendon sheaths of the foot
fibula, through which pass the tendons of the peroneus and ankle include soft tissue swelling; change in contour,
brevis and peroneus longus muscles, is the smallest and calcification, or ossification of a tendon; bone proliferation;
most shallow, perhaps explaining the occurrence of sub- fracture fragments; and sesamoid displacement. Achilles
luxation of these tendons. tendinosis or rupture of the Achilles tendon may be
Disorders of the tendons about the ankle and in the accompanied by edema or hemorrhage, with obscuration
foot include the following: tenosynovitis, tethering, tears, of portions of the pre-Achilles fat body. Associated retro-
dislocations, tumors and pseudotumors, ossification, con- calcaneal bursitis (which may occur in systemic rheumatic
genital anomalies, contractures, and iatrogenic injuries. disorders and in Haglund’s syndrome) leads to obliteration
Inflammatory changes about a tendon, often designated of the normal radiolucent retrocalcaneal recess between
tenosynovitis, can be more precisely defined according to the Achilles tendon and the posterosuperior portion of
the type of tissue affected. If inflammation affects a tendon the calcaneus (Fig. 59–129).
sheath, the term tenosynovitis is most appropriate. MR imaging has been applied to the assessment of
Paratenosynovitis refers to inflammation of tissues about tendons and other structures in the ankle and foot. The
a tendon sheath, and paratendinitis refers to inflamma- tibialis posterior tendon and the Achilles tendon have
tion about a tendon that has no sheath. Peritendinitis is received the greatest attention. The most commonly used
defined as inflammation of a peritenon. Tendinosis (or MR imaging sequences in the ankle and foot are standard
tendinopathy) indicates degeneration within the substance spin echo, fast spin echo, MPGR, and STIR sequences.
Chapter 59 Internal Derangements of Joints 977
A B
Figure 59–129. Retrocalcaneal bursa: normal appearance and retrocalcaneal bursitis. A, Normal appear-
ance. Normally, with the foot in neutral position, fat about the retrocalcaneal bursa produces a triangular
radiolucent area (arrows), designated the retrocalcaneal recess, between the Achilles tendon and the calca-
neus on lateral radiographs of the ankle. B, Retrocalcaneal bursitis. Inflammation, synovial hypertrophy, and
effusion have resulted in an enlarged, fluid-filled retrocalcaneal bursa that extends above the calcaneus as a
radiodense area (open arrow). The Achilles tendon and surrounding tissue are also thickened (solid arrow).
With minor exceptions, the normal tendons in the ankle that of a tendon disruption, particularly disruption of
and foot are homogeneous and of low signal intensity the tibialis posterior tendon.
with all MR imaging sequences. They are generally equal
4. Tendon striations. When three-dimensional gradient
in size on the two sides of the body and have a smooth
recalled MR images are obtained, longitudinal lines of
contour. Some exceptions to these general rules may be
intermediate signal intensity in the distal portion of
noted, however:
the tibialis posterior tendon may be noted. These lines
1. Magic angle effect. As indicated earlier, increased signal probably represent branches of the tendon. Similar
intensity may be seen in normal tendons oriented striations are caused by fat interspersed among the
obliquely with respect to the main magnetic field, an fibers of some of the ankle ligaments.
effect that is greatest when this orientation is at 55
degrees to that of the magnetic field (Fig. 59–130). This In common with the findings derived by ultrasonography
effect is greater when the MR imaging examination and CT, the major MR imaging finding of tenosynovitis
uses spin echo techniques with short TEs or gradient is abnormal accumulation of fluid within the tendon
echo techniques with short TEs and high flip angles. sheath. This fluid has low signal intensity on T1-weighted
spin echo MR images and high signal intensity on T2-
2. Tenosynovial fluid. Differentiation of a thickened tendon weighted spin echo images. Pannus and scar formation
from one surrounded by a fluid-filled synovial sheath is about a tendon are characterized by intermediate signal
difficult on T1-weighted spin echo MR images. Further, intensity on T1-weighted spin echo MR images and inter-
the presence of small or even moderate amounts of mediate to high signal intensity on T2-weighted spin echo
fluid within a tendon sheath is, by itself, not diagnostic MR images. Intratendinous tears are accompanied by
of an abnormality, because such fluid is seen in asymp- focal areas of high signal intensity within the substance of
tomatic persons. Tenosynovial fluid is more frequent the tendon on intermediate- and T2-weighted spin echo
in flexor tendons than extensor tendons and may be MR images. With chronic tendinosis, the tendon is enlarged
particularly prominent about the flexor hallucis longus and of low or intermediate signal intensity on both T1-
tendon. and T2-weighted spin echo MR images.
3. Bulbous tendon insertion sites. Insertion sites of tendons Tendon ruptures may be acute or chronic and partial
may appear bulbous (Fig. 59–131), perhaps related to or complete. With MR imaging, recent tendon tears fre-
volume averaging of their signal intensity with that of quently reveal regions of increased signal intensity on
adjacent cortical bone. This appearance can simulate T2-weighted spin echo MR images and certain gradient
978 Section XVI Internal Derangement of Joints
A B
Figure 59–130. Tendons about the ankle: normal variations—magic angle phenomenon. A, Peroneus
longus and brevis tendons. Sagittal T1-weighted (TR/TE, 600/20) spin echo MR image reveals a slight
increase in signal intensity (arrows) of the peroneus tendons as they course about the distal portion of the
fibula (arrowhead). This alteration in signal intensity relates to the magic angle phenomenon. B, Flexor
hallucis longus tendon. Sagittal T1-weighted (TR/TE, 600/20) spin echo MR image shows a slight increase
in signal intensity (arrow) in the flexor hallucis longus tendon near the sustentaculum tali (arrowhead) related
to the magic angle phenomenon. (Courtesy of D. Goodwin, MD, Hanover, N.H., and D. Salonen, MD,
Toronto, Ontario, Canada.)
A C
Figure 59–132. Tendons about the ankle: types of tendon
tears. Type 1 tears are characterized by partial disruption of the Figure 59–133. Achilles tendon: normal appearance. A, Sagittal
tendon, with vertical splits and a bulbous or hypertrophied T1-weighted (TR/TE, 650/20) spin echo MR image shows the
appearance. Type 2 tears are characterized by partial disruption smooth, straight contour of the Achilles tendon (solid arrows)
of the tendon, which appears attenuated. Type 3 tears are charac- and its sharp interface with the pre-Achilles fat pad. Note the
terized by complete disruption of the tendon and retraction of triangular region of fat (open arrow) that separates the Achilles
the torn ends. (Modified from Rosenberg ZS, Cheung Y, Jahss tendon and the posterosuperior portion of the calcaneus. B and
MH, et al: Rupture of posterior tibial tendon: CT and MR C, Transaxial T1-weighted (TR/TE, 650/20) spin echo MR
imaging with surgical correlation. Radiology 169:229, 1988.) images at the level of the ankle joint (B) and just above the
calcaneal atttachment of the Achilles tendon (C) show the
changing shape of the Achilles tendon (arrows). In B, the ante-
rior margin of the Achilles tendon is flat or slightly concave; in
spiral twist. The vulnerable region of the Achilles tendon C, a wider structure with a flat anterior margin is evident.
is just proximal to the inception of this spiral twist and
involves a segment between 2 and 6 cm from its insertion
into the calcaneus. The normal blood supply in this
region is tenuous. The Achilles tendon does not possess
a tendon sheath.
The MR imaging features of the Achilles tendon
parallel the anatomic features (Fig. 59–133). The average
anteroposterior diameter of the Achilles tendon is 5.2 mm.
The anterior margin of the tendon is mildly convex in
10% of asymptomatic persons.
Chronic tendinosis leads to thickening of the Achilles
tendon. The involved tendon feels thickened, fibrotic, and
nodular on physical examination, and it may be tender to
palpation. Radiography, CT, and ultrasonography can all
document the enlarged Achilles tendon that characterizes
chronic tendinosis. The classic MR imaging feature of
chronic Achilles tendinosis, with or without surrounding
A B
peritendinitis and paratendinitis, is a diffusely or focally
enlarged or thickened tendon (Fig. 59–134). The signal Figure 59–134. Chronic Achilles tendinosis. Transaxial
intermediate-weighted (TR/TE, 2500/20) spin echo MR image
intensity in the involved portion of the Achilles tendon is at the level of the distal portions of the tibia and fibula (A) and
generally low; when intratendinous foci of increased transaxial T2-weighted (TR/TE, 2500/70) spin echo MR
signal intensity are observed on T2-weighted spin echo image at a slightly lower level (B) show a markedly enlarged
MR images, an accompanying partial tear of the Achilles Achilles tendon (arrows) that is of low signal intensity on both
tendon is likely. images.
980 Section XVI Internal Derangement of Joints
C D E
Chapter 59 Internal Derangements of Joints 981
Figure 59–137. Normal tendons of
the ankle: sagittal MR images. A, T1-
weighted (TR/TE, 600/20) spin echo
MR image through the lateral malleolus
(arrowhead) shows portions of the per-
oneus longus (open arrows) and peroneus
brevis (solid arrow) tendons. B, T1-
weighted (TR/TE, 600/20) spin echo
MR image through the medial malleolus
(arrowhead) reveals the tibialis posterior
(open arrow) and flexor digitorum longus
(solid arrow) tendons.
A B
bone, the inferior capsule of the cuneonavicular joint, With both CT and MR imaging, type 1 partial tears are
and the medial cuneiform bone. Disruption of the tibialis associated with tendon hypertrophy and, when longitu-
posterior tendon, which is being recognized with increasing dinal splitting is prominent, tendon division into two
frequency, is associated with an inability to supinate and parts. With MR imaging, an abnormal increase in signal
invert the foot and, in some cases, with progressive and intensity may be seen within the enlarged or bifurcated
painful planovalgus deformity of the foot. tibialis posterior tendon, especially on intermediate-
Degenerative changes in the tendon and inflammatory weighted spin echo MR images, but also occasionally on
changes in the tendon sheath of the tibialis posterior T2-weighted spin echo and STIR images. Type 2 partial
muscle are encountered as a result of altered mechanics tears lead to a diminution in size of the tibialis posterior
of the foot or as a response to a systemic articular disease tendon, especially its width. Complete tears of the tibialis
such as rheumatoid arthritis or a seronegative spondy- posterior tendon are associated with tendon retraction
loarthropathy. Spontaneous rupture of the tibialis poste- and a gap (Fig. 59–138).
rior tendon is classically seen in patients in the fifth and
sixth decades of life, with two thirds of cases occurring in
women. Initial manifestations include pain, swelling, and Abnormalities of the Flexor Hallucis Longus
tenderness to palpation of the affected tendon. Progressive Tendon
involvement results in weakness of inversion of the foot Abnormalities of the flexor hallucis longus tendon are
and flattening of the medial longitudinal arch of the foot, encountered less commonly than those of the Achilles
with the eventual appearance of severe pes planus
deformity. Spontaneous rupture of the tibialis posterior
tendon unrelated to a systemic rheumatic disease appears
to result from local mechanical factors. Acute angulation
of the tendon behind the medial malleolus and, perhaps,
a vulnerable blood supply make it susceptible to injury.
Further, an accessory ossicle of the navicular bone may
provide the site of attachment of some of the fibers of the
tibialis posterior tendon, also predisposing to failure of
the tendon mechanism.
Although routine radiographs and those obtained
during weight bearing allow the diagnosis of long-
standing disruption of the tibialis posterior tendon, these
methods are less useful in the diagnosis of early disrup-
tion because of the presence of a number of deformities
(e.g., an increase in the angle between the long axis of the
talus and that of the calcaneus, a sag at the calcaneo-
navicular and naviculocuneiform articulations, and lateral
subluxation of the navicular bone with respect to the Figure 59–138. Injuries of the tibialis posterior tendon: acute
talus). CT and MR imaging appear to be more valuable complete tear. Sagittal T1-weighted (TR/TE, 800/12) spin
than ultrasonography and tenography in the evaluation echo MR image shows disorganization of the tibialis posterior
of degeneration and disruption of the tibialis posterior tendon (arrows) near its navicular site of insertion. Note a mass
tendon. of intermediate signal intensity about the tendon.
982 Section XVI Internal Derangement of Joints
and tibialis posterior tendons. Such abnormalities include the ankle; such tethering leads to unexplained flexion
tendinosis and partial or complete disruption, partial tether- contracture of the interphalangeal joint of the hallux with
ing (i.e., os trigonum syndrome), and complete tethering a mild extension contracture of the first metatarsopha-
(i.e., checkrein deformity). Flexor hallucis longus tendi- langeal joint (claw hallux). A healing fracture of the lower
nosis usually occurs behind the medial malleolus. portion of the tibia may lead to this deformity as a result
MR imaging can be used to assess degeneration or rup- of post-traumatic adhesions.
ture of the flexor hallucis longus tendon (Fig. 59–139),
although fluid in the sheath of this tendon may be a normal
finding in patients with large ankle effusions because of Abnormalities of the Tibialis Anterior Tendon
communication between the sheath and the ankle, which Spontaneous rupture of the tibialis anterior tendon is
occurs in approximately 20% of normal persons. Partial rare. Spontaneous ruptures typically appear in patients
tethering of the tendon of the flexor hallucis longus older than 45 or 50 years, and they may be complete or
muscle may result from hypertrophy of the os trigonum, partial in extent. Rupture of the tibialis anterior tendon may
a condition designated the os trigonum syndrome. The also develop in athletes such as runners, soccer players,
posterior aspect of the talus normally has two tubercles, and hikers. On clinical examination, a mass related to pro-
the medial tubercle and the lateral tubercle, between trusion of the proximal portion of the torn tendon above
which is found the fibro-osseous tunnel of the flexor the inferior retinaculum may be noted. Retraction of the
hallucis longus tendon. The os trigonum is the ununited torn end beneath the superior retinaculum may also be
lateral tubercle of the talus, is present in approximately seen. Inflammation of the adjacent tendon sheaths is
10% of persons, and is bilateral in 50%. The medial edge frequently apparent. MR imaging (Fig. 59–140) and CT
of the os trigonum (or trigonal process of the talus) lies allow an accurate diagnosis in most cases.
under the flexor hallucis longus tendon, on the lateral
side of the flexor hallucis longus tunnel, and it may lead
to compression of the tendon in this area. CT or MR Abnormalities of the Peroneal Tendons
imaging is useful in defining the relationship between the The peroneus longus and brevis muscles are important
os trigonum and flexor hallucis longus tendon in some lateral stabilizers of the ankle joint. The peroneus longus
patients with this syndrome. muscle serves to evert and dorsiflex the foot, and the brevis
The checkrein deformity consists of a fixed tethering muscle contributes to eversion of the foot. During its course,
of the flexor hallucis longus tendon under or just proximal the peroneus longus tendon undergoes an abrupt change in
to the flexor retinaculum (internal annular ligament) of direction at two points: at the tip of the lateral malleolus,
and at the distal (lateral) edge of the cuboid bone. In both
these locations, the tendon is thickened, and at the point
of contact with the smooth surface of the edge of the cuboid
bone, a sesamoid composed of fibrocartilage or bone may
be found within the tendon. The tendon of the peroneus
brevis muscle, after passing behind the fibula, runs forward
on the lateral surface of the calcaneus, above the peroneus
longus tendon to insert into the tuberosity of the lateral
aspect of the base of the fifth metatarsal bone.
A B
984 Section XVI Internal Derangement of Joints
A B
Figure 59–144. Medial (tibial) collateral ligament: normal anatomy. (From Kelikian H, Kelikian AS:
Disorders of the Ankle. Philadelphia, WB Saunders, 1985, p 20.)
deltoid ligament extends from the tip of the medial component of the lateral ligamentous complex is the
malleolus to the entire nonarticular medial surface of the weakest and is injured most often. The calcaneofibular
body of the talus. The tendons of the tibialis posterior and ligament extends across two joints (the talocrural and
flexor digitorum longus muscles are located superficial to posterior subtalar joints) in an inferior and posterior
the deltoid ligament. The fibers of the deep portion of the direction from a depression in front of the apex of the
deltoid ligament prevent the talus from being displaced lateral malleolus to attach to a tubercle on the lateral sur-
laterally. face of the calcaneus. The tendons of the peroneus
longus and peroneus brevis muscles and their sheaths
Lateral Complex. The lateral ligamentous complex of the cross over this ligament. This ligament serves to stabilize
ankle, referred to as the fibular collateral ligament, con- the posterior subtalar joint. The posterior talofibular
sists of three components, or fasciculi, all of which attach ligament is a strong, horizontally oriented structure that
to the lateral (fibular) malleolus (Fig. 59–145). The passes from the lower part of the fossa of the lateral
anterior talofibular ligament passes from the anterior malleolus to the lateral tubercle of the posterior process
margin of the fibular malleolus in a forward and medial of the talus.
direction to attach to the lateral articular facet and lateral
aspect of the neck of the talus. The anterior talofibular
Pathologic Considerations
ligament is almost horizontal with the talus in a neutral
position, is directed slightly upward as it passes medially Injuries to the ligaments about the ankle are one of the
with the talus in dorsiflexion, and is directed downward most frequently encountered consequences of trauma.
as it passes medially with the talus in plantar flexion. This They are typically seen in young adults, particularly those
involved in sports. The lateral ligamentous complex is
injured more commonly than the medial or tibiofibular
complex. Of the lateral ligaments, the anterior talofibular
ligament is affected most frequently, either alone or in
combination with an injury to the calcaneofibular
ligament.
Classification of ligamentous injuries is based on the
specific site or sites of involvement and their severity.
Injuries to the collateral ligaments of the ankle are often
designated low ankle sprains, and injuries to the
syndesmotic ligaments are commonly termed high ankle
sprains. The severity of the injury may be categorized as
grade I, II, or III. Grade I injuries are the least severe,
apparently representing failure of some of the tendinous
fibers; grade III injuries are the most severe and are
associated with complete ligamentous disruption and
instability of the ankle joint; and grade II injuries are
A B intermediate in severity. Instability of the ankle implies
Figure 59–145. Lateral ligamentous complex of the ankle: abnormal joint motion during the application of varus or
normal anatomy. (From Kelikian H, Kelikian AS: Disorders of valgus stress or positive results during an anterior drawer
the Ankle. Philadelphia, WB Saunders, 1985, p 20.) test.
986 Section XVI Internal Derangement of Joints
Imaging Considerations
Initial assessment of injuries to the medial, lateral, and
syndesmotic ligaments about the ankle requires routine
radiography, which allows the detection of any accom-
panying fractures (see Chapter 57), and radiography
obtained during the application of stress. Complementary
imaging methods are arthrography and MR imaging;
ultrasonography may also be helpful.
MR imaging has been used extensively to evaluate
ligamentous injuries about the ankle. Whether the ankle
ligaments are evaluated with the foot in a neutral posi-
tion, plantar-flexed, dorsiflexed, or in some combination
of these positions, three MR imaging planes are usually
required to fully evaluate all the ligaments of the ankle.
Normal ligaments are thin and of low signal intensity.
The anterior talofibular ligament is the easiest to identify,
particularly on transaxial MR images (Fig. 59–146). It is
usually homogeneous in appearance. Similarly, the posterior
talofibular ligament is identified most readily in the
transaxial plane (although it is also seen on coronal images),
and its posterior aspect is intimately related to the
peroneal longus tendon and its sheath (see Fig. 59–146). Figure 59–146. Normal ligaments of the ankle: anterior and
posterior talofibular ligaments. Transaxial T1-weighted (TR/TE,
The MR imaging findings associated with disruption of 600/20) spin echo MR image shows the anterior talofibular liga-
the ligaments about the ankle include interruption of part ment (curved arrow) and posterior talofibular ligament (open arrow).
or all of a ligament, ligament laxity or waviness, thickening
and irregularity of the ligament, surrounding hemor-
rhage and edema (in acute injuries), and accumulation of
abnormal amounts of fluid in adjacent joints and tendon Sinus Tarsi Syndrome
sheaths (Fig. 59–147). An inability to visualize a ligament Between the two subtalar joints—the posterior subtalar
may indicate a tear, but the reliability of this finding and the talocalcaneonavicular joints—a cone-shaped region
depends on the ligament being considered and the designated the tarsal sinus and tarsal canal is found. The
technical aspects of the MR imaging examination. apex of the cone is located posteromedially and represents
A B
Figure 59–147. Injuries of the talofibular ligaments: acute complete tear of the anterior talofibular liga-
ment and acute partial tear of the posterior talofibular ligament. A, Coronal T1-weighted (TR/TE, 600/20)
spin echo MR image reveals subcutaneous edema laterally and an osteochondral fracture of the talus
(arrowhead). B, Transaxial intermediate-weighted (TR/TE, 2000/20) spin echo MR image obtained with fat
suppression at a level just below the ankle shows soft tissue edema and hemorrhage (of high signal intensity),
complete disruption of the anterior talofibular ligament (solid arrow), and attenuation of the posterior
talofibular ligament (open arrow). (Courtesy of C. Sebrechts, MD, San Diego, Calif.)
Chapter 59 Internal Derangements of Joints 987
the tarsal canal, and the expanded anterolateral portion Impingement Syndromes
of the cone represents the tarsal sinus. The contents of the Several impingement syndromes may lead to restricted
tarsal canal and sinus include fat, an arterial anastomosis motion of the ankle joint. These syndromes can be
with branches from the posterior tibial and peroneal divided into six types: anterior, posterior, posterolateral,
arteries, joint capsules, nerve endings, and five ligaments. anterolateral, syndesmotic, and medial (Table 59–7).
These ligaments are the medial, intermediate, and lateral
roots of the inferior extensor retinaculum; the cervical
ligament; and the ligament of the tarsal canal (i.e., talo- Anterior Impingement Syndrome
calcaneal interosseous ligament). Injury to these structures The anterior impingement syndrome results from impinge-
leads to the sinus tarsi syndrome, which is associated with ment of the anterior aspect of the tibia against the
lateral foot pain, tenderness, and hindfoot instability. The superior portion of the neck of the talus. It is commonly
high association between tarsal sinus and canal injury and observed in young athletic persons. Clinical findings
injury to the lateral ligaments of the ankle requires careful include pain and tenderness in the anterior region of the
analysis of the sinus tarsi in all patients with inversion ankle that are accentuated with dorsiflexion of the joint.
injuries of the ankle. Routine radiography reveals osteophytes in the anterior sur-
MR imaging has been used to study the normal contents face of the tibia, neck of the talus, or both (Fig. 59–150).
of the tarsal sinus and canal and to investigate patients Arthroscopic resection of the osteophytes may lead to
with the sinus tarsi syndrome. The cervical ligament and symptomatic relief and increased ankle motion.
portions of the inferior extensor retinaculum are seen in
all normal ankles, and the interosseous ligament is evident
in most normal ankles (Fig. 59–148). Fluid collections Posterior Impingement Syndrome
with increased signal intensity on T2-weighted spin echo The posterior impingement syndrome may be related to
MR images are normally evident in the recesses about the the presence of an os trigonum (Fig. 59–151) or a
talocalcaneal interosseous ligament, although absence of prominent Stieda’s process in the posterior surface of the
these recesses on MR imaging does not allow a diagnosis talus; alternatively, it may result from soft tissue injury.
of sinus tarsi syndrome. MR imaging findings of this The first of these two types of posterior impingement is
syndrome include poor definition or disruption of soft often referred to as the os trigonum or talar compression
tissue structures, edema or fibrosis, and abnormal fluid syndrome; the second type is sometimes designated
collections (Fig. 59–149). Abnormalities of adjacent struc- posterior soft tissue impingement. With regard to the
tures, such as the anterior talofibular ligament, calcaneo- talar compression syndrome, symptoms are common
fibular ligament, and tibialis posterior tendon, may also because of limitation of plantar flexion at the ankle. For
be evident. athletes such as professional ballet dancers, the condition
A B C
Figure 59–148. Sinus tarsi syndrome. A, Normal appearance. Coronal T1-weighted (TR/TE, 500/18)
spin echo MR image reveals a normal talocalcaneal interosseous ligament (arrow), which is surrounded by
fat. B and C, Sinus tarsi syndrome. Coronal reconstructions from a three-dimensional gradient echo
(TR/TE, 30/10; flip angle, 70 degrees) MR image, with B being slightly more posterior than C, show diffuse
infiltration of the tarsal sinus (black arrow). In B, partial disruption of the talocalcaneal interosseous ligament
has led to a wavy, discontinuous appearance (solid white arrows). A small osteochondral fracture of the medial
talar dome (open white arrow) is seen. In C, partial disruption of the cervical ligament (white arrows) is evident.
(From Klein MA, Spreitzer AM: MR imaging of the tarsal sinus and canal: normal anatomy, pathologic
findings, and features of the sinus tarsi syndrome. Radiology 186:233, 1993.)
988 Section XVI Internal Derangement of Joints
A B
Figure 59–149. Sinus tarsi syndrome. Sagittal T1-weighted (TR/TE, 760/20) spin echo (A) and STIR
(TR/TE, 5300/30; inversion time, 150 msec) (B) MR images reveal obliteration of the fat in the tarsal sinus,
with high signal intensity in B. (Courtesy of J. Edwards, MD, Savannah, Ga.)
A B
Figure 59–151. Posterior impingement syndrome. In this 45-year-old woman, a conventional radiograph
(A) shows an os trigonum (arrow). A sagittal STIR (TR/TE, 5100/30; inversion time, 150 msec) MR image
(B) shows high signal intensity (arrows) in the os trigonum and adjacent talus, with fluid in the ankle,
posterior subtalar joint, and sinus tarsi.
A B
C
Chapter 59 Internal Derangements of Joints 991
The peroneus quartus muscle, also designated the Abnormalities of the Plantar Soft Tissues
peroneus accessorius, peroneus externus, and peroneus
calcaneus externus muscle, has been associated with Plantar Fasciitis
chronic pain and swelling about the ankle. Its reported The plantar aponeurosis is a thick, strong band of tissue
frequency, based on results of cadaveric dissections, extending from the calcaneus posteriorly to the region
varies from approximately 12% to 22%. Its site of origin of the metatarsal heads and beyond. It is composed of
may include the distal lateral portion of the fibula and the a prominent central part and lateral and medial parts.
peroneus brevis or longus muscle, and its site of insertion Plantar fasciitis represents inflammation of the plantar
may include the phalanges or metatarsal bone of the fifth fascia and perifascial structures. Its causes are divided
toe, the calcaneus, the cuboid bone, and the lateral reti- into three categories: mechanical, degenerative, and
naculum of the ankle. Accurate diagnosis is provided by systemic. Mechanical factors associated with plantar
MR imaging (Fig. 59–154). fasciitis include pes cavus, a pronated foot, and an
externally rotated lower extremity. Degenerative
causes of plantar fasciitis include atrophy of the heel
Muscle Hernia
pad and age-related increases in foot pronation. Systemic
Herniation of a portion of a muscle through its fascial causes include a wide variety of rheumatic disorders,
sheath is encountered most often in the leg. Although the especially rheumatoid arthritis and the seronegative
tibialis anterior muscle is the typical site of involvement, spondyloarthropathies.
other muscles can be affected, such as the peroneus Clinical findings include pain and tenderness localized
longus muscle. Most persons with muscle hernias are to the regions of inflammation. Dorsiflexion of the toes
asymptomatic, and multiple hernias with bilateral may lead to an exacerbation of the findings as a result of
involvement are not uncommon. Accurate diagnosis can stretching of the fibers of the plantar fascia. Although
be accomplished clinically in many persons. Indeed, the bone scintigraphy may reveal regions of increased
symmetry of bilateral lesions is an important diagnostic accumulation of the radiopharmaceutical agent at the site
clue. MR imaging allows a correct diagnosis in most cases of inflammation, the increased scintigraphic activity may
because the subcutaneous masses are seen to be portions be more widespread and produce a nonspecific pattern.
of the muscle that extend through defects in the With ultrasonography, findings of plantar fasciitis include
overlying fascia. Ultrasonography is another excellent thickening of the fascia, abnormal echogenicity, and peri-
diagnostic method. fascial edema. MR imaging appears to be both sensitive and
specific in the diagnosis of plantar fasciitis, demonstrating of all ages (including children) and is more common in
thickening of the plantar fascia (which may grow to 7 or men. Lesions may be solitary or multiple and unilateral
8 mm from the normal 3 or 4 mm thickness) and intrafas- or bilateral in distribution. Affected persons are usually
cial alterations in signal intensity. On T1-weighted spin asymptomatic, with the nodules being discovered by
echo MR images, foci of intermediate signal intensity palpation.
may be seen within the plantar fascia, which is normally With MR imaging, single or multiple nodules in the
of uniform low signal intensity. On T2-weighted spin plantar fascia and subcutaneous tissue are observed. The
echo and STIR MR images, areas of high signal intensity medial and central portions of the plantar fascia are typi-
may be evident in the plantar fascia and subcutaneous cally involved. The lesions are of low signal intensity on
tissue (Fig. 59–155). Alterations in signal intensity of the T1-weighted spin echo images and low to intermediate
marrow of the calcaneus, reflective of edema, may also be signal intensity on T2-weighted spin echo MR images. In
apparent. In some cases, partial fascial tears are seen. some cases, regions of high signal intensity are seen on
T2-weighted spin echo or STIR MR images. Enhance-
ment of signal intensity is variable after the intravenous
Plantar Fibromatosis administration of a gadolinium compound.
Plantar fibromatosis is a common condition associated
with fibrous proliferation and replacement of portions of
the plantar aponeurosis. The disorder is seen in persons Abnormalities of Nerves
Entrapment Neuropathies
Entrapment neuropathies occurring in the foot and ankle
are described in Chapter 65. The most important of
these neuropathies relate to compression of the tibial
nerve in the medial aspect of the ankle (i.e., the tarsal
tunnel syndrome) and entrapment of the deep peroneal
nerve.
The tarsal tunnel is located behind and below the
medial malleolus; its floor is bony, and its roof is formed
by the flexor retinaculum. The resulting fibro-osseous
channel allows passage of the tendons of the tibialis
posterior, flexor digitorum longus, and flexor hallucis
longus muscles; the posterior tibial artery and vein; and
the tibial nerve. Causes of compression of the tibial nerve
within the tarsal tunnel include tumors of nerves and other
structures, tenosynovitis, ganglion cysts (Fig. 59–156), post-
traumatic fibrosis, and dilated or tortuous veins. Many of
A the causes can be defined with CT or MR imaging.
Kursunoglu-Brahme S, Riccio T, Weisman MH, et al: Rheumatoid Peterfy CG, Majumdar S, Lang P, et al: MR imaging of the
knee: Role of gadopentetate-enhanced MR imaging. Radiology arthritic knee: Improved discrimination of cartilage, syn-
176:831, 1990. ovium, and effusion with pulsed saturation transfer and fat-
Lektrakul N, Chung CB, Lai Y, et al: Tarsal sinus: Arthro- suppressed T1-weighted sequences. Radiology 191:413,
graphic, MR imaging, MR arthrographic, and pathologic 1994.
findings in cadavers and retrospective study data in patients Quinn SF, Murray WT, Clark RA, et al: Achilles tendon: MR
with sinus tarsi syndrome. Radiology 219:802, 2001. imaging at 1.5T. Radiology 164:767, 1987.
Linscheid RL, Dobyns JH, Beabout JW, et al: Traumatic insta- Recht MP, Kramer J, Marcelis S, et al: Abnormalities of articular
bility of the wrist: Diagnosis, classification, and pathome- cartilage in the knee: Analysis of available MR techniques.
chanics. J Bone Joint Surg Am 54:1612, 1972. Radiology 187:473, 1993.
Manaster BJ, Remley K, Newman AP, et al: Knee ligament Rockwood CA Jr, Lyons FR: Shoulder impingement syndrome:
reconstruction: Plain film analysis. AJR Am J Roentgenol Diagnosis, radiographic evaluation and treatment with a
150:337, 1988. modified Neer acromioplasty. J Bone Joint Surg Am 75:409,
Mesgarzadeh M, Moyer R, Leder DS, et al: MR imaging of the 1993.
knee: Expanded classification and pitfalls to interpretation of Rosenberg ZS, Beltran J, Bencardino JT: MR imaging of the
meniscal tears. Radiographics 13:489, 1993. ankle and foot. Radiographics 20(Suppl):153, 2000.
Mesgarzadeh M, Schneck CD, Bonakdarpour A, et al: Carpal Rosenberg ZS, Cheung Y, Jahss MH, et al: Rupture of posterior
tunnel: MR imaging. II. Carpal tunnel syndrome. Radiology tibial tendon: CT and MR imaging with surgical correlation.
171:749, 1989. Radiology 169:229, 1988.
Mink JH: Ligaments of the ankle. In Deutsch AL, Mink JH, Shellock FG, Mink JH, Fox JM: Patellofemoral joint: Kinematic
Kerr R (eds): MRI of the Foot and Ankle. New York, Raven MR imaging to assess tracking abnormalities. Radiology
Press, 1992, p 173. 168:551, 1988.
Mink JH: Tendons. In Deutsch AL, Mink JH, Kerr R (eds): Smith DK: Volar carpal ligaments of the wrist: Normal appear-
MRI of the Foot and Ankle. New York, Raven Press, 1992, ance on multiplanar reconstructions of three-dimensional
p 135. Fourier transform MR imaging. AJR Am J Roentgenol
Mink JH, Deutsch AL: Occult cartilage and bone injuries of the 161:353, 1993.
knee: Detection, classification, and assessment with MR Vahey TN, Broome DR, Kaye KJ, et al: Acute and chronic tears
imaging. Radiology 170:823, 1989. of the anterior cruciate ligament: Differential features at
Mitchell DG, Rao VM, Dalinka MK, et al: Femoral head MR imaging. Radiology 181:251, 1991.
avascular necrosis: Correlation of MR imaging, radiographic Weinreb JC, Cohen JM, Maravilla KR: Iliopsoas muscles: MR
staging, radionuclide imaging, and clinical findings. study of normal anatomy and disease. Radiology 156:435,
Radiology 162:709, 1987. 1985.
Murphy BJ: MR imaging of the elbow. Radiology 184:525, Zlatkin MB, Chao PC, Osterman AL, et al: Chronic wrist pain:
1992. Evaluation with high-resolution MR imaging. Radiology
Narváez JA, Narváez J, Ortega R, et al: Painful heel: MR 173:723, 1989.
imaging findings. Radiographics 20:333, 2000. Zlatkin MB, Iannotti JP, Roberts MC, et al: Rotator cuff tears:
O’Donoghue DH: Surgical treatment of fresh injuries to major Diagnostic performance of MR imaging. Radiology
ligaments of the knee. J Bone Joint Surg Am 32:721, 1950. 172:223, 1989.
Pao DG: The lateral femoral notch sign. Radiology 219:800,
2001.
SECTION
XVII
Thermal, Iatrogenic, Nutritional, and
Neurogenic Diseases
CHAPTER 60
Thermal and Electrical Injuries TABLE 60–1
Radiographic Findings Associated with Thermal and
Electrical Injuries
SUMMARY OF KEY FEATURES Soft tissue swelling, loss, or contracture
The radiographic features of the skeleton after thermal Osteoporosis
and electrical injuries are varied. In many cases, they are Acro-osteolysis
not specific and must be interpreted with knowledge of Periostitis
the mechanism of injury. Of particular interest are the Epiphyseal injury and growth disturbance
appearance of epiphyseal abnormalities in frostbite, Articular abnormalities
Periarticular calcification and ossification
periarticular calcification and ossification after thermal Osteolysis, osteosclerosis, and fracture
burns, and pathologic fractures associated with
electrocution.
Differential Diagnosis
The normal occurrence of sclerosis of one or more
epiphyses (ivory epiphyses) should not be misinterpreted
as evidence of epiphyseal necrosis after frostbite. Thiemann’s
disease (epiphyseal acrodysplasia) is a poorly defined
condition occurring predominantly in men in their late
teens; in this disease, swelling of the fingers is associated
with epiphyseal irregularity, sclerosis, and fragmentation.
The distribution of epiphyseal abnormalities, with sparing
of the distal phalanges, differs from that of frostbite.
Ungual tuftal resorption can occur in a variety of condi-
Figure 60–1. Frostbite: late changes. Note brachydactyly,
irregularity, and contour abnormalities of the proximal, middle,
tions other than frostbite, including collagen vascular
and distal phalanges; epiphyseal destruction; and premature disease, epidermolysis bullosa, neuropathic osteoarthrop-
physeal fusion in the proximal phalanges of the second and athy, hyperparathyroidism, sarcoidosis, and psoriatic
third fingers. (Courtesy of D. Wilcox, MD, Kansas City, Mo.) arthritis. In these conditions, the thumb is frequently
affected, and other more diagnostic findings are gener-
ally present.
ELECTRICAL BURNS
General Abnormalities
Electricity can produce severe injury or death. The
extent of bodily harm depends on the type of electricity,
the voltage, the water content of tissues, and the points of
entry and exit of the charge. Electrical injury associated
with a relatively short route—such as a point of entry in
the arm to a point of exit in a finger—produces less tissue
damage than an injury associated with a longer route—
such as entry in the head and exit through a foot. With
alternating currents, muscular contraction may prevent a
person from releasing the source of electricity, leading to
more prolonged and severe tissue damage.
Electrical energy is converted to heat as it traverses
the skin. Resulting burns are accentuated by vascular
spasm, leading to electrical necrosis. Death from low-
voltage electrical injury (<200 volts) usually is caused by
Figure 60–5. Thermal burn: osteophytes, soft tissue calcifi- ventricular fibrillation; death related to high-voltage
cation, and ossification. Several months after a thermal burn, electricity (>1000 volts) is caused by inhibition of the
local ossification (arrowheads) appears along the posterior sur- respiratory center in the brain.
face of the humerus and ulna.
Musculoskeletal Abnormalities
develop within a few months (Fig. 60–7). Initially, fusi- The hand, because of its grasping function, is the most
form soft tissue swelling and osteoporosis are observed commonly affected area. Severe skin burns are encoun-
about the involved joint. Progressive loss of interosseous tered at the points of entry and exit of the electrical
space and subchondral irregularity are seen. Eventually, charge. Osseous and articular changes are related to the
intra-articular osseous fusion may result. Thermal injury, effects of heat, mechanical trauma from accompanying
mechanical trauma, infection, neuropathic osteoarthrop- uncoordinated muscle spasm, neural and vascular tissue
athy, and immobilization are all possible factors in the damage, infection, disuse or immobilization, and, perhaps,
pathogenesis of these articular changes. Contractures a specific effect of the electricity itself.
caused by soft tissue and muscular changes are also fre- Initial radiographic features include loss of cutaneous,
quently observed in burn patients, especially about the subcutaneous, and osseous tissues owing to tissue char-
elbow and hand. ring. Other findings are soft tissue hematomas; compres-
sion fractures of the spine, dislocation of joints, and
avulsions at tendinous insertions related to muscle spasm;
Differential Diagnosis
and various fractures resulting from accompanying falls
The radiographic findings of osteoporosis, periarticular (Fig. 60–8). Small, rounded, dense osseous lesions resem-
calcification and ossification, joint space loss, intra- bling wax drippings may be attributable to the melting of
articular bony ankylosis, and contracture that are bone from the intense heat. Focal, tiny disruptions in
encountered in burn patients may also be seen after bony continuity (microfractures) may also be a direct
paralysis (or immobilization). Heterotopic ossification in effect of the heat. They may have a zigzag contour with
Differential Diagnosis
Figure 60–7. Thermal burn: articular and osseous changes.
In this burned hand, note osteolysis with deformity of The radiographic findings occurring after this type of
phalanges and intra-articular osseous fusion. injury represent a combination of thermal, mechanical,
Figure 60–8. Electrical burn. This man developed changes Figure 60–9. Electrical burn. This 21-year-old man came
in his hands after touching a high-tension wire. The severe into contact with a high-tension wire, resulting in severe injury
alterations in the left hand resulted from sustained grasping to both hands (entrance wounds) and to the pretibial regions
of the wire. They include contracture, soft tissue injury, (exit wounds). Radiograph of a hand reveals contractures of
subluxation, dislocation, and fracture (arrows). The right hand multiple digits and periarticular osteoporosis. (Courtesy of J.
was affected similarly. Barry, MD, Seattle, Wash.)
1000 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
and electrical effects on bones and joints. They are com- Fleckenstein JL, Chason DP, Bonte FJ, et al: High-voltage
plicated by vascular and neurologic damage and second- electric injury: Assessment of muscle viability with MR
ary infection. Some of the imaging abnormalities are imaging and Tc-99m pyrophosphate scintigraphy. Radiology
identical to those that occur after trauma from any cause, 195:205, 1995.
Gralino BJ, Porter JM, Rosch J: Angiography in the diagnosis
whereas others resemble those of septic arthritis, neuro-
and therapy of frostbite. Radiology 119:301, 1976.
pathic osteoarthropathy, osteoporosis, thermal burns, frost- Ogden JA, Southwick WO: Electrical injury involving the
bite, and neurologic deficit. immature skeleton. Skeletal Radiol 6:187, 1981.
Reed MH: Growth disturbances in the hands following thermal
injuries in children. 2. Frostbite. J Can Assoc Radiol 39:95,
FURTHER READING 1988.
Salimi Z, Vas W, Tang-Barton P, et al: Assessment of tissue
Brinn LB, Moseley JE: Bone changes following electrical viability in frostbite by 99mTc pertechnetate scintigraphy.
injury: Case report and review of literature. AJR Am J AJR Am J Roentgenol 142:415, 1984.
Roentgenol 97:682, 1966. Schiele HP, Hubbard RB, Bruck HM: Radiographic changes in
Carrera GF, Kozin F, Flaherty L, et al: Radiographic changes burns of the upper extremity. Radiology 104:13, 1972.
in the hands following childhood frostbite injury. Skeletal Selke AC Jr: Destruction of phalangeal epiphyses by frostbite.
Radiol 6:33, 1981. Radiology 93:959, 1969.
Evans EB, Smith JR: Bone and joint changes following burns: Tishler J: The soft tissue and bone changes in frostbite injuries.
A roentgenographic study—preliminary report. J Bone Joint Radiology 102:511, 1972.
Surg Am 41:785, 1959.
61
CHAPTER 61
Radiation Changes
sometimes multicentric, are seen. The average latent
period of these sarcomas is 23 years. Carcinomas of the
Murray K. Dalinka and Tamara Miner Haygood paranasal sinuses and mastoids have also been reported.
THORIUM
SUMMARY OF KEY FEATURES
Thorium dioxide in dextran (Thorotrast) was introduced
The effects of radiation on bone occur as a result of as a contrast agent in 1928 and was widely used in the
accidental exposure (e.g., radium dial workers) and of United States between 1930 and the early 1950s. Its
diagnostic (e.g., Thorotrast) and therapeutic procedures. clinically inert properties and high atomic number made
Radiation therapy may affect bone growth, cause osseous it the agent of choice for hepatolienography, peripheral
necrosis, and induce neoplasia. Fortunately, these and cerebral angiography, and the opacification of body
complications have decreased in frequency owing to cavities.
advances in the methods of providing such therapy and Extravasation of Thorotrast at the site of injection
to the use of both radiation and chemotherapy. leads to continuous alpha particle irradiation, resulting in
an expanding cicatricial mass (Thorotrastoma) that
invades contiguous structures, leading to tissue destruc-
tion and vascular compromise. The liver, spleen, and
INTRODUCTION bone marrow are subjected to continuous low-dose alpha
radiation. The reticuloendothelial system and adjacent
Within a year after Roentgen published his discovery, the
tissues are therefore at risk for radiation-induced
new technique was in clinical use, chiefly for the diag-
neoplasia, particularly hemangioendothelial sarcoma of
nosis of fractures and the localization of metallic foreign
the liver.
bodies. Deleterious effects of radiation were noted as early
The injection of Thorotrast into growing children may
as 6 months after Roentgen’s initial description, including
give rise to a “bone-within-bone” or “ghost vertebra”
the development of pigmentation, telangiectasia, fibrosis,
appearance (Fig. 61–3). Thorotrast deposition causes
alopecia, scarring, ulceration, and dermatitis. More serious
constant alpha radiation and temporary growth arrest.
was the finding several years later that malignant lesions
The size of the ghost vertebra corresponds to the
could be induced by exposure to diagnostic and thera-
vertebral size at the time of injection.
peutic irradiation, as well as to radioactive agents such as
radium and thorium.
EFFECTS OF RADIATION THERAPY
RADIUM Bone Growth
Radium was used therapeutically, both orally and intra- The effects of radiation on bone growth depend on dosage,
venously, in the treatment of many ailments between quality of the x-ray beam, age of the patient, method of
1910 and 1930. Orally ingested radium is deposited mainly fractionation, length of time after therapy, specific bone
in the outer cortex of bone, and in the 1920s, it was found or bones involved, and coexistence of trauma or infec-
to cause radium jaw (a type of osteomyelitis), severe aplastic tion. These effects include disruption of normal growth
anemia, and osseous neoplasms in radium dial painters. and maturation, scoliosis, osteonecrosis, and neoplasm.
When large quantities of radium are deposited in bone The epiphysis is the area of the bone that is most
for more than 20 years, mostly dead osteoid tissue remains. sensitive to radiation. Decreased growth can occur with
Normal bone physiology becomes erratic, and large resorp- as little as 400 cGy. With a low dose of 600 to 1200 cGy,
tion cavities are formed. These cavities contain gelatinous rapid histologic recovery of radiation-induced changes
material with osteoid-like matrix and appear as sharply occurs. Secondary changes are limited, but some residua
defined bone lesions resembling those of multiple myeloma. may remain. With 1200 cGy or more, the damage is
They occur in the long bones (Fig. 61–1) and skull increased and is maximal to the chondroblasts. Delayed
(Fig. 61–2) and increase in size over time. These areas of changes take 6 months or longer to appear and are dose
cortical resorption are caused by constant alpha particle related; they include vascular damage, premature cartilage
irradiation. degeneration, and bone marrow atrophy. Growth impair-
Metaphyseal sclerosis is frequent, particularly in ment is related to the age of the patient, with the effect
patients who ingest radium before physeal closure. Areas of any given dose being greater at a younger age. The
of increased density probably represent the deposition of greater the growth potential of a particular bone, the more
new bone on unresorbed trabeculae. These density changes drastic is the effect. Any skeletal part capable of growth
may simulate those of Paget’s disease, but the bone is of that is exposed to 2000 cGy or more will show growth
normal size. Pathologic fractures can occur, and frequently disturbances. In pediatric patients, irradiation of the
they heal normally. Osteosarcomas and fibrosarcomas, growing epiphysis or apophysis may cause shortening of
1001
1002 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
Scoliosis
Irradiation to the spine is noted to produce scoliosis. No
radiographic abnormality is seen in patients treated with
Figure 61–2. Skull of a radium dial worker demonstrates less than 1000 cGy. Children who receive 1000 to 2000 cGy
multiple small lucent areas within the calvaria, mainly in the develop changes secondary to growth arrest 9 to 12 months
parietal region, simulating the lesions of multiple myeloma. (From after therapy; these changes consist of horizontal lines of
Dalinka MK, Edeiken J, Finkelstein JB: Complications of increased density parallel to the vertebral endplates and,
radiation therapy: Adult bone. Semin Roentgenol 9:29, 1974.) on occasion, a bone-within-bone appearance. These abnor-
A B
Figure 61–4. Radiation-induced lesion of the distal tibial metaphysis after therapy for Ewing’s tumor of
the proximal end of the tibia. A, Six months after the completion of therapy, a lucent lesion is observed in the
distal metaphysis; it is well defined and has sclerotic margins. A small fracture is also present. B, Six months
after (A), the lesion has filled in with normal-appearing bone. A lucent band is present at the proximal extent
of the lesion. (From Dalinka MK, Mazzeo VP Jr: CRC Crit Rev Diagn Imaging 23:235, 1985.)
Regional Effects
Mandible. Osteonecrosis is considerably more common
in the mandible than in the maxilla because of its com-
pact bone and poor blood supply. The mandible, because
of its superficial location, receives a large dose of radiation
during the treatment of intraoral cancer. Osteonecrosis is
more common when the tumor involves or is adjacent to
bone. The necrosis is usually of a mild and temporary
Figure 61–7. Spinal changes following radiation. This patient nature. Mandibular necrosis may be aseptic or septic; the
was irradiated for a right-sided Wilms’ tumor in childhood.
Note the severe decrease in the height of the vertebral bodies
aseptic or simple type is usually of no consequence.
and a slight scoliosis convex to the left. The ribs and iliac crest In children, radiation to the mandible may result in
on the right side are also hypoplastic. The long tube is present altered patterns of tooth eruption, including malformation
because of intestinal obstruction, which was later shown to be of the tooth or its root. These findings, which are well
secondary to radiation enteritis. displayed with panoramic radiography, may be more
prominent when both radiation and chemotherapy are
employed. Mandibular osteonecrosis may be difficult to
differentiate from tumor recurrence. Recurrence and
entire vertebral body may mitigate the resulting scoliosis. osteoradionecrosis usually both occur within 1 year after
In general, a dose less than 2000 cGy produces no defor- therapy. Mandibular necrosis frequently manifests as a
mity, a dose between 2000 and 3000 cGy is associated poorly defined destructive lesion without sequestration
with a scoliosis of not more than 20 degrees, and a dose (Fig. 61–8). The absence of a soft tissue mass helps in
greater than 3000 cGy produces a curvature greater than differentiating necrosis from tumor recurrence, but an
20 degrees. It has also become apparent that radiation- inflammatory mass may be present with osteonecrosis.
induced scoliosis can be progressive, particularly when
high doses are given to young patients. Skull. Radiation injury to the calvaria may occur after a
maximum absorbed dose of 3600 cGy. The typical
finding of radiation change in the calvaria is a mixed
Radiation Osteitis and Osteonecrosis region of lysis and sclerosis that starts in the epicenter of
The pathologic effects of radiation are independent of the radiation portal and extends outward to the margins
their method of production. The effects of radiation in of the portal (Fig. 61–9). If soft tissue necrosis is
mature bone are mainly on the osteoblasts, with the pri- associated with the calvarial changes, secondary infection
mary event being decreased matrix production. Immediate with osteomyelitis, mastoiditis, or meningitis may occur.
or delayed cell death, injury with recovery, arrest of cel-
lular division, abnormal repair, or neoplasia may occur. Shoulder. Radiation changes in the shoulder girdle after
Historically, Ewing used the term radiation osteitis to therapy for breast carcinoma have been reported in 1%
define the effects of radiation on bone. Abnormalities to 3% of patients. Osteopenia is common after irradia-
include temporary cessation of growth with recovery, tion but also occurs in a substantial number of patients
periostitis, bone sclerosis with increased fragility, ischemic treated by radical mastectomy alone. Osteopenia is com-
necrosis, and infection with osteoradionecrosis. Despite monly associated with a coarse, disorganized trabecular
disagreement about the nature of the bone abnormalities pattern, which may resemble Paget’s disease superficially.
that occur after radiation, it has been established that Rib fractures are also common; they may be subtle and
they are dependent on dosage, quality of the x-ray beam, are frequently painless. The early finding is often a sharp
method of fractionation, length of time after therapy, change in alignment with or without a fracture line. The
Chapter 61 Radiation Changes 1005
Figure 61–8. Oblique view of the
mandible in a patient previously
treated for carcinoma of the floor of
the mouth. There is a poorly defined
destructive lesion in the body of the
mandible, with a pathologic fracture.
No discernible soft tissue mass is
present. This represents mandibular
necrosis.
lesions commonly occur in the anterior or anterolateral resorption. Radiation necrosis of the humerus (see
aspects of the ribs and are usually multiple (Fig. 61–10). Fig. 61–10) can be seen 7 to 10 years after therapy, and
The edges of the fracture fragments are frequently such necrosis is frequently symptomatic. Associated
resorbed, and they may show sclerotic or pointed ends. findings in the ribs, clavicle, and scapula are frequent.
In some cases, the bone resorption is progressive. Changes in the humerus may include resorption cavities,
Clavicular fractures are commonly associated with rib fractures, and ischemic necrosis of the humeral head.
fractures and may also be associated with adjacent bone
Pelvis. Fractures of the femoral neck occurring after
radiation therapy to the pelvis (Fig. 61–11) are reported
to occur in approximately 2% of patients treated with
pelvic radiation. The fractures are usually subcapital in
location and typically involve osteopenic bone. Such frac-
tures may be unilateral or bilateral. Fractures of the femoral
A B
Figure 61–14. Radiation changes of the bony pelvis. This 82-year-old man underwent radiation therapy
for carcinoma of the prostate. A, Coronal T1-weighted (TR/TE, 300/14) spin echo MR image shows regions
of low signal intensity (arrows) involving the ilium and sacrum about both sacroiliac joints. B, Coronal fat-
suppressed fast spin echo (TR/TE, 4400/105) MR image reveals edema (arrows) at the fracture sites. The
fracture lines are poorly seen, but the locations of the lesions are virtually diagnostic of radiation-induced
insufficiency fractures. (Courtesy of C. Beaulieu, MD, Palo Alto, Calif.)
1008 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
A B
Figure 61–16. Ewing’s tumor followed by late radiation
necrosis. A, Oblique view of the femur demonstrates a destruc-
Figure 61–17. Radiation-induced exostosis in the proximal
portion of the humerus after radiation therapy. This appearance
tive diaphyseal lesion with saucerization, perpendicular periosteal
is identical to that of a spontaneously occurring osteochon-
new bone formation, and a large soft tissue mass. Codman’s
droma. (Courtesy of W. E. Berdon, MD, New York.)
triangles are present at both edges of the lesion. B, In the same
patient 31 years later, note the multiple well-defined lucent
areas in the shaft of the femur secondary to radiation necrosis.
irradiated field and are reported with doses ranging from lished as a histologic entity and is the most frequent diagnosis
1600 to 6425 cGy. Malignant degeneration of radiation- in many series of radiation-induced malignancies. Many
induced osteochondromas is exceedingly rare but has soft tissue tumors that would have been considered fibrosar-
been reported. comas in the past are now being called malignant fibrous
histiocytomas.
Malignant Neoplasms. Radiation-induced malignant neo- The evidence that radiation can induce sarcomas is
plasia is a well-recognized complication of radiation therapy. overwhelming. Although sarcomas have been reported
Osseous changes usually precede the development of with doses as low as 800 cGy for the treatment of bursitis,
radiation-induced tumors (Fig. 61–18). Radiation- radiation-induced sarcomas usually require a dose of at
induced neoplasms form in areas that receive radiation least 3000 cGy in 3 weeks, with a threshold appearing at
sufficient to induce mutation but not enough to destroy about 1000 cGy. A higher prevalence of radiation-induced
the regenerating capacity of the bone. tumors would be expected in children because of the
The criteria for the diagnosis of radiation-induced longer potential period at risk; however, the latent period
sarcoma are as follows: does not seem to differ between children and adults. It
1. There must be microscopic or radiographic evidence varies between 4 and 42 years, with an average of 11 years
of a nonmalignant condition. (Fig. 61–19). Radiation-induced bone sarcoma may arise
in previously normal or abnormal bone; however, in one
2. Sarcoma must arise within the irradiated field. series, 70% of patients had preexisting bone disease.
3. A long latent period must be present (at least 4 years). Radiation necrosis can usually be differentiated from
sarcoma arising in irradiated bone, although this may be
4. Histologic proof of sarcoma must be available. difficult in some cases. It may not be possible to distin-
On the basis of these criteria, radiation-induced sarco- guish a new radiation-induced sarcoma from recurrence
mas have been documented in both soft tissue and bone. of the original tumor. Sarcoma, necrosis, and recurrences
Before the mid-1980s, osteosarcoma was the most common all occur within the field of radiation and may be seen
radiation-induced sarcoma in most series. Since the mid- years after treatment. The presence of pain, a soft tissue
1980s, malignant fibrous histiocytoma has become estab- mass, or a new focal lucent area favors the diagnosis of
Chapter 61 Radiation Changes 1009
A B
Figure 61–18. Sarcoma arising in irradiated bone. A, Radiograph 8 years after radiation therapy for breast
carcinoma shows a previous pathologic fracture and pseudarthrosis of the humerus, with radiation changes
in the scapula and humerus. B, In the same patient 6 years later, note an undifferentiated radiation-induced
sarcoma of the humerus. (A, From Dalinka MK, Edeiken J, Finkelstein JB: Complications of radiation
therapy: Adult bone. Semin Roentgenol 9:29, 1974.)
Figure 61–20. Radiation changes on MR imaging. This patient Figure 61–21. Radiation changes in the marrow. This patient
underwent radiation therapy for a tibial lymphoma 2 years underwent radiation therapy to the left pelvis. Coronal T1-
previously. On this sagittal intermediate-weighted (TR/TE, weighted (TR/TE, 622/17) spin echo MR image shows
1600/16) MR image, areas of signal void probably represent increased fatty marrow signal from the left sacrum, ilium and
extremely dense sclerotic bone. This appearance was unchanged ischium.
for more than a year, which, together with involvement of both
the tibia and the fibula, suggests radiation change rather than
new or recurrent tumor.
Thalidomide Osteoporosis
Osteonecrosis
Thalidomide was previously used to induce sleep in
Neuropathic-like arthropathy
pregnant women, with disastrous results. When it is Osteomyelitis (septic arthritis)
ingested during the first trimester, thalidomide exposure Tendinous injury or rupture
produces reduction deformities of the limbs of the fetus. Soft tissue atrophy
The anomalies include dysplasia of the thumb and radial Intra-articular calcification
hemimelia, phocomelia, or complete four-limb amelia; Periarticular calcification
these deformities are associated with hypoplasia or aplasia Accumulation of fat
of the external ear and canal, congenital heart defects,
1012
Chapter 62 Disorders Due to Medications and Other Chemical Agents 1013
Osteoporosis
The occurrence of generalized osteoporosis in patients
receiving systemic corticosteroids has been noted by
many observers. Stress (insufficiency) fractures occur and
heal with extensive callus formation. Collapse of single or
multiple vertebral bodies, with condensation of bone at
the superior and inferior surfaces (Fig. 62–1), and infrac-
tions of ribs are particularly characteristic. The radi-
ographic diagnosis of insufficiency fractures related to
corticosteroid therapy is often difficult, owing to the
associated generalized osteopenia. Although bone scinti-
graphy can be used to enhance the diagnostic accuracy,
false-negative results may be encountered. Computed
tomography and magnetic resonance (MR) imaging are
useful in detecting such fractures in anatomically com-
plex regions. The precise mechanism by which corti-
costeroids lead to bone loss is not known.
Osteonecrosis
The true frequency of steroid-induced osteonecrosis is
difficult to determine because of the many variables
included in different studies. In patients receiving corti-
costeroids after renal transplantation, the frequency
A
appears to be approximately 5% to 10%. In many reviews
of patients with osteonecrosis, steroid medications are
implicated in 20% to 35% of cases. The occurrence of
steroid-induced bone necrosis appears to be directly related
to the dosage level and duration of the medication, and it
may become prominent in many persons after prolonged
high intake. Single or multiple osseous sites can be affected;
the femoral head, humeral head, distal end of the femur,
and proximal part of the tibia are altered most commonly,
in decreasing order of frequency (Fig. 62–2). Radiographic
and pathologic characteristics of osteonecrosis due to
corticosteroids are not unique, being evident in cases of
bony necrosis from other causes (see Chapter 51). As
observed in ischemic necrosis in general, bone scanning
and MR imaging are more sensitive than plain film radi-
ography in detecting sites of osseous necrosis in patients B
receiving corticosteroids (Fig. 62–3). The pathogenesis of
steroid-associated osteonecrosis is not clear (Table 62–2). Figure 62–1. Steroid-induced osteoporosis. This 22-year-old
Changes in the diaphyses and metaphyses of tubular woman, who had received systemic corticosteroid therapy for
bones are encountered less frequently than epiphyseal many months as a child, developed cushingoid features, with
alterations in patients being treated with steroids or other deposition of fat in the shoulders and back and muscle weak-
ness. A, Lateral radiograph obtained during a lumbar myelo-
anti-inflammatory agents. Osteonecrosis of vertebral
gram (for investigation of low back pain) reveals osteopenia,
bodies in association with steroid medications has been biconcave deformities of multiple vertebral bodies, and well-
recognized. Radiolucent linear shadows in the subchon- defined subchondral bone plates. B, Sagittal T1-weighted
dral bone reflect fractures of necrotic bone and are remi- (TR/TE, 200/26) spin echo MR image of the lumbar spine
niscent of the radiolucent areas that appear at other sites shows the biconcave deformities to good advantage. The signal
of osteonecrosis, such as the femoral and humeral heads intensity of the bone marrow reflects the presence of
(Fig. 62–4). This phenomenon is termed the vacuum hematopoietic tissue and is within normal limits. (Courtesy of
vertebral body. Although it is not associated universally G. Greenway, MD, Dallas.)
with ischemic necrosis of bone, it is most suggestive of
that diagnosis.
drug (Table 62–3). It may be associated with osteoporosis,
osteonecrosis, and an underlying articular disorder, such
Neuropathic-Like Articular Destruction as rheumatoid arthritis or osteoarthritis (Fig. 62–5).
A neuropathic-like, rapidly progressive joint disease Although any joint can be affected, the hip and knee are
characterized by severe osseous and cartilaginous involved most frequently. The rapidity of the process can
destruction represents one variety of steroid arthropathy be remarkable. The pathogenesis of the process is not
that typically appears after intra-articular injection of the certain.
1014 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
TABLE 62–2
Pathogenesis of Steroid-Induced Osteonecrosis
TABLE 62–3
Steroid-Induced Neuropathic-Like Alterations
Accumulation of Fat
Endogenous or exogenous corticosteroid excess leads to
abnormal accumulations of fat. Mediastinal lipomatosis Figure 62–7. Steroid-induced intra-articular and periarticular
calcification. In this 57-year-old woman with osteoarthritis
represents one manifestation of this phenomenon, which of the interphalangeal joints of both hands, multiple intra-
may resolve if the steroid dosage is lowered or termi- articular injections of triamcinolone hexacetonide were followed
nated. Similarly, paraspinal localization may be seen. by the development of intra-articular and periarticular calcifi-
Although the masses of fat in the thorax and paraspinal cations (arrow), presumably related to calcium hydroxyapatite
regions rarely lead to clinical manifestations, epidural crystal deposition. (Courtesy of M. Dalinka, MD, Philadelphia.)
1016 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
A B
Figure 62–8. Fluorosis: axial skeletal abnormalities. A, Osteosclerosis and vertebral osteophytosis are
evident in a patient with fluorosis. Note the bony eburnation about the sacroiliac joints. B, In a different
patient, note the extreme increase in radiodensity of the cervical spine, with spinal osteophytes and
ossification of the posterior longitudinal ligament (arrows). (B, Courtesy of G. Beauregard, MD, Montreal,
Quebec, Canada.)
Although the radiographic appearance of osteoporotic certain plasma cell dyscrasias. Periostitis similar to that
bone in patients receiving sodium fluoride is one of pro- seen in fluorosis can be detected in hypertrophic osteo-
gressive osteosclerosis, it is not clear whether a commen- arthropathy, pachydermoperiostosis, and thyroid acropathy.
surate increase in osseous strength occurs. In fact,
fractures of the vertebral bodies and tubular bones during
fluoride treatment have been reported. CALCIUM GLUCONATE
Intravenous administration of calcium gluconate has been
Differential Diagnosis. The combination of findings noted used in the treatment of neonatal tetany and neonatal
on the radiographs of patients with skeletal fluorosis is asphyxia. Subcutaneous masses of calcification can appear
virtually diagnostic. Osteosclerosis, osteophytosis, and at sites of recent or previous injections. Clinical findings
ligamentous calcification represent a useful triad of abnor- include erythema and bulla formation, which become
malities that are evident on pelvis and spine radiographs. evident in days and may be followed by skin sloughing
The other alterations that occur in both axial and extra- and secondary infection. Calcification, which is related to
axial locations, such as periostitis and osseous excrescences tissue necrosis, can be noted within 4 or 5 days or as late
at sites of tendinous and ligamentous attachment, provide as 3 weeks after injection (Fig. 62–9). It is amorphous
additional clues to the correct diagnosis. and can be localized or distributed along fascial planes.
Osteosclerosis alone is not diagnostic of fluorosis; it is Vascular calcification may also be apparent. With heal-
evident in skeletal metastasis, myelofibrosis, mastocytosis, ing, clinical and radiographic manifestations may disappear.
certain hemoglobinopathies, renal osteodystrophy, Paget’s The radiographic changes simulate those in subcuta-
disease, congenital disorders, and other conditions as well. neous fat necrosis and hematomas after trauma.
Likewise, vertebral osteophytosis or similar outgrowths
can accompany many diseases, including fluorosis, spondy-
losis deformans, diffuse idiopathic skeletal hyperostosis, MILK-ALKALI
ankylosing spondylitis, the spondylitis of psoriasis, Reiter’s Milk-alkali syndrome was initially observed in patients
syndrome and inflammatory bowel disorders, acromegaly, with chronic peptic ulcer disease and renal insufficiency
neuropathic osteoarthropathy, and alkaptonuria. Prolifera- in whom excessive intake of milk and alkali (over a few to
tive changes at ligamentous and tendinous insertions in many years) had led to metastatic calcification. These
bones are apparent not only in fluorosis but also in patients exhibit hypercalcemia without hypercalciuria or
diffuse idiopathic skeletal hyperostosis, hypoparathy- hypophosphatemia, normal serum alkaline phosphatase
roidism, X-linked hypophosphatemic osteomalacia, and levels, azotemia, and mild alkalosis. Soft tissue calcifi-
1018 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
PROSTAGLANDINS RETINOIDS
Prostaglandin E1 is commonly used to maintain the Natural and synthetic forms of vitamin A, known collec-
patency of the ductus arteriosus in neonates with ductus- tively as retinoids, have been employed in the treatment
Chapter 62 Disorders Due to Medications and Other Chemical Agents 1019
Figure 62–11. Prostaglandin periostitis.
In this 3-month-old infant receiving
prostaglandin E1 infusions, exuberant
periosteal new bone is observed in the ribs,
humerus, radius, and ulna. (Courtesy of T.
Broderick, MD, Orange, Calif.)
of various dermatologic disorders. Reports indicate that to larger osteophytic outgrowths (Fig. 62–13). In the
the long-term use of isotretinoin is associated with skeletal appendicular skeleton, pointlike hyperostosis is seen at
hyperostosis resembling that seen in chronic vitamin A the corners and promontories of bones, such as the tarsal
intoxication. Both axial and appendicular skeletal sites are navicular and calcaneus. Bilateral and symmetrical changes
affected. Vertebral alterations predominate in the cervical occur more frequently than unilateral abnormalities.
region, although thoracic and lumbar spinal changes may Clinical manifestations are absent or mild.
also be evident. The findings, which are usually subtle and
require comparison with previous radiographic studies
QUINOLONES
for identification, consist of pointed osseous excrescences
arising from the anterior and posterior margins of the A number of complications, including arthralgias, arthritis,
vertebral bodies; with time, these excrescences progress myalgia, tendinosis, and spontaneous tendon ruptures,
A B
Figure 62–12. Prostaglandin periostitis. This male infant was noted to have multiple congenital
abnormalities at birth. A cardiac catheterization confirmed the presence of a severe tetralogy of Fallot. An
infusion of prostaglandin E was begun when the patient was 3 weeks old and maintained for 7 weeks, after
which it was discontinued. Bone changes were observed initially in the ribs and clavicle after 20 days of
therapy. A, Radiograph of the forearm, obtained when the infant was 43 days old, reveals extensive periosteal
elevation in the ulna. Note the absence of the radius, a finding of the VATER syndrome (consisting of
vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dyslasia). B, At 6 months
of age, the periosteal bone has been incorporated into the diaphysis of the ulna. (From Poznanski AK,
Fernbach SK, Berry TE: Bone changes from prostaglandin therapy. Skeletal Radiol 14:20, 1985.)
B
Figure 62–13. Isotretinoin hyperostosis. A, Lateral radiograph of the cervical spine obtained after 1 year
of therapy shows “pointing” of the bone at the anteroinferior and posteroinferior portions of the vertebral
bodies (arrowheads). B, In a different patient, prominent ligamentous ossification (arrows) is apparent in the
midcervical spine. (A, From Pennes DR, Ellis CN, Madison KC, et al: Early skeletal hyperostoses secondary
to 13-cis-retinoic acid. AJR 142:979, 1984.)
1022
Chapter 63 Hypervitaminosis and Hypovitaminosis 1023
Figure 63–1. Hypervitaminosis
A: periostitis and cortical hyper-
ostosis. A, Note periosteal bone
formation in the diaphysis of the
ulna (arrowheads). B, In a different
child, periosteal proliferation is
evident in the diaphyses of multi-
ple metatarsals (arrowheads). (A,
Courtesy of F. Silverman, MD,
Stanford, Calif.)
A B
TABLE 63–1
Hypervitaminosis A versus Infantile Cortical Hyperostosis
(Caffey’s Disease)
Infantile Cortical
Factor Hypervitaminosis A Hyperostosis
Age of onset End of first year of life First 6 mo of life
Findings Soft tissue nodules Soft tissue nodules
Periostitis and Periostitis and
hyperostosis hyperostosis
Metaphyseal changes Growth disturbances
Growth disturbances
Increased intracranial
pressure
Sites of hyperostosis Ulna Mandible
(descending order Metatarsal Clavicle
of frequency) Clavicle Scapula
Tibia Rib
Fibula Tubular bones
Metacarpal
Other tubular bones
Rib
Etiology Vitamin A poisoning Unknown; possibly
a viral disease
A radiograph of the end of an involved tubular bone between the provisional zone of calcification and the
reveals several zones (Table 63–2; Fig. 63–4). A radio- heavy spongiosa deeper in the shaft is a transverse band
dense line borders on the growth plate, representing the of diminished density, the scurvy line. Small, beaklike
sclerotic provisional zone. On its metaphyseal side outgrowths of the metaphysis, incomplete or complete
separation of the plate from the shaft due to subepi-
physeal marginal clefts and infractions (corner sign or
TABLE 63–2
Hypovitaminosis C (Scurvy): Radiographic-Pathologic
Correlation
A B
angle sign), extensive physiolysis with osseous malalign- cupping in a bilateral or unilateral distribution can lead
ment, and periosteal elevation with new bone formation to intrusion of the epiphysis into the exaggerated con-
due to subperiosteal hemorrhage complete the distinctive cavity of the metaphysis and apparent early fusion of the
radiographic picture of the metaepiphyseal regions of growth plate. This complication is not frequent, and the
tubular bones (and costochondral junctions). These pathogenesis of metaphyseal cupping is not clear.
changes are most marked in areas of active endochondral
bone formation. Effects of Therapy. With treatment of the disease, there is
thickening of the cortex, increased density of the radio-
Epiphyseal Changes. In the ossification centers of the lucent zone of the metaphysis, transverse densities within
epiphyses of tubular bones and in the carpus and tarsus, the shaft caused by burying of the thickened provisional
similar but less marked alterations are seen. Persistence zone of calcification, massive subperiosteal bone formation
and thickening of the provisional zone of calcification that later merges with the underlying cortex, sponta-
produce a radiodense shell around the ossification center neous shifting of the diaphysis to realign with the dis-
that is accentuated by central rarefaction owing to atrophy placed epiphysis, and increased density of the epiphysis.
of the adjacent spongiosa (Wimberger’s sign of scurvy).
Differential Diagnosis. The appearance of a radiolucent
Diaphyseal Changes. Atrophy of spongiosa in the shafts metaphyseal band in scurvy is not a pathognomonic
of tubular bones accounts for a nonspecific decreased finding and may be seen in other chronic illnesses, such
radiodensity and a “ground-glass” appearance of the as leukemia and neuroblastoma. The identification of
diaphyses. Cortical diminution is common and often radiodense lines at the metaphysis and about the epiph-
severe, yet fracture of the shafts is unusual. Subperiosteal ysis, metaphyseal fracture, osseous beaks, epiphyseal
hemorrhage is most frequent in the larger tubular bones displacement, and diaphyseal periostitis allows an accu-
such as the femur, tibia, and humerus. The degree of rate diagnosis of scurvy. Leukemia can lead to periostitis
hemorrhage is variable and can produce focal or diffuse and diaphyseal destruction in combination with bandlike
elevation of the periosteal membrane. Radiographically, metaphyseal radiolucency, but fracture and epiphyseal
soft tissue masses of increased density, displacement of separation are not present. Syphilis produces sym-
adjacent bones, and small or large shells of periosteal metrical destructive foci in the metaphyses, particularly
bone, particularly during the healing phase of the disease, in the proximal tibia, but the distinctive findings are not
are observed. confused with those of scurvy. Metaphyseal changes can
also accompany rubella, cytomegalic inclusion disease,
Articular Changes. Hemarthrosis, a rare manifestation of toxoplasmosis, and a variety of traumatic and dysplastic
scurvy, demonstrates a predilection for the large weight- disorders.
bearing joints of the lower extremities.
Adult Scurvy
Growth Disturbances. Permanent growth disturbances are
unusual after scurvy, despite the frequency and severity of Currently, scurvy is encountered infrequently in adults,
epiphyseal separations. Central segmental metaphyseal although it may be observed in severely malnourished
1026 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
persons, especially the elderly. A protracted period of Metastatic soft tissue calcification accompanies a
vitamin deficiency is required for clinical expression. variety of disorders, including hypervitaminosis D, milk-
Nonspecific weakness, anorexia, weight loss, and fatigue alkali syndrome, hyperparathyroidism, plasma cell
usually antedate the more diagnostic hemorrhagic mani- myeloma, and skeletal metastasis. Further, soft tissue
festations, such as petechiae and ecchymoses of the skin, calcinosis may be evident in various collagen vascular
subcutaneous tissues, and gums. disorders, and dystrophic calcification can occur after
Hemarthrosis and bleeding at synchondroses may be tissue injury or devitalization from any cause. Periartic-
observed in adult scurvy. Osteoporosis is prominent in ular deposits are most characteristic of hypervitaminosis
the axial skeleton, especially the spine, and in the tubular D, milk-alkali syndrome, hyperparathyroidism, renal
bones of the appendicular skeleton. osteodystrophy, collagen vascular disorders, and idio-
pathic tumoral calcinosis.
HYPERVITAMINOSIS D
Excessive intake of vitamin D can be associated with Chronic Idiopathic Hypercalcemia
clinical and radiographic manifestations in both children Chronic idiopathic hypercalcemia relates to excessive
and adults. Initial reports noted findings of intoxication ingestion of vitamin D over prolonged periods by infants
in infants and young children who had been given exces- who are slightly sensitive to this vitamin. The major
sive levels of vitamin D for the treatment of rickets and clinical manifestations of this disorder include a peculiar
other skeletal disorders; similar intoxication was later facies, anorexia, hypotonia, mental and physical retarda-
identified in adult patients who were receiving inordinate tion, and vomiting. Radiographically, a generalized increase
amounts of vitamin D for the treatment of Paget’s in skeletal density is observed.
disease. Infants with Williams’ syndrome demonstrate a facies
similar to that of patients with idiopathic hypercalcemia;
they also have supravalvular aortic stenosis and mental
Musculoskeletal Abnormalities
retardation. The detection of hypercalcemia and these
Vitamin D poisoning can be acute or chronic. The level variable vascular anomalies suggests that vitamin D excess
of the vitamin required to produce toxic symptoms and or hypersensitivity in the expectant mother may be a
signs is extremely variable. Acute clinical manifestations fundamental cause of congenital malformations of the
may appear within 3 to 9 days after massive doses of the cardiovascular system of the fetus.
vitamin and include vomiting, fever, dehydration, abdom-
inal cramps, bone pain and tenderness, convulsions, and
coma. With chronic poisoning, lassitude, thirst, anorexia,
and polyuria are followed by vomiting, abdominal pain,
and diarrhea.
In infants and children, metaphyseal bands of increased
density, reflecting heavy calcifications of the matrix of the
proliferating cartilage, alternating with areas of increased
lucency are evident in the tubular bones. Cortical thick-
ening due to periosteal apposition may be observed at
certain sites, and osteoporosis and thinning of the cortices
may be evident at other locations. Metastatic calcification
of viscera, blood vessels, periarticular structures, muscles,
laryngeal and tracheal cartilage, falx cerebri, and tento-
rium cerebelli may be noted.
In adults, hypervitaminosis D can lead to focal or
generalized osteoporosis. The bones of the appendicular
skeleton, the spine, the pelvis, and even the skull reveal
varying degrees of bone loss. Massive soft tissue calcifi-
cation can become apparent. Lobulated, smooth, amor-
phous masses of calcium are evident in periarticular
regions, bursae, tendon sheaths, joint capsules, and intra-
articular cavities (Fig. 63–5). Pathologic evaluation reveals
thick, white, granular calcareous material, usually repre-
senting calcium hydroxyapatite, and an inflammatory Figure 63–5. Hypervitaminosis D: soft tissue abnormalities.
reaction in the bursal wall or adjacent soft tissues can be In a patient with rheumatoid arthritis who was treated with
seen. vitamin D, note massive soft tissue calcification about the wrist.
Chapter 63 Hypervitaminosis and Hypovitaminosis 1027
FURTHER READING Keating JP, Feigin RD: Increased intracranial pressure associated
with probable vitamin A deficiency in cystic fibrosis. Pediatrics
Caffey J: Chronic poisoning due to excess of vitamin A: 46:41, 1970.
Description of the clinical and roentgen manifestations in Miller JH, Hayon II: Bone scintigraphy in hypervitaminosis A.
seven infants and young children. Pediatrics 5:672, 1950. AJR 144:767, 1985.
Caffey J: Traumatic cupping of the metaphyses of growing Shiers JA, Neuhauser EBD, Bowman JR: Idiopathic hyper-
bones. AJR 108:451, 1970. calcemia. AJR 78:19, 1957.
Christensen WR, Liebman C, Sosman MC: Skeletal and peri- Singleton EB: The radiographic features of severe idiopathic
articular manifestations of hypervitaminosis D. AJR 65:27, hypercalcemia of infancy. Radiology 68:721, 1957.
1951. Sprague PL: Epiphyseo-metaphyseal cupping following infantile
Holman CB: Roentgenologic manifestations of vitamin D scurvy. Pediatr Radiol 4:122, 1976.
intoxication. Radiology 59:805, 1952.
Joffe N: Some radiological aspects of scurvy in the adult. Br J
Radiol 34:429, 1961.
64
CHAPTER 64
Heavy Metal Poisoning and
underscores the early belief that their appearance signi-
Deficiency fied an arrest in normal growth; however, they are more
properly termed recovery lines, indicating periods of
renewed or increased growth, presumably after a period
of inhibited growth of the bone.
SUMMARY OF KEY FEATURES Growth arrest (or recovery) lines are also observed on
In some cases of heavy metal poisoning, radiodense magnetic resonance (MR) images. They appear as curvi-
lines or bands may appear in the metaphyses of tubular linear regions of low signal intensity, producing a bone-
bones and within flat or irregular bones. The resulting within-bone appearance (Fig. 64–3). The more central
radiographic findings, which are characteristically aspect often contains fatty marrow of higher signal intensity
observed in lead, phosphorus, and bismuth poisoning, than that in the periphery.
must be differentiated from normal variants, stress lines Transversely or obliquely oriented radiodense lines
of Park or Harris, and changes in various metabolic, are regularly encountered in the tubular bones of adults
endocrine, and infectious disorders. Significant with osteopenia. These lines, which are termed bone bars or
radiographic abnormalities are also observed in the reinforcement lines, are discussed more fully in Chapter
aluminum accumulation that accompanies dialysis and 41. They are especially frequent in the distal portion of the
in nutritional or inherited deficiencies of copper. femur and the tibia in persons with long-term osteo-
Finally, metal debris (metallosis) may complicate joint porosis, and they should be regarded as an adaptive response
surgery. of chronically weakened bone to normal or abnormal
stress, not as a manifestation of growth disturbance.
LEAD POISONING
INTRODUCTION
General Abnormalities
Poisoning with certain heavy metals can produce charac-
teristic musculoskeletal alterations. One of the most Lead poisoning results from prolonged ingestion of lead-
well-recognized changes associated with the ingestion, containing materials such as paint chips, ceramics, and
inhalation, or injection of lead or other heavy metals is drinking water; inhalation of fumes from burning storage
the appearance of radiodense lines at the ends of tubular batteries or similar substances; or, infrequently, absorption
bones or along the contours of flat and irregular bones. of the material from bullets or buckshot within a serous
These lines must be differentiated from the transverse cavity after a wound. Lead poisoning may also occur in
radiodense areas that are commonly observed at some of the fetus of a pregnant woman exposed to lead, because
these locations in persons who have not been poisoned lead can cross the placenta. Delayed dental and skeletal
with metals. development, lead lines, and osteosclerosis may be evident
in infants with congenital lead poisoning.
The onset of symptoms and signs after chronic (sub-
TRANSVERSE OR STRESS LINES clinical) lead poisoning may be abrupt. Cramping abdomi-
The appearance of opaque transverse lines that extend nal pain; encephalopathy with convulsions, delirium, coma,
across the metaphyses of tubular bones is a common or death; peripheral neuritis with muscular paralysis; and
radiographic phenomenon that may be observed in children mild anemia are recognized clinical manifestations of the
and adults (Fig. 64–1). They are often referred to as Park chronic disorder. Examining the urine for porphyrin is a
or Harris lines or as transverse or growth arrest lines. useful screening test to establish the diagnosis.
Investigations of these lines have emphasized that they
can occur in both healthy and sick persons, that similar
Musculoskeletal Abnormalities in Infants
lines have been encountered in patients poisoned with a
and Children
variety of heavy metals, and that the lines may be used as
a determinant of growth potential and magnitude. Lead poisoning is associated with the appearance of thick,
Transverse radiodense lines may be evident at birth or transverse, radiodense lines in the metaphyses of growing
during infancy; they do not appear after growth has ceased, tubular bones. Deposition of calcium is the basis of the
but once formed, they persist into adult life. Similar lines transverse lines; even though lead is deposited in the meta-
can appear about the margins of the round and the flat physis, the amounts are minute in relation to the content
bones and in both the appendicular and axial portions of of calcium. Lead lines, which are almost invariable in
the skeleton (Fig. 64–2). chronic infantile and juvenile plumbism, are not an early
The close proximity of and similarity between the lines manifestation of the process and, therefore, are of little
and the growth plate suggest that the bands are related to diagnostic aid. The radiodense zones are especially promi-
a disturbance of normal growth patterns. Indeed, the term nent in the bones about the knee (Fig. 64–4); identifi-
arrest lines, which is still applied to these dense shadows, cation of density in the proximal fibular metaphysis may
1028
Chapter 64 Heavy Metal Poisoning and Deficiency 1029
A B
Figure 64–3. Transverse growth recovery lines. In sagittal intermediate-weighted (TR/TE, 2500/16)
(A) and transaxial T1-weighted (TR/TE, 722/20) (B) spin echo MR images, note the bone-within-bone
appearance (arrows), with the marrow within the central bone having a higher signal intensity (arrowheads).
1030 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
Differential Diagnosis
Radiodense lines in the metaphyses of tubular bones can
be seen as a normal variant, as an indication of previous
stress, in heavy metal poisoning (including lead poisoning),
and in the healing stages of leukemia, rickets, and scurvy
(Table 64–1). Similar findings may accompany hypothy-
roidism, hypervitaminosis D, and transplacental infections
(rubella, cytomegalic inclusion disease, herpes simplex,
toxoplasmosis, and syphilis). Metaphyseal flaring or
widening is seen not only in lead poisoning but also in
various anemias (sickle cell anemia, thalassemia), storage
Figure 64–5. Lead poisoning: wrist. Transverse radiodense disorders (Gaucher’s disease, Niemann-Pick disease), and
bands of the metaphyses of radius and ulna. congenital syndromes (Pyle’s disease, multiple familial
exostoses, osteopetrosis, Ollier’s disease) and as a normal
variant.
PHOSPHORUS POISONING
Phosphorus poisoning was previously seen in rachitic and
tuberculous children who were being treated with phos-
phorized cod liver oil, although it is encountered infre-
quently today. Single or multiple deep bands of increased
radiodensity are produced in the ends of growing tubular
bones and within flat or irregular bones and persist for
many years.
BISMUTH POISONING
Figure 64–6. Lead poisoning: radiographic abnormalities.
Observe fragmentation of a missile (arrow), with opaque fragments In a pregnant woman with syphilis, injected bismuth may
present in the knee joint. cross the placenta and enter the fetal circulation to be
Chapter 64 Heavy Metal Poisoning and Deficiency 1031
TABLE 64–1
Historical Summary of Early Published Studies on Lines and Bands
1032 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
TABLE 64–1
Historical Summary of Early Published Studies on Lines and Bands Continued
From Garn SM, Silvermann FN, Hertzog KP, et al: Lines and bands of increased density: Their implication to growth and development. Med Radiogr Photogr 44:60,
1968.
deposited in the skeleton. Single or multiple radiodense dialysis encephalopathy. Further, bone toxicity, mani-
bands or lines in the metaphyses of the tubular bones in fested clinically as pain, tenderness, and deformity, is
cases of bismuth poisoning share radiographic and mor- accompanied by the accumulation of aluminum in
phologic characteristics with those that appear in cases of osseous tissue.
lead poisoning. Osteopenia (related to osteomalacia), Looser’s zones
(insufficiency fractures), and complete fractures affecting
the ribs, spine, and tubular bones are the principal radio-
ALUMINUM TOXICITY
graphic manifestations of aluminum-induced osteopathy.
The oral or parenteral administration of aluminum com- Periosteal new bone in the shafts of the tibia and fibula
pounds may lead to its deposition in the body’s tissues and in the pelvis has also been observed. In children,
and, in some sites—such as the brain and bones—to toxic rickets-like metaphyseal abnormalities appear.
effects. This situation can arise in nonuremic adults, some
of whom have peptic ulcer disease, who are ingesting
large amounts of aluminum carbonate; in uremic patients COPPER DEFICIENCY
who ingest aluminum salts as phosphate binders and A disorder associated with hypocupremia has been reported
whose renal clearance of aluminum is reduced or who are in infants who are malnourished, those receiving long-
undergoing hemodialysis, and in patients who have lost term parenteral nutrition, or those born prematurely and
small bowel function and are maintained on chronic total maintained on diets low in copper. Radiographic findings
parenteral nutrition. include osteopenia, metaphyseal radiodense lines and
The toxic effects of aluminum on the brain and bones spurs, fractures, and physeal disruptions with malalign-
have been well studied in patients with chronic renal ment of the epiphysis and metaphysis. Periostitis, which
failure who are maintained on regular hemodialysis or, may become profound, is also observed. Reversal of the
rarely, on peritoneal dialysis (see Chapter 46). Accu- osseous abnormalities occurs when copper supplementation
mulation of aluminum appears to play a major role in is employed.
Chapter 64 Heavy Metal Poisoning and Deficiency 1033
Menkes’ syndrome (kinky-hair syndrome) is an inherited FURTHER READING
disorder with an X-linked pattern of transmission that
relates to a defect in copper absorption from the gut. Andreoli SP, Smith JA, Bergstein JM: Aluminum bone disease
Radiographic findings are similar to those in nutritional in children: Radiographic features from diagnosis to reso-
copper deficiency, consisting of osteopenia, flaring of the lution. Radiology 156:663, 1985.
Ashkenazai A, Levin S, Djaldetti M, et al: The syndrome of
ends of the tubular bones, metaphyseal cupping and neonatal copper deficiency. Pediatrics 52:525, 1971.
irregularity, and osseous spurs. The changes resemble Blickman JG, Wilkinson RH, Graef JW: The radiologic “lead
those of rickets and the abused child syndrome. band” revisited. AJR Am J Roentgenol 146:245, 1986.
Chew FS, Ramsdell MG, Keel SB: Metallosis after total knee
replacement. AJR Am J Roentgenol 170:1556, 1998.
METAL DEBRIS (METALLOSIS) ASSOCIATED Danks DM, Campbell PE, Stevens BJ, et al: Menkes’ kinky hair
WITH JOINT IMPLANTS syndrome: An inherited defect in copper absorption with
Although the complications resulting from joint surgery widespread effects. Pediatrics 50:188, 1972.
are discussed in Chapter 14, a few comments regarding DeMartini J, Wilson A, Powell JS, et al: Lead arthropathy and
one of these, metal debris (or metallosis), are appropriate systemic lead poisoning from an intraarticular bullet. AJR
Am J Roentgenol 176:1144, 2001.
here. Such debris may occur in large amounts in tissues Edwards DK III: Skeletal growth lines on radiographs of
surrounding failed prostheses, especially those con- newborn infants: Prevalence and possible association with
taining titanium. Although metallosis is relatively rare, obstetric abnormalities. AJR Am J Roentgenol 161:141,
increased serum and urine levels of some metals (e.g., 1993.
cobalt, chromium, nickel) have been noted in patients Follis RH Jr, Park EA: Some observations on bone growth with
with knee and hip prostheses, especially if the prostheses particular respect to zones and transverse lines of increased
are loose. Dissemination of metal debris to regional density in the metaphysis. AJR Am J Roentgenol 68:709,
lymph nodes has been documented. Concern has been 1952.
expressed about the effects of particulate metal debris on Garn SM, Silverman FN, Hertzog KP, et al: Lines and bands of
the immune system, with the potential development of increased density: Their implication to growth and develop-
ment. Med Radiogr Photogr 44:58, 1968.
hypersensitivity reactions, infections, and tumors. Garrett P, McWade M, O’Callaghan J: Radiological assessment
Within the involved joint, metal debris in the synovial of aluminum-related bone disease. Clin Radiol 37:63, 1986.
membrane leads to a diagnostic radiographic appearance, Klinenberg JR: Saturnine gout—a moonshine malady. N Engl
with radiodense material observed at the periphery of the J Med 280:1238, 1969.
joint (Fig. 64–7). Arthrocentesis documents the presence Leonard MH: The solution of lead of synovial fluid. Clin Orthop
of thick, dark gray or black fluid. 64:255, 1969.
Leone AJ Jr: On lead lines. AJR Am J Roentgenol 103:165,
1968.
Park EA: The imprinting of nutritional disturbances on the
growing bone. Pediatrics 33:815, 1964.
Pearl M, Boxt LM: Radiographic findings in congenital lead
poisoning. Radiology 136:83, 1980.
Pease CN, Newton GG: Metaphyseal dysplasia due to lead
poisoning in children. Radiology 79:233, 1962.
Sachs HK: The evolution of the radiologic lead line. Radiology
139:81, 1981.
Sclafani SJA, Vuletin JC, Twersky J: Lead arthropathy: Arthritis
caused by retained intra-articular bullets. Radiology 156:
299, 1985.
Sebes JI, Pinstein ML, Massie JD, et al: Radiographic
manifestations of aluminum-induced bone disease. AJR Am
J Roentgenol 142:424, 1984.
Wesenberg RL, Gwinn JL, Barnes GR Jr: Radiological findings
in the kinky hair syndrome. Radiology 92:500, 1969.
Figure 65–5. Pathogenesis of coxa valga. A, Normal. The Osseous, Articular, and Soft Tissue Infection
growth zones in this region are the articular cartilage, longi-
tudinal growth plate (LGP), femoral neck isthmus (FNI), and Generalized or localized infections frequently develop in
trochanteric growth plate (TGP). The triradiate cartilage (TRC) immobilized and debilitated patients with neuromuscular
is present on the acetabular side of the joint. The direction of diseases. Skin breakdown over pressure points is a common
growth is indicated by small and large bold arrows. The double- and frustrating problem in these patients, and with penetra-
headed arrows indicate the normal neck-shaft angle. B, Coxa tion of the subcutaneous and fascial tissue, infection may
valga. Weakness in the abductor musculature reduces the growth reach the underlying bones and joints. The ischial tuberosi-
stimulus to the trochanteric growth plate and the femoral neck
isthmus (open arrows). The horizontally oriented longitudinal
ties, femoral trochanters, hip joints, and other bony protu-
growth plate continues to lengthen the femoral neck in line berances are typically affected, and the characteristic
with the femoral shaft (small and large bold arrows). A valgus radiographic findings of osteomyelitis or septic arthritis
angulation results (double-headed arrows). (Modified from Siffert (or both) ensue.
RS: Patterns of deformity in the developing hip. Clin Orthop
160:14, 1981.)
A B
Figure 65–9. Heterotopic ossification: MR imaging. In this 37-year-old man with quadriplegia and soft
tissue swelling about the hip, axial (A) and coronal (B) fat-suppressed T1-weighted (TR/TE, 500/14) spin
echo MR images obtained after intravenous gadolinium administration show muscle and soft tissue
enhancement of signal intensity, with central nonenhancing areas (arrows). Aspiration failed to document
infection. Also note the increased signal intensity in the ischial tuberosity in (A), related to chronic stress in
this bedridden patient.
narrowing, which may progress to intra-articular bony and consist of disc space loss, bone sclerosis, fragmentation,
ankylosis, simulating that of ankylosing spondylitis osteophytosis, and subluxation; they are similar to abnor-
(Fig. 65–11). malities appearing in diabetes mellitus, syringomyelia,
and syphilis (see Chapter 66). In other instances, bony
outgrowths of the spine develop, with characteristics that
Spondylopathy resemble those of the paravertebral ossification that
A variety of spinal abnormalities occur in paralyzed per- accompanies psoriatic arthritis or Reiter’s syndrome, the
sons. Some of them represent neuropathic osteoarthropathy flowing ossification of diffuse idiopathic skeletal hyper-
ostosis, or the thin excrescences of ankylosing spondylitis
(see Fig. 65–11).
Entrapment Neuropathies
Peripheral nerve entrapment syndromes involve the com-
pression of a short segment of a single nerve at a specific
site, frequently as a result of the vulnerability of that nerve
as it passes through a fibro-osseous tunnel or an opening
in fibrous or muscular tissue. The clinical manifestations
Figure 65–11. Sacroiliac joint and spinal abnormalities. In vary with the specific site of involvement. In some cases,
this patient, who had been quadriplegic for more than 5 years, the manifestations are persistent; in others, they occur
radiograph shows loss of interosseous space in both sacroiliac only with certain maneuvers of the extremity. The latter
joints (arrowheads) and spinal ossification. situation is often referred to as dynamic compression neu-
ropathy. A variety of provocative clinical tests have been
described in which gentle pressure on the nerve at the
site of entrapment is accompanied by paresthesias in the
Miscellaneous Abnormalities distribution of that nerve. These provocative tests, when
Significant digital clubbing may be encountered in applied carefully and combined with data derived from
paralyzed patients. This finding is seen in patients who the clinical history and routine physical examination, aid
have been paralyzed for more than a year, and it appears in the assessment of entrapment neuropathies. Entrapment
to correlate with the presence of decubitus ulcers and neuropathies typically involve the median, ulnar, radial,
persistent subcutaneous edema. musculocutaneous, suprascapular, dorsoscapular, or brachial
TABLE 65–1
Classification of Peripheral Neuropathies
From Sherman DG: Peripheral neuropathies. In Stein JG (ed): Internal Medicine. Boston, Little, Brown, 1983, p 872.
1040 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
plexus nerve in the upper extremities and the sciatic, Pronator Syndrome. An entrapment neuropathy of the
common peroneal, tibial, femoral, saphenous, lateral femoral median nerve (Fig. 65–13) can occur in the antecubital
cutaneous, obturator, ilioinguinal, or genitofemoral nerve area where the nerve passes between the two heads of the
in the lower extremity (Table 65–2). pronator teres muscle and then under the edge of the
flexor digitorum sublimis muscle.
Median Nerve Anterior Interosseous Nerve Syndrome (Kiloh-Nevin
Entrapment of the median nerve occurs most frequently Syndrome). The anterior interosseous nerve, the largest
in the wrist as the carpal tunnel syndrome, but it may also branch of the median nerve, is purely motor and supplies
develop in the region of the distal end of the humerus or the flexor pollicis longus, pronator quadratus, and radial
proximal end of the forearm. part of the flexor digitorum profundus muscles. Its com-
pression leads to weakness of the thumb and index finger
Ligament of Struthers. In approximately 1% of limbs, the and occurs on an idiopathic basis, after radial or supra-
ligament of Struthers connects an anomalous bony excres- condylar humeral fractures.
cence (the supracondylar process) arising from the
anteromedial surface of the distal portion of the humerus Carpal Tunnel Syndrome. The most frequent entrapment
to the medial epicondyle of the humerus. Compromise of syndrome of the median nerve occurs as the nerve passes
the median nerve is a possible complication (Fig. 65–12). through the narrow fibro-osseous tunnel that exists between
TABLE 65–2
Entrapment Neuropathies
TABLE 65–2
Entrapment Neuropathies Continued
TABLE 65–2
Entrapment Neuropathies Continued
the carpal bones and a roof consisting of the inelastic cles. Physical examination documents a positive Tinel’s
transverse carpal ligament. Although the carpal tunnel sign (paresthesia after percussion of the nerve in the volar
view allows analysis of the osseous components of this aspect of the wrist).
passageway, delineation of the fibrous roof and the struc- Four general findings characterize the MR imaging
tures within the canal requires ultrasonography or MR appearance of carpal tunnel syndrome: (1) swelling of the
imaging (Fig. 65–14). median nerve, best evaluated at the level of the pisiform
Causes of carpal tunnel syndrome are numerous bone; (2) flattening of the median nerve, judged most
(Table 65–3), but many cases occur on an idiopathic basis. reliably at the level of the hamate bone; (3) palmar bowing
Most patients are between the ages of 35 and 65 years. of the flexor retinaculum, best visualized at the level of
Women are affected more frequently than men, and the hamate bone; and (4) increased signal intensity of the
although bilateral involvement is common, the dominant median nerve on T2-weighted spin echo MR images
hand is usually involved first and more severely. Aggra- (Fig. 65–15).
vating factors include occupations that require a great
deal of repetitive hand and wrist motion, pregnancy, and
the use of oral contraceptive agents. Clinical findings Ulnar Nerve
include sensory and motor deficits, with numbness and
paresthesias of the thumb, index, middle, and half of the Entrapment of the ulnar nerve occurs most commonly
ring finger and atrophy and weakness of the thenar mus- near the elbow or wrist and rarely in the forearm.
1046 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
C
Figure 65–12. Entrapment of the median nerve: supracondylar process. A and B, Radiograph (A) and
photograph (B) show an osseous outgrowth (arrows) from the anterior surface of the distal portion of the
humerus. The ligament of Struthers may extend from its tip to the medial epicondyle of the humerus and
result in compromise of the median nerve. C, In a different patient, transverse fat-suppressed T1-weighted
(TR/TE, 500/13) spin echo MR image obtained after intravenous gadolinium administration shows a
supracondylar process (arrow) under which lies the ulnar nerve (arrowhead).
Cubital Tunnel Syndrome. Ulnar nerve entrapment is seen Guyon’s Canal Syndrome (Ulnar Tunnel Syndrome). An
most frequently at the level of the cubital tunnel. In this entrapment neuropathy of the ulnar nerve may occur in
location, the ulnar nerve extends through a fibro-osseous the wrist where the nerve enters the palm through the
canal formed by the medial epicondyle and an aponeu- canal of Guyon (the ulnar tunnel) (Fig. 65–17). The walls
rotic band bridging the dual origin of the flexor carpi of the canal consist of the pisiform bone medially and the
ulnaris muscle. Cubital tunnel syndrome is probably the hook of the hamate laterally. The floor of the canal is
second most common compressive neuropathy of the composed of the flexor retinaculum and the origin of the
upper extremity (after carpal tunnel syndrome). Typical hypothenar muscles, and the roof is composed of the
causes of compression of the ulnar nerve in the cubital volar carpal ligament, palmaris brevis muscle, and fibers
tunnel are trauma, progressive cubitus valgus deformity, from the palmar fascia. The contents of the canal are the
and various masses (Fig. 65–16). Clinical findings are ulnar nerve, ulnar artery, and fatty tissue. The effects of ulnar
sensory deficits and weakness of the flexor carpi ulnaris nerve compression in the ulnar tunnel are dependent on
muscle, flexor digitorum profundus muscle of the fourth the specific anatomic site affected: with proximal involve-
and fifth fingers, and intrinsic hand muscles. ment, motor and sensory disturbances are evident; with
Chapter 65 Neuromuscular Disorders 1047
Radial Nerve
more distal involvement, either sensory or motor nerve
involvement predominates. The most frequent causes of Entrapment of the radial nerve may occur in the axilla,
ulnar nerve entrapment in Guyon’s canal are ganglia upper part of the arm, elbow, proximal portion of the
(Fig. 65–18) and accidental, occupational, or recreational forearm, and wrist.
(bicycling) trauma.
TABLE 65–3
Causes of Carpal Tunnel Syndrome
A B
Figure 65–15. Entrapment of the median nerve: carpal tunnel syndrome. Transverse T1-weighted
(TR/TE, 517/17) (A) and fat suppressed fast T2-weighted (TR/TE, 3610/77) (B) spin echo MR images at
the level of the hook of the hamate show flattening of the median nerve (arrow), mild retinacular bowing,
and increased signal from the median nerve in (B).
Saturday Night Palsy. Radial nerve dysfunction may of the supinator muscle. Causes of this compression neu-
develop because of compression of the radial nerve in the ropathy include dislocation of the elbow, fracture, rheuma-
axilla or spiral groove of the humerus in persons who toid arthritis, soft tissue tumor, ganglia, and traumatic or
sleep in a position that exerts pressure on the proximal developmental fibrous bands.
medial aspect of the arm. This type of sleep palsy is
particularly common in alcoholics and drug abusers and Radial Tunnel Syndrome. At the elbow, radial nerve com-
in patients using crutches. pression occurs in the radial tunnel, the most common
type of compression of the radial nerve. The motor
Posterior Interosseous Nerve Syndrome. Compression of weakness associated with this entrapment or compressive
the posterior interosseous nerve, a purely motor branch neuropathy is mild. Causes leading to this syndrome are
of the radial nerve, occurs just distal to the elbow. Com- similar to those producing the posterior interosseous
pression of the nerve occurs beneath the proximal edge nerve syndrome (some consider both syndromes to be
Chapter 65 Neuromuscular Disorders 1049
Suprascapular Nerve
edema or atrophy, and, in some cases, a rotator cuff tear
Entrapment of the suprascapular nerve occurs most are evident (Fig. 65–20).
commonly as it passes through the suprascapular notch
(Fig. 65–19). This nerve, which contains motor and
sensory fibers, arises from the upper trunk of the brachial Axillary Nerve
plexus, runs deep to the trapezius and omohyoid muscles,
and enters the supraspinous fossa through the suprascap- Although rare, a quadrilateral space syndrome is produced
ular notch below the superior transverse scapular by compression of the posterior circumflex humeral
ligament. After the suprascapular nerve passes through artery and the axillary nerve. The syndrome predominates
the notch, it supplies the supraspinatus and infraspinatus in young men and women, usually in the third or fourth
muscles. The most frequent site of nerve encroachment decade of life, and is more common in the dominant upper
is at the point where the suprascapular nerve traverses extremity. The quadrilateral space syndrome may also
the suprascapular notch adjacent to the suprascapular develop in athletes, especially baseball pitchers. Symptoms
ligament. As the nerve continues over the lateral part of include paresthesias in the upper extremity and anterior
the scapular spine and spinoglenoid notch and under the shoulder pain that may interfere with daily physical activity.
inferior transverse ligament and the spinoglenoid ligament MR imaging allows an accurate diagnosis, showing changes
to the infraspinatus fossa, other sites of entrapment are in signal intensity (representing regions of edema or fat)
possible, particularly in the spinoglenoid notch. Potential within the deltoid and teres minor muscles (Fig. 65–21).
causes of suprascapular nerve entrapment include a scapular
fracture or glenohumeral joint dislocation, occupational Digital Branch of the Medial or Lateral Plantar
or recreational injury, ganglia, and tumors. The major Nerves
clinical findings, which may be unilateral or bilateral,
are shoulder pain and weakness and atrophy of the Entrapment of a digital branch of the medial or lateral
supraspinatus or infraspinatus muscle, or both. plantar nerves of the foot as a result of injury to these
Entrapment neuropathy of the suprascapular nerve by nerves near the transverse intertarsal ligament leads to
a ganglion deserves special emphasis. Such ganglia arising the development of Morton’s neuroma. Ultrasonography
at the scapular notch compress both motor branches of and MR imaging can be used to make the diagnosis. The
the suprascapular nerve, with subsequent weakness or typical sonographic appearance is that of an ovoid,
atrophy of both the supraspinatus and the infraspinatus hypoechoic mass oriented parallel to the long axis of the
muscles; ganglia arising near the spinoglenoid notch affect metatarsal bones. MR imaging characteristics include a
a more distal segment of the nerve and result in selective mass with decreased signal intensity that is well demar-
involvement of the infraspinatus muscle. On MR images, cated from adjacent fat tissue on T1-weighted spin echo
a fluid-filled mass communicating with the joint, muscle MR images and is isointense or slightly hypointense to
1050 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
A B
Figure 65–20. Entrapment of the suprascapular nerve: ganglia. A and B, Two oblique coronal fat-
suppressed fast spin echo (TR/TE, 2650/48) MR images reveal a ganglion cyst (arrow) in the spinoglenoid
notch, with intense signal in the infraspinatus muscle related to denervation. Also note an undersurface tear
of the infraspinatus tendon and a humeral cyst.
are found within the proximal tarsal tunnel, and the bifur-
cation of the tibial nerve into medial and lateral plantar
nerves generally occurs within the tarsal tunnel.
The distal tarsal tunnel contains the medial and lateral
plantar nerves, which pass through fibrous openings in
the origin of the abductor hallucis muscle. The classic
tarsal tunnel syndrome refers to compression of nerves
within the proximal tarsal tunnel, behind the medial
malleolus. A specific cause of this classic syndrome can be
identified in about 60% to 80% of cases. Trauma is the
leading cause, including such specific injuries as fracture
of the distal portion of the tibia. Tumors and tumor-like
lesions may also cause the syndrome. An insidious onset
of pain and burning sensation aggravated by activity in
the plantar aspect of the foot is typical.
Distal tarsal tunnel syndromes relate to entrapment
Figure 65–21. Quadrilateral space syndrome: neurologic or compression of one or more branches of the tibial
abnormalities. Oblique sagittal fast spin echo (TR/TE, nerve. MR imaging is an effective technique for assessing
3000/16) MR image shows selective atrophy of the teres minor the classic tarsal tunnel syndrome or its variations
muscle (arrow). (Fig. 65–23).
A B
Figure 65–22. Morton’s neuroma. A, Coronal T1-weighted (TR/TE, 600/20) spin echo MR image shows
a mass (arrow) of low signal intensity between the third and fourth metatarsal heads. B, This mass (arrow)
has high signal intensity on a coronal fat-suppressed fast spin echo (TR/TE, 3500/50) MR image. A small
amount of fluid may be present in the intermetatarsal bursa (arrowhead).
Figure 65–23. Entrapment of the tibial nerve: tarsal tunnel syndrome. A, Normal anatomy. The tibial
nerve (T) passes beneath the flexor retinaculum (FR) and then divides into various nerves, including the
medial and lateral plantar nerves (arrows). B, Ganglion. Sagittal gradient recalled acquisition in the steady
state (GRASS) image obtained with volumetric acquisition (TR/TE, 30/12; flip angle, 40 degrees) show a
ganglion (arrows) on the medial and posterior portions of the ankle in this 48-year-old man. (A, From Kopell
HP, Thompson WAL: Peripheral entrapment neuropathies of the lower extremity. N Engl J Med 262:56,
1960. B, Courtesy of G. Bock, MD, Winnipeg, Manitoba, Canada.)
Sciatic Nerve
through the suprapiriformis region. The sciatic nerve
Most of the important structures connecting the gluteal leaves the pelvis through the infrapiriformis region.
region with the pelvis pass through the greater sciatic Although an extruded intervertebral disc is the most
foramen. The piriformis muscle splits the greater sciatic common cause of sciatica, entrapment neuropathies and
foramen into the suprapiriformis and infrapiriformis regions. other processes of the sciatic nerve are also encountered.
The superior gluteal nerve and vessels exit the pelvis The proximal portion of the sciatic nerve is infrequently
1052 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
Sciatic nerve
A 84.2%
TABLE 65–4
Classification of Human Muscular Dystrophies
From Rowland LP: Diseases of muscle and neuromuscular junction. In Beeson PB, McDermott W, Wyngaarden JB (eds): Cecil Textbook of Medicine, 15th ed.
Philadelphia, WB Saunders, 1979, p 916.
Chapter 65 Neuromuscular Disorders 1055
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paraplegia: A possible disturbance of the paravertebral 137:439, 1980.
venous plexus. Radiology 136:797, 1980. Thomas PK: Inherited neuropathies. Mayo Clin Proc 58:476,
Martinoli C, Bianchi S, Gandolfo N, et al: US of nerve entrap- 1983.
ments in osteofibrous tunnels of the upper and lower limbs. Woodlief RM: Superior marginal rib defects in traumatic
Radiographics 20:199, 2000. quadriplegia. Radiology 126:673, 1978.
66
CHAPTER 66
Neuropathic Osteoarthropathy No agreement exists on the pathogenesis of the articular
changes. Two fundamental theories of pathogenesis are
(1) the “French theory,” holding that joint changes are
SUMMARY OF KEY FEATURES the result of damage to the central nervous system trophic
centers, which control nutrition of the bones and joints,
The effect of the deprivation of sensory feedback on the and (2) the “German theory,” which maintains that unusual
musculoskeletal system can be profound. An anesthetized mechanical stresses about a weight-bearing joint in an
joint or bone that is subject to continuing stress ataxic person lead to recurrent subclinical trauma. Although
deteriorates progressively, with the appearance of other theories exist, there is considerable evidence that
specific radiographic abnormalities. These changes, both mechanical and vascular factors contribute to subse-
which include articular space narrowing, bony eburnation, quent disintegration of the joint (Fig. 66–1). What is
osteophytosis, fragmentation, fracture, and subluxation, clear is that loss of normal neurologic function renders a
can accompany a variety of disorders but are most joint (and adjacent bone) susceptible to a sequence of
common in diabetes mellitus, tabes dorsalis, and pathologic and radiographic alterations that may ulti-
syringomyelia. When severe, the resulting radiographic mately produce its disintegration. Joint instability that
picture is diagnostic; in earlier stages, however, the precedes neurologic dysfunction may make this sequence
findings may resemble those of osteoarthritis, calcium more likely.
pyrophosphate dihydrate crystal deposition disease,
calcium hydroxyapatite crystal deposition disease,
osteonecrosis, or, in the spine, intervertebral GENERAL RADIOGRAPHIC AND
(osteo)chondrosis. PATHOLOGIC ABNORMALITIES
Although the pathologic and radiographic features of
advanced neuropathic osteoarthropathy are characteristic,
early features may simulate those of osteoarthritis
INTRODUCTION (Fig. 66–2). The presence of an enlarging and persistent
In 1868, Charcot described an apparent cause-and-effect effusion, minimal subluxation, fracture, and fragmentation
relationship between primary lesions of the central nervous should alert a radiologist to the possibility of neuropathic
system and certain arthropathies. Charcot continued to osteoarthropathy; similarly, the finding of considerable
study and characterize the disorder for the next 15 years, amounts of cartilaginous and osseous debris attached to
and although his description was virtually confined to and incorporated into the synovial membrane (detritic
patients with tabes dorsalis, the name “Charcot joint” has synovitis) in a patient who is presumed to have osteoarthritis
become synonymous with all articular abnormalities should suggest to a pathologist that the changes may
related to neurologic deficits, regardless of the nature of represent joint manifestations of neuropathic disease
the primary disease. Other terms applied to this disorder (Table 66–1). These early changes may show rapid pro-
are neuroarthropathy and neurotrophic or neuropathic gression, and the joint may appear to fall apart in a matter
joint disease. None of these terms is ideal, however, of days or weeks (Fig. 66–3).
because the initial event in some cases occurs not in the More advanced radiographic abnormalities consist of
joint itself but in the adjacent bone or even some distance depression, absorption, and shattering of subchondral bone;
from the articulation. Therefore, the designations neuro- osseous proliferation in the form of significant sclerosis
pathic osteoarthropathy and neuropathic bone and joint and osteophytosis; intra-articular osseous fragments; sub-
disease are more suitable. luxation; massive soft tissue enlargement and effusion;
and fracture of neighboring bones (Fig. 66–4). On patho-
logic examination, the capsule is found to be irregularly
CAUSE AND PATHOGENESIS thickened by fibrous tissue and ossified; the synovial
Central (upper motor neuron) and peripheral (lower motor membrane is indurated, with villous transformation; and
neuron) lesions can lead to neuropathic osteoarthropathy. considerable fluid and connective tissue adhesions are
Among the central lesions that may produce neuropathic evident within the joints. In the synovial membrane,
osteoarthropathy are syphilis, syringomyelia, meningomye- diffuse metaplasia of the synovium, with the formation of
locele, trauma, multiple sclerosis, Charcot-Marie-Tooth cartilage and calcification of cartilage within the deeper
disease, congenital vascular anomalies, and other causes layers of the membrane, may be noted; this can produce
of cord compression, injury, or degeneration; peripheral radiographically detectable calcific and ossific dense
causes include diabetes mellitus, alcoholism, amyloidosis, lesions that may eventually become far removed from the
infection (tuberculosis, yaws, leprosy), pernicious anemia, joint itself (Fig. 66–5).
trauma, and intra-articular or systemic administration of In long-standing neuropathic osteoarthropathy, the
steroids. Additionally, specific syndromes leading to con- radiographic picture is that of a disorganized joint charac-
genital insensitivity to pain and dysautonomia produce terized by simultaneous bone resorption and formation.
similar alterations. The degree of sclerosis, osteophytosis, and fragmentation
1056
Chapter 66 Neuropathic Osteoarthropathy 1057
in this disorder is greater than that in any other process.
Yet the bone shards and irregular articular surfaces pro-
duced by the considerable osseous fragmentation and
collapse accompanying this disease are generally well defined
and sharp. Poorly marginated, “fuzzy” bone contours, as
occur in osteomyelitis and septic arthritis, are not usually
evident unless infection has become superimposed on the
neuropathic process.
SPECIFIC DISORDERS
Neuropathic osteoarthropathy can accompany many
disorders that lead to sensory disturbances. Although
some motor function is fundamental in the pathogenesis
of the lesions, neuropathic osteoarthropathy can develop
in patients with both sensory and motor loss, presumably
related to vigorous physical therapy. The radiographic and
Figure 66–1. Pathogenesis of neuropathic osteoarthropathy. pathologic features of this condition are generally similar
Probable sequential steps in the development of the disease are in these various disorders, but certain subtle differences
indicated.
C D
1058 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
TABLE 66–1 patients, the affected joints may be painful. Axial neuro-
pathic osteoarthropathy is not uncommon in tabes and
Causes of Detritic Synovitis may represent 20% of all cases of neuropathic disease
encountered in tabetic patients. It is most frequent in the
Neuropathic osteoarthropathy lumbar spine, where one or more vertebrae may be
Osteonecrosis affected. As opposed to the situation in peripheral neuro-
Calcium pyrophoshate dihydrate crystal deposition disease pathic osteoarthropathy, axial involvement is often symp-
Psoriatic arthritis tomatic and results in significant pain.
Osteoarthritis Radiographic features of tabetic axial neuropathic disease
Osteolysis with detritic synovitis may be productive or destructive. In the former group,
changes such as intervertebral disc space and apophyseal
joint narrowing, sclerosis, and osteophytosis are generally
more exaggerated than those of degenerative joint disease
and represent “osteoarthritis with a vengeance” (Fig. 66–7).
may be evident, and the distribution of abnormalities Fracture with osseous fragmentation in spinal and
varies among the disorders (Table 66–2). paraspinal locations, as well as malalignment, may also be
evident. The resulting radiographic findings, when severe,
are unique to this disorder and differ from the spinal
Tabes Dorsalis manifestations of degenerative joint disease, infection,
It is estimated that 5% to 10% of patients with tabes skeletal metastasis, and Paget’s disease (Table 66–3). Less
dorsalis have neuropathic osteoarthropathy. The joints of commonly, osteolytic or destructive changes predominate
the lower extremity are affected in 60% to 75% of cases. in axial neuropathic osteoarthropathy. These alterations
The knee, hip, ankle, shoulder, and elbow are altered, in can appear acutely, progress rapidly, and lead to significant
descending order of frequency (see Figs. 66–3 to 66–6). bony dissolution in a period of weeks or months. The
Other involved sites include the joints of the forefoot, appearance of this lytic variety of neuropathic disease can
midfoot, vertebral column, and fingers and the temporo- resemble that of infection or skeletal metastasis.
mandibular and sternoclavicular joints. Polyarticular
alterations are not unusual.
Syringomyelia
Typically, painless swelling, deformity, weakness, and
instability represent the clinical manifestations of tabetic It has been estimated that neuropathic osteoarthropathy
neuropathic osteoarthropathy. In as many as one third of develops in 20% to 25% of patients with syringomyelia.
A B
Figure 66–3. General radiographic and pathologic abnormalities of neuropathic osteoarthropathy: rapid
disease progression. A and B, The appearance and progression of articular destruction can occur rapidly.
Initial foci of chondral and osseous destruction can lead to fragmentation and collapse in a matter of weeks.
This patient has tabes dorsalis.
Chapter 66 Neuropathic Osteoarthropathy 1059
A B
Neuropathic changes in syringomyelia are common in on neuropathic changes in diabetic patients, especially in
the joints of the upper extremity, especially the gleno- superficially located joints. The addition of suppuration
humeral articulation (Fig. 66–8). In the lower extremity, can accentuate and accelerate the joint destruction.
the knee, ankle, and hip are affected with approximately Although the exact frequency of neuropathic bone and
equal frequency; in both the upper and lower limbs, joint changes in diabetic persons is not clear, this disease
bilateral symmetrical changes are not as common as in has certainly overtaken both syphilis and syringomyelia
tabes. as the leading cause of neuropathic osteoarthropathy; it
Generally, neuropathic osteoarthropathy occurs during represents the underlying disorder in more than 98% of
the later phases of syringomyelia, but occasionally, joint cases. Typically, diabetic neuropathic osteoarthropathy
findings may be the initial or predominant manifestation appears in a man or woman with long-standing diabetes
of the disease. The clinical, radiographic, and pathologic mellitus, generally in the fifth to seventh decades of life.
alterations of neuropathic osteoarthropathy in syringomyelia The joints of the forefoot and midfoot are altered most
are similar to those in tabes. The degree of fragmentation commonly. Painless soft tissue swelling, skin ulceration,
and sclerosis of the humeral head can be striking, and and joint deformity are encountered. Monoarticular or
these changes may be associated with fractures of neigh- polyarticular involvement can occur.
boring bones, including the scapula, clavicle, and ribs. Destructive or resorptive bony abnormalities can pre-
Further, spontaneous dislocation of the glenohumeral dominate, depending on the location of the neuropathic
joint may be the initial manifestation of neuropathic changes. In the intertarsal or tarsometatarsal joints, osseous
osteoarthropathy in syringomyelia. fragmentation, sclerosis, and subluxation or dislocation
may be prominent, and complete disintegration of one or
more tarsal bones can occur rapidly. Calcaneal fragmen-
Diabetes Mellitus
tation is typical (Fig. 66–9). Talar disruption and dorso-
The complexity of diabetic neuropathic osteoarthropathy lateral displacement of the metatarsal bones in relation to
is well known. Loss of pain and loss of proprioceptive the cuneiform and cuboid bones are also characteristic,
sensation appear to be of major importance in diabetic and the resulting radiographic picture may resemble that
neuropathic osteoarthropathy and, in the presence of observed in an acute Lisfranc’s fracture-dislocation
repetitive microtrauma or macrotrauma, promote the (Fig. 66–10). At the metatarsophalangeal joints, osseous
changes. Infectious processes are commonly superimposed resorption is frequent and leads to partial or complete
1060 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
A B
Figure 66–7. Tabes dorsalis: neuropathic osteoarthropathy of the axial skeleton—spine. A, Localized
disease. Frontal and lateral radiographs of the lumbar spine reveal extensive disorganization involving two
vertebral bodies (arrows) and the intervening intervertebral disc. Note the loss of intervertebral disc space,
sclerosis, and osteophytosis. The resulting osseous contours are relatively well defined. B, Generalized disease.
Widespread abnormalities in the lumbar spine consist of loss of height of multiple intervertebral discs,
extreme sclerosis, osteophytes, subluxation, and vertebral angulation.
TABLE 66–3
Axial Neuropathic Osteoarthropathy and Its Differential Diagnosis
A B C
Figure 66–13. Diabetes mellitus: spine. Progressive deterioration of the lumbar spine is apparent during
a 3-year period of observation. Initially (A), the changes at the lower lumbar level resemble degenerative disc
disease. Subsequently (B and C), this level deteriorates slowly; one level above, however, rapid destruction
of the intervertebral disc and bone is evident. The vacuum phenomenon in the upper disc, as well as the
well-defined and sclerotic bone in (B), makes infection unlikely. In (C), the pattern of disc destruction is
identical to that of infection, although the latter was not apparent clinically. (Courtesy of U.S. Naval Hospital,
San Diego, Calif.)
1064 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
A B
Figure 66–14. Syndromes of congenital indifference to pain. A, In this 7-year-old boy, fragmentation of
the lateral femoral condyle (arrow) and patella is associated with a joint effusion and intra-articular osseous
bodies. The opposite side (not shown) was similarly affected. B, In a 17-year-old patient, osteolysis and
autoamputation of multiple phalanges are evident. The opposite hand was similarly involved. (Courtesy of
M. Mitchell, MD, Halifax, Nova Scotia, Canada.)
A B
Figure 66–16. Neuropathic osteoarthropathy: diabetes mellitus. As shown on these transverse MR images,
marrow edema may accompany acute neuropathic changes, which in this case include dislocation and
fragmentation at the tarsometatarsal joints. The edema is displayed on a T1-weighted (TR/TE, 600/14) spin
echo MR image (A) and a short tau inversion recovery (STIR; TR/TE, 4000/20; inversion time, 140 msec)
MR image (B).
are consistently observed in the feet of diabetic patients, tract, and cortical interruption. These are best identified on
and this may simulate findings of septic arthritis. T1-weighted and gadolinium-enhanced fat-suppressed
Differences in the MR imaging appearances of T1-weighted images. Identification of abscesses is also
infection and neuropathic osteoarthropathy may be seen. strong evidence that infection is present (Fig. 66–17).
In general, pedal osteomyelitis results almost exclusively
from contiguous infection and occurs most frequently
DIFFERENTIAL DIAGNOSIS
around the first and fifth metatarsophalangeal joints.
Although primary signs of osteomyelitis are not specific, When severe, neuropathic osteoarthropathy is associated
secondary MR imaging signs of osteomyelitis have a high with radiographic changes that are almost pathogno-
specificity and include adjacent cutaneous ulcer, sinus monic. Bony eburnation, fracture, subluxation, and joint
A B
1066 Section XVII Thermal, Iatrogenic, Nutritional, and Neurogenic Diseases
disorganization are more profound in this disorder than Campbell WL, Feldman F: Bone and soft tissue abnormalities
in any other condition. In the joints of the appendicular of the upper extremity in diabetes mellitus. AJR Am J
skeleton, joint space loss, sclerosis, and fragmentation in Roentgenol 124:7, 1975.
the early stages of neuropathic osteoarthropathy can Citron ND, Paterson FWN, Jackson AM: Neuropathic
osteonecrosis of the lateral femoral condyle in childhood: A
resemble the changes in osteoarthritis. With progressive
report on four cases. J Bone Joint Surg Br 68:96, 1986.
flattening and deformity of the articular surfaces, the pro- Clouse ME, Gramm HF, Legg M, et al: Diabetic osteoarthropa-
duction of numerous intra-articular osseous dense areas, thy: Clinical and roentgenographic observations in 90 cases.
and the appearance of increasing sclerosis and osteophy- AJR Am J Roentgenol 121:22, 1974.
tosis, the diagnosis of neuropathic osteoarthropathy Eichenholtz SN: Charcot Joints. Springfield, Ill, Charles C
becomes more obvious. Thomas, 1966.
In calcium pyrophosphate dihydrate crystal deposition El-Khoury GY, Kathol MH: Neuropathic fractures in patients
disease, a neuropathic-like arthropathy characterized by with diabetes mellitus. Radiology 134:313, 1980.
joint space narrowing, eburnation, and fragmentation can Feldman F, Johnson AM, Walter JF: Acute axial neuroarthropa-
appear, especially in the knee, wrist, and metacarpopha- thy. Radiology 111:1, 1974.
Forrester DM, Magre G: Migrating bone shards in dissecting
langeal joints. Identification of articular and periarticular
Charcot joints. AJR Am J Roentgenol 130:1133, 1978.
calcification, involvement of specific areas of the joint Giesecke SB, Dalinka MK, Kyle GC: Lisfranc’s fracture-dislo-
such as the radiocarpal compartment of the wrist and the cation: A manifestation of peripheral neuropathy. AJR Am J
patellofemoral compartment of the knee, and the vari- Roentgenol 131:139, 1978.
ability in osteophyte formation are helpful clues to the Goldman AB, Freiberger RH: Localized infectious and
accurate diagnosis of pyrophosphate arthropathy. Intra- neuropathic diseases. Semin Roentgenol 14:19, 1979.
articular deposition of calcium hydroxyapatite crystals Gondos B: The pointed tubular bone: Its significance and
may lead to progressive destruction of a joint, especially pathogenesis. Radiology 105:541, 1972.
in the shoulder, with fracture and dissolution of bone. A Hodgson J, Pugh D, Young H: Roentgenologic aspects of certain
similar appearance in the shoulder has been reported as a lesions of bone: Neurotropic or infectious? Radiology 50:65,
1948.
manifestation of idiopathic “rapid destructive arthropathy.”
Jones EA, Manaster BJ, May DA, et al: Neuropathic
Bony fragmentation and collapse are also manifes- osteoarthropathy: Diagnostic dilemmas and differential
tations of osteonecrosis, post-traumatic osteoarthritis, diagnosis. Radiographics 20:279, 2000.
intra-articular steroid arthropathy, neglected infection, Kathol MH, El-Khoury GY, Moore TE, et al: Calcaneal insuffi-
and alkaptonuria. In the joints of the axial skeleton, early ciency avulsion fractures in patients with diabetes mellitus.
findings of neuropathic osteoarthropathy, such as inter- Radiology 180:725, 1991.
vertebral disc space narrowing and vertebral sclerosis, Katz I, Rabinowitz JG, Dziadiw R: Early changes in Charcot’s
resemble those of intervertebral osteochondrosis, infection, joints. AJR Am J Roentgenol 86:965, 1961.
or alkaptonuria. Kirkpatrick RH, Riley CR: Roentgenographic findings in familial
Once the radiographic findings are interpreted as those dysautonomia. Radiology 68:654, 1957.
Ledermann HP, Morrison WB, Schweitzer ME: MR image analysis
of neuropathic osteoarthropathy, identification of the
of pedal osteomyelitis: Distribution, patterns of spread, and
underlying disorder usually depends on the location of frequency of associated ulceration and septic arthritis.
the changes. Tabes typically produces changes in the hip, Radiology 223:747, 2002.
knee, ankle, and spine; diabetes mellitus leads to alterations Meyer GA, Stein J, Poppel MH: Rapid osseous changes in
in the midfoot and forefoot; syringomyelia affects the syringomyelia. Radiology 69:415, 1957.
articulations of the upper extremity and cervical spine; Morrison WB, Schweitzer ME, Batte WG, et al: Osteomyelitis
and the syndromes of congenital indifference to pain and of the foot: Relative importance of primary and secondary
meningomyelocele commonly localize in the joints MR imaging signs. Radiology 207:625, 1998.
(including the physes) of the lower extremity. The presence Norman A, Robbins H, Milgram JE: The acute neuropathic
of metaphyseal and growth plate destruction in an imma- arthropathy—a rapid severely disorganizing form of
arthritis. Radiology 90:1159, 1968.
ture skeleton is especially characteristic of congenital
Pogonowska MJ, Collins LC, Dobson HL: Diabetic osteopathy.
indifference to pain and meningomyelocele. Radiology 89:265, 1971.
Schneider R, Goldman AB, Bohne WH: Neuropathic injuries
to the lower extremities in children. Radiology 128:713,
FURTHER READING 1978.
Siegelman S, Heimann WG, Manin MC: Congenital indifference
Bjorkengren AG, Weisman M, Pathria MN, et al: Neu- to pain. AJR Am J Roentgenol 97:242, 1966.
roarthropathy associated with chronic alcoholism. AJR Am J Silverman FN, Gilden JJ: Congenital insensitivity to pain: A
Roentgenol 151:743, 1988. neurologic syndrome with bizarre skeletal lesions. Radiology
Blanford AT, Keane SP, McCarty DJ, et al: Idiopathic Charcot 72:176, 1959.
joint of the elbow. Arthritis Rheum 21:723, 1978. Westcott MA, Dynes MC, Remer EM, et al: Congenital and
Brower AC, Allman RM: Pathogenesis of the neurotrophic acquired orthopedic abnormalities in patients with
joint: Neurotraumatic versus neurovascular. Radiology myelomeningocele. Radiographics 12:1155, 1992.
139:349, 1981.
SECTION
XVIII
Osteonecrosis and Osteochondrosis
CHAPTER 67
Osteonecrosis: Pathogenesis, TABLE 67–1
1067
1068 Section XVIII Osteonecrosis and Osteochondrosis
and the marrow cavity makes it possible for temporary injured marrow and bone, active hyperemia, and viable
anoxia to result in the death of hematopoietic elements marrow and bone. Infarcts occurring within an epiphysis
without necessarily being sufficient to cause osteocytic or or a small, round bone demonstrate a similar three-
marrow fat cell death. Once ischemic marrow fat cell dimensional pattern, except that one surface is almost
death occurs, the involved segment of bone and marrow always covered by compact subchondral bone and articu-
can clearly be labeled infarcted. lar cartilage. Because articular cartilage receives the bulk
Infarcts, including those in bone, are three dimen- of its nourishment from synovial fluid, its viability is
sional and can be subdivided into four zones: a central usually not significantly affected at first by the underlying
zone of cell death surrounded by successive zones of osteonecrosis, except for the cartilage cells below the
ischemic injury, active hyperemia, and, finally, normal tidemark, which may die. Because the osteonecrotic
tissue. The ischemic zone reflects a gradation of hypoxic segment is by definition avascular, repair begins along its
injury ranging from severe cell damage immediately adja- outer perimeter at the junction between the ischemic
cent to the central zone of cell death to marginal cellular zone surrounding the dead area and the viable area with
alterations adjacent to the hyperemic zone. Once ischemic an intact circulation (hyperemic zone). This reparative
necrosis is established, the breakdown products of dying response results in the progressive development of a
and severely damaged cells provoke the initial inflam- reactive margin (interface) between the dead zone and
matory response consisting of vasodilatation, transuda- adjacent viable tissue.
tion of fluid, fibrin precipitation, and local infiltration of Because tissue death is essentially a cellular phenomenon,
inflammatory cells. This response forms the basis for the mineralized bone matrix does not appear to be materially
development of the hyperemic zone and also represents altered by ischemic necrosis directly; hence, bone struc-
the initial step of repair, removal, and reconstruction of ture is initially unaltered as a direct result of osteocyte
the infarcted area. death or osteonecrosis. Therefore, any alteration in bone
Bone infarcts occurring in the metadiaphyseal intra- density—either a real increase or a real decrease in radi-
medullary cavity (Fig. 67–1) have a central core of dead ographic density—is an indication of viability requiring
marrow and bone surrounded by zones of ischemically osteoblastic or osteoclastic cell activity, respectively, and
will initially be perceived in the viable bone and marrow
surrounding the osteonecrotic segment.
MARROW CAVITY
The medullary cavity is an admixture of cancellous bone,
hematopoietic marrow, fatty marrow, and a sinusoidal
vascular bed confined by a nonexpandable shell of cortical
bone. Radiographically, ischemic necrosis of bone is
encountered most commonly within the epiphyseal (espe-
cially the femoral and humeral heads) and metadiaphyseal
marrow cavities of adult long tubular bones. Occasional
involvement occurs in the distal femoral condyles, small
bones of the wrist and ankle, and other sites.
The extensive arterial supply to adult long tubular
bones—including the nutrient artery, with its ascending
and descending diaphyseal branches, and the penetrating
metaphyseal and epiphyseal (retinacular) arteries, as well
as the superficial periosteal vessels and the diffuse intra-
medullary sinusoidal vascular bed—provides excellent
collateral circulation. Venous outflow is also extensive,
as evidenced by numerous perforating channels exiting
from the cortex.
Despite the extensive circulation available to the meta-
diaphyseal cortex and intramedullary cavity, the epiphy-
seal ends of long bones have limited arterial access and
venous outflow because much of their surface is covered
by articular cartilage. Arterial access to the epiphysis is
further compromised in a growing skeleton because the
epiphysis is separated from the metaphysis by the growth
plate. Although occasional small arterioles penetrate the
Figure 67–1. Intramedullary bone infarct. Specimen radi- physeal growth plate (physis), little or no significant
ograph of a coronally sectioned distal end of the femur reveals
unaltered density within the central necrotic zone (N), which is collateral circulation exists between a developing epiphy-
separated from the adjacent viable bone and marrow (V) by an sis and its adjacent metaphysis. In the absence of collateral
irregular linear margin of increased density. Note the localized circulation, the likelihood of a single or dominant artery
area of increased density that appears to be within the inferior supplying an entire epiphysis or a significant segment of
portion of the infarct. an epiphysis is increased. The small carpal and tarsal
Chapter 67 Osteonecrosis: Pathogenesis, Diagnostic Techniques, Specific Situations, and Complications 1069
bones are also covered to a large extent by articular carti- the viable and osteonecrotic zones, consisting of ischemic
lage. In many respects, their ossification centers are analo- fiber bone formation and dystrophic mineralization of
gous to the developing epiphyses of a long bone with dead marrow fat within the necrotic zone adjacent to the
regard to blood supply. fibrous interface. As a result of the continuing hyperemia,
Development of a sinusoidal vascular bed appears to the supporting bony architecture may become sufficiently
be an important prerequisite for the emergence of normal weakened by continued resorption of trabecular bone
hematopoietic activity. This demand for hematopoiesis is and subchondral bone plate along the reactive interface
largely a function of age. The marrow cavities of all bones that the stress of weight bearing results in subchondral
in newborns and young children are actively engaged in bone plate fracture, with focal articular cartilage buckling
hematopoiesis. As the total marrow volume increases and eventual collapse. Fragmentation and compaction of
with skeletal growth, normal hematopoietic demand can subchondral bony fracture debris lead to the develop-
eventually be satisfied by the marrow capacity of the axial ment of a subchondral lucent area along the fracture line,
skeleton. Therefore, the marrow cavities of the adoles- designated the crescent sign (Fig. 67–3). This sign is often
cent and adult appendicular skeleton normally contain best seen radiographically on a frog-leg view. In time,
predominantly fatty marrow, unless some special circum- flattening of the articular surface becomes apparent.
stance requires increased hematopoiesis.
Thus, both radiographic and anatomic observations
indicate that ischemic necrosis of bone, either avascular DIAGNOSTIC TECHNIQUES
necrosis or bone infarction, occurs almost invariably
Radiography
within areas of predominantly fatty marrow. The con-
verse is equally true; ischemic necrosis in areas of normal The radiographic findings of osteonecrosis of an epiphy-
active hematopoiesis is distinctly unusual except in sickle sis, metaphysis, or diaphysis in a tubular bone or a flat or
cell disease and related hemoglobinopathies or after com- irregular bone are characteristic. Arclike subchondral
plete traumatic disruption of the arterial blood supply. radiolucent lesions, patchy lucent areas and sclerosis,
Ischemic necrosis of cortical bone is relatively rare and osseous collapse, and preservation of the joint space in an
occurs only when the arterial blood supply has been epiphyseal region (Fig. 67–4); lucent shadows with a
extensively interrupted, as in osteomyelitis. peripheral rim of sclerosis and periostitis in a diameta-
physeal region (Fig. 67–5); and patchy lucent areas and
sclerosis with bony collapse in a flat or irregular bone
HISTOLOGIC-RADIOGRAPHIC CORRELATION (Fig. 67–6) are typical radiographic signs of osteonecro-
Once osteonecrosis or ischemic injury is an established sis. Unfortunately, these abnormalities do not appear for
anatomic fact, the body sets into motion the initial inflam- several months after the onset of clinical manifestations
matory host response as the first step toward revival, in many persons and are therefore not a sensitive indi-
rehabilitation, or removal and reconstruction (repair). cation of early disease (Fig. 67–7). Further, the initial
In the early stages of osteonecrosis, no gross architec- radiographic features of osteonecrosis, especially in the
tural or radiographic alteration is observed. Following diametaphyseal portions of tubular bones, may lack speci-
this initial phase, there is cell modulation in the zone of ficity. Subtle, mottled, poorly defined radiolucent lesions
ischemic injury and associated hyperemia. The general- may simulate the aggressive pattern of bone destruction
ized active hyperemia in response to the injured and that accompanies a variety of malignant tumors, as well as
dying cells results in osteoporosis (mediated through osteomyelitis.
osteoclastic activity) of the adjacent viable bone, the first
recognizable radiographic alteration in osteonecrosis; the
Computed Tomography
dead area remains unchanged. As the marrow cells in
the ischemic zone undergo morphologic changes, the The use of computed tomography (CT) to evaluate
reactive interface begins to emerge. ischemic necrosis of bone (especially in the femoral head)
The reactive interface demonstrates an increase in has two goals: to allow early diagnosis of the condition,
vascularity associated with infiltration of inflammatory and to identify the presence and extent of bone collapse.
cells along the outer viable margin and further modula- Although it appears that CT can delineate subtle alter-
tion of ischemic marrow fat toward fibroblastic cells, ations in bone when routine radiographs are normal, it is
which may elaborate an atypical-appearing ischemic fiber also apparent that scintigraphy and magnetic resonance
bone. This ischemic bone may also appear as an appliqué (MR) imaging are far more sensitive in this respect
on the surface of preexisting dead bone trabeculae within (see later discussion). CT may also be a useful adjunct in
the ischemic zone. While these changes evolve in the patients unable to undergo MR imaging.
reactive interface, sustained active hyperemia causes pro- In patients with more advanced changes of osteonecrosis
gressive osteoporosis in the remaining viable bone. of the femoral head, transaxial CT scans reveal centrally
Emergence of the reactive interface within the previously or peripherally located circular areas of decreased attenu-
ischemic zone (with focal cancellous bone resorption, ation. At this stage, reformation of the CT data in a
minimal trabecular bone reinforcement, and deposition of sagittal or coronal plane is more useful because it delin-
ischemic fiber bone within the reactive fibrosis) accounts eates areas of subchondral fracture and subtle buckling or
for the earliest alterations in radiographic density between collapse of the articular surface (Fig. 67–8). The latter
the infarcted and viable zones (Fig. 67–2). With continued finding has therapeutic significance because the presence
repair and remodeling, a reactive interface forms between of even minimal bone collapse indicates a more advanced
1070 Section XVIII Osteonecrosis and Osteochondrosis
A B
Figure 67–3. Ischemic necrosis of the femoral head (nontraumatic). A, Radiograph demonstrates flat-
tening of the weight-bearing surface of the femoral head, with a subtle crescent sign (arrow) indicating
collapse of the articular surface. A serpentine rind of sclerosis surrounding the osteonecrotic region of the
femoral head is not well defined. B, Specimen radiograph from a different patient shows the crescent sign
and fracture to better advantage. The osteonecrotic region of the femoral head has a surrounding rind of
sclerosis (reactive interface).
Chapter 67 Osteonecrosis: Pathogenesis, Diagnostic Techniques, Specific Situations, and Complications 1071
Scintigraphy
Radionuclide examination is one of the most attractive
techniques for the early diagnosis of osteonecrosis. Single
photon emission computed tomography (SPECT) may
further improve radionuclide sensitivity for this diag-
nosis. Establishing a correct diagnosis at an early stage is
important, because the success of some of the proposed
orthopedic procedures requires their application before
TABLE 67–2
Criteria for Staging Osteonecrosis
Stage Characteristics
0 Normal or nondiagnostic radiograph, bone scan,
and MR examination
I* Normal radiograph; abnormal bone scan or MR
examination, or both
II* Abnormal radiograph showing cystic and sclerotic
changes in the femoral head
III* Subchondral collapse producing a crescent sign
IV* Flattening of the femoral head
V* Joint space narrowing with or without acetabular
involvement
Figure 67–5. Osteonecrosis: diametaphysis (bone infarct).
Radiograph of the lower half of the left femur of a 69-year-old VI Advanced degenerative changes
man with intermittent claudication of 4 years’ duration demon-
strates a bone infarction. Observe the typical shell-like calci- *The extent or grade of involvement should also be indicated as A, mild;
fication of the lesion. (From Jaffe HL: Ischemic necrosis of B, moderate; or C, severe.
bone. Med Radiogr Photogr 45:57, 1969.) Modified from Steinberg ME, et al: J Bone Joint Surg Br 77:34, 1995.
1072 Section XVIII Osteonecrosis and Osteochondrosis
A B
Figure 67–7. Osteonecrosis: inadequacy of radiographic examination. This 35-year-old man had hip pain
at the time of his initial radiographic examination that progressed over the next 6 to 9 months. A, The initial
radiograph was interpreted as normal. Neither a bone scan nor MR imaging was performed. B, Seven
months later, significant collapse of the superolateral aspect of the femoral head is identified (arrows). The
joint is not narrowed.
A C
B D
Figure 67–8. Osteonecrosis: CT. A and B, Transaxial CT scan (A) and a coronal reconstruction (B)
illustrates a curvilinear zone of sclerosis, diagnostic of ischemic necrosis of the femoral head. No collapse of
the articular surface is evident. C and D, In similar images from a different patient, observe the degenerative
subchondral cysts and articular collapse (arrows) of the left femoral head. The joint space is preserved.
Chapter 67 Osteonecrosis: Pathogenesis, Diagnostic Techniques, Specific Situations, and Complications 1073
TABLE 67–3
Surgical Methods of Treating Ischemic Necrosis of the
Femoral Head
Method Rationale
Drilling or forage Multiple drill holes in the femoral
head and neck to establish
channels for revascularization
Core decompression Coring device to create large and
small channels from the
trochanteric region to the femoral
head to allow decompression of
elevated intraosseous pressure
Free bone grafts with Placement of cortical bone (for
cancellous or cortical mechanical support) or cancellous
bone bone (for rapid incorporation)
into channels in the femoral head
and neck
Vascularized bone grafts Same as for free bone grafts, except
with attached muscle that living rather than dead bone
pedicle is used
Osteochondral allograft Replacement of the collapsed
portion of the articular surface
with an allograft
Osteotomy
Varus or valgus Shift of the femoral head in the
acetabulum to provide a new
weight-bearing area
Rotational Rotation of the femoral head in Figure 67–9. Osteonecrosis: radionuclide bone scan. In this
the acetabulum to provide a new 75-year-old woman with widespread infarction, a bone scan
weight-bearing area shows absence of uptake of the radionuclide throughout much
Electrical stimulation Use of electrical current to induce of the length of the left femur, consistent with the clinical diag-
bone formation nosis of acute bone infarction. Osteoarthritis in the right hip is
associated with increased accumulation of the radiopharma-
Arthroplasty Replacement of the abnormal ceutical agent. (Courtesy of K. Nguyen, MD, Wichita, Kans.)
femoral head (and acetabulum)
with a prosthesis
A B
C D
A B
healing process, or lack of participation of the bone in the into the epiphysis can lead to significant disruption of
hyperemia and osteoporosis of neighboring viable bone. blood supply and subsequent osteonecrosis. The vessels
within the ligamentum teres vary in diameter, and their
role in the supply of blood to the femoral head is debated.
Femoral Head Fractures of the femoral neck in adults (and in children)
Osteonecrosis of the femoral head is a well-recognized can be complicated by osteonecrosis of the femoral head
complication of femoral neck fractures and hip disloca- (Fig. 67–18). This complication is more frequent in intra-
tions. The principal blood supply to the adult femoral capsular fractures (subcapital, transcervical) than in extra-
head is via the circumflex femoral branches of the pro- capsular fractures (intertrochanteric); in the latter situation,
funda femoris artery (Fig. 67–17). A second supply of the blood supply is not compromised by the fracture line.
blood is derived from vessels of the ligamentum teres, The use of MR imaging to detect acute ischemia of the
which enters the bone of the fovea capitis. It has been femoral head, and the use of contrast-enhanced MR
established that the superior (lateral) retinacular vessels imaging in cases of early ischemia, is valuable in patients
represent the most important source of blood to the with acute fractures of the femoral neck.
femoral head, and a fracture of the femoral neck that Other traumatic causes of osteonecrosis of the femoral
traverses the entry site of the superior retinacular vessels head include dislocation of the hip and a slipped capital
A B
Figure 67–13. Osteonecrosis: MR imaging. Coronal T1-weighted (TR/TE, 600/20) (A) and T2-weighted
(TR/TE, 2500/70) (B) spin echo MR images reveal an area of osteonecrosis in the left femoral head, where
a serpentine region of low signal intensity surrounds a central region whose signal characteristics are iden-
tical to those of fat.
Chapter 67 Osteonecrosis: Pathogenesis, Diagnostic Techniques, Specific Situations, and Complications 1077
A B
1078 Section XVIII Osteonecrosis and Osteochondrosis
Humeral Head
The blood supply of the head of the humerus is derived
from several major sources (Fig. 67–21). The vascular
supply pierces the bony cortex just distal to the anatomic
B
Figure 67–17. Femoral head: vascular supply in adults. A,
The major blood supply is derived from the profunda femoris
artery (1), from which arise the lateral (2) and medial (3)
circumflex arteries. (The medial and lateral circumflex arteries
may arise from the femoral artery rather than the profunda
femoris artery in some persons.) As these latter vessels pass
anterior and posterior to the femur to anastomose at the level
of the trochanters, they send off small branches beneath the
capsule of the hip joint. These branches, including the superior
retinacular (lateral epiphyseal) arteries (4) and the inferior
retinacular (inferior metaphyseal) arteries (5), raise the synovial
membrane into folds, or retinacula. A second supply of blood is
derived from the vessels of the ligamentum teres. Here, the
foveal (medial epiphyseal) arteries (6) can be noted. Additional
regional vessels are the inferior gluteal artery (7) and the obtu-
rator artery (8). B, Cross section of the proximal end of the
femur at the base of the neck better delineates the nature of the
blood supply. The greater trochanter (arrow) and iliopsoas muscle
(open arrow) are indicated. Note the profunda femoris artery (1)
and the lateral (2) and medial (3) circumflex arteries. From the
lateral circumflex arteries are derived the anterior ascending
cervical arteries (9). From the medial circumflex artery are derived
the medial (10), posterior (11), and lateral (12) ascending cervical
arteries, which, in combination with the anterior ascending
cervical arteries, form a subsynovial anastomotic ring on the
surface of the femoral neck at the margin of the articular Figure 67–19. Post-traumatic osteonecrosis: talus. In this
cartilage. The inset shows the plane of section (line a–a′). person who sustained a fracture of the body of the talus, as well
(A, After Graham J, Wood S: In Davidson JK [ed]: Aseptic as a fracture of the medial malleolus (which was transfixed with
Necrosis of Bone. New York, American Elsevier, 1976, p 101. a screw), note the increased radiodensity of the proximal half of
B, After Chung SM: J Bone Joint Surg Am 58:961, 1976.) the bone (arrowheads).
Chapter 67 Osteonecrosis: Pathogenesis, Diagnostic Techniques, Specific Situations, and Complications 1079
A B
Figure 67–22. Post-traumatic osteonecrosis: humeral head. A, Although a fracture of the anatomic neck
of the humerus has healed, observe the flattened and irregular humeral head, indicative of ischemic necrosis.
B, In a different patient with a comminuted fracture of the proximal end of the humerus, note a large focus
of osteonecrosis in the humeral head on an oblique coronal T1-weighted (TR/TE, 666/20) spin echo MR
image. (A, Courtesy of J. Schils, MD, Cleveland, Ohio.)
1080 Section XVIII Osteonecrosis and Osteochondrosis
varies with the nature and severity of the fracture or injury. The proximal pole of the capitate is the site of
dislocation; a frequency of 11% to 65% has been noted, ischemic necrosis. Osteonecrosis of this bone is a recog-
but 10% to 15% is the most common estimated frequency nized complication of a transscaphoid, transcapitate, per-
of this complication. The radiographic diagnosis depends ilunate fracture-dislocation (scaphocapitate syndrome).
on a relative increase in density of the devitalized portion
of the scaphoid in comparison to the osteoporotic viable
Vertebral Body
bone (Fig. 67–23), an appearance that is delayed for 4 to
8 weeks and may be associated with delayed union or Delayed post-traumatic collapse of vertebral bodies is
nonunion of the fracture, collapse of the necrotic segment, termed Kümmell’s disease. Current evidence suggests that
and, eventually, secondary degenerative joint disease of vertebral collapse occurs as a consequence of a vascular
the radiocarpal compartment of the wrist. MR imaging insult leading to secondary bone necrosis. The frequency
has also been used to diagnose osteonecrosis of the scaphoid of Kümmell’s disease is not known, but it is probably not
(Fig. 67–24). Regions of low signal intensity in the marrow uncommon. Affected patients are generally middle-aged
of the proximal pole of the scaphoid on T2-weighted or elderly men or women. The interval between the acute
spin echo MR images and, more importantly, the pres- traumatic episode and the vertebral collapse varies from
ence of such regions after the intravenous injection of days to years, and the lower thoracic and upper lumbar
a gadolinium-containing contrast agent are suggestive of vertebral bodies are principally involved (Fig. 67–25). A
osteonecrosis. gas collection within the collapsed vertebra may be seen
(vacuum vertebral body), which may extend into the adja-
cent psoas musculature.
Capitate
Ischemic necrosis of the capitate typically occurs after
accidental or occupational trauma or recreational stress, Other Sites
as well as spontaneously in the absence of any type of After significant injury or prolonged stress, avascular
necrosis can appear at other sites, especially when an
osseous fragment loses its soft tissue attachments. Areas
at risk include the lunate, other carpal bones, tarsal navic-
ular bone, mandibular condyle, patella, femoral condyles,
and even metatarsal and metacarpal heads.
DYSBARIC OSTEONECROSIS
General Features
Exposure to high-pressure environments occurs as a result
of scuba diving, underwater and space exploration, and
off-shore oil drilling. One late complication of such expo-
sure is osteonecrosis, which has been designated dysbaric
osteonecrosis, caisson disease, pressure-induced osteo-
arthropathy, and barotraumatic osteoarthropathy.
The term decompression sickness indicates the acute
consequences of the liberation of gas bubbles, principally
nitrogen, in the blood and tissues of a person who has
undergone decompression too rapidly after a period of
exposure to a hyperbaric environment. In a high-pressure
environment, a person’s blood and tissues are saturated
with atmospheric gases, and after a rapid return to normal
atmospheric pressure, the various gases come out of solu-
tion and produce supersaturation of tissues. Ventilation
may allow dispersion of the excess oxygen and carbon
dioxide, but the released nitrogen may produce bubbles
within the vascular tree that act as air emboli. Nitrogen
accumulation is greatest in tissues rich in fat, and the
fatty marrow does not escape this accumulation.
The association of decompression sickness and dysbaric
osteonecrosis is controversial, and in general, there appears
to be a lack of correlation between these two conditions.
Figure 67–23. Post-traumatic osteonecrosis: carpal scaphoid.
Radiograph obtained 8 weeks after traumatic scaphoid fracture Despite this dissociation of findings, however, it is gener-
shows increased radiodensity of the proximal half of the ally assumed that gas bubbles initiate the manifestations
scaphoid, accentuated by osteoporosis of the distal half of the of decompression sickness and that they are probably the
bone, indicative of the presence of osteonecrosis and reparative cause of osteonecrosis as well. The frequency of dysbaric
bone formation. osteonecrosis appears to be exaggerated in patients with
Chapter 67 Osteonecrosis: Pathogenesis, Diagnostic Techniques, Specific Situations, and Complications 1081
A C
Figure 67–24. Post-traumatic osteonecrosis: carpal scaphoid. A, Routine radiography reveals typical
features of osteonecrosis involving the proximal pole of the bone. No evidence of bone union is apparent at
the fracture site in the midportion of the scaphoid bone. B and C, Coronal intermediate-weighted (TR/TE,
2500/30) (B) and T2-weighted (TR/TE, 2500/80) (C) spin echo MR images document the abnormally low
signal intensity of the marrow in the proximal pole of the bone. In (C), observe fluid in the midcarpal joint,
with extension into the fracture gap (arrow).
A B C
Figure 67–25. Post-traumatic osteonecrosis: vertebral body (Kümmell’s disease). A to C, Delayed post-
traumatic collapse of the second lumbar vertebral body developed in this 71-year-old man. A, Radiograph
shows an intraosseous vacuum at this spinal level. B, In a sagittal T1-weighted (TR/TE, 300/13) spin echo
MR image, low signal intensity is seen in this vertebral body. Narrowing of the spinal canal is evident. C, In
a sagittal fat-suppressed T1-weighted (TR/TE, 450/11) spin echo MR image obtained after the intravenous
administration of a gadolinium compound, low signal intensity persists in the central portion of the vertebral
body (consistent with fluid or gas), with enhancement of other portions of the vertebral body. (Courtesy of
G. Greenway, MD, Dallas.)
1082 Section XVIII Osteonecrosis and Osteochondrosis
Radiographic Abnormalities
Radiographic abnormalities are typically divided into
juxta-articular lesions (occurring most frequently in the
head of the humerus and femur; Fig. 67–26) and diaphy-
seal and metaphyseal lesions (situated at a distance from
the joint; Fig. 67–27). Juxta-articular alterations consist
of radiodense areas between 3 and 20 mm in diameter
that are slightly less discrete than bone islands (enos- Figure 67–27. Dysbaric osteonecrosis: diametaphyseal lesions.
toses); spherical, segmental opaque areas that may even- In this 28-year-old male scuba diver, a patchy area of increased
tually produce a “snow-capped” configuration; radiolucent sclerosis is evident in the proximal femoral shaft (arrows). Note
subcortical bands (crescent sign), indicating a fracture of the increased density of the periphery of the lesion. Biopsy with
necrotic bone; and osseous collapse and fragmentation. histologic evaluation indicated osseous and marrow necrosis
compatible with an infarct.
Diaphyseal and metaphyseal abnormalities consist of
indistinct, poorly defined radiodense foci; irregular calci-
fied areas with a shell-like configuration; and, rarely,
radiolucent lesions, perhaps resulting from sites of necrosis.
Multiple or single, bilateral or unilateral alterations can
be seen. The distal femoral shaft, humeral head, femoral
head, and tibial shaft, in descending order of frequency,
are the most typical sites of involvement. An infarct that
is limited to the shaft of a tubular bone is not usually
associated with clinical complaints; when involvement of
an epiphysis leads to collapse of the articular surface, pain
and swelling may become apparent.
Differential Diagnosis
The radiographic findings associated with dysbaric osteo-
necrosis are generally indistinguishable from those asso-
ciated with osteonecrosis of other causes such as sickle
cell anemia, Gaucher’s disease, and steroid medication.
Small, dense foci in dysbaric osteonecrosis are virtually
identical to bone islands, and only foci that are larger and
more numerous may allow an accurate diagnosis of this
condition.
IATROGENIC OSTEONECROSIS
A number of iatrogenic factors can lead to single or
multiple sites of osteonecrosis. Certain medications (e.g.,
Figure 67–26. Dysbaric osteonecrosis: juxta-articular lesions. corticosteroids) and irradiation are well known in this
The sclerosis (“snow-capped” appearance) of the humeral head regard. One additional iatrogenic cause relates to the use
(arrowheads) is typical of dysbaric osteonecrosis but may be of lasers for orthopedic applications such as meniscec-
evident in other varieties of bone necrosis as well. tomy and discectomy.
Chapter 67 Osteonecrosis: Pathogenesis, Diagnostic Techniques, Specific Situations, and Complications 1083
IDIOPATHIC (PRIMARY OR SPONTANEOUS) bone (crescent sign). As the disease progresses, a focus of
OSTEONECROSIS increased radiodensity or a lucent zone with a peripheral
Osteonecrosis may appear in certain locations in the rim of increased radiodensity is observed.
absence of any recognizable underlying disorder or event. A milder form of osteonecrosis of the femoral head,
Characteristically, the femoral head or femoral condyles typically found contralateral to a femoral head with the
are involved, although rarely, spontaneous osteonecrosis classic features of ischemic necrosis, has been designated
can appear at other sites. minimal or segmental osteonecrosis. This type of involve-
ment is not progressive and affects the superficial zone of
the femoral head, producing a wedge-shaped involved
Spontaneous Osteonecrosis of the Femoral area that is related to vascular interruption at the wedge
Head in Adults apex and a base situated on the articular surface (Fig.
Idiopathic, or spontaneous, osteonecrosis of the femoral 67–28). The resulting radiographic findings are localized
head in adults, which is also designated Chandler’s disease, to a small portion of the femoral head but otherwise
affects men more frequently than women and is usually resemble those seen with more extensive osteonecrosis.
seen between the fourth and seventh decades of life.
Unilateral or bilateral involvement may be detected.
Spontaneous Osteonecrosis about the Knee
Despite the frequency of bilateral involvement, the condi-
in Adults
tion usually first manifests as a unilateral symptomatic
hip related to osseous collapse on the more severely This disorder, which is sometimes referred to as Ahlbäck’s
affected side. disease, is distinct from osteochondritis dissecans occur-
The radiographic features vary with the stage of the ring in adolescence (Table 67–4). The cause and patho-
disorder. Initially, a femoral head of normal osseous con- genesis of this condition are not clear; however, the
tour may reveal mottled radiodense areas scattered through- dominant opinion implicates vascular insufficiency lead-
out its anterosuperior region, with a faint curvilinear ing to infarction of bone. It typically affects older patients,
band of diminished density in the anterior subchondral more frequently women. Onset occurs with abrupt pain
A B
TABLE 67–4
Spontaneous Osteonecrosis versus Osteochondritis Dissecans (about the Knee)
A B
COMPLICATIONS
Spontaneous Osteonecrosis of the Tarsal
Navicular Bone in Adults (Mueller-Weiss Cartilaginous Abnormalities
Syndrome) One of the striking pathologic features of osteonecrosis
Rarely, atraumatic osteonecrosis of the tarsal navicular is the intactness of the chondral surface, despite the pres-
bone occurs in adults, especially women. Radiographic ence of adjacent severe osseous abnormality. It is also
characteristics include medial or dorsal protrusion of recognized that when osteonecrosis leads to significant
portions of the bone or the entire navicular bone and a collapse of the articular surface, incongruities of apposing
comma-shaped deformity caused by collapse of the bony margins may lead to secondary degenerative joint
lateral portion of the bone. Bilateral distribution, asym- disease. In this situation, cartilaginous fibrillation and
metrical involvement, and pathologic fractures represent erosion may be revealed on radiographs as diminution of
additional findings in the disease. Also termed Mueller- the interosseous space (Fig. 67–33).
Weiss syndrome, this form of osteonecrosis is distinct
from the well-recognized osteochondrosis of the tarsal
Intra-articular Osseous Bodies
navicular bone occurring in children (Köhler’s disease).
Mueller-Weiss syndrome is a disorder of adults that is Infrequently, one or more chondral or osteochondral
characterized by a chronic clinical course, severe and fragments can appear in osteonecrosis. They may exist
sometimes devastating pain and disability, and progres- free in the articular cavity, in situ in the depressed bony
sive deformity. area, or embedded in the synovium at a distant site.
1086 Section XVIII Osteonecrosis and Osteochondrosis
Cyst Formation
Cystic degeneration in areas of bone infarction predom-
inates in the diaphyses of tubular bones, especially the
tibia (Fig. 67–34) and the humerus. An expanding oste-
olytic area that erodes the cortex is seen. As it evolves, the
cyst becomes sharply marginated. This feature and the
absence of cortical disruption suggest the correct diag-
nosis, although differentiation of a cyst and malignant
degeneration of an osseous infarct may be difficult.
Malignant Degeneration
Sarcoma arising in areas of bone infarction has been
documented in both idiopathic cases and those related to
caisson disease or other disorders. Men are affected more
commonly, and the patient is usually in the fifth to seventh
decade of life. Multiple bone infarcts are frequently
present. Typically, the distal end of the femur (Fig.
67–35) or the proximal part of the tibia is the site of
neoplasm, but other sites may be altered as well. The
diametaphyseal portion of the bone is affected almost
invariably. The malignant tumor is most often fibrosar-
coma or malignant fibrous histiocytoma; more rarely, it is
osteosarcoma, angiosarcoma, or pleomorphic sarcoma. It
is suggested that these tumors arise from some of the
cellular elements involved in the repair process.
There is a long latent period between bone infarction
and malignant transformation. Although the prognosis in
patients with bone infarction and sarcoma is guarded,
and disseminated metastasis and death commonly occur
within a short period, the data in no way justify surgical
Figure 67–34. Osteonecrosis: complications—cyst formation.
Observe a bone infarct (upper arrow) in the shaft of the tibia. A ablation of infarcts because of the apparent rarity of the
cystic area (lower arrow) has led to erosion of the endosteal complication. Radiographic diagnosis is not difficult,
margin of the cortex. (From Norman A, Steiner GC: Radi- inasmuch as a soft tissue mass and osseous destruction
ographic and morphological features of cyst formation in idio- appear at a site of obvious infarction. MR imaging may
pathic bone infarction. Radiology 146:335, 1983.) be another useful diagnostic method.
Chapter 67 Osteonecrosis: Pathogenesis, Diagnostic Techniques, Specific Situations, and Complications 1087
Gelberman RH, Menon J: The vascularity of the scaphoid
bone. J Hand Surg 5:508, 1980.
Glimcher MJ, Kenzora JE: The biology of osteonecrosis of the
human femoral head and its clinical implications. II. The
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Gohel VK, Dalinka MK, Edeiken J: Ischemic necrosis of the
femoral head simulating chondroblastoma. Radiology
107:545, 1973.
Haller J, Sartoris DJ, Resnick D, et al: Spontaneous osteonec-
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Am J Roentgenol 151:355, 1988.
Houpt JB, Alpert B, Lotem M, et al: Spontaneous osteonecrosis
of the medial tibial plateau. J Rheumatol 9:81, 1982.
Jaffe HL: Ischemic necrosis of bone. Med Radiogr Photogr
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Kim Y-M, Oh HC, Kim HJ: The pattern of bone marrow
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A B Maldague BE, Noel HM, Malghem JJ: The intravertebral
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Figure 67–35. Osteonecrosis: complications—malignant
Mankin HJ, Brower TD: Bilateral idiopathic aseptic necrosis of
transformation. In this 76-year-old woman with bone infarc-
the femur in adults: “Chandler’s disease.” Bull Hosp Joint
tion, a fibrosarcoma developed at the site of bone necrosis in
Dis 23:42, 1962.
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Martel W, Poznanski AK: The effect of traction on the hip in
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osteonecrosis: A comment on the “radiolucent crescent line.”
pathologic fracture representing a fibrosarcoma. (Courtesy of
Radiology 94:505, 1970.
V. Vint, MD, La Jolla, Calif.)
Mirra JM, Bullough PG, Marcove RC, et al: Malignant fibrous
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Joint Surg Am 56:932, 1974.
FURTHER READING Mitchell DG, Kressel HY, Arger PH, et al: Avascular necrosis
of the femoral head: Morphologic assessment by MR imag-
Ahlbäck S, Bauer GCH, Bohne WH: Spontaneous osteonecrosis ing with CT correlation. Radiology 161:739, 1986.
of the knee. Arthritis Rheum 11:705, 1968. Mitchell DG, Rao V, Dalinka M, et al: MRI of joint fluid in the
Barquet A: Natural history of avascular necrosis following trau- normal and ischemic hip. AJR Am J Roentgenol 146:1215,
matic hip dislocation in childhood: A review of 145 cases. 1986.
Acta Orthop Scand 53:815, 1982. Mont MA, Baumgarten KM, Rifai A, et al: Atraumatic osteo-
Bell ALL, Edson GN, Hornick N: Characteristic bone and joint necrosis of the knee. J Bone Joint Surg Am 82:1279, 2000.
changes in compressed air workers: A survey of symptomless Morris HD: Aseptic necrosis of the talus following injury.
cases. Radiology 38:698, 1913. Orthop Clin North Am 5:177, 1974.
Bjorkengren AG, AlRowaih A, Lindstrand A, et al: Spontaneous Mulfinger GL, Trueta J: The blood supply of the talus. J Bone
osteonecrosis of the knee: Value of MR imaging in deter- Joint Surg Br 52:160, 1970.
mining prognosis. AJR Am J Roentgenol 154:331, 1990. Nellen JR, Kindwall EP: Aseptic necrosis of bone secondary
Brower AC, Downey EF Jr: Kümmell disease: Report of a case to occupational exposure to compressed air: Roentgeno-
with serial radiographs. Radiology 141:363, 1981. logic findings in 59 cases. AJR Am J Roentgenol 115:512,
Burt RW, Matthews TJ: Aseptic necrosis of the knee: Bone 1972.
scintigraphy. AJR Am J Roentgenol 138:571, 1982. Norman A, Baker ND: Spontaneous osteonecrosis of the knee
Catto M: A histological study of avascular necrosis of the and medial meniscal tears. Radiology 129:653, 1978.
femoral head after transcervical fracture. J Bone Joint Surg Norman A, Steiner GC: Radiographic and morphological fea-
Br 47:749, 1965. tures of cyst formation in idiopathic bone infarction. Radio-
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Dihlmann W: CT analysis of the upper end of the femur: The Saito S, Ohzono K, Ono K: Minimal osteonecrosis as a seg-
asterisk knee. Investigation by radionuclide scintimetry and mental infarct within the femoral head. Clin Orthop 231:35,
radiography. J Bone Joint Surg Br 53:605, 1970. 1988.
1088 Section XVIII Osteonecrosis and Osteochondrosis
Shimizu K, Moriya H, Akita T, et al: Prediction of collapse with Vande Berg B, Malghem J, Labaisse MA, et al: Avascular
magnetic resonance imaging of avascular necrosis of the necrosis of the hip: Comparison of contrast-enhanced and
femoral head. J Bone Joint Surg Am 76:215, 1994. nonenhanced MR imaging with histologic correlation.
Totty WG, Murphy WA, Ganz WI, et al: Magnetic resonance Radiology 182:445, 1992.
imaging of the normal and ischemic femoral head. AJR Am Vogler JB III, Murphy WA: Bone marrow imaging. Radiology
J Roentgenol 143:1273, 1984. 168:679, 1988.
Vance RM, Gelberman RH, Evans EF: Scaphocapitate frac- Williams JL, Cliff MM, Bonakdarpour A: Spontaneous
tures. J Bone Joint Surg Am 62:271, 1980. osteonecrosis of the knee. Radiology 107:15, 1973.
68
CHAPTER 68
Osteochondroses The concept that generalized factors may initiate or
aggravate some of the osteochondroses is supported by
numerous observations, including their occurrence in several
members of a single family, in children who are below
SUMMARY OF KEY FEATURES average in size and have a delay in skeletal maturation, and
The osteochondroses are a heterogeneous group of in those who have congenital anomalies. The importance
disorders that are usually characterized by fragmentation of trauma as an initiating event or common pathway in
and sclerosis of an epiphyseal or apophyseal center in an most of these disorders cannot be denied.
immature skeleton. Reossification and reconstitution of Although the radiographic features of the osteochon-
osseous contour may be evident in some cases. These droses have been well described and documented,
disorders can be divided into three major categories: interpretation of the changes in some of the syndromes is
(1) conditions characterized by primary or secondary debated because of the irregularity in endochondral
osteonecrosis; (2) conditions related to trauma or ossification at certain sites, such as the calcaneus and the
abnormal stress, without evidence of osteonecrosis; and tarsal navicular bone. Nevertheless, the identification of
(3) conditions that represent variations in normal patterns similar irregularities on radiographs of sites that nor-
of ossification. In some cases, a definite pathogenesis mally ossify in a uniform fashion and the identification of
cannot be identified. necrosis, granulation tissue, disorderly ossification, bone
absorption, and reparative osteogenesis on histologic inspec-
tion support the belief that some of the osteochondroses
should not be regarded as variations of normal.
TABLE 68–1
Osteochondroses
2. Smallness of the femoral ossification nucleus. A diminu- in as many as 85% of cases, may relate to synovitis with
tive ossification center may be apparent in as many as intra-articular fluid accumulation or cartilaginous
50% of patients. The pathogenesis of this finding is hypertrophy.
not clear, but it may represent an actual retardation of 4. Fissuring and fracture of the femoral ossific nucleus.
bone growth. This sign may be detected only on radiographs obtained
3. Lateral displacement of the femoral ossification nucleus. in the frog-leg position. Linear or curvilinear radiolu-
The ossific nucleus may be laterally displaced 2 to cent shadows are seen at the margin of the epiphysis,
5 mm, producing enlargement of the medial portion of and the fracture fragment may remain in situ or be
the joint space. This change, which has been reported slightly displaced.
A B
Figure 68–1. Legg-Calvé-Perthes disease: radiographic abnormalities. A, Early radiographic changes are
demonstrated. Note soft tissue distortion (arrowheads), a sclerotic femoral ossification center that is laterally
displaced and contains radiolucent fissures, and metaphyseal irregularity. B, In a different child, a frog-leg
projection delineates the fragmented sclerotic femoral ossification center containing several radiolucent fissures.
Chapter 68 Osteochondroses 1091
5. Flattening and sclerosis of the femoral ossific nucleus. Course of the Disease. The course of Legg-Calvé-Perthes
Flattening of the epiphyseal nucleus predominates near disease is variable. The degree of reconstitution of the
the fracture lines in the anterolateral superior segment ossific nucleus and the ultimate shape of the femoral head
of the femoral head. The frog-leg position is optimal depend on the amount of necrosis, its exact location, and the
for visualizing the degree of bone flattening. Mild to magnitude of forces across the joint. In some instances,
moderate sclerosis accompanies other radiographic the eventual radiographic appearance of the involved
signs of the disease, and on the anteroposterior view, head may be indistinguishable from that of its uninvolved
the entire femoral head may appear radiodense. counterpart, whereas in others, coxa plana, shortening and
6. Intraepiphyseal gas. A vacuum phenomenon caused by widening of the femoral neck, osteochondroma-like lesions
the release of gas into clefts and gaps in the subchon- of the femoral neck, degenerative joint disease, and intra-
dral trabeculae accentuates the exaggerated radiolucent articular osseous bodies may be identified (Fig. 68–3). A
appearance in this area. In most cases, however, this radiodense curvilinear shadow at the base of the femoral
finding is not present. neck, termed the “sagging rope” sign, probably represents
the radiodense shadow cast by the anterior or lateral edge
7. Metaphyseal “cysts.” Although the progression and of a severely deformed femoral head (Fig. 68–4).
extent of the disease are highly variable, further com- In most cases of Legg-Calvé-Perthes disease, changes
pression, disintegration, fragmentation, and sclerosis are isolated to or predominantly involve one hip. When
of the epiphysis may be seen, along with characteristic bilateral abnormalities are encountered, symmetrical
radiolucent lesions of the metaphysis (Fig. 68–2). The involvement is exceedingly rare, a feature that distin-
pathogenesis is debated, with some theories supporting guishes Legg-Calvé-Perthes disease from other disorders
osteonecrosis or a disturbance in endochondral bone that affect the hip. Although the chondral surface is
formation. relatively uninvolved in this disease, joint incongruity can
8. Widening and shortening of the femoral neck. lead to secondary osteoarthritis with cartilaginous fibril-
Widening and irregularity of the growth plate and lation and erosion. A detached osteochondral fragment
broadening of the metaphysis are additional manifes- (osteochondritis dissecans) can be seen in this disease
tations of this disorder. The greater trochanter may (Fig. 68–5). The reported frequency of this complication
appear disproportionately large in comparison to the is approximately 2% to 4%, and it is observed almost
shortened femoral neck. exclusively in male patients.
Pathologic Abnormalities. The fundamental pathologic Classification and Prognosis. The variable course of
aberration of the femoral head in this disease is Legg-Calvé-Perthes disease has stimulated investigation
osteonecrosis, accompanied by structural failure of the into the predictive value of certain radiographic or clinical
femoral head with resultant flattening and collapse. As in signs in determining the eventual outcome of the disorder.
other varieties of osteonecrosis, the chondral surfaces are
remarkably well preserved during the early and interme-
diate stages of Legg-Calvé-Perthes disease. Healing is
characterized by revascularization of the necrotic portion
of the femoral head.
Figure 68–2. Legg-Calvé-Perthes disease: metaphyseal Figure 68–3. Legg-Calvé-Perthes disease: coxa plana and
“cysts.” Observe the large cystic lesion of the medial metaphysis coxa magna. Note residual flattening and enlargement of the
of the femur (arrow), which is associated with a fragmented, femoral head, with surface irregularity and widening of the
sclerotic, and laterally placed ossific nucleus. femoral neck. The acetabulum is mildly flattened.
1092 Section XVIII Osteonecrosis and Osteochondrosis
C D
In general, a better prognosis is observed in boys and in The purpose of this classification system is to identify
younger patients (younger than 5 or 6 years), perhaps the degree of epiphyseal involvement and to relate this
indicating that a longer elapsed time before skeletal involvement to the prognosis of the disease. In general,
maturity allows more extensive remodeling and healing patients in groups III and IV have a relatively poor prog-
of the femoral head. Other prognostic signs relate to the nosis, whereas those in groups I and II do better. These
extent of epiphyseal involvement, and the disease can be generalizations are not without exception, however. An
separated into four groups on the basis of this involve- additional limitation of this classification system is the
ment (Table 68–2): difficulty in recognizing which pattern is present in the
Group I. The anterior part of the epiphysis is the only early phase of the disease; some patients initially placed
affected site, and collapse and sequestration are not in one category shift to another group on follow-up
evident. Metaphyseal changes are unusual but may radiography.
occur in later stages. Radiographically, the course of the Early radiographic changes useful to indicate a capital
disease is characterized by absorption of the involved femoral epiphysis at risk for collapse include (1) Gage’s
segment, followed by regeneration commencing from sign (a small, osteoporotic segment that forms a transra-
the periphery. diant V on the lateral side of the epiphysis), (2) calcifica-
tion lateral to the epiphysis (reflecting the presence of
Group II. Greater involvement is apparent. Collapse and
extruded cartilage), (3) lateral subluxation of the femoral
sequestration are followed by absorption and healing.
head, and (4) a transverse physeal line.
Group III. Only a small portion of the epiphysis is not It is clear that no single radiographic observation is
sequestered. Anteroposterior radiographs reveal a “head- indicative of a good or poor result and that a combination
within-a-head” appearance, and collapse of a centrally of findings must be used for prognostic accuracy. Most
placed sequestrum can be identified. Subsequent patients are symptom free 30 to 40 years after diagnosis,
broadening of the femoral neck is common. The course although persistent radiographic alterations are usually evi-
of the disease is similar to that in group II, although dent. A mild limp, leg shortening, and pain can be seen.
the metaphyseal changes are more generalized.
Group IV. The whole epiphysis is affected. Total collapse Other Diagnostic Methods. Arthrography may be used in
may be associated with metaphyseal changes. the evaluation of Legg-Calvé-Perthes disease. In the early
Chapter 68 Osteochondroses 1093
TABLE 68–2
Grades of Femoral Involvement in Legg-Calvé-Perthes Disease
(Catterall Classification)
Grade
I II III IV
Site of epiphyseal involvement Anterior part Anterior part Almost whole epiphysis Whole epiphysis
Sequestrum No Yes Yes Yes
Crescent sign No Anterior Anterior and extends posteriorly Anterior and posterior
Collapse No Yes Yes Yes
Metaphyseal abnormalities No Localized Diffuse Diffuse
1094 Section XVIII Osteonecrosis and Osteochondrosis
Freiberg’s Infraction
In 1914, Freiberg described a series of patients with
metatarsalgia in whom the metatarsal head appeared to
TABLE 68–3
Causes of Femoral Head Irregularity and Collapse
in Infants and Children
Disease Distribution
Legg-Calvé-Perthes disease Unilateral or bilateral
Meyer’s dysplasia Unilateral or bilateral
Hypothyroidism Bilateral
Epiphyseal dysplasia Bilateral
Figure 68–8. Legg-Calvé-Perthes disease: MR imaging Spondyloepiphyseal dysplasia Bilateral
abnormalities. In this 14-year-old boy, a coronal T1-weighted Sickle cell anemia Unilateral or bilateral
(TR/TE, 600/20) spin echo MR image reveals evidence of Gaucher’s disease Unilateral or bilateral
osteonecrosis of the left femoral head. The ossified portion
Infection Unilateral
of the capital epiphysis is flattened and contains superior and
lateral regions of abnormal signal intensity. The cartilage sur- Eosinophilic granuloma Unilateral
face, of intermediate signal intensity, appears more congruent. Hemophilia Unilateral or bilateral
The acetabulum is slightly flattened. The opposite hip is normal.
Chapter 68 Osteochondroses 1095
be crushed or collapsed, and he termed the condition an
infraction of bone. The head of the second metatarsal is
typically involved, although it is now recognized that the
third and fourth metatarsal heads can also be affected.
Unilateral changes are characteristic, although bilateral
involvement and alterations of more than one digit can
be encountered (Fig. 68–9). The disease is usually seen in
adolescents (especially girls) between the ages of 13 and
18 years. Clinical features consist of local pain, tenderness,
swelling, and limitation of motion of the corresponding
metatarsophalangeal joint.
The radiographic features of Freiberg’s infraction are
virtually pathognomonic. Initial radiographic abnormali-
ties include subtle flattening, increased radiodensity, and
cystic lucent lesions of the metatarsal head and widening
of the metatarsophalangeal articulation; subsequently, an
osteochondral fragment with progressive flattening and
sclerosis of the metatarsal head, and periostitis with
increased cortical thickening of the adjacent metaphysis
and diaphysis of the bone, may be seen (Fig. 68–10).
Premature closure of the growth plate, intra-articular
osseous bodies, deformity and enlargement of the metatarsal
head, and secondary degenerative joint disease are recog-
nized complications of the process. The nearby pha-
langeal base may enlarge and appear irregular. Increased Figure 68–10. Freiberg’s infraction: late radiographic abnor-
accumulation of bone-seeking radiopharmaceutical malities. A loose body had been removed from the second
agents is evident in the abnormal sites, and MR imaging metatarsophalangeal joint 2 years before this evaluation. Note
reveals abnormalities in osseous contour and alterations the flattened metatarsal head with two osteochondral fragments
of signal intensity in the bone marrow (Fig. 68–11). (arrowheads), osteophytosis, joint space narrowing, and widen-
The prevailing opinion suggests that single or ing of the phalangeal base.
multiple episodes of trauma represent the primary event
in Freiberg’s infraction. The high rate of occurrence in
women could conceivably be related to the wearing of
high-heeled shoes, which creates increased stress on the
second metatarsal bone. Repeated injury at this site may
lead to disruption of articular cartilage, ischemic necrosis
of subchondral bone, compression fracture, collapse, and
fragmentation.
Kienböck’s Disease
Also termed lunatomalacia, this peculiar affliction of the
carpal lunate was described by Kienböck in 1910.
Kienböck’s disease is most commonly observed in patients
A B
Figure 68–12. Kienböck’s disease. A, Magnification radiograph demonstrates patchy increased density of
the lunate, without alterations in the shape of the bone. B, In a different patient, observe the collapse of a
sclerotic lunate bone. The ulna is shortened (ulna minus variant).
Chapter 68 Osteochondroses 1097
A B
Figure 68–14. Kienböck’s disease: MR imaging abnormalities. A, In this 43-year-old woman, a coronal
T1-weighted (TR/TE, 600/15) spin echo MR image shows diffuse low signal intensity in the lunate. Note
the scapholunate dissociation. B, Coronal gradient echo (TR/TE, 600/15; flip angle, 35 degrees) MR image
reveals patchy high signal intensity in the lunate, disruption of the scapholunate interosseous ligament,
disruption of the radial aspect of the triangular fibrocartilage, and effusions in the radiocarpal, midcarpal,
and inferior radioulnar joints.
Panner’s Disease
Panner’s disease, or osteochondrosis of the capitulum of the
humerus, is a rare disorder that usually appears between
the ages of 5 and 10 years. Boys are affected almost exclu- Figure 68–17. Panner’s disease. Findings include fissuring
sively, and the condition is commonly linked to a history and fragmentation of the capitulum (arrow) and deformity of
of trauma. It is sometimes termed “Little Leaguer’s the adjacent radial head in this child with elbow pain and
elbow” because of its frequency in young baseball pitchers. swelling. (Courtesy of V. Vint, MD, La Jolla, Calif.)
Bilateral involvement is rare. Clinical manifestations are
typically mild, and complete recovery is frequent. Pain
and stiffness with restricted range of motion of the elbow appear sclerotic and fragmented and may contain medial
are seen. and lateral osseous excrescences (Fig. 68–18). Eventually,
Radiographs reveal fissuring and increased density of the joint space becomes narrowed, the base of the phalanx
the capitulum, decreased size and condensation of bone thickens, and phalangeal shortening is seen (Fig. 68–19).
with an increase in the radiohumeral space, fragmentation, The differential diagnosis of the phalangeal alterations
and resorption (Fig. 68–17). Subsequent regeneration of Thiemann’s disease includes trauma, infection, thermal
and reconstitution of the capitulum are observed, and in injury, and juvenile chronic arthritis.
most cases, no residual deformity or disability is seen.
Hyperemia can lead to abnormal skeletal maturation of
the radial head. The disease is differentiated from osteo- DISORDERS RELATED TO TRAUMA OR
chondritis dissecans of the elbow principally on the basis ABNORMAL STRESS WITHOUT EVIDENCE
of the age of the patient. Osteochondritis dissecans typi- OF OSTEONECROSIS
cally occurs in adolescents or adults, at a time when
ossification of the capitulum is complete. Osgood-Schlatter Disease
Osgood-Schlatter disease occurs in adolescents, usually
between the ages of 11 and 15 years. Boys are affected
Thiemann’s Disease
more frequently than girls, and a history of participation
Thiemann’s disease is characterized by progressive in sports and a rapid growth spurt before the onset of
enlargement of the proximal interphalangeal joints of the symptoms and signs are typical. Although the disease is
fingers, an onset in the second decade of life, and a predilec- generally unilateral in distribution, bilateral alterations
tion for boys. Some reports indicate that the principal are detected in approximately 25% of cases. Clinically,
clinical manifestations of this disease are painless swelling patients usually have local pain and tenderness of variable
of the proximal interphalangeal articulations, digital severity. Soft tissue swelling and firm masses can be
shortening, and deformity. Thiemann’s disease is familial, palpated in the involved region, but no synovial effusion
probably transmitted as a dominant trait with virtually is present in the knee.
complete penetrance. Initial radiographic abnormalities include soft tissue
Radiographs reveal irregularity of the epiphyses of the swelling in front of the tuberosity, resulting from edema
phalanges, especially in the middle fingers. The epiphyses of the skin and subcutaneous tissue (Fig. 68–20). The
Chapter 68 Osteochondroses 1099
A B
Figure 68–22. Osgood-Schlatter disease: MR imaging abnormalities. A, Sagittal T1-weighted (TR/TE,
850/25) spin echo MR image demonstrates fragmentation and irregularity of the tibial tuberosity at the
insertion site of the patellar tendon (arrow). B, On a sagittal gradient echo (TR/TE, 750/20; flip angle, 45
degrees) MR image, note the joint effusion and fluid (arrow) in the deep infrapatellar bursa adjacent to the
site of fragmentation of the tuberosity.
A fragmented tuberosity can occur in other conditions dysplasia, which leads to abnormalities that may simulate
and, in the absence of current or previous symptoms, may those of classic Blount’s disease.
indicate only a normal ossification pattern. Soft tissue
swelling is fundamental to the radiographic diagnosis of Infantile Tibia Vara. Infantile tibia vara appears to develop
Osgood-Schlatter disease. In the past, Osgood-Schlatter when normal physiologic bowing persists rather than
disease was considered by some investigators to be a type progressively changes to a straight leg or slight valgus
of osteonecrosis. More recent studies, however, indicate position; it worsens once a growing child becomes heavier
that the tibial tuberosity possesses an excellent blood and begins to put weight on the knee joint. Additional
supply and that the pathologic findings in this condition mechanical factors that may contribute to infantile tibia
are most consistent with traumatically induced disruption vara include excessive body weight and abnormal articular
somewhere along the site of attachment of the patellar laxity, but these factors are not present consistently.
tendon to the tibial tuberosity. The appearance of frag- Altered mechanical forces in the proximal end of the tibia
mentation of the inferior pole of the patella in some
patients with Osgood-Schlatter disease supports the
concept of a traumatic insult, as does evidence of patellar
tendinosis provided by MR imaging. TABLE 68–4
Infantile versus Adolescent Tibia Vara
Blount’s Disease
Infantile Adolescent
Blount’s disease, also called tibia vara or osteochondrosis Age of onset 1–3 yr 8–15 yr
deformans tibiae, is a local disturbance in growth of the
Distribution Bilateral: 50–75% Unilateral: 90%
medial aspect of the proximal tibial epiphysis. The condi-
tion is generally classified into two types: an infantile Clinical findings Obesity Normal body
Absent pain, weight
type in which deformity is noted in the first few years of tenderness Pain and
life, and an adolescent type in which deformity appears Prominent deformity tenderness
between the ages of 8 and 15 years (Table 68–4). The Slight leg shortening Mild deformity
infantile type is approximately five to eight times more Moderate, severe
frequent than the adolescent variety. Recently, the classi- leg shortening
fication of tibia vara has been modified and expanded to Cause or Trauma Trauma
include a third and fourth type—late-onset tibia vara, pathogenesis Growth arrest or Growth arrest
which typically appears in obese black children between dysplasia
the ages of 6 and 13 years, and focal fibrocartilaginous
Chapter 68 Osteochondroses 1101
from various primary causes can increase the mobility of Radiographic abnormalities are seldom evident before
the tibia on the femur and result in a change in direction age 2 years. They simulate those of physiologic bowing
of weight-bearing forces on the upper tibial epiphysis but are more severe and can be unilateral or asymmetrical
from perpendicular to oblique. This obliquity tends to in distribution. Further, altered alignment in Blount’s
displace the tibial epiphysis in a lateral direction, with disease occurs in the proximal portion of the tibia, not
subsequent overloading of the mediodorsal segment of between the femur and tibia, as occurs in physiologic
the bone. Histologic examination confirms the absence bowing. In Blount’s disease, the tibia is in a varus position
of changes attributable to infection or osteonecrosis. The because of angulation of the metaphysis, and the tibial
microscopic findings are consistent with the effects of shaft is adducted without intrinsic curvature. A depressed
persistent abnormal pressure on the growth plate. medial tibial metaphysis with an osseous excrescence, or
Clinically, progressive bilateral (60%) or unilateral outgrowth, is seen. Six stages have been recognized on
(40%) bowing of the leg during the first year of life is radiographs (Figs. 68–23 and 68–24):
difficult to differentiate from physiologic changes. The Stage I (2 to 3 years). A progressive increase in the degree
tibia may be angulated acutely inward just below the of varus deformity of the tibia is associated with irregu-
knee. Pain is not evident. Associated findings are obesity, larity of the entire growth plate. The medial part of the
shortening of the leg, tibial torsion, and pronated feet. metaphysis protrudes with a medial and distal beak.
Figure 68–23. Blount’s disease: infantile tibia vara. Six stages of the disease (see text for details). (After
Langenskiöld A: Tibia vara. Osteochondritis deformans tibiae. A survey of 23 cases. Acta Chir Scand 103:1,
1952.)
A B
Figure 68–24. Blount’s disease: infantile tibia vara. The various stages of the disease are well illustrated
in this child. A, Stage II, at age 4 years. B, Stage III–IV, at age 8 years. (Courtesy of L. Danzig, MD, Santa
Ana, Calif.)
1102 Section XVIII Osteonecrosis and Osteochondrosis