SYSTEMIC DISEASES

MANIFESTED
IN THE JAWS

Endocrine disorders
1. Parathyroid gland: hyper & hypoparathyroidism
2. Pituitry gland: hyper & hypopituitarism
3. Thyroid gland: hyper & hypo thyroidism
4. Adrenal gland: Cushing syndrome
Bone metabolism disorders
5. Vitamin D related: Rickets, Osteomalacia. Renal osteodystrophy,
Hypophosphatemia
6. Osteoporosis, hypophosphatasia : Bone loss
7. Osteopetrosis: Lack of resorption> increased bone
Blood disorders & others
8. Sickle cell anaemia
9. Thalassemia
10.Progressive systemic sclerosis

Radiographic Features
The general changes include the following:
1. A change in size and shape of the bone
2. A change in the number, size, and orientation of
trabeculae
3. Altered thickness and density of cortical structures
4. An increase or decrease in overall bone density

Changes to teeth and associated structures include

the
following:
1.
2.
3.
4.

Accelerated or delayed eruption

Hypoplasia
Hypocalcification
Loss of a distinct lamina dura

HYPERPARATHYROIDISM
Radiographic Features
Only about one in five patients with hyperparathyroidism has
radiographically
observable bone changes.
General Radiographic Features. The following are the major
manifestations of hyperparathyroidism:
1. The earliest and most reliable changes of hyperparathyroidism
are subtle erosions of bone from the subperiosteal surfaces of the
phalanges of the hands.
2. Demineralization of the skeleton results in an unusual radiolucent
appearance.
3. Osteitis fibrosa cystica are localized regions of bone loss produced
by osteoclastic activity resulting in a loss of all apparent bone
structure.
4. Brown tumors occur late in the disease and in about 10% of
cases. These peripheral or central tumors of bone are radiolucent.
The gross specimen has a brown or reddish-brown color.
5. Pathologic calcifications in soft tissues have a punctate or nodular

Axial and, B, sagittal computed tomographic images with bone

algorithm of a case of
secondary hyperparathyroidism. Note the lack of normal cortical
bone at the inner and outer tables of the skull, internal granular
bone pattern, and generalized lack of defined outer cortical

HYPERPARATHYROIDISM

Panoramic image. The loss of bone in

hyperparathyroidism results in the radiopaque teeth
standing out in contrast to the radiolucent jaws.

Axial and coronal computed tomographic images secondary

hyperparathyroidism with a brown tumor involving the
maxilla.
This tumor has features of a central giant cell granuloma
with a granular expanded cortex of the maxilla and very
subtle and ill-defined internal septa.

the loss of a distinct lamina dura and the granular texture

of the bone pattern in this periapical film

Granular bone pattern that was characteristic in all intraoral

films. Note the loss of a distinct lamina dura and floor of the
maxillary antrum.
This view of the same case reveals a brown tumor related to
the apical region of the second and third molars.

HYPOPARATHYROIDISM AND
PSEUDOHYPOPARATHYROIDISM
Most often this includes sharp flexion (tetany)
of the wrist and ankle joints (carpopedal
spasm).
Some patients have sensory abnormalities
consisting of paresthesia of the hands, feet, or
the area around the mouth
The principal radiographic change is
calcification of the basal ganglia. On skull
radiographs this calcification appears flocculent
and paired within the cerebral hemispheres on
the posteroanterior view.

 Radiographic
examination of the
jaws may reveal
dental enamel
hypoplasia, external
root resorption,
delayed eruption, or
root dilaceration

HYPERPITUITARISM
Acromegaly and Giantism

A, An example of acromegaly manifesting as excessive growth

of the mandible, resulting in a class III skeletal relationship of
the jaws. B, A portion of a lateral skull view of the same patient
demonstrating enlargement of the sella turcica.

Radiographic Features of
the Jaws
The increase in the length of the dental arches results in
spacing of the teeth
In acromegaly the angle between the ramus and body of
the mandible may increase
in combination with enlargement of the tongue
(macroglossia), may result in anterior flaring of the teeth
and the development of an anterior open bite
class III skeletal relationship between the jaws. The
thickness and height of the alveolar processes may also
increase.
The tooth crowns are usually normal in size, although the
roots of posterior teeth often enlarge as a result of
hypercementosis.

HYPOPHOSPHATASIA
Hypophosphatasia is a rare inherited disorder that is caused by
either a reduced production or a defective function of alkaline
phosphatase

the large pulp chambers in the deciduous dentition and

the premature loss of the mandibular incisors.

Radiographic Features
long bones show irregular defects in the epiphysis, and
the skull is poorly calcified.
In older children with premature closure of the skull
sutures, multiple lucent areas of the calvarium may exist,
called gyral or convolutional markings. These markings
resemble hammered copper
skull may assume a brachycephalic shape
A generalized reduction in bone density
Jaws. A generalized radiolucency of the mandible and
maxilla is evident. The cortical bone and lamina dura are
thin, and the alveolar bone is poorly calcified and may
appear deficient.
Teeth. Both primary and permanent teeth have a thin
enamel layer and large pulp chambers and root canals

HYPOPHOSPHATEMIA
Vitamin D resistant rickets and hypophosphatemic rickets

Hypophosphatemia represents a group of

inherited conditions that produce renal tubular
disorders resulting in excessive loss of
phosphorus.

A panoramic image of hypophosphatemia. Note the

radiolucent appearance of the

These periapical films of a different case of hypophosphatemia

demonstrate apparent bone loss around the teeth, a granular
bone pattern, large pulp chambers, and external root resorption.

OSTEOPETROSIS
Albers-Schnberg and marble bone disease

Osteopetrosis is a disorder of bone that results

from a defect in the differentiation and
function of osteoclasts. The lack of normally
functioning osteoclasts results in abnormal
formation of the primary skeleton and a
generalized increase in bone mass
1. autosomal recessive type (osteopetrosis
congenita)
2. autosomal dominant type (osteopetrosis
tarda).

 Osteopetrosis,
showing dense
calcifi cation of all
the bones.
A, Skull and
facial bones. B,
Chest.
C, Pelvis and
femurs (note the
fracture of the
proximal right
femur).

A panoramic fi lm of a patient with osteopetrosis; note the

increased density of the
jaws, lack of eruption of the mandibular second bicuspids,
narrow inferior alveolar nerve canal, and development of
osteomyelitis in the body of the left mandible with periostitis

Differential Diagnosis
The differential diagnosis includes other sclerosing
bone dysplasias
sclerosteosis,
infantile cortical hyperostosis,
pyknodysostosis,
craniometaphyseal dysplasia,
diaphyseal dysplasia, melorheostosis,
osteopathia striata.
Osteosclerosis from fluoride poisoning and
secondary hyperparathyroidism from renal disease
also may have a general sclerotic appearance.

PROGRESSIVE SYSTEMIC SCLEROSIS

Scleroderma

Progressive systemic sclerosis (PSS)

is a generalized connective tissue
disease that causes excessive
collagen deposition resulting in
hardening (sclerosis) of the skin and
other tissues.

Progressive systemic sclerosis demonstrating a loss

of bone in the region of the angle of the mandible
(arrows) and at the right coronoid process (arrow),
which are locations of muscle attachments.

Two periapical fi lms of two different patients with PSS; note

the widening of the periodontal membrane space around
some of the teeth.

SICKLE CELL ANEMIA

Sickle cell anemia is an autosomal
recessive, chronic hemolytic blood
disorder. Patients with this disorder
have abnormal hemoglobin
(deoxygenated hemoglobins), which
under low oxygen tension results in
sickling of the red blood cells.

SICKLE CELL ANEMIA

Radiograph of a patient with sickle cell anemia showing a

thickened diploic space and thinning of the skull cortex. B,
Normal skull for comparison.

Skull showing the hair-on-end bone pattern.

THALASSEMIA
Cooley s anemia, Mediterranean anemia, and
erythroblastic anemi

Thalassemia is a hereditary disorder

that results in a defect in hemoglobin
synthesis
- or globulin genes.
1. The heterozygous form (thalassemia
minor) is mild.
2. The homozygous form (thalassemia
major) may be severe. A less severe
form
3. Thalassemia intermedia

A skull radiograph of a patient with thalassemia showing a

granular appearance of
the skull and thickening of the diploic space. B, An axial CT
image of the skull of a patient with thalassemia; note the
thickened diploic space and that there is hint linear orientation
of the trabeculae, especially in the frontal bone

A panoramic film of a patient with

thalassemia; note the thickened body of the
mandible and the sparse trabeculae and lack
of maxillary antra.
B, Radiograph of a different patient with
thalassemia with thick trabeculae and large
bone marrow spaces.