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C H A P T E R 1 2 

B. Stephens Richards  
Scoliosis Daniel J. Sucato  
Charles E. Johnston
only after a thorough physical and radiographic examination
Chapter Outline has ruled out neurologic causes, syndromes, and congenital
anomalies. Idiopathic scoliosis may have its onset at any age
Definition          206 during growth, but three fairly well defined peak periods
Classification of Scoliotic Curves          206 are accepted: (1) in the first year of life, (2) at 5 to 6 years
Idiopathic Scoliosis          206 of age, and (3) after 11 years of age to the end of skeletal
Adolescent Idiopathic Scoliosis          206 growth.
Juvenile Idiopathic Scoliosis          247 The term adolescent idiopathic scoliosis (AIS) is used
Congenital Spinal Deformities          247 when the deformity is recognized after the child has reached
Early-Onset Scoliosis          265 10 years of age but before skeletal maturity, although it is
Other Causes of Scoliosis          281 typically noted before the onset of puberty. Infantile idio-
pathic (younger than 3 years) and juvenile idiopathic scolio-
sis (3 to 10 years old) are now included within “early-onset”
scoliosis, a group that includes any type of scoliosis diag-
Definition nosed before the age of 10 years.
Scoliosis recognized after skeletal maturity is defined as
The term scoliosis, first used by Galen (131-201 AD), is adult scoliosis.
derived from the Greek word meaning “crooked.”514 One
of the most common deformities of the spine, scoliosis has
been recognized since ancient times, with descriptions of
normal and abnormal spinal curves found in the Corpus Adolescent Idiopathic Scoliosis
Hippocraticum. In 1741, André devised the crooked spine Prevalence
as his symbol for orthopaedics.23
Currently, scoliosis is defined as lateral deviation of the The prevalence of radiographic curves measuring at least 10
normal vertical line of the spine, which when measured on degrees ranges from 1.5% to 3.0%, that of curves exceeding
a radiograph, is greater than 10 degrees (Fig. 12-1). Because 20 degrees is between 0.3% and 0.5%, and that of curves
the lateral curvature of the spine is associated with rotation exceeding 30 degrees is between 0.2% and 0.3%.
of the vertebrae within the curve, a three-dimensional A definite relationship between idiopathic scoliosis and
deformity occurs. This complex deformity represents sex has been noted, particularly as the magnitude of the
abnormal movement in three planes: (1) intervertebral curve increases. The ratio of affected females to males has
extension in the sagittal plane leading to lordosis of the been reported to be 1 : 1 for curves between 6 and 10
scoliotic segment, (2) lateral intervertebral tilting in the degrees, 1.4 : 1 for curves between 11 and 20 degrees, 5.4 : 1
frontal plane, and (3) a rotatory component in the axial for curves exceeding 21 degrees but not requiring treat-
plane.592 This results in torsion of the spine, with the most ment, and 7.2 : 1 for curves requiring orthopaedic interven-
significant abnormality located in the apical region. As the tion.650 This sex prevalence in idiopathic scoliosis—that is,
deformity worsens, structural changes develop in the verte- an equal prevalence between the sexes for small curves
brae and rib cage. Relationships between intrathoracic and (<10 degrees), with increasing female prevalence for larger
abdominal organs may be distorted as the deformity and progressive curves—has been reported by several
becomes severe, but rarely are the organs’ functions authors.29,162,454,650 The clinical significance of these observa-
compromised. tions is that curve progression is more common in girls.

Natural History
Classification of Scoliotic Curves
Few current natural history studies have examined curve
A variety of terms are used to describe the different types progression in the untreated, skeletally immature scoliosis
of scoliotic curves. Box 12-1 provides definitions for the population,96,455,534 and consensus is lacking in the literature
most common ones. regarding the definition of curve progression. Measurable
increases in curve size of 5, 6, and 10 degrees have all been
reported as being representative of progression.* Most
Idiopathic Scoliosis studies use increases of more than 5 or 6 degrees as indica-
tive of definite progression.
Idiopathic scoliosis, for which a definitive cause of the
deformity has not been established, is the most common
type and accounts for nearly 80% of patients with structural
scoliosis. The diagnosis of idiopathic scoliosis can be made *References 140, 157, 257, 359, 362, 610, 724, 774, 816.


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CHAPTER 12  Scoliosis 207

velocity (PHV), and in females, menarchal status (a physi-

ologic marker).
The Risser sign is a radiographic measurement based on
ossification of the iliac apophysis, which is divided into four
quadrants,645 beginning on the lateral aspect of the iliac
apophysis and progressing medially (Fig. 12-2). The Risser
sign proceeds from grade 0, no ossification, to grade 4, in
which all four quadrants of the apophysis show ossification
(“capping”). When the ossified apophysis has fused com-
pletely to the ilium (Risser grade 5), the patient is fully
skeletally mature. Patients with Risser grade 0 or 1 (and to
a lesser extent, grade 2) are at greatest risk for curve pro-
gression because a significant amount of spinal growth
remains. A modified Risser grading system has been created
in which a new group, Risser 0 with closed triradiate carti-
lage, and Risser 1 were found to be the best predictors of
the beginning of rapid curve progression.539 The triradiate
cartilage cannot be used as an independent predictor of
curve stability, but it may serve as an additional indicator of
skeletal maturity.658
Menarchal status is a clinical measurement applicable
only to females. A premenarchal girl is still in the active
growth period. After menarche, she enters the deceleration
phase of growth, and the likelihood of curve progression
lessens. The Tanner index of maturity,758 which is based on
assessment of breast and genital development, is another
clinical index that has been used to determine a child’s
remaining growth and thus can indirectly predict the risk
for curve progression.
PHV is a measurement of the maximal skeletal growth
that occurs during the adolescent growth spurt (Fig.
12-3).442,673,716 Calculated from changes in a patient’s height
measurements over time, PHV is fairly consistent in the
FIGURE 12-1  Posteroanterior radiograph of the thoracolumbar published literature and is reported to be about 8.0 cm/yr
spine of a 13-year-old girl showing right thoracic scoliosis of   for girls and 9.5 cm/yr for boys.92,224,758 The reported average
45 degrees. age at PHV in North American girls is approximately 11.5
years. Closure of the triradiate cartilage, a radiographic
index of maturity, occurs after PHV and before Risser grade
1 and menarche. For PHV to be clinically useful, serial
Natural History Before Skeletal Maturity height measurements must be obtained. Six-month inter-
Individuals with untreated curves of less than 20 degrees vals are preferred because shorter intervals may result
are at low risk for progression, particularly as they approach in significant measurement error. If height data are not
skeletal maturity.455 Because some patients, however, have available from the patient’s records, the information can
curves that progress over the years and ultimately lead to often be obtained from the family, school, or pediatrician.
health problems, it is important to recognize the factors Although PHV requires analysis of serial height measure-
associated with curve progression, including patient sex, ments collected over time, it is the earliest and best index
remaining growth, curve magnitude, and curve pattern.96,455,534 available to demonstrate that growth is slowing and the risk
Factors of no predictive value for curve progression before for curve progression is diminishing. In boys, use of PHV
skeletal maturity include a family history of scoliosis, patient to predict the period of remaining growth is superior to the
height-to-weight ratio, lumbosacral transitional anomalies, Risser sign and chronologic age, and closure of the triradiate
thoracic kyphosis, lumbar lordosis, and spinal balance.96 cartilage approximates the time of PHV.676
A simplified skeletal maturity scoring system for AIS
Sex that uses radiographs of the hand has also been developed.672
The majority of patients whose curves progress and ulti- The Tanner-Whitehouse III score, which is based on the
mately require treatment are female.29,162,454,650 Although radiographic appearance of the epiphyses of the distal ends
the exact reason for this phenomenon remains unknown, of the radius and ulna, the small bones of the hand, and the
hormonal influences have been proposed.9,283,708 digital skeletal maturity scoring system (which is based on
the metacarpals and phalanges), correlates highly with curve
Remaining Growth acceleration in girls with AIS. This system is reliable and
A young patient’s remaining growth is usually assessed by correlates more strongly with the behavior of idiopathic
four maturity indices: the Risser sign (a skeletal marker of scoliosis than does the Risser sign. It appears to be strongly
the pelvis), hand and wrist skeletal maturity,672 peak height prognostic of future scoliosis curve behavior.

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208 SECTION II  Anatomic Disorders

Box 12-1  Types of Scoliosis and Scoliotic Curves

Adult scoliosis: Spinal curvature present after skeletal maturity represent such excessive rotation of the spine that a lateral
as a result of any cause. radiograph is actually reflecting the scoliotic deformity.  
Cervicothoracic curve: Any spinal curvature in which the apex (In idiopathic scoliosis, true kyphotic deformity does not
is at C7 or T1. occur.)
Compensatory curve: Secondary curve located above or Lordoscoliosis: Structural scoliosis associated with increased
below the structural component that develops to maintain swayback or loss of normal kyphosis within the measured
normal body alignment. curve; it is nearly always present in idiopathic scoliosis.
Congenital scoliosis: Scoliosis caused by bony abnormalities Lumbar curve: Spinal curvature in which the apex is between
of the spine that are present at birth. The anomalies are L1 and L4.
classified as failure of vertebral formation or failure of Lumbosacral curve: Spinal curvature in which the apex is at
segmentation. L5 or below.
Double curve: Scoliosis in which two lateral curves are present Neuromuscular scoliosis: Scoliosis caused by a neurologic
in the same section of spine. disorder of the central nervous system or muscle.
Double major curve: Scoliosis in which two structural curves, Nonstructural (functional) curve: Curvature that does not
usually of similar size and rotation, are present. have a fixed deformity and may be compensatory in nature.
Double thoracic curve: Scoliosis with a structural upper The curve may be a result of leg length discrepancy (in which
thoracic curve; a larger, more deforming lower thoracic curve; case it disappears when the patient is supine), poor posture,
and a relatively nonstructural lumbar curve. muscle spasm, or some other cause.
Hysterical scoliosis: Nonstructural deformity of the spine that Primary curve: The first or earliest curve present.
is a manifestation of a psychological disorder. Structural curve: Segment of the spine that has a fixed lateral
Idiopathic scoliosis: Structural spinal curvature, the cause of curvature.
which has not been definitely established. Thoracic curve: Spinal curvature in which the apex is between
Kyphoscoliosis: Seen as an increased round back on a lateral T2 and T11.
radiograph, this condition may represent a true kyphotic Thoracolumbar curve: Spinal curvature in which the apex is
deformity (as occurs in some pathologic conditions), or it may at T12, L1, or the T12-L1 interspace.

3 4

TRC closure
Height Risser 1 and menarche
Risser 2

Crankshaft No crankshaft


FIGURE 12-3  Schematic drawing of height velocity. Closure of the

triradiate cartilage (TRC) occurs after the period of peak height
velocity (PHV) and before Risser grade 1 and menarche are
attained. (Modified from Sanders JO, Little DG, Richards BS:
FIGURE 12-2  The Risser sign proceeds from grade 0 (no
Prediction of the crankshaft phenomenon by peak height velocity,
ossification) to grade 4 (all four quadrants show ossification of the
Spine 22:1352, 1997.)
iliac apophysis). When the ossified apophysis has fused completely
to the ilium (Risser grade 5), the patient is skeletally mature.

Curve Magnitude Table 12-1  Incidence of Curve Progression

The size of the existing curve when scoliosis is recognized Based on Curve Magnitude and Risser Grade
is helpful in predicting curve progression. The combination
Percentage of Curves That Progress
of this factor and assessment of remaining growth is used Risser
to predict the natural history in young patients with scolio- Grade Curves 5-19 Degrees Curves 20-29 Degrees
sis. Immature patients (premenarchal, Risser grade 0) with
0 or 1 22 68
curves greater than 20 degrees are at substantial risk for
progression of spinal deformity (Table 12-1).96,265,450,451,534,650 2, 3, or 4 1.6 23
For immature patients with curves exceeding 25 to 30
Modified from Lonstein JE, Carlson JM: The prediction of curve
degrees, the risk for curve progression is believed to be progression in untreated idiopathic scoliosis during growth, J Bone Joint
significant enough to recommend orthotic management at Surg Am 66:1061, 1984, with permission from The Journal of Bone
the time of initial evaluation.364,456,534,610,724,774 and Joint Surgery, Inc.

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CHAPTER 12  Scoliosis 209

those greater than 50 degrees, are also likely to progress in

Curve Pattern adulthood and lead to osteoarthritis. Therefore, even when
The curve pattern is useful for predicting curve progression. cosmetic factors are not taken into account, aggressive
Double curves and thoracic curves are most likely to pro­ treatment of a child with a significant spinal deformity is
gress, followed by thoracolumbar curves. Lumbar scoliosis justified.
is reportedly the least likely to worsen.96,455
Scoliosis Screening
Prognostic Testing
A genetic screening test called the ScoliScore is available as School Screening Programs
an adjunct to clinical and radiographic information to deter- A number of medical organizations support the general
mine the risk for progression of AIS.563,808 This test can be screening of children for scoliosis. In 2008, the Scoliosis
used in white (North American, European, Eastern Euro- Research Society (SRS), the American Academy of
pean, Middle Eastern) patients between the ages of 9 and Orthopaedic Surgeons (AAOS), the Pediatric Orthopaedic
13 years with a mild scoliotic curve (<25 degrees). The Society of North America (POSNA), and the American
stated goal of the test is to determine the risk that the curve Academy of Pediatrics (AAP) endorsed an information
will increase to 40 degrees or greater. Further independent statement in support of screening for AIS.638 This 2008
verification of the test is needed before its usefulness for statement was a response to a recommendation made by
this condition can be determined. the U.S. Preventive Service Task Force.785 For years this task
force stated that the evidence was insufficient to recom-
Natural History After Skeletal Maturity mend either for or against routine screening of asymptom-
In general, the rate of progression of scoliosis in adulthood atic adolescents for idiopathic scoliosis.57,782-784 In 2004,
is much slower than that in adolescence and depends on the based on a brief evidence update, the task force changed its
size of the curve once skeletal maturity has been reached. position and recommended against routine screening.785 It
Regardless of the curve pattern, curves of less than 30 reported that no good evidence could be found that screen-
degrees in a mature individual are unlikely to progress. ing of asymptomatic adolescents detects idiopathic scoliosis
Conversely, approximately two thirds of curves that exceed at an earlier stage. However, as the primary care providers
50 degrees worsen, with thoracic curves progressing nearly for adolescents with idiopathic scoliosis, the SRS, AAOS,
1 degree per year.811 Lumbar curves also tend to progress POSNA, and AAP do not support any recommendation
in adulthood and may do so at a magnitude smaller than against scoliosis screening given the available literature. If
50 degrees if they are accompanied by a transitory shift scoliosis screening is undertaken, the SRS, AAOS, POSNA,
between the lower vertebrae. and AAP agree that girls should be screened twice, at ages
In terms of the long-term status of adults with untreated 10 and 12 (grades 5 and 7), and boys once, at age 13 or 14
scoliosis, several Swedish studies have reported an overall (grades 8 or 9).
mortality rate greater than that predicted by national mor- The clinical logic behind school screening for idiopathic
tality statistics.533,551,587 However, these studies included scoliosis assumes that screening is an accurate and reliable
patients with nonidiopathic scoliosis and those with infan- method of detecting curvatures, early detection results in
tile deformities. When examined selectively, the mortality improved health outcomes, and brace therapy is effective
rate of patients with AIS appeared to be the same as that in altering the natural history of the deformity.16,524,783,796,819
in the general population.587 A significant increase in the The implications of these assumptions are that small curva-
mortality rate was identified for patients with infantile and tures detected through screening are likely to progress to
juvenile idiopathic scoliosis. Respiratory failure and cardio- curvatures of clinical significance, that scoliosis causes sig-
vascular disease accounted for most early deaths. Respira- nificant health problems, and that the benefits of early
tory failure developed in adults with severe scoliosis (>110 detection outweigh the potential adverse effects of screen-
degrees) as normal aging further reduced their ventilatory ing and treatment. The many proponents of school screen-
capacity.586 In patients who underwent surgery for scoliosis, ing believe that these assumptions are successfully addressed
respiratory failure tended to not develop, thus suggesting a by school screening programs.453,506 The idea of general sco-
preventive effect of corrective surgery.588 liosis screening is not universally accepted, however, and is
Chronic back pain is common in adults with scoliosis, becoming more controversial.525,785,796,862,863 Some authors
although it is not related to the size or location of the argue that school screening programs have not reduced the
curvature.145,533,809 The pain does not usually interfere with prevalence or incidence of scoliosis requiring treatment, are
the patient’s ability to work or perform daily activities. not cost-effective, and result in children with no scoliosis
Lumbar osteoarthritis may also be seen in up to 83% of or only a mild degree of curvature that does not require
adults with scoliosis, but it is not necessarily associated with treatment being unnecessarily referred to orthopaedic sur-
the duration or intensity of back pain. Despite outwardly geons or radiologists.862,863 However, a study involving more
apparent deformities because of long-standing untreated than 150,000 students reported that school screening is
scoliosis, most individuals have no significant psychological predictive and sensitive with a low referral rate.469 The
difficulties when compared with persons without scoliosis authors recommended that screening be continued to facili-
(the sole exception being a slight dissatisfaction with body tate early initiation of conservative treatment.
In summary, thoracic scoliosis of greater than 50 to 60 Screening Methods
degrees in adulthood may progressively worsen and poten- Several clinical signs are indicative of possible scoliosis
tially reduce pulmonary function. Lumbar curves, especially and are frequently used in screening programs, including

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210 SECTION II  Anatomic Disorders

shoulder asymmetry, unequal scapular prominence, appear- One of the constant features of structural scoliosis is
ance of an elevated or prominent hip, greater space between axial rotation of the vertebrae affected by the curve. The
the arm and body on one side (with the arms hanging spinous processes almost always rotate toward the concavity
loosely at the side), head not centered over the pelvis, and of the curvature. Rotation of the thoracic vertebrae is also
a positive Adams forward-bending test. The Adams test is impaired by rotation and deformity of the attached rib cage,
performed by having the child bend forward until the spine with elevation on the side of the convexity and depression
is horizontal and, while examining the patient from the rear, on the side of the concavity. This asymmetry is significantly
noting whether one side of the back appears higher than the accentuated when the patient bends forward. Examining
other (Fig. 12-4). This test is the most common noninvasive patients in the forward-bent position is the standard method
clinical method for evaluating scoliosis.7,95,146,525 used to detect mild degrees of curvature in mass screening
programs. In an effort to quantitatively assess the asym-
metry and thus establish an appropriate degree of deformity
that justifies referral for medical evaluation, Bunnell intro-
duced the scoliometer in 1984 (Fig. 12-5).95 This specially
designed inclinometer (similar to a level used in a wood
shop) measures the angle of vertebral rotation. When using
the scoliometer, it is important that the screener stand
behind the patient to view the back (as in the Adams
forward-bending test). The screener’s eyes should be on a
horizontal plane with the maximal deformity of the back.
If the patient bends forward approximately 45 degrees, the
outline of the trunk at the level of the thoracic spine is seen.
With further bending, the outline of the trunk at the level
of the thoracolumbar spine is seen, followed by the outline
at the level of the lumbar spine.
If a rotational deformity of the back is noted at any level,
the scoliometer is placed gently on the person’s back at
the apex of the deformity, perpendicular to the long axis
of the body, and the angle of inclination is read directly from
the scale. Originally, the recommendation for orthopaedic
referral was a 5-degree angle of trunk rotation at any level
of the spine,95 which meant that the chance of missing a
curve exceeding 20 degrees was small. However, because of
excessive referrals, this recommendation has been modified
to a 7-degree angle of trunk rotation.97 With this criterion
the chance of missing a curve greater than 30 degrees (the
FIGURE 12-4  Adams forward-bending test. The patient is viewed curve magnitude at which bracing is usually initiated) is low.
from behind and is asked to bend forward until the spine is When this approach is used, the referral rate is approxi-
horizontal. When scoliosis is present, one side of the back appears mately 3% of persons screened, with a 95% detection rate
higher than the other. of curves requiring brace treatment.

FIGURE 12-5  The scoliometer is a specially designed inclinometer that is used clinically to measure the angle of vertebral rotation. A, In
the lumbar spine the scoliometer is used to assess paravertebral muscle asymmetry. B, In the thoracic spine the scoliometer is used to
assess rib asymmetry.

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CHAPTER 12  Scoliosis 211

in patients with scoliosis.128,460,462,854 Differences in collagen

have been found between normal individuals and those with
The exact cause of idiopathic scoliosis remains unknown AIS; however, this finding is not universal.797 These changes
despite considerable investigation. Although growth has a may be secondary to the mechanical effects of the spinal
significant influence on the deformity, it is not considered deformity rather than reflecting mutations in collagen itself.
a causative factor. Since the 1990s, much of the research This theory has been substantiated by segregation analysis
on the etiology of scoliosis has focused on central neurologic of genetic markers linked to the structural genes encoding
dysfunction, connective tissue abnormalities, and most types I and II collagen.115
recently, genetic factors. These influences have supplanted Other components of connective tissue may be abnor-
previous theories that idiopathic scoliosis was caused by a mal as well. In histologic studies of the ligamentum flavum
biochemical or nutritional deficiency,294,339,849 structural in scoliotic patients, the elastic fiber system was found to
defects,106 or endocrine abnormality.708 The true cause is have disarranged fibers, a marked decrease in fiber density,
probably multifactorial and involves several of the afore- and a nonuniform distribution of fibers throughout the
mentioned factors. ligament.282 These findings suggest that the elastic fiber
system (which is predominantly fibrillin) may play a sig-
Neurologic Dysfunction nificant role in the pathogenesis of idiopathic scoliosis in
The literature in the past supported an underlying neuro- some individuals. Bone mineral density has also been shown
logic abnormality as the primary etiologic factor in idio- to be lower in young adolescents with scoliosis.126,128 It is
pathic scoliosis. Dysfunction of the vestibular, ocular, or uncertain, however, whether this finding is related to the
proprioceptive systems causes an interruption of equilib- primary cause of the disease or whether it is secondary to
rium that is indicative of abnormalities involving the poste- the asymmetric mechanical forces associated with the
rior column of the proximal portion of the spinal cord, back deformities. Further investigation is needed to deter-
brainstem, and cerebral cortex.† Somatosensory evoked mine whether those with osteopenia have the same cause,
potentials are useful parameters for evaluating neurologic pathogenesis, and risk for progression as those without
function. Responses to vibratory stimuli are reportedly osteopenia.
reduced significantly and asymmetric between the left and The paravertebral musculature in patients with scoliosis
right sides in scoliotic patients when compared with con- may exhibit abnormalities in the muscle spindle,459 in indi-
trols.39,565,850 These findings support the concept that an vidual muscle fiber morphology,325,661,876 in histochemis-
aberration in function of the posterior column pathway of try,719 and on electromyography.17,625 Some of these changes
the spinal cord may play a role. Other investigators, however, are more pronounced with severe curves, but they are
have been unable to corroborate this opinion.495 Altered believed to be secondary to muscle adaptation to the curve
balance affecting foot posture and gait, particularly pes and not a primary cause of the deformity.325
cavus, has been reported.114,258 In addition to abnormalities Abnormal platelet structure and function have been
in the sensory pathways, motor dysfunction has been reported in patients with scoliosis.‡ Calmodulin, a calcium-
reported, thus suggesting that the organization of the entire binding receptor protein found in platelets and skeletal
brain is asymmetric in individuals with scoliosis.264 Regional muscle, regulates the contractile protein system (actin and
differences in brain volume have also been reported in myosin). If an underlying systemic contractile disorder is
patients with AIS.443 present, both platelets and skeletal muscle will be affected.
Another neurologically based theory regarding the cause Thus, measurable abnormalities of calmodulin in platelets
of idiopathic scoliosis involved the role of melatonin in regu- are indicative of skeletal muscle abnormalities. Platelet
lating normal spine growth. Secreted by the pineal gland, calmodulin levels in adolescents with progressive scoliosis
this neurohormone controls the circadian rhythm. Experi- are significantly higher than those in normal individuals, in
ments on pinealectomized chickens revealed that melatonin patients with stable curves, and in those whose progressive
deficiency contributes to the development of scoliosis in curves were stabilized by bracing or spinal fusion.3,383,460
this model, probably by interfering with the normal sym- Although this finding cannot be implicated as a direct cause
metric growth of the proprioceptive system involving of scoliosis, it may become a useful predictor of curve pro-
the paraspinal muscles and the spine.36,68,130,335,474-479,865 gression, but controversy remains.462
However, melatonin therapy after pinealectomy in
chickens had no effect on the development or progression Genetic Factors
of scoliosis, thus raising doubts about its role.35,780 Signifi- Because idiopathic scoliosis can be seen in multiple
cantly lower melatonin levels and impaired melatonin sig- members of the same family, attempts have been made to
naling have been reported in patients with scoliosis versus determine the genetic factors involved. Several extensive
controls,477,523 but other investigators have refuted this clinical studies of affected families conducted in the 1960s
finding.3,89,217,311 In addition, studies report no evidence to and 1970s revealed a high prevalence of familial scoliosis
support mutations in the gene coding for human melatonin (6.9% to 11.1% of first-degree relatives).149,644,852 Both
receptor.522,542,700 dominant and multifactorial inheritance patterns have
been suggested to explain the genetic contributions to AIS
Connective Tissue Abnormalities (Fig. 12-6). Not all studies suggested a genetic basis,
Another focus of research is on alterations in connective however; some implicated older maternal age at the time
tissue involving the spine, paraspinal muscles, and platelets of childbirth.167
† ‡
References 102, 105, 127, 232, 255, 297, 480, 538, 565, 765, 814. References 238, 383, 437, 460, 529, 589, 659.

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212 SECTION II  Anatomic Disorders

= Affected with curve ≥15°

= Unaffected
= Deceased
= Male
= Female

FIGURE 12-6  A, Family tree of five

generations demonstrating an apparent
dominant pattern of inheritance. B, Three
other small family trees reflecting probable
multifactorial modes of inheritance. B

More recent literature has shown evidence of a strong the vertebral body is rotated toward the convex side of the
genetic tendency in some families of patients with AIS.§ In lateral curvature, so the spinous processes of the vertebrae
a meta-analysis of scoliosis in twins, monozygous twins had are rotated toward the concavity of the curve. The asym-
a significantly higher rate of concordance than did dizygous metric deformities found within the bodies of scoliotic ver-
twins, and the curves in monozygous twins developed and tebrae differ substantially from the vertebrae in normal
progressed together.372 spines.438,441,582,614,727 Forces during compression and distrac-
Studies are now under way to identify the genes that tion act on the growing spine and produce wedge-shaped
cause scoliosis and its progression.251,279,686,841 In this new changes in the vertebrae, which become higher on the
investigative frontier, genomic DNA from families with convex side and lower on the concave side (Fig. 12-7). The
apparent autosomal dominant inheritance of adolescent sco- vertebral body becomes condensed on the concave side as
liosis is analyzed for linkage with disease. To date, findings a result of the greater pressure, and it is expanded and
have been reported that involve chromosomes 3p26.3 single thinned on the convex side. The concave facets have a sig-
nucleotide polymorphism,686 6,508 8,41,42 9,508 10,840 16,508 nificantly thicker cortex than the convex facets do.688
17,508,663 18q,279 and 19,119 as well as the X chromosome.353 In addition to changes in the frontal and axial planes, the
Familial analysis using this approach may enable investiga- scoliotic portion of the spine is lordotic in the sagittal
tors to track causative genes, such as the CHD7 gene associ- plane.182,183 This three-dimensional deformity is appropri-
ated with the CHARGE syndrome (coloboma of the eye, ately termed torsion of the spine and is greatest at the apical
heart anomaly, choanal atresia, retardation, and genital and region.179,180,195,591-593
ear anomalies) and later-onset idiopathic scoliosis,251 thereby A scoliotic spine has associated changes in the neural
providing more insight into the etiology of idiopathic scolio- canal and posterior arch. Abnormality in the neurocentral
sis. It may also allow the identification of diagnostic markers. synchondrosis may contribute to the scoliotic deformity, as
has been shown experimentally.878,879 With more severe
deformities, the laminae on the convex side are broad and
widely separated, whereas those on the concave side are
The extent of structural changes varies with the degree of narrow and close together (Fig. 12-8). The pedicles are
scoliosis.210,646 These changes are greatest at the apex of the shorter and thinner (narrower endosteal transverse width)
curve and diminish toward each end. In structural scoliosis, on the concave side.441,582 The transverse processes more
closely approach the sagittal plane on the convex side and
References 41, 42, 119, 353, 372, 508, 509, 663, 805. are more in the frontal plane on the concave side. The

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CHAPTER 12  Scoliosis 213

FIGURE 12-9  The intraspinal canal is slightly distorted because

of short, misshapen pedicles. The transverse processes are
asymmetric. (From James JIP: Scoliosis, Baltimore, 1967, Williams &
Wilkins, p 15.)

FIGURE 12-7  Gross anatomic specimen of a spine showing

changes that developed with severe right thoracic scoliosis. The
vertebral bodies became trapezoidal, with the narrower side on intraspinal canal becomes distorted because of the mis-
the concavity. The rotation of the spine is so severe in this shapen pedicles and articular processes (Fig. 12-9).
specimen that the anterior aspect of the apical region is facing   As a result of pressure over time, the intervertebral disks
90 degrees to the right. (From James JIP: Scoliosis, Baltimore, on the concave side narrow and may show degenerative
1967, Williams & Wilkins, p 13.) changes in adulthood. The adjoining portion of the vertebra
becomes sclerotic, with marginal lipping.
In patients with right thoracic idiopathic scoliosis, the
aorta is positioned more laterally and posteriorly relative to
the vertebral body than its position in patients without
spinal deformity.682,735 As demonstrated by magnetic reso-
nance imaging (MRI) studies, this finding is even more
striking with increasing curve severity and apical vertebral
The thoracic cage is also affected by the deformity.
Because of rotation of the thoracic vertebrae, the ribs on
the convex side are directed posteriorly, which produces a
rib prominence that in severe cases may be referred to as a
“razorback.” On the concave side the ribs are rotated
forward, which can potentially produce prominence of the
anterior chest wall. The sternum may be asymmetric
and laterally displaced from the midline. The breasts are
often mildly asymmetric as a result of the chest wall defor-
mity. This breast asymmetry is often a major concern of
Because of the spinal deformity, the thoracic cavity is no
longer symmetric. Its capacity is diminished on the convex
side and increased on the concave side. In severe cases with
marked angulation of the ribs posteriorly, lung function may
be altered.586,782
In severe cases of scoliosis in which the shape of the
FIGURE 12-8  The posterior elements of the spine at the apical intraspinal canal is distorted, the spinal cord may be
region of this severe scoliosis show notable deformity, with the stretched over the concave side, but rarely is any neurologic
laminae on the concave side being narrow and close together. deficit present. Cord compression with neurologic deficit
(From James JIP: Scoliosis, Baltimore, 1967, Williams & Wilkins, usually occurs only with extreme deformities that are
p 15.) accentuated by marked thoracic kyphosis.

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214 SECTION II  Anatomic Disorders

Clinical Features Neurologic deficits are also rare in persons with AIS.
Should an adolescent describe any suspicious symptoms
Initial Signs and Symptoms (e.g., persistent neck pain, frequent headaches, ataxia,
Adolescents with scoliosis do not usually seek medical weakness), meticulous attention must be paid to the neu-
evaluation because of back discomfort but rather because rologic portion of the physical examination. If any neuro-
of some physical aspect of their deformity, such as a high logic deficits are found or if the convexity of the thoracic
shoulder, one-sided prominence of a scapula or breast, curve is to the left, appropriate imaging of the neural axis
elevated or protuberant iliac crest, and asymmetry in is undertaken.120,220,554,616,771 Normally, the convexity of tho-
flank creases and the trunk. Except for being noticed racic curves in AIS is directed to the right. Abnormal left
personally by the adolescent, these findings are often first thoracic curves are more common in those with an underly-
appreciated during school screening programs for scoliosis ing syrinx.571,616,681
or during back-to-school examinations by the family
physician. Physical Examination
Though uncommon, back pain is present in individuals Physical examination of an adolescent with idiopathic sco-
with idiopathic scoliosis more often than was previously liosis should be performed with the patient properly draped.
thought. Nearly 32% of adolescents with idiopathic scoliosis It is convenient if the patient wears a swimsuit. Alterna-
complain of back discomfort at some point (23% at initial tively, the patient may be dressed in underpants and an
evaluation and 9% during the period of observation).616 A examination gown open at the back. The patient’s entire
significant association has been found between back pain back, including the shoulders and iliac crests, must be
and age older than 15 years, Risser grade 2 or greater skel- visible.
etal maturity, postmenarchal status, and a history of injury. The skin is inspected closely for abnormalities such as
Back pain does not seem to be related to the sex of the midline hemangiomas, hair tufts, and dimpling in the lum-
patient, family history of scoliosis, limb length discrepan- bosacral region. Any of these surface findings may indicate
cies, magnitude or type of curve, or spinal alignment. In the presence of an underlying spinal cord abnormality such
patients with back pain, the source of discomfort can be as a tethered cord or diastematomyelia. The spinous pro-
identified only 10% of the time despite the use of appropri- cesses are palpated from the cervical region to the sacrum
ate imaging studies. The most common causes of discomfort for any deficiencies or areas of discomfort. Occasionally,
are associated spondylolysis, spondylolisthesis, and Scheuer- absence of a spinous process is noted, which usually cor-
mann kyphosis. Less likely causes include spinal cord syrinx, responds to spina bifida occulta seen on a spinal radiograph
disk herniation, tethered spinal cord, and tumor. A painful (Fig. 12-10).
left thoracic curve or an abnormal neurologic finding is most With the patient standing, the examiner should deter-
predictive of an underlying pathologic condition of the mine whether the iliac crests are level. If they are not, a
spinal cord. lower limb length discrepancy is likely, which can be quanti-
When an adolescent with presumed idiopathic scoliosis fied by placing measured blocks under the short extremity
has back pain, a careful history should be obtained, a thor- until the iliac crests are level. Leg length discrepancy can
ough physical examination performed, and plain radiographs be responsible for the appearance of scoliosis, and the con-
ordered. If findings on this initial evaluation are normal, a dition must not be overlooked. The back is then examined
diagnosis of idiopathic scoliosis can be made, the scoliosis for asymmetry of the shoulders and flank creases, unequal
can be treated appropriately, and nonsurgical treatment of scapular prominence, prominent iliac crest, and increased
the back pain can be initiated. It is not necessary to perform space between the arm and body on one side with respect
extensive diagnostic studies in every adolescent with scolio- to the other with the arms hanging loosely at the side
sis and back pain. If the patient’s symptoms persist and (Fig. 12-11).
significantly restrict normal activities and if the findings on Although these findings are consistent with scoliosis, the
neurologic examination are normal, a technetium bone scan best noninvasive clinical test for evaluating spinal curvature
may be useful. If the neurologic findings are abnormal, MRI is the Adams forward-bending test (see Fig. 12-4).7,95,146
of the spinal cord is indicated. Unlike backache in adults With this test, the degree and direction of associated rota-
with lumbar scoliosis, backache in adolescents is not usually tion of the vertebrae are clearly demonstrated. The exam-
due to degenerative arthritis in the posterior articulations iner observes the adolescent from behind as the patient
or to nerve root irritation. bends forward at the waist until the spine is horizontal. The
Respiratory symptoms are uncommon in patients with patient’s knees should be straight, the feet together, the
AIS. Studies have shown that clinically significant cardio- arms dependent, and the palms in opposition. Vertebral
pulmonary compromise does not usually occur until the rotation causes one side of the back to appear higher. This
magnitude of the curve approaches 100 degrees, vital is noted as rib prominence in the thoracic region or as para-
capacity becomes less than 45%, or thoracic lordosis signifi- spinal fullness in the lumbar region. Such asymmetry can
cantly narrows the anteroposterior (AP) dimensions of the be quantified with a scoliometer, which can provide mea-
chest.586,588 Most curves are treated operatively before the surements documenting change over time (see Fig. 12-5).95
spinal deformity becomes this severe. However, when Frequently, if the patient is inspected from the front,
the kinematics of the chest cage and spine during breathing asymmetry of the pectoral regions, breasts, or rib cage may
is evaluated, individuals with AIS have decreased motion in be evident. Although these asymmetries are probably
comparison to healthy individuals.390,392,429 In patients with related to the spinal curvature, they may also occur in indi-
impaired pulmonary function, this stiffness plays a signifi- viduals without scoliosis. Occasionally, breast asymmetry is
cant role. the primary concern of the patient and parents. Families

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CHAPTER 12  Scoliosis 215

FIGURE 12-11  Clinical appearance of a 13-year-old girl with right

thoracic and left lumbar scoliosis. The right scapula is prominent,
and the space between the left arm and body is increased. The
shoulders are level.
FIGURE 12-10  Posteroanterior radiograph of the spine of a
12-year-old girl. The spinous processes of T11 and T12 were not assessing the AP aspect of the spinal curvature when viewing
detectable on palpation. The radiograph shows spina bifida
the spine from the side. The resulting clinical appearance is
occulta at the same levels (arrows).
an apparently increased kyphosis in the sagittal plane
because in reality, the scoliotic deformity is being viewed
should be informed that correcting the scoliosis may have from the side (see Fig. 12-7).
little, if any, influence on this asymmetry. Sagittal plane deformity in an individual with apparent
Spinal balance is assessed by two different methods. The idiopathic scoliosis may also be an indicator of syringomy-
first method, known as coronal balance, is to determine the elia.571,636 If hypokyphosis is absent clinically and radio-
alignment of the head over the pelvis. The head is almost graphically and if little or no rotation (rib prominence) is
always positioned directly above the gluteal crease in present, a diagnosis of idiopathic scoliosis should be made
patients with idiopathic scoliosis. To assess this balance, a only after a syrinx has been ruled out.
plumb line is held from the base of the skull or from the
spinous process of C7. Normally, the plumb line should not Neurologic Examination
deviate from the center of the gluteal crease by more than Because idiopathic scoliosis is basically a diagnosis of exclu-
1 to 2 cm (Fig. 12-12). If it does, this finding should be sion, a thorough evaluation is necessary to rule out a neu-
considered atypical, and a meticulous neurologic examina- rologic cause of the deformity. The neurologic examination
tion is necessary to rule out coexisting neurologic pathology. begins by assessing the patient’s reflexes. Examination of
The second method, known as trunk balance, is to assess the superficial abdominal reflexes is useful for determining
the position of the trunk over the pelvis.43,239,628 Unlike the which patients should undergo MRI to rule out syringomy-
position of the head over the pelvis, patients with idiopathic elia (Fig. 12-14).336,864,870
scoliosis may have significant imbalance of the trunk over The abdominal reflex examination is performed with the
the pelvis (Fig. 12-13), particularly with single thoracic patient supine on an examination table and the arms relaxed
curve patterns. along the side of the body. An area approximately 10 cm
Next, the examiner inspects the patient from the side above and below the umbilicus and to each anterior axillary
and observes the sagittal contours of the spine. Normally, line is exposed. With the patient relaxed, the bluntly
in individuals with idiopathic scoliosis the sagittal plane pointed handle of a reflex hammer is used to lightly stroke
appears hypokyphotic throughout the scoliotic segment, the skin in each quadrant over a distance of 10 cm (Fig.
with actual lordosis being present radiographically in the 12-15). The stroke starts lateral to the umbilicus near the
apex of the deformity. In more severe cases the entire sagit- anterior axillary line and is directed diagonally toward the
tal plane may actually be lordotic and lead to a very narrow umbilicus in each quadrant. The umbilicus is observed for
AP diameter of the thoracic cage. Rarely, as much as 90 deviation toward the side on which the test is performed.
degrees of rotation may be present in the apical vertebrae If these reflexes are consistently present on one side and
within the curve. In this instance the examiner is actually absent on the other side, further evaluation is warranted

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216 SECTION II  Anatomic Disorders

FIGURE 12-12  Coronal balance. A plumb line held at the spinous process of C7 should not deviate from the center of the gluteal fold
(center sacral line) by more than 1 to 2 cm.

FIGURE 12-13  Trunk balance. To measure trunk balance, two vertical lines are drawn on a radiograph: the first vertical line is the center
sacral line, and the second vertical line bisects a horizontal line drawn from the peripheral edges of the ribs of the apical vertebra. The
distance between the two vertical lines quantifies the amount of trunk imbalance.

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CHAPTER 12  Scoliosis 217

pubic hair development in boys. Although the Tanner

system may provide an indication of the patient’s physical
maturity, more practical clinical emphasis is placed on the
patient’s menarchal status and increase in height over time
and on assessment of skeletal indicators of maturity (e.g.,
Risser sign, open or closed triradiate cartilage, and the
Tanner-Whitehouse III score, which is based on the radio-
graphic appearance of the epiphyses of the distal ends of
the radius and ulna, the small bones of the hand, and the
digital skeletal maturity scoring system).

Radiographic Findings
Plain Radiography
FIGURE 12-14  Diagnostic imaging of the spinal cord and canal is If older conventional radiology equipment is used, the
necessary in children with abnormal neurologic findings. Magnetic initial examination of the spine should include posteroan-
resonance imaging is the optimal study for assessing the neural terior (PA) and lateral radiographs on 36 × 14-inch film
axis. A large cervical syringomyelia (arrow) is evident on this cassettes. With these long cassettes, nearly all the impor-
magnetic resonance image of the head and neck. (From Richards tant radiographic features can be assessed on a single film.
BS: Back pain in childhood and adolescence: the clinical
On the PA projection, such features include the curve
assessment, J Musculoskelet Med 15:39, 1998.)
pattern in its entirety, the type of scoliosis (congenital or
idiopathic), the overall balance of the spine and trunk,
skeletal maturity (as determined by the Risser sign, triradi-
ate cartilage, or capital femoral physis), and the presence
of a lower limb length discrepancy (pelvic tilt). The lateral
projection is useful initially to evaluate the global sagittal
contour of the thoracic and lumbar spine, determine the
presence and severity of thoracic hypokyphosis, and screen
for spondylolysis and spondylolisthesis. In very young chil-
dren, 17 × 14-inch film cassettes may be large enough to
provide all this information; however, these shorter cas-
settes are too small to be used for adolescent patients.
With female patients 10 years or older, the radiology tech-
nologist should always inquire about the patient’s last
menstrual period and the possibility of pregnancy; if preg-
nancy is suspected, the radiographic evaluation can be
FIGURE 12-15  The abdominal reflex examination is performed postponed.
with the patient supine. The bluntly pointed handle of a reflex Today, with the more common use of computed radiog-
hammer is used to lightly stroke the skin in each quadrant over   raphy and the picture archive and communication system
a distance of 10 cm. Asymmetry of the reflex between sides is (PACS), further technologic development in scoliosis
abnormal. imaging with less ionizing radiation has been achieved.
Older conventional film images can be digitized into a PACS
for monitor viewing. With computed radiography, an imaging
because this finding does not occur in neurologically normal plate inside a cassette is exposed and subsequently pro-
patients with scoliosis. However, other variations might cessed by a reader that converts the image into a digital
occur, such as absent reflexes in all quadrants. format to be viewed and stored in a PACS. A series of up
The patellar and Achilles tendon reflexes should also be to four plates can be placed in a long-length cassette holder
tested, with the expectation that they will be symmet- and exposed simultaneously to acquire a long scoliosis image
ric.336,864 Muscle testing and examination of the range of in both the PA and lateral projections. Special software
motion of all four extremities should always be conducted. electronically stitches the images together for monitor
The hands and feet should be examined for abnormal display or, if needed, printing. Newer imaging opportunities
posture and for evidence of abnormal sensation (excessive are available with the use of a recently introduced low-dose
callus formation or nail bed irregularities). Abnormal find- radiation digital stereoradiography system known as the
ings may be the only clinical evidence of underlying pathol- EOS system (EOS Imaging, Paris).261,497 At this point this
ogy of the neural axis, such as syringomyelia or tethered system is found primarily in academic institutions, but its
cord. use will probably expand over time.
After the initial radiographic evaluation has been accom-
Patient Maturity plished, effort is made to limit the number of follow-up
Sexual maturity can be assessed during the physical exami- films, thereby reducing the amount of radiation exposure.
nation according to the Tanner system,757 which assesses During the course of routine follow-up examinations, only
breast and pubic hair development in girls and genital and the PA projection is needed. No set interval from one

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218 SECTION II  Anatomic Disorders

FIGURE 12-16  During radiographic evaluation the patient stands erect with the knees straight and the feet together. The posteroanterior
projection (A) reduces exposure to breast tissue. During the lateral view (B), the arms are held forward to allow clear visualization of the

radiographic examination to the next has been determined Measurement of Curve Magnitude
for all patients. The period between evaluations depends The Cobb method is considered the standard for measuring
on the maturity of the patient and the size of the spinal curve size.31 The measurement is started by determining the
curvature. For example, a premenarchal, Risser grade 0, end vertebrae (top and bottom of the curve). The cephalic
11-year-old girl with a 25-degree thoracic curve should end vertebra’s superior surface and the caudal end verte-
return for radiographic reevaluation after a 4-month inter- bra’s inferior surface have the greatest amount of tilt into
val, whereas a 2-year postmenarchal, Risser grade 4, 14-year- the curve (Fig. 12-17). The intervertebral space on the
old girl with a 30-degree curve need not return for concave side of the curve is generally wider above the
reevaluation before 1 year. In most cases the interval cephalic (top) vertebra and narrower below it. The opposite
between radiographic evaluations ranges from 4 to 6 months. applies to the inferior surface of the caudal (bottom) ver-
During radiographic evaluation the patient should stand tebra. Using a transparent plastic goniometer, the examiner
as erect as possible with the knees straight and the feet draws lines perpendicular to the top vertebra’s superior
together (Fig. 12-16). The patient should be barefoot so surface and the bottom vertebra’s inferior surface. The
that if lower limb length inequality is suspected, the appro- angle formed by the intersection of these lines is the Cobb
priate lift can be placed under the short limb. Unsupported angle. If a second curve is present below the primary curve,
sitting views are taken if the patient is unable to stand. the original curve’s bottom vertebra becomes the cephalic
Twisting of the trunk should be avoided. To ensure sufficient end vertebra for the second curve, and the same line along
cephalic visualization, the upper limit of the cassette should its inferior surface is used. With the use of PACSs in radiol-
extend to the external auditory meatus. In the upright ogy departments today, electronic angular measurements
lateral projection, the patient’s shoulders are flexed forward, simplify this process.
the elbows are fully flexed, and the fists should rest on the Although the Cobb method has good overall reliability,396
clavicles. For the lateral radiograph, this position allows some variation among different observers’ measurements is
the best representation of the patient’s functional sagittal always present. Such variability averages 7.2 degrees if the
balance while still providing adequate lateral radiographic end vertebrae are not preselected but improves to 6.3
visualization of the spine.222,322 Although AP radiographs of degrees when they are preselected.526 Another aspect of the
the hand and wrist are used today to determine skeletal age, accuracy of the Cobb method is that to achieve 95% statisti-
maturity continues to be more commonly assessed with the cal confidence that a true change in curve size has occurred,
Risser sign of the iliac crest. a measurement difference of 10 degrees between radio-
Bending radiographs (including the fulcrum bend test) graphs taken at different times would be needed.113 This
and traction radiographs obtained with the patient supine finding is of particular interest because many studies use a
are usually reserved for preoperative evaluation of spinal criterion of a 5- to 6-degree change in curve size to deter-
flexibility.131,290,466 The information gained from these AP mine the success or failure of brace treatment for scoliosis.
radiographs can be helpful in determining appropriate This information reinforces the importance of meticulous
fusion levels. line drawings and precise measurements.

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CHAPTER 12  Scoliosis 219



FIGURE 12-17  Cobb angle measurement. The vertebrae with the

greatest amount of tilt are selected as the end vertebrae. Lines are
drawn perpendicular to the end-plates of the vertebrae. The angle
formed at the intersection of these lines is the Cobb angle. If a
second curve is present below the primary curve, the original FIGURE 12-18  Left, Perdriolle torsionometer—a clear template.
curve’s lower vertebra becomes the top vertebra when measuring Right, The torsionometer’s outer margins are aligned over the
the second curve, and the same line along its surface is used. vertebra’s lateral borders. The line intersecting the center of the
pedicle shadow (convex side) estimates the amount of spinal
rotation. (From Richards BS: Measurement error in assessment of
vertebral rotation using the Perdriolle torsionometer, Spine 17:514,
Measurement of Vertebral Rotation 1992.)
The Perdriolle method and the Nash-Moe method are the
two most common means of assessing vertebral rotation on
a plain frontal radiograph. The Perdriolle method uses a
transparent torsionometer that is overlaid on the radiograph Measurement of Spinal Balance
(Fig. 12-18).590 The edges of the curve’s apical vertebra and Measurements of spinal balance are important to assess
its rotated pedicle constitute the landmarks. This method the amount of decompensation that exists preoperatively
is accurate for measuring rotations that are less than 30 or that can occur postoperatively. Coronal balance repre-
degrees.568 However, once the scoliotic spine has undergone sents the horizontal distance between the midpoint of C7
instrumentation, the landmarks of the apical vertebra may and the center of the pelvis (Fig. 12-19). Coronal balance
become obstructed by the shadows of the rods or hooks, is considered poor, or decompensated, if this distance
thus making accurate measurements difficult to obtain.629 exceeds 2  cm.203,611 Trunk balance assesses the position of
In the Nash-Moe method, the relationship of the pedicle the thorax over the pelvis and is measured via lateral trunk
to the center of the vertebral body is observed on AP radio- shift. Lateral trunk shift is measured by drawing a hori-
graphs, and the rotation is divided into five grades: grade 0 zontal line to the edges of the ribs of the trunk and a
when both pedicles are symmetric, grade I when the convex perpendicular line that bisects this horizontal line; the dis-
pedicle has moved away from the side of the vertebral body, tance between this perpendicular line and the center of
grade III when the convex pedicle is in the center of the the pelvis represents lateral trunk shift (Fig. 12-20).
vertebral body, grade II when the rotation is between grades Another parameter used to indirectly assess trunk balance
I and III, and grade IV when the convex pedicle has moved is thoracic apical vertebral translation,203,419 which is the
past the midline.537 This method has only fair reliability.396 distance measured between the midpoint of the apical
Computed tomography (CT) can also be used to assess thoracic vertebra and the C7 plumb line. Because the mid-
vertebral rotation.154,321,848 Although CT is more expensive, point of C7 rarely corresponds to the exact center of the
it is more accurate than the Nash-Moe method. For example, pelvis, thoracic apical vertebral translation must be used
Nash-Moe grade 0 vertebrae have been found to have up to in combination with the coronal balance measurement to
11 degrees of rotation when measured with CT.314 reflect trunk balance.635

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220 SECTION II  Anatomic Disorders

the two perpendicular lines represents the degree of tho-

racic kyphosis. Normal thoracic kyphosis ranges from 20 to
45 degrees. No kyphosis or lordosis is present at the thora-
columbar junction (between T11 and L1-2).60 Lumbar lor-
dosis usually begins at L1-2 and gradually increases caudally
to the sacrum. To measure lumbar lordosis, the lower end
vertebra for the thoracic curve becomes the upper end
vertebra. The lower end vertebra for the measurement of
lumbar lordosis is usually L5 or S1. Although attempts have
been made to determine normal lumbar lordosis, no con-
sensus has been achieved; reported normal values range
from 50 to 65 degrees.129,775 Thoracic and lumbar regional
alignment is very similar between adolescents and adults.795
Effort has been made to standardize levels of sagittal plane
measurement to achieve more consistency between
studies.396 Recommendations for sagittal plane measure-
ments include T2-5, T5-12, T2-12, T10-L2, and T12-S1.
All but the T2-5 measurement have shown interobserver
Surface Imaging
FIGURE 12-19  Coronal balance. A plumb line held at the spinous In an effort to decrease the amount of radiation exposure
process of C7 (x) should not deviate from the center of the gluteal during the course of scoliosis management, techniques have
fold (center sacral line) by more than 1 to 2 cm. been developed to assess changes in body surface in patients
with scoliosis. The goals of surface imaging are to appropri-
ately identify scoliosis, monitor curve progression, and
provide information that can be used to make treatment
decisions. However, natural history data and, in most cases,
treatment decisions are based on Cobb angle measurements
made from upright radiographs.
For surface-imaging systems to be useful, they must
demonstrate some consistency with Cobb angle measure-
ments. Moire topography, the Quantec spinal image system
(raster-stereophotography), and the Integrated Shape
Imaging System (ISIS) are three sophisticated techniques
that use computer analysis of digitized topographic informa-
tion (Fig. 12-21). The presence, level, and side of the sco-
liosis curvature have been documented by these topographic
techniques in patients with standard rotation; however, it is
not possible to determine the magnitude of the scoliosis
with sufficient accuracy to be clinically useful.‖ These tech-
niques continue to be investigated in an effort to determine
their role in the management of scoliosis.684
Magnetic Resonance Imaging
By providing a clear anatomic picture of abnormalities that
occur within the spinal canal, MRI can be an extremely
FIGURE 12-20  To measure trunk balance, two vertical lines are valuable tool in the assessment of scoliosis. Syringomyelia,
drawn on a radiograph (arrows). The first vertical line (pink) is Arnold-Chiari malformations, abnormalities in the brain-
the center sacral line. The second vertical line (green) bisects a stem, hydromyelia, spinal cord tumors, spinal cord tether-
horizontal line drawn from the peripheral edges of the ribs of the ing, and diastematomyelia have all been identified in
apical vertebra. The distance between the two vertical lines individuals previously thought to have idiopathic scoliosis.¶
quantifies the amount of trunk imbalance. However, because these abnormalities are rare, performing
MRI as part of routine screening programs is impractical
and cost prohibitive.188 MRI is usually reserved for patients
Measurement of Kyphosis and Lordosis with an atypical manifestation of idiopathic scoliosis.521
on Lateral Radiographs Although atypical manifestations have never been specifi-
The end vertebrae are the last vertebrae that are maximally cally defined, they generally include patients with neck pain
tilted into the concavity of the curve. In the thoracic area, and headache (particularly with exertion) and abnormal
the upper end vertebra is usually T3 or T4, and the lower
end vertebra is T12. Perpendicular lines are drawn to the ‖
References 161, 444, 471, 662, 728, 773, 843.
inferior and superior end-plates. The angle formed between ¶
References 175, 255, 336, 434, 574, 636, 681, 689, 833.

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CHAPTER 12  Scoliosis 221

Table 12-2  Guidelines for Treating Patients With Idiopathic Scoliosis

Risser Sign
Curve Magnitude
(Degrees) Grade 0/Premenarchal Grade 1 or 2 Grade 3, 4, or 5

<25 Observation Observation Observation

30-40 Brace therapy (begin when Brace therapy Observation
the curve is >25 degrees)
>45 Surgery Surgery Surgery (when the curve is
>50 degrees)

spine’s anatomy.733 It also remains a useful tool postopera-

tively (particularly with three-dimensional reconstruction)
for assessing bone fusion mass if pseudarthrosis is suspected,
for evaluating changes in spinal rotation, and for verifying
pedicle screw placement.# In addition, CT-myelography
affords improved evaluation of the spinal cord when retained
metal implants limit the effectiveness of MRI.

Most adolescents with idiopathic scoliosis do not require
treatment because of the low probability that their curves
will progress.16,455 Treatment is warranted only for patients
whose scoliotic curves are at substantial risk of worsening
over time or for those with severe curves at initial evalua-
tion. A clear understanding of the risk factors discussed
earlier in the natural history section is useful in determining
which patients need treatment, regardless of whether they
are skeletally immature or mature.
In selecting treatment, the physician must consider the
adolescent’s remaining growth potential, the severity of the
curve at the time of detection, and the pattern and location
of the scoliosis. The cosmetic appearance and social factors
that may have an impact on treatment also enter into the
decision-making process. The treatment choices available
FIGURE 12-21  Moire topographic photograph. This surface- are observation, nonsurgical intervention, and surgical inter-
imaging system produces an image that can be read in the same vention, and it is imperative that physicians know which
way as contour lines on a map. (From Stokes IA, Moreland MS: options are appropriate for each individual patient (Table
Concordance of back surface asymmetry and spine shape in 12-2 provides general guidelines). Actively growing adoles-
idiopathic scoliosis, Spine 14:73, 1989.) cents (Risser grade 2 or lower) with curves between 30 and
45 degrees should start brace therapy at the time of the
initial visit.653 In very immature patients (Risser grade 0 and
neurologic findings such as ataxia, weakness, and progressive premenarchal if female) with curves exceeding 25 degrees,
foot deformities; patients with unusually rapid curve pro- bracing should be started immediately.456,653 In most cases,
gression or excessive thoracic kyphosis; or patients requiring growing adolescents with curves exceeding 45 to 50 degrees
surgery who have left thoracic curves or asymmetric abdom- require operative stabilization because other forms of treat-
inal reflexes. Curves greater than 70 degrees do not increase ment are ineffective in controlling or correcting the scolio-
the likelihood of finding a spinal cord anomaly.556 Routine sis. Skeletally mature individuals with curves exceeding 50
preoperative MRI is not indicated for typical AIS if findings to 55 degrees are also at risk for continued curve progression
on the neurologic examination are normal.175,556,689,833 and should be considered for surgical treatment.811 Possible
exceptions include patients with well-balanced double
Computed Tomography curves less than 60 degrees whose clinical appearance is
Although CT may clearly demonstrate congenital abnor- acceptable to them. Continued observation would be neces-
malities in the spine, it is rarely needed in the routine sary to document progression of the scoliosis, which would
assessment of individuals with idiopathic scoliosis. However, necessitate surgery.
with the emerging use of vertebral column resection (VCR)
in those with extremely severe AIS, preoperative three-
dimensional CT imaging is indicated to clarify the deformed #
References 154, 225, 287, 407, 418, 440, 619, 847, 848.

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222 SECTION II  Anatomic Disorders

advocated, such as the suspensory plaster cast of Sayre and

Observation the hinge or turnbuckle cast of Hibbs and Risser. In 1946
In general, no treatment is needed for curves less than 25 the Milwaukee brace was developed to replace postopera-
degrees, regardless of the patient’s maturity. Follow-up tive plaster immobilization. Later, the brace was used as a
examinations are necessary, with the interval between visits nonoperative method of treatment when passive, active,
depending on the patient’s maturity and the size of the and distraction forces were thought to be necessary to
curve. For example, a premenarchal Risser grade 0 adoles- prevent curve progression. Subsequent studies have shown
cent with an initial curve measuring 24 degrees should that the corrective forces of a brace are passive and that the
undergo follow-up examinations every 3 to 4 months, and predominant corrective component is transverse loading of
a brace may be needed if the curve progresses. For more the spine through the use of corrective pads.24,121,580,851 In
skeletally mature patients (Risser grade 3 or higher), longer the 1960s, thermoplastics were introduced into orthosis
intervals between visits (e.g., 6 months) are appropriate manufacturing, which led to the thoracolumbosacral ortho-
because curve progression usually occurs at a slower rate, if ses (TLSOs) of today. In recent years, computer-assisted
at all. Clearly, predetermined guidelines do not apply to all design and computer-assisted manufacturing have been
cases, and follow-up must be individualized. used to fashion spinal orthoses.157,205
The magnitude of the patient’s curve at initial evaluation
helps determine the frequency of follow-up visits. In Indications for Brace Treatment. Brace treatment is
general, for growing children with small curves (<20 restricted to immature children in an attempt to prevent
degrees), the next follow-up evaluation should be approxi- curve progression during further skeletal growth. In general,
mately 6 months later. If the curve is between 20 and 30 bracing is indicated in growing adolescents (Risser grade 0,
degrees, radiographs should be obtained 3 to 4 months later 1, or 2) who on initial evaluation have curves in the range
because treatment may be necessary if the curve progresses of 30 to 45 degrees or who have documented progression
5 degrees or more. For patients whose curves do not pro­ exceeding 5 degrees in curves that initially measured 20 to
gress, observation continues and the interval between visits 30 degrees. Those who are Risser grade 0 should be consid-
gradually lengthens as maturity approaches. ered candidates for bracing when their curves reach 25
What constitutes true curve progression is a matter of degrees. Patients should consider their existing deformities
some debate. Traditionally, an increase in curve size of more cosmetically acceptable and must be willing to wear the
than 5 to 6 degrees has been taken as representing progres- brace the prescribed amount of time. Low-profile braces
sion; however, a 7- to 10-degree change in measurement is (TLSOs) are the most commonly used orthoses today, but
more accurate if a 95% confidence level is used to deter- their use is restricted to patients whose curve apex is at T7
mine true progression.113,526 This should be taken into con- or below. Fortunately, this is the case with most curve pat-
sideration when deciding whether the measured change in terns in adolescents with idiopathic scoliosis. In 2005 the
the patient’s scoliosis warrants either nonsurgical or surgical Bracing Committee of the SRS made recommendations
intervention. Nevertheless, throughout the literature a 5- to concerning inclusion criteria for future brace studies involv-
6-degree measured change is considered indicative of curve ing AIS.632 Optimal inclusion criteria are age 10 years and
progression. Not all progressive curves exceeding 30 degrees older when the orthosis is prescribed, Risser grade 0 to 2,
require treatment; the decision depends on the adolescent’s primary curve magnitude of 25 to 40 degrees, no previous
maturity and the size of the curve. treatment, and if female, either premenarchal or less than
1 year postmenarchal.
Nonsurgical Treatment
To be considered effective, nonsurgical treatment must Contraindications to Brace Treatment. Brace treatment has
prevent curve progression in those who are most at risk several contraindications. First, most studies concur that
(curves of 25 to 45 degrees in patients with Risser grade 0 large curves (>45 degrees) in a growing adolescent cannot
or 1), be of benefit in all curve patterns, result in an accept- be effectively controlled by a brace and that these patients
able cosmetic appearance at the end of treatment, and need surgical treatment. Even if progression could be con-
reduce the need for surgery. In other words, nonsurgical trolled with a brace, the cosmetic appearance associated
treatment must improve the patient’s outcome with respect with these large curves is often unacceptable because of
to the expected natural history. Over the years a great deal excessive trunk shift and rib prominence. An exception to
of experience has been gained with various forms of non- this general rule involves very immature adolescents with
surgical treatment, some of which orthopaedists consider large curves (approximately 50 degrees) who have not yet
effective (e.g., bracing) and others that have shown no reached their PHV. These patients may benefit from bracing
beneficial effect (e.g., electrical stimulation). In Europe, the to delay curve progression until greater maturity is reached;
use of physical therapy exercises and biofeedback to enhance this may avoid the need for additional anterior spinal fusion
the effectiveness of bracing (Schroth technique and others) to prevent the crankshaft phenomenon.
is common.540,541,710,871 Although its use in North America In addition, bracing is not indicated for patients who find
has not yet become established, this treatment method is wearing an orthosis to be emotionally intolerable, although
being studied in various centers. appropriate psychological counseling may result in eventual
acceptance of a brace by an adolescent. Extreme thoracic
Orthotic (Brace) Treatment hypokyphosis precludes the use of an orthosis. In these
Historically, Pare is credited with being the first to use metal cases, normal positioning of the pads within the brace could
braces, in the form of armor, to treat patients with scoliosis. exacerbate the rib deformity. If the hypokyphosis is 20
Since then, various types of braces and casts have been degrees or less, corrective pads should be lateralized to

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CHAPTER 12  Scoliosis 223

eliminate any anteriorly directed derotation forces. Finally, patient’s pelvis. The suprastructure consists of one anterior
skeletally mature adolescents (Risser grade 4 or 5 and, if and two widely separated posterior uprights, plus a cervical
female, 2 years postmenarchal) should not be treated with ring with a throat mold and occipital piece. In many cases
braces. a low-profile, over-the-shoulder structure may be used in
Relative contraindications to bracing include a high tho- place of the more standard neck-ring design. Today, because
racic or cervicothoracic curve, which ordinarily does not of the strong emphasis on self-image, use of the Milwaukee
respond to orthotic treatment, and male sex. A relative lack brace has decreased greatly, and it has largely been replaced
of effectiveness of bracing in boys has been documented, in by equally effective but lower-profile braces. Low-profile
part because of extremely poor compliance.359,867 TLSOs, such as the Boston, Wilmington, and Miami
braces,494 can often be hidden under loose shirts or
Comparison of Orthoses. Numerous reports in the litera- sweaters, thus providing adolescents a more acceptable
ture attest to the effectiveness of brace treatment.*a In alternative.
most of these studies, bracing was considered effective if Boston Brace. The Boston brace was introduced in 1971
the curve remained within 5 to 6 degrees of its original by Hall and associates.286 Its design consists of a prefabri-
magnitude on completion of treatment. Some of these cated, symmetric thoracolumbar-pelvic module with built-in
studies included low-risk patients (Risser grades 3 to 5, lumbar flexion and areas of relief opposite areas of pressure
curves <20 degrees), patients still undergoing treatment, (Fig. 12-22). Braces are individually constructed by an
patients who had previously undergone treatment, and chil- orthotist by using a blueprint created from the patient’s
dren younger than 10 years. In some studies, patients were full-length radiograph. This brace is the most commonly
eliminated from the study population because of noncom- used TLSO today and is effective in controlling curve pro-
pliance. All these factors make comparisons among studies gression, including larger curves measuring 35 to 45
difficult, particularly when one is trying to assess the effec- degrees.§a It has been reported that the Boston brace exerts
tiveness of bracing in patients most at risk (Risser grade 0 derotational force on the scoliosis; however, a long-term
or 1, premenarchal girls, 25- to 45-degree curves). Some of study found no lasting improvement in derotation of the
the more recent literature has been more consistent in spine.818 The brace is effective in treating either single- or
focusing on the population at greatest risk.†a These studies, double-curve patterns in which the apex of the most
along with a 1997 meta-analysis of the bracing literature,653 cephalic curve is located at T7 or below.
strongly reinforce the idea that bracing is effective in con- Wilmington Brace. The Wilmington brace was described
trolling curve progression. Perhaps the most compelling in 1980.98 It is custom-made from a positive mold of the
evidence for this at present comes from a study by Katz and patient’s torso in which the scoliosis is maximally corrected
colleagues.363 In this study, braced patients had compliance in a Risser- or Cotrel-type cast. Indications for the
objectively verified through the use of a heat sensor in the Wilmington brace are the same as those for the Boston
orthosis. The total number of hours of brace wear correlated brace. This brace has not enjoyed the popularity of the
with curve progression. This effect was most significant in Boston brace, although it continues to be used by several
patients who were at Risser stage 0 or 1 at the beginning of institutions.18,249
treatment. Curves did not progress in 82% of patients who Charleston Brace. Development of the Charleston brace
wore the brace more than 12 hr/day, as opposed to only was based on the concept that part-time use may be effec-
31% of those who wore the brace less than 7 hr/day. tive.226,609,610 This brace holds the patient in maximal side-
The perception of brace effectiveness is not universal, bending correction and is worn only at night for 8 to 10
however, in that some studies question whether orthotic hours. The side-bending force exerted by the brace does not
management provides any benefit in those with scoliosis.‡a allow its use in the upright position, thus making wear fea-
Numerous orthoses are available. Most are named after sible only when the patient is recumbent. The main appeal
their place of origin, such as the Milwaukee brace,456 the of this brace is the limited number of hours of daily wear,
Boston brace,208,286 the Wilmington brace,18,45 the Charles- all of which are accomplished during sleep.
ton brace,610 and the Providence brace.157 All these braces Despite several studies in which the Charleston brace
are effective in preventing curve progression. Before decid- was found to be as effective as the Milwaukee and Boston
ing which brace to use, the orthopaedist should be familiar braces,226,257,609,610,774 some skeptics doubt that such a limited
with the advantages and disadvantages of each. amount of time in a brace can successfully control curve
Milwaukee Brace. The Milwaukee brace, introduced by progression. A comparison of the Boston brace and the
Blount, Schmidt, and Bidwell in 1946, was the original Charleston brace found that in patients with curves between
modern design. The device consisted of three main compo- 36 and 45 degrees, 83% of those treated with a Charleston
nents: a pelvic girdle, a suprastructure, and lateral pads. brace experienced curve progression of more than 5 degrees
Over time, some design modifications have been made, and as compared with 43% of those treated with a Boston
in the current model the pelvic girdle is made of thermo- brace.364 The authors concluded that the Charleston brace
plastic material and is created from a positive mold of the should be reserved for single lumbar or thoracolumbar
curves less than 35 degrees.
Providence Brace. The Providence brace, like the Charles-
*aReferences 18, 24, 43, 44, 121, 139-141, 157, 159, 202, 208, 233, ton brace, is used only at night (Fig. 12-23). Made with
235, 249, 250, 257, 272, 273, 324, 340, 362-364, 368, 400, 404, computer-assisted design and manufacturing technology, it
456, 516, 534, 553, 566, 609, 610, 653, 676, 774, 787, 816, 827,
References 18, 157, 362, 364, 456, 534, 610, 774, 816.
‡a §a
References 181, 192, 193, 265, 268, 553, 724. References 208, 362, 364, 404, 516, 566, 816, 818.

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224 SECTION II  Anatomic Disorders

FIGURE 12-22  A, Scoliosis deformity

before brace application. B, Posterior view
of the Boston brace. C, Correction of A B C
scoliosis with the patient in the brace.

FIGURE 12-23  A and B, Patient

with thoracolumbar scoliosis A B
treated with a Providence brace.

is reported to be effective.157,340 We now use this brace in distress, and poor compliance with brace wear was
place of the Charleston brace for the treatment of thoraco- common.183 As a result, the idea of part-time brace use
lumbar or lumbar curves. evolved, with the goal of approximately 16 hr/day of
Spine-Cor Brace. This is a dynamic flexible brace. Several bracing. Although several studies have reported that part-
studies have reported its effectiveness for AIS.139,141,250 time use of orthoses appears to be as effective as full-time
However, a failure rate significantly higher than that of a wear in controlling curve progression,9,121,165 other reports
rigid spinal orthosis has been reported.845 emphasize that the outcome is better when more hours per
day are spent in the brace.362,816 This was confirmed by Katz
Brace Treatment Protocols. The number of hours per day and colleagues in a 2010 study that objectively assessed
that the brace needs to be worn remains uncertain. Origi- brace compliance by using heat sensors in the orthosis.363
nally, 20 to 22 hr/day was advocated for the Milwaukee In an effort to form a consensus from the literature on
brace in immature adolescents with progressive curves; the the effectiveness of bracing (including whether part-time
same recommendation applied to the lower-profile TLSOs. bracing controls curve progression as effectively as full-
Understandably, this caused adolescents some emotional time bracing does), the Prevalence and Natural History

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CHAPTER 12  Scoliosis 225

FIGURE 12-24  Radiographic findings with brace wear. A, Initially, this premenarchal girl aged 12 years 7 months had a 30-degree
thoracic curve and was Risser grade 0. B, Treatment in a Boston brace was begun, with in-brace correction to 18 degrees. C, Brace wear
was continued until the patient was 2 years postmenarchal and Risser grade 4. D, Thirty months later, the curve remained stable at 26

Committee of the SRS conducted a meta-analysis on more effect on both the curve and spinal balance.450 However,
than 1900 patients from 20 studies.653 It concluded that curve progression may be missed if the patient undergoes
bracing (with TLSOs or the Milwaukee brace) is effective imaging while wearing the brace.
in controlling curve progression in individuals with idio- With female patients, if the brace has been successful in
pathic scoliosis and that full-time bracing (23 hr/day) is controlling curve progression, plans can be made to discon-
more effective than part-time bracing (8 to 16 hr/day). The tinue treatment when the girl is approximately 18 to 24
latter finding is supported by more recent studies.362,816 months postmenarchal and Risser grade 4 and when no
When brace treatment is chosen for a patient, certain further increase in her height has occurred (Fig. 12-24).
general guidelines should be followed. Once the brace has Rather than tapering use of the brace, we discontinue it
been constructed and fitted to the patient by the orthotist, completely at that time. In male patients, curves exceeding
the patient should work up to the prescribed number of 25 degrees have a tendency to progress even when Risser
hours of wear per day. After 2 to 4 weeks the adolescent grade 4 maturity has been reached.360 Therefore, in boys,
should return to the orthopaedist’s office for an initial brace bracing may need to be continued until Risser grade 5 is
evaluation. At that time, any problems (e.g., intolerable achieved. Frequently, this does not occur until the later
pressure points) will have been identified by the patient and teenage years, which makes compliance with brace wear a
can be addressed by the orthotist. Equally important, an challenge.
in-brace radiograph should be obtained to verify the amount
of curve correction being achieved. With the Boston brace, Electrical Stimulation
a minimum of 40% to 50% curve correction should be Electrical stimulation was used as an alternative to bracing
obtained in the brace.364 With the Charleston and Provi- in the early 1980s. Surface muscle stimulators were placed
dence braces, the amount of in-brace correction should over the muscles on the convex side of the scoliotic curve
approach 90% for flexible curves and 70% for rigid curves. and were activated for approximately 8 to 10 hours each
Regardless of the type of brace used, insufficient in-brace night. In Canada, electrode stimulators were actually
curve correction leads to an unsatisfactory outcome that implanted in the paraspinal muscles. Although some pre-
differs little from the expected natural history.553 If proper liminary success was reported with transcutaneous stimula-
correction cannot be obtained with brace use, orthotic tion,21 most studies found that this form of treatment did
treatment should be discontinued. nothing to favorably alter the natural history of scolio-
During brace management, follow-up visits are sched- sis.62,198,534,559 Today, electrical stimulation is no longer con-
uled at 4-month intervals for rapidly growing adolescents sidered a useful method in the management of idiopathic
with large curves. The interval may be extended to 6 months scoliosis.
for patients nearing maturity whose curves have shown no
recent changes. During these visits a single standing PA Physical Therapy and Biofeedback
thoracolumbar radiograph is obtained with the patient out Consistent asymmetry in torso rotation strength has been
of the brace. Curve progression, if it has occurred, is readily documented in patients with AIS when parameters such as
identifiable, and appropriate adjustments to the treatment specific strength testing and myoelectric activity recording
program can be made. Some physicians obtain radiographs are used.518 Although muscle conditioning is beneficial to a
with the patient wearing the brace to show the brace’s patient’s overall well-being, only modest evidence supports

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226 SECTION II  Anatomic Disorders

the concept that exercises or physical therapy programs are these cases, surgery should be considered when the curves
helpful in controlling or improving scoliosis.540,541,710,871 Like- exceed 40 to 45 degrees.
wise, spinal manipulations and biofeedback have yet to be In addition to curve magnitude, the patient’s appearance
shown to alter the natural history of scoliosis. (as perceived by the patient, the family, and the surgeon)
plays a role in surgical decision making. Patients and their
Surgical Treatment families are usually most concerned about this aspect of the
The primary goals of surgical intervention in the treatment deformity. The patient’s spinal balance may be decompen-
of scoliosis are to reduce the magnitude of the deformity, sated, with the thorax shifted noticeably away from the
to obtain fusion for prevention of future curve progression, midline; rib prominence may be severe because of excessive
and to do so safely. Operative treatment should result in a rotation; and the shoulders and hips may appear uneven.
well-balanced spine in which the patient’s head, shoulders, It is uncommon for back pain alone to serve as an indica-
and trunk are centered over the pelvis. Ideally, when this is tion for scoliosis surgery. Historically, 30% of patients with
accomplished with newer surgical implants, a significant scoliosis describe associated back pain; however, more
amount of curve correction can be achieved in all three recently studies suggest that up to 75% of patients have
planes. back pain that may be improved with surgery.869 Less than
Improved correction of deformity has resulted from a 10% of these symptomatic patients have a definite cause
combination of improved instrumentation systems that found for the discomfort.616 In general, the treating ortho-
impart more corrective force on scoliotic spines and paedist should not assume that the patient’s discomfort will
advances in surgical techniques that allow greater mobiliza- be remedied by spinal fusion.
tion and derotation of the spine. All pedicle screw–systems Curve patterns in males appear to be more rigid than
provide greater opportunity to gain control of each vertebra, those in females with idiopathic scoliosis.305,738 When plan-
which allows greater correction of each segment either indi- ning surgery in males, less curve correction should be
vidually or through “en bloc” techniques. Today, with the expected, but short- and long-term functional outcomes
introduction of pedicle screw fixation at nearly every level and complication rates can be expected to be similar to
of the spinal segment requiring instrumentation, curve cor- those in female patients.869
rection is even greater than that achieved with hook–rod
segmental fixation systems. These new pedicle screw Preoperative Planning
implant systems and the surgical techniques are complex Preoperative planning must take into consideration the
and require a significant amount of training. The established patient’s curve pattern and spinal balance, curve flexibility,
segmental fixation systems—including Cotrel-Dubousset neurologic status, rib deformities, physical maturity and
(CD), Texas Scottish Rite Hospital (TSRH), and Isola future growth potential, and other surgery-related needs
instrumentation—became popular in the mid-1980s and, (e.g., transfusion requirements, bone grafting, spinal cord
with refinements that allow the use of more pedicle screw monitoring, postoperative pain management). The surgeon’s
fixation points, remain so today. Each instrumentation selection of instrumentation depends on personal experi-
system allows the surgeon to achieve increased curve cor- ence, availability of the various systems, and the choice of
rection, improved sagittal contouring, brace-free postopera- anterior or posterior instrumentation.
tive mobilization, and improved MRI compatibility (with
the availability of titanium components). Curve Patterns
The expansion of technology since the early 1990s has King Classification System. In 1983, King and colleagues
resulted in the availability of numerous other systems, with introduced a radiographic classification system for AIS in
many manufacturers developing very similar concepts cen- which five different thoracic curve types were described
tered around multiple fixation points with pedicle screws (Fig. 12-25).384 This system provided useful recommenda-
that provide the opportunity to correct all three planes with tions for surgical intervention with Harrington implants;
especial focus on the use of axial plane correction strategies
while attempting to maintain the sagittal plane, particularly
thoracic kyphosis. Familiarity with one or more of these
systems, including their limitations, is helpful when plan-
ning surgical treatment of the various curve patterns seen
with idiopathic scoliosis.
Indications for Surgery
Although various considerations enter into surgical decision
making, curve magnitude remains the primary factor.
Curves less than 30 degrees at skeletal maturity are unlikely Type I Type II Type III Type IV Type V
to progress, regardless of the curve pattern, and do not
FIGURE 12-25  Diagrammatic representation of the King
require surgery. Thoracic curves and double major curves
classification of idiopathic scoliosis, which consists of five distinct
that exceed 50 degrees at skeletal maturity have a signifi-
curve patterns. This classification continues to be used regularly,
cant probability of worsening over time and nearly always although its reliability and reproducibility have been questioned.
warrant operative intervention.811 Thoracolumbar and (Redrawn from King HA, Moe JH, Bradford DS, et al: The selection
lumbar curves of less magnitude, when associated with of fusion levels in thoracic idiopathic scoliosis, J Bone Joint Surg Am
marked apical rotation or translational shift, also have a 1983;65:1302, with permission from The Journal of Bone and
propensity to worsen over time in mature patients.811 In Joint Surgery, Inc.)

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CHAPTER 12  Scoliosis 227

however, with the evolution of multiple hook or screw assessed to gain a better understanding of the three-
implant systems in the late 1980s, which allowed increased dimensional deformity. The sagittal modifier is hypoky-
three-dimensional curve correction, several shortcomings in photic (<10 degrees), normal (10 to 40 degrees), or
the descriptions and recommendations of the King system hyperkyphotic (>40 degrees). In addition to radiographic
became evident. The one King curve pattern that is not characterization, the clinical appearance is critical to deter-
better described in other systems is type IV, a thoracic curve mine curves requiring surgical treatment. A comparison of
in which the L4 vertebra is tilted into the curve. This curve right and left shoulder height is important to determine
pattern requires instrumentation to a more distal segment when inclusion of the proximal thoracic curve is necessary,
into the lumbar curve, generally the vertebra last touched waistline asymmetry helps determine when inclusion of the
by the center sacral vertical line (CSVL). lumbar curve is necessary, and the axial plane rotational
Lenke Classification System. Lenke and co-workers deformities are critical when deciding which curves are
developed a new classification system for AIS in 1997 that structural.
recognized the important contributions already made by
King and colleagues.416 Specific goals for the new classifica- Construct Selection. Three major trends with respect to
tion system were to allow more acceptable comparisons surgical treatment of scoliosis have occurred: first, the pos-
among the various types of operative treatment available, terior approach has had greater use than the anterior
to support a treatment-based approach by determining approach for nearly all curves, including Lenke 5 and 6
which of a patient’s scoliotic curves should be included thoracolumbar and lumbar curves; second, pedicle screws
in the instrumentation and fusion, to encourage three- have been used in all regions, including the thoracic spine,
dimensional analysis of scoliosis, and to achieve greater in place of hooks and hybrids380,412; and third, because of
intraobserver and interobserver reliability.415 The Lenke greater use of posterior releases and more powerful poste-
classification system is dependent on curve measurements rior implants, use of an anterior release has declined.164,254,435
in both the frontal and sagittal planes.417 It is comprehensive Proponents of screws suggest they provide stronger fixa-
(42 different curve patterns can be derived) yet is fairly tion than hooks do, are safe when inserted by experienced
easy for surgeons to learn quickly.415 The three main vari- surgeons, avoid placing metal within the canal, provide a
ables requiring evaluation are curve type (Table 12-3), stronger corrective force for three-dimensional curve cor-
lumbar spine modifiers, and thoracic sagittal modifiers (Fig. rection, may obviate the need for anterior fusion to avoid
12-26). the crankshaft phenomenon because of the strength of the
Using standard-size projection-type slides of photo- construct, and may avoid the need to perform thoraco-
graphs of premeasured radiographs, the developers of the plasty. Those who question the routine use of pedicle screw
Lenke classification system found both interobserver and placement for thoracic scoliosis cite the significant increase
intraobserver reliability to be excellent.415,417 However, in in cost with no documentation of measurable improvement
clinical practice, physicians vary significantly both in their in outcome studies, early reports of loss of thoracic kyphosis
selection of end vertebrae and in their angular measure- with screws, and the potential risk for injury to the spinal
ments of curves in individuals with idiopathic scoliosis.113,526 cord by surgeons inexperienced in the technique.
Because scoliosis surgeons rarely have access to premea- Fusion selection recommendations are based on correct
sured radiographs when they evaluate patients and formu- identification of curve patterns and the clinical deformity
late surgical plans, the reliability of the Lenke classification in each patient. Some generalities on selection of the
system is less impressive when tested with unmarked radio- level can be taken into consideration when developing
graphs. When all three of the main variables are combined, plans for fusion. The upper instrumented vertebra (UIV)
its overall reliability is fair.564,637 When each variable is is usually the proximal end vertebra for thoracic and
reviewed separately, its reliability improves. Despite this thoracolumbar/lumbar curves and, most often, T2 when
limitation, the Lenke classification system offers a more fusing a structural proximal thoracic curve. The lowest
comprehensive preoperative radiographic evaluation of instrumented vertebra (LIV) for lumbar/thoracolumbar
patients with AIS than was available with previous systems curves is usually the distal end vertebra when performing
and appears to correlate with surgical treatment of struc- posterior instrumentation. When fusing vertebrae for idio-
tural regions of the spine.57,414,424 pathic scoliosis via the anterior approach, they should gener-
The first parameter to identify in the Lenke classification ally be the UIV and LIV. In addition to these guidelines,
system is curve type (see Fig. 12-26), which is determined each curve pattern has some specific rules to follow.
by first identifying the largest curve. The other curves are Lenke type 1A and 1B curves have single thoracic struc-
then deemed structural by magnitudes that are greater than tural curves with nonstructural lumbar curves that do not
25 degrees on the supine best-bend radiograph or if junc- cross the midline (CSVL). Clinically, trunk imbalance is
tional kyphosis (measured between T2 and T5 for the proxi- more pronounced to the right in these patients than in those
mal and main thoracic curves and between T10 and L2 for with double-curve patterns and responds nicely to surgical
the main thoracic and thoracolumbar/lumbar curves) is treatment. For posterior constructs the UIV is generally the
greater than 20 degrees. Once the type of curve is identi- proximal end vertebra or one proximal to it when it is sig-
fied, the second main variable, the lumbar spine modifier, nificantly off midline. The rules for selection of the LIV are
is assessed (Fig. 12-27). The CSVL (vertical line drawn more varied, with three general rules: first, fusion to one or
upward from the center of the sacrum) is drawn, and its two levels proximal to the stable vertebra633; second, fusion
position relative to the concave pedicle of the apical lumbar to the vertebra that last touches the CSVL; and third, as
vertebra determines the lumbar modifier. The final main reported by Suk and colleagues, rules based on the relative
variable, the thoracic sagittal modifier (T5-12), is then position of the neutral vertebra (the most proximal vertebra

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228 SECTION II  Anatomic Disorders

Table 12-3  Lenke Curve Types

Characteristic Curve Patterns

Curve Thoracolumbar
Type Description Proximal Thoracic Main Thoracic or Lumbar Structural Region

1 Main thoracic Nonstructural Structural (major) Nonstructural Main thoracic

Cobb angle: ≥25°
on side-bending
Kyphosis: +20°
between T10
and L2
2 Double thoracic Structural Structural (major) Nonstructural Proximal thoracic,
Cobb angle: ≥25° Cobb angle: ≥25° main thoracic
on side-bending on side-bending
radiographs radiographs
Kyphosis: +20° Kyphosis: +20°
between T2 between T10
and T5 and L2
3 Double major Nonstructural Structural (major) Structural Main thoracic,
Cobb angle: ≥25° Cobb angle: ≥25° thoracolumbar,
on side-bending on side-bending or lumbar
radiographs radiographs
Kyphosis: +20° Kyphosis: +20°
between T10 between T10
and L2 and L2
4 Triple major Structural Structural (major)* Structural (major)* Proximal thoracic,
Cobb angle: ≥25° Cobb angle: ≥25° Cobb angle: ≥25° main thoracic,
on side-bending on side-bending on side-bending thoracolumbar,
radiographs radiographs radiographs or lumbar
Kyphosis: +20° Kyphosis: +20° Kyphosis: +20°
between T2 between T10 between T10
and T5 and L2 and L2
5 Thoracolumbar Nonstructural Nonstructural Structural (major) Thoracolumbar or
or lumbar Cobb angle: ≥25° lumbar
on side-bending
Kyphosis: +20°
between T10
and L2
6 Thoracolumbar Nonstructural Structural Structural (major) Thoracolumbar or
or lumbar, Cobb angle: ≥25° Cobb angle: ≥25° lumbar, main
main thoracic on side-bending on side-bending thoracic
radiographs radiographs
Kyphosis: +20° Kyphosis: +20°
between T10 between T10
and L2 and L2

Modified from Lenke LG, Betz RR, Harms J, et al: Adolescent idiopathic scoliosis: a new classification to determine extent of spinal arthrodesis,  
J Bone Joint Surg Am 83:1169, 2001, with permission from The Journal of Bone and Joint Surgery, Inc.
*Either the main thoracic curve or the thoracolumbar or lumbar curve can be the major curve.

that has neutral axial plane rotation based on the position vertebra is L4, and posterior instrumentation can be stopped
of the pedicles) and the position of the distal end verte- at L2 (two levels cephalic to the stable vertebra). Anterior
bra.747,751 Preoperative planning for posterior instrumenta- instrumentation has shown that even more inferior motion
tion using only screws is shown in Figure 12-28. Frequently, segments can be preserved because the implant extends
the implant is a hybrid, with screws used as an anchor at down to only the lowest vertebra included in the measured
the bottom of the constructs and some combination of curve.64,358,413
hooks, sublaminar wires, or tape and screws used Patients with Lenke type 1C curves have single thoracic
above.118,332,666,858 For the King-Moe type IV curve (a Lenke structural curves with nonstructural lumbar curves, and the
1A curve when L4 is tilted into the curve), the end vertebra apical lumbar vertebra completely crosses the midline (i.e.,
may be chosen as the LIV, especially when the stable is not in contact with the CSVL). In the late 1980s and

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CHAPTER 12  Scoliosis 229

Lumbar spine modifier Curve type (1–6)

Type 1 Type 2 Type 3 Type 4 Type 5 Type 6
(main (double (double (triple (TL/L) (TL/L-MT)
thoracic) thoracic) major) major)

(No to minimal

1A* 2A* 3A* 4A*

(Moderate curve)

1B* 2B* 3B* 4B*

(Large curve)

1C* 2C* 3C* 4C* 5C* 6C*

Possible sagittal ≥+20° ≥+20°

structural criteria
(to determine
≥+20° ≥+20°
specific curve type)

Normal PT TL PT+TL
kyphosis kyphosis kyphosis

* T5-12 sagittal alignment modifier: –, N, or +

– is <10° N (normal) is 10-40° + is >40°
FIGURE 12-26  Schematic drawings of the curve types, lumbar modifiers, and sagittal structural criteria that determine specific curve
patterns. MT, main thoracic; PT, proximal thoracic; TL, thoracolumbar; TL/L, thoracolumbar/lumbar. (Redrawn from Lenke LG, Betz RR,
Harms J, et al: Adolescent idiopathic scoliosis: a new classification to determine extent of spinal arthrodesis, J Bone Joint Surg Am 83:1169,
2001, with permission from The Journal of Bone and Joint Surgery, Inc.)

1990, use of “derotation” instrumentation for selective tho- was most likely multifactorial, and improvement occurred
racic fusions often led to spinal imbalance manifested as a over time as the uninstrumented lumbar curve adapted
shift in the patient’s trunk or head (or both) to the left of (Fig. 12-29).489,628
midline—so-called decompensation.‖a When using this method of instrumentation for selective
Despite many purported reasons, including improper fusions in individuals with Lenke type 1C curves, the
choice of fusion levels, overcorrection of the thoracic curve, surgeon should keep in mind some principles that can mini-
incorrect identification of curve patterns, lumbar curve mize the chance of postoperative imbalance. First, the
stiffness, and progression, the cause of decompensation instrumentation should not extend beyond the stable ver-
tebra; second, if using the rod rotation maneuver, incom-
References 46, 55, 88, 419, 489, 493, 519, 628, 633, 764. plete rotation should occur to avoid overcorrection or

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Lumbar spine modifier A, B, C rules

1. Examine upright coronal radiograph.

2. Accept pelvic obliquity <2 cm. If >2 cm, must block out leg length
inequality to level pelvis.
3. Draw CSVL with a fine-tip pencil or marker. Bisects proximal sacrum and
line drawn vertical to parallel lateral edge of radiograph.
4. Stable vertebra—Most proximal lower thoracic or lumbar vertebra most CSVL
closely bisected by CSVL. If a disk is most closely bisected then choose
next caudal vertebra as stable.
5. Apex of curve is the most horizontal and laterally placed vertebral body
or disk.
6. SRS definitions:
Curves Apex
Thoracic T2 to T11-12
Thoracolumbar T12-L1
Lumbar L1-2 to L4
Lumbar modifier A

CSVL falls between lumbar pedicles up to

stable vertebra
Must have a thoracic apex
If in doubt whether CSVL touches medial
aspect of lumbar apical pedicle, choose type B

Includes King types III, IV, and V

CSVL between pedicles up to stable vertebra,

no to minimal scoliosis and rotation of L-spine

Lumbar modifier B

CSVL falls between medial border of lumbar

concave pedicle and lateral margin of apical
vertebral body or bodies (if apex is a disk)

Must have a thoracic apex

If in doubt whether CSVL touches lateral
margin of apical vertebral body or bodies,
choose type B
Includes King types II, III, and V

CSVL touches apical vertebral bodies or pedicles,

minimal to moderate L-spine rotation

Lumbar modifier C

CSVL falls medial to lateral aspect of lumbar

apical vertebral body or bodies (if apex is a disk)
May have a thoracic, thoracolumbar, and/or
lumbar apex
If in doubt whether CSVL actually touches
lateral aspect of vertebral body or bodies,
Apical Apical choose type B
disk body
Includes King types I, II, V, double major, triple
major thoracolumbar, and lumbar curves

CSVL does not touch apical vertebral body or the bodies

immediately above and below the apical disk
FIGURE 12-27  Rules and definitions for determining the lumbar spine modifiers A, B, and C. A, Steps required to determine the stable
vertebra. B, Description of lumbar modifiers A, B, and C. CSVL, Center sacral vertical line; SRS, Scoliosis Research Society. (Redrawn from
Lenke LG, Betz RR, Harms J, et al: Adolescent idiopathic scoliosis: a new classification to determine extent of spinal arthrodesis, J Bone Joint
Surg Am 83:1169, 2001, with permission from The Journal of Bone and Joint Surgery, Inc.)
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CHAPTER 12  Scoliosis 231



FIGURE 12-29  A, Preoperative anteroposterior radiographs of
a Lenke 3C curve with an 80-degree right thoracic curve and a
63-degree left lumbar curve in a 13-year-old girl. B, Two years
after surgery, the overall coronal plane correction is satisfactory.
Greater implant density was used on the right side than in Figure
FIGURE 12-28  Construct planning for the use of all pedicle screws 12-28 because of the large stiff nature of the curve.
varies among surgeons, and no specific pattern has been fully
adopted or studied. However, the most common scenario is to
use pedicle screws at each level in a segmental fashion for the  
rod that is used for the main portion of the correction. excessive straightening of the thoracic curve; and third,
For the double major curve shown, the most common screw other correction strategies such as apical translation with
pattern is segmental screw fixation for the left rod, which is used
fixed proximal and distal anchors or a cantilever can be
to correct the coronal plane while maintaining the sagittal plane
performed. Preoperative planning involving pedicle screw
or correcting any junctional kyphosis between the main thoracic
and lumbar curves. Typically, variable-angle or polyaxial screws anchors for selective thoracic instrumentation and fusion of
can be used in the lumbar spine and fixed-angle screws in the a Lenke type 1C curve is shown in Figure 12-30.
thoracic spine. If a segmental screw pattern is not used, an Failure to properly distinguish Lenke type 1C or King
alternative screw pattern on the left side should always include type II curves from true double major curves (Lenke type
segmental fixation of the lumbar curve and a more typical 3C) may be responsible for some cases of postoperative
hook–screw construct for the thoracic curve. However, to achieve imbalance following selective thoracic fusions. Useful
maximal correction, segmental fixation with pedicle screws is guidelines have been developed to help differentiate these
generally used for the left rod. curve patterns.419 Relative ratios between the thoracic and
The screw pattern on the right rod generally includes at least
lumbar curves with regard to their size, rotation, and devia-
two screws in the distal segment of the lumbar spine to allow
tion from midline can be assessed preoperatively on a stand-
fine-tuning of the lowest instrumented vertebra to ensure that it  
is horizontal and neutral. L2 and L3 screws are used for complete ing radiograph. If thoracic curve parameter–lumbar curve
fixation of the lumbar curve, and T11 and T12 screws are used for parameter ratios are less than 1.0, both curves will require
fine-tuning of the lumbar curve. Screws are used at the apex of fusion. If the ratios are greater than 1.2 for curve size and
the convexity of the thoracic curve to provide an opportunity to deviation and greater than 1.0 for rotation, selective tho-
perform a convex apical derotation maneuver to correct the axial racic fusion can be performed safely. With true double
plane deformity in the thoracic spine. Two screws are then used at major curves, both curves must be included in the posterior
T4 and T5 to provide good fixation proximally. fusion to achieve a balanced spine with segmental fixation
Once the screws are placed on the left side, a rod rotation systems.
maneuver is performed to correct the coronal plane and sagittal
Selective anterior fusion on the convexity of the thoracic
plane deformity. The apical convex screws can then be used to
component in Lenke type 1C curves (using screws and
derotate the apical thoracic spine after loosening the rod on the
concave apical segments. Downward and lateral pressure on the either a threaded or a smooth rod) has been advo-
convex screws with upward and medial pressure on the concave cated.66,413,844 Reported advantages over posterior instru-
screws allows apical derotation. Following this, the right rod can mentation include improved balance, correction of a
be placed, and fine-tuning of the coronal and sagittal planes is hypokyphotic thoracic spine, and preservation of more infe-
performed. rior motion segments.

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232 SECTION II  Anatomic Disorders

FIGURE 12-30  Planning fusion for a Lenke 1 curve using an all-screw construct
and preoperative, postoperative, and follow-up standing anteroposterior
radiographs following posterior spinal fusion of a Lenke 1C pattern. A, Fusion
selection in this case was to L1. For the left side, two distal screws provide a
nice foundation to build the construct. To translate the apex medially and
posteriorly, two (shown here) or three (for stiffer curves) apical screws are used.
For stiff curves, one, two, or three screws may be reduction-type screws to
allow gradual reduction of the apex to the left and posteriorly. Alternatively,
some systems allow attachment to a regular polyaxial screw, which permits this
translation to occur. Proximally, two screws provide excellent control of the
proximal segments of the spine. For the right rod, a similar two-screw construct
proximally and distally controls these segments of the spine; the three screws
shown here provide excellent axial plane correction. Alternatively, two screws
can be used at the apex on the convex side. B, Preoperative images of a Lenke
1C pattern in a 14-year-old demonstrating a main thoracic curve of 57 degrees
and a lumbar curve measuring 46 degrees. C, Six weeks following selective
thoracic fusion from T4 to T12, the patient has undergone shift of the trunk to
the left, which is expected. D, At 6 months the lumbar curve has appropriately
increased so that the patient has become nicely balanced.


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CHAPTER 12  Scoliosis 233

Preliminary studies have reported breakage of the

threaded rod in 31% of patients,39 but this complication can
be remedied by the use of a larger rod and thorough dis­
kectomies and bone grafting. Thoracoscopically placed
anterior instrumentation has become used less often but
overall demonstrates comparable results to posterior fusion
with inclusion of fewer fusion levels.544,547,739,844
Patients with Lenke type 2 curves have double thoracic
structural curves with nonstructural lumbar curves. The
first thoracic vertebra is tilted into the upper curve, with
junctional kyphosis between the proximal and main thoracic
curves. The patient’s shoulder on the side of the convexity
of the upper curve is nearly always elevated. The upper
curve and the shoulder elevation may worsen if only the
lower main thoracic component is instrumented. Therefore,
most Lenke type 2 curves require posterior instrumentation
of both thoracic curves. Selective main thoracic fusion using
anterior instrumentation has been successful when the
proximal thoracic curve is sufficiently flexible. The poste-
rior instrumentation should extend up to the second tho- A B
racic vertebra if T1 is tilted into the upper curve and the
shoulder is elevated on the convex side of the upper curve,
if the upper curve is greater than 30 degrees with limited
flexibility, or if the transitional vertebra between the curves
is located at T6 or below.394,408,423,826,829 Lenke type 3 curves
represent double major structural curve patterns in which
both the thoracic and lumbar components require instru-
mentation. Preoperative planning for instrumentation of
double major curves is shown in Figure 12-31, with LIV
selection principally to the distal end vertebra. Curve char- FIGURE 12-31  Planning fusion for a Lenke type 3C (double major)
acteristics that allow stopping short of the end vertebra curve using a hook–screw construct.
include thoracolumbar or lumbar curves smaller than 55 1. The hook sites used for the thoracic component of the
degrees, flexible curves, and apex of the thoracolumbar or deformity are almost identical to those used for selective
lumbar curves that is two or more levels proximal to the thoracic fusion.
intended LIV. Lenke type 4 curves are triple major curve 2. For the lumbar component, the instrumentation usually
patterns in which both thoracic curves, in addition to the extends to L3. Fusion to L4 should be considered only if the L3
lumbar curve, are structural. All three curves require pos- vertebral body deviates significantly from the midline and if the
L3-4 disk space remains wedged open on the side of the
terior instrumentation.
convexity. Fusion to L5 for idiopathic scoliosis is almost never
In Lenke type 5 curves, only the thoracolumbar or indicated.
lumbar curve is structural. The anterior approach has tradi- 3. The convexity of the lumbar curve is approached first. Pedicle
tionally been used for these curves with excellent three- screws are placed at every level (in this example, from T12
dimensional correction.254,369,806,807 However, the use of through L3) (A). This provides firm fixation and allows
posterior instrumentation with aggressive release techniques correction (and compression) across the entire convexity of the
provides a viable alternative to the anterior approach. Pro- lumbar curve.
ponents of the posterior approach suggest that the same 4. On the concavity of the lumbar curve, a pedicle screw is placed
number of levels are fused (proximal end vertebra to distal at T12 and L3 (B). Another pedicle screw may be added at L2
end vertebra), coronal plane correction is the same, surgical if desired.
time is shorter, no chest tube is necessary, and intensive care
unit and hospital stay is shorter. Advocates of the anterior
approach suggest that not all curve patterns will allow accompanied by a smaller but structural main thoracic
similar fusion levels and the axial plane correction is less curve. Selective fusion of the thoracolumbar or lumbar
because the disk and soft tissues are still intact with the curve will not yield similar results as selective thoracic
posterior approach. Anterior spine surgery for these curves fusion because the thoracic curve will not respond to main-
has always had challenges in achieving fusion; however, con- tain balance.670,678 Posterior fusion and instrumentation are
structs using one or two solid rods were developed and necessary in this circumstance.
resulted in a decreased incidence of pseudarthrosis, better
maintenance of restored sagittal lordosis, and elimination of Preoperative Curve Flexibility. Preoperative curve flexibil-
postoperative brace immobilization.320,343,356,357,781 Preopera- ity can be assessed by a number of different techniques,
tive planning for anterior or posterior instrumentation of a including supine best-effort, side-bending radiographs; the
thoracolumbar curve is shown in Figure 12-32. fulcrum bend test; or a supine resting radiograph. We use
Lenke type 6 curves represent double curve patterns, supine best-bend radiographs because they realistically
with the primary thoracolumbar or lumbar curve reflect the amount of curve correction that can be achieved

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234 SECTION II  Anatomic Disorders

FIGURE 12-32  Planning posterior instrumentation of a Lenke type 5C

thoracolumbar curve and a case example. A, Generally, screws in each pedicle
from end vertebra to end vertebra are used. B, Preoperative radiographs of a Lenke
5C curve with a 24-degree right thoracic curve and a 48-degree lumbar curve in a
14-year-old girl. C, Two years following posterior spinal fusion and instrumentation
from T11 to L3.



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CHAPTER 12  Scoliosis 235

posteriorly and determine flexibility of the spine with the Future Growth Potential. Correction of scoliosis by poste-
newer generation of instrumentation systems. These radio- rior spinal instrumentation and fusion is usually maintained
graphs are used to determine the flexibility of the remaining over time and is not adversely affected by any remaining
curves to provide a Lenke classification. They can also be anterior spinal growth; however, the crankshaft phenome-
used to help determine or confirm the LIV for isolated non (resumption of the curve secondary to anterior growth
thoracolumbar or lumbar curves.122,388,794 For left thoraco- in patients with posterior fusion) can still occur. Dubousset
lumbar or lumbar curves, a right-bend film should demon- coined the term when he observed that the entire spine and
strate that the proposed LIV is centered over the sacrum trunk gradually rotated and deformed as the anterior portion
and, on the left-bend film, should demonstrate “reversal” of the spine continued to grow and twist around the axis of
of the disk above the intended LIV. Large curves (>75 the fusion mass (in a manner similar to an automobile
degrees) are best analyzed with traction films because the crankshaft)196 (Fig. 12-33). Methods to prevent this phe-
mechanics of the bend films is minimized for these large nomenon include careful assessment of the growth remain-
and stiff curves.603 The push-prone radiograph was shown ing, the use of anterior fusion when appropriate, and greater
to be the best preoperative indicator of flexibility for pre- use of and correction with pedicle screw fixation. Although
dicting the final lumbar curve measurement in patients its severity is difficult to quantify, the crankshaft phenom-
undergoing selective thoracic fusion for Lenke type 1B and enon can best be appreciated by examining serial clinical
1C curves.189 Significant study using the fulcrum bend test photographs that demonstrate progressive changes in rib
has demonstrated that for the thoracic curve, this test best deformities, narrowing of the chest, and imbalance in the
predicts the status of the thoracic curve following posterior thoracic and lumbar spine. Radiographs can also demon-
spinal fusion and instrumentation.227,298,466,467 strate progressive changes over time, such as alterations in
curve size, rotation, and rib–vertebral angle difference;
Neurologic Status. If a subtle neurologic abnormality (e.g., translation of the apical vertebra toward the chest wall on
asymmetric abdominal reflexes) is detected in an otherwise the convexity; and changes in the vertical inclination of the
normal individual, MRI of the entire spinal canal should be instrumentation. Radiographic changes of more than 10
considered to rule out syrinx, cord tethering, or diastema- degrees in curve size, apical vertebral rotation, and the
tomyelia.864 Preoperative MRI should also be performed in rib–vertebral angle difference are all thought to reflect pro-
patients with left thoracic curves and in those in whom the gression of the deformity secondary to the crankshaft phe-
typical apical lordotic sagittal deformity is absent because nomenon.288,405,671 However, during the first 6 to 12 months
of the association with intracanal abnormalities.231,482,571,616,681
The MRI study can be ordered when surgery is scheduled.
Studies have demonstrated that excessive rotation or
kyphosis in the thoracic spine is an indication to perform

Rib Deformities. The rotational deformity inherent in AIS

is often seen most by families and is generally mild to
moderate with rare occurrence of the razorback deformity
(it is more common in nonidiopathic conditions such as
neurofibromatosis). Suggested indications for thoracoplasty
include a preoperative rib prominence exceeding 10
degrees (measured from a tangential radiograph with the
patient bent forward 90 degrees), preoperative curves
greater than 60 degrees, and flexibility less than 20%.295
Surgeon preference and experience play a large role in
determining the indications for thoracoplasty; however, the
general trend has been less use of this procedure because
of improved methods for direct vertebral rotation (DVR).
Nonetheless, Professor Suk, the developer of the DVR
technique, continues to perform thoracoplasty in all
patients with AIS. In addition to improving the patient’s
cosmetic appearance, partial resection of three to five apical
ribs provides bone graft in amounts sufficient to obviate
an iliac crest graft.40,256,295,668,746 A percutaneous thoraco-
plasty technique has resulted in excellent correction but
some loss of coronal plane correction.859 Because some
studies report a decline in pulmonary function following
thoracoplasty, this technique is contraindicated in patients
with compromised preoperative pulmonary or cardiac FIGURE 12-33  Crankshaft phenomenon in an 11-year-old girl
status. More recent studies have demonstrated an early 1 year after undergoing posterior spinal fusion with
decline in pulmonary function test results without long- instrumentation consisting of all pedicle screws. She grew 7 cm
lasting effects when compared with a cohort that did not since surgery and has had a trunk shift to the right with a
undergo thoracoplasty.134,411,746,859 significantly worse clinical rotational deformity as shown here.

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236 SECTION II  Anatomic Disorders

after surgery, it is important to not automatically assume Bone Grafting. The primary goal of scoliosis surgery is to
that changes in radiographic measurements are a result of achieve a solid arthrodesis, which is enhanced by meticulous
the crankshaft phenomenon; these changes are often due to cleaning of soft tissue from the spine, facetectomies, decor-
stress relaxation of the spine, gradual maturation of the tication, and adequate bone grafting. Although autogenous
fusion mass, and realignment of the curve. iliac crest bone grafting has previously been the stan-
For female adolescents in need of surgery who have not dard,133,801 significant postoperative pain at the donor site
yet reached their PHV, who are premenarchal, and whose persists and remains the greatest problem with the use of
triradiate cartilage remains open, strong consideration autogenous grafting.274,707 Because of these postoperative
should be given to combining anterior and posterior fusion symptoms, alternative bone graft substitutes have been
to prevent the crankshaft phenomenon.191,405,673,697 For ante- sought.67,333,430,431,860 Numerous studies of successful fusions
rior spinal fusion, a conventional open thoracotomy approach using allografts of frozen, bank-stored bone as a substitute
has been compared with the newer, less invasive video- for autogenous bone have been reported34,75,190,214,274,730
assisted thoracoscopic surgery (VATS).316,503,549,737,804 Advan- without an increase in pseudarthrosis rates.190,214
tages of VATS include muscle sparing, improved cosmetic To minimize the risk of transmitting human immunode-
results (less scarring), greater access to the entire length of ficiency virus, hepatitis virus, and any other potential viral
the thoracic spine, and less effect on pulmonary function pathogens, the donor blood and tissue are tested at the site
than with open thoracotomy. Instruments are used through of recovery, and testing is usually continued throughout the
multiple intercostal portals to resect disk material, perform harvesting process. Freeze-dried cancellous bone is usually
anterior release, and insert bone graft. However, surgeons exposed to low-dose gamma radiation to sterilize all non-
require extensive training in the VATS technique. systemic bacterial and fungal contaminants. Bone morpho-
Some reports suggest that stiff posterior constructs, par- genetic protein has become popular for use in single-level
ticularly when screws are used at nearly every level in the lumbar spinal fusions but has yet to become cost-effective
segment fused, may be strong enough to prevent the crank- for routine use in multisegment scoliosis fusions.5,99,496,606,789
shaft phenomenon in immature patients, thus avoiding the The authors’ preferred technique is to perform thorough
need for anterior fusion.100,412,759 More research is needed to stripping of the spine, followed by aggressive facetectomies,
prove the effectiveness of this approach. decortications of the spine, and the use of allograft bone,
without any pseudarthrosis in individuals with AIS being
Transfusion Requirements. Several procedures are available documented in the past 8 years.
to reduce the need for homologous blood transfusions in
patients undergoing posterior spinal instrumentation for Spinal Cord Monitoring. Spinal cord monitoring using both
scoliosis, including controlled hypotensive anesthesia, autol- spinal somatosensory evoked potentials (SSEPs) and motor
ogous blood predonation of 1 or 2 units, acute normovole- evoked potentials (MEPs) is the standard of care during
mic hemodilution, intraoperative and postoperative salvage scoliosis surgery and is critical to the safety of any spine
of lost blood, intraoperative use of antifibrinolytics, and deformity surgery. SSEPs record the sensory function of the
transfusion decisions based on clinical judgment rather than spinal cord and provide continuous monitoring throughout
on a predetermined hemoglobin value. the procedure.#a This test may, however, be adversely
Various combinations of these methods have been shown affected by changes in anesthetic level and perfusion, and
to significantly reduce exposure of patients to homologous critical changes tend to lag behind MEPs. With impending
blood products during scoliosis surgery.¶a The combination neurologic deficit, MEPs are used to monitor the anterior
of predonated autologous blood, hypotensive anesthesia, spinal cord motor tracts and are ideally performed by apply-
and intraoperative salvage of lost blood is probably the ing a stimulus to the motor cortex of the brain (transcranial
one used most frequently for healthy individuals with idio- MEPs [tcMEPs]).59,263,575,680,726,754 This provides direct stim-
pathic scoliosis.520,532 Intraoperative salvage of lost blood, ulation of the motor cortex, which then travels through the
the most expensive of the available techniques, is most anterior column tracts with responses noted in the upper
effective when blood loss is expected to exceed 1000 mL. and lower extremity musculature (Fig. 12-34). When MEPs
Acute normovolemic hemodilution appears to be a satisfac- are used in conjunction with SSEPs, the chance of unrec-
tory alternative to the use of predonated autologous ognized injury to the spinal cord is minimized. A large
blood.143,567 multicenter study of 1121 patients demonstrated that 38
The antifibrinolytic agent ε-aminocaproic acid (Amicar) (3.4%) had a critical change on monitoring when tcMEPs
is reportedly a safe, effective, and inexpensive method of and SSEPs were used. Hypotension was responsible for nine
reducing perioperative blood loss in patients with scolio- changes (corrected by elevating blood pressure), whereas
sis.174,241 We generally prefer tranexamic acid (TXA), which three were due to segmental vessel ligation. The tcMEP/
has also been shown to reduce intraoperative and postopera- SSEP combination did not miss any patient with a transient
tive blood loss during posterior spinal fusion and instrumen- motor or sensory deficit.679 Similar studies have demon-
tation for AIS in a matched cohort.856 The response to TXA strated excellent results with combined multimodal intra-
depends on the dose administered, with a higher loading operative neuromonitoring during AIS surgery.230,584,767,868
dose of 20 mg/kg followed by a 10-mg/kg/hr infusion Although total intravenous anesthesia with propofol is
appearing to have a greater effect.270 necessary to obtain good tcMEP data, other techniques
to assist in obtaining good monitoring data have

References 20, 142, 143, 229, 240, 241, 253, 329, 350, 393, 520,
532, 567, 598, 701, 703, 788. References 25, 123-125, 307, 468, 527, 555, 579.

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CHAPTER 12  Scoliosis 237

Postoperative Pain Management. Patient-controlled analge-

sia (PCA) and epidural analgesia are the two methods used
regularly for the management of postoperative pain. PCA
provides safe and effective analgesia in children as young as
5 years. It allows the patient to self-administer small, pre-
programmed doses of opioids via a pump connected to the
patient’s intravenous tubing.485 This enables the patient to
titrate an opioid blood level in direct response to the chang-
ing intensity of pain. The built-in safety mechanism of PCA
systems prevents oversedation. In addition, PCA devices
can deliver a continuous infusion so that therapeutic levels
of analgesia are maintained during sleep.
The use of epidural analgesia for scoliosis surgery has
become increasingly popular because it provides excellent
pain relief; however, meticulous attention to detail is
required.*b At the end of the surgical procedure but before
closure, the surgeon inserts an epidural catheter. The cath-
eter is tunneled lateral to the incision and is usually left in
place for 48 to 72 hours. Low-dose opioids are infused to
provide effective analgesia, usually under the direction of
pain management teams experienced in this technique.
FIGURE 12-34  Output of a typical response when using Close monitoring of the patient’s respiratory status and the
transcranial motor evoked potentials (tcMEPs). The left extremities use of pulse oximetry are necessary for 24 hours after the
(left) and right extremities (right) are shown. The red response is infusion has been discontinued. Postoperative pulmonary
the baseline data, whereas the green notes the latest output from toileting is optimized with this technique.
the most recent “run” of tcMEPs. The muscle group in the upper
Ketorolac, an injectable nonsteroidal antiinflammatory
extremity is the abductor pollicis brevis, and the lower extremities
are evaluated with three muscle groups—the tibialis anterior (LTA
drug, is effective for the short-term management of moder-
and RTA), the soleus (LSOL and RSOL), and the abductor hallucis ate to severe postoperative pain. It is often used in conjunc-
(LAH and RAH). Note that the most recent amplitudes (green) are tion with opioids because the combination provides more
the same as the baseline (red) in all muscle groups demonstrating effective analgesia than either drug alone does. Although its
good responses. use has been associated with pseudarthrosis after adult low
back surgery,262,488 this problem has not been demonstrated
in large series of patients with AIS undergoing surgical
been evaluated.370,481,868 The current increase in the use of correction.740,803
thoracic pedicle screws for fixation points has led some
surgeons to use triggered electromyographic (EMG) moni- Antibiotic Prophylaxis for Dental Procedures. Antibiotic
toring; however, this has been relatively unreliable in the prophylaxis for dental procedures in patients with spinal
thoracic region without clearly identified thresholds to indi- instrumentation is a controversial issue.631 Currently, no
cate when medial screw penetration is seen.620 scientific evidence supports the position that antibiotics
The wake-up test, a gross evaluation of motor function, should be given during routine dental care. Streptococcus
is no longer used routinely if spinal cord monitoring is avail- viridans, the predominant bacterium in normal human oral
able and results are normal throughout surgery. The wake-up flora and the most common organism isolated from blood
test can be performed if changes in SSEPs or MEPs are after dental procedures, has not been reported in delayed
noted during correction of the spine because spinal cord deep wound infections following spinal instrumentation. In
injury may exist even when monitored variables return to those in whom early postoperative wound infections
baseline.552 The authors generally do not perform this test develop, Staphylococcus aureus is the predominant organ-
during AIS surgery because first, a “normal” wake-up test ism. Yet S. aureus accounts for only 0.005% of the normal
does not provide a detailed examination and strength testing oral flora and is rarely isolated after dental procedures.166
is not possible and, second, even if normal, tcMEP/SSEP Guidelines similar to those provided in the advisory
monitoring can indicate some stress or subclinical deficit in statement issued by the AAOS regarding antibiotic prophy-
the spinal cord that may become a permanent deficit with laxis for dental patients with total joint replacement should
continued surgery. For this test the anesthesiologist allows be used for those who have undergone spinal instrumenta-
the patient to regain partial consciousness and motor func- tion.19 If antibiotic prophylaxis is given, the following
tion during the surgical procedure.285 Intraoperative neuro- regimen is recommended: patients who are not allergic to
monitoring is especially useful when intraoperative traction penicillin can be treated with cephalexin, cephradine, or
is used because critical changes develop in a third of patients amoxicillin, 2 g orally 1 hour before the dental procedure;
during surgery and are related to having a thoracic curve, a patients who are allergic to penicillin should receive
larger Cobb angle, and a rigid curve. A stepwise response clindamycin, 600 mg orally 1 hour before the dental
to these changes, including removal of the traction, resulted procedure.
in overall good results, and the presence of MEP recordings
at the completion of the surgery was associated with normal
neurologic function.432 *bReferences 26, 204, 373, 464, 485, 687, 736, 769.

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238 SECTION II  Anatomic Disorders

Posterior Spinal Instrumentation

Exposure of the spine for posterior instrumentation must
be meticulous and thorough, regardless of the implant
system selected (see Plate 12-1 on page 292).

Harrington Instrumentation. Harrington developed his

technique in the late 1950s and first reported it in 1962.292,293
In this system, hooks apply distraction forces to the concave
side of the spinal curve via a ratchet mechanism. Compres-
sion force is applied to the convex side of the thoracic curve
at the base of the transverse processes, with the amount of
force adjusted by tightening nuts on a threaded rod.
Long-term follow-up studies have reported that approxi-
mately 30% to 40% of curve correction is maintained
through the years with Harrington instrumentation.†b
However, minimal, three-dimensional correction of the
spine was achieved because distraction forces flattened the
spine and the implants provided insufficient stability to
allow brace-free postoperative mobilization. The technique
is detailed in the second edition of Tachdjian’s Pediatric

Multiple-Hook Segmental Instrumentation. The CD

instrumentation system was developed in France by Cotrel
and Dubousset and was introduced in the United States in
the mid-1980s (Fig. 12-35).147 The system revolutionized
posterior instrumentation for idiopathic scoliosis by enhanc-
ing the surgeon’s ability to improve the three-dimensional
orientation of the spine. This was accomplished through the
“derotation maneuver” popularized by Dubousset,37,148
whereby the contoured rod is secured to the spine with
various hooks and rotated 90 degrees to bring the concave FIGURE 12-35  Cotrel-Dubousset (CD) instrumentation. The first
spine posterior and medial for correction. This maneuver, contoured rod is secured to the concave side of the spine with
which continues to be used today, improves the sagittal multiple hooks and is rotated 90 degrees. This maneuver improves
contour, achieves significant curve correction, and improves the sagittal contour and achieves significant correction of the
the rotation or translation of the spine. The second rod curve. Placement of the second rod increases the construct’s
increases the construct’s strength and torsional stability, strength. CD instrumentation uses numerous hooks but no
particularly when rigidly united to the first rod via a rod- sublaminar wires.
connecting device.
Numerous reports have documenteded significant
improvement in the correction of idiopathic scoliosis with
CD instrumentation. Rib deformities were reduced, curve illustrated in Plate 12-2 on page 294 on the accompanying
correction in the range of 48% to 69% was achieved and website.
maintained, and nearly normal sagittal alignment was
restored.‡b The ability to preserve lumbar lordosis in curves Pedicle Screws. The use of pedicle screws has dramatically
requiring long fusion to L3 or L4 avoided the long-term “flat changed the operative treatment of all spinal deformity,
back” problems that occurred with Harrington distraction including AIS. The use of pedicle screw fixation for correc-
instrumentation. tion of deformity was first described in the lumbar spine in
The TSRH instrumentation system was introduced in the mid-1990s.38,289,749 These studies demonstrated that
1988 and, like the CD system, uses multiple hooks and pedicle screws provide greater ability to obtain and maintain
screws to attach smooth, precontoured rods to the spine.32,345 coronal plane correction of the thoracolumbar or lumbar
Once the system is assembled, selective compression, dis- curves in individuals with double major idiopathic scoliosis.
traction, and rotation maneuvers can be performed to When compared with hooks, initial correction was 72%
correct the spinal deformity. These maneuvers follow the versus 60% with hooks, and less loss of correction occurred
principles introduced by Cotrel and Dubousset148 and have at follow-up (5% versus 13%).38 Significant improvement in
resulted in outcomes similar to those with CD instrumenta- LIV tilt (82% versus 50%) and translation (50% versus 23%)
tion.72,634,718 The technique of a multiple-hook system is was seen when compared with hooks.289
Pedicle screws placed in the lumbar spine for pediatric
References 160, 178, 304, 328, 458, 507, 576, 817. spinal deformity have a very good track record, with few
References 71, 87, 154, 236, 276, 328, 399, 401, 420, 421, 422, 601, complications.76,91,212,453,677 The improved correction of
612, 633, 654, 696, 793, 812, 842, 848. deformity and maintenance of the correction achieved with

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CHAPTER 12  Scoliosis 239


FIGURE 12-36  Thoracic pedicle screw fixation. A, Preoperative

posteroanterior (left) and lateral (right) radiographs of a 13-year-old girl
with a double thoracic (Lenke type 2A) curve pattern. The main thoracic
curve measures 62 degrees, and the upper thoracic curve measures 40
degrees. B, Supine-bending radiographs to the left (left) and to the right
(right) demonstrate improvement of the main thoracic curve to 37
degrees and the upper thoracic curve to 36 degrees. C, Postoperative
C posteroanterior (left) and lateral (right) radiographs demonstrate excellent
coronal correction and maintenance of the sagittal profile.

lumbar pedicle screws led to their use in the thoracic spine compared with more traditional hook constructs.§b The
(Fig. 12-36). Suk and coauthors first reported the routine improved coronal plane correction achieved with pedicle
use of pedicle screws in the thoracic spine for spinal defor- screw fixation can be attributed to several factors. First,
mity surgery in 1995 and achieved improved coronal plane surgeons generally place pedicle screws at more levels than
correction in the screw group (72%) versus hooks (55%) when hooks or other anchors are used; second, the three-
and hybrids (66%).750 The initial reports of their safety column fixation of the spine provides better “grip” of the
demonstrated overall good results, with a pedicle screw vertebrae, so correction maneuvers yield greater improve-
breech rate of between 1.5% and 15% and few neurologic ment in the spinal deformity; third, the use of procedures
complications.2,52,186,395,748 The learning curve is steep, and to mobilize the spine has expanded and included greater use
greater surgeon experience leads to improved accuracy.2,669 of Ponte-style or Smith-Petersen osteotomies, concave and
Reports of improved correction of spinal deformity have led convex rib osteotomies, and for very severe curves, use of
to fairly enthusiastic adoption of the technique by surgeons,
with overall improved radiographic correction when §b
References 100, 380, 381, 395, 440, 605, 715, 729, 752, 798.

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240 SECTION II  Anatomic Disorders

the VCR procedure; and finally, with improved fixation, the to the width, height, and depth of the pedicles in the
surgeon can use a number of correction strategies, including various regions of the thoracic spine.‖b In addition, an
the traditional rod rotation maneuver, segmental distraction understanding of the surrounding anatomic structures later-
or compression, and segmental in situ bending. Fixation of ally and medially is important to avoid injury.439,735 In
each vertebra in the instrumented segment may be an general, the width of the thoracic pedicle is smaller in the
important factor when coronal plane correction with tho- proximal part of the thoracic spine, on the concavity of the
racic pedicle screws is analyzed. However, the appropriate upper and main thoracic curves, and with greater curve
screw “density” (the number of pedicles filled with pedicle magnitude. The spinal cord is positioned adjacent to the
screws relative to the total number that are available) is not concave pedicles with less than 1 mm of epidural space, as
well known, and early studies demonstrated conflicting compared with 3 to 5 mm on the convex side.439 At the
results, with some showing a positive effect with improved apex of a right thoracic scoliosis, the aorta is positioned
coronal correction136 and others demonstrating no differ- more lateral and posterior to the vertebral body than in a
ence between high and low screw density.69 In North normal, straight spine.439,735 The combination of narrow
America, longer-term follow-up has demonstrated mainte- pedicles, dural sac proximity medially, and aorta proximity
nance of correction with nearly 70% coronal plane laterally makes safe screw placement challenging on the
correction.411 concavity of these thoracic curves. The most challenging
Perhaps the greatest advantage of thoracic pedicle screws pedicles are those in the proximal part of the thoracic spine,
is improved axial plane correction. Lee and associates especially on the concave aspect. The freehand technique
described a DVR maneuver in which the concave and entails identifying the starting point for screw insertion,
convex screws in the juxtaapical vertebrae were rotated decorticating that level with a burr, entering the cancellous
opposite the direction of the rod rotation maneuver.410 With channel with a pedicle finder, and traveling down the pedicle
CT they demonstrated better apical rotation in the group via manual pressure. The channel is then probed to ensure
that underwent DVR than in those who did not (42.5% that all five walls (anterior, medial, lateral, superior, and
versus 2.4%). The technique of apical derotation can be inferior) of the pedicle are intact, followed by tapping the
performed via a number of methods, and all have demon- pedicle, reprobing to ensure maintenance of the pedicle
strated some success in improving the rotational deformity walls, and placing the screw. Fluoroscopy or plain radiogra-
in a variety of AIS curves.185,330,331 Use of this technique may phy is then used to check the position of the screws. Kim
obviate the need for thoracoplasty and the associated and co-workers reported on 3204 screws placed in the
detrimental effect on pulmonary function (see Plate 12-3 thoracic spine for spinal deformity and randomly analyzed
on page 297).156,354 However, care in maintaining thoracic 577 screws with CT imaging.379 They demonstrated that
kyphosis when performing these DVRs is necessary to avoid 6.2% of the screws had moderate cortical perforation,
creation of lordosis as one pushes anteriorly on the spine. including 1.7% with medial wall violation; however, no neu-
This loss of thoracic kyphosis is accompanied by a loss of rologic or vascular complications occurred. Pedicle screws
lumbar lordosis.550 Large-diameter rods with an accentuated have been placed successfully in patients with severe defor-
thoracic kyphosis contour help maintain the thoracic kypho- mities and scoliosis curves greater than 90 degrees, with a
sis with the posterior approach,512 whereas the anterior thoracic pedicle screw accuracy rate of 96.3% and no neu-
approach maintains kyphosis because correction of the rologic complications.395
coronal plane is achieved with shortening of the anterior Image-Guided Techniques. A variety of image guidance
column.734 It is important to preserve the tension band of techniques have been described, from plain radiography
the soft tissues proximal to the planned instrumented levels following guidewire placement to continuous stepwise fluo-
to prevent junctional kyphosis, which has been reported roscopic evaluation299,398,748 to true surgical navigation using
with the use of pedicle screws.306 preoperative or intraoperative CT scanning and intraopera-
Surgical treatment of spinal deformity has three main tive computer navigation.¶b Suk and colleagues described a
aspects. The first is to grab onto the spine with anchors method in which anatomic landmarks are used to identify
strategically placed on the spine, typically with pedicle the starting point and guidewires are drilled into the center
screws today. The second is to mobilize the spine when of the pedicle, which are then visualized on a plain PA
necessary through a variety of techniques, including intra- radiograph.748 Based on these radiographic images, adjust-
operative traction, multiple Ponte-style osteotomies, or ments are made in the position of the guidewires before
even resection procedures. Finally, once the anchor points placing the screws. A review of their experience in placing
are placed and the spine is flexible (either inherently so or 4604 thoracic pedicle screws in 462 patients demonstrated
following mobilization techniques), a variety of surgical that 1.5% of the screws were malpositioned in 10.4% of the
techniques can be used to improve the spinal deformity. patients, with only 4 screws (0.09%) placed medially. One
Thoracic pedicle screws can be placed in several ways, patient experienced transient paraparesis, and three had
but generally two main methods are used: freehand, in dural tears.
which the screws are placed without the use of fluoroscopic True image guidance systems rely on either a preopera-
guidance, and image guided, in which the screws are tive or an intraoperative CT scan to define the spinal
inserted under the guidance of some radiographic imaging anatomy (initial data acquisition), followed intraoperatively
modality.379 by image-to-patient registration. Surgical navigation is
Freehand Technique. The freehand technique relies on a then performed during placement of the pedicle screws.
thorough understanding of pedicle anatomy in the thoracic
spine, including the anatomic landmarks for the starting ‖b
References 391, 557, 577, 581, 582, 790, 791, 880, 881.
point and the trajectory and general guidelines with regard ¶b
References 6, 247, 280, 355, 402, 618, 866.

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CHAPTER 12  Scoliosis 241

Early results demonstrated similar or improved screw constructs because of the higher cost per implant, as well
accuracy when compared with more conventional as the increased number of screws used in each case.639
imaging.247,280,402,406,618 One study reported that a potentially
unsafe screw was 3.8 times less likely to be inserted with Posterior Mobilization Techniques. Removal of the inferior
navigation and that the chance of a significant medial breach facet at all instrumented levels has been the standard tech-
was 7.6 times higher without navigation.786 Alternatively, nique to achieve fusion when performing surgery to correct
CT imaging may be used to confirm accurate screw place- spinal deformity. The opportunity for greater three-
ment, although the systems currently available provide less dimensional spine correction with the use of pedicle screws
detail than a typical CT scanner found in radiology suites has been accentuated by a trend toward performing more
does. All these image guidance techniques expose the aggressive spine mobilization procedures, thereby providing
patient and surgeon to increased radiation, which may have an opportunity to gain greater correction of deformity.22
long-term effects.346,660,709 Beyond standard facetectomies, the gradual resection of
Other Methods of Assessing Screw Placement. Other more posterior structures provides greater spine mobility in
methods to determine safe and accurate screw placement the following order: complete facetectomies/ligamentum
include EMG stimulation, which has produced reliable release (Ponte or Smith-Petersen osteotomies), concave or
results and clear guidelines when lumbar pedicle screws are convex rib resections (or both), vertebral body decancella-
within the pedicle.137,427,690 The premise of this technique is tion with wedge resection of the vertebra, pedicle subtrac-
that when the electrical current is passed through a com- tion osteotomy, and finally, complete VCR.
pletely intraosseous pedicle screw, it will not result in a A Ponte-style osteotomy generally refers to complete
triggered EMG peripheral response. With greater stimulus removal of both the superior and inferior facets at all levels,
intensity, however, even a well-placed screw will trigger a typically in the setting of Scheuermann kyphosis. However,
peripheral response, so guidelines have been established this nomenclature has been applied to the scoliotic spine
based on the level of stimulation required to elicit a response: deformity as well, especially when the anterior column is
greater than 8 mA defines a screw completely within the unfused. The Smith-Petersen osteotomy, in contrast, typi-
pedicle; between 4 and 8 mA is intermediate, which means cally refers to performance of these complete facetectomies
that the screw should be removed and the medial wall in the setting of a fully or partially arthrodesed anterior
probed; and less than 4 mA indicates a strong likelihood of column. The ability to achieve correction with Ponte or
a pedicle wall defect. Although good success has been Smith-Petersen osteotomies for AIS was characterized well
achieved in the lumbar spine, studies involving the thoracic for thoracolumbar and lumbar curves when it was first done
spine have not been able to clearly define threshold values on the convex spine, with overall 80% correction of these
for determining a safe screw.433,620,624 The technique of curves.699 Correction of thoracic curves with these osteoto-
EMG stimulation for ascertaining thoracic pedicle screw mies is most likely less than that seen in the lumbar spine
placement is technician dependent and takes some experi- because the ribs and chest wall limit correction and blood
ence to perform. It should serve only as an adjunct to loss and operative time can be higher.284 However, bilateral
thorough understanding of the pedicle anatomy, meticulous complete facetectomies allow one to lengthen the posterior
surgical technique, and good imaging. column to restore thoracic kyphosis while also “untether-
The improved radiographic correction seen with the use ing” the concave spine to allow shortening and correction
of thoracic pedicle screws has not been directly associated of the coronal plane deformity. Following complete removal
with improved clinical outcomes. Comparisons of thoracic of the inferior facet, the ligamentum flavum is removed
pedicle screws and hook constructs or a hybrid construct with a large Leksell rongeur in the midline until epidural fat
(hooks proximally and screws distally) have demonstrated is seen, and completion of the flavectomy is then performed
no difference in 2-year postoperative scores and little or no with a Kerrison rongeur (Fig. 12-37). The superior facet on
correlation between coronal plane correction in AIS and the the convex side can be largely removed with a small Leksell
SRS outcome instrument.158 rongeur because the dural sac is positioned closer to the
The advantages of improved radiographic correction concave spine. Care is taken to limit the depth of penetra-
when using thoracic pedicle screws for the treatment of tion of the rongeurs on the convex spine because the epi-
spinal deformity must be weighed against the risk and cost dural veins are more prominent on this side. The concave
of these implants. In most series the incidence of the most superior facet is more safely removed with Kerrison ron-
feared complications—neurologic injury and major injury to geurs because the dural sac is in close proximity and care
soft tissue structures, including the great vessels—is very must be taken to remain directly on the bone when resec-
low (<1%).#b A study of more than 19,000 patients who tion is performed. Subtle SSEP changes may be seen fol-
underwent surgery for pediatric spinal deformity demon- lowing these osteotomies.
strated fewer neurologic deficits with pedicle screws (0.7%) The next level of posterior release would be rib resec-
than with wires (1.7%).621 This may be due to the significant tions on the concave side of the spine to allow lateral and
experience gained by the initial users of the technique, as posterior translation of the spine. Three to five ribs are
well as the relatively easy transition from the lumbar spine resected by first performing subperiosteal dissection, typi-
to the thoracic spine. Neurologic injuries directly related to cally beginning a couple of centimeters lateral to the trans-
the use of thoracic pedicle screws have been reported, verse process and continuing for 3 to 5 cm. Because the ribs
however.15,580 The final consideration is the cost of the are close to the anterior aspect of the chest, small tears in
implants, which is higher than that of hook or hybrid the parietal pleura tend to occur and should be recognized
at the time of surgery; placement of a chest tube may be
References 51, 52, 379, 715, 729, 748, 798. required to prevent pneumothorax.206 The authors do not

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242 SECTION II  Anatomic Disorders

waking with a decline in neurologic function, which returned

to baseline.741
The indications for posterior VCR are not fully defined
for AIS; however, given its significant neurologic risk and
long operative times with associated potential for significant
blood loss and pulmonary complications, this procedure
should be chosen as the technique of last resort. The authors
generally use this procedure in individuals with very severe
deformity—angular scoliosis or kyphosis—especially when
previous fusion has been performed. This procedure should
be performed only by the most skilled and experienced
surgeons who have worked their way up the learning curve
by performing less arduous spine mobilization procedures.
A multicenter study of 147 pediatric VCR procedures per-
formed by senior surgeons reported complications in 59%
of patients, including 27% who had an intraoperative neu-
rologic event without paraplegia.425 The posterior approach
FIGURE 12-37  Ponte osteotomy. A Kerrison rongeur is completing to the VCR procedure begins with rigid fixation of the spine
removal of the posterior elements of the spine. Epidural fat is seen. with pedicle screws proximal and distal to the planned
resection levels (Fig. 12-38, A). Typically, long-tabbed
reduction screws are placed distal to the planned resection
generally perform these resections because experience has levels. The ribs associated with the resected levels are
shown limited appreciable correction and the risk for post- removed entirely, including the rib heads. A laminectomy
operative pulmonary issues and pneumothorax is high. is next performed to identify the neural elements. The
The VCR procedure is the most aggressive technique for authors prefer to ligate the nerve roots on the convex side
achieving correction of the spine because it removes one or first and to remove the pedicle or pedicles to the level of
more vertebral segments at the apex of the deformity. It the floor of the spinal canal (Fig. 12-38, B). A provisional
was initially described for severe spine deformity and trunk rod is then placed on the convex side and the concave
shift and used an anterior and posterior approach to the osseous elements are removed, including the pedicle and
spine, with overall outstanding radiographic and clinical the concave side of the vertebral body after the concave
results. Bradford and Tribus reported on 24 patients with nerve roots have been ligated and transected. The concave
severe curves in which greater than 80% correction of the pedicle and floor of the canal are very difficult to remove
coronal and sagittal plane translation was achieved along because the dural sac and spinal cord are tightly adherent
with 50% correction of the curves. Despite complications to these structures and the cancellous channel to the
in 14 of the patients, the overall satisfaction rate in patients concave pedicle is limited. Following complete resection of
was extremely high.84 Suk and co-workers described the the concave bone, a second provisional rod is secured so
posterior-only approach for the VCR procedure and that two provisional rods (one on the concave and one on
reported 62% correction in the coronal plane and 45 degrees the convex side) are in place (Fig. 12-38, C). The remainder
of correction in the sagittal plane in 70 patients with severe of the convex bony anatomy can be removed with both rods
deformity from kyphoscoliosis, postinfectious kyphosis, and in place because the convex bone is anterior to the convex
adult scoliosis. Similar to previous reports, the complication rod. Following complete resection, exchange of the provi-
rate was relatively high, with 24 patients having complica- sional rods for the final rods is accomplished. The authors
tions, including 2 with postoperative spinal cord deficits typically remove the convex provisional rod and replace it
who had neurologic deficits preoperatively.745 Lenke and with an undercontoured final rod that is seated in the proxi-
coauthors reported their experience in performing posterior mal screws and secured firmly and then partially seated in
VCR for pediatric spinal deformity in 35 consecutive the tips of the first two reduction screws distal to the
patients. The patients were divided into five diagnostic cat- resected area (Fig. 12-38, D). The final concave rod is
egories, including severe scoliosis, global kyphosis, angular placed similarly. The distal aspects of the two final rods are
kyphosis, kyphoscoliosis, and congenital scoliosis, with then pushed anteriorly to unhinge them from the set screws
reported correction of 51%, 55%, 58%, 54%, and 60%, on the reduction screws while still providing some guidance
respectively. No spinal cord–related complications were to the spine segments. The spine can then be manually
reported; however, two patients lost intraoperative neuro- compressed without jeopardizing the screw–bone interface.
monitoring data during correction with return of baseline Before this manipulation an anterior cage is placed in the
monitoring, which resulted in prompt surgical interven- resected area to maintain the length of the spine and to
tion.426 The authors’ experience is similar in 32 consecutive avoid kinking of the dura and potential neurologic deficits
VCR procedures: 4 were done via an anterior/posterior (Fig. 12-38, D). Sequential reduction of the distal aspect of
approach and the remainder via a posterior-only approach, the rod to the remaining screws is performed, compression
with coronal plane correction from 122 degrees to 53 is subsequently applied to achieve the final correction, and
degrees and an average increase in T1 to T12 height of the rib grafts initially harvested at the time of resection are
6.1 cm associated with improvement in pulmonary func- placed over the exposed dura (Fig. 12-38, E).
tion. The incidence of intraoperative changes in neuromoni- The choice of spine mobilization procedure is dependent
toring parameters during surgery was 35%, with two patients on several factors related to the spine deformity, including

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CHAPTER 12  Scoliosis 243


FIGURE 12-38  Vertebral column resection. A, Proximal (right) and distal fixation are seen with the planned removal of thoracic vertebrae
6 and 7 uninstrumented. B, A provisional right rod is in place and the seventh thoracic nerve root on the left convex side is being
isolated and ligated. C, At completion of the resection, two provisional rods are in place and the spinal cord is completely free. A metal
suction tip is seen anterior to the spinal cord. D, The spine has been reduced with the anterior cage in place to maintain overall length of
the spine and prevent shortening with kinking of the spinal cord. E, Final intraoperative photo with the rib grafts in place spanning the
resected segment and protecting the exposed spinal cord.

its severity, flexibility, amount of previous fusion, and expe- thoracic pedicle screws and the use of rods with various
rience of the surgeon. The major risk is neurologic deficit, characteristics of stiffness and strength, together with spine
which occurs more commonly with more aggressive release mobilization techniques and more advanced instrumenta-
procedures. The most recent review of the SRS database tion, provide an opportunity to better achieve axial plane
demonstrated an overall 0.9% incidence of neurologic defi- correction.
cits for all AIS surgery; however, the incidence increased Rod rotation continues to be used as the initial step in
when a Smith-Petersen osteotomy was performed (1.1%) correction and is maximized with segmental fixation and
and was greatest when VCR was performed (7.3%).621 larger-diameter rods. Alternatively, the apical reduction
screws can be used to translate the apex of the spine pos-
Anterior Mobilization Techniques.  Anterior release includ- teriorly and laterally with an overcontoured rod that is fixed
ing discectomy, and possibly rib head resection when neces- in its final position proximally and distally. This requires
sary, can be performed through an open thoracotomy or significant releases for stiff spines to allow this translation
through a thoracoscopic approach. The thoracoscopic to occur. A stiff cobalt-chrome 6.25-mm rod is preferred
approach has some advantage over the open thoracotomy in general by the authors, especially for severely hypoky-
because smaller incisions are used with less chest wall dis- photic spines.
ruption and less detrimental impact on pulmonary function, Following initial placement of the left rod, in situ coronal
especially when it is performed in the prone position with bending can be performed together with compression on
double lung ventilation using a regular single-lumen endo- the convex side and distraction on the concave side. At this
tracheal tube (Video 12-1). The combined thoracoscopic point, axial plane correction at the apex can be performed
release in the prone position and posterior instrumented with a variety of techniques to correct this component of
fusion has resulted in overall excellent radiographic and the deformity via DVR, which allows one to directly rotate
clinical results. each spinal segment individually, or, with the use of connec-
tors for the DVR instruments, en bloc rotation can be
Correction Techniques performed. A counterforce proximal and distal to the apex
With introduction of the CD implant system, the 90-degree should be imparted to most effectively improve the axial
concave rod rotation maneuver was used in an attempt to plane deformity. The set screws of the apical segments
improve the three-dimensional deformity. The rod was must be loosened on the concave rod to allow maximal axial
shaped to the coronal plane deformity, and for the typical plane correction. The DVR technique is most effective
right thoracic curve, the left rod was rotated counterclock- when only a single rod is in place to allow maximal correc-
wise so that the scoliotic bend in the rod became the tion. The axial plane correction maneuver can be performed
kyphotic bend in the rod to maintain or restore kyphosis. segmentally, with each screw head being manipulated indi-
Excellent coronal and sagittal correction could be vidually to correct the axial plane (Fig. 12-39, A and B), or
achieved with hook constructs; however, the axial plane the screws can be linked together and rotated en bloc
correction was somewhat limited. The introduction of (Fig. 12-39, C and D).14,513,744 Suk and colleagues reported

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244 SECTION II  Anatomic Disorders


FIGURE 12-39  Direct vertebral rotation can

be performed in several ways, including
those shown in A and B. For segmental
rotation, the manipulation sticks are
attached to the convex screws and the
spine is derotated. C and D, For en bloc
rotation, the convex and concave screws  
are connected to allow rotation of the entire
apical segments. A counterforce should be
applied above and below these segments to
prevent imparting the derotation forces to C D
the adjacent segments.

greater coronal plane correction and rib hump height cor- The most feared complication in spine deformity surgery
rection when the DVR technique was used together with is neurologic deficit, whose incidence has remained steady
thoracoplasty than when thoracoplasty was used alone.746 through the years and is still below 1%.138,472,621 Reames and
coauthors reported the most recent analysis of the SRS
Complications of Posterior Instrumentation and Fusion database, which found a 0.8% incidence of neurologic deficit
for Adolescent Idiopathic Scoliosis. The primary complica- at the time of surgery for AIS.621 The likelihood of complete
tions associated with posterior surgery and instrumentation or partial recovery of neurologic deficits is high in all series.
include infection, pseudarthrosis, neurologic deficit, and The incidence of neurologic deficit is generally regarded to
implant-related problems such as prominence, discomfort, be higher with combined anterior/posterior surgery and
and implant failure associated with pseudarthrosis. The when osteotomies are performed and is generally thought
overall incidence of reoperation after posterior instrumenta- to be of vascular origin.88,621
tion and fusion varies but is below 10% in more recent
series.30,116,242,465,617 A 1% to 10% incidence of delayed Anterior Spinal Instrumentation
wound infection has been reported, presumably related Anterior Approach to Thoracolumbar and Lumbar Curves.
either to the increased amount of hardware or to the mul- The anterior approach to thoracolumbar and lumbar curves
tiple hook–rod connections.*c Some of these episodes of has been successful in correcting deformity and preventing
delayed drainage were culture negative and attributed to curve progression. However, its use for these curves has
micromotion at the hook–rod interface.155,197,820 Micromo- diminished with greater use of pedicle screws and spine
tion causes metallic debris, which leads to a foreign body mobilization procedures. Shufflebarger and associates
reaction, formation of a false membrane and fluid, and reported 80% correction of these curves with a posterior
finally loosening of the implant. Rather than representing approach that involved the use of segmental pedicle screws
an aseptic process, it is more likely that these delayed infec- at each level with wide Ponte-style osteotomies and convex
tions result from low-virulence organisms that are seeded compression.698,699 Comparison studies between the poste-
at the time of surgery and remain quiescent over an extended rior and anterior approaches have demonstrated similar
period.135,627,630,800 levels of instrumentation with similar correction of defor-
Risk factors for the development of a delayed infection mity; however, the posterior approach is associated with a
are the presence of a significant past medical history, receiv- shorter operative time and hospital stay without the need
ing a blood transfusion, and not having a deep drain placed.313 for a chest tube or a general surgeon to perform the surgical
The incidence of pseudarthrosis is very low with modern approach.101,254 In general, we treat all thoracolumbar
double-rod systems, segmental instrumentation, and the and lumbar curves via a posterior approach except when
use of allograft bone. the planned LIV is significantly translated from the CSVL,
in which case the anterior approach with complete diskec-
tomies and powerful derotation of the spine may allow
better correction of the three-dimensional deformity of
*cReferences 135, 197, 627, 630, 640, 800, 820. the spine.

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CHAPTER 12  Scoliosis 245

FIGURE 12-40  A, A 14-year-old female with 56 degrees thoracolumbar idiopathic scoliosis. She underwent anterior instrumentation
between T10 and L3, with autogenous bone graft and spacers between L1-L2 and L2-L3. At 2 years postoperative, she had a balanced
spine and solid fusion (B and C).

Solid-Rod Anterior Instrumentation. TSRH instrumenta- stiffness of dual-rod systems is better than that of single-rod
tion extended the concepts of Zielke by using a stiff, systems in flexion, extension, and torsion, with no increase
smooth, solid rod as the longitudinal connection between in lateral-bending stiffness.246,558,692,693 The addition of ante-
vertebral screws (Fig. 12-40).343,345,781 The resulting stiffer, rior structural support to a dual-rod system increases overall
fatigue-resistant construct enhances the maintenance of construct stiffness in flexion but not in other loading
correction and the likelihood of arthrodesis without post- conditions.246
operative external immobilization in most cases. Deformity The surgical technique for dual-rod systems is to initially
is corrected by rotation of a 6.4-mm rod that is precon- place two screws in each vertebra, with the posterior screw
toured for lordosis (similar to the CD instrumentation traversing along the posterior cortex of the vertebral body
principles for thoracic curves posteriorly, but in reverse). and the anterior screw directed in an anterior-to-posterior
Because correction of deformity is achieved by gradual rod direction to provide increased pullout and plow resistance
rotation, corrective forces are evenly distributed all along (see Plate 12-4, I-M on page 306.) A staple is similarly used
the construct simultaneously rather than applied acutely or to capture both screws. A contoured rod with the desired
gradually at a single segment. lordosis is placed into the posterior screws, and a rod rota-
The early results of anterior single-rod treatment of tho- tion maneuver is performed in a similar manner as a single-
racolumbar and lumbar curves included a high incidence of rod technique to correct the scoliosis and restore or maintain
pseudarthrosis, which improved with rib strut grafts.570,781 lordosis. Anterior structural support in the form of a tita-
The addition of anterior interbody structural support in the nium mesh cage filled with the autologous rib graft har-
form of a titanium cage or femoral ring allograft significantly vested during the surgical approach is next placed to help
increased the flexion–extension stiffness of the construct maintain lordosis and assist in achieving correction of scolio-
when using single-rod implants,246,463,558 and it appears to sis. The second rod is then placed and slight compression is
increase the likelihood of fusion. It is important to place applied to lock in the anterior structural support. Kaneda
the structural interbody support in the most anterior aspect and associates reported their results with Kaneda’s dual-rod
of the intervertebral disk space to obtain optimal load system without anterior structural support in 25 patients
sharing602 and in the concavity of the curve to aid in coronal (20 with idiopathic scoliosis and 5 others) with thoracolum-
plane correction. The concept of dual-rod constructs bar or lumbar curves; they demonstrated average coronal
for anterior scoliosis surgery was popularized by Kaneda correction of 83%, restoration of 9 degrees of lordosis
as a method of increasing the stiffness of the single-rod (7 degrees of preoperative kyphosis), and no cases of pseud-
construct while maintaining coronal and sagittal plane arthrosis.357 Others have demonstrated excellent correction
correction and preventing pseudarthrosis.319,320,357 The and maintenance of correction with a dual-rod system.93,101

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246 SECTION II  Anatomic Disorders

The Halm-Zielke system is composed of a lid plate fixed to including the relationship between the rib head and the
the lateral aspect of the vertebral body with two screws—a vertebral body. The correct starting point for the screw
screw sunk anteriorly and a second ventral derotation spon- must be identified to avoid the spinal canal.877 On the con-
dylodesis screw posteriorly. This low-profile system has tralateral side of the chest, the aorta is positioned more
yielded excellent correction with few complications.94 The laterally and posteriorly than is the case with a straight,
dual-rod systems emphasize the importance of construct normal spine. This exposes the aorta to a higher risk
stiffness and the stability provided by the second rod. for injury when placing anterior screws.735 Transvertebral
Fusion levels for thoracolumbar and lumbar scoliosis are anterior screws should penetrate the far cortex only
generally from the proximal end vertebra to the distal end slightly to avoid injuring these important soft tissue
vertebra, as assessed on the preoperative PA radiograph. structures.93,394,652,739
Deciding on the distal fusion level is challenging because The traditional surgical approach for ASFI of the tho-
of the relatively common occurrence of a postoperative racic spine was through an open thoracotomy incision (Fig.
oblique disk (which opens into the convexity of the curve) 12-41). Thoracoscopic ASFI was first introduced in the
below the fusion levels. This problem is more likely to mid-1990s following experience with thoracoscopy for
occur when the disk below the distal end vertebra is parallel anterior release and fusion in conjunction with posterior
and the LIV is closer to the apex of the curve on the pre- spinal fusion and instrumentation.63,153,326,599,732 Advantages
operative radiograph.675 Despite these challenges, relatively of the thoracoscopic technique include smaller incisions,
good success has been achieved with the so-called short- which results in improved cosmesis, and less chest wall dis-
segment anterior fusion for mild to moderate curves that section, which results in both less postoperative pain and
are relatively flexible.61,74,90 The fusion levels are based on improved pulmonary function.221,412 The disadvantage of the
the level of the apex of the deformity: when a disk is the technique is that it is technically demanding, which results
apex, fusion should be two levels above and two levels in a fairly steep learning curve, long operative times, and a
below the disk; when the apex is a vertebra, fusion is one relatively high pseudarthrosis rate.409,449,548,742
level above and one below the apex. The success of this Ideal patients for thoracoscopic ASFI include those with
surgical strategy is dependent on achieving overcorrection good pulmonary function; active patients with high bone
of the instrumented segments to translate the spine and mineral density for good screw purchase; tall, thin patients
balance the patient.61 to allow easy portal placement and manipulation; and those
with thoracic curves that have a coronal magnitude of less
Anterior Instrumentation for Thoracic Deformity. Dwyer
and colleagues proposed this technique in the 1960s but
found that correction was unsatisfactory with the cable
systems.200 Since then, use of anterior instrumentation for
thoracic deformity has reemerged. In 1988, Harms began
repopularizing the idea after conjecturing that anterior cor-
rection without a posterior derotation maneuver of the tho-
racic curve in King type II deformities would prevent the
lumbar curve from decompensating, as had been described
following selective posterior instrumentation.64
The technique of anterior spinal fusion and instrumenta-
tion (ASFI) of thoracic curves achieves excellent coronal
curve correction and restoration of thoracic kyphosis, and
the lumbar curve responds nicely in selected thoracic fusion
situations.189,413,461,626,678 Advantages of the anterior approach
over the posterior approach include the ability to save
motion segments, no stripping of the paraspinal muscles and
thus avoidance of scarring and perhaps subsequent pain,
limited development of late operative site pain (seen with
posterior implants), and a lower incidence of infection. Betz
and colleagues, in a comparison of anterior and posterior
approaches for thoracic scoliosis, found 58% correction of
the coronal curve and an average of 2.5 levels that were
saved with the use of anterior surgery; however, a high
incidence (31%) of rod fracture occurred when a small-
diameter rod (3.2 mm) was used.65 More recent studies
using rods 4.0, 5.0, or 5.5 mm in diameter showed 47%
curve correction, but pseudarthrosis developed in 7% of
patients.753 A lower incidence of pseudarthrosis is seen
with dual rods, but the challenge of placing two screws in FIGURE 12-41  Anterior thoracic instrumentation. A threaded
the thoracic spine may prevent widespread use of this rod is attached to vertebral body screws on the convexity of  
technique.358 the thoracic curve. A shorter segment of thoracic fusion can  
When placing anterior instrumentation in the thoracic be accomplished with this system than with posterior
spine it is necessary to understand the anatomic landmarks, instrumentation.

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CHAPTER 12  Scoliosis 247

FIGURE 12-42  Thoracoscopic anterior spinal fusion and instrumentation for a thoracic curve. A, Preoperative posteroanterior (left) and
lateral (right) radiographs demonstrate a 58-degree right thoracic curve with the end vertebra at L2 and the stable vertebra at L4.
B, Posteroanterior (left) and lateral (right) radiographs following thoracoscopic anterior spinal fusion and instrumentation from T7 to T12.
Anterior correction mechanics allowed the fusion to stop at L2 in this case despite significant translation from the center sacral line.

than 70 degrees and a flexibility index greater than 50%. two other factors once thought to be associated with a poor
Follow-up has demonstrated that the results of thoraco- prognosis—thoracic kyphosis of less than 20 degrees and
scopic AFSI are similar to those of open ASFI, with 55% left-sided curves in boys—is uncertain.
coronal plane correction and a pseudarthrosis rate of 6%.546
When compared directly with a posterior approach, thora-
Neural Axis Abnormalities
coscopic ASFI is associated with similar coronal plane cor-
rection and less intraoperative blood loss but longer surgical MRI studies have provided greater insight into juvenile idio-
times (Fig. 12-42).448,844 pathic scoliosis.213,278,434,681,720 The incidence of neural axis
abnormalities in these patients is 18% to 26%. Most of these
children are asymptomatic and have no physical signs (other
Juvenile Idiopathic Scoliosis than scoliosis) of an underlying neural axis abnormality. MRI
abnormalities include Chiari type I malformations with cer-
The prevalence of juvenile idiopathic scoliosis (3 to 10 years vical syrinx, thoracic syrinx, brainstem tumor, dural ectasia,
of age) is 8% to 12% in Europe and 13% to 16% in the and low-lying conus. Many of these abnormalities may
United States.338,649 The deformity is usually recognized benefit from neurosurgical treatment. As a result, some
clinically by the age of 6 to 7 years.486 The female-to-male authors recommend MRI during the initial evaluation of
ratio ranges from 1.6 : 1 to 4.4 : 1, with the difference patients presumed to have juvenile idiopathic scoliosis. If
increasing with age.234,649 Convex right thoracic curve pat- scoliosis surgery is planned, it is imperative that preopera-
terns are most common, and relatively few patients have tive MRI evaluation be undertaken. Neurologic deficits
thoracolumbar or lumbar curves. following spinal surgery have been reported in patients
with neural axis abnormalities that were not recognized
Predicting Curve Progression
Treatment options for children with juvenile scoliosis are
Juvenile scoliosis is more likely to progress, less likely to outlined in the section “Early-Onset Scoliosis.” As the child
respond to bracing, and more likely to require surgical treat- approaches 8 to 10 years of age, a definitive surgical proce-
ment than AIS is.490,828 Unlike infantile scoliosis, use of the dure should be considered (Fig. 12-43).
rib–vertebral angle difference (RVAD) does not predict
curve progression in juvenile scoliosis.770 Patients with pro-
gressive curves have a steady increase in the RVAD, whereas Congenital Spinal Deformities
those whose curves will resolve usually show a decrease in
the RVAD. If the RVAD does not improve following bracing Congenital deformities of the spine are caused by anomalies
of a progressive curve, spinal fusion will probably be required in the growing vertebrae. These anomalies may be subtle
as definitive treatment. The level of the most rotated ver- and found incidentally on radiographs obtained for some
tebra at the apex of the primary curve appears to be the other reason, or they may be complex and lead to severe
most useful factor in determining the prognosis of patients spinal deformity with accompanying neurologic deficits.
with juvenile idiopathic scoliosis. Those with a curve apex Congenital scoliosis, congenital kyphosis, and a combination
at T8, T9, or T10 have an 80% chance of requiring spinal of the two are the deformities encountered. They are much
arthrodesis by 15 years of age.649 The predictive value of less common than idiopathic scoliosis.

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248 SECTION II  Anatomic Disorders


FIGURE 12-43  Treatment of juvenile scoliosis. A skeletally immature patient underwent anterior and posterior spinal fusion to prevent
progressive changes caused by the crankshaft phenomenon. Preoperative radiographic (A) and clinical (B) appearance at the age of
8 years 6 months. Radiographic (C) and clinical (D) appearance 7 months after surgery. Radiographic (E) and clinical (F) appearance
6 years after surgery. Although the trunk is slightly shorter than normal, it is without deformity. (B, D, and F, From Richards BS: The
effects of growth on the scoliotic spine following posterior spinal fusion. In Buckwalter JA, Ehrlich MG, Sandell LJ, et al, editors: Skeletal
and growth development: clinical issues and basic science advances, Rosemont, Ill, 1998, American Academy of Orthopaedic Surgeons,
p 585.)

increase in exposure to fumes (chemical fumes and carbon

monoxide) in the mothers of children with congenital spinal
The cause of congenital vertebral anomalies remains deformities.445
unknown. During embryologic development, these abnor- Investigation of genetic causes has provided modest
malities develop in the spine between the fifth and eighth insight. A positive family history can be found in approxi-
weeks of gestation, but it is very uncommon to identify any mately 1% of patients with congenital spinal deformities.823
traumatic or teratologic type of maternal insult during this Idiopathic scoliosis has been reported in 17% of families of
stage of pregnancy. children with congenital scoliosis.613 An isolated anomaly,
Research has found that carbon monoxide exposure and such as a hemivertebra, usually occurs as a sporadic event
the resulting hypoxia can lead to reproducible congenital and carries no risk for a similar abnormality in other off-
spinal deformities in mice offspring.218,446 Such deformities spring.853 Studies of identical twins, only one of whom was
include wedged vertebrae, hemivertebrae, fused vertebrae, affected, showed no genetic pattern,296,596,604 but other
and missing vertebrae, as well as fused ribs. The severity reports of twins with similar congenital deformities sug-
of the deformities is related to both the dose of carbon gested the possibility of genetic causes.237,517,731 Scientists
monoxide and the time during gestation that exposure have identified the human gene HuP48, a member of the
occurred. Correlating with this basic science study, the Pax family of developmental control genes, as having a role
same institution reported clinical data indicating a potential in establishing the segmented pattern of the vertebral

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CHAPTER 12  Scoliosis 249



FIGURE 12-44  A and B, Congenital spinal deformity in a girl aged 12 years 11 months
with normal findings on neurologic examination. Significant rotational deformity is evident
clinically. C and D, Radiographs demonstrate numerous congenital thoracolumbar
abnormalities associated with the 65-degree scoliosis. E and F, Preoperative magnetic
resonance imaging demonstrates a large diastematomyelia (arrow) at the second lumbar
D vertebra and resulting diplomyelia. Both are well visualized on the transverse (E) and
sagittal (F) images. Postoperative results are shown in Figure 12-55.

column.711 As yet, no mutations in this gene have been rent epigenesis.266 The most common associated finding
found in those with vertebral segmentation defects. A chro- is intraspinal anomaly, a general category that includes
mosomal aberration, deletion of 17p11.2, has been reported numerous abnormalities such as tethered cord, diastemato-
in congenital scoliosis but needs further verification.334 myelia, syringomyelia, diplomyelia, Arnold-Chiari malfor-
Analysis of the candidate gene DLL3 has raised the possibil- mations, and intraspinal tumors.†c The incidence of one of
ity of its involvement in congenital scoliosis.211,483 However, these associated neural axis abnormalities developing ranges
no definitive cause of anomalous vertebral development has from 21% to 37%. All these abnormalities are best identified
yet been established. with MRI.
Once an intraspinal abnormality (such as a diastemato-
myelia spur) has been identified, it should be addressed
Associated Abnormalities
neurosurgically if a progressive neurologic deficit has devel-
The neural axis, vertebral column, and other organ systems oped or if surgical correction of the scoliotic deformity is
develop at a similar stage in utero. A nonspecific insult needed (Fig. 12-44).498,554 To some physicians the mere
during this embryonic period has been suggested to desta- presence of a potentially tethering intraspinal lesion may be
bilize the developmental control systems and may result in
congenital malformation of any organ undergoing concur- †c
References 47, 53, 83, 498, 500, 607, 608, 743.

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250 SECTION II  Anatomic Disorders

sufficient reason for prophylactic surgical treatment. The scoliosis can be difficult. Close examination of radiographs
rationale for this early aggressive approach is to address the usually reveals the vertebral abnormalities present in chil-
lesion before the development of any neural dysfunction.177 dren with congenital scoliosis.
Any of these neural axis lesions may be associated with a The variety of vertebral anomalies that can exist in those
more visible clinical abnormality such as a hairy patch, a with congenital scoliosis leads to an unpredictable natural
nevus, or a distinct neurologic deficit. Subtle deficits can history. The deformity may remain mild, or it may progress
also be present, thus making a careful neurologic examina- dramatically over time and ultimately result in severe spinal
tion imperative for any patient with a congenital spinal deformity and pulmonary compromise.156 Understanding
abnormality. In view of the relatively high incidence of which vertebral anomalies place the scoliotic spine at great-
intraspinal anomalies and the fact that clinical manifesta- est risk for progressive deformity allows the treating physi-
tions may not be detectable initially, MRI has been recom- cian to intervene at the appropriate time.
mended as part of the initial evaluation in all patients with
congenital spinal deformities, even in the absence of clinical Classification
findings. Two basic types of abnormalities lead to congenital scoliosis:
In addition to neural axis abnormalities, approximately defects of vertebral formation and defects of vertebral seg-
60% of patients have associated abnormalities affecting mentation (Fig. 12-45). Hemivertebrae and wedged verte-
other systems.49 Approximately 20% of patients have an brae are examples of defects of formation. Defects of
anomaly of the genitourinary system,47,150,201,275,473 and segmentation include block vertebrae, unilateral bars, and
cardiac anomalies are seen in approximately 12% to 26% of unilateral bars accompanied by hemivertebrae. Approxi-
patients.47,49,73 Other abnormalities include cranial nerve mately 80% of the vertebral anomalies associated with con-
palsy, radial hypoplasia, clubfoot, dislocated hip, Sprengel genital scoliosis can easily be classified into one of the two
deformity, imperforate anus, and hemifacial microsomia. types; the remaining 20% cannot be precisely classified.
Many patients have a combination of deformities in which
one type predominates.
Congenital Scoliosis
A newer classification system introduced in 2009 uses
Congenital scoliosis may not become evident until later three-dimensional CT images of congenital spinal deformi-
childhood, even though the vertebral anomalies are present ties.367,535 Four types of congenital vertebral abnormalities
at birth. In a child younger than 3 years, differentiation were introduced: type 1, solitary simple; type 2, multiple
between infantile idiopathic scoliosis and congenital simple; type 3, complex; and type 4, segmentation failure

Defects of Segmentation

Block vertebra Unilateral bar Unilateral bar and hemivertebra

Bilateral Unilateral
failure of failure of
segmentation segmentation

Defects of Formation
Hemivertebra Wedge vertebra

Unilateral partial failure
complete of formation
failure of

Fully segmented Semisegmented Incarcerated Nonsegmented

FIGURE 12-45  Congenital scoliosis: defects of formation and defects of segmentation. (Redrawn from McMaster MJ: Congenital scoliosis.
In Weinstein SL, editor: The pediatric spine: principles and practice, New York, 1994, Raven Press, p 229.)

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CHAPTER 12  Scoliosis 251

Table 12-4  Classification of Congenital Vertebral

Abnormalities (Based on Presence or Absence of
Abnormal Formation)
Type 1 Solitary simple (unison) type
  Wedged vertebra
  Butterfly vertebra
Type 2 Multiple simple (unison) type
  Combination of hemivertebra, wedge vertebra,
or butterfly vertebra
  Discrete, adjacent, or others
Type 3 Complex (discordant) type
  Mismatched complex type
  Mixed complex type
Type 4 No abnormal formation type
  Pure segmentation failure

From Kawakami N, Tsuji T, Imagama S, et al: Classification of

congenital scoliosis and kyphosis: a new approach to the three-
dimensional classification for progressive vertebral anomalies requiring
operative treatment, Spine 34:1756, 2009.

(Table 12-4). This improved three-dimensional understand-

ing of the variations in congenital scoliosis may be helpful
FIGURE 12-46  Spinal radiograph of a 2-year-old girl with
in preoperative planning for correction of these complex 53-degree congenital scoliosis and semisegmented hemivertebrae
deformities. at levels T8 and T10. A rib accompanies each of these
hemivertebrae. This deformity is partially balanced by a left-sided
Defects of Formation hemivertebra at the T4 level. The deformity slowly progressed to
Defects of formation may be partial or complete. Partial 61 degrees by 9 years of age, at which time she underwent
unilateral failure of formation produces a wedged or fusion.
trapezoid-shaped vertebra that contains two pedicles,
although one of them may be hypoplastic. The associated
scoliosis worsens slowly and may not require treatment. lumbosacral junction creates significant obliquity between
True hemivertebrae are caused by complete failure of the spine and pelvis and is usually accompanied by a long
formation on one side and result in laterally based wedges compensatory scoliosis in the lumbar or thoracolumbar
consisting of half the vertebral body, a single pedicle, and a region. This readily apparent deformity is best treated surgi-
hemilamina. Occasionally, the lamina associated with the cally (usually by hemivertebrectomy) at an early age, before
hemivertebra may be incorporated into that of the adjacent the compensatory curve becomes fixed (Fig. 12-47).
normal-appearing vertebra. When this occurs, differentiat- A semisegmented hemivertebra is separated from one
ing between the anterior vertebral abnormality and the adjacent vertebra (superior or inferior) by a normal verte-
corresponding posterior abnormality becomes difficult. bral growth plate and disk but is fused to the other adjacent
Hemivertebrae in the thoracic spine are usually accompa- vertebra. Although growth of the spine should remain bal-
nied by an extra rib. Hemivertebrae may be fully segmented anced, the hemivertebra can induce a slowly progressive
(most common), semisegmented, nonsegmented, or incar- scoliosis. Treatment is necessary only if the deformity is
cerated (least common) (Fig. 12-46). Distinguishing among progressive (see Fig. 12-46).
these various types is important because the associated dif- A nonsegmented hemivertebra is fused to both adjacent
ferences in growth potential have a profound effect on the vertebrae (above and below) and therefore has no vertebral
eventual severity of the spinal deformity. end-plates or adjacent disks. In the absence of any asym-
A fully segmented hemivertebra has the highest likeli- metric growth, a nonsegmented hemivertebra does not
hood of progressive deformity because it is separated from cause progressive spinal deformity. An incarcerated hemi-
the adjacent vertebrae by intact end-plates and interverte- vertebra is more ovoid and smaller than a fully segmented
bral disks. The hemivertebra is nearly always located at the (nonincarcerated) hemivertebra. The vertebrae above and
apex of the scoliosis. Lower thoracic and thoracolumbar below compensate for this hemivertebra, and as a result,
curves tend to worsen more rapidly than do curves at other minimal if any scoliosis is present.
levels. When two or more hemivertebrae are present on
the same side of the spine, the deformity progresses at a Defects of Segmentation
faster rate. Conversely, the spinal deformity may be bal- Defects of segmentation result in a bony bar or bridge
anced and nonprogressive if two hemivertebrae are situated between two or more vertebrae, either unilaterally or
opposite each other. A fully segmented hemivertebra at the involving the entire segment. Circumferential, symmetric

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252 SECTION II  Anatomic Disorders

Unilateral failure of segmentation of two or more verte-

brae (unilateral bar) is the most common cause of congeni-
tal scoliosis. Usually, a bar of bone fuses the disk spaces,
pedicles, and facet joints on one side of the spine, thus
precluding growth on the side of the concavity (Fig. 12-49).
Growth usually proceeds on the convexity and leads to
worsening of the deformity. Rib fusions or other rib abnor-
malities on the concavity of the scoliosis are often seen
adjacent to the bony bar bridging the vertebrae.
Some patients with unilateral failure of segmentation
have one or more hemivertebrae located on the opposite
(convex) side of the curve. For an individual with congenital
scoliosis, this combination carries the worst prognosis
because it produces the most severe and rapidly progressive
deformity. Curves of this kind located in the thoracolumbar
spine can be expected to exceed 50 degrees by 2 years of
age. Without treatment, patients with thoracolumbar, mid-
thoracic, or lumbar curves become severely deformed at an
FIGURE 12-47  Standing radiograph of an 18-month-old boy early age because of a combination of shoulder imbalance,
showing significant obliquity of the pelvis because of a severe distortion of the rib cage, decompensation of the
hemivertebra at the L5 level. Postoperative findings are shown in
trunk, and pelvic obliquity that produces an apparent leg
Figure 12-56.
length discrepancy.
In addition to deformities involving the thoracic and
lumbar spine, congenital scoliosis affecting the cervical
and cervicothoracic spine can lead to significant deformi-
ties of the neck and an abnormal head position (Fig.
12-50).712 The neck deformities can result in persistent
tilt of the head (apparent torticollis) because the rela-
tively few normal vertebrae above the area of the seg-
mentation defects cannot provide sufficient compensation
for balance. Nearly 50% of those with congenital cervical
or cervicothoracic scoliosis have associated Klippel-Feil
Rib anomalies in patients with congenital deformities of
the spine have been found to be much more frequently
associated with congenital scoliosis than with congenital
kyphoscoliosis or kyphosis and to be more frequent in those
whose scoliosis was caused by unilateral failure of vertebral
segmentation than in those whose scoliosis was due to a
hemivertebra alone. Rib anomalies are more common with
thoracic or thoracolumbar scoliosis and are most often
located on the concavity of the curve. The rib anomalies do
not appear to affect the rate of curve progression.
Natural History
The rate of curve progression and the final severity of con-
genital scoliosis are related to two factors: the type of ver-
tebral anomalies present and the patient’s remaining growth
at the time of diagnosis. The two periods of accelerated
growth during which congenital scoliosis worsens most
rapidly are the first 2 years of life and the adolescent growth
FIGURE 12-48  Radiographic appearance of a 10-year-old girl with spurt.
52-degree scoliosis and block vertebrae at T5-6 and T9-10. The Curve progression is certain in patients with a unilateral
deformity cannot be attributed to the mere presence of these unsegmented bar and a contralateral hemivertebra (one
abnormalities. Findings on magnetic resonance imaging of the or more).79,499,500,821,824 Thoracolumbar curves of this type
spinal canal were normal.
have the worst prognosis and deteriorate 7 degrees per year
before the age of 10 years; this increases to 14 degrees
failure of segmentation leads to a block vertebra (Fig. per year during the adolescent growth spurt. Severe
12-48). This does not cause any angular or rotational spinal spinal deformity occurs unless surgical intervention is
deformity but does lead to some loss of longitudinal growth. undertaken.
Klippel-Feil syndrome in the cervical spine represents a Severe deformities are also caused by (in order of
severe form of this failure of segmentation. decreasing severity) isolated unilateral unsegmented bars,

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CHAPTER 12  Scoliosis 253

FIGURE 12-49  A, An 11-month-old boy with unilateral failure of segmentation that was noted on a radiograph obtained because of mild
back asymmetry. B, On a radiograph obtained at the age of 16 years, no significant change had occurred in the spinal alignment, even
though no intervention had been undertaken.

FIGURE 12-50  Clinical (A) and radiographic (B) appearance of a 2-year-old girl with Klippel-Feil syndrome and congenital cervicothoracic
scoliosis caused by numerous vertebral abnormalities. Eleven years later, without operative intervention the curve was similar in size, and
the head tilt was mild (C). Neurologic findings were normal.

multiple fully segmented hemivertebrae, a single fully seg- respiration cannot be supported.111 This concept is explained
mented hemivertebra, and a block vertebra. in more detail in the section “Early-Onset Scoliosis.”
The future behavior of congenital scoliosis caused by a Compensatory curves in an otherwise normal spine
combination of the previously described abnormalities is develop more commonly in patients with congenital scolio-
extremely difficult to predict, and numerous visits may be sis and a curve apex at T5, T6, or T7 (Fig. 12-51). As the
required before the nature of the curve becomes evident. congenital curve deteriorates, this secondary curve may
The most worrisome potential outcome is the development worsen, become inflexible, and require treatment.499
of thoracic insufficiency syndrome, in which growth of the Patients with severe congenital lumbar or thoracolumbar
thorax is so retarded that normal lung growth and curves may be unable to develop compensatory curves large

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254 SECTION II  Anatomic Disorders

FIGURE 12-51  Radiographic appearance of a girl with numerous congenital abnormalities in the upper thoracic spine, including
hemivertebrae on the convexity and fused ribs on the concavity. These abnormalities are well demonstrated on a radiograph obtained at
7 months of age (A). Because of the high risk for progression, anterior and posterior in situ fusion between T2 and T8 was performed at
16 months of age. A compensatory curve developed below that measured 40 degrees at 7 years of age (B). This compensatory curve was
subsequently managed with a brace.

enough to maintain a balanced trunk. In this instance, If comparisons are made with radiographs obtained several
notable pelvic obliquity and apparent lower limb length years earlier, the changes become more evident. Consistent
inequality are unsightly compensatory mechanisms used to measurement of the secondary curve may also reveal its
keep the trunk vertical. progression and indicate the need for treatment.
In severe congenital scoliosis, plain radiographs may not
Radiographic Findings provide sufficient detail of the vertebral abnormalities.
Radiographic details of the vertebral abnormalities are best Should surgical intervention be necessary, CT with three-
seen on films obtained before the development of significant dimensional reconstruction is helpful for preoperative plan-
deformity—often during infancy—on a radiograph taken ning, particularly in visualizing posterior vertebral anomalies
while the child is supine. As the child grows and the con- associated with hemivertebrae.103,300,367,505,535,545
genital scoliosis progressively worsens, the bony detail MRI of the spine should be performed in all patients
becomes less clear. At initial evaluation, coned-down radio- with congenital scoliosis who are undergoing surgical inter-
graphs of the affected area provide the most information vention and should also be strongly considered during the
about the vertebral anomalies. Associated abnormalities initial evaluation because up to 37% of patients have an
that may also be noted on plain radiographs include diaste- intraspinal abnormality (Fig. 12-52).47,500 MRI reconstruc-
matomyelia (midline bone spur), spina bifida occulta, and tion can provide a clear picture of the canal contents despite
congenital rib fusions on the concavity of the curve. the severe three-dimensional deformity associated with
Although early supine radiographs reveal bony detail, some cases of congenital scoliosis.
they cannot be used to assess curve progression. The initial
upright radiograph must serve as the baseline study against Nonoperative Treatment
which further curve progression is measured. The variability Bracing is much less successful in the treatment of congeni-
in measuring angles in congenital scoliosis is reportedly tal scoliotic deformities than it is for idiopathic scoliosis.
larger than that in idiopathic scoliosis because of skeletal Use of a brace to control curve progression secondary to
immaturity, incomplete ossification, and anomalous devel- unsegmented bars or hemivertebrae is universally unsuc-
opment of the end vertebrae.215,447 Concerted effort should cessful and should not be attempted. However, bracing can
be made to measure the curves with similar end points to be considered as a means of temporarily controlling a long,
detect subtle yet steady progression of the curvature and flexible compensatory curve below the congenital compo-
assess secondary or compensatory curves. The most recent nent and thereby allowing further spinal growth before
radiographs should be carefully compared with one of the operative intervention. If the congenital or compensatory
earliest upright radiographs to ascertain whether slow but component worsens during the period of brace wear, opera-
steady progression has occurred. It is not uncommon for tive intervention should be undertaken without delay. In
radiographs taken 4 to 6 months previously to reveal only general, few patients with congenital scoliosis benefit from
slight progression when compared with current radiographs. the use of an orthosis.

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CHAPTER 12  Scoliosis 255


FIGURE 12-52  A and B, A neurologically normal 13-year-old girl had 53-degree scoliosis that required operative correction. C, Close
examination of the radiographs revealed block vertebrae extending from T3 to T5. D, Because of this finding, magnetic resonance
imaging was performed and demonstrated a diastematomyelia that bisected the spinal canal at T4 and split the spinal cord. E, The
diastematomyelia was better demonstrated on computed tomography. Neurosurgical resection of the diastematomyelia was performed
through a laminectomy. F, By the time that the patient had recovered sufficiently from this operation to undergo a scoliosis procedure,
the curve had progressed to 62 degrees. G and H, Two years after posterior spinal instrumentation and fusion, the spine was well
balanced and the patient remained neurologically normal.

Operative Treatment develop above or below the fused sites. Additional surgery
The primary goal of surgery is to stop progression of the may be required in these individuals.
congenital spinal deformity. If partial correction can be Various operative approaches can be used; the choice
achieved safely, this is an added benefit. depends on the maturity of the patient, the location of the
Fusionless techniques in the form of growing rods and deformity, and the type of congenital deformity. Approaches
expansion thoracoplasty are presented in detail in the include anterior and posterior spinal fusion, posterior fusion
section “Early-Onset Scoliosis.” Many patients with con- with or without instrumentation, hemivertebra excision,
genital scoliosis are very young, have severe thoracic defor- and spinal osteotomies.
mities, and are in need of greater trunk height and thoracic
volume to survive into adulthood (Fig. 12-53). Fusion of the Anterior and Posterior Fusion
spine in these young individuals must be avoided until the This approach is used in immature individuals in whom
age of 8 to 10 years. continued anterior growth on the convexity would lead to
For older children with congenital scoliosis, even with development of the crankshaft phenomenon.196,303,761 Chil-
relatively small curves (<40 degrees), once progression has dren most in need of this approach are those who have
been confirmed, surgical intervention should be undertaken unilateral unsegmented bars with (or sometimes without)
if a significant amount of growth remains. This concept contralateral hemivertebrae (see Fig. 12-54). In a young
must be emphasized: surgery should be performed before a child, fusion should extend to one level above and one level
major deformity develops (Fig. 12-54). Children and ado- below the anomalous vertebrae; this may prevent “adding
lescents who have undergone spinal fusion should be moni- on” of the curve in subsequent years. Postoperatively, a cast
tored to maturity because a progressive deformity can is needed for 4 to 6 months until healing has been achieved.

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256 SECTION II  Anatomic Disorders

fusion is performed. If spinal instrumentation is used, spinal

cord monitoring is imperative, and if reliable waves are not
obtainable, use of the wake-up test is necessary.831 Any cor-
rection of deformity is obtained through the flexible, normal
portion of the curve, not in the rigid, congenitally anoma-
lous region (Fig. 12-55). The exception to this is when VCR
is used for severe deformities, in which case the correction
is obtained at the apical rigid portion. Of all the forms of
scoliosis, congenital scoliosis carries the highest risk for neu-
rologic complications following intraoperative correction.
Slow, gradual correction of severe deformities can be
achieved in some individuals by means of preoperative halo
traction for 6 to 12 weeks. At our institution, patients use
it while sleeping, walking, or in a wheelchair.705 A home
traction program is possible but requires very close monitor-
ing for any neurologic change (e.g., numbness, tingling,
weakness). When correction has been achieved or has
reached a plateau, the spine is stabilized by instrumentation
and fusion.
Hemivertebra Excision
Relatively few patients with congenital scoliosis secondary
to a hemivertebra need to have the hemivertebra excised.
Most can be managed with safer procedures already
described. Hemivertebra excision carries a risk for tempo-
rary and occasionally permanent neurologic injury to a nerve
root.318 This procedure is indicated for patients with a fixed
decompensation in whom adequate alignment cannot be
achieved through other procedures—usually those with a
hemivertebra at the fourth or fifth lumbar level. Excision of
the hemivertebra at this level is safer than in the upper
lumbar or thoracic region because the cauda equina is more
tolerant of manipulation than is the area surrounding the
spinal cord. The major advantage of resection of the hemi-
vertebra is that it allows maximal correction of the defor-
mity and realignment of the spine.‡c
Excision of a hemivertebra can be accomplished by
several surgical techniques (Video 12-2). One method uses
FIGURE 12-53  Hybrid rib-to-spine construct intended to expand
the left thorax for congenital spine/rib deformity.
two exposures—first an anterior approach and then a pos-
terior one.78,302,387,855 The anterior approach allows removal
of the body of the hemivertebra and its adjacent disks back
to the spinal canal, along with removal of the anterior half
Posterior Spinal Fusion of the pedicle. The patient is next repositioned and the
Posterior spinal fusion is indicated in older children with posterior elements are excised through a secondary midline
progressive congenital scoliosis in whom the crankshaft approach. Correction is then achieved internally with pos-
phenomenon is unlikely to develop or in younger children terior compression instrumentation on the convexity or
who do not have normal anterior growth potential. Unlike externally with a cast during the postoperative period.
children with idiopathic scoliosis, crankshaft progression Immobilization in a cast is needed for 4 to 6 months until
does not develop in many young children with congenital fusion has been achieved (Fig. 12-56). A number of studies
scoliosis because the anterior growth plates are abnor- have reported successful hemivertebra excision via a poste-
mal.371,457,830,836,838 These patients are not always easily iden- rior approach alone.337,536,655,656,695,861 This method, com-
tified preoperatively, so the decision whether to include bined with transpedicular instrumentation, is safe and
anterior fusion is difficult. The use of reduced-size spinal provides excellent correction in both the frontal and sagittal
instrumentation in young patients is safe and efficacious.301 planes. Its use should be reserved for experienced spinal
With instrumentation, curve correction, length of immobi- deformity surgeons.
lization, and the fusion rate are improved in comparison to
similar patients treated without instrumentation. Osteotomy of the Spine
If any correction of the deformity with instrumentation VCR is reserved for older children with rigid, severe, angular
is anticipated, preoperative MRI of the neural canal is essen- scoliosis and significant spinal decompensation.33,426,428,745
tial to rule out diastematomyelia, syrinx, tumor, and other Anterior and posterior wedge resection osteotomies are
abnormalities. If any of these lesions are identified, they
need to be addressed neurosurgically before the spinal ‡c
References 81, 107, 173, 318, 323, 385, 387, 536, 655, 656, 695.

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CHAPTER 12  Scoliosis 257

FIGURE 12-54  A, A neurologically normal 6-year-old girl had a left congenital thoracic curve measuring 47 degrees. (Five years
previously, the patient had undergone resection of a diastematomyelia at the thoracolumbar junction.) The curve increased a small
amount (6 degrees) over a period of 4 months, and it was decided to operate before a major deformity developed. B, The preoperative
bending radiograph clearly demonstrated a bar formation in the concavity at T8-10. Anterior and posterior fusion without instrumentation
was performed. After surgery, the patient was immobilized for 4 months in a Risser cast. C, Four years later the curve measured 25
degrees, the patient had good spinal balance, and she remained neurologically normal.

FIGURE 12-55  The patient shown in Figure 12-44 underwent posterior spinal instrumentation and fusion 4 weeks after resection of the
diastematomyelia. Eighteen months later (at 14 years 6 months of age), her spine remained balanced, with a residual 49-degree curve  
(A and B).

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258 SECTION II  Anatomic Disorders

FIGURE 12-56  A, The patient shown in Figure 12-47 had significant pelvic obliquity because of an L5 hemivertebra. At 3 years of age
he underwent resection of the L5 hemivertebra through both anterior and posterior approaches. Correction was maintained with a cast
rather than with internal fixation. B and C, Twenty-seven months later the obliquity was much improved. Although a stable, fibrous
nonunion was present between L4 and the sacrum, the patient remained asymptomatic.

FIGURE 12-57  A, A neurologically normal 12-year-old boy had severe fixed pelvic obliquity. Only one motion segment (L1-2 disk) was
present between T7 and the pelvis because of numerous congenital abnormalities and previous spinal fusions. B, Three-dimensional
reconstruction of computed tomography images allowed viewing of the spine throughout a 360-degree rotation. C, A 32-degree wedge
osteotomy performed through a posterior exposure improved but did not fully correct the pelvic obliquity.

Congenital Kyphosis
performed, and the spine is then instrumented for correc-
tion (Fig. 12-57).239,318 If this procedure is undertaken in Congenital kyphosis represents an abrupt posterior angula-
the thoracic spine, costotransversectomy and resection of tion of the spine because of a localized congenital malforma-
the ribs are required.289 These operations should be per- tion of one or more vertebrae.194,277,821,831 Although this
formed only by very experienced spinal surgeons because condition is less common than congenital scoliosis, paraple-
the risk for neurologic complications is high. gia is a far greater risk in those with congenital kyphosis.

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CHAPTER 12  Scoliosis 259

with kyphosis resulting from failure of segmentation. These

Classification vertebral abnormalities may also lead to frontal plane defor-
Congenital kyphosis is caused by defects of vertebral body mity and result in kyphoscoliosis.
formation (type I), defects of vertebral body segmentation
(type II), or a combination of the two (type III) (Fig. Defects of Formation (Type I)
12-58). In contrast to congenital scoliosis, failure of forma- In kyphosis caused by defects in vertebral body formation,
tion is the most common type of congenital kyphosis, and part or all of the vertebral body is deficient (Fig. 12-59).
it tends to produce more severe deformities than those seen Several contiguous levels may be affected, which produces

Type I Type II Type III

Defects of Vertebral Body Defects of Vertebral Body

Mixed Anomalies
Formation Segmentation

Anterior and unilateral aplasia Anterior and median aplasia Partial

Posterolateral quadrant vertebra Butterfly vertebra Anterior unsegmented bar Anterolateral bar
and contralateral
Anterior aplasia Anterior hypoplasia Complete quadrant vertebra

Posterior hemivertebra Wedged vertebra Block vertebra

FIGURE 12-58  Congenital kyphosis. Type I results from defects in vertebral body formation, type II results from defects in vertebral body
segmentation, and type III results from a combination of the two. (Redrawn from McMaster MJ, Singh H: Natural history of congenital
kyphosis and kyphoscoliosis, J Bone Joint Surg Am 81:1369, 1999, with permission from The Journal of Bone and Joint Surgery, Inc.)

FIGURE 12-59  A to C, Chest radiographs obtained in a 14-month-old girl to evaluate an upper respiratory tract infection showed an
abnormality at T11. On further radiographic evaluation the abnormality was determined to be kyphosis caused by failure of vertebral
body formation. A 51-degree kyphosis was measured. D, Magnetic resonance imaging demonstrated abrupt angulation of the spinal cord
at this level. The child was neurologically normal.

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260 SECTION II  Anatomic Disorders

greater deformity. In general, the posterior elements relentlessly progressive.487,501,597,837 Deformities caused by
(spinous processes, pedicles, transverse processes) are two adjacent type I vertebral anomalies progress more
present and accompany the deficient vertebral body. Growth rapidly and with more severity than do deformities caused
continues normally in the posterior portion of the spine, but by a similar single anomaly. Kyphosis from failure of seg-
not anteriorly. As a result, relentless progression of the mentation (type II) is much less progressive, produces less
deformity usually occurs. severe deformity, and has a very low likelihood of resulting
Defects of formation place the patient at a much greater in paraplegia.
risk for the development of paraplegia than do defects of
segmentation. The kyphotic junction may be unstable, par- Clinical Features
ticularly when the apex is between T4 and T9. Paraplegia Although congenital kyphosis has been diagnosed prenatally,
can occur at any age but is most common during the ado- it may not be clinically evident in a newborn or infant.717
lescent growth spurt. Reports of acute-onset paraplegia fol- Suspicion may first be raised after a chest radiograph is
lowing minimal trauma in young children testify to their obtained for evaluation of an unrelated event, such as a
fragile neurologic status. respiratory infection. As the child begins standing and
walking, a localized prominence may become noticeable or
Defects of Segmentation (Type II) palpable. The child is usually asymptomatic and has no
In kyphosis caused by failure of segmentation, the spinal tenderness. In adolescents, the predominant clinical
anterior portions of two or more adjacent vertebral complaint tends to be lower back discomfort caused by
bodies are fused. This deformity tends to be less progres- secondary lumbar hyperlordosis. Mild scoliosis may accom-
sive, produces less deformity, and is associated with a pany the kyphosis.
much lower risk for paraplegia than is kyphosis caused On occasion, myelopathy or paraplegia secondary to
by defects in formation.492 The area most commonly spinal cord compression may develop in a child with con-
affected is the lower thoracic or thoracolumbar spine genital kyphosis.374 Reports of mild trauma producing a
(Fig. 12-60). sudden onset of paraplegia in children who have unrecog-
nized, acute type I kyphosis highlight the delicate underly-
Natural History ing neurologic status in this condition. When congenital
The apical area of the kyphosis can occur at any level but kyphosis caused by a defect in vertebral formation is diag-
is most commonly located between the tenth thoracic and nosed, a meticulous neurologic examination should be per-
first lumbar levels.501 There appears to be no relationship formed to identify any subtle abnormalities. Plans for
between the severity of the kyphosis and its location in the surgical intervention should begin immediately.
spine. Progression of these deformities is most rapid during
the adolescent growth spurt. Radiographic Findings
Congenital kyphosis from either failure of formation Congenital kyphosis is best visualized on a lateral radiograph
(type I) or mixed anomalies (type III) tends to be of the spine. It may not be evident on the frontal view. Once

FIGURE 12-60  This neurologically normal 14-year-old boy had back pain and progressive worsening of his appearance. A and B,
Radiographs demonstrated failure of segmentation at T12-L1 and L2-3, with a resulting 82-degree localized kyphosis. C, Magnetic
resonance imaging showed a normal spinal cord.

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CHAPTER 12  Scoliosis 261

FIGURE 12-61  The patient whose images are shown in Figure 12-59 was treated with an anterior rib strut graft between T10 and T12,
followed by posterior in situ fusion. A small amount of correction of the kyphosis was achieved. A, Two years postoperatively, the kyphosis
measured 35 degrees and the rib strut had been incorporated but was still visible radiographically. B, Ten years postoperatively, the
kyphosis remained unchanged. The patient was neurologically normal.

identified, a coned-down lateral view of the specific area fusion without instrumentation may be considered.
provides greater bony detail. A hyperextension cast is used postoperatively for 4 to
MRI provides the clearest picture of the spinal cord and 6 months, followed by a TLSO for another 6 months.
vertebral bodies in very young children. It should be ordered Successful outcomes with posterior fusion have been
immediately for those whose kyphosis is due to failure of reported.501,502,834 This approach allows some growth to
formation (see Fig. 12-59). Spinal cord compression may occur anteriorly in the abnormal region of the spine, which
be evident on MRI before any clinical neurologic deficits may result in progressive improvement in the localized
become apparent. Three-dimensional CT imaging of the kyphosis over time. Reexploration and augmentation of the
spine with reconstructed images is very useful in the evalu- graft at 6 months have been advocated. An alternative
ation of vertebral anomalies, especially in older children.545 approach for young children is to combine anterior fusion
Both MRI and CT should be performed before any opera- using a rib strut with posterior fusion during the same surgi-
tive intervention is undertaken. cal intervention (Fig. 12-61). This approach produces some
immediate improvement in sagittal plane alignment and
Treatment increases the likelihood of a solid fusion, but it eliminates
Nonoperative treatment has no beneficial effect on congeni- any further correction that might occur as a result of ante-
tal kyphosis, and use of an orthosis is inappropriate. Once rior growth.
type I or type III kyphosis is recognized, plans for surgical In an older child or adult, two approaches can be used.
intervention should be made. For adolescents with mild The first and older approach consists of the combination of
type II kyphosis, close monitoring for progression is reason- anterior and posterior arthrodesis.839 The anterior arthro­
able. If the deformity is recognized at a younger age, opera- desis is performed first. Following excision of the gristlelike
tive intervention should be considered. soft tissue anteriorly, some distraction is attempted. Any
distraction that is achieved can then be maintained with rib
Type I Kyphosis strut grafts. Vascularized rib struts heal more rapidly and
Defects of formation are more common than defects of should always be used in those who have had previously
segmentation, can lead to more severe deformity, and have unsuccessful attempts at anterior fusion; they may also be
a greater potential for producing paraplegia. For these considered for the initial fusion procedure (Fig. 12-62).80,82,683
reasons, once this form of congenital kyphosis is diagnosed, Spinal cord monitoring is essential. In an older child, instru-
surgical intervention is indicated, even in an infant. The mentation should be used during the posterior arthrodesis
main goal is to prevent paraplegia. All other goals, such as if it is not too prominent.
improved spinal alignment and cosmetic appearance, are The newer second approach involves posterior surgery
secondary. only. Through VCR, the anterior deformity can be
If the kyphosis is recognized in a child younger than 5 resected, decompression achieved, and an interbody spacer
years and is less than 45 to 50 degrees, simple posterior placed.33,426,428,573,733,745 Posterior pedicle screw fixation is

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262 SECTION II  Anatomic Disorders

required to maintain spinal stability during resection of the posterior arthrodesis, these subtle neurologic deficits may
anterior elements (Fig. 12-63). Spinal cord monitoring is resolve. On occasion, patients have mild paraparesis of
essential during this procedure. recent onset. In these individuals the apical flexibility of the
If a neurologic deficit is present at the time that the kyphotic deformity should be assessed with a hyperexten-
congenital kyphosis is recognized, treatment should be sion radiograph. If the apex is flexible, some improvement
undertaken immediately. If the deficit is minimal (increased in the paraparesis may be achieved by resting the recently
reflexes, Babinski sign, or both, but no loss of motor, bowel, compromised spinal cord with a halo vest, cast, or minimal
or bladder function), formal anterior decompression of the halo traction.194 Halo traction should not be considered in
spinal cord is not necessary. Following a solid anterior and those with a rigid, inflexible kyphotic apex because of the


Rib bundle
graft Neurovascular

Rib graft
in place

FIGURE 12-62  A and B, Radiographic appearance of a 10-year-old boy with spondyloepiphyseal dysplasia and an abrupt 93-degree
kyphosis at L1 (as well as 47-degree scoliosis) because of a small vertebra in the posterolateral quadrant. A previous attempt at anterior
and posterior fusion with posterior instrumentation had been complicated by infection. The hardware was removed and the deformity
worsened. He remained neurologically normal. C, Magnetic resonance imaging demonstrates the abrupt kyphosis and its effect on the
spinal cord. D and E, A vascularized rib strut graft was used during the repeated anterior and posterior fusion. The neurovascular bundle
was isolated from the rib near its origin to allow the rib to be cut. The rib selected for a graft usually corresponds to the upper vertebral
level requiring fusion (D). When the rib is seated into the vertebral bodies, the vascular bundle must be free of tension. The ends of the
ribs should be exposed subperiosteally for a length of 1 cm to allow secure fixation into the vertebral bodies (E). F, The vascularized rib
was fixed into T10 and L3.

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CHAPTER 12  Scoliosis 263

FIGURE 12-62, cont’d  G and H, Fifteen months postoperatively, the kyphosis was stable at 50 degrees and the scoliosis measured
40 degrees. Five years later, the radiographic appearance was unchanged and the patient remained neurologically normal.

Segmental Spinal Dysgenesis, Congenital

risk for progressive neurologic deterioration. Very close mon-
Vertebral Displacement, and Congenital
itoring is needed. If recovery occurs, spinal fusion can be
Dislocation of the Spine
performed without the need for decompression.382 If the
deficits do not resolve, arthrodesis must be combined with Segmental spinal dysgenesis, congenital vertebral displace-
anterior decompression of the spinal cord. Unless the child ment, and congenital dislocation of the spine are similar
is very small, these procedures can be accomplished during conditions. In fact, they may be variations of the same
the same operative episode. The decompression must be deformity, although this is not universally accepted.176 They
performed anterior to the compressed cord by removing the all create severe localized kyphosis of the spine and lead to
posterior aspect of the vertebral body. Posterior laminec- a neurologic deficit in 50% to 60% of patients.216,243,327,685,874
tomy does not relieve the spinal cord compression. VCR Segmental spinal dysgenesis is characterized by a focal
through a costotransversectomy is an effective approach for spinal deformity, usually located at the thoracolumbar junc-
these complex kyphotic deformities of the thoracic spine. tion or in the upper lumbar spine.216,243,244,327,772,872 The
It should be undertaken only by those experienced with this deformity frequently includes severe kyphosis; anterior,
technique. posterior, or lateral subluxation of the spine; scoliosis in
association with a severely stenotic spinal canal; and absent
Type II Kyphosis nerve roots. All these patients have localized stenosis of the
Defects of segmentation are best treated at a young age, spinal canal at the level of involvement, and the osseous
before significant deformity has developed. Posterior spinal canal has an hourglass shape. No pedicles, spinous pro-
fusion followed by cast immobilization is sufficient treat- cesses, or transverse processes are seen at the level of
ment. The fusion should span the unsegmented levels plus involvement. Commonly, an offset in the sagittal plane is
one level farther both cephalad and caudad. Correction of present between the cephalic and caudal segments of the
the kyphosis should not be expected, although mild spine at the level of dysgenesis. Decompression of the ste-
improvement from the cephalic and caudal extensions is notic canal results in some improvement in neurologic func-
possible.378 Posterior compression instrumentation may tion in 20% of patients. Early anterior and posterior
lessen the need for external immobilization. arthrodesis in patients with segmental spinal dysgenesis is
In an older child with severe kyphosis, some correction indicated because progressive kyphosis inevitably develops
of the deformity may be achieved through osteotomy of the and often results in neurologic deficits.
unsegmented anterior region.713 When combined with pos- Type I congenital kyphosis can be similar to and may be
terior compression instrumentation, this approach may confused with segmental spinal dysgenesis. Type I congeni-
result in some improvement in the sagittal plane. tal kyphosis represents failure of formation of the vertebral
body; however, the pedicles and posterior elements are
Type III Kyphosis present. The severe spinal stenosis associated with segmen-
Mixed anomalies are least common but usually produce a tal spinal dysgenesis is not present in congenital kyphosis.
kyphoscoliotic deformity. Because of their association with Although many patients with segmental spinal dysgenesis
failure of segmentation, type III anomalies generally require have fixed neurologic deficits, neurologic function in patients
posterior arthrodesis only. with congenital kyphosis is generally good at birth, with

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264 SECTION II  Anatomic Disorders


FIGURE 12-63  Twenty-year-old man with type I congenital kyphosis and back pain. A and B, Preoperative photo and radiograph.
C, Lateral magnetic resonance image of the thoracolumbar region (supine). D and E, Postoperative photo and radiograph.

paraplegia subsequently developing as a result of untreated be present. As with segmental spinal dysgenesis, the poten-
instability and worsening kyphotic deformity. tial for severe neurologic deficits is high. Combined anterior
Congenital vertebral displacement occurs when the and posterior arthrodesis of the spine is needed in an effort
spinal column is displaced at a single vertebral level and to prevent the development of such deficits. For those with
results in abrupt displacement of the neural canal (Fig. neurologic deficits of recent onset or progressive neurologic
12-64).685,776,799,873 The displacement can occur in the pres- deficits, decompression of the spinal cord is indicated.
ence of a posteriorly located hemivertebra in which the The congenitally dislocated spine was first described in
pedicles, transverse processes, and spinous processes may 1973 by Dubousset.874 It, too, is associated with spinal

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CHAPTER 12  Scoliosis 265


FIGURE 12-64  Two types of congenital vertebral displacement are recognized. A, Type
A consists of posterior displacement of the caudal vertebrae with anterior angulation  
of the spinal canal. A hemivertebra is noted in the area of the deformity. B, Type B is
characterized by rotatory subluxation with resulting rotatory translation of the neural
canal. C, Radiograph showing a type B deformity in a girl aged 2 years 8 months
D with abrupt congenital vertebral displacement at the T10 level. This was further
demonstrated on magnetic resonance imaging (D) and computed tomography (E).

kyphosis and a high likelihood of neurologic deterioration. and the appropriate operative intervention should be under-
The posterior elements are abnormal in all patients with taken. Prenatal diagnosis is possible and can be useful for
congenital dislocation of the spine. The various stages of parental counseling and obstetric management.245,664
posterior dysraphism range from agenesis of the laminae
with pathologic changes in the articular facets to total
absence of the posterior elements and the spinal cord under Early-Onset Scoliosis
otherwise normal skin. Anterior and posterior spinal fusion
is indicated because posterior fusion alone is insufficient to An increasing body of evidence suggests that patients in
achieve solid fusion with this type of congenital instability. whom scoliosis develops at an early age, regardless of the
Exploration and augmentation of the posterior fusion mass cause, are at significant risk for thoracic insufficiency syn-
should be considered because of the high rate of pseudar- drome,111 defined as an inability of the thorax to support
throsis with this abnormality. No sudden extemporary cor- normal respiration and lung growth. The increased respira-
rection should be attempted in older patients with severe tory morbidity and mortality now recognized in patients
angular kyphosis and progressive neurologic deficits. Func- with early-onset scoliosis make a compelling case for clas-
tion must be favored over cosmetic appearance. Neurosurgi- sifying such patients as a separate group in which the
cal decompression should be used only for a proven recent emphasis is not necessarily on treating the spine itself but
and progressive neurologic deficit. rather on maintaining growth of the thorax to promote
For these three entities, all of which involve a severe increased lung volume throughout the critical first decade
form of localized kyphosis, early recognition is imperative, of life.

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266 SECTION II  Anatomic Disorders

correlated with increasing deformity, whereas in adolescent-

Effect on Respiratory Function
onset deformity, no effect on vital capacity was noted with
Respiratory failure secondary to untreated scoliosis before an increasing Cobb angle.530 In general, the combination of
5 to 8 years of age has been documented for at least 2 onset before 7 years or a Cobb magnitude of 100 degrees
decades,85,271,702 with double the mortality rate as in the and associated rib anomalies or muscle weakness can
general population.271 Swedish patients with infantile (0 to produce respiratory failure as early as the third decade
3 years) and juvenile (4 to 9 years) onset were found to (Fig. 12-65).702
have a significant increase in observed mortality in compari- The source of the respiratory failure is twofold: intrinsic
son to the general population; in contrast, patients with alveolar hypoplasia and extrinsic disturbance of chest wall
adolescent (older than 10 years) onset had the same mortal- function.
ity rate as the general population.587 Not surprisingly, the
magnitude of the scoliosis also plays a role in the demise of Intrinsic Alveolar Hypoplasia
untreated patients: curves greater than 70 degrees result in Limited autopsy material has shown that failure of alveolar
a higher mortality rate than do smaller curves.587 In a Scot- multiplication is probably a major source of respiratory
tish study of children with infantile-onset idiopathic or con- demise with early-onset scoliosis because the chest defor-
genital curves, a decrease in vital capacity was directly mity prevents hyperplasia of the lung tissue.56,77,163,623


FIGURE 12-65  A and B, A 5-year-old boy

with severe, fixed left cervicothoracic scoliosis
secondary to cervical dysraphism with
myelocele that produced a dramatic
torticollis. The thorax was congenitally
normal, but a rigid compensatory scoliosis
developed secondary to the fixed cervical
deformity, which could not be corrected
because of the overwhelming neurologic  
risk. Thus, the thoracic curve could not be
corrected either without making the head  
tilt worse, so it was simply fused in situ.  
C to E, By 20 years of age the patient was in
respiratory failure, with severe loss of volume
in the convex (right) hemithorax as a result of
the severe deformity and loss of respiratory
function because of severe crowding of the
D E ribs in the left hemithorax. The patient died
of respiratory failure.

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CHAPTER 12  Scoliosis 267

Indeed, the alveoli that are present appear emphysematous,

as though attempting compensatory hypertrophy; however, Extrinsic Disturbance of Chest Wall Function
actual compression of the alveoli, which might be predicted Extrinsic disturbance of respiratory function is the result of
by the chest wall deformity, is not observed. In a normal rib or associated chest wall deformities producing loss of
lung, alveolar hyperplasia continues until about 8 years of compliance or functional incompetence of the chest
age, with hypertrophy possible until growth of the thorax wall.58,109 The latter is most severe in patients with rib
is complete.623 Other investigators have found that the adult fusion, in whom normal chest wall movements are inhib-
number of alveoli is attained even earlier, by the age of 2 ited, or in those with rib absence, in whom a localized “flail”
years. Subsequent expansion of lung volume up to 5 years or paradoxical segment of the chest wall is present. The
of age occurs through the rapid increase in peripheral thorax is thus unable to change volume effectively because
airway conductance that accompanies airway enlarge- of chest wall dysfunction. Patients with congenital scoliosis
ment.167,312,315,768,875 Most authors agree that lung “growth” have decreased vital capacity in comparison to those with
(hyperplasia and airway expansion) is essentially complete idiopathic scoliosis and the same magnitude of Cobb angle,
by the age of 8 years, with the “golden period” of maximal presumably as a result of concomitant rib anomalies produc-
growth occurring before 5 years. Deformities developing ing additional chest wall dysfunction.572 Congenital dia-
after this age have less effect on lung growth.85,586 In this phragmatic hernia, which produces ineffective volume
period of alveolar hyperplasia, rapid growth of the bony expansion because of diaphragm dysfunction, may be
thorax also occurs. The length of the thoracic spine increases another example of extrinsic dysfunction. In noncongenital
by 50% (from 12 to 18 cm) from birth to 5 years of age,182 deformities, the rib deformity secondary to the scoliosis
with some 60% of the adult length being achieved by this produces inefficient respiration. The intercostal spaces on
age (Fig. 12-66). The circumference of the thorax, which the concave hemithorax are narrowed and unable to expand;
is only 7% of adult size at birth, increases to 30% by 5 years thus, end-inspiratory volume is restricted (see Fig. 12-65).
of age and to 50% by 10 years.182 The “golden period” of Meanwhile, the convex hemithorax has widened intercostal
rapid growth of the thoracic spine and rib cage thus coin- spaces that cannot generate normal expiratory function.349
cides with lung development. Increasing rotational deformity of the ribs accompanying

T1 T1

26 cm

T1 T1

12 cm

16 cm T12

7 cm
L5 S1


FIGURE 12-66  Growth in length of the lumbar (L1-5) (A) and thoracic (T1-12) (B) spine from birth to maturity. The thoracic spine grows
6 cm (from 12 to 18 cm) in the first 5 years of life, with 60% of the adult length being achieved by this age.

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268 SECTION II  Anatomic Disorders

increasing spinal deformity can produce further loss of chest 100

wall function as the more deformed rib cage becomes stiff 90
and noncompliant and thereby lead to the restrictive lung 70
disease typical of patients with severe scoliosis. 60

Spinal fusion in a young child, the traditional method of 50
halting curve progression, is now recognized to have the 40
potential to produce thoracic insufficiency by eliminating 30
growth of the thoracic spine.109 Thoracic volume—and 10
hence lung volume—depends on the length of the T1-12 0
segment, as well as thoracic coronal width and sagittal depth 20 40 60 80 100
provided by the rib cage. Fusion of the thoracic spine, which % Thoracic fusion
shortens the T1-12 segment, can impair growth of the A
entire thorax, depending on the age at which the fusion is 100
performed and the number and location of segments 90
fused.209,361 Thus, fusion itself is a cause of respiratory insuf-

% Normal FVC
ficiency and adds to the spinal deformity with loss of pul- 60
monary function. 50
Poor respiratory outcomes have been reported in patients 40
with infantile (noncongenital) scoliosis operated on at a 30
mean age of 4 years.267 When tested at a mean age of 20 10
years, forced expiratory volume in 1 second and forced vital 0
capacity averaged around 41% of predicted values, with 1 1 1 1 1 2 22 2 2 2 3 4 4 4 5 55 5 6 6 7 8 9 9 9
individual patients testing as low as 12% to 14%. Because Proximal level of fusion
vital capacity less than 43% of the predicted normal value B
is considered a risk factor for respiratory failure,586 these FIGURE 12-67  A, Forced vital capacity (FVC) versus percentage of
patients have a guarded prognosis for long-term pulmonary the thoracic spine fused. FVC may decrease to less than 50% of
function. In contrast, patients whose surgery could be predicted volume if more than 60% of the thoracic spine (i.e.,
eight thoracic levels) is fused before 8 years of age. B, FVC versus
delayed by nonoperative means until 10 years or older
proximal extent of fusion. Fusions extending to T1 or T2 before  
(mean, 12.9 years) had mean pulmonary function around
8 years of age are likely to produce an FVC of less than 50% of
70% of predicted values, with the worst patients having 45% predicted volume. (Redrawn from Karol LA, Johnston CE,
of the predicted value and the best patients being normal Mladenov K, et al: The effect of early thoracic fusion on
(100%). A disturbing finding was that in the early fusion pulmonary function in non-neuromuscular scoliosis. 40th Annual
group, scoliosis was not controlled by surgery: the mean Meeting of the Scoliosis Research Society, 2005, Miami.)
Cobb magnitude was 70 degrees preoperatively and 80
degrees at 16 years of age. In contrast, in patients operated
on after 10 years of age, the curves were corrected from 81 It is important to reexamine our approach to young
degrees to 63 degrees at follow-up. Although the ineffective children with large spinal deformities. The principle that a
scoliosis management clearly contributed to the poor pul- short, straight spine produced by early fusion is better than
monary outcomes in the early-fusion patients, consistent a long, curved spine is no longer generally accepted. It is
with Pehrsson and co-workers’ data587 on greater mortality important to avoid fusion of the thoracic spine, especially
in those with curves larger than 70 degrees, the benefit of the proximal segments, before 5 years of age. The goal of
controlling the scoliosis through nonoperative means and management must be to control the spinal deformity
delaying surgery until after age 10 is incontrovertible. without impeding thoracic growth. Other innovative
Additional pulmonary outcome data on patients with techniques, such as expansion thoracoplasty, offer the pos-
congenital scoliosis have confirmed the detrimental effect sibility of preventing thoracic insufficiency from spinal
on pulmonary function when patients younger than 5 years deformity.110,207
undergo fusion of four or more thoracic segments, especially
if the fusion includes upper thoracic segments above T6
(Fig. 12-67).209,361 The effect on pulmonary function is most Treatment
damaging if the fusions must include T1 or T2.361 Day and
associates demonstrated that patients with congenital sco- Nonoperative Delaying Tactics
liosis treated nonsurgically (mean age, 11.7 years) had Attempting to delay definitive spinal fusion until 10 years
nearly normal pulmonary function despite multiple thoracic of age by nonoperative means is usually the first approach
vertebral anomalies whereas surgically treated patients with in a patient at risk for thoracic insufficiency.
similar anomalies but larger curve magnitudes (mean age,
16 years; 7.6 years after surgery) had only 45% to 65% of Bracing
predicted volume.165 In addition, these same surgically Bracing is a time-honored method of controlling noncon-
treated patients with multiple anomalies had significantly genital deformities if the patient has no coexisting neuro-
worse pulmonary function test results than did surgically muscular or other medical conditions in which a brace
treated patients with two or fewer anomalies. The growth would adversely affect respiratory function by circumferen-
disturbance from more extensive anomalies and the surgery tial chest or abdominal compression (Fig. 12-68). Bracing
to fuse them both contributed to a poor outcome.165 efficacy is difficult to define or prove by the standard

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CHAPTER 12  Scoliosis 269


FIGURE 12-68  A and B, A 4-year-old boy with Eagle-Barrett (prune-belly) syndrome. Lack of an abdominal wall, an abnormally
compressible chest wall, and bilateral nephrostomy stomata posteriorly prevented any attempt at orthotic management. C, The scoliosis
progressed to 60 degrees, at which time instrumentation without fusion was performed. D and E, Two years postoperatively, the curve is
controlled and the patient has gained 5 cm in length of the T1-12 segment. The instrumentation has been lengthened twice since the
original implantation.

criteria of success because most curves can be temporarily When brace management is used in a rapidly growing
improved radiographically by well-constructed orthoses, child younger than 5 years, the patient must be carefully
even in the smallest patients (Fig. 12-69), only to progress evaluated for brace-induced rib deformity produced by
with growth. Orthotic management can be considered suc- pressure on the soft infantile rib cage, as well as obliteration
cessful if progression is prevented for several years, thereby of the normal sagittal contour (lumbar hypolordosis and
delaying the need for fusion until the child is older. If fusion thoracic hypokyphosis). Iatrogenic rib and sagittal spine
can be delayed until 10 years of age, the need for anterior deformities (Fig. 12-70) and volume restriction must not
fusion may be avoided.344 add to the existing deformity.

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270 SECTION II  Anatomic Disorders

FIGURE 12-69  A, A 1-year-old child with spinal muscular atrophy and collapsing kyphoscoliosis. B, A modified, miniature Milwaukee-type
brace was devised for this patient. The brace is less than 11 inches long. C, Patient sitting in the brace.

between anterior and posterior uprights without constrict-

ing the thorax or abdomen (see Fig. 12-69); thus, respira-
tory effort and rib compression are minimally affected. A
well-fitted orthosis is generally accepted readily by children
younger than 6 years; poor acceptance is often an indication
of poor brace fit, which may be due to progression and
increased rigidity of the deformity.
Serial casting is often useful in prolonging the nonoperative
management of a young child with a progressive deformity.
The primary indication for casting is an inability to control
curve progression with a brace in an effort to delay surgical
intervention. As the curve increases in severity and stiffness,
the child may be unable to tolerate a brace. A series of casts
applied under anesthesia can provide significant curve cor-
rection and may improve the flexibility of the spine as well
(Videos 12-3 and 12-4).When the child resumes brace wear
after several months in a cast or casts, the deformity is
smaller and the brace is more likely to be comfortable and
control the curve. In addition, because the cast is equivalent
to a full-time brace that cannot be removed, many parents
prefer it. Casting eliminates the problem of poor compli-
ance and the difficulty of donning braces in uncooperative
FIGURE 12-70  This child’s chest and spinal deformities were young children.
worsened by bracing. The right thoracic pad deformed the lower Moreover, casting can be a more definitive method of
ribs posteriorly (arrows) and probably added to the left lumbar
management rather than simply a delaying tactic. Mehta
deformity by creating a posterolateral force at the right
thoracolumbar junction.
reviewed a 20-year experience of treating infantile-onset,
noncongenital scoliosis with serial casts.504 She showed that
patients who were treated by aggressive casting beginning
The Milwaukee brace, in some form, is often the orthosis at a mean of 19 months of age with an average curve of 32
of choice in early-onset patients because of its ability to degrees had their scoliosis reduced to less than 10 degrees
apply corrective forces directly at the apex of the curve, at at maturity. Patients who started treatment at a mean of 30
the pelvis, and at the neck while minimizing the constrictive months of age with larger curves averaging 52 degrees did
aspects of a TLSO. Precise custom construction is essential not gain significant correction, but their deformities had not
for success. In a small child, the rib pad can be applied progressed (averaging 46 degrees) at follow-up. Her casting

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CHAPTER 12  Scoliosis 271

protocol required serial cast changes under anesthesia every removed for respiratory excursion, and other cast relief is
2 to 3 months in children younger than 2 years, with a applied liberally in portions that do not involve spinal cor-
minimum of five casts. The goal was to achieve a straight rective forces. A window to allow the spine to move into
spine, at which time the patient was switched to a brace. the concavity is useful to gain further correction.504 Improve-
Children older than 2 years required cast changes every 3 ment in the deformity can be dramatic in the short term
to 4 months. Older children demonstrating “recurrence” (see Fig. 12-71, C and D), but the apparent correction is
were placed back in a cast for 4 months to recorrect the somewhat misleading because the radiograph is taken with
deformity before continuing with brace management. the patient supine (and is usually compared with a pretreat-
Mehta’s important contribution was to demonstrate that ment standing film). Nevertheless, with serial cast changes
serial casting in young children (even infants), if pursued performed every 2 to 3 months for 6 to 9 months (usually
aggressively and for a long enough time, can correct the totaling three casts), an apparently uncontrollable, rapidly
deformity over a long follow-up period. progressive deformity can be corrected sufficiently to return
Our experience with serial casting has been less aggres- to orthotic management and further delay the need for
sive and more variable. We apply the cast under general surgery.
anesthesia on a Risser table, with neck halter and pelvic The superior mesenteric artery syndrome and neurologic
strap longitudinal traction and appropriate localized hand dysfunction, particularly affecting the cranial nerves (see
pressure or straps applied directly to the rib hump (Fig. Fig. 12-71, E) and the brachial plexus if the neck portion is
12-71). Properly molded chin and neck pieces are generally applied with too much head or shoulder distraction, are the
required to maintain the longitudinal corrective force once main acute complications of applying corrective casts under
the traction is removed. A large abdominal window is anesthesia. In the longer term, pressure sores over the rib


FIGURE 12-71  A, Cast application on the Risser table with the

patient under anesthesia. Traction is applied via a head halter
and pelvic straps. B, Completion of cast trimming. Note the
large abdominal window. C and D, Radiographs showing the
immediate correction achieved by casting. E, Seventh cranial
nerve palsy in a patient in a Risser cast, presumably caused by
excessive traction on the neck by the neck piece. Once the cast
was removed, the palsy resolved.

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272 SECTION II  Anatomic Disorders

prominence and other bony prominences are not uncom- by traction noted two possible neurologic complications:
mon, especially in patients who are not cognitively normal. nystagmus and dizziness.641
For this reason, casting may be relatively contraindicated in Long-term halo traction is especially useful for correcting
such patients. trunk shift, trunk height, and sagittal plane deformity. The
It may be argued that a cast restricts pulmonary function ability to mobilize patients with weakness, osteopenia, and
more than a brace does and may cause more rib deforma- respiratory compromise during the traction period is invalu-
tions, but these drawbacks can be minimized with a well- able in preparing such patients for surgery. This traction
managed casting protocol. We find the casting alternative protocol avoids enforced bed rest, which is required by
to be useful in delaying surgery, as do many European halo-femoral traction, and significant pulmonary benefit is
centers.504,667 achieved with an upright thorax as these patients are readied
for surgery. Finally, because these children have diminutive,
Traction often osteopenic spinal elements and more rigid deformi-
Some patients with progressive deformities are not candi- ties, acute correction of the deformity with instrumentation
dates for casting, such as those with weakness, skin or chest is often fraught with the risk for bone–implant interface
wall defects, intolerance, or mental retardation. Large, stiff failure and neurologic compromise. Thus, correction of the
curves may not benefit from serial casting, and the cast may spine, not to mention the length of the thorax, must
be poorly tolerated. In these instances, halo–gravity traction be done gradually with increasing traction (Fig. 12-73).
is an invaluable method to achieve correction of the defor- The definitive stabilization procedure using instrumentation
mity and, indirectly, improve respiratory mechanics.705 may then be essentially an in situ fixation to maintain the
The technique is not new, having been developed origi- position achieved by traction.
nally by Stagnara725 and later demonstrated to one of the
authors (CEZ) by Zielke on a visit to the latter’s clinic in Operative Treatment
1984. A halo is first applied with six to eight pins and the
child under general anesthesia (Fig. 12-72).528,705 Experi- Instrumentation Without Fusion (“Growing Rods”)
ence has shown that the use of numerous pins actually (Video 12-5)
decreases the chance of pin infection or loosening of any In an effort to treat uncontrollable progressive scoliosis in
single pin. Pins are tightened to a torque equaling the age young children, Moe and colleagues introduced the tech-
of the child; for example, a 4-year-old patient’s pins are nique of subcutaneous Harrington instrumentation, first
tightened to 4 inch-pounds of torque with a calibrated reported to the SRS in 1978.515 With this technique the
torque wrench. The following day the patient is placed spine is exposed subperiosteally only at the end vertebrae
upright in overhead traction via a traction bale attached to in the hope of allowing continued growth of intercalary
a wheelchair or a standing frame via a spring-loaded fish segments after distraction between these end hooks. In
scale or other dynamic traction device (see Fig. 12-72, D), Moe’s original method, Harrington rods were modified so
with an initial traction of 5 to 10 lb. The time in traction that a short threaded segment was present at each end of
and the amount of weight are increased to tolerance, with the rod with a long nonthreaded segment in between so that
careful neurologic monitoring. All patients need cranial the rods could be exchanged easily through subcutaneous
nerve testing once during each shift while upright in trac- tunnels without soft tissue growth into the threads and be
tion, and in patients with preexisting neurologic signs, lower contoured without damaging the threaded portion. Single
extremity strength and reflexes are monitored as well during hooks were seated at the selected end vertebrae through
the phase of increasing traction. Eventually, a traction force small, limited incisions, and then the modified rod was tun-
exceeding 50% of body weight may be achieved. The trac- neled subcutaneously or submuscularly to engage the hooks.
tion is increased so that the patient’s buttocks are lifted Later, subfascial placement through a single long incision
slightly off the wheelchair seat while sitting; in the standing was described to add stability to the construct and obtain
frame, the patient should be up just on tiptoes (see Fig. better skin coverage.386 The hooks were then reexposed
12-72, D and E). The safety of this method is ensured by every 4 to 6 months or when 10 degrees of correction had
the patient’s ability to automatically relieve the traction by been lost, and the rod was lengthened by advancing a nut
pushing up on the wheelchair arms or walker hand rails in on the threaded portion to redistract the hooks. Patients
response to pain or neurologic symptoms. were maintained in Milwaukee braces full-time. Eventually,
We have treated many children as outpatients once their definitive fusion was performed to complete the treatment
caretakers are educated on the use of traction and comfort- protocol. Following the initial experience, standard non-
able with its supervision. Only two neurologic complica- threaded Harrington rods were substituted to avoid rod
tions have occurred in a series of nearly 100 patients. In 1 fracture, which occurred at the junction of the smooth and
patient with Klippel-Feil syndrome and multiple cervical threaded portions of Moe’s modified rods.511 Localized end
synostoses, mouth and facial numbness developed when fusions were also added in an attempt to prevent hook
C3-4 distraction occurred at the only nonfused level in the migration and dislodgement. Moe and co-workers reported
neck (see Fig. 12-72, F). The numbness resolved after trac- that in the early cases, the apex of the curve remained
tion was discontinued and converted to a halo vest for 6 unfused until the definitive fusion whereas the end verte-
weeks. In another patient with incomplete resection of a brae uniformly fused spontaneously.515 Subsequent reports
benign ganglioneuroma of the conus medullaris and pretrac- showed that the intercalary segments ankylosed even though
tion hyperreflexia, weakness of multiple muscle groups in they were never actually exposed.4 Decreasing effectiveness
one lower extremity developed and necessitated that trac- of repeated distractions often resulted in little additional
tion be discontinued. Another report of 33 patients treated correction of the stiffened spine.386,760

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CHAPTER 12  Scoliosis 273


FIGURE 12-72  A, Halo application in a young child. As many as 8 to 10 pins should be used, with low amounts of torque per pin, to
gain safe and adequate skull fixation. B, Dynamic traction device using multiple pulleys in sequence. C, By shortening the traction rope
through the pulleys in sequence, the longitudinal pull can be increased smoothly and gradually (in this case, to 15 lb). D, Spring-loaded
“fish scale” traction with an overhead standing frame. The amount of traction with the overhead standing frame should be sufficient to
pull the patient up on tiptoes. E, The activity level of some patients in traction is essentially unrestricted. F, Cervical radiograph of a
patient with Klippel-Feil anomalies and distraction through the only nonfused segment in the neck. The only neurologic deficit was facial
and intraoral numbness, which resolved when the traction was discontinued (B, Courtesy D. Ross.)

The long-term results of the Moe method over 21 years column while the anterior column continues to grow in the
indicate that scoliosis can generally be arrested or improved intervals between the planned lengthenings, with no control
by repeated distractions without producing significant sagit- over rotational deformity. Rotation and kyphosis (presumed
tal plane deformities.386,760 However, significant rotational to be related to crankshafting) have been reported to be
deformity can occur because of the crankshaft phenomenon worse when short apical convex fusions are added to the
inherent in treating growing spines with posterior instru- single concave distraction rod method.763 The spontaneous
mentation only.4,511,763 The construct tethers the posterior ankylosis that may occur also acts as a posterior tether, thus

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274 SECTION II  Anatomic Disorders

FIGURE 12-73  A, Initial radiograph of a 15-month-old girl with a 100-degree infantile idiopathic curve. She weighed 5.2 kg at this time
(<5th percentile), and T1-12 length was 10.3 cm. Any surgical options using instrumentation were rejected because of the child’s size.
B, Improvement to 65 degrees in traction. C, The patient at 4 years 4 months of age following two halo traction sessions, each lasting
about 4 to 5 months, followed by bracing once the curve had been reduced to about 60 degrees. She now weighs 12 kg, and T1-12
length is 12.1 cm. Despite no improvement in the original magnitude of the curve, the delay of 3 years has allowed time for growth,  
so surgical treatment is now feasible.

making the definitive surgical correction less efficient unless period in halo traction may reduce the kyphosis and allow
anterior releases or mobilizing osteotomies are added.4 more effective growing instrumentation to be implanted
The true amount of growth achieved by the Moe method (Fig. 12-75). What remains to be determined is the long-
is limited and reported to range from 1.2 to 3.1 cm, with term pulmonary benefit, if any, in patients with potential
complication rates ranging from 11% to 30%. thoracic insufficiency who receive spinal column treatment
In an effort to improve the complication rate and only—a subject that is under prospective investigation.
outcome with “growing” instrumentation, dual-rod subcu-
taneous instrumentation (Fig. 12-74) was introduced. The Instrumentation Without Fusion: Current Technique
second rod with claw anchors at the end vertebrae was The end vertebrae are fused to an adjacent vertebra beyond
thought to provide better stability, avoid the need for long- the instrumentation. Distal fixation is frequently achieved
term bracing,13 and provide better control of rotational with double-level pedicle screws. Proximal fixation is
deformity. Early results in patients with primarily noncon- achieved with pedicle–transverse claw hook constructs,
genital deformities confirmed these advantages, with a with the levels staggered on each side (see Figs. 12-68 and
reported increase in T1-S1 length of 5 cm at the initial 12-74). The proximal level of instrumentation should be T1
procedure and an additional 4.6 cm in serial lengthenings. or T2, if possible, to avoid junctional kyphosis above the
No significant improvement occurred during the lengthen- instrumentation. Sublaminar wires may be used to augment
ing period, thus suggesting that most of the thoracic length fixation proximal to the hook or hooks, which is the most
ratio change took place with the 5-cm elongation at the frequent site of failure (Fig. 12-76). A longitudinal tubular
initial procedure. connector (the tandem connector) housing two rod seg-
The dual-rod technique rekindled enthusiasm for the ments end to end allows lengthening of the construct by
“growing rod” concept by eliminating some of the draw- distracting the rods through a slot in the connector (see Fig.
backs of the original Moe single-rod method.763 However, 12-74). This connector cannot be contoured and must be
patients with significant preoperative kyphosis continue to placed in a straight sagittal segment of the spine (usually
be poor candidates for growing rod instrumentation because the thoracolumbar junction).
of the increased likelihood of failure of the proximal anchors An alternative construct connects two overlapping rods
and the biomechanical inefficiency of correcting kyphosis with side-to-side “domino” or “wedding band” connectors.
by distraction. In these patients a preliminary treatment This construct can be contoured for sagittal curvature and

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CHAPTER 12  Scoliosis 275

FIGURE 12-74  Dual-rod subcutaneous instrumentation. A, The proximal

anchors include bilateral pedicle hook–transverse process claw constructs
over two thoracic levels. Distal consecutive lumbar segment pedicle
screws form the distal claw. Each set of anchors is connected by
submuscular rods tunneled to an overlap point, where they are linked
with side-by-side “domino” implants. Transverse cross-links to further
stabilize the anchor are optional and depend considerably on implant
profile and wound closure issues. B, Lateral view. Appropriate
contouring of the thoracic and lumbar sets of rods is crucial to avoid
long-term problems of junctional kyphosis proximally and flat back
distally. With the use of domino implants in the thoracolumbar region,
sagittal alignment can be addressed without concern about the location
of rod connectors (as with tandem connectors). C, Lengthening
procedure. Through a small incision over the domino, lengthening is
carried out at approximately 6-month intervals. D, Lengthening
procedure using end-to-end tandem connectors. Because the connector
and rods within must not bend, their location is limited to an area
where the spine is straight in the sagittal plane—usually at the
thoracolumbar junction. E, Rib–rib anchor using lamina hooks.
F, Cephalic fixation method for the rib–pelvis construct (scapula
retracted superolaterally).


Loosen and retighten

set screw Set screw

Vice grips
tight on rod



Set screw Rod

Hook Rib

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276 SECTION II  Anatomic Disorders


FIGURE 12-75  A and B, Eight-year-old boy with collapsing kyphosis secondary to spastic cervical myelopathy associated with Conradi-
Hünermann syndrome (note the previous cervical fusion). C, After 2 months in halo–wheelchair traction, the kyphosis has markedly
improved. Instrumentation without fusion can now be attempted. D and E, The patient 2 years later, after three lengthenings of fusionless
instrumentation and revision of the upper anchors.

can be placed in an optimal location for soft tissue coverage In some patients with adequate bone stock, no postopera-
(see Figs. 12-68 and 12-75). This construct also allows tive protection is necessary, and ambulatory patients are
longer overlapping rod segments and greater eventual allowed to resume normal nonvigorous activity, including
lengthening. some sports. Current practice is to routinely lengthen the
Postoperative immobilization (bracing) can be used on construct every 6 months.86
an ad hoc basis, depending on the strength and security of The ultimate spinal instrumentation and fusion are per-
the fixation, while the initial localized fusions are maturing. formed when little additional length is gained by rod

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CHAPTER 12  Scoliosis 277

FIGURE 12-76  A and B, Bilateral rib fixation (original construct) in the patient shown in Figure 12-75. C, Because of fracture of one of
the rib fixation sites, the rib anchors were revised to obtain spinal fixation, with protection of the hook site by sublaminar wires at the
next cephalic segment (compare with Fig. 12-75, E).

lengthening—ideally, not before 10 years of age. Complica- by the pins. To deal with the new problem of how to con-
tions may occur that cause the surgeon to abandon the tinue treatment as growth inevitably occurred, an expand-
fusionless method and perform an earlier fusion. able rib prosthesis had to be developed. Thus, the concept
An alternative fusionless fixation in which a subcutane- of expansion thoracoplasty by rib distraction was born, and
ous rodding technique is combined with an expansion tho- this led to the development of a unique prosthetic rib
racoplasty technique may be appropriate in certain patients implant—the vertically expandable prosthetic titanium
with chest wall dysfunction but without congenital rib rib (VEPTR) (Fig. 12-77, A). The VEPTR can be length-
abnormalities and spinal deformities. In this setting, one ened periodically to produce an opening wedge correction
that is often associated with syndromic or neuromuscular of the scoliosis from the concavity while providing chest
conditions, the patient requires correction of spinal defor- wall stability for flail segments and volume expansion of
mity and expansion of thoracic volume (see later), which is the hypoplastic thorax in a patient with, for example, fused
constricted because of rib deformity related to the spinal ribs (Fig. 12-77, B and C). Concomitant growth of the spine
deformity and often trunk weakness or rigidity. Bilateral use has been documented, even in patients with congenital
of spinal instrumentation or expandable rib prostheses in a unsegmented bars in whom it was assumed that growth of
subcutaneous technique, with the cephalic anchors being rib the concavity was impossible as a result of the congenital
rather than spine attachments and the caudal anchors seated fusion.109
on the iliac crests, can provide both thoracic expansion and The expansion thoracoplasty technique has revolution-
spinal correction without actual exposure of the spine at ized the treatment of young children with congenital scolio-
any point (see Fig. 12-76).110 Thoracic expansion takes place sis and chest wall abnormalities. Previously, treatment of
by distraction of the rib attachment on the upper part of these children emphasized stopping the progressive defor-
the thorax away from the spinal or pelvic attachment mity at all cost by early spinal fusion. As discussed earlier,
without any formal rib resection or chest wall mobilization the pulmonary cost of this early fusion has been well docu-
surgery. The results of this hybrid technique (rib to pelvis mented, with follow-up studies finding that many patients
or rib to lumbosacral spine) are still anecdotal and prelimi- suffered from thoracic insufficiency because of lack of
nary,714 but it is a promising alternative for patients with growth of the thorax (see Fig. 12-65).109,165,209,267,361
potential or actual thoracic insufficiency associated with a The ability to lengthen the thoracic spine and simultane-
collapsing noncongenital spinal deformity. ously correct the scoliosis without performing surgery on
the spine itself has been the major contribution of the
Expansion Thoracoplasty expansion thoracoplasty technique (Fig. 12-78). Other
In 1987, faced with what appeared to be untreatable scolio- radiographic parameters of growth, such as the sagittal
sis because of a congenital flail chest in a 6-month-old infant depth of the thorax (distance from the anterior body of T6
who was respirator dependent and could not be weaned, to the sternum; Fig. 12-79) and the space available for the
Campbell and associates109 implanted a “chest wall prosthe- lung ratio (relative height of the concave and convex hemi-
sis” made of vertically oriented Steinmann pins wired to the thoraces),109 can also be corrected significantly.
vestigial ribs. Not only was the child subsequently weaned Pulmonary outcomes were somewhat equivocal in
from the respirator, but the scoliosis was significantly Campbell and colleagues’ report,112 primarily because of an
improved by the rib distraction produced and maintained inability to test young patients preoperatively and thus have

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278 SECTION II  Anatomic Disorders

FIGURE 12-77  A, Evolution of the vertical expandable prosthetic

titanium rib implant, from the original implant created from
smooth Steinmann pins (far left) to the current expandable
device for rib anchorage (far right). B to D, Correction of chest
wall constriction secondary to fused ribs with congenital scoliosis
by an opening wedge thoracoplasty and implantation of an
expandable rib prosthesis. (A, Courtesy Robert M. Campbell,
Jr, MD.)


a baseline for comparison. However, patients operated on primarily infection, skin sloughing, and device migration
before 2 years of age had a significantly higher predicted or dislodgement—are comparable to those seen with
vital capacity at follow-up (58%) than did those older than single growing rod techniques. These complications can be
2 years at the initial operation (44% of predicted), thus managed satisfactorily with standard surgical wound care
attesting to the importance of early treatment during the and repositioning of the device or revision of the construct.
“golden period” of alveolar growth.168 By using volumes Brachial plexus injury from extreme cephalic placement of
obtained from CT scans of the thorax (Fig. 12-80), Emans the proximal rib cradle (first or second rib), combined with
and colleagues were able to clearly demonstrate an increased traction on the scapula during wound closure to cover the
anatomic volume of the thorax, especially the hemithorax implants, is a unique complication of expansion thoraco-
on the side where the VEPTR was placed.207 The increase plasty. Upper extremity neurologic and vascular monitoring
in CT-derived total lung volume was 147% from preopera- is required to avoid this complication. In the longer term,
tive values to the last follow-up, and the lung on the VEPTR the effects of chest wall stiffening, mentioned anecdotally
side had an increase in volume of 219%. Unfortunately, the as a consequence of serial device lengthening,207 is of suf-
correlation between such increases in anatomic volume and ficient concern that most surgeons are reluctant to use a
concomitant physiologic function tests (e.g., forced vital chest wall expansion technique in patients who do not have
capacity, forced expiratory volume in 1 second) is unknown primary chest wall anomalies.
at this time, thus making the interpretation of anatomic The technique of expansion thoracoplasty and VEPTR
changes in volume uncertain. implantation has been widely publicized,110,112,207 but
Despite an average of 1.3 to 1.8 procedures per year per indications for its use are frequently subjective. It is difficult
patient, complication rates of expansion thoracoplasty— to predict with objectivity the likelihood of thoracic

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CHAPTER 12  Scoliosis 279


FIGURE 12-78  Preoperative (A) and postoperative (B) radiographs and clinical appearance before (C) and after (D) expansion
thoracoplasty in a 4-year-old with congenital scoliosis and impending thoracic insufficiency. (Courtesy Robert M. Campbell, Jr, MD.)

insufficiency in young children and infants because “control” determined by an elevated resting respiratory rate, easy
patients are usually young adults who have undergone con- fatigability, frequent respiratory infections, hypoxia and
ventional fusion and thus may or may not have respiratory hypercapnia, primary diaphragmatic breathing, and trunk
insufficiency related to the extent and age of their fusions. and shoulder elevation and depression with respiration
A 10% reduction in the radiographic space available for (“marionette sign”).109 Paradoxical chest movement may
the lung on the convex side has been cited as an indication indicate more advanced respiratory compromise. Ideally,
for chest expansion. Clinically, thoracic insufficiency is treatment should begin before these signs of significant

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280 SECTION II  Anatomic Disorders


T6 T6-sternum
Thoracic width Thoracic depth

Thoracic length


FIGURE 12-79  A, T1-12 spinal length and coronal thoracic width at T6. B, Sagittal thoracic depth from T6 to the sternum.

FIGURE 12-80  A, Girl aged 1 year 6 months with congenital scoliosis and rib fusions. B, Computed tomography (CT) lung volumes at
the age of 2 years 6 months. (The concave right lung is seen at the left of the CT image.) C, CT lung volumes at 4 years 6 months of
age. Based on the minimal increase in lung volume, expansion thoracoplasty was indicated.

compromise are present. The window of opportunity to thoracoplasty and, indeed, any of the treatments of early-
preserve alveolar function may start to close by 2 years of onset scoliosis discussed in this chapter.
age, and earlier treatment should be considered in patients
who are clearly at risk.110 Determination of thoracic volume
by CT scanning may be useful because pulmonary function
testing is unsatisfactory in children younger than 5 years Several treatment regimens are appropriate and still under
(see Fig. 12-80),207 but its prognostic accuracy remains investigation for these difficult disorders. Serial casting and
unknown.269 bracing are appropriate approaches for children without
Design and execution of a prospective study of anatomic major chest wall deformities in an effort to delay spinal
volume outcomes of treated and control patients and cor- fusion until 10 years of age. Larger and stiffer curves may
relation of those outcomes with physiologic pulmonary respond to halo traction programs, followed by either a
function remain the greatest challenges to the development return to bracing or growing rod instrumentation. Growing
of objective indications for the rational use of expansion rod instrumentation is recommended for patients who have

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CHAPTER 12  Scoliosis 281

FIGURE 12-81  A, Clinical appearance of a 16-year-old boy with neurofibromatosis and thoracolumbar scoliosis with the convexity to the
right. B and C, Posteroanterior and lateral radiographs of the spine demonstrate the dystrophic nature of the curve, as evidenced by
erosions of the vertebral bodies.

failed both bracing and casting and for those with neuro- and management differ significantly. Dystrophic scoliosis is
muscular conditions or iatrogenic rib deformities. Expan- more common, has a greater tendency to progress, and
sion thoracoplasty is reserved for those with spinal includes a subgroup of patients (those with severe kypho-
deformities complicated by chest wall deformities that limit scoliosis) at risk for neurologic deficits.706 Nondystrophic
pulmonary function. scoliosis more closely resembles idiopathic scoliosis in both
curve patterns and behavior. It is now recognized that non-
dystrophic curves in younger children can modulate into the
more worrisome dystrophic type over the course of several
Other Causes of Scoliosis years.152
Neurofibromatosis Nondystrophic Scoliosis
Scoliosis is the most common skeletal manifestation of neu- The nondystrophic type of scoliosis has a more favorable
rofibromatosis (Fig. 12-81).151,152,377,778 Typically, it is located outlook in patients with neurofibromatosis. The clinical
in the thoracic spine; has a short, sharply angled curve; and appearance, radiographic findings, and behavior of the curve
involves four to six vertebrae. The reported incidence is tend to be similar to what is found with idiopathic scoliosis.
between 10% and 60%.§c Reports citing high incidence rates However, nondystrophic deformities usually become appar-
may have been biased, however, in that they were derived ent at an earlier age than idiopathic curves do and have a
from populations of patients with neurofibromatosis slightly higher likelihood of progressive deformity. Manage-
managed by musculoskeletal specialists. A 10% to 20% inci- ment of nondystrophic curves is similar to that for idiopathic
dence of scoliosis appears to more accurately reflect the scoliosis. Curves less than 25 degrees can be observed closely
entire population with neurofibromatosis.12 without active intervention. Brace treatment appears to be
The cause of spinal deformity in patients with neurofi- effective for skeletally immature individuals with curves
bromatosis is unknown. Proposed explanations include between 25 and 40 degrees.377 However, once the nondys-
primary mesodermal dysplasia, osteomalacia, erosion or trophic curves of neurofibromatosis exceed 40 degrees, pos-
infiltration of bone by localized neurofibromatosis tumors, terior spinal fusion with instrumentation is recommended
and endocrine disturbances.152 (Fig. 12-82). Close follow-up after surgery is needed because
Scoliosis secondary to neurofibromatosis can be either of the higher likelihood of pseudarthrosis in this population,
nondystrophic or dystrophic, depending on accompanying and over time, some nondystrophic curves evolve and
abnormalities specific to this disorder.‖c Differentiation exhibit characteristics of dystrophic scoliosis.
between the two types is important because the prognosis
Dystrophic Scoliosis
References 12, 117, 152, 187, 228, 317, 341, 706. In dystrophic scoliosis, short, sharply angled curves develop
References 108, 152, 248, 377, 403, 560, 706, 815. at an early age, often as young as 3 years. Radiographic

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282 SECTION II  Anatomic Disorders


FIGURE 12-82  A and B, Clinical appearance of a boy aged 7 years 3 months with neurofibromatosis and right thoracic scoliosis. He had
previously undergone resection of several plexiform neurofibromas of the chest wall. C, Radiographically, the 55-degree thoracic curve
showed no dystrophic changes. D, No localized area of increased kyphosis was present. E, Posterior instrumentation and fusion were
performed. The child’s short stature allowed the use of only a single rod. Because the crankshaft phenomenon was highly likely to occur
with subsequent anterior growth, anterior fusion was performed at the same time. F, Two years later, the curve correction was
maintained, and solid fusion was evident anteriorly. G, Sagittal plane alignment remained satisfactory.

features that help differentiate dystrophic from nondystro- of neurologic deficits. Kyphosis may occur in one of two
phic curves include vertebral scalloping, spindled transverse ways. An abrupt angular kyphosis may be present in the
processes, severe apical vertebral wedging and rotation, very early stages of the deformity, or a more gradual kyphos-
foraminal enlargement, defective pedicles, penciling (nar- ing scoliosis might result from progression and rotation of
rowing of the proximal portion) of the ribs, the presence of the scoliosis (Fig. 12-83).248 Recognition of either type is
paravertebral soft tissue lesions, and rarely, subluxation important because once kyphosis is established, prompt
between vertebral bodies. Some of these findings may result combined anterior and posterior spinal fusions are required.
from direct erosion of the bone by intraspinal neurofibro- Nonoperative management of dystrophic scoliosis is
mas, paraspinal neurofibromas, or dural ectasia. Dural almost always unsuccessful. These curve patterns need early
ectasia is an expansion in the width of the thecal sac thought and aggressive surgical intervention, even in a young child.
to be due to an increase in hydrostatic pressure. Delay leads only to progressive deformity, which may be as
Fortunately, most dystrophic curves are not accompanied rapid as 8 degrees per year in the frontal plane and 11
by an excessive amount of kyphosis.706 Individuals with this degrees per year in the sagittal plane.108 Most patients
combination have significant potential for the development exhibit marked progression before 10 years of age, and

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CHAPTER 12  Scoliosis 283

FIGURE 12-83  A and B, Severe kyphosis in a 15-year-old girl with neurofibromatosis. She remained ambulatory but complained of
progressive weakness. C, A radiograph obtained elsewhere at 10 years of age showed a scoliosis of approximately 60 degrees and
moderate thoracic kyphosis, but no intervention was undertaken.

severe deformity can be seen before the adolescent growth occur in patients with neurofibromatosis, even in those with
spurt. Characteristics of dystrophic scoliosis that correlate a solid arthrodesis.815
with excessive risk for progression include early age at Severe kyphoscoliosis absolutely requires anterior fusion
onset, a high Cobb angle at the time of initial evaluation, in addition to posterior fusion. Thorough anterior diskec-
and the presence of vertebral scalloping, penciling of mul- tomy, bone grafting, and rib (or tibia) strut graft placement
tiple ribs, and apical vertebral rotation exceeding 11 degrees are needed (Fig. 12-85). In some patients with exaggerated
(Perdriolle measurements590).248 kyphosis, the apical rotation may be so severe that the
Before surgery, a thorough neurologic examination is vertebral body faces posterolaterally. With this deformity,
essential to identify any subtle abnormalities. MRI and CT placement of the strut graft can be extremely difficult, and
should always be performed.778 MRI demonstrates neurofi- the anterior approach to the spine may need to be under-
bromatosis lesions in the neck, thorax, paravertebral region, taken from the concave side. Vertebral body erosion sec-
neural foramina, or spinal canal. Chiari I malformations, ondary to intrathoracic neurofibroma or dural ectasia can
dural ectasia, pseudomeningoceles, and spinal cord com- also significantly interfere with anterior exposure and
pression (secondary to localized kyphosis, rib impingement, fusion. Dysplastic posterior elements limit the ability to
or a mass effect from neurofibromas) can also be detected achieve strong posterior internal fixation. Every effort
with MRI.¶c CT demonstrates scalloping of the vertebral should be made to stabilize the spine because stabilization
bodies anteriorly, erosion of the posterior portion of the improves the likelihood of a successful outcome. Postopera-
vertebral body or lamina from dural ectasia, and the pres- tive immobilization in a cast or orthosis is clearly indicated
ence of ribs within the spinal canal (Fig. 12-84).1,780,857 when the vertebrae are weak, the severity and location of
Three-dimensional CT reconstruction is invaluable in clari- the kyphosis cause excessive strain at certain hook or screw
fying the anatomy of severe deformities and is helpful in sites, or the quality of bone does not allow fixation points
preoperative planning. for instrumentation. Despite meticulous attempts at ante-
Posterior spinal fusion with instrumentation alone can be rior and posterior fusion, pseudarthrosis is a significant
used for certain patients with dystrophic curves between concern.152,706,835
20 and 50 degrees and kyphosis of less than 50 degrees (no Excessive kyphosis is the most frequent cause of neuro-
sharp angulation). Because the risk for pseudarthrosis is logic deficits in patients with neurofibromatosis and spinal
higher than that in the idiopathic scoliosis population, con- deformities. Should a neurologic deficit be present, VCR is
sideration should be given to performing imaging studies needed to decompress the spinal cord. Laminectomy for
(tomography) 6 months after surgery. If the fusion mass spinal cord decompression or prophylactic laminectomy for
appears inadequate, repeated bone graft augmentation may kyphoscoliosis should be avoided because it destabilizes the
be necessary. spine, increases the kyphosis, removes bone stock needed
Anterior fusion in addition to posterior fusion is needed for successful posterior fusion, and most important, does
for most patients with dystrophic curves.291,389,583,704 The not relieve the anterior compression on the spinal cord.
combination of anterior and posterior fusion increases the Neurologic deficits can also result from cord impingement
likelihood of successful fusion. Longer fusions are generally by neurofibroma lesions within the spinal canal.600 Differ-
indicated, even in young patients. Curve progression can entiation of neurofibroma impingement from kyphotic
impingement is required to correctly address the problem
References 28, 170, 252, 375, 376, 615, 777, 779. surgically. MRI can help clarify the situation.

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284 SECTION II  Anatomic Disorders




FIGURE 12-84  A and B, Clinical appearance of a girl aged 14 years 4 months with an extremely large café au lait discoloration on her
trunk. C and D, Radiographs show 54-degree lumbar scoliosis with flattening of the L3 body and posterior scalloping of numerous lumbar
vertebrae. E and F, Preoperative magnetic resonance imaging shows a large neurofibroma in the concavity of the lumbar spine. G and H,
Computed tomography demonstrates significant enlargement of the spinal canal, narrow depth of the vertebral bodies, thin posterior
elements, and spondylolysis of the pars and pedicle of L3. I to L, Anterior instrumentation and fusion between L1 and L4 improved the
patient’s frontal and sagittal plane balance.
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CHAPTER 12  Scoliosis 285


FIGURE 12-85  A and B, Radiographic appearance of a boy aged 3 years 2 months with neurofibromatosis and severe upper thoracic
kyphoscoliosis. C and D, A rib strut was placed anteriorly, and a single rod was used posteriorly during fusion. E and F, Nearly 5 years
later, frontal plane alignment remains satisfactory, but the kyphosis is increasing above the instrumentation.

The sagittal profile of the spine in Marfan syndrome

Marfan Syndrome
varies widely and requires close examination when planning
Marfan syndrome, one of the more common connective treatment.347 Increased lordosis in the thoracic spine was
tissue disorders, has a 0.01% prevalence in the general pop- thought to be common,822,825 but one report found that
ulation.722 Scoliosis is the most common spinal deformity in increased thoracic kyphosis (>50 degrees) may be found in
this condition, with a prevalence approaching 63%.647,648,722,756 as many as 45% of patients with Marfan syndrome and
In addition, 6% of patients with Marfan syndrome have scoliosis.722 When the transitional point between thoracic
spondylolisthesis. Although Marfan syndrome is an autoso- kyphosis and lumbar lordosis is below the second lumbar
mal dominant disorder, no familial pattern of scoliosis has vertebra, a long, broad thoracic kyphosis is evident. If a
been identified. localized kyphosis exists at the thoracolumbar junction, the
The curve patterns seen in Marfan syndrome are similar thoracic spine above is generally hypokyphotic.
to those seen in idiopathic scoliosis, although Marfan syn- Back pain is more frequent in patients with Marfan syn-
drome has a slightly higher rate of triple curves and thora- drome than in the general population. However, no signifi-
columbar curves (Fig. 12-86). Scoliosis is equally distributed cant difference in back pain between patients with scoliosis
between males and females, in contrast to the female pre- and those without has been noted. Back pain is associated
ponderance in idiopathic scoliosis. Limb length discrepan- with the presence of dural ectasia, a finding that is more
cies in patients with Marfan syndrome are associated with common in those with Marfan syndrome than in the normal
increased structural scoliosis.348 population.10,11,223,281,721

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286 SECTION II  Anatomic Disorders

FIGURE 12-86  A and B, Clinical appearance of a girl aged 12 years 2 months with Marfan syndrome. C and D, Despite progressive
thoracic and thoracolumbar scoliosis, the spine remains well balanced. Brace treatment was unsuccessful in preventing curve progression.

No well-defined natural history studies of scoliosis in Marfan syndrome tend to have a higher incidence of pseud-
patients with Marfan syndrome exist, although certain arthrosis, although its true incidence is unknown.347,397 A
trends are evident. Curves identified in infancy progress higher incidence of perioperative complications, including
dramatically.723 These curves do not resemble the curves of increased blood loss, infection, and curve decompensation,
infantile idiopathic scoliosis in that they are not expected has been reported.347
to resolve spontaneously and are largely right thoracic in Instrumentation and fusion in patients who are identified
configuration. In older but still skeletally immature patients, during infancy usually produce modest correction, in the
all curves greater than 30 degrees will probably progress at range of 20%.723 Surgery should be delayed until the child
least 10 degrees and will reach at least 40 degrees by is older than 5 years. When surgery is performed earlier,
maturity. many patients with large curves die of cardiac complica-
Unfortunately, brace treatment is not effective in con- tions. If possible, anterior fusion should be added to prevent
trolling scoliosis in Marfan syndrome,70,171,397,722,723,822 with a development of the crankshaft phenomenon or to address
reported success rate of just 17%.721 Most skeletally imma- excessive kyphosis.
ture patients (Risser grade 2 or less) with curves exceeding Relative contraindications to performing corrective
25 degrees will reach the stage at which surgery is necessary, surgery for spinal deformity in patients with Marfan syn-
even with brace treatment. In infants, curves almost always drome include cardiac insufficiency and a dissecting aortic
progress to the point of needing operative intervention. aneurysm. These conditions should be treated before ortho-
Nevertheless, bracing curves less than 40 degrees in infants paedic intervention is undertaken. Splenic rupture has been
may be a useful technique for postponing surgery. This is reported following posterior spinal instrumentation.132
important because in children with Marfan syndrome and
scoliosis, surgical intervention before 4 years of age is associ-
Congenital Heart Disease
ated with significant cardiac morbidity. Bracing in older
children may also be temporarily beneficial in that it may The association between scoliosis and congenital heart
allow sufficient maturity to be gained that only posterior disease is well established.#c Because of advances in heart
surgery is needed. surgery, children are now living longer than in the past, and
Spinal stabilization for scoliotic deformities is indicated many with severe scoliosis are candidates for operative
when the magnitude of the curve exceeds 45 degrees in correction.
adolescents or 50 degrees in adults. Because of an increased The incidence of scoliosis associated with congenital
risk for atlantoaxial rotatory instability, special attention to heart disease is approximately 4%.694 For those with con-
intubation and positioning, both intraoperatively and post- genital heart disease who have undergone cardiac surgery,
operatively, is necessary.310 The spinal procedure of choice the incidence of scoliosis is higher (11% to 28%).308,309,366
is posterior spinal fusion with segmental instrumenta- This observation has led some authors to conclude that
tion.184,352,397,436,822 When compared with idiopathic scolio- performance of thoracotomy or sternotomy in patients with
sis, patients with Marfan syndrome will have atypical congenital heart disease may be associated with the devel-
curve patterns; require more levels of surgical correction, opment of scoliosis.309,366,393 One study reported that scolio-
more distal fusion, greater correction of sagittal balance, sis exceeding 20 degrees later developed in 12.5% of 128
and more reoperations; and have more cerebrospinal mature patients who underwent median sternotomy for the
fluid leaks and instrumentation-related complications (Fig. treatment of congenital heart disease before 8 years of
12-87).171,259,260,347 Thoracolumbar or lumbar kyphosis may age.657 Patients operated on before 18 months of age had a
require anterior release before posterior instrumentation to
achieve sufficient sagittal plane flexibility. Patients with #c
References 48, 54, 219, 351, 366, 470, 562, 595, 622, 651, 694.

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CHAPTER 12  Scoliosis 287


FIGURE 12-87  A and B, The patient shown in Figure 12-86 underwent posterior instrumentation and fusion, and excellent correction was
achieved. C and D, Five months later she noticed worsening balance in the lower part of her spine. Radiographs showed that the inferior
portion of the instrumentation had dislodged. The patient was decompensated to the left and had lost correction of the lumbar curve.
The lower segment was revised with pedicle screws, rod extensions, and rod cross-links. E and F, Three years later her balance remained
improved and she was asymptomatic.

significantly increased risk for the development of scoliosis Two types of scoliosis are seen in conjunction with con-
when compared with those operated on later. The presence genital heart disease: congenital scoliosis and developmental
of scoliosis was not related to the type of congenital heart scoliosis. In congenital scoliosis, the curve patterns and
disease. Although surgical intervention for heart disease in natural history appear to be unaffected by the coexisting
children may be associated with the development of scolio- heart disease. Curve progression requires limited spinal
sis, it appears that these two events have a multifactorial fusion performed according to the standard guidelines
relationship. described previously in the section on congenital scoliosis.

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288 SECTION II  Anatomic Disorders

Children with developmental curves are initially seen at Those with mild developmental curves (<30 degrees)
an average age of 11 to 14 years.219,309,366 Left and right require only observation. In children with developmental
convexities occur with equal frequency. Usually, however, curves exceeding 30 degrees, the curves may progress
convex left thoracic curves are found in the upper thoracic as much as 9 degrees per year, tend not to respond
spine, and convex right curves are seen in the lower thoracic to bracing, and are likely to require posterior spinal
region.366 No significant relationship between the age of the fusion. Before spinal surgery is initiated, repair of cardiac
child at the time of cardiac surgery, the age at the onset of anomalies or temporary cardiac shunting procedures should
scoliosis, and the severity of the scoliosis has been reported be completed. Intraoperative management by experi-
(Fig. 12-88). enced cardiopulmonary anesthesiologists and postoperative


FIGURE 12-88  A and B, Clinical appearance of a girl aged 9 years 6 months who was born with double-outlet right ventricle mitral
atresia. She had previously undergone pulmonary artery banding, a Blalock-Taussig shunt, and bilateral Glenn shunts with takedown  
of the Blalock shunt. C, The initial radiograph showed a 70-degree thoracolumbar curve accompanied by a small thoracic prominence.
D, The patient underwent anterior instrumentation and fusion between T11 and L3. Postoperatively, the curve measured 40 degrees.
E, Four years later the scoliosis had changed. The patient had a 67-degree thoracic curve and a 62-degree lumbar curve. F and G, The
curves were stabilized with posterior hook-rod instrumentation (circa 2001).

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CHAPTER 12  Scoliosis 289

intensive care are requisite for orthopaedic surgical plane deformities, scoliosis and rotatory deformities may
intervention.144,802 occur. Many of the conditions for which laminectomies are
performed (e.g., trauma, neurofibromatosis, syringomyelia)
can, by themselves, produce a spinal deformity.
Spinal deformities develop in more than 50% of children
Many patients in whom scoliosis develops after thoracot- undergoing multilevel laminectomies in the cervical
omy have congenital heart disease,657,792 but other condi- region.27,50 Most of these deformities are kyphotic and
tions requiring thoracotomy (e.g., repair of tracheoesophageal usually span short segments. Some of the deformities are
fistula) can also lead to scoliosis.813,846 Most, but not all more gradual and involve more vertebrae (Fig. 12-89).
curves have the convexity toward the operated side. On Swan-neck lordotic deformities can also occur and are
occasion, two ribs fuse together at the thoracotomy site and thought to represent compensatory mechanisms to maintain
function as a tether. In this instance the concavity of the alignment of the head over the thorax.
scoliosis is toward the operated side. In the thoracic spine, kyphosis may occur as short,
Young patients who have a large number of ribs resected sharply angled deformities or may have a more gradual angle
or who have undergone multiple thoracotomies are at higher and extend over several vertebrae (Fig. 12-90). These sagit-
risk for the development of scoliosis.172,199 Usually, resection tal plane abnormalities result from the destabilizing effect
of the posterior portion of the ribs leads to the deformity. of laminectomies, during which the spinous processes,
Anterior resection of the ribs does not tend to produce interspinous and supraspinous ligaments, laminae, and liga-
significant scoliosis. menta flava are all removed. Preservation of the facet joints
Brace treatment of larger curves is usually ineffective, is important because they contribute significantly to stabil-
possibly because of the inability to apply corrective force to ity of the spine.531,595
the abnormal chest wall. Operative intervention with pos- Several other factors may contribute to postlaminectomy
terior spinal instrumentation generally results in a successful kyphotic spinal deformities. Vertebral body defects caused
outcome. by trauma or tumor (e.g., eosinophilic granuloma) lessen the
resistance to compressive flexion forces. Radiation therapy,
which is often required in patients who have undergone
laminectomies for spinal cord tumors, damages the growth
Spinal deformity following a one-level laminectomy is plates and thus adds to the deformity.169,365,585
uncommon.595 Usually, the deformities encountered in chil- Scoliosis occurs less frequently than sagittal plane defor-
dren result from multilevel laminectomies performed for mity. It also generally involves the area of the laminecto-
intraspinal tumors or trauma.104,169,365,569,578,585 The age of the mies. Less commonly, collapsing scoliosis develops below
patient and the anatomic level of the laminectomy are this area. In this case it is usually related to a neuromuscular
important factors. Laminectomies performed in the cervical deficit that resulted from a spinal cord tumor or its
or thoracic spine commonly lead to progressive kyphosis. treatment.
When laminectomies are performed in the lumbar spine, In the lumbar spine, extensive laminectomy may result
excessive lordosis may result. In addition to these sagittal in lumbar hyperlordosis.452 Rhizotomy, which has recently

FIGURE 12-89  A, Radiographic appearance of a girl aged 5 years 8 months with excessive cervicothoracic kyphosis secondary to multiple
laminectomies. B, At 10 months of age a small cell neural ectodermal tumor of the cervical spinal cord had been diagnosed. Craniotomy
and laminectomies down to T5 were performed, followed by chemotherapy and radiation therapy. At the age of 5 years 8 months she
underwent posterolateral fusion with an autogenous iliac crest used as the graft material; this was followed by immobilization in a halo
vest. C, Three years later the fusion remained solid, with excellent sagittal plane alignment.

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290 SECTION II  Anatomic Disorders

FIGURE 12-90  A, A 13-year-old boy was evaluated because of increasing kyphosis in the thoracic spine. It measured 62 degrees
between T5 and T10. B, One year previously he had undergone resection of an arachnoid cyst through laminectomies over three
levels. C, Magnetic resonance image showed no cyst but did demonstrate progressive localized kyphosis. D, Anterior and posterior fusion
was performed with posterior titanium instrumentation. The improvement seen in the sagittal plane at 6 months has been maintained
over time.

become popular in the treatment of cerebral palsy, requires column doses exceeding 1000 rad may have an inhibitory
lumbar laminectomies to expose the nerve rootlets. effect on the physeal regions.543 As a result, asymmetric
Although rhizotomy reportedly does not result in increased growth may develop and lead to a scoliotic or kyphotic
changes in sagittal plane deformities,594 at our institution deformity. Spinal deformity may also result from soft tissue
several examples of relentlessly progressive postoperative fibrosis and contractures.
hyperlordotic deformities have been encountered. This Very young children who undergo radiation therapy
same finding was reported elsewhere in 50% of 34 patients (often for Wilms tumor or neuroblastoma) are at greatest
after selective dorsal rhizotomy.342 risk for the development of spinal deformities.365,484,491,585,642,643
Treatment of an established postlaminectomy spinal Long-term follow-up of these individuals is necessary
deformity generally requires further stabilization surgery. because the deformities can worsen notably during the ado-
Therefore, all effort should be directed toward preventing lescent growth spurt. Every effort should be made to
this deformity. Laminoplasties in the thoracolumbar spine exclude the spine or pelvis from the radiation field in young
at the time of initial surgery have been helpful in preventing children.
the occurrence of kyphosis.510,691 Facetectomies should be Bracing has not proved effective in arresting the progres-
avoided if possible. Before tumor resection, neurosurgeons sion of irradiation-induced spinal deformity. However, it
and orthopaedic surgeons should discuss whether immedi- continues to be used in patients with scoliosis exceeding 20
ate operative fusion would be beneficial. degrees in an effort to delay progression of the deformity.
Preventive bracing of the spine following laminectomies When the scoliosis exceeds 40 to 45 degrees, operative
has not been effective in stopping subsequent deformities. intervention should be undertaken. Healing may be pro-
Therefore, if a brace is used, it should be understood that longed, and consideration should be given to repeated bone
it may provide only temporary benefit. Once notable kypho- grafting 6 months postoperatively. The risk for postopera-
sis has been identified in the cervical or thoracic spine, surgi- tive complications, including pseudarthrosis and infection,
cal fusion is needed to correct and stabilize the deformity. is increased.
In the thoracic spine, if the deformity is mild, posterior For postirradiation kyphosis, anterior fusion is needed
fusion with instrumentation may be sufficient. If the defor- along with posterior fusion in an effort to avoid the likeli-
mity is more severe or if the kyphotic deformity is short hood of pseudarthrosis.561 For sharply angled kyphotic
and sharply angled, anterior fusion (with a rib strut graft as deformities, a vascularized rib strut graft is recommended.
needed) should precede the posterior spinal instrumenta- Postoperative bracing for 6 to 12 months should be consid-
tion and fusion.569 In the cervical spine, anterior fusion is ered in these patients.
often performed in combination with posterior fusion.27,50
Hysterical Scoliosis
Hysterical scoliosis is a diagnosis of exclusion.8,561,757 The
For some spinal cord tumors, radiation therapy may be the curvature generally has a long C-shaped appearance, with
primary form of treatment. In a growing child, vertebral trunk imbalance, lack of abnormal neurologic or other

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CHAPTER 12  Scoliosis 291

physical findings, and no radiographic evidence of vertebral been ruled out, treatment of hysterical scoliosis requires
rotation. A change in the pattern or severity of the scoliosis psychological (or psychiatric) therapy. Orthotic manage-
from day to day may be seen. The curve generally resolves ment should not be undertaken because it may reinforce
when the individual is supine. the underlying personality disorder.
A thorough neurologic evaluation is necessary to rule out
rare causes such as spinal cord tumor. Bone scanning and
MRI may be necessary for confirmation. Laboratory studies
(complete blood cell count with differential and sedimenta- For References, see 
tion rate) can rule out infection. Once organic causes have

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CHAPTER 12  Scoliosis 291.e1

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Cotrel-Dubousset (CD) instrumentation for King type II and III congenital scoliosis. An analysis of the cases of two hundred and
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16:1124, 1991. history and treatment as observed in a study of one hundred and
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1994. rior and posterior fusion for congenital kyphosis. A case report,
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scoliosis, Spine 25:2326, 2000. to familial idiopathic scoliosis, Spine 25:2372, 2000.
817. Willers U, Hedlund R, Aaro S, et al: Long-term results of 841. Wise CA, Gao X, Shoemaker S, et al: Understanding genetic
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291.e20 SECTION II  Anatomic Disorders

850. Wyatt MP, Barrack RL, Mubarak SJ, et al: Vibratory response in 866. Youkilis AS, Quint DJ, McGillicuddy JE, et al: Stereotactic navi-
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292 SECTION II  Anatomic Disorders

Plate 12-1  Exposure of the Spine for Posterior Instrumentation and Fusion


Operative Technique
General anesthesia is administered via endotracheal intu-
bation. Intravenous access is obtained, followed by place-
ment of a radial arterial line. Perioperative antibiotics,
usually first-generation cephalosporins, are administered.
A, Positioning the patient. Under the supervision of the
surgeon, the patient is placed prone on an OSI frame. Gel
pads may be placed over the four support pads to further
cushion the chest and inguinal region. The abdomen is free
of any contact to minimize blood loss. The upper pads rest
on the upper part of the chest just lateral to the nipple
region. The shoulders are abducted and the elbows are
flexed. The axillae should be free of pressure, without
stretching across the brachial plexus or pressure over the B
ulnar nerve (at the elbow). The lower pads make contact
at the ilioinguinal region. Unless the lateral femoral cuta-
neous nerve is padded satisfactorily, pressure can lead to
temporary postoperative dysesthesia in the anterior aspect
of the thigh. When instrumented into the lumbar spine,
the hips should be extended by elevating the legs with
pillows placed under the anterior part of the thigh to down through the dermis into subcutaneous tissue. An
ensure that lumbar lordosis is maintained. alternative to this technique is to infiltrate the intradermal
The entire back is prepared with povidone-iodine tissue with epinephrine and then sharply incise down to
(Betadine) or chlorhexidine, beginning at the base of the the subcutaneous tissue. Self-retaining retractors are next
hairline and continuing to the gluteal cleft. Both iliac crests placed into the wound to keep the skin edges under
are included in the surgical field. After preparation and tension and provide exposure of the spinous processes.
draping, a Betadine-impregnated, sticky drape is applied. (Although minimally invasive surgery has been attempted
An adequate area must be exposed so that the incision for adolescent idiopathic scoliosis, the results have not
never extends to the edge of the drapes. been widely reported and it probably does not have any
B, Incision. The length of the skin incision is determined significant advantages. Early reports suggest longer surgery
by the number of levels requiring fusion. The incision is and little benefit with respect to shorter hospital stays,
taken down to the dermis with a scalpel. To minimize and it is far too early to determine the incidence of pseud-
bleeding, electrocautery is used to continue the incision arthrosis, a significant risk given the limited exposure.674)

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CHAPTER 12  Scoliosis 293

Cobb periosteal elevator

reflecting cartilaginous
caps to expose spinous
processes on both sides

Incision of periosteum
over spinous processes
to bony tip

NOTE: Subperiosteal stripping
to facet joints begins distally
and proceeds proximally in the
T4 thoracic spine


C, Once the spinous processes have

been exposed, the median raphe is
incised sharply down to bone. A
Kelly clamp may provide proper ori- processes
entation for the incision. Dissection exposed
in this avascular plane minimizes
blood loss.
D, Cobb elevators are used to sub-
periosteally expose the posterior
elements. The exposure extends lat-
erally to the tips of the transverse
processes of all the levels included
in the fusion. Meticulous dissection
should be performed to prepare the
posterior elements for fusion.
E, As the subperiosteal dissection
continues, each level is packed firmly NOTE: Fluoroscopy is
with gauze to minimize bleeding. used at this step to
F, Once the dissection is completed, E confirm correct level of
the packing is removed and self- exposure
retaining retractors are placed at the
proximal, distal, and intermediate
areas. Further cleaning of the
surgical field is then performed with rongeurs, curets, and After exposure of the surgical field, anchor sites (hooks,
electrocautery. wires, or screws) are prepared. Preoperative planning for
Intraoperative fluoroscopy is used to confirm the proper proper hook and screw placement should be noted on the
levels for fusion. Regardless of the surgeon’s expertise, radiograph and should be familiar to all those assisting in
fluoroscopy should always be performed to avoid inadver- the operation (see Fig. 12-28 for techniques to select
tent selection of the wrong vertebral level. appropriate hook sites).

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294 SECTION II  Anatomic Disorders

Plate 12-2  Posterior Spinal Instrumentation and Fusion Using Hooks


Implant Components C, The cephalic-facing laminar hook is placed after a sharp

lamina elevator is used to dissect the ligamentum flavum
A and B, The first step in placing a pedicle hook is to
from the ventral surface of the lamina. Caudal-facing
remove a portion of the inferior facet with an osteotome.
laminar hooks are placed after excision of a portion of the
When the hook is inserted against the pedicle, the shoe of
inferior lamina above the interspace and removal of a
the hook should abut the remaining lamina in a snug fit.
portion of the ligamentum flavum. The hooks should have
a “press fit” with the lamina.

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CHAPTER 12  Scoliosis 295


D, The hooks are placed in the “right thoracic pattern,”

which consists of four hooks, two facing cephalically and
two caudally. Before rod placement, facetectomies are per-
formed on the concavity, and the laminae and transverse
processes are decorticated.
E, After bone graft is placed over the exposed laminae, a
rod that has been contoured to the curvature is placed.
Eyebolts on the rod are seated into the hooks or screws by
using seating devices such as the “corkscrew.” The nuts are
tightened moderately and the hooks distracted lightly to
better seat them. The rod can then be rotated with the hex
wrench. The nuts must not be so tight that they rotate the
hooks and may need to be adjusted during the rotation
maneuver. (Caution: injury to the spinal cord could occur
if the hooks are allowed to rotate as the rod is rotated.)
F, An intermediate thoracic lamina hook has been placed
into the canal via a laminotomy. A corkscrew device is used
to push the rod and preplaced eyebolt down into the
uprights of the hook.
G, Once the rod is provisionally secured to the hooks, the G
rotational maneuver is performed. The spine lengthens
slightly with this maneuver. Distraction should be main-
tained between the intermediate hook sites to ensure that
they stay engaged during the rotation maneuver.

Continued on following page

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296 SECTION II  Anatomic Disorders

Plate 12-2  Posterior Spinal Instrumentation and Fusion Using Hooks, cont’d


H, The spinal deformity is corrected by rotating the rod downwardly directed lamina hook over the transverse
90 degrees. Rotation is greatly facilitated by using the process at the most cephalic vertebral site. An upwardly
small wrench over the hexagonal end of the rod. On occa- directed pedicle hook is placed one level lower. These two
sion, a vise grip applied to the distal end of the rod can hooks are then compressed along the rod to provide firm
assist in this rotation maneuver. Rotation should be per- proximal fixation. Once the claw is firmly secured on the
formed slowly to avoid possible intermediate-level hook uppermost segments of the convex rod, compression can
disengagement. The most cephalic pedicle hook and most be applied along the rod at the remaining hook sites.
caudal lamina hook usually maintain excellent purchase J, An alternative method of fixation of the proximal site
during the correction maneuver. of the convex rod is to use a downwardly directed sub-
The amount of curve correction is greatly determined laminar hook. This configuration is used if the transverse
by the flexibility of the spine. All the correction is achieved process site does not provide satisfactory fixation for the
with the first rod. After rod rotation, slight distraction is claw configuration. At the intermediate hook site on the
placed at each of the hook sites to ensure firm purchase. convex rod is an upwardly directed pedicle hook, which
The nut on each eyebolt is tightened completely. is usually the most prominent hook in this two-rod con-
I, Further correction of the spinal deformity should not struct. If this hook is too prominent, it may be excluded.
be expected with the second rod. However, this rod At the inferior hook site on the convex rod is an upwardly
does significantly increase the torsional strength of the directed sublaminar hook. The eyebolts for the cross-link
construct. The proximal end of the rod is secured to the should be placed on the rod before engaging the rod with
spine with a “claw.” This claw is created by placing a the hooks.

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CHAPTER 12  Scoliosis 297

Plate 12-3  Posterior Spinal Instrumentation and Fusion Using Pedicle Screws


Unsafe Cephalad-Caudad Starting

A Safe Level

T1 T1 Midpoint TP

A, Safe placement of segmental pedicle T2 T2 Midpoint TP

screws is essential. The starting point
must never be medial to the midpoint of
the superior facet. (Redrawn from
T3 T3 Midpoint TP
Medtronic Sofamor Danek USA, Inc.,
Memphis, Tenn, with permission.)
B, Using the straightforward approach,
the thoracic vertebral cephalad–caudad T4 T4 Proximal third TP
starting points vary slightly depending on
the levels being instrumented. TP, Trans-
verse process. (Redrawn from Medtronic
Sofamor Danek USA, Inc. Memphis, T5 T5 Proximal third TP
Tenn, with permission.)

T6 T6 Proximal third TP

T7 T7 Proximal TP

T8 T8 Proximal TP

T9 T9 Proximal TP

T10 Junction:
Proximal edge–
proximal third TP

T11 T11 Proximal third TP

T12 T12 Midpoint TP

Continued on following page

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298 SECTION II  Anatomic Disorders

Plate 12-3  Posterior Spinal Instrumentation and Fusion Using Pedicle Screws, cont’d

1 3

2 4

C, 1 and 2: Preparation for the pedicle screw sites should 4: After insertion to 15 to 20 mm, the pedicle finder is
begin at the most distal vertebra to be instrumented and reversed (medial orientation) to continue penetration into
proceed cephalad. Either the freehand technique or the the vertebral body to a depth approximating 30 to 40 mm,
fluoroscopy-guided technique can be used. A thoracic gear- depending on the vertebra level. Once this is accomplished,
shift (2-mm blunt-tipped curved pedicle finder) is used to a ball-tipped probe is used to palpate all four walls of the
enter the pedicle. pedicle and the floor (anterior vertebral body). The depth
3: To avoid medial wall penetration, the gearshift is ini- that is measured will approximate the screw length that will
tially pointed laterally when the pedicle is entered. be needed. (From Kim YJ, Lenke LG, Bridwell KH, et al:
Free hand pedicle screw placement in the thoracic spine: is
it safe? Spine 29:333, 2004.)

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CHAPTER 12  Scoliosis 299



D, 1: The pedicle is undertapped (over a guide pin) 0.5 to

1.0 mm smaller than the intended screw diameter.
2: The screw is then slowly inserted freehand.
E, All screws are placed.
1a and 1b: Preoperative anteroposterior (AP) and lateral
radiographs of a Lenke 6C curve.


Continued on following page

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300 SECTION II  Anatomic Disorders

Plate 12-3  Posterior Spinal Instrumentation and Fusion Using Pedicle Screws, cont’d




F, 1: A precountoured provisional convex right lumbar rod

is placed.
2: Following rod rotation of the lumbar provisional right


2: Fluoroscopic images demonstrating accurate place-

ment of the lumbar screws.
3: Lumbar screws.

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CHAPTER 12  Scoliosis 301




G, 1: The left final rod with a precontour is placed with H, Final AP and lateral radiographs at 2 years after surgery
the right thoracic kyphosis and left lumbar lordosis while demonstrating good correction in the AP and lateral
the provisional right lumbar rod is in place. planes.
2: An apical derotation maneuver is performed via the
en bloc rotation technique with the left rod in place.
3: The left rod is in place.

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302 SECTION II  Anatomic Disorders

Plate 12-4  Anterior Instrumentation of the Spine for Thoracolumbar or Lumbar Scoliosis

Incision Cut line at P

costocartilage angle r

(along path of 10th rib)
Spinous process of T10

Periosteal bed of
resected 10th rib

Sutures are
placed prior
to splitting
of cartilage

10th rib cartilage

is split with scalpel

requiring instrumentation. For instrumentation between

Operative Technique
T11 and L3, removal of the tenth rib allows adequate
In this plate the thoracoabdominal approach for exposure exposure.
of the lower thoracic and lumbar spine is described. Skin incision. The incision begins lateral to the spinous
A, Positioning. Under the direction of the surgeon, the process of T10 (or T9) and extends along the course of the
patient is placed in the lateral decubitus position (the con- tenth rib to the costocartilaginous junction and then across
vexity of the curve is upward). A roll is placed under the the upper part of the abdomen to the lateral edge of the
axilla of the dependent arm. The body is supported with a rectus abdominis. Here, it turns distally toward the sym-
deflatable beanbag. The upper part of the arm is flexed physis pubis and stops at the level of the umbilicus.
forward and slightly abducted. The operating table may be B, The tenth rib is freed subperiosteally, divided at its cos-
temporarily flexed (at the apex of the scoliosis) to facilitate tocartilaginous junction, and removed. This creates a larger
excision of the intervertebral disks. working aperture and provides a source of autogenous bone
Approach. It will be necessary to remove a rib for expo- graft.
sure of the spine. Ideally, the rib that is removed is the one C, Once the costal cartilage of the tenth rib is split, the
immediately cephalad to the uppermost vertebral body retroperitoneal space is identified and entered.

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CHAPTER 12  Scoliosis 303

Guide sutures placed on either side of intended

line of section of diaphragm

Retroperitoneal space

Finger inserted into abdominal

cavity to strip peritoneum from
undersurface of diaphragm

Diaphragm (free from peritoneum

on undersurface) is divided
circumferentially 1.5 cm from
its costal attachment

D, Using blunt finger dissection, the operator separates the to ¾ inch from its periphery. Placement of several of these
peritoneum from the inferior aspect of the diaphragm. sutures facilitates proper closure of the diaphragm later.
Once freed, the viscera lie safely away from the vertebral E, The diaphragm is sectioned from its costal
bodies. Identification sutures are placed on either side of attachments.
the intended line of division of the diaphragm, which is ½

Continued on following page

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304 SECTION II  Anatomic Disorders

Plate 12-4  Anterior Instrumentation of the Spine for Thoracolumbar or Lumbar Scoliosis, cont’d

Line of division of parietal pleura

Psoas muscle

Vertebral body

Lumbar segment
arteries and veins




Abdominal contents

F, Next, the parietal pleura is incised along the thoracic the middle of each vertebral body included in the fusion.
vertebral bodies that are to be included in the fusion. The aorta and vena cava are protected with retractors,
G, In the lumbar region, the psoas muscle is gently elevated and the anterior longitudinal ligament is partially excised
off the vertebral bodies and intervertebral disks and with a sharp scalpel. Each disk within the levels selected for
retracted posteriorly. The segmental vessels are ligated in fusion is removed with various rongeurs and curets.

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CHAPTER 12  Scoliosis 305

Osteotome and mallet

used to remove
vertebral end-plate

Disks removed

Space packed with

sponge and Gelfoam

Instrument to
protect aorta
and vena cava

H, With a curet or sharp osteotome and mallet, the operator annular fibers need not be fully removed. The disk spaces
removes the vertebral cartilaginous end-plates and reta