C H A P T E R 1 2 
B. Stephens Richards  
Scoliosis
Chapter Outline
Definition          206
Classification of Scoliotic Curves          206
Idiopathic Scoliosis          206
Adolescent Idiopathic Scoliosis          206
Juvenile Idiopathic Scoliosis          247
Congenital Spinal Deformities          247
Early-Onset Scoliosis          265
Other Causes of Scoliosis          281
Definition
The term scoliosis, first used by Galen (131-201 AD), is
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
Hippocraticum. In 1741, André devised the crooked spine
as his symbol for orthopaedics.23
Currently, scoliosis is defined as lateral deviation of the
normal vertical line of the spine, which when measured on
a radiograph, is greater than 10 degrees (Fig. 12-1). Because
the lateral curvature of the spine is associated with rotation
of the vertebrae within the curve, a three-dimensional
deformity occurs. This complex deformity represents
abnormal movement in three planes: (1) intervertebral
extension in the sagittal plane leading to lordosis of the
scoliotic segment, (2) lateral intervertebral tilting in the
frontal plane, and (3) a rotatory component in the axial
plane.592 This results in torsion of the spine, with the most
significant abnormality located in the apical region. As the
deformity worsens, structural changes develop in the verte-
brae and rib cage. Relationships between intrathoracic and
abdominal organs may be distorted as the deformity
becomes severe, but rarely are the organs’ functions
compromised.
Classification of Scoliotic Curves
A variety of terms are used to describe the different types
of scoliotic curves. Box 12-1 provides definitions for the
most common ones.
Idiopathic Scoliosis
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
206
Daniel J. Sucato  
Charles E. Johnston
only after a thorough physical and radiographic examination
has ruled out neurologic causes, syndromes, and congenital
anomalies. Idiopathic scoliosis may have its onset at any age
during growth, but three fairly well defined peak periods
are accepted: (1) in the first year of life, (2) at 5 to 6 years
of age, and (3) after 11 years of age to the end of skeletal
growth.
The term adolescent idiopathic scoliosis (AIS) is used
when the deformity is recognized after the child has reached
10 years of age but before skeletal maturity, although it is
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-
nosed before the age of 10 years.
Scoliosis recognized after skeletal maturity is defined as
adult scoliosis.
Adolescent Idiopathic Scoliosis
Prevalence
The prevalence of radiographic curves measuring at least 10
degrees ranges from 1.5% to 3.0%, that of curves exceeding
20 degrees is between 0.3% and 0.5%, and that of curves
exceeding 30 degrees is between 0.2% and 0.3%.
A definite relationship between idiopathic scoliosis and
sex has been noted, particularly as the magnitude of the
curve increases. The ratio of affected females to males has
been reported to be 1 : 1 for curves between 6 and 10
degrees, 1.4 : 1 for curves between 11 and 20 degrees, 5.4 : 1
for curves exceeding 21 degrees but not requiring treat-
ment, and 7.2 : 1 for curves requiring orthopaedic interven-
tion.650 This sex prevalence in idiopathic scoliosis—that is,
an equal prevalence between the sexes for small curves
(<10 degrees), with increasing female prevalence for larger
and progressive curves—has been reported by several
authors.29,162,454,650 The clinical significance of these observa-
tions is that curve progression is more common in girls.
Natural History
Few current natural history studies have examined curve
progression in the untreated, skeletally immature scoliosis
population,96,455,534 and consensus is lacking in the literature
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
studies use increases of more than 5 or 6 degrees as indica-
tive of definite progression.
*References 140, 157, 257, 359, 362, 610, 724, 774, 816.

FIGURE 12-1  Posteroanterior radiograph of the thoracolumbar
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.
Natural History Before Skeletal Maturity
Individuals with untreated curves of less than 20 degrees
are at low risk for progression, particularly as they approach
skeletal maturity.455 Because some patients, however, have
curves that progress over the years and ultimately lead to
health problems, it is important to recognize the factors
associated with curve progression, including patient sex,
remaining growth, curve magnitude, and curve pattern.96,455,534
Factors of no predictive value for curve progression before
skeletal maturity include a family history of scoliosis, patient
height-to-weight ratio, lumbosacral transitional anomalies,
thoracic kyphosis, lumbar lordosis, and spinal balance.96
Sex
The majority of patients whose curves progress and ulti-
mately require treatment are female.29,162,454,650 Although
the exact reason for this phenomenon remains unknown,
hormonal influences have been proposed.9,283,708
Remaining Growth
A young patient’s remaining growth is usually assessed by
four maturity indices: the Risser sign (a skeletal marker of
the pelvis), hand and wrist skeletal maturity,672 peak height
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
published literature and is reported to be about 8.0 cm/yr
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
height measurements must be obtained. Six-month inter-
vals are preferred because shorter intervals may result
in significant measurement error. If height data are not
available from the patient’s records, the information can
often be obtained from the family, school, or pediatrician.
Although PHV requires analysis of serial height measure-
ments collected over time, it is the earliest and best index
available to demonstrate that growth is slowing and the risk
for curve progression is diminishing. In boys, use of PHV
to predict the period of remaining growth is superior to the
Risser sign and chronologic age, and closure of the triradiate
cartilage approximates the time of PHV.676
A simplified skeletal maturity scoring system for AIS
that uses radiographs of the hand has also been developed.672
The Tanner-Whitehouse III score, which is based on the
radiographic 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 (which is based on
the metacarpals and phalanges), correlates highly with curve
acceleration in girls with AIS. This system is reliable and
correlates more strongly with the behavior of idiopathic
scoliosis than does the Risser sign. It appears to be strongly
prognostic of future scoliosis curve behavior.
208 SECTION II  Anatomic Disorders
Box 12-1  Types of Scoliosis and Scoliotic Curves
Adult scoliosis: Spinal curvature present after skeletal maturity
as a result of any cause.
Cervicothoracic curve: Any spinal curvature in which the apex
is at C7 or T1.
Compensatory curve: Secondary curve located above or
below the structural component that develops to maintain
normal body alignment.
Congenital scoliosis: Scoliosis caused by bony abnormalities
of the spine that are present at birth. The anomalies are
classified as failure of vertebral formation or failure of
segmentation.
Double curve: Scoliosis in which two lateral curves are present
in the same section of spine.
Double major curve: Scoliosis in which two structural curves,
usually of similar size and rotation, are present.
Double thoracic curve: Scoliosis with a structural upper
thoracic curve; a larger, more deforming lower thoracic curve;
and a relatively nonstructural lumbar curve.
Hysterical scoliosis: Nonstructural deformity of the spine that
is a manifestation of a psychological disorder.
Idiopathic scoliosis: Structural spinal curvature, the cause of
which has not been definitely established.
Kyphoscoliosis: Seen as an increased round back on a lateral
radiograph, this condition may represent a true kyphotic
deformity (as occurs in some pathologic conditions), or it may
3 4
5 PHV
2
1
FIGURE 12-2  The Risser sign proceeds from grade 0 (no
ossification) to grade 4 (all four quadrants show ossification of the
iliac apophysis). When the ossified apophysis has fused completely
to the ilium (Risser grade 5), the patient is skeletally mature.
Curve Magnitude
The size of the existing curve when scoliosis is recognized
is helpful in predicting curve progression. The combination
of this factor and assessment of remaining growth is used
to predict the natural history in young patients with scolio-
sis. Immature patients (premenarchal, Risser grade 0) with
curves greater than 20 degrees are at substantial risk for
progression of spinal deformity (Table 12-1).96,265,450,451,534,650
For immature patients with curves exceeding 25 to 30
degrees, the risk for curve progression is believed to be
significant enough to recommend orthotic management at
the time of initial evaluation.364,456,534,610,724,774
represent such excessive rotation of the spine that a lateral
radiograph is actually reflecting the scoliotic deformity.  
(In idiopathic scoliosis, true kyphotic deformity does not
occur.)
Lordoscoliosis: Structural scoliosis associated with increased
swayback or loss of normal kyphosis within the measured
curve; it is nearly always present in idiopathic scoliosis.
Lumbar curve: Spinal curvature in which the apex is between
L1 and L4.
Lumbosacral curve: Spinal curvature in which the apex is at
L5 or below.
Neuromuscular scoliosis: Scoliosis caused by a neurologic
disorder of the central nervous system or muscle.
Nonstructural (functional) curve: Curvature that does not
have a fixed deformity and may be compensatory in nature.
The curve may be a result of leg length discrepancy (in which
case it disappears when the patient is supine), poor posture,
muscle spasm, or some other cause.
Primary curve: The first or earliest curve present.
Structural curve: Segment of the spine that has a fixed lateral
curvature.
Thoracic curve: Spinal curvature in which the apex is between
T2 and T11.
Thoracolumbar curve: Spinal curvature in which the apex is
at T12, L1, or the T12-L1 interspace.
TRC closure
Height Risser 1 and menarche
velocity
Risser 2
Crankshaft No crankshaft
Age
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:
Prediction of the crankshaft phenomenon by peak height velocity,
Spine 22:1352, 1997.)
Table 12-1  Incidence of Curve Progression
Based on Curve Magnitude and Risser Grade
Percentage of Curves That Progress
Risser
Grade Curves 5-19 Degrees Curves 20-29 Degrees
0 or 1 22 68
2, 3, or 4 1.6 23
Modified from Lonstein JE, Carlson JM: The prediction of curve
progression in untreated idiopathic scoliosis during growth, J Bone Joint
Surg Am 66:1061, 1984, with permission from The Journal of Bone
and Joint Surgery, Inc.

Curve Pattern
The curve pattern is useful for predicting curve progression.
Double curves and thoracic curves are most likely to pro­
gress, followed by thoracolumbar curves. Lumbar scoliosis
is reportedly the least likely to worsen.96,455
Prognostic Testing
A genetic screening test called the ScoliScore is available as
an adjunct to clinical and radiographic information to deter-
mine the risk for progression of AIS.563,808 This test can be
used in white (North American, European, Eastern Euro-
pean, Middle Eastern) patients between the ages of 9 and
13 years with a mild scoliotic curve (<25 degrees). The
stated goal of the test is to determine the risk that the curve
will increase to 40 degrees or greater. Further independent
verification of the test is needed before its usefulness for
this condition can be determined.
Natural History After Skeletal Maturity
In general, the rate of progression of scoliosis in adulthood
is much slower than that in adolescence and depends on the
size of the curve once skeletal maturity has been reached.
Regardless of the curve pattern, curves of less than 30
degrees in a mature individual are unlikely to progress.
Conversely, approximately two thirds of curves that exceed
50 degrees worsen, with thoracic curves progressing nearly
1 degree per year.811 Lumbar curves also tend to progress
in adulthood and may do so at a magnitude smaller than
50 degrees if they are accompanied by a transitory shift
between the lower vertebrae.
In terms of the long-term status of adults with untreated
scoliosis, several Swedish studies have reported an overall
mortality rate greater than that predicted by national mor-
tality statistics.533,551,587 However, these studies included
patients with nonidiopathic scoliosis and those with infan-
tile deformities. When examined selectively, the mortality
rate of patients with AIS appeared to be the same as that
in the general population.587 A significant increase in the
mortality rate was identified for patients with infantile and
juvenile idiopathic scoliosis. Respiratory failure and cardio-
vascular disease accounted for most early deaths. Respira-
tory failure developed in adults with severe scoliosis (>110
degrees) as normal aging further reduced their ventilatory
capacity.586 In patients who underwent surgery for scoliosis,
respiratory failure tended to not develop, thus suggesting a
preventive effect of corrective surgery.588
Chronic back pain is common in adults with scoliosis,
although it is not related to the size or location of the
curvature.145,533,809 The pain does not usually interfere with
the patient’s ability to work or perform daily activities.
Lumbar osteoarthritis may also be seen in up to 83% of
adults with scoliosis, but it is not necessarily associated with
the duration or intensity of back pain. Despite outwardly
apparent deformities because of long-standing untreated
scoliosis, most individuals have no significant psychological
difficulties when compared with persons without scoliosis
(the sole exception being a slight dissatisfaction with body
image).810
In summary, thoracic scoliosis of greater than 50 to 60
degrees in adulthood may progressively worsen and poten-
tially reduce pulmonary function. Lumbar curves, especially
CHAPTER 12  Scoliosis 209
those greater than 50 degrees, are also likely to progress in
adulthood and lead to osteoarthritis. Therefore, even when
cosmetic factors are not taken into account, aggressive
treatment of a child with a significant spinal deformity is
justified.
Scoliosis Screening
School Screening Programs
A number of medical organizations support the general
screening of children for scoliosis. In 2008, the Scoliosis
Research Society (SRS), the American Academy of
Orthopaedic Surgeons (AAOS), the Pediatric Orthopaedic
Society of North America (POSNA), and the American
Academy of Pediatrics (AAP) endorsed an information
statement in support of screening for AIS.638 This 2008
statement was a response to a recommendation made by
the U.S. Preventive Service Task Force.785 For years this task
force stated that the evidence was insufficient to recom-
mend either for or against routine screening of asymptom-
atic adolescents for idiopathic scoliosis.57,782-784 In 2004,
based on a brief evidence update, the task force changed its
position and recommended against routine screening.785 It
reported that no good evidence could be found that screen-
ing of asymptomatic adolescents detects idiopathic scoliosis
at an earlier stage. However, as the primary care providers
for adolescents with idiopathic scoliosis, the SRS, AAOS,
POSNA, and AAP do not support any recommendation
against scoliosis screening given the available literature. If
scoliosis screening is undertaken, the SRS, AAOS, POSNA,
and AAP agree that girls should be screened twice, at ages
10 and 12 (grades 5 and 7), and boys once, at age 13 or 14
(grades 8 or 9).
The clinical logic behind school screening for idiopathic
scoliosis assumes that screening is an accurate and reliable
method of detecting curvatures, early detection results in
improved health outcomes, and brace therapy is effective
in altering the natural history of the deformity.16,524,783,796,819
The implications of these assumptions are that small curva-
tures detected through screening are likely to progress to
curvatures of clinical significance, that scoliosis causes sig-
nificant health problems, and that the benefits of early
detection outweigh the potential adverse effects of screen-
ing and treatment. The many proponents of school screen-
ing believe that these assumptions are successfully addressed
by school screening programs.453,506 The idea of general sco-
liosis screening is not universally accepted, however, and is
becoming more controversial.525,785,796,862,863 Some authors
argue that school screening programs have not reduced the
prevalence or incidence of scoliosis requiring treatment, are
not cost-effective, and result in children with no scoliosis
or only a mild degree of curvature that does not require
treatment being unnecessarily referred to orthopaedic sur-
geons or radiologists.862,863 However, a study involving more
than 150,000 students reported that school screening is
predictive and sensitive with a low referral rate.469 The
authors recommended that screening be continued to facili-
tate early initiation of conservative treatment.
Screening Methods
Several clinical signs are indicative of possible scoliosis
and are frequently used in screening programs, including
210 SECTION II  Anatomic Disorders
shoulder asymmetry, unequal scapular prominence, appear-
ance of an elevated or prominent hip, greater space between
the arm and body on one side (with the arms hanging
loosely at the side), head not centered over the pelvis, and
a positive Adams forward-bending test. The Adams test is
performed by having the child bend forward until the spine
is horizontal and, while examining the patient from the rear,
noting whether one side of the back appears higher than the
other (Fig. 12-4). This test is the most common noninvasive
clinical method for evaluating scoliosis.7,95,146,525
FIGURE 12-4  Adams forward-bending test. The patient is viewed
from behind and is asked to bend forward until the spine is
horizontal. When scoliosis is present, one side of the back appears
higher than the other.
A B
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.
One of the constant features of structural scoliosis is
axial rotation of the vertebrae affected by the curve. The
spinous processes almost always rotate toward the concavity
of the curvature. Rotation of the thoracic vertebrae is also
impaired by rotation and deformity of the attached rib cage,
with elevation on the side of the convexity and depression
on the side of the concavity. This asymmetry is significantly
accentuated when the patient bends forward. Examining
patients in the forward-bent position is the standard method
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
curve magnitude at which bracing is usually initiated) is low.
When this approach is used, the referral rate is approxi-
mately 3% of persons screened, with a 95% detection rate
of curves requiring brace treatment.

Etiology
The exact cause of idiopathic scoliosis remains unknown
despite considerable investigation. Although growth has a
significant influence on the deformity, it is not considered
a causative factor. Since the 1990s, much of the research
on the etiology of scoliosis has focused on central neurologic
dysfunction, connective tissue abnormalities, and most
recently, genetic factors. These influences have supplanted
previous theories that idiopathic scoliosis was caused by a
biochemical or nutritional deficiency,294,339,849 structural
defects,106 or endocrine abnormality.708 The true cause is
probably multifactorial and involves several of the afore-
mentioned factors.
Neurologic Dysfunction
The literature in the past supported an underlying neuro-
logic abnormality as the primary etiologic factor in idio-
pathic scoliosis. Dysfunction of the vestibular, ocular, or
proprioceptive systems causes an interruption of equilib-
rium that is indicative of abnormalities involving the poste-
rior column of the proximal portion of the spinal cord,
brainstem, and cerebral cortex.† Somatosensory evoked
potentials are useful parameters for evaluating neurologic
function. Responses to vibratory stimuli are reportedly
reduced significantly and asymmetric between the left and
right sides in scoliotic patients when compared with con-
trols.39,565,850 These findings support the concept that an
aberration in function of the posterior column pathway of
the spinal cord may play a role. Other investigators, however,
have been unable to corroborate this opinion.495 Altered
balance affecting foot posture and gait, particularly pes
cavus, has been reported.114,258 In addition to abnormalities
in the sensory pathways, motor dysfunction has been
reported, thus suggesting that the organization of the entire
brain is asymmetric in individuals with scoliosis.264 Regional
differences in brain volume have also been reported in
patients with AIS.443
Another neurologically based theory regarding the cause
of idiopathic scoliosis involved the role of melatonin in regu-
lating normal spine growth. Secreted by the pineal gland,
this neurohormone controls the circadian rhythm. Experi-
ments on pinealectomized chickens revealed that melatonin
deficiency contributes to the development of scoliosis in
this model, probably by interfering with the normal sym-
metric growth of the proprioceptive system involving
the paraspinal muscles and the spine.36,68,130,335,474-479,865
However, melatonin therapy after pinealectomy in
chickens had no effect on the development or progression
of scoliosis, thus raising doubts about its role.35,780 Signifi-
cantly lower melatonin levels and impaired melatonin sig-
naling have been reported in patients with scoliosis versus
controls,477,523 but other investigators have refuted this
finding.3,89,217,311 In addition, studies report no evidence to
support mutations in the gene coding for human melatonin
receptor.522,542,700
Connective Tissue Abnormalities
Another focus of research is on alterations in connective
tissue involving the spine, paraspinal muscles, and platelets
† ‡
References 102, 105, 127, 232, 255, 297, 480, 538, 565, 765, 814.
CHAPTER 12  Scoliosis 211
in patients with scoliosis.128,460,462,854 Differences in collagen
have been found between normal individuals and those with
AIS; however, this finding is not universal.797 These changes
may be secondary to the mechanical effects of the spinal
deformity rather than reflecting mutations in collagen itself.
This theory has been substantiated by segregation analysis
of genetic markers linked to the structural genes encoding
types I and II collagen.115
Other components of connective tissue may be abnor-
mal as well. In histologic studies of the ligamentum flavum
in scoliotic patients, the elastic fiber system was found to
have disarranged fibers, a marked decrease in fiber density,
and a nonuniform distribution of fibers throughout the
ligament.282 These findings suggest that the elastic fiber
system (which is predominantly fibrillin) may play a sig-
nificant role in the pathogenesis of idiopathic scoliosis in
some individuals. Bone mineral density has also been shown
to be lower in young adolescents with scoliosis.126,128 It is
uncertain, however, whether this finding is related to the
primary cause of the disease or whether it is secondary to
the asymmetric mechanical forces associated with the
back deformities. Further investigation is needed to deter-
mine whether those with osteopenia have the same cause,
pathogenesis, and risk for progression as those without
osteopenia.
The paravertebral musculature in patients with scoliosis
may exhibit abnormalities in the muscle spindle,459 in indi-
vidual muscle fiber morphology,325,661,876 in histochemis-
try,719 and on electromyography.17,625 Some of these changes
are more pronounced with severe curves, but they are
believed to be secondary to muscle adaptation to the curve
and not a primary cause of the deformity.325
Abnormal platelet structure and function have been
reported in patients with scoliosis.‡ Calmodulin, a calcium-
binding receptor protein found in platelets and skeletal
muscle, regulates the contractile protein system (actin and
myosin). If an underlying systemic contractile disorder is
present, both platelets and skeletal muscle will be affected.
Thus, measurable abnormalities of calmodulin in platelets
are indicative of skeletal muscle abnormalities. Platelet
calmodulin levels in adolescents with progressive scoliosis
are significantly higher than those in normal individuals, in
patients with stable curves, and in those whose progressive
curves were stabilized by bracing or spinal fusion.3,383,460
Although this finding cannot be implicated as a direct cause
of scoliosis, it may become a useful predictor of curve pro-
gression, but controversy remains.462
Genetic Factors
Because idiopathic scoliosis can be seen in multiple
members of the same family, attempts have been made to
determine the genetic factors involved. Several extensive
clinical studies of affected families conducted in the 1960s
and 1970s revealed a high prevalence of familial scoliosis
(6.9% to 11.1% of first-degree relatives).149,644,852 Both
dominant and multifactorial inheritance patterns have
been suggested to explain the genetic contributions to AIS
(Fig. 12-6). Not all studies suggested a genetic basis,
however; some implicated older maternal age at the time
of childbirth.167
References 238, 383, 437, 460, 529, 589, 659.
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
genetic tendency in some families of patients with AIS.§ In
a meta-analysis of scoliosis in twins, monozygous twins had
a significantly higher rate of concordance than did dizygous
twins, and the curves in monozygous twins developed and
progressed together.372
Studies are now under way to identify the genes that
cause scoliosis and its progression.251,279,686,841 In this new
investigative frontier, genomic DNA from families with
apparent autosomal dominant inheritance of adolescent sco-
liosis is analyzed for linkage with disease. To date, findings
have been reported that involve chromosomes 3p26.3 single
nucleotide polymorphism,686 6,508 8,41,42 9,508 10,840 16,508
17,508,663 18q,279 and 19,119 as well as the X chromosome.353
Familial analysis using this approach may enable investiga-
tors to track causative genes, such as the CHD7 gene associ-
ated with the CHARGE syndrome (coloboma of the eye,
heart anomaly, choanal atresia, retardation, and genital and
ear anomalies) and later-onset idiopathic scoliosis,251 thereby
providing more insight into the etiology of idiopathic scolio-
sis. It may also allow the identification of diagnostic markers.
Pathophysiology
The extent of structural changes varies with the degree of
scoliosis.210,646 These changes are greatest at the apex of the
curve and diminish toward each end. In structural scoliosis,
§
References 41, 42, 119, 353, 372, 508, 509, 663, 805.
the vertebral body is rotated toward the convex side of the
lateral curvature, so the spinous processes of the vertebrae
are rotated toward the concavity of the curve. The asym-
metric deformities found within the bodies of scoliotic ver-
tebrae differ substantially from the vertebrae in normal
spines.438,441,582,614,727 Forces during compression and distrac-
tion act on the growing spine and produce wedge-shaped
changes in the vertebrae, which become higher on the
convex side and lower on the concave side (Fig. 12-7). The
vertebral body becomes condensed on the concave side as
a result of the greater pressure, and it is expanded and
thinned on the convex side. The concave facets have a sig-
nificantly thicker cortex than the convex facets do.688
In addition to changes in the frontal and axial planes, the
scoliotic portion of the spine is lordotic in the sagittal
plane.182,183 This three-dimensional deformity is appropri-
ately termed torsion of the spine and is greatest at the apical
region.179,180,195,591-593
A scoliotic spine has associated changes in the neural
canal and posterior arch. Abnormality in the neurocentral
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
narrow and close together (Fig. 12-8). The pedicles are
shorter and thinner (narrower endosteal transverse width)
on the concave side.441,582 The transverse processes more
closely approach the sagittal plane on the convex side and
are more in the frontal plane on the concave side. The

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
the concavity. The rotation of the spine is so severe in this
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,
1967, Williams & Wilkins, p 13.)
FIGURE 12-8  The posterior elements of the spine at the apical
region of this severe scoliosis show notable deformity, with the
laminae on the concave side being narrow and close together.
(From James JIP: Scoliosis, Baltimore, 1967, Williams & Wilkins,
p 15.)
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.)
intraspinal canal becomes distorted because of the mis-
shapen pedicles and articular processes (Fig. 12-9).
on the concave side narrow and may show degenerative
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
rotation.
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
patients.
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
intraspinal canal is distorted, the spinal cord may be
stretched over the concave side, but rarely is any neurologic
deficit present. Cord compression with neurologic deficit
usually occurs only with extreme deformities that are
accentuated by marked thoracic kyphosis.
214 SECTION II  Anatomic Disorders
Clinical Features
Initial Signs and Symptoms
Adolescents with scoliosis do not usually seek medical
evaluation because of back discomfort but rather because
of some physical aspect of their deformity, such as a high
shoulder, one-sided prominence of a scapula or breast,
elevated or protuberant iliac crest, and asymmetry in
flank creases and the trunk. Except for being noticed
personally by the adolescent, these findings are often first
appreciated during school screening programs for scoliosis
or during back-to-school examinations by the family
physician.
Though uncommon, back pain is present in individuals
with idiopathic scoliosis more often than was previously
thought. Nearly 32% of adolescents with idiopathic scoliosis
complain of back discomfort at some point (23% at initial
evaluation and 9% during the period of observation).616 A
significant association has been found between back pain
and age older than 15 years, Risser grade 2 or greater skel-
etal maturity, postmenarchal status, and a history of injury.
Back pain does not seem to be related to the sex of the
patient, family history of scoliosis, limb length discrepan-
cies, magnitude or type of curve, or spinal alignment. In
patients with back pain, the source of discomfort can be
identified only 10% of the time despite the use of appropri-
ate imaging studies. The most common causes of discomfort
are associated spondylolysis, spondylolisthesis, and Scheuer-
mann kyphosis. Less likely causes include spinal cord syrinx,
disk herniation, tethered spinal cord, and tumor. A painful
left thoracic curve or an abnormal neurologic finding is most
predictive of an underlying pathologic condition of the
spinal cord.
When an adolescent with presumed idiopathic scoliosis
has back pain, a careful history should be obtained, a thor-
ough physical examination performed, and plain radiographs
ordered. If findings on this initial evaluation are normal, a
diagnosis of idiopathic scoliosis can be made, the scoliosis
can be treated appropriately, and nonsurgical treatment of
the back pain can be initiated. It is not necessary to perform
extensive diagnostic studies in every adolescent with scolio-
sis and back pain. If the patient’s symptoms persist and
significantly restrict normal activities and if the findings on
neurologic examination are normal, a technetium bone scan
may be useful. If the neurologic findings are abnormal, MRI
of the spinal cord is indicated. Unlike backache in adults
with lumbar scoliosis, backache in adolescents is not usually
due to degenerative arthritis in the posterior articulations
or to nerve root irritation.
Respiratory symptoms are uncommon in patients with
AIS. Studies have shown that clinically significant cardio-
pulmonary compromise does not usually occur until the
magnitude of the curve approaches 100 degrees, vital
capacity becomes less than 45%, or thoracic lordosis signifi-
cantly narrows the anteroposterior (AP) dimensions of the
chest.586,588 Most curves are treated operatively before the
spinal deformity becomes this severe. However, when
the kinematics of the chest cage and spine during breathing
is evaluated, individuals with AIS have decreased motion in
comparison to healthy individuals.390,392,429 In patients with
impaired pulmonary function, this stiffness plays a signifi-
cant role.
Neurologic deficits are also rare in persons with AIS.
Should an adolescent describe any suspicious symptoms
(e.g., persistent neck pain, frequent headaches, ataxia,
weakness), meticulous attention must be paid to the neu-
rologic portion of the physical examination. If any neuro-
logic deficits are found or if the convexity of the thoracic
curve is to the left, appropriate imaging of the neural axis
is undertaken.120,220,554,616,771 Normally, the convexity of tho-
racic curves in AIS is directed to the right. Abnormal left
thoracic curves are more common in those with an underly-
ing syrinx.571,616,681
Physical Examination
Physical examination of an adolescent with idiopathic sco-
liosis should be performed with the patient properly draped.
It is convenient if the patient wears a swimsuit. Alterna-
tively, the patient may be dressed in underpants and an
examination gown open at the back. The patient’s entire
back, including the shoulders and iliac crests, must be
visible.
The skin is inspected closely for abnormalities such as
midline hemangiomas, hair tufts, and dimpling in the lum-
bosacral region. Any of these surface findings may indicate
the presence of an underlying spinal cord abnormality such
as a tethered cord or diastematomyelia. The spinous pro-
cesses are palpated from the cervical region to the sacrum
for any deficiencies or areas of discomfort. Occasionally,
absence of a spinous process is noted, which usually cor-
responds to spina bifida occulta seen on a spinal radiograph
(Fig. 12-10).
With the patient standing, the examiner should deter-
mine whether the iliac crests are level. If they are not, a
lower limb length discrepancy is likely, which can be quanti-
fied by placing measured blocks under the short extremity
until the iliac crests are level. Leg length discrepancy can
be responsible for the appearance of scoliosis, and the con-
dition must not be overlooked. The back is then examined
for asymmetry of the shoulders and flank creases, unequal
scapular prominence, prominent iliac crest, and increased
space between the arm and body on one side with respect
to the other with the arms hanging loosely at the side
(Fig. 12-11).
Although these findings are consistent with scoliosis, the
best noninvasive clinical test for evaluating spinal curvature
is the Adams forward-bending test (see Fig. 12-4).7,95,146
With this test, the degree and direction of associated rota-
tion of the vertebrae are clearly demonstrated. The exam-
iner observes the adolescent from behind as the patient
bends forward at the waist until the spine is horizontal. The
patient’s knees should be straight, the feet together, the
arms dependent, and the palms in opposition. Vertebral
rotation causes one side of the back to appear higher. This
is noted as rib prominence in the thoracic region or as para-
spinal fullness in the lumbar region. Such asymmetry can
be quantified with a scoliometer, which can provide mea-
surements documenting change over time (see Fig. 12-5).95
Frequently, if the patient is inspected from the front,
asymmetry of the pectoral regions, breasts, or rib cage may
be evident. Although these asymmetries are probably
related to the spinal curvature, they may also occur in indi-
viduals without scoliosis. Occasionally, breast asymmetry is
the primary concern of the patient and parents. Families

FIGURE 12-10  Posteroanterior radiograph of the spine of a
12-year-old girl. The spinous processes of T11 and T12 were not
detectable on palpation. The radiograph shows spina bifida
occulta at the same levels (arrows).
should be informed that correcting the scoliosis may have
little, if any, influence on this asymmetry.
Spinal balance is assessed by two different methods. The
first method, known as coronal balance, is to determine the
alignment of the head over the pelvis. The head is almost
always positioned directly above the gluteal crease in
patients with idiopathic scoliosis. To assess this balance, a
plumb line is held from the base of the skull or from the
spinous process of C7. Normally, the plumb line should not
deviate from the center of the gluteal crease by more than
1 to 2 cm (Fig. 12-12). If it does, this finding should be
considered atypical, and a meticulous neurologic examina-
tion is necessary to rule out coexisting neurologic pathology.
The second method, known as trunk balance, is to assess
the position of the trunk over the pelvis.43,239,628 Unlike the
position of the head over the pelvis, patients with idiopathic
scoliosis may have significant imbalance of the trunk over
the pelvis (Fig. 12-13), particularly with single thoracic
curve patterns.
Next, the examiner inspects the patient from the side
and observes the sagittal contours of the spine. Normally,
in individuals with idiopathic scoliosis the sagittal plane
appears hypokyphotic throughout the scoliotic segment,
with actual lordosis being present radiographically in the
apex of the deformity. In more severe cases the entire sagit-
tal plane may actually be lordotic and lead to a very narrow
AP diameter of the thoracic cage. Rarely, as much as 90
degrees of rotation may be present in the apical vertebrae
within the curve. In this instance the examiner is actually
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.
assessing the AP aspect of the spinal curvature when viewing
the spine from the side. The resulting clinical appearance is
an apparently increased kyphosis in the sagittal plane
because in reality, the scoliotic deformity is being viewed
from the side (see Fig. 12-7).
Sagittal plane deformity in an individual with apparent
idiopathic scoliosis may also be an indicator of syringomy-
elia.571,636 If hypokyphosis is absent clinically and radio-
graphically and if little or no rotation (rib prominence) is
present, a diagnosis of idiopathic scoliosis should be made
only after a syrinx has been ruled out.
Neurologic Examination
Because idiopathic scoliosis is basically a diagnosis of exclu-
sion, a thorough evaluation is necessary to rule out a neu-
rologic cause of the deformity. The neurologic examination
begins by assessing the patient’s reflexes. Examination of
the superficial abdominal reflexes is useful for determining
which patients should undergo MRI to rule out syringomy-
elia (Fig. 12-14).336,864,870
The abdominal reflex examination is performed with the
patient supine on an examination table and the arms relaxed
along the side of the body. An area approximately 10 cm
above and below the umbilicus and to each anterior axillary
line is exposed. With the patient relaxed, the bluntly
pointed handle of a reflex hammer is used to lightly stroke
the skin in each quadrant over a distance of 10 cm (Fig.
12-15). The stroke starts lateral to the umbilicus near the
anterior axillary line and is directed diagonally toward the
umbilicus in each quadrant. The umbilicus is observed for
deviation toward the side on which the test is performed.
If these reflexes are consistently present on one side and
absent on the other side, further evaluation is warranted
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.

FIGURE 12-14  Diagnostic imaging of the spinal cord and canal is
necessary in children with abnormal neurologic findings. Magnetic
resonance imaging is the optimal study for assessing the neural
axis. A large cervical syringomyelia (arrow) is evident on this
magnetic resonance image of the head and neck. (From Richards
BS: Back pain in childhood and adolescence: the clinical
assessment, J Musculoskelet Med 15:39, 1998.)
FIGURE 12-15  The abdominal reflex examination is performed
with the patient supine. The bluntly pointed handle of a reflex
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
abnormal.
because this finding does not occur in neurologically normal
patients with scoliosis. However, other variations might
occur, such as absent reflexes in all quadrants.
The patellar and Achilles tendon reflexes should also be
tested, with the expectation that they will be symmet-
ric.336,864 Muscle testing and examination of the range of
motion of all four extremities should always be conducted.
The hands and feet should be examined for abnormal
posture and for evidence of abnormal sensation (excessive
callus formation or nail bed irregularities). Abnormal find-
ings may be the only clinical evidence of underlying pathol-
ogy of the neural axis, such as syringomyelia or tethered
cord.
Patient Maturity
Sexual maturity can be assessed during the physical exami-
nation according to the Tanner system,757 which assesses
breast and pubic hair development in girls and genital and
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
If older conventional radiology equipment is used, the
initial examination of the spine should include posteroan-
terior (PA) and lateral radiographs on 36 × 14-inch film
cassettes. With these long cassettes, nearly all the impor-
tant radiographic features can be assessed on a single film.
On the PA projection, such features include the curve
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
postponed.
Today, with the more common use of computed radiog-
(PACS), further technologic development in scoliosis
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
plate inside a cassette is exposed and subsequently pro-
cessed by a reader that converts the image into a digital
format to be viewed and stored in a PACS. A series of up
to four plates can be placed in a long-length cassette holder
and exposed simultaneously to acquire a long scoliosis image
in both the PA and lateral projections. Special software
electronically stitches the images together for monitor
display or, if needed, printing. Newer imaging opportunities
are available with the use of a recently introduced low-dose
radiation digital stereoradiography system known as the
EOS system (EOS Imaging, Paris).261,497 At this point this
system is found primarily in academic institutions, but its
use will probably expand over time.
After the initial radiographic evaluation has been accom-
plished, effort is made to limit the number of follow-up
films, thereby reducing the amount of radiation exposure.
During the course of routine follow-up examinations, only
the PA projection is needed. No set interval from one
218 SECTION II  Anatomic Disorders
A B
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
spine.
radiographic examination to the next has been determined
for all patients. The period between evaluations depends
on the maturity of the patient and the size of the spinal
curvature. For example, a premenarchal, Risser grade 0,
11-year-old girl with a 25-degree thoracic curve should
return for radiographic reevaluation after a 4-month inter-
val, whereas a 2-year postmenarchal, Risser grade 4, 14-year-
old girl with a 30-degree curve need not return for
reevaluation before 1 year. In most cases the interval
between radiographic evaluations ranges from 4 to 6 months.
During radiographic evaluation the patient should stand
as erect as possible with the knees straight and the feet
together (Fig. 12-16). The patient should be barefoot so
that if lower limb length inequality is suspected, the appro-
priate lift can be placed under the short limb. Unsupported
sitting views are taken if the patient is unable to stand.
Twisting of the trunk should be avoided. To ensure sufficient
cephalic visualization, the upper limit of the cassette should
extend to the external auditory meatus. In the upright
lateral projection, the patient’s shoulders are flexed forward,
the elbows are fully flexed, and the fists should rest on the
clavicles. For the lateral radiograph, this position allows
the best representation of the patient’s functional sagittal
balance while still providing adequate lateral radiographic
visualization of the spine.222,322 Although AP radiographs of
the hand and wrist are used today to determine skeletal age,
maturity continues to be more commonly assessed with the
Risser sign of the iliac crest.
Bending radiographs (including the fulcrum bend test)
and traction radiographs obtained with the patient supine
are usually reserved for preoperative evaluation of spinal
flexibility.131,290,466 The information gained from these AP
radiographs can be helpful in determining appropriate
fusion levels.
Measurement of Curve Magnitude
The Cobb method is considered the standard for measuring
curve size.31 The measurement is started by determining the
end vertebrae (top and bottom of the curve). The cephalic
end vertebra’s superior surface and the caudal end verte-
bra’s inferior surface have the greatest amount of tilt into
the curve (Fig. 12-17). The intervertebral space on the
concave side of the curve is generally wider above the
cephalic (top) vertebra and narrower below it. The opposite
applies to the inferior surface of the caudal (bottom) ver-
tebra. Using a transparent plastic goniometer, the examiner
draws lines perpendicular to the top vertebra’s superior
surface and the bottom vertebra’s inferior surface. The
angle formed by the intersection of these lines is the Cobb
angle. If a second curve is present below the primary curve,
the original curve’s bottom vertebra becomes the cephalic
end vertebra for the second curve, and the same line along
its inferior surface is used. With the use of PACSs in radiol-
ogy departments today, electronic angular measurements
simplify this process.
Although the Cobb method has good overall reliability,396
some variation among different observers’ measurements is
always present. Such variability averages 7.2 degrees if the
end vertebrae are not preselected but improves to 6.3
degrees when they are preselected.526 Another aspect of the
accuracy of the Cobb method is that to achieve 95% statisti-
cal confidence that a true change in curve size has occurred,
a measurement difference of 10 degrees between radio-
graphs taken at different times would be needed.113 This
finding is of particular interest because many studies use a
criterion of a 5- to 6-degree change in curve size to deter-
mine the success or failure of brace treatment for scoliosis.
This information reinforces the importance of meticulous
line drawings and precise measurements.

Thoracic
Lumbar
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
curve’s lower vertebra becomes the top vertebra when measuring
the second curve, and the same line along its surface is used.
Measurement of Vertebral Rotation
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
(Fig. 12-18).590 The edges of the curve’s apical vertebra and
its rotated pedicle constitute the landmarks. This method
is accurate for measuring rotations that are less than 30
degrees.568 However, once the scoliotic spine has undergone
instrumentation, the landmarks of the apical vertebra may
become obstructed by the shadows of the rods or hooks,
thus making accurate measurements difficult to obtain.629
In the Nash-Moe method, the relationship of the pedicle
to the center of the vertebral body is observed on AP radio-
graphs, and the rotation is divided into five grades: grade 0
when both pedicles are symmetric, grade I when the convex
pedicle has moved away from the side of the vertebral body,
grade III when the convex pedicle is in the center of the
vertebral body, grade II when the rotation is between grades
I and III, and grade IV when the convex pedicle has moved
past the midline.537 This method has only fair reliability.396
Computed tomography (CT) can also be used to assess
vertebral rotation.154,321,848 Although CT is more expensive,
it is more accurate than the Nash-Moe method. For example,
Nash-Moe grade 0 vertebrae have been found to have up to
11 degrees of rotation when measured with CT.314
CHAPTER 12  Scoliosis 219
FIGURE 12-18  Left, Perdriolle torsionometer—a clear template.
Right, The torsionometer’s outer margins are aligned over the
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,
1992.)
Measurement of Spinal Balance
Measurements of spinal balance are important to assess
the amount of decompensation that exists preoperatively
or that can occur postoperatively. Coronal balance repre-
sents the horizontal distance between the midpoint of C7
and the center of the pelvis (Fig. 12-19). Coronal balance
is considered poor, or decompensated, if this distance
exceeds 2  cm.203,611 Trunk balance assesses the position of
the thorax over the pelvis and is measured via lateral trunk
shift. Lateral trunk shift is measured by drawing a hori-
zontal line to the edges of the ribs of the trunk and a
perpendicular line that bisects this horizontal line; the dis-
tance between this perpendicular line and the center of
the pelvis represents lateral trunk shift (Fig. 12-20).
Another parameter used to indirectly assess trunk balance
is thoracic apical vertebral translation,203,419 which is the
distance measured between the midpoint of the apical
thoracic vertebra and the C7 plumb line. Because the mid-
point of C7 rarely corresponds to the exact center of the
pelvis, thoracic apical vertebral translation must be used
in combination with the coronal balance measurement to
reflect trunk balance.635
220 SECTION II  Anatomic Disorders
FIGURE 12-19  Coronal balance. A plumb line held at the spinous
process of C7 (x) should not deviate from the center of the gluteal
fold (center sacral line) by more than 1 to 2 cm.
FIGURE 12-20  To measure trunk balance, two vertical lines are
drawn on a radiograph (arrows). The first vertical line (pink) is
the center sacral line. The second vertical line (green) 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.
Measurement of Kyphosis and Lordosis
on Lateral Radiographs
The end vertebrae are the last vertebrae that are maximally
tilted into the concavity of the curve. In the thoracic area,
the upper end vertebra is usually T3 or T4, and the lower
end vertebra is T12. Perpendicular lines are drawn to the
inferior and superior end-plates. The angle formed between
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
reliability.
Surface Imaging
In an effort to decrease the amount of radiation exposure
during the course of scoliosis management, techniques have
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
valuable tool in the assessment of scoliosis. Syringomyelia,
Arnold-Chiari malformations, abnormalities in the brain-
stem, hydromyelia, spinal cord tumors, spinal cord tether-
ing, and diastematomyelia have all been identified in
individuals previously thought to have idiopathic scoliosis.¶
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
with an atypical manifestation of idiopathic scoliosis.521
Although atypical manifestations have never been specifi-
cally defined, they generally include patients with neck pain
and headache (particularly with exertion) and abnormal
‖
References 161, 444, 471, 662, 728, 773, 843.
¶
References 175, 255, 336, 434, 574, 636, 681, 689, 833.

Table 12-2  Guidelines for Treating Patients With Idiopathic Scoliosis
Curve Magnitude
(Degrees) Grade 0/Premenarchal
<25 Observation
30-40 Brace therapy (begin when
the curve is >25 degrees)
>45 Surgery
FIGURE 12-21  Moire topographic photograph. This surface-
imaging system produces an image that can be read in the same
way as contour lines on a map. (From Stokes IA, Moreland MS:
Concordance of back surface asymmetry and spine shape in
idiopathic scoliosis, Spine 14:73, 1989.)
neurologic findings such as ataxia, weakness, and progressive
foot deformities; patients with unusually rapid curve pro-
gression or excessive thoracic kyphosis; or patients requiring
surgery who have left thoracic curves or asymmetric abdom-
inal reflexes. Curves greater than 70 degrees do not increase
the likelihood of finding a spinal cord anomaly.556 Routine
preoperative MRI is not indicated for typical AIS if findings
on the neurologic examination are normal.175,556,689,833
Computed Tomography
Although CT may clearly demonstrate congenital abnor-
malities in the spine, it is rarely needed in the routine
assessment of individuals with idiopathic scoliosis. However,
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
CHAPTER 12  Scoliosis 221
Risser Sign
Grade 1 or 2 Grade 3, 4, or 5
Observation Observation
Brace therapy Observation
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.
Treatment
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
are observation, nonsurgical intervention, and surgical inter-
vention, and it is imperative that physicians know which
options are appropriate for each individual patient (Table
12-2 provides general guidelines). Actively growing adoles-
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
premenarchal if female) with curves exceeding 25 degrees,
bracing should be started immediately.456,653 In most cases,
growing adolescents with curves exceeding 45 to 50 degrees
require operative stabilization because other forms of treat-
ment are ineffective in controlling or correcting the scolio-
sis. Skeletally mature individuals with curves exceeding 50
to 55 degrees are also at risk for continued curve progression
and should be considered for surgical treatment.811 Possible
exceptions include patients with well-balanced double
curves less than 60 degrees whose clinical appearance is
acceptable to them. Continued observation would be neces-
sary to document progression of the scoliosis, which would
necessitate surgery.
#
References 154, 225, 287, 407, 418, 440, 619, 847, 848.
222 SECTION II  Anatomic Disorders
Observation
In general, no treatment is needed for curves less than 25
degrees, regardless of the patient’s maturity. Follow-up
examinations are necessary, with the interval between visits
depending on the patient’s maturity and the size of the
curve. For example, a premenarchal Risser grade 0 adoles-
cent with an initial curve measuring 24 degrees should
undergo follow-up examinations every 3 to 4 months, and
a brace may be needed if the curve progresses. For more
skeletally mature patients (Risser grade 3 or higher), longer
intervals between visits (e.g., 6 months) are appropriate
because curve progression usually occurs at a slower rate, if
at all. Clearly, predetermined guidelines do not apply to all
cases, and follow-up must be individualized.
The magnitude of the patient’s curve at initial evaluation
helps determine the frequency of follow-up visits. In
general, for growing children with small curves (<20
degrees), the next follow-up evaluation should be approxi-
mately 6 months later. If the curve is between 20 and 30
degrees, radiographs should be obtained 3 to 4 months later
because treatment may be necessary if the curve progresses
5 degrees or more. For patients whose curves do not pro­
gress, observation continues and the interval between visits
gradually lengthens as maturity approaches.
What constitutes true curve progression is a matter of
some debate. Traditionally, an increase in curve size of more
than 5 to 6 degrees has been taken as representing progres-
sion; however, a 7- to 10-degree change in measurement is
more accurate if a 95% confidence level is used to deter-
mine true progression.113,526 This should be taken into con-
sideration when deciding whether the measured change in
the patient’s scoliosis warrants either nonsurgical or surgical
intervention. Nevertheless, throughout the literature a 5- to
6-degree measured change is considered indicative of curve
progression. Not all progressive curves exceeding 30 degrees
require treatment; the decision depends on the adolescent’s
maturity and the size of the curve.
Nonsurgical Treatment
To be considered effective, nonsurgical treatment must
prevent curve progression in those who are most at risk
(curves of 25 to 45 degrees in patients with Risser grade 0
or 1), be of benefit in all curve patterns, result in an accept-
able cosmetic appearance at the end of treatment, and
reduce the need for surgery. In other words, nonsurgical
treatment must improve the patient’s outcome with respect
to the expected natural history. Over the years a great deal
of experience has been gained with various forms of non-
surgical treatment, some of which orthopaedists consider
effective (e.g., bracing) and others that have shown no
beneficial effect (e.g., electrical stimulation). In Europe, the
use of physical therapy exercises and biofeedback to enhance
the effectiveness of bracing (Schroth technique and others)
is common.540,541,710,871 Although its use in North America
has not yet become established, this treatment method is
being studied in various centers.
Orthotic (Brace) Treatment
Historically, Pare is credited with being the first to use metal
braces, in the form of armor, to treat patients with scoliosis.
Since then, various types of braces and casts have been
advocated, such as the suspensory plaster cast of Sayre and
the hinge or turnbuckle cast of Hibbs and Risser. In 1946
the Milwaukee brace was developed to replace postopera-
tive plaster immobilization. Later, the brace was used as a
nonoperative method of treatment when passive, active,
and distraction forces were thought to be necessary to
prevent curve progression. Subsequent studies have shown
that the corrective forces of a brace are passive and that the
predominant corrective component is transverse loading of
the spine through the use of corrective pads.24,121,580,851 In
the 1960s, thermoplastics were introduced into orthosis
manufacturing, which led to the thoracolumbosacral ortho-
ses (TLSOs) of today. In recent years, computer-assisted
design and computer-assisted manufacturing have been
used to fashion spinal orthoses.157,205
Indications for Brace Treatment. Brace treatment is
restricted to immature children in an attempt to prevent
curve progression during further skeletal growth. In general,
bracing is indicated in growing adolescents (Risser grade 0,
1, or 2) who on initial evaluation have curves in the range
of 30 to 45 degrees or who have documented progression
exceeding 5 degrees in curves that initially measured 20 to
30 degrees. Those who are Risser grade 0 should be consid-
ered candidates for bracing when their curves reach 25
degrees. Patients should consider their existing deformities
cosmetically acceptable and must be willing to wear the
brace the prescribed amount of time. Low-profile braces
(TLSOs) are the most commonly used orthoses today, but
their use is restricted to patients whose curve apex is at T7
or below. Fortunately, this is the case with most curve pat-
terns in adolescents with idiopathic scoliosis. In 2005 the
Bracing Committee of the SRS made recommendations
concerning inclusion criteria for future brace studies involv-
ing AIS.632 Optimal inclusion criteria are age 10 years and
older when the orthosis is prescribed, Risser grade 0 to 2,
primary curve magnitude of 25 to 40 degrees, no previous
treatment, and if female, either premenarchal or less than
1 year postmenarchal.
Contraindications to Brace Treatment. Brace treatment has
several contraindications. First, most studies concur that
large curves (>45 degrees) in a growing adolescent cannot
be effectively controlled by a brace and that these patients
need surgical treatment. Even if progression could be con-
trolled with a brace, the cosmetic appearance associated
with these large curves is often unacceptable because of
excessive trunk shift and rib prominence. An exception to
this general rule involves very immature adolescents with
large curves (approximately 50 degrees) who have not yet
reached their PHV. These patients may benefit from bracing
to delay curve progression until greater maturity is reached;
this may avoid the need for additional anterior spinal fusion
to prevent the crankshaft phenomenon.
In addition, bracing is not indicated for patients who find
wearing an orthosis to be emotionally intolerable, although
appropriate psychological counseling may result in eventual
acceptance of a brace by an adolescent. Extreme thoracic
hypokyphosis precludes the use of an orthosis. In these
cases, normal positioning of the pads within the brace could
exacerbate the rib deformity. If the hypokyphosis is 20
degrees or less, corrective pads should be lateralized to

eliminate any anteriorly directed derotation forces. Finally,
skeletally mature adolescents (Risser grade 4 or 5 and, if
female, 2 years postmenarchal) should not be treated with
braces.
Relative contraindications to bracing include a high tho-
racic or cervicothoracic curve, which ordinarily does not
respond to orthotic treatment, and male sex. A relative lack
of effectiveness of bracing in boys has been documented, in
part because of extremely poor compliance.359,867
Comparison of Orthoses. Numerous reports in the litera-
ture attest to the effectiveness of brace treatment.*a In
most of these studies, bracing was considered effective if
the curve remained within 5 to 6 degrees of its original
magnitude on completion of treatment. Some of these
studies included low-risk patients (Risser grades 3 to 5,
curves <20 degrees), patients still undergoing treatment,
patients who had previously undergone treatment, and chil-
dren younger than 10 years. In some studies, patients were
eliminated from the study population because of noncom-
pliance. All these factors make comparisons among studies
difficult, particularly when one is trying to assess the effec-
tiveness of bracing in patients most at risk (Risser grade 0
or 1, premenarchal girls, 25- to 45-degree curves). Some of
the more recent literature has been more consistent in
focusing on the population at greatest risk.†a These studies,
along with a 1997 meta-analysis of the bracing literature,653
strongly reinforce the idea that bracing is effective in con-
trolling curve progression. Perhaps the most compelling
evidence for this at present comes from a study by Katz and
colleagues.363 In this study, braced patients had compliance
objectively verified through the use of a heat sensor in the
orthosis. The total number of hours of brace wear correlated
with curve progression. This effect was most significant in
patients who were at Risser stage 0 or 1 at the beginning of
treatment. Curves did not progress in 82% of patients who
wore the brace more than 12 hr/day, as opposed to only
31% of those who wore the brace less than 7 hr/day.
The perception of brace effectiveness is not universal,
however, in that some studies question whether orthotic
management provides any benefit in those with scoliosis.‡a
Numerous orthoses are available. Most are named after
their place of origin, such as the Milwaukee brace,456 the
Boston brace,208,286 the Wilmington brace,18,45 the Charles-
ton brace,610 and the Providence brace.157 All these braces
are effective in preventing curve progression. Before decid-
ing which brace to use, the orthopaedist should be familiar
with the advantages and disadvantages of each.
Milwaukee Brace. The Milwaukee brace, introduced by
Blount, Schmidt, and Bidwell in 1946, was the original
modern design. The device consisted of three main compo-
nents: a pelvic girdle, a suprastructure, and lateral pads.
Over time, some design modifications have been made, and
in the current model the pelvic girdle is made of thermo-
plastic material and is created from a positive mold of the
*aReferences 18, 24, 43, 44, 121, 139-141, 157, 159, 202, 208, 233,
235, 249, 250, 257, 272, 273, 324, 340, 362-364, 368, 400, 404,
456, 516, 534, 553, 566, 609, 610, 653, 676, 774, 787, 816, 827,
832.
†a
References 18, 157, 362, 364, 456, 534, 610, 774, 816.
‡a
References 181, 192, 193, 265, 268, 553, 724.
CHAPTER 12  Scoliosis 223
patient’s pelvis. The suprastructure consists of one anterior
and two widely separated posterior uprights, plus a cervical
ring with a throat mold and occipital piece. In many cases
a low-profile, over-the-shoulder structure may be used in
place of the more standard neck-ring design. Today, because
of the strong emphasis on self-image, use of the Milwaukee
brace has decreased greatly, and it has largely been replaced
by equally effective but lower-profile braces. Low-profile
TLSOs, such as the Boston, Wilmington, and Miami
braces,494 can often be hidden under loose shirts or
sweaters, thus providing adolescents a more acceptable
alternative.
Boston Brace. The Boston brace was introduced in 1971
by Hall and associates.286 Its design consists of a prefabri-
cated, symmetric thoracolumbar-pelvic module with built-in
lumbar flexion and areas of relief opposite areas of pressure
(Fig. 12-22). Braces are individually constructed by an
orthotist by using a blueprint created from the patient’s
full-length radiograph. This brace is the most commonly
used TLSO today and is effective in controlling curve pro-
gression, including larger curves measuring 35 to 45
degrees.§a It has been reported that the Boston brace exerts
derotational force on the scoliosis; however, a long-term
study found no lasting improvement in derotation of the
spine.818 The brace is effective in treating either single- or
double-curve patterns in which the apex of the most
cephalic curve is located at T7 or below.
Wilmington Brace. The Wilmington brace was described
in 1980.98 It is custom-made from a positive mold of the
patient’s torso in which the scoliosis is maximally corrected
in a Risser- or Cotrel-type cast. Indications for the
Wilmington brace are the same as those for the Boston
brace. This brace has not enjoyed the popularity of the
Boston brace, although it continues to be used by several
institutions.18,249
Charleston Brace. Development of the Charleston brace
was based on the concept that part-time use may be effec-
tive.226,609,610 This brace holds the patient in maximal side-
bending correction and is worn only at night for 8 to 10
hours. The side-bending force exerted by the brace does not
allow its use in the upright position, thus making wear fea-
sible only when the patient is recumbent. The main appeal
of this brace is the limited number of hours of daily wear,
all of which are accomplished during sleep.
Despite several studies in which the Charleston brace
was found to be as effective as the Milwaukee and Boston
braces,226,257,609,610,774 some skeptics doubt that such a limited
amount of time in a brace can successfully control curve
progression. A comparison of the Boston brace and the
Charleston brace found that in patients with curves between
36 and 45 degrees, 83% of those treated with a Charleston
brace experienced curve progression of more than 5 degrees
as compared with 43% of those treated with a Boston
brace.364 The authors concluded that the Charleston brace
should be reserved for single lumbar or thoracolumbar
curves less than 35 degrees.
Providence Brace. The Providence brace, like the Charles-
ton brace, is used only at night (Fig. 12-23). Made with
computer-assisted design and manufacturing technology, it
§a
References 208, 362, 364, 404, 516, 566, 816, 818.
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
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
place of the Charleston brace for the treatment of thoraco-
lumbar or lumbar curves.
Spine-Cor Brace. This is a dynamic flexible brace. Several
studies have reported its effectiveness for AIS.139,141,250
However, a failure rate significantly higher than that of a
rigid spinal orthosis has been reported.845
Brace Treatment Protocols. The number of hours per day
that the brace needs to be worn remains uncertain. Origi-
nally, 20 to 22 hr/day was advocated for the Milwaukee
brace in immature adolescents with progressive curves; the
same recommendation applied to the lower-profile TLSOs.
Understandably, this caused adolescents some emotional
C
distress, and poor compliance with brace wear was
common.183 As a result, the idea of part-time brace use
evolved, with the goal of approximately 16 hr/day of
bracing. Although several studies have reported that part-
time use of orthoses appears to be as effective as full-time
wear in controlling curve progression,9,121,165 other reports
emphasize that the outcome is better when more hours per
day are spent in the brace.362,816 This was confirmed by Katz
and colleagues in a 2010 study that objectively assessed
brace compliance by using heat sensors in the orthosis.363
In an effort to form a consensus from the literature on
the effectiveness of bracing (including whether part-time
bracing controls curve progression as effectively as full-
time bracing does), the Prevalence and Natural History

A B
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
degrees.
Committee of the SRS conducted a meta-analysis on more
than 1900 patients from 20 studies.653 It concluded that
bracing (with TLSOs or the Milwaukee brace) is effective
in controlling curve progression in individuals with idio-
pathic scoliosis and that full-time bracing (23 hr/day) is
more effective than part-time bracing (8 to 16 hr/day). The
latter finding is supported by more recent studies.362,816
When brace treatment is chosen for a patient, certain
general guidelines should be followed. Once the brace has
been constructed and fitted to the patient by the orthotist,
the patient should work up to the prescribed number of
hours of wear per day. After 2 to 4 weeks the adolescent
should return to the orthopaedist’s office for an initial brace
evaluation. At that time, any problems (e.g., intolerable
pressure points) will have been identified by the patient and
can be addressed by the orthotist. Equally important, an
in-brace radiograph should be obtained to verify the amount
of curve correction being achieved. With the Boston brace,
a minimum of 40% to 50% curve correction should be
obtained in the brace.364 With the Charleston and Provi-
dence braces, the amount of in-brace correction should
approach 90% for flexible curves and 70% for rigid curves.
Regardless of the type of brace used, insufficient in-brace
curve correction leads to an unsatisfactory outcome that
differs little from the expected natural history.553 If proper
correction cannot be obtained with brace use, orthotic
treatment should be discontinued.
During brace management, follow-up visits are sched-
uled at 4-month intervals for rapidly growing adolescents
with large curves. The interval may be extended to 6 months
for patients nearing maturity whose curves have shown no
recent changes. During these visits a single standing PA
thoracolumbar radiograph is obtained with the patient out
of the brace. Curve progression, if it has occurred, is readily
identifiable, and appropriate adjustments to the treatment
program can be made. Some physicians obtain radiographs
with the patient wearing the brace to show the brace’s
CHAPTER 12  Scoliosis 225
C D
effect on both the curve and spinal balance.450 However,
curve progression may be missed if the patient undergoes
imaging while wearing the brace.
With female patients, if the brace has been successful in
controlling curve progression, plans can be made to discon-
tinue treatment when the girl is approximately 18 to 24
months postmenarchal and Risser grade 4 and when no
further increase in her height has occurred (Fig. 12-24).
Rather than tapering use of the brace, we discontinue it
completely at that time. In male patients, curves exceeding
25 degrees have a tendency to progress even when Risser
grade 4 maturity has been reached.360 Therefore, in boys,
bracing may need to be continued until Risser grade 5 is
achieved. Frequently, this does not occur until the later
teenage years, which makes compliance with brace wear a
challenge.
Electrical Stimulation
Electrical stimulation was used as an alternative to bracing
in the early 1980s. Surface muscle stimulators were placed
over the muscles on the convex side of the scoliotic curve
and were activated for approximately 8 to 10 hours each
night. In Canada, electrode stimulators were actually
implanted in the paraspinal muscles. Although some pre-
liminary success was reported with transcutaneous stimula-
tion,21 most studies found that this form of treatment did
nothing to favorably alter the natural history of scolio-
sis.62,198,534,559 Today, electrical stimulation is no longer con-
sidered a useful method in the management of idiopathic
scoliosis.
Physical Therapy and Biofeedback
Consistent asymmetry in torso rotation strength has been
documented in patients with AIS when parameters such as
specific strength testing and myoelectric activity recording
are used.518 Although muscle conditioning is beneficial to a
patient’s overall well-being, only modest evidence supports
226 SECTION II  Anatomic Disorders
the concept that exercises or physical therapy programs are
helpful in controlling or improving scoliosis.540,541,710,871 Like-
wise, spinal manipulations and biofeedback have yet to be
shown to alter the natural history of scoliosis.
Surgical Treatment
The primary goals of surgical intervention in the treatment
of scoliosis are to reduce the magnitude of the deformity,
to obtain fusion for prevention of future curve progression,
and to do so safely. Operative treatment should result in a
well-balanced spine in which the patient’s head, shoulders,
and trunk are centered over the pelvis. Ideally, when this is
accomplished with newer surgical implants, a significant
amount of curve correction can be achieved in all three
planes.
Improved correction of deformity has resulted from a
combination of improved instrumentation systems that
impart more corrective force on scoliotic spines and
advances in surgical techniques that allow greater mobiliza-
tion and derotation of the spine. All pedicle screw–systems
provide greater opportunity to gain control of each vertebra,
which allows greater correction of each segment either indi-
vidually or through “en bloc” techniques. Today, with the
introduction of pedicle screw fixation at nearly every level
of the spinal segment requiring instrumentation, curve cor-
rection is even greater than that achieved with hook–rod
segmental fixation systems. These new pedicle screw
implant systems and the surgical techniques are complex
and require a significant amount of training. The established
segmental fixation systems—including Cotrel-Dubousset
(CD), Texas Scottish Rite Hospital (TSRH), and Isola
instrumentation—became popular in the mid-1980s and,
with refinements that allow the use of more pedicle screw
fixation points, remain so today. Each instrumentation
system allows the surgeon to achieve increased curve cor-
rection, improved sagittal contouring, brace-free postopera-
tive mobilization, and improved MRI compatibility (with
the availability of titanium components).
The expansion of technology since the early 1990s has
resulted in the availability of numerous other systems, with
many manufacturers developing very similar concepts cen-
tered around multiple fixation points with pedicle screws
that provide the opportunity to correct all three planes with
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
to progress, regardless of the curve pattern, and do not
require surgery. Thoracic curves and double major curves
that exceed 50 degrees at skeletal maturity have a signifi-
cant probability of worsening over time and nearly always
warrant operative intervention.811 Thoracolumbar and
lumbar curves of less magnitude, when associated with
marked apical rotation or translational shift, also have a
propensity to worsen over time in mature patients.811 In
these cases, surgery should be considered when the curves
exceed 40 to 45 degrees.
In addition to curve magnitude, the patient’s appearance
(as perceived by the patient, the family, and the surgeon)
plays a role in surgical decision making. Patients and their
families are usually most concerned about this aspect of the
deformity. The patient’s spinal balance may be decompen-
sated, with the thorax shifted noticeably away from the
midline; rib prominence may be severe because of excessive
rotation; and the shoulders and hips may appear uneven.
It is uncommon for back pain alone to serve as an indica-
tion for scoliosis surgery. Historically, 30% of patients with
scoliosis describe associated back pain; however, more
recently studies suggest that up to 75% of patients have
back pain that may be improved with surgery.869 Less than
10% of these symptomatic patients have a definite cause
found for the discomfort.616 In general, the treating ortho-
paedist should not assume that the patient’s discomfort will
be remedied by spinal fusion.
Curve patterns in males appear to be more rigid than
those in females with idiopathic scoliosis.305,738 When plan-
ning surgery in males, less curve correction should be
expected, but short- and long-term functional outcomes
and complication rates can be expected to be similar to
those in female patients.869
Preoperative Planning
Preoperative planning must take into consideration the
patient’s curve pattern and spinal balance, curve flexibility,
neurologic status, rib deformities, physical maturity and
future growth potential, and other surgery-related needs
(e.g., transfusion requirements, bone grafting, spinal cord
monitoring, postoperative pain management). The surgeon’s
selection of instrumentation depends on personal experi-
ence, availability of the various systems, and the choice of
anterior or posterior instrumentation.
Curve Patterns
King Classification System. In 1983, King and colleagues
introduced a radiographic classification system for AIS in
which five different thoracic curve types were described
(Fig. 12-25).384 This system provided useful recommenda-
tions for surgical intervention with Harrington implants;
Type I Type II Type III Type IV Type V
FIGURE 12-25  Diagrammatic representation of the King
classification of idiopathic scoliosis, which consists of five distinct
curve patterns. This classification continues to be used regularly,
although its reliability and reproducibility have been questioned.
(Redrawn from King HA, Moe JH, Bradford DS, et al: The selection
of fusion levels in thoracic idiopathic scoliosis, J Bone Joint Surg Am
1983;65:1302, with permission from The Journal of Bone and
Joint Surgery, Inc.)

however, with the evolution of multiple hook or screw
implant systems in the late 1980s, which allowed increased
three-dimensional curve correction, several shortcomings in
the descriptions and recommendations of the King system
became evident. The one King curve pattern that is not
better described in other systems is type IV, a thoracic curve
in which the L4 vertebra is tilted into the curve. This curve
pattern requires instrumentation to a more distal segment
into the lumbar curve, generally the vertebra last touched
by the center sacral vertical line (CSVL).
Lenke Classification System. Lenke and co-workers
developed a new classification system for AIS in 1997 that
recognized the important contributions already made by
King and colleagues.416 Specific goals for the new classifica-
tion system were to allow more acceptable comparisons
among the various types of operative treatment available,
to support a treatment-based approach by determining
which of a patient’s scoliotic curves should be included
in the instrumentation and fusion, to encourage three-
dimensional analysis of scoliosis, and to achieve greater
intraobserver and interobserver reliability.415 The Lenke
classification system is dependent on curve measurements
in both the frontal and sagittal planes.417 It is comprehensive
(42 different curve patterns can be derived) yet is fairly
easy for surgeons to learn quickly.415 The three main vari-
ables requiring evaluation are curve type (Table 12-3),
lumbar spine modifiers, and thoracic sagittal modifiers (Fig.
12-26).
Using standard-size projection-type slides of photo-
graphs of premeasured radiographs, the developers of the
Lenke classification system found both interobserver and
intraobserver reliability to be excellent.415,417 However, in
clinical practice, physicians vary significantly both in their
selection of end vertebrae and in their angular measure-
ments of curves in individuals with idiopathic scoliosis.113,526
Because scoliosis surgeons rarely have access to premea-
sured radiographs when they evaluate patients and formu-
late surgical plans, the reliability of the Lenke classification
system is less impressive when tested with unmarked radio-
graphs. When all three of the main variables are combined,
its overall reliability is fair.564,637 When each variable is
reviewed separately, its reliability improves. Despite this
limitation, the Lenke classification system offers a more
comprehensive preoperative radiographic evaluation of
patients with AIS than was available with previous systems
and appears to correlate with surgical treatment of struc-
tural regions of the spine.57,414,424
The first parameter to identify in the Lenke classification
system is curve type (see Fig. 12-26), which is determined
by first identifying the largest curve. The other curves are
then deemed structural by magnitudes that are greater than
25 degrees on the supine best-bend radiograph or if junc-
tional kyphosis (measured between T2 and T5 for the proxi-
mal and main thoracic curves and between T10 and L2 for
the main thoracic and thoracolumbar/lumbar curves) is
greater than 20 degrees. Once the type of curve is identi-
fied, the second main variable, the lumbar spine modifier,
is assessed (Fig. 12-27). The CSVL (vertical line drawn
upward from the center of the sacrum) is drawn, and its
position relative to the concave pedicle of the apical lumbar
vertebra determines the lumbar modifier. The final main
variable, the thoracic sagittal modifier (T5-12), is then
CHAPTER 12  Scoliosis 227
assessed to gain a better understanding of the three-
dimensional deformity. The sagittal modifier is hypoky-
photic (<10 degrees), normal (10 to 40 degrees), or
hyperkyphotic (>40 degrees). In addition to radiographic
characterization, the clinical appearance is critical to deter-
mine curves requiring surgical treatment. A comparison of
right and left shoulder height is important to determine
when inclusion of the proximal thoracic curve is necessary,
waistline asymmetry helps determine when inclusion of the
lumbar curve is necessary, and the axial plane rotational
deformities are critical when deciding which curves are
structural.
Construct Selection. Three major trends with respect to
surgical treatment of scoliosis have occurred: first, the pos-
terior approach has had greater use than the anterior
approach for nearly all curves, including Lenke 5 and 6
thoracolumbar and lumbar curves; second, pedicle screws
have been used in all regions, including the thoracic spine,
in place of hooks and hybrids380,412; and third, because of
greater use of posterior releases and more powerful poste-
rior implants, use of an anterior release has declined.164,254,435
Proponents of screws suggest they provide stronger fixa-
tion than hooks do, are safe when inserted by experienced
surgeons, avoid placing metal within the canal, provide a
stronger corrective force for three-dimensional curve cor-
rection, may obviate the need for anterior fusion to avoid
the crankshaft phenomenon because of the strength of the
construct, and may avoid the need to perform thoraco-
plasty. Those who question the routine use of pedicle screw
placement for thoracic scoliosis cite the significant increase
in cost with no documentation of measurable improvement
in outcome studies, early reports of loss of thoracic kyphosis
with screws, and the potential risk for injury to the spinal
cord by surgeons inexperienced in the technique.
Fusion selection recommendations are based on correct
identification of curve patterns and the clinical deformity
in each patient. Some generalities on selection of the
level can be taken into consideration when developing
plans for fusion. The upper instrumented vertebra (UIV)
is usually the proximal end vertebra for thoracic and
thoracolumbar/lumbar curves and, most often, T2 when
fusing a structural proximal thoracic curve. The lowest
instrumented vertebra (LIV) for lumbar/thoracolumbar
curves is usually the distal end vertebra when performing
posterior instrumentation. When fusing vertebrae for idio-
pathic scoliosis via the anterior approach, they should gener-
ally be the UIV and LIV. In addition to these guidelines,
each curve pattern has some specific rules to follow.
Lenke type 1A and 1B curves have single thoracic struc-
tural curves with nonstructural lumbar curves that do not
cross the midline (CSVL). Clinically, trunk imbalance is
more pronounced to the right in these patients than in those
with double-curve patterns and responds nicely to surgical
treatment. For posterior constructs the UIV is generally the
proximal end vertebra or one proximal to it when it is sig-
nificantly off midline. The rules for selection of the LIV are
more varied, with three general rules: first, fusion to one or
two levels proximal to the stable vertebra633; second, fusion
to the vertebra that last touches the CSVL; and third, as
reported by Suk and colleagues, rules based on the relative
position of the neutral vertebra (the most proximal vertebra
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
radiographs
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
radiographs
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
of the pedicles) and the position of the distal end verte-
bra.747,751 Preoperative planning for posterior instrumenta-
tion using only screws is shown in Figure 12-28. Frequently,
the implant is a hybrid, with screws used as an anchor at
the bottom of the constructs and some combination of
hooks, sublaminar wires, or tape and screws used
above.118,332,666,858 For the King-Moe type IV curve (a Lenke
1A curve when L4 is tilted into the curve), the end vertebra
may be chosen as the LIV, especially when the stable
vertebra is L4, and posterior instrumentation can be stopped
at L2 (two levels cephalic to the stable vertebra). Anterior
instrumentation has shown that even more inferior motion
segments can be preserved because the implant extends
down to only the lowest vertebra included in the measured
curve.64,358,413
Patients with Lenke type 1C curves have single thoracic
structural curves with nonstructural lumbar curves, and the
apical lumbar vertebra completely crosses the midline (i.e.,
is not in contact with the CSVL). In the late 1980s and
 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)

A
(No to minimal
curve)

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

B
(Moderate curve)

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

C
(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-
racic fusions often led to spinal imbalance manifested as a
shift in the patient’s trunk or head (or both) to the left of
midline—so-called decompensation.‖a
Despite many purported reasons, including improper
choice of fusion levels, overcorrection of the thoracic curve,
incorrect identification of curve patterns, lumbar curve
stiffness, and progression, the cause of decompensation
‖a
References 46, 55, 88, 419, 489, 493, 519, 628, 633, 764.
was most likely multifactorial, and improvement occurred
over time as the uninstrumented lumbar curve adapted
(Fig. 12-29).489,628
When using this method of instrumentation for selective
fusions in individuals with Lenke type 1C curves, the
surgeon should keep in mind some principles that can mini-
mize the chance of postoperative imbalance. First, the
instrumentation should not extend beyond the stable ver-
tebra; second, if using the rod rotation maneuver, incom-
plete rotation should occur to avoid overcorrection or
 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
A
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
B
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.)

T4
L3
FIGURE 12-28  Construct planning for the use of all pedicle screws
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.
For the double major curve shown, the most common screw
pattern is segmental screw fixation for the left rod, which is used
to correct the coronal plane while maintaining the sagittal plane
or correcting any junctional kyphosis between the main thoracic
and lumbar curves. Typically, variable-angle or polyaxial screws
can be used in the lumbar spine and fixed-angle screws in the
thoracic spine. If a segmental screw pattern is not used, an
alternative screw pattern on the left side should always include
segmental fixation of the lumbar curve and a more typical
hook–screw construct for the thoracic curve. However, to achieve
maximal correction, segmental fixation with pedicle screws is
generally used for the left rod.
The screw pattern on the right rod generally includes at least
two screws in the distal segment of the lumbar spine to allow
fine-tuning of the lowest instrumented vertebra to ensure that it  
is horizontal and neutral. L2 and L3 screws are used for complete
fixation of the lumbar curve, and T11 and T12 screws are used for
fine-tuning of the lumbar curve. Screws are used at the apex of
the convexity of the thoracic curve to provide an opportunity to
perform a convex apical derotation maneuver to correct the axial
plane deformity in the thoracic spine. Two screws are then used at
T4 and T5 to provide good fixation proximally.
Once the screws are placed on the left side, a rod rotation
maneuver is performed to correct the coronal plane and sagittal
plane deformity. The apical convex screws can then be used to
derotate the apical thoracic spine after loosening the rod on the
concave apical segments. Downward and lateral pressure on the
convex screws with upward and medial pressure on the concave
screws allows apical derotation. Following this, the right rod can
be placed, and fine-tuning of the coronal and sagittal planes is
performed.
CHAPTER 12  Scoliosis 231
A B
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
12-28 because of the large stiff nature of the curve.
excessive straightening of the thoracic curve; and third,
other correction strategies such as apical translation with
fixed proximal and distal anchors or a cantilever can be
performed. Preoperative planning involving pedicle screw
anchors for selective thoracic instrumentation and fusion of
a Lenke type 1C curve is shown in Figure 12-30.
Failure to properly distinguish Lenke type 1C or King
type II curves from true double major curves (Lenke type
3C) may be responsible for some cases of postoperative
imbalance following selective thoracic fusions. Useful
guidelines have been developed to help differentiate these
curve patterns.419 Relative ratios between the thoracic and
lumbar curves with regard to their size, rotation, and devia-
tion from midline can be assessed preoperatively on a stand-
ing radiograph. If thoracic curve parameter–lumbar curve
parameter ratios are less than 1.0, both curves will require
fusion. If the ratios are greater than 1.2 for curve size and
deviation and greater than 1.0 for rotation, selective tho-
racic fusion can be performed safely. With true double
major curves, both curves must be included in the posterior
fusion to achieve a balanced spine with segmental fixation
systems.
Selective anterior fusion on the convexity of the thoracic
component in Lenke type 1C curves (using screws and
either a threaded or a smooth rod) has been advo-
cated.66,413,844 Reported advantages over posterior instru-
mentation include improved balance, correction of a
hypokyphotic thoracic spine, and preservation of more infe-
rior motion segments.
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.

B C D
 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
 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.
B

T-12

B C
 CHAPTER 12  Scoliosis 235

posteriorly and determine flexibility of the spine with the
newer generation of instrumentation systems. These radio-
graphs are used to determine the flexibility of the remaining
curves to provide a Lenke classification. They can also be
used to help determine or confirm the LIV for isolated
thoracolumbar or lumbar curves.122,388,794 For left thoraco-
lumbar or lumbar curves, a right-bend film should demon-
strate that the proposed LIV is centered over the sacrum
and, on the left-bend film, should demonstrate “reversal”
of the disk above the intended LIV. Large curves (>75
degrees) are best analyzed with traction films because the
mechanics of the bend films is minimized for these large
and stiff curves.603 The push-prone radiograph was shown
to be the best preoperative indicator of flexibility for pre-
dicting the final lumbar curve measurement in patients
undergoing selective thoracic fusion for Lenke type 1B and
1C curves.189 Significant study using the fulcrum bend test
has demonstrated that for the thoracic curve, this test best
predicts the status of the thoracic curve following posterior
spinal fusion and instrumentation.227,298,466,467
Neurologic Status. If a subtle neurologic abnormality (e.g.,
asymmetric abdominal reflexes) is detected in an otherwise
normal individual, MRI of the entire spinal canal should be
considered to rule out syrinx, cord tethering, or diastema-
tomyelia.864 Preoperative MRI should also be performed in
patients with left thoracic curves and in those in whom the
typical apical lordotic sagittal deformity is absent because
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
MRI.175,636
Future Growth Potential. Correction of scoliosis by poste-
rior spinal instrumentation and fusion is usually maintained
over time and is not adversely affected by any remaining
anterior spinal growth; however, the crankshaft phenome-
non (resumption of the curve secondary to anterior growth
in patients with posterior fusion) can still occur. Dubousset
coined the term when he observed that the entire spine and
trunk gradually rotated and deformed as the anterior portion
of the spine continued to grow and twist around the axis of
the fusion mass (in a manner similar to an automobile
crankshaft)196 (Fig. 12-33). Methods to prevent this phe-
nomenon include careful assessment of the growth remain-
ing, the use of anterior fusion when appropriate, and greater
use of and correction with pedicle screw fixation. Although
its severity is difficult to quantify, the crankshaft phenom-
enon can best be appreciated by examining serial clinical
photographs that demonstrate progressive changes in rib
deformities, narrowing of the chest, and imbalance in the
thoracic and lumbar spine. Radiographs can also demon-
strate progressive changes over time, such as alterations in
curve size, rotation, and rib–vertebral angle difference;
translation of the apical vertebra toward the chest wall on
the convexity; and changes in the vertical inclination of the
instrumentation. Radiographic changes of more than 10
degrees in curve size, apical vertebral rotation, and the
rib–vertebral angle difference are all thought to reflect pro-
gression of the deformity secondary to the crankshaft phe-
nomenon.288,405,671 However, during the first 6 to 12 months

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.
236 SECTION II  Anatomic Disorders
after surgery, it is important to not automatically assume
that changes in radiographic measurements are a result of
the crankshaft phenomenon; these changes are often due to
stress relaxation of the spine, gradual maturation of the
fusion mass, and realignment of the curve.
For female adolescents in need of surgery who have not
yet reached their PHV, who are premenarchal, and whose
triradiate cartilage remains open, strong consideration
should be given to combining anterior and posterior fusion
to prevent the crankshaft phenomenon.191,405,673,697 For ante-
rior spinal fusion, a conventional open thoracotomy approach
has been compared with the newer, less invasive video-
assisted thoracoscopic surgery (VATS).316,503,549,737,804 Advan-
tages of VATS include muscle sparing, improved cosmetic
results (less scarring), greater access to the entire length of
the thoracic spine, and less effect on pulmonary function
than with open thoracotomy. Instruments are used through
multiple intercostal portals to resect disk material, perform
anterior release, and insert bone graft. However, surgeons
require extensive training in the VATS technique.
Some reports suggest that stiff posterior constructs, par-
ticularly when screws are used at nearly every level in the
segment fused, may be strong enough to prevent the crank-
shaft phenomenon in immature patients, thus avoiding the
need for anterior fusion.100,412,759 More research is needed to
prove the effectiveness of this approach.
Transfusion Requirements. Several procedures are available
to reduce the need for homologous blood transfusions in
patients undergoing posterior spinal instrumentation for
scoliosis, including controlled hypotensive anesthesia, autol-
ogous blood predonation of 1 or 2 units, acute normovole-
mic hemodilution, intraoperative and postoperative salvage
of lost blood, intraoperative use of antifibrinolytics, and
transfusion decisions based on clinical judgment rather than
on a predetermined hemoglobin value.
Various combinations of these methods have been shown
to significantly reduce exposure of patients to homologous
blood products during scoliosis surgery.¶a The combination
of predonated autologous blood, hypotensive anesthesia,
and intraoperative salvage of lost blood is probably the
one used most frequently for healthy individuals with idio-
pathic scoliosis.520,532 Intraoperative salvage of lost blood,
the most expensive of the available techniques, is most
effective when blood loss is expected to exceed 1000 mL.
Acute normovolemic hemodilution appears to be a satisfac-
tory alternative to the use of predonated autologous
blood.143,567
The antifibrinolytic agent ε-aminocaproic acid (Amicar)
is reportedly a safe, effective, and inexpensive method of
reducing perioperative blood loss in patients with scolio-
sis.174,241 We generally prefer tranexamic acid (TXA), which
has also been shown to reduce intraoperative and postopera-
tive blood loss during posterior spinal fusion and instrumen-
tation for AIS in a matched cohort.856 The response to TXA
depends on the dose administered, with a higher loading
dose of 20 mg/kg followed by a 10-mg/kg/hr infusion
appearing to have a greater effect.270
¶a
References 20, 142, 143, 229, 240, 241, 253, 329, 350, 393, 520,
532, 567, 598, 701, 703, 788.
Bone Grafting. The primary goal of scoliosis surgery is to
achieve a solid arthrodesis, which is enhanced by meticulous
cleaning of soft tissue from the spine, facetectomies, decor-
tication, and adequate bone grafting. Although autogenous
iliac crest bone grafting has previously been the stan-
dard,133,801 significant postoperative pain at the donor site
persists and remains the greatest problem with the use of
autogenous grafting.274,707 Because of these postoperative
symptoms, alternative bone graft substitutes have been
sought.67,333,430,431,860 Numerous studies of successful fusions
using allografts of frozen, bank-stored bone as a substitute
for autogenous bone have been reported34,75,190,214,274,730
without an increase in pseudarthrosis rates.190,214
To minimize the risk of transmitting human immunode-
ficiency virus, hepatitis virus, and any other potential viral
pathogens, the donor blood and tissue are tested at the site
of recovery, and testing is usually continued throughout the
harvesting process. Freeze-dried cancellous bone is usually
exposed to low-dose gamma radiation to sterilize all non-
systemic bacterial and fungal contaminants. Bone morpho-
genetic protein has become popular for use in single-level
lumbar spinal fusions but has yet to become cost-effective
for routine use in multisegment scoliosis fusions.5,99,496,606,789
The authors’ preferred technique is to perform thorough
stripping of the spine, followed by aggressive facetectomies,
decortications of the spine, and the use of allograft bone,
without any pseudarthrosis in individuals with AIS being
documented in the past 8 years.
Spinal Cord Monitoring. Spinal cord monitoring using both
spinal somatosensory evoked potentials (SSEPs) and motor
evoked potentials (MEPs) is the standard of care during
scoliosis surgery and is critical to the safety of any spine
deformity surgery. SSEPs record the sensory function of the
spinal cord and provide continuous monitoring throughout
the procedure.#a This test may, however, be adversely
affected by changes in anesthetic level and perfusion, and
critical changes tend to lag behind MEPs. With impending
neurologic deficit, MEPs are used to monitor the anterior
spinal cord motor tracts and are ideally performed by apply-
ing a stimulus to the motor cortex of the brain (transcranial
MEPs [tcMEPs]).59,263,575,680,726,754 This provides direct stim-
ulation of the motor cortex, which then travels through the
anterior column tracts with responses noted in the upper
and lower extremity musculature (Fig. 12-34). When MEPs
are used in conjunction with SSEPs, the chance of unrec-
ognized injury to the spinal cord is minimized. A large
multicenter study of 1121 patients demonstrated that 38
(3.4%) had a critical change on monitoring when tcMEPs
and SSEPs were used. Hypotension was responsible for nine
changes (corrected by elevating blood pressure), whereas
three were due to segmental vessel ligation. The tcMEP/
SSEP combination did not miss any patient with a transient
motor or sensory deficit.679 Similar studies have demon-
strated excellent results with combined multimodal intra-
operative neuromonitoring during AIS surgery.230,584,767,868
Although total intravenous anesthesia with propofol is
necessary to obtain good tcMEP data, other techniques
to assist in obtaining good monitoring data have
#a
References 25, 123-125, 307, 468, 527, 555, 579.

FIGURE 12-34  Output of a typical response when using
transcranial motor evoked potentials (tcMEPs). The left extremities
(left) and right extremities (right) are shown. The red response is
the baseline data, whereas the green notes the latest output from
the most recent “run” of tcMEPs. The muscle group in the upper
extremity is the abductor pollicis brevis, and the lower extremities
are evaluated with three muscle groups—the tibialis anterior (LTA
and RTA), the soleus (LSOL and RSOL), and the abductor hallucis
(LAH and RAH). Note that the most recent amplitudes (green) are
the same as the baseline (red) in all muscle groups demonstrating
good responses.
been evaluated.370,481,868 The current increase in the use of
thoracic pedicle screws for fixation points has led some
surgeons to use triggered electromyographic (EMG) moni-
toring; however, this has been relatively unreliable in the
thoracic region without clearly identified thresholds to indi-
cate when medial screw penetration is seen.620
The wake-up test, a gross evaluation of motor function,
is no longer used routinely if spinal cord monitoring is avail-
able and results are normal throughout surgery. The wake-up
test can be performed if changes in SSEPs or MEPs are
noted during correction of the spine because spinal cord
injury may exist even when monitored variables return to
baseline.552 The authors generally do not perform this test
during AIS surgery because first, a “normal” wake-up test
does not provide a detailed examination and strength testing
is not possible and, second, even if normal, tcMEP/SSEP
monitoring can indicate some stress or subclinical deficit in
the spinal cord that may become a permanent deficit with
continued surgery. For this test the anesthesiologist allows
the patient to regain partial consciousness and motor func-
tion during the surgical procedure.285 Intraoperative neuro-
monitoring is especially useful when intraoperative traction
is used because critical changes develop in a third of patients
during surgery and are related to having a thoracic curve, a
larger Cobb angle, and a rigid curve. A stepwise response
to these changes, including removal of the traction, resulted
in overall good results, and the presence of MEP recordings
at the completion of the surgery was associated with normal
neurologic function.432
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.
Close monitoring of the patient’s respiratory status and the
use of pulse oximetry are necessary for 24 hours after the
infusion has been discontinued. Postoperative pulmonary
toileting is optimized with this technique.
Ketorolac, an injectable nonsteroidal antiinflammatory
drug, is effective for the short-term management of moder-
ate to severe postoperative pain. It is often used in conjunc-
tion with opioids because the combination provides more
effective analgesia than either drug alone does. Although its
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
correction.740,803
Antibiotic Prophylaxis for Dental Procedures. Antibiotic
prophylaxis for dental procedures in patients with spinal
instrumentation is a controversial issue.631 Currently, no
scientific evidence supports the position that antibiotics
should be given during routine dental care. Streptococcus
viridans, the predominant bacterium in normal human oral
flora and the most common organism isolated from blood
after dental procedures, has not been reported in delayed
deep wound infections following spinal instrumentation. In
those in whom early postoperative wound infections
develop, Staphylococcus aureus is the predominant organ-
ism. Yet S. aureus accounts for only 0.005% of the normal
oral flora and is rarely isolated after dental procedures.166
Guidelines similar to those provided in the advisory
statement issued by the AAOS regarding antibiotic prophy-
laxis for dental patients with total joint replacement should
be used for those who have undergone spinal instrumenta-
tion.19 If antibiotic prophylaxis is given, the following
regimen is recommended: patients who are not allergic to
penicillin can be treated with cephalexin, cephradine, or
amoxicillin, 2 g orally 1 hour before the dental procedure;
patients who are allergic to penicillin should receive
clindamycin, 600 mg orally 1 hour before the dental
procedure.
*bReferences 26, 204, 373, 464, 485, 687, 736, 769.
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
Orthopaedics.755

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
spine posterior and medial for correction. This maneuver,
which continues to be used today, improves the sagittal
contour, achieves significant curve correction, and improves
the rotation or translation of the spine. The second rod
increases the construct’s strength and torsional stability,
particularly when rigidly united to the first rod via a rod-
connecting device.
Numerous reports have documenteded significant
improvement in the correction of idiopathic scoliosis with
CD instrumentation. Rib deformities were reduced, curve
correction in the range of 48% to 69% was achieved and
maintained, and nearly normal sagittal alignment was
restored.‡b The ability to preserve lumbar lordosis in curves
requiring long fusion to L3 or L4 avoided the long-term “flat
back” problems that occurred with Harrington distraction
instrumentation.
The TSRH instrumentation system was introduced in
1988 and, like the CD system, uses multiple hooks and
screws to attach smooth, precontoured rods to the spine.32,345
Once the system is assembled, selective compression, dis-
traction, and rotation maneuvers can be performed to
correct the spinal deformity. These maneuvers follow the
principles introduced by Cotrel and Dubousset148 and have
resulted in outcomes similar to those with CD instrumenta-
tion.72,634,718 The technique of a multiple-hook system is
†b
References 160, 178, 304, 328, 458, 507, 576, 817.
‡b
References 71, 87, 154, 236, 276, 328, 399, 401, 420, 421, 422, 601,
612, 633, 654, 696, 793, 812, 842, 848.
FIGURE 12-35  Cotrel-Dubousset (CD) instrumentation. The first
contoured rod is secured to the concave side of the spine with
multiple hooks and is rotated 90 degrees. This maneuver improves
the sagittal contour and achieves significant correction of the
curve. Placement of the second rod increases the construct’s
strength. CD instrumentation uses numerous hooks but no
sublaminar wires.
illustrated in Plate 12-2 on page 294 on the accompanying
website.
Pedicle Screws. The use of pedicle screws has dramatically
changed the operative treatment of all spinal deformity,
including AIS. The use of pedicle screw fixation for correc-
tion of deformity was first described in the lumbar spine in
the mid-1990s.38,289,749 These studies demonstrated that
pedicle screws provide greater ability to obtain and maintain
coronal plane correction of the thoracolumbar or lumbar
curves in individuals with double major idiopathic scoliosis.
When compared with hooks, initial correction was 72%
versus 60% with hooks, and less loss of correction occurred
at follow-up (5% versus 13%).38 Significant improvement in
LIV tilt (82% versus 50%) and translation (50% versus 23%)
was seen when compared with hooks.289
Pedicle screws placed in the lumbar spine for pediatric
spinal deformity have a very good track record, with few
complications.76,91,212,453,677 The improved correction of
deformity and maintenance of the correction achieved with
 CHAPTER 12  Scoliosis 239

A B

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
(Fig. 12-36). Suk and coauthors first reported the routine
use of pedicle screws in the thoracic spine for spinal defor-
mity surgery in 1995 and achieved improved coronal plane
correction in the screw group (72%) versus hooks (55%)
and hybrids (66%).750 The initial reports of their safety
demonstrated overall good results, with a pedicle screw
breech rate of between 1.5% and 15% and few neurologic
complications.2,52,186,395,748 The learning curve is steep, and
greater surgeon experience leads to improved accuracy.2,669
Reports of improved correction of spinal deformity have led
to fairly enthusiastic adoption of the technique by surgeons,
with overall improved radiographic correction when
compared with more traditional hook constructs.§b The
improved coronal plane correction achieved with pedicle
screw fixation can be attributed to several factors. First,
surgeons generally place pedicle screws at more levels than
when hooks or other anchors are used; second, the three-
column fixation of the spine provides better “grip” of the
vertebrae, so correction maneuvers yield greater improve-
ment in the spinal deformity; third, the use of procedures
to mobilize the spine has expanded and included greater use
of Ponte-style or Smith-Petersen osteotomies, concave and
convex rib osteotomies, and for very severe curves, use of
§b
References 100, 380, 381, 395, 440, 605, 715, 729, 752, 798.
240 SECTION II  Anatomic Disorders
the VCR procedure; and finally, with improved fixation, the
surgeon can use a number of correction strategies, including
the traditional rod rotation maneuver, segmental distraction
or compression, and segmental in situ bending. Fixation of
each vertebra in the instrumented segment may be an
important factor when coronal plane correction with tho-
racic pedicle screws is analyzed. However, the appropriate
screw “density” (the number of pedicles filled with pedicle
screws relative to the total number that are available) is not
well known, and early studies demonstrated conflicting
results, with some showing a positive effect with improved
coronal correction136 and others demonstrating no differ-
ence between high and low screw density.69 In North
America, longer-term follow-up has demonstrated mainte-
nance of correction with nearly 70% coronal plane
correction.411
Perhaps the greatest advantage of thoracic pedicle screws
is improved axial plane correction. Lee and associates
described a DVR maneuver in which the concave and
convex screws in the juxtaapical vertebrae were rotated
opposite the direction of the rod rotation maneuver.410 With
CT they demonstrated better apical rotation in the group
that underwent DVR than in those who did not (42.5%
versus 2.4%). The technique of apical derotation can be
performed via a number of methods, and all have demon-
strated some success in improving the rotational deformity
in a variety of AIS curves.185,330,331 Use of this technique may
obviate the need for thoracoplasty and the associated
detrimental effect on pulmonary function (see Plate 12-3
on page 297).156,354 However, care in maintaining thoracic
kyphosis when performing these DVRs is necessary to avoid
creation of lordosis as one pushes anteriorly on the spine.
This loss of thoracic kyphosis is accompanied by a loss of
lumbar lordosis.550 Large-diameter rods with an accentuated
thoracic kyphosis contour help maintain the thoracic kypho-
sis with the posterior approach,512 whereas the anterior
approach maintains kyphosis because correction of the
coronal plane is achieved with shortening of the anterior
column.734 It is important to preserve the tension band of
the soft tissues proximal to the planned instrumented levels
to prevent junctional kyphosis, which has been reported
with the use of pedicle screws.306
Surgical treatment of spinal deformity has three main
aspects. The first is to grab onto the spine with anchors
strategically placed on the spine, typically with pedicle
screws today. The second is to mobilize the spine when
necessary through a variety of techniques, including intra-
operative traction, multiple Ponte-style osteotomies, or
even resection procedures. Finally, once the anchor points
are placed and the spine is flexible (either inherently so or
following mobilization techniques), a variety of surgical
techniques can be used to improve the spinal deformity.
Thoracic pedicle screws can be placed in several ways,
but generally two main methods are used: freehand, in
which the screws are placed without the use of fluoroscopic
guidance, and image guided, in which the screws are
inserted under the guidance of some radiographic imaging
modality.379
Freehand Technique. The freehand technique relies on a
thorough understanding of pedicle anatomy in the thoracic
spine, including the anatomic landmarks for the starting
point and the trajectory and general guidelines with regard
to the width, height, and depth of the pedicles in the
various regions of the thoracic spine.‖b In addition, an
understanding of the surrounding anatomic structures later-
ally and medially is important to avoid injury.439,735 In
general, the width of the thoracic pedicle is smaller in the
proximal part of the thoracic spine, on the concavity of the
upper and main thoracic curves, and with greater curve
magnitude. The spinal cord is positioned adjacent to the
concave pedicles with less than 1 mm of epidural space, as
compared with 3 to 5 mm on the convex side.439 At the
apex of a right thoracic scoliosis, the aorta is positioned
more lateral and posterior to the vertebral body than in a
normal, straight spine.439,735 The combination of narrow
pedicles, dural sac proximity medially, and aorta proximity
laterally makes safe screw placement challenging on the
concavity of these thoracic curves. The most challenging
pedicles are those in the proximal part of the thoracic spine,
especially on the concave aspect. The freehand technique
entails identifying the starting point for screw insertion,
decorticating that level with a burr, entering the cancellous
channel with a pedicle finder, and traveling down the pedicle
via manual pressure. The channel is then probed to ensure
that all five walls (anterior, medial, lateral, superior, and
inferior) of the pedicle are intact, followed by tapping the
pedicle, reprobing to ensure maintenance of the pedicle
walls, and placing the screw. Fluoroscopy or plain radiogra-
phy is then used to check the position of the screws. Kim
and co-workers reported on 3204 screws placed in the
thoracic spine for spinal deformity and randomly analyzed
577 screws with CT imaging.379 They demonstrated that
6.2% of the screws had moderate cortical perforation,
including 1.7% with medial wall violation; however, no neu-
rologic or vascular complications occurred. Pedicle screws
have been placed successfully in patients with severe defor-
mities and scoliosis curves greater than 90 degrees, with a
thoracic pedicle screw accuracy rate of 96.3% and no neu-
rologic complications.395
Image-Guided Techniques. A variety of image guidance
techniques have been described, from plain radiography
following guidewire placement to continuous stepwise fluo-
roscopic evaluation299,398,748 to true surgical navigation using
preoperative or intraoperative CT scanning and intraopera-
tive computer navigation.¶b Suk and colleagues described a
method in which anatomic landmarks are used to identify
the starting point and guidewires are drilled into the center
of the pedicle, which are then visualized on a plain PA
radiograph.748 Based on these radiographic images, adjust-
ments are made in the position of the guidewires before
placing the screws. A review of their experience in placing
4604 thoracic pedicle screws in 462 patients demonstrated
that 1.5% of the screws were malpositioned in 10.4% of the
patients, with only 4 screws (0.09%) placed medially. One
patient experienced transient paraparesis, and three had
dural tears.
True image guidance systems rely on either a preopera-
tive or an intraoperative CT scan to define the spinal
anatomy (initial data acquisition), followed intraoperatively
by image-to-patient registration. Surgical navigation is
then performed during placement of the pedicle screws.
‖b
References 391, 557, 577, 581, 582, 790, 791, 880, 881.
¶b
References 6, 247, 280, 355, 402, 618, 866.

Early results demonstrated similar or improved screw
accuracy when compared with more conventional
imaging.247,280,402,406,618 One study reported that a potentially
unsafe screw was 3.8 times less likely to be inserted with
navigation and that the chance of a significant medial breach
was 7.6 times higher without navigation.786 Alternatively,
CT imaging may be used to confirm accurate screw place-
ment, although the systems currently available provide less
detail than a typical CT scanner found in radiology suites
does. All these image guidance techniques expose the
patient and surgeon to increased radiation, which may have
long-term effects.346,660,709
Other Methods of Assessing Screw Placement. Other
methods to determine safe and accurate screw placement
include EMG stimulation, which has produced reliable
results and clear guidelines when lumbar pedicle screws are
within the pedicle.137,427,690 The premise of this technique is
that when the electrical current is passed through a com-
pletely intraosseous pedicle screw, it will not result in a
triggered EMG peripheral response. With greater stimulus
intensity, however, even a well-placed screw will trigger a
peripheral response, so guidelines have been established
based on the level of stimulation required to elicit a response:
greater than 8 mA defines a screw completely within the
pedicle; between 4 and 8 mA is intermediate, which means
that the screw should be removed and the medial wall
probed; and less than 4 mA indicates a strong likelihood of
a pedicle wall defect. Although good success has been
achieved in the lumbar spine, studies involving the thoracic
spine have not been able to clearly define threshold values
for determining a safe screw.433,620,624 The technique of
EMG stimulation for ascertaining thoracic pedicle screw
placement is technician dependent and takes some experi-
ence to perform. It should serve only as an adjunct to
thorough understanding of the pedicle anatomy, meticulous
surgical technique, and good imaging.
The improved radiographic correction seen with the use
of thoracic pedicle screws has not been directly associated
with improved clinical outcomes. Comparisons of thoracic
pedicle screws and hook constructs or a hybrid construct
(hooks proximally and screws distally) have demonstrated
no difference in 2-year postoperative scores and little or no
correlation between coronal plane correction in AIS and the
SRS outcome instrument.158
The advantages of improved radiographic correction
when using thoracic pedicle screws for the treatment of
spinal deformity must be weighed against the risk and cost
of these implants. In most series the incidence of the most
feared complications—neurologic injury and major injury to
soft tissue structures, including the great vessels—is very
low (<1%).#b A study of more than 19,000 patients who
underwent surgery for pediatric spinal deformity demon-
strated fewer neurologic deficits with pedicle screws (0.7%)
than with wires (1.7%).621 This may be due to the significant
experience gained by the initial users of the technique, as
well as the relatively easy transition from the lumbar spine
to the thoracic spine. Neurologic injuries directly related to
the use of thoracic pedicle screws have been reported,
however.15,580 The final consideration is the cost of the
implants, which is higher than that of hook or hybrid
#b
References 51, 52, 379, 715, 729, 748, 798.
CHAPTER 12  Scoliosis 241
constructs because of the higher cost per implant, as well
as the increased number of screws used in each case.639
Posterior Mobilization Techniques. Removal of the inferior
facet at all instrumented levels has been the standard tech-
nique to achieve fusion when performing surgery to correct
spinal deformity. The opportunity for greater three-
dimensional spine correction with the use of pedicle screws
has been accentuated by a trend toward performing more
aggressive spine mobilization procedures, thereby providing
an opportunity to gain greater correction of deformity.22
Beyond standard facetectomies, the gradual resection of
more posterior structures provides greater spine mobility in
the following order: complete facetectomies/ligamentum
release (Ponte or Smith-Petersen osteotomies), concave or
convex rib resections (or both), vertebral body decancella-
tion with wedge resection of the vertebra, pedicle subtrac-
tion osteotomy, and finally, complete VCR.
A Ponte-style osteotomy generally refers to complete
removal of both the superior and inferior facets at all levels,
typically in the setting of Scheuermann kyphosis. However,
this nomenclature has been applied to the scoliotic spine
deformity as well, especially when the anterior column is
unfused. The Smith-Petersen osteotomy, in contrast, typi-
cally refers to performance of these complete facetectomies
in the setting of a fully or partially arthrodesed anterior
column. The ability to achieve correction with Ponte or
Smith-Petersen osteotomies for AIS was characterized well
for thoracolumbar and lumbar curves when it was first done
on the convex spine, with overall 80% correction of these
curves.699 Correction of thoracic curves with these osteoto-
mies is most likely less than that seen in the lumbar spine
because the ribs and chest wall limit correction and blood
loss and operative time can be higher.284 However, bilateral
complete facetectomies allow one to lengthen the posterior
column to restore thoracic kyphosis while also “untether-
ing” the concave spine to allow shortening and correction
of the coronal plane deformity. Following complete removal
of the inferior facet, the ligamentum flavum is removed
with a large Leksell rongeur in the midline until epidural fat
is seen, and completion of the flavectomy is then performed
with a Kerrison rongeur (Fig. 12-37). The superior facet on
the convex side can be largely removed with a small Leksell
rongeur because the dural sac is positioned closer to the
concave spine. Care is taken to limit the depth of penetra-
tion of the rongeurs on the convex spine because the epi-
dural veins are more prominent on this side. The concave
superior facet is more safely removed with Kerrison ron-
geurs because the dural sac is in close proximity and care
must be taken to remain directly on the bone when resec-
tion is performed. Subtle SSEP changes may be seen fol-
lowing these osteotomies.
The next level of posterior release would be rib resec-
tions on the concave side of the spine to allow lateral and
posterior translation of the spine. Three to five ribs are
resected by first performing subperiosteal dissection, typi-
cally beginning a couple of centimeters lateral to the trans-
verse process and continuing for 3 to 5 cm. Because the ribs
are close to the anterior aspect of the chest, small tears in
the parietal pleura tend to occur and should be recognized
at the time of surgery; placement of a chest tube may be
required to prevent pneumothorax.206 The authors do not
242 SECTION II  Anatomic Disorders
FIGURE 12-37  Ponte osteotomy. A Kerrison rongeur is completing
removal of the posterior elements of the spine. Epidural fat is seen.
generally perform these resections because experience has
shown limited appreciable correction and the risk for post-
operative pulmonary issues and pneumothorax is high.
The VCR procedure is the most aggressive technique for
achieving correction of the spine because it removes one or
more vertebral segments at the apex of the deformity. It
was initially described for severe spine deformity and trunk
shift and used an anterior and posterior approach to the
spine, with overall outstanding radiographic and clinical
results. Bradford and Tribus reported on 24 patients with
severe curves in which greater than 80% correction of the
coronal and sagittal plane translation was achieved along
with 50% correction of the curves. Despite complications
in 14 of the patients, the overall satisfaction rate in patients
was extremely high.84 Suk and co-workers described the
posterior-only approach for the VCR procedure and
reported 62% correction in the coronal plane and 45 degrees
of correction in the sagittal plane in 70 patients with severe
deformity from kyphoscoliosis, postinfectious kyphosis, and
adult scoliosis. Similar to previous reports, the complication
rate was relatively high, with 24 patients having complica-
tions, including 2 with postoperative spinal cord deficits
who had neurologic deficits preoperatively.745 Lenke and
coauthors reported their experience in performing posterior
VCR for pediatric spinal deformity in 35 consecutive
patients. The patients were divided into five diagnostic cat-
egories, including severe scoliosis, global kyphosis, angular
kyphosis, kyphoscoliosis, and congenital scoliosis, with
reported correction of 51%, 55%, 58%, 54%, and 60%,
respectively. No spinal cord–related complications were
reported; however, two patients lost intraoperative neuro-
monitoring data during correction with return of baseline
monitoring, which resulted in prompt surgical interven-
tion.426 The authors’ experience is similar in 32 consecutive
VCR procedures: 4 were done via an anterior/posterior
approach and the remainder via a posterior-only approach,
with coronal plane correction from 122 degrees to 53
degrees and an average increase in T1 to T12 height of
6.1 cm associated with improvement in pulmonary func-
tion. The incidence of intraoperative changes in neuromoni-
toring parameters during surgery was 35%, with two patients
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
to the VCR procedure begins with rigid fixation of the spine
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
levels. The ribs associated with the resected levels are
removed entirely, including the rib heads. A laminectomy
is next performed to identify the neural elements. The
authors prefer to ligate the nerve roots on the convex side
first and to remove the pedicle or pedicles to the level of
the floor of the spinal canal (Fig. 12-38, B). A provisional
rod is then placed on the convex side and the concave
osseous elements are removed, including the pedicle and
the concave side of the vertebral body after the concave
nerve roots have been ligated and transected. The concave
pedicle and floor of the canal are very difficult to remove
because the dural sac and spinal cord are tightly adherent
to these structures and the cancellous channel to the
concave pedicle is limited. Following complete resection of
the concave bone, a second provisional rod is secured so
that two provisional rods (one on the concave and one on
the convex side) are in place (Fig. 12-38, C). The remainder
of the convex bony anatomy can be removed with both rods
in place because the convex bone is anterior to the convex
rod. Following complete resection, exchange of the provi-
sional rods for the final rods is accomplished. The authors
typically remove the convex provisional rod and replace it
with an undercontoured final rod that is seated in the proxi-
mal screws and secured firmly and then partially seated in
the tips of the first two reduction screws distal to the
resected area (Fig. 12-38, D). The final concave rod is
placed similarly. The distal aspects of the two final rods are
then pushed anteriorly to unhinge them from the set screws
on the reduction screws while still providing some guidance
to the spine segments. The spine can then be manually
compressed without jeopardizing the screw–bone interface.
Before this manipulation an anterior cage is placed in the
resected area to maintain the length of the spine and to
avoid kinking of the dura and potential neurologic deficits
(Fig. 12-38, D). Sequential reduction of the distal aspect of
the rod to the remaining screws is performed, compression
is subsequently applied to achieve the final correction, and
the rib grafts initially harvested at the time of resection are
placed over the exposed dura (Fig. 12-38, E).
The choice of spine mobilization procedure is dependent
on several factors related to the spine deformity, including
 CHAPTER 12  Scoliosis 243

A B C

D E
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-
rience of the surgeon. The major risk is neurologic deficit,
which occurs more commonly with more aggressive release
procedures. The most recent review of the SRS database
demonstrated an overall 0.9% incidence of neurologic defi-
cits for all AIS surgery; however, the incidence increased
when a Smith-Petersen osteotomy was performed (1.1%)
and was greatest when VCR was performed (7.3%).621
Anterior Mobilization Techniques.  Anterior release includ-
ing discectomy, and possibly rib head resection when neces-
sary, can be performed through an open thoracotomy or
through a thoracoscopic approach. The thoracoscopic
approach has some advantage over the open thoracotomy
because smaller incisions are used with less chest wall dis-
ruption and less detrimental impact on pulmonary function,
especially when it is performed in the prone position with
double lung ventilation using a regular single-lumen endo-
tracheal tube (Video 12-1). The combined thoracoscopic
release in the prone position and posterior instrumented
fusion has resulted in overall excellent radiographic and
clinical results.
Correction Techniques
With introduction of the CD implant system, the 90-degree
concave rod rotation maneuver was used in an attempt to
improve the three-dimensional deformity. The rod was
shaped to the coronal plane deformity, and for the typical
right thoracic curve, the left rod was rotated counterclock-
wise so that the scoliotic bend in the rod became the
kyphotic bend in the rod to maintain or restore kyphosis.
Excellent coronal and sagittal correction could be
achieved with hook constructs; however, the axial plane
correction was somewhat limited. The introduction of
thoracic pedicle screws and the use of rods with various
characteristics of stiffness and strength, together with spine
mobilization techniques and more advanced instrumenta-
tion, provide an opportunity to better achieve axial plane
correction.
Rod rotation continues to be used as the initial step in
correction and is maximized with segmental fixation and
larger-diameter rods. Alternatively, the apical reduction
screws can be used to translate the apex of the spine pos-
teriorly and laterally with an overcontoured rod that is fixed
in its final position proximally and distally. This requires
significant releases for stiff spines to allow this translation
to occur. A stiff cobalt-chrome 6.25-mm rod is preferred
in general by the authors, especially for severely hypoky-
photic spines.
Following initial placement of the left rod, in situ coronal
bending can be performed together with compression on
the convex side and distraction on the concave side. At this
point, axial plane correction at the apex can be performed
with a variety of techniques to correct this component of
the deformity via DVR, which allows one to directly rotate
each spinal segment individually, or, with the use of connec-
tors for the DVR instruments, en bloc rotation can be
performed. A counterforce proximal and distal to the apex
should be imparted to most effectively improve the axial
plane deformity. The set screws of the apical segments
must be loosened on the concave rod to allow maximal axial
plane correction. The DVR technique is most effective
when only a single rod is in place to allow maximal correc-
tion. The axial plane correction maneuver can be performed
segmentally, with each screw head being manipulated indi-
vidually to correct the axial plane (Fig. 12-39, A and B), or
the screws can be linked together and rotated en bloc
(Fig. 12-39, C and D).14,513,744 Suk and colleagues reported
244 SECTION II  Anatomic Disorders
A B
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-
rection when the DVR technique was used together with
thoracoplasty than when thoracoplasty was used alone.746
Complications of Posterior Instrumentation and Fusion
for Adolescent Idiopathic Scoliosis. The primary complica-
tions associated with posterior surgery and instrumentation
include infection, pseudarthrosis, neurologic deficit, and
implant-related problems such as prominence, discomfort,
and implant failure associated with pseudarthrosis. The
overall incidence of reoperation after posterior instrumenta-
tion and fusion varies but is below 10% in more recent
series.30,116,242,465,617 A 1% to 10% incidence of delayed
wound infection has been reported, presumably related
either to the increased amount of hardware or to the mul-
tiple hook–rod connections.*c Some of these episodes of
delayed drainage were culture negative and attributed to
micromotion at the hook–rod interface.155,197,820 Micromo-
tion causes metallic debris, which leads to a foreign body
reaction, formation of a false membrane and fluid, and
finally loosening of the implant. Rather than representing
an aseptic process, it is more likely that these delayed infec-
tions result from low-virulence organisms that are seeded
at the time of surgery and remain quiescent over an extended
period.135,627,630,800
Risk factors for the development of a delayed infection
are the presence of a significant past medical history, receiv-
ing a blood transfusion, and not having a deep drain placed.313
The incidence of pseudarthrosis is very low with modern
double-rod systems, segmental instrumentation, and the
use of allograft bone.
*cReferences 135, 197, 627, 630, 640, 800, 820.
The most feared complication in spine deformity surgery
is neurologic deficit, whose incidence has remained steady
through the years and is still below 1%.138,472,621 Reames and
coauthors reported the most recent analysis of the SRS
database, which found a 0.8% incidence of neurologic deficit
at the time of surgery for AIS.621 The likelihood of complete
or partial recovery of neurologic deficits is high in all series.
The incidence of neurologic deficit is generally regarded to
be higher with combined anterior/posterior surgery and
when osteotomies are performed and is generally thought
to be of vascular origin.88,621
Anterior Spinal Instrumentation
Anterior Approach to Thoracolumbar and Lumbar Curves.
The anterior approach to thoracolumbar and lumbar curves
has been successful in correcting deformity and preventing
curve progression. However, its use for these curves has
diminished with greater use of pedicle screws and spine
mobilization procedures. Shufflebarger and associates
reported 80% correction of these curves with a posterior
approach that involved the use of segmental pedicle screws
at each level with wide Ponte-style osteotomies and convex
compression.698,699 Comparison studies between the poste-
rior and anterior approaches have demonstrated similar
levels of instrumentation with similar correction of defor-
mity; however, the posterior approach is associated with a
shorter operative time and hospital stay without the need
for a chest tube or a general surgeon to perform the surgical
approach.101,254 In general, we treat all thoracolumbar
and lumbar curves via a posterior approach except when
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
the spine.

A B
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-
tion extended the concepts of Zielke by using a stiff,
smooth, solid rod as the longitudinal connection between
vertebral screws (Fig. 12-40).343,345,781 The resulting stiffer,
fatigue-resistant construct enhances the maintenance of
correction and the likelihood of arthrodesis without post-
operative external immobilization in most cases. Deformity
is corrected by rotation of a 6.4-mm rod that is precon-
toured for lordosis (similar to the CD instrumentation
principles for thoracic curves posteriorly, but in reverse).
Because correction of deformity is achieved by gradual rod
rotation, corrective forces are evenly distributed all along
the construct simultaneously rather than applied acutely or
gradually at a single segment.
The early results of anterior single-rod treatment of tho-
racolumbar and lumbar curves included a high incidence of
pseudarthrosis, which improved with rib strut grafts.570,781
The addition of anterior interbody structural support in the
form of a titanium cage or femoral ring allograft significantly
increased the flexion–extension stiffness of the construct
when using single-rod implants,246,463,558 and it appears to
increase the likelihood of fusion. It is important to place
the structural interbody support in the most anterior aspect
of the intervertebral disk space to obtain optimal load
sharing602 and in the concavity of the curve to aid in coronal
plane correction. The concept of dual-rod constructs
for anterior scoliosis surgery was popularized by Kaneda
as a method of increasing the stiffness of the single-rod
construct while maintaining coronal and sagittal plane
correction and preventing pseudarthrosis.319,320,357 The
CHAPTER 12  Scoliosis 245
C
stiffness of dual-rod systems is better than that of single-rod
systems in flexion, extension, and torsion, with no increase
in lateral-bending stiffness.246,558,692,693 The addition of ante-
rior structural support to a dual-rod system increases overall
construct stiffness in flexion but not in other loading
conditions.246
The surgical technique for dual-rod systems is to initially
place two screws in each vertebra, with the posterior screw
traversing along the posterior cortex of the vertebral body
and the anterior screw directed in an anterior-to-posterior
direction to provide increased pullout and plow resistance
(see Plate 12-4, I-M on page 306.) A staple is similarly used
to capture both screws. A contoured rod with the desired
lordosis is placed into the posterior screws, and a rod rota-
tion maneuver is performed in a similar manner as a single-
rod technique to correct the scoliosis and restore or maintain
lordosis. Anterior structural support in the form of a tita-
nium mesh cage filled with the autologous rib graft har-
vested during the surgical approach is next placed to help
maintain lordosis and assist in achieving correction of scolio-
sis. The second rod is then placed and slight compression is
applied to lock in the anterior structural support. Kaneda
and associates reported their results with Kaneda’s dual-rod
system without anterior structural support in 25 patients
(20 with idiopathic scoliosis and 5 others) with thoracolum-
bar or lumbar curves; they demonstrated average coronal
correction of 83%, restoration of 9 degrees of lordosis
(7 degrees of preoperative kyphosis), and no cases of pseud-
arthrosis.357 Others have demonstrated excellent correction
and maintenance of correction with a dual-rod system.93,101
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.
 CHAPTER 12  Scoliosis 247

A B
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%.
Follow-up has demonstrated that the results of thoraco-
scopic AFSI are similar to those of open ASFI, with 55%
coronal plane correction and a pseudarthrosis rate of 6%.546
When compared directly with a posterior approach, thora-
coscopic ASFI is associated with similar coronal plane cor-
rection and less intraoperative blood loss but longer surgical
times (Fig. 12-42).448,844
Juvenile Idiopathic Scoliosis
The prevalence of juvenile idiopathic scoliosis (3 to 10 years
of age) is 8% to 12% in Europe and 13% to 16% in the
United States.338,649 The deformity is usually recognized
clinically by the age of 6 to 7 years.486 The female-to-male
ratio ranges from 1.6 : 1 to 4.4 : 1, with the difference
increasing with age.234,649 Convex right thoracic curve pat-
terns are most common, and relatively few patients have
thoracolumbar or lumbar curves.
Predicting Curve Progression
Juvenile scoliosis is more likely to progress, less likely to
respond to bracing, and more likely to require surgical treat-
ment than AIS is.490,828 Unlike infantile scoliosis, use of the
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
those whose curves will resolve usually show a decrease in
the RVAD. If the RVAD does not improve following bracing
of a progressive curve, spinal fusion will probably be required
as definitive treatment. The level of the most rotated ver-
tebra at the apex of the primary curve appears to be the
most useful factor in determining the prognosis of patients
with juvenile idiopathic scoliosis. Those with a curve apex
at T8, T9, or T10 have an 80% chance of requiring spinal
arthrodesis by 15 years of age.649 The predictive value of
two other factors once thought to be associated with a poor
prognosis—thoracic kyphosis of less than 20 degrees and
left-sided curves in boys—is uncertain.
Neural Axis Abnormalities
MRI studies have provided greater insight into juvenile idio-
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
than scoliosis) of an underlying neural axis abnormality. MRI
abnormalities include Chiari type I malformations with cer-
vical syrinx, thoracic syrinx, brainstem tumor, dural ectasia,
and low-lying conus. Many of these abnormalities may
benefit from neurosurgical treatment. As a result, some
authors recommend MRI during the initial evaluation of
patients presumed to have juvenile idiopathic scoliosis. If
scoliosis surgery is planned, it is imperative that preopera-
tive MRI evaluation be undertaken. Neurologic deficits
following spinal surgery have been reported in patients
with neural axis abnormalities that were not recognized
preoperatively.554
Treatment options for children with juvenile scoliosis are
outlined in the section “Early-Onset Scoliosis.” As the child
approaches 8 to 10 years of age, a definitive surgical proce-
dure should be considered (Fig. 12-43).
Congenital Spinal Deformities
Congenital deformities of the spine are caused by anomalies
in the growing vertebrae. These anomalies may be subtle
and found incidentally on radiographs obtained for some
other reason, or they may be complex and lead to severe
spinal deformity with accompanying neurologic deficits.
Congenital scoliosis, congenital kyphosis, and a combination
of the two are the deformities encountered. They are much
less common than idiopathic scoliosis.
248 SECTION II  Anatomic Disorders

A B C

D E F
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

Etiology
The cause of congenital vertebral anomalies remains
unknown. During embryologic development, these abnor-
malities develop in the spine between the fifth and eighth
weeks of gestation, but it is very uncommon to identify any
traumatic or teratologic type of maternal insult during this
stage of pregnancy.
Research has found that carbon monoxide exposure and
the resulting hypoxia can lead to reproducible congenital
spinal deformities in mice offspring.218,446 Such deformities
include wedged vertebrae, hemivertebrae, fused vertebrae,
and missing vertebrae, as well as fused ribs. The severity
of the deformities is related to both the dose of carbon
monoxide and the time during gestation that exposure
occurred. Correlating with this basic science study, the
same institution reported clinical data indicating a potential
monoxide) in the mothers of children with congenital spinal
deformities.445
Investigation of genetic causes has provided modest
insight. A positive family history can be found in approxi-
mately 1% of patients with congenital spinal deformities.823
Idiopathic scoliosis has been reported in 17% of families of
children with congenital scoliosis.613 An isolated anomaly,
such as a hemivertebra, usually occurs as a sporadic event
and carries no risk for a similar abnormality in other off-
spring.853 Studies of identical twins, only one of whom was
affected, showed no genetic pattern,296,596,604 but other
reports of twins with similar congenital deformities sug-
gested the possibility of genetic causes.237,517,731 Scientists
have identified the human gene HuP48, a member of the
Pax family of developmental control genes, as having a role
in establishing the segmented pattern of the vertebral
 CHAPTER 12  Scoliosis 249

A B C

E F

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

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

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.)
 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
  Hemivertebra
  Wedged vertebra
  Butterfly vertebra
  Defect
  Others
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
in preoperative planning for correction of these complex
deformities.
Defects of Formation
Defects of formation may be partial or complete. Partial
unilateral failure of formation produces a wedged or
trapezoid-shaped vertebra that contains two pedicles,
although one of them may be hypoplastic. The associated
scoliosis worsens slowly and may not require treatment.
True hemivertebrae are caused by complete failure of
formation on one side and result in laterally based wedges
consisting of half the vertebral body, a single pedicle, and a
hemilamina. Occasionally, the lamina associated with the
hemivertebra may be incorporated into that of the adjacent
normal-appearing vertebra. When this occurs, differentiat-
ing between the anterior vertebral abnormality and the
corresponding posterior abnormality becomes difficult.
Hemivertebrae in the thoracic spine are usually accompa-
nied by an extra rib. Hemivertebrae may be fully segmented
(most common), semisegmented, nonsegmented, or incar-
cerated (least common) (Fig. 12-46). Distinguishing among
these various types is important because the associated dif-
ferences in growth potential have a profound effect on the
eventual severity of the spinal deformity.
A fully segmented hemivertebra has the highest likeli-
hood of progressive deformity because it is separated from
the adjacent vertebrae by intact end-plates and interverte-
bral disks. The hemivertebra is nearly always located at the
apex of the scoliosis. Lower thoracic and thoracolumbar
curves tend to worsen more rapidly than do curves at other
levels. When two or more hemivertebrae are present on
the same side of the spine, the deformity progresses at a
faster rate. Conversely, the spinal deformity may be bal-
anced and nonprogressive if two hemivertebrae are situated
opposite each other. A fully segmented hemivertebra at the
FIGURE 12-46  Spinal radiograph of a 2-year-old girl with
53-degree congenital scoliosis and semisegmented hemivertebrae
at levels T8 and T10. A rib accompanies each of these
hemivertebrae. This deformity is partially balanced by a left-sided
hemivertebra at the T4 level. The deformity slowly progressed to
61 degrees by 9 years of age, at which time she underwent
fusion.
lumbosacral junction creates significant obliquity between
the spine and pelvis and is usually accompanied by a long
compensatory scoliosis in the lumbar or thoracolumbar
region. This readily apparent deformity is best treated surgi-
cally (usually by hemivertebrectomy) at an early age, before
the compensatory curve becomes fixed (Fig. 12-47).
A semisegmented hemivertebra is separated from one
adjacent vertebra (superior or inferior) by a normal verte-
bral growth plate and disk but is fused to the other adjacent
vertebra. Although growth of the spine should remain bal-
anced, the hemivertebra can induce a slowly progressive
scoliosis. Treatment is necessary only if the deformity is
progressive (see Fig. 12-46).
A nonsegmented hemivertebra is fused to both adjacent
vertebrae (above and below) and therefore has no vertebral
end-plates or adjacent disks. In the absence of any asym-
metric growth, a nonsegmented hemivertebra does not
cause progressive spinal deformity. An incarcerated hemi-
vertebra is more ovoid and smaller than a fully segmented
(nonincarcerated) hemivertebra. The vertebrae above and
below compensate for this hemivertebra, and as a result,
minimal if any scoliosis is present.
Defects of Segmentation
Defects of segmentation result in a bony bar or bridge
between two or more vertebrae, either unilaterally or
involving the entire segment. Circumferential, symmetric
252 SECTION II  Anatomic Disorders
FIGURE 12-47  Standing radiograph of an 18-month-old boy
showing significant obliquity of the pelvis because of a
hemivertebra at the L5 level. Postoperative findings are shown in
Figure 12-56.
FIGURE 12-48  Radiographic appearance of a 10-year-old girl with
52-degree scoliosis and block vertebrae at T5-6 and T9-10. The
deformity cannot be attributed to the mere presence of these
abnormalities. Findings on magnetic resonance imaging of the
spinal canal were normal.
failure of segmentation leads to a block vertebra (Fig.
12-48). This does not cause any angular or rotational spinal
deformity but does lead to some loss of longitudinal growth.
Klippel-Feil syndrome in the cervical spine represents a
severe form of this failure of segmentation.
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
early age because of a combination of shoulder imbalance,
severe distortion of the rib cage, decompensation of the
trunk, and pelvic obliquity that produces an apparent leg
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
abnormalities.712,762,766
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
spurt.
Curve progression is certain in patients with a unilateral
unsegmented bar and a contralateral hemivertebra (one
or more).79,499,500,821,824 Thoracolumbar curves of this type
have the worst prognosis and deteriorate 7 degrees per year
before the age of 10 years; this increases to 14 degrees
per year during the adolescent growth spurt. Severe
spinal deformity occurs unless surgical intervention is
undertaken.
Severe deformities are also caused by (in order of
decreasing severity) isolated unilateral unsegmented bars,
 CHAPTER 12  Scoliosis 253

A B
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.

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

A B
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,
notable pelvic obliquity and apparent lower limb length
inequality are unsightly compensatory mechanisms used to
keep the trunk vertical.
Radiographic Findings
Radiographic details of the vertebral abnormalities are best
seen on films obtained before the development of significant
deformity—often during infancy—on a radiograph taken
while the child is supine. As the child grows and the con-
genital scoliosis progressively worsens, the bony detail
becomes less clear. At initial evaluation, coned-down radio-
graphs of the affected area provide the most information
about the vertebral anomalies. Associated abnormalities
that may also be noted on plain radiographs include diaste-
matomyelia (midline bone spur), spina bifida occulta, and
congenital rib fusions on the concavity of the curve.
Although early supine radiographs reveal bony detail,
they cannot be used to assess curve progression. The initial
upright radiograph must serve as the baseline study against
which further curve progression is measured. The variability
in measuring angles in congenital scoliosis is reportedly
larger than that in idiopathic scoliosis because of skeletal
immaturity, incomplete ossification, and anomalous devel-
opment of the end vertebrae.215,447 Concerted effort should
be made to measure the curves with similar end points to
detect subtle yet steady progression of the curvature and
assess secondary or compensatory curves. The most recent
radiographs should be carefully compared with one of the
earliest upright radiographs to ascertain whether slow but
steady progression has occurred. It is not uncommon for
radiographs taken 4 to 6 months previously to reveal only
slight progression when compared with current radiographs.
If comparisons are made with radiographs obtained several
years earlier, the changes become more evident. Consistent
measurement of the secondary curve may also reveal its
progression and indicate the need for treatment.
In severe congenital scoliosis, plain radiographs may not
provide sufficient detail of the vertebral abnormalities.
Should surgical intervention be necessary, CT with three-
dimensional reconstruction is helpful for preoperative plan-
ning, particularly in visualizing posterior vertebral anomalies
associated with hemivertebrae.103,300,367,505,535,545
MRI of the spine should be performed in all patients
with congenital scoliosis who are undergoing surgical inter-
vention and should also be strongly considered during the
initial evaluation because up to 37% of patients have an
intraspinal abnormality (Fig. 12-52).47,500 MRI reconstruc-
tion can provide a clear picture of the canal contents despite
the severe three-dimensional deformity associated with
some cases of congenital scoliosis.
Nonoperative Treatment
Bracing is much less successful in the treatment of congeni-
tal scoliotic deformities than it is for idiopathic scoliosis.
Use of a brace to control curve progression secondary to
unsegmented bars or hemivertebrae is universally unsuc-
cessful and should not be attempted. However, bracing can
be considered as a means of temporarily controlling a long,
flexible compensatory curve below the congenital compo-
nent and thereby allowing further spinal growth before
operative intervention. If the congenital or compensatory
component worsens during the period of brace wear, opera-
tive intervention should be undertaken without delay. In
general, few patients with congenital scoliosis benefit from
the use of an orthosis.
 CHAPTER 12  Scoliosis 255

A B C D

E F G H
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
The primary goal of surgery is to stop progression of the
congenital spinal deformity. If partial correction can be
achieved safely, this is an added benefit.
Fusionless techniques in the form of growing rods and
expansion thoracoplasty are presented in detail in the
section “Early-Onset Scoliosis.” Many patients with con-
genital scoliosis are very young, have severe thoracic defor-
mities, and are in need of greater trunk height and thoracic
volume to survive into adulthood (Fig. 12-53). Fusion of the
spine in these young individuals must be avoided until the
age of 8 to 10 years.
For older children with congenital scoliosis, even with
relatively small curves (<40 degrees), once progression has
been confirmed, surgical intervention should be undertaken
if a significant amount of growth remains. This concept
must be emphasized: surgery should be performed before a
major deformity develops (Fig. 12-54). Children and ado-
lescents who have undergone spinal fusion should be moni-
tored to maturity because a progressive deformity can
develop above or below the fused sites. Additional surgery
may be required in these individuals.
Various operative approaches can be used; the choice
depends on the maturity of the patient, the location of the
deformity, and the type of congenital deformity. Approaches
include anterior and posterior spinal fusion, posterior fusion
with or without instrumentation, hemivertebra excision,
and spinal osteotomies.
Anterior and Posterior Fusion
This approach is used in immature individuals in whom
continued anterior growth on the convexity would lead to
development of the crankshaft phenomenon.196,303,761 Chil-
dren most in need of this approach are those who have
unilateral unsegmented bars with (or sometimes without)
contralateral hemivertebrae (see Fig. 12-54). In a young
child, fusion should extend to one level above and one level
below the anomalous vertebrae; this may prevent “adding
on” of the curve in subsequent years. Postoperatively, a cast
is needed for 4 to 6 months until healing has been achieved.
256 SECTION II  Anatomic Disorders
FIGURE 12-53  Hybrid rib-to-spine construct intended to expand
the left thorax for congenital spine/rib deformity.
Posterior Spinal Fusion
Posterior spinal fusion is indicated in older children with
progressive congenital scoliosis in whom the crankshaft
phenomenon is unlikely to develop or in younger children
who do not have normal anterior growth potential. Unlike
children with idiopathic scoliosis, crankshaft progression
does not develop in many young children with congenital
scoliosis because the anterior growth plates are abnor-
mal.371,457,830,836,838 These patients are not always easily iden-
tified preoperatively, so the decision whether to include
anterior fusion is difficult. The use of reduced-size spinal
instrumentation in young patients is safe and efficacious.301
With instrumentation, curve correction, length of immobi-
lization, and the fusion rate are improved in comparison to
similar patients treated without instrumentation.
If any correction of the deformity with instrumentation
is anticipated, preoperative MRI of the neural canal is essen-
tial to rule out diastematomyelia, syrinx, tumor, and other
abnormalities. If any of these lesions are identified, they
need to be addressed neurosurgically before the spinal
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
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
of the pedicle. The patient is next repositioned and the
posterior elements are excised through a secondary midline
approach. Correction is then achieved internally with pos-
terior compression instrumentation on the convexity or
externally with a cast during the postoperative period.
Immobilization in a cast is needed for 4 to 6 months until
fusion has been achieved (Fig. 12-56). A number of studies
have reported successful hemivertebra excision via a poste-
rior approach alone.337,536,655,656,695,861 This method, com-
bined with transpedicular instrumentation, is safe and
provides excellent correction in both the frontal and sagittal
planes. Its use should be reserved for experienced spinal
deformity surgeons.
Osteotomy of the Spine
VCR is reserved for older children with rigid, severe, angular
scoliosis and significant spinal decompensation.33,426,428,745
Anterior and posterior wedge resection osteotomies are
‡c
References 81, 107, 173, 318, 323, 385, 387, 536, 655, 656, 695.
 CHAPTER 12  Scoliosis 257

A B C
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.

A B
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).
258 SECTION II  Anatomic Disorders

A B C
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.

A B C
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.

performed, and the spine is then instrumented for correc-
tion (Fig. 12-57).239,318 If this procedure is undertaken in
the thoracic spine, costotransversectomy and resection of
the ribs are required.289 These operations should be per-
formed only by very experienced spinal surgeons because
the risk for neurologic complications is high.
Congenital Kyphosis
Congenital kyphosis represents an abrupt posterior angula-
tion of the spine because of a localized congenital malforma-
tion of one or more vertebrae.194,277,821,831 Although this
condition is less common than congenital scoliosis, paraple-
gia is a far greater risk in those with congenital kyphosis.
 CHAPTER 12  Scoliosis 259

with kyphosis resulting from failure of segmentation. These

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

A B C D
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.
260 SECTION II  Anatomic Disorders
greater deformity. In general, the posterior elements
(spinous processes, pedicles, transverse processes) are
present and accompany the deficient vertebral body. Growth
continues normally in the posterior portion of the spine, but
not anteriorly. As a result, relentless progression of the
deformity usually occurs.
Defects of formation place the patient at a much greater
risk for the development of paraplegia than do defects of
segmentation. The kyphotic junction may be unstable, par-
ticularly when the apex is between T4 and T9. Paraplegia
can occur at any age but is most common during the ado-
lescent growth spurt. Reports of acute-onset paraplegia fol-
lowing minimal trauma in young children testify to their
fragile neurologic status.
Defects of Segmentation (Type II)
In kyphosis caused by failure of segmentation, the
anterior portions of two or more adjacent vertebral
bodies are fused. This deformity tends to be less progres-
sive, produces less deformity, and is associated with a
much lower risk for paraplegia than is kyphosis caused
by defects in formation.492 The area most commonly
affected is the lower thoracic or thoracolumbar spine
(Fig. 12-60).
Natural History
The apical area of the kyphosis can occur at any level but
is most commonly located between the tenth thoracic and
first lumbar levels.501 There appears to be no relationship
between the severity of the kyphosis and its location in the
spine. Progression of these deformities is most rapid during
the adolescent growth spurt.
Congenital kyphosis from either failure of formation
(type I) or mixed anomalies (type III) tends to be
A B
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.
relentlessly progressive.487,501,597,837 Deformities caused by
two adjacent type I vertebral anomalies progress more
rapidly and with more severity than do deformities caused
by a similar single anomaly. Kyphosis from failure of seg-
mentation (type II) is much less progressive, produces less
severe deformity, and has a very low likelihood of resulting
in paraplegia.
Clinical Features
Although congenital kyphosis has been diagnosed prenatally,
it may not be clinically evident in a newborn or infant.717
Suspicion may first be raised after a chest radiograph is
obtained for evaluation of an unrelated event, such as a
respiratory infection. As the child begins standing and
walking, a localized prominence may become noticeable or
palpable. The child is usually asymptomatic and has no
spinal tenderness. In adolescents, the predominant clinical
complaint tends to be lower back discomfort caused by
secondary lumbar hyperlordosis. Mild scoliosis may accom-
pany the kyphosis.
On occasion, myelopathy or paraplegia secondary to
spinal cord compression may develop in a child with con-
genital kyphosis.374 Reports of mild trauma producing a
sudden onset of paraplegia in children who have unrecog-
nized, acute type I kyphosis highlight the delicate underly-
ing neurologic status in this condition. When congenital
kyphosis caused by a defect in vertebral formation is diag-
nosed, a meticulous neurologic examination should be per-
formed to identify any subtle abnormalities. Plans for
surgical intervention should begin immediately.
Radiographic Findings
Congenital kyphosis is best visualized on a lateral radiograph
of the spine. It may not be evident on the frontal view. Once
C
 CHAPTER 12  Scoliosis 261

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

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

A B C

Neurovascular
Rib bundle
graft Neurovascular
bundle

Rib graft
in place

D E
F
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.

G H
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.
risk for progressive neurologic deterioration. Very close mon-
itoring is needed. If recovery occurs, spinal fusion can be
performed without the need for decompression.382 If the
deficits do not resolve, arthrodesis must be combined with
anterior decompression of the spinal cord. Unless the child
is very small, these procedures can be accomplished during
the same operative episode. The decompression must be
performed anterior to the compressed cord by removing the
posterior aspect of the vertebral body. Posterior laminec-
tomy does not relieve the spinal cord compression. VCR
through a costotransversectomy is an effective approach for
these complex kyphotic deformities of the thoracic spine.
It should be undertaken only by those experienced with this
technique.
Type II Kyphosis
Defects of segmentation are best treated at a young age,
before significant deformity has developed. Posterior spinal
fusion followed by cast immobilization is sufficient treat-
ment. The fusion should span the unsegmented levels plus
one level farther both cephalad and caudad. Correction of
the kyphosis should not be expected, although mild
improvement from the cephalic and caudal extensions is
possible.378 Posterior compression instrumentation may
lessen the need for external immobilization.
In an older child with severe kyphosis, some correction
of the deformity may be achieved through osteotomy of the
unsegmented anterior region.713 When combined with pos-
terior compression instrumentation, this approach may
result in some improvement in the sagittal plane.
Type III Kyphosis
Mixed anomalies are least common but usually produce a
kyphoscoliotic deformity. Because of their association with
failure of segmentation, type III anomalies generally require
posterior arthrodesis only.
CHAPTER 12  Scoliosis 263
Segmental Spinal Dysgenesis, Congenital
Vertebral Displacement, and Congenital
Dislocation of the Spine
Segmental spinal dysgenesis, congenital vertebral displace-
ment, and congenital dislocation of the spine are similar
conditions. In fact, they may be variations of the same
deformity, although this is not universally accepted.176 They
all create severe localized kyphosis of the spine and lead to
a neurologic deficit in 50% to 60% of patients.216,243,327,685,874
Segmental spinal dysgenesis is characterized by a focal
spinal deformity, usually located at the thoracolumbar junc-
tion or in the upper lumbar spine.216,243,244,327,772,872 The
deformity frequently includes severe kyphosis; anterior,
posterior, or lateral subluxation of the spine; scoliosis in
association with a severely stenotic spinal canal; and absent
nerve roots. All these patients have localized stenosis of the
spinal canal at the level of involvement, and the osseous
canal has an hourglass shape. No pedicles, spinous pro-
cesses, or transverse processes are seen at the level of
involvement. Commonly, an offset in the sagittal plane is
present between the cephalic and caudal segments of the
spine at the level of dysgenesis. Decompression of the ste-
notic canal results in some improvement in neurologic func-
tion in 20% of patients. Early anterior and posterior
arthrodesis in patients with segmental spinal dysgenesis is
indicated because progressive kyphosis inevitably develops
and often results in neurologic deficits.
Type I congenital kyphosis can be similar to and may be
confused with segmental spinal dysgenesis. Type I congeni-
tal kyphosis represents failure of formation of the vertebral
body; however, the pedicles and posterior elements are
present. The severe spinal stenosis associated with segmen-
tal spinal dysgenesis is not present in congenital kyphosis.
Although many patients with segmental spinal dysgenesis
have fixed neurologic deficits, neurologic function in patients
with congenital kyphosis is generally good at birth, with
264 SECTION II  Anatomic Disorders

A B C

D E
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
instability and worsening kyphotic deformity.
Congenital vertebral displacement occurs when the
spinal column is displaced at a single vertebral level and
results in abrupt displacement of the neural canal (Fig.
12-64).685,776,799,873 The displacement can occur in the pres-
ence of a posteriorly located hemivertebra in which the
pedicles, transverse processes, and spinous processes may
be present. As with segmental spinal dysgenesis, the poten-
tial for severe neurologic deficits is high. Combined anterior
and posterior arthrodesis of the spine is needed in an effort
to prevent the development of such deficits. For those with
neurologic deficits of recent onset or progressive neurologic
deficits, decompression of the spinal cord is indicated.
The congenitally dislocated spine was first described in
1973 by Dubousset.874 It, too, is associated with spinal
 CHAPTER 12  Scoliosis 265

C
A B

E
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.
The posterior elements are abnormal in all patients with
congenital dislocation of the spine. The various stages of
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
otherwise normal skin. Anterior and posterior spinal fusion
is indicated because posterior fusion alone is insufficient to
achieve solid fusion with this type of congenital instability.
Exploration and augmentation of the posterior fusion mass
should be considered because of the high rate of pseudar-
throsis with this abnormality. No sudden extemporary cor-
rection should be attempted in older patients with severe
angular kyphosis and progressive neurologic deficits. Func-
tion must be favored over cosmetic appearance. Neurosurgi-
cal decompression should be used only for a proven recent
and progressive neurologic deficit.
For these three entities, all of which involve a severe
form of localized kyphosis, early recognition is imperative,
and the appropriate operative intervention should be under-
taken. Prenatal diagnosis is possible and can be useful for
parental counseling and obstetric management.245,664
Early-Onset Scoliosis
An increasing body of evidence suggests that patients in
whom scoliosis develops at an early age, regardless of the
cause, are at significant risk for thoracic insufficiency syn-
drome,111 defined as an inability of the thorax to support
normal respiration and lung growth. The increased respira-
tory morbidity and mortality now recognized in patients
with early-onset scoliosis make a compelling case for clas-
sifying such patients as a separate group in which the
emphasis is not necessarily on treating the spine itself but
rather on maintaining growth of the thorax to promote
increased lung volume throughout the critical first decade
of life.
266 SECTION II  Anatomic Disorders
Effect on Respiratory Function
Respiratory failure secondary to untreated scoliosis before
5 to 8 years of age has been documented for at least 2
decades,85,271,702 with double the mortality rate as in the
general population.271 Swedish patients with infantile (0 to
3 years) and juvenile (4 to 9 years) onset were found to
have a significant increase in observed mortality in compari-
son to the general population; in contrast, patients with
adolescent (older than 10 years) onset had the same mortal-
ity rate as the general population.587 Not surprisingly, the
magnitude of the scoliosis also plays a role in the demise of
untreated patients: curves greater than 70 degrees result in
a higher mortality rate than do smaller curves.587 In a Scot-
tish study of children with infantile-onset idiopathic or con-
genital curves, a decrease in vital capacity was directly
A B
D E
correlated with increasing deformity, whereas in adolescent-
onset deformity, no effect on vital capacity was noted with
an increasing Cobb angle.530 In general, the combination of
onset before 7 years or a Cobb magnitude of 100 degrees
and associated rib anomalies or muscle weakness can
produce respiratory failure as early as the third decade
(Fig. 12-65).702
The source of the respiratory failure is twofold: intrinsic
alveolar hypoplasia and extrinsic disturbance of chest wall
function.
Intrinsic Alveolar Hypoplasia
Limited autopsy material has shown that failure of alveolar
multiplication is probably a major source of respiratory
demise with early-onset scoliosis because the chest defor-
mity prevents hyperplasia of the lung tissue.56,77,163,623
C
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
ribs in the left hemithorax. The patient died
of respiratory failure.

Indeed, the alveoli that are present appear emphysematous,
as though attempting compensatory hypertrophy; however,
actual compression of the alveoli, which might be predicted
by the chest wall deformity, is not observed. In a normal
lung, alveolar hyperplasia continues until about 8 years of
age, with hypertrophy possible until growth of the thorax
is complete.623 Other investigators have found that the adult
number of alveoli is attained even earlier, by the age of 2
years. Subsequent expansion of lung volume up to 5 years
of age occurs through the rapid increase in peripheral
airway conductance that accompanies airway enlarge-
ment.167,312,315,768,875 Most authors agree that lung “growth”
(hyperplasia and airway expansion) is essentially complete
by the age of 8 years, with the “golden period” of maximal
growth occurring before 5 years. Deformities developing
after this age have less effect on lung growth.85,586 In this
period of alveolar hyperplasia, rapid growth of the bony
thorax also occurs. The length of the thoracic spine increases
by 50% (from 12 to 18 cm) from birth to 5 years of age,182
with some 60% of the adult length being achieved by this
age (Fig. 12-66). The circumference of the thorax, which
is only 7% of adult size at birth, increases to 30% by 5 years
of age and to 50% by 10 years.182 The “golden period” of
rapid growth of the thoracic spine and rib cage thus coin-
cides with lung development.
T1
T1
L1
16 cm
L1
7 cm
L5
L5
A B
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.
CHAPTER 12  Scoliosis 267
Extrinsic Disturbance of Chest Wall Function
Extrinsic disturbance of respiratory function is the result of
rib or associated chest wall deformities producing loss of
compliance or functional incompetence of the chest
wall.58,109 The latter is most severe in patients with rib
fusion, in whom normal chest wall movements are inhib-
ited, or in those with rib absence, in whom a localized “flail”
or paradoxical segment of the chest wall is present. The
thorax is thus unable to change volume effectively because
of chest wall dysfunction. Patients with congenital scoliosis
have decreased vital capacity in comparison to those with
idiopathic scoliosis and the same magnitude of Cobb angle,
presumably as a result of concomitant rib anomalies produc-
ing additional chest wall dysfunction.572 Congenital dia-
phragmatic hernia, which produces ineffective volume
expansion because of diaphragm dysfunction, may be
another example of extrinsic dysfunction. In noncongenital
deformities, the rib deformity secondary to the scoliosis
produces inefficient respiration. The intercostal spaces on
the concave hemithorax are narrowed and unable to expand;
thus, end-inspiratory volume is restricted (see Fig. 12-65).
Meanwhile, the convex hemithorax has widened intercostal
spaces that cannot generate normal expiratory function.349
Increasing rotational deformity of the ribs accompanying
T1
26 cm
T1
T12
12 cm
T12
S1
S1
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
80
and noncompliant and thereby lead to the restrictive lung 70
disease typical of patients with severe scoliosis. 60

% FVC
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
20
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
80
fused.209,361 Thus, fusion itself is a cause of respiratory insuf-

ficiency and adds to the spinal deformity with loss of pul-
monary function.
Poor respiratory outcomes have been reported in patients
with infantile (noncongenital) scoliosis operated on at a
mean age of 4 years.267 When tested at a mean age of 20
years, forced expiratory volume in 1 second and forced vital
capacity averaged around 41% of predicted values, with
individual patients testing as low as 12% to 14%. Because
vital capacity less than 43% of the predicted normal value
is considered a risk factor for respiratory failure,586 these
patients have a guarded prognosis for long-term pulmonary
function. In contrast, patients whose surgery could be
delayed by nonoperative means until 10 years or older
(mean, 12.9 years) had mean pulmonary function around
70% of predicted values, with the worst patients having 45%
of the predicted value and the best patients being normal
(100%). A disturbing finding was that in the early fusion
group, scoliosis was not controlled by surgery: the mean
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
degrees to 63 degrees at follow-up. Although the ineffective
scoliosis management clearly contributed to the poor pul-
monary outcomes in the early-fusion patients, consistent
with Pehrsson and co-workers’ data587 on greater mortality
in those with curves larger than 70 degrees, the benefit of
controlling the scoliosis through nonoperative means and
delaying surgery until after age 10 is incontrovertible.
Additional pulmonary outcome data on patients with
congenital scoliosis have confirmed the detrimental effect
on pulmonary function when patients younger than 5 years
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
damaging if the fusions must include T1 or T2.361 Day and
associates demonstrated that patients with congenital sco-
liosis treated nonsurgically (mean age, 11.7 years) had
nearly normal pulmonary function despite multiple thoracic
vertebral anomalies whereas surgically treated patients with
similar anomalies but larger curve magnitudes (mean age,
16 years; 7.6 years after surgery) had only 45% to 65% of
predicted volume.165 In addition, these same surgically
treated patients with multiple anomalies had significantly
worse pulmonary function test results than did surgically
treated patients with two or fewer anomalies. The growth
disturbance from more extensive anomalies and the surgery
to fuse them both contributed to a poor outcome.165
% Normal FVC
70
60
50
40
30
20
10
0
1 1 1 1 1 2 22 2 2 2 3 4 4 4 5 55 5 6 6 7 8 9 9 9
Proximal level of fusion
B
FIGURE 12-67  A, Forced vital capacity (FVC) versus percentage of
the thoracic spine fused. FVC may decrease to less than 50% of
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
proximal extent of fusion. Fusions extending to T1 or T2 before  
8 years of age are likely to produce an FVC of less than 50% of
predicted volume. (Redrawn from Karol LA, Johnston CE,
Mladenov K, et al: The effect of early thoracic fusion on
pulmonary function in non-neuromuscular scoliosis. 40th Annual
Meeting of the Scoliosis Research Society, 2005, Miami.)
It is important to reexamine our approach to young
children with large spinal deformities. The principle that a
short, straight spine produced by early fusion is better than
a long, curved spine is no longer generally accepted. It is
important to avoid fusion of the thoracic spine, especially
the proximal segments, before 5 years of age. The goal of
management must be to control the spinal deformity
without impeding thoracic growth. Other innovative
techniques, such as expansion thoracoplasty, offer the pos-
sibility of preventing thoracic insufficiency from spinal
deformity.110,207
Treatment
Nonoperative Delaying Tactics
Attempting to delay definitive spinal fusion until 10 years
of age by nonoperative means is usually the first approach
in a patient at risk for thoracic insufficiency.
Bracing
Bracing is a time-honored method of controlling noncon-
genital deformities if the patient has no coexisting neuro-
muscular or other medical conditions in which a brace
would adversely affect respiratory function by circumferen-
tial chest or abdominal compression (Fig. 12-68). Bracing
efficacy is difficult to define or prove by the standard
 CHAPTER 12  Scoliosis 269

A B

C D E
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
improved radiographically by well-constructed orthoses,
even in the smallest patients (Fig. 12-69), only to progress
with growth. Orthotic management can be considered suc-
cessful if progression is prevented for several years, thereby
delaying the need for fusion until the child is older. If fusion
can be delayed until 10 years of age, the need for anterior
fusion may be avoided.344
When brace management is used in a rapidly growing
child younger than 5 years, the patient must be carefully
evaluated for brace-induced rib deformity produced by
pressure on the soft infantile rib cage, as well as obliteration
of the normal sagittal contour (lumbar hypolordosis and
thoracic hypokyphosis). Iatrogenic rib and sagittal spine
deformities (Fig. 12-70) and volume restriction must not
add to the existing deformity.
270 SECTION II  Anatomic Disorders
A B
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.
FIGURE 12-70  This child’s chest and spinal deformities were
worsened by bracing. The right thoracic pad deformed the lower
ribs posteriorly (arrows) and probably added to the left lumbar
deformity by creating a posterolateral force at the right
thoracolumbar junction.
The Milwaukee brace, in some form, is often the orthosis
of choice in early-onset patients because of its ability to
apply corrective forces directly at the apex of the curve, at
the pelvis, and at the neck while minimizing the constrictive
aspects of a TLSO. Precise custom construction is essential
for success. In a small child, the rib pad can be applied
C
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.
Casting
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
young children.
Moreover, casting can be a more definitive method of
management rather than simply a delaying tactic. Mehta
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
at a mean of 19 months of age with an average curve of 32
degrees had their scoliosis reduced to less than 10 degrees
at maturity. Patients who started treatment at a mean of 30
months of age with larger curves averaging 52 degrees did
not gain significant correction, but their deformities had not
progressed (averaging 46 degrees) at follow-up. Her casting

protocol required serial cast changes under anesthesia every
2 to 3 months in children younger than 2 years, with a
minimum of five casts. The goal was to achieve a straight
spine, at which time the patient was switched to a brace.
Children older than 2 years required cast changes every 3
to 4 months. Older children demonstrating “recurrence”
were placed back in a cast for 4 months to recorrect the
deformity before continuing with brace management.
Mehta’s important contribution was to demonstrate that
serial casting in young children (even infants), if pursued
aggressively and for a long enough time, can correct the
deformity over a long follow-up period.
Our experience with serial casting has been less aggres-
sive and more variable. We apply the cast under general
anesthesia on a Risser table, with neck halter and pelvic
strap longitudinal traction and appropriate localized hand
pressure or straps applied directly to the rib hump (Fig.
12-71). Properly molded chin and neck pieces are generally
required to maintain the longitudinal corrective force once
the traction is removed. A large abdominal window is
A B
C D
CHAPTER 12  Scoliosis 271
removed for respiratory excursion, and other cast relief is
applied liberally in portions that do not involve spinal cor-
rective forces. A window to allow the spine to move into
the concavity is useful to gain further correction.504 Improve-
ment in the deformity can be dramatic in the short term
(see Fig. 12-71, C and D), but the apparent correction is
somewhat misleading because the radiograph is taken with
the patient supine (and is usually compared with a pretreat-
ment standing film). Nevertheless, with serial cast changes
performed every 2 to 3 months for 6 to 9 months (usually
totaling three casts), an apparently uncontrollable, rapidly
progressive deformity can be corrected sufficiently to return
to orthotic management and further delay the need for
surgery.
The superior mesenteric artery syndrome and neurologic
dysfunction, particularly affecting the cranial nerves (see
Fig. 12-71, E) and the brachial plexus if the neck portion is
applied with too much head or shoulder distraction, are the
main acute complications of applying corrective casts under
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.
272 SECTION II  Anatomic Disorders
prominence and other bony prominences are not uncom-
mon, especially in patients who are not cognitively normal.
For this reason, casting may be relatively contraindicated in
such patients.
It may be argued that a cast restricts pulmonary function
more than a brace does and may cause more rib deforma-
tions, but these drawbacks can be minimized with a well-
managed casting protocol. We find the casting alternative
to be useful in delaying surgery, as do many European
centers.504,667
Traction
Some patients with progressive deformities are not candi-
dates for casting, such as those with weakness, skin or chest
wall defects, intolerance, or mental retardation. Large, stiff
curves may not benefit from serial casting, and the cast may
be poorly tolerated. In these instances, halo–gravity traction
is an invaluable method to achieve correction of the defor-
mity and, indirectly, improve respiratory mechanics.705
The technique is not new, having been developed origi-
nally by Stagnara725 and later demonstrated to one of the
authors (CEZ) by Zielke on a visit to the latter’s clinic in
1984. A halo is first applied with six to eight pins and the
child under general anesthesia (Fig. 12-72).528,705 Experi-
ence has shown that the use of numerous pins actually
decreases the chance of pin infection or loosening of any
single pin. Pins are tightened to a torque equaling the age
of the child; for example, a 4-year-old patient’s pins are
tightened to 4 inch-pounds of torque with a calibrated
torque wrench. The following day the patient is placed
upright in overhead traction via a traction bale attached to
a wheelchair or a standing frame via a spring-loaded fish
scale or other dynamic traction device (see Fig. 12-72, D),
with an initial traction of 5 to 10 lb. The time in traction
and the amount of weight are increased to tolerance, with
careful neurologic monitoring. All patients need cranial
nerve testing once during each shift while upright in trac-
tion, and in patients with preexisting neurologic signs, lower
extremity strength and reflexes are monitored as well during
the phase of increasing traction. Eventually, a traction force
exceeding 50% of body weight may be achieved. The trac-
tion is increased so that the patient’s buttocks are lifted
slightly off the wheelchair seat while sitting; in the standing
frame, the patient should be up just on tiptoes (see Fig.
12-72, D and E). The safety of this method is ensured by
the patient’s ability to automatically relieve the traction by
pushing up on the wheelchair arms or walker hand rails in
response to pain or neurologic symptoms.
We have treated many children as outpatients once their
caretakers are educated on the use of traction and comfort-
able with its supervision. Only two neurologic complica-
tions have occurred in a series of nearly 100 patients. In 1
patient with Klippel-Feil syndrome and multiple cervical
synostoses, mouth and facial numbness developed when
C3-4 distraction occurred at the only nonfused level in the
neck (see Fig. 12-72, F). The numbness resolved after trac-
tion was discontinued and converted to a halo vest for 6
weeks. In another patient with incomplete resection of a
benign ganglioneuroma of the conus medullaris and pretrac-
tion hyperreflexia, weakness of multiple muscle groups in
one lower extremity developed and necessitated that trac-
tion be discontinued. Another report of 33 patients treated
by traction noted two possible neurologic complications:
nystagmus and dizziness.641
Long-term halo traction is especially useful for correcting
trunk shift, trunk height, and sagittal plane deformity. The
ability to mobilize patients with weakness, osteopenia, and
respiratory compromise during the traction period is invalu-
able in preparing such patients for surgery. This traction
protocol avoids enforced bed rest, which is required by
halo-femoral traction, and significant pulmonary benefit is
achieved with an upright thorax as these patients are readied
for surgery. Finally, because these children have diminutive,
often osteopenic spinal elements and more rigid deformi-
ties, acute correction of the deformity with instrumentation
is often fraught with the risk for bone–implant interface
failure and neurologic compromise. Thus, correction of the
spine, not to mention the length of the thorax, must
be done gradually with increasing traction (Fig. 12-73).
The definitive stabilization procedure using instrumentation
may then be essentially an in situ fixation to maintain the
position achieved by traction.
Operative Treatment
Instrumentation Without Fusion (“Growing Rods”)
(Video 12-5)
In an effort to treat uncontrollable progressive scoliosis in
young children, Moe and colleagues introduced the tech-
nique of subcutaneous Harrington instrumentation, first
reported to the SRS in 1978.515 With this technique the
spine is exposed subperiosteally only at the end vertebrae
in the hope of allowing continued growth of intercalary
segments after distraction between these end hooks. In
Moe’s original method, Harrington rods were modified so
that a short threaded segment was present at each end of
the rod with a long nonthreaded segment in between so that
the rods could be exchanged easily through subcutaneous
tunnels without soft tissue growth into the threads and be
contoured without damaging the threaded portion. Single
hooks were seated at the selected end vertebrae through
small, limited incisions, and then the modified rod was tun-
neled subcutaneously or submuscularly to engage the hooks.
Later, subfascial placement through a single long incision
was described to add stability to the construct and obtain
better skin coverage.386 The hooks were then reexposed
every 4 to 6 months or when 10 degrees of correction had
been lost, and the rod was lengthened by advancing a nut
on the threaded portion to redistract the hooks. Patients
were maintained in Milwaukee braces full-time. Eventually,
definitive fusion was performed to complete the treatment
protocol. Following the initial experience, standard non-
threaded Harrington rods were substituted to avoid rod
fracture, which occurred at the junction of the smooth and
threaded portions of Moe’s modified rods.511 Localized end
fusions were also added in an attempt to prevent hook
migration and dislodgement. Moe and co-workers reported
that in the early cases, the apex of the curve remained
unfused until the definitive fusion whereas the end verte-
brae uniformly fused spontaneously.515 Subsequent reports
showed that the intercalary segments ankylosed even though
they were never actually exposed.4 Decreasing effectiveness
of repeated distractions often resulted in little additional
correction of the stiffened spine.386,760
 CHAPTER 12  Scoliosis 273

A C

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

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

A B

Loosen and retighten

set screw Set screw

Vice grips
tight on rod

Rods

C D

Set screw Rod

Hook Rib
E F
276 SECTION II  Anatomic Disorders

A B

C D E
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
(see Figs. 12-68 and 12-75). This construct also allows
longer overlapping rod segments and greater eventual
lengthening.
Postoperative immobilization (bracing) can be used on
an ad hoc basis, depending on the strength and security of
the fixation, while the initial localized fusions are maturing.
In some patients with adequate bone stock, no postopera-
tive protection is necessary, and ambulatory patients are
allowed to resume normal nonvigorous activity, including
some sports. Current practice is to routinely lengthen the
construct every 6 months.86
The ultimate spinal instrumentation and fusion are per-
formed when little additional length is gained by rod

A B
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-
tions may occur that cause the surgeon to abandon the
fusionless method and perform an earlier fusion.
An alternative fusionless fixation in which a subcutane-
ous rodding technique is combined with an expansion tho-
racoplasty technique may be appropriate in certain patients
with chest wall dysfunction but without congenital rib
abnormalities and spinal deformities. In this setting, one
that is often associated with syndromic or neuromuscular
conditions, the patient requires correction of spinal defor-
mity and expansion of thoracic volume (see later), which is
constricted because of rib deformity related to the spinal
deformity and often trunk weakness or rigidity. Bilateral use
of spinal instrumentation or expandable rib prostheses in a
subcutaneous technique, with the cephalic anchors being rib
rather than spine attachments and the caudal anchors seated
on the iliac crests, can provide both thoracic expansion and
spinal correction without actual exposure of the spine at
any point (see Fig. 12-76).110 Thoracic expansion takes place
by distraction of the rib attachment on the upper part of
the thorax away from the spinal or pelvic attachment
without any formal rib resection or chest wall mobilization
surgery. The results of this hybrid technique (rib to pelvis
or rib to lumbosacral spine) are still anecdotal and prelimi-
nary,714 but it is a promising alternative for patients with
potential or actual thoracic insufficiency associated with a
collapsing noncongenital spinal deformity.
Expansion Thoracoplasty
In 1987, faced with what appeared to be untreatable scolio-
sis because of a congenital flail chest in a 6-month-old infant
who was respirator dependent and could not be weaned,
Campbell and associates109 implanted a “chest wall prosthe-
sis” made of vertically oriented Steinmann pins wired to the
vestigial ribs. Not only was the child subsequently weaned
from the respirator, but the scoliosis was significantly
improved by the rib distraction produced and maintained
CHAPTER 12  Scoliosis 277
C
by the pins. To deal with the new problem of how to con-
tinue treatment as growth inevitably occurred, an expand-
able rib prosthesis had to be developed. Thus, the concept
of expansion thoracoplasty by rib distraction was born, and
this led to the development of a unique prosthetic rib
implant—the vertically expandable prosthetic titanium
rib (VEPTR) (Fig. 12-77, A). The VEPTR can be length-
ened periodically to produce an opening wedge correction
of the scoliosis from the concavity while providing chest
wall stability for flail segments and volume expansion of
the hypoplastic thorax in a patient with, for example, fused
ribs (Fig. 12-77, B and C). Concomitant growth of the spine
has been documented, even in patients with congenital
unsegmented bars in whom it was assumed that growth of
the concavity was impossible as a result of the congenital
fusion.109
The expansion thoracoplasty technique has revolution-
ized the treatment of young children with congenital scolio-
sis and chest wall abnormalities. Previously, treatment of
these children emphasized stopping the progressive defor-
mity at all cost by early spinal fusion. As discussed earlier,
the pulmonary cost of this early fusion has been well docu-
mented, with follow-up studies finding that many patients
suffered from thoracic insufficiency because of lack of
growth of the thorax (see Fig. 12-65).109,165,209,267,361
The ability to lengthen the thoracic spine and simultane-
ously correct the scoliosis without performing surgery on
the spine itself has been the major contribution of the
expansion thoracoplasty technique (Fig. 12-78). Other
radiographic parameters of growth, such as the sagittal
depth of the thorax (distance from the anterior body of T6
to the sternum; Fig. 12-79) and the space available for the
lung ratio (relative height of the concave and convex hemi-
thoraces),109 can also be corrected significantly.
Pulmonary outcomes were somewhat equivocal in
Campbell and colleagues’ report,112 primarily because of an
inability to test young patients preoperatively and thus have
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.)
B C
a baseline for comparison. However, patients operated on
before 2 years of age had a significantly higher predicted
vital capacity at follow-up (58%) than did those older than
2 years at the initial operation (44% of predicted), thus
attesting to the importance of early treatment during the
“golden period” of alveolar growth.168 By using volumes
obtained from CT scans of the thorax (Fig. 12-80), Emans
and colleagues were able to clearly demonstrate an increased
anatomic volume of the thorax, especially the hemithorax
on the side where the VEPTR was placed.207 The increase
in CT-derived total lung volume was 147% from preopera-
tive values to the last follow-up, and the lung on the VEPTR
side had an increase in volume of 219%. Unfortunately, the
correlation between such increases in anatomic volume and
concomitant physiologic function tests (e.g., forced vital
capacity, forced expiratory volume in 1 second) is unknown
at this time, thus making the interpretation of anatomic
changes in volume uncertain.
Despite an average of 1.3 to 1.8 procedures per year per
patient, complication rates of expansion thoracoplasty—
D
primarily infection, skin sloughing, and device migration
or dislodgement—are comparable to those seen with
single growing rod techniques. These complications can be
managed satisfactorily with standard surgical wound care
and repositioning of the device or revision of the construct.
Brachial plexus injury from extreme cephalic placement of
the proximal rib cradle (first or second rib), combined with
traction on the scapula during wound closure to cover the
implants, is a unique complication of expansion thoraco-
plasty. Upper extremity neurologic and vascular monitoring
is required to avoid this complication. In the longer term,
the effects of chest wall stiffening, mentioned anecdotally
as a consequence of serial device lengthening,207 is of suf-
ficient concern that most surgeons are reluctant to use a
chest wall expansion technique in patients who do not have
primary chest wall anomalies.
The technique of expansion thoracoplasty and VEPTR
implantation has been widely publicized,110,112,207 but
indications for its use are frequently subjective. It is difficult
to predict with objectivity the likelihood of thoracic
 CHAPTER 12  Scoliosis 279

A B

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

T1

T6 T6-sternum
Thoracic width Thoracic depth

T1-12
Thoracic length

T12

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

A B C
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
preserve alveolar function may start to close by 2 years of
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
(see Fig. 12-80),207 but its prognostic accuracy remains
unknown.269
Design and execution of a prospective study of anatomic
volume outcomes of treated and control patients and cor-
relation of those outcomes with physiologic pulmonary
function remain the greatest challenges to the development
of objective indications for the rational use of expansion
thoracoplasty and, indeed, any of the treatments of early-
onset scoliosis discussed in this chapter.
Summary
Several treatment regimens are appropriate and still under
investigation for these difficult disorders. Serial casting and
bracing are appropriate approaches for children without
major chest wall deformities in an effort to delay spinal
fusion until 10 years of age. Larger and stiffer curves may
respond to halo traction programs, followed by either a
return to bracing or growing rod instrumentation. Growing
rod instrumentation is recommended for patients who have

A B
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-
muscular conditions or iatrogenic rib deformities. Expan-
sion thoracoplasty is reserved for those with spinal
deformities complicated by chest wall deformities that limit
pulmonary function.
Other Causes of Scoliosis
Neurofibromatosis
Scoliosis is the most common skeletal manifestation of neu-
rofibromatosis (Fig. 12-81).151,152,377,778 Typically, it is located
in the thoracic spine; has a short, sharply angled curve; and
involves four to six vertebrae. The reported incidence is
between 10% and 60%.§c Reports citing high incidence rates
may have been biased, however, in that they were derived
from populations of patients with neurofibromatosis
managed by musculoskeletal specialists. A 10% to 20% inci-
dence of scoliosis appears to more accurately reflect the
entire population with neurofibromatosis.12
The cause of spinal deformity in patients with neurofi-
bromatosis is unknown. Proposed explanations include
primary mesodermal dysplasia, osteomalacia, erosion or
infiltration of bone by localized neurofibromatosis tumors,
and endocrine disturbances.152
Scoliosis secondary to neurofibromatosis can be either
nondystrophic or dystrophic, depending on accompanying
abnormalities specific to this disorder.‖c Differentiation
between the two types is important because the prognosis
§c
References 12, 117, 152, 187, 228, 317, 341, 706.
‖c
References 108, 152, 248, 377, 403, 560, 706, 815.
CHAPTER 12  Scoliosis 281
C
and management differ significantly. Dystrophic scoliosis is
more common, has a greater tendency to progress, and
includes a subgroup of patients (those with severe kypho-
scoliosis) at risk for neurologic deficits.706 Nondystrophic
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
years.152
Nondystrophic Scoliosis
The nondystrophic type of scoliosis has a more favorable
outlook in patients with neurofibromatosis. The clinical
appearance, radiographic findings, and behavior of the curve
tend to be similar to what is found with idiopathic scoliosis.
However, nondystrophic deformities usually become appar-
ent at an earlier age than idiopathic curves do and have a
slightly higher likelihood of progressive deformity. Manage-
ment of nondystrophic curves is similar to that for idiopathic
scoliosis. Curves less than 25 degrees can be observed closely
without active intervention. Brace treatment appears to be
effective for skeletally immature individuals with curves
between 25 and 40 degrees.377 However, once the nondys-
trophic curves of neurofibromatosis exceed 40 degrees, pos-
terior spinal fusion with instrumentation is recommended
(Fig. 12-82). Close follow-up after surgery is needed because
of the higher likelihood of pseudarthrosis in this population,
and over time, some nondystrophic curves evolve and
exhibit characteristics of dystrophic scoliosis.
Dystrophic Scoliosis
In dystrophic scoliosis, short, sharply angled curves develop
at an early age, often as young as 3 years. Radiographic
282 SECTION II  Anatomic Disorders

A B C

D E F G
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-
phic curves include vertebral scalloping, spindled transverse
processes, severe apical vertebral wedging and rotation,
foraminal enlargement, defective pedicles, penciling (nar-
rowing of the proximal portion) of the ribs, the presence of
paravertebral soft tissue lesions, and rarely, subluxation
between vertebral bodies. Some of these findings may result
from direct erosion of the bone by intraspinal neurofibro-
mas, paraspinal neurofibromas, or dural ectasia. Dural
ectasia is an expansion in the width of the thecal sac thought
to be due to an increase in hydrostatic pressure.
Fortunately, most dystrophic curves are not accompanied
by an excessive amount of kyphosis.706 Individuals with this
combination have significant potential for the development
of neurologic deficits. Kyphosis may occur in one of two
ways. An abrupt angular kyphosis may be present in the
very early stages of the deformity, or a more gradual kyphos-
ing scoliosis might result from progression and rotation of
the scoliosis (Fig. 12-83).248 Recognition of either type is
important because once kyphosis is established, prompt
combined anterior and posterior spinal fusions are required.
Nonoperative management of dystrophic scoliosis is
almost always unsuccessful. These curve patterns need early
and aggressive surgical intervention, even in a young child.
Delay leads only to progressive deformity, which may be as
rapid as 8 degrees per year in the frontal plane and 11
degrees per year in the sagittal plane.108 Most patients
exhibit marked progression before 10 years of age, and

A B
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
spurt. Characteristics of dystrophic scoliosis that correlate
with excessive risk for progression include early age at
onset, a high Cobb angle at the time of initial evaluation,
and the presence of vertebral scalloping, penciling of mul-
tiple ribs, and apical vertebral rotation exceeding 11 degrees
(Perdriolle measurements590).248
Before surgery, a thorough neurologic examination is
essential to identify any subtle abnormalities. MRI and CT
should always be performed.778 MRI demonstrates neurofi-
bromatosis lesions in the neck, thorax, paravertebral region,
neural foramina, or spinal canal. Chiari I malformations,
dural ectasia, pseudomeningoceles, and spinal cord com-
pression (secondary to localized kyphosis, rib impingement,
or a mass effect from neurofibromas) can also be detected
with MRI.¶c CT demonstrates scalloping of the vertebral
bodies anteriorly, erosion of the posterior portion of the
vertebral body or lamina from dural ectasia, and the pres-
ence of ribs within the spinal canal (Fig. 12-84).1,780,857
Three-dimensional CT reconstruction is invaluable in clari-
fying the anatomy of severe deformities and is helpful in
preoperative planning.
Posterior spinal fusion with instrumentation alone can be
used for certain patients with dystrophic curves between
20 and 50 degrees and kyphosis of less than 50 degrees (no
sharp angulation). Because the risk for pseudarthrosis is
higher than that in the idiopathic scoliosis population, con-
sideration should be given to performing imaging studies
(tomography) 6 months after surgery. If the fusion mass
appears inadequate, repeated bone graft augmentation may
be necessary.
Anterior fusion in addition to posterior fusion is needed
for most patients with dystrophic curves.291,389,583,704 The
combination of anterior and posterior fusion increases the
likelihood of successful fusion. Longer fusions are generally
indicated, even in young patients. Curve progression can
¶c
References 28, 170, 252, 375, 376, 615, 777, 779.
CHAPTER 12  Scoliosis 283
C
occur in patients with neurofibromatosis, even in those with
a solid arthrodesis.815
Severe kyphoscoliosis absolutely requires anterior fusion
in addition to posterior fusion. Thorough anterior diskec-
tomy, bone grafting, and rib (or tibia) strut graft placement
are needed (Fig. 12-85). In some patients with exaggerated
kyphosis, the apical rotation may be so severe that the
vertebral body faces posterolaterally. With this deformity,
placement of the strut graft can be extremely difficult, and
the anterior approach to the spine may need to be under-
taken from the concave side. Vertebral body erosion sec-
ondary to intrathoracic neurofibroma or dural ectasia can
also significantly interfere with anterior exposure and
fusion. Dysplastic posterior elements limit the ability to
achieve strong posterior internal fixation. Every effort
should be made to stabilize the spine because stabilization
improves the likelihood of a successful outcome. Postopera-
tive immobilization in a cast or orthosis is clearly indicated
when the vertebrae are weak, the severity and location of
the kyphosis cause excessive strain at certain hook or screw
sites, or the quality of bone does not allow fixation points
for instrumentation. Despite meticulous attempts at ante-
rior and posterior fusion, pseudarthrosis is a significant
concern.152,706,835
Excessive kyphosis is the most frequent cause of neuro-
logic deficits in patients with neurofibromatosis and spinal
deformities. Should a neurologic deficit be present, VCR is
needed to decompress the spinal cord. Laminectomy for
spinal cord decompression or prophylactic laminectomy for
kyphoscoliosis should be avoided because it destabilizes the
spine, increases the kyphosis, removes bone stock needed
for successful posterior fusion, and most important, does
not relieve the anterior compression on the spinal cord.
Neurologic deficits can also result from cord impingement
by neurofibroma lesions within the spinal canal.600 Differ-
entiation of neurofibroma impingement from kyphotic
impingement is required to correctly address the problem
surgically. MRI can help clarify the situation.
284 SECTION II  Anatomic Disorders

A B C

D E F

G H

I J K L
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.
 CHAPTER 12  Scoliosis 285

A B C

D E F
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
Marfan syndrome, one of the more common connective
tissue disorders, has a 0.01% prevalence in the general pop-
ulation.722 Scoliosis is the most common spinal deformity in
this condition, with a prevalence approaching 63%.647,648,722,756
In addition, 6% of patients with Marfan syndrome have
spondylolisthesis. Although Marfan syndrome is an autoso-
mal dominant disorder, no familial pattern of scoliosis has
been identified.
The curve patterns seen in Marfan syndrome are similar
to those seen in idiopathic scoliosis, although Marfan syn-
drome has a slightly higher rate of triple curves and thora-
columbar curves (Fig. 12-86). Scoliosis is equally distributed
between males and females, in contrast to the female pre-
ponderance in idiopathic scoliosis. Limb length discrepan-
cies in patients with Marfan syndrome are associated with
increased structural scoliosis.348
varies widely and requires close examination when planning
treatment.347 Increased lordosis in the thoracic spine was
thought to be common,822,825 but one report found that
increased thoracic kyphosis (>50 degrees) may be found in
as many as 45% of patients with Marfan syndrome and
scoliosis.722 When the transitional point between thoracic
kyphosis and lumbar lordosis is below the second lumbar
vertebra, a long, broad thoracic kyphosis is evident. If a
localized kyphosis exists at the thoracolumbar junction, the
thoracic spine above is generally hypokyphotic.
Back pain is more frequent in patients with Marfan syn-
drome than in the general population. However, no signifi-
cant difference in back pain between patients with scoliosis
and those without has been noted. Back pain is associated
with the presence of dural ectasia, a finding that is more
common in those with Marfan syndrome than in the normal
population.10,11,223,281,721
286 SECTION II  Anatomic Disorders
A B
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
patients with Marfan syndrome exist, although certain
trends are evident. Curves identified in infancy progress
dramatically.723 These curves do not resemble the curves of
infantile idiopathic scoliosis in that they are not expected
to resolve spontaneously and are largely right thoracic in
configuration. In older but still skeletally immature patients,
all curves greater than 30 degrees will probably progress at
least 10 degrees and will reach at least 40 degrees by
maturity.
Unfortunately, brace treatment is not effective in con-
trolling scoliosis in Marfan syndrome,70,171,397,722,723,822 with a
reported success rate of just 17%.721 Most skeletally imma-
ture patients (Risser grade 2 or less) with curves exceeding
25 degrees will reach the stage at which surgery is necessary,
even with brace treatment. In infants, curves almost always
progress to the point of needing operative intervention.
Nevertheless, bracing curves less than 40 degrees in infants
may be a useful technique for postponing surgery. This is
important because in children with Marfan syndrome and
scoliosis, surgical intervention before 4 years of age is associ-
ated with significant cardiac morbidity. Bracing in older
children may also be temporarily beneficial in that it may
allow sufficient maturity to be gained that only posterior
surgery is needed.
Spinal stabilization for scoliotic deformities is indicated
when the magnitude of the curve exceeds 45 degrees in
adolescents or 50 degrees in adults. Because of an increased
risk for atlantoaxial rotatory instability, special attention to
intubation and positioning, both intraoperatively and post-
operatively, is necessary.310 The spinal procedure of choice
is posterior spinal fusion with segmental instrumenta-
tion.184,352,397,436,822 When compared with idiopathic scolio-
sis, patients with Marfan syndrome will have atypical
curve patterns; require more levels of surgical correction,
more distal fusion, greater correction of sagittal balance,
and more reoperations; and have more cerebrospinal
fluid leaks and instrumentation-related complications (Fig.
12-87).171,259,260,347 Thoracolumbar or lumbar kyphosis may
require anterior release before posterior instrumentation to
achieve sufficient sagittal plane flexibility. Patients with
C D
Marfan syndrome tend to have a higher incidence of pseud-
arthrosis, although its true incidence is unknown.347,397 A
higher incidence of perioperative complications, including
increased blood loss, infection, and curve decompensation,
has been reported.347
Instrumentation and fusion in patients who are identified
during infancy usually produce modest correction, in the
range of 20%.723 Surgery should be delayed until the child
is older than 5 years. When surgery is performed earlier,
many patients with large curves die of cardiac complica-
tions. If possible, anterior fusion should be added to prevent
development of the crankshaft phenomenon or to address
excessive kyphosis.
Relative contraindications to performing corrective
surgery for spinal deformity in patients with Marfan syn-
drome include cardiac insufficiency and a dissecting aortic
aneurysm. These conditions should be treated before ortho-
paedic intervention is undertaken. Splenic rupture has been
reported following posterior spinal instrumentation.132
Congenital Heart Disease
The association between scoliosis and congenital heart
disease is well established.#c Because of advances in heart
surgery, children are now living longer than in the past, and
many with severe scoliosis are candidates for operative
correction.
The incidence of scoliosis associated with congenital
heart disease is approximately 4%.694 For those with con-
genital heart disease who have undergone cardiac surgery,
the incidence of scoliosis is higher (11% to 28%).308,309,366
This observation has led some authors to conclude that
performance of thoracotomy or sternotomy in patients with
congenital heart disease may be associated with the devel-
opment of scoliosis.309,366,393 One study reported that scolio-
sis exceeding 20 degrees later developed in 12.5% of 128
mature patients who underwent median sternotomy for the
treatment of congenital heart disease before 8 years of
age.657 Patients operated on before 18 months of age had a
#c
References 48, 54, 219, 351, 366, 470, 562, 595, 622, 651, 694.
 CHAPTER 12  Scoliosis 287

A B C

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

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

A B C

D E F G
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).
 CHAPTER 12  Scoliosis 289

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

A B C
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.
290 SECTION II  Anatomic Disorders
A B
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
lumbar laminectomies to expose the nerve rootlets.
Although rhizotomy reportedly does not result in increased
changes in sagittal plane deformities,594 at our institution
several examples of relentlessly progressive postoperative
hyperlordotic deformities have been encountered. This
same finding was reported elsewhere in 50% of 34 patients
after selective dorsal rhizotomy.342
Treatment of an established postlaminectomy spinal
deformity generally requires further stabilization surgery.
Therefore, all effort should be directed toward preventing
this deformity. Laminoplasties in the thoracolumbar spine
at the time of initial surgery have been helpful in preventing
the occurrence of kyphosis.510,691 Facetectomies should be
avoided if possible. Before tumor resection, neurosurgeons
and orthopaedic surgeons should discuss whether immedi-
ate operative fusion would be beneficial.
Preventive bracing of the spine following laminectomies
has not been effective in stopping subsequent deformities.
Therefore, if a brace is used, it should be understood that
it may provide only temporary benefit. Once notable kypho-
sis has been identified in the cervical or thoracic spine, surgi-
cal fusion is needed to correct and stabilize the deformity.
In the thoracic spine, if the deformity is mild, posterior
fusion with instrumentation may be sufficient. If the defor-
mity is more severe or if the kyphotic deformity is short
and sharply angled, anterior fusion (with a rib strut graft as
needed) should precede the posterior spinal instrumenta-
tion and fusion.569 In the cervical spine, anterior fusion is
often performed in combination with posterior fusion.27,50
Irradiation
For some spinal cord tumors, radiation therapy may be the
primary form of treatment. In a growing child, vertebral
C D
column doses exceeding 1000 rad may have an inhibitory
effect on the physeal regions.543 As a result, asymmetric
growth may develop and lead to a scoliotic or kyphotic
deformity. Spinal deformity may also result from soft tissue
fibrosis and contractures.
Very young children who undergo radiation therapy
(often for Wilms tumor or neuroblastoma) are at greatest
risk for the development of spinal deformities.365,484,491,585,642,643
Long-term follow-up of these individuals is necessary
because the deformities can worsen notably during the ado-
lescent growth spurt. Every effort should be made to
exclude the spine or pelvis from the radiation field in young
children.
Bracing has not proved effective in arresting the progres-
sion of irradiation-induced spinal deformity. However, it
continues to be used in patients with scoliosis exceeding 20
degrees in an effort to delay progression of the deformity.
When the scoliosis exceeds 40 to 45 degrees, operative
intervention should be undertaken. Healing may be pro-
longed, and consideration should be given to repeated bone
grafting 6 months postoperatively. The risk for postopera-
tive complications, including pseudarthrosis and infection,
is increased.
For postirradiation kyphosis, anterior fusion is needed
along with posterior fusion in an effort to avoid the likeli-
hood of pseudarthrosis.561 For sharply angled kyphotic
deformities, a vascularized rib strut graft is recommended.
Postoperative bracing for 6 to 12 months should be consid-
ered in these patients.
Hysterical Scoliosis
Hysterical scoliosis is a diagnosis of exclusion.8,561,757 The
curvature generally has a long C-shaped appearance, with
trunk imbalance, lack of abnormal neurologic or other

physical findings, and no radiographic evidence of vertebral
rotation. A change in the pattern or severity of the scoliosis
from day to day may be seen. The curve generally resolves
when the individual is supine.
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-
tion rate) can rule out infection. Once organic causes have
CHAPTER 12  Scoliosis 291
been ruled out, treatment of hysterical scoliosis requires
psychological (or psychiatric) therapy. Orthotic manage-
ment should not be undertaken because it may reinforce
the underlying personality disorder.
References
For References, see expertconsult.com. 

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292 SECTION II  Anatomic Disorders

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

Self-retaining
retractors

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)
 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

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

T4

C, Once the spinous processes have

been exposed, the median raphe is
incised sharply down to bone. A
Transverse
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
F
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).
294 SECTION II  Anatomic Disorders

Plate 12-2  Posterior Spinal Instrumentation and Fusion Using Hooks

B C

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.
 CHAPTER 12  Scoliosis 295

D E

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

296 SECTION II  Anatomic Disorders
Plate 12-2  Posterior Spinal Instrumentation and Fusion Using Hooks, cont’d
H I
H, The spinal deformity is corrected by rotating the rod
90 degrees. Rotation is greatly facilitated by using the
small wrench over the hexagonal end of the rod. On occa-
sion, a vise grip applied to the distal end of the rod can
assist in this rotation maneuver. Rotation should be per-
formed slowly to avoid possible intermediate-level hook
disengagement. The most cephalic pedicle hook and most
caudal lamina hook usually maintain excellent purchase
during the correction maneuver.
The amount of curve correction is greatly determined
by the flexibility of the spine. All the correction is achieved
with the first rod. After rod rotation, slight distraction is
placed at each of the hook sites to ensure firm purchase.
The nut on each eyebolt is tightened completely.
I, Further correction of the spinal deformity should not
be expected with the second rod. However, this rod
does significantly increase the torsional strength of the
construct. The proximal end of the rod is secured to the
spine with a “claw.” This claw is created by placing a
J
downwardly directed lamina hook over the transverse
process at the most cephalic vertebral site. An upwardly
directed pedicle hook is placed one level lower. These two
hooks are then compressed along the rod to provide firm
proximal fixation. Once the claw is firmly secured on the
uppermost segments of the convex rod, compression can
be applied along the rod at the remaining hook sites.
J, An alternative method of fixation of the proximal site
of the convex rod is to use a downwardly directed sub-
laminar hook. This configuration is used if the transverse
process site does not provide satisfactory fixation for the
claw configuration. At the intermediate hook site on the
convex rod is an upwardly directed pedicle hook, which
is usually the most prominent hook in this two-rod con-
struct. If this hook is too prominent, it may be excluded.
At the inferior hook site on the convex rod is an upwardly
directed sublaminar hook. The eyebolts for the cross-link
should be placed on the rod before engaging the rod with
the hooks.
 CHAPTER 12  Scoliosis 297

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

A B

Unsafe Cephalad-Caudad Starting

A Safe Level
Point

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:
T10
Proximal edge–
proximal third TP

T11 T11 Proximal third TP

T12 T12 Midpoint TP

Continued on following page

298 SECTION II  Anatomic Disorders
Plate 12-3  Posterior Spinal Instrumentation and Fusion Using Pedicle Screws, cont’d
1 3
2 4
C
C, 1 and 2: Preparation for the pedicle screw sites should
begin at the most distal vertebra to be instrumented and
proceed cephalad. Either the freehand technique or the
fluoroscopy-guided technique can be used. A thoracic gear-
shift (2-mm blunt-tipped curved pedicle finder) is used to
enter the pedicle.
3: To avoid medial wall penetration, the gearshift is ini-
tially pointed laterally when the pedicle is entered.
4: After insertion to 15 to 20 mm, the pedicle finder is
reversed (medial orientation) to continue penetration into
the vertebral body to a depth approximating 30 to 40 mm,
depending on the vertebra level. Once this is accomplished,
a ball-tipped probe is used to palpate all four walls of the
pedicle and the floor (anterior vertebral body). The depth
that is measured will approximate the screw length that will
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.)
 CHAPTER 12  Scoliosis 299

E1a

2
D

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.

E1b

Continued on following page

300 SECTION II  Anatomic Disorders

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

F1

E2

F2

F, 1: A precountoured provisional convex right lumbar rod

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

E3

2: Fluoroscopic images demonstrating accurate place-

ment of the lumbar screws.
3: Lumbar screws.
 CHAPTER 12  Scoliosis 301

G3

G1

G2
H

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.
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

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

Periosteal bed of
resected 10th rib

A
Sutures are
placed prior
to splitting
of cartilage

10th rib cartilage

is split with scalpel

requiring instrumentation. For instrumentation between

Operative Technique
In this plate the thoracoabdominal approach for exposure
of the lower thoracic and lumbar spine is described.
A, Positioning. Under the direction of the surgeon, the
patient is placed in the lateral decubitus position (the con-
vexity of the curve is upward). A roll is placed under the
axilla of the dependent arm. The body is supported with a
deflatable beanbag. The upper part of the arm is flexed
forward and slightly abducted. The operating table may be
temporarily flexed (at the apex of the scoliosis) to facilitate
excision of the intervertebral disks.
Approach. It will be necessary to remove a rib for expo-
sure of the spine. Ideally, the rib that is removed is the one
immediately cephalad to the uppermost vertebral body
T11 and L3, removal of the tenth rib allows adequate
exposure.
Skin incision. The incision begins lateral to the spinous
process of T10 (or T9) and extends along the course of the
tenth rib to the costocartilaginous junction and then across
the upper part of the abdomen to the lateral edge of the
rectus abdominis. Here, it turns distally toward the sym-
physis pubis and stops at the level of the umbilicus.
B, The tenth rib is freed subperiosteally, divided at its cos-
tocartilaginous junction, and removed. This creates a larger
working aperture and provides a source of autogenous bone
graft.
C, Once the costal cartilage of the tenth rib is split, the
retroperitoneal space is identified and entered.
 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
peritoneum from the inferior aspect of the diaphragm.
Once freed, the viscera lie safely away from the vertebral
bodies. Identification sutures are placed on either side of
the intended line of division of the diaphragm, which is ½
to ¾ inch from its periphery. Placement of several of these
sutures facilitates proper closure of the diaphragm later.
E, The diaphragm is sectioned from its costal
attachments.

Continued on following page

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

Disk

Hemiazygos
vein

Aorta

G
Abdominal contents
retracted

F, Next, the parietal pleura is incised along the thoracic
vertebral bodies that are to be included in the fusion.
G, In the lumbar region, the psoas muscle is gently elevated
off the vertebral bodies and intervertebral disks and
retracted posteriorly. The segmental vessels are ligated in
the middle of each vertebral body included in the fusion.
The aorta and vena cava are protected with retractors,
and the anterior longitudinal ligament is partially excised
with a sharp scalpel. Each disk within the levels selected for
fusion is removed with various rongeurs and curets.
 CHAPTER 12  Scoliosis 305

Osteotome and mallet

used to remove
vertebral end-plate

Disks removed

Space packed with

sponge and Gelfoam

H
Instrument to
protect aorta
and vena cava

H, With a curet or sharp osteotome and mallet, the operator
removes the vertebral cartilaginous end-plates and retained
pieces of disk. For correction of kyphosis, most of the
annular ligamentous tissue down to the posterior longitudi-
nal ligament is removed. For scoliosis, however, the outer
annular fibers need not be fully removed. The disk spaces
are then temporarily packed with Gelfoam to minimize
bleeding. If the operating table was flexed to facilitate exci-
sion of intervertebral disks, it should be flattened at this
time.

Continued on following page

306 SECTION II  Anatomic Disorders

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

I J

K L

I, Following complete disk excision, the dual screws are
placed via some guidance with a dual-headed staple. Retrac-
tion of the abdominal contents is seen at the top of the
photo.
J, The precontoured posterior rod has been placed and a
rod rotation maneuver was performed to correct the scolio-
sis and improve the lumbar lordosis.
K, Following rod rotation, anterior structural support is
placed to maintain the lumbar lordosis, to assist in correc-
tion of the coronal plane deformity, and to increase the
stiffness of the construct.
L, Final construct after the anterior rod has been placed.
 CHAPTER 12  Scoliosis 307

M, Preoperative and 2-year radiographs following anterior

spinal fusion and instrumentation with a dual-rod system
and anterior autologous rib bone graft.