Chapter 66
Pediatric Spinal Deformity
Daniel J. Sucato, MD
B. Stephens Richards III, MD
Idiopathic Scoliosis
Idiopathic scoliosis is the most common type of scoliosis
and, as its name implies, there is no known definitive
etiology for this condition. It is defined as a lateral cur-
vature of the spine with a Cobb angle of 10 or greater
and axial plane rotation. The sagittal plane usually dem-
onstrates hypokyphosis in the thoracic spine, junctional
kyphosis between two structural curves, and segmental
hypolordosis of the lumbar spine when a structural
curve is present. Classification of idiopathic scoliosis is
usually defined according to the age of the patient at
the time of curve development as follows: infantile
(from birth to age 3 years), juvenile (from age 3 years to
10 years), and adolescent (from age 10 years to 18
years). The age classifications, although somewhat arbi-
trary, allow the surgeon to characterize curves and assist
in treatment algorithms from the outset.
Infantile Idiopathic Scoliosis
Infantile idiopathic scoliosis is uncommon, and its etiol-
ogy is not determined. However, patients who present
with this presumed diagnosis require more careful eval-
uation because an underlying cause is more often found
with infantile idiopathic scoliosis than juvenile and ado-
lescent scoliosis. A recent multicenter study demon-
strated that 21.7% of patients who presented with infan-
tile idiopathic scoliosis measuring greater than or equal
to 20 had neural axis abnormalities, and 80% of these
patients required neurosurgical intervention. The au-
thors of this study recommended that an MRI scan be
obtained at the time of presentation for any patient with
presumed infantile idiopathic scoliosis whose curve
measures 20 or greater.
Infantile idiopathic scoliosis is the least common
type of idiopathic scoliosis. It is more commonly seen in
males when compared with juvenile or adolescent idio-
pathic scoliosis (1:1 male to female ratio), and left
curves are more often reported.
Spontaneous correction of infantile idiopathic scolio-
sis may occur, and can be predicted based on the posi-
tion of the rib relative to the vertebra and the rib-
American Academy of Orthopaedic Surgeons
vertebral angle difference (RVAD) as described by
Metha. If the rib overlaps the vertebral body at the apex
of the curve (phase 2 rib), the curve is likely to progress,
whereas a phase 1 rib pattern (no overlap of the rib) re-
quires measurement of the RVAD to assess for curve
progression. A patient with a phase 1 rib and an RVAD
greater than 20 has a significant risk for curve progres-
sion. Careful observation is warranted in patients with
these curves, and treatment with an orthotic device is in-
dicated for those curves that have shown progression or
are greater than 30. For the young patient with a large
curve or those patients who do not tolerate an orthotic
device, a Risser cast is appropriate to loosen up the
spine and allow for improved wear of the orthotic de-
vice.
Surgical treatment should be delayed as long as pos-
sible, primarily because of the concern with creating a
small chest and its detrimental effect on pulmonary func-
tion. Although there are no definitive indications for sur-
gical treatment, progression despite orthotic treatment
or curves greater than 50 are generally accepted indica-
tions. A variety of surgical treatment strategies have
been described, with the most predictable outcome re-
sulting from an anterior and posterior fusion. Posterior
instrumentation is generally recommended, and it is pos-
sible in even small patients because of the newer and
smaller instrumentations that are currently available. A
5-year follow-up study of 13 patients demonstrated that
convex epiphysiodesis combined with a Luque trolley re-
sulted in overall good results, and instrumented growth
was 32% of what was expected. Newer instrumentation
using dual posterior rods and connectors in the middle
of the construct to allow intermittent lengthenings have
demonstrated favorable early results. This approach ap-
pears to limit the typically high complication rate ob-
served in the traditional growing rod scenario. Longer
follow-up is required for these patients.
Juvenile Idiopathic Scoliosis
Juvenile idiopathic scoliosis occurs in patients between
the ages of 3 to 10 years; approximately 15% of all pa-
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Pediatric Spinal Deformity
tients with idiopathic scoliosis have juvenile idiopathic
scoliosis. Females are more commonly affected than
males, and this disparity increases with age. As with ado-
lescent idiopathic scoliosis, right thoracic curves are
more common.
The natural history of juvenile idiopathic scoliosis is
one of steady progression until age 10 years, when
curves generally progress fairly rapidly. Unlike infantile
idiopathic scoliosis, 95% of all patients with juvenile id-
iopathic scoliosis have progressive curves, whereas only
5% resolve. The thoracic curves tend to progress more
commonly and require fusion more often. Curve pat-
terns may change with extension of the primary curve
or a development of secondary structural curves.
As with those with infantile idiopathic scoliosis, pa-
tients with juvenile idiopathic scoliosis should have MRI
studies to evaluate the neural axis because of a 20% to
25% incidence of abnormalities. These abnormalities of-
ten require surgical treatment; therefore, it has been
recommended that MRI be done at the time of the ini-
tial evaluation.
The nonsurgical treatment of juvenile idiopathic
scoliosis is similar to that for adolescent idiopathic scoli-
osis. Bracing is used to treat patients with curves that
have progressed to 30 or for those with curves exceed-
ing 20 that have demonstrated 5 of progression. Surgi-
cal management is also similar to that for patients with
adolescent idiopathic scoliosis, although the crankshaft
phenomenon assumes greater importance in the skele-
tally immature patient.
Adolescent Idiopathic Scoliosis
Adolescent idiopathic scoliosis is relatively common,
with a reported incidence of 2% to 3% for curves be-
tween 10 and 20, and an incidence of 0.3% for curves
greater than 30. There is an equal incidence of smaller
curves among males and females, whereas the female to
male ratio of curves greater than 30 is 10:1.
The natural history of adolescent idiopathic scoliosis
has been well studied, and it is generally accepted that
curves progress in two scenarios: (1) continued spine
growth and (2) large curve magnitude despite the com-
pletion of spine growth. Continued spine growth is as-
sessed using an array of both clinical and radiographic
parameters. Clinical parameters include female menar-
cheal status and height measurements performed at
each clinic visit to determine growth velocity. Peak
growth velocity is approximately 10 cm per year and oc-
curs just before the onset of menses in females. Radio-
graphic parameters that indicate skeletal immaturity in-
clude an open triradiate cartilage, and a Risser grade 0
to 1. Following the completion of growth, curve progres-
sion is more likely for patients with thoracic curves ex-
ceeding 45 to 50 and thoracolumbar/lumbar curves ex-
ceeding 40, especially when coronal imbalance is
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Orthopaedic Knowledge Update 8
present. The rate of progression in these curves is ap-
proximately 1 per year.
Patients with adolescent idiopathic scoliosis should
have a complete and organized clinical evaluation to
confirm the diagnosis, rule out neural axis abnormalities,
and assist in determining treatment. A careful inspec-
tion of the skin is necessary to rule out lesions such as
caf-au-lait spots (neurofibromatosis) and cutaneous
manifestations of dysraphism. The extremities, especially
the feet, should be inspected to ensure that no deformi-
ties (cavovarus feet indicating neural axis abnormalities)
are present. Observation of gait (including heel and toe
walking) will allow the surgeon to obtain a general
sense of the patients strength and coordination. The
neurologic examination should include an assessment of
motor function in all muscle groups, a sensory examina-
tion, and deep tendon reflexes. Abdominal reflexes that
are symmetric confirm a normal neural axis; when ab-
dominal reflexes are asymmetric, the surgeon should ob-
tain an MRI scan. Examination of the patients back in-
cludes an assessment of balance, trunk imbalance, waist
asymmetry, pelvic tilt, and limb-length discrepancy.
Shoulder height differences are important, especially
when deciding whether an upper thoracic curve requires
fusion. The Adams forward bend test is performed to
analyze axial plane deformity, which is an indication
that the curve is structural. Asymmetric forward bend-
ing of the patient or tenderness of the spine to palpation
may indicate a neural axis abnormality.
Initial radiographic examination should include PA
and lateral radiographs of the entire spine (lower cervi-
cal spine down to the hips). The PA radiograph should
be used to evaluate the spine and determine the Cobb
angle for all curves. Skeletal maturity should be assessed
using the Risser sign and the maturity of the triradiate
cartilage (open or closed). The PA radiograph should al-
ways be assessed to ensure that two pedicles are present
for each vertebra, no signs of congenital vertebra exist,
and that there is no evidence for other abnormalities
such as neurofibromatosis (penciling of the ribs, en-
dosteal scalloping, or significantly wedged vertebra). An
assessment of spinal balance can be made by dropping a
C7 plumb line and comparing it with the center sacral
vertical line. Structural characteristics of the proximal
thoracic curve include T1 tilt, Cobb angle of greater
than 30, Nash-Moe apical rotation of II or greater, and
a transitional vertebra between the upper and middle
thoracic curves of T6 or lower. Recently, the clavicle an-
gle (the angle subtended by the intersection of a hori-
zontal line and the line that is tangential to the highest
points of each clavicle) has been shown to be the best
predictor of postoperative shoulder balance. The lateral
radiograph can be used to assess sagittal balance, which
is variable, and to ensure that thoracic hypokyphosis is
present. The rib heads of the thoracic curve should dem-
onstrate apical lordosis in patients with adolescent idio-
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Orthopaedic Knowledge Update 8
pathic scoliosis, and its absence should warrant investi-
gation of the neural axis with MRI. A recent study
demonstrated that 97% of patients with adolescent idio-
pathic scoliosis and a normal MRI study had apical lor-
dosis, whereas this sign was absent in 75% of patients
with scoliosis and a syringomyelia. The presence of a
spondylolysis and/or spondylolisthesis should always be
evaluated at the lumbosacral junction and is present in
1% to 2% of patients with adolescent idiopathic scolio-
sis. Preoperative radiographic assessment should include
supine best-effort bend films to the right and left.
MRI should not be used as a screening tool and is
indicated when there is an atypical curve pattern, ab-
sence of thoracic lordosis, atypical pain, neurologic ab-
normalities or foot deformities, or extremely rapid curve
progression.
The King-Moe classification has been traditionally
used for determining curve types in patients with ado-
lescent idiopathic scoliosis. This is a five-part classifica-
tion that was developed to describe thoracic curve pat-
terns to help guide the surgeon when implanting
Harrington instrumentation. As with many orthopaedic
classifications, it has fair interobserver and intraobserver
reliability. The newer classification system described by
Lenke and associates provides a more comprehensive
evaluation of both the PA and lateral radiographs and
also uses best-effort supine bend films. The following
three components are defined in this classification sys-
tem: curve types (1 through 6), lumbar modifiers (A, B,
or C), and thoracic kyphosis (negative, N = normal, or
positive). Although more comprehensive, this classifica-
tion system has 42 possible combinations and therefore
introduces more complexity into the curve evaluations.
When each of the three components were evaluated in-
dividually, the reliability was similar to that of the King-
Moe classification. As with any classification system for
adolescent idiopathic scoliosis, it should always be used
in conjunction with a careful clinical evaluation of the
patient.
Nonsurgical treatment includes observation and
management with an orthotic device. Observation is in-
dicated for any curve less than 45 in a skeletally mature
patient without significant clinical deformity. In the skel-
etally immature patient, observation of curves less than
25 is warranted. Bracing is used for the skeletally im-
mature patient (Risser grades 0, 1, or 2) with a curve be-
tween 30 and 45 or an initial curve between 20 and
25 that has demonstrated 5 of progression. Although
numerous braces are available today, the Boston and
Charleston braces are most commonly used. The Boston
brace can be used to treat all curve patterns. Successful
treatment depends on the amount of time the brace is
worn, as was reported in a recent study in which a com-
pliance monitor was incorporated into the brace. The
Charleston (nighttime) brace has been shown to be ef-
American Academy of Orthopaedic Surgeons
Chapter 66 Pediatric Spinal Deformity
fective in treating thoracolumbar/lumbar curves that
measure between 25 and 35.
Surgical Treatment
Generally, the indications for surgical treatment of pa-
tients with adolescent idiopathic scoliosis are thoracic
curves greater than 45 in the skeletally immature pa-
tient or greater than 50 in the skeletally mature pa-
tient. Because thoracolumbar/lumbar curves are more
likely to progress despite a smaller curve magnitude,
surgical intervention is indicated for patients with
curves greater than 40 to 45, especially when there is
significant rotation and/or translation. The first goal of
surgery is to prevent curve progression with spinal ar-
throdesis. The second goal of surgery is to safely im-
prove the three-dimensional deformity. Surgical plan-
ning depends on the radiographic and clinical deformity
present and the skeletal maturity of the patient. Fusion
levels depend on the surgical approach used and a care-
ful assessment of the radiographs and clinical deformity.
The standard, more traditional posterior approach can
be used for all curve patterns, and it is best for double
or triple curves. The anterior approach is more com-
monly used for thoracolumbar/lumbar curve patterns
because removal of the disk assists in achieving im-
provement in coronal plane deformity, and lumbar lor-
dosis can be restored. The anterior approach can also be
used for thoracic curve patterns, especially when hy-
pokyphosis is present. In this instance, correction is
achieved with convex compression, which produces ky-
phosis. Use of anterior instrumentation requires close
attention to screw length and direction (in the vertebral
body) because of the proximity of the aorta to the left
side of the spine in patients with right thoracic scoliosis.
For those undergoing posterior instrumentation and
fusion, an anterior diskectomy and fusion should also be
performed in those who are skeletally immature (Risser
grade 0 with open triradiate cartilage) and in those pa-
tients who have very large (> 80) and stiff (< 50% flex-
ibility index) curves.
Advances in spinal instrumentation have improved
the correction of scoliotic deformities. Studies have
demonstrated improvement and maintenance of defor-
mity when pedicle screws are used in the thora-
columbar/lumbar spine (Figure 1). The use of pedicle
screws in the thoracic spine also improves curve correc-
tion when compared with hooks, and initial reports
demonstrate safe placement (Figure 2). However, ana-
tomic studies demonstrate that it may be challenging to
place thoracic pedicle screws, especially on the concav-
ity of the curve, because of the narrow width of the
pedicle and the proximity of the aorta laterally and spi-
nal cord medially. Improvement in lateral and posterior
translation of the thoracic spine may not be significantly
improved when compared with segmental hook and
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Pediatric Spinal Deformity Orthopaedic Knowledge Update 8

Figure 1 Preoperative AP (A) and lateral (B) and 3-year postoperative AP (C) and lateral (D) radiographs of a 14-year-old girl who underwent posterior spinal fusion and
instrumentation with a combination of hooks, sublaminar wires, and pedicle screw fixation. Restoration of coronal and sagittal balance is seen in the postoperative radiographs,
with excellent correction of the lumbar curve.

Figure 2 Preoperative (A and B) and 2-year postoperative (C and D) radiographs of a 14-year-old boy after posterior spinal fusion and instrumentation using pedicle screw
fixation alone.

wire fixation; however, the improvement in the ability to
correct axial rotation may prove to be its greatest ad-
vantage. Confirmation of screw placement is more diffi-
cult in the thoracic spine because radiographic visualiza-
tion is obscured by the ribs and soft tissues. The results
of using electromyographic stimulation of screws to con-
firm intrapedicle placement is not as reliable as lumbar
screw stimulation.
The thoracoscopic approach to perform an anterior
release appears as effective as open thoracotomy and
has minimized the incisions required for anterior access
to the spine. A recent study demonstrated that an ante-
rior thoracoscopic release/fusion performed with the pa-
tient in the prone position is very effective and better
tolerated when compared with a thoracoscopic release
with the patient in the lateral position. Thoracoscopic
instrumentation for single thoracic curves achieves cor-
rection comparable to open anterior or posterior instru-
mentation (Figure 3). Although less scarring provides
excellent cosmetic improvement, the duration of thora-
coscopic surgery continues to be significantly longer
when compared with more conventional approaches.
Despite recent advancements in spinal instrumenta-
tion and techniques, the ultimate goal of surgical treat-

778 American Academy of Orthopaedic Surgeons

 Orthopaedic Knowledge Update 8
Figure 3 Preoperative (A and B) and 3-year postoperative (C and D) radiographs of a 14-year-old girl after a thoracoscopic anterior spinal fusion and instrumentation.
ment is to achieve solid fusion while minimizing compli-
cations. Autogenous iliac crest bone continues to be the
gold standard to promote fusion in adolescent idiopathic
scoliosis. Neurologic monitoring using somatosensory-
evoked potentials and/or motor-evoked potentials is now
the standard of care. A 50% decrease in amplitude and/
or an increase in latency of 10% are generally considered
thresholds for concern for neurologic injury when assess-
ing patients using somatosensory-evoked potentials. Crit-
ical threshold values for motor-evoked potentials are not
as clear and are dependent on the mode of stimulation.
The Stagnara wake-up test, which is used when neurolog-
ic injury is suspected, is the gold standard for neurologic
assessment. Acute complications from the surgical treat-
ment of adolescent idiopathic scoliosis are relatively rare.
However, the need for revision following a posterior spi-
nal fusion can be as high as 19%, with revision for late-
onset surgical pain from prominent hardware occurring
in 8% of patients. Delayed infections can occur up to 3 to
4 years postoperatively and can present as a small drain-
ing wound or fluctuance, accompanied by low-grade fe-
vers and a mildly elevated erythrocyte sedimentation
rate. Treatment consists of removal of the instrumenta-
tion and primary closure, followed by oral administration
of antibiotics. Intraoperative cultures usually grow Sta-
phylococcus epidermidis or Propionibacterium acnes, an
organism that requires culture incubation up to 2 weeks.
Scoliosis Research Society-22 Patient Outcome
Instrument
This health-related quality-of-life patient questionnaire
was developed to correlate patients perceptions and
satisfaction with their scoliotic deformities. Studies have
shown this instrument to be simple to use, internally
American Academy of Orthopaedic Surgeons
Chapter 66 Pediatric Spinal Deformity
consistent, and comparable in reliability and validity to
the Medical Outcomes Study Short Form-36 Health Sur-
vey Questionnaire (SF-36). The Scoliosis Research
Society-22 Patient Outcome Instrument (SRS-22) is
shorter and more focused on health issues related to
scoliosis than is the SF-36. Following surgical treatment
for adolescent idiopathic scoliosis, outcomes using the
SRS-22 have shown significant improvement from the
preoperative status in the domains of pain, general self-
image, function from back condition, and level of activ-
ity. Improvement in coronal Cobb angle correction does
not correlate with improved SRS-22 scores.
One study reported that minimum 20-year follow-up
for patients who had posterior surgery with Harrington
instrumentation for idiopathic scoliosis demonstrated
similar back function when these patients were com-
pared with an age-matched control group from the gen-
eral population. Those who underwent surgery had a
greater likelihood of having back pain (78% versus
58%) and lumbar pain (65% versus 47%). In a 50-year
follow-up study of untreated patients compared with a
matched control group, the authors reported a greater
incidence of shortness of breath (22% versus 15%),
which was associated with a Cobb angle of greater than
80. Despite a greater likelihood of chronic back pain
(61% versus 35%), patients with scoliosis were produc-
tive and functioning at a high level.
Congenital Spinal Deformities
Congenital vertebral abnormalities lead to a variety of
spinal deformities, including scoliosis, kyphosis, or a
combination of the two. Its cause remains unknown. Ge-
netic abnormalities or any other traumatic or teratologic
type of maternal insults during pregnancy are rarely Ki-
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Pediatric Spinal Deformity
dentified in patients with congenital vertebral abnor-
malities. Recently, congenital scoliosis has been docu-
mented in two monozygotic twins, a finding that
contradicts most of the findings in the congenital defor-
mity literature. A potential increase in exposure to
chemical fumes and carbon monoxide in mothers of
children with congenital spine deformities has also been
reported.
Spinal dysraphism, which includes numerous abnor-
malities such as diastematomyelia, syringomyelia, diplo-
myelia, Arnold-Chiari malformations, intraspinal tumors,
and tethering of the spinal cord, is consistently found in
30% to 40% of patients with congenital spinal deformi-
ties. MRI, which best identifies these dysraphic abnor-
malities, has been recently recommended for all patients
with congenital spinal deformity as part of the initial
evaluation, even in the absence of clinical findings. The
mere presence of a potentially tethering intraspinal le-
sion may be sufficient reason for prophylactic surgical
treatment to address the lesion before the development
of any neural dysfunction. This approach, in the absence
of neurologic findings, remains controversial.
Congenital Scoliosis
The variety of vertebral anomalies found in congenital
scoliosis makes its natural history uncertain. The two ba-
sic types, defects of vertebral formation and defects of
vertebral segmentation, may occur separately or in com-
bination. In 80% of patients with congenital scoliosis,
the anomalies can be classified into one of the two
types.
Defects of vertebral formation may be partial or
complete. True hemivertebrae result from the complete
failure of formation on one side and cause the forma-
tion of laterally based wedges consisting of half the ver-
tebral body, a single pedicle, and hemilamina. When
present in the thoracic spine, hemivertebrae are usually
accompanied by extra ribs. When located at the lum-
bosacral junction, a significant obliquity between the
spine and pelvis can result and is usually accompanied
above by a long compensatory scoliosis. This lumbosac-
ral deformity is best treated surgically (usually with
hemivertebrectomy) at an early age before the compen-
satory curve becomes fixed.
Defects of segmentation result in an osseous bridge
between two or more vertebrae, either unilaterally or
involving the entire segment. The combination of unilat-
eral failure of segmentation and contralateral hemiver-
tebra carries the worst prognosis in congenital scoliosis
because it produces the most severe and rapidly pro-
gressive deformity. Curves of this kind located in the
thoracolumbar spine can be expected to exceed 50 by
the age of 2 years. Without treatment, patients with tho-
racolumbar, midthoracic, or lumbar curves experience
severe deformity at an early age. Rib fusions that ac-
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Orthopaedic Knowledge Update 8
company unilateral segmentation defects on the concav-
ity of the curve may adversely affect thoracic growth, re-
sulting in severe limitations in pulmonary function and
growth. This rare condition is known as thoracic insuffi-
ciency syndrome, which is defined as the inability of the
thorax to support normal respiration or lung growth.
Left untreated, progressive deterioration occurs and can
result in death at an early age. Methods to expand the
thoracic cage are being investigated, with the goals be-
ing to provide an acute increase in the thoracic volume
with stabilization of any flail chest wall defects and to
maintain these improvements as the patient grows.
In patients with severe congenital scoliosis, plain ra-
diographs may not provide sufficient information re-
garding vertebral abnormalities. Should surgical inter-
vention be necessary, a CT scan with reformatted three-
dimensional reconstruction provides excellent detail for
the understanding of the deformity. These reconstruc-
tions are extremely valuable in the preoperative plan-
ning for severe deformities. MRI of the spine also must
be done for all patients with congenital scoliosis who
are undergoing surgical intervention to assess intracanal
abnormalities (30% to 40% prevalence). Currently, re-
formatted images from MRI can provide a clear picture
of the canal contents despite the severe three-
dimensional deformity associated with some congenital
scoliosis patients.
Although bracing has no beneficial effect on congen-
ital curves, it may help to control long flexible compen-
satory curves below the congenital component. The pri-
mary goal of surgical intervention is to stop further
progression. Partial correction that can be obtained
safely is an added benefit. Even if the curves are rela-
tively small (< 40), once curve progression has been
confirmed, surgical intervention should be undertaken.
Surgery should be performed prior to the development
of notable deformity, a concept different from that in id-
iopathic scoliosis. Various surgical approaches are used
depending on patient maturity, deformity location, and
type of deformity. These approaches include anterior
and posterior convex hemiepiphysiodesis, anterior and
posterior spinal fusion, posterior fusion with or without
instrumentation, hemivertebra excision, and spine os-
teotomies. Expansion thoracoplasty, a new approach to
surgery for congenital scoliosis, is indicated for very
young patients who either have or will potentially have
thoracic insufficiency syndrome. It consists of lengthen-
ing the concave hemithorax with rib distraction by
means of a prosthetic rib distractor (Figure 4). This re-
sults in increased growth of both the concave and con-
vex sides of the curve, thus allowing additional volume
for growth of the underlying lungs. This technique, cur-
rently undergoing clinical trials, appears very promising.
In patients with severe congenital spinal deformities,
slow gradual correction has recently been reported to
be successful in some individuals using preoperative
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 Orthopaedic Knowledge Update 8
Figure 4 A, Radiograph of a 4-month-old girl with congenital scoliosis, fused ribs, and a unilateral unsegmented bar. B, Radiograph of the same patient at age 11 years. After
several expansion thoracoplasty procedures, the patients hemithorax is increased in size beyond that expected with no intervention.
halo traction for 6 to 12 weeks. If this method is used,
very close monitoring for any neurologic change (numb-
ness, tingling, and weakness) is essential. When partial
correction is obtained or a plateau has been reached,
the spine is stabilized by instrumentation and fusion.
Few patients with congenital scoliosis secondary to a
hemivertebra need to have the hemivertebra excised.
The main indication for hemivertebra excision is a fixed
decompensation in a patient in whom adequate align-
ment cannot be achieved through other procedures
(usually involving a hemivertebra of the fourth or fifth
lumbar level). Although a combined anterior and poste-
rior resection has been the standard procedure for
hemivertebra excision, several recent studies report suc-
cess with excision through a posterior approach only,
with correction maintained using transpedicular instru-
mentation.
Congenital Kyphosis
Congenital kyphosis represents an abrupt posterior an-
gulation of the spine resulting from a localized congeni-
tal malformation of one or more vertebrae. This defor-
mity is caused either by defects of formation (type 1),
defects of segmentation (type 2), or a combination of
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Chapter 66 Pediatric Spinal Deformity
the two. In contrast to congenital scoliosis, failure of seg-
mentation in congenital kyphosis is less common and
produces much less deformity than failure of formation.
In type 1 kyphosis, there is a partial (or complete) defi-
ciency of the vertebral body, but the posterior elements
remain present. With growth, a relentless progression in
the kyphosis occurs, leading to anterior impingement on
the spinal cord. When this type of deformity is diag-
nosed, plans for surgical intervention should begin im-
mediately because of the risk of neurologic deficits.
In type 2 kyphosis, the anterior portion of two or
more adjacent vertebral bodies are fused, which leads to
a deformity that is less progressive, produces less defor-
mity, and has a much lower risk of paraplegia than that
seen in patients with type 1 kyphosis.
When imaging congenital kyphosis, MRI will pro-
vide the clearest picture of the spinal cord and, in very
young patients, the clearest picture of the vertebral bod-
ies. Cord compression may be evident on MRI before
any clinical neurologic deficits. Three-dimensional imag-
ing of the spine using CT scan reconstructions is useful
for the evaluation of the vertebral anomalies, especially
in the older child. Both tests should be obtained before
any surgical intervention.
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Pediatric Spinal Deformity
Because nonsurgical treatment has no beneficial ef-
fect on congenital kyphosis, the use of an orthotic de-
vice is inappropriate. In patients with type 1 kyphosis,
surgical intervention should be considered, even in the
infant, with the primary goal being prevention of future
paraplegia. If the deformity is recognized in patients
younger than age 3 to 5 years and before the kyphosis
exceeds 50, simple posterior fusion without instrumen-
tation may be considered. A hyperextension cast is used
postoperatively for 4 to 6 months followed by a thora-
columbosacral orthosis for another 6 months. This ap-
proach may allow for some growth anteriorly in the ab-
normal region of the spine, which, over time, may result
in progressive improvement in the localized kyphosis.
Alternatively, in patients with kyphosis that exceeds 50,
an anterior release with strut graft must accompany the
posterior fusion. In the older child or the adult, com-
bined anterior and posterior arthrodesis is mandatory.
Rotatory Dislocation
Segmental spinal dysgenesis, congenital dislocation of
the spine, and congenital vertebral displacement of the
spine are conditions that create the most severe local-
ized kyphosis of the spine and lead to a neurologic defi-
cit in 50% to 60% of patients. These conditions can be
difficult to differentiate from one another. The deformi-
ties include severe kyphosis; anterior, posterior, or lat-
eral subluxation of the spine; and scoliosis in association
with a severely stenotic spinal canal. The treatment re-
quires combined anterior and posterior spinal fusion be-
cause posterior fusion alone is insufficient to achieve
solid arthrodesis in patients with these types of congeni-
tal instabilities. Exploration and augmentation of the
posterior fusion mass should be considered because of a
high occurrence of pseudarthrosis. No sudden correction
should be attempted in older patients with severe angu-
lar kyphosis and progressive neurologic deficit. Function
must be favored over cosmetic appearance. Neurosurgi-
cal decompression should be used only for patients with
a proven recent and progressive neurologic deficit.
Spondylolysis and Spondylolisthesis
Spondylolysis is a defect of one or both of the pars in-
terarticularis in the posterior element of a lower lumbar
vertebra. Although it generally results from repetitive
stress to the pars interarticularis, it can occur abruptly.
Seen in 4% to 6% of the general population, spondylo-
lysis has a predilection in those whose activities involve
increased hyperextension of the lumbar spine. The con-
dition primarily affects L5 (in 87% to 95% of patients)
and less frequently L4 (in up to 10%) and L3 (in up to
3%). Spondylolisthesis represents a forward slippage of
all or part of one vertebra on another and in children is
nearly always located between L5 and the sacrum.
782
Orthopaedic Knowledge Update 8
Patients usually present with pain localized to the
low back that is usually aggravated by extension activi-
ties and relieved by rest. Radiation of the pain into the
buttocks or posterior thighs is uncommon and a neuro-
logic deficit is rare. Unless the slippage is high grade, the
back alignment appears normal and no localized tender-
ness is present. Hamstring tightness may be present and
can lead to a shortened stride length. In patients with a
high-grade slip, the buttocks may appear flattened, a
step-off may be palpable between the area of the unaf-
fected lumbar spine and loose posterior elements, the
torso may appear short, and an olisthetic scoliosis may
be present. L5 or S1 nerve root compression symptoms
are rare.
Spondylolisthesis is always evident on an upright lat-
eral radiograph of the lumbosacral junction, but spondy-
lolysis may be difficult to visualize. If suspicion of
spondylolysis is high, oblique lumbar radiographs
should demonstrate sclerosis, elongation, or a distinct
defect in the pars interarticularis. If these radiographs
are not conclusive, single photon emission CT may dem-
onstrate increased uptake in patients with recent
spondylolysis or prefractured stressed regions, but may
be normal in patients with established spondylolysis. CT
will definitively demonstrate the occult fracture that
may not be evident on oblique radiographs. MRI is not
needed for diagnosis.
The two most common types of spondylolysis seen
in children are isthmic and dysplastic spondylolysis. Isth-
mic spondylolysis is more common and represents a fa-
tigue fracture of the pars interarticularis. The dysplastic
type occurs only at L5-S1 and results from congenital
dysplasia of the L5-S1 facet joints. Patients with this
type of spondylolysis have an elongated pars interarticu-
laris and are more prone to developing neurologic
symptoms and deformity during growth.
Traditionally, radiographic sagittal descriptions of
spondylolisthesis have involved the Meyerding classifi-
cation (grades I through IV) and the slip angle. The
Meyerding classification measures the forward transla-
tion of L5 on the sacrum. The slip angle measures the
sagittal rotation of L5 on the sacrum and reflects the lo-
calized kyphosis at this junction, increasing in higher-
grade deformities (Meyerding grade IV or spondy-
loptosis). Pelvic incidence, a newer radiographic
measurement that assesses the sacral anatomy and its
relationship to the pelvis and spine balance, appears to
be more predictive of spondylolisthesis progression. It
describes the obliquity of the sacrum to the pelvis and is
measured on the lateral radiograph by the angle be-
tween the line perpendicular to the middle of the sacral
plate and the line joining the middle of the sacral plate
to the center of the acetabular axis (femoral heads)
(Figure 5). This fixed angle is significantly larger in
those patients with higher-grade deformities (when
compared with those with low-grade slips and control
American Academy of Orthopaedic Surgeons
Orthopaedic Knowledge Update 8 Chapter 66 Pediatric Spinal Deformity

subjects). Pelvic incidence has a strong correlation with

the Meyerding classification; therefore, it may be used
early to predict the ultimate severity of spondylolisthe-
sis in early adolescence.
The risk of progression in spondylolisthesis is great-
est during the adolescent growth spurt, especially for
patients with dysplastic spondylolysis. Findings, such as a
vertical, dome-shaped sacrum, a trapezoidal L5, and a
kyphotic slip angle increase the risk for progression.
Asymptomatic patients with low-grade spondylolis-
thesis do not require treatment or activity restrictions.
Symptomatic patients will need to be counseled regard-
ing their disorder and temporarily curtail participation
in athletic activities. Ultimately, those participating in
high-risk sports are five times more likely to have an
unfavorable clinical outcome. An acute spondylolysis
may heal following the use of a thermoplastic lumbosac-
ral orthosis worn full-time for 6 months, particularly if
the spondylolysis is unilateral. If the pars interarticularis
defect is chronic, a brace can be used until the child is
asymptomatic. Exercises that include hamstring stretch-
ing, pelvic tilts, and abdominal strengthening are begun
when the patient is pain free. Athletic activities may
then be resumed with or without a low-profile antilor-
dotic brace. Should the symptoms recur after a return to
higher levels of activity, the following two options need
to be discussed: (1) discontinue the activity that pro-
duces the symptoms or (2) proceed with surgical treat-
Figure 5 Illustration showing how pelvic incidence in patients with spondylolisthesis
ment to either repair the lytic defect or eliminate move- is determined by the angle between the line perpendicular to the middle of the sacral
ment at the spondylolytic (or spondylolisthetic) segment plate and the line joining the middle of the sacral plate to the center of the acetabular
by a single-level fusion. axis. Pelvic incidence is significantly larger in patients with higher-grade deformities.
Surgical treatment options include repair of a
spondylolytic defect, single-level (or two-level) fusions,
and reduction of higher-grade spondylolisthesis defor- and pedicle screws with hooks/rods over the lamina.
mities. The high-grade deformities are uncommon (1% When a patient with spondylolysis at L5-S1 or low-
of patients), yet much of the recent literature focuses on grade spondylolisthesis requires surgical treatment, a bi-
the treatment of these deformities. Options that have lateral posterolateral L5-S1 in situ fusion remains the
successfully been described in the recent literature in-
gold standard, and it should be done early in patients
clude partial reduction, decompression, and posterior
with a dysplastic lesion because of the high propensity
lumbosacral fixation; additional anterior column sup-
for progression. Patients with higher-grade spondylolis-
port; and even in situ posterolateral fusion followed by
thesis may require fusion extending to L4. Postoperative
cast immobilization. Partial (or complete) reduction and
brace immobilization may be used for comfort. These
instrumentation should be undertaken only by experi-
treatment options will successfully manage most adoles-
enced spine surgeons (see chapter 45 for more in-depth
cent patients.
information regarding the treatment of adult patients
with spondylolisthesis).
Direct repair of spondylolytic defects in patients Scheuermanns Kyphosis
without spondylolisthesis is usually reserved for those Scheuermanns disease represents an exaggerated struc-
patients with defects at L4 or L3. Defects that occur at tural kyphosis involving the thoracic spine. The primary
L5 can be effectively treated by single-level in situ report of poor posture in the adolescent is commonly
L5-S1 fusion. If a direct repair is considered, preopera- accompanied by a dull, aching, midscapular, nonradiat-
tive MRI should be done to rule out adjacent disk pa- ing discomfort. Physical examination demonstrates an
thology. A variety of techniques have been described, in- increased, inflexible thoracic kyphosis, which is most ev-
cluding screws across the pars defects, compression ident during forward bending. A compensatory lumbar
wires from transverse process to the spinous process, hyperlordosis is common, but it remains debatable
pedicle screws with wires around the spinous process, whether this leads to an increased incidence of spondy-

American Academy of Orthopaedic Surgeons 783

Pediatric Spinal Deformity
lolysis. Mild scoliosis is present in 15% of patients, but
the neurologic examination is almost always normal.
Radiographic features of Scheuermanns disease in-
clude kyphosis exceeding 45, with the apex usually in
the middle to lower thoracic spine. If the exaggerated
kyphosis is mild, then other accompanying features
must be recognized to differentiate Scheuermanns dis-
ease from postural kyphosis. These include anterior
wedging of three or more adjacent vertebrae in the api-
cal region, end plate irregularities, and narrowing of disk
spaces. Schmorls nodes (herniation of the disk into the
vertebral end plate) are occasionally present. Hyperex-
tension lateral radiographs are also helpful in differenti-
ating Scheuermanns disease from postural kyphosis be-
cause the apical region in patients with Scheuermanns
disease will remain relatively inflexible. Preoperative
MRI can be used to rule out disk herniation if neces-
sary.
Nonsurgical treatment consists of exercises and brac-
ing. Although exercises can help alleviate thoracic and
lumbar discomfort, they will not result in improvement
of the kyphosis. Bracing has been reported to be effec-
tive in improving moderate deformity, but lack of pa-
tient compliance is a primary limitation.
Surgical indications include large deformities
(> 70), curve progression, persistent pain despite non-
surgical treatment, and, most importantly, genuine cos-
metic concerns of the patient. Over the past 15 years,
anterior release followed by posterior instrumentation
and fusion has been most commonly used. However, re-
cent reports documenting the success of posterior fusion
alone, particularly with the use of threaded 4.8-mm
compression rods, may obviate the need for additional
anterior surgery. Progressive localized kyphosis above
or below the fusion can be avoided if the implant ex-
tends from the second thoracic vertebra proximally to
one level beyond the first lordotic lumbar disk space in-
feriorly and if the correction is not excessive (> 50%).
Annotated Bibliography
Idiopathic Scoliosis
Asher M, Min Lai S, Burton D, Manna B: The reliability
and concurrent validity of the Scoliosis Research
Society-22 patient questionnaire for idiopathic scoliosis.
Spine 2003;28:63-69.
Asher M, Min Lai S, Burton D, Manna B: Scoliosis Re-
search Society-22 patient questionnaire: Responsiveness
to change associated with surgical treatment. Spine
2003;28:70-73.
These two studies confirm the reliability and validity of
the SRS-22 questionnaire when compared with the SF-36. The
authors conclude that the SRS-22 is responsive to changes in
the postoperative period.
784
Orthopaedic Knowledge Update 8
Danielsson A: Back pain and function 23 years after fu-
sion for adolescent idiopathic scoliosis: A case control
study. Part II. Spine 2003;28:E373-E383.
This is a study of 142 patients who had posterior spinal fu-
sion and instrumentation using Harrington rods at a minimum
20-year follow-up; these patients were compared with 100 age-
and sex-matched control subjects. Patients with scoliosis had
significantly more degenerative disk changes and lumbar pain
than control subjects (65% versus 47%, respectively). How-
ever, only 25% of patients who underwent fusion had daily
pain, and there were no differences in back function or gen-
eral health-related quality of life. Patients who underwent fu-
sion also reported pain over their iliac crest incisions when it
was made separately.
Dobbs M, Lenke LG, Szymanski DA, et al: Prevalence
of neural axis abnormalities in patients with infantile id-
iopathic scoliosis. J Bone Joint Surg Am 2002;84:2230-
2234.
This multicenter study analyzed 46 consecutive patients
between 1992 and 2000 with infantile idiopathic scoliosis and a
curve magnitude greater than or equal to 20. Ten of the 46
patients (21%) had evidence of neural axis abnormalities on
MRI. Eight of these 10 patients had neurosurgical treatment
of these abnormalities.
Kuklo TR, Lenke LG, Graham EJ, et al: Correlation of
radiographic, clinical, and patient assessment of shoul-
der balance following fusion versus nonfusion of the
proximal thoracic curve in adolescent idiopathic scolio-
sis. Spine 2002;27:2013-2020.
In this retrospective review of 112 patients who underwent
surgical treatment for a double thoracic curve pattern, the au-
thors evaluated T1 tilt, clavicle angle, coracoid height differ-
ence, trapezius length, first rib-clavicle height difference, shoul-
der asymmetry as measured by soft-tissue shadows on
radiographs, and the translation of the structural curves. They
reported that the clavicle angle was the best preoperative pre-
dictor of postoperative shoulder balance.
Lenke LG, Betz RR, Harms J, et al: Adolescent idio-
pathic scoliosis: A new classification to determine extent
of spinal arthrodesis. J Bone Joint Surg Am 2001;83:
1169-1181.
This study introduced the new scoliosis classification,
which includes the following three components: curve type,
lumbar spine modifier, and a sagittal thoracic modifier. The
authors who developed the new system and seven randomly
selected surgeons from the Scoliosis Research Society then
tested its reliability. The interobserver and intraobserver cap
of values for the components of the classification were ana-
lyzed and demonstrated good interobserver and intraobserver
reliability.
Liljenqvist UR, Allkemper T, Hackenberg L, Link TM,
Steinbeck J, Halm HF: Analysis of vertebral morphol-
ogy in idiopathic scoliosis with use of magnetic reso-
American Academy of Orthopaedic Surgeons
 Orthopaedic Knowledge Update 8
nance imaging and multiplanar reconstruction. J Bone
Joint Surg Am 2002;84:359-368.
The authors analyzed the morphology of the thoracic pedi-
cles in 307 vertebrae using MRI in multiplanar reconstruction.
Maximum intravertebral deformity at the apex of the curve
was noted, with transverse endosteal width of the apical pedi-
cles between 2.3 to 3.2 mm on the concavity and 3.9 to 4.4 mm
on the convexity.
Merola AA, Haher TR, Brkaric M, et al: A multi-center
study of the outcomes of the surgical treatment of ado-
lescent idiopathic scoliosis using the Scoliosis Research
Society (SRS) Outcome Instrument. Spine 2002;27:2046-
2051.
This multicenter study measured outcomes following sur-
gical treatment of adolescent idiopathic scoliosis in 242 pa-
tients. Outcome categories included improvement from preop-
erative pain, general self-image, function from back condition,
and level of activity. Overall, the patients were satisfied with
the results of surgery. The authors concluded that preoperative
pain is typically present in patients with adolescent idiopathic
scoliosis, and it improves significantly following surgical treat-
ment.
Newton PO, Betz R, Clements DH, et al: Anterior tho-
racoscopic instrumentation: A matched comparison to
anterior open instrumentation and posterior open in-
strumentation. 70th Annual Meeting Proceedings. Rose-
mont, IL, American Academy of Orthopaedic Surgeons,
2003.
This multicenter study compared three treatment ap-
proaches (thoracoscopic, open anterior, and posterior) for pa-
tients with right thoracic curves. The radiographic and func-
tional outcomes were similar for the three approaches;
however, the patients who had thoracoscopic anterior instru-
mentation and fusion had longer surgical times.
Ouellet JA, LaPlaza J, Erickson M, Birch JG, Burke S,
Browne R: Sagittal plane deformity in the thoracic
spine: A clue to the presence of syringomyelia as a
cause of scoliosis. Spine 2003;28:2147-2151.
Thirty patients with scoliosis and documented evidence of
syringomyelia were compared with 54 patients with adolescent
idiopathic scoliosis and a normal MRI. The authors analyzed
the lateral radiographs for the presence or absence of Dickson
apical lordosis. Apical lordosis was seen in 97% of patients
with adolescent idiopathic scoliosis and normal MRI scan,
whereas only 25% of patients with syringomyelia-associated
scoliosis had apical lordosis.
Pratt R, Webb JK, Burwell RG, Cummings SL: Luque
trolley and convex epiphysiodesis in the management of
infantile and juvenile idiopathic scoliosis. Spine 1999;24:
1538-1547.
The authors reported 5-year follow-up of patients in whom
either a Luque trolley alone or a Luque trolley together with a
convex epiphysiodesis was used to treat progressive infantile
American Academy of Orthopaedic Surgeons
Chapter 66 Pediatric Spinal Deformity
or juvenile idiopathic scoliosis. The authors concluded that
convex epiphysiodesis together with Luque trolley instrumen-
tation may slow down or improve progressive infantile or ju-
venile idiopathic curves.
Richards BS: Delayed infections following posterior spi-
nal instrumentation for the treatment of idiopathic
scoliosis. J Bone Joint Surg Am 1995;77:524-529.
Ten patients (average age, 25 months) with delayed treat-
ment of deep wound infections were observed after undergo-
ing posterior instrumentation for adolescent idiopathic scolio-
sis. The authors reported that the patients usually had
drainage from the wound, fluctuance, and mildly elevated
erythrocyte sedimentation rates and were treated using instru-
mentation removal, primary wound closure, and short-term
administration of antibiotics. The authors also discuss the im-
portance of longer culture incubation to identify the infectious
organisms.
Richards BS, Sucato DJ, Konigsberg DE, Ouellet JA:
Comparison of reliability between the Lenke and King
classification systems for adolescent idiopathic scoliosis
using radiographs that were not premeasured. Spine
2003;28:1148-1157.
Four surgeons analyzed 50 radiographs that had not been
premeasured and assigned a classification using both the King
and the Lenke classification. The intraobserver and interob-
server reliability of the King classification were 83.5% and
68.0%, respectively. These values were similar when only the
Lenke curve types were analyzed; however, when the com-
plete classifications were assigned (curve type, lumbar modi-
fier, and thoracic sagittal modifier), there was fair intraob-
server and interobserver reliability (65.0% and 55.5%,
respectively).
Sucato DJ, Duchene C: The position of the aorta rela-
tive to the spine: A comparison of patients with and
without idiopathic scoliosis. J Bone Joint Surg Am 2003;
85:1461-1469.
Axial T1-weighted MRI scans of the thoracic and lumbar
spine were compared for normal control subjects and patients
with right idiopathic scoliosis. The aorta was positioned more
laterally and posteriorly to the vertebral bodies in patients
with idiopathic scoliosis, which was in line with a well-placed
vertebral body screw and would be in jeopardy of a laterally
misplaced left pedicle screw.
Sucato DJ, Elerson E: A comparison between the prone
and lateral position for performing a thoracoscopic an-
terior release and fusion for pediatric spinal deformity.
Spine 2003;28:2176-2180.
The technique of an anterior thoracoscopic release using a
regular endotracheal tube is described, with ventilation of
both lungs with lower tidal volumes. The patient is positioned
prone. When compared with patients who underwent this pro-
cedure in the lateral position (double lumen endotracheal
tube for single-lung ventilation), the anesthesia preparation
785
Pediatric Spinal Deformity
time and the delay between the anterior and posterior proce-
dure were both shorter and there were fewer respiratory com-
plications (in 0 versus 14.8% of patients).
Suk SI, Kim WJ, Lee SM, Kim JH, Chung ER: Thoracic
pedicle screw fixation in spinal deformities: Are they re-
ally safe? Spine 2001;26:2049-2057.
This study analyzed 462 patients who had 4,604 thoracic
screws placed to treat spinal deformity. Neurologic complica-
tions directly related to the screws occurred in four patients
(0.8%), one of whom had transient paraparesis and three had
dural tears. The authors concluded that thoracic pedicle screw
fixation is safe when treating spinal deformity.
Weinstein SL, Dolan LA, Spratt KF, Peterson KK,
Spoonamore MJ, Ponseti IV: Health and function of pa-
tients with untreated idiopathic scoliosis. JAMA 2003;
289:559-567.
In this prospective natural history study, 117 patients with
untreated scoliosis were compared with 62 age- and sex-
matched control subjects. The minimum follow-up was 50
years, and multiple parameters were evaluated. The probabil-
ity of survival was similar between the two groups, however,
the incidence of shortness of breath and chronic back pain
were greater in patients with scoliosis.
Congenital Spinal Deformities
Basu PS, Elsebaie H, Noordeen MH: Congenital spinal
deformity: A comprehensive assessment at presentation.
Spine 2002;27:2255-2259.
A series of 126 consecutive patients with congenital spinal
deformity were assessed for incidence of intraspinal anomaly.
This incidence was found in 37% of patients (26% of patients
had cardiac defects and 21% had urogenital anomalies). The
authors concluded that MRI and echocardiography should be
an essential part of the evaluation of patients with congenital
spinal deformity.
Campbell RM, Hell-Vocke AK: Growth of the thoracic
spine in congenital scoliosis after expansion thoraco-
plasty. J Bone Joint Surg Am 2003;85:409-420.
Expansion thoracoplasty consists of osteotomizing fused
ribs on the concavity of the spine followed by expansion of the
chest cage using a vertical, expandable prosthetic titanium rib
implant. The authors reported that longitudinal growth of the
spine was achieved using this technique, likely providing addi-
tional volume for growth of the underlying lungs.
Campbell RM, Smith MD, Mayes TC, et al: The charac-
teristics of thoracic insufficiency syndrome associated
with fused ribs and congenital scoliosis. J Bone Joint
Surg Am 2003;85-A:399-408.
This landmark article introduces and defines thoracic in-
sufficiency syndrome, the inability of the thorax to support
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Orthopaedic Knowledge Update 8
normal respiration or lung growth, which is caused by a rare
condition of multiple fused ribs and congenital scoliosis.
Kim YJ, Otsuka NY, Flynn JM, et al: Surgical treatment
of congenital kyphosis. Spine 2001;26:2251-2257.
In this study, 26 patients were retrospectively reviewed.
The authors found a low rate of pseudarthrosis even without
routine augmentation of the fusion mass if instrumentation
was used. They report that although gradual correction of ky-
phosis occurs with growth in patients younger than 3 years of
age with type II and type III deformities after posterior fusion,
it appears to be unpredictable.
Klemme WR, Polly DW, Orchowske JR: Hemivertebral
excision for congenital scoliosis in very young children.
J Pediatr Orthop 2001;21:761-764.
Six children (average age, 19 months) underwent anterior-
posterior hemivertebra excision. Correction was maintained
with plaster immobilization for 3 months. The authors reported
that excellent improvements in the curves were obtained and
maintained at a minimum 2-year follow-up.
McMaster MJ, Singh H: The surgical management of
congenital kyphosis and kyphoscoliosis. Spine 2001;26:
2146-2154.
In this study, 65 patients with congenital kyphosis or ky-
phoscoliosis were treated with spine arthrodesis. The authors
concluded that all patients with type I or type III congenital
kyphosis or kyphoscoliosis should be treated using posterior
arthrodesis before age 5 years and before the kyphosis ex-
ceeds 50. If the kyphosis does not reduce to less than 50, an
anterior release and arthrodesis using strut grafting is needed
before posterior arthrodesis can be done.
Ruf M, Harms J: Hemivertebra resection by a posterior
approach: Innovative operative technique and first re-
sults. Spine 2002;27:1116-1123.
In this retrospective study, 21 consecutive patients with
congenital scoliosis were treated with hemivertebra resection
using a posterior approach only with transpedicular instru-
mentation. Early surgery is recommended to avert severe local
deformities, to prevent secondary structural changes, and to
avert extensive fusions.
Sink EL, Karol LA, Sanders J, et al: Efficacy of periop-
erative halo-gravity traction in the treatment of severe
scoliosis in children. J Pediatr Orthop 2001;21:519-524.
Perioperative halo traction was used in 19 patients, includ-
ing those with congenital scoliosis. The technique improved bal-
ance and frontal and sagittal alignment. No neurologic compli-
cations occurred.
Sturm PF, Chung R, Bormze SR: Hemivertebra in
monozygotic twins. Spine 2001;26:1389-1391.
This is a report on two monozygotic female twins with tho-
racic hemivertebrae that led to scoliosis.
American Academy of Orthopaedic Surgeons
 Orthopaedic Knowledge Update 8
Suh SW, Sarwark JF, Vora A, et al: Evaluating congeni-
tal spine deformities for intraspinal anomalies with mag-
netic resonance imaging. J Pediatr Orthop 2001;21:525-
531.
In this study, 41 children with congenital spinal deformities
underwent MRI. Evidence of intraspinal anomalies were visi-
ble for 31% of the patients, including tethered cord, syringo-
myelia, and diastematomyelia. The authors recommend MRI
as part of the initial evaluation, even in the absence of clinical
findings.
Spondylolysis and Spondylolisthesis
Grzegorzewski A, Kumar SJ: In situ posterolateral spine
arthrodesis for grades III, IV, and V spondylolisthesis in
children and adolescents. J Pediatr Orthop 2000;20:506-
511.
This study reports on 21 patients who underwent in situ
posterolateral L4-S1 fusions to treat severe spondylolisthesis,
followed by pantaloon cast for 4 months. The authors report
satisfactory results using this technique.
Hanson DS, Bridwell KH, Rhee JM, Lenke LG: Corre-
lation of pelvic incidence with low and high-grade isth-
mic spondylolisthesis. Spine 2002;27:2026-2029.
In this study, pelvic incidence, a fixed angle in an individ-
ual, was reported to be significantly higher in patients with
low-grade and high-grade isthmic spondylolisthesis when com-
pared with control subjects and correlated significantly with
the Meyerding grades of severity.
Lenke LG, Bridwell KH: Evaluation and surgical treat-
ment of high-grade isthmic dysplastic spondylolisthesis.
Instr Course Lect 2003;52:525-532.
The authors reported that high-grade isthmic dysplastic
spondylolisthesis should be treated surgically with appropriate
central and foraminal decompressions at the L5-S1 level, fol-
lowed by lumbosacral fusion. Partial reduction (to improve
the slip angle) provides less risk to the L5 nerve root than
complete reduction. Anterior and posterior fusion at L5-S1
appears to provide the best long-term results.
Scheuermanns Kyphosis
Johnston CE, Sucato DJ, Elerson E: Correction of ado-
lescent hyperkyphosis with posterior-only threaded rod
compression instrumentation. 38th Annual Scoliosis Re-
search Society Meeting Manual. Quebec, Canada, Scolio-
sis Research Society, 2003, p 121.
In this study, threaded 4.8-mm posterior compression rods
were used to treat 14 patients with thoracic kyphosis (average
kyphosis, 78.6 preoperatively). Anterior release was not per-
formed. Correction to 40 was maintained 2.5 years postopera-
tively. The authors concluded that anterior spinal fusion is not
necessary when kyphosis is corrected using this technique.
American Academy of Orthopaedic Surgeons
Chapter 66 Pediatric Spinal Deformity
Papagelopoulos PJ, Klassen RA, Peterson HA, et al:
Surgical treatment of Scheuermanns disease with seg-
mental compression instrumentation. Clin Orthop 2001;
386:139-149.
Twenty-one patients with kyphotic deformities of 50 or
greater underwent posterior compression instrumentation.
Seven patients also had anterior release. The authors con-
cluded that the use of the posterior procedure by itself pro-
vided significant correction, thereby avoiding the development
of any secondary deformity in most patients.
Poolman RW, Been HD, Ubags LH: Clinical outcome
and radiographic results after operative treatment of
Scheuermanns disease. Eur Spine J 2002;11:561-569.
In this study, 23 patients underwent combined anterior and
posterior fusion of their kyphotic deformities. On extended
follow-up, thoracic kyphosis significantly increased, which was
thought to be caused primarily by removal of the posterior im-
plant. This occurred despite solid fusions being shown at the
time of implant removal. Use of the SRS-22 questionnaire
showed only fair outcomes after surgical intervention, leading
the authors to question the indications for surgery.
Stotts AK, Smith JT, Santora SD, et al: Measurement of
spinal kyphosis: Implications for the management of
Scheuermanns kyphosis. Spine 2002;27:2143-2146.
In this study, a broad range of intraobserver and interob-
server differences occur in the measurement of thoracic ky-
phosis in patients with Scheuermanns disease.
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Metha MH: The rib-vertebra angle in the early diagnosis
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Nachemson AL, Peterson L-E: Effectiveness of treat-
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