CURRENT ISSUES
Congenital Scoliosis
A Review and Update
Daniel Hedequist, MD and John Emans, MD
Abstract: Vertebral anomalies causing congenital scoliosis are
classified on the basis of failures of formation, segmentation, or both.
The natural history depends on the type of anomaly and the location
of anomaly. Patient evaluation focuses on the history and physical
examination, followed by appropriate imaging modalities. The
hallmark of surgical treatment is early intervention before the
development of large curvatures. The surgical treatment of a
congenital deformity mandates the use of neurological monitoring to
minimize the risk of perioperative neurological deficit. Modern
surgical techniques have evolved to include the routine use of spinal
instrumentation. Patients with associated chest wall deformities or
large compensatory curves may be candidates for vertical expansion
prosthetic titanium rib placement or growing rods insertion to
maximize growth.
Key Words: congenital scoliosis, surgical treatment, VEPTR
(J Pediatr Orthop 2007;27:106Y116)
T he prevalence rate of congenital scoliosis is thought to be
approximately 1 in 1000 live births.1,2 There is currently
no known cause for the development of a congenital vertebral
anomaly. Strong evidence based on basic science research in
mice suggests that maternal exposure to toxins, such as
carbon monoxide exposure, may cause congenital scolio-
sis.3,4 Associations with maternal diabetes and ingestion of
antiepileptic drugs during pregnancy have also been postu-
lated as possible causes.5,6 Genetic inheritance has been
shown responsible for some congenital vertebral anomalies;
however, there is no clear-cut genetic etiology of congenital
scoliosis to date.2,7,8 Although fetal imaging modalities, such
as magnetic resonance imaging (MRI) and ultrasound, have
improved our ability to diagnose vertebral anomalies in utero,
they do not have any therapeutic role in the clinical
setting.9,10 The goal of treatment of congenital scoliosis is
early diagnosis and treatment, if indicated. Modern imaging
modalities have improved our diagnostic capabilities and our
ability to screen for spinal dysraphism.11,12 Surgical treat-
ment revolves around early arthrodesis for progressive
deformities and has evolved to include the routine use of
spinal instrumentation. Patients with congenital rib fusions in
From the Childrens Hospital Boston, Harvard Medical School, Boston, MA.
The authors state that they no proprietary interest in the products named in
this article.
Reprints: Daniel Hedequist, MD, Childrens Hospital Boston, 300 Longwood
Ave, Department of Orthopedics, Hunnewell 2, Boston, MA 02114. E-mail:
daniel.hedequist@childrens.harvard.edu.
Copyright * 2007 by Lippincott Williams & Wilkins
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concert with congenital scoliosis are at risk of severe
restrictive lung disease and of thoracic insufficiency.13
Expansion thoracoplasty and placement of vertical expansion
prosthetic titanium rib (VEPTR) devices have evolved into a
treatment option for children with the most difficult problems.
The purpose of this article is to serve as a review and update
of congenital scoliosis.
CLASSIFICATION
Vertebral anomalies causing congenital scoliosis may
be caused by a failure of formation, by failure of segmenta-
tion, or by a combination of these 2 factors, resulting in a
mixed deformity.14 An incomplete failure of formation leads
to a wedge vertebra (Fig. 1). A wedge vertebra has asymmetry
in height, with 1 side being hypoplastic; however, there are
bilateral pedicles. Complete failure of formation results in a
hemivertebra, with the absence of 1 pedicle and a region of
vertebral body. Hemivertebra may be further classified on the
basis of the presence or the absence of fusion to the vertebral
bodies above and/or below.15 An unsegmented hemivertebra
is fused to the vertebral body above and below; a partially
segmented hemivertebra is fused to the vertebral body either
above or below; and a fully segmented hemivertebra is
separated from the body above and below by disk space.
Hemivertebra may occur at ipsilateral adjacent levels of the
spine, which produces significantly asymmetrical spine
growth, or a hemivertebra may be counterbalanced by a
hemivertebra on the contralateral side of the spine in the same
region, separated by 1 or several healthy vertebrae (this is
termed a hemimetameric shift).16
The defects of segmentation are characterized by
abnormal bony connections between vertebrae (Fig. 2).
These bony connections may be bilateral and symmetrical,
resulting in a block vertebra. Segmentation defects caused by
unilateral bony fusions are termed bars and may act as a
unilateral growth tether. Occasionally, a segmentation defect
may span an ipsilateral formation defect, resulting in a
unilateral bar and a contralateral hemivertebra.17
Mixed deformities are common and may be difficult to
define, given the abnormal anatomy and the resultant occa-
sionally severe deformity.18 Scoliosis caused by multiple
vertebral anomalies may also be associated with rib abnorm-
alities; this may be associated with severe stunting of thoracic
volume and a restriction of pulmonary function.13,19
NATURAL HISTORY
The progression of congenital scoliosis depends on both
the type and the location of the vertebral anomaly.18 Curve
progression is caused by unbalanced growth of 1 side of the
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J Pediatr Orthop & Volume 27, Number 1, January/February 2007
FIGURE 1. Schematic representation of formation failures. A,
Wedge vertebra. B, Fully segmented hemivertebra. C, Partially
segmented hemivertebra. D, Unsegmented hemivertebra.
Reproduced with permission from Hedequist and Emans.14
spine relative to the other. Radiographically, definable disks
signify the presence of vertebral growth plates and, when
asymmetrical or more present on 1 side of the spine than on
the other, have potential for asymmetrical growth in that area
of the spine. Thus, fully segmented hemivertebra with
healthy, definable disks above and below have much more
potential to cause curvature compared with an unsegmented
hemivertebra, which is fused to the vertebra above and
below.15 Likewise, the asymmetrical tethering of the spine
leads to curvature with growth, as is seen with bars or rib
fusions on the concavity of a curve.
The rate of curve progression depends on the type of
anomaly, the age of the patient, and the location of the curve
(Fig. 3). Curve progression occurs more rapidly during the
first 5 years of life and, again, during the adolescent growth
period of puberty; these 2 periods represent the most rapid
stages of spine growth.20 Anomalies at the cervicothoracic
and lumbosacral junctions produce more visible deformities
FIGURE 2. Schematic representations of failures of
segmentation. A, Block vertebra. B, Bar. C, Bar with
contralateral hemivertebra. Reproduced with permission
from Hedequist and Emans.14
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Congenital Scoliosis: A Review and Update
FIGURE 3. Radiograph, taken from an adolescent patient,
of an untreated lumbar hemivertebra causing progressive
deformity.
than those seen at other areas of the spine. The anomaly most
probable to produce the most severe scoliosis is the unilateral
bar with contralateral hemivertebra, followed by a unilateral
bar, a hemivertebra, a wedge vertebra, and, finally, the most
benign of all anomaliesVthe block vertebra.18 Mixed
deformities are unpredictable, and their severity depends on
the amount of unbalanced growth potential.
PATIENT EVALUATION
The evaluation of a patient with congenital scoliosis
focuses on the physical examination, the search for other
anomalies, and radiographic evaluation. The physical exam-
ination should start with the height and the weight of the
patient, given that growth plays a significant role in curve
progression. The skin needs to be evaluated for any evidence
of spinal dysraphism, such as abnormal pigmentation, hairy
patches, or skin tags over the cutaneous region of the spine.
Spinal dysraphism may also manifest itself in the lower
extremities, and signs would include asymmetrical calves,
cavus feet, clubfeet, vertical tali, and abnormal neurological
findings. The spinal examination itself focuses on any
evidence of truncal or pelvic imbalance. Rib cage deformities
and anomalies need to be evaluated, as does the inspiratory
and expiratory capacity of the chest wall, given the possibility
of any associated restrictive lung disease. Spinal balance in
both the coronal and the sagittal planes needs to be evaluated.
Truncal imbalance, head tilt, shoulder inequality, and pelvic
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Hedequist and Emans J Pediatr Orthop & Volume 27, Number 1, January/February 2007

balance all need to be addressed and recorded. Given the

association of neural axis abnormalities and the possibility of
neurological compromise in congenital spine deformities, a
thorough neurological examination of strength, sensation, and
reflexes, including abdominal reflexes, becomes mandatory.

ASSOCIATED ANOMALIES
Neural axis abnormalities are present in up to 35% of
patients, as detected with MRI.21 These abnormalities include
(but are not limited to) diastematomyelia (split cord), cord
tethering, Chiari malformations, and intradural lipomas. The
absence of cutaneous signs of dysraphism and the absence of
neurological deficit do not rule out an intraspinal dysraphism.
Congenital heart disease is observed in up to 25% of
patients with congenital scoliosis.22 The abnormalities may
be benign and may be detected during a routine preoperative
appointment; however, they may be severe, and the child may
have an already extensive cardiac history. The cardiac defects
range from atrial and ventricular septal defects, which are the
most common abnormalities, to complex congenital heart
defects, such as tetralogy of Fallot and transposition of the
great vessels. Patients who are undergoing an operation for a
congenital spine deformity need a screening echocardiogram, FIGURE 4. Three-dimensional CT scan showing the anatomical
with referral to a cardiologist if indicated. detail of a lumbar partially segmented hemivertebra.
Genitourinary anomalies are observed in up to 20% of
radiation that children receive during CT examination, and
patients with congenital scoliosis.22 The abnormalities may
surgeons should urge that these protocols be used. The ability
be asymptomatic and may be detected on a routine screening
to create 3-dimensional images is software based and does not
test, or they may be significant enough to have already been
require additional radiation exposure. Hedequist and
diagnosed and have required treatment. Anomalies may
Emans,11 in a retrospective study comparing the findings at
affect the kidneys, ureters, bladder, or urethra. These include
operation compared with the findings seen on preoperative
horseshoe kidney, renal aplasia, duplicate ureters, and
radiographs and CT scans, concluded that CT scans were
hypospadias. A renal ultrasound remains to be the criterion
100% accurate in defining the anatomy and the unrecognized
standard for urological screening in these patients, and an
anomalies not seen on plain films. Newton et al27 found that
abnormal ultrasound result demands a referral to a urologist.
in 17 of 31 patients with congenital spine deformities, CT
Musculoskeletal anomalies occur frequently in associa-
scanning with image reformatting allowed for the identifica-
tion with congenital spine anomalies. Disorders, such as
tion of unrecognized malformations not seen on plain films
clubfeet, Sprengel deformity, Klippel-Feil deformity, devel-
alone. Preoperative CT scans help in clearly defining the
opmental dysplasia of the hip, and upper and lower limb
anatomy and avoid any unexpected encounters with posterior
deformities, need to be evaluated and treated appropriately if
element deficiencies at the time of surgical intervention.
present in these patients.
Computed tomography scans that include the chest and
ribs are useful for the evaluation of chest wall deformity and
IMAGING lung volume in congenital deformities with chest wall anoma-
Plain radiographs remain a reliable standard for lies, chest deformity, or thoracic insufficiency. Smith et al61
diagnosis of congenital anomalies and for following curve were able to use 3-dimensional CT data to define lung volumes
progression.23 The details of vertebral anomalies may be in patients who were too young for pulmonary function tests;
particularly evident on plain x-ray results of the infant. The subsequently, they were able to use this data as a measure of
advent of computed tomography (CT) and MRI have improvement in lung function after expansion thoracoplasty.
improved our ability to study spinal anatomy and to screen Others have used this tool to measure improvements in chest
for spinal dysraphism.11,21,24 wall, lung volume, and spinal growth measurements after
We routinely use CT with 3-dimensional reconstruc- expansion thoracoplasty.29,30
tions for preoperative assessment and evaluation of complex Magnetic resonance imaging has replaced myelogram
deformities, but not for routine observation or serial as the procedure of choice in detecting occult spinal
documentation11 (Fig. 4). Concern exists regarding the dysraphism. The efficacy of MRI has been widely studied
significant radiation exposure during CT examination.25,26 and documented in patients with congenital scoliosis.12,21
Tube current (in milliamperes), kilovoltage, and, particularly, The prevalence rate of spinal dysraphism detected using MRI
slice thickness all contribute to the amount of radiation that a approaches 30% in patients with congenital spine deformi-
patient receives in the CT scanner.11,25 Most probably, ties. We do not routinely instruct to perform an MRI on all
institutions do have written protocols in place to minimize the patients with congenital spine deformities; however, patients

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J Pediatr Orthop & Volume 27, Number 1, January/February 2007
FIGURE 5. A, Preoperative MRI scan taken from a patient with
congenital kyphoscoliosis causing cord compression (arrow)
and myelopathy. B, Postoperative MRI scan showing the
decompression of the spinal cord after partial apical
vertebral resection (arrow).
with progressive deformities, major extremity anomalies,
abnormal reflexes, or neurological deficits are best evaluated
with a spinal MRI. We have also found magnetic resonance
imaging helpful in documenting any canal stenosis or cord
impingement in patients with kyphoscoliosis, both as a
preoperative and a postoperative measure to determine the
efficacy of decompression (Fig. 5).
Patients undergoing operative treatment of their spinal
deformity may benefit from specialized radiographs before
surgery to determine the flexibility of the spine. These
radiographs include traction views, push-prone views, supine
bending films, and films over a bolster. These curves help
determine flexibility and help determine the stable vertebra
for instrumentation.
SURGICAL PRINCIPLES
Surgical treatment of patients with congenital spinal
deformities carry a risk of neurological injury greater than
that of patients with idiopathic spinal deformity.31 The
occurrence of a perioperative neurological deficit may be
minimized in multiple ways, the first being the routine use of
MRI evaluation of the spinal cord. The risk of neurological
injury from surgical manipulation of a congenital spine
deformity with an associated spinal cord anomaly may be
reduced by earlier treatment of the spinal cord anomaly.
Occasionally, neurosurgical treatment may be performed at
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Congenital Scoliosis: A Review and Update
the same time as the deformity surgery; however, this
depends on the magnitude of each procedure and the opinion
of the neurosurgeon.32
Early and aggressive treatment of deformities before
they become severe helps in minimizing the risk to the
patient. The avoidance of lengthening the spinal cord
intraoperatively by avoiding intraoperative distraction and
by using shortening procedures during surgery also mini-
mizes the chance of a perioperative neurological deficit. The
use of controlled hypotension to minimize blood loss should
be monitored carefully to minimize the occurrence of
unwanted cord ischemia, especially during any corrective
maneuvers.
Motor- and sensory-evoked potential monitoring is
suggested whenever possible for any surgical case. There is
an increased risk of a perioperative neurological injury when
baseline monitoring cannot be established.33 The intraopera-
tive changes in neurological monitoring that do not return to
baseline may be investigated further by performing a wake-
up test.34,35 At our institution, we also perform a wake-up test
at the end of each deformity case to minimize any chance of a
neurological deficit. The ability to perform wake-up tests
even on younger patients has been shown effective.36 The
postoperative monitoring of a patient`s neurological status
also remains paramount, given that paraplegia after deformity
surgery may present in a delayed fashion, especially in the
first 72 hours.35,37
SPINAL INSTRUMENTATION
The use of spinal instrumentation for congenital spine
deformities has evolved since the description by Hall et al38 in
1981. Newer, downsized implants are available, whereas
titanium implants have allowed for increased MRI compat-
ibility. Hedequist et al39 studied the use of downsized
instrumentation in patients with congenital spine deformities.
In that series, the average patient was aged 3.3 years, with a
preoperative Cobb measurement of 41 degrees; all patients
were treated with an instrumented fusion. There were no
neurological complications; the implant maintained correc-
tion in the coronal plane and, at follow-up after more than
2 years, there were no pseudarthroses. They concluded
that instrumentation was safe and effective in congenital
scoliosis even in the youngest of patients. This data was
further elucidated in a study of 103 patients with congenital
spine deformities treated with instrumentation at an age
younger than 18 years. In this series, the pseudarthrosis
rate and the curve correction were similar to those in the
previously described series, with no reported neurological
abnormalities.40
The efficacy of instrumentation in congenital deformity
surgery was supported by Ruf and Harms41 who studied
newer generation implants in younger patients during poste-
rior-only hemivertebra resection. They found that instrumen-
tation could be safely used in this population. They further
studied spinal implants in younger patients by looking at the
efficacy of pedicle screws in patients younger than 2 years.42
Screw insertion was safe and feasible in 1-year-old patients;
in addition, they found no instances of canal stenosis
associated with instrumentation crossing the neurocentral
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synchondrosis. Kim et al,43 in a study of patients with
congenital kyphosis, found that the use of spinal implants
increased union rate and maintained curve correction bet-
ter than in the case of uninstrumented patients. Thus, we
recommend that newer, downsized implants be safely used
in patients with congenital spine deformities. We also
recommend that implants, if at all possible, should be
titanium, given the increased use of MRI for monitoring not
only spinal dysraphism but also cardiac disease and
genitourinary anomalies.44
FUSION IN SITU
In situ fusion is a safe technique and a good choice for
many progressive curves with minimal deformity involving a
relatively short section of the spine.45 Prophylactic in situ
fusion may be justified for fully segmented hemivertebra,
given that the rates of progression for these deformities are
extremely high.15 In situ arthrodesis over a short segment of
the spine for these types of deformity is associated with only
limited loss in spinal height and good long-term results, even
when performed in younger children.46 Successful arthrod-
esis is based on thorough facet resection, decortication, and
placement of abundant bone graft. The use of spinal
instrumentation has been shown safe and efficacious in
younger children with congenital scoliosis, probably
increases the fusion rate, and may diminish the time needed
in a brace or a cast.39 Given the paucity of graft available
from the iliac crest in smaller children, allograft is an
attractive alternative and has been shown effective in
obtaining fusion.40
The need for an anterior fusion with disk excision and a
posterior in situ fusion and arthrodesis depends on the growth
potential of disks viewed anteriorly, the amount of growth
remaining, and the magnitude and direction of curvature. The
disk quality and, by inference, the growth potential of the
adjacent vertebral endplates may be evaluated using plain
radiographs and preoperative MRI and CT scans. Although
the disk spaces are frequently small remnants, the failure to
perform an anterior procedure in the face of healthy disks
may lead to the crankshaft phenomena, with progression of
curvature in the face of a solid posterior arthrodesis.47,48 In
lordotic deformities, anterior-only in situ fusion may be
preferable and sufficient to arrest progression. Kyphotic
deformities may profit most from posterior-only fusion, with
anticipated anterior growth and slow deformity improvement
occurring relative to a posteriorly created tether. The anterior
procedure may be performed either through an anterior, open
technique, thoracoscopically, or through a posterior approach
via the pedicles, depending on the location of the anomaly
and the preference of the surgeon.49,50
CONVEX HEMIEPIPHYSIODESIS
Convex hemiepiphysiodesis, as the name implies, is a
partial growth arrest procedure. For this procedure to be
effective, little or no concave growth potential. Thus, failures
of segmentation with no little or no growth potential cannot
be successfully treated this way. The most common
indication is a unilateral failure of formation, a hemivertebra.
Much of the correction associated with this procedure is
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& Volume 27, Number 1, January/February 2007
achieved acutely at the time of the initial procedure using a
corrective postoperative cast. The total correction obtained by
performing a convex hemiepiphysiodesis varies because the
younger the child at the time of the operation, the more
potential that exists for correction over time. In general, this
procedure should be reserved for patients younger than 5 years
who have modest deformity, given that the long-term results
yield less than 15 degrees of total correction, with some
patients obtaining no correction.51 Possibly more predictable
alternatives to convex hemiepiphysiodesis include hemiver-
tebra resection or wedge resection, when the deformity
involves a short segment of spine, and growth-oriented proce-
dures, such as VEPTR placement or growing rods insertion,
when a longer segment of spine is involved.
HEMIVERTEBRA EXCISION
Hemivertebra excision remains a safe and effective tool
for treating an isolated hemivertebra that produces curve
progression and causes truncal imbalance. The options of in
situ fusion and convex epiphysiodesis have been shown
reliable at obtaining a growth arrest and stopping curve
progression; however, they afford no correction of deformity
and truncal imbalance.51,52 The optimal indication for
hemivertebra resection remains the same: a patient younger
than 5 years with a thoracolumbar, lumbar, or lumbosacral
hemivertebra and associated truncal imbalance. The surgical
technique of hemivertebra excision varies from staged
anterior and posterior procedures to isolated posterior
wedge resections, with the decision based on the experience
and preference of the surgeon.53,54
The excision of a hemivertebra may be performed by
using combined anterior and posterior procedures. This
technique allows for the circumferential exposure of the
spine, with the ability to obtain a complete excision of the
disks above and below the hemivertebra. Anterior and
posterior exposure of the spine may be performed as
sequential procedures under a single anesthetic. Although
this affords excellent visualization, the operative time tends
to be longer, given the magnitude of the surgery and the need
to reposition and drape the patient.55 Anterior and posterior
exposure has been shown effective for hemivertebra excision
when performed as simultaneous procedures. Hedequist et
al56 reported on their series of 18 patients treated by means of
simultaneous exposures with excision and instrumentation.
The average age of the patients was 3 years, with an average
curve correction of 70%. There were no neurological
complications, and all patients obtained fusion from the
index operation.
Posterior-only hemivertebra excision in growing chil-
dren has recently been reported with successful results.54,57
We have found the ideal indication to be the hemivertebra
located at the thoracolumbar junction or in the lumbar spine,
with some associated kyphosis. Ruf and Harms54 reported
their results on hemivertebra excision using posterior-only
approach and segmental transpedicular instrumentation. They
reported excellent results in patients younger than 6 years,
with an average Cobb measurement of 45 degrees. At 3.5 years
follow-up, the Cobb measurement had been maintained at
14 degrees, with no patient having a neurological complication.
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J Pediatr Orthop & Volume 27, Number 1, January/February 2007
Shono et al58 reported on their experience involving 12 pa-
tients treated with hemivertebra excision and segmental
instrumentation during adolescence. Their correction rate was
64%, with all patients obtaining fusion and no patient having
a neurological deficit. The posterior resection of hemivertebra
is a demanding procedure that may be performed safely by
experienced hands with good correction rate and minimal
neurological risk.
CORRECTION AND FUSION WITH
INSTRUMENTATION
Partial or complete correction of a congenital spine
deformity may be possible by means of arthrodesis and
instrumentation. The partial or complete correction of a
deformity is based on the congenital anomaly itself, the
degree of deformity, and the magnitude of surgery. The stable
zones of operation may be defined by the standing spinal
radiographs, with additional information regarding the
flexibility of the anomaly and the adjacent spine deemed by
bending x-rays or traction x-rays.
Congenital anomalies associated with relatively normal
segmentation, flexibility (as revealed by radiographs), and
less severe truncal deformity may be managed by means of
standard posterior arthrodesis and instrumentation. The use
of modern neurological monitoring techniques and good
surgical technique allows this to be a relatively safe surgical
option for mild to moderate deformities.39,40 The addition of
anterior surgery in these cases may be done in 2 situations
(1) The patient has well-defined disk spaces seen on imaging
FIGURE 6. A, Plain radiograph depicting significant coronal imbalance in a patient with multiple congenital anomalies
(arrows). B and C, Postoperative radiographs depicting coronal and sagittal balance after anteroposterior surgery with osteotomies
and instrumentation.
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Congenital Scoliosis: A Review and Update
studies and has significant remaining growth; this patient
may be at risk of crankshaft and should have either an open
or a thoracoscopic anterior release and fusion.47 (2) The
patient has a moderately sized deformity, has well-defined
disks on imaging studies, and has less flexibility as revealed
by bending radiographs. An anterior procedure with
discectomies and bone grafting performed in these patients
aids in obtaining a well-balanced spine and aids in helping
obtain fusion.
The correction of more severe deformities may be
significantly more challenging with a greater prevalence of
neurological compromise. Osteotomies of vertebral congeni-
tal fusions and bars may aid in correction but are also fraught
with more risks associated with either direct or indirect cord
injury and significant intraoperative hemorrhage.57,59 The
surgery for these deformities may be either combined anterior
and posterior procedures or posterior-only procedures with
instrumentation (Fig. 6). Anterior surgery for more severe
deformities should be performed as an open procedure, with
discectomies and osteotomies at a single level or multiple
levels being performed, depending on the degree of
deformity. The addition of anterior surgery to a posterior
procedure may be conducted at the same anesthetic or as a
separate procedure, depending on the magnitude of surgery.
Posterior-only procedures, such as pedicle subtraction
osteotomies or vertebral column resection, offer correction of
severe deformities without a separate anterior surgical
approach. These procedures are technically demanding and
are associated with significant blood loss and neurological
risk. The use of pedicle subtraction techniques as used in
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Hedequist and Emans
adults for kyphotic deformity may be beneficial.57,59 The
disadvantages of posterior-only procedures include the
difficulty with anterior column visualization during any
period of significant blood loss, the need for spinal cord and
dural manipulation, and the potential for perioperative
displacement at the osteotomy site. Planning of circumfer-
ential osteotomies should be conducted preoperatively by
using 3-dimensional CT to understand the deformity and to
anticipate the anomalous anatomy. As with any procedure
being performed on the spine, the planned osteotomy should
be one that shortens the spinal column and relies on
compression rather than on distraction and lengthening.
Anterior access to the spinal column may be most
readily managed by using a posterior approach when there is
an associated kyphosis or extreme degrees of rotation.57,59Y61
A patient with congenital kyphosis or congenital kyphosco-
liosis may require circumferential treatment for curve
correction and/or decompression. Given the kyphotic nature
of some deformities and the posterior positioning of the apex,
an anterior surgery may be technically unfeasible, given the
difficulty of access in a thoracotomy (Fig. 7). Access to the
anterior column may be provided through a costotransverse-
ctomy, which allows access to the anterior portion of the
spine through a posterior incision. Smith et al61 reported on a
FIGURE 7. Preoperative 3-dimensional CT scan, taken from a
patient with congenital kyphoscoliosis, depicting the potential
difficulty in obtaining apical access through an anterior
approach. This patient was treated with a decompression
procedure via costotransversectomy and fusion with spinal
instrumentation.
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J Pediatr Orthop & Volume 27, Number 1, January/February 2007
series of 16 patients treated in this manner for congenital
kyphosis. Vertebral resection or osteotomies were performed
using a costotransversectomy approach, with satisfactory
results in 13 of 16 patients. In their series, the mean kyphosis
was corrected from 65 to 34 degrees, and instrumentation was
used in all but 1 patient. The authors concluded that for
complex kyphotic deformities of the thoracic spine, costo-
transversectomy should be considered.
TRACTION
Congenital spine deformities occasionally present as
rigid, severe curves, which may be impossible to correct
using standard instrumentation techniques. The safety of
traction in congenital spine deformities, particularly in cases
involving preexisting neurological deficits, has been ques-
tioned in the past reports by MacEwen et al31 with regard to
the risk of traction-induced paraplegia in congenital defor-
mities. Recently, the use of traction has been popularized for
severe deformities, including congenital deformities.62,63 The
use of halo gravity traction has been reported on by Sink
et al62 and then expanded on by Rinella et al63; both series had
no permanent neurological deficits. Halo gravity traction
allows patients to have gradual weight applied to the halo ring
either while in bed or while in a wheelchair or walker device.
Weight is applied daily until partial curve correction is
attained; any evidence of neurological demise calls for
decreasing the traction weight. This technique allows for
some gradual curve correction before an operation. It may be
used before or after an associated anterior release.
Halo-femoral traction has recently been described by
Mehlman et al.64 They used this method of traction after an
associated spinal release and described the technique they
used on 24 patients, with an average pretraction radiograph of
95 degrees and a posttraction radiograph of 44 degrees. The
patients were maintained in traction at an average of 54% of
body weight. The final curve correction was 71% with no
permanent neurological deficits.
FUSIONLESS SURGERY
Patients younger than 5 years who have congenital
deformities involving long sections of the spine or with large
compensatory curves in normally segmented regions present a
great challenge for treatment. In the past, the early arthrodesis
of the spine was thought to be beneficial and to have a
tolerable effect on sitting and trunk height.46 However, recent
studies have shown that early arthrodesis over a section of the
thoracic spine before the age of 5 years may be associated with
a significant reduction in pulmonary function.65
The growth of the spine is greatest during the first
5 years of life as sitting height reaches two thirds of the adult
level by age 5, whereas the thorax has achieved less of its adult
volume. Congenital scoliosis is associated with short stature
and diminished trunk height. Long fusions performed on
younger children may have a further deleterious effect on
trunk height and thoracic volume, leading to thoracic
insufficiency. In the absence of congenital rib fusions, treating
patients with early progressive deformities may best be
conducted by using a growing rod technique.
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J Pediatr Orthop & Volume 27, Number 1, January/February 2007
Surgically, the treatment of deformity by way of
instrumentation without fusion was first pioneered by Paul
Harrington66 in the 1960s. His experiences led him to believe
that a definitive fusion should not be performed before age 10
and that instrumentation without fusion should be considered
in younger patients. Moe et al67 then reported their technique
of limited exposure in the placement of implants, with
resultant growth seen in the instrumented areas of the spine.
Several authors have continued to study this technique and,
recently, Blakemore et al68 reported on the technique they
used on a heterogeneous group of patients with newer
generation implants. They reported improvement on Cobb
angles and sagittal contouring in the patients, although the
final growth measurements were not reported.
The problems associated with a single-rod implant,
including hook dislodgement and rod breakage, have led
Akbarnia et al69 to report their series of a dual growing rod
construct. This is performed by subperiosteal dissection of the
anchor sites proximally and distally and by placement of claw
constructs. Rods are then placed subcutaneously on each side
and joined with tandem connectors placed at the thoraco-
lumbar junction, where the lengthening may occur. Their
patients had an average Cobb angle improvement from 82 to
38 degrees at follow-up and an average growth of the T1-S1
segment of 1.2 cm per year. Thompson et al70 have reported
further on this technique, with encouraging results on dual
rods without apical fusion. Although these reports have not
been about homogenous groups of patients and include
limited numbers of patients with congenital scoliosis, the
results are interesting and the technique merits attention.
For patients with congenital deformities involving long
regions of the spine, we have thought that this procedure is a
good surgical option. At our institution, we place anchors
FIGURE 8. A, Preoperative radiograph taken from a 2-year-old
patient with congenital scoliosis and rib fusions (arrows). Note
the hypoplastic right hemithorax, B, Postoperative radiograph
from the same patient after expansion thoracoplasty
and VEPTR placement. Note the improvement in the volume
of the right hemithorax.
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Congenital Scoliosis: A Review and Update
proximally and distally after subperiosteal dissection and add
bone graft to obtain fusion at the anchor sites. Dual rods,
connected to the anchor points, are placed deep to the
muscular fascia to obtain partial correction, and then
lengthened every 4 to 6 months. We have found this useful
in occasional patients with congenital spine deformities.
Preoperative evaluation via plain film and 3-dimensional CT
scanning has been useful to determine whether the anatomy
will allow for the placement of proximal and distal anchors.
In general, the technique is most useful in patients who have
compensatory curves in normally segmented regions above
and below the congenital anomalies, allowing for anchor
placement and relying on growth through more normally
segmented areas (Fig. 8).
For a patient with progressive curves that either have
congenital anomalies that will not allow for anchor placement
or have associated congenital rib fusions that require
thoracostomy, we will choose expansion thoracostomy and
VEPTR.
EXPANSION THORACOPLASTY AND VEPTR
Congenital spine deformities with rib fusions may be
associated with a constricted thorax, leading to poor thoracic
and lung parenchymal growth and to thoracic insufficiency.
This term has been coined by Dr Robert Campbell to describe
a continuum of problems associated with spine and chest
growth that lead to the inability to support normal lung
function and growth.13 The tethering effect of congenital rib
fusions add to the scoliotic effect of the spine to produce a
concave, constricted hemithorax, which is diminished in
height and function.19 Campbell has also described the
Bwindswept thorax,[ in which progressive thoracic scoliosis
and rotation lead to a foreshortened hemithorax on the
concave side and a collapsed hemithorax on the convex side
of the severe scoliosis. The growth of the lung, respiratory
branches, and alveoli is greatest in the first 8 years of life.
Fifty percent of the thoracic volume is obtained by age 10;
thus, early fusions may have an even more profound effect on
thoracic development than on spine height.20 The possible
effect of early spinal fusion on thoracic development may
compound the preexisting thoracic insufficiency associated
with congenital scoliosis and fused ribs.13 This interdepen-
dent relationship of the spine and the chest wall has led to the
development of expansion thoracoplasty and VEPTR place-
ment as a possible treatment of congenital spine deformities
with associated chest wall anomalies.71,72
The evaluation of these patients is similar to that of all
patients with congenital spine deformities; however, they
need additional studies to document lung volumes and
pulmonary function tests. Younger children are not able to
reliably perform pulmonary function tests; however, their
pulmonary function can be gleamed from the data obtained by
3-dimensional CT scanning.28,73 Gollogly et al28 published a
series on younger patients with thoracic insufficiency who
were being treated with expansion thoracoplasty. Their
results indicated that measurements of lung volumes could
predictably be performed using data obtained from the 3-
dimensional reconstruction of CT scan data. These studies
could also be conducted postoperatively to quantify an
113
Hedequist and Emans
FIGURE 9. A, Radiograph taken from a 4-year-old patient with
a lumbosacral hemivertebra and lumbar congenital vertebral
anomalies (arrows). Note the large amount of curvature
through normally segmented spine. B, Postoperative
radiograph from the same patient after the insertion of bilateral
growing rods with upper thoracic and pelvic anchors.
increase in the volume of lung parenchyma after expansion
thoracoplasty.
Expansion thoracoplasty is a technique that involves
the expansion of the hemithorax by osteotomizing the
segments of congenitally fused ribs or the areas of rib
adhesions between anomalous sections of ribs.72 The surgical
technique involves a standard thoracotomy incision with
division of the latissimus and the serratus. Once exposed, the
areas on constriction may be isolated and then osteotomized
by using a rongeur and a craniotome. Once the osteotomy or
lysis of adhesions is done, the area may be opened with
lamina spreaders. The expansion of the chest and the rib
separation are performed by using VEPTR (Fig. 9). The
VEPTR devices can be attached spanning the ribs (rib to rib
devices) or as hybrid devices (rib to spine, rib to pelvis).
Multiple devices may be used depending on the chest
expansion and the spine control needed.
Campbell et al71 have reported their results on using
this technique in patients with congenital scoliosis and fused
ribs. They found that the patients showed significant
improvement in Cobb measurement of the spine from 74 to
49 degrees. The patients also had an average increase of 8 mm
per year in the length of both the convex and the concave
sides of the spine compared with baseline; the patients also
had an increase in vital capacity. Most complications treated
in the series of Campbell et al were device related and readily
treatable. Emans et al29 reviewed their series on 31 patients
with thoracic insufficiency and fused ribs; they found that the
growth of the thoracic spine after expansion was similar to
that in healthy controls. They also found that the increased
volume of the constricted hemithorax and the total lung
volumes obtained during expansion were maintained at
follow-up. In general, we have found this treatment beneficial
114
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J Pediatr Orthop & Volume 27, Number 1, January/February 2007
to those patients with congenital scoliosis and chest wall
deformities that require surgical intervention to control both
deformities and to allow growth.
SUMMARY
The treatment of congenital scoliosis focuses on early
diagnosis and appropriate surgical management before the
development of large curves. Vertebral anomalies that have a
natural history of progression need to be managed aggres-
sively. All patients with the diagnosis of congenital scoliosis
need a preoperative screening MRI of the spinal axis and a
screening evaluation for renal and cardiac anomalies. The
hallmark of treatment remains to be the early diagnosis before
the development of a large curve. The surgical treatment
options include in situ fusion procedures and convex
hemiepiphysiodesis in those cases where a progressive
curve is present in the face of minimal or no deformity.
Moderate deformities may be partially corrected through
instrumentation and arthrodesis, whereas more severe
deformities may be managed by corresponding osteotomies
or vertebrectomies. Surgical correction of congenital spinal
deformity carries significant risk of neurological injury,
making early, simple treatment preferable. Thoracic insuffi-
ciency syndrome associated with congenital scoliosis and
fused ribs is perhaps best managed during growth by
expansion thoracostomy and insertion of expandable
VEPTR devices. Growing rods may be used in younger
patients with severe curves involving long areas of normally
segmented spine. The use of neurological monitoring is
paramount during any surgical procedure because the goal
remains the same: to achieve a balanced spine with no
resultant neurological deficit.
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