This document provides a review and update on congenital scoliosis. It begins by classifying vertebral anomalies causing congenital scoliosis into failures of formation, segmentation, or both. The natural history and progression of curves depends on the type and location of the anomaly. Patient evaluation focuses on physical exam, imaging to identify the anomaly, and screening for associated anomalies. Surgical treatment aims to intervene early before large curves develop and utilizes modern techniques like spinal instrumentation.

1. CURRENT ISSUES
Congenital Scoliosis
A Review and Update
Daniel Hedequist, MD and John Emans, MD
concert with congenital scoliosis are at risk of severe
Abstract: Vertebral anomalies causing congenital scoliosis are
restrictive lung disease and of thoracic insufficiency.13
classified on the basis of failures of formation, segmentation, or both.
Expansion thoracoplasty and placement of vertical expansion
The natural history depends on the type of anomaly and the location
prosthetic titanium rib (VEPTR) devices have evolved into a
of anomaly. Patient evaluation focuses on the history and physical
treatment option for children with the most difficult problems.
examination, followed by appropriate imaging modalities. The
The purpose of this article is to serve as a review and update
hallmark of surgical treatment is early intervention before the
of congenital scoliosis.
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
CLASSIFICATION
surgical techniques have evolved to include the routine use of spinal Vertebral anomalies causing congenital scoliosis may
instrumentation. Patients with associated chest wall deformities or be caused by a failure of formation, by failure of segmenta-
large compensatory curves may be candidates for vertical expansion tion, or by a combination of these 2 factors, resulting in a
prosthetic titanium rib placement or growing rods insertion to mixed deformity.14 An incomplete failure of formation leads
maximize growth. to a wedge vertebra (Fig. 1). A wedge vertebra has asymmetry
in height, with 1 side being hypoplastic; however, there are
Key Words: congenital scoliosis, surgical treatment, VEPTR bilateral pedicles. Complete failure of formation results in a
(J Pediatr Orthop 2007;27:106Y116)
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
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
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
anomaly. Strong evidence based on basic science research in separated from the body above and below by disk space.
mice suggests that maternal exposure to toxins, such as Hemivertebra may occur at ipsilateral adjacent levels of the
carbon monoxide exposure, may cause congenital scolio- spine, which produces significantly asymmetrical spine
sis.3,4 Associations with maternal diabetes and ingestion of growth, or a hemivertebra may be counterbalanced by a
antiepileptic drugs during pregnancy have also been postu- hemivertebra on the contralateral side of the spine in the same
lated as possible causes.5,6 Genetic inheritance has been region, separated by 1 or several healthy vertebrae (this is
shown responsible for some congenital vertebral anomalies; termed a hemimetameric shift).16
however, there is no clear-cut genetic etiology of congenital The defects of segmentation are characterized by
scoliosis to date.2,7,8 Although fetal imaging modalities, such abnormal bony connections between vertebrae (Fig. 2).
as magnetic resonance imaging (MRI) and ultrasound, have These bony connections may be bilateral and symmetrical,
improved our ability to diagnose vertebral anomalies in utero, resulting in a block vertebra. Segmentation defects caused by
they do not have any therapeutic role in the clinical unilateral bony fusions are termed bars and may act as a
setting.9,10 The goal of treatment of congenital scoliosis is unilateral growth tether. Occasionally, a segmentation defect
early diagnosis and treatment, if indicated. Modern imaging may span an ipsilateral formation defect, resulting in a
modalities have improved our diagnostic capabilities and our unilateral bar and a contralateral hemivertebra.17
ability to screen for spinal dysraphism.11,12 Surgical treat- Mixed deformities are common and may be difficult to
ment revolves around early arthrodesis for progressive define, given the abnormal anatomy and the resultant occa-
deformities and has evolved to include the routine use of sionally severe deformity.18 Scoliosis caused by multiple
spinal instrumentation. Patients with congenital rib fusions in 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
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. NATURAL HISTORY
Reprints: Daniel Hedequist, MD, Childrens Hospital Boston, 300 Longwood
Ave, Department of Orthopedics, Hunnewell 2, Boston, MA 02114. E-mail:
The progression of congenital scoliosis depends on both
daniel.hedequist@childrens.harvard.edu. the type and the location of the vertebral anomaly.18 Curve
Copyright * 2007 by Lippincott Williams & Wilkins progression is caused by unbalanced growth of 1 side of the
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2. J Pediatr Orthop & Volume 27, Number 1, January/February 2007 Congenital Scoliosis: A Review and Update
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 FIGURE 3. Radiograph, taken from an adolescent patient,
fusions on the concavity of a curve. of an untreated lumbar hemivertebra causing progressive
The rate of curve progression depends on the type of deformity.
anomaly, the age of the patient, and the location of the curve
(Fig. 3). Curve progression occurs more rapidly during the than those seen at other areas of the spine. The anomaly most
first 5 years of life and, again, during the adolescent growth probable to produce the most severe scoliosis is the unilateral
period of puberty; these 2 periods represent the most rapid bar with contralateral hemivertebra, followed by a unilateral
stages of spine growth.20 Anomalies at the cervicothoracic bar, a hemivertebra, a wedge vertebra, and, finally, the most
and lumbosacral junctions produce more visible deformities 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
FIGURE 2. Schematic representations of failures of and expiratory capacity of the chest wall, given the possibility
segmentation. A, Block vertebra. B, Bar. C, Bar with of any associated restrictive lung disease. Spinal balance in
contralateral hemivertebra. Reproduced with permission both the coronal and the sagittal planes needs to be evaluated.
from Hedequist and Emans.14 Truncal imbalance, head tilt, shoulder inequality, and pelvic
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3. 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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4. J Pediatr Orthop & Volume 27, Number 1, January/February 2007 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
FIGURE 5. A, Preoperative MRI scan taken from a patient with first 72 hours.35,37
congenital kyphoscoliosis causing cord compression (arrow)
and myelopathy. B, Postoperative MRI scan showing the SPINAL INSTRUMENTATION
decompression of the spinal cord after partial apical The use of spinal instrumentation for congenital spine
vertebral resection (arrow). 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-
with progressive deformities, major extremity anomalies, ibility. Hedequist et al39 studied the use of downsized
abnormal reflexes, or neurological deficits are best evaluated instrumentation in patients with congenital spine deformities.
with a spinal MRI. We have also found magnetic resonance In that series, the average patient was aged 3.3 years, with a
imaging helpful in documenting any canal stenosis or cord preoperative Cobb measurement of 41 degrees; all patients
impingement in patients with kyphoscoliosis, both as a were treated with an instrumented fusion. There were no
preoperative and a postoperative measure to determine the neurological complications; the implant maintained correc-
efficacy of decompression (Fig. 5). tion in the coronal plane and, at follow-up after more than
Patients undergoing operative treatment of their spinal 2 years, there were no pseudarthroses. They concluded
deformity may benefit from specialized radiographs before that instrumentation was safe and effective in congenital
surgery to determine the flexibility of the spine. These scoliosis even in the youngest of patients. This data was
radiographs include traction views, push-prone views, supine further elucidated in a study of 103 patients with congenital
bending films, and films over a bolster. These curves help spine deformities treated with instrumentation at an age
determine flexibility and help determine the stable vertebra younger than 18 years. In this series, the pseudarthrosis
for instrumentation. rate and the curve correction were similar to those in the
previously described series, with no reported neurological
SURGICAL PRINCIPLES abnormalities.40
Surgical treatment of patients with congenital spinal The efficacy of instrumentation in congenital deformity
deformities carry a risk of neurological injury greater than surgery was supported by Ruf and Harms41 who studied
that of patients with idiopathic spinal deformity.31 The newer generation implants in younger patients during poste-
occurrence of a perioperative neurological deficit may be rior-only hemivertebra resection. They found that instrumen-
minimized in multiple ways, the first being the routine use of tation could be safely used in this population. They further
MRI evaluation of the spinal cord. The risk of neurological studied spinal implants in younger patients by looking at the
injury from surgical manipulation of a congenital spine efficacy of pedicle screws in patients younger than 2 years.42
deformity with an associated spinal cord anomaly may be Screw insertion was safe and feasible in 1-year-old patients;
reduced by earlier treatment of the spinal cord anomaly. in addition, they found no instances of canal stenosis
Occasionally, neurosurgical treatment may be performed at associated with instrumentation crossing the neurocentral
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5. Hedequist and Emans J Pediatr Orthop & Volume 27, Number 1, January/February 2007
synchondrosis. Kim et al,43 in a study of patients with achieved acutely at the time of the initial procedure using a
congenital kyphosis, found that the use of spinal implants corrective postoperative cast. The total correction obtained by
increased union rate and maintained curve correction bet- performing a convex hemiepiphysiodesis varies because the
ter than in the case of uninstrumented patients. Thus, we younger the child at the time of the operation, the more
recommend that newer, downsized implants be safely used potential that exists for correction over time. In general, this
in patients with congenital spine deformities. We also procedure should be reserved for patients younger than 5 years
recommend that implants, if at all possible, should be who have modest deformity, given that the long-term results
titanium, given the increased use of MRI for monitoring not yield less than 15 degrees of total correction, with some
only spinal dysraphism but also cardiac disease and patients obtaining no correction.51 Possibly more predictable
genitourinary anomalies.44 alternatives to convex hemiepiphysiodesis include hemiver-
tebra resection or wedge resection, when the deformity
FUSION IN SITU involves a short segment of spine, and growth-oriented proce-
In situ fusion is a safe technique and a good choice for dures, such as VEPTR placement or growing rods insertion,
many progressive curves with minimal deformity involving a when a longer segment of spine is involved.
relatively short section of the spine.45 Prophylactic in situ
fusion may be justified for fully segmented hemivertebra, HEMIVERTEBRA EXCISION
given that the rates of progression for these deformities are Hemivertebra excision remains a safe and effective tool
extremely high.15 In situ arthrodesis over a short segment of for treating an isolated hemivertebra that produces curve
the spine for these types of deformity is associated with only progression and causes truncal imbalance. The options of in
limited loss in spinal height and good long-term results, even situ fusion and convex epiphysiodesis have been shown
when performed in younger children.46 Successful arthrod- reliable at obtaining a growth arrest and stopping curve
esis is based on thorough facet resection, decortication, and progression; however, they afford no correction of deformity
placement of abundant bone graft. The use of spinal and truncal imbalance.51,52 The optimal indication for
instrumentation has been shown safe and efficacious in hemivertebra resection remains the same: a patient younger
younger children with congenital scoliosis, probably than 5 years with a thoracolumbar, lumbar, or lumbosacral
increases the fusion rate, and may diminish the time needed hemivertebra and associated truncal imbalance. The surgical
in a brace or a cast.39 Given the paucity of graft available technique of hemivertebra excision varies from staged
from the iliac crest in smaller children, allograft is an anterior and posterior procedures to isolated posterior
attractive alternative and has been shown effective in wedge resections, with the decision based on the experience
obtaining fusion.40 and preference of the surgeon.53,54
The need for an anterior fusion with disk excision and a The excision of a hemivertebra may be performed by
posterior in situ fusion and arthrodesis depends on the growth using combined anterior and posterior procedures. This
potential of disks viewed anteriorly, the amount of growth technique allows for the circumferential exposure of the
remaining, and the magnitude and direction of curvature. The spine, with the ability to obtain a complete excision of the
disk quality and, by inference, the growth potential of the disks above and below the hemivertebra. Anterior and
adjacent vertebral endplates may be evaluated using plain posterior exposure of the spine may be performed as
radiographs and preoperative MRI and CT scans. Although sequential procedures under a single anesthetic. Although
the disk spaces are frequently small remnants, the failure to this affords excellent visualization, the operative time tends
perform an anterior procedure in the face of healthy disks to be longer, given the magnitude of the surgery and the need
may lead to the crankshaft phenomena, with progression of to reposition and drape the patient.55 Anterior and posterior
curvature in the face of a solid posterior arthrodesis.47,48 In exposure has been shown effective for hemivertebra excision
lordotic deformities, anterior-only in situ fusion may be when performed as simultaneous procedures. Hedequist et
preferable and sufficient to arrest progression. Kyphotic al56 reported on their series of 18 patients treated by means of
deformities may profit most from posterior-only fusion, with simultaneous exposures with excision and instrumentation.
anticipated anterior growth and slow deformity improvement The average age of the patients was 3 years, with an average
occurring relative to a posteriorly created tether. The anterior curve correction of 70%. There were no neurological
procedure may be performed either through an anterior, open complications, and all patients obtained fusion from the
technique, thoracoscopically, or through a posterior approach index operation.
via the pedicles, depending on the location of the anomaly Posterior-only hemivertebra excision in growing chil-
and the preference of the surgeon.49,50 dren has recently been reported with successful results.54,57
We have found the ideal indication to be the hemivertebra
CONVEX HEMIEPIPHYSIODESIS located at the thoracolumbar junction or in the lumbar spine,
Convex hemiepiphysiodesis, as the name implies, is a with some associated kyphosis. Ruf and Harms54 reported
partial growth arrest procedure. For this procedure to be their results on hemivertebra excision using posterior-only
effective, little or no concave growth potential. Thus, failures approach and segmental transpedicular instrumentation. They
of segmentation with no little or no growth potential cannot reported excellent results in patients younger than 6 years,
be successfully treated this way. The most common with an average Cobb measurement of 45 degrees. At 3.5 years
indication is a unilateral failure of formation, a hemivertebra. follow-up, the Cobb measurement had been maintained at
Much of the correction associated with this procedure is 14 degrees, with no patient having a neurological complication.
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6. J Pediatr Orthop & Volume 27, Number 1, January/February 2007 Congenital Scoliosis: A Review and Update
Shono et al58 reported on their experience involving 12 pa- studies and has significant remaining growth; this patient
tients treated with hemivertebra excision and segmental may be at risk of crankshaft and should have either an open
instrumentation during adolescence. Their correction rate was or a thoracoscopic anterior release and fusion.47 (2) The
64%, with all patients obtaining fusion and no patient having patient has a moderately sized deformity, has well-defined
a neurological deficit. The posterior resection of hemivertebra disks on imaging studies, and has less flexibility as revealed
is a demanding procedure that may be performed safely by by bending radiographs. An anterior procedure with
experienced hands with good correction rate and minimal discectomies and bone grafting performed in these patients
neurological risk. aids in obtaining a well-balanced spine and aids in helping
obtain fusion.
The correction of more severe deformities may be
CORRECTION AND FUSION WITH significantly more challenging with a greater prevalence of
INSTRUMENTATION neurological compromise. Osteotomies of vertebral congeni-
Partial or complete correction of a congenital spine tal fusions and bars may aid in correction but are also fraught
deformity may be possible by means of arthrodesis and with more risks associated with either direct or indirect cord
instrumentation. The partial or complete correction of a injury and significant intraoperative hemorrhage.57,59 The
deformity is based on the congenital anomaly itself, the surgery for these deformities may be either combined anterior
degree of deformity, and the magnitude of surgery. The stable and posterior procedures or posterior-only procedures with
zones of operation may be defined by the standing spinal instrumentation (Fig. 6). Anterior surgery for more severe
radiographs, with additional information regarding the deformities should be performed as an open procedure, with
flexibility of the anomaly and the adjacent spine deemed by discectomies and osteotomies at a single level or multiple
bending x-rays or traction x-rays. levels being performed, depending on the degree of
Congenital anomalies associated with relatively normal deformity. The addition of anterior surgery to a posterior
segmentation, flexibility (as revealed by radiographs), and procedure may be conducted at the same anesthetic or as a
less severe truncal deformity may be managed by means of separate procedure, depending on the magnitude of surgery.
standard posterior arthrodesis and instrumentation. The use Posterior-only procedures, such as pedicle subtraction
of modern neurological monitoring techniques and good osteotomies or vertebral column resection, offer correction of
surgical technique allows this to be a relatively safe surgical severe deformities without a separate anterior surgical
option for mild to moderate deformities.39,40 The addition of approach. These procedures are technically demanding and
anterior surgery in these cases may be done in 2 situations are associated with significant blood loss and neurological
(1) The patient has well-defined disk spaces seen on imaging risk. The use of pedicle subtraction techniques as used in
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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7. Hedequist and Emans J Pediatr Orthop & Volume 27, Number 1, January/February 2007
adults for kyphotic deformity may be beneficial.57,59 The series of 16 patients treated in this manner for congenital
disadvantages of posterior-only procedures include the kyphosis. Vertebral resection or osteotomies were performed
difficulty with anterior column visualization during any using a costotransversectomy approach, with satisfactory
period of significant blood loss, the need for spinal cord and results in 13 of 16 patients. In their series, the mean kyphosis
dural manipulation, and the potential for perioperative was corrected from 65 to 34 degrees, and instrumentation was
displacement at the osteotomy site. Planning of circumfer- used in all but 1 patient. The authors concluded that for
ential osteotomies should be conducted preoperatively by complex kyphotic deformities of the thoracic spine, costo-
using 3-dimensional CT to understand the deformity and to transversectomy should be considered.
anticipate the anomalous anatomy. As with any procedure
being performed on the spine, the planned osteotomy should TRACTION
be one that shortens the spinal column and relies on
Congenital spine deformities occasionally present as
compression rather than on distraction and lengthening.
rigid, severe curves, which may be impossible to correct
Anterior access to the spinal column may be most
using standard instrumentation techniques. The safety of
readily managed by using a posterior approach when there is
traction in congenital spine deformities, particularly in cases
an associated kyphosis or extreme degrees of rotation.57,59Y61
involving preexisting neurological deficits, has been ques-
A patient with congenital kyphosis or congenital kyphosco-
tioned in the past reports by MacEwen et al31 with regard to
liosis may require circumferential treatment for curve
the risk of traction-induced paraplegia in congenital defor-
correction and/or decompression. Given the kyphotic nature
mities. Recently, the use of traction has been popularized for
of some deformities and the posterior positioning of the apex,
severe deformities, including congenital deformities.62,63 The
an anterior surgery may be technically unfeasible, given the
use of halo gravity traction has been reported on by Sink
difficulty of access in a thoracotomy (Fig. 7). Access to the
et al62 and then expanded on by Rinella et al63; both series had
anterior column may be provided through a costotransverse-
no permanent neurological deficits. Halo gravity traction
ctomy, which allows access to the anterior portion of the
allows patients to have gradual weight applied to the halo ring
spine through a posterior incision. Smith et al61 reported on a
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
FIGURE 7. Preoperative 3-dimensional CT scan, taken from a
patient with congenital kyphoscoliosis, depicting the potential younger children may have a further deleterious effect on
difficulty in obtaining apical access through an anterior trunk height and thoracic volume, leading to thoracic
approach. This patient was treated with a decompression insufficiency. In the absence of congenital rib fusions, treating
procedure via costotransversectomy and fusion with spinal patients with early progressive deformities may best be
instrumentation. conducted by using a growing rod technique.
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8. J Pediatr Orthop & Volume 27, Number 1, January/February 2007 Congenital Scoliosis: A Review and Update
Surgically, the treatment of deformity by way of proximally and distally after subperiosteal dissection and add
instrumentation without fusion was first pioneered by Paul bone graft to obtain fusion at the anchor sites. Dual rods,
Harrington66 in the 1960s. His experiences led him to believe connected to the anchor points, are placed deep to the
that a definitive fusion should not be performed before age 10 muscular fascia to obtain partial correction, and then
and that instrumentation without fusion should be considered lengthened every 4 to 6 months. We have found this useful
in younger patients. Moe et al67 then reported their technique in occasional patients with congenital spine deformities.
of limited exposure in the placement of implants, with Preoperative evaluation via plain film and 3-dimensional CT
resultant growth seen in the instrumented areas of the spine. scanning has been useful to determine whether the anatomy
Several authors have continued to study this technique and, will allow for the placement of proximal and distal anchors.
recently, Blakemore et al68 reported on the technique they In general, the technique is most useful in patients who have
used on a heterogeneous group of patients with newer compensatory curves in normally segmented regions above
generation implants. They reported improvement on Cobb and below the congenital anomalies, allowing for anchor
angles and sagittal contouring in the patients, although the placement and relying on growth through more normally
final growth measurements were not reported. segmented areas (Fig. 8).
The problems associated with a single-rod implant, For a patient with progressive curves that either have
including hook dislodgement and rod breakage, have led congenital anomalies that will not allow for anchor placement
Akbarnia et al69 to report their series of a dual growing rod or have associated congenital rib fusions that require
construct. This is performed by subperiosteal dissection of the thoracostomy, we will choose expansion thoracostomy and
anchor sites proximally and distally and by placement of claw VEPTR.
constructs. Rods are then placed subcutaneously on each side
and joined with tandem connectors placed at the thoraco- EXPANSION THORACOPLASTY AND VEPTR
lumbar junction, where the lengthening may occur. Their Congenital spine deformities with rib fusions may be
patients had an average Cobb angle improvement from 82 to associated with a constricted thorax, leading to poor thoracic
38 degrees at follow-up and an average growth of the T1-S1 and lung parenchymal growth and to thoracic insufficiency.
segment of 1.2 cm per year. Thompson et al70 have reported This term has been coined by Dr Robert Campbell to describe
further on this technique, with encouraging results on dual a continuum of problems associated with spine and chest
rods without apical fusion. Although these reports have not growth that lead to the inability to support normal lung
been about homogenous groups of patients and include function and growth.13 The tethering effect of congenital rib
limited numbers of patients with congenital scoliosis, the fusions add to the scoliotic effect of the spine to produce a
results are interesting and the technique merits attention. concave, constricted hemithorax, which is diminished in
For patients with congenital deformities involving long height and function.19 Campbell has also described the
regions of the spine, we have thought that this procedure is a Bwindswept thorax,[ in which progressive thoracic scoliosis
good surgical option. At our institution, we place anchors 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
FIGURE 8. A, Preoperative radiograph taken from a 2-year-old
patient with congenital scoliosis and rib fusions (arrows). Note were being treated with expansion thoracoplasty. Their
the hypoplastic right hemithorax, B, Postoperative radiograph results indicated that measurements of lung volumes could
from the same patient after expansion thoracoplasty predictably be performed using data obtained from the 3-
and VEPTR placement. Note the improvement in the volume dimensional reconstruction of CT scan data. These studies
of the right hemithorax. could also be conducted postoperatively to quantify an
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9. Hedequist and Emans 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
FIGURE 9. A, Radiograph taken from a 4-year-old patient with instrumentation and arthrodesis, whereas more severe
a lumbosacral hemivertebra and lumbar congenital vertebral deformities may be managed by corresponding osteotomies
anomalies (arrows). Note the large amount of curvature or vertebrectomies. Surgical correction of congenital spinal
through normally segmented spine. B, Postoperative deformity carries significant risk of neurological injury,
radiograph from the same patient after the insertion of bilateral making early, simple treatment preferable. Thoracic insuffi-
growing rods with upper thoracic and pelvic anchors.
ciency syndrome associated with congenital scoliosis and
fused ribs is perhaps best managed during growth by
increase in the volume of lung parenchyma after expansion expansion thoracostomy and insertion of expandable
thoracoplasty. VEPTR devices. Growing rods may be used in younger
Expansion thoracoplasty is a technique that involves patients with severe curves involving long areas of normally
the expansion of the hemithorax by osteotomizing the segmented spine. The use of neurological monitoring is
segments of congenitally fused ribs or the areas of rib paramount during any surgical procedure because the goal
adhesions between anomalous sections of ribs.72 The surgical remains the same: to achieve a balanced spine with no
technique involves a standard thoracotomy incision with resultant neurological deficit.
division of the latissimus and the serratus. Once exposed, the
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