CHAPTER
107
Congenital Scoliosis
Congenital scoliosis is a lateral curvature of the spine due to
developmental vertebral anomalies that produce a localized
imbalance in the lateral longitudinal growth of the spine
occurring in the frontal plane. These vertebral anomalies are
present at birth, but the clinical deformity may not become
evident until later childhood when the diagnosis is made
radiographically.
It is important to appreciate that congenital scoliosis should
not be regarded as a separate entity but is on the spectrum of
deformities, ranging from a congenital scoliosis through
kyphoscoliosis to a pure kyphosis. These deformities are all due
to developmental vertebral anomalies that produce a localized
imbalance in the longitudinal growth of the spine. However,
the type of spinal curvature that develops depends on whether
the spinal growth imbalance occurs laterally producing a scoli-
osis or anterior or anterolateral to the transverse axis of verte-
bral rotation in the sagittal plane producing a kyphosis or
kyphoscoliosis. Of 750 patients with congenital spine deformi-
ties attending my Edinburgh clinic, I found that 80% had a
scoliosis, 14% had a kyphoscoliosis, and 6 % had a pure kypho-
sis. This chapter deals only with congenital scoliosis. Patients
with a congenital kyphoscoliosis or kyphosis have a different
natural history and require different management.26,27
All degrees of severity of congenital scoliosis are seen at all
ages. Some patients present with small curves that progress
minimally, whereas others progress rapidly to become an
extreme deformity at an early age, and this can, on occasion,
impair lung growth and development. If left untreated until
the teenage years, some patients will have developed a very
severe rigid spinal deformity with spinal imbalance that can be
very difficult to treat surgically.
The skill in managing a patient with a congenital scoliosis
lies not just in the ability to perform major complex spine sur-
gery at a late stage but primarily, and more importantly, in rec-
ognizing those curves that are at risk of rapid deterioration at
an early stage. It is in this patient group that prophylactic surgi-
cal treatment to prevent curve progression is indicated to mini-
mize impairment of pulmonary function. It is much better to
treat a patient at an early stage when the curve is small, rather
than to wait until a severe rigid deformity has developed when
it is necessary to perform a higher-risk salvage procedure.
To plan an appropriate course of treatment, it is necessary
to understand the natural history of congenital scoliosis and
correlate the principles of normal growth of the spine and tho-
rax with the pathological anatomy and growth imbalance pro-
duced by the various types of congenital vertebral anomalies
that may produce a scoliosis.
1118
LWBK836_Ch107_p1118-1138.indd 1118
Michael J. McMaster
PATHOGENESIS
SPINE
Normal longitudinal growth of the spine is the sum total of
growth occurring at the epiphyseal end plates on the upper
and lower surfaces of the vertebral bodies. This growth occurs
symmetrically and as a result the spine remains balanced in the
frontal and sagittal planes. However, in the presence of a con-
genital vertebral anomaly, there is an absence or deficiency in
either the number of growth plates or the rate of growth on
one side of the spine, resulting in a localized longitudinal
imbalance of growth and an increasing spinal curvature as the
child grows.
The rate of deterioration and final severity of a congenital
scoliosis is proportional to the degree of growth imbalance pro-
duced by the vertebral anomalies. In general, the greater the
growth imbalance is, the more severe the deformity. This dete-
rioration continues until skeletal maturity when the growth
plates fuse. However, the rate of spinal growth is not uniform.
There are two periods of accelerated growth during which time
the scoliosis deteriorates more rapidly. The first occurs during
the first 2 years of life and the second during the adolescent
growth spurt, which usually occurs in girls between the ages of
10 and 13 years and in boys 2 years later. It is during these two
periods of accelerated growth that a congenital scoliosis is usu-
ally first diagnosed. On average, 25% of curves are nonprogres-
sive, 25% progress slowly, and 50% progress more rapidly.25
CHEST
The interrelationship between the growth of the spine as well
as the growth of the thoracic cage and the development of the
lungs is now more fully appreciated. At birth, the thoracic cage
is 6.7% of its adult volume after which it enlarges five-fold, up
to 30%, by the age of 5 years. At the age of 10 years, it is 50%
adult size and then doubles to adult volume by skeletal matu-
rity. The volume of the thorax determines the volume of the
lungs.
Lung growth is dependent on the normal increasing tho-
racic cage volume. From birth to the age of 8 years, the lungs
grow by alveolar multiplication with the most rapid phase
occurring in the first 2 years. The development of the ancillary
arteries and capillaries parallels the development of the alveoli.
After the age of 8 years, the number of alveoli remains relatively
unchanged but hypertrophy with normal growth of the thorax
until skeletal maturity.
8/25/11 10:07:27 PM

An increasing thoracic scoliosis occurring in infancy will dis-
tort the rib cage and result in a loss of thoracic volume and the
space available for normal lung growth and development. In
addition, an early and extensive spinal fusion of the thoracic
spine will impair the longitudinal growth in this region and
adversely affect the development of the thorax. Campbell
et al11 have described the development of a thoracic insufficiency
syndrome (TIS), in which there is an inability of the thorax to
support normal lung growth and respiration. This can occur in
patients with a severe early-onset congenital thoracic scoliosis,
especially with congenitally fused ribs on the concavity of the
curve. Davies and Reid13 studied four cadavers with severe rib
cage deformities secondary to scoliosis developing in early
childhood. They found that the alveoli had failed to multiply
and may even have atrophied in the compressed lung. If surgi-
cal treatment is required to control a thoracic scoliosis during
this early period, it should encourage not only longitudinal
growth of the spine but also normal development of the thorax
to facilitate growth and development of the lungs.
After skeletal maturity, normal aging can also adversely
affect pulmonary function. A child with a severely deformed
thorax due to a congenital scoliosis and associated chest wall
anomalies will probably have an additional gradual loss of lung
function with an increased morbidity and possible cardiorespi-
ratory failure in later life.
CLASSIFICATION AND NATURAL
HISTORY
The major advances in the management of congenital scoliosis
have been a better understanding of the natural history of the
condition and the possible adverse effects on lung growth and
Figure 107.1. Congenital scoliosis.
LWBK836_Ch107_p1118-1138.indd 1119
Chapter 107 Congenital Scoliosis 1119
Types of Developmental
TABLE 107.1 Vertebral Anomalies Causing
Congenital Scoliosis
Failure of vertebral segmentation
Unilateral
Unsegmented bar
Unsegmented bar with contralateral hemivertebrae
Bilateral
Block vertebra
Failure of vertebral formation
Complete unilateral
Hemivertebra: fully segmented, semisegmented,
nonsegmented, incarcerated
Partial unilateral
Wedge vertebra
Mixed or unclassifiable vertebral anomalies
knowing when to apply prophylactic surgical treatment before
there is a significant deformity of the spine or chest.
The classification of the congenital vertebral anomalies that
may produce a congenital scoliosis is based on the embryologi-
cal maldevelopment of the spine25,37 (Table 107.1, Fig. 107.1).
There are two basic groups of vertebral anomalies that can pro-
duce a scoliosis. First, there are those caused by a unilateral
failure of vertebral formation, the most common of which is a
hemivertebra. Second, there are those caused by a unilateral
failure of segmentation of two or more vertebrae, which result
in a unilateral unsegmented bar, with or without contralateral
hemivertebrae at the same level. There is also a group of
patients with a complex mixture of defects of vertebral forma-
tion and segmentation that cannot be specifically classified.
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 1120 Section IX Dysplastic and Congenital Deformities
Vertebral anomalies may also be present in other areas of the
spine outside the scoliotic curve, but these are ignored for the
purpose of classification of the scoliosis if they do not contrib-
ute to the deformity.
This classification is based on plain spinal radiographs, which
most clearly identify malformations of the vertebral bodies and
pedicles but do not clearly show the posterior elements. However,
recent developments in three-dimensional (3D) computed
tomography (CT) of the spine have provided more detailed
information with regard to both the anterior and posterior com-
ponents of the malformed vertebrae. This has shown that, in
some patients, there may also be anomalies of the posterior ele-
ments such as partially or fully fused lamina, either unilaterally
or bilaterally, as well as occult spina bifida areas with exposed
neural elements. These anomalies in the posterior elements may
or may not match the anterior anomalies in the vertebral bodies
and may also have an effect on curve progression.
In a study of 560 patients with congenital scoliosis, by using
plain spinal radiographs, I found that the cause of the deformity
was due to one or more hemivertebrae in 38%, a unilateral
unsegmented bar in 30%, a unilateral unsegmented bar with
contralateral hemivertebrae at the same level in 12%, and an
additional 12% of patients could not be classified because they
had a complex mixture of anomalies. Wedged and block verte-
brae are not often seen by themselves as a cause of scoliosis
because the resulting deformity is so small that it is not usually
recognized. These anomalies are more commonly seen as part of
a complex mixture of vertebral anomalies that cannot be specifi-
cally diagnosed but can produce a significant deformity.
Predicting curve progression is not always easy, but some
generalizations can be made in planning an appropriate course
of treatment.
HEMIVERTEBRA
A hemivertebra is the most common cause of a congenital sco-
liosis, but the severity of the deformity varies greatly, and there
is debate as to the necessity and timing of treatment. Opinions
range through the spectrum from total excision to total neglect
of the hemivertebra. To rationalize this problem, it is necessary
to have an understanding of the problems associated with the
different types and sites of hemivertebrae.
The potential for a hemivertebra to cause a significant spinal
deformity depends on three factors. The most important factor
is the pathological anatomy and relationship of the hemivertebra
to the adjacent vertebrae in the spine (Fig. 107.1). The hemiver-
tebra may be fully segmented, which is most common; semiseg-
mented; or incarcerated, which is least common. It is important
to distinguish between these three types because each has a dif-
ferent potential for growth. The severity of the resulting scoliosis
and necessity for surgical treatment is related to the degree of
segmentation. Second, the site of the hemivertebra is important,
especially in those cases that occur at the lumbosacral junction.
Third is the number of hemivertebrae and their relationship to each
other in the spine. Is there a single hemivertebra or two hemiver-
tebrae? Are they on the same side? Or, are they opposing?
FULLY SEGMENTED HEMIVERTEBRA
This type of hemivertebra has a normal disc above and below
and is completely separate from its adjacent vertebrae. There is
LWBK836_Ch107_p1118-1138.indd 1120
an absence of two growth plates on the unformed side, whereas
relatively normal growth occurs on the upper and lower sur-
faces of the hemivertebrae. As the hemivertebra grows, it acts
as an enlarging wedge resulting in an increasing scoliosis. The
hemivertebra may occur anywhere in the spine, including the
lumbosacral junction, where it can cause a major problem.
Without treatment, progression of a scoliosis caused by a single
fully segmented hemivertebra can be difficult to predict and
requires carefully monitoring. However, most curves progress
relatively slowly, at 1 or 2 per year.
The most pernicious and deforming type of hemivertebra
occur at the lumbosacral junction. Here, it causes an oblique
takeoff of the lumbar spine from the sacrum. A compensatory
curve develops in the normal spine above, but this is never suf-
ficient to balance the spine and the patient lists due to trunk
shift to the side opposite the hemivertebra. It is the spinal
imbalance rather than the size of the curve that is the major
problem. Initially, the compensatory curve is mobile and cor-
rectable but, with time, becomes fixed and rotated and becomes
the major deformity. The time to treat these patients is in the
first few years of life, before the compensatory curve becomes
fixed, when the hemivertebra can be excised and the scoliosis
completely corrected, and not at a late stage.
A hemivertebra in the cervical spine or at the cervicothoracic
junction occurs infrequently but can cause tilting of the head.
This is often associated with a congenitally fused block of verte-
brae (a KlippelFeil deformity) occurring above in the cervical
spine restricting movement and preventing the development of
a compensatory curve in this region. The patient attempts to
level the head by either tilting the whole of the upper body to
one side or, alternatively, developing a compensatory curve in
the upper thoracic region. The optimal time to treat these
patients is at an early stage when a localized in situ fusion will
prevent increasing deformity. This is preferable to waiting until
a significant deformity with spinal imbalance has developed
when correction can be achieved only by excision of the
hemivertebra, which, in this region, is a more difficult and
potentially hazardous procedure.
Two unilateral hemivertebrae are less common but have a much
worse prognosis. The hemivertebrae are usually separated by
several normal vertebrae. Here, there is an absence of four
growth plates on one side of the spine, resulting in a much
greater growth imbalance. These curves usually progress 3 to
4 per year, and without treatment, the majority will exceed 50
by the age of 10 years. By skeletal maturity, most curves will be
more than 70. All of these patients require prophylactic surgi-
cal treatment to balance the growth of the spine at an early
stage and before the age of 10 years.
Two opposing hemivertebrae represent a hemimetameric shift
and have a more variable prognosis depending on the type of
hemivertebra and whether the hemivertebrae are close together
or more widely separated in different regions of the spine. If
the hemivertebrae are close together in the same region, they
tend to balance each other and cause only a minimal cosmetic
deformity, and no treatment is required. However, if the
hemivertebrae are more widely separated in different regions
of the spine, the curves are often unbalanced, causing spinal
decompensation, which is much more deforming and requires
prophylactic treatment at an early stage. The most difficult situ-
ation is when there is a hemivertebra at the lumbosacral junc-
tion with another hemivertebra on the opposite side in the
upper lumbar region causing significant spinal imbalance.
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Both of these hemivertebrae may have to be excised to balance
the spine.
SEMISEGMENTED HEMIVERTEBRA
This type of hemivertebra is synostosed to its neighboring ver-
tebra and has only one disc space either above or below. As a
result, two growth plates are obliterated on the convexity, and
this tends to balance the absence of the two growth plates on
the unformed side of the hemivertebra. Although growth of
the spine is theoretically balanced, the hemivertebra can cause
a tilting of the spine and induce a slowly progressive scoliosis.
This does not usually require treatment unless the semiseg-
mented hemivertebra is at the lumbosacral junction where it
may cause spinal imbalance.
INCARCERATED HEMIVERTEBRA
This is a small, ovoid piece of bone lying within a niche in the
spine, which remains straight. The disc spaces above and below
the hemivertebra are usually narrow and poorly formed, indi-
cating a poor growth potential. This type of hemivertebra usu-
ally occurs in the thoracic region. The resulting scoliosis
deteriorates very slowly, if at all, and rarely exceeds 20 at skel-
etal maturity. This does not require treatment.
When treating a patient with a congenital scoliosis caused by
a fully segmented hemivertebra, it is important to appreciate
that it is not possible to create growth on the unformed side of
the hemivertebra that is not growing. It is only possible to bal-
ance growth by retarding growth on the convexity. This retarda-
tion can be achieved either by a convex growth arrest proce-
dure or by excising the hemivertebra. A convex growth arrest
procedure tethers the growth of the spine on the convexity,
with the objective of allowing continuing growth on the concav-
ity slowly to correct the deformity. Excision of the hemivertebra
removes the primary cause of the scoliosis, which is the enlarg-
ing wedge on the convexity at the apex of the deformity. For
either of these procedures to be effective, they should be per-
formed before the age of 5 years, when the scoliosis is still small
and before the compensatory curves become fixed.
UNILATERAL UNSEGMENTED BAR
A unilateral unsegmented bar is the second most common
cause of a congenital scoliosis. Here, there is a unilateral failure
of segmentation of two or more vertebrae. The unsegmented
bar does not contain growth plates and, therefore, does not
grow longitudinally, whereas some degree of growth occurs on
the convexity, resulting in a progressive scoliosis. This has a bad
prognosis.25
The unsegmented bar may occur anywhere in the spine
from the upper thoracic region to the sacrum, and no one
region is more commonly affected than the other. The mean
extent of the unsegmented bar is over three vertebrae (range:
two to eight vertebrae). One would assume that the more exten-
sive the bar, the more severe the rate of progression and final
severity of the scoliosis. However, there is not always a direct
relationship between the length of the bar and the rate of dete-
rioration of the scoliosis. Of much more importance is the
growth potential on the convexity of the curve. It is the unbal-
anced growth of the spine on the convexity that drives the
LWBK836_Ch107_p1118-1138.indd 1121
Chapter 107 Congenital Scoliosis 1121
deformity and not the unsegmented bar. If there was no convex
growth, there would be no deformity. It is, therefore, important
when assessing prognosis to look at both sides of the spine and
not just at the extent of the unsegmented bar. If the disc spaces
on the convexity are widely open, it is more likely that there will
be a greater growth potential than if the disc spaces are nar-
rowed. The age of the patient and the onset of the adolescent
growth spurt also have an effect with much more rapid progres-
sion during puberty.
Without treatment, most patients with a unilateral unseg-
mented bar have a scoliosis that will exceed 50 by the age of
10 years, and there will be a severe rigid deformity. These
patients require early prophylactic surgical treatment to bal-
ance the growth of the spine at an early stage, before the age of
10 years and before there is a severe deformity.
UNILATERAL UNSEGMENTED BAR
WITH CONTRALATERAL
HEMIVERTEBRAE
We know that a congenital scoliosis caused by a unilateral
unsegmented bar has a bad prognosis. However, there is a
smaller, less well-recognized group of patients who have an
even worse prognosis. These patients have not only a unilateral
unsegmented bar but also one or more hemivertebrae on the
contralateral side at the same level. These hemivertebrae pro-
duce an even greater growth imbalance than if there had been
an unsegmented bar alone. If left untreated, this can result in
some of the most rapidly progressive and severe deformities
seen by the spine surgeon.23
These vertebral anomalies occur in all regions of the spine,
with the unsegmented bar extending over a mean five verte-
brae and a mean of three contralateral hemivertebrae (range:
1 to 8 vertebrae) at the same level. The diagnosis of this type of
vertebral anomaly is not always easy at a late stage. Radiographi-
cally, the vertebral anomalies are most clearly seen in the first
few years of life, but as the curve deteriorates, the hemiverte-
brae often become obscured by the severity of the deformity. In
addition, the crankshaft effect produced by the continuing
growth of the hemivertebrae anterolaterally combined with the
tethering effect of the unsegmented bar on the concavity results
in severe vertebral rotation and distortion of the rib cage.
Without surgical treatment, these curves will progress on aver-
age 6 per year, and the majority will exceed 50 by 2 years of
age. The deformity of the chest, which usually occurs at an early
age, will impair lung growth and development and can be an
indication for surgical treatment by means of a vertical expand-
able prosthetic titanium rib (VEPTR) procedure and expan-
sion thoracoplasty.12
COMPLEX MIXED VERTEBRAL
ANOMALIES
Congenital scoliosis caused by a jumble of unclassifiable verte-
bral anomalies can be difficult to predict and requires careful
monitoring.25 In some patients, the vertebral anomalies are
well balanced, and there is very little spinal deformity other
than a slight shortening of the trunk, and no treatment is
required. However, in others, there may be a severe spinal
growth imbalance leading to a severe scoliosis at an early age.
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 1122 Section IX Dysplastic and Congenital Deformities
If the vertebral anomalies extend over a long segment of the
spine, there may be too few normal vertebrae either above or
below the anomalous segment to allow for the development of
compensatory curves to balance the spine. This results in severe
malalignment of the body, which can be associated with tilting
of the head, shoulder imbalance, spinal decompensation, tho-
racic translocation, pelvic obliquity, and an apparent leg length
discrepancy. If there is also an associated deformity of the tho-
racic cage developing at an early age, this can lead to a TIS.
Early spinal fusion of such a long segment of the spine would
lead to severe stunting of the trunk and would also contribute
to a TIS. This can be an indication for a growing rods or VEPTR
procedure with spinal fusion being postponed until the child is
older.
CONGENITAL RIB AND CHEST WALL
ANOMALIES
Congenital malformation of the ribs and chest wall, as well as
anomalies of the scapulae, are often found in association with
congenital deformities of the spine.34
Chest wall deformities may be simple or complex. A simple
anomaly consists of a localized fusion of two or three ribs,
whereas patients with complex anomalies have multiple exten-
sive rib fusions, usually without set pattern, combined with an
adjacent chest wall defect due to an absence or deviation of the
ribs. These anomalies occur most commonly (38%) on the con-
cavity of a thoracic or thoracolumbar congenital scoliosis due
to a unilateral failure of vertebral segmentation (with or with-
out contralateral hemivertebrae).
Complex rib fusions on the concavity may have a unilateral
tethering effect on the spine, but this is of minor importance in
comparison with the main driving force for the scoliosis, which
is the severe growth imbalance produced by the vertebral
anomalies. However, congenital rib fusions and an increasing
scoliosis can contribute to a TIS and require early prophylactic
treatment by means of a VEPTR procedure.
Sprengel deformity (congenital elevation of the scapula) occurs
most commonly (60%) in association with a cervicothoracic or
thoracic scoliosis due to a unilateral failure of vertebral seg-
mentation.34 This combination of a congenitally elevated scap-
ula and its occurrence on the convexity of an upper thoracic
congenital scoliosis will cause a significant deformity because of
an elevation of the shoulder line and an impairment of shoul-
der function. These deformities usually require surgical treat-
ment both to correct the scoliosis and to perform a distal dis-
placement of the scapula in relation to the vertebral column.
However, when the Sprengel deformity is on the concavity of
the scoliosis, it often partially compensates for the cosmetic
deformity caused by the elevation of the contralateral shoulder
on the convexity of the scoliotic curve. This minimizes shoul-
der asymmetry and usually does not require reduction of the
congenitally elevated scapula.
SECONDARY DEFORMING FEATURES
REQUIRING TREATMENT
There are also important secondary features, common to all
types of congenital scoliosis, relating to the site of the vertebral
anomalies. These features contribute significantly to the overall
LWBK836_Ch107_p1118-1138.indd 1122
deformity of the patient and must be taken into consideration
when planning treatment.
Cervicothoracic congenital scoliosis will cause a tilting of the
head to one side. The patient will attempt to level the head by
either tilting the upper body to one side or developing a com-
pensatory curve on the opposite side in the upper thoracic
region below the congenital scoliosis. This requires early pro-
phylactic surgical treatment usually by means of an in situ
fusion of the anomalous segment before there is a significant
deformity.
Upper thoracic congenital scoliosis may never become large but
can cause a significant cosmetic deformity as a consequence of
an elevation of the shoulder line on the convexity of the curve
and less frequently, tilting of the head.25 In general, the higher
the apex of the curve is, the more severe the deformity. A 30
curve is probably at the upper limit of acceptability especially in
girls and requires early prophylactic surgical treatment usually
by means of a spinal fusion in situ.
Midthoracic congenital curves with their apices at T4, T5, T6, or
T7, especially those caused by unilateral unsegmented bar with or
without contralateral hemivertebrae, are frequently associated
with the development of a secondary structural curve in the lower
thoracic or thoracolumbar regions.23 As the congenital curve
deteriorates, it produces a rotational torque that is transmitted
into the lower thoracic or thoracolumbar regions, resulting in a
secondary scoliosis on the opposite side. This secondary curve,
which does not contain any congenital anomalies, is initially com-
pensatory and correctable. However, it later becomes fixed and
may deteriorate even more rapidly than the primary congenital
curve above. The congenital thoracic curve may be only moder-
ately rotated, but the lower secondary curve is frequently severely
rotated, producing the major cosmetic deformity with spinal
imbalance and a large rib prominence. In these circumstances, a
severe, apparently idiopathic curve develops below an upper
thoracic congenital anomaly whose significance may not be fully
appreciated. If not treated early, surgical treatment of the con-
genital scoliosis will often require to be combined with control of
the secondary structural curve by means of a growth rod.
Thoracic congenital scoliosis can be associated with severe dis-
tortion of the rib cage. When this occurs before the child is
8 years of age, it can interfere with the normal growth and func-
tion of the lungs.13 Increasing deformity is associated with
diminishing vital capacity and, in severe cases without treat-
ment, may lead to cor pulmonale and possible death in early
adult life. Surgical treatment to fuse a long segment of the tho-
racic spine in these young children is also associated with stunt-
ing of the spine and an additional reduction in vital capacity.16
An alternative to early spinal fusion in these patients is a grow-
ing rod or VEPTR procedure.
Lower thoracic, thoracolumbar, and lumbar congenital scoliosis,
especially those due to a unilateral failure of vertebral segmen-
tation or multiple unclassifiable vertebral anomalies, often fail
to develop a compensatory curve on the opposite side in the
lower lumbar region that is sufficient to balance the spine. The
reason for this is that there are too few normal mobile verte-
brae between the rigid anomalous segment and the sacrum.
This results in a significant cosmetic deformity with severe
malalignment of the body, often associated with pelvic obliquity
and an apparent leg length discrepancy.
Lumbosacral congenital scoliosis, usually due to a hemivertebra
at the lumbosacral junction, frequently results in the develop-
ment of a secondary structural noncongenital scoliosis on the
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 Chapter 107 Congenital Scoliosis 1123

opposite side in the upper lumbar or thoracolumbar regions PREOPERATIVE ASSESSMENT

with spinal imbalance. This secondary curve, which becomes
fixed and rotated, produces the major deformity and also
requires treatment.
PROGNOSIS
The prognosis for a congenital scoliosis with regard to its rate
of deterioration, final severity, and resulting disability depends
on three factors (Table 107.2).
1. The type of vertebral anomaly and the degree of growth imbalance it
produces. The type of anomaly that causes the most severe
scoliosis is a unilateral unsegmented bar with contralateral
hemivertebrae at the same level followed in diminishing
order of severity by a unilateral unsegmented bar alone, two
unilateral fully segmented hemivertebrae, a single fully seg-
mented hemivertebra, a wedged vertebra, and least deform-
ing is a block vertebra.25 The poor prognosis associated with
a unilateral unsegmented bar with contralateral hemiverte-
brae is so predictable that these curves should be treated
immediately without a period of observation.23 Congenital
scoliosis caused by a jumble of unclassifiable anomalies can
be difficult to predict and requires careful monitoring.
2. The site of the anomaly. For any type of vertebral anomaly, the
rate of deterioration of the resulting scoliosis is most severe
in the thoracic and thoracolumbar regions and usually less
severe in the upper thoracic and lumbar regions. The site of
the anomaly, especially at the lumbosacral junction, is also
related to the possible development of secondary structural
curves and spinal imbalance.
3. The age of the patient at the time of diagnosis. Congenital scoliosis
presenting as a clinical deformity in the first few years of life
usually has a bad prognosis because this indicates a marked
growth imbalance that will continue until skeletal maturity
when the vertebral growth plates fuse. In addition, the rate of
deterioration is not uniform and becomes more severe after
the age of 10 years, during the adolescent growth spurt. Even
after skeletal maturity, very severe curves may continue to
deteriorate slowly due to either plastic deformation of the
spine or the onset of secondary degenerative changes.
SPINE
Plain radiographs: The diagnosis of a congenital scoliosis, its site,
and its size as well as an assessment of head tilt, shoulder asym-
metry, decompensation of the trunk, and pelvic obliquity is made
on full-length anteroposterior and lateral spine radiographs
taken with the patient standing. In infants, the radiograph may
have to be taken sitting or lying. The rigidity of the deformity is
assessed on spinal radiographs taken with the patient bending to
either side. Cervical spine radiographs are also necessary to
exclude a KlippelFeil deformity or cervical hemivertebra.
However, more precise identification of the vertebral anomalies
that cause the scoliosis will require good-quality anteroposterior
and lateral spinal radiographs taken with the patient supine and
centered on the abnormal levels. A coned-down oblique radio-
graph of the apex of the scoliosis gives a true anteroposterior
view of the more severely rotated region of the spine and may
detect hidden vertebral anomalies. An anteroposterior view
aligned though the lumbosacral junction may show whether a
lumbosacral vertebra is fully segmented or semisegmented.
Often the most informative radiographs, if available, are those
taken shortly after birth. However, a misdiagnosis is possible in
infants with a short unilateral unsegmented bar, which may not
appear radiographically until it is fully ossified.
It is important to carefully examine the lateral spine radio-
graph to detect any associated kyphosis because this will affect
treatment. If only the anteroposterior radiograph is viewed, a
patient with a posterolateral quadrant vertebra producing a
kyphoscoliosis may be misdiagnosed as having a lateral hemiver-
tebra producing only a scoliosis. Treatment by means of a com-
bined anterior and posterior convex epiphysiodesis, which may
be appropriate for a lateral hemivertebra, would be contraindi-
cated for a kyphoscoliosis because it could increase the defor-
mity in the sagittal plane.
After the vertebral anomalies have been clearly identified
on the anteroposterior radiograph, an attempt should be made
to count the number of growth plates on both sides of the spine
and estimate the potential growth imbalance. Allowance should
be made for any disc space that is narrowed or ill defined

Mean Yearly Rate of Deterioration (in Degrees) Without Treatment for Each Type of
TABLE 107.2
Congenital Scoliosis in Each Region of the Spine (Before 10 Years to After 10 Years)

Type of Congenital Vertebral Anomaly

Hemivertebra
(Fully Segmented) Unilateral Unsegmented
Block Unilateral Bar and Contralateral
Site of Curvature Vertebra Wedge Vertebra Single Double Unsegmented Bar Hemivertebrae

Upper thoracic 11  2 1-2 22.5 24 56

Lower thoracic 11 22 22.5 23 35 58
Thoracolumbar 11 1.52 23.5 5  37 714
Lumbar 1 1 11  35 
Lumbosacral   11.5   

 No treatment required; May require spinal surgery;  Requires spinal surgery;  Too few or no curves.
A study of 560 patients with congenital scoliosis: McMaster MJ (2006).

LWBK836_Ch107_p1118-1138.indd 1123 8/25/11 10:07:28 PM

 1124 Section IX Dysplastic and Congenital Deformities
because this could indicate an impaired growth potential. It
may be difficult to assess the prognosis in young infants with
mixed vertebral anomalies because the spine is only 30% ossi-
fied at birth and possible deterioration of the scoliosis requires
careful radiographic follow-up.
Follow-up radiographic assessment of a congenital scoliosis
does not require detailed visualization of the vertebral anoma-
lies after they have been fully identified. Serial full-length
anteroposterior spinal radiographs taken with the patient
standing are best for assessing deterioration. The Cobb angle
of the scoliosis is always measured at exactly the same vertebral
levels with reference to the standing radiograph taken when
the patient was first seen. Errors in measurement are mini-
mized if one surgeon measures all the radiographs, using con-
sistent landmarks with reference to the first radiograph and
attention to detail. Serial radiographs taken every 4 to 6 months
will show progression of the scoliosis and also any change in the
shoulder asymmetry, decompensation of the trunk, or pelvic
obliquity.
Computed tomography can be used to obtain a 3D reconstruc-
tion of the anomalous segment of the spine. This is often help-
ful in defining more complex congenital vertebral anomalies
and identifying previously unrecognized anomalies. The CT
reconstruction should be confined only to the abnormal seg-
ment of the spine so as to limit the amount of radiation to these
growing children. Precise identification of the vertebral anom-
alies allows the surgeon better to assess the likelihood of pro-
gression of the scoliosis and plan precise surgical treatment
such as excision of a hemivertebra, which may be fully seg-
mented or semisegmented, and also to evaluate the presence of
a spina bifida occulta with exposed neural structures.
Sprengel deformity (congenital elevation of the scapula) is
often found in association with congenital scoliosis in the upper
thoracic or cervicothoracic region. When it is present on the
convexity of the curve, the combination of these two anomalies
causes a significant deformity because of the elevation of the
shoulder line. In these circumstances, the elevated scapula
should be reduced when the scoliosis is treated surgically.
However, if the elevated scapula is on the concavity of the curve,
it may partially compensate for the scoliotic deformity by level-
ing the shoulders. In these circumstances, it should not be
operated upon providing shoulder function is acceptable.
INTRASPINAL ANOMALIES
The embryological development of the neural axis and the ver-
tebral column occur synchronously, and it is therefore not sur-
prising that neural and vertebral malformations often coexist.
Abnormalities of the spinal cord can also be associated with a
failure of development of the posterior neural arches and over-
lying soft tissues, including the skin, resulting in an open spi-
nal defect such as a myelomeningocele. This chapter deals only
with closed congenital scoliosis, in which the skin overlying
the spine remains intact. However, there may be occult intraspi-
nal anomalies that can be associated with mild neurological
abnormalities in the lower limbs. It is important to detect these
hidden intraspinal anomalies because they may restrict move-
ment of the spinal cord within the spinal canal, leading to neu-
rological deterioration with spinal growth and also during
attempts to surgically correct the scoliosis. However, a congeni-
tal scoliosis without an intraspinal anomaly will not cause spinal
cord compression or result in a neurological deficit no matter
LWBK836_Ch107_p1118-1138.indd 1124
how severe the scoliosis. This is very different from a congenital
kyphosis or kyphoscoliosis that can cause spinal cord compres-
sion at the apex of the deformity.26
Intraspinal anomalies may be found in association with all
types and sites of congenital scoliosis, but the most common
association (50%) is with a unilateral unsegmented bar with
contralateral hemivertebrae, producing a scoliosis in the lower
thoracic or thoracolumbar regions.24 In the past, myelography
was used to diagnose intraspinal anomalies and a diastemato-
myelia was found in 5% to 21% of all patients.24
Clinical Examination
An intraspinal anomaly may not be immediately obvious, but
there are clinical clues as to its presence. An abnormality of the
skin overlying the spine, such as a dimple, nevus, hairy patch,
or lipoma is present in approximately 70% of affected patients.
However, these stigmata do not always overlie the intraspinal
anomaly and may also occur without an accompanying intraspi-
nal anomaly. Neurological abnormalities affecting the lower
limbs may be present but are often mild and easily missed. Fre-
quently, only one leg is affected, and this may be slightly short
with a small foot and mild cavus deformity with slight clawing
of the toes.
Plain Radiographs
The spinal radiograph may show a spina bifida occulta affect-
ing one or more adjacent vertebrae at the site of the intraspinal
anomaly associated with widening of the interpedicular dis-
tance and narrowing of the disc spaces. Occasionally, a bony
spur associated with a diastematomyelia may be visible on the
plain spinal radiograph, but magnetic resonance imaging
(MRI) is necessary to confirm its presence and reveal other
possible neural abnormalities.
Magnetic Resonance Imaging
MRI is a noninvasive and sensitive method of identifying soft
tissue abnormalities of the spinal cord and showing the carti-
laginous end plates of the vertebral bodies and providing infor-
mation on possible growth potential. However, an MRI requires
a general anesthetic for children younger than 5 years and
sedation for those between 5 and 10 years. In these circum-
stances, MRI should be applied selectively and only to those
with suspicious clinical or radiological findings and also to
those who are about to undergo surgery to correct a spinal
deformity.
Basu et al2 investigated 110 patients with congenital scoliosis
by using MRI and found a 34% prevalence of intraspinal anom-
alies. The most common anomalies were a tethered cord
(16 patients), a syrinx (13 patients), followed by a thickened
and fatty filum (11 patients), a low conus (10 patients), a
diastematomyelia (8 patients), and, less frequently, a Chiari
malformation or an arachnoid cyst.
Neurosurgical Treatment
It is widely accepted that an intraspinal anomaly that tethers
the spinal cord should be surgically excised or released if there
is a progressive neurological deficit or before attempting to sur-
gically correct a spine deformity. Excision or release of the
8/25/11 10:07:28 PM

intraspinal anomaly will not improve the neurological status,
but it will prevent further neurological deterioration and
decrease the risk of complications occurring during correction
of the scoliosis.24 If the intraspinal anomaly is diagnosed during
the routine assessment of a child younger than 5 years, it should
probably be removed as a prophylactic measure to prevent the
possible later development of neurological complications.
However, in an older child, the presence of an intraspinal
anomaly is not necessarily an indication for its removal unless
there are symptoms or corrective surgery is planned. Excision
or release of the intraspinal anomaly is a neurosurgical proce-
dure, which in my opinion is best performed as a separate
operation performed 2 to 3 months prior to the surgical treat-
ment of the congenital scoliosis.
RESPIRATORY FUNCTION
Respiratory function should be assessed by a pulmonologist
experienced in seeing children with spinal deformities. Rou-
tine spirometry may be used in patients older than 6 years,
but in younger children, this is not usually possible due to a
failure to comply. Younger children can have their lung vol-
umes assessed by a CT scan, but unfortunately this provides
little information with regard to pulmonary function. A com-
monly used index of vital capacity is the percentage of the
normal predicted value based on the standing height. How-
ever, this can be misleading because the vertical height of the
spine is reduced by the scoliosis and, in addition, children
with congenital vertebral anomalies are usually short for their
age and remain smaller than normal children. In these cir-
cumstances, arm span is best used when assessing the per-
centage of the normal vital capacity for their age in these
patients.
OTHER ASSOCIATED CONGENITAL ANOMALIES
Congenital scoliosis is frequently associated with congenital
anomalies in other systems. These anomalies are often asymp-
tomatic and may remain undetected until the patient is fully
assessed after diagnosis of a congenital scoliosis. Beals and asso-
ciates3 found that up to 60% of patients with vertebral anoma-
lies had one or more additional anomalies in other systems and
that many of these were medically important. The prognosis for
these patients is excellent if the associated anomalies are
detected and, if necessary, treated.
Congenital heart disease diagnosed by echocardiography was
found in 23% of 110 patients with congenital scoliosis studied
by Basu et al.2 The common abnormalities were atrial or ven-
tricular septal defects and a persistent ductus arteriosus, and
some of these required surgical treatments. Echocardiography
is, therefore, necessary for all patients in whom surgery is
planned for a congenital scoliosis.
Genitourinary anomalies have been reported in 13% to 37% of
patients.2 The most common anomalies diagnosed by means of
renal ultrasound are a unilateral kidney, horseshoe kidney, or
ureteric obstruction. These renal anomalies may also show on
the MRI of the spine, which is performed as part of the routine
preoperative assessment. The highest rates of renal abnormali-
ties are often found in association with vertebral anomalies in
the cervical and upper thoracic spine. Most of these anomalies
are benign, but some may require treatment before surgically
treating the congenital scoliosis.
LWBK836_Ch107_p1118-1138.indd 1125
Chapter 107 Congenital Scoliosis 1125
Other Anomalies
The VATER syndrome is an association of congenital abnor-
malities including vertebral anomalies, anorectal atresia, tra-
cheoesophageal fistula, and renal anomalies. The additions of
cardiac defects and limb defects such as radial club hand and
thumb hypoplasia have been described as the VACTERL
syndrome.
THE TREATMENT OF CONGENITAL
SCOLIOSIS IS VERY DIFFERENT FROM
ALL OTHER TYPES OF SCOLIOSIS
NONOPERATIVE TREATMENT
Orthotic treatment is never an alternative to appropriate surgi-
cal treatment for congenital scoliosis because the problem
being treated is an imbalance of spinal growth and the scoliosis
if often rigid. A brace cannot overcome a rigid deformity or
create growth on the side of the spine that is not growing.
However, after early prophylactic surgery, a brace may be
helpful in maintaining spinal alignment and preventing the
progression of compensatory curves that were not included in
the fusion or controlled by a growth rod. These secondary
structural curves are easier to control in a brace than the pri-
mary congenital curve because they occur in regions of the
spine where the vertebrae are relatively normal and, therefore,
much more mobile than the anomalous segment. However,
care should be taken in young children to ensure that pressure
from the brace does not result in a secondary chest wall defor-
mity and additional respiratory impairment.
SURGICAL TREATMENT
Surgical treatment is frequently necessary for congenital scolio-
sis and is performed at any age if the curve has a bad prognosis
or there is already a significant deformity.
The object of surgery is to correct or prevent increasing spi-
nal deformity and produce a spine that at the end of growth
will be as balanced as possible in the frontal and sagittal planes,
with as short a spinal fusion segment as possible while preserv-
ing respiratory function. We are also now much more aware of
the possible adverse affects on lung growth and development of
an extensive thoracic spinal fusion performed at an early age.16
This differs from adolescent idiopathic scoliosis in which sur-
gery is performed during the teenage years when the lungs are
fully formed and a spinal fusion is unlikely to have a significant
affect on respiratory function.
There is no one operative procedure that can be applied to
all types of congenital scoliosis. The method of surgery selected
depends on the age of the patient, the site and type of the ver-
tebral anomaly, the size of the congenital scoliosis, and spinal
balance, as well as the presence of structural compensatory
curves or a significant chest deformity. Successful surgical treat-
ment depends on selecting the correct procedure and applying
it at the appropriate time. There are three groups of surgical
procedures (Table 107.3).
Prophylactic Surgical Treatment
The goal of prophylactic treatment is to anticipate progres-
sion of the scoliosis at an early age when the deformity is still
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 1126 Section IX Dysplastic and Congenital Deformities

TABLE 107.3 Surgical Options for Progressive Congenital Scoliosis

PROPHYLACTIC PROCEDURES Patients younger than 5 years

Scoliosis less than 40
Unilateral failure of vertebral formation
Hemivertebra
Single fully segmented hemivertebra
Spine balanced- convex epiphysiodesis
Or hemivertebra excision
Spine unbalanced- hemivertebra excision
Two unilateral hemivertebrae
Excision of both hemivertebraeposterior-only approach
Or posterior growth rod convex epiphysiodesis
Unilateral failure of vertebral segmentation
Unilateral unsegmented bar with of without contralateral hemivertebrae
Short anomalous segmentfour vertebrae or less
Chest wall normal
In situ localized posterior spine fusion growth rod
Chest wall normal but severe vertebral rotation and/or rapidly progressing scoliosis (crankshaft effect possible)
Anterior spine fusion + posterior growth rod
Chest wall abnormal with concave fused ribs
VEPTR and expansion thoracostomy
Long anomalous segmentmore than four vertebrae concave fused ribs
VEPTR and expansion thoracostomy
Multiple unclassifiable vertebral anomalies
Short anomalous segmentfour vertebrae or less
Growth rod convex epiphysiodesis
Long anomalous segmentmore than four vertebrae
Chest wall normal
Posterior growth rod
Chest wall abnormal with concave fused ribs
VEPTR and expansion thoracoplasty
CORRECTIVE PROCEDURES IN LATER CHILDHOOD
Older but skeletally immature patient
Moderate to severe progressing scoliosis
Less than 50
Posterior spine fusion with instrumentation
Greater than 50
Anterior release and posterior spinal fusion with instrumentation OR posterior spinal fusion with all-pedicle screw instrumentation
VERTEBRAL COLUMN RESECTION
Severe rigid scoliosis with fixed spinal imbalance
Combined anterior and posterior approach vertebral column resection with pedicle screw and rod instrumentation
Or posterior-only approach vertebral column resection with pedicle screw and rod instrumentation

VEPTR, vertical expandable prosthetic titanium rib.

small and perform a limited surgical procedure to balance the
growth of the spine and prevent the later development of a
much more severe deformity. However, it is not possible to
create normal growth on the concavity of a congenital scolio-
sis where growth is either retarded or nonexistent, and for
some patients with a marked spinal growth imbalance, there
is no perfect treatment.
Anticipation of curve progression and the type of prophylac-
tic spinal surgery required is based on the type and site of the
congenital vertebral anomaly and the degree of growth imbal-
ance that it is likely to produce. The development of secondary
structural compensatory curves or spinal imbalance can be pre-
vented only by early correction or stabilization of the primary
congenital scoliosis. Delayed treatment for the same problem
would necessitate a much more extensive surgical procedure
requiring a spinal fusion of not only the congenital scoliosis but
also the secondary structural compensatory curves. Correction
is also much more difficult to achieve in large rigid curves and
with a higher risk of neurological complications.
The main indication for prophylactic surgery is a child
younger than 5 years with a small congenital scoliosis less than
40, with radiographic evidence of curve progression, especially
if this is likely to cause spinal imbalance. The objective is to bal-
ance the growth of the spine and if possible achieve some
degree of correction and minimize any possible adverse effects
on lung growth if the curvature is in the thoracic region.
Treatment options include (1) an in situ fusion, which is
simplest but provides no correction, (2) a convex growth arrest
procedure (hemiepiphysiodesis), which can be unpredictable
and relies on continuing growth on the concavity to achieve
correction, (3) excision of a hemivertebra will achieve immedi-
ate correction but has a limited application, or (4) a VEPTR
with expansion thoracostomy for those with a thoracic defor-
mity that would impair lung growth and development.

LWBK836_Ch107_p1118-1138.indd 1126 8/25/11 10:07:29 PM


A unilateral failure of vertebral formation resulting in a fully seg-
mented hemivertebra requires either a hemivertebra excision
or a localized anterior and posterior convex epiphysiodesis.
A unilateral failure of vertebral segmentation extending over less
than four vertebrae can be treated by a localized in situ fusion,
which is unlikely to have any significant adverse effect on a nor-
mal thorax. The objective of the surgery is to transform the
anomalous segment, due to the unilateral unsegmented bar,
into a block vertebra with more balanced although restricted
longitudinal spinal growth. This may be combined with a poste-
rior growth rod to control the unfused normal vertebrae above
and below the anomalous segment, which are also contained
within the Cobb measurement of the congenital scoliosis. In
addition, the growth rod can also be used to prevent or control
the development of a structural compensatory curve develop-
ing below the congenital scoliosis. However, a spinal fusion of a
long segment (more than four vertebrae) of the thoracic spine,
in a young child, is likely to have an adverse effect on the growth
of the thorax and lung development.16 In these circumstances,
the patient is probably best treated by an expansion thoracos-
tomy with a VEPTR in an attempt to optimize lung growth and
development as well as control the scoliosis.
Mixed or unclassifiable vertebral anomalies extending over a few
segments and producing a progressive scoliosis with a normal
thorax can be treated by a growth rod with or without a convex
epiphysiodesis. However, vertebral anomalies extending over
multiple segments in the thoracic spine with fused ribs on the
concavity are best treated by a VEPTR procedure with an expan-
sion thoracostomy.
After prophylactic surgery, all curves must be carefully mon-
itored to skeletal maturity, not only to serially lengthen either a
growth rod or a VEPTR every 6 months but also to detect any
further curve progression that may necessitate additional sur-
gery especially during the adolescent growth spurt. In these
circumstances, it may be necessary to extend the spinal fusion
over the whole extent of the deformity including not only the
congenital scoliosis but also any secondary structural curves.
Corrective Procedures for Moderate to Severe Congenital
Scoliosis in Older Children
These older children have already developed a significant sco-
liosis that, without treatment, will continue to progress until
skeletal maturity. The object of surgery is to correct partly and
stabilize the spine in the optimum position by means of a spinal
fusion extending over the whole length of the scoliosis includ-
ing any structural compensatory curves. This necessitates a pos-
terior spinal fusion with or without an associated anterior
release and fusion depending on the severity and rigidity of the
curve and the type of construct (i.e., hook vs. hybrid vs. pedicle
screw). Posterior instrumentation is used mainly as an internal
strut to balance the spine rather than to achieve excessive cor-
rection, which could be neurologically dangerous.
Salvage Procedure for Severe Rigid Unbalanced
Congenital Scoliosis at a Late Stage
Patients who present at a late stage with a severe rigid unbal-
anced scoliosis will require, if thought appropriate, a vertebral
column resection to achieve significant correction and realign
the spine. However, the primary object is still to prevent further
deterioration and correction of the deformity is only attempted
LWBK836_Ch107_p1118-1138.indd 1127
Chapter 107 Congenital Scoliosis 1127
if it can be safely achieved. Correction of these rigid deformi-
ties is difficult with a higher risk of neurological complications
and a less certain result.
PROPHYLACTIC SURGICAL
PROCEDURES
CONVEX GROWTH ARREST
(HEMIEPIPHYSIODESIS)
A combined anterior and posterior convex epiphysiodesis is a
method of modifying the growth of the spine and is used as a
prophylactic surgical procedure for patients with a small pro-
gressive congenital scoliosis due to a unilateral failure of verte-
bral formation, such as a hemivertebra, in which there is some
growth potential on the concavity of the curve. Tethering
growth on the convex side of the curve can lead to spontaneous
correction of the deformity due to continued growth on the
concavity.
This procedure, which does not provide immediate correc-
tion, is best applied to patients who have a balanced and cos-
metically acceptable deformity but with a poor prognosis for
progression. The best results are achieved in patients operated
upon before the age of 5 years with a single fully segmented
hemivertebra producing a short curve extending over five seg-
ments or less correcting to less than 40 with the patient supine
(Fig. 107.2A and B). More severe or unbalanced curves are bet-
ter treated by excising the hemivertebra with the objective of
obtaining immediate correction and balancing the spine.
Convex hemiepiphysiodesis of the spine is a relatively safe
operation when compared with other surgical procedures for
congenital scoliosis. The disadvantage is a limited application
with a slow and often uncertain correction because of the
unpredictable growth potential on the concavity of the curve.
However, even if there is no correction of the scoliosis, the con-
vex growth arrest is often sufficient to stabilize the deformity. A
convex epiphysiodesis is likely to result in an improvement of
the congenital scoliosis in approximately 50% (epiphysiodesis
effect), no change in 40% (fusion effect), and continued pro-
gression in 10%.
This technique, which depends for its success on continuing
growth on the concavity of the curve, will not be effective as a
corrective procedure in patients with a unilateral unsegmented
bar with or without contralateral hemivertebrae because there
is no concave growth potential. The presence of a kyphosis in
association with the scoliosis is also a contraindication because
the anterior convex growth arrest could exacerbate the kyphotic
deformity. Patients with a small kyphoscoliosis are best treated
by a posterior growth arrest procedure.27
The surgery for a convex growth arrest procedure is per-
formed in two stages under the same anesthetic. The spine is
first approached anteriorly on the convexity of the scoliosis
through a minithoracotomy, a thoracoabdominal retroperito-
neal exposure, or a purely retroperitoneal approach beneath
the diaphragm, depending on the site of the hemivertebra.
The lateral one third to one half of the discs and their adjacent
end plates are removed not only at the site of the hemivertebra
but also at one normal intervertebral disc above and below.
This removes the anterior growth plates at the site of the anom-
aly, which are the main cause of the increasing scoliosis.
However, failure to extend the convex growth arrest to two
8/25/11 10:07:29 PM
 1128 Section IX Dysplastic and Congenital Deformities
A B
disc spaces above and below the hemivertebra will not control
the curvature. To create an anterior convex fusion, the excised
disc spaces are packed with chips of bone taken from the
excised rib or preferably the excised rib is used as an inlay graft
inserted into a trough cut laterally in the vertebral bodies and
inserted in niches cut into the vertebral bodies at either end.
The second stage of the procedure is performed through a
separate posterior midline subperiosteal exposure of convexity
of the curve at the site of the hemivertebra and extending to
two normal vertebrae above and below. Care is taken not to
strip the paraspinal muscles on the concavity of the curve so as
not to interfere with the growth potential on this side of the
spine. A posterior convex fusion is performed by excising the
facet joints and decorticating the posterior elements. Strips of
excised rib are applied to the fusion area. Several days after the
surgery, and once the chest drain has been removed, partial
correction of the deformity may be attempted by means of
manual traction and the application of an underarm plaster
jacket, which is maintained for 4 months until the convex
growth arrest has healed.
Minimally invasive thoracoscopic and laparoscopic proce-
dures can also be used to perform an anterior convex epiphysi-
odesis or arthrodesis as well as excise the body of a hemiverte-
bra. However, these are technically much more demanding
procedures with a relatively long learning curve and should be
performed only in highly specialized centers where the tech-
nique is being used regularly. The advantage is a more cosmeti-
cally pleasing result because of small incisional scars and reduc-
ing postoperative pain and recovery time.
All of these patients require careful radiographic follow-up
to skeletal maturity because of the possibility that the convex
epiphysiodesis may not completely control the deformity espe-
cially during the adolescent growth spurt and additional sur-
gery may be necessary.
LWBK836_Ch107_p1118-1138.indd 1128
Figure 107.2. (A) An infant aged
2 years with a 33 right thoracolumbar
scoliosis due to a single fully seg-
mented hemivertebra at L1. A com-
bined anterior and posterior convex
growth arrest procedure was per-
formed from T11 to L3. (B) The scolio-
sis resolved and this was maintained at
the age of 16 years 8 month.
EXCISION OF A HEMIVERTEBRA
Total excision of a hemivertebra is attractive as a prophylactic
procedure because it removes the primary cause of the scolio-
sis, which is the enlarging wedge on the convexity at the apex
of the curve (Fig. 107.3A to D). The surgery may be performed
through either sequential or simultaneous anterior and poste-
rior approaches or by means of a posterior-only approach to
the spine.
The main indication for the procedure is a fully segmented
hemivertebra, which is producing a significant progressive sco-
liosis and/or spinal imbalance, especially when the hemiverte-
bra occurs at the lumbosacral junction and causes the lumbar
spine to take off obliquely from the sacrum so that the patient
lists to one side. The procedure is most safely performed in the
lumbar region where the cauda equina is less easily injured and
more hazardous in the cervicothoracic, thoracic, or thora-
columbar regions where the neural structures are less mobile.
The advantage of resecting a hemivertebra is that it creates
a wedge osteotomy of the spine, which, when closed, produces
immediate correction and realignment of the spine with the
fusion being confined to only one segment. With a convex
growth arrest procedure, there is a slow and often uncertain
correction of the deformity and a more extensive fusion extend-
ing to two normal vertebrae above and below the site of the
hemivertebra. This can cause a loss of mobility in the lumbar
spine. The disadvantage of a hemivertebral resection is that it is
a technically more demanding procedure that requires enter-
ing the spinal canal both anteriorly and posteriorly, with the
possibility of neurological complications18 and bleeding from
the epidural veins.
Hemivertebral resection became accepted as a method of
treatment when Leatherman and Dickson22 described two-stage
anterior and posterior spine surgery to perform a closing wedge
8/25/11 10:07:29 PM

A B
Figure 107.3. (A and B) An infant aged 1 year and 6 months with a 38 left thoracolumbar scoliosis due
to a single fully segmented hemivertebra at L2. A single-stage posterior approach excision of the hemiverte-
bra was performed and the scoliosis corrected using convex pedicle screw and rod instrumentation.
(C and D) At the age of 4 years 8 months, there is a single-level fusion at the site of the excised hemivertebra,
and the scoliosis is stable at 12.
osteotomy to shorten the spine and correct a deformity.
Shortening of the vertebral column is much less likely to cause
traction on the spinal cord and neurological complications.
Until recently, combined anterior and posterior surgery has
been the most commonly used technique for the total removal
of a hemivertebra. Initially, because of the fear of precipitating
spinal cord ischemia and neurological complications, this was
performed as two separate procedures separated by 5 to 7 days.
However, this risk has proven to be unfounded and both proce-
dures are now usually performed under the same anesthetic as
a combined procedure.
In a combined anterior and posterior resection, the hemivertebra
is approached both anteriorly and posteriorly in a manner
similar to that for a convex growth arrest procedure. In the
anterior-first stage, the body of the hemivertebra and anterior
part of the pedicle are removed along with the adjacent discs
and vertebral end plates. This approach provides good visual-
ization and the ability for an extensive removal of the vertebral
body along with the adjacent intervertebral discs and end plates.
It is important to excise the entire disc to the concavity of the
curve to create mobility and allow the wedge osteotomy to close
more easily. This is followed under the same anesthetic by the
second stage of the procedure in which the spine is exposed pos-
teriorly to excise the posterior aspect of the hemivertebra and
the residual pedicle to create a wedge osteotomy.
Closure of the wedge osteotomy after resection of the
hemivertebra was in the past achieved by manipulation of the
spine followed by the application of a spinal spica cast, which
was worn for up to 6 months. However, spinal instrumentation
is now used and provides a much more effective means of clos-
ing the space left after excising the hemivertebra with stable
fixation and a reduced period in a spinal jacket.
LWBK836_Ch107_p1118-1138.indd 1129
Chapter 107 Congenital Scoliosis 1129
C D
Hemivertebral resection before the mean age of 5 years by using a
combined anterior and posterior approach with posterior
instrumentation has been reported in six series totaling 83
patients.6,5,8,17,20,21 Preoperatively, the mean scoliosis was 38
(range 28 to 47), and postoperatively this was reduced to a
mean 12 (range 11 to 16), which was a mean improvement
of 68% (range 64% to 71%). Bollini et al5 recommended plac-
ing a fibular graft anteriorly to act as a strut between the two
adjacent vertebral bodies at the site of the hemivertebral exci-
sion to prevent later development of a kyphosis. Neurological
complications due to nerve root injury occurred in 3 (4%) of
the 83 patients, with recovery occurring in 2, and 1 was perma-
nent. A pseudarthrosis occurred in two patients (2%), and
there were no vascular complications.
Hemivertebral resection after the mean age of 12 years using
the combined approach with instrumentation has been
reported in three series totaling 73 patients.4,14,18 Preoperatively,
the mean scoliosis was larger than in the younger patients at
59 (range 45 to 78), and postoperatively, this was reduced to
27 (range 17 to 38), a mean improvement of 54% (range
44% to 61%), which was less than in the younger patients. In
the series reported by Benli et al,4 12 patients had an anterior
followed by a posterior approach with posterior instrumenta-
tion and the remaining 14 had a posterior followed by an ante-
rior approach with anterior compression instrumentation to
obtain correction. They found no statistically significant differ-
ence in the degree of correction achieved between the two
groups. They concluded that hemivertebrectomy could be
safely performed in adolescents and the space left after exci-
sion closed by spinal instrumentation. However, anterior instru-
mentation tended to have a kyphotic effect in the thoracic
region and posterior instrumentation had a lordotic effect in
8/25/11 10:07:29 PM
 1130 Section IX Dysplastic and Congenital Deformities
the lumbar region. Complications were reported in only one of
these series. Holte et al18 reported neurological complications
with a nerve root injury in 8 (22%) of 37 patients, with recovery
occurring in 7 patients and 1 was permanent. A vascular com-
plication due to a laceration of the iliac vein occurred in one
patient with a loss of 7200 mL blood. Three patients had a
pseudarthrosis that required to be repaired.
In authors opinion, hemivertebra resection is best performed
early when the scoliosis is mobile and before the development
of secondary structural changes in either the primary congeni-
tal or compensatory curves. A hemivertebra may be diagnosed
in utero by ultrasound scanning or shortly after birth on rou-
tine chest or abdominal radiographs. However, the best age for
surgery is usually in the second year of life and before the age
of 5 years. At this time, the bony anatomy of the spine has
sufficiently developed to allow the application of pediatric
pedicle screw and rod instrumentation and apply sufficient
force to close the wedge osteotomy and achieve near-total cor-
rection of both the congenital and compensatory curves. Care
must be taken not to compress the emerging nerve roots as the
osteotomy is closed, especially at the lumbosacral junction.
Fusion at the site of the hemivertebra excision is also more
likely when the vertebrae are more fully ossified.
I have found that the best long-term results after hemiverte-
bral resection with compression instrumentation are achieved
if the scoliosis can be nearly fully corrected and the spine bal-
anced in both the frontal and sagittal planes. Larger congenital
curves, especially those that have already developed secondary
structural changes or fixed compensatory curves above or
below, may not fully correct after hemivertebral resection and
continue to deteriorate. This may be partially prevented when
performing a combined anterior and posterior excision of the
hemivertebra by extending the surgery to include an anterior
and posterior convex epiphysiodesis of the normal discs one
level above and below. This, combined with a posterior growth
rod extending over the entire length of the deformity includ-
ing any residual compensatory curve, will maintain as much
longitudinal growth as possible as well as balancing the spine
and hopefully defer a definitive extensive posterior spinal
fusion until the adolescent years. Failure to stabilize the whole
length of a large residual congenital scoliotic curve with fixed
compensatory curves either above or below will lead to a slow
progressive loss of correction, which becomes more rapid dur-
ing the adolescent growth spurt and may necessitate a much
more complex vertebral column resection with instrumenta-
tion to achieve significant correction and balance the spine.
It is also possible to excise a hemivertebra in the cervical
spine or at the cervicothoracic junction by using a combined
anterior and posterior approach.30 However, this is a much
more hazardous procedure that should be attempted only by
the most experienced spinal surgeons. The surgical approach is
much more difficult because of the presence of the vertebral
arteries, which usually pass through the transverse foramen of
C6 and above. Preoperative MRI angiography is essential to
assess for any anatomical variation. The vertebral arteries are
carefully exposed both anteriorly and posteriorly at the site of
the hemivertebra resection and protected along with the nerve
roots when closing the osteotomy. Correction is achieved at sur-
gery by manipulation of the head and stabilization using ante-
rior and posterior screw and rod instrumentation as well as a
halo body jacket worn for 12 weeks. Ruf et al30 have reported
three patients with a significant tilt of the head due to a cervical
LWBK836_Ch107_p1118-1138.indd 1130
hemivertebra treated in this manner. The mean age at surgery
was 9.3 years. The mean Cobb angle was 29 before surgery and
6 after a mean follow-up of 4.8 years. One patient had a C5
palsy that resolved after change of one of the screws.
More recently, a posterior-only approach to total hemivertebral
resection has been described.28,29,31 In this procedure, the
hemivertebra is exposed posteriorly by a midline bilateral sub-
periosteal approach. By using an operating microscope or
loupes, the posterior elements of the hemivertebra are removed
along with the transverse process and medial portion of the rib
if in the thoracic region. The spinal cord and nerve roots, at
the site of the hemivertebra, are visualized and carefully pro-
tected at all times. The pedicle is entered and the body of the
hemivertebra decancellated by using a curette. Posterior exci-
sion of the body of the hemivertebra can usually be performed
relatively safely because the spinal cord has moved toward the
concavity of the curve and allows space to resect the discs above
and below the hemivertebra. The lateral aspect of the body of
the hemivertebra is exposed subperiosteally and the remnants
of the hemivertebra removed with rongeurs. The hemivertebra
lies within a fibrous capsule that is much thicker than that in
the adult and allows the body of the hemivertebra to be shelled
out with relative safety. However, during this procedure, the
restricted access and blood loss from the bone and epidural
veins, especially in young children, can make it difficult to
excise fully the discs and end plates of the adjacent vertebral
bodies. In order that the scoliosis will fully correct, it is neces-
sary to remove the entire disc to the opposite side of the spine
to create a mobile wedge osteotomy. Closure of the wedge
osteotomy can be achieved, even in very young children, by
using compression instrumentation applied to the convexity of
the curve by laminar hooks or pedicle screws inserted into the
adjacent normal vertebrae above and below. The anchor sites
for the internal fixation should be prepared before excising the
body of the hemivertebra. During closure of the osteotomy, the
spine remains relatively stable because the ligaments have been
maintained on the concavity and act as a stabilizing hinge as
the wedge is closed. Correction is achieved more easily and
safely in the mobile lumbar spine where the cauda equina is
more resilient and is more difficult in the thoracic region where
the spinal cord is less mobile and should not be manipulated
and the scoliosis is more rigid due to the supporting rib cage.
In 2001, Shono et al31 reported a single-stage posterior-only
approach in 12 patients to excise a hemivertebra that was tho-
racic in 9 and lumbar in 3. The mean age at surgery was
14 years, and the mean scoliosis reduced from 49 to 18 (mean
improvement 64%) after a mean follow-up of 5.9 years. Hook
and rod instrumentation was used, and there were no neuro-
logical complications. Nakamura et al28 in 2002 reported five
patients with a hemivertebral excision using a posterior-only
approach at a mean age of 10 years. Harrington instrumenta-
tion was used in all but one patient, and there were no neuro-
logical complications. However, the authors advised against
using this approach above the thoracolumbar region because
of the risk to the spinal cord. In the largest series of posterior-
only surgery to excise a hemivertebra, Ruf and Harms29 in 2003
reported 28 patients in whom they had used pedicle screw and
rod instrumentation to obtain correction and stability. The sites
of the hemivertebrae were in the thoracic region in 12, thora-
columbar in 12, and lumbar in 4. There were no lumbosacral
hemivertebrae. Eight of these patients also had a contralateral
bar at the same level, and this was osteotomized along with
8/25/11 10:07:30 PM

excision of concave fused ribs. Before surgery, the mean Cobb
angle of the scoliosis was 45, and this was reduced to 13 (mean
improvement 72%) after a mean follow-up of 3.5 years. All of
the patients were mobilized in the first postoperative week and
wore a spinal brace for 12 weeks. There were no neurological
complications. However, there were two pedicle fractures, three
failures of instrumentation, and one infection. Two additional
operations were necessary because of curve progression.
A concern in using pedicle screws in very young children is
that this could impair growth of the pedicles and lead to spinal
stenosis in later childhood. However, anatomical studies have
shown that the diameter of the spinal canal has reached adult
size within the first few years of life. Three of the children
treated by Ruf and Harms29 with pedicle screws inserted before
the age of 6 years had MRI and CT scans performed postopera-
tively after a minimum of 5 years. These scans showed relatively
normal growth of the instrumented vertebrae, and there was
no major retardation of the growth of the pedicles or spinal
stenosis.
In the authors opinion, the advantage of a single-stage
posterior-only approach to excise a hemivertebra combined
with pedicle screw and rod instrumentation is that it is a less
invasive procedure with less blood loss and postoperative scar-
ring and does not require a thoracotomy or chest drain that
may be associated with potential respiratory complications. In
addition, pedicle screws are less invasive within the spinal canal
than laminar hooks and are more effective in applying the
forces necessary to close the osteotomy, especially in young
children with soft bones. The disadvantage is that visualization
can be difficult, especially in young children, because of the
limited access and blood loss. Combined anterior and posterior
surgery is a more major procedure but allows for maximum
visualization with complete resection of the hemivertebra
including the adjacent discs.
IN SITU FUSION
An in situ localized spinal fusion as a prophylactic treatment
may be indicated for a congenital scoliosis due to a unilateral
unsegmented bar, with or without contralateral hemivertebrae
at the same level. These anomalies produce very rigid deformi-
ties with a known potential for severe progression. The objec-
tive of surgery is to stabilize the scoliosis at an early stage before
there is significant deformity by transforming the anomalous
segment, due to a unilateral unsegmented bar, into a block ver-
tebra with more balanced, although still restricted, longitudi-
nal growth of the spine. This is usually achieved by creating a
solid posterior spinal fusion extending over the anomalous seg-
ment and to one normal segment above and below. A convex
growth arrest procedure will not correct this type of congenital
scoliosis because there is no growth potential in the unseg-
mented bar on the concavity of the curve. An osteotomy of the
unsegmented bar is of no value as a prophylactic procedure
because the bar contains no growth plates and the osteotomy
heals rapidly and will continue to act as a unilateral tether. If a
severe scoliosis is allowed to develop, it can be significantly cor-
rected only by a vertebral column resection, which can be a
difficult and hazardous procedure.
Traditional teaching has been that patients with a unilateral
failure of vertebral segmentation are best treated prophylacti-
cally by an in situ posterior spinal fusion performed before the
age of 2 years. The argument that an early spine fusion would
LWBK836_Ch107_p1118-1138.indd 1131
Chapter 107 Congenital Scoliosis 1131
stunt the growth of the spine was thought to be of no relevance
with this type of congenital scoliosis because the abnormal seg-
ment was not contributing to vertical height and was only mak-
ing the spine more crooked. It was thought that it was much
better to achieve a short relatively straight spine that was bal-
anced than a spine that was even shorter because of the severe
curvature. However, this did not take into consideration the
possible adverse effects that a short thoracic spine might have
on the development of the thoracic cage and the space avail-
able for lung growth and development in young children.
Recent studies have shown that although an extensive spinal
fusion of four or more thoracic spinal segments performed
before the age of 5 years may control the scoliosis, it will also
have an adverse effect on normal lung growth and develop-
ment, which occurs up to the age of 8 years.16 Vitale et al35 stud-
ied the pulmonary function and the quality of life in 21 patients
with a progressive congenital scoliosis who were treated by
means of a posterior fusion performed at a mean age of 4 years
9 months (range 1 to 10 years). They found that compared with
healthy children, the pulmonary function and quality of life
scores were significantly worse when assessed, on average,
7 years after the surgery. In these circumstances, it is probably
best to modify but not necessarily exclude spinal fusion in situ
as a method of early prophylactic treatment.
Loss of correction after early posterior spinal fusion has
been attributed to the development of a pseudarthrosis, bend-
ing of a solid but weak fusion mass, adding on of normal verte-
brae to the scoliosis above and below the fused anomalous seg-
ment, and increasing rotation of the spine resulting from the
crankshaft effect produced by continuing unbalanced anterior
growth of the spine in the presence of a posterior tether caused
by the fusion.
The crankshaft effect does not usually occur to a great extent
in congenital scoliosis because the anterior growth plates in
the anomalous segment are abnormal with a diminished
growth potential. This differs from infantile and juvenile idio-
pathic scoliosis in which the anterior growth plates are rela-
tively normal. Winter and Moe36 reported a crankshaft phe-
nomenon with loss of more than 10 of correction occurring
in 3 (6%) of 49 patients treated by a posterior spinal fusion
before the age of 5 years. They subsequently reported on
290 patients with congenital scoliosis treated by a posterior spi-
nal fusion at various ages with a crankshaft incidence of 14%.38
They concluded that crankshafting was most likely in those
curves that were progressing most rapidly before spinal fusion.
Terek et al33 found that 30% of 21 patients with congenital
scoliosis had curves that progressed by more than 10 or
showed signs of increasing vertebral rotation after a posterior
fusion performed before the age of 10 years. Kesling et al19
reviewed 54 patients with congenital scoliosis treated by a pos-
terior spinal fusion before the age of 10 years and found a 15%
incidence of crankshafting, which was more frequent the ear-
lier the surgery and in curves greater than 50. The number of
discs in the fused segment and the length of the fusion were
not found to be of significance.
In the authors opinion, an in situ localized posterior spinal
fusion is best applied to a child seen before the age of 5 years
with a relatively small progressive scoliosis due to a short unilat-
eral failure of vertebral segmentation with the fusion extending
over no more than four vertebrae and this combined with a
posterior growth rod extending over the whole length of the
scoliosis from the upper to the lower neutral vertebrae. This
8/25/11 10:07:30 PM
 1132 Section IX Dysplastic and Congenital Deformities
can produce an acceptable result providing there is a strong
thick fusion extending to the tips of the transverse process
bilaterally and capable of overcoming the anterior growth in
the anomalous segment. The posterior growth rod will hope-
fully prevent increasing deformity due to adding on and tilting
into the curvature of normal and unfused vertebrae above and
below the anomalous segment. If the curve continues to dete-
riorate, early exploration of the posterior fusion is essential to
detect and repair a pseudarthrosis, strengthen a weak fusion
mass by the addition of further bone graft material or extend
the fusion.
Young children with large curves, especially those with sig-
nificant vertebral rotation and severe antero-lateral growth
imbalance resulting in a rapidly progressive scoliosis, are better
treated by an anterior fusion of the anomalous vertebral seg-
ments combined with a posterior growth rod. In these circum-
stances, a posterior fusion is not necessary because the major
growth imbalance is occurring antero-laterally in the vertebral
bodies and not in the posterior elements. This combined pro-
cedure has the advantage of directly overcoming any possible
crankshaft effect and controlling the whole of the spinal defor-
mity and allowing continued longitudinal spinal growth in the
unfused normal vertebrae included within the instrumented
levels. The rib excised at the time of the thoracotomy provides
a good source of autogenous bone graft, which may not be
available from the iliac crest in these very young children.
Postoperatively, a spinal jacket is applied for 4 to 6 months to
allow the fusion to heal.
GROWTH ROD
An extensive spinal fusion in a young child with congenital sco-
liosis may lead to stunting of the trunk. The objectives of insert-
ing a growth rod without a spinal fusion in a patient with a
congenital scoliosis are to correct the spinal deformity as much
as is safely possible and to prevent further progression and to
preserve longitudinal growth in the normal segments of the
curve.
A single rod on the concavity or two bilateral rods are
applied to the spine posteriorly extending from the upper to
the lower neutral vertebrae of the scoliosis. The single rod or
bilateral rods are contoured to the normal thoracic kyphosis
and lumbar lordosis. Each rod is cut into upper and lower seg-
ments, which are rejoined in an overlapping position by a side-
to-side tandem or axial connector placed at the thoracolumbar
junction to allow for lengthening by distraction. The rods are
placed either submuscularly or subcutaneously, so as not to
stimulate an unwanted spinal fusion, and secured to the spine
by means of hooks or pedicle screws applied in a claw pattern
spanning two levels at the upper and lower ends of the curva-
ture. Subperiosteal dissection is performed only at the upper
and lower anchor sites where local bone graft or synthetic graft
is applied around the hooks or screws to provide extra strength.
Partial correction of the scoliosis is achieved by distracting the
rods while the neurological status is assessed by using spinal
cord monitoring with evoked potentials.
Postoperatively, a spinal jacket is worn for 3 months to allow
for consolidation of the bone grafts around the anchor sites.
Further lengthening of the construct is performed every
6 months to keep up with spinal growth until such time as the
scoliosis can either be no longer controlled or preferably when
the patient is at a more appropriate age during the teenage
LWBK836_Ch107_p1118-1138.indd 1132
years when a definitive spinal fusion is performed extending
over the whole length of the deformity. The best results are
achieved when rod lengthening is performed routinely every
6 months and not at greater intervals.
As yet, there are no long-term outcome studies of this proce-
dure in children with a progressive congenital scoliosis. The
Growing Spine Study Group database1 has a 2-year follow-up on
19 patients with congenital scoliosis having single or dual
growth rod surgical treatment. The mean age at the time of the
initial implantation of the growth rod was 6.9 years. Five patients
had a failure of segmentation, 4 had a failure of formation, and
10 had mixed or unclassifiable vertebral anomalies. A mean
number of affected anomalous vertebrae per patient was 5.2
(range 2 to 9 vertebrae). The mean Cobb angle of 65 was
reduced to 45 after the insertion of the growth rod and was
47 after a mean follow-up of 3.6 years. The mean number of
lengthening was 4.3 per patient. Spinal growth and the space
available for lung development were improved, but no informa-
tion was provided on lung function studies. Five patients
reached final fusion.
Complications were common in patients with a congenital
scoliosis treated with a growth rod because of the necessity for
repeat surgery every 6 months through the same incision as well
as the mechanical stress on the implant in the absence of a spinal
fusion 1. There were 11 implant-related failures, 2 infections,
and 2 respiratory infections, but no neurological complications.
In the authors opinion, because of the growth imbalance in
the anomalous segment, a growth rod is probably best used in
conjunction with a localized in situ fusion or convex epiphysi-
odesis of a short anomalous segment of a congenital scoliosis.
The congenital scoliotic curve contains not only the anomalous
vertebrae but also a number of relatively normal vertebrae at
the upper and lower ends, within Cobb measurement, that are
also tilted into the curve. Failure to stabilize all of these verte-
brae will result in continued progression of the scoliosis. This is
best prevented at the time of the in situ fusion or convex
epiphysiodesis by the simultaneous insertion of a posterior
growth rod extending over the whole length of the scoliosis
(Fig. 107.4A to E). The growth rod may also be used to stabilize
the secondary structural scoliosis that occurs in the lower tho-
racic or thoracolumbar regions on the opposite side below a
congenital scoliosis with its apex at T4, T5, T6, or T7. The
growth rod is lengthened sequentially every 6 months with the
object of not only stabilizing the spine but also allowing contin-
ued longitudinal spinal growth in the normal and unfused ver-
tebrae within the whole deformity. It is hoped that this will
allow a definitive fusion of the whole of the deformity to be
postponed for as long as possible. Unfortunately, this technique
is ineffective in derotating the unfused vertebrae within the
curvature, and the mechanical advantage is too small effectively
to expand the lateral part of the rib cage constricted by rib
fusions.
RIB EXPANSION SURGERY
Campbell et al11,12 have pioneered the use of an opening wedge
expansion thoracostomy combined with a VEPTR in the treat-
ment of patients with a progressive congenital thoracic scolio-
sis with concave rib fusions presenting at an early age and
likely to result in a TIS. This represents a shift in emphasis
from concentrating only on the spinal deformity to managing
8/25/11 10:07:30 PM
 Chapter 107 Congenital Scoliosis 1133

A B C D

Figure 107.4. (A) A boy aged 2 years with a 30 left thoracic scoliosis due to a hemivertebra at T6. No
treatment was given. (B) By the age of 4 years 8 months, the scoliosis increased to 55, and the spine was
unbalanced. (C) An anterior convex growth arrest procedure was performed from T4 to T8 through a thora-
cotomy combined with a posterior growth rod extending from T1 to L3 performed as a single-stage combined
procedure. The scoliosis was corrected to 34. (D and E) The growth rod was lengthened every 6 months, and
E by the age of 7 years 7 months, there was a further improvement in the scoliosis to 19. The sagittal profile
has been maintained.

the related problems due to the rib cage deformity and the
possible adverse effect that this might have on thoracic volume
and lung growth. A vital capacity of less than 43% of predicted
normal associated with a scoliosis is a risk factor for eventual
respiratory failure.
The objective of an expansion thoracostomy and VEPTR is
serially to lengthen the constricted hemithorax on the concave
side of the scoliosis and increase the space available for poten-
tial lung growth and development that would otherwise deteri-
orate. In addition, the procedure has the secondary effect of
indirectly partly correcting the thoracic scoliosis and balancing
the spine and optimizing the potential for continued longitudi-
nal growth and spinal mobility.
The indications for an opening wedge thoracostomy and
VEPTR are in patients older than 1 year with a progressive con-
genital scoliosis due to four or more anomalous thoracic verte-
brae with at least three fused ribs on the concavity at the apex
of the scoliosis and a greater than 16 per reduction in the
height of the hemithorax on the concave side compared with
that on the convex side of the curve. VEPTR should not be
used in patients with a short segment spinal anomaly such as a
single hemivertebra, where the rib cage and height of the tho-
racic spine are normal. This type of patient is better treated by
a hemivertebral excision or convex epiphysiodesis.
Contraindications to the VEPTR procedure are inadequate
bone strength in the ribs for the attachment of the device and
deficient diaphragmatic function and a patient who is younger
than 6 months. However, even at 1 year or older, implanting
the device can be a problem because of the size of the patient
and the relatively large prosthesis.

LWBK836_Ch107_p1118-1138.indd 1133 8/25/11 10:07:30 PM

 1134 Section IX Dysplastic and Congenital Deformities
The technique requires a thoracotomy performed at the
apex of the scoliosis on the concave side and extended through
the congenitally fused ribs at the apex of the deformity.12 The
thoracotomy is expanded widely by using rib spreaders to cre-
ate an opening wedge thoracostomy, which is propped open by
the insertion of a prosthetic rib (VEPTR). In older children, a
second prosthetic rib is then applied vertically spanning the
thoracostomy and attached to normal ribs in the upper tho-
racic region and distally to either normal caudal ribs or subcu-
taneously as a hybrid device extending to a laminar hook in the
lumbar spine or to a hook placed over the ilium. The defect
created in the chest wall by the thoracostomy is repaired, if nec-
essary, by using a prosthetic patch. With this technique, the
bony spine is not exposed, other than at the site of the lumbar
hook, and the scoliosis is indirectly partly corrected and bal-
anced by using a distraction force in a manner similar to a
growth rod. Spinal cord monitoring using evoked potentials
should be used throughout the procedure.
Postoperatively, the prosthetic ribs are routinely lengthened
every 6 months by means of a much less invasive procedure.
The object is to maintain thoracic cage volume and the poten-
tial for lung growth before finally performing a spinal fusion at
an optimum age usually during the teenage years when the
lungs are more fully developed and the majority of spinal
growth has been completed. A brace is not used because of the
possible adverse effect that this may have on the developing
thorax.
In 2004, Campbell et al12 reported their results in 27 patients
with a congenital scoliosis and fused ribs treated by an opening
wedge thoracostomy and VEPTR. Twenty-five of these patients
had a unilateral unsegmented bar with contralateral hemiverte-
brae at the same level. The mean age at surgery was 3.2 years
and a mean follow-up duration was 5.7 years. During this
follow-up period, there was a mean 10.4 extension procedures.
Only three patients were old enough before surgery to cooper-
ate with lung function tests using spirometry. However, postop-
erative CT scanning showed that the space available for lung
growth had increased in all patients. As the children became
older, it was possible to perform postoperative serial pulmonary
function studies using spirometry in 16 patients. In these
patients, the first obtainable postoperative vital capacity was a
mean 49% of predicted normal, and at last follow-up, this was
47%. Patients treated before the age of 2 years, when the lungs
were growing most rapidly by alveolar cell multiplication, had
the most favorable percentage of normal vital capacity at the
last follow-up. In addition, there was also an indirect beneficial
effect on the severity of the congenital scoliosis. Preoperatively,
the mean Cobb angle was 74, and postoperatively, this was cor-
rected to 56, and after serial lengthening, there was further
improvement to 49 (mean improvement 34%) at the last
follow-up.
A total of 52 complications occurred in 22 of these patients.
The most common (26%) was a gradual upward migration of
the device through its attachment to the ribs with complete cut-
out after a mean of 3 years requiring reattachment at the time
of the next expansion. The laminar hook of the hybrid device
also migrated downward in 15%. Two patients had an upper
extremity brachioplexopathy, which recovered after reposition-
ing of the upper attachment. One patient who had a spinal
cord injury, occurring during resection of the concave fused
ribs with an encroachment into the spinal canal, made an
almost full recovery after 2 years. Of the lesser complications,
LWBK836_Ch107_p1118-1138.indd 1134
the most common was infection at the site of the device in 11%
and after lengthening in 2%. This was always accompanied by a
skin slough.
Emans et al16 reported 31 patients with congenital spinal
deformities associated with fused ribs treated by VEPTR and
expansion thoracostomy. The mean age at surgery was
4.2 years with a mean follow-up of 2.6 years during which time
there were a mean 3.5 device lengthenings. By using CT scan
to measure lung volume, they found that the procedure
increased volume, larger than before the surgery, but that the
chest wall on the treated side was stiffer than normal. In those
patients old enough to have their respiratory function esti-
mated by spirometry, there was deterioration in the immedi-
ate postoperative period, and this had only slightly improved
at the last follow-up.
In the authors opinion, the potential long-term benefits of
VEPTR and expansion thoracostomy on lung growth and devel-
opment have not as yet been established. There are also diffi-
culties in measuring lung function as distinct from lung volume
in very young children. It is possible that there could be an
improvement in lung growth in very young children, but it is
more likely that the major long-term effect will be to maintain
thoracic volume and prevent further deterioration in lung
function rather than any significant improvement.
The VEPTR and expansion thoracostomy also appear to
have an effect similar to a growth rod in preserving potential
longitudinal spinal growth. However, a growth rod has no effect
on preventing increasing thoracic cage deformity. In addition,
the VEPTR may have a mechanical advantage over a growth
rod. The reason for this is that the upper end of the VEPTR
construct is placed at a site on the ribs, more lateral than the
upper vertebral attachment of a growth rod, resulting in greater
leverage and a better overall balance of the spine. Campbell
et al9 have shown that a midthoracic VEPTR and opening
wedge thoracostomy has the ability to rotate a stiff upper tho-
racic and cervical spine as a unit so that it becomes more
upright with an improved cervical tilt and balanced shoulders.
This would not be possible with a growth rod. The disadvantage
of both the VEPTR and growth rod procedures is the necessity
for repeat surgery through the same incision to lengthen or
revise the implants with the possibility of skin problems and
secondary infection at the site of the surgery.
Campbell and Hell-Vocke10 have used CT scans to measure
longitudinal spinal growth in 18 patients with a unilateral
unsegmented bar with contralateral hemivertebrae and con-
cave rib fusions following primary treatment by a VEPTR and
expansion thoracostomy. Surgery was performed at a mean age
of 2.7 years and the baseline CT scan was at a mean age of
3.3 years. They found that there was no significant change in
the Cobb angle of the scoliosis after a mean follow-up of
4.2 years during which time there were a mean 7.6 expansion
procedures. The CT scan measurements showed relatively
equal but not normal growth on the concave and convex sides
of the scoliosis as well as an elongation of the unsegmented bar.
They suggested that the distracting force on the spine pro-
duced by the VEPTR unloaded the relatively normal but nar-
rowed discs on the concavity of the curve above and below the
unsegmented bar and allowed continued growth. However, the
CT scan confirmed the absence of growth plates in the unseg-
mented bar, and the reason for its elongation remains unknown
but could possibly be due to appositional bone growth. It was
hoped that if this balanced spinal growth was maintained that
8/25/11 10:07:32 PM
 Chapter 107 Congenital Scoliosis 1135

the final height of the thoracic spine at skeletal maturity would
be greater than it would have been if a spinal fusion had been
performed at an early age. Hopefully, this would have a benefi-
cial effect on maintaining the volume of the thoracic cage and
the potential for lung growth and development. However, there
has, as yet, been no long-term follow-up, and the mean age
when these patients were last seen was only 7.5 years. Further
careful follow-up is required, and in my opinion, it is possible
that there could be a recurrence of unbalanced spinal growth
during the adolescent growth spurt with further curve progres-
sion requiring additional surgery to fuse the spine before skel-
etal maturity.
CORRECTION AND SPINAL FUSION
WITH INSTRUMENTATION
Attempted correction of the deformity and posterior spinal
arthrodesis with instrumentation is the usual surgical proce-
dure for an older child with a moderate to severe congenital
scoliosis that is progressing and still relatively flexible. This may
be combined with an anterior spinal release and arthrodesis if
the curve is more severe and/or rigid. These procedures are
usually performed after the age of 12 years, when the lungs are
nearly fully formed and the majority of thoracic spinal growth
has already occurred.
The objective of the surgery is to achieve overall balance of
the spine rather than excessive correction of the congenital
curve, which could be hazardous. Posterior arthrodesis and
instrumentation is performed over the whole length of the con-
genital curve and including any upper or lower structural com-
pensatory curves that are contributing to the deformity. The
extent of the arthrodesis is usually from the neutral vertebra at
the upper end of the congenital curve or more proximal to
include an upper structural compensatory curve causing shoul-
der imbalance, to the stable vertebra at the lower end of either
the congenital or lower structural compensatory curve if pres-
ent (Fig. 107.5A to D). Although it is not usually possible to
obtain significant correction at the site of the anomalous verte-
brae, moderate correction may be achieved in the relatively
normal vertebral levels that lie above and below this area and
are still within the congenital scoliosis and in the structural
compensatory curves (Fig. 107.6A to C).
An MRI is essential in all patients before operative correction
of the deformity is attempted. This may reveal the presence of
an intraspinal anomaly, such as a diastematomyelia, that could
be tethering the spinal cord. If this tether is not removed or
released, the patient could develop serious neurological compli-
cations if a distraction force is applied to the spine to correct the
deformity. In my experience, because of the complexity of the
two operative procedures, it is best to have the intraspinal anom-
aly treated surgically as a separate neurosurgical procedure per-
formed 2 to 3 months before the attempted correction of the
spinal deformity and posterior arthrodesis. It is also best to use
titanium instrumentation, which will allow for further imaging
of the spinal cord if necessary.
The use of posterior segmental spinal instrumentation to
correct the scoliosis at the time of spinal arthrodesis will usually
achieve moderate correction. However, of the various types of
scoliosis, the congenital variety carries the highest risk of neu-
rological complications after intraoperative correction under a
general anesthetic. Hooks, pedicle screws, and sublaminar
wires have been used to secure the rods to the spine. However,
congenital anomalies affecting the lamina may make it difficult

A B C D

Figure 107.5. (A and B) A girl aged 14 years and 9 months with a 68 left upper thoracic scoliosis due to
a unilateral unsegmented bar extending from T4 to T5 combined with a 45 right lower thoracic structural
compensatory scoliosis that contains no congenital vertebral anomalies. At this time, a posterior spinal fusion
with pedicle hook, screw, and rod instrumentation was performed from T1 to L1 to include both the congen-
ital and structural compensatory curves. (C and D) At the age of 16 years 6 months, the spine was balanced
with a solid posterior spine fusion. The congenital scoliosis was corrected to 36 and the structural compensa-
tory curve to 20.

LWBK836_Ch107_p1118-1138.indd 1135 8/25/11 10:07:32 PM

 1136 Section IX Dysplastic and Congenital Deformities

A B C

Figure 107.6. (A) A boy aged 13 years 6 months with a 70 right thoracolumbar scoliosis due to a unilat-
eral unsegmented bar extending from T10 to T13 with a contralateral hemivertebra at T12. There were con-
genital rib fusions on the concavity of the curve. At this time, a posterior spine fusion with pedicle screw and
rod instrumentation was performed from T6 to L4. (B and C) At the age of 15 years 2 months, the scoliosis
remained well corrected at 42, and there was a solid fusion.

to apply these types of fixation, and it may be dangerous to pass
wires or hooks into the spinal canal at the site of the anomalous
segment, which could be congenitally narrowed or contain an
abnormal spinal cord. Pedicle screws can be difficult to insert
because of the abnormal anatomy that may obscure the land-
marks at the site of their insertion, and there may also be the
possibility of absent or defective pedicles. Attempted correc-
tion of the deformity also carries a risk of neurological compli-
cations if a significant distraction force is applied to the spine.
It is, therefore, important to use translational and compression
forces on the convexity to correct the deformity, and the instru-
mentation on the concavity of the curve should act mainly as an
internal strut to support the spine rather than to apply major
distraction.
The advantage of the spinal instrumentation is the ability to
not only correct but also to balance the spine and maintain cor-
rection until the arthrodesis is fused. It also eliminates the need
for a spinal jacket or brace and reduces the risk of pseudarthro-
sis. However, regardless of the method of instrumentation, it is
important to remember that the main objective is to achieve
trunk balance rather than excessive correction of the scoliosis,
which could result in neurological complications.
Specific measures must be taken during the surgery to detect
the possible development of neurological complications. Spinal
cord monitoring using evoked potentials is essential, but if in
doubt, the wake-up test or ankle clonus test should be used
immediately after correction of the deformity.
VERTEBRAL COLUMN RESECTION,
CORRECTION, AND ARTHRODESIS
Vertebral column resection and arthrodesis is a major salvage
procedure, which theoretically should never be necessary in
congenital scoliosis. Its use is indicative of a failure to apply the
basic principles of early diagnosis, anticipation, and prevention
of increasing deformity. Patients who require a vertebral col-
umn resection would have been much better treated at an ear-
lier stage by much simpler surgical procedures.
The indication for a vertebral column resection is a very
severe rigid scoliotic deformity with fixed spinal imbalance that
does not significantly correct on spinal radiographs taken with
the patient bending to either side. This is frequently associated
with marked decompensation of the upper trunk relative to the
pelvis as well as fixed pelvic obliquity and an apparent leg
length discrepancy. The primary objective is to restore spinal
balance in the frontal and sagittal planes.
These severe deformities usually occur as a consequence of
a unilateral unsegmented bar with or without contralateral
hemivertebrae. In these patients, a posterior arthrodesis with
instrumentation or an anterior release combined with a poste-
rior arthrodesis and instrumentation would provide only lim-
ited correction and would not achieve spinal balance. In addi-
tion, any attempt to correct the rigid spine by applying an
excessive distraction force could lead to stretching of the spinal
cord and compromise neural function.

LWBK836_Ch107_p1118-1138.indd 1136 8/25/11 10:07:33 PM


Correction of a severe rigid deformity and restoration of spi-
nal balance can be achieved only by a circumferential resection
of a segment of the vertebral column at the apex of the defor-
mity.7,32 This shortens the spine with relaxation of the neural
structures and creates immediate mobility that allows for translo-
cation of the vertebral column in three planes and the ability to
balance the head and upper trunk over a level pelvis. A spinal
osteotomy without vertebral resection provides correction only
through a hinge action in a single plane and will not allow trans-
location of the spine without neural compromise.
In 1997, Bradford and Tribus7 described a combined anterior
and posterior vertebral column resection (PVCR) with spinal shorten-
ing and posterior segmental spinal instrumentation and arthro-
desis for the treatment of 24 patients with a severe rigid scoliosis
of various etiologies causing spinal imbalance. Six of these
patients had a congenital scoliosis. The spine was first
approached anteriorly on the convexity of the scoliosis through
a thoracotomy or thoracoabdominal approach. An osteope-
riosteal flap was elevated over the vertebral bodies to be resected.
If the deformity was sharply angulated, it was usually sufficient
to excise one vertebral body at the apex of the curve. However,
if there was a long and sweeping deformity, it was often neces-
sary to excise two or three apical vertebrae to achieve balance
without stretching the spinal cord. The convex pedicle was
removed during the anterior approach but the concave pedicle
could only be partly resected with safety. They recommended
that if the anterior procedure resulted in a blood loss of less
than 1000 mL and had taken less than 3 hours, it should be pos-
sible to proceed with the posterior procedure under the same
anesthetic. If not, the posterior procedure was performed
1 week later. In the posterior approach, the entire length of the
scoliosis was exposed subperiosteally through a midline inci-
sion. Rib resections were performed as necessary on both sides
of the spine. The posterior elements of the previously anteriorly
resected vertebrae were removed including any residual pedicle.
Correction was achieved at the site of the vertebral resection by
applying segmental spinal instrumentation to shorten and trans-
late the vertebral column. The posterior spinal instrumentation
extended over the whole length of the deformity and was com-
bined with a posterior arthrodesis. The patients were mobilized
in a spinal orthosis 2 to 3 days after the surgery.
By using this technique, Bradford and Tribus7 were able to
correct a preoperative mean scoliosis of 103 to 49 (52%
improvement). Coronal and sagittal imbalances were corrected
by a mean 82% and 87%, respectively. The fusion extent was over
a mean 8.3 vertebrae, and the mean operating time was 730 min-
utes. However, there was a large mean blood loss of 5500 mL,
and complications occurred in 14 patients (58%); 3 patients had
a neurological deficit that either resolved or improved; 8 patients
had a dural tear. Three patients had a wound infection.
In an attempt to overcome some of these problems, Suk
et al32 described a technique of posterior-only surgery, in which
vertebral column resection with pedicle screw and rod instru-
mentation was performed through a single posterior midline
subperiosteal approach. Pedicle screws were first inserted with
at least four fixation points on either side of the spinal segment
that was to be resected. A single temporary rod contoured to the
shape of the deformity was placed on the concavity of the curve
and secured to the pedicle screws. The objective was to provide
stability to the vertebral column during the bony resection pro-
cedure. Vertebral resection was performed at the apex of the
deformity, and the number of vertebrae to be removed was
LWBK836_Ch107_p1118-1138.indd 1137
Chapter 107 Congenital Scoliosis 1137
determined by the type of the deformity and the degree of cor-
rection required achieving spinal balance. A total laminectomy
was performed over the site of the resection combined with
foraminal unroofing to expose the nerve roots. The transverse
processes and corresponding ribs were removed as necessary on
both sides of the spine. Subperiosteal resection was then per-
formed down the lateral wall of the vertebral body on the con-
vexity to its anterior surface. The pedicle and lateral portion of
the vertebral body on the convexity was removed along with the
adjacent discs. The temporary fixation rod was then moved to
the convex side and the same bony resection performed on the
concavity along with removal of the posterior wall of the verte-
bral body. After this circumferential vertebral body resection,
and to avoid inadvertent distraction of the neural elements, the
vertebral column was shortened by slight compression on the
instrumentation over the resected gap. The deformity was then
corrected by exchanging the temporary rod with precontoured
rods to the degree of correction required in all three planes.
These rods were sequentially changed from those with minimal
correction to moderate and then to the final desired shape. The
exchanges were done alternating from one side of the spine to
the other to avoid loss of shortening of the resected gap or
uncontrolled displacement. Special reduction pedicle screws
were also used to gradually bring the vertebral column to the
prebent contoured rods using translocation, rotation, and com-
pression maneuvers to correct the scoliosis and maintain short-
ening of the vertebral column. Any bony defect in the anterior
column was filled with either bone chips or titanium mesh filled
with bone chips. The patients were mobilized 24 hours after sur-
gery in a spinal jacket, which was worn for 6 months.
By using this technique, Suk et al32 in 2005 reported their
results in 16 patients with a very rigid scoliosis and spinal imbal-
ance similar to those described by Bradford and Tribus.7 Three of
these patients had a congenital scoliosis due to a unilateral unseg-
mented bar. The indication for PVCR was a rigid scoliosis more
than 80 with spinal imbalance and flexibility less than 25%. The
preoperative mean scoliosis of 107 was corrected to 46 (59%
improvement). Coronal and sagittal imbalances were corrected
by a mean 79% and 55%, respectively. The fusion extent was over
a mean 10.6 vertebrae. The operating time was a mean 370 min-
utes, with a mean blood loss of 7034 mL, which was even greater
then the mean blood loss reported by Bradford and Tribus.7
Complications occurred in four patients (25%), complete paraly-
sis (one patient), hematoma (one patient), hemopneumothorax
(one patient), proximal junction kyphosis (one patient).
In the authors opinion, a vertebral column resection by either
a combined anterior and posterior procedure or a
posterior-only approach is a technically demanding procedure
with significant intraoperative blood loss and neurological risks.
These procedures should be performed only by an experienced
spinal surgeon after full consultation with the patient or par-
ents and only if there are no alternatives. Both procedures pro-
vide similar degrees of correction of a severe rigid scoliosis and
spinal imbalance. However, both procedures may result in a
large blood loss from the epidural veins and the venous sinuses
within the vertebrae. In these circumstances, preparation
should be made for significant blood replacement, and surgery
is contraindicated if the patient will not tolerate a large blood
loss. The advantage of a posterior-only approach is that it
requires only a single-stage procedure with a reduction in oper-
ating time when compared with combined anterior and poste-
rior surgery. A thoracotomy is not required, and this may allow
8/25/11 10:07:34 PM
 1138 Section IX Dysplastic and Congenital Deformities
surgery to be performed on patients with reduced lung func-
tion. Neurological complications can occur in both procedures
either by direct trauma to the spinal cord occurring at the time
of the bony resection or if there is a sudden uncontrolled dis-
placement of the vertebral column once the bony resection has
been completed. Excessive shortening of the vertebral column
can also result in neural impairment due to dural buckling.
Spinal cord ischemia is a rare but possible complication after
extensive bony resection, especially in the midthoracic region
where the segmental blood supply to the spinal cord is nor-
mally poorest or may already be congenitally defective. The
advantage of pedicle screw and rod instrumentation, which can
be applied to both procedures, is that it provides stability
throughout the bony resection and the ability for controlled
intraoperative correction of the deformity in three dimensions
with spinal reconstruction and rigid postoperative fixation. The
absence of secure bony anchors for rigid internal fixation is a
contraindication for the procedure.
SUMMARY
Congenital scoliosis is a potentially serious condition that can,
in some patients, result in an extreme spinal deformity with
malalignment of the body and possible constriction of the tho-
racic cage in young children interfering with normal lung
growth and function.
The objective of surgery is to correct or prevent increasing
spinal deformity and produce a spine, which, at the end of
growth, will be as balanced as possible in the frontal and sagittal
planes with as short a spinal fusion segment as possible and
preserving respiratory function.
The most common errors in management are (1) failure to
recognize the significance of congenital vertebral anomalies
when seen on radiographs taken in infancy, for reasons other
than the spine, and before there is an obvious clinical deformity;
(2) failure to observe and prevent progression of the scoliosis
before there is a severe rigid deformity of the spine or the tho-
rax; and (3) failure to surgically stabilize the whole of the spinal
deformity including any structural compensatory curves.
The key to successful management depends on three princi-
ples. First, early diagnosis prior to 5 years of age, before the scolio-
sis exceeds 40 and before the compensatory curves have become
fixed or there is a significant deformity of the thorax. Second,
the ability to anticipate what is likely to happen based on the type
and sites of the vertebral anomalies, as well as the age of the
patient and the amount of spinal growth remaining. Third, the
application of prophylactic surgical treatment at an early age to bal-
ance the growth of the spine and, in some patients, prevent
increasing deformity of the thorax. A scoliosis that is at risk of
progressing requires immediate prophylactic treatment, no mat-
ter how young the patient. It is much better to conduct relatively
simple surgery at an early age than to wait until the scoliosis has
become severe and rigid when it is necessary to perform hazard-
ous surgical salvage procedures with possible neurological com-
plications and a result that is likely to be less than optimal.
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