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Congenital
Scoliosis
Congenital scoliosis is
defined as a curvature of the spine that is the result
of malformations of the vertebral elements. The fact
that the spine and spinal column ever form correctly is
amazing given the complexity of the process from an
embryological standpoint. Most of this development
happens during the third to sixth week in utero (after
conception). In spite of the opportunities for error,
congenital malformations are relatively
rare.

The elements of the spinal
column develop at the same time as several other major
organ systems such as the bladder, kidneys, and heart.
As a result, there is an association between congenital
vertebral anomalies and other malformations. For
example, there is a 20% incidence of associated
genitourinary anomalies in children with congenital
scoliosis. Malformations of the spinal cord are also
common, and medical attention should be sought if there
is any suggestion of an intraspinal problem such as
dimples or hairy patches over the skin of the back,
pain, or spasticity in the lower
extremities.
If your child is thought to
have congenital scoliosis, the evaluation should begin
with a thorough physical examination in search of
associated congenital anomalies. Taking x-rays of the
entire spine will determine if congenital scoliosis is
present. X-rays will also allow your doctor to determine
the type and severity of the congenital vertebral
malformations, which helps predict the long-term risk
that your child's curve may get worse with growth. A
renal ultrasound is usually recommended as part of the
routine screening assessment if congenital scoliosis has
been confirmed. An MRI of the spinal cord may be
indicated if there is suspicion of a spinal cord
abnormality or unexplained rapid progression of the
curve.
Doctors think about
congenital scoliosis in three groups: failures of
formation, failures of segmentation, and combinations of
these defects. The most common failure of formation is
called a hemivertebra. Hemivertebra produce a growth
imbalance in the spine and, therefore, result in the
spine growing crooked. Failures of segmentation include
block vertebra and unilateral bars, which produce a
growth tether of the spine. Finally, when these occur in
combination, such as a hemivertebra on one side and a
bar on the other, the scoliosis can progress in very
rapid manner.
When your doctor recommends a
treatment plan for congenital scoliosis, it is based on
the prediction of the potential abnormal growth
associated with your child's particular pattern of
spinal malformations. Some patterns of congenital
scoliosis have a low potential for significant
progression with growth, whereas others will progress
aggressively at a very early age. Each pattern of
malformation is unique and a treatment plan is made
based on the risk of curve progression during
growth.
Treatment options in congenital
scoliosis include observation, bracing, or surgery. The
goals of treatment are to allow the child to reach the
end of growth with a reasonably straight, balanced
spine, and to allow the spine to grow as much as
possible. For younger children, allowing for the chest
cavity to grow and develop and allowing the lungs to
increase in size is becoming increasingly recognized as
an important consideration in the overall treatment
plan.
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