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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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