Congenital scoliosis is the abnormal development of the spine resulting
in a missing portion, partial formation, or lack of separation of the
vertebras. Unlike Idiopathic Scoliosis,
it is not believed to be hereditary.
The spine and major organs develop between three and six weeks in utero
(after conception). Therefore, it is common to see other congenital malformations
such as heart problems, kidney or bladder problems, and spinal cord malformations
in patients with Congenital Scoliosis. Congenital malformations are relatively
rare.
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 will include a physical examination for other
congenital anomalies. Standing full spine x-rays will
allow your doctor to diagnose the type and severity of
the congenital vertebral malformations, and track the
progression of the curvature over time. An MRI may be
ordered to determine if there are spinal cord abnormalities.
After Congenital Scoliosis is confirmed by x-rays, a
renal ultrasound is generally performed to rule out renal
abnormalities such as a single kidney.
Congenital Scoliosis has three classifications: Failure of formation, Failure
of segmentation, and a combination of both. Failure of formation
usually presents as a Hemiverterbra (portion of a vertebra).
A hemivetebra creates an imbalance in the spinal column forcing the
spine to curve as the child grows.
Failure of segmentation can present as a Block Vertebra (where vertebras
are fused together) or as a Unilateral Bar (when on side of the
vertebras fuse together) 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.
The treatment plan is based on the the progression
of the spinal curvature. The chances of progression
of the curve (in all congenital scoliosis cases):
50% > 30 degrees
25% (5-30 degrees)
25% are non-progressive.
Each patient is unique and a treatment plan is made based on the risk of
curve progression during growth. The treatment options include observation,
bracing, and surgical intervention. 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. Early surgical intervention
is vital to prevent the worsening of the curvature as the patient grows.
To view video of imaging navigation for a complex scoliosis procedure,
click on the image below. The surgical images may not be suitable for all
audiences.
