Congenital scoliosis is the curvature due to imbalance of growth of the spine caused by the presence of vertebral defects. Most of these defects develop during the first 6 weeks of embryological life.
Classification of Congenital Scoliosis
Failure of formation
- Partial failure of formation (wedge vertebra)
- Complete failure of formation (hemivertebra)
Failure of segmentation
- Unilateral failure of segmentation (unilateral unsegmented bar)
- Bilateral failure of segmentation (block vertebra)
In addition to this regions of the spine involved are also mentioned in the classification like the cervicothoracic spine, thoracic spine, thoracolumbar spine, and lumbosacral spine.
Unilateral unsegmented bar with a convex hemivertebra produces most progressive curve followed by unilateral unsegmented bar and a double convex hemivertebra. Thoracolumbar curves have maximum progression, followed by lower thoracic and upper thoracic regions. The least severe scoliosis is caused by a block vertebra.
The deformity produced by a failure of formation is much more difficult to predict than that caused by failure of segmentation.
Diastemotmyelia, tethered cord, spinal cord tumor, kyphosis, arnold chiari malformation and syringomelia in spine are frequent associated abnormalities. Apart from this congenital heart defects, pulmonary dysfunction,cor pulmonale and back pain can be associated.
The patient is evaluated as per routine spinal evaluation first. In addition, specific findings should be looked for in congenital scoliosis
In addition to the routine spinal evaluation, some specific physical findings should be sought in patients with congenital scoliosis.
- Hair patches, lipomata, dimples, and scars may indicate an underlying vertebral defect and should be looked for.
- A complete neurological evaluation should be done.
- Clubfoot, calf atrophy, absent reflexes, and limb atrophy could be signs of neurological involvement or associated anomalies
Imaging in Congenital Scoliosis
Anteroposterior and lateral radiographs are the basic imaging studies required for this. Sserial radiographic investigation would tell about the progression of the curve.The radiograph also let us know if the curve is due to failure of segmentation or a failure of formation.
Growth potential of the curves should be estimated to better determine the possibility of curve progression.
Myelography or MRI should be considered if diastematomyelia or a neurological abnormality is suspected. Some authors recommend urological evaluation of all patients with congenital scoliosis with ultrasound.
Treatment of Congenital Scoliosis
Nonoperative treatment has a limited role in congenital scoliosis.
Nonprogressive curves require regular observation with six monthly xrays.75% of congenital curves are progressive and only 5% to 10% can be treated with bracing
Brace management can be considered for
- Long, flexible curves
- Curves correctable on traction or on side bending
- Curves with a mixture of anomalous and nonanomalous vertebrae.
Short, sharp, and rigid curves do not respond to brace treatment.
Surgery remains the main treatment in congenital scoliosis.
Following operative procedures are used in treatment of congenital scoliosis
- Posterior fusion without instrumentation
- Posterior fusion with instrumentation
- Combined anterior and posterior fusion
- Combined anterior and posterior convex hemiepiphysiodesis
- Hemivertebra excision
- Instrumentation without fusion
Get more stuff on Musculoskeltal Health
Subscribe to our Newsletter and get latest publications on Musculoskeletal Health your email inbox.
Thank you for subscribing.