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.