Definition:

Congenital scoliosis is a lateral curvature of the spine that is due to the presence of vertebral anomalies causing an imbalance in the longitudinal growth of the spine. Most congenital scoliosis is often recognized at birth, but more subtle spinal defects can remain undetected. A close interaction of genes and environment regulates the development of normal spine. Developmental studies in animal models have identified many genes regulating somite formation and segmentation. Patients with congenital scoliosis frequently have other associated anomalies. Sometimes rib fusion or absence can be observed along with the spinal anomalies since ribs are formed in close association with the vertebrae. When the number of ribs on the right or left side do not match, congenital vertebral anomalies should be suspected. An extensive thoracic congenital scoliosis associated with fused ribs may affect thoracic function and the growth of the lungs in young children and lead to a thoracic insufficiency syndrome. The vertebral expandable prosthetic titanium rib (VEPTR) holds the expanded hemithorax. This improves the thoracic height, lung volume and lung function.