Studies have shown that about 20% of people with EA have some degree of deformity of the chest wall, and between one-quarter and one-half of people with EA have a mild (or greater) curve of the spine, called scoliosis. The chest deformity is sometimes an indentation of the front of the chest, called pectus excavatum. This usually doesn’t affect health. Other patients have different heights of the shoulders.
Scoliosis, on the other hand, can compress the lungs, and sometimes needs surgery. Many factors play a role in chest deformities and scoliosis in people with EA. Congenital abnormalities of the ribs and vertebrae occur in about a quarter of VATER or VACTERL Association, and can cause scoliosis. Patients with scoliosis from this cause are more likely to need surgery. Major chest surgery early in life can lead to ribs and the muscles between the ribs getting scarred together. This reduces growth of the chest cage on that side, and also increases the risk of surgery. For many people, both factors play a role. Modern surgical techniques avoid affecting the ribs as much as possible, reducing the risk of this complication, and, hopefully, with new endoscopic techniques, this complication can now be prevented.
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