Spinal deformities in Marfan's syndrome

Abstract

Marfan's syndrome is an hereditary disorder of the connective tissue which in its most classic form includes cardiovascular, ocular and skeletal manifestaions. Scoliosis is frequently present (40-75 per cent); the type of curve is similar to that seen in idiopathic scoliosis, but progression is often much more severe. One of our patients, a woman aged thirty-two who had never been treated, had two 180 degree curves. There is a high incidence (50 per cent) of double curves (right thoracic and left lumbar) and a 23 per cent incidence of triple curves. Vertebral inversion (thoracic lordoscoliosis) is very common and often accompanied by severe respiratory insufficiency, particularly if it is associated with funnel chest. Conservative treatment is almost invariably ineffective and should be reserved only for very slight curves. All curves of 50 degrees or more should be surgically corrected. A 40-50 per cent correction can be achieved without risk. In view of the high incidence of pseudarthrosis, systematic review and, if necessary, revision of the arthrodesis is to be recommended. In the more severe cases of kyphosis it is advisable also to perform an anterior arthrodesis. Surgery is contraindicated in the presence of severe mitral or aortic insufficiency or aortic aneurysm.