抄録
We report a 14-year-old girl with Marfan syndrome whose pulmonary function improved after surgical treatment for scoliosis. The patient was refered to our hospital with dyspnea, and progressive scoliosis. Her spine had a right convex single thoracic curve (T5—12), and the Cobb angle was 100°. The curve was corrected to 60°with traction. It was classified as King and Moe type III, and Lenke type 1A-. The curvature had progressed from 40°at 11 years of age to 100°just prior to the operation. Her pulmonary function was impaired with a % vital capacity (%VC) of 58.7%. First, we performed anterior release (T7—10) and bone graft. Two weeks later, posterior correction using the Cotrel-Dubousset system was performed with rib resection on the right side between the 7 th rib to 10 th, and between 5 th rib and 10 th on the left side. The curve was corrected to 60°just after surgery. Two years after the final operation, the Cobb angle was 76°with a correction loss of 26.6%. Her pulmonary function had improved to a %VC of 89.7%.
Scoliosis associated with Marfan syndrome tends to be progressive, and has many complications. Fortunately, this case had no severe complications, and had a good clinical course after operation. We considered that rib resection was useful in correcting the pulmonary dysfunction in this case.
