Surgical resection of giant fibrous dysplasia for near respiratory collapse.

Abstract

Fibrous dysplasia may involve the ribs or thoracic spine and cause progressive asphyxiation. We present a 41-year-old man with polyostotic fibrous dysplasia who was admitted to the hospital with progressive shortness of breath requiring initiation of supplemental oxygen. Pulmonary function test results revealed severely limited function with forced expiratory volume in 1 second (FEV1) of 14% predicted and diffusion capacity of 17%. As a lifesaving effort, the patient was offered resection, decortication, and chest wall reconstruction, after which the lung reexpanded. At 6 months, his FEV1 was 49% and his diffusion capacity was 56%. He no longer required supplemental oxygen and now exercises daily.