Osteogenesis imperfecta and aortic dissection

Abstract

Osteogenesis imperfecta (OI) is a group of autosomal dominant disorders affecting Type I collagen synthesis with the predominant clinical manifestations in skeleton, skin, eyes, and teeth. Valvular cardiac manifestations and aortic root changes have long been reported. However, aortic dissection and repair were not described until 1993, with only 3 more proximal aortic dissections and repair noted. We report a fifth case and the first distal dissection. Surgeons performing vascular operations will in all probability never have encountered vascular repair in those with OI. The reports and this case are strong evidence that special efforts are required because of the fragility of tissues and propensity for bleeding. Reinforcement of any vascular suture line should be considered to reduce failure and bleeding.