Osteogenesis imperfecta and aortic dissection.

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.