Aortic dissection: A rare complication of osteogenesis imperfecta

Osteogenesis imperfecta (OI) is an inherited connective tissue disorder, a group that includes Ehlers-Danlos syndrome, Marfan's syndrome and pseudoxan thoma elasticum. OI is a heterogeneous disease of collagen I biosynthesis c haracterized by variable clinical phenotypes, including skeletal and cardio vascular manifestations. A 65-year-old man with OI who had extensive prior successful cardiac valve surgeries is described. He survived for 18 years a fter his initial valve surgery, but died of multiorgan failure and sepsis a fter repair of a spontaneous type A aortic dissection. This is the fourth r eported case of aortic dissection secondary to OI and illustrates the exten sive cardiovascular pathology associated with OI. Aggressive management of arterial dissection risk factors, such as systemic arterial hypertension, i s advocated for patients with OI.