Fatality in a Type IV Ehlers-Danlos Syndrome Patient Following Rupture of an Abdominal Aortic Aneurysm 1 Year after Treatment for a Ruptured Subclavian Artery

Abstract

Type IV Ehlers-Danlos syndrome (EDS) is characterized by many vascular complications, including aortic and visceral arterial ruptures, aneurysms, and dissection. The surgical management of these complications is exceedingly challenging because of the high morbidity and mortality resulting from the profound fragility of the arterial tissue. We report a case of a 20-year-old man with type IV EDS associated with a subclavian arterial rupture, who was successfully treated with transcatheter coil embolization, but who suddenly died because of rupture of dissecting abdominal aortic aneurysm 1 year later.