Review of Ehlers-Danlos syndrome. Successful repair of rupture and dissection of abdominal aorta.

Abstract

Successful surgical treatment of spontaneous rupture and dissection of the abdominal aorta in Ehlers-Danlos syndrome has not been previously reported. A 16-year-old male sustained spontaneous rupture and dissection of the abdominal aorta. Successful surgical treatment included placement of an abdominal aortic bifurcation graft. Genetical, biochemical and clinical differences of seven types of the syndrome are outlined. A brief guideline for treatment and prevention of vascular complications is discussed.