Ruptured dissecting aneurysm in bilateral iliac arteries caused by Ehlers-Danlos syndrome type IV: Report of a case

Ehlers-Danlos syndrome (EDS) is an inherited disorder of connective tissue characterized by hyperextensible skin, hypermobile joints, and abnormalitie s of the cardiovascular system. Ten types and several subtypes of EDS have so far been recognized based on genetic, clinical, and biochemical characte ristics. The spectrum of the disorder varies from mild to life-threatening vascular complications. EDS type IV is a particularly dangerous form with a lethal spontaneous rupture of the major arteries and aneurysmal formation. We present herein a case of a ruptured dissecting aneurysm in the bilatera l iliac arteries caused by EDS type IV. A previously healthy 33-year-old ma n without any physical features of this connective tissue disorder experien ced a metachronous vascular rupture two times. Successful synthetic bypass grafting was performed with great difficulty. The diagnosis of EDS type IV was made afterwards based on an electrophoresis analysis of a skin biopsy s pecimen which revealed a lack of type III collagen. Surgical intervention i n cases of arterial complications in EDS type IV patients have been reporte d to be both difficult and frequently unsuccessful. The early clinical reco gnition of this syn drome is therefore of great importance due to the hazar ds of such surgical therapies.