Vascular Manifestations of Type IV Ehlers-Danlos Syndrome

Ehlers-Danlos syndrome (EDS) is a hereditary disorder of the connective tissue. Ten different types of EDS have been described, most of which are associated with skin hyperflexibility and joint hypermobility. The type most frequently encountered by surgeons is the arterial ecchymotic type, or type IV. The phenotypical variance that characterizes EDS can make recognition difficult. The diagnosis is often made after vascular or gastrointestinal complications have occurred. We are describing a young pregnant patient with EDS type IV who developed a carotico-cavernous fistula, a crural artery pseudoaneurysm and died due to spontaneous intestinal rupture.