Spontaneous common iliac arteries rupture in Ehlers-Danlos syndrome type IV: report of two cases and review of the literature.

Abstract

Two patients with previously undiagnosed Ehlers-Danlos syndrome type IV (EDS IV) presented acutely with clinical features suggestive of hypovolemic shock. Emergency laparotomies in both of them revealed spontaneous rupture of the common iliac arteries. The clinical features, operative findings, surgical approach, outcome and implications are discussed.