Spontaneous carotid-cavernous fistula and multiple arterial dissections in Type IV Ehlers-Danlos syndrome

Abstract

A 43-year-old woman without phenotypic expression of Ehlers-Danlos syndrome developed a spontaneous unilateral carotid-cavernous fistula (CCF). Attempts at balloon occlusion of the CCF were unsuccessful, and caused multiple arterial dissections and lacerations eventually leading to massive retroperitoneal hemorrhage and death. The CCF was demonstrated during postmortem studies. In addition, the patient had independent dissections of the internal carotid artery at the site of the fistula and an intracavernous aneurysm of the contralateral internal carotid artery. Despite widespread dissections, the major arteries showed only mild histological abnormalities. Morphometric analysis of collagen from the aorta revealed an increase in large-sized fibers, consistent with deficiency of Type III collagen.