Spontaneous carotid-cavernous fistula and multiple arterial dissections in type IV Ehlers-Danlos syndrome. Case report.

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 …