Spontaneous carotid-cavernous fistula and the Ehlers-Danlos syndromes

A 22-year-old woman with one of the Ehlers-Danlos syndromes (EDS) developed a spontaneous carotid-cavernous fistula (CCF), demonstrated by ophthalmic ultrasound and cerebral angiography. Therapy consisted of two balloon embolization procedures that significantly reduced blood flow through the fistula. The patient subsequently had a cerebral hemorrhage and expired. The cardinal features of EDS and its ophthalmologic findings are reviewed. Spontaneous CCF have been described in EDS patients, but case documentation is lacking in the ophthalmic literature. The rationale for treating a CCF and the various therapeutic approaches are discussed; the serious risks involved in any attempt to save vision in these patients are considered.