Carotid-cavernous fistula in ehlers-danlos syndrome by pure transvenous approach

We describe a carotid-cavernous fistula (CCF) in a middle aged woman with Ehlers-Danlos syndrome (EDS) type IV, which manifested with a left-sided ophthalmoplegia. The CCF was diagnosed on magnetic resonance imaging. To prevent potential lethal arterial wall injury, the CCF was treated endovascularly under local anesthesia and exclusively by a transvenous approach. The fistula was successfully closed with Guglielmi Detachable Coils. Notwithstanding these precautionary measures, the patient suffered an intraperitoneal and a small retroperitoneal bleed during the procedure and died suddenly ten days after intervention in hemorrhagic shock. A review of recent literature focussing on the technique of transvenous approach and the catheterization risks of CCF in Ehlers-Danlos syndrome is presented.