Carotid cavernous fistula in a patient with Ehlers-Danlos syndrome type IV: a case report

Abstract

Objective: We report a case of carotid cavernous fistula (CCF) in a patient with the vascular type Ehlers-Danlos syndrome (EDS), type IV .
Case presentation: A 41-year-old female complained of sudden onset of left eye pain and presented with conjunctival congestion without trauma. The left internal carotid artery showed a high flow direct carotid cavernous fistula draining into the left superior ophthalmic vein and the cortical vein retrogradely. The lesion was successfully treated with transarterial coil embolization. After endovascular surgery, hemorrhagic events (retroperitoneal hemorrhage, internal iliac artery extravasation, and splenic artery aneurysm) recurred. We suspected vascular type EDS, which was confirmed biochemically. A COL3A1 gene mutation was identified.
Conclusion: In view of the risks of hemorrhagic events after arteriography, early diagnosis of the vascular type EDS is important in patients with spontaneous direct carotid-cavernous fistulas.