Carotid cavernous fistula is a well-known clinical and angiographic entity responsible for ocular signs and symptoms. On the contrary, ocular signs are unusual in the presentation of cranial dural arteriovenous fistulas at locations other than the cavernous sinus. We retrospectively analyzed data focusing on the pathophysiology of ophthalmologic signs and their angiographic explanations. Thirteen patients were included with a mean age of 50 years. The most common signs were chemosis (61.5%), loss of visual acuity (38.5%), exophthalmia (38.5%), and ocular hypertension (7.7%). Dural arteriovenous fistulas presenting with ocular signs could be classified into 4 types due to their pathologic mechanism (local venous reflux into the superior ophthalmic vein, massive venous engorgement of the cerebrum responsible for intracranial hypertension, compression of an oculomotor nerve by a venous dilation, or intraorbital fistula with drainage into the superior ophthalmic vein).
