Atypical Presentation of Direct Carotid Cavernous Fistula

Abstract

An atypical case of carotid cavernous fistula (CCF) is presented which was challenging to diagnose even with investigations. A 50-year old female, presented with complaints of increasing chemosis, diplopia and proptosis of her right eye following a fall from stairs. Her IOP was 32 mm Hg in right and 18 in left eye. Absence of other classic clinical signs like bruit/thrill of carotid cavernous fistula led to difficulty in diagnosing. There was absence of radiological evidence like enlargement of superior ophthalmic vein on MRI, MRV and MRA. We started symptomatic treatment for increased IOP and exposure keratopathy. Right sided CCF diagnosis was confirmed on CT angiogram and Digital Subtraction Angiography. Coil embolization was done which resulted in symptomatic and clinical relief. Carotid cavernous fistula with absence of typical signs is sometimes challenging to diagnose and treat. Importance of Digital Subtraction Study and CT Angiogram is highlighted.