A Case of Spontaneous Bilateral Direct Carotid-Cavernous Fistula

A 92-year-old female with poorly controlled systemic hypertension presented with bilateral eye redness, lid fullness, conjunctival chemosis, ophthalmoplegia, and ptosis for two days. A neuro-ophthalmic evaluation revealed bilateral proptosis, severe conjunctival chemosis and congestion, and an almost complete bilateral ophthalmoplegia with a complete right superior eyelid ptosis. Computed tomography (CT) scans demonstrated bilateral dilation of the superior ophthalmic veins, and a CT angiography (CTA) showed a direct high-flow carotid-cavernous fistula (CCF) with secondary extraocular muscle enlargement. Clinicians should be aware that a typical direct high-flow CCF, although usually occurs after trauma and unilaterally, can present spontaneously without trauma and bilaterally.