Objective:

We report a case of a carotid cavernous fistula (CCF) with arterial blood flow originating from bilateral anterior internal maxillary arteries and contralateral meningeal artery branches of the internal carotid artery into the left cavernous sinus.

Background:

Carotid cavernous fistulas are rare vascular abnormalities caused by abnormal shunting between the carotid artery and the cavernous sinus. They are classified by hemodynamics (high or low flow), etiology (traumatic vs. nontraumatic), or anatomical location. Spontaneous CCFs are often linked to ruptured aneurysms, fibromuscular dysplasia, or Ehlers-Danlos syndrome type IV. The incidence is approximately 0.37 per 100,000 people annually.

Design/Methods:

A 79-year-old male with a history of benign prostatic hyperplasia presented with a 3-week history of left eye pain, edema, and decreased visual acuity. Physical examination revealed left eye proptosis, chemosis, periorbital edema, ophthalmoplegia, and near-total vision loss without pulsatile exophthalmos. Orbital CT showed reduced enhancement of the left cavernous sinus with a prominent superior ophthalmic vein (SOV). MRI confirmed these findings. CTA identified a CCF from the left cavernous internal carotid artery (ICA) draining into the left SOV. A subsequent cerebral angiogram confirmed a Type D CCF of the left cavernous sinus, supplied by bilateral internal maxillary artery branches and meningeal branches from the right ICA, with retrograde flow into the dilated SOV.

Results:

The patient underwent successful catheterization and transvenous coil embolization of the left SOV and cavernous sinus.

Conclusions:

While CCFs are rare, they should be considered in patients presenting with ophthalmoplegia, proptosis, and chemosis, even in the absence of pulsatile exophthalmos. CCFs can be supplied by contralateral or bilateral arteries traversing the cavernous sinus.