Carotid Cavernous Fistula CCF

This is a high flow shunting of blood from the carotid artery into the cavernous sinus. It is usually due to carotid trauma. Other causes are intracavernous aneurysm, Ehlers-Danlos syndrome, fibromuscular dysplasia, and pseudoxanthoma elasticum. Ocular signs and symptoms are pulsatile exopthalmos, periorbital congestion, opthalmoplegia (even a "frozen" globe), and increased intraocular pressure.

Two features distinguish CCF from cavernous sinus thrombosis: an audible supraorbital bruit (may or may not be present) and the presence of arterialized corkscrew episcleral veins. Marked enlargement of the superior opthalmic vein is easily visible on a CT or MRI. CCF is life threatening due to the possibility of intracranial hemorrhage or massive epistaxis. Vision loss, seizures and neurologic deficits are bad prognostic signs. Rapid diagnosis is critical. Transarterial balloon embolization is the preferred initial treatment, with a success rate over 90 %.