Carotid-Cavernous Fistula (CCF)

Updated: Sep 05, 2017
  • Author: Ingrid U Scott, MD, MPH; Chief Editor: Hampton Roy, Sr, MD  more...
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Overview

Background

Carotid-cavernous sinus fistula is an abnormal communication between the internal or external carotid arteries and the cavernous sinus. These lesions may be classified based on the following: etiology (traumatic vs spontaneous), velocity of blood flow (high vs low), and anatomy (direct vs dural, or internal carotid vs external carotid).

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Pathophysiology

Carotid-cavernous sinus fistulae occur because of traumatic or spontaneous rents in the walls of the intracavernous internal carotid artery or its branches. This results in short-circuiting of the arterial blood into the venous system of the cavernous sinuses. [1]

Direct carotid-cavernous sinus fistulae, which represent 70-90% of all carotid-cavernous sinus fistulae in most series, are characterized by a direct connection between the intracavernous segment of the internal carotid artery and the cavernous sinus. These fistulae usually have high rates of arterial blood flow and most commonly are caused by a single traumatic tear in the arterial wall.

Dural carotid-cavernous sinus fistulae are characterized by a communication between the cavernous sinus and one or more meningeal branches of the internal carotid artery, external carotid artery, or both. These fistulae usually have low rates of arterial blood flow and almost always produce symptoms and signs spontaneously, without any antecedent trauma or manipulation. The lesions may represent congenital arteriovenous malformations, which develop spontaneously or in association with atherosclerosis, systemic hypertension, collagen vascular disease, pregnancy, and during or after childbirth.

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Epidemiology

Frequency

United States

Carotid-cavernous fistula is rare.

International

Carotid-cavernous fistula is rare.

Mortality/Morbidity

Nearly all patients with a direct carotid-cavernous sinus fistula experience progressive ocular complications if the fistula is left untreated. Increasing proptosis, conjunctival chemosis, and visual loss occur over months to years, with central retinal vein occlusion and secondary glaucoma representing the most severe ocular complications.

Several investigators have reported severe epistaxis and intracerebral hemorrhage, potentially fatal, in patients with traumatic carotid-cavernous sinus fistulae. Subarachnoid hemorrhage also may complicate the course of a traumatic carotid-cavernous sinus fistula. A 3% incidence of spontaneous intracerebral hemorrhage caused by carotid-cavernous sinus fistulae has been reported.

Visual loss, although less frequent than in patients with direct carotid-cavernous sinus fistulae, occurs in 20-30% of patients with dural carotid-cavernous sinus fistulae and may be due to secondary ischemic optic neuropathy, chorioretinal dysfunction, including central retinal vein occlusion, or uncontrolled glaucoma.

Sex

While direct carotid-cavernous sinus fistulae generally are associated with trauma or surgical manipulation, dural carotid-sinus fistulae occur more commonly in middle-aged to elderly women.

Age

Traumatic carotid-cavernous sinus fistulae occur more commonly in young individuals.

Dural carotid-cavernous sinus fistulae usually occur in middle-aged to elderly women but may produce symptoms at any age, including infancy.

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