Carotid-cavernous fistulas

Kim Rickert MD (Dr. Rickert of the Guthrie Clinic has no relevant financial relationships to disclose.)
Steven R Levine MD, editor. (Dr. Levine of the SUNY Health Science Center at Brooklyn has received honorariums from Genentech for service on a scientific advisory committee and a research grant from Genentech as a principal investigator.)
Originally released June 30, 1999; last updated May 21, 2017; expires May 21, 2020

This article includes discussion of carotid-cavernous fistulas, carotid-cavernous sinus dural arteriovenous malformation, carotid-cavernous fistulae, carotid-cavernous sinus fistulas, CCF, CCSF, direct carotid-cavernous sinus dural arteriovenous malformation, direct carotid-cavernous fistula, indirect carotid-cavernous sinus dural arteriovenous malformation, indirect carotid-cavernous fistula, aneurysms of the cavernous sinus, and arteriovenous fistulas. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Carotid-cavernous fistulas are abnormal connections between the cavernous sinus and the internal carotid artery, external carotid artery, their branches, or both. There are 2 broad categories of carotid-cavernous fistulas—direct and indirect—each of which has a markedly different clinical presentation. In this article, the author discusses the clinical presentation, pathogenesis, and novel methods of diagnosis for this disease entity. This article provides an in-depth review of the current treatments for carotid-cavernous sinus fistulas, including the endovascular repair via transarterial or transvenous embolization.

Key points

 

• Carotid-cavernous sinus fistulas are direct communications between the cavernous sinus and the carotid arterial system.

 

• “Direct” carotid-cavernous sinus fistulas are abnormal communications between the internal carotid artery and the cavernous sinus, whereas “indirect” carotid-cavernous fistulas are aberrant connections between the branches of the internal or external carotid arterial system and the cavernous sinus.

 

• “Direct” carotid-cavernous sinus fistulas are typically caused by head trauma or a ruptured cavernous carotid aneurysm and present in a dramatic fashion with proptosis, ophthalmoplegia, and loss of vision. “Indirect” or “dural” carotid-cavernous fistulas typically present in elderly women with hypertension in a more subtle fashion. Both types of carotid-cavernous sinus fistulas can be closed successfully with modern neuro-interventional techniques.

Historical note and terminology

Carotid-cavernous fistulas are abnormal communications between the cavernous sinus and the carotid arterial system, which includes the internal carotid artery, external carotid artery, and their meningeal branches. Historically, carotid-cavernous fistulas have been classified into 2 broad categories: direct and indirect. “Direct” or “high flow” carotid-cavernous fistulas are defined as direct connections between the internal carotid artery and cavernous sinus, whereas “indirect” or “dural” carotid-cavernous fistulas are characterized by connections between the meningeal branches of the internal carotid artery or external carotid artery and the cavernous sinus. A more specific anatomical-angiographic classification for carotid-cavernous fistulas was proposed by Barrow and colleagues who differentiated between 4 types of carotid-cavernous fistulas (Barrow et al 1985). Type A is a direct fistula between the cavernous internal carotid artery and cavernous sinus and is most often caused by a traumatic tear in the arterial wall or a rupture of an cavernous carotid aneurysm.

Image: Direct carotid-cavernous fistula, type A
Type B is a fistula between meningeal branches of the internal carotid artery and cavernous sinus. Type C is a fistula between meningeal branches of the external carotid artery and cavernous sinus. Lastly, Type D is a fistula between meningeal branches of both the internal carotid artery and external carotid artery and the cavernous sinus.
Image: Carotid-cavernous fistula, type D: digital angiography of the external and interal carotid artery
Thus, the traditional “direct” carotid-cavernous fistula is equivalent to a Type A carotid-cavernous fistula, whereas “indirect” (or “dural”) carotid-cavernous fistulas encompass Types B, C, and D. This classification gives the treating physician a more precise anatomical guideline on which treatment can be based.

The content you are trying to view is available only to logged in, current MedLink Neurology subscribers.

If you are a subscriber, please log in.

If you are a former subscriber or have registered before, please log in first and then click select a Service Plan or contact Subscriber Services. Site license users, click the Site License Acces link on the Homepage at an authorized computer.

If you have never registered before, click Learn More about MedLink Neurology  or view available Service Plans.