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.
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 authors discuss the clinical presentation, pathogenesis, and diagnostic steps for this disease entity. Furthermore, this article provides an in-depth review of the current and recently described treatments for carotid-cavernous sinus fistulas, including the most common strategy of endovascular repair via transarterial or transvenous embolization.
• 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 cavernous sinus, while “indirect” carotid-cavernous fistulas are aberrant connections between the branches of the internal or external carotid arterial system.
• “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.
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