Carotid-cavernous fistula: current concepts in aetiology, investigation, and management

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Abstract

A carotid-cavernous fistula (CCF) is an abnormal communication between arteries and veins within the cavernous sinus and may be classified as either direct or dural. Direct CCFs are characterized by a direct connection between the internal carotid artery (ICA) and the cavernous sinus, whereas dural CCFs result from an indirect connection involving cavernous arterial branches and the cavernous sinus. Direct CCFs frequently are traumatic in origin and also may be caused by rupture of an ICA aneurysm within the cavernous sinus, Ehlers-Danlos syndrome type IV, or iatrogenic intervention. Causes of dural CCFs include hypertension, fibromuscular dysplasia, Ehlers-Danlos type IV, and dissection of the ICA. Evaluation of a suspected CCF often involves non-invasive imaging techniques, including standard tonometry, pneumotonometry, ultrasound, computed tomographic scanning and angiography, and/or magnetic resonance imaging and angiography, but the gold standard for classification and diagnosis remains digital subtraction angiography. When a direct CCF is confirmed, first-line treatment is endovascular intervention, which may be accomplished using detachable balloons, coils, liquid embolic agents, or a combination of these tools. As dural CCFs often resolve spontaneously, low-risk cases may be managed conservatively. When invasive treatment is warranted, endovascular intervention or stereotactic radiosurgery may be performed. Modern endovascular techniques offer the ability to successfully treat CCFs with a low morbidity and virtually no mortality.

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Most cited references 61

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Classification and treatment of spontaneous carotid-cavernous sinus fistulas.

G T Tindall, Saori I. Braun, S C Tindall … (1985)

An anatomical-angiographic classification for carotid-cavernous sinus fistulas is introduced and a series of 14 patients with spontaneous carotid-cavernous sinus fistulas is reviewed to illustrate the usefulness of such a classification for patient evaluation and treatment. Fistulas are divided into four types: Type A are direct high-flow shunts between the internal carotid artery and the cavernous sinus; Type B are dural shunts between meningeal branches of the internal carotid artery and the cavernous sinus; Type C are dural shunts between meningeal branches of the external carotid artery and the cavernous sinus; and Type D are dural shunts between meningeal branches of both the internal and external carotid arteries and the cavernous sinus. The anatomy, clinical manifestations, angiographic evaluation, indications for therapy, and therapeutic options for spontaneous carotid-cavernous sinus fistulas are discussed.

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Dural carotid cavernous fistula: definitive endovascular management and long-term follow-up.

Philip M Meyers, Van V. Halbach, Christopher Dowd … (2002)

To describe the endovascular treatment and clinical outcome in patients with indirect carotid cavernous fistulas (CCFs) over a 15-year period. To our knowledge, this is the largest series in the medical literature. Interventional case series. A retrospective evaluation of 135 consecutive patients who underwent examination and treatment for indirect CCF was performed. Patients received independent evaluations by ophthalmologists, neurologists, or neuro-ophthalmologists before, during, and after endovascular treatment. Patients initially received noninvasive imaging followed by cerebral arteriography for definitive diagnosis and stratification by angiographic risk factors. Endovascular treatment was performed in 133 (98%) patients and clinical follow-up was achieved in 135 (100%) patients on an average of 56 +/- 4.3 months (range: 2 months-14 years). Angiographic follow-up was performed in 72 (54%) patients with ongoing symptoms or a history of fistula with high-risk angiographic features. Arteriographic cure with long-term clinical outcome is summarized by modified Rankin scale (mRS) and Barthel index (BI). At a mean follow-up of 56 months, 121 (90%) patients were clinically cured. At latest clinical follow-up, 131 (97%) patients showed good recovery (mRS, 1-2; BI 90-100), one (1%) had moderate disability (mRS, 3; BI, 50-60), and three (2%) (mRS, 4; BI, 40-50) were severely disabled. Procedure-related permanent morbidity was 2.3%. There was no operative mortality. With the observed favorable outcomes and low rate of procedural morbidity in this patient population with long-term angiographic and clinical follow-up, endovascular therapy should be the primary treatment for patients with indirect (dural) fistulas of the cavernous sinus.

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Carotid-cavernous and orbital arteriovenous fistulas: ocular features, diagnostic and hemodynamic considerations in relation to visual impairment and morbidity.

R Keizer (2003)

The author investigated 101 cases with direct dural carotid-cavernous and orbital arteriovenous fistulas (CCF). The characteristic clinical findings, such as specific epibulbar arterialized loops, are described and the differential diagnosis of the striking diagnostic triad (exophthalmos, the above-mentioned loops and glaucoma) is discussed, together with the exclusion criteria for other causes of red eyes, episcleral measurements and blood flow. The results of various diagnostic procedures, such as ultrasonography, Doppler hematotachography and color Doppler of the orbit and carotid systems, magnetic resonance imaging and angiography, and of conservative treatment and embolization processes are dealt with successively. The classification of different types of carotid-cavernous fistulas is presented,(1-3) together with the clinical signs in relation to morbidity and mortality during or after conservative or intervention therapies. The importance of patient follow-up, in the clinic as well as with Doppler methods, is emphasized in order to differentiate a progressive or diminished clinical condition caused by spontaneous thrombosis in the healing process or more arteriovenous flow. A 'decision tree' for use in daily practice is provided. In this study, of the 101 cases in which the localization was diagnosed by angiography, 42 were direct (30 traumatic, 12 spontaneous), 31 were dural (3 traumatic, 28 spontaneous) and 10 were orbital CCFs. In 18 other cases, usually dural or orbital shunts, angiography was not performed. For the management of 42 direct fistulas, conservative treatment was used in 12 cases (7 with success; 58%) and balloon embolization was performed in 18 cases (17 with success; 94.5%); the other cases were treated by direct or indirect surgery. Of the 48 (spontaneous and traumatic) dural fistulas, 39 were treated conservatively (32 recovered or were much improved: 82%, of the total cases, 67%). All seven cases in which embolization was performed were cured and/or much improved. In two cases, one fistula was conservatively treated while one was embolized at another location, both with success. Of the 10 orbital arteriovenous shunts showing signs of dural fistulas, the features disappeared in 8 cases, although after a much longer follow-up period than for the typical dural carotid-cavernous sinus fistulas; in one patient, direct surgery was performed successfully and in one patient the original, non-progressive, orbital features could still be observed.