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Valerie Touitou, Zoltan Szatmary, Nader Sourour, Bahram Bodaghi, Phuc LeHoang; Carotido-cavernous Fistulas: Anatomical And Clinical Correlations. Invest. Ophthalmol. Vis. Sci. 2012;53(14):4895.
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To recognize clinical features of direct and indirect carotidocavernous fistulas (CCF) and compare them to the anatomical features on the angiogram.
Patients seen between Nov 2009 and Nov 2010 in a single tertiary center, with angiogram-confirmed diagnosis of CCF, were retrospectively reviewed. Functional and clinical manifestations were reviewed and compared with the antomical site of the CCF on the angiogram.
Six patients (3F/3M) were included in this study. Four patients had a direct CCF (DCCF), and two patients had an indirect CCF (ICCF). Mean age at diagnosis was 59.7 years for patients with DCCF, and 60 years for patients with ICCF. All patients with DCCF presented with pulsatile proptosis (mean 3.5mm), dilatation of episcleral vessels, and a moderate to severe orbital bruit. Diplopia was reported by three patients (75%). Patients with ICCF had no episcleral vessel dilatation, no propotosis (mean : 0.5mm) but complained of tinnitus. One patient had binocular diplopia (50%) one patient denied any other symptoms. Both patients had a mild to moderate bruit, best heard in the orbital region for one patient, and in the pretragial area for one patient. When comparing the clinical symptoms and the angiogram, posterior drainage was observed in all cases with tinnitus. A bruit in the pretrageal or temporal area was related to ICCF, whereas an orbital bruit was related to DCCF. The intensity of the bruit was related to the CCF flow on the angiogram.Mean delay between onset of symptoms and diagnosis was 7 weeks for DCCF and 24 weeks for ICCF. A history of trauma was reported in two patients (50%) in the DCCF group, and one patient (50%) in the ICCF group.
High flow CCFs (DCCFs) are often caracterized by severe ophtalmological involvement from orbital congestion, allowing prompt diagnosis. ICCFs have a more torpid evolution, responsible for a major diagnostic delay. Tinnitus is very evocative of CCFs, and particularly of posteriorly draining ICCF. Facial bruit is pathognomonic of CCFs and was heard in all patients. The location in which it is best heard, and its intensity seem to be related to the anatomic type of the CCF on the angiogram. ICCF are life-threatening conditions which are frequently misdiagnosed. Diagnostic delay is significantly longer than for DCCF. Our study demonstrates that, although the diagnosis of ICCF is challenging because of the absence of congestive orbital signs, it can be suspected on clinical examination, with a good correlation between the clinical signs and the angiogram findings.
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