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      Carotid Cavernous Sinus Fistula with Contralateral Feed Not Diagnosed by Virtual Assessment or by Non-Invasive Vascular Imaging


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          A 67-year-old woman had delayed initial diagnosis of her right low flow carotid cavernous fistula (CCF) during the coronavirus disease (COVID-19) pandemic due to difficulty detecting ocular signs via online virtual examinations. Her right eye conjunctival erythema and proptosis with medial rectus enlargement on computed tomography scan was initially misdiagnosed as euthyroid thyroid-associated orbitopathy without lid retraction. She developed vision loss, and increasing episcleral venous congestion and CCF was suspected. Computed tomographic angiography did not show an obvious fistula. Digital subtraction angiography revealed the right-sided low flow CCF, which was fed from vessels from the contralateral side.

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          Most cited references9

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          Classification and treatment of spontaneous carotid-cavernous sinus fistulas.

          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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            The epidemiologic characteristics and clinical course of ophthalmopathy associated with autoimmune thyroid disease in Olmsted County, Minnesota.

            G Bartley (1993)
            Among incident cases of GO in Olmsted County, Minnesota: GO affected females six times more frequently than males (86% versus 14% of cases, respectively). The age-adjusted incidence rate was 16 cases per 100,000 population per year for females and 2.9 cases per 100,000 population for males. The peak incidence rates were bimodal, occurring in the age groups 40 to 44 years and 60 to 64 years in females and 45 to 49 years and 65 to 69 years in males. Among patients with GO, approximately 90% had Graves' hyperthyroidism, 1% had primary hypothyroidism, 3% had Hashimoto's thyroiditis, and 5% were euthyroid. Eyelid retraction was the most common ophthalmic feature of autoimmune thyroid disease, being present either unilaterally or bilaterally in more than 90% of patients at some point in their clinical course. Exophthalmos of one or both eyes affected approximately 60% of patients, restrictive extraocular myopathy was apparent in about 40% of patients, and optic nerve dysfunction occurred in either one or both eyes in 6% of patients with autoimmune thyroid disease. Only 5% of patients had the complete constellation of classic findings: eyelid retraction, exophthalmos, optic nerve dysfunction, extraocular muscle involvement, and hyperthyroidism. Upper eyelid retraction, either unilateral or bilateral, was documented in approximately 75% of patients at the time of diagnosis of GO. Lid lag also was a frequent early sign, being present either unilaterally or bilaterally in 50% of patients at the initial examination. At the time of diagnosis of GO, the most frequent ocular symptom was pain or discomfort, which affected 30% of patients. Some degree of diplopia was noted by approximately 17% of patients, lacrimation or photophobia was present in about 15% to 20% of patients, and 7.5% of patients complained of blurred vision. Decreased vision attributable to optic neuropathy was present in less than 2% of eyes at the time of diagnosis of GO. Thyroid dermopathy and acropachy accompanied GO in approximately 4% and 1% of patients, respectively. Myasthenia gravis occurred in less than 1% of patients. Superior limbic keratoconjunctivitis was documented in less than 4% of patients. The median age at the time of diagnosis of GO was 43 years (range, 8 to 88). Among patients with hyperthyroidism, 61% developed ophthalmopathy within 1 year of the onset of thyrotoxicosis. Symptoms and signs for which statistically significant changes occurred between the initial and final examinations included lacrimation, pain or ocular discomfort, photophobia, eyelid retraction, lid lag, eyelid fullness, conjunctival injection, chemosis, and exophthalmos.(ABSTRACT TRUNCATED AT 400 WORDS)
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              Carotid-cavernous fistula: current concepts in aetiology, investigation, and management

              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.

                Author and article information

                Case Rep Ophthalmol
                Case Rep Ophthalmol
                Case Reports in Ophthalmology
                S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.com )
                May-Aug 2021
                24 August 2021
                24 August 2021
                : 12
                : 2
                : 712-716
                [1] aOphthalmology, University of Toronto, Toronto, Ontario, Canada
                [2] bNeurology, University of Toronto, Toronto, Ontario, Canada
                [3] cInterventional Radiology, University of Toronto, Toronto, Ontario, Canada
                Author notes
                Copyright © 2021 by S. Karger AG, Basel

                This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.

                : 9 June 2021
                : 20 July 2021
                : 2021
                Page count
                Figures: 3, References: 10, Pages: 5
                Case Report

                carotid cavernous fistula,dural fistula,digital subtraction angiography,contralateral feed


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