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A 56-year-old man presented with subacute injection of his right eye and diplopia. He had no head trauma. His exam demonstrated right eye proptosis, chemosis (Fig. 1a), afferent pupillary defect, abducens nerve palsy (Video, available online), decreased visual acuity, and increased intraocular pressure. Non-contrast CT revealed right superior ophthalmic vein distention (Fig. 1b) suggesting vascular pathology. Non-enhanced MRI demonstrated increased signal intensity in the right cavernous sinus (Fig. 1c) consistent with a carotid cavernous fistula (CCF). Indirect CCF was confirmed by cerebral angiography.
Demonstrated is the patient’s right abducens nerve palsy. (M4V 2028 kb)
CCFs are classified using the Barrow classification.1 Type A CCFs are direct high-flow connections between the internal carotid artery and cavernous sinus. Young men are at highest risk, as the major cause is head trauma.2 The presentation is abrupt, and headache and bruits (subjective or auscultated over the globe) are common. Type B–D CCFs are low-flow, indirect connections; risk factors include atherosclerosis, hypertension, diabetes, and collagen disease. Indirect CCFs are more common in postmenopausal women.3 Unlike direct CCFs, they typically lack a bruit and are more insidious in onset. Direct CCFs are at risk of progression and morbidity without closure.4 Indirect CCFs rarely result in morbidity and may spontaneously close. Our patient’s ocular findings spontaneously improved.
References
Barrow DL, Spector RH, Braun IF, et al. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg. 1985;62(2):248–256.
Lewis AI, Tomsick TA, Tew JM Jr. Management of 100 consecutive direct carotid-cavernous fistulas: results of treatment with detachable balloons. Neurosurgery. 1995;36:239–244.
Biousse V, Mendicino ME, Simon DJ, Newman NJ. The Ophthalmology of Intracranial Vascular Abnormalities. Am J Ophthalmol. 1998;125:527–544.
Naesens R, Mestdagh C, Breemersch M, Defreyne L. Direct carotid-cavernous fistula: a case report and review of the literature. Bull Soc Belge Ophtalmol. 2006;299:43–54.
Acknowledgments
We would like to thank Dr. Izlem Izbudak, Associate Professor of Neuroradiology at Johns Hopkins Hospital, for her help with image interpretation and selection.
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Bailey, C.R., Ray-Mazumder, N. & Sedighi Manesh, R. Carotid Cavernous Fistula. J GEN INTERN MED 32, 483–484 (2017). https://doi.org/10.1007/s11606-016-3860-6
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DOI: https://doi.org/10.1007/s11606-016-3860-6