JKOS Journal of the Korean Ophthalmological Society J Korean Ophthalmol Soc 0378-6471 2092-9374 Korean Ophthalmological Society 10.3341/jkos.2016.57.6.977 jkos-57-977 Case Report A Case of Cavernous Sinus Dural Arteriovenous Fistula Following Tolosa-Hunt Syndrome ParkAram MD KimSeung Woo MD ChungSeung Ah MD, PhD Department of Ophthalmology, Ajou University School of Medicine, Suwon, Korea Address reprint requests to Seung Ah Chung, MD, PhD Department of Ophthalmology, Ajou University Hospital, #164 World cup-ro, Yeongtong-gu, Suwon 16499, Korea Tel: 82-31-219-5257, Fax: 82-31-519-5259 E-mail: mingming8@naver.com 09 2016 25 09 2016 576 977 982 29102015 19012016 25012016 Copyright © 2016 Korean Ophthalmological Society 2016 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Abstract Purpose

To report a case of cavernous sinus dural arteriovenous fistula following Tolosa-Hunt syndrome.

 Case summary

A 64-year-old female with a history of hypertension, presented with blepharoptosis and periorbital pain in the right eye and diplopia. Her right pupil was dilated. She had right exotropia and right hypertropia with inability to elevate, depress, and adduct the right eye. Magnetic resonance imaging including angiography, revealed hyperintensities in the right cavernous sinus consistent with inflammation and no vascular abnormalities. Three days after oral corticosteroid therapy, the pain disappeared. A presumptive diagnosis was Tolosa-Hunt syndrome presenting as a form of complete oculomotor nerve palsy. Two months later, she experienced severe pain in the right periocular area, even though blepharoptosis was resolved and ocular motility was improved. A rapid response to high-dose intravenous corticosteroids was consistent with recurrence of Tolosa-Hunt syndrome. Three months later, she had normal ocular motility, but developed a conjunctival injection, pulsatile orbital bruits, and exophthalmos in the right eye. Cerebral angiography showed a cavernous sinus dural arteriovenous fistula. She received a transvenous coil embolization and her symptoms markedly improved over 2 months.

 Conclusions

Tolosa-Hunt syndrome, a granulomatous inflammation in the cavernous sinus may be followed by cavernous sinus dural arteriovenous fistula and should be considered during follow-up.

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Clinical findings and imaging at initial presentation. (A) Gaze photographs showing severe limitation on supraduction, adduction, and infraduction in the right eye. (B) Photographs showing blepharoptosis, and dilated pupil in the right eye. (C) Brain magnetic resonance angiography (MRA) not showing any abnormality such as aneurysmal dilatation or compressed lesion. (D) Gadolinium enhanced T1-weighted brain magnetic resonance image (MRI) showing increased signal intensity in the right cavernous sinus, indicated by red circle. P = posterior aspect; A = anterior aspect.

Gaze photographs 2 months after initial presentation when she suffered from severe periocular pain and headache, showing resolution of blepharoptosis and improvement in ocular motility in the right eye.

Clinical findings and imaging 5 months after initial presentation. (A) Gaze photographs showing resolution of the right oculomotor nerve palsy, but severe redness in the right eye. Slit lamp photographs showing engorged vessels on the conjunctiva of right eye. (B) Cerebral angiography showing arteriovenous shunt from the right middle meningeal artery and ascending pharyngeal artery, supplied from the right external carotid artery to the cavernous sinus (type C in Barrow classification,10 red arrow, left column), and no more shunt after coil embolization (right column).