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Dural arteriovenous fistula presenting with exophthalmos and seizures

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Abstract

Concomitant seizures and exophthalmos in the context of a temporal dural arteriovenous fistula (dAVF) has not been described before. Here, we report a 55-year-old-male who presented with an 8-month history of progressive painless exophthalmos of his left eye, conjunctival chemosis, reduced vision and new onset complex partial seizures. Cerebral angiography demonstrated Cognard Type IIa left cerebral dAVF fed by branches from the left occipital artery and an accessory meningeal artery, with drainage to the superior ophthalmic vein. Following surgical obliteration of dAVF feeding vessels, our patient had dramatic improvement in visual acuity, proptosis and chemosis along with cessation of clinical seizures.

Introduction

Dural arteriovenous fistulas (dAVFs) are rare dural-based pathologic shunts representing 10–15% of all cerebrovascular malformation [1]. Although there are several classification systems for intracranial dAVFs, Cognard classification (Types I–V) that correlates with venous drainage patterns with increasingly aggressive neurological clinical course, is most widely used and accepted [2]. dAVFs can also be classified based on their location: transverse-sigmoid sinus, cavernous sinus, tentorum cerebri, superior sagittal sinus and anterior cranial fossa dAVFs. Concomitant seizures and exophthalmos in the context of direct carotid-cavernous sinus dAVF was reported once [3]; however, this association has not been described with a temporal dAVF. Herein, we report a patient with a dAVF who presented with an 8-month history of progressive painless exophthalmos of his left eye, conjunctival chemosis, reduced vision and new onset complex partial seizures (CPS).

Section snippets

Case report

A previously healthy 55-year-old-male presented with an 8 month history of progressive painless exophthalmos of his left eye, associated with increasing conjunctival injection, worsening diplopia, and reduced vision. One day prior to admission, he became confused and was witnessed to have three generalized tonic–clonic seizures. On admission, he was somnolent but arousable. Neurologic examination also revealed non-pulsating exopththalmos of the left eye with periorbital ecchymosis, conjunctival

Discussion

Presenting signs and symptoms, management, and risk of hemorrhage of dAVFs vary greatly depending on the type, location, size, progression, and direction of drainage [1]. Symptoms include headache, proptosis, conjunctival chemosis, papilledema, tinnitus, seizures, vision loss, diplopia, and various degrees of motor and sensory deficits [2]. Anterior fossa and cavernous sinus dAVFs classically present with ophthalmoplegia, proptosis, chemosis, retro-orbital pain, or decreased visual acuity

Conflict of interest

None of the authors have any conflicts of interest to state.

Disclosures

None.

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    The use of AEDs alone often fails to control seizures in patients with DAVF owing to the nature of venous congestion–related epileptogenicity, while DAVF treatment in combination with AEDs can effectively control seizures in these patients.17,19 Based on these findings, seizure-related DAVFs should be treated aggressively through endovascular intervention or microsurgical disconnection, which have been shown to rapidly reverse venous hypertension and to reduce potential epileptogenicity.20,21 We retrospectively reviewed 48 patients with seizure-associated DAVFs that were successfully treated at our center.

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    However, a more common mechanism involves arterialization of the SOV due to changes in the venous drainage pattern. Noncavernous AVFs with eye symptoms often reside near the orbit,3,4 but some dural AVFs with extensive rerouting of venous drainage may be located remote from the CS.5-8 We report the unusual case of a dural AVF involving the vein of Trolard (VOT) presenting with eye symptoms where the communication to the CS was not immediately obvious from the diagnostic angiogram.

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