Simultaneous unilateral moyamoya disease and ipsilateral dural arteriovenous fistula: case report.

Abstract

Previous reports have described an association between moyamoya disease and arteriovenous malformations, but this unusual case is the first report of a unilateral moyamoya pattern simultaneously associated with an ipsilateral dural arteriovenous fistula (dAVF). A 44-year-old man presented with headache, tinnitus, and an intraventricular hemorrhage, as seen on computed tomographic scans. Cerebral angiography showed a right moyamoya pattern and an ipsilateral dAVF fed by branches of the external carotid artery and draining into the transverse sinus. After the intraventricular hemorrhage resolved, the patient underwent transarterial embolization of the dAVF with Onyx (EV3, Inc. Irvine, CA), followed by a direct superficial temporal artery-to-middle cerebral artery bypass. Subsequent reconstitution of the dAVF required transvenous embolization, which obliterated the dAVF. At his 2-month follow-up examination, the patient was asymptomatic and neurologically intact. Recent investigations have established that both moyamoya disease and dAVFs are associated with dural angiogenesis. These factors may represent a mechanism for vaso-occlusive ischemia contributing to the formation of dAVF. This extremely rare coincidental presentation may have deeper pathogenic implications.