Management of Concomitant Moyamoya Disease, Arterial Venous Malformation, and Intracranial Aneurysm: Case Illustration, Literature Review, and Management Algorithm

Abstract

Background: Moyamoya disease (MMD), arterial venous malformations (AVMs), and intracranial aneurysms are distinct cerebrovascular disease processes that most commonly occur in isolation. The literature on the treatment algorithm of each individual condition is well established. An association between MMD and intracranial aneurysms is also known, but MMD in association with AVM is rare.

Case descriptions: The authors discuss various dilemmas in the multimodality management of these conditions when they coexist with an illustrative case of a 46-year-old woman who presented following a stroke, with 1) left-sided MMD, 2) left-sided frontal AVM, and 3) an aneurysm arising from the A1 segment of the right anterior cerebral artery. These were managed respectively by 1) the left external carotid artery to M2 segment of middle cerebral artery bypass using an autologous radial artery graft and left indirect superior temporal artery-encephaloduroarteriosynangiosis, 2) stereotactic radiosurgery, and 3) endovascular coiling of the aneurysm. Three years following intervention, cerebral angiography showed a patent bypass, complete obliteration of the AVM, and no residual filling of the coiled aneurysm.

Conclusions: Our strategy of surgical revascularization for MMD, radiosurgery for AVM, and endovascular coiling for aneurysm resulted in a positive long-term clinical outcome. In view of the rarity of the condition, the authors propose a management algorithm for such patients.

Keywords: Arterial venous malformation; Cerebral aneurysm; EC-IC bypass; Endovascular coiling; Moyamoya disease; Stereotactic radiosurgery.