Anaesthetic management of children with Moya Moya Disease

Moyamoya syndrome (MMD) is a progressive occlusive cerebrovascular disorder that usually presents as recurrent strokes in children. Distal internal carotid and basilar arteries gradually narrow, leading to proliferation of penetrating arteries, primarily at the base of the brain. Moya moya in japanese, means "something hazy, like a puff of cigarette smoke drifting in the air." Because of precarious cerebral circulation these patients represent an anaesthetic challenge. The risk factors for perioperative complications, predominantly the cerebral ischemic events in patients with MMD are: history of transient ischemic attacks, severity of disease, type of revascularistion procedure, significant reduction in hematocrit, intraoperative hypotension, intraoperative hypercapnia and reduction in circulating blood volume.(1-3) We describe the anaesthetic management of a case undergoing surgery for moya moya disease.