Indirect bypass surgery as a line of management of pediatric moyamoya disease

Abstract

Moyamoya disease (MMD) is a chronic, rare cerebrovascular disorder of unknown cause featured by extensive narrowing of the terminal internal carotid artery (ICA) and its main branches. As a compensatory mechanism, perforators dilate forming fragile collateral vessels that appear as an abnormal vascular network resembling ‘puff of smoke drifting in the air’ observed via angiography around stenotic area in the brain. The database of the current hospital was retrospectively studied. All patients with ischemic strokes owing to MMD treated with surgical revascularization between January 2015 and December 2022 were included. MMD was identified on catheter-based digital subtraction angiography (DSA) by the presence of stenosis at the terminal portions of both internal carotid arteries and the development of abnormal collateral “moyamoya vessels” around the base of the brain. Clinical, radiological and follow-up data were retrospectively analyzed. During the mentioned time window, nine patients were diagnosed with moyamoya disease among all pediatric patients with established diagnosis of ischemic stroke in our institute; six of them underwent surgery. Unilateral EDMS was performed in 5 cases (83.3%), while one patient (16.67%) underwent staged bilateral surgeries with no added neurological deficit. Indirect revascularization surgery for pediatric moyamoya-related stroke patients has high rates of reperfusion and promising outcomes.