Evaluation of cerebral blood flow and hemodynamic reserve in symptomatic moyamoya disease using stable Xenon-CT blood flow.

Moyamoya disease is a vascular abnormality seen in children and adults characterized by progressive narrowing of the internal carotid, middle, anterior, and posterior cerebral arteries and the development of leptomeningeal and proximal internal carotid artery collaterals, which appear diaphanous on angiogram. Although adults tend to present with subarachnoid hemorrhage and children with ischemic events, the clinical sequelae in these two populations overlap. Expanding upon work done at this institution using stable xenon computer tomographic blood flow determinations with acetazolamide and carbon dioxide challenge to predict which population of patients with severe carotid disease and hemodynamic compromise would benefit from surgical intervention, we used similar rationale to determine which patients with moyamoya disease would likely benefit from revascularization. Data and outcome concerning four such patients make up the body of this report.