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Moyamoya disease: Treatment and prognosis

Author
Nijasri Charnnarong Suwanwela, MD
Section Editors
Jose Biller, MD, FACP, FAAN, FAHA
Douglas R Nordli, Jr, MD
Deputy Editor
John F Dashe, MD, PhD

INTRODUCTION

Moyamoya disease is a unique chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation.

This topic will review the prognosis and treatment of moyamoya. Other aspects of the disease are discussed separately. (See "Moyamoya disease: Etiology, clinical features, and diagnosis".)

ACUTE MANAGEMENT

For children and adults with moyamoya and acute stroke, acute treatment is mainly symptomatic and directed towards reducing elevated intracranial pressure, improving cerebral blood flow, and controlling seizures [1]. Ventricular drainage and/or hematoma removal is often required for patients with intracerebral hemorrhage.

During hospitalization for acute stroke or surgery, the following management issues deserve special attention, especially in children with moyamoya [2]:

Precautions to minimize crying and hyperventilation: both can lower PaCO2 and thereby induce or worsen cerebral ischemia by causing vasoconstriction [2,3].

        

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Literature review current through: Nov 2016. | This topic last updated: Tue Jan 05 00:00:00 GMT+00:00 2016.
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