Moyamoya disease is a rare chronic cerebrovascular disease seen both in children and adults. It has a progressive course, but may have a variable clinical presentation. The disease causes ischemic stroke, intracranial hemorrhage, headache, seizures, and transient ischemia attack in children and in adults. Although the pathogenesis of the disease remains unknown, research suggests a genetic predisposition. There are also undefined systemic processes involved in this vasculopathy. Better noninvasive diagnostic techniques for diagnosis of the Moyamoya disease have been developed, but medical treatment can still be challenging. However, various surgical revascularization procedures have shown to provide symptomatic benefit in a majority of these patients. In addition, the anesthetic management of these patients has evolved over the years with an increased understanding of the disease. These have specifically resulted from the identification of risk factors for perioperative complications and outcomes related to the use of anesthetic agents. Finally, research in the last 3 decades has led to the recognition of the importance of pain control, the increased use of regional anesthesia, and better monitoring techniques in providing high quality and safe patient care to patients with Moyamoya disease. This article will provide a comprehensive review of the disease and its anesthetic management.

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