Moyamoya disease: pathophysiology and anesthetic management

Moyamoya disease, an ischemic cerebrovascular disease, is characterized by a slowly increasing bilateral occlusion of the internal carotid circulation. Although collateral pathways are formed, therapy is aimed at further increasing blood flow by surgical anastomosis before ischemic events and fixed neurologic defects occur. This disease remains one of the few indications for performance of the operation of superficial temporal artery to middle cerebral artery anastomosis. Anesthetic considerations involve increasing substrate supply and decreasing demand for its use. Two cases of moyamoya disease are described, noteworthy not only because of the rarity of the disease but because of its occurrence in the Hispanic race rather than the traditional appearance in those only of Japanese descent. The anesthetic management is outlined and the literature reviewed.