Under general anesthesia, with the patient in supine position, head kept neutral and flexed so that there is good exposure of the calvaria, a bicoronal skin flap was marked. Adenosine brain SPECT study for evaluation of cerebrovascular reserve in stress and at rest showed a matched perfusion defect in the right temporo-occipital area, with no evidence of a new perfusion defect. Single photon emission computerized tomography (SPECT) images of the brain showed hypoperfusion in the right temporo-occipital area suggestive of an old infarct, with other areas in the brain parenchyma showing normal perfusion. Digital subtraction four-vessel angiography confirmed bilateral multiple stenosis of the supraclinoid portion of the ICA as well as stems of the ACA and MCAs and the posterior circulation with multiple collaterals arising proximal to the occluded vessels and from the external carotid artery. MR angiogram of the brain showed bilateral stenosis of the ICA distal to the supraclinoid segment and in the posterior circulation. MRI and CT scan of the brain plain and contrast study showed multiple lacunar infarcts bilaterally. Most surgical procedures have aimed at increasing the blood supply primarily to the MCA territory and do not directly benefit the ACA territory. Direct revascularization by superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis and indirect methods such as encephalomyosynangiosis (EMS), encephaloduroarteriosynangiosis (EDAS), encephaloduroarteriomyosynangiosis (EDAMS), omental graft have been performed with varying results and a combination of direct and indirect revascularization techniques have also been attempted. Several surgical techniques have been described, in which revascularization of the ischemic regions of the brain is attempted. However, the benefits of revascularization surgery for the ischemic type of MMD are well-established. Very little is known about the pathogenesis of MMD, and thus curative treatment is still elusive. The clinical presentation of moyamoya disease (MMD) in children usually includes episodes of transient ischemic attacks (TIAs), whereas in adults it is intracranial hemorrhage. It is the most common pediatric cerebrovascular disease in Eastern Asia. It is a chronic, occlusive cerebrovascular disease involving bilateral stenosis or occlusion of the terminal portion of the internal carotid arteries (ICAs) and/or the proximal portions of the anterior cerebral arteries (ACAs), middle cerebral arteries (MCAs) and also the posterior circulation, with an abnormal vascular network at the base of the brain. Moyamoya, meaning a “hazy puff of smoke” in Japanese, was first described by Takeuchi and Shimizu in 1957.
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