Moyamoya Disease: The Disorder and Surgical Treatment

To discuss the clinical features of moyamoya disease, the studies that aid in diagnosing this disorder, and the reported outcomes of surgical treatment. We review the manifestations of moyamoya disease in children and adults and the recent reports of the various surgical procedures. Moyamoya disease is a chronic cerebrovascular disorder in which stenosis of the major arteries of the circle of Willis at the base of the skull progresses to occlusion. The diagnosis is based on the angiographic findings of the “puff of smoke” appearance of the abnormal capillary vessels at the base of the skull. Three surgical procedures are used to manage this disease: anastomosis of the superficial temporal artery to the middle cerebral artery, encephalomyosynangiosis, and encephaloduroarteriosynangiosis. In children with this disease, cerebral ischemic events, including strokes, occur. In adults, the fragile abnormal vessels can rupture and cause intracerebral hemorrhage. The mortality rate for adults is higher than that for children. Most published reports support the efficacy of surgical treatment in children but not in adults. The natural history of moyamoya disease is poor; neurologic deterioration due to strokes and hemorrhage is progressive. Seizures and intellectual deterioration can occur.