Rational approach to treatment of moyamoya disease in childhood

Early diagnosis and treatment of moyamoya disease in children is essential to minimize residual mental and physiologic deficits. Current treatment of childhood moyamoya disease in Japan, preoperative evaluation of perfusion r eserve as a surgical indication, and the role of noninvasive follow-up by m agnetic resonance angiography are reported. Approximately 20% of children w ith definite moyamoya disease were observed or treated medically. Among sur gical procedures, single indirect bypass surgery was used in approximately 30% of all patients; combinations of direct and indirect bypass surgery, 20 %; and multiple-indirect bypass surgery, 18%. Both adequate understanding o f the primary condition and determination of optimal treatment, including s pecific operative procedures, required evaluation of cerebral circulation a nd metabolism. Surgical indications included reduced perfusion reserve in a ffected brain by positron emission tomography or single photon emission tom ography with administration of acetazolamide or a CO, load. Postoperative i mprovements of cerebral perfusion reserve show better correlation with disa ppearance of ischemic attacks than does angiographically demonstrated colla teral formation. Follow-up evaluation with magnetic resonance angiography h as advantages over conventional angiography because it is noninvasive and a voids general anesthesia.