Abstract
We review recent progress and perspectives in etiology, pathophysiology, diagnosis, surgical treatment, and perioperative management of moyamoya disease. Endothelial progenitor cells may closely be involved in the development of the disease. The clinical impact of microbleeds seen on T2*-weighted imaging should be clarified. Long-term outcome and treatment strategy in asymptomatic moyamoya disease should also be assessed. A novel multi-center study, called the Asymptomatic Moyamoya Registry (AMORE) study, will be started very soon. Surgical revascularization is accepted as a useful option for moyamoya disease with ischemic onset. An ongoing randomized clinical trial, the Japan Adult Moyamoya (JAM) trial, is expected to elucidate the efficacy of direct or combined bypass surgery on moyamoya disease with hemorrhagic onset. Direct bypass procedures help lower the incidence of perioperative ischemic stroke, but careful management should be emphasized to reduce the incidence of major complications due to postoperative hyperperfusion. The pathophysiology and treatment strategy of headaches in pediatric patient should be further discussed. Infantile cases are at very high risk for repeated ischemic stroke and perioperative ischemic complications, and should be intensively managed before and after surgery.
Based on these considerations, we should further clarify the unsolved issues and improve the short- and long-term outcome by advancing our basic research, diagnostic modalities, surgical technique, and perioperative management.
