[Diagnosis and Management Guidelines for Moyamoya Disease].

Abstract

Moyamoya disease(MMD) is a rare cerebrovascular disease characterized by progressive stenosis of the internal carotid artery terminus and an abnormal formation of a vascular network at the base of the brain. Superficial temporal artery-middle cerebral artery(STA-MCA) bypass, either as a direct or combined revascularization procedure, is a reasonable management choice for patients with symptomatic MMD. STA-MCA bypass prevents cerebral ischemic attacks by improving cerebral blood flow. Recent evidence further suggests that direct revascularization reduces the potential risk of rebleeding in patients with MMD with posterior hemorrhage who have an extremely high annual rebleeding rate. Despite the favorable long-term outcomes of STA-MCA bypass, cerebral hyperperfusion syndrome is a potential complication of this procedure that can result in focal neurologic deficits and/or delayed intracerebral hemorrhage. Therefore, recent guideline recommendations indicate that STA-MCA bypass is a reasonable choice for symptomatic patients with MMD, together with intensive perioperative care for blood pressure control.