Current Approaches to Moyamoya Disease

Abstract

  Moyamoya disease (MMD) is a unique cerebrovascular disease with unknown etiology, characterized by progressive stenosis at the internal carotid artery terminus and formation of an abnormal vascular network at the base of the brain. Superficial temporal artery-middle cerebral artery anastomosis with/without indirect pial synangiosis is a standard management option for symptomatic MMD patients, either those with ischemic or hemorrhagic onset. Occipital artery-posterior cerebral artery bypass is also a reasonable additional surgery for recurrent ischemic attack or re-bleeding after standard revascularization. Endovascular stenting or angioplasty for affected vessels is generally not recommended, but targeted embolization on the bleeding point, including periventricular anastomosis or microaneurysms, can be the management choice for patients with repeated hemorrhage. Genetic analysis of RNF213 variants may be useful to predict the postoperative development of pial synangiosis, facilitating precise treatment of MMD.