Progress of diagnosis and surgical treatment in children with moyamoya disease

Moyamoya disease (MMD), also known as abnormal intracranial vascular network, is a group of chronic progressive stenosis or occlusion of bilateral main branches of Willis ring (siphon segment of internal carotid artery and anterior and middle cerebral arteries, sometimes including the origin of posterior cerebral artery), followed by abnormal collateral small vascular network. The disease is known as MMD because it presents many dense piles of small blood vessels that appear as “puff of smoke” on cerebral angiogram. At present, great progress has been made in the field of epidemiology, pathogenesis, clinical manifestations, diagnosis, and treatment of adult MMD. However, the disease characteristics considerably differ in children. Therefore, this article will review the diagnosis, treatment, and prognosis of MMD in children.