Approach to A Pregnant Woman with Moyamoya Syndrome

Abstract

Many female patients with the moyamoya disease have normal fertility functions, including those diagnosed before they enter their childbearing years. Moya moya syndrome (MMS) is a rare cerebrovascular disorder caused by segmentary stenosis and occlusions of the small vessels around the circle of Willis and distal branches of carotid arteries. The name of “moyamoya” means “puff of smoke” in Japanese and describes the look of the tangle of tiny vessels formed to compensate for the blockage. This disorder may presented transient paresis, headache, epilepsy, dementia, aphasia, ataxia, and subarachnoid hemorrhage. Although MMS is considered a disease of unknown etiology, infectious and genetic causes have been blamed. However, there has been no comprehensive review of the management of pregnancy and delivery associated with Moyamoya disease. The purpose of this report is to describe a pregnant woman diagnosed with moyamoya disease and to review the literature on other such cases.