Moyamoya disease during pregnancy and childbirth

Moyamoya disease (MMD) is a chronic vasculopathy characterized by progressive bilateral stenosis and occlusion of the terminal portion of the internal carotid artery (ICA) and the presence of an abnormal vascular network at the base of the brain, termed Moyamoya vessels (MMV). The main presentations of MMD are ischemia and hemorrhage, and diagnosis is done via angiography. Cerebral events were reported in 5.1% of pregnancies of MMD diagnosed mothers, in Japan. In the case of MMD diagnosis due to cerebrovascular events during gestation, 34.7% of patients presented with an ischemic event, while 69.5% suffered from hemorrhage. During gestation, hemorrhagic events were found to occur mostly antepartum, after 24 weeks of gestation, while cerebral infarction peaked 3-7 days after delivery. No treatment has been found to halt or reverse the progress of the disease. Interventions focus on reducing the risk of stroke and cognitive disfunction as a result of ischemia. In the case of pregnant women suffering hemorrhage due to MMD, conservative treatment, as well as ventricle puncture and drainage were to be efficient, and did not severely impact the child. When diagnosed before pregnancy occurs, MMD under treatment does not pose a significantly increased risk of complications, compared to pregnancies in unaffected women. No evidence suggests that MMD is a contraindication for pregnancy.