Pregnancy and Delivery in Moyamoya Disease: Results of a Nationwide Survey in Japan

Abstract

Stroke during pregnancy associated with moyamoya disease (MMD) has been reported sporadically, but no systematic surveys have been undertaken. To reveal the current clinical situation, the authors conducted Japan's first nationwide survey of pregnancy and delivery associated with MMD. A questionnaire was sent to all 270 perinatal medical centers across Japan to survey their experiences with delivery associated with MMD within the preceding 5 years (Survey I); another questionnaire was sent to 554 adult female patients with MMD regarding their experience with childbirth (Survey II). Survey I included 59 deliveries among patients with previously diagnosed MMD. The incidence of perinatal neurological events and morbidity was 5.1% and 1.7%, respectively. In another five cases, newly diagnosed after perinatal attacks, disability was noted in three cases, including one death from intracranial hemorrhage. Survey II included 278 deliveries. The perinatal attack rate was 6.6% in 76 previously diagnosed cases and 2.0% in 202 cases undiagnosed at pregnancy, but neither group reported permanent morbidity. Caesarean section in previously diagnosed cases accounted for 76.3% of deliveries in Survey I and 69.7% in Survey II, but no significant difference in event rate was found between caesarean section and vaginal delivery in either survey. Although the incidence of perinatal neurological events is low when MMD has been diagnosed, careful monitoring is required in light of the potential for stroke. Serious events, especially intracranial hemorrhage, can occur if MMD has not been diagnosed at pregnancy. Further efforts to establish management guidelines are required to ensure safer childbirth in patients with MMD.

Moyamoya Disease in Pregnancy: A Single Institute Experience

“…5) Therefore, neurosurgeons are more frequently encountering pregnant patients with moyamoya disease than before. Although reports about moyamoya disease and pregnancy are limited, 4,8) pregnant patients diagnosed with moyamoya disease before pregnancy are known to have relatively lower potential risk for cerebrovascular events. 4,8) Strict blood pressure control, in particular precautions against pregnancy-induced hypertension, might lead to better prognosis for the patients.…”

“…Although reports about moyamoya disease and pregnancy are limited, 4,8) pregnant patients diagnosed with moyamoya disease before pregnancy are known to have relatively lower potential risk for cerebrovascular events. 4,8) Strict blood pressure control, in particular precautions against pregnancy-induced hypertension, might lead to better prognosis for the patients. 8) This recommendation is based on the observations that, in addition to the increase in circulating plasma volume and enhanced blood coagulation during pregnancy, pregnancy-induced hypertension may result in blood-brain barrier disruption and vasospasm, and subsequently cause neurological deterioration of patients with moyamoya disease.…”

“…4,8) Strict blood pressure control, in particular precautions against pregnancy-induced hypertension, might lead to better prognosis for the patients. 8) This recommendation is based on the observations that, in addition to the increase in circulating plasma volume and enhanced blood coagulation during pregnancy, pregnancy-induced hypertension may result in blood-brain barrier disruption and vasospasm, and subsequently cause neurological deterioration of patients with moyamoya disease. 3) In our present series, all four patients who had been diagnosed with moyamoya disease before pregnancy underwent scheduled cesarean section, and none of them suffered neurological events in pregnancy and puerperium.…”

“…5,7) Moyamoya disease is a rare entity, but particularly affects children and young adults with female predominance, 5) so it is not uncommon in the clinical setting to encounter patients with moyamoya disease at pregnancy and/or delivery. 4,6,8) In fact, pregnant patients with moyamoya disease are often referred to the neurological service, but there are no established management guidelines for moyamoya disease in pregnancy and puerperium. The present retrospective analysis of 4,400 consecutive deliveries in our hospital investigated the incidence and clinical picture of pregnant patients with moyamoya disease.…”

The case of completed pregnancy after hemorrhagic stroke in the patient with moyamoya disease

“…Ведение родов через естественные ро-довые пути возможно, предпочтение отдают эпиду-ральной анестезии при влагалищном родоразреше-нии. Родоразрешение проводится в условиях много-профильного стационара, имеющего в структуре отделение нейрореанимации [14][15][16].…”