Objective and importance: Many female patients with moyamoya disease are of childbearing years, including those who were diagnosed before entering their childbearing years. However, there have been no extensive reviews of the management of pregnancy and delivery in association with moyamoya disease. The purpose of this report is to describe the case of a patient with moyamoya disease complicated by pregnancy and to review the literature on other such cases.
Clinical presentation and intervention: We report a 23-year-old primipara with moyamoya disease who delivered uneventfully by cesarean section under spinal anesthesia at 38 weeks of gestation. In the literature, 30 cases were reported of patients who had been diagnosed with moyamoya disease before pregnancy and delivery, and 23 patients who were symptomatic and were diagnosed for the first time with moyamoya disease in association with pregnancy.
Conclusion: There is no evidence that pregnancy increases the risk of cerebrovascular accident or that bypass surgery decreases its risk. Poor prognosis of the patient or the newborn is mostly caused by cerebral hemorrhage and not by cerebral ischemia. It is important to control blood pressure and especially to avoid toxemia during pregnancy. Either cesarean section or vaginal delivery can be accomplished safely. Any anesthetic method can be used, provided special attention is given to avoiding hypocapnia, hypotension, and hypertension. Oral contraceptives should be avoided.