Moyamoya disease and pregnancy: A case report

Background: Moyamoya (puff of smoke) is the Japanese name for a cerebral angiographic picture of bilateral intracranial carotid artery occlusion associated with telangiectatic vessels in the region of the basal ganglia. In childhood the condition usually presents as hemiplegia of sudden onset and epileptic seizures; adults more commonly develop intraventricular hemorrhage. The disease occurs mainly in females. The incidence is about 0.07% in Japan. Case report: A 25-year-old primigravida with moyamoya disease developed sudden intraventricular hemorrhage and loss of consciousness at 24 weeks' gestation. She underwent bilateral ventricular drainage and anticoagulation during a 3-week period in the intensive care unit. A healthy girl was delivered by cesarean section at 34 weeks' gestation. Extra-intracranial arterial bypass surgery was performed some months after delivery. Ten months later the patient shows only mild neurologic deficits. Conclusion: This suggests that prompt diagnostic measures and aggressive treatment can lead to acceptable maternal and neonatal outcome in pregnant patients with moyamoya disease.