Anestesia e Pesquisa Clínica
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Two Patients with Moyamoya Disease Underwent Emergency Cesarean Delivery Resulted in Opposite Outcomes

T. Yamashita and S. Shibuta

Moyamoya disease is a rare cerebrovascular disorder, characterized by bilateral stenosis or occlusion of arteries at the base of the brain and formation of an abnormal network of collateral vessels, called moyamoya vessels, associated with cerebral hemorrhage and infarction. No appropriate method of delivery, anesthesia, and perioperative management of pregnant patients with moyamoya disease has yet been established. We report 2 cases of pregnant patients with moyamoya disease who underwent emergency cesarean delivery under general anesthesia. The postoperative courses were different, which might help us establish a protocol for the standard management of this disease. Case 1: A 33-year-old primipara was diagnosed as having moyamoya disease when she was 4-year-old. She underwent emergency cesarean delivery under general anesthesia at 37 weeks’ gestation due to early rupture of the membranes. The perioperative period was uneventful. The patient was discharged without aggravation of neurological symptoms. Case 2: A 35-year-old multipara with no medical history experienced sudden nausea and headache disturbance at 30 weeks’ gestation. Brain CT showed intracranial hemorrhage, and the patient was diagnosed moyamoya disease by cerebral angiography. The patient recovered after intraventricular drainage but lost consciousness at 32 weeks’ gestation due to repeated hemorrhage. Cesarean delivery, aneurysm excision, direct hematoma evacuation, and external decompression were performed shortly thereafter under general anesthesia. The intracranial pressure continued to increase, and the patient died on the ninth postoperative day. In pregnant patients with moyamoya disease, careful management during the perinatal period is needed to decide the timing of emergency cesarean delivery, which affects prognosis.