Purpose: To present a case of Moyamoya disease with intracranial hemorrhage complicating pregnancy.
Clinical features: A 36-yr-old parturient at 34 wk gestation presented with left hemiparesis, headache, nausea and vomiting. Subsequent deterioration in level of consciousness and the development of a dilated right pupil necessitated immediate intubation. Urgent non-contrast CT scan revealed a large right intracerebral hematoma with transtentorial herniation. The patient underwent simultaneous emergency Cesarean section and craniotomy. A postoperative angiogram revealed findings consistent with Moyamoya disease. The neonate survived but the patient developed severe cerebral edema and died eleven days postoperatively.
Conclusion: Adult patients with Moyamoya disease often present with intracranial hemorrhage which poses unique anesthetic challenges. We report a case of intracerebral hemorrhage during pregnancy, which is known to be associated with high morbidity and mortality. The anesthetic techniques are reviewed and discussed.