Hemorrhagic Moyamoya disease during pregnancy

Background: Intracranial hemorrhage in pregnant patients with Moyamoya dise ase is rare. We review the case of one such patient who presented with pre- eclampsia and a catastrophic intracerebral hemorrhage in order to highlight the associated management difficulties. Methods: A case of a pregnant (31 weeks) female brought to the emergency department with hypertension and a p rogressive decrease in her level of consciousness is presented. She rapidly developed a dilated right pupil and left extensor posturing. A CT scan of her head showed a large putamenal intracerebral hemorrhage. She was intubat ed, ventilated and given intravenous mannitol and magnesium sulfate. She un derwent a simultaneous craniotomy and Cesarean section. Post-operatively th e patient's ICP and jugular venous saturation were monitored in the intensi ve care unit. Results: The patient delivered a 1185g infant who did well. T he patient's ICP was well controlled until the tenth post-operative day whe n she developed malignant brain edema and died. Conclusion: This case highl ights three important points. First, simultaneous craniotomy and Cesarean s ection can be performed. Second, intraoperative control of bleeding Moyamoy a vessels is described. Third, the difficult post-operative management of t hese cases is highlighted. The literature regarding Moyamoya disease and pr egnancy is reviewed and some recommendations for the management of this rar e but potentially deadly condition are presented.