Moyamoya disease diagnosed during pregnancy

Introduction: Moyamoya is a rare cerebrovascular disease in which the terminal, intracranial portions of the carotid arteries are progressively narrowed, resulting in the development of a tenuous network of small, collateral vessels. The exact etiology is unknown; however, these patients are at greater risk for both ischemic and hemorrhagic strokes. The risk of stroke in patients with Moyamoya disease increases during pregnancy, likely due to changes in vascular dynamics and other pregnancy-related physiological changes. Most cases of Moyamoya in pregnancy describe patient’s diagnosed preconception. The handful of case reports of moyamoya diagnosed in pregnancy have guarded outcomes. Case Report: This case report describes the pregnancy of a patient in her 30s who was diagnosed with Moyamoya at 13 weeks of gestation. She suffered three ischemic strokes prior to iatrogenic preterm delivery at 27 weeks and 5 days of pregnancy. Postpartum, she had a massive left hemispheric infarct leading to her demise. Conclusion: The clinical course and outcome for this patient suggests that Moyamoya disease without evidence of medical or surgical control may be a contraindication to pregnancy.