Key result
Multidisciplinary management yields favorable outcomes in pregnant moyamoya disease, though preeclampsia poses fatal hemorrhagic risk.
Why the study?
Moyamoya disease in pregnancy is rare and lacks standardized obstetric management guidelines, complicating care due to physiologic changes and cerebrovascular risks.
Case Report (n=3)
No
Pregnant patients with Moyamoya disease require individualized multidisciplinary care and strict blood pressure management, as superimposed hypertensive disorders can trigger fatal intracranial hemorrhage even in those with prior cerebral revascularization.
Multidisciplinary care may support favorable outcomes in moyamoya pregnancies; this series leaves open optimal strategies to prevent hemorrhage with preeclampsia.
Objective Moyamoya disease is a rare progressive cerebrovascular disorder characterized by stenosis of the intracranial internal carotid arteries and formation of fragile collateral vessels, predisposing patients to ischemic and hemorrhagic stroke. Physiologic changes of pregnancy—including increased blood volume, systemic vasodilation, and augmented cerebral blood flow—may increase cerebrovascular vulnerability and complicate management. Study Design We describe three pregnancies complicated by moyamoya disease managed at a tertiary referral center in West Texas. All patients had undergone prior cerebral revascularization and were followed by neurology before conception. Antithrombotic strategies varied by individual risk factors and included aspirin or low-molecular-weight heparin. Comorbidities included pregestational diabetes with dichorionic diamniotic twins, homozygous factor V Leiden, and Chiari malformation with MTHFR mutation. Results Two patients underwent planned cesarean delivery with favorable maternal and neonatal outcomes following multidisciplinary management. The third developed preeclampsia with severe features at 32 weeks and experienced sudden neurologic deterioration. Despite emergent cesarean delivery, she suffered a fatal intracerebral hemorrhage. Conclusion These cases highlight the spectrum of pregnancy outcomes in patients with moyamoya disease and emphasize the importance of multidisciplinary care, strict blood pressure control, and careful neurologic surveillance.
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McCool et al. (2026) conducted a case report in Moyamoya disease in pregnancy (n=3). Pregnancy with moyamoya disease was evaluated on Maternal and neonatal outcomes. In a series of three pregnant women with moyamoya disease, two had favorable outcomes following multidisciplinary management, while one suffered a fatal intracerebral hemorrhage related to severe preeclampsia.
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