Moyamoya Disease in Pregnancy: A Case Series.

Abstract

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.