Moyamoya Disease in Pregnancy: A 20-Year Single-Center Experience and Literature Review

Abstract

Background: Pregnancy is a potential risk factor for stroke in women with Moyamoya disease. However, the rarity of the disease has limited clinical expertise to ensure a healthy pregnancy. The aim of the present study was to explore the possible risk factors for hemorrhagic and ischemic stroke in pregnant women with Moyamoya disease.

Methods: A retrospective review of cases in our hospital during a 20-year period and a review of the reported data were conducted to investigate pregnancy-related cerebrovascular events in women with Moyamoya disease.

Results: Thirty pregnancies in 20 women with Moyamoya disease were identified in the case review of our hospital. All were previously diagnosed cases, and no stroke had occurred during the study period. In the reported data review, pregnancy-related stroke in women with Moyamoya disease was identified in 54 (44 intracranial hemorrhage and 10 cerebral infarction). Intracranial hemorrhage occurred most commonly during the antepartum period (n = 39; 88.6%), with most events occurring at ≥24 weeks. Of the intracranial hemorrhage cases, 7 (15.9%) were complicated by hypertensive disorders of pregnancy, and 8 patients (18.2%) died of stroke. The onset of cerebral infarction was either in the antepartum (n = 4; 40.0%) or postpartum (n = 6; 60.0%) period. All postpartum cases occurred within 3-7 days after delivery.

Conclusion: Pregnancy-related stroke in patients with Moyamoya disease might be susceptible to gestational age. Intracranial hemorrhage is prone to occur during the antepartum period, especially at ≥24 weeks, and cerebral infarction tends to occur postpartum.

Keywords: Cerebral infarction; Intracranial hemorrhage; Moyamoya disease; Pregnancy.