Association Between Moyamoya Revascularization Surgery and Neurologic Events During Pregnancy: Systematic Review

The management of moyamoya disease during pregnancy and recommendations for the mode of delivery remain controversial. We investigated factors associated with neurologic events during pregnancy in women with moyamoya disease and its association with prepregnancy surgical revascularization.

We performed a literature search from January 1, 1970, through September 30, 2021, using Embase, Web of Science, Medline, and Cochrane to identify cases of moyamoya disease with pregnancy. Primary outcome was neurologic events during pregnancy and were subcategorized into antepartum, intrapartum (within 24 hours of delivery), and postpartum events. Univariate and multivariate regression analyses using pooled results were performed to assess risk factors associated with neurologic events.

Fifty-two relevant studies with 182 individuals diagnosed with moyamoya before pregnancy, and 229 pregnancies were included in the study. 59% underwent surgical revascularization before pregnancy. Of the 229 pregnancies, 22 (9.6%) patients had ischemic events and 3 (1.3%) had hemorrhagic events. In addition, there were 7 (3%) seizures and 4 (1.7%) other neurologic events not associated with ischemia or hemorrhage. There were fewer neurologic events during pregnancy in patients treated with surgery than those without surgery (11% surgical vs 24% medical, P = .009). Multivariable regression analysis demonstrated prior surgical revascularization as the only factor associated with lower number of neurologic events during pregnancy (odds ratio 0.42 [95% CI 0.19-0.96]). Mode of delivery (vaginal vs cesarean section) was not associated with a difference in overall intrapartum and postpartum neurologic events.

We found that prior revascularization surgery was the only factor associated with fewer neurologic events during pregnancy in women with moyamoya disease. Mode of delivery was not associated with increased neurologic events during or after delivery.