Cerebral venous sinus thrombosis in pregnancy and the postpartum period: epidemiology, management, and maternal mortality-a systematic review and meta-analysis.

BACKGROUND: Cerebral venous sinus thrombosis is a rare cerebrovascular disease of importance both during pregnancy and in the puerperium. However, the existing cohort literature relates to two distinct clinical populations: women with acute onset of pregnancy-/puerperium-associated cerebral venous thrombosis (CVST), and women with known previous CVST who were assessed in connection with a subsequent pregnancy. As these populations address distinct clinical questions and have distinct denominators and follow-up structures, this review has distinguished outcomes from the two populations and synthesized them separately. METHODS: A PRISMA-compliant systematic review was conducted searching six bibliographic databases for evidence on CVST/central venous thrombosis (CVT), pregnancy/puerperium, and follow-up cohorts. Cohort studies only have been eligible for inclusion, with the additional criterion of a follow-up period of ≥5 years or participant-level follow-up period of ≥5 years. This particular criterion was used mainly as an aid to interpreting data on recurrence or subsequent pregnancy, while for outcomes related to acute obstetric CVST, it was used simply as a measure of study period. Outcomes have been summarized as pooled proportions by random-effects meta-analysis with pre-specified stratification in follow-up cohorts (women with known CVST in the past) and study period cohorts (acute obstetric CVST). RESULTS: Twelve studies were included. In follow-up cohorts assessing subsequent pregnancy following previous CVST, deliveries/livebirths pooled to 69.6% (95% CI 59.2 to 78.4%, I2 = 51.7%), spontaneous miscarriage pooled to 19.4% (95% CI 13.8 to 26.5%, I2 = 20.6%), and recurrence of cerebral venous sinus thrombosis during subsequent pregnancy/puerperium pooled to 3.17% (95% CI 1.43 to 6.89%, I2 = 0%). In acute pregnancy/puerperium cerebral venous sinus thrombosis cohorts, maternal mortality pooled to 8.28% (95% CI 5.65 to 11.97%, I2 = 0%), while acute-phase anticoagulation use pooled to 92.9% (95% CI 64.8 to 98.9%) with high heterogeneity (I2 = 81.2%). These outcomes were thus interpreted within the context of their particular population and observation period. CONCLUSION: Long-term follow-up cohorts indicated that subsequent pregnancy following previous cerebral venous sinus thrombosis was associated with good delivery outcomes and low recurrence in cases when CVST management included structured risk stratification and appropriate prophylaxis. Acute obstetric CVST cohorts, on the other hand, dealt with acute morbidity, mortality, and treatment, and not with future pregnancy safety, with significant maternal mortality burden. SYSTEMATIC REVIEW REGISTRATION: PROSPERO registration number CRD420261328921.