A Systematic Review of Race or Ethnicity and Risk of Death in Spina Bifida in the United States.

ObjectiveWe performed a systematic review of studies of mortality risk in spina bifida by race or ethnicity to determine if risks were relatively greater in Black or in Hispanic people with spina bifida than in White people in most studies.MethodsWe included studies of myelomeningocele that were peer-reviewed, recently published (1983-2022), and from the United States. We searched MEDLINE, Embase, and Scopus. We used PRISMA 2020 Guidelines and Cochrane Handbook for Interventions GRADE "confidence in certainty of quality of evidence" to evaluate studies. We assessed duplication of data sources during study periods in permutations of all possible pairs of studies.ResultsWe identified 4675 reports. After sequential review, 9 studies met inclusion criteria. We eliminated 1 study for design flaws, leaving 8 studies for systematic review synthesis. The median final endpoint at which survival was ascertained was 1 year old (range neonatal hospitalization discharge to ≤28 years old). There were 7 population-based, longitudinal, observational birth cohort studies and 1 cross-sectional study that used the Nationwide Inpatient Sample. Five studies found no increased mortality risk at study final endpoints in either Black or Hispanic participants (983 total decedents/9571 total participants), and 3 found a greater mortality risk in either Black (2) or "non-White" (1) participants (286 total decedents/7826 total participants). The median GRADE rating of the quality of evidence of the 8 studies was only "moderate." Duplicated data sources during study periods were present in 28 of the 42 permutations of the 21 possible study pairs (67%), with a median duplication of 24%.ConclusionThis systematic review synthesis was inconclusive for racial or ethnic disparities in risk of death in young people with spina bifida. Results were variable across studies; potential bias from duplicated data sources was present in most pairs of studies; and quality of evidence was not high. Disparities in risk of death require further study.PROSPERO registrationCRD42021244766.Funded by philanthropic donations.

Duke Scholars

Author Joan Mary Jasien Pediatrics, Neurology

Author Gordon Worley Pediatrics, Neurology

Author Phillip Brian Smith Pediatrics, Neonatology