Survival Trends of People With Spina Bifida From 1950-2020

People with spina bifida historically have worse survival. Despite health care improvements, survival improvement data are mixed.

To compare survival for people with spina bifida to an unaffected cohort and to determine if survival for people with spina bifida has improved from 1950 to 2020.

This retrospective cohort study was conducted using state databases to determine survival of individuals with spina bifida and an unaffected population. Demographic, health care, and epidemiological data were extracted from the Utah Population Database from the period January 1950 to December 2019. People with spina bifida were matched by birth year, sex, and county of residence to Utah residents without spina bifida. Data were analyzed from March 2025 to April 2026.

Diagnosis of spina bifida.

The primary outcome was all-cause mortality. Survival rates for people with spina bifida were compared to an unaffected cohort. Interaction terms were used to determine if geography or birth decade modified survival. The secondary analysis quantified longitudinal trends in survival among those with spina bifida from 1950 to 2020.

A total of 942 people with spina bifida (487 female [51.7%]) were matched by birth year, sex, and county of residence to 9420 Utah residents without spina bifida (4870 female [51.7%]). Cohorts were similar in demographics but differed by median (IQR) birth weight (spina bifida: 3118 g [2693-3480]; unaffected cohort: 3330 g [3010-3630]; P < .001) and gestational age at birth (spina bifida: 39 weeks [37-40]; unaffected cohort: 39 weeks [38-40]; P < .001). Deaths occurred in 270 spina bifida cases (29%; 1.7/100 person-years) and 257 controls (2.7%; 0.1/100 person-years) (P < .001). Compared to the unaffected cohort, spina bifida survival was worse at 1 month (hazard ratio [HR], 23.8; 95% CI, 18.0-31.5), 1 year (HR, 15.7; 95% CI, 13.0-19.1), 10 years (HR, 10.7; 95% CI, 8.6-13.3), and 30 years (HR, 8.9; 95% CI, 6.9-11.5). Geography did not modify mortality risk. Decadal survival improvements in spina bifida paralleled the unaffected cohort. Compared to those born in the 1980s, survival was worse for those born with the condition in the 1950s and 1960s (1960s 1-year HR: 3.1; 95% CI, 2.0-5.0; 1960s 30-year HR: 3.2; 95% CI, 1.4-7.3). Also relative to the 1980s, there was improved survival for those born with spina bifida in more recent decades at many but not all time points (1990s 20-year HR: 0.4; 95% CI, 0.1-1.2 vs 2000s 20-year HR: 0.2; 95% CI, 0.0-0.7).

Per the results of this cohort study, survival for those with spina bifida improved markedly between 1950 and 2010, yet it was still substantially worse than population levels. Spina bifida survival did not improve faster than the unaffected population, suggesting ongoing unmet health care needs for spina bifida survival.