Analysis of Mortality among Neonates and Children with Spina Bifida: An International Registry‐Based Study, 2001‐2012

• Published in: Paediatric and perinatal epidemiology Vol. 33; no. 6; pp. 436 - 448
• Main Authors: Bakker, Marian K., Kancherla, Vijaya, Canfield, Mark A., Bermejo‐Sanchez, Eva, Cragan, Janet D., Dastgiri, Saeed, De Walle, Hermien E. K., Feldkamp, Marcia L., Groisman, Boris, Gatt, Miriam, Hurtado‐Villa, Paula, Kallen, Karin, Landau, Daniella, Lelong, Nathalie, Lopez Camelo, Jorge S., Martínez, Laura, Morgan, Margery, Mutchinick, Osvaldo M., Nembhard, Wendy N., Pierini, Anna, Rissmann, Anke, Sipek, Antonin, Szabova, Elena, Tagliabue, Giovanna, Wertelecki, Wladimir, Zarante, Ignacio, Mastroiacovo, Pierpaolo
• Format: Journal Article
• Language: English
• Published: England Wiley Subscription Services, Inc 01-11-2019; John Wiley and Sons Inc
• Subjects: Asia - epidemiology; Birth defects; Child; Child Mortality; Child, Preschool; Children; Clinical Medicine; Confidence intervals; Congenital defects; epidemiology; Europe - epidemiology; Fatalities; Female; Fetuses; Folic acid; Folkhälsovetenskap, global hälsa, socialmedicin och epidemiologi; Gestation; Health Sciences; Humans; Hälsovetenskap; Infant; Infant Mortality; Infant, Newborn; Klinisk medicin; Life expectancy; Life span; Live Birth - epidemiology; Male; Medical and Health Sciences; Medicin och hälsovetenskap; Morbidity; Mortality; Neonates; North America - epidemiology; Original; Pediatrics; Pediatrik; Perinatal Health; Pregnancy; Prevalence; Public Health, Global Health, Social Medicine and Epidemiology; Registries; registry-based study; South America - epidemiology; Spina bifida; Spinal Dysraphism - epidemiology; Spinal Dysraphism - mortality; Stillbirth; Stillbirth - epidemiology; Surveillance; Survival; South America; Asia; North America; Europe; mortality; epidemiology; registry-based study; spina bifida
• ISSN: 0269-5022; 1365-3016
• DOI: 10.1111/ppe.12589

Background
Medical advancements have resulted in better survival and life expectancy among those with spina bifida, but a significantly increased risk of perinatal and postnatal mortality for individuals with spina bifida remains.
Objectives
To examine stillbirth and infant and child mortality among those affected by spina bifida using data from multiple countries.
Methods
We conducted an observational study, using data from 24 population‐ and hospital‐based surveillance registries in 18 countries contributing as members of the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR). Cases of spina bifida that resulted in livebirths or stillbirths from 20 weeks' gestation or elective termination of pregnancy for fetal anomaly (ETOPFA) were included. Among liveborn spina bifida cases, we calculated mortality at different ages as number of deaths among liveborn cases divided by total number of liveborn cases with spina bifida. As a secondary outcome measure, we estimated the prevalence of spina bifida per 10 000 total births. The 95% confidence interval for the prevalence estimate was estimated using the Poisson approximation of binomial distribution.
Results
Between years 2001 and 2012, the overall first‐week mortality proportion was 6.9% (95% CI 6.3, 7.7) and was lower in programmes operating in countries with policies that allowed ETOPFA compared with their counterparts (5.9% vs. 8.4%). The majority of first‐week mortality occurred on the first day of life. In programmes where information on long‐term mortality was available through linkage to administrative databases, survival at 5 years of age was 90%‐96% in Europe, and 86%‐96% in North America.
Conclusions
Our multi‐country study showed a high proportion of stillbirth and infant and child deaths among those with spina bifida. Effective folic acid interventions could prevent many cases of spina bifida, thereby preventing associated childhood morbidity and mortality.