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

• Type of Material: Article
• Authors: Mariam K. Bakker, University of Groningen; Vijaya Kancherla, Emory University; Mark A. Canfield, Texas Department of State Health Services; Eva Bermejo-Sanchez, Instituto de Salud Carlos III; Janet D. Cragan, Centers for Disease Control and Prevention; Saeed Dastgiri, Tabriz University of Medical Sciences; Hermien E. K. De Walle, University of Groningen; Marcia L. Feldkamp, University of Utah; Boris Groisman, National Network of Congenital Anomalies of Argentina; Miriam Gatt, Directorate for Health Information and Research; Paula Hurtado-Villa, Pontificia Universidad Javeriana; Karin Kallen, National Board of Health and Welfare and University of Lund; Daniella Landau, Soroka University Medical Center; Nathalie Lelong, Universite Paris Descartes; Jorge S. Lopez Camelo, Center for Medical Education and Clinical Research; Laura Martínez, Universidad Autónoma de Nuevo León; Margery Morgan, Singleton Hospital; Osvaldo M. Mutchinick, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán; Wendy N. Nembhard, University of Arkansas; Anna Pierini, Gabriele Monasterio Foundation
• Language: English
• Date: 2019-11-01
• Publisher: Wiley: 12 months
• Publication Version: Final Published Version
• Copyright Statement: © 2019 The Authors. Paediatric and Perinatal Epidemiology published by John Wiley & Sons Ltd.
• License: Creative Commons Attribution 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1111/ppe.12589
• Title of Journal or Parent Work: Paediatric and Perinatal Epidemiology
• ISSN: 0269-5022
• Volume: 33
• Issue: 6
• Start Page: 436
• End Page: 448
• Grant/Funding Information: This research received no specific grant from any funding agency in the public, commercial, or not‐for‐profit sectors.; The programmes participating in this study may receive local funding.
• Keywords: epidemiology; mortality; spina bifida; registry-based study
• Research Categories: Health Sciences, Human Development; Health Sciences, Epidemiology

Abstract

Paediatric and Perinatal Epidemiology published by John Wiley & Sons Ltd. 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.