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Observational Study
. 2019 Nov;33(6):436-448.
doi: 10.1111/ppe.12589. Epub 2019 Oct 21.

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

Marian K Bakker  1 , Vijaya Kancherla  2 , Mark A Canfield  3 , Eva Bermejo-Sanchez  4 , Janet D Cragan  5 , Saeed Dastgiri  6 , Hermien E K De Walle  1 , Marcia L Feldkamp  7 , Boris Groisman  8 , Miriam Gatt  9 , Paula Hurtado-Villa  10 , Karin Kallen  11 , Daniella Landau  12 , Nathalie Lelong  13 , Jorge S Lopez Camelo  14 , Laura Martínez  15 , Margery Morgan  16 , Osvaldo M Mutchinick  17 , Wendy N Nembhard  18 , Anna Pierini  19 , Anke Rissmann  20 , Antonin Sipek  21 , Elena Szabova  22 , Giovanna Tagliabue  23 , Wladimir Wertelecki  24 , Ignacio Zarante  25 , Pierpaolo Mastroiacovo  26
Affiliations
Observational Study

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

Marian K Bakker et al. Paediatr Perinat Epidemiol. 2019 Nov.

Abstract

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.

Keywords: epidemiology; mortality; registry-based study; spina bifida.

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Figures

Figure 1
Figure 1
Spina bifida surveillance period by country and registry, International Clearinghouse for Birth Defects Surveillance and Research. 1Spain included information on elective termination of pregnancy for fetal anomalies from 1995 to 2014. 2Sweden included information on elective terminations of pregnancy for fetal anomalies from 1999 to 2014. 3Number of surveillance years. ECEMC, Registry of the Spanish Collaborative Study of Congenital Malformations; ECLAMC, Latin American Collaborative Study of Congenital Malformations; MCAR, Malta Congenital Anomalies Registry; OMNI‐Net = Ukraine Birth Defects Prevention Program; RENAC, National Network of Congenital Anomalies of Argentina; RYVEMCE, Mexican Registry and Epidemiological Surveillance of External Congenital Malformations; TROCA, Tabriz Registry of Congenital Anomalies; SMC, Soroka Medical Center; UK, United Kingdom; USA, United States of America
Figure 2
Figure 2
Survival in livebirths with spina bifida for surveillance period 2001‐2012, from European and North American registries (with linkage to administrative databases) contributing to the International Clearinghouse for Birth Defects Surveillance and Research
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