Prevalence and Mortality in Children with Congenital Diaphragmatic Hernia: A Multi-Country Study

International Clearinghouse for Birth Defects Surveillance and Research, Maria D Politis, Eva Bermejo-Sánchez, Mark A Canfield, Paolo Contiero, Janet D Cragan, Saeed Dastgiri, Hermien Ek de Walle, Marcia L Feldkamp, Amy Nance, Boris Groisman, Miriam Gatt, Adriana Benavides-Lara, Paula Hurtado-Villa, Kärin Kallén, Danielle Landau, Nathalie Lelong, Jorge Lopez-Camelo, Laura Martinez, Margery MorganOsvaldo M Mutchinick, Anna Pierini, Anke Rissmann, Antonin Šípek, Elena Szabova, Wladimir Wertelecki, Ignacio Zarante, Marian K Bakker, Vijaya Kancherla, Pierpaolo Mastroiacovo, Wendy N Nembhard*

*Bijbehorende auteur voor dit werk

OnderzoeksoutputAcademicpeer review

21 Citaten (Scopus)
49 Downloads (Pure)


PURPOSE: This study determined the prevalence, mortality and time trends of children with congenital diaphragmatic hernia (CDH).

METHODS: Twenty-five hospital- and population-based surveillance programs in 19 International Clearinghouse for Birth Defects Surveillance and Research member countries provided birth defects mortality data between 1974 and 2015. CDH cases included live births, stillbirths, or elective termination of pregnancy for fetal anomalies. Prevalence, cumulative mortality rates, and 95% confidence intervals (CI) were calculated using Poisson regression and Kaplan-Meier product-limit method. Joinpoint regression analyses were conducted to assess time trends.

RESULTS: The prevalence of CDH was 2.6 per 10,000 total births (95% CI: 2.5-2.7), slightly increasing between 2001 and 2012 (average annual percent change [AAPC] = 0.5%; 95% CI:-0.6-1.6). The total percent mortality of CDH was 37.7%, with hospital-based registries having more deaths among live births than population-based registries (45.1% vs. 33.8%). Mortality rates decreased over time (AAPC = -2.4%; 95% CI: -3.8--1.1). Most deaths due to CDH occurred among 2- to 6-day-old infants for both registry types (36.3%, hospital-based; 12.1%, population-based).

CONCLUSION: The mortality of CDH has decreased over time. Mortality remains high during the first week and varied by registry type.

Originele taal-2English
Pagina's (van-tot)61-69.e3
Aantal pagina's12
TijdschriftAnnals of Epidemiology
Vroegere onlinedatum27-nov-2020
StatusPublished - apr-2021

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