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

Ann Epidemiol

. 2020 Nov 27;S1047-2797(20)30415-4. doi: 10.1016/j.annepidem.2020.11.007. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/33253899/

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

Maria D Politis 1Eva Bermejo-Sánchez 2Mark A Canfield 3Paolo Contiero 4Janet D Cragan 5Saeed Dastgiri 6Hermien Ek de Walle 7Marcia L Feldkamp 8Amy Nance 9Boris Groisman 10Miriam Gatt 11Adriana Benavides-Lara 12Paula Hurtado-Villa 13Kärin Kallén 14Danielle Landau 15Nathalie Lelong 16Jorge Lopez-Camelo 17Laura Martinez 18Margery Morgan 19Osvaldo M Mutchinick 20Anna Pierini 21Anke Rissmann 22Antonin Šípek 23Elena Szabova 24Wladimir Wertelecki 25Ignacio Zarante 26Marian K Bakker 7Vijaya Kancherla 27Pierpaolo Mastroiacovo 28Wendy N Nembhard 29International Clearinghouse for Birth Defects Surveillance and ResearchAffiliations expand

Abstract

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.

Keywords: Registry; hospital-based; mortality; population-based; prevalence.

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