Ann Epidemiol

. 2021 Apr;56:61-69.e3. doi: 10.1016/j.annepidem.2020.11.007. Epub 2020 Nov 27. https://pubmed.ncbi.nlm.nih.gov/33253899/

Prevalence and mortality in children with congenital diaphragmatic hernia: a multicountry study

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

• PMID: 33253899
• PMCID: PMC8009766 (available on 2022-04-01)
• DOI: 10.1016/j.annepidem.2020.11.007

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 (CIs) were calculated using Poisson regression and a 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 = 0.5%; 95% CI:-0.6 to 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 (average annual percent change = -2.4%; 95% CI: -3.8 to 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).

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

Keywords: Hospital-based; Mortality; Population-based; Prevalence; Registry.

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