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.

Arkansas Center for Birth Defects Research and Prevention and Arkansas Reproductive Health Monitoring System and Department of Epidemiology Fay W Boozman College of Public Health University of Arkansas for Medical Sciences Little Rock AR

Arkansas Center for Birth Defects Research and Prevention and Department of Epidemiology Fay W Boozman College of Public Health University of Arkansas for Medical Sciences Little Rock AR

CARIS the Congenital Anomaly Register for Wales Singleton Hospital Swansea Wales UK

Costa Rican Birth Defects Registry Cartago Costa Rica

Department of Basic Sciences of Health School of Health Pontificia Universidad Javeriana Cali Colombia

Department of Epidemiology Emory University Rollins School of Public Health Atlanta GA

Department of Genetics RYVEMCE Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán México City Mexico

Department of Genetics University of Groningen University Medical Center Groningen Eurocat Northern Netherlands Groningen the Netherlands

Department of Medical Genetics Thomayer Hospital Prague Czech Republic

Department of Neonatology Soroka Medical Center Beer Sheva Israel

Division of Family Health and Preparedness Utah Department of Health Utah Birth Defect Network Bureau of Children with Special Health Care Needs Salt Lake City UT

Division of Medical Genetics Department of Pediatrics University of Utah School of Medicine Salt Lake City UT

ECEMC Instituto de Salud Carlos 3 Madrid Spain

ECLAMC Center for Medical Education and Clinical Research Buenos Aires Argentina

Genetics Department Hospital Universitario Dr Jose E Gonzalez Universidad Autonóma de Nuevo León Mexico

Health Services Management Research Centre Tabriz University of Medical Sciences Tabriz Iran

Institute of Clinical Physiology National Research Council and Fondazione Toscana Gabriele Monasterio Tuscany Registry of Congenital Defects Pisa Italy

International Center on Birth Defects International Clearinghouse for Birth Defects Surveillance and Research Rome Italy

Lombardy Congenital Anomalies Registry Cancer Registry Unit Fondazione IRCCS Istituto Nazionale Tumori Italy

Malformation Monitoring Centre Saxony Anhalt Medical Faculty Otto von Guericke University Magdeburg Germany

Malta Congenital Anomalies Registry Directorate for Health Information and Research Malta

Metropolitan Atlanta Congenital Defects Program National Center on Birth Defects and Development Disabilities Centers for Disease Control and Prevention Atlanta GA

National Board of Health and Welfare Stockholm Sweden

National Network of Congenital Anomalies of Argentina National Center of Medical Genetics National Ministry of Health Buenos Aires Argentina

Omni Net for Children International Charitable Fund Rivne Ukraine

Pontificia Universidad Javeriana Bogotá Colombia

REMAPAR Paris Registry of Congenital Malformations Inserm UMR 1153 Obstetrical Perinatal and Pediatric Epidemiology Research Team Center for Epidemiology and Statistics Sorbonne Paris Cité DHU Risks in Pregnancy Paris Descartes University France

Slovak Teratologic Information Centre Slovak Medical University Bratislava Slovak Republic

Texas Department of State Health Services Birth Defects Epidemiology and Surveillance Branch Austin TX

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