Prevalence and Mortality among Children with Congenital Diaphragmatic Hernia: A Multi-Country Analysis

Details

• Alternative Title:
  Ann Epidemiol
• Personal Author:
  Politis, Maria D. ; Bermejo-Sánchez, Eva ; Canfield, Mark A. ; Contiero, Paolo ; Cragan, Janet D. ; Dastgiri, Saeed ; de Walle, Hermien ; Feldkamp, Marcia L. ; Nance, Amy ; Groisman, Boris ; Gatt, Miriam ; Benavides-Lara, Adriana ; Hurtado-Villa, Paula ; Kallén, Kärin ; Landau, Danielle ; Nathalie Lelong, ; Lopez-Camelo, Jorge ; Martinez, Laura ; Morgan, Margery ; Mutchinick, Osvaldo M. ; Pierini, Anna ; Rissmann, Anke ; Šípek, Antonin ; Szabova, Elena ; Wertelecki, Wladimir ; Zarante, Ignacio ; Bakker, Marian K. ; Kancherla, Vijaya ; Mastroiacovo, Pierpaolo ; Nembhard, Wendy N.
• Description:
  Importance
  
  Congenital diaphragmatic hernia (CDH), a severe birth defect characterized by a diaphragmatic malformation allowing herniation by abdominal organs into the thorax, is associated with high mortality.
  
  Objective
  
  The purpose of our study was to examine (1) the overall CDH prevalence and (2) mortality and survival trends of infants with CDH using data collected by hospital- and population-based birth defects surveillance programs from multiple countries affiliated with the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR).
  
  Design, Setting, and Participants Methods
  
  Twenty-five hospital- and population-based surveillance programs in 19 countries from members of the ICBDSR provided birth defects mortality data between 1974 and 2015. Prevalence estimates and mortality rates from 2001 to 2012, a period in which the majority of the programs had the most complete data, were further examined. Included were CDH cases involving live births, stillbirths, or elective termination of pregnancy for fetal anomalies.
  
  Main Outcomes and Measures
  
  Prevalence and 95% confidence intervals (CI) from Poisson regression and cumulative mortality rates and 95% CI from the Kaplan-Meier Product-Limit method were calculated for each country and registry type. Joinpoint regression analyses were conducted to assess time trends.
  
  Results
  
  Overall, the prevalence of CDH from all countries combined 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]=0.47%). The overall percent mortality of CDH was 37.7%, with hospital-based registries having more deaths involving live births than population-based registries (45.1% compared to 33.8%). Mortality rates decreased over time (AAPC=−2.43%). Infants with multiple congenital anomalies and syndromes had higher 1-week mortality rates (45.2% and 40.8%) than those with isolated defects (28.6%) overall. 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 and Relevance
  
  The prevalence of CDH has increased over time; although the mortality rate has slightly decreased, it remains high especially during the first week of life and varied by registry type. Further research is needed to inform development of measures and interventions to decrease deaths among infants with CDH.
  
  
• Subjects:
  Article Child Female Hernias, Diaphragmatic, Congenital Humans Infant Live Birth Pregnancy Prevalence Registries Stillbirth

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• Source:
  Ann Epidemiol. 56:61-69.e3
• Pubmed ID:
  33253899
• Pubmed Central ID:
  PMC8009766
• Document Type:
  Journal Article
• Funding:
  CC999999/ImCDC/Intramural CDC HHSUnited States/ ; U01 DD000491/DD/NCBDD CDC HHSUnited States/ ; U01 DD001285/DD/NCBDD CDC HHSUnited States/
• Volume:
  56
• Collection(s):
  CDC Public Access
• Main Document Checksum:
  urn:sha256:74b22821bf7bb7177b33902a4b6b745cbc21aca7e95f8446137107733f00b0ed
• Download URL:
  https://stacks.cdc.gov/view/cdc/104769/cdc_104769_DS1.pdf
• File Type:
  [PDF - 674.39 KB ]