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Original article
Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study
  1. Mark R McGivern1,
  2. Kate E Best1,
  3. Judith Rankin1,
  4. Diana Wellesley2,
  5. Ruth Greenlees3,
  6. Marie-Claude Addor4,
  7. Larraitz Arriola5,
  8. Hermien de Walle6,
  9. Ingeborg Barisic7,
  10. Judit Beres8,
  11. Fabrizio Bianchi9,
  12. Elisa Calzolari10,
  13. Berenice Doray11,
  14. Elizabeth S Draper12,
  15. Ester Garne13,
  16. Miriam Gatt14,
  17. Martin Haeusler15,
  18. Babak Khoshnood16,
  19. Kari Klungsoyr17,
  20. Anna Latos-Bielenska18,
  21. Mary O'Mahony19,
  22. Paula Braz20,
  23. Bob McDonnell21,
  24. Carmel Mullaney22,
  25. Vera Nelen23,
  26. Anette Queisser-Luft24,
  27. Hanitra Randrianaivo25,
  28. Anke Rissmann26,
  29. Catherine Rounding27,
  30. Antonin Sipek28,
  31. Rosie Thompson29,
  32. David Tucker30,
  33. Wladimir Wertelecki31,
  34. Carmen Martos32
  1. 1Institute of Health & Society, Newcastle University, Newcastle upon Tyne, UK
  2. 2Faculty of Medicine and Wessex Clinical Genetics Service, University Hospitals Southampton, Southampton, UK
  3. 3University of Ulster, Ulster, UK
  4. 4Service de Genetique Medicale Maternite, CHUV, Lausanne, Switzerland
  5. 5Public Health Division of Gipuzkoa, Instituto Bio-Donostia, Basque Government, CIBER Epidemiología y Salud Pública, CIBERESP, Spain
  6. 6Eurocat Northern Netherlands, Department of Genetics, University of Groningen, University Medical Center, Groningen, The Netherlands
  7. 7Children's Hospital Zagreb, University of Zagreb, School of Medicine, Zagreb, Croatia
  8. 8Department of Hungarian Congenital Abnormality Registry & Surveillance, National Institute of Health Development, Budapest, Hungary
  9. 9Department of Medical Genetics, ARNAS Garibaldi Nesima, Catania, Italy
  10. 10IMER Registry (Emila Romagna Registry of Birth Defects), Ferrara, Italy
  11. 11Department of de Genetique Medicale, Hopital de Hautepierre, Strasbourg, France
  12. 12Department of Health Sciences, University of Leicester, Leicester, UK
  13. 13Hospital Lillebaelt, Kolding, Denmark
  14. 14Department of Health Information and Research, Guardamangia, Malta
  15. 15Medical University of Graz, Graz, Austria
  16. 16Paris Registry of Congenital Malformations, INSERM U953, Paris, France
  17. 17Medical Birth Registry of Norway, Norwegian Institute of Public Health and Department of Global Public Health and Primary Care, University of Bergen, Bergen, Norway
  18. 18Polish Registry of Congenital Malformations, Poznan, Poland
  19. 19Health Service Executive, Cork, Ireland
  20. 20Instituto Nacional de Saude Dr Ricardo Jorge, Lisbon, Portugal
  21. 21Health Service Executive, Dublin, Ireland
  22. 22Public Health Department, HSE South, Kilkenny, Ireland
  23. 23Provinciaal Instituut voor Hygiene, Antwerp, Belgium
  24. 24Birth Registry Mainz Model, Childrens Hospital, University Medical Center, Johannes Gutenberg-University, Mainz, Germany
  25. 25Medical Genetics Unit of CHU Sud Réunion, Ile de la Reunion, France
  26. 26Malformation Monitoring Centre Saxony-Anhalt, Medical Faculty Otto-von-Guericke University, Magdeburg, Germany
  27. 27National Perinatal Epidemiology Unit, University of Oxford, Oxford, UK
  28. 28National Registry of Congenital Anomalies, Department of Medical Genetics, Thomayer Hospital, Prague, Czech Republic
  29. 29South West England Congenital Anomaly Register, Bristol, UK
  30. 30Public Health Wales, Wales, UK
  31. 31OMNI-Net Ukraine Birth Defects Program, Rivne- Khmelnytskyy, Ukraine
  32. 32Centro Superior de Investigación en Salud Pública—FISABIO, Valencia, Spain
  1. Correspondence to Professor Judith Rankin, Institute of Health & Society, Baddiley Clark Building, Newcastle University, Newcastle upon Tyne NE2 4AX, UK; judith.rankin{at}ncl.ac.uk

Abstract

Introduction Published prevalence rates of congenital diaphragmatic hernia (CDH) vary. This study aims to describe the epidemiology of CDH using data from high-quality, population-based registers belonging to the European Surveillance of Congenital Anomalies (EUROCAT).

Methods Cases of CDH delivered between 1980 and 2009 notified to 31 EUROCAT registers formed the population-based case series. Prevalence over time was estimated using multilevel Poisson regression, and heterogeneity between registers was evaluated from the random component of the intercept.

Results There were 3373 CDH cases reported among 12 155 491 registered births. Of 3131 singleton cases, 353 (10.4%) were associated with a chromosomal anomaly, genetic syndrome or microdeletion, 784 (28.2%) were associated with other major structural anomalies. The male to female ratio of CDH cases overall was 1:0.69. Total prevalence was 2.3 (95% CI 2.2 to 2.4) per 10 000 births and 1.6 (95% CI 1.6 to 1.7) for isolated CDH cases. There was a small but significant increase (relative risk (per year)=1.01, 95% credible interval 1.00–1.01; p=0.030) in the prevalence of total CDH over time but there was no significant increase for isolated cases (ie, CDH cases that did not occur with any other congenital anomaly). There was significant variation in total and isolated CDH prevalence between registers. The proportion of cases that survived to 1 week was 69.3% (1392 cases) for total CDH cases and 72.7% (1107) for isolated cases.

Conclusions This large population-based study found an increase in total CDH prevalence over time. CDH prevalence also varied significantly according to geographical location. No significant association was found with maternal age.

  • congenital diaphragmatic hernia
  • Maternal age
  • Population based
  • Prevalence
  • Survival

https://doi.org/10.1136/archdischild-2014-306174

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