title: Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study.
abstract: BACKGROUND
  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.
authors.affiliation: Institute of Health & Society, Newcastle University, Newcastle upon Tyne, UK.
authors.forename: Mark R
authors.initials: MR
authors.lastname: McGivern
authors.affiliation: Institute of Health & Society, Newcastle University, Newcastle upon Tyne, UK.
authors.forename: Kate E
authors.initials: KE
authors.lastname: Best
authors.affiliation: Institute of Health & Society, Newcastle University, Newcastle upon Tyne, UK.
authors.forename: Judith
authors.initials: J
authors.lastname: Rankin
authors.affiliation: Faculty of Medicine and Wessex Clinical Genetics Service, University Hospitals Southampton, Southampton, UK.
authors.forename: Diana
authors.initials: D
authors.lastname: Wellesley
authors.affiliation: University of Ulster, Ulster, UK.
authors.forename: Ruth
authors.initials: R
authors.lastname: Greenlees
authors.affiliation: Service de Genetique Medicale Maternite, CHUV, Lausanne, Switzerland.
authors.forename: Marie-Claude
authors.initials: MC
authors.lastname: Addor
authors.affiliation: Public Health Division of Gipuzkoa, Instituto Bio-Donostia, Basque Government, CIBER Epidemiología y Salud Pública, CIBERESP, Spain.
authors.forename: Larraitz
authors.initials: L
authors.lastname: Arriola
authors.affiliation: Eurocat Northern Netherlands, Department of Genetics, University of Groningen, University Medical Center, Groningen, The Netherlands.
authors.forename: Hermien
authors.initials: H
authors.lastname: de Walle
authors.affiliation: Children's Hospital Zagreb, University of Zagreb, School of Medicine, Zagreb, Croatia.
authors.forename: Ingeborg
authors.initials: I
authors.lastname: Barisic
authors.affiliation: Department of Hungarian Congenital Abnormality Registry & Surveillance, National Institute of Health Development, Budapest, Hungary.
authors.forename: Judit
authors.initials: J
authors.lastname: Beres
authors.affiliation: Department of Medical Genetics, ARNAS Garibaldi Nesima, Catania, Italy.
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Bianchi
authors.affiliation: IMER Registry (Emila Romagna Registry of Birth Defects), Ferrara, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Calzolari
authors.affiliation: Department of de Genetique Medicale, Hopital de Hautepierre, Strasbourg, France.
authors.forename: Berenice
authors.initials: B
authors.lastname: Doray
authors.affiliation: Department of Health Sciences, University of Leicester, Leicester, UK.
authors.forename: Elizabeth S
authors.initials: ES
authors.lastname: Draper
authors.affiliation: Hospital Lillebaelt, Kolding, Denmark.
authors.forename: Ester
authors.initials: E
authors.lastname: Garne
authors.affiliation: Department of Health Information and Research, Guardamangia, Malta.
authors.forename: Miriam
authors.initials: M
authors.lastname: Gatt
authors.affiliation: Medical University of Graz, Graz, Austria.
authors.forename: Martin
authors.initials: M
authors.lastname: Haeusler
authors.affiliation: Paris Registry of Congenital Malformations, INSERM U953, Paris, France.
authors.forename: Babak
authors.initials: B
authors.lastname: Khoshnood
authors.affiliation: Medical Birth Registry of Norway, Norwegian Institute of Public Health and Department of Global Public Health and Primary Care, University of Bergen, Bergen, Norway.
authors.forename: Kari
authors.initials: K
authors.lastname: Klungsoyr
authors.affiliation: Polish Registry of Congenital Malformations, Poznan, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Latos-Bielenska
authors.affiliation: Health Service Executive, Cork, Ireland.
authors.forename: Mary
authors.initials: M
authors.lastname: O'Mahony
authors.affiliation: Instituto Nacional de Saude Dr Ricardo Jorge, Lisbon, Portugal.
authors.forename: Paula
authors.initials: P
authors.lastname: Braz
authors.affiliation: Health Service Executive, Dublin, Ireland.
authors.forename: Bob
authors.initials: B
authors.lastname: McDonnell
authors.affiliation: Public Health Department, HSE South, Kilkenny, Ireland.
authors.forename: Carmel
authors.initials: C
authors.lastname: Mullaney
authors.affiliation: Provinciaal Instituut voor Hygiene, Antwerp, Belgium.
authors.forename: Vera
authors.initials: V
authors.lastname: Nelen
authors.affiliation: Birth Registry Mainz Model, Childrens Hospital, University Medical Center, Johannes Gutenberg-University, Mainz, Germany.
authors.forename: Anette
authors.initials: A
authors.lastname: Queisser-Luft
authors.affiliation: Medical Genetics Unit of CHU Sud Réunion, Ile de la Reunion, France.
authors.forename: Hanitra
authors.initials: H
authors.lastname: Randrianaivo
authors.affiliation: Malformation Monitoring Centre Saxony-Anhalt, Medical Faculty Otto-von-Guericke University, Magdeburg, Germany.
authors.forename: Anke
authors.initials: A
authors.lastname: Rissmann
authors.affiliation: National Perinatal Epidemiology Unit, University of Oxford, Oxford, UK.
authors.forename: Catherine
authors.initials: C
authors.lastname: Rounding
authors.affiliation: National Registry of Congenital Anomalies, Department of Medical Genetics, Thomayer Hospital, Prague, Czech Republic.
authors.forename: Antonin
authors.initials: A
authors.lastname: Sipek
authors.affiliation: South West England Congenital Anomaly Register, Bristol, UK.
authors.forename: Rosie
authors.initials: R
authors.lastname: Thompson
authors.affiliation: Public Health Wales, Wales, UK.
authors.forename: David
authors.initials: D
authors.lastname: Tucker
authors.affiliation: OMNI-Net Ukraine Birth Defects Program, Rivne- Khmelnytskyy, Ukraine.
authors.forename: Wladimir
authors.initials: W
authors.lastname: Wertelecki
authors.affiliation: Centro Superior de Investigación en Salud Pública-FISABIO, Valencia, Spain.
authors.forename: Carmen
authors.initials: C
authors.lastname: Martos
mesh_terms: D000015:Abnormalities, Multiple / Q000453:epidemiology; D000293:Adolescent; D000328:Adult; D001724:Birth Weight; D005060:Europe / Q000453:epidemiology; D005260:Female; D005865:Gestational Age; D065630:Hernias, Diaphragmatic, Congenital / Q000453:epidemiology*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008423:Maternal Age; D011159:Population Surveillance; D015995:Prevalence; D012042:Registries; D016019:Survival Analysis; D055815:Young Adult
keywords: Maternal age; Population based; Prevalence; Survival; congenital diaphragmatic hernia