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Arch Dis Child Fetal Neonatal Ed. 2015 Mar;100(2):F137-44. doi: 10.1136/archdischild-2014-306174. Epub 2014 Nov 19.

Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study.

Author information

1
Institute of Health & Society, Newcastle University, Newcastle upon Tyne, UK.
2
Faculty of Medicine and Wessex Clinical Genetics Service, University Hospitals Southampton, Southampton, UK.
3
University of Ulster, Ulster, UK.
4
Service de Genetique Medicale Maternite, CHUV, Lausanne, Switzerland.
5
Public Health Division of Gipuzkoa, Instituto Bio-Donostia, Basque Government, CIBER Epidemiología y Salud Pública, CIBERESP, Spain.
6
Eurocat Northern Netherlands, Department of Genetics, University of Groningen, University Medical Center, Groningen, The Netherlands.
7
Children's Hospital Zagreb, University of Zagreb, School of Medicine, Zagreb, Croatia.
8
Department of Hungarian Congenital Abnormality Registry & Surveillance, National Institute of Health Development, Budapest, Hungary.
9
Department of Medical Genetics, ARNAS Garibaldi Nesima, Catania, Italy.
10
IMER Registry (Emila Romagna Registry of Birth Defects), Ferrara, Italy.
11
Department of de Genetique Medicale, Hopital de Hautepierre, Strasbourg, France.
12
Department of Health Sciences, University of Leicester, Leicester, UK.
13
Hospital Lillebaelt, Kolding, Denmark.
14
Department of Health Information and Research, Guardamangia, Malta.
15
Medical University of Graz, Graz, Austria.
16
Paris Registry of Congenital Malformations, INSERM U953, Paris, France.
17
Medical Birth Registry of Norway, Norwegian Institute of Public Health and Department of Global Public Health and Primary Care, University of Bergen, Bergen, Norway.
18
Polish Registry of Congenital Malformations, Poznan, Poland.
19
Health Service Executive, Cork, Ireland.
20
Instituto Nacional de Saude Dr Ricardo Jorge, Lisbon, Portugal.
21
Health Service Executive, Dublin, Ireland.
22
Public Health Department, HSE South, Kilkenny, Ireland.
23
Provinciaal Instituut voor Hygiene, Antwerp, Belgium.
24
Birth Registry Mainz Model, Childrens Hospital, University Medical Center, Johannes Gutenberg-University, Mainz, Germany.
25
Medical Genetics Unit of CHU Sud Réunion, Ile de la Reunion, France.
26
Malformation Monitoring Centre Saxony-Anhalt, Medical Faculty Otto-von-Guericke University, Magdeburg, Germany.
27
National Perinatal Epidemiology Unit, University of Oxford, Oxford, UK.
28
National Registry of Congenital Anomalies, Department of Medical Genetics, Thomayer Hospital, Prague, Czech Republic.
29
South West England Congenital Anomaly Register, Bristol, UK.
30
Public Health Wales, Wales, UK.
31
OMNI-Net Ukraine Birth Defects Program, Rivne- Khmelnytskyy, Ukraine.
32
Centro Superior de Investigación en Salud Pública-FISABIO, Valencia, Spain.

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.

KEYWORDS:

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

[Indexed for MEDLINE]

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