Article Text

This article has a correction. Please see:

Download PDFPDF
Epidemiology of small intestinal atresia in Europe: a register-based study
  1. Kate E Best1,
  2. Peter W G Tennant1,
  3. Marie-Claude Addor2,
  4. Fabrizio Bianchi3,
  5. Patricia Boyd4,
  6. Elisa Calzolari5,
  7. Carlos Matias Dias6,
  8. Berenice Doray7,
  9. Elizabeth Draper8,
  10. Ester Garne9,
  11. Miriam Gatt10,
  12. Ruth Greenlees11,
  13. Martin Haeusler12,
  14. Babak Khoshnood13,
  15. Bob McDonnell14,
  16. Carmel Mullaney15,
  17. Vera Nelen16,
  18. Hanitra Randrianaivo17,
  19. Anke Rissmann18,
  20. Joaquin Salvador19,
  21. David Tucker20,
  22. Diana Wellesly21,
  23. Judith Rankin1,22
  1. 1Institute of Health & Society, Newcastle University, Newcastle upon Tyne, UK
  2. 2Division Autonome de Genetique Medicale, Registre Vaudois des Malformations, Vaud, Switzerland
  3. 3CNR Institute of Clinical Physiology, Unit of Epidemiology, Tuscany, Italy
  4. 4National Perinatal Epidemiology Unit, University of Oxford, Oxford, UK
  5. 5Neonatal Intensive Care Unit and Neonatology Hospital, Universita di Ferrara, Emilia Romagna, Italy
  6. 6Centro de Estudos e registo de A C, Lisbon, Portugal
  7. 7Service de Genetique Medicale, Hopitale de Hautepierre, Strasbourg, France
  8. 8Department of Epidemiology & Public Health, University of Leicester, Leicester, UK
  9. 9Paediatric Department, Hospital Lillebaelt, Kolding, Odense, Denmark
  10. 10Department of Health Information, Malta Congenital Anomalies Registry, G’mangia, Malta
  11. 11The Institute of Nursing Research, University of Ulster, Ulster, UK
  12. 12Styrian Malformation Registry, University of Graz, Graz, Austria
  13. 13INSERM U953, Hopital Saint Vincent de Paul, Paris, France
  14. 14Health Information Unit, Dr Steevens Hospital, Dublin, Ireland
  15. 15Department of Public Health, Health Service Executive, Kilkenny, Ireland
  16. 16Provinciaal Instituut voor Hygiene, Antwerp, New York, USA
  17. 17Naitre Aujhourd'hui, Ile de la Reunion, France
  18. 18Malformation Monitoring Centre Saxony-Anhalt, Otto-von-Guericke University, Saxony-Anhalt, Germany
  19. 19Regional Maternity Survey Office, Newcastle upon Tyne, UK
  20. 20SESIS, Agencia de Salut Publica de Barcelona, Barcelona, Spain
  21. 21Congenital Anomaly Register & Info Service, Singleton Hospital, Swansea, United Kingdom
  22. 22Wessex Clinical Genetics Service, Princess Anne Hospital, Southampton, United Kingdom
  1. Correspondence to Professor Judith Rankin, Institute of Health & Society, Newcastle University, Baddiley-Clark Building, Richardson Road, Newcastle upon Tyne, NE2 4AX, England, UK; judith.rankin{at}


Background The epidemiology of congenital small intestinal atresia (SIA) has not been well studied. This study describes the presence of additional anomalies, pregnancy outcomes, total prevalence and association with maternal age in SIA cases in Europe.

Methods Cases of SIA delivered during January 1990 to December 2006 notified to 20 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 In total 1133 SIA cases were reported among 5126, 164 registered births. Of 1044 singleton cases, 215 (20.6%) cases were associated with a chromosomal anomaly. Of 829 singleton SIA cases with normal karyotype, 221 (26.7%) were associated with other structural anomalies. Considering cases with normal karyotype, the total prevalence per 10 000 births was 1.6 (95% CI 1.5 to 1.7) for SIA, 0.9 (95% CI 0.8 to 1.0) for duodenal atresia and 0.7 (95% CI 0.7 to 0.8) for jejunoileal atresia (JIA). There was no significant trend in SIA, duodenal atresia or JIA prevalence over time (RR=1.0, 95% credible interval (CrI): 1.0 to 1.0 for each), but SIA and duodenal atresia prevalence varied by geographical location (p=0.03 and p=0.04, respectively). There was weak evidence of an increased risk of SIA in mothers aged less than 20 years compared with mothers aged 20 to 29 years (RR=1.3, 95% CrI: 1.0 to 1.8).

Conclusion This study found no evidence of a temporal trend in the prevalence of SIA, duodenal atresia or JIA, although SIA and duodenal atresia prevalence varied significantly between registers.

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.


  • Correction notice This article has been corrected since it was published Online First. The last 4 authors were not in the correct order and this has now been rectified.

  • Funding The North of England register is funded by the UK Healthcare Quality Improvement Partnership. The Malformation Monitoring Centre Saxony-Anhalt as a register is funded by the Ministry of Health and Social Affairs of the state of Saxony-Anhalt, Germany. JR is funded by a Personal Award Scheme Career Scientist Award from the UK National Institute of Health Research. This project was funded by the Newlife Foundation. This paper reports on an independent study which is part-funded by the Policy Research Programme in the Department of Health, UK. The views expressed are not necessarily those of the Department.

  • Competing interests None.

  • Provenance and peer review Not commissioned; externally peer reviewed.

Linked Articles

  • Corrections
    BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health