Small intestinal atresia in europe: prevalence, associated anomalies and pregnancy outcomes. (7th June 2011)

• Record Type: Journal Article
• Authors: Best, K E; Tennant, P W G; Rankin, J
• Is Part Of: Archives of disease in childhood. Volume 96(2011)Supplement 1
• Page Start: Fa56
• Page End: Fa57
• Publication Date: 2011-06-07
• Subjects: Infants -- Diseases -- Periodicals; Newborn infants -- Diseases -- Periodicals; Fetus -- Diseases -- Periodicals; 618.920105
• DOI: 10.1136/adc.2011.300161.11
• Languages: English
• ISSNs: 1359-2998

Abstract

Abstract : Background: Small intestinal atresia (SIA) is a congenital anomaly characterised by the abnormal closure, discontinuity or narrowing of the duodenum, jejunum or ileum. This study aimed to describe the epidemiology of SIA in Europe. Methods: SIA cases delivered during 1990–2006 and notified to 21 European congenital anomaly registers formed this population-based case series. Risk of spontaneous fetal loss, accounting for terminations, was examined by Cox regression. Age-adjusted prevalence was modelled using multilevel Poisson regression. Results: 1154 SIA cases were reported among 5 383 099 births. Of 1092 singleton cases, 222 (20.3%) were associated with chromosomal and 227 (20.8%) with structural anomalies. Chromosomal and structural anomalies occurred more frequently in duodenal atresia than jejunoileal atresia (JIA) (p<0.001 for both). The prevalence for singleton cases of normal karyotype was 1.6 per 10, 000 births (95% CI: 1.5 to 1.7) for SIA, 0.9 (95% CI: 0.8 to 1.0) for duodenal atresia and 0.8 (95% CI: 0.7 to 0.8) for JIA. Of the singleton cases of normal karyotype, 91.4% resulted in live birth, 4.3% in spontaneous fetal loss (≤20 weeks gestation) and 4.4% in termination of pregnancy. The risk of spontaneous fetal loss was 6.2% for SIA, varying significantly between 9.4% for duodenal atresia and 2.4% for JIA (p<0.001). There was no evidence of an increased risk of SIA in mothers aged ≤20 years compared to mothers aged 20 to 29 (relative risk=1.3, 95% credible interval: 1.0 to 1.8; p=0.08). Conclusion: Chromosomal and structural anomalies are more common, and the risk of fetal loss greater, in cases of duodenal atresia compared to JIA.