Small intestinal atresia in a defined population: occurrence, prenatal diagnosis and survival

Abstract

Objective: To describe the prevalence, associated anomalies, prenatal diagnosis, and survival of cases of congenital small intestinal atresia (SIA).

Method: Data were extracted from the Northern Congenital Abnormality Survey (NorCAS) for cases delivered during 1991 to 2001.

Results: A total of 99 cases of SIA were notified from 372 717 registered births. Twenty-five (25.8%) SIA cases were associated with a chromosomal anomaly. Of the 72 SIA cases with normal karyotype, 18 (25%) were associated with other structural anomalies. The total prevalence for all SIA cases over the 11 years was 2.66 per 10 000 registered births (95% CI 2.13, 3.18), and the livebirth prevalence was 2.37 per 10 000 livebirths (95% CI 1.88, 2.87). For jejunoileal atresia, the total prevalence increased from 0.54 per 10 000 births during 1991-1995 to 1.11 per 10 000 births during 1996-2001, and livebirth prevalence increased from 0.49 to 1.06 per 10 000 livebirths. The more proximal the lesion the prenatal diagnosis sensitivity was slightly better with 19 (44.2%) cases of duodenal atresia and 3 (42.8%) cases of jejunal atresia diagnosed prenatally by routine ultrasonography.

Conclusion: This population-based study of SIA has confirmed several previous findings but, by considering subtypes separately, it also suggests a trend towards an increase in the prevalence of jejunoileal atresia (JIA).