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Multicenter Study
. 2020 Apr;36(4):477-483.
doi: 10.1007/s00383-020-04628-w. Epub 2020 Feb 29.

The impact of trisomy 21 on epidemiology, management, and outcomes of congenital duodenal obstruction: a population-based study

George S Bethell  1 , Anna-May Long  2   3 , Marian Knight  2 , Nigel J Hall  4 ; BAPS-CASS
Collaborators, Affiliations
Multicenter Study

The impact of trisomy 21 on epidemiology, management, and outcomes of congenital duodenal obstruction: a population-based study

George S Bethell et al. Pediatr Surg Int. 2020 Apr.

Abstract

Purpose: Congenital duodenal obstruction (CDO) is associated with trisomy 21 (T21), or Down's syndrome, in around a third of infants. The aim of this study was to explore the impact of T21 on the epidemiology, management, and outcomes of infants with CDO.

Methods: Data were prospectively collected from specialist neonatal surgical centres in the United Kingdom over a 12 month period from March 2016 using established population-based methodology for all babies with CDO. Infants with T21 were compared to those without any chromosomal anomaly.

Results: Of 102 infants with CDO that underwent operative repair, T21 was present in 33 [32% (95% CI 23-41%)] babies. Cardiac anomalies were more common in those with T21 compared to those without a chromosomal anomaly (91 vs 17%, p < 0.001), whereas associated gastrointestinal anomalies were less common in infants with T21 (3 vs 12%, p = 0.03). Surgical management was not influenced by T21. Time to achieve full enteral feed, need for repeat related surgery, and mortality were similar between groups. Infants with T21 had a longer median initial inpatient stay (23 vs 16.5 days, p = 0.02).

Conclusions: Infants with T21 have a higher incidence of cardiac anomalies and a longer initial inpatient stay; however, it does not change CDO management or outcomes. This information is important for prenatal and postnatal counselling of parents of infants with CDO and T21.

Keywords: Congenital cardiac disease; Down syndrome; Duodenal atresia; Duodenal stenosis; Trisomy 21.

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Conflict of interest statement

All authors declare that they have no conflicts of interest.

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