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Does Down syndrome affect the outcome of congenital duodenal obstruction?
M V A Singh1, C Richards, J C Bowen
1Department of Paediatric Surgery, Royal Manchester Children's Hospital, Hospital Road, Pendlebury, Manchester M27 4HA , UK. mvasingh@hotmail.com
Insights
Congenital duodenal obstruction (DO) in children with Down syndrome (DS) shows similar postoperative outcomes but a higher rate of cardiac defects. DS does not impact DO morbidity or mortality.
Area of Science:
- Pediatric Surgery
- Clinical Genetics
- Neonatology
Background:
- Congenital duodenal obstruction (DO) is frequently associated with Down syndrome (DS) and other congenital anomalies.
- Previous studies have not fully elucidated the impact of DS on the spectrum of malformations and postoperative outcomes in DO patients.
Purpose of the Study:
- To investigate the influence of Down syndrome on associated congenital malformations and postoperative morbidity and mortality in infants with congenital duodenal obstruction.
Main Methods:
- Retrospective analysis of 79 infants with congenital duodenal obstruction over an 11-year period.
- Patients were divided into two groups: those with DS (n=28) and those without DS (n=51).
- Comparison of gestational age, birth weight, incidence of coexisting malformations, time to full feeds, complications, and mortality between groups.
Main Results:
- No significant differences were observed in gestational age, birth weight, time to full feeds, or postoperative complications between the DS and non-DS groups.
- Congenital malformations were present in 68% of patients; gastrointestinal anomalies were most common in the non-DS group, while cardiac anomalies predominated in the DS group (81.5%).
- Mortality rates did not differ significantly between groups, though delayed mortality in the DS group was linked to cardiac and respiratory conditions.
Conclusions:
- Down syndrome does not significantly influence the short-term morbidity and mortality associated with congenital duodenal obstruction.
- Infants with Down syndrome and duodenal obstruction have a notably higher incidence of congenital cardiac abnormalities.
- Coexisting cardiac and respiratory diseases are significant factors in delayed mortality for infants with Down syndrome and duodenal obstruction.
Abstract:
Congenital duodenal obstruction (DO) has a well-known association with Down syndrome (DS) and other congenital malformations. Previously reported series on DO have not examined the influence of DS on associated congenital malformations and postoperative morbidity and mortality. We report on a retrospective study of all children born with DO over an 11-year period to investigate this. A total of 79 patients with DO were studied: group 1 consisted of 51 (64.6%) children without DS, and group 2 consisted of 28 (35.4%) children with DS. There was no significant difference in mean gestational age and birth weight between groups 1 and 2. A coexisting congenital malformation was found in 68% of patients. Gastrointestinal malformations were the most common in group 1 (71.1%), and cardiac malformations were the most common in group 2 (81.5%). The mean time to reach full intragastric feeds was 12 days, with no significant difference between the two groups ( p=0.383). Seven (8.9%) patients developed a postoperative complication, with no significant difference between both groups ( p=0.853). A total of 11 patients died: six (12%) in group 1 and five (28%) in group 2, with no significant difference between the groups ( p=0.454). DS does not influence the morbidity and mortality of DO but does carry a higher incidence of congenital cardiac abnormalities. Delayed mortality was a result of coexisting congenital cardiac and respiratory disease.
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