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BACKGROUND/PURPOSE: Primary pull-through without a stoma has become preferred practice in managing Hirschsprung disease (HD). The aims of this study were to establish stoma rate and identify factors associated with stoma formation in a population-based cohort in the UK and Ireland. METHODS: Live-born infants with HD were prospectively identified in all 28 specialist pediatric surgical units in the UK and Ireland between October 2010 to September 2012. Method of colonic decompression was recorded and multivariable logistic regression was used to identify factors associated with stoma formation. RESULTS: 305 infants with HD were identified. Rectal washouts were initially used in 86% (263) with a defunctioning stoma formed as the primary management in 13% (39). Ultimately, 36% (111) required a stoma prior to definitive surgery. Compared to infants managed with rectal washouts alone; infants managed with a stoma were more likely to have a transition zone proximal to the splenic flexure, Down (or another) syndrome, and HD diagnosis established more than 28days after presentation. CONCLUSIONS: Although rectal washouts are commonly employed, a stoma prior to definitive surgery was required in 36% of infants in a national cohort. Delayed diagnosis, aganglionosis proximal to the splenic flexure and presence of other anomalies are associated with stoma formation. TYPE OF STUDY AND LEVEL OF EVIDENCE: Prognosis study (high-quality prospective cohort study with 80% follow-up and all patients enrolled at same time point in disease).

Original publication




Journal article


J Pediatr Surg

Publication Date





1451 - 1457


Hirschsprung disease, Incidence, Management, Presentation, Stoma, Female, Hirschsprung Disease, Humans, Infant, Ireland, Male, Prospective Studies, Surgical Stomas, United Kingdom