ArticleArchives of disease in childhood2017
Hirschsprung's disease in the UK and Ireland: incidence and anomalies.
Article in Archives of disease in childhood, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers, 4 of them syntheses that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
39 citing papers in PubMed, 4 syntheses or guidelines pooled it, 105 citations in OpenAlex.
- Updated European Reference Network for rare Inherited and Congenital Digestive and Gastrointestinal Anomalies guidelines for the management of rectosigmoid Hirschsprung's disease 2025.Journal of pediatric gastroenterology and nutrition · 2026Guideline
- Systematic appraisal of the guidelines for the diagnosis and treatment of Hirschsprung's disease.Pediatric surgery international · 2022Pooled it
- The prevalence and clinical presentation of Hirschsprung's disease in preterm infants: a systematic review and meta-analysis.Pediatric surgery international · 2022Pooled it
- Hirschsprung's disease in twins: a systematic review and meta-analysis.Pediatric surgery international · 2017Pooled it
- Combinatorial multiomic analysis from a pedigree of Sox10Dom Hirschsprung mice identifies multiple high confidence candidate modifiers of Enteric Nervous System development.PLoS computational biology · 2026Article
- Article
- Trends in Paediatric surgerical volume and associated mortality in England: a nationwide study over an eight year period.BJA open · 2025Article
- Epidemiology and Surgical Management of Hirschsprung's Disease in France: A 12-Year Retrospective Analysis (2012-2023).Acta paediatrica (Oslo, Norway : 1992) · 2025Article
- Impaired Fertility and Sexual Function in Women With Hirschsprung Disease: Results From an International Multi-Centre Cross-Sectional Study.BJOG : an international journal of obstetrics and gynaecology · 2025Article
- EMB is essential for enteric nervous system development mediated by PI3K signaling.Genome medicine · 2025Article
- Post pull- through bowel function outcome and contributing factors in children; a cross-sectional study from low resource setting.BMC surgery · 2025Article
- Risk factors of bowel perforation in neonates with Hirschsprung disease.Pediatric surgery international · 2025Article
- Hirschsprung disease at a tertiary hospital: Patient profile, management and outcomes.Health SA = SA Gesondheid · 2025Article
- Early presentation of Hirschsprung's disease with initial meconium passage: A diagnostic challenge.SAGE open medical case reports · 2025Article
- Investigations, management and outcome of neonates presenting with distal intestinal obstruction: challenging the need for contrast enemas.Pediatric surgery international · 2024Article
- Hirschprung's disease and postpartum trauma leading to fecal incontinence: Why? How?Revista da Associacao Medica Brasileira (1992) · 2024Article
- Early Detection and Intervention for Hirschsprung's Disease: A Key to Successful Outcomes.Clinical medicine insights. Case reports · 2024Article
- Hirschsprung disease.Nature reviews. Disease primers · 2023Review
- A Rare Case of Left Hemicolectomy Masking the Signs and Symptoms of Underlying Hirschsprung's Disease in an Adult.Cureus · 2023Article
- Neonatal intestinal failure - improved outcomes.Frontline gastroenterology · 2023Article
Corrections and comments
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Authors and funding
6 authors at 3 institutions in 1 country.
Funding
Abstract
objectivesTo describe clinical characteristics and preoperative management of a national cohort of infants with Hirschsprung's disease (HD).
designPopulation-based cohort study of all live-born infants with HD born in the UK and Ireland from October 2010 to September 2012.
settingAll 28 paediatric surgical centres in the UK and Ireland.
participants305 infants presenting before 6 months of age with histologically proven HD.
main outcome measuresIncidence, clinical characteristics including gestational age, birth weight, gender, associated anomalies; age and clinical features at presentation; and use of rectal washouts or stoma.
resultsThe incidence of HD in the UK and Ireland was 1.8 per 10 000 live births (95% CI 1.5 to 1.9). Male to female ratio was 3.3:1. An associated anomaly was identified in 23% (69), with 15% (47) having a recognisable syndrome. The proportion of infants who presented and were diagnosed in the neonatal period was 91.5% (279) and 83.9% (256), respectively. 23.9% (73) and 44.2% (135) passed meconium within 24 and 48 hours of birth. 81% (246) first presented to a hospital without tertiary paediatric surgical services, necessitating interhospital transfer. Initial colonic decompression was by rectal washouts in 86.2% (263) and by defunctioning stoma in 12.8% (39). Subsequently, 27.4% (72) of infants failed management with rectal washouts and required a delayed stoma, resulting in 36.4% (111) of infants having a stoma.
conclusionsIn this population-based cohort, presentation outside the neonatal period was rare. Nearly half of the infants with HD passed meconium within 48 hours of birth and over one third were managed with a stoma.
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