Hirschsprung's disease in the UK and Ireland: incidence and anomalies. Issue 8 (9th March 2017)

• Record Type: Journal Article
• Authors: Bradnock, T J; Knight, M; Kenny, S; Nair, M; Walker, G M
• Other Names: author non-byline.; Draper Elizabeth author non-byline.; Aslam Adil author non-byline.; Kazmierski Marcin author non-byline.; Jawaid Wajid author non-byline.; Jester Ingo author non-byline.; Niyogi Anindya author non-byline.; Cusick Eleri author non-byline.; McNally Janet author non-byline.; Clarke Simon author non-byline.; Galea Julie author non-byline.; Parker Shabnam author non-byline.; Broadis Emily author non-byline.; Brownlee Ewan author non-byline.; Keys Charles author non-byline.; Kufeji Dorothy author non-byline.; Alexander Nicholas author non-byline.; Curry Jo author non-byline.; Gupta Samir author non-byline.; Paramalingam Saravanakumar author non-byline.; Besarovic Sanja author non-byline.; Johnson Paul author non-byline.; Jones Ceri author non-byline.; Lakshminarayanan Bhanu author non-byline.; Carnaghan Helen author non-byline.; Patel Shailesh author non-byline.; Sugarman Ian author non-byline.; Nour Shawqui author non-byline.; Tsang Thomas author non-byline.; Kurinczuk Jenny author non-byline.; Puri Prem author non-byline.; McMahon Suzanne author non-byline.; Motiwale Sandeep author non-byline.; Singh Shailinder author non-byline.; Thompson Richard author non-byline.; Healy Costa author non-byline.; Mahomed Anies author non-byline.; Marshall David author non-byline.; Ervine Evelyn author non-byline.; Shipley Lara author non-byline.; Zani Augusto author non-byline.; Charlesworth Paul author non-byline.; Joshi Ashwini author non-byline.; Ragazzi Simone author non-byline.; Craigie Ross author non-byline.; Chhabra Sumita author non-byline.; Jaffray Bruce author non-byline.; Lawson Anne author non-byline.; Marven Sean author non-byline.; Lloyd Karen author non-byline.; Murthi Govind author non-byline.; Stanton Michael author non-byline.; Blackburn Simon author non-byline.; Giuliani Stefano author non-byline.; Nicholls Eric author non-byline.; Okoye Bruce author non-byline.; McDowell Dermot author non-byline.; Mortell Alan author non-byline.; McMahon Suzanne author non-byline.; Huddart Simon author non-byline.; Shrestha Ram author non-byline.; Rowsell Julianne author non-byline.;
• Is Part Of: Archives of disease in childhood. Volume 102:Issue 8(2017)
• Page Start: 722
• Page End: 727
• Publication Date: 2017-03-09
• Subjects: Hirschsprung's disease -- Incidence -- Associated anomalies -- Management; Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/archdischild-2016-311872
• Languages: English
• ISSNs: 0003-9888

Abstract

Abstract : Objectives: To describe clinical characteristics and preoperative management of a national cohort of infants with Hirschsprung's disease (HD). Design: Population-based cohort study of all live-born infants with HD born in the UK and Ireland from October 2010 to September 2012. Setting: All 28 paediatric surgical centres in the UK and Ireland. Participants: 305 infants presenting before 6 months of age with histologically proven HD. Main outcome measures: Incidence, clinical characteristics including gestational age, birth weight, gender, associated anomalies; age and clinical features at presentation; and use of rectal washouts or stoma. Results: The 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. Conclusions: In 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.