title: Functional outcomes in Hirschsprung disease: A single institution's 12-year experience.
abstract: OBJECTIVE
  Hirschsprung disease (HD) is a chronic condition associated with long-term morbidity. We assessed the short and long-term functional outcomes of operated patients in a single institution over a 12-year period.
  
  
  METHODS
  We conducted a retrospective review of all children operated for HD between 2002 and 2014. Postoperative functional outcomes were assessed using the Rintala Bowel Function Score (BFS, 0-20, 20=best score). We assessed hospital admissions, complications including Hirschsprung associated enterocolitis (HAEC) and the need for further surgical procedures.
  
  
  RESULTS
  72 (52 male) patients were studied, of whom, 6 (8%) had a positive family history, 5 (7%) had Trisomy 21 and 5 (7%) had total colonic HD. The median age at diagnosis was 6.5days (2 days-6.7 years) and median follow-up was 6years (1-12years). All patients except two underwent a Duhamel pull-through procedure. The median age at surgery was 4months (6days-90months). 37 (51%) procedures were performed single-stage and 7 (10%) were laparoscopically assisted. Our early complication rate was 15%; 11 (15%) patients were treated for HAEC and 43 (60%) did not require any further surgery. 12 (17%) underwent injection of botulinum toxin, 7 (10%) needed residual spur division and 4 (5%) required an unplanned, post pull-through enterostomy for obstructive defecation symptoms and HAEC. Two (3%) patients underwent an Antegrade Colonic Enema (ACE) stoma. The median BFS was 17 (5-20). There were two deaths both out of hospital.
  
  
  CONCLUSIONS
  Long-term functional outcomes following Duhamel Pull-Through surgery are satisfactory although 40% of patients needed some form of further surgical intervention. The management of anal sphincter achalasia has improved with the use of botulinum toxin and we advocate aggressive and early management of this condition for symptoms of obstructive defecation and HAEC.
  
  
  METHODS
  III.
authors.affiliation: Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH.
authors.forename: Hemanshoo S
authors.initials: HS
authors.lastname: Thakkar
authors.affiliation: Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH.
authors.forename: Christopher
authors.initials: C
authors.lastname: Bassett
authors.affiliation: Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH.
authors.forename: Andy
authors.initials: A
authors.lastname: Hsu
authors.affiliation: Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH.
authors.forename: Riccardo
authors.initials: R
authors.lastname: Manuele
authors.affiliation: Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH.
authors.forename: Dorothy
authors.initials: D
authors.lastname: Kufeji
authors.affiliation: Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH.
authors.forename: Catherine A
authors.initials: CA
authors.lastname: Richards
authors.affiliation: Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH.
authors.forename: Meena
authors.initials: M
authors.lastname: Agrawal
authors.affiliation: Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH. Electronic address: ali.keshtgar@gstt.nhs.uk.
authors.forename: Alireza S
authors.initials: AS
authors.lastname: Keshtgar
mesh_terms: D001003:Anal Canal / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003248:Constipation / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D013505:Digestive System Surgical Procedures; D005260:Female; D005500:Follow-Up Studies; D006627:Hirschsprung Disease / Q000503:physiopathology* / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications / Q000175:diagnosis / Q000503:physiopathology; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Duhamel operation; Functional outcomes; Hirschsprung disease