title: Adult outcomes after surgery for Hirschsprung's disease: Evaluation of bowel function and quality of life.
abstract: OBJECTIVE
  Hirschsprung's disease (HSCR) is treated surgically. There is a risk for faecal incontinence and constipation postoperatively. The long-term bowel functional outcome in adults and quality of life are sparsely studied. The aim of this study was to assess bowel function and quality of life in patients who had undergone surgery for HSCR during childhood.
  
  
  METHODS
  All patients treated between 1969 and 1994 at St. Göran's Children's Hospital in Stockholm were invited to participate in the study. After consent, the patients received questionnaires containing general questions, validated questions on bowel function, questions about urinary function, SF-36 health survey (SF-36) and the Gastrointestinal Quality of Life Index (GIQLI). Clinical data were extracted from the case records. Controls matched for sex and age were randomly selected from the National Swedish Population Register.
  
  
  RESULTS
  48 of 60 (80%) invited patients responded to the questionnaires. Nine patients were excluded since the HSCR diagnosis could not be confirmed. The median age of the included patients was 28 (20-43) years. Most patients had undergone Soave's operation (73.4%) and two patients had a stoma at the time of follow-up. The bowel function was impaired in the HSCR group compared to controls, especially problems with flatulence, need to strain at defecation and several defecations for emptying. Patients in the HSCR group also had significantly more problems with faecal incontinence than controls. Quality of life according to SF-36 did not differ significantly between patients and controls, but the GIQLI score showed a significantly worse outcome in the HSCR group compared to the controls.
  
  
  CONCLUSIONS
  General quality of life in adults treated for HSCR during childhood is comparable to controls. However, they have impaired bowel function and gastrointestinal quality of life.
authors.affiliation: Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden; Division of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden. Electronic address: anna.lof@ki.se.
authors.forename: Anna Löf
authors.initials: AL
authors.lastname: Granström
authors.affiliation: Institution of Women's and Children's Health, Uppsala University, Uppsala, Sweden.
authors.forename: Johan
authors.initials: J
authors.lastname: Danielson
authors.affiliation: Department of CLINTEC, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Britt
authors.initials: B
authors.lastname: Husberg
authors.affiliation: Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden; Division of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Agneta
authors.initials: A
authors.lastname: Nordenskjöld
authors.affiliation: Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden; Division of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Tomas
authors.initials: T
authors.lastname: Wester
mesh_terms: D000328:Adult; D000367:Age Factors; D003248:Constipation / Q000209:etiology*; D003672:Defecation; D005242:Fecal Incontinence / Q000209:etiology*; D005260:Female; D005414:Flatulence / Q000209:etiology*; D006627:Hirschsprung Disease / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D008297:Male; D011183:Postoperative Complications; D011184:Postoperative Period; D011788:Quality of Life; D011795:Surveys and Questionnaires; D013548:Sweden; D016896:Treatment Outcome; D014554:Urination; D055815:Young Adult
keywords: Bowel function; Gastrointestinal quality of life; Hirschsprung’s disease; Long-term follow-up; Quality of life