title: Patient-reported Swedish nationwide outcomes of children and adolescents with total colonic aganglionosis.
abstract: BACKGROUND
  The aim of this study was to evaluate the nationwide outcome of children with total colonic aganglionosis (TCA) during the last 20years.
  
  
  METHODS
  This was an observational, cross-sectional study where all patients with TCA, including aganglionosis of 0-50cm of ileum, born in Sweden 1995-2014 were included. Data were collected from the medical records. Patients >4years old without stoma answered a questionnaire regarding bowel function (bowel function score, BFS, score 1-20), medical treatment and nutrition.
  
  
  RESULTS
  Twenty-seven children were included. Twenty-five children were reconstructed at median age of 56 (4-236) weeks. Reconstruction procedures included Swenson (6), Soave (5), mucosectomy with short muscular cuff with or without J-pouch (9), Duhamel (3) and Rehbein (2). There was no mortality. The median follow-up time was 9.5years (8months-20years). At follow-up 7 (26%) patients had an ileostomy, 4 with a syndrome. Eight patients required parenteral support, until a median age of 11 (2-24) months. Oral energy support was used by 5/27 (15%), still 5/22 (23%) were underweighted. Obstructive symptoms were reported by 7/20 (31%). All 17 patients >4years old completed the BFS questionnaire at median age of 10 (4-20) years. Median stool frequency/24h was 5 (1-30). Fecal accidents at least once per week was reported by 4 (24%), and social problems by 8 (47%). The median BFS was 15 (11-19) without any gender differences.
  
  
  CONCLUSIONS
  One-third of patients with TCA report obstructive symptoms, one-third need additional nutrition and one-fifth require a permanent stoma. TCA have a negative impact on social life. Subsequently, children with TCA need a careful lifelong follow-up of specialized teams.
authors.affiliation: Lund University, Department of Pediatric Surgery, Skane University Hospital Lund, 22185 Lund, Sweden. Electronic address: pernilla.stenstrom@med.lu.se.
authors.forename: Pernilla
authors.initials: P
authors.lastname: Stenström
authors.affiliation: The Queen Silvia Children's Hospital, The Sahlgrenska Academy University of Gothenburg, Gothenburg, Sweden.
authors.forename: Matilda
authors.initials: M
authors.lastname: Brautigam
authors.affiliation: The Queen Silvia Children's Hospital, The Sahlgrenska Academy University of Gothenburg, Gothenburg, Sweden.
authors.forename: Helena
authors.initials: H
authors.lastname: Borg
authors.affiliation: Lund University, Department of Pediatric Surgery, Skane University Hospital Lund, 22185 Lund, Sweden.
authors.forename: Christina
authors.initials: C
authors.lastname: Graneli
authors.affiliation: Unit of Pediatric Surgery, Department of Women's and Children's Health, University Children's Hospital, Uppsala, Sweden.
authors.forename: Helene Engstrand
authors.initials: HE
authors.lastname: Lilja
authors.affiliation: Unit of Pediatric Surgery, Department of Women's and Children's Health, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Tomas
authors.initials: T
authors.lastname: Wester
mesh_terms: D000293:Adolescent; D002648:Child; D003430:Cross-Sectional Studies; D003672:Defecation / Q000502:physiology*; D013505:Digestive System Surgical Procedures / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D005544:Forecasting; D006627:Hirschsprung Disease / Q000453:epidemiology / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D007082:Ileum / Q000601:surgery*; D015994:Incidence; D008297:Male; D000071066:Patient Reported Outcome Measures; D011183:Postoperative Complications / Q000453:epidemiology*; D012189:Retrospective Studies; D011795:Surveys and Questionnaires; D013548:Sweden / Q000453:epidemiology; D016896:Treatment Outcome; D055815:Young Adult
keywords: Bowel function; Hirschsprung disease; Nutrition; Total colonic aganglionosis