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Patient-reported Swedish nationwide outcomes of children and adolescents with total colonic aganglionosis.
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2016 (English)In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, S0022-3468(16)30580-2Article in journal (Refereed) Epub ahead of print
Abstract [en]

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.

CONCLUSION: 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.

Place, publisher, year, edition, pages
2016. S0022-3468(16)30580-2
Keyword [en]
Bowel function, Hirschsprung disease, Nutrition, Total colonic aganglionosis
National Category
Pediatrics
Identifiers
URN: urn:nbn:se:uu:diva-319274DOI: 10.1016/j.jpedsurg.2016.11.033PubMedID: 27912975OAI: oai:DiVA.org:uu-319274DiVA: diva2:1086473
Available from: 2017-04-03 Created: 2017-04-03 Last updated: 2017-04-03

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Engstrand Lilja, Helene
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