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Patient-reported Swedish nationwide outcomes of children and adolescents with total colonic aganglionosis

Stenström, Pernilla LU ; Brautigam, Matilda; Borg, Helena; Graneli, Christina LU ; Lilja, Helene Engstrand and Wester, Tomas (2017) In Journal of Pediatric Surgery 52(8). p.1302-1307
Abstract

Background: The aim of this study was to evaluate the nationwide outcome of children with total colonic aganglionosis (TCA) during the last 20. years. Methods: This was an observational, cross-sectional study where all patients with TCA, including aganglionosis of 0-50. cm of ileum, born in Sweden 1995-2014 were included. Data were collected from the medical records. Patients>. 4. years 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... (More)

Background: The aim of this study was to evaluate the nationwide outcome of children with total colonic aganglionosis (TCA) during the last 20. years. Methods: This was an observational, cross-sectional study where all patients with TCA, including aganglionosis of 0-50. cm of ileum, born in Sweden 1995-2014 were included. Data were collected from the medical records. Patients>. 4. years 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 9.5. years (8. months-20. years). 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>. 4. years old completed the BFS questionnaire at median age of 10 (4-20) years. Median stool frequency/24. h 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.

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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Bowel function, Hirschsprung disease, Nutrition, Total colonic aganglionosis
in
Journal of Pediatric Surgery
volume
52
issue
8
pages
1302 - 1307
publisher
Elsevier
external identifiers
  • scopus:85007287189
  • wos:000406421300015
ISSN
0022-3468
DOI
10.1016/j.jpedsurg.2016.11.033
language
English
LU publication?
yes
id
038cfdb6-ebb4-4d38-8cbf-2184b79ca2c8
date added to LUP
2017-01-17 14:43:38
date last changed
2018-01-07 11:45:21
@article{038cfdb6-ebb4-4d38-8cbf-2184b79ca2c8,
  abstract     = {<p>Background: The aim of this study was to evaluate the nationwide outcome of children with total colonic aganglionosis (TCA) during the last 20. years. Methods: This was an observational, cross-sectional study where all patients with TCA, including aganglionosis of 0-50. cm of ileum, born in Sweden 1995-2014 were included. Data were collected from the medical records. Patients&gt;. 4. years 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 9.5. years (8. months-20. years). 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&gt;. 4. years old completed the BFS questionnaire at median age of 10 (4-20) years. Median stool frequency/24. h 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.</p>},
  author       = {Stenström, Pernilla and Brautigam, Matilda and Borg, Helena and Graneli, Christina and Lilja, Helene Engstrand and Wester, Tomas},
  issn         = {0022-3468},
  keyword      = {Bowel function,Hirschsprung disease,Nutrition,Total colonic aganglionosis},
  language     = {eng},
  number       = {8},
  pages        = {1302--1307},
  publisher    = {Elsevier},
  series       = {Journal of Pediatric Surgery},
  title        = {Patient-reported Swedish nationwide outcomes of children and adolescents with total colonic aganglionosis},
  url          = {http://dx.doi.org/10.1016/j.jpedsurg.2016.11.033},
  volume       = {52},
  year         = {2017},
}