Colectomy Followed by J-Pouch Reconstruction to Correct Total Colonic Aganglionosis

Background: Patients suffering from complete colonic aganglionosis (TCA) require the best surgical care possible. Only a few studies reported J-Pouch repair as the primary reconstructive surgery in TCA patients. This study adds to the current literature a thorough clinical and functional outcomes gr...

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Main Authors: Konrad Reinshagen, Gunter Burmester, Johanna Hagens, Thomas Franz Krebs, Christian Tomuschat
Format: Article
Language:English
Published: MDPI AG 2022-01-01
Series:Children
Subjects:
Online Access:https://www.mdpi.com/2227-9067/9/1/101
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author Konrad Reinshagen
Gunter Burmester
Johanna Hagens
Thomas Franz Krebs
Christian Tomuschat
author_facet Konrad Reinshagen
Gunter Burmester
Johanna Hagens
Thomas Franz Krebs
Christian Tomuschat
author_sort Konrad Reinshagen
collection DOAJ
description Background: Patients suffering from complete colonic aganglionosis (TCA) require the best surgical care possible. Only a few studies reported J-Pouch repair as the primary reconstructive surgery in TCA patients. This study adds to the current literature a thorough clinical and functional outcomes group. Methods: Between 2011 and 2021, medical records of Hirschsprung disease (HD) patients who underwent J-Pouch reconstruction during infancy (n = 12) were reviewed. In close follow-up, bowel function and satisfaction with operation results were evaluated. The median age at the time of J-Pouch reconstruction was 16 months, and covering ileostomies were closed four months later. There were no postoperative problems. After the final repair, Pouch-related problems (PRP) occurred in 27% of the children and were treated conservatively. There was no histological evidence of pouchitis in any of the individuals. The median 24-h stooling frequency was 4–5 at the latest follow-up 51 months following enterostomy closure. Conclusions: The current study’s findings support the existing literature and advocate for J-pouch repair in TCA patients. However, more research will be needed to determine the best time to undergo pouch surgery and ileostomy closure in TCA patients.
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spelling doaj.art-9cbae9b41bad4dca9771c2ffac04aa022023-11-23T13:21:55ZengMDPI AGChildren2227-90672022-01-019110110.3390/children9010101Colectomy Followed by J-Pouch Reconstruction to Correct Total Colonic AganglionosisKonrad Reinshagen0Gunter Burmester1Johanna Hagens2Thomas Franz Krebs3Christian Tomuschat4Department of Pediatric Surgery, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, GermanyDepartment of Pediatrics, Altonaer Kinderkrankenhaus, 22763 Hamburg, GermanyDepartment of Pediatric Surgery, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, GermanyDepartment of Pediatric Surgery, Children’s Hospital of Eastern Switzerland, 9006 St. Gallen, SwitzerlandDepartment of Pediatric Surgery, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, GermanyBackground: Patients suffering from complete colonic aganglionosis (TCA) require the best surgical care possible. Only a few studies reported J-Pouch repair as the primary reconstructive surgery in TCA patients. This study adds to the current literature a thorough clinical and functional outcomes group. Methods: Between 2011 and 2021, medical records of Hirschsprung disease (HD) patients who underwent J-Pouch reconstruction during infancy (n = 12) were reviewed. In close follow-up, bowel function and satisfaction with operation results were evaluated. The median age at the time of J-Pouch reconstruction was 16 months, and covering ileostomies were closed four months later. There were no postoperative problems. After the final repair, Pouch-related problems (PRP) occurred in 27% of the children and were treated conservatively. There was no histological evidence of pouchitis in any of the individuals. The median 24-h stooling frequency was 4–5 at the latest follow-up 51 months following enterostomy closure. Conclusions: The current study’s findings support the existing literature and advocate for J-pouch repair in TCA patients. However, more research will be needed to determine the best time to undergo pouch surgery and ileostomy closure in TCA patients.https://www.mdpi.com/2227-9067/9/1/101hirschsprung diseaseTotal Colonic AganglionosisJ-pouch reconstructionrestora-tive proctocolectomy
spellingShingle Konrad Reinshagen
Gunter Burmester
Johanna Hagens
Thomas Franz Krebs
Christian Tomuschat
Colectomy Followed by J-Pouch Reconstruction to Correct Total Colonic Aganglionosis
Children
hirschsprung disease
Total Colonic Aganglionosis
J-pouch reconstruction
restora-tive proctocolectomy
title Colectomy Followed by J-Pouch Reconstruction to Correct Total Colonic Aganglionosis
title_full Colectomy Followed by J-Pouch Reconstruction to Correct Total Colonic Aganglionosis
title_fullStr Colectomy Followed by J-Pouch Reconstruction to Correct Total Colonic Aganglionosis
title_full_unstemmed Colectomy Followed by J-Pouch Reconstruction to Correct Total Colonic Aganglionosis
title_short Colectomy Followed by J-Pouch Reconstruction to Correct Total Colonic Aganglionosis
title_sort colectomy followed by j pouch reconstruction to correct total colonic aganglionosis
topic hirschsprung disease
Total Colonic Aganglionosis
J-pouch reconstruction
restora-tive proctocolectomy
url https://www.mdpi.com/2227-9067/9/1/101
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AT johannahagens colectomyfollowedbyjpouchreconstructiontocorrecttotalcolonicaganglionosis
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