Abstract
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.
Highlights
Complete colectomy is the only curative treatment for total colonic aganglionosis (TCA)
Utsonomiya popularised the J-Pouch reconstruction, which revolutionised pouch surgery primarily because it was easier to create while providing the obvious benefits of a pouch reservoir following colectomy [4]
Pouch repair is the standard surgery for individuals who have had a total colectomy for a variety of reasons [3]
Summary
Complete colectomy is the only curative treatment for total colonic aganglionosis (TCA). Ravitch originally described colectomy with continent repair in adult patients in 1951 utilising a straight ileoanal anastomosis [1]. Sir Alan Parks and Nichols created the S-Pouch in 1978, allowing continent reconstruction following a total colectomy [2]. Utsonomiya popularised the J-Pouch reconstruction, which revolutionised pouch surgery primarily because it was easier to create while providing the obvious benefits of a pouch reservoir following colectomy [4]. Pouch repair is the standard surgery for individuals who have had a total colectomy for a variety of reasons [3]. A few studies reported J-Pouch repair as the primary reconstructive surgery in TCA patients. More research will be needed to determine the best time to undergo pouch surgery and ileostomy closure in TCA patients
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