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

• Published in: Children (Basel) Vol. 9; no. 1; p. 101
• Main Authors: Reinshagen, Konrad, Burmester, Gunter, Hagens, Johanna, Krebs, Thomas Franz, Tomuschat, Christian
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 12.01.2022; MDPI
• Subjects: Abdomen; Age; Biopsy; Constipation; Electrolytes; Failure to thrive; Fecal incontinence; hirschsprung disease; Hospitals; J-pouch reconstruction; Mortality; Ostomy; Patients; Pediatrics; Quality of life; restora-tive proctocolectomy; Sodium; Surgery; Total Colonic Aganglionosis; Weaning; hirschsprung disease; restora-tive proctocolectomy; J-pouch reconstruction; Total Colonic Aganglionosis
• ISSN: 2227-9067; 2227-9067
• DOI: 10.3390/children9010101

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. 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. 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.