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KMID : 1152420180240020086
Advances in Pediatric Surgery
2018 Volume.24 No. 2 p.86 ~ p.93
Long-Term Outcome of Patients Undergoing Total Proctocolectomy with Ileal Pouch-Anal Anastomosis in Childhood
Sim Yoon-Jung

Oh Chae-Youn
Youn Joong-Kee
Kim Soo-Hong
Park Kyu-Joo
Jeong Seung-Yong
Jung Sung-Eun
Park Kwi-Won
Kim Hyun-Young
Abstract
Purpose: Total proctocolectomy with ileal pouch-anal anastomosis (T-IPAA) in childhood is a surgical procedure mainly applied to familial adenomatous polyposis (FAP) or ulcerative colitis (UC), but it can be applied to non-FAP/non-UC disease (NFNU). Studies regarding the role of T-IPAA who underwent the operation in childhood, especially in terms of long-term gastrointestinal function, complications, and quality of life (QOL) are limited. The aim of this study was to evaluate the characteristics of patients receiving T-IPAA and to compare their bowel function outcomes and QOL.

Methods: Patients aged ¡Â18 years at the time of T-IPAA were included. Their medical records were retrospectively reviewed. Krickenbeck classification, Cleveland Clinic Incontinence (CCI) score, 36-item Short-form Health Survey Questionnaire, and Gastrointestinal Quality of Life Index were used for the evaluation of bowel function and QOL. The median follow-up period was 9.8 years.

Results: Of the 25 patients, 9 had FAP, 9 had UC, and 7 had NFNU. NFNU include 3 of Hirschsprung disease, 2 of intestinal neuronal dysplasia, and 2 of imperforate anus. The median age at T-IPAA was 17.8, 14.2, and 9.3 years for FAP, UC, and NFNU, respectively (p=0.001). Bowel function was satisfactory in terms of voluntary bowel movement (VBM), soiling, and constipation. VBM and constipation were not different between the groups, but soiling was most in NFNU (100%, p=0.047). However, QOL was best in the NFNU group in surveys (p=0.034 and 0.004, respectively).

Conclusion: T-IPAA could be safely applied not only for FAP and UC but also for other diseases in selective cases, with caution.
KEYWORD
Ileoanal pouches, Familial adenomatous polyposis, Ulcerative colitis, Pediatrics, Hirschsprung disease
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