Adv Pediatr Surg.  2018 Dec;24(2):86-94. 10.13029/aps.2018.24.2.86.

Long-Term Outcome of Patients Undergoing Total Proctocolectomy with Ileal Pouch-Anal Anastomosis in Childhood

• Sim YJ
• Oh C
• Youn JK
• Kim SH
• Park KJ
• Jeong SY
• Jung SE
• Park KW
• Kim HY

• KMID: 2429805
• DOI: http://doi.org/10.13029/aps.2018.24.2.86

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 ≤18 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

MeSH Terms

Adenomatous Polyposis Coli
Anus, Imperforate
Classification
Colitis, Ulcerative
Colonic Pouches
Constipation
Follow-Up Studies
Health Surveys
Hirschsprung Disease
Humans
Medical Records
Neurons
Pediatrics
Quality of Life
Retrospective Studies
Soil
Soil

Figure

• 

  Fig. 1 Detailed results of quality of life evaluation by questionnaires. SF-36, Short-Form 36 Health Survey Questionnaire; GIQLI, Gastrointestinal Quality of Life Index; FAP, familial adenomatous polyposis; UC, ulcerative colitis; NFNU, non-familial adenomatous polyposis/non-ulcerative colitis disease.

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