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.
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.
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).
T-IPAA could be safely applied not only for FAP and UC but also for other diseases in selective cases, with caution.
Citations
Ulcerative colitis, one of the inflammatory bowel disease, is primarily managed medically with combinations of 5-ASA and steroids. However, this chronic disease requires surgical management if symptoms persist or complications develop despite medical management. The clinical course, the indications and outcome for surgical management of pediatric ulcerative colitis patients were studied from medical records retrospectively. Twenty-one patients under the age of 15 who were endoscopically diagnosed with ulcerative colitis at the Seoul National University Children's Hospital between January, 1988 and January, 2003 were subjected to the study. Mean follow up period was 3 years and 10 months. The mean age was 10.3 years old. All patients received medical management primarily after diagnosis and 8 patients (38%) eventually required surgical management. Of 13 patients who received medical management only, 7 patients (53%) showed remission, 4 patients are still on medical management, and 2 patients expired due to congenital immune deficiency and hepatic failure owing to sclerosing cholangitis respectively. In 8 patients who received surgical management, 1 patient underwent surgery due to sigmoid colon perforation and 7 patients due to intractability of medical management. The perforated case received colon segmental resection and the other 7 patients recieved total protocolectomy with ileal pouch-anal anastomosis. One patient expired postoperatively due to pneumonia and sepsis. One patient is still on medical management because of mild persistent hematochezia after surgery. The other operated patients are showing good prognosis without any management. Pediatric ulcerative colitis patients can be surgically managed if the patient is intractable to medical management or if complications such as perforation is present. Total protocolectomy & ileal pouch-anal anastomosis is thought to be the adequate surgical method.
First
Prev
