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Case Report

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

Advances in Pediatric Surgery 2018;24(2):86-93.
Published online: November 26, 2018

1Department of Pediatric Surgery, Seoul National University College of Medicine, Seoul, Korea.

2Department of Surgery, Korea University Anam Hospital, Seoul, Korea.

3Department of Pediatric Surgery, Pusan National University Yangsan Hospital, Yangsan, Korea.

4Department of Surgery, Seoul National University College of Medicine, Seoul, Korea.

5Department of Surgery, Chung-Ang University Hospital, Seoul, Korea.

Correspondence to Hyun-Young Kim. Department of Pediatric Surgery, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul 03080, Korea. spkhy02@snu.ac.kr
• Received: October 16, 2018   • Revised: October 27, 2018   • Accepted: October 29, 2018

Copyright © 2018 Korean Association of Pediatric Surgeons

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (https://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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  • Colon polyps in children
    A. L. Ionov, M. V. Pichugina, A. V. Myzin, V. A. Luka, T. D. Kostomarova, Ya. P. Sulavko
    Koloproktologia.2022; 21(2): 64.     CrossRef

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Long-Term Outcome of Patients Undergoing Total Proctocolectomy with Ileal Pouch-Anal Anastomosis in Childhood
Adv Pediatr Surg. 2018;24(2):86-93.   Published online November 26, 2018
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Long-Term Outcome of Patients Undergoing Total Proctocolectomy with Ileal Pouch-Anal Anastomosis in Childhood
Adv Pediatr Surg. 2018;24(2):86-93.   Published online November 26, 2018
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Long-Term Outcome of Patients Undergoing Total Proctocolectomy with Ileal Pouch-Anal Anastomosis in Childhood
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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.
Long-Term Outcome of Patients Undergoing Total Proctocolectomy with Ileal Pouch-Anal Anastomosis in Childhood

Demographics and operation-related data

Variables Total FAP UC NFNU p-value
No. of patients 25 (100.0) 9 (36.0) 9 (36.0) 7 (28.0)
Male 16 (64.0) 5 (55.6) 6 (66.7) 5 (71.4) 0.871
Age (yr)
at diagnosis 11.6 (0.2–18.7) 16.6 (13.6–18.7) 11.1 (5.1–13.7) 5.8 (0.2–15.0) 0.001
at T-IPAA 14.1 (0.7–18.9) 17.8 (15.1–18.9) 14.2 (12.6–16.0) 9.3 (0.7–15.4) 0.001
Weight (kg) at T-IPAA 42.6 (8.0–70.1) 55.4 (36.7–67.1) 41.9 (22.5–70.1) 27.2 (8.0–43.0) 0.103
Preoperative medication 10 (40.0) 1 (11.1) 9 (100.0) 0 0.001
Laparoscopic surgery 3 (12.0) 2 (22.2) 0 1 (14.3) 0.156
IPAA anastomosis method 0.959
Hand-sewing 13 (52.0) 4 (44.4) 5 (55.6) 4 (57.1%) 0.655
Using stapler 12 (48.0) 5 (55.6) 4 (44.4) 3 (42.9) 0.712
Ileostomy formation 22 (88.0) 7 (77.8) 8 (88.9) 7 (100.0) 0.411
Operation time (min) 243 (145–415) 224 (150–390) 202 (145–340) 301.8 (155–415) 0.075
Postoperative hospital stays (day) 19.3 (7–56) 11 (7–23) 24 (10–56) 15.0 (9–46) 0.049
Follow-up (yr) 9.8 (2.1–25.9) 8.1 (2.1–17) 13.8 (6.3–25.9) 6.9 (5.4–6.8) 0.615

Values are presented as number of patients (%) or median (range).

FAP, familial adenomatous polyposis; UC, ulcerative colitis; NFNU, non-familial adenomatous polyposis/non-ulcerative colitis disease; IPAA, ileal pouch-anal anastomosis.

Complications

Variables Total FAP UC NFNU p-value
No. of patients 25 (100.0) 9 (36.0) 9 (36.0) 7 (28.0)
Early 6 (24.0) 2 (22.2) 4 (44.4) 0 0.128
Fluid collection 1 (4.0) 0 1 (11.1) 0 0.435
Wound complication 1 (4.0) 0 1 (11.1) 0 0.435
Ileus 4 (16.0) 2 (22.2) 2 (22.2) 0 0.384
Internal herniation 1 (4.0) 0 1 (11.1) 0 0.411
Late 16 (64.0) 8 (88.9) 4 (44.4) 4 (57.1) 0.143
Wound complication 3 (12.0) 2 (22.2) 0 1 (14.3) 0.309
Perianal fistula 3 (12.0) 0 1 (11.1) 2 (28.6) 0.260
Rectovaginal fistula 2 (8.0) 0 2 (22.2) 0 0.435
Anal stricture 2 (8.0) 0 2 (22.2) 0 0.395
Ileus 8 (32.0) 5 (55.5) 2 (22.2) 1 (14.3) 0.105
Pouchitis 3 (12.0) 1 (11.1) 2 (22.2) 0 0.175

Values are presented as number of patients (%).

FAP; familial adenomatous polyposis, UC; ulcerative colitis; NFNU, non-familial adenomatous polyposis/non-ulcerative colitis disease.

Evaluation of bowel functions by applying scoring methods

Variables Total FAP UC NFNU p-value
No. of patients 21 (100.0) 8 (38.1) 7 (33.3) 6 (28.6)
Krickenbeck continence score
Voluntary bowel movement 20 (95.2) 7 (87.5) 7 (100.0) 6 (100.0) 0.287
Soiling (no) 10 (47.6) 5 (62.5) 5 (71.4) 0 0.047
Grade 1 8 (38.1) 3 (37.5) 1 (14.3) 4 (66.7) 0.394
Grade 2 1 (4.8) 0 0 1 (16.7) 0.287
Grade 3 2 (9.5) 0 1 (14.3) 1 (16.7) 0.287
Constipation (no) 1 (4.8) 0 1 (14.3) 0 0.368
Grade 1 18 (85.7) 7 (87.5) 6 (85.7) 5 (83.3) 0.977
Grade 2 2 (9.5) 1 (12.5) 0 1 (16.7) 0.572
Grade 3 0 0 0 0 1.00
CCI score 4.2 3.9 2.3 5.2 0.409

FAP, familial adenomatous polyposis; UC, ulcerative colitis; NFNU, non-familial adenomatous polyposis/non-ulcerative colitis disease; CCI, Cleveland Clinic Incontinence.

Clinical features of NFNU patients

Case Sex Diagnosis At IPAA operation Cause of total proctocolectomy with IPAA No. of operations before IPAA
Age (yr) Weight (kg)
1 M Hirschsprung's disease (RSA) 13 29.8 Septic shock with total colonic necrosis 3
2 M Intestinal neuronal dysplasia, type B 12.7 36.9 Persistent colonic dilatation and fecal incontinence 7
3 M Hirschsprung's disease (TCA) 3.66 13.9 Duhamel anastomosis failure 3
4 M Hirschsprung's disease (TCA) 0.72 8 Duhamel anastomosis failure 1
5 F Intestinal neuronal dysplasia, type B 13.7 40 Persistent colonic dilatation and fecal incontinence 9
6 M IA (RUF), Visceral neuropathy 15.4 43 Persistent colonic dilatation and fecal incontinence 4
7 F IA (RVF), Hypoganglinosis with immature ganglion cell 6.5 18.6 Persistent colonic dilatation and fecal incontinence 4

NFNU, non-familial adenomatous polyposis/non-ulcerative colitis disease; IPAA, ileal pouch anal anastomosis; RSA, rectosigmoid aganglinosis; TCA, total colonic aganglionosis; IA, imperforate anus; RUF, recto-urethral fistula; RVF, recto-vaginal fistula.

Evaluation of quality of life by applying scoring system

Variables Total FAP UC NFNU p-value
SF-36 75 (61–92) 74 (61–92) 62 (52–82) 78 (68–87) 0.034
GIQLI 116 (73–133) 111 (91–125) 89 (73–122) 125 (113–133) 0.004

Values are presented as median (range).

FAP, familial adenomatous polyposis; UC, ulcerative colitis; NFNU, non-familial adenomatous polyposis/non-ulcerative colitis disease; SF-36, Short-Form 36 Health Survey Questionnaire; GIQLI, Gastrointestinal Quality of Life Index.

Table 1 Demographics and operation-related data

Values are presented as number of patients (%) or median (range).

FAP, familial adenomatous polyposis; UC, ulcerative colitis; NFNU, non-familial adenomatous polyposis/non-ulcerative colitis disease; IPAA, ileal pouch-anal anastomosis.

Table 2 Complications

Values are presented as number of patients (%).

FAP; familial adenomatous polyposis, UC; ulcerative colitis; NFNU, non-familial adenomatous polyposis/non-ulcerative colitis disease.

Table 3 Evaluation of bowel functions by applying scoring methods

FAP, familial adenomatous polyposis; UC, ulcerative colitis; NFNU, non-familial adenomatous polyposis/non-ulcerative colitis disease; CCI, Cleveland Clinic Incontinence.

Table 4 Clinical features of NFNU patients

NFNU, non-familial adenomatous polyposis/non-ulcerative colitis disease; IPAA, ileal pouch anal anastomosis; RSA, rectosigmoid aganglinosis; TCA, total colonic aganglionosis; IA, imperforate anus; RUF, recto-urethral fistula; RVF, recto-vaginal fistula.

Table 5 Evaluation of quality of life by applying scoring system

Values are presented as median (range).

FAP, familial adenomatous polyposis; UC, ulcerative colitis; NFNU, non-familial adenomatous polyposis/non-ulcerative colitis disease; SF-36, Short-Form 36 Health Survey Questionnaire; GIQLI, Gastrointestinal Quality of Life Index.