Juvenile Polyposis Syndrome

Overview

Journal J Indian Assoc Pediatr Surg

Specialty Pediatrics

Date 2009 Dec 17

PMID 20011494

Citations 2

Authors

Vijai D Upadhyaya

A N Gangopadhyaya

S P Sharma

S C Gopal

D K Gupta

Vijayendra Kumar

Affiliations

Soon will be listed here.

Abstract

Aim: Report of a series of 12 cases of juvenile polyposis coli.

Methods: The study period was from 1995 to 2005. All the patients were treated by total colectomy with rectal mucosectomy and endorectal ileoanal pullthrough with or without ileal pouch formation. Covering ileostomy was avoided in all the cases. Time taken for the surgery, postoperative complications and continence were documented.

Results: The mean operating time was 4.2 h (range: 4-5 h). The mean duration of hospital stay was 16.3 days (range: 15-18 days). The most common postoperative complication was pouchitis and perianal excoriation. Initially, all the patients were passing stools at an interval of 2 h, and after 3 weeks, the frequency has reduced to 6-8 stools per day. In the follow-up after 3 months, the frequency was 3-5 per day with minimal soiling.

Conclusions: Single-stage total colectomy with rectal mucosectomy and endorectal ileoanal pull-through without covering ileostomy and pouch formation is a safe and definitive treatment for juvenile polyposis coli if the patient selection is appropriate.

Citing Articles

Clinical and functional characterisation of the SMAD4 germline variant c.1035C > A in a family with juvenile polyposis syndrome by whole-exome sequencing.

Dang Y, Xu Q, Liu X, Wang L, Lin C Med Mol Morphol. 2022; 56(1):78-83.

PMID: 35851413 DOI: 10.1007/s00795-022-00333-w.

Juvenile polyposis syndrome: An unusual case report of anemia and gastrointestinal bleeding in young infant.

Hsiao Y, Wei C, Chang S, Chang L, Fu Y, Lee H Medicine (Baltimore). 2016; 95(37):e4550.

PMID: 27631205 PMC: 5402548. DOI: 10.1097/MD.0000000000004550.

References

Coran A . A personal experience with 100 consecutive total colectomies and straight ileoanal endorectal pull-throughs for benign disease of the colon and rectum in children and adults. Ann Surg. 1990; 212(3):242-7; discussion 247-8. PMC: 1358148. DOI: 10.1097/00000658-199009000-00002. View

Dawson I . Hamartomas in the alimentary tract. Gut. 1969; 10(8):691-4. PMC: 1552916. DOI: 10.1136/gut.10.8.691. View

Bussey H, Veale A, MORSON B . Genetics of gastrointestinal polyposis. Gastroenterology. 1978; 74(6):1325-30. View

Yoshida T, Haraguchi Y, Tanaka A, Higa A, Daimon Y, Mizuta Y . A case of generalized juvenile gastrointestinal polyposis associated with gastric carcinoma. Endoscopy. 1988; 20(1):33-5. DOI: 10.1055/s-2007-1018122. View

Haggitt R, Reid B . Hereditary gastrointestinal polyposis syndromes. Am J Surg Pathol. 1986; 10(12):871-87. DOI: 10.1097/00000478-198612000-00006. View

Fonkalsrud E, Thakur A, Beanes S . Ileoanal pouch procedures in children. J Pediatr Surg. 2001; 36(11):1689-92. DOI: 10.1053/jpsu.2001.27961. View

Wirtzfeld D, Petrelli N, Rodriguez-Bigas M . Hamartomatous polyposis syndromes: molecular genetics, neoplastic risk, and surveillance recommendations. Ann Surg Oncol. 2001; 8(4):319-27. DOI: 10.1007/s10434-001-0319-7. View

Brosens L, van Hattem W, Jansen M, de Leng W, Giardiello F, Offerhaus G . Gastrointestinal polyposis syndromes. Curr Mol Med. 2007; 7(1):29-46. DOI: 10.2174/156652407779940404. View

Grigioni W, Alampi G, Martinelli G, PICCALUGA A . Atypical juvenile polyposis. Histopathology. 1981; 5(4):361-76. DOI: 10.1111/j.1365-2559.1981.tb01798.x. View

10.

Brosens L, van Hattem A, Hylind L, Iacobuzio-Donahue C, Romans K, Axilbund J . Risk of colorectal cancer in juvenile polyposis. Gut. 2007; 56(7):965-7. PMC: 1994351. DOI: 10.1136/gut.2006.116913. View

11.

Jones M, Hebert J, Trainer T . Juvenile polyp with intramucosal carcinoma. Arch Pathol Lab Med. 1987; 111(2):200-1. View

12.

Castro A . Adenomatous polyps of the rectum and colon in children. Clin Proc Child Hosp Dist Columbia. 1955; 11(2):29-32. View

13.

Durno C . Colonic polyps in children and adolescents. Can J Gastroenterol. 2007; 21(4):233-9. PMC: 2657698. DOI: 10.1155/2007/401674. View

14.

Sturniolo G, Montino M, DallIgna F, DInca R, Messineo A, Cecchetto A . Familial juvenile polyposis coli: results of endoscopic treatment and surveillance in two sisters. Gastrointest Endosc. 1993; 39(4):561-5. DOI: 10.1016/s0016-5107(93)70174-1. View

15.

Gathright Jr J, Cofer Jr T . Familial incidence of juvenile polyposis coli. Surg Gynecol Obstet. 1974; 138(2):185-8. View

16.

Coburn M, Pricolo V, DELUCA F, Bland K . Malignant potential in intestinal juvenile polyposis syndromes. Ann Surg Oncol. 1995; 2(5):386-91. DOI: 10.1007/BF02306370. View

17.

Howe J, Mitros F, Summers R . The risk of gastrointestinal carcinoma in familial juvenile polyposis. Ann Surg Oncol. 1998; 5(8):751-6. DOI: 10.1007/BF02303487. View

18.

Oncel M, Church J, Remzi F, Fazio V . Colonic surgery in patients with juvenile polyposis syndrome: a case series. Dis Colon Rectum. 2005; 48(1):49-55. DOI: 10.1007/s10350-004-0749-y. View

19.

Jass J, Williams C, Bussey H, MORSON B . Juvenile polyposis--a precancerous condition. Histopathology. 1988; 13(6):619-30. DOI: 10.1111/j.1365-2559.1988.tb02093.x. View

20.

Koivusalo A, Pakarinen M, Rintala R . Surgical complications in relation to functional outcomes after ileoanal anastomosis in pediatric patients with ulcerative colitis. J Pediatr Surg. 2007; 42(2):290-5. DOI: 10.1016/j.jpedsurg.2006.10.001. View