Gastroenterology

Gastroenterology

Volume 119, Issue 6, December 2000, Pages 1454-1460
Gastroenterology

Alimentary Tract
Ileorectal anastomosis is appropriate for a subset of patients with familial adenomatous polyposis☆

https://doi.org/10.1053/gast.2000.20180Get rights and content

Abstract

Background & Aims: This study reevaluates the risk of rectal cancer and the frequency of subsequent proctectomy for nonmalignant causes in patients with familial adenomatous polyposis (FAP) who have undergone colectomy with ileorectal anastomosis (IRA). Potential risk factors for rectal cancer in this setting are also examined, and recommendations for the choice of surgical procedure are made. Methods: The national polyposis registries in Denmark, Finland, The Netherlands, and Sweden included 659 patients undergoing surgery with IRA in 1940–1997. Kaplan–Meier analysis and Cox regression analysis were performed to evaluate cumulative risk, survival, and predictive risk factors. Results: Rectal carcinoma was diagnosed in 47 patients, with a cumulative 40-year risk of 0.32. The cumulative risk according to chronologic age was 0.30 at age 60, and higher in patients undergoing surgery above age 25 (P = 0.0016). Chronologic age was the only independent risk factor (P = 0.0016). The cumulative 5-year survival rate after rectal carcinoma was 0.60. The apc mutation was known in 167 patients, of whom 7 had rectal cancer. The cumulative 40-year risk of secondary proctectomy was 0.70, and higher in patients with a mutation in codon 1250–1500 than outside this region (P = 0.005). However, all 7 rectal cancers were found in the latter group. None of the 18 patients with attenuated FAP (mutation in codon 0–200 or >1500) had a secondary proctectomy. Conclusions: IRA is recommended in (1) young patients with few rectal adenomas and a family history of a mild phenotype and (2) patients with attenuated FAP (a mutation in codon 0–200 or >1500), provided there is acceptance of life-long rectal surveillance. Patients with many rectal polyps and/or a family history of severe polyposis should be offered a restorative proctocolectomy with an ileal pouch–anal anastomosis.

GASTROENTEROLOGY 2000;119:1454-1460

Section snippets

Materials and methods

The national polyposis registries of Denmark, Finland, Holland, and Sweden were searched for patients undergoing surgery from January 1, 1940, to December 31, 1997, with a colectomy and an ileorectal anastomosis as the primary procedure. Patients in regular followup with at least one annual proctoscopy were included in the study.

The registered data included date of birth, sex, proband/call-up case, familial/isolated case, location of mutation in the apc gene, date of IRA, colon cancer at IRA,

Results

The study consisted of 659 patients, 349 men (53%) and 310 women, from Denmark (126), Finland (105), Holland (240), and Sweden (188). The median age at IRA was 26 years (range, 7–75 years). Of them, 193 were probands (29%) and 418 (63%) were call-up patients, and the status proband/call-up case was unknown in 48 patients. Seventy-five patients (11%) were isolated cases with no family history of FAP. The specific apc gene mutation was known in 167 of 366 (46%) Dutch and Danish patients (Figure

Discussion

The choice of surgical method in FAP has remained controversial over the past 30 years, primarily because of different results on the long-term risk of secondary rectal cancer. The first surgical method was total proctocolectomy with ileostomy, which was gradually replaced by colectomy and IRA during the 1940s. IRA is a one-step procedure with almost no mortality, low postoperative morbidity, and good functional results, but the patients are left with their rectum in situ, and the risk of

Acknowledgements

The authors gratefully acknowledge the work of Dr. Carli Tops.

References (39)

  • T Iwama et al.

    Factors affecting the risk of rectal cancer following rectum-preserving surgery in patients with familial adenomatous polyposis

    Dis Colon Rectum

    (1994)
  • KP Nugent et al.

    Rectal cancer risk in older patients with familial adenomatous polyposis and an ileorectal anastomosis: a cause for concern

    Br J Surg

    (1992)
  • P Duijvendijk et al.

    Cumulative risk of developing polyps or malignancy at the ileal pouch–anal anastomosis in patients with familial adenomatous polyposis

    J Gastrointest Surg

    (1999)
  • WJ Ambroze et al.

    Familial adenomatous polyposis: results following ileal pouch–anal anastomosis and ileorectostomy

    Dis Colon Rectum

    (1992)
  • MV Madden et al.

    Comparison of morbidity and function after colectomy with ileorectal anastomosis or restorative proctocolectomy for familial adenomatous polyposis

    Br J Surg

    (1991)
  • HJR Bussey

    Familial polyposis coli

    (1975)
  • CG Moertel et al.

    Management of multiple polyposis of the large bowel

    Cancer

    (1971)
  • MA Bess et al.

    Rectal cancer following colectomy for polyposis

    Arch Surg

    (1980)
  • RG Sarre et al.

    Colectomy with ileorectal anastomosis for familial adenomatous polyposis: the risk of rectal cancer

    Surgery

    (1987)

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    ☆

    Address requests for reprints to: Steffen Bülow, M.D., D.M.Sc., The Danish Polyposis Register, Department of Surgical Gastroenterology 435, Hvidovre University Hospital, DK-2650 Hvidovre, Copenhagen, Denmark. e-mail: [email protected]; fax: (45) 3632-3200.

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    Copyright © 2000 American Gastroenterological Association. Published by Elsevier Inc. All rights reserved.