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Deciding on an IRA vs. IPAA for FAP

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Difficult Decisions in Colorectal Surgery

Part of the book series: Difficult Decisions in Surgery: An Evidence-Based Approach ((DDSURGERY))

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Abstract

Total abdominal colectomy or proctocolectomy is eventually required for nearly all patients with familial adenomatous polyposis (FAP). These operations are most often performed to prevent or treat colon or rectal cancer. Total abdominal colectomy with ileorectal anastomosis (TAC-IRA) is generally recommended for patients who have a low risk of cancer progression in the retained rectum. Less than 1000 colorectal adenomas, and less than 20 rectal adenomas, and no known diagnosis of high-grade dysplasia (HGD) or cancer, are typical scenarios where TAC-IRA is advised. Alternatively, for patients with >1000 colorectal adenomas, or > 20 rectal adenomas, or a diagnosis of HGD or colorectal cancer, proctocolectomy with ileal pouch anal anastomosis (IPAA) or end ileostomy creation is recommended. Operative risk, postoperative complication risk, functional outcomes, the risk of postoperative desmoid tumor formation, and for women, concerns about fecundity, also figure in to the decision on which operation is best for each individual with FAP.

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References

  1. Aretz S, Uhlhaas S, Caspari R, et al. Frequency and parental origin of de novo APC mutations in familial adenomatous polyposis. Eur J Hum Genet. 2004;12(1):52–8. https://doi.org/10.1038/sj.ejhg.5201088.

    Article  CAS  PubMed  Google Scholar 

  2. Olkinuora AP, Peltomäki PT, Aaltonen LA, Rajamäki K. From APC to the genetics of hereditary and familial colon cancer syndromes. Hum Mol Genet. 2021;30(R2):R206–24. https://doi.org/10.1093/hmg/ddab208.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Björk J, Åkerbrant H, Iselius L, et al. Periampullary adenomas and adenocarcinomas in familial adenomatous polyposis: cumulative risks and APC gene mutations. Gastroenterology. 2001;121(5):1127–35. https://doi.org/10.1053/gast.2001.28707.

    Article  PubMed  Google Scholar 

  4. Herzig D, Hardimann K, Weiser M, et al. The American Society of Colon and Rectal Surgeons clinical practice guidelines for the management of inherited polyposis syndromes. Dis Colon Rectum. 2017;60(9):881–94. https://doi.org/10.1097/DCR.0000000000000912.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Bülow S, Bülow C, Vasen H, Järvinen H, Björk J, Christensen IJ. Colectomy and ileorectal anastomosis is still an option for selected patients with familial adenomatous polyposis. Dis Colon Rectum. 2008;51(9):1318–23. https://doi.org/10.1007/s10350-008-9307-3.

    Article  PubMed  Google Scholar 

  6. Koskenvuo L, Mustonen H, Renkonen-Sinisalo L, Järvinen HJ, Lepistö A. Comparison of proctocolectomy and ileal pouch-anal anastomosis to colectomy and ileorectal anastomosis in familial adenomatous polyposis. Familial Cancer. 2015;14(2):221–7. https://doi.org/10.1007/s10689-014-9773-9.

    Article  CAS  PubMed  Google Scholar 

  7. Soravia C, Klein L, Berk T, O’Connor B, Cohen Z, McLeod RS. Comparison of ileal pouch-anal anastomosis and ileorectal anastomosis in patients with familial adenomatous polyposis. Dis Colon Rectum. 1999;42(8):1028–33.

    Article  CAS  PubMed  Google Scholar 

  8. Campos FG, Perez RO, Imperiale AR, Seid VE, Nahas SC, Cecconello I. Surgical treatment of familial adenomatous polyposis: Ileoretal anastomosis or restorative proctolectomy? Arq Gastroenterol. 2009;46(4):294–9. https://doi.org/10.1590/S0004-28032009000400009.

    Article  PubMed  Google Scholar 

  9. Ambroze WL, Dozois RR, Pemberton JH, Beart RW, Ilstrup DM. Familial adenomatous polyposis: results following ileal pouch-anal anastomosis and ileorectostomy. Dis Colon Rectum. 1992;35(1):12–5. https://doi.org/10.1007/BF02053332.

    Article  PubMed  Google Scholar 

  10. Tonelli F, Valanzano R, Monaci I, Mazzoni P, Anastasi A, Ficari F. Restorative proctocolectomy or rectum-preserving surgery in patients with familial adenomatous polyposis: results of a prospective study. World J Surg. 1997;21(6):653–9. https://doi.org/10.1007/s002689900289.

    Article  CAS  PubMed  Google Scholar 

  11. Van Duijvendijk P, Slors JFM, Taat CW, et al. Quality of life after total colectomy with ileorectal anastomosis or proctocolectomy and ileal pouch-anal anastomosis for familial adenomatous polyposis. Br J Surg. 2000;87(5):590–6. https://doi.org/10.1046/j.1365-2168.2000.01442.x.

    Article  PubMed  Google Scholar 

  12. Björk J, Åkerbrant H, Iselius L, et al. Outcome of primary and secondary ileal pouch-anal anastomosis and ileorectal anastomosis in patients with familial adenomatous polyposis. Dis Colon Rectum. 2001;44(7):984–92. https://doi.org/10.1007/BF02235487.

    Article  PubMed  Google Scholar 

  13. Konishi T, Ishida H, Ueno H, et al. Feasibility of laparoscopic total proctocolectomy with ileal pouch–anal anastomosis and total colectomy with ileorectal anastomosis for familial adenomatous polyposis: results of a nationwide multicenter study. Int J Clin Oncol. 2016;21(5):953–61. https://doi.org/10.1007/s10147-016-0977-x.

    Article  PubMed  Google Scholar 

  14. Mozafar M, Shateri K, Tabatabaey A, Lotfollahzadeh S, Atqiaee K. Familial adenomatous polyposis: Ileo-anal pouch versus ileo-rectal anastomosis. Gastroenterol Hepatol Bed Bench. 2014;7(4):206–10.

    PubMed  PubMed Central  Google Scholar 

  15. Hassan I, Chua HK, Wolff BG, et al. Quality of life after ileal pouch-anal anastomosis and ileorectal anastomosis in patients with familial adenomatous polyposis. Dis Colon Rectum. 2005;48(11):2032–7. https://doi.org/10.1007/s10350-005-0169-7.

    Article  PubMed  Google Scholar 

  16. Ko CY, Rusin LC, Schoetz DJ, et al. Does better functional result equate with better quality of life? Implications for surgical treatment in familial adenomatous polyposis. Dis Colon Rectum. 2000;43(6):829–37. https://doi.org/10.1007/bf02238022.

    Article  CAS  PubMed  Google Scholar 

  17. Madden MV, Neale KF, Nicholls RJ, et al. Comparison of morbidity and function after colectomy with ileorectal anastomosis or restorative proctocolectomy for familial adenomatous polyposis. Br J Surg. 2005;78(7):789–92.

    Article  Google Scholar 

  18. Aziz O, Athanasiou T, Fazio VW, et al. Meta-analysis of observational studies of ileorectal versus ileal pouch-anal anastomosis for familial adenomatous polyposis. Br J Surg. 2006;93(4):407–17. https://doi.org/10.1002/bjs.5276.

    Article  CAS  PubMed  Google Scholar 

  19. Hartley JE, Church JM, Gupta S, McGannon E, Fazio VW, Phillips RKS. Significance of incidental desmoids identified during surgery for familial adenomatous polyposis. Dis Colon Rectum. 2004;47(3):334–40. https://doi.org/10.1007/s10350-003-0063-0.

    Article  CAS  Google Scholar 

  20. Nieuwenhuis MH, Lefevre JH, Bülow S, et al. Family history, surgery, and APC mutation are risk factors for desmoid tumors in familial adenomatous polyposis: an international cohort study. Dis Colon Rectum. 2011;54(10):1229–34. https://doi.org/10.1097/DCR.0b013e318227e4e8.

    Article  PubMed  Google Scholar 

  21. Sommovilla J, Liska D, Jia X, et al. Ileal Pouch anal anastomosis is more “Desmoidogenic” than ileorectal anastomosis in familial adenomatous polyposis. Dis Colon Rectum. 2021; Publish Ah:1351–1361. https://doi.org/10.1097/dcr.0000000000002172.

  22. Burgess A, Xhaja X, Church J. Does intra-abdominal desmoid disease affect patients with an ileal pouch differently than those with an ileorectal anastomosis? Dis Colon Rectum. 2011;54(11):1388–91. https://doi.org/10.1097/DCR.0b013e31822f8d91.

    Article  CAS  PubMed  Google Scholar 

  23. Vasen HFA, Möslein G, Alonso A, et al. Guidelines for the clinical management of familial adenomatous polyposis (FAP). Gut. 2008;57(5):704–13. https://doi.org/10.1136/gut.2007.136127.

    Article  CAS  PubMed  Google Scholar 

  24. Vogel J, Church JMLL. Minimally invasive pouch surgery predisposes to desmoid tumor formation in patients with familial adenomatous polyposis. Dis Colon Rectum. 2005;48:662.

    Google Scholar 

  25. Saito Y, Hinoi T, Ueno H, et al. Risk factors for the development of Desmoid tumor after colectomy in patients with familial adenomatous polyposis: multicenter retrospective cohort study in Japan. Ann Surg Oncol. 2016;23:559–65. https://doi.org/10.1245/s10434-016-5380-3.

    Article  PubMed  Google Scholar 

  26. Walter T, Zhenzhen Wang C, Guillaud O, et al. Management of desmoid tumours: a large national database of familial adenomatous patients shows a link to colectomy modalities and low efficacy of medical treatments. United Eur Gastroenterol J. 2017;5(5):735–41. https://doi.org/10.1177/2050640616678150.

    Article  CAS  Google Scholar 

  27. Da Luz MA, Church JM, Burke CA. The evolution of prophylactic colorectal surgery for familial adenomatous polyposis. Dis Colon Rectum. 2009;52(8):1481–6. https://doi.org/10.1007/DCR.0b013e3181ab58fb.

    Article  Google Scholar 

  28. Xie M, Chen Y, Wei W, et al. Does ileoanal pouch surgery increase the risk of desmoid in patients with familial adenomatous polyposis? Int J Color Dis. 2020;35(8):1599–605. https://doi.org/10.1007/s00384-020-03578-y.

    Article  Google Scholar 

  29. Fábio GC. Surgical treatment of familial adenomatous polyposis: dilemmas and current recommendations. World J Gastroenterol. 2014;20(44):16620–9. https://doi.org/10.3748/wjg.v20.i44.16620.

    Article  Google Scholar 

  30. Church J, Burke C, McGannon E, Pastean O, Clark B. Risk of rectal cancer in patients after colectomy and ileorectal anastomosis for familial adenomatous polyposis: a function of available surgical options. Dis Colon Rectum. 2003;46(9):1175–81. https://doi.org/10.1007/s10350-004-6710-2.

    Article  PubMed  Google Scholar 

  31. Sinha A, Tekkis PP, Rashid S, Phillips RKS, Clark SK. Risk factors for secondary proctectomy in patients with familial adenomatous polyposis. Br J Surg. 2010;97(11):1710–5. https://doi.org/10.1002/bjs.7202.

    Article  CAS  PubMed  Google Scholar 

  32. Bülow C, Vasen H, Järvinen H, Björk J, Bisgaard ML, Bülow S. Ileorectal anastomosis is appropriate for a subset of patients with familial adenomatous polyposis. Gastroenterology. 2000;119(6):1454–60. https://doi.org/10.1053/gast.2000.20180.

    Article  PubMed  Google Scholar 

  33. Koskenvuo L, Renkonen-Sinisalo L, Järvinen HJ, Lepistö A. Risk of cancer and secondary proctectomy after colectomy and ileorectal anastomosis in familial adenomatous polyposis. Int J Color Dis. 2014;29(2):225–30. https://doi.org/10.1007/s00384-013-1796-4.

    Article  CAS  Google Scholar 

  34. Debinski HS, Love S, Spigelman AD, Phillips RKS. Colorectal polyp counts and cancer risk in familial adenomatous polyposis. Gastroenterology. 1996;110(4):1028–30. https://doi.org/10.1053/gast.1996.v110.pm8612989.

    Article  CAS  PubMed  Google Scholar 

  35. Wu JS, Paul P, McGannon EA, Church JM. APC genotype, polyp number, and surgical options in familial adenomatous polyposis. Ann Surg. 1998;227(1):57–62. https://doi.org/10.1097/00000658-199801000-00009.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  36. Church J, Burke C, McGannon E, Pastean O, Clark B, Cohen Z. Predicting polyposis severity by proctoscopy: how reliable is it? Dis Colon Rectum. 2001;44(9):1249–52. https://doi.org/10.1007/bf02234779.

    Article  CAS  PubMed  Google Scholar 

  37. Talseth-Palmer BA. The genetic basis of colonic adenomatous polyposis syndromes. Hered Cancer Clin Pract. 2017;15(1):1–7. https://doi.org/10.1186/s13053-017-0065-x.

    Article  CAS  Google Scholar 

  38. Nieuwenhuis MH, Mathus-Vliegen LM, Slors FJ, et al. Genotype-phenotype correlations as a guide in the management of familial adenomatous polyposis. Clin Gastroenterol Hepatol. 2007;5(3):374–8. https://doi.org/10.1016/j.cgh.2006.12.014.

    Article  PubMed  Google Scholar 

  39. Church JM. Controversies in the surgery of patients with familial adenomatous polyposis and lynch syndrome. Familial Cancer. 2016;15(3):447–51. https://doi.org/10.1007/s10689-016-9886-4.

    Article  PubMed  Google Scholar 

  40. Möslein G. Surgical considerations in FAP-related pouch surgery: could we do better? Familial Cancer. 2016;15(3):457–66. https://doi.org/10.1007/s10689-016-9904-6.

    Article  PubMed  Google Scholar 

  41. Van Duijvendijk P, JFM S, Taat CW, Oosterveld P. Functional outcome after colectomy and ileorectal anastomosis compared with proctocolectomy and ileal pouch anal anastomosis in familial adenomatous polyposis. Ann Surg. 1999;230(5):648–54.

    Google Scholar 

  42. Olsen K, Juul S, Bülow S, et al. Female fecundity before and after operation for familial adenomatous polyposis. Br J Surg. 2003;90(2):227–31. https://doi.org/10.1002/bjs.4082.

    Article  CAS  PubMed  Google Scholar 

  43. Rajaratnam SG, Eglinton TW, Hider P, Fearnhead NS. Impact of ileal pouch-anal anastomosis on female fertility: meta-analysis and systematic review. Int J Color Dis. 2011;26(11):1365–74. https://doi.org/10.1007/s00384-011-1274-9.

    Article  Google Scholar 

  44. Melnitchouk N, Saadat LV, Bleday R, Goldberg JE. A decision analysis for rectal-sparing familial adenomatous polyposis: total colectomy with Ileorectal anastomosis versus proctocolectomy with IPAA. Dis Colon Rectum. 2019;62(1):27–32. https://doi.org/10.1097/DCR.0000000000001186.

    Article  PubMed  Google Scholar 

  45. Dossa F, Morris AM, Wilson AR, Baxter NN. Life after surgery: surgeon assessments of quality of life among patients with familial adenomatous polyposis. Dis Colon Rectum. 2018;61(10):1217–22. https://doi.org/10.1097/DCR.0000000000001146.

    Article  PubMed  Google Scholar 

  46. Kuruvilla K, Osler T, Hyman NH. A comparison of the quality of life of ulcerative colitis patients after IPAA vs ileostomy. Dis Colon Rectum. 2012;55(11):1131–7. https://doi.org/10.1097/DCR.0b013e3182690870.

    Article  PubMed  Google Scholar 

  47. Murphy PB, Khot Z, Vogt KN, Ott M, Dubois L. Quality of life after total proctocolectomy with ileostomy or IPAA: a systematic review. Dis Colon Rectum. 2015;58(9):899–908. https://doi.org/10.1097/DCR.0000000000000418.

    Article  PubMed  Google Scholar 

  48. Seidel S, Newman M, Sharp K. Ileoanal pouch versus ileostomy: is there a difference in quality of life? Am Surg. 2000;66(6):540–6.

    Article  CAS  PubMed  Google Scholar 

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Correspondence to Jon D. Vogel .

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Lai, S.H., Vogel, J.D. (2023). Deciding on an IRA vs. IPAA for FAP. In: Umanskiy, K., Hyman, N. (eds) Difficult Decisions in Colorectal Surgery. Difficult Decisions in Surgery: An Evidence-Based Approach. Springer, Cham. https://doi.org/10.1007/978-3-031-42303-1_37

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  • DOI: https://doi.org/10.1007/978-3-031-42303-1_37

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