Optimizing the timing of colorectal surgery in patients with familial adenomatous polyposis in clinical practice

Abstract Background: Familial adenomatous polyposis (FAP) is characterized by the development of hundreds of colorectal adenomas in the second decade of life, and prophylactic colectomy is usually performed around age of 20. A common question is the appropriate timing of surgery and which endoscopic findings indicate surgery. Methods: All FAP patients known at Leiden University Medical Centre from 1985 onwards were included. The patients were then subdivided into those diagnosed before or after 2000. Patient information included age at diagnosis, colonic phenotype, age at surgery, pathological findings and the outcome of follow-up colonoscopies in whom surgery was postponed. Results: The 72 FAP patients identified consisted of 33 patients diagnosed before (group A) and 39 after (group B) 2000. The median age at diagnosis for patients with classical FAP was 18 in groups A and B. All patients diagnosed before 2000 underwent colorectal surgery versus 68% of those diagnosed >2000. The median age at surgery for classical FAP patients was 19 and 24 years in groups A and B, respectively. In patients with intact colon, the number of adenomas gradually increased over many years. Although most adenomas remained <5 mm, the proportion of 5–15 mm adenomas slowly increased. Only one patient developed a high-grade adenoma. None of the patients developed CRC. Conclusions: Surgery today in FAP is performed less often and at a more advanced age. Our experience also suggests that surgery can be safely postponed in selected patients. The most important endoscopic indication for surgery is substantial number of large adenomas of >5–10 mm.

[1]  Jeffrey S. Morris,et al.  A proposed staging system and stage-specific interventions for familial adenomatous polyposis. , 2016, Gastrointestinal endoscopy.

[2]  G. Möslein Surgical considerations in FAP-related pouch surgery: Could we do better? , 2016, Familial Cancer.

[3]  Video Comment on Ghanouni et al. , 2016, Endoscopy.

[4]  J. Dominitz,et al.  Can individuals with colonic polyposis be managed safely with colonoscopic surveillance? , 2015, Endoscopy.

[5]  T. Iwama,et al.  Endoscopic management of familial adenomatous polyposis in patients refusing colectomy , 2015, Endoscopy.

[6]  G. Möslein,et al.  Prophylactic surgery in familial adenomatous polyposis (FAP)—a single surgeon’s short- and long-term experience with hand-assisted proctocolectomy and smaller J-pouches , 2015, International Journal of Colorectal Disease.

[7]  Randall W Burt,et al.  ACG Clinical Guideline: Genetic Testing and Management of Hereditary Gastrointestinal Cancer Syndromes , 2015, The American Journal of Gastroenterology.

[8]  D. Evans,et al.  Systematic review of the impact of registration and screening on colorectal cancer incidence and mortality in familial adenomatous polyposis and Lynch syndrome , 2013, The British journal of surgery.

[9]  Elena A. Manilich,et al.  Ileal Pouch Anal Anastomosis: Analysis of Outcome and Quality of Life in 3707 Patients , 2013, Annals of surgery.

[10]  N. D. Wolf,et al.  Quality of life after restorative proctocolectomy and ileal pouch–anal anastomosis in patients with familial adenomatous polyposis: a matter of adjustment , 2011, Colorectal disease : the official journal of the Association of Coloproctology of Great Britain and Ireland.

[11]  H. Järvinen,et al.  Family History, Surgery, and APC Mutation Are Risk Factors for Desmoid Tumors in Familial Adenomatous Polyposis: An International Cohort Study , 2011, Diseases of the colon and rectum.

[12]  N. Aaronson,et al.  Quality of life and consequences for daily life of familial adenomatous polyposis (FAP) family members , 2011, Colorectal disease : the official journal of the Association of Coloproctology of Great Britain and Ireland.

[13]  I. Tomlinson,et al.  Attenuated familial adenomatous polyposis: results from an international collaborative study , 2010, Colorectal disease : the official journal of the Association of Coloproctology of Great Britain and Ireland.

[14]  C. Herfarth,et al.  Quality of Life Ten and More Years After Restorative Proctocolectomy for Patients With Familial Adenomatous Polyposis Coli , 2010, Diseases of the colon and rectum.

[15]  P. Møller,et al.  Guidelines for the clinical management of familial adenomatous polyposis (FAP) , 2008, Gut.

[16]  H. Morreau,et al.  Germline mutations in APC and MUTYH are responsible for the majority of families with attenuated familial adenomatous polyposis , 2007, Clinical genetics.

[17]  M. Nieuwenhuis,et al.  Correlations between mutation site in APC and phenotype of familial adenomatous polyposis (FAP): a review of the literature. , 2007, Critical reviews in oncology/hematology.

[18]  H. Nelson,et al.  Quality of Life After Ileal Pouch-Anal Anastomosis and Ileorectal Anastomosis in Patients With Familial Adenomatous Polyposis , 2005, Diseases of the colon and rectum.

[19]  M. Leppert,et al.  Genetic testing and phenotype in a large kindred with attenuated familial adenomatous polyposis. , 2004, Gastroenterology.

[20]  S. Bülow,et al.  Results of national registration of familial adenomatous polyposis , 2003, Gut.

[21]  B. Bittorf,et al.  Patients with familial adenomatous polyposis experience better bowel function and quality of life after ileorectal anastomosis than after ileoanal pouch , 2003, Colorectal disease : the official journal of the Association of Coloproctology of Great Britain and Ireland.

[22]  M. Sprangers,et al.  Quality of life after total colectomy with ileorectal anastomosis or proctocolectomy and ileal pouch—anal anastomosis for familial adenomatous polyposis , 2000, The British journal of surgery.

[23]  I. Heiskanen, T. Luostarinen, H. J. Järvinen Impact of Screening Examinations on Survival in Familial Adenomatous Polyposis , 2000 .

[24]  R. Hultcrantz,et al.  Epidemiology of familial adenomatous polyposis in Sweden: changes over time and differences in phenotype between males and females. , 1999, Scandinavian journal of gastroenterology.

[25]  H. Vasen,et al.  Functional outcome after colectomy and ileorectal anastomosis compared with proctocolectomy and ileal pouch-anal anastomosis in familial adenomatous polyposis. , 1999, Annals of surgery.

[26]  S. Bülow,et al.  [Prognosis in familial adenomatous polyposis. Results from the Polyposis Registry]. , 1996, Ugeskrift for laeger.

[27]  T. Muto,et al.  [Familial polyposis coli]. , 1995, Nihon rinsho. Japanese journal of clinical medicine.

[28]  S. Bülow,et al.  Centralized registration, prophylactic examination, and treatment results in improved prognosis in familial adenomatous polyposis. Results from the Danish Polyposis Register. , 1995, Scandinavian journal of gastroenterology.

[29]  M. Kida,et al.  Simultaneous side-by-side self-expanding metal stent placement using a two-channel endoscope for a bilioenteric stricture after Child’s resection , 2016, Endoscopy.