Predictive models forprophylactic surgery for familial adenomatous polyposis - development of a new scoring system: B5

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Aim:Colectomy with ileo-rectal anastomosis (IRA) is performed forprophylaxis in familial adenomatous polyposis (FAP). Post-IRA patients may require completionproctectomy for worsening polyposis or rectal cancer. We evaluated the outcome after IRA and identified risk factorspredictingprogression of rectal disease.Method:Hazard ratios (HR) were calculated for phenotype, genotype, gender, age at surgery andpresence of colonic cancer. We developed a weighted scoring systempredicting rectal survival post-IRA.Results:Of the 427 patients having IRA analysed, 77(18%) requiredproctectomy for polyposis and 39(9%) for rectal cancer. Nine (2%) developed rectal cancer but did not undergoproctectomy. Fifty percent retained a healthy rectum at age 60 years. Multivariate analysis identified a rectal polyp count > 20 (HR31, 95%CI 9.6-100, P < 0.0001), an APC mutation at codon 1250-1450 (HR3.9, 95%CI 1.5-10.5, P = 0.007), colonic polyp count > 500 (HR2.2, 95%CI 1.2-3.8, P = 0.006) and age at surgery below 25 (HR2, 95%CI 1.2-3.4, P = 0.011) years aspredictors ofprogressive rectal disease. Ourpredictive scoring system yielded a maximum score of 5. Patients scoring 3 or more were significantly less likely to retain a healthy rectum.Conclusion:The scoring system may be useful for the stratification of risk to assist the choice between IRA and restorativeproctocolectomy forprimary surgery for FAP

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