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      Risk of desmoid formation after laparoscopic versus open colectomy and ileorectal anastomosis for familial adenomatous polyposis

      , 1 , 1 , 1 , 1

      BJS Open

      John Wiley & Sons, Ltd

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          Laparoscopy is used increasingly in prophylactic surgery for patients with familial adenomatous polyposis (FAP) undergoing colectomy with ileorectal anastomosis (IRA). Little is known about the impact of laparoscopy on subsequent desmoid risk. This study documented the risk of desmoid in patients undergoing laparoscopic and open IRA.


          This was an observational study of patients with FAP and known germline APC mutation, undergoing IRA at a tertiary referral centre between 1996 and 2016. Patients were retrieved from a prospectively maintained polyposis registry. Data included genotype, family history of desmoid, sex, surgical approach at IRA and postoperative complications. The main outcome was development of either a clinically or radiologically significant desmoid.


          Some 112 patients (61 female) underwent colectomy and IRA. A laparoscopic approach was used in 69 patients (61·6 per cent). Baseline characteristics did not differ between patients having an open or laparoscopic approach. Median follow‐up was 5·8 (i.q.r. 2·4–11·2) years. Patients who underwent laparoscopic IRA had a reduced risk of desmoid formation (3 of 69 (4 per cent) versus 7 of 43 (16 per cent) in the open group; P = 0·043).


          Laparoscopic IRA may reduce risk of subsequent desmoid formation in patients with FAP.

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          Most cited references 9

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          Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey.

          Although quality assessment is gaining increasing attention, there is still no consensus on how to define and grade postoperative complications. This shortcoming hampers comparison of outcome data among different centers and therapies and over time. A classification of complications published by one of the authors in 1992 was critically re-evaluated and modified to increase its accuracy and its acceptability in the surgical community. Modifications mainly focused on the manner of reporting life-threatening and permanently disabling complications. The new grading system still mostly relies on the therapy used to treat the complication. The classification was tested in a cohort of 6336 patients who underwent elective general surgery at our institution. The reproducibility and personal judgment of the classification were evaluated through an international survey with 2 questionnaires sent to 10 surgical centers worldwide. The new ranking system significantly correlated with complexity of surgery (P < 0.0001) as well as with the length of the hospital stay (P < 0.0001). A total of 144 surgeons from 10 different centers around the world and at different levels of training returned the survey. Ninety percent of the case presentations were correctly graded. The classification was considered to be simple (92% of the respondents), reproducible (91%), logical (92%), useful (90%), and comprehensive (89%). The answers of both questionnaires were not dependent on the origin of the reply and the level of training of the surgeons. The new complication classification appears reliable and may represent a compelling tool for quality assessment in surgery in all parts of the world.
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            Familial adenomatous polyposis (FAP): frequency, penetrance, and mutation rate.

            The nationwide Danish polyposis register includes all known Danish cases of familial adenomatous polyposis (FAP) and their relatives. By identifying all FAP patients born between 1920 and 1949, we found the frequency of the disease to be 1 in 13,528. By comparing the number of affected and nonaffected offspring born to affected parents during the same period we found the penetrance of the disease for inherited cases to be close to 100% at the age of 40 years. The mutation rate found by the direct method was 9 mutations per million gametes per generation and the proportion of new mutants was estimated to 25%. Fitness for patients between 15 and 29 years was found close to one, while for patients older than 30 the fitness was reduced, but increasing during the three decades (from 0.44 to 0.71) probably because treatment became more widespread and efficient. As we have used the overall fitness in the period, 0.87, to estimate the mutation rate by the indirect method, we found a lower value than by the direct method, namely 5 mutations per million gametes per generation.
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              Genotype and phenotype factors as determinants of desmoid tumors in patients with familial adenomatous polyposis.

               ,  M Pierotti,  Debra A Russo (2001)
              Desmoids represent the most important cause of death, after colorectal cancer, in patients affected with familial adenomatous polyposis (FAP), an inherited disease due to mutations in the APC gene. The aims of our study were to estimate the risk of developing desmoids in FAP patients and to evaluate the association between desmoids and different risk factors. The occurrence of desmoids, colorectal cancer and other extra-colonic manifestations were assessed in 897 FAP patients, 653 of whom were also investigated for APC mutations. Odds ratios (OR) and corresponding 95% confidence intervals (CI) were computed using an unconditional multiple logistic regression model. Desmoids developed in 107 patients (11.9%), with a cumulative risk of 20.6%. Females had a significantly higher risk than males (OR = 2.1; 95% CI 1.4-3.1). Family history of desmoids (OR = 8.75; 95% CI 5.66-13.51), osteomas (OR = 2.9; 95% CI 1.8-4.8) and epidermoid cysts (OR = 1.8; 95% CI 1.1-3.2) was also significantly associated with the occurrence of disease. Subjects with APC mutations beyond codon 1444 had a 12-fold increased risk, compared with patients with mutations located upstream. Mutations beyond codon 1309 conferred a 17-fold higher risk, compared with mutations upstream codon 452. Multivariate analysis identified as independent predictors mutation beyond codon 1444 (OR = 6.2; 95% CI 2.5-15.8), family history of desmoids (OR = 5.8; 95% CI 3.1-10.6), female gender (OR = 2.1; 95% CI 1.1-3.8) and the presence of osteomas (OR = 1.9; 95% CI 1.1-3.4). Our results indicate that integrating genetic and clinical data is helpful in defining subgroups of patients at higher risk for desmoids, who may benefit from specific prevention programs. Copyright 2001 Wiley-Liss, Inc.

                Author and article information

                BJS Open
                BJS Open
                BJS Open
                John Wiley & Sons, Ltd (Chichester, UK )
                08 August 2018
                December 2018
                : 2
                : 6 ( doiID: 10.1002/bjs5.2018.2.issue-6 )
                : 452-455
                [ 1 ] The Polyposis Registry, Level 5 St Mark's Hospital Northwick Park, Watford Road, Harrow HA1 3UJ UK
                Author notes
                [* ] Correspondence to: Mr A. Sinha (e‐mail: ashishsinha@ )
                © 2018 The Authors. BJS Open published by John Wiley & Sons Ltd on behalf of BJS Society Ltd

                This is an open access article under the terms of the License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

                Page count
                Figures: 1, Tables: 3, Pages: 4, Words: 2048
                Original Article
                Original Articles
                Custom metadata
                December 2018
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