Association of cancer with comorbid inflammatory conditions and treatment in patients with Lynch syndrome.

BACKGROUND: Individuals with Lynch syndrome (LS) and hereditary non-polyposis colorectal cancer (HNPCC) are at increased risk of both colorectal cancer and other cancers. The interplay between immunosuppression, a comorbid inflammatory condition (CID), and HNPCC on cancer risk is unclear. AIM: To evaluate the impact of CIDs, and exposure to monoclonal antibodies and immunomodulators, on cancer risk in individuals with HNPCC. METHODS: Individuals prospectively followed in a hereditary cancer registry with LS/HNPCC with the diagnosis of inflammatory bowel disease or rheumatic disease were identified. We compared the proportion of patients with cancer in LS/HNPCC group with and without a CID. We also compared the proportion of patients who developed cancer following a CID diagnosis based upon exposure to immunosuppressive medications. RESULTS: A total of 21 patients with LS/HNPCC and a CID were compared to 43 patients with LS/HNPCC but no CID. Cancer occurred in 84.2% with a CID compared to 76.7% without a CID (P = 0.74) with no difference in age at first cancer diagnosis 45.5 ± 14.6 vs 43.8 ± 7.1 years (P = 0.67). LS specific cancers were diagnosed in 52.4% with a CID vs 44.2% without a CID (P = 0.54). Nine of 21 (42.9%) patients were exposed to biologics or immunomodulators for the treatment of their CID. Cancer after diagnosis of CID was seen in 7 (77.8%) of exposed individuals vs 5 (41.7%) individuals unexposed to biologics/immunomodulators (P = 0.18). All 7 exposed compared to 3/5 unexposed developed a LS specific cancer. The exposed and unexposed groups were followed for a median 10 years and 8.5 years, respectively. The hazard ratio for cancer with medication exposure was 1.59 (P = 0.43, 95%CI: 0.5-5.1). CONCLUSION: In patients with LS/HNPCC, the presence of a concurrent inflammatory condition, or use of immunosuppressive medication to treat the inflammatory condition, might not increase the rate of cancer occurrence in this limited study.

The relationship between mesorectal grading and oncological outcome in rectal adenocarcinoma.

AIM: The prognostic association between mesorectal grading and oncological outcome in patients undergoing resection for rectal adenocarcinoma is controversial. The aim of this retrospective chart review was to determine the individual impact of mesorectal grading on rectal cancer outcomes. METHOD: We compared oncological outcomes in patients with complete, near-complete and incomplete mesorectum who underwent rectal excision with curative intent from 2009 to 2014 for Stage cI-III rectal adenocarcinoma. We also assessed the independent association of mesorectal grading and oncological outcome using multivariate models including other relevant variables. RESULTS: Out of 505 patients (339 men, median age of 60 years), 347 (69%) underwent a restorative procedure. There were 452 (89.5%), 33 (6.5%) and 20 (4%) patients with a complete, near-complete and incomplete mesorectum, respectively. Local recurrence was seen in 2.4% (n = 12) patients after a mean follow-up of 3.1 ± 1.7 years. Unadjusted 3-year Kaplan-Meier analysis by mesorectal grade showed decreased rates of overall, disease-free and cancer-specific survival and increased rates of overall and distant recurrence with a near-complete mesorectum, while local recurrence was increased in cases of an incomplete mesorectum (all P < 0.05). On multivariate analyses, a near-complete mesorectum was independently associated with decreased cancer-specific survival (hazard ratio 0.26, 95% CI 0.1-0.7; P = 0.007). There were no associations between mesorectal grading and overall survival, disease-free survival, overall recurrence or distant recurrence (all P > 0.05). CONCLUSION: Mesorectal grading is independently associated with oncological outcome. It provides unique information for optimizing surgical quality in rectal cancer.

Colorectal surveillance after segmental resection for young-onset colorectal cancer: is there evidence for extended resection?

AIM: Although sporadic colorectal cancer (CRC) usually occurs in patients aged over 50, recent evidence suggests that the incidence is increasing in younger patients. Such patients are theoretically at high risk of metachronous neoplasia and may be candidates for extended prophylactic colectomy. This study aimed to define the risk of metachronous cancer/adenomas during follow-up of younger patients who underwent segmental colectomy for CRC. METHOD: A CRC database was used to identify patients aged under 50 who underwent surgery for CRC between 1994 and 2010. Patients diagnosed with hereditary cancer or inflammatory bowel disease were excluded. The primary end-points were frequency of extended resection and the rates of metachronous cancer and high-risk adenomas during follow-up. RESULTS: There were 284 young patients with a resectable primary tumour, of whom 280 (98.6%) underwent segmental resection, 3 (1%) extended resection and 1 (0.4%) local resection. Endoscopic follow-up was available for 150 of the patients who had segmental colectomy, with a mean age of 42.6 (±5.8) years at diagnosis and median follow-up time of 68 months (interquartile range 45-105). Out of these 150 patients, 4 (2.7%) developed metachronous colonic adenocarcinoma at 24, 71, 151 and 228 months after index surgery. Thirty additional patients had at least one adenoma identified during surveillance, and three had sessile serrated polyps. Out of the three patients undergoing extended resection, none had metachronous cancer or advanced adenomas at an average follow-up of 17 years. CONCLUSION: A segmental colectomy or proctectomy is adequate treatment for patients presenting with CRC under the age of 50.

Restorative proctocolectomy: an example of how surgery evolves in response to paradigm shifts in care.

AIM: Surgical technique constantly evolves in response to the pressure of progress. Ileal pouch anal anastomosis (IPAA) is a good example. We analysed the effect of changes in practice on the technique of IPAA and its outcomes. METHOD: Patients undergoing primary IPAA at this institution were divided into three groups by date of the IPAA: those operated from 1983 to 1993, from 1994 to 2004 and from 2005 to 2015. Demographics, patient comorbidity, surgical techniques, postoperative outcomes, pouch function and quality of life were analysed. RESULTS: In all, 4525 patients had a primary IPAA. With each decade, increasing numbers of surgeons were involved (decade I, 8; II, 16; III, 31), patients tended to be sicker (higher American Society of Anesthesiologists score) and three-staged pouches became more common. After an initial popularity of the S pouch, J pouches became dominant and a mucosectomy rate of 12% was standard. The laparoscopic technique blossomed in the last decade. 90-day postoperative morbidity by decade was 38.3% vs 50% vs 48% (P < 0.0001), but late morbidity decreased from 74.2% through 67.1% to 30% (P < 0.0001). Functional results improved, but quality of life scores did not. Pouch survival rate at 10 years was maintained (94% vs 95.2% vs 95.2%; P = 0.06). CONCLUSION: IPAA is still evolving. Despite new generations of surgeons, a more accurate diagnosis, appropriate staging and the laparoscopic technique have made IPAA a safer, more effective and enduring operation.

Genotype-defined cancer risk in juvenile polyposis syndrome.

BACKGROUND: Germline mutations in SMAD4 and BMPR1A disrupt the transforming growth factor ß signal transduction pathway, and are associated with juvenile polyposis syndrome. The effect of genotype on the pattern of disease in this syndrome is unknown. This study evaluated the differential impact of SMAD4 and BMPR1A gene mutations on cancer risk and oncological phenotype in patients with juvenile polyposis syndrome. METHODS: Patients with juvenile polyposis syndrome and germline SMAD4 or BMPR1A mutations were identified from a prospectively maintained institutional registry. Medical records were reviewed and the clinical patterns of disease were analysed. RESULTS: Thirty-five patients had germline mutations in either BMPR1A (8 patients) or SMAD4 (27). Median follow-up was 11 years. Colonic phenotype was similar between patients with SMAD4 and BMPR1A mutations, whereas SMAD4 mutations were associated with larger polyp numbers (number of patients with 50 or more gastric polyps: 14 versus 0 respectively). The numbers of patients with rectal polyps was comparable between BMPR1A and SMAD4 mutation carriers (5 versus 17). No patient was diagnosed with cancer in the BMPR1A group, whereas four men with a SMAD4 mutation developed gastrointestinal (3) or extraintestinal (1) cancer. The gastrointestinal cancer risk in patients with juvenile polyposis syndrome and a SMAD4 mutation was 11 per cent (3 of 27). CONCLUSION: The SMAD4 genotype is associated with a more aggressive upper gastrointestinal malignancy risk in juvenile polyposis syndrome.

The impact of mesenteric tension on pouch outcome and quality of life in patients undergoing restorative proctocolectomy.

AIM: The study aimed to establish a method for the measurement of mesenteric tension after ileal pouch-anal anastomosis (IPAA) and to evaluate the impact of tension on clinical outcome and quality of life. METHODS: All consecutive patients undergoing an open IPAA from July 2008 to October 2009 were prospectively enrolled. After the creation of the anastomosis, mesenteric tension was estimated by the surgeon in the operating room on a 10-point scale (1, least tension; 10, most tension). The association was analysed between mesenteric tension defined as low (1-2), medium (3-7) and high (8-10) and postoperative complications and quality of life (Cleveland Clinic Global Scale). RESULTS: A mesenteric tension score was obtained in 134 patients (71 men, 53.0%). Median age was 38.5 (29.3-47.0) years. Fifty-six patients (41.8%) had a low, 59 (44.0%) a medium and 19 (14.2%) a high degree of mesenteric tension. Patients with a high mesenteric tension had a shorter anal transitional zone, a longer distance from the upper border of the symphysis pubis to the apex of the small bowel loop designated for the ileoanal anastomosis, a thinner abdominal wall at the stoma site and a longer distance from the pouch to the ileostomy. The proportion of patients with high mesenteric tension was less after stapled anastomosis. On long-term follow-up, patients with high mesenteric tension were more likely to suffer from anastomotic stricture and pouch failure. Pouch function was not influenced by mesenteric tension. CONCLUSION: High mesenteric tension after IPAA is adversely associated with postoperative complications and pouch survival.

Results from an American Society of Colon and Rectal Surgeons survey on the management of young-onset colorectal cancer.

BACKGROUND: Young patients with colorectal cancer (CRC) present a diagnostic and clinical challenge. The aim of our study was to survey the approaches to preoperative evaluation and clinical management of young patients with CRC by colorectal surgeons in North America. METHODS: A standard electronic survey was sent to the members of the American Society of Colon and Rectal Surgeons. The survey polled management decisions in various clinical scenarios for CRC patients less than 50 years old. Survey responses were collated and analyzed. RESULTS: One hundred ninety surgeons responded and 140 completed the entire survey (response rate 10%). Eighty percent of surgeons would offer preoperative genetic testing if the patient's family met the Amsterdam criteria compared to only 67% if the criteria were not met. Of those offering preoperative tumor testing, 48% test microsatellite instability, 19% mismatch repair protein expression by immunohistochemistry, and 24% offer both. Decisions regarding the extent of the resection for cancer were dependent on family history: Most members (86%) would perform a segmental colectomy for CRC in a patient without family history. Eighty-four percent of respondents would offer a total abdominal colectomy if preoperative tests indicated Lynch syndrome. When questioned about MYH-associated polyposis, only 27% recognized the appropriate diagnosis. CONCLUSIONS: Among the American Society of Colon and Rectal Surgeons, family history influences preoperative testing and surgical management decisions. A significant portion of surgeons do not offer preoperative genetic testing, despite implications on operative management, postoperative surveillance, and screening of family members.

Key factors associated with postoperative complications in patients undergoing colorectal surgery.

BACKGROUND: Surgical outcomes are determined by complex interactions among a variety of factors including patient characteristics, diagnosis, and type of procedure. OBJECTIVE: The aim of this study was to prioritize the effect and relative importance of the surgeon (in terms of identity of a surgeon and surgeon volume), patient characteristics, and the intraoperative details on complications of colorectal surgery including readmission, reoperation, sepsis, anastomotic leak, small-bowel obstruction, surgical site infection, abscess, need for transfusion, and portal and deep vein thrombosis. DESIGN: This study uses a novel classification methodology to measure the influence of various risk factors on postoperative complications in a large outcomes database. METHODS: Using prospectively collected information from the departmental outcomes database from 2010 to 2011, we examined the records of 3552 patients who underwent colorectal surgery. Instead of traditional statistical methods, we used a family of 7000 bootstrap classification models to examine and quantify the impact of various factors on the most common serious surgical complications. For each complication, an ensemble of multivariate classification models was designed to determine the relative importance of potential factors that may influence outcomes of surgery. This is a new technique for analyzing outcomes data that produces more accurate results and a more reliable ranking of study variables in order of their importance in producing complications. PATIENTS: Patients who underwent colorectal surgery in 2010 and 2011 were included. SETTINGS: This study was conducted at a tertiary referral department at a major medical center. MAIN OUTCOME: Postoperative complications were the primary outcomes measured. RESULTS: Factors sorted themselves into 2 groups: a highly important group (operative time, BMI, age, identity of the surgeon, type of surgery) and a group of low importance (sex, comorbidity, laparoscopy, and emergency). ASA score and diagnosis were of intermediate importance. The outcomes most influenced by variations in the highly important factors included readmission, transfusion, surgical site infection, and abscesses. LIMITATIONS: This study was limited by the use of data from a single tertiary referral department at a major medical center. CONCLUSIONS: Body mass index, operative time, and the surgeon who performed the operation are the 3 most important factors influencing readmission rates, rates of transfusions, and surgical site infection. Identification of these contributing factors can help minimize complications.

Prognostic modeling of preoperative risk factors of pouch failure.

BACKGROUND: The prospect of pouch failure needs to be considered when evaluating the management strategy for patients who may be candidates for an ileo anal pouch. An ability to predict the likelihood and timing of failure preoperatively may influence surgical decision making. OBJECTIVE: The aim of this study was to define a preoperative prognostic model for ileoanal pouch failure. DESIGN: A novel random forest methodology was used to evaluate the prognostic significance of 21 preoperative potential risk factors for pouch failure. A forest of 3000 random survival trees was grown to estimate pouch failure for each patient and to identify important risk factors that maximize survival prediction. SETTINGS: This study took place at a tertiary referral department at a major academic medical center. PATIENTS: Patients undergoing an ileoanal pouch at this institution between 1983 and 2008 were included. MAIN OUTCOME MEASURES: The primary outcome measured was pouch survival. RESULTS: Between 1983 and 2008, 3754 patients underwent ileoanal pouch. Type of resection (total proctocolectomy vs completion proctectomy), type of anastomosis (stapled vs mucosectomy), patient diagnosis (mucosal ulcerative colitis and others vs Crohn's disease) and diagnosis of diabetes had the strongest effect on pouch survival. Predicted survival was worse for completion proctectomy (HR, 1.44; 95% CI, 1.08-1.93), Crohn's disease (HR, 2.37; 95% CI, 1.48-3.79), handsewn anastomosis (HR, 1.72; 95% CI, 1.23-2.42), and diabetes (HR, 2.31; 95% CI, 1.25-4.24). Pouch survival was worse for the oldest group of patients. LIMITATIONS: This study was limited by its retrospective nature. CONCLUSION: Random forest techniques applied to a large number of patients undergoing the ileoanal pouch identify factors associated with pouch failure. Attention directed at these factors may improve outcomes for these patients.

Biological immunomodulators improve the healing rate in surgically treated perianal Crohn's fistulas.

AIM: The role of biological therapy in perianal fistulas associated with Crohn's disease (CD) is uncertain as available data are confused and conflicting. In order to provide some clarity to the issue we have examined a large cohort of patients with perianal fistulas and CD and stratified them according to use of biological agents. METHOD: Patients with perianal Crohn's fistulas treated between June 1999 and June 2009 were stratified according to use of biological agents and outcome was examined. Healing was defined as absence of fistula or drainage. Prior to surgery perianal sepsis was eradicated with drains or setons. Endpoints were defined as either complete healing, improvement (minimal symptoms and drainage) or unhealed, as noted at subsequent outpatient follow-up. Variables assessed were age, body mass index, smoking, perineal involvement with Crohn's granuloma and type of procedure. Fisher's exact test and χ(2) test were used for analysis. RESULTS: Two hundred and eighteen patients had anal fistulas and CD. Mean follow-up was 3.2±3 years with mean age 38.8±12.2years and body mass index of 25.3±6. One hundred and seventeen patients (53.7%) underwent surgery alone (Group A) and 101 patients (46.3%) underwent surgery and biological immunomodulator treatments (Group B). Demographic data and CD history were similar between groups. Surgeries included seton drainge (n=90), fistulotomy (n=22), rectal advancement flap (n=39), fistulotomy plus seton (n=47) and others (n=20). Overall improvement in Group A was in 42 patients (35.9%) vs 72 patients (71.3%) in Group B (P=0.001). There was no significant difference in other studied variables between both groups. CONCLUSIONS: There is a definite role for biological therapy as an adjuvant to surgery in patients with perianal fistulas and CD.

Influence of age at ileoanal pouch creation on long-term changes in functional outcomes.

AIM: We reviewed the functional results and quality of life (QOL) of patients who had had an ileoanal pouch (IPAA) for at least 15 years. METHOD: Retrospective analysis was undertaken of data accrued prospectively into a pouch database since 1983. Patients who had retained an IPAA for at least 15 years were identified. Trends in IPAA function and QOL of the patients were determined over a time-period of 15 years after formation of the IPAA. Data were compared for patients who were < 35, 35-55 and > 55 years of age when the IPAA was formed. RESULTS: Three hundred and ninety-six of a total of 3276 patients in the database (53% men, median age 36 years and median follow-up 17.1 years) underwent IPAA with at least 15 years of follow-up. The final pathology was ulcerative colitis in 78%; 66.4% of patients had a restorative proctocolectomy, 91.4% underwent temporary diversion, 59% had a J-pouch configuration and 63.1% a stapled anastomosis. The frequency of bowel movements remained the same over the follow-up period. There was an increase in the incidence of incontinence and urgency after 15 years with no significant change in dietary, social, work and sexual restrictions during follow-up. Patients in all three age groups experienced deterioration in pouch function at 15 years of follow up compared with the function at 5 years. The QOL of the patients remained high and stable. CONCLUSION: There is a deterioration of pouch function after 15 years, irrespective of the age of the patient when the IPAA was formed. Despite this, QOL appears to be high for all patients who retain their pouch.

Genetic and epigenetic classifications define clinical phenotypes and determine patient outcomes in colorectal cancer.

BACKGROUND: A molecular classification of colorectal cancer has been proposed based on microsatellite instability (MSI), CpG island methylator phenotype (CIMP), and mutations in the KRAS and BRAF oncogenes. This study examined the prevalence of these molecular classes, and differences in clinical presentation and outcome. METHODS: Demographics, tumour characteristics and survival were recorded for 391 subjects with colorectal cancer. Tumour DNA was analysed for MSI (high (MSI-H) or microsatellite stable (MSS)), CIMP (high (CIMP-H) or no (CIMP-neg)) and BRAF and KRAS mutations. Clinical differences between four phenotypes were examined. RESULTS: Most tumours were MSS/CIMP-neg (69.8 per cent), with a nearly equal distribution of MSI-H/CIMP-H, MSI-H/CIMP-neg and MSS/CIMP-H types. MSS/CIMP-neg tumours were less likely to be poorly differentiated (P = 0.009). CIMP-H tumours were more common in older patients (P < 0.001). MSI-H/CIMP-H tumours had a high frequency of BRAF mutation and a low rate of KRAS mutation; the opposite was true for MSS/CIMP-neg tumours (P < 0.001). The four molecular phenotypes tended towards divergent survival (P = 0.067 for stages 1-III). MSI-H cancers were associated with better disease-free survival (hazard ratio 2.00 (95 per cent confidence interval 1.03 to 3.91); P = 0.040). CONCLUSION: Colorectal cancers are molecularly and clinically heterogeneous. These different molecular phenotypes may reflect variable prognosis.

Measuring sexual and urinary outcomes in women after rectal cancer excision.

PURPOSE: This study was designed to investigate sexual and urinary dysfunction in women who underwent rectal cancer excision, and the influence of tumor and treatment variables on long-term outcomes. METHODS: Data were prospectively collected on 295 women who underwent rectal cancer excision at a tertiary referral colorectal center from 1998 to 2006. Sexual and urinary function was assessed preoperatively and at intervals up to five years after surgery. Functional outcomes were assessed by using univariate and multivariate regression analysis, chi-squared test for trend, or Kruskal-Wallis test. RESULTS: The mean age of the patients was 60.9 years. Anterior resection was performed in 222 patients (75.2 percent) and abdominoperineal resection in 73 patients (24.7 percent). Patients who underwent abdominoperineal resection were less sexually active (25 vs. 50 percent; P = 0.02) and had a lower frequency of intercourse than anterior resection patients at one year after surgery (anterior resection, 3 (0-5) (median interquartile range); abdominoperineal resection 0 (0-4); P = 0.029). The frequency of intercourse improved over time for abdominoperineal resection (4 months, 0 (0-0) median interquartile range; 5 years, 3 (0.25-4) median interquartile range; P = 0.028). Abdominoperineal resection was associated with increased dyspareunia (odds ratio, 5.75; 95 percent confidence interval (CI), 1.87-17.6; P = 0.002), urinary urgency (odds ratio, 8.52; 95 percent CI, 2.81-25.8; P < 0.001), incontinence (odds ratio, 2.41; 95 percent CI, 1.11-5.26; P = 0.026), poor stream (odds ratio, 5.64, 95 percent CI, 2.55-12.5; P

Endoscopic surveillance of the ileoanal pouch following restorative proctocolectomy for familial adenomatous polyposis.

Persistence of underlying disease in the residual rectal mucosa and anal transition zone occurs following mucosectomy with either a hand-sewn anastomosis or a double-stapled anastomosis. Furthermore, recent reports have suggested an increased incidence of neoplasia in the pouch body. For this reason, endoscopic surveillance is performed not only as a screening tool to detect significant intraepithelial neoplastic lesions but also with secondary therapeutic intent aimed at reducing the adenoma burden within the ileoanal pouch. Conventional endoscopic assessment of the ileoanal pouch can be challenging. In the future, novel adjunctive endoscopic technologies such as magnification endoscopy and confocal endomicroscopy may improve our diagnostic and therapeutic management of this group.

Complications and functional results after ileoanal pouch formation in obese patients.

OBJECTIVE: Ileoanal pouch formation (IPAA) can be technically challenging in obese patients, and there is little data evaluating results after the procedure in these patients. We compare outcomes for patients with a body mass index (BMI) > or =30 undergoing IPAA when compared with those for patients with BMI <30. METHODS: Retrospective analysis of prospectively accrued data for patients with BMI > or =30 undergoing IPAA. Patient and disease-related characteristics, complications, long-term function, and quality of life (QOL) using the Cleveland Global Quality of Life scale (CGQL) were determined for this group of patients (group B) and compared with those for patients with BMI <30 (group A). Kruskal-Wallis and Wilcoxon rank sum tests were used to compare quantitative or ordinal data and chi-square or Fisher's exact tests for categorical variables. Long-term mortality and complication rates were estimated using the Kaplan-Meier method with group comparisons performed using log rank tests. RESULTS: There were 345 patients (median BMI 32.7) in group B and 1,671 patients in group A. When the cumulative risk of complications over 15 years was compared, group B patients had a significantly higher chance of getting a complication (94.9% vs 88%, p = 0.006). The rates of pelvic sepsis (6.7% vs 5.3%, p = 0.3), pouchitis (58.1 vs 54.4%, p = 0.9), pouch failure (6% vs 4.5%, p = 0.9), and hemorrhage (5.6% vs 4.8%, p = 0.7) were similar for group B and group A. Group B patients, however, had a significantly higher risk of the development of wound infection (18.8% vs 8.1%, p < 0.001) and anastomotic separation (10.4% vs 5.4%, p < 0.001), whereas group A patients had a higher rate of development of obstruction over time (26.7% vs 22.3%, p = 0.02). Long-term outcome including QOL and function after 15 years was comparable between groups. CONCLUSIONS: Although technically demanding, IPAA can be undertaken in obese patients with acceptable morbidity. Good long-term functional results and QOL that is comparable to nonobese patients may be anticipated.

Analysis of the outcome of ileal pouch-anal anastomosis in patients with Crohn's disease.

PURPOSE: Ileal pouch-anal anastomosis has come to represent the procedure of choice for patients requiring surgery for mucosal ulcerative colitis. In contrast, a proven diagnosis of Crohn's disease is generally held to preclude ileal pouch-anal anastomosis. However, patients with ileal pouch-anal anastomosis for apparent mucosal ulcerative colitis who are subsequently found to have Crohn's disease have a variable course. We reviewed our experience in this scenario to determine whether selected patients with Crohn's disease may be candidates for ileal pouch-anal anastomosis. METHODS: A retrospective review of the prospectively maintained ileal pouch-anal anastomosis database was undertaken to identify patients with a diagnosis of Crohn's disease after ileal pouch-anal anastomosis. Clinical outcome and quality-of-life data were obtained from the database and chart review. End points were the development of recrudescent Crohn's disease, pouch failure, and quality of life and functional outcome at the time of data collection. Differences between groups were calculated using the chi-squared test. Cumulative incidence of recrudescent Crohn's disease and pouch loss were calculated by the Kaplan-Meier method. Factors predictive of development of recrudescent Crohn's disease and pouch loss were examined by univariate analysis. RESULTS: Sixty patients (32 females; median age, 33 (range, 15-74) years) who underwent ileal pouch-anal anastomosis for mucosal ulcerative colitis subsequently had that diagnosis revised to Crohn's disease. Median follow-up of all patients was 46 (range, 4-158) months at time of data collection by which time 21 patients (35 percent) had developed recrudescent Crohn's disease. No pre-ileal pouch-anal anastomosis factors examined were predictors of the development of recrudescent Crohn's disease on univariate analysis. Median follow-up of the latter group was 63 (range, 0-132) months from time of diagnosis, by which time six patients underwent pouch excision and another patient was permanently defunctioned. The overall pouch loss rate for the entire cohort was 12 percent and 33 percent for those with recrudescent Crohn's disease. Median daily bowel movements in those with ileal pouch-anal anastomosis in situ at the time of data collection was 7 (range, 3-20), with 50 percent of patients rarely or never experiencing urgency and 59 percent reporting perfect or near perfect continence. Median quality of life, health, and happiness scores were 9.9 and 10 of 10. CONCLUSIONS: The secondary diagnosis of Crohn's disease after ileal pouch-anal anastomosis is associated with protracted freedom from clinically evident Crohn's disease, low pouch loss rate, and good functional outcome. Such results only can be improved by the continued development of medical strategies for the long-term suppression of Crohn's disease. These data support a prospective evaluation of ileal pouch-anal anastomosis in selected patients with Crohn's disease.

Benefits of 'clean sweep' in Peutz-Jeghers patients.

OBJECTIVE: Laparotomy is the treatment of choice in Peutz-Jeghers Syndrome (PJS) patients for endoscopically irretrievable symptomatic polyps and polyp-related complications. During the last decade, we have operated on majority of the PJS patients with the purpose of removing all the gastrointestinal polyps (clean sweep), when an operation was indicated. The aim of this study is to evaluate the effect of clean sweep technique on the need for repeated surgery compared to a problem focused approach. PATIENTS AND METHODS: All patients with PJS treated in our institution since 1964 were studied. They were placed into two groups; those who had a problem-focused operation and those who were operated with the purpose of removing all small and large intestinal polyps. Demographics, presentation, follow-up period and the need for recurrent surgery were compared. RESULTS: We identified 11 patients (4 males, 7 females). Eight patients (5 females; median age 18.5) had problem-focused surgery for bleeding-anaemia (n = 3) or obstruction-intussusception (n = 5). These patients required 23 further operations within 87 patient-follow-up-years (2.64 operations per 10 years). Three patients (2 females; median age 6) were operated for bleeding-anaemia (n = 1) or obstruction-intussusception (n = 2) using the 'clean sweep' approach. These patients did not require any further surgery within 21 patient-follow-up-years. The gender, presentation and follow-up periods were similar between the groups. However, the 'clean sweep' technique appears to have reduced the need for further operations when it is compared with problem-focused approach (P = 0.01). CONCLUSION: To reduce the need for abdominal surgery and consequent problems in PJS patients, an attempt to remove all detected polyps (clean sweep technique) may be beneficial in these patients.

Significance of incidental desmoids identified during surgery for familial adenomatous polyposis.

PURPOSE: The behavior of intra-abdominal desmoids in familial adenomatous polyposis is incompletely understood. Findings range from typical mass lesions to flat sheets, termed the desmoid reaction or desmoid precursor lesion. The latter often are incidental findings of uncertain significance. The study was designed to describe the natural history of incidental intra-abdominal desmoid tumors with particular reference to the desmoid reaction. METHODS: Patients who underwent laparotomy for familial adenomatous polyposis at the Cleveland Clinic Foundation were identified. The incidence of incidental intra-abdominal desmoid tumors was determined by review of operative records. Intra-abdominal desmoid tumors were classified as mass lesions if three-dimensional or desmoid reaction if two-dimensional. The incidence of clinically apparent intra-abdominal desmoid tumors (typical mass lesions on physical examination or cross-sectional imaging in symptomatic patients) was determined by chart review. The incidence of clinical intra-abdominal desmoid tumors between groups was compared by Fisher's exact test. RESULTS: A total of 266 patients (153 females; median age, 26 (range, 9-63) years) underwent abdominal surgery for familial adenomatous polyposis. Incidental intra-abdominal desmoid tumors were identified in 34 patients: 8 at the index surgery and 26 at relaparotomy. These lesions influenced the planned procedure in eight cases (26 percent), including preventing ileoanal pouch in 3 of 19 patients in whom this was intended. The median follow-up from the time of identification of intra-abdominal desmoids was 42 (range, 2-178) months at which point four patients (11 percent) had developed clinical intra-abdominal desmoid tumors. There was no significant difference in incidence of clinical intra-abdominal desmoid tumors between mass and desmoid reaction groups ( P = 0.27). CONCLUSIONS: Incidental intra-abdominal desmoid tumors are a common finding at relaparotomy in patients with familial adenomatous polyposis. These lesions influence planned surgery in a minority of cases. Desmoid reaction may have little bearing on the subsequent development of clinically significant intra-abdominal desmoid tumors.