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Full diagnostic colonoscopy often is performed to exclude significant pathology in patients presenting with rectal bleeding. In patients with classic "outlet" bleeding, defined as bright red blood after or during defecation, with no family history of colorectal neoplasia or change in bowel habits, we hypothesize that the diagnostic yield of complete colonoscopy will be low. The purpose of this study was to determine whether complete colonoscopy is necessary in the evaluation of patients with "outlet" rectal bleeding.Information for all patients undergoing colonoscopy by a single endoscopist was prospectively recorded. Before each colonoscopy, a complete history, including indication for the examination, was obtained. Using standard definitions, patients with outlet bleeding, suspicious bleeding, hemorrhage, and occult bleeding were accessed and the findings of their colonoscopies were analyzed. Institutional permission was obtained.A total of 9,098 patients had colonoscopy recorded in the database, and 703 had the indication of outlet bleeding, 251 suspicious bleeding, 204 occult bleeding, and 67 hemorrhage. Of the patients with outlet bleeding, only 47 (6.7 percent) had significant lesions on colonoscopy (adenomas1 cm, villous adenomas, cancer in situ, or invasive cancer). By contrast a greater number of significant lesions were present in patients with all other types of bleeding (17.2 percent; P0.001). The incidence of invasive cancer was significantly lower in the outlet bleeding group compared with other types of bleeding (1 vs. 3.6 percent; P0.01). Patients with outlet bleeding were much less likely than patients with other bleeding to have isolated right-sided colonic pathology. Younger patients with outlet bleeding have a particularly low yield on colonoscopy. In 182 patients younger than aged 50 years with outlet bleeding, only 3 (1.6 percent) had adenomas1 cm and no invasive cancers were detected.In patients with classic outlet bleeding, the yield of a complete diagnostic colonoscopy is low. If the history is classic for outlet bleeding and no other indication for colonoscopy exists, flexible sigmoidoscopy is enough to exclude significant pathology.

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) ≥30 undergoing IPAA when compared with those for patients with BMI

Background: The aim of the present study was to evaluate the influence of a younger age at the time of ileal pouch-anal anastomosis (IPAA) on functional outcome and quality of life in patients with familial adenomatous polyposis (FAP). Method: A total of 105 patients with FAP who had undergone IPAA were retrieved from a dedicated Institutional Review Board (IRB)-approved IPAA database. Fifty-three patients who had complete follow-up data at 1, 3 and 5 years were included in the study. Patients were categorized into three groups according to their ages at the time of surgery. Group 1, patients ≤20 years of age (n = 9); group 2, patients >20 and ≤40 years of age (n = 23); and group 3, patients >40 years of age (n = 21). Perioperative characteristics, postoperative complications, quality of life scores and functional outcome at 1, 3 and 5 years of postoperative follow-up were assessed. Results: At any time point, there were no significant differences among the groups in terms of perioperative characteristics, quality of life scores or level of satisfaction with the procedure. However, younger patients had significantly lower nocturnal bowel movements at the first and third years of follow-up and tended to have less bowel movements per day at all stages of follow-up. Conclusion: These findings indicate that younger patients with FAP are inclined to have better functional outcome after IPAA in the early postoperative period. This finding may be encouraging for colorectal surgeons who will operate on young patients with FAP.

Traditional length of hospital stay after ileal pouch-anal anastomosis is 8 to 15 days. Fast track rehabilitation programs reduce stay, but there are concerns that readmission and complication rates may be increased. This study evaluated a fast track pathway after ileoanal pouch surgery.One hundred three consecutive patients underwent ileal pouch-anal anastomosis on two colorectal services using a fast track protocol with early ambulation, diet, and defined discharge criteria. Direct hospital costs and 30-day and long-term complication data were collected. Patients were matched to controls managed with traditional care pathways by other colorectal staff.Matching was established for 97 patients. Fast track patients had shorter hospital stay than controls (median 4 vs. 5 days; mean 5.0 vs. 5.9, P = 0.012). Readmission and recurrent operation rates were similar (24 vs. 20 percent, P = 0.49, and 9 vs. 10 percent, P = 0.8, fast track vs. control, respectively). Median direct costs per patient (US$) within 30 days were lower with fast track (5692 vs. 6672, P = 0.001), primarily because of reductions in postoperative management expenses. Complication rates, including pouch failure, bowel obstruction, pouchitis, and anastomotic stricture were comparable. Early discharge (or = 5 days from surgery) occurred in 79 (77 percent) fast track patients. Failure with early discharge was associated with male gender, reoperations, and anastomotic complications.Fast track protocol after ileoanal pouch surgery reduces length of stay and hospital costs without increasing complication rates. Successful early discharge usually signals a benign postoperative course.

Colorectal cancer is typically a condition of older patients with only 10 % diagnosed under the age of 50 years. Often, diagnosis is delayed, but certain factors such as inherited syndromes, inflammatory bowel disease, or a family history of colorectal cancer should heighten the clinician’s awareness. This study of young colorectal cancers describes the incidence of potential contributory factors that warrant early investigations and their effect on survival outcomes. A single-institution colorectal cancer database was queried for patients diagnosed with colorectal cancer under the age of 50. Medical records were reviewed, and patients were grouped into familial, inflammatory bowel disease-related, or sporadic cancers. Sporadic cancers without existing family history were further evaluated for genetic and molecular changes including mutations in the oncogenes KRAS and BRAF, microsatellite instability, and methylator phenotype. One hundred thirty-five patients under the age of 50 with colorectal cancer diagnosed between 1994 and 2004 were identified. Slightly under half, (44.4 %) were women. Mean age at surgery was 42.1 ± 6.7 years. Nineteen patients (14 %) had a hereditary colorectal cancer syndrome (11 hereditary non-polyposis colorectal cancer (HNPCC), 8 familial adenomatous polyposis (FAP)), and 19 (14 %) had inflammatory bowel disease (14 ulcerative colitis, 5 Crohn’s). Three patients had other cancers (brain, breast, and endometrial) and 20 % of patients had a family history of colorectal cancer outside of a defined syndrome. Overall, age-standardized 5-year survival was 66.8 % (stage I 100 %, stage II 76.5 %, stage III 63.0 %, and stage IV 0 %). Patients with genetic predisposition and inflammatory bowel disease had better 5-year survival when compared to the sporadic group (p = 0.025). Molecular profiles were available for 71 of the 77 sporadic cancers. All 71 tumors were microsatellite stable, and none had CpG island methylator phenotype. Twenty-three (32.4 %) were KRAS mutant. In our cohort, a family history of colorectal cancer, known hereditary colorectal cancer syndrome, and inflammatory bowel disease account for nearly half of all cases of young colorectal cancer. Prompt investigation of symptoms is essential in patients with Sporadic early-onset colorectal cancers, which appear to arise through the classical adenoma-to-carcinoma sequence.

Background Lateral internal sphincterotomy cures chronic anal fissure by preventing internal sphincter hypertonia. However, cutting sphincter predisposes to sphincter dysfunction, manifests as incontinence of gas, liquid, or stool. Surgeons, therefore, can be too cautious in its use, making ineffective superficial incisions or avoiding the operation altogether. This study is designed to confirm the role of redo lateral internal sphincterotomy in the treatment of surgically recurrent chronic anal fissure. Methods Patients undergoing repeat lateral internal sphincterotomy for surgically recurrent chronic anal fissure were accessed from a prospectively maintained database. Chronicity was defined by symptoms persisting more than 3 weeks. Contralateral sphincterotomy was performed with electrocautery through a stab incision over the intersphincteric plane. The length of sphincter division was the same as the length of the fissure. Phone questionnaire was administered and fecal continence was assessed by modified Cleveland Clinic Incontinence Score. Patients were asked to rank their overall satisfaction with the operation, and pre- and postoperative quality of life. Results There were 57 patients, 24 women and 33 men; mean age was 47.9 ± 14.8 years. Mean follow-up was 12.5 ± 4.2 years (range 6.2 to 25.2 years). Presenting symptoms included pain (100%), bleeding (80%), pruritus ani (39%), constipation (26%), and diarrhea. Fifty patients (90%) presented with 1 fissure, and 40 were posterior. Most procedures were performed on an outpatient basis. Fissure healing rate was 98%, and 2 patients (4%) developed minor incontinence postoperatively (one of gas, the other, gas and seepage). Overall satisfaction was 9.7 ± .9 out of 10 with a significant improvement in the quality of life from 5.7 ± 2.4 out of 10 to 9.3 ± 1.4 out of 10 (P Conclusion Judicious repeat lateral sphincterotomy cures recurrent chronic fissures with minimal risk of incontinence.

10 This guideline presents recommendations for the management of patients with hereditary gastrointestinal cancer syndromes. The initial assessment is the collection of a family history of cancers and premalignant gastrointestinal conditions and should provide enough information to develop a preliminary determination of the risk of a familial predisposition to cancer. Age at diagnosis and lineage (maternal and/or paternal) should be documented for all diagnoses, especially in fi rst- and second-degree relatives. When indicated, genetic testing for a germline mutation should be done on the most informative candidate(s) identifi ed through the family history evaluation and/or tumor analysis to confi rm a diagnosis and allow for predictive testing of at-risk relatives. Genetic testing should be conducted in the context of pre- and post-test genetic counseling to ensure the patient's informed decision making. Patients who meet clinical criteria for a syndrome as well as those with identifi ed pathogenic germline mutations should receive appropriate surveillance measures in order to minimize their overall risk of developing syndrome- specifi c cancers. This guideline specifi cally discusses genetic testing and management of Lynch syndrome, familial adenomatous polyposis (FAP), attenuated familial adenomatous polyposis (AFAP), MUTYH -associated polyposis (MAP), Peutz-Jeghers syndrome, juvenile polyposis syndrome, Cowden syndrome, serrated (hyperplastic) polyposis syndrome, hereditary pancreatic cancer, and hereditary gastric cancer.

Familial adenomatous polyposis (FAP) is a hereditary colon cancer syndrome that involves multiple extracolonic organs, including the thyroid. Several studies have estimated the rate of thyroid cancer in FAP to occur at five times the rate of the general population, but no current consensus defines screening for thyroid cancer in this cohort. This study seeks to define the features of benign and malignant thyroid disease in FAP patients, to compare thyroid cancer cases found through screening with those found incidentally, and to propose disease surveillance recommendations.Prospective screening for early thyroid cancer detection with thyroid ultrasound (US) was performed on FAP patients at the time of annual colonoscopy since November 2008. Clinical and US data were reviewed to characterize the observed thyroid nodules. Nonscreening-detected cases (NSD) were found through review of the colon cancer registry database.Eighteen NSD were found, compared with 15 screening-detected (SD) cases, out of 205 total patients screened (Mage=42 years; 55% female). The mean tumor size was larger in the NSD group than the SD group (p=0.04), and they tended to demonstrate more positive lymph nodes and more complications than the SD group. In the screened cohort, at least one thyroid nodule was detected in 106 (51.7%) patients, with 90% of these seen on initial exam. A total of 40/106 (37.7%) patients required fine-needle aspiration biopsy of a dominant nodule (Msize=14 mm), and 28/40 (70%) of these were performed at the first US visit. Suspicious US features were present in 16/40 (40%) patients, including five sub-centimeter nodules. Cytology and/or nodule US was abnormal in 15/205 screened patients, leading to surgery and revealing 14 papillary and one medullary thyroid cancer.Given the age and sex distribution of the screened cohort, this study reveals a higher-than-expected prevalence of both benign and malignant thyroid disease in the FAP population. Additionally, SD cases seemed to consist of smaller-sized cancers that required less radical therapy compared to NSD cases. Since it was found that the initial US in the screening program accounted for the majority of detected nodules (90%) and biopsies (70%), baseline and subsequent thyroid US surveillance is recommended in all FAP patients.

Hereditary colorectal cancer affects the rectum as well as the colon. Treatment strategies need to take into account the cancer risks posed by a retained rectum, and the threats to lifestyle caused by proctectomy. This chapter will discuss decision making in detail. Desmoid disease is a manifestation of the germline APC mutation that causes familial adenomatous polyposis. The relationship of desmoid disease to surgery in general and the rectum in particular will also be discussed.

Local recurrence of rectal cancer is the result either of potentially removable tumor cells left in situ or cells already disseminated to areas where surgery cannot reach them. The first scenario infers inadequate surgery, the second implies unfavorable biology. Surgeons who operate for rectal cancer must know local recurrence rates in their patients, and be able to relate them to outcomes achieved by others. We have performed this study to facilitate such a comparison. Methods: Systematic review of the literature from 1990 to 2010 was performed for publications which reported local recurrence after proctectomy for rectal cancer. Inclusion criteria were: studies of more than 80 patients and local recurrence stratified by histopathologic stage. Pooled local recurrence rates were tabulated by 5 percentile levels, stratified according to TNM stage (I,II,III) and surgical technique (total mesorectal excision or standard), as well as laparoscopic versus open. Results: Thirty-six studies comprising 16425 patients were pooled for final analysis: Mean follow-up is 40.9 months (1.3-188mths). The table shows local recurrence stratified by tumor biology (stage), operative technique (total mesorectal excision vs. standard) and operative approach (open vs. laparoscopic). The percentiles provide standards against which surgeons can compare their own outcomes Conclusion: Oncologic outcome of the treatment of rectal cancer is the result of interaction of therapeutic expertise and tumor biology. The percentile tables allow the use of local recurrence rates as an indirect parameter of surgical quality.

Restorative proctocolectomy is the procedure of choice for patients undergoing proctocolectomy for familial adenomatous polyposis or ulcerative colitis. This meta-analysis was designed to identify differences in adverse events and functional outcomes between these two groups. Studies published between 1986 and 2003 that compared outcomes between patients with familial adenomatous polyposis and ulcerative colitis were included. Meta-analytical techniques using random effect models were used to compare short-term and long-term adverse events as well as functional outcomes between the groups. Nineteen studies comprising 5,199 patients (familial adenomatous polyposis, 782; ulcerative colitis, 4,417) were analyzed. There were no significant differences in immediate postoperative adverse events between the two groups. Pouch-related fistulation was significantly increased in the ulcerative colitis group (10.5 percent vs. familial adenomatous polyposis 4.8 percent; odds ratio 2.31; P < 0.001). There was no significant difference in pouch failure between the two groups (ulcerative colitis 5.8 percent vs. familial adenomatous polyposis 4.5 percent; odds ratio 1.22; P = 0.43). The incidence of pouchitis was significantly greater in the ulcerative colitis group (30.1 vs. 5.5 percent; odds ratio 6.44; P < 0.001). Patients with familial adenomatous polyposis had a significant advantage in stool frequency with one less motion per 24 hours (95 percent confidence interval, 0.21–1.76; P = 0.01). In contrast to studies reporting similar outcomes for patients undergoing restorative proctocolectomy for familial adenomatous polyposis or ulcerative colitis, the present meta-analysis suggested that patients with ulcerative colitis are at greater risk of pouch-related fistulation and pouchitis. Although there was an increase in the 24-hour stool frequency in the ulcerative colitis group, this may be accounted for by the younger age at surgery in the familial adenomatous polyposis group.

Familial adenomatous polyposis (FAP) and hereditary nonpolyposis colorectal cancer (HNPCC) are the two most common forms of hereditary colorectal cancer (CRCA), representing approximately 5% of all CRCA. Endoscopic surveillance of at-risk colorectal mucosa is one of the key issues in the management of each of these diseases. In patients with FAP who are treated with total abdominal colectomy and ileorectal anastomosis, the risk of a metachronous rectal cancer is 4% to 10% at 10 years and increases with the duration of follow-up. After proctocolectomy with ileal pouch anal anastomosis, both the ileal pouch and the anal transition zone are at risk for adenomas and invasive adenocarcinoma. Patients with FAP need yearly proctoscopy or pouchoscopy, performed with a flexible endoscope. Increasing adenoma numbers, sizes, and dysplasia are indications to consider proctectomy or chemoprevention (in patients with a pouch). In patients with HNPCC who undergo segmental or subtotal colectomy, the risk for a second cancer developing in the remaining colon or rectum is in the range of 4% to 15%. These patients need yearly colonoscopy or proctoscopy (in patients with an IRA). In this chapter, details of technique and the management of metachronous neoplasia are discussed.

Controversy exists regarding the safety for omission of diverting ileostomy in restorative proctocolectomy because of fears of increased septic complications. This study was designed to evaluate the outcomes of restorative proctocolectomy in a consecutive series of patients by comparing postoperative complications, functional results, and quality of life in patients with and without diverting ileostomy.Data regarding demographics, length of stay, surgical characteristics, and complications were reviewed and recorded according to the presence (n= 1,725) or absence (n = 277) of a diverting ileostomy at the time of pelvic pouch surgery. Criteria for omission of ileostomy included: stapled anastomosis, tension-free anastomosis, intact tissue rings, good hemostasis, absence of airleaks, malnutrition, toxicity, anemia, and prolonged consumption of steroids. Functional outcome and quality of life indicators were prospectively recorded and compared.Patients in the ileostomy group had greater body surface area and older mean age at time of surgery, were taking greater doses of steroids preoperatively, and required more blood transfusions at the time of surgery compared with the one-stage (P0.05). There were no differences between the two groups in septic complications (P0.05). Early postoperative ileus was more common in the one-stage group (P0.001). There were no differences between the groups in quality of life and functional outcomes.For carefully selected patients undergoing restorative proctocolectomy with ileal pouch-anal anastomosis, omission of diverting ileostomy is a safe procedure that does not lead to an increase in septic complications or mortality. Quality of life and functional results are similar to those who undergo ileal pouch-anal anastomosis with diversion, provided that certain selection factors are considered.

This study was designed to evaluate the impact of childbirth on anal sphincter integrity and function, functional outcome, and quality of life in females with restorative proctocolectomy and ileal pouch-anal anastomosis. The patients who had at least one live birth after ileal pouch-anal anastomosis were asked to return for a comprehensive assessment. They were asked to complete the following questionnaires: the Short Form-36, Cleveland Global Quality of Life scale, American Society of Colorectal Surgeons fecal incontinence severity index, and time trade-off method. Additionally, anal sphincter integrity (endosonography) and manometric pressures were measured by a medical physician blinded to the delivery technique. Anal sphincter physiology also was evaluated with electromyography and pudendal nerve function by nerve terminal motor latency technique. Of 110 eligible females who had at least one live birth after ileal pouch-anal anastomosis, 57 participated in the study by returning for clinical evaluation to the clinic and 25 others by returning the quality of life and functional outcome questionnaires. Patients were classified into two groups: patients who had only cesarean section delivery after ileal pouch-anal anastomosis (n = 62) and patients who had at least one vaginal delivery after ileal pouch-anal anastomosis (n = 20). The mean follow-up from the date of the most recent delivery was 4.9 years. The vaginal delivery group had significantly higher incidence of an anterior sphincter defect by anal endosonography (50 percent) vs. cesarean section delivery group (13 percent; P = 0.012). The mean squeeze anal pressure was significantly higher in the patients who had only cesarean section delivery (150 mmHg) after restorative proctocolectomy than patients who had at least one vaginal delivery (120 mmHg) after restorative proctocolectomy (P = 0.049). Quality of life evaluated by time trade-off method also was significantly better in the cesarean section delivery group (1) vs. vaginal delivery group (0.9; P < 0.001). The risk of the sphincter injury and quality of life measured by time trade-off method are significantly worse after vaginal delivery compared with cesarean section in patients with ileal pouch-anal anastomosis. In the short-term, this does not seem to substantially influence pouch function or quality of life; however, the long-term effects remain unknown, thus obstetric concern may not be the only factor dictating the type of delivery in this group of patients. A planned cesarean section may eliminate these potential and factual concerns in ileal pouch-anal anastomosis patients.

The technology of the seven-minute scientific presentation has changed greatly with the universal acceptance of computerized slides and graphics. We performed this study to see whether the quality of delivery has improved pari passu with technical advances in presentation of data.In 1993 and in 2003 a sample of the podium presentations given at The American Society of Colon and Rectal Surgeons annual scientific meeting was analyzed. Number of slides, type of slides, time of presentation, and quality of presentation were recorded. Quality was scored by the presence of poor presentation techniques such as presenting too fast for the slide to be comprehended, presenting data that were different to what was on the slide, using fonts that were too small to be read, and reading the exact wording of the slide. One point was awarded for each transgression and for any talk lasting more than 7.9 minutes; the higher the score, the worse the presentation.Thirty-eight presentations were analyzed from the 1993 meeting in Orlando and 40 were analyzed from the 2003 meeting in New Orleans. There was no difference in mean time of the presentation (1993, 7.5 minutes +/- 1.5 standard deviation vs. 2003, 7.2 minutes +/- 0.7 standard deviation), in mean number of slides used per presentation (1993, 16.4 +/- 3.3 standard deviation vs. 2003, 17.1 +/- 5.4 standard deviation), or in slides per minute (1993, 2.3 +/- 0.8 standard deviation vs. 2003, 2.5 +/- 1.0 standard deviation). Presentation technique was much worse in 1993, however, with a mean quality score of 2.2 +/- 1.1 standard deviation vs. 0.8 +/- 0.9 standard deviation in 2003 (P0.001). The most common fault in both years was presenting a slide too rapidly, a fault more pervasive in 1993 than in 2003 (1993, 30/38 presentations vs. 2003, 16/40). Only ten presentations went over the allotted time in 2003, by an average of one minute. In 1993, 18 presentations were too long, by an average of 1.8 minutes each.The basic structure of the seven-minute podium presentation has changed little over the last ten years, despite major advances in technology. The most notable improvements have been in the technique of delivering the talk.

Background Small intestinal (SI) surveillance is recommended for polyposis patients. The utility and safety of capsule endoscopy (CE) for surveillance of SI neoplasia in patients with familial adenomatous polyposis (FAP) and Peutz-Jeghers syndrome (PJS) is unknown. Methods CE was offered to consecutive FAP and PJS patients due for routine upper endoscopic surveillance. The prevalence, location (jejunum, ileum), size (1-5 mm, 6-10 mm, >10 mm) and number (1-5, 6-12, >20) of polyps detected by CE was assessed. Results 19 subjects (15 FAP/4 PJS) with a mean age of 43 were included. All subjects had previous intestinal surgery. No complications occurred with CE. CE in FAP: 9/15 (60%) of subjects with FAP had SI polyps. The prevalence of SI polyps was related to the duodenal polyposis stage and subject age. The location, size and number of polyps progressed as duodenal polyposis stage advanced. CE in PJS: 3/4 (75%) of subjects with PJS had SI polyps. The polyps were diffuse in 2/4 and only in the ileum in one subject. CE findings led to laparotomy with intra-operative endoscopic polypectomy in two PJS patients. Conclusion SI polyps are common in FAP but their importance is unknown. CE should be performed in FAP patients with stage III and IV duodenal disease. Clinically significant polyps are commonly detected by CE in PJS and lead to change in management in 50% of PJS subjects. CE should replace radiographic SI surveillance for PJS patients. CE is safe in polyposis patients who have undergone major intestinal surgery.

Frequent loose stools test the integrity of sphincter function in patients undergoing ileal pouch-anal anastomosis. The authors hypothesized that women with anal sphincter defects were more likely to experience incontinence episodes than women with intact sphincter muscles following ileal pouch-anal anastomosis. From 1996 to 1998, 42 women with a mean age of 42 (range, 22–63) years were prospectively evaluated by anorectal manometry and endoanal ultrasound before pouch surgery. Forty women underwent a stapled ileal pouch-anal anastomosis and two underwent a handsewn anastomosis. All patients considered themselves continent of stool before the procedure. A postoperative survey including the Cleveland Clinic Florida scale, Fecal Incontinence Severity Index, and Fecal Incontinence Quality of Life scale was sent to study participants. Nineteen women with an obstetrical history had significant sphincter defects associated with significant lower mean resting pressure, mean squeeze pressure, and shorter anal canal length (3 vs. 3.7 cm, P = 0.0007). Thirty-five women (83 percent) responded resulting in a mean follow-up of 62 (range, 49-72) months. Fourteen responders (mean age, 46 years) had sphincter defects but no significant difference was found in Cleveland Clinic Florida scale, Fecal Incontinence Severity Index, or Fecal Incontinence Quality of Life scale scores when compared with those without defects. Although almost all women reported episodes of seepage, marked sphincter defects associated with low anal pressures and shorter anal canal length did not affect anal function following pouch surgery. This study supports the findings that continent women with significant sphincter defects on ultrasound evaluation may be considered for restorative proctocolectomy.

Family history of colorectal cancer is associated with an increased risk for the disease, although there are many combinations of family history that are hard to correlate with risk status. A scoring system for family history of colorectal cancer was designed to make risk more readily quantifiable. A colonoscopy database was used to test the following points system: each first-degree relative with colorectal cancer = 3 points; each second-degree relative with colorectal cancer = 1 point. Families with one or more first-degree relative affected under 50 years of age = an extra 3 points. Families with one or more second-degree relative affected under 50 years of age = an extra 1 point. Families with multiple relatives on the same side of the family = an extra 3 points (first-degree relatives), 1 point (second-degree relatives), or 2 points (first-degree and second-degree relatives). Points were added and categories defined as follows: low risk, 1 to 4 points; medium risk, 5 to 7 points; high risk, 8 to 10 points; very high risk, >10 points. A control group of average-risk patients having screening colonoscopy was used. Categories were compared in number of adenomas, hyperplastic polyps, and cancers. The records of 992 patients were used to test the system. Mean adenomas per patient per group were 0.4 for controls, 1.0 for low risk, 1.0 for medium risk, 1.7 for high risk, and 1.7 for very high risk. Cancers per group were 2 of 196 for controls, 8 of 513 for low risk, 3 of 171 for medium risk, 3 of 84 for high risk, and 1 of 28 for very high risk. The score categories were combined to produce revised risk levels of low (score 1 to 7) and high (>7). Average adenomas per patient in the revised categories were 0.4 (control), 1.0 (low risk), and 1.7 (high risk). The odds ratio of having one to two adenomas was 1.73 (1.19–2.50, 95% confidence limits) in the low-risk group and 2.39 (1.41–4.01) in the high-risk group. Odds ratios for having three or more adenomas were 5.70 (2.44–13.32) in the low-risk group and 10.35 (3.97–26.97) in the high-risk group. In the two-category system proposed here of quantifying familial risk of colorectal cancer, patients having less than 8 points were at low risk and those with 8 or more were at high risk. Surveillance and chemoprevention protocols can be designed through use of these risk categories. A scoring system for family history of colorectal cancer can make risk assessment easier and facilitate both collaborative studies and patient triage into appropriate screening programs.

The colonic J-pouch has been used to improve bowel function in patients undergoing low colorectal or coloanal anastomosis. However, a narrow pelvis, difficulties in reach, a long anal canal with prominent sphincters, or a fatty mesentery may turn this technique into a technically challenging procedure in certain patients. In these circumstances, “coloplasty” offers an alternative to a straight anastomosis. The purpose of this study was to compare the quality of life, functional outcome, and complications between patients undergoing coloplasty, colonic J-pouch, or straight anastomosis. Altogether, 162 patients who underwent coloanal or low colorectal anastomosis between 1998 and 2001 were studied. Data collected included demographics, length of follow-up, technique and type of anastomosis, complications, quality of life, and functional outcome. Results were analyzed according to use of a coloplasty (n = 69), colonic J-pouch (n = 43), or straight anastomosis (n = 50). The choice of the technique was based on the surgeon’s preference. Usually coloplasty or straight anastomosis was favored in male patients with a narrow pelvis or when a handsewn anastomosis was used. Quality of life assessment with the short form-36 questionnaire revealed better scores in coloplasty and colonic J-pouch groups. The coloplasty (1.0 ± 1.7) and colonic J-pouch (1.0 ± 1.2) groups had fewer night bowel movements than the straight anastomosis group (1.5 ± 2.0) (P < 0.05). The coloplasty group also had fewer bowel movements per day than the straight anastomosis group (3.8 ± 2.9 vs. 4.8 ± 3.6; P < 0.05); also, less clustering and less antidiarrheal medication use were observed than in the straight anastomosis group. Colonic J-pouch patients with handsewn anastomosis had a higher anastomotic leak rate (44 percent) than the patients in the coloplasty with hand-sewn anastomosis group (3.6 percent). Coloplasty seems to be a safe, effective technique for improving the outcome of low colorectal or coloanal anastomosis. It is especially applicable when a colonic J-pouch anastomosis is technically difficult.

Juvenile polyposis syndrome is characterized by multiple hamartomatous polyps in the large intestine. When indicated, the surgical choices in symptomatic juvenile polyposis syndrome patients are colectomy with ileorectal anastomosis or proctocolectomy with pouch. The aim of this study was to evaluate the long-term outcomes of the surgical options in juvenile polyposis syndrome patients who present with symptomatic colonic polyps. The charts of all juvenile polyposis syndrome patients who had had at least one colonic operation since 1953 in our institution were reviewed. The following data were abstracted: demographics, the number and site of the polyps, symptoms, the intervals and types of the colonic operation, follow-up, and the patients’ current status. There were 13 patients (6 males) with a median age of 10 years (range, 1–50 years) at the time of diagnosis. Patients had colonic (n = 13), rectal (n = 12), and gastric (n = 6) polyps. Rectal bleeding (n = 11) was the most common presenting symptom. Three patients underwent proctectomy as the initial operation. Although a rectum-preserving operation was initially performed in ten patients, a subsequent proctectomy was required in five of them within a median of 9 years (range, 6–34 years). Therefore, eight patients had their rectum removed during the study period; five had an ileal pouch–anal anastomosis, one had a Koch pouch as a restorative surgery, and two had an end ileostomy. No relation was observed between the number of colonic and rectal polyps and the type of surgery or the need for proctectomy. Patients were followed up a median of 3 years (range, 2–24 years) after their ultimate operations. During this period, one patient (20 percent) who underwent restorative proctectomy and 4 patients (80 percent) whose rectums were preserved required multiple endoscopic polypectomies for recurrent polyps in the pouch (first patient) or their rectums (the other four patients). The patient who underwent the Koch procedure required surgery for recurrent polyps in her pouch. One-half of the patients who initially underwent rectal preservation required subsequent proctectomy. The number of colonic or rectal polyps does not influence the choice of the surgical procedure. Both restorative proctocolectomy and subtotal colectomy with ileorectal anastomosis need endoscopic follow-up because of the high recurrence rates of juvenile polyps in the remnant rectum or pouch.

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. 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. 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. 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.

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.

The prevalence of familial adenomatous polyposis-associated cancers in the United States has been conservatively estimated at 15,000, and that of hereditary nonpolyposis colorectal cancer (HNPCC)-associated cancers at 30,000. Every blood descendant of each of these patients is at risk of carrying a germline mutation predisposing them to early onset colorectal and other cancers. Optimal care of these high-risk families involves a center with expertise in the syndromes. This study was performed to see how many such centers exist in the United States and to learn something of how they work. The mailing lists of three international societies concerned with inherited colorectal cancer were used to send surveys inquiring about the presence of a registry or center, and how that center worked. The Collaborative Group of the Americas, the Leeds Castle Polyposis Group, and the International Collaborative Group on hereditary nonpolyposis colorectal cancer were queried. There were 30 centers in the United States: 26 responded, representing 15 states. Eighteen centers that responded had registries for inherited colorectal cancer. There were 1,396 familial adenomatous polyposis families among the 18 registries, 2,058 hereditary nonpolyposis colorectal cancer families, 42 with juvenile polyposis, and 216 with Peutz-Jehger’s syndrome. The 18 registries employed 29 genetic counselors or coordinators. Seven used Microsoft Access™ as a database, five used Progeny™, three a SQL server-based system, one Filemaker Pro™, one Microsoft Excel™ and one used Oracle™. Cyrillic™ was the pedigree-drawing program in 6 registries, Progeny™ in 12, and Ped Draw™ in 1. Hereditary nonpolyposis colorectal cancer was defined using the Amsterdam I criteria by four registries, Amsterdam II by five, both criteria by six, Bethesda guidelines by one, and by genotype alone in two registries. The United States is underserved by registries for inherited colorectal cancer, having enrolled only a small proportion of the families theoretically available. Registries differ in fundamental aspects of function. More collaboration and more registries are needed.

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. 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. 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). 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.

Background: Large colonic polyps present a particular challenge to endoscopists because of the risks of significant haemorrhage, perforation, inadequate polypectomy, or trying to snare an unrecognized cancer. The alternative to endoscopic therapy of large polyps is surgical resection and although minimally invasive techniques are available, risks are significant. Although neither surgery nor endoscopy is a perfect way of treating large colonic polyps, endoscopic resection is usually tried first. Most series of endoscopic polypectomies are small, include both rectal and colonic polyps and have varying size and shape criteria. The purpose of the present study is to describe a large consecutive series of colonic polyps evaluated endoscopically, to determine the chances of performing a safe, effective endoscopic polypectomy. Methods: All colonic polyps>20 mm in maximum dimension assessed during colonoscopy from 1989 to 2002 were reviewed. Rectal polyps were excluded. Demographic data for the patients were abstracted, as were data regarding the outcomes of polyp assessment and treatment. Primary end-points were: the need for surgical resection, the incidence of postpolypectomy complications and the persistence of the index polyp at follow up. Independent variables included the endoscopically assessed size of the polyps, the year in which the polypectomy took place, the shape of the polyps and their location within the colon. Results: During the period under review 311 large polyps were removed from 252 different patients. Of these, 263 polyps were removed endoscopically and 48 polyps were removed surgically. An additional 18 endoscopically removed polyps ultimately needed surgery for recurrence or malignancy. There were no deaths but 19 complications of endoscopic polypectomy (17 late haemorrhage and two postpolypectomy syndrome). At first follow up, 22% of polyps had persisted, this decreased to 14% at second follow up and 7% at third. Complications were more common in right sided polyps and in flat or sessile lesions. Pedunculated polyps never persisted or recurred and had the lowest rate of surgery. Larger polyps had higher rates of advanced histology, complications, polyp persistence and the need for surgery. Conclusions: Polyp size, location and shape influence the results of endoscopic resection of large colonic polyps. Polyps>30 mm in maximum diameter are significantly more advanced histologically but also significantly more difficult to treat successfully than those

PURPOSE: Colonic polyps are sometimes difficult to remove endoscopically and are referred for surgical resection. This study was performed to determine how many polyps referred for surgery could actually be managed endoscopically. METHODS: An endoscopic database with data entered prospectively and consecutively was used to identify patients referred for surgery for a colonic polyp. Rectal polyps were excluded. All patients underwent colonoscopy before surgery to see if the polyp could be managed endoscopically. Cases were reviewed to see the method and outcome of treatment. RESULTS: The study population consists of 58 patients referred for surgical resection of a colorectal polyp. Endoscopic polypectomy was initially successful in 48. Five of the 48 needed surgery later for a final success rate of 43/58 avoiding surgery. There were no deaths, four complications of endoscopic polypectomy (three bleeds, one post-polypectomy syndrome) and two patients had complications of surgery (one splenic injury, one ventral hernia). Polyps ranged in size from 1.5 cm to 8.0 cm. Seven polyps contained invasive cancer (three needing surgical resection), eight contained intramucosal cancer (one operated) and 11 had severe dysplasia (three operated). Rate of persistent polyp was 16/37 at first follow up, 7/23 at second, 1/14 at third and 0/8 at fourth. CONCLUSION: Most polyps referred for surgical resection were successfully managed endoscopically. Patients with colonic polyps that are difficult or potentially dangerous to remove endoscopically should be sent for a second opinion before surgery is performed.

PURPOSE: Restorative proctocolectomy with ileal pouch-anal anastomosis is accepted as the surgical treatment of choice for many patients with familial adenomatous polyposis. The risk of cancer developing in the ileal pouch after this surgery is unknown. Cancer may arise from the ileal pouch after restorative proctocolectomy, but that arising from the anal transitional zone has not been documented in familial adenomatous polyposis. We report two cases of this cancer from the anal transitional zone in patients with familial adenomatous polyposis, with a review of the literature. METHODS: All patients with familial adenomatous polyposis treated with restorative proctocolectomy and ileal pouch-anal anastomosis in The Cleveland Clinic were included in the study. Patients whose surveillance biopsy of the anal transitional zone revealed invasive adenocarcinoma were studied. RESULTS: Among a total of 146 patients with familial adenomatous polyposis who underwent restorative proctocolectomy and ileal pouch-anal anastomosis from 1983 to 2001 in our institution, none developed cancer of the anal transitional zone at up to 18 years of follow-up. However, there were two patients, both of whom underwent surgery elsewhere but who were followed up here, who developed invasive adenocarcinoma of the anal transitional zone. In one of them, cancer was diagnosed three years after a double-stapled ileal pouch-anal anastomosis, whereas in the other, cancer occurred eight years after a straight ileoanal anastomosis with mucosectomy. CONCLUSIONS: Cancer may develop in the anal transitional zone after restorative proctocolectomy with ileal pouch-anal anastomosis for familial adenomatous polyposis. Long-term surveillance of the anal transitional zone needs to be emphasized.

PURPOSE: Involvement of the urinary tract by colorectal cancer is sufficiently rare to be encountered by an individual surgeon on an infrequent basis. The aim of this review is to highlight technical and oncologic issues that should be considered when dealing with complex colorectal cancer that involves the urinary tract. METHODS: The relevant literature from 1975 to 2001 was identified using the MEDLINE database of the U.S. National Library of Medicine and reviewed. Because of the diversity of forms of presentation of urologic involvement, few randomized, controlled trials are available, with most evidence derived from retrospective studies. RESULTS: Three distinct clinical situations in which the urinary tract may be affected by colorectal cancer were identified: involvement by primary colorectal cancer, involvement by recurrent cancer, and unexpected intraoperative findings of urinary tract involvement. Management strategies to identify and treat locally advanced primary or recurrent colorectal cancer involving the urinary tract improve survival with acceptable morbidity and mortality. Careful preoperative assessment of all patients with colorectal cancer will reduce unexpected identification of urinary tract invasion at the time of surgery. In patients in whom cure is not possible, endourologic techniques combined with judicious surgical resection can provide high-quality palliation. Optimal care of many of these conditions is facilitated by specialist urologic advice. CONCLUSIONS: The wide spectrum of possible urinary tract involvement by colorectal cancer requires individual patient-specific and disease-specific consideration. The literature offers important guidelines that aid decision making and improve management of these challenging problems.

PURPOSE: One of the concerns with colectomy and ileorectal anastomosis as a prophylactic procedure for patients with familial adenomatous polyposis is the risk of metachronous rectal cancer, estimated at from 12 to 43 percent. These estimates are based largely on surgeries performed at a time when the only alternative option to ileorectal anastomosis for patients with severe familial adenomatous polyposis was proctocolectomy and ileostomy. This study was designed to test the hypothesis that in the pouch era severe polyposis is now treated by proctocolectomy and ileal pouch-anal anastomosis. Ileorectal anastomosis is performed mostly in mildly affected patients and will therefore carry a very low risk of metachronous rectal cancer. METHODS: Patients undergoing primary prophylactic surgery for familial adenomatous polyposis between 1950 and 1999 were categorized according to the year of their surgery: prepouch era (before 1983) or pouch era (after 1983). Patients undergoing colectomy and ileorectal anastomosis were the focus of the study, and rate of proctectomy and the incidence of rectal cancer were recorded for each group. Data on the severity of the polyposis for each group were abstracted. RESULTS: A total of 197 patients underwent ileorectal anastomosis, 62 in the prepouch era (median follow-up, 212 months; interquartile range, 148 months) and 135 in the pouch era (median follow-up, 60 months; interquartile range, 80 months). Patients in the prepouch era came to surgery at the same median age as those in the pouch era (median age 23.0 years, interquartile ranges 15.5 years for prepouch and 17 years for pouch). Similar proportions of patients in the prepouch era had severe polyposis (49 percent) as in the pouch era (44 percent), although all severely affected patients had an ileorectal anastomosis in the prepouch era vs. 39 percent in the pouch era. Twenty (32 percent) prepouch-era patients underwent proctectomy compared with three (2 percent) pouch-era patients. No pouch-era patient had rectal cancer on follow-up; eight (12.9 percent) prepouch-era patients did. CONCLUSION: Although follow-up is shorter, ileorectal anastomosis for familial adenomatous polyposis performed since 1983 carries a much lower rate of rectal cancer and proctectomy than ileorectal anastomosis performed before this time, when restorative proctocolectomy was not an option. This is related, at least in part, to a greater number of patients with severe polyposis having their rectum initially spared.

OBJECTIVE To evaluate how age affects functional outcome and quality of life after ileal pouch anal anastomosis (IPAA). SUMMARY BACKGROUND DATA Because of the limited number of older patients undergoing IPAA, it has been difficult to assess functional outcome and quality of life stratified by age. METHODS IPAA was performed in 1895 patients. Patients were stratified by age into

PURPOSE: The purpose of this article is to review the different aspects of the colonic J-pouch reconstruction with special focus on functional results and complications METHODS: A MEDLINE search from 1965 to the present with manual search for older articles was used as the basis of this review. RESULTS: Rectal reconstruction using a colonic J-pouch-anal anastomosis after excision of extraperitoneal cancers can be performed with acceptable morbidity and good functional results. CONCLUSIONS: Among the different methods of rectal reconstruction now available after total rectal resection for cancer, the colonic J-pouch may be the optimal choice.

The effect of surgery on quality of life (QOL) in the early postoperative period is important in Crohn's disease because of the multiple surgical procedures that patients undergo and the acute QOL benefits that might occur as a result of modifications of medical treatment. Earlier studies of the effect of surgery on QOL have been retrospective and assessed changes 3 to 24 months after surgery. This study prospectively assesses the effect of surgery on QOL in the early postoperative period.Patients requiring surgical management of sequelae of Crohn's disease were obtained from a prospectively entered database including data on QOL. Preoperative and 30-day postoperative QOL were determined in 82 patients using Cleveland Global Quality of Life (CGQL) scores (range from 0 [worst] to 10 [best possible] QOL). Preoperative and postoperative scores were compared using a paired t-test to determine the significance of any change in QOL after surgery. The effect of other variables on change in QOL after surgery was assessed using the t-test or analysis of variance. Multifactor analysis of variance was used to assess the effect of several independent variables.Eighty-two patients (41 women) of 142 patients who had had surgery (58%) had complete preoperative and 30-day postoperative scores. The incidence of complications was 23% (11% were major). There was a significant improvement in QOL 30 days after surgery as measured by CGQL (0.6 +/- 0.2 preoperative to 0.7 +/- 0.2 postoperative; mean +/- SD; p0.001). The mean preoperative CGQL was 0.56 +/- 0.24 and the mean improvement was 0.11 +/- 0.20 toward a better QOL. Female patients (p0.05) and those who did not develop complications within 30 days of surgery (p0.05) had a significantly greater improvement in CGQL after surgery than other groups. No other factor was predictive of improved outcomes.QOL as measured by CGQL improves early after surgery (30 days postoperatively). Improvement in CGQL is greater in female patients and patients who do not develop complications in the postoperative period. It is not affected by other patient characteristics, nature of disease, indication, or procedure performed. Most patients who undergo surgery for Crohn's disease feel that surgery has helped them and would undergo surgery again.

INTRODUCTION: The phenotype of hereditary nonpolyposis colorectal cancer includes an 80 percent lifetime risk of colorectal cancer, a predominance of lesions proximal to the splenic flexure, and a high incidence of synchronous and metachronous neoplasia. Although prophylactic colectomy is rarely advised for patients with a hereditary nonpolyposis colorectal cancer genotype and a normal colon, the presence of advanced neoplasia in the context of a qualifying family history or a hereditary nonpolyposis colorectal cancer genotype has led to such recommendations. We performed this study to document the patterns of colorectal surgery performed for cancer-bearing patients who are part of an Amsterdam criteria–positive family and to compare rates of metachronous cancers that follow index total or segmental colectomy. METHODS: Family trees fulfilling the classic Amsterdam criteria for hereditary nonpolyposis colorectal cancer were identified, and all patients for whom surgical and pathology records were available were included in the study. Type of surgery and the outcome of subsequent follow-up were abstracted. Patients were divided into those treated at the Cleveland Clinic and those treated elsewhere. RESULTS: There were 39 families with 93 affected patients. These patients had 127 colorectal cancers, 76 (60 percent) of which were right sided (proximal to the splenic flexure). Median age at diagnosis of the index cancer was 47 (range, 26–81) years. Sixteen patients (17 percent) had metachronous cancers and multiple surgeries, whereas four (4 percent) had synchronous cancers. Median follow-up for patients who underwent surgery at the Cleveland Clinic was 13 (range, 4–49) years, whereas that for those who underwent surgery elsewhere was 14 (range, 1–42) years. Sixteen (48 percent) of the 33 patients who underwent surgery at the Cleveland Clinic had a total colectomy vs. 7 (12 percent) of the 60 who had surgery elsewhere (Fisher’s exact test, P < 0.001). Only one patient who had surgery at the Cleveland Clinic had a second operation for a metachronous cancer (1/17 patients having a segmental resection). Fifteen patients who underwent surgery elsewhere needed a second resection for metachronous cancer (15/53 patients having a segmental resection; Fisher’s exact test, P = 0.094). CONCLUSION: We conclude that there is high risk of metachronous colorectal cancer if an index cancer in a hereditary nonpolyposis colorectal cancer patient (defined according to Amsterdam criteria) is treated by partial colectomy. However, this risk can be lowered by performing a total colectomy at the time of index surgery, or possibly by effective postoperative surveillance.

INTRODUCTION: Historically, rectal cancer with transmural spread and/or lymph node involvement has presented a major challenge to surgeons, with a variable and often high risk of local recurrence and poor survival outcomes. In recent years a large amount of literature has focused attention on the importance of surgical technique, tumor staging, and the optimal integration of CT and radiation therapy. METHODS: This article reviews the clinical trials that have defined the current approach to rectal cancer, the controversies regarding what should be considered the standard of care, and the ongoing clinical studies that will resolve some of these issues. RESULTS: The preoperative staging of rectal cancer can be improved with the use of endorectal ultrasound and (where available) magnetic resonance imaging. Careful pathologic analysis, particularly of the radial margin, provides important prognostic information that enables better allocation of postoperative care. Although both radiation therapy and CT have a proven role in adjuvant therapy, the interpretation of many studies is confounded by unacceptably poor outcomes in the control arm, and in older studies the use of inferior chemotherapy and radiation therapy techniques. Ongoing studies will better define the optimal combination and timing of chemotherapy and radiation therapy, with respect to both toxicity and survival endpoints. CONCLUSIONS: A combined modality approach to rectal cancer, integrating the colon and rectal surgeon, pathologist, medical oncologist, and radiation oncologist, is necessary to achieve optimal outcomes. The achievements to date and the ongoing vigorous debates regarding standard care continue to highlight the importance of quality ongoing research in a rapidly changing clinical environment.

INTRODUCTION: Insufflation of air is a cause of discomfort during and after colonoscopy. Although this can be minimized by good technique, the use of carbon dioxide insufflation may provide further benefits. Carbon dioxide is rapidly absorbed and excreted through the lungs. We hypothesized that carbon dioxide would alleviate postcolonoscopy discomfort. METHODS: After they had provided informed consent, patients presenting for colonoscopy were randomized into two groups: those in whom air was used for colonoscopy and those in whom carbon dioxide was used. Pain during and ten minutes after colonoscopy was measured on a ten-point analog scale. Data are mean and 95 percent confidence limits. RESULTS: There were 124 patients in the air group and 123 in the carbon dioxide group. Age, body mass index, indication, diagnosis, and number of procedures were similar for the two groups. There were no differences between the groups in the amounts of sedation or analgesia used, the percentage of examinations that were complete (air, 98.4 percent; carbon dioxide, 95.2 percent), or patient satisfaction (on a scale of 1 to 10: air, 9.4; carbon dioxide, 9.5). Although there were more females in the carbon dioxide group (69 vs. 51), hysterectomy rates were the same. Pain scores (mean ± 95 percent confidence interval; scale of 1 to 10) immediately after the examination were 4.3 ± 0.3 for air and 3.6 ± 0.3 for carbon dioxide (no significant difference). Pain scores 10 minutes later were 2.1 ± 0.2 for air and 0.9 ± 0.2 for carbon dioxide (P < 0.05, Student’s t-test). CONCLUSION: Because there was significantly less abdominal pain ten minutes after colonoscopy in the group in whom carbon dioxide was used, carbon dioxide should be considered as an insufflating gas for colonoscopy.

PURPOSE: Stapling of the ileal pouch-anal anastomosis with preservation of the anal transitional zone remains controversial because of concerns about the potential risk of dysplasia and cancer. The natural history and optimal treatment of anal transitional zone dysplasia ten or more years after surgery are unknown. This study establishes the risk of dysplasia in the anal transitional zone and the outcome of a conservative management policy for anal transitional zone dysplasia, with a minimum of ten years' follow-up after ileal pouch-anal anastomosis. METHODS: A total of 289 patients undergoing anal transitional zone-sparing stapled ileal pouch-anal anastomosis for inflammatory bowel disease between 1986 and 1990 were studied. Patients undergoing anal transitional zone-sparing ileal pouch-anal anastomosis who were studied with serial anal transitional zone biopsies for at least ten years postoperatively were included (n = 178). Median follow-up was 130 (range, 120-157) months. RESULTS: Anal transitional zone dysplasia developed in 8 patients 4 to 123 (median, 9) months after surgery. There was no association with gender, age, preoperative disease duration, or extent of colitis, but the risk of anal transitional zone dysplasia was significantly associated with cancer or dysplasia as a preoperative diagnosis or in the proctocolectomy specimen. Dysplasia was high grade in two patients and low grade in six. Two patients with low-grade dysplasia on two or more occasions after detection of low-grade dysplasia underwent completion mucosectomy and perineal pouch advancement with neo-ileal pouch-anal anastomosis. One patient with high-grade dysplasia on two occasions was to undergo completion mucosectomy, but this was not technically feasible. Partial mucosectomy with vigorous anal transitional zone biopsy was performed with close postoperative surveillance. Biopsies were negative for dysplasia. The second recently diagnosed patient with high-grade dysplasia underwent examination under anesthesia with negative anal transitional zone biopsies and will be kept under close surveillance. No cancer in the anal transitional zone was found during the study period. The 4 other patients with low-grade dysplasia on 1 or 2 occasions were treated expectantly and have been dysplasia free for a median of 119 (range, 103-133) months. CONCLUSIONS: Anal transitional zone dysplasia after stapled ileal pouch-anal anastomosis is infrequent and is usually self-limiting. Anal transitional zone preservation did not lead to the development of cancer in the anal transitional zone with a minimum of ten years of follow-up. Long-term surveillance is recommended to monitor dysplasia. If repeat biopsy confirms persistent dysplasia, mucosectomy with perineal pouch advancement and neo-ileal pouch-anal anastomosis is recommended.

PURPOSE: The aim of this retrospective study was to determine which aspects of tumor morphology and histology influenced the incidence of local recurrence after curative resection of colonic adenocarcinoma. METHODS: Patients who had a curative resection for a primary colonic adenocarcinoma between 1980 and 1993 (inclusive) were identified from the colorectal cancer database in the Department of Colorectal Surgery. The charts of patients diagnosed with a local recurrence were then reviewed and their findings at operation and histologic assessment analyzed. Patients were followed up for at least five years or until death. RESULTS: Over the period of study, 1,031 patients had a curative resection for colonic adenocarcinoma. Local recurrences were detected in 32 patients (3.1 percent). The gender distribution of patients with local recurrence was 18 males (56.3 percent) and 14 females (43.7 percent) with a mean age of 63.4 years. The median time to local recurrence was 13 (range, 2–71) months. The distribution of primary tumors that recurred locally favored the cecum (n = 9; 28.1 percent) and sigmoid colon (n = 14; 43.7 percent) over other locations; these were, however, the most common sites of primary lesions. Less common sites included the ascending colon (n = 0; 0 percent), hepatic flexure (n = 2; 6.3 percent), transverse colon (n = 1; 3.1 percent), splenic flexure (n = 3; 9.4 percent), and descending colon (n = 3; 9.4 percent). Of the total number of tumors, 101 were found to be adherent to at least 1 other intra-abdominal viscus, and 12 (11.9 percent) recurred locally. Other factors associated with local recurrence were tumor perforation and fistulation. Overall, 30 tumors (2.9 percent) were perforated, and 6 (20 percent) recurred locally. Four tumors (0.4 percent) were fistulating; of these, 2 (50 percent) recurred locally. Advanced tumor stage was also associated with an increased rate of local recurrence (Stage I, 0 percent; Stage II, 2.05 percent; Stage III, 7.0 percent; and Stage IV, 6.1 percent). Similarly, tumor differentiation was related to local recurrence, with no instances in well-differentiated tumors, 2.8 percent in moderately differentiated tumors, and 6.8 percent in poorly differentiated tumors. CONCLUSIONS: The location of the primary tumor is not a factor in producing local recurrence. Fixity to another viscus, perforation or fistulation, advanced stage of disease, and differentiation of tumor appear to increase the chances of recurrence of curatively resected colonic carcinoma. Although the recurrence rate is higher in these groups than for tumors overall, definitive oncologic surgery prevents recurrence in the majority of cases. No colonic tumor that was T1 or T2 (N0, N1, or N2) or that was well differentiated recurred locally.

Quality in the technical performance of colonoscopy and the continuous quality improvement process for colonoscopy: recommendations of the U.S. Multi-Society Task Force on Colorectal Cancer

PURPOSE: As an alternative to resection, strictureplasty may allow for preservation of intestinal length and avoidance of short-bowel syndrome in patients with diffuse Crohn's jejunoileitis. However, the long-term durability of the procedure and its safety have not been confirmed. The purpose of this study was to report our experience with strictureplasty for diffuse Crohn's jejunoileitis. METHODS: Between 1984 and 1999, 123 patients underwent a laparotomy that included an index strictureplasty for diffuse jejunoileitis. Patient history, operative details, and postoperative morbidities were obtained by chart review. Nineteen patients (15 percent) were receiving total parenteral nutrition for short-bowel syndrome, and 81 (66 percent) were taking chronic steroids. Total number of strictureplasties performed was 701 (median, 5/patient). Seventy percent of patients underwent a synchronous bowel resection. Follow-up information was determined by personal or phone interviews. Recurrence was defined as the need for reoperation, and risk was calculated by the Kaplan-Meier method. Patients with diffuse jejunoileitis were also compared with 219 patients with limited small-bowel Crohn's disease undergoing strictureplasty. RESULTS: The overall morbidity rate was 20 percent, with septic complications occurring in 6 percent. The surgical recurrence rate was 29 percent with a median follow-up period of 6.7 (range, 1-16) years. The recurrence rate in diffuse jejunoileitis patients did not differ from that seen in patients with limited small-bowel Crohn's disease (P= 0.38). Short duration of disease and short interval since last surgery were significant predictors of accelerated recurrence (P= 0.008 and 0.04, respectively). CONCLUSIONS: Strictureplasty is a safe and durable alternative to resection in diffuse Crohn's jejunoileitis. Patients with a short duration of disease and short interval since last surgery are at higher risk for accelerated recurrence. Patients with diffuse jejunoileitis do not appear to be at higher risk for recurrence than patients with more limited Crohn's disease.

Cecal volvulus is second only to sigmoid volvulus in its frequency of occurrence. Diagnostic doubt is not uncommon in cecal volvulus; nonoperative decompression is rarely achievable; and if gangrene supervenes, mortality rises appreciably. Resection is mandatory for gangrene and a grossly distended, thin-walled cecum. Cecopexy and cecostomy seem less-effective and more morbid options than resection and anastomosis for viable bowel. However, their role needs reappraisal in the light of advances in minimally invasive techniques.