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  • 1
    Electronic Resource
    Electronic Resource
    Functional outcome of conversion of ileorectal anastomosis to ileal pouch-anal anastomosis in patients with familial adenomatous polyposis and ulcerative colitis (1999)
    Springer
    Diseases of the colon & rectum 42 (1999), S. 903-908 
    Details
    ISSN: 1530-0358
    Keywords: Ulcerative colitis ; Familial adenomatous polyposis ; Anastomosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract PURPOSE: The aim of this study was to review the functional outcome in 20 patients with familial adenomatous polyposis and ulcerative colitis who were converted from ileorectal anastomosis to ileal pouch-anal anastomosis. METHODS: From 1985 to 1997, 12 patients with familial adenomatous polyposis (5 males; mean age, 39.1 years) and 8 patients with ulcerative colitis (5 males; mean age, 36.7 years) underwent conversion from ileorectal anastomosis to ileal pouch-anal anastomosis. Clinical and operative data were analyzed retrospectively. Functional results were obtained by telephone interview in 16 patients (94 percent) after pouch construction. Four patients were not interviewed (2 were deceased, 1 was lost to follow-up, and 1 was not reachable). RESULTS: Indications for conversion were uncontrollable rectal polyps (10 patients) and colonic cancer found in the pathology specimen after ileorectal anastomosis in patients with familial adenomatous polyposis (2 patients), intractable proctitis (5 patients), colonic cancer found in the pathology specimen of patients with ulcerative colitis after ileorectal anastomosis (2 patients), and rectal dysplasia (1 patients). Mean follow-up time was 5 (range, 1–11) years. Ileal pouch-anal anastomosis was handsewn in 14 patients, and the remaining cases were double-stapled in 4 patients with ulcerative colitis. No intraoperative difficulties were reported in 13 cases; technical problems were related to adhesions (3 cases), difficult rectal dissection (2 cases), and stapler-related difficulties (2 cases). Postoperative complications after ileal pouch-anal anastomosis included small-bowel obstruction (4 patients) and ileal pouch-anal anastomosis leak (1 patient). Patients with ileorectal anastomosisvs. those with ileal pouch-anal anastomosis had a better functional outcome with regard to nighttime continence (14 (88 percent)vs. 6 (38 percent) patients) and average bowel movements (〈6/day; 12 (75 percent)vs. 4 (25 percent) patients). Complete daytime continence, 15 (94 percent)vs. 10 (62 percent) patients, was similar in the two groups. Physical and emotional well-being were similarly rated as very good to excellent. CONCLUSIONS: In patients with familial adenomatous polyposis and ulcerative colitis with ileorectal anastomosis, conversion to ileal pouch-anal anastomosis may be required. In view of the risk of rectal cancer or intractable proctitis, patients seem to accept the conversion in spite of poorer bowel function.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02237099
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  • 2
    Electronic Resource
    Electronic Resource
    Comparison of ileal pouch-anal anastomosis and ileorectal anastomosis in patients with familial adenomatous polyposis (1999)
    Springer
    Diseases of the colon & rectum 42 (1999), S. 1028-1033 
    Details
    ISSN: 1530-0358
    Keywords: Ileal pouch-anal anastomosis ; Ileorectal anastomosis ; Familial adenomatous polyposis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract PURPOSE: The aim of this study was to evaluate the surgical complications and long-term outcome and assess the functional results and quality of life after ileorectal anastomosis and ileal pouch-anal anastomosis in patients with familial adenomatous polyposis. METHODS: From 1980 to 1997, 131 patients with familial adenomatous polyposis were operated on or were followed up or both at the Familial Gastrointestinal Cancer Registry at Mount Sinai Hospital. Demographic and operative data were prospectively collected in the ileal pouch-anal anastomosis group, and retrospectively in the ileorectal anastomosis group. A questionnaire or telephone interview or both were undertaken to evaluate functional outcome and quality of life. RESULTS: The ileorectal anastomosis group consisted of 60 patients (mean age, 31 years; mean follow-up, 7.7 years). In the ileal pouch-anal anastomosis group there were 50 patients (mean age, 35 years; mean follow-up, 6 years). There were no statistically significant differences with respect to anastomotic leak rate in ileal pouch-anal anastomosisvs. ileorectal anastomosis (12vs. 3 percent;P=0.21), risk of small-bowel obstruction (24vs. 15 percent;P=0.58), and risk of intra-abdominal sepsis (3vs. 2 percent;P=0.86). Reoperation rate was similar in the two groups (14vs. 16 percent;P=0.94). Twenty-one patients (37 percent) with ileorectal anastomosis were converted to ileal pouch-anal anastomosis (12 patients) or proctocolectomy (9 patients), because of rectal cancer (5 patients), dysplasia (1 patient), or uncontrollable rectal polyps (15 patients). Two pelvic pouches were excised, and another one was defunctioned. Information regarding functional results and quality of life was obtained in 40 patients (66.6 percent) in the ileorectal anastomosis group and in 43 patients (86 percent) in the ileal pouch-anal anastomosis group. Patients with ileorectal anastomosis had a significantly better functional outcome with regard to nighttime continence and perineal skin irritation. But otherwise, functional results and quality of life were similar. CONCLUSIONS: Although ileorectal anastomosis has a better functional outcome, ileal pouch-anal anastomosis may be preferable because of the lower long-term failure rate. Ileorectal anastomosis is still an option in patients with familial adenomatous polyposis with rectal polyp sparing and good compliance for follow-up.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02236696
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  • 3
    Electronic Resource
    Electronic Resource
    Desmoid disease in patients with familial adenomatous polyposis (2000)
    Springer
    Diseases of the colon & rectum 43 (2000), S. 363-369 
    Details
    ISSN: 1530-0358
    Keywords: Familial adenomatous polyposis ; Desmoid tumors ; Surgery ; Chemotherapy ; Sulindac
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract PURPOSE: The aim of this retrospective study was to review the clinical features, and surgical and medical management of patients with familial adenomatous polyposis-associated desmoid tumors. METHODS: From 1980 to 1997, 97 of 780 patients with familial adenomatous polyposis developed desmoid disease. Clinical and demographic data; operative notes; and histologic, radiologic, and follow-up reports were retrieved from patients' medical records. Risk factors for desmoid disease, such as prior surgery, age at desmoid tumor diagnosis, pregnancy, and family history were sought. The outcome after noncytotoxic and cytotoxic therapy was evaluated with respect to improvement of symptoms. RESULTS: There were 38 males with a mean age of 32.1 years and 59 females with a mean age of 29.1 years. A family history of desmoid tumors was found in 41 patients (42 percent), and a history of pregnancy was documented in 33 females (56 percent). The most common clinical presentation was small-bowel obstruction (58 percent). One-half of the desmoids were located in the mesentery, and 32 percent were located in the mesentery and the abdominal wall. Desmoids developed after colectomy in 77 cases (80 percent), after a mean time of 4.6 years. Partial resection of desmoid tumor was performed in 46 patients (47 percent), resection of extra-abdominal desmoid tumors was performed in 17 cases (17 percent), and biopsy only was performed in 34 patients (35 percent). Postoperative morbidity was 23 percent after desmoid tumor resection. Eight patients (8 percent) died of their intra-abdominal desmoid. Mean follow-up time was 5.3 years. Sulindac, tamoxifen, or toremifene therapy was able to alleviate symptoms in only 4 of 31 patients. Symptomatic improvement was noted after chemotherapy in six of ten patients with extremely complex desmoids. CONCLUSION: Desmoid disease was found in 12.4 percent of our patients with familial adenomatous polyposis. In view of the high rate of morbidity, indication for surgery should be limited mainly to acute or chronic small-bowel obstruction, because resection triggers a high recurrence rate. Noncytotoxic therapy was not effective for progressive desmoid tumors, whereas chemotherapy was effective in aggressive cases of intra-abdominal desmoid tumors.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02258303
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  • 4
    Electronic Resource
    Electronic Resource
    Family history characteristics, tumor microsatellite instability and germline MSH2 and MLH1 mutations in hereditary colorectal cancer (1999)
    Springer
    Human genetics 〈Berlin〉 104 (1999), S. 167-176 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Recent characterization of the molecular genetic basis of hereditary nonpolyposis colorectal cancer provides an important opportunity for identification of individuals and their families with germline mutations in mismatch repair genes. Cancer family history criteria that accurately define hereditary colorectal cancer are necessary for cost-effective testing for germline mutations in mismatch repair genes. The present report describes the results of analysis of 33 colorectal cancer cases/families that satisfy our modified family history criteria (Mount Sinai criteria) for colorectal cancer. Fourteen of these families met the more stringent Amsterdam criteria. Germline MSH2 and MLH1 mutations were identified by the reverse transcription-polymerase chain reaction and the protein truncation test, and confirmed by sequencing. Microsatellite instability analysis was performed on available tumors from affected patients. MSH2 or MLH1 mutations were detected in 8 of 14 Amsterdam criteria families and in 5 of the remaining 19 cases/families that only satisfied the Mount Sinai criteria. Three of the latter families had features of the Muir-Torre syndrome. A high level of microsatellite instability (MSI-H) was detected in almost all (16/18) colorectal cancers from individuals with MSH2 and MLH1 mutations, and infrequently (1/21) in colorectal cancer specimens from cases without detectable mutations. Families with germline MSH2 and MLH1 mutations tended to have individuals affected at younger ages and with multiple tumors. The Amsterdam criteria are useful, but not sufficient, for detecting hereditary colorectal cancer families with germline MSH2 and MLH1 mutations, since a proportion of cases and families with mutations in mismatch repair genes will be missed. Further development of cancer family history criteria are needed, using unbiased prospectively collected cases, to define more accurately those who will benefit from MSH2 and MLH1 mutation analysis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004390050931
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