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  • 1
    Electronic Resource
    Electronic Resource
    Treatment of rectovaginal fistulas that has failed previous repair attempts (1995)
    Springer
    Diseases of the colon & rectum 38 (1995), S. 921-925 
    Details
    ISSN: 1530-0358
    Keywords: Rectovaginal fistula ; Advancement flap ; Gracilis transfer ; Coloanal anastomosis ; Sphincteroplasty
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract PURPOSE: The purpose of this study was to assess results of treatment of rectovaginal fistulas (excluding pouch vaginal fistulas) that have failed previous attempts at repair. METHOD: A retrospective chart review of all patients presenting with nonhealing rectovaginal fistula was performed. RESULTS: Twenty eight patients with persistent fistulas were identified. In 18 patients the fistula was classified as simple, and in 10 the fistula was complex. Fourteen fistulas were secondary to obstetric injury, five were caused by Crohn's disease, and nine patients had miscellaneous etiologies for their fistulas. Of patients with persistent simple fistulas, 13 (72 percent) of the fistulas healed, 5 after advancement flaps, 5 following sphincteroplasty, and 3 after coloanal anastomoses. Of persistent complex fistulas, only four of ten (40 percent) healed, one following sphincteroplasty, one with coloanal anastomosis, and two after gracilis transposition. A total of 23 advancement flaps were done in 17 patients with five fistulas healing (29 percent). Sphincteroplasty and fistulectomy was successful in six of seven patients (86 percent). Coloanal anastomosis resulted in healing of four of six patients (67 percent) in whom it was attempted. Gracilis muscle transfer was successful in two of two patients (100 percent). CONCLUSION: Persistent rectovaginal fistula presents a difficult management problem. Choice of operation must be tailored to the underlying pathology and type of repair previously done. Advancement flap repair is generally not recommended for persistent complex fistulas or for simple fistulas that have failed a previous advancement flap repair.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02049726
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  • 2
    Electronic Resource
    Electronic Resource
    Changing causes of mortality in patients with familial adenomatous polyposis (1996)
    Springer
    Diseases of the colon & rectum 39 (1996), S. 384-387 
    Details
    ISSN: 1530-0358
    Keywords: Familial adenomatous polyposis ; Colorectal carcinoma ; Desmoid tumors ; Periampullary tumors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Widespread use of prophylactic colectomy has resulted in a reduction in the incidence of colorectal cancer in familial adenomatous polyposis (FAP) patients. A retrospective chart review of families registered at the Steve Atanas Stavro Familial Gastrointestinal Cancer Registry in Toronto was performed to determine whether the decrease in the number of patients developing colorectal cancer implies that causes of mortality in FAP patients are shifting to that of extracolonic manifestations of FAP. Information was available on 140 deaths within 158 families and among 461 individuals with FAP. When stratified by decade, from the 1930s to the 1990s, the ratio of deaths caused by extracolonic manifestations of FAP compared with deaths caused by colorectal cancer was noted to have risen. Even though most deaths in FAP patients are still from colorectal cancer, it appears that screening policies and prophylactic colectomy have resulted in a reduction in the number of FAP patients who develop colorectal cancer. Thus, in recent decades, a greater percentage of deaths in FAP patients appears to be attributable to extracolonic manifestations of the disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02054051
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  • 3
    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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  • 4
    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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  • 5
    Electronic Resource
    Electronic Resource
    Using genetic information to make surgical decisions (1997)
    Springer
    Diseases of the colon & rectum 40 (1997), S. 240-243 
    Details
    ISSN: 1530-0358
    Keywords: Hereditary nonpolyposis colorectal carcinoma ; Microsatellite instability ; Genetic counseling ; Registries
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract PURPOSE: We report the case of a boy aged 13 years who was diagnosed with a Dukes B obstructing cancer of the sigmoid colon. At the time of diagnosis, he underwent a Hartmann's procedure with end colostomy. Because of his unusually young age, he was referred to the Familial GI Cancer Registry at Mount Sinai Hospital for genetic assessment. A detailed pedigree revealed no significant history other than lung cancer in his maternal grandfather. METHODS: We obtained his tumor specimen and performed molecular analysis of both normal colonic and tumor DNA. Specifically, we identified replication errors (RER) in the patient's tumor DNA when compared with normal colonic DNA. RER has been found in more than 90 percent of tumors from patients with Hereditary Nonpolyposis Colon Cancer (HNPCC) and is, thus, considered to be one of the hallmarks of this disease. Because HNPCC patients have a 40 percent risk of synchronous or metachronous tumors, the recommended surgery for HNPCC should be at least a subtotal colectomy with ileorectal anastomosis. RESULTS: Based on molecular results, we were able to recommend that the patient have a subtotal colectomy performed instead of merely colostomy closure, to reduce his lifetime risk of developing further colon tumors and to make surveillance of the remaining rectum relatively easy. In this patient, we subsequently identified a germline mutation of the mismatch repair gene hMSH2 that is implicated in HNPCC. The possibility of HNPCC should be considered in adolescents who are diagnosed with colorectal cancer, so appropriate surgical decisions can be made.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02054995
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  • 6
    Electronic Resource
    Electronic Resource
    Risk factors for pelvic pouch failure (1997)
    Springer
    Diseases of the colon & rectum 40 (1997), S. 257-262 
    Details
    ISSN: 1530-0358
    Keywords: Heal pouch-anal anastomosis ; Ulcerative colitis ; Crohn's disease ; Familial adenomatous polyposis ; Complications
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract PURPOSE: This study was designed to identify factors associated with pelvic pouch failure. METHOD: A retrospective review of patients undergoing the pelvic pouch procedure with a minimum of 30 months follow-up was conducted. RESULTS: A total of 551 patients had pelvic pouch procedures from 1981 to 1992. Forty-nine patients (8.8 percent) have undergone pouch excision, and 9 (1.6 percent) have been defunctioned, for 58 (10.5 percent) patients with pouch failure. Cause of failure was leakage from the ileoanal anastomosis (IAA) in 21 (39 percent) patients, poor functional results in 13 (23 percent), pouchitis in 7 (12 percent), pouch leakage in 7 (12 percent), perianal disease in 7 (12 percent), and miscellaneous in 3 (5.2 percent). Nine of 22 patients (41 percent) had pouch failure during the first two years, with 2 of 147 patients (1 percent) having failure during the last two years of the study. The 58 patients whose pouches failed (Group 1) were compared with the 493 patients whose pouches did not fail (Group 2). Handsewn IAA (P 〈0.001), tension on the IAA (P 〈0.001), use of a defunctioning ileostomy (P 〈0.01), a diagnosis of Crohn's disease (P 〈0.001), and a leak from the pouch (P 〈0.001) or the IAA (P 〈0.001) were associated with pouch failure. Pouchitis was not a risk factor. CONCLUSION: The majority of pouch failures were caused by leaks at the IAA. Although the leak rate remained stable, leaks following a stapled anastomosis seemed to have a better prognosis than leaks following a handsewn anastomosis. Experience with the pouch procedure and the management of complications likely plays an important role in decreasing the risk of pouch failure.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02050412
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  • 7
    Electronic Resource
    Electronic Resource
    Effect of intervention in inflammatory bowel disease on health-related quality of life: A critical review (1995)
    Springer
    Diseases of the colon & rectum 38 (1995), S. 1147-1161 
    Details
    ISSN: 1530-0358
    Keywords: Quality of life ; Ulcerative colitis ; Crohn's disease ; Inflammatory bowel disease ; Pelvic pouch ; Ileostomy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Health-related quality of life (HRQOL) is a quantitative measurement of subjective perception of health state, including emotional and social aspects. It can be reliably measured with several valid instruments. Previous reviews of the literature suggested inadequate attention to HRQOL in studies of interventions in inflammatory bowel disease. PURPOSE: This study was undertaken to assess the current status of the quality of measurement of HRQOL in studies of inflammatory bowel disease and to review the clinical conclusions warranted by the literature. METHOD: Medline was searched for articles relating to ulcerative colitis, Crohn's disease, or inflammatory bowel disease and quality of life since 1981. The articles found were reviewed for citations of further articles. The adequacy of HRQOL measure was assessed and graded, and the study design was categorized to assess the strength of the literature on the whole. RESULTS: A trend was found toward higher quality of HRQOL measurement in the period 1988 to 1994 compared with 1981 to 1987. Most of the improvement was because of increased use of standardized and multidomain but unvalidated and unpublished questionnaires for measurement. CONCLUSIONS: Confidence in the following clinical conclusions in studies of surgical interventions in inflammatory bowel disease is limited by study design: that pelvic pouch is not inferior to ileostomy, that specific domains of HRQOL are differentially affected by different surgical procedures, and (with less confidence) that surgery is helpful in Crohn's disease. Medical studies have demonstrated that high quality HRQOL measures can be integrated into randomized, prospective trials. Clinically equivalent treatments have shown differential effects on HRQOL: 9 mg daily of budesonide is superior to 15 mg, and hydrocortisone foam enemas are superior to prednisolone. Home parenteral nutrition has received modest support, limited again by study design. It is recommended that standard tests of HRQOL be used to increase comparability of studies and to increase the quality of this literature in general. In particular the Inflammatory Bowel Disease Questionnaire, Rating Form of Inflammatory Bowel Disease Concerns, and Direct Questioning of Objectives are recommended.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02048330
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  • 8
    Electronic Resource
    Electronic Resource
    Chemotherapy for desmoid tumors in association with familial adenomatous polyposis (1997)
    Springer
    Diseases of the colon & rectum 40 (1997), S. 798-801 
    Details
    ISSN: 1530-0358
    Keywords: Desmoids ; Familial polyposis ; Doxorubicin ; Dacarbazine ; Carboplatin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract PURPOSE: This study was designed to assess the effect of chemotherapy on complex desmoid tumors associated with familial adenomatous polyposis. METHODS: Five patients (3 males, 2 females; age range, 29–45 years) had symptomatic, unresectable intra-abdominal desmoid tumors in association with familial adenomatous polyposis that were unresponsive to conventional medical therapy. Each patient was treated with a cytotoxic chemotherapeutic regimen consisting of doxorubicin and dacarbazine followed by carboplatin and dacarbazine. Response to treatment was assessed by measurement of tumor size using computerized tomography. Follow-up has been for a mean of 22 (range, 10–30) months. RESULTS: One patient has had a complete response, and three patients have had a partial response, with a reduction in tumor volume of at least 50 percent. One patient had a minimum response to treatment and developed a rapid increase in tumor size on cessation of therapy. Complications of treatment included febrile neutropenia, severe epistaxis, and subclavian vein thrombosis. CONCLUSIONS: The cytotoxic chemotherapeutic regimen described is effective in the treatment of selected unresectable desmoid tumors associated with familial adenomatous polyposis and should be considered in symptomatic patients who do not respond to conventional medical therapy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02055435
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  • 9
    Electronic Resource
    Electronic Resource
    Invited editorial (1998)
    Springer
    Diseases of the colon & rectum 41 (1998), S. 1355-1356 
    Details
    ISSN: 1530-0358
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02237047
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  • 10
    Electronic Resource
    Electronic Resource
    Negative genetic test result in familial adenomatous polyposis (1999)
    Springer
    Diseases of the colon & rectum 42 (1999), S. 307-310 
    Details
    ISSN: 1530-0358
    Keywords: Familial adenomatous polyposis ; Genetic testing ; Registries ; APC gene
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract PURPOSE: The goal of genetic testing is to define individual risk, which in turn may guide clinical management. METHODS: Thirty-two international familial adenomatous polyposis registries were surveyed regarding their approach to a specific clinical management question. There were 30 respondents. Respondents declared their clinical policy for an at-risk, first-degree relative who undergoes direct mutation analysis and does not have anAPC gene mutation known to be present in his or her family. RESULTS: Nineteen of 30 (63.3 percent) registries would discharge this negativeAPC mutation case from clinical screening. Eleven of 30 (37 percent) registries would maintain clinical screening. Reasons offered for maintaining surveillance included the need for additional confirmation of theAPC mutation in two affected relatives, the possibility of sampling error or two different mutations in an affected family, limited prospective data, and patient anxiety. CONCLUSIONS: The discrepancy in response to the survey suggests that some clinicians are as yet reluctant to accept fully that predictive genetic analysis is a definitive guide to clinical management in familial adenomatous polyposis. Nevertheless, we believe that use of a predictive gene test for familial adenomatous polyposis should alter management, decrease cost, and reduce psychological trauma for the tested individual. Although the benefit ofAPC analysis is accepted for the positive gene carrier, the challenge remains to reclassify the negative gene carriers who are no longer at risk for familial adenomatous polyposis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02236343
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