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  • 1990-1994  (14)
  • Familial adenomatous polyposis  (8)
  • Restorative proctocolectomy  (4)
  • dysplasia  (3)
  • 1
    Electronic Resource
    Electronic Resource
    Spontaneous mutation in familial adenomatous polyposis (1990)
    Springer
    Diseases of the colon & rectum 33 (1990), S. 52-55 
    Details
    ISSN: 1530-0358
    Keywords: Familial adenomatous polyposis ; Spontaneous mutation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A retrospective review of the familial adenomatous polyposis registry at the Cleveland Clinic Foundation revealed an incidence of spontaneous mutation in familial adenomatous polyposis (FAP) of 22 percent of family kindreds. These patients were reviewed retrospectively and compared with the total FAP population followed at The Cleveland Clinic Foundation with respect to the onset of disease, the incidence of carcinoma in the resected colon, and incidence of extracolonic manifestations. Review of the characteristics and presentations of these patients suggested that these individuals may harbor a more severe form of FAP. This may be due, in part, to the delay in diagnosis and, therefore, a higher rate of development of colorectal carcinoma and possibly duodenal adenomas. There is also a demonstrable higher rate of extracolonic manifestations of FAP present in this subset of patients. When selecting the initial type of prophylactic colonic resection the surgeon should bear in mind the increased incidence of extracolonic manifestations of the disease in this group of patients and their potential for complications.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02053203
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  • 2
    Electronic Resource
    Electronic Resource
    Predictive value of congenital hypertrophy of the retinal pigment epithelium as a clinical marker for familial adenomatous polyposis (1990)
    Springer
    Diseases of the colon & rectum 33 (1990), S. 1003-1008 
    Details
    ISSN: 1530-0358
    Keywords: Familial adenomatous polyposis ; Congenital hypertrophy ; Retinal pigment epithelium ; Extracolonic manifestations
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract One hundred forty-eight members of 53 kindreds with familial adenomatous polyposis (FAP) were examined for congenital hypertrophy of the retinal pigment epithelium (CHRPE) and extracolonic manifestations (ECM) to assess the value of CHRPE as a predictive marker for FAP. Based on eye examination results, the families were divided into 2 groups. In a first group of 34 families, all 61 members diagnosed as having polyps and 13 of the 33 patients at risk had 4 or more lesions distributed in both eyes. By contrast, in a second group of 18 families, all 32 polyposis patients and all 18 members at risk had less than 4 lesions. Extracolonic manifestations were present in 26 of 34 families in the first group and in 11 of 18 families in the second group. Data on one family with ambiguous ancestry were reviewed separately. The existence of 4 or more CHRPE lesions distributed in both eyes seems to be a congenital marker for FAP, present in 65.4 percent of families. When present in a family: 1) it is found in all diagnosed patients in that family, 2) can therefore be considered predictive for the development of polyps in other family members who carry the trait, and 3) if confirmed by longer follow-up, may possibly preclude members without the trait from further evaluation and surveillance.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02139213
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  • 3
    Electronic Resource
    Electronic Resource
    Noncytotoxic drug therapy for intra-abdominal desmoid tumor in patients with familial adenomatous polyposis (1992)
    Springer
    Diseases of the colon & rectum 35 (1992), S. 29-33 
    Details
    ISSN: 1530-0358
    Keywords: Intra-abdominal desmoid tumor ; Familial adenomatous polyposis ; Nonsteroidal anti-inflammatory drugs ; Antiestrogen drugs ; Prostaglandin synthesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Forty of 416 patients with familial adenomatous polyposis were noted to have intra-abdominal desmoid tumors, and a subgroup of 16 were treated with noncytotoxic drug therapy. Drugs used were sulindac (14 patients), sulindac plus tamoxifen (3 patients), indomethacin (4 patients), tamoxifen (4 patients), progesterone (DEPO-PROVERA®; Upjohn Co., Kalamazoo, MI) (2 patients), and testolactone (1 patient). Therapy with these drugs for continuous periods of six months or more resulted in three complete and seven partial remissions. When treated patients were compared with untreated patients (n=12), there were significant benefits for the treated group, both in reduction of desmoid size and in improvement of symptoms, despite the inherent selection bias against this. Sulindac was the only drug used in enough patients to permit independent evaluation of its effect, with one complete and seven partial reductions of tumor size. Some patients had a delayed response to sulindac, with tumor shrinkage occurring after an initial period of tumor enlargement. When using sulindac for the treatment of desmoid tumors, this phenomenon should be considered.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02053335
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  • 4
    Electronic Resource
    Electronic Resource
    Retained mucosa after double-stapled ileal reservoir and ileoanal anastomosis (1992)
    Springer
    Diseases of the colon & rectum 35 (1992), S. 1051-1056 
    Details
    ISSN: 1530-0358
    Keywords: Ileoanal reservoir ; Ileal pouch-anal anastomosis ; Mucosal ulcerative colitis ; Ulcerative colitis ; Double-stapled ; Dysplasia ; Restorative proctocolectomy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A study was undertaken to assess the incidence of inflammation and dysplasia in retained mucosa after double-stapled ileoanal reservoir (IAR) for mucosal ulcerative colitis (MUC). Between September 1988 and February 1992, 56 patients with MUC underwent an IAR. Forty-five patients had a double-stapled IAR (DS-IAR), seven patients had a transanal pursestring stapled IAR (PS-IAR), and four patients had a PS-IAR with mucosectomy. Distal donuts obtained from the stapled IAR were submitted for pathologic review in 55 patients. Nine patients had only small bowel, connective tissue, and/or muscle noted on review. Mucosa was qualified as squamous epithelium (SE), transitional epithelium (TE), or columnar epithelium (CE). All samples were examined for evidence of inflammation and dysplasia. Four patients had SE only, one patient had TE, and 18 had CE. In addition, three patients had SE and CE, seven patients had SE and TE, two patients had CE and TE, and nine patients had all three types. The distance from the dentate line to the anastomosis ranged from 0 to 2.5 cm (mean, 1 cm). In 19 patients (35 percent), the distal donut revealed MUC. Of these 19 patients, six had persistent MUC (43 percent) at the time of subsequent biopsy. An additional four patients had MUC evident on follow-up biopsy but not on distal donuts; two of these four patients had no mucosa in their distal donuts. Only one of the patients with evidence of MUC on donuts and/or biopsy experienced any symptoms referable to active MUC (1.8 percent). None of the specimens examined had any evidence of dysplasia. In 31 patients, no MUC was present in the initial donuts or follow-up biopsies. Although the double-stapled technique appears safe, periodic monitoring is suggested.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02252995
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  • 5
    Electronic Resource
    Electronic Resource
    Mortality in patients with familial adenomatous polyposis (1990)
    Springer
    Diseases of the colon & rectum 33 (1990), S. 639-642 
    Details
    ISSN: 1530-0358
    Keywords: Familial adenomatous polyposis ; Colorectal carcinoma ; Desmoid tumors ; Periampullary carcinoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The authors identified 132 patients who died with a documented diagnosis of familial adenomatous polyposis (FAP). A review of the medical records, autopsy reports, and in-depth discussion with local physicians and well-informed family members was performed. It was impossible, even after the review, to ascertain the exact cause of death in 22 patients. In the remaining patients, the cause of death was as follows: metastatic colorectal carcinoma, 64 patients (58.2 percent), (colon, 49 [44.5 percent], rectal, 15 [13.6 percent]); desmoid tumors, 12 (10.9 percent); periampullary carcinoma, 9 (8.2 percent); brain tumors, 8 (7.3 percent); perioperative mortalities, 5 (4.5 percent); adrenal carcinoma, 1 (0.9 percent); and abdominal carcinomatosis, 1 (0.9 percent). Ten patients died of causes not related to FAP. The major causes of death in 36 patients who underwent prophylactic colectomy were desmoid tumor and periampullary malignancy. This finding underscores the importance of lifelong surveillance and periodic endoscopic evaluation in patients with FAP.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02150736
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  • 6
    Electronic Resource
    Electronic Resource
    Optimization of sphincter function after the ileoanal reservoir procedure (1994)
    Springer
    Diseases of the colon & rectum 37 (1994), S. 419-423 
    Details
    ISSN: 1530-0358
    Keywords: Kegel exercises ; Pelvic muscle exercises ; Ileoanal reservoir ; J-pouch ; Restorative proctocolectomy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract PURPOSE: Impairment of sphincter function in patients who undergo ileoanal reservoir is usually most severe immediately after ileostomy closure. Therefore, a prospective, randomized trial was undertaken to assess the potential value of preileostomy closure sphincter-strengthening exercises to improve early functional outcome. METHODS: Patients were randomized either to a control group (Group 1) or to undergo a five-week pelvic floor exercise program (Group 2). An incontinence score from 0 to 20 was used to clinically assess the functional results. Anorectal manometric assessment included: highpressure zone length, mean resting pressure, highest resting pressure, mean squeezing pressure, and highest squeezing pressure. The paired t-test was used to compare the functional results preoperatively and at the time of ileostomy closure. This time corresponded to the conclusion of the exercise program or the equivalent time period for the control group. RESULTS: Twenty-six patients who underwent double-stapled ileoanal reservoir between July 1991 and June 1992 were studied. They included 16 males and 10 females with a mean age of 38 (range, 17–69) years. When both evaluations were compared, the mean incontinence score decreased from 0.2 to 2.8 (Δ=2.6) in Group 1 and from 0.2 to 2.0 (Δ = 1.8) in Group 2 (P=0.07). None of the changes between the preoperative and postoperative clinical and physiologic evaluations were statistically significant (P〉0.05). CONCLUSION: Sphincter-strengthening exercises before ileostomy closure did not minimize the transient impairment of functional results.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02076184
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  • 7
    Electronic Resource
    Electronic Resource
    Recovery of anal sphincter function after the ileoanal reservoir procedure in patients over the age of fifty (1994)
    Springer
    Diseases of the colon & rectum 37 (1994), S. 1002-1005 
    Details
    ISSN: 1530-0358
    Keywords: Ileoanal reservoir ; Pouch ; Restorative proctocolectomy ; Continence ; Ileoanal anastomosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract PURPOSE: This study was undertaken to postoperatively assess the progression of anal sphincter function and clinical outcome in patients ≥50 years old (Group I) compared with those 〈50 years old (Group II). METHODS: Clinical data were assessed after ileostomy closure by a questionnaire. These data were compiled to obtain an incontinence score, which ranged from 0 (perfect continence) to 20 (total incontinence). Anorectal manometry was performed preoperatively (MN1) and postoperatively, before (MN2) and after (MN3) ileostomy closure. Wilcoxon and paired t-test were used to compare the clinical and functional results, respectively. RESULTS: Group I consisted of 22 patients (mean age, 56 years) and Group II, 50 patients (mean age, 32 years). No differences were found relative to either preoperative pressures or clinical outcome. However, both the mean and high resting pressures were significantly lower in Group I at the MN2 examination. CONCLUSION: The effect on anal sphincters of ileoanal reservoir in patients over the age of 50 years is similar to that noted in younger patients. Transient impairment of internal anal sphincter function observed after ileoanal reservoir is more severe in older patients (P=0.01). However, as in younger patients, it does completely recover after ileostomy closure.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02049312
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  • 8
    Electronic Resource
    Electronic Resource
    The double-stapled ileal reservoir and ileoanal anastomosis (1991)
    Springer
    Diseases of the colon & rectum 34 (1991), S. 487-494 
    Details
    ISSN: 1530-0358
    Keywords: Ileoanal reservoir ; Stapling device ; Mucosal ulcerative colitis ; Familial adenomatous polyposis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fifteen consecutive patients (nine males and six females) who underwent construction of a double-stapled ileoanal reservoir (DS-IAR) were prospectively evaluated. Mean and maximal resting pressures preoperatively, before ileostomy closure, and at 12 months, were 53 and 84 mm Hg, 39 and 62 mm Hg, and 62 and 81 mm Hg. Mean and maximal squeeze pressures at those same time periods were 96 and 153 mm Hg, 111 and 173 mm Hg, and 95 and 168 mm Hg. There were no significant decreases in either resting or squeeze pressure between preoperative values and those obtained 12 months after surgery. However, the length of the high pressure zone decreased from 3–8 cm preoperatively to 2.3 cm at 12 months. This reflects the sacrifice of the cephalad 1.5 cm of the internal anal sphincter necessary to effect this anastomosis at a mean of 1.4 cm from the dentate line. However, this maneuver did not result in poor continence. Eleven patients whose ileostomies were closed for a mean of 9 months, ranging from 3 to 15 months, were evaluated regarding functional outcome. Only one patient had any incontinence and this patient had incomplete circularstapled tissue rings, which necessitated transanal suture repair of the anastomotic defect. Similarly, three of the four patients who sometimes or rarely use a pad at night had transanal-suture reinforcement. Ten of the 11 patients never wear a pad during the day. No pelvic or perianal sepsis occurred. Stratified squamous epithelium was found in 6 of the 13 distal stapler “donuts” that were examined. In addition, 10 patients underwent biopsy of the tissue immediately caudad to the circular staple line at the time of ileostomy closure; in five, only stratified squamous epithelium was noted. The DS-IAR is associated with excellent objective physiologic and subjective functional results.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02049935
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  • 9
    Electronic Resource
    Electronic Resource
    Evaluation of polymorphic genetic markers for linkage to the familial adenomatous polyposis locus on chromosome 5 (1990)
    Springer
    Diseases of the colon & rectum 33 (1990), S. 740-744 
    Details
    ISSN: 1530-0358
    Keywords: Familial adenomatous polyposis ; Restriction fragment length polymorphism ; Chromosome 5q
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A gene associated with the inherited syndrome, familial adenomatous polyposis (FAP), has been localized to the long arm of chromosome 5 near the 5q21-22 region, and markers that identify genetic polymorphisms near this locus are now available. The authors evaluated several of these markers for linkage to the FAP trait in 11 families entered in the Cleveland Clinic Polyposis Registry. The original probe that established linkage to the FAP locus (C11p11) has limited utility for family studies because of low heterozygosity and distance from the FAP gene. Other probes, however, should be useful for assessing FAP inheritance by restriction fragment length polymorphism analysis, for presymptomatic diagnosis of the disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02052318
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  • 10
    Electronic Resource
    Electronic Resource
    Systemic cytotoxic chemotherapy and radiation therapy for desmoid in familial adenomatous polyposis (1991)
    Springer
    Diseases of the colon & rectum 34 (1991), S. 1090-1092 
    Details
    ISSN: 1530-0358
    Keywords: Desmoid tumor ; Familial adenomatous polyposis ; Systemic cytotoxic chemotherapy ; Radiation therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Forty-two of 416 familial adenomatous polyposis (FAP) patients in the FAP registry at the Cleveland Clinic had desmoid tumors. The role of cytotoxic chemotherapy and radiation therapy in the management of these patients was investigated. Eight intra-abdominal desmoid tumors were treated by systemic cytotoxic chemotherapy. Two had complete remission, and one had partial remission. Five patients died as a result of the desmoid tumor or late complications of chemotherapy. Three intra-abdominal desmoids were treated by radiation therapy with no response. Neither cytotoxic chemotherapy nor radiation therapy is recommended as a first-choice treatment for intra-abdominal desmoid tumors in patients with FAP.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02050067
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