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PubMed1998

Extracolonic manifestations of familial adenomatous polyposis: desmoid tumours, and upper gastrointestinal adenomas and carcinomas.

It is well known that patients with familial adenomatous polyposis (FAP) are at considerable risk of developing extracolonic manifestations of the disease. Particularly, desmoid tumours of the abdominal cavity, and duodenal adenomas and carcinomas are the most serious ones. It is estimated that some 10% of the FAP patients will develop desmoids, whereas 50-90% of the FAP patients will get duodenal adenomas predominantly concentrated on or around the major papilla. Desmoid tumours and duodenal carcinomas are major causes of death in those patients in whom a prophylactic (procto)colectomy has been performed. Desmoids are histologically benign tumours, composed of mature fibroblasts. They usually grow slowly but they can become quite large and may compress or infiltrate surrounding viscera, which might cause significant morbidity as well as mortality. Successful treatment of these tumours is extremely difficult as surgical therapy often requires the removal of considerable lengths of small bowel. Moreover, surgical therapy may lead to uncontrollable bleeding and is seldom radical. Chemotherapy with cytoxic agents seems promising but so far the data are too few for firm conclusions to be drawn. The same holds true for drug regimens which interfere with the metabolic and hormonal metabolism of the tumour. Although various lines of evidence suggest that the adenoma-carcinoma sequence, which is generally accepted for colorectal adenomas, also applies for the duodenal adenomas in FAP patients, it is not clear whether we should screen these patients for upper gastrointestinal adenomas or not. As these polyps are usually small, sessile, multiple and difficult to remove, the benefit of endoscopic surveillance would be the early detection of cancer rather than eradication of the polyps. In addition, evidence that screening and early treatment leads to improvement of the prognosis is not available. Although the role of (procto)colectomy in the treatment of large-bowel polyps is well established in FAP patients, the treatment of their duodenal counterparts is still open for debate. The risk of the development of periampullary cancer is not high enough to warrant an aggressive prophylactic surgical approach, i.e. a Whipple's procedure, immediately after the discovery of duodenal adenomas. The considerable morbidity and mortality rates of this procedure must be weighted against a putative benefit of screening.

Adenoma↗
PubMed1999

Proctocolectomy is associated with a higher complication rate but carries a lower recurrence rate than total colectomy and ileorectal anastomosis in Crohn colitis.

BACKGROUND: Most patients with extensive colonic Crohn disease are treated with total colectomy and ileorectal anastomosis or, when there is severe anorectal disease, with proctocolectomy. This study was undertaken to compare postoperative complications and recurrence rates for these two operations. METHODS: Eighty-six patients who underwent a single-stage proctocolectomy and 65 who underwent total colectomy and ileorectal anastomosis for colonic Crohn disease were retrospectively reviewed. RESULTS: Anorectal disease (severe proctitis, perianal sepsis, complex fistula) was seen in 77 patients (90%) at proctocolectomy, compared with 7 patients (11%) at colectomy and ileorectal anastomosis (P < 0.0001). After proctocolectomy the commonest complication was perineal wound sepsis (36%). After colectomy and ileorectal anastomosis only three patients (5%) developed anastomotic leak. The overall complication rate was 53% after proctocolectomy compared with 32% after colectomy and ileorectal anastomosis (P = 0.02). Twenty-four patients (29%) after proctocolectomy and 43 patients (68%) after colectomy and ileorectal anastomosis developed symptomatic recurrence (P < 0.0001). After proctocolectomy the 5-, 10-, and 15-year cumulative reoperation rate for recurrence were 13%, 16%, and 26%, which were significantly lower than the 29%, 46%, and 48% after colectomy and ileorectal anastomosis (P = 0.002). CONCLUSIONS: The overall complication rate was lower after colectomy and ileorectal anastomosis than after proctocolectomy. However, proctocolectomy was associated with a lower incidence of reoperation for recurrence than colectomy and ileorectal anastomosis.

Anastomosis, Surgical↗
PubMed1999

Health-related quality of life after ileal J-pouch-anal anastomosis for ulcerative colitis: long-term results.

BACKGROUND: This questionnaire study examined associations between health-related quality of life and long-term follow-up results with J-pouch anal anastomosis for ulcerative colitis. METHODS: A two-part questionnaire was sent to patients treated for ulcerative colitis by handsewn J-pouch-anal anastomosis at our institute in the period 1985-95. The health-related quality of life measurement was based on a RAND SF-36 Finnish version of the questionnaire. Scores were compared with the normal Finnish population. The functional status was analysed with a 34-item questionnaire on pouch function and incontinence. RESULTS: The response rate was 62%. The median follow-up time was 8 years (3 to 13 years). Functional results were acceptable: median number of bowel movements per 24 h was six. Soiling at night occurred in 32%, and inability to differentiate pouch contents in 24% cases. Twenty-four per cent used a pad occasionally, and 9% every day. The functional score and the health-related quality of life score correlated strongly. Poor functional status and a history of chronic pouchitis were associated with lower score. The limiting value after which the score was lower was 10 bowel movements per 24 h in 16%. A compromised anal continence proved to decrease the health-related quality of life as well. CONCLUSIONS: RAND SF-36 scores seem to provide a useful tool to evaluate the health-related quality of life after pelvic pouch operation for ulcerative colitis. Frequent bowel movements, impaired anal continence, and pouchitis are common in long-term follow-up after ileal pouch operation. The scores were, however, comparable to those in the normal population in terms of adaptation to daily life with a pelvic pouch.

Adult↗
PubMed1999

Impaired release of peptide YY in patients with proctocolectomy and ileal pouch-anal anastomosis.

BACKGROUND: Peptide YY (PYY) is a gut hormone produced by endocrine cells in the distal small bowel, colon, and rectum. PYY inhibits upper gastrointestinal secretory and motor functions. The aim of this study was to determine whether basal and postprandial plasma PYY levels in patients with proctocolectomy and ileal pouch-anal anastomosis (IPAA) are reduced and to determine the relationship between plasma PYY and plasma cholecystokinin (CCK) levels. METHODS: Plasma concentrations of PYY and CCK were measured before and after ingestion of a standardized breakfast in 14 IPAA patients and in 12 healthy control subjects. RESULTS: Basal PYY was slightly lower in the IPAA patients than in the controls (8.3 +/- 0.3 versus 9.3 +/- 1.1 pM; not significant). Ingestion of the meal induced a small but significant increase of PYY to a maximum of 10.9 +/- 0.9 pM in patients. Integrated postprandial PYY was markedly reduced in patients when compared with the controls (1725 +/- 66 pM*180min versus 3194 +/- 480 pM*180 min; P < 0.005). Plasma PYY concentrations were inversely correlated with plasma CCK concentrations in the 2nd and 3rd h after the meal (r = -0.86; P = 0.0001). CONCLUSION: PYY release in response to meal ingestion is markedly reduced but not completely absent in patients with proctocolectomy and ileal pouch-anal anastomosis. The inverse relationship between circulating PYY and CCK in the late postprandial phase is compatible with a negative feedback regulation of CCK release by endogenous PYY.

Adult↗
PubMed1999

Evaluation of Kock urinary reservoir function in children and adolescents at 3-10 years' follow-up.

The aim of this study was to evaluate Kock reservoir function in children and adolescents operated for congenital urinary incontinence and to determine the complication rate. Reservoir function was investigated in 13 children (age range 10.8-16 years) and 7 adolescents (age range 16-18 years) through enterocystometry, enterocystoscopy and patient history. Patients were followed up for 3-10 years. The follow-up was reported as early (3 months-2 years) and late (2-10 years) postoperative periods. At early follow-up reservoir capacity was high in four patients and normal in the remaining patients. A low reservoir pressure was accompanied by high capacity and compliance. On enterocystometry first sensation for emptying was experienced in 82% of patients at early follow-up and 92% at late follow-up. Reservoir contractions were recorded in 60% of patients at early follow-up and 65% at late follow-up. The contractions were recorded at an average reservoir capacity of 270 ml at early follow-up and 340 ml at late follow-up. The complication rate was high in the child group compared with that in adolescents. Of 13 patients with at least one reservoir complication 10 were from the child group. Nipple dysfunction (angled nipple, prolapsed or stenosed stoma) occurred in 35% of patients, stones in 40% and bleeding during catheterization in 15%. Revision was performed in 38% of the child group and 15% of the adolescents. Reservoir perforation was observed in two patients at 6 and 9 years postoperatively. At late follow-up continence was excellent in 17 of 19 patients and good in 2. We conclude that the Kock reservoir is a good modality for urinary diversion, but the complication incidence is high in the child group (<16 years). Stability of the reservoir in terms of volume and low internal pressures was achieved one year after operation, except in the patients with infrequent reservoir emptying. A time-related increase in the reservoir sensitivity and contractility was reported on enterocystometry. Nipple dysfunction is common during the first two years after surgery, particularly in the child group. Stones may form 3-4 years after surgery. Satisfactory continence was achieved in all patients, although a revisional operation was necessary in some patients in order to obtain permanent continence.

Adolescent↗
PubMed1999

Determinants of bone loss in patients with Kock ileal urinary reservoir.

The purpose of the present study was to determine the change in bone mineral density (BMD) measured with dual energy X-ray absorptiometry (DXA) in patients with Kock reservoirs for urinary diversion who were examined with the same technique 3 years earlier, and relate the changes to kidney function and variables reflecting bone metabolism. A total of 28 patients with Kock ileal reservoirs to the skin (23) or urethra (5) were reinvestigated 3 years after the first measurement. BMD was measured in the lumbar spine, femur and whole body with DXA. Bone specific alkaline phosphatase, osteocalcin, parathyroid hormone (PTH), calcitonin and chloride were also determined in serum. GFR was determined from the plasma clearance of 51Cr-EDTA. The mean values for BMD expressed in percentage of corresponding mean values for age-matched controls (BMD%) were almost identical after 3 years. Only osteocalcin levels correlated with the BMD% values. However, significant positive correlations were found between GFR and the observed individual changes in BMD% over the 3 years in spite of the fact that most GFR values were fairly normal. Enhanced bone loss was associated with high concentrations of osteocalcin and bone specific alkaline phosphatase. Comparisons with blood gas analyses and determination of 1,25 dihydroxyvitamin D performed in the previous study indicate to us that the relation between reduced GFR and low mineral content might, in part, be related to a low-grade metabolic acidosis and reduced availability of the biologically active vitamin D hormone. The conclusion to be drawn is that urinary diversion with a Kock reservoir does not regularly cause bone demineralization. However, patients with even moderately reduced GFR appear to be at risk for developing osteoporosis in the long-time run.

Adult↗
PubMed1997

Bacterial fermentation of fructooligosaccharides and resistant starch in patients with an ileal pouch-anal anastomosis.

Patients with large bowel disease may undergo ileal pouch-anal anastomosis, in which the colon is removed and part of the distal ileum is used to construct a pelvic reservoir. Competence of the ileal pouch to ferment carbohydrates is associated with the absence of pouchitis. However, the extent to which bacterial fermentation takes place and whether it is affected by diet are unclear. We investigated fermentation of two nondigestible carbohydrates, fructooligosaccharides and resistant starch, in 15 healthy patients with an ileal pouch by using a placebo-controlled crossover design (with glucose as the placebo). Apparent fermentability of fructooligosaccharides was 83%; that of resistant starch was 46%. Resistant starch increased fecal excretion of butyrate by 69% whereas fructooligosaccharides reduced excretion of amino acid-derived isobutyrate by 94% and of isovalerate by 77%. Fructooligosaccharides also significantly increased fecal weight (651 compared with 541 g/d) and breath-hydrogen excretion (286 compared with 85 ppm x h). Bacterial fermentation of nondigestible carbohydrates in pouches takes place to an appreciable extent and in a substrate-specific manner. The relation between such fermentation and inflammation of the pouch (pouchitis) deserves study.

Adult↗
PubMed1996

Bilateral compartment syndrome complicating prolonged lithotomy position.

A case of bilateral compartment syndrome after prolonged Lloyd-Davies lithotomy position is described. The diagnosis was made early, despite effective extradural bupivacaine-fentanyl analgesia. The aetiology, diagnosis, pathology and treatment of compartment syndrome are described. Complications of the syndrome may be life-threatening and permanently disabling. The anaesthetist should be aware of the potential complications of the operative positions of the unconscious patient.

Adult↗
PubMed1994

Is the construction of an ileoanal pouch associated with the development of arthritis?

The construction of an ileoanal pouch in patients who have undergone total colectomy for ulcerative colitis is claimed to be associated with the development of arthritis. The pattern and prevalence of reported musculoskeletal symptoms in the 15 patients with ileoanal pouches attending our hospital was not significantly different to a control group of 60 patients with an ileostomy for inflammatory bowel disease. There was no clinical or serological evidence of arthritis in the eight patients from the study group reporting symptoms. We conclude that the majority of symptoms occurring following pouch construction are not attributable to an inflammatory arthropathy.

Adolescent↗
PubMed1992

Long-term results after colectomy and endorectal ileal pullthrough procedure in children.

Since 1987, 67 children with ulcerative colitis, 14 with colonic polyposis, and one with Hirschsprung's disease have undergone colectomy and endorectal ileal pullthrough procedures (ERP). Seventy-six children had a lateral ileal reservoir and six had a straight pullthrough. Seventy-seven patients had a diverting ileostomy for 4 months; five polyposis patients underwent ERP without ileostomy. Forty-three (52%) developed complications, including ileoanal stenosis with reservoir outlet obstruction (14), elongated ileal spout (2), elongated ileal reservoir greater than 15 cm (4), and a combination of outflow obstruction, reservoir enlargement causing stasis (14), and intestinal obstruction causing obstruction (10). Thirty-two children required reoperation. Sixty per cent of complications occurred in the first 30 patients. Only three of the last 40 patients had reoperation. There were no deaths. Four children (two with Crohn's disease) returned to a permanent ileostomy (4.9%); 10 had a temporary ileostomy. Five straight pullthroughs were converted to a reservoir because of stool frequency; 19 underwent reservoir reconstruction because of stasis. Seventy-five children (91.5%) are currently progressing very well. Features for optimal function include a short rectal muscle cuff, a short reservoir and spout, and aggressive correction of rectal strictures. The ERP is a desirable option to proctocolectomy and ileostomy for children with colitis or polyposis.

Adenomatous Polyposis Coli↗
PubMed1992

Continuing evolution of the pelvic pouch procedure.

The results of the pelvic pouch procedure were reviewed to assess the surgical complication rate and outcome of patients who had had the procedure performed with a stapled ileo-anal anastomosis with and without a defunctioning ileostomy. Between December 1982 and March 1992, 483 patients underwent a pelvic pouch (PP) procedure. Patients were divided into three groups: group I consisted of 325 patients (178 men and boys and 147 women and girls) who underwent a PP procedure with a handsewn ileoanal anastomosis (IAA) with a defunctioning loop ileostomy. In group II, there were 87 patients (47 men and boys and 40 women and girls) who had a stapled IAA with a defunctioning ileostomy. Group III patients consisted of 71 patients (43 men and boys and 28 women and girls) who had a stapled IAA with no covering ileostomy. Assessment was made of the IAA leak rate, the surgical complications, the reoperation rate, and functional outcome. Early surgical complications included 40 (12%) IAA leaks in group I patients compared with only six (7%) leaks in group II patients who had a stapled IAA (p < 0.05). In group III patients, who had a stapled IAA but no covering ileostomy, there were 13 leaks (18%). Eleven of these 13 leaks healed spontaneously with tube drainage; one patient remains with a rectal tube in place 6 weeks after operation, and only one patient has required a reoperation (defunctioning ileostomy). Functionally, all patients with a healed IAA after a leak have had an excellent result comparable to those without a leak. Patients who were male, older than age 40, on steroids, and had had a true one-stage PP procedure, had a greater risk of developing an IAA leak. In two patients, there was intraoperative difficulty, and one of these patients had an IAA leak after operation. Disease activity at the resection margin and patient weight did not affect the leak rate. Our results suggest that the IAA leak rate is significantly reduced in patients with a stapled IAA with an ileostomy compared with those with a handsewn IAA. Omission of the defunctioning ileostomy is associated with a higher IAA leak rate, but spontaneous healing occurs in almost all patients without impairment of functional results. In patients in whom the ileostomy is omitted, the IAA leak rate is greatest in male patients who have undergone a true one-stage PP procedure, are on steroids, and are older than age 40.

Adenomatous Polyposis Coli↗
PubMed1993

The impact of familial adenomatous polyposis on the tumorigenesis and mortality at the several organs. Its rational treatment.

The authors reviewed the case records of 1050 familial adenomatous polyposis (FAP) patients who were registered at their institution. The organ-specific morbidity and mortality rates of malignant tumor in FAP patients were compared with those of the general population of Japan, and the prognosis after rectum-preserving operation also was calculated. The cumulative prevalence of colorectal carcinoma at the age of 44 years was 0.52 for men and 0.61 for women. The observed/expected morbidity ratio was 20.9 (95% confidence interval, 10.8-36.6) for thyroid carcinoma, 3.08 (2.03-7.75) for gastric carcinoma, and 295 (263-330) for colorectal carcinoma. The observed/expected mortality ratios was 250 (112-447) for periampullary and small intestinal carcinoma, 3.43 (1.77-6.0) for gastric carcinoma, and 210 (183-241) for colorectal carcinoma. The risk of rectal carcinoma after ileorectal anastomosis was 13% (8.5-17.5%) at 10 years and 37% at 20 years. The results clarified the impact of FAP on the carcinogenesis in several organs as a whole including preserved rectum, and suggested a direction of the rational treatment of FAP.

Adenomatous Polyposis Coli↗
PubMed1993

Axonal necrosis of enteric autonomic nerves in continent ileal pouches. Possible implications for pathogenesis of Crohn's disease.

OBJECTIVE: Axonal necrosis was first described in samples of small intestine from patients with Crohn's disease (A.M. Dvorak et al. Hum Pathol 1980; 11:620-634). Clinically evident inflammation of continent ileal reservoirs (pouches) has clinical features that resemble Crohn's disease. Possible similarities in the pathogenesis of Crohn's disease and pouchitis were sought using ultrastructural and microbiologic tools to identify damaged enteric nerves and tissue bacteria. METHODS: An encoded ultrastructural and microbiologic study of replicate biopsies from 114 samples of human intestine was done. Biopsies from ileum, colon, conventional ileostomy or continent pouch were obtained from patients with ulcerative colitis, Crohn's disease, or familial polyposis and grouped into three clinical study groups (control, normal pouch, pouchitis), based on clinical and endoscopic criteria. Biopsies were prepared for electron microscopy with standard methods; replicate biopsy samples were washed extensively before preparing cultures designed to identify aerobic as well as facultative and obligate anaerobic bacteria (Onderdonk et al. J Clin Microbiol 1992; 30:312-317). The ultrastructural diagnosis of damaged enteric nerves was based on previously published criteria for axonal necrosis (A.M. Dvorak and W. Silen. Ann Surg 1985; 201:53-63). Intergroup comparisons were tested for significance using Chi-square analysis. RESULTS: The highest incidence of axonal necrosis was present in Crohn's disease control biopsies (53%), regardless of whether bacteria were present (or not) in cultures of replicate biopsies. Axonal necrosis also occurred in more ulcerative colitis and familial polyposis biopsies (regardless of biopsy site) that had positive bacterial cultures than in those that did not (p < 0.001). In addition, axonal necrosis was documented in 42% of the pouch biopsies from ulcerative colitis and familial polyposis patients, particularly in those pouches that were found to be inflamed by clinical criteria and that also had positive bacterial cultures of the biopsied tissues. Control biopsies from patients with ulcerative colitis and familial polyposis had significantly less nerve damage than pouch biopsies in the presence of positive cultures (p < 0.01). Among the clinically inflamed pouches biopsied in ulcerative colitis or familial polyposis patients, we found that none had damaged enteric nerves when bacterial cultures were negative (p < 0.005). If the presence of axonal necrosis alone was compared with the presence of undamaged enteric nerves in all biopsies from patients with ulcerative colitis, a highly significant number of ulcerative colitis biopsies with axonal necrosis occurred in pouches (72%) compared with controls (p < 0.001). CONCLUSIONS: The ultrastructural finding of axonal necrosis in Crohn's disease confirms previous studies. The presence of damaged enteric nerves in patients with pouchitis provides ultrastructural support to the clinical impression of similarities between pouchitis and Crohn's disease. The association of damaged nerves and invasive bacteria in pouchitis suggests mechanistic similarities for the pathogenesis of Crohn's disease that requires further investigation.

Adenomatous Polyposis Coli↗