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Surgery for pediatric ulcerative colitis.

Restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) has become the "gold standard" operation for treatment of ulcerative colitis refractory to medical therapy. The J-pouch is the simplest to construct, produces the fewest complications, and is used most commonly. Long-term function with the IPAA is good in approximately 90% of cases, and patient satisfaction is high. Controversial aspects of the operation include 1) whether mucosectomy and hand-sewn IPAA is better than anal transition zone preservation and stapled anastomosis, 2) whether a temporary diverting ileostomy should be used, 3) the role for IPAA in the treatment of indeterminate colitis, 4) the etiology and the most effective treatment for pouchitis, and 5) the best method for salvaging the failed pelvic pouch.

Anal Canal↗

Azathioprine or 6-mercaptopurine before colectomy for ulcerative colitis is not associated with increased postoperative complications.

AIM: To determine whether the use of azathioprine/6-mercaptopurine before colectomy is associated with an increased rate of postoperative complications. METHODS: All patients who underwent colectomy with ileal pouch-anal anastomosis for ulcerative colitis between 1997 and 1999 were identified. Medical records were abstracted for demographics, extent and duration of disease, dose and duration of corticosteroids and azathioprine/6-mercaptopurine, albumin, and Truelove/Witts score. Early (30-day) and late (6-month) complications were identified. Noncorticosteroid immunosuppressive use was coded as none, azathioprine/6-mercaptopurine within 1 week of surgery, or therapy with other immunosuppressive agents within 1 month of surgery. A logistic regression analysis assessed the association between these variables and complications. RESULTS: Early complications occurred in 49 of 151 (32%) patients not treated with immunosuppressive agents, 12 of 46 (26%) azathioprine/6-mercaptopurine-treated patients, and 4 of 12 (33%) patients treated with other immunosuppressive agents (p = 0.71). Late complications occurred in 72 of 148 (49%), 20 of 46 (43%), and 8 of 12 (67%) patients in these same groups, respectively. Intravenous or oral steroids at doses of 40 mg/d or greater (p < 0.01) and severe or fulminant disease (p = 0.0094) were associated with greater early complication rates. CONCLUSION: Early complications after restorative proctocolectomy for ulcerative colitis are associated with high dose steroids and severe disease but not use of azathioprine/6-mercaptopurine.

Adolescent↗

Current status of ileoanal reservoirs: 1992.

Restorative proctocolectomy with ileoanal reservoir has been a significant development in the surgical treatment of mucosal ulcerative colitis and familial adenomatous polyposis. It enables excision of the diseased colorectal mucosa with preservation of continence and avoidance of a permanent stoma. The surgical techniques, criteria for patient selection, and understanding of the physiology and outcome of the procedure, however, continue to evolve.

Postoperative Complications↗

Quantification of risk for pouch failure after ileal pouch anal anastomosis surgery.

OBJECTIVE: To identify risk factors associated with ileal pouch failure and to develop a multifactorial model for quantifying the risk of failure in individual patients. SUMMARY BACKGROUND DATA Ileal pouch anal anastomosis (IPAA) has become the treatment choice for most patients with ulcerative colitis and familial adenomatous polyposis who require surgery. At present, there are no published studies that investigate collectively the interrelation of factors related to ileal pouch failure, nor are there any predictive indices for risk stratification of patients undergoing IPAA surgery. METHODS: Data from 23 preoperative, 7 intraoperative, and 10 postoperative risk factors were recorded from 1,965 patients undergoing restorative proctocolectomy in a single center between 1983 and 2001. Primary end point was ileal pouch failure during the follow-up period of up to 19 years. The "CCF ileal pouch failure" model was developed using a parametric survival analysis and a 70%:30% split-sample validation technique for model training and testing. RESULTS: The median patient follow-up was 4.1 year (range, 0-19 years). Five-year ileal pouch survival was 95.6% (95% CI, 94.4-96.7). The following risk factors were found to be independent predictors of pouch survival and were used in the final multivariate model: patient diagnosis, prior anal pathology, abnormal anal manometry, patient comorbidity, pouch-perineal or pouch-vaginal fistulae, pelvic sepsis, anastomotic stricture and separation. The model accurately predicted the risk of ileal pouch failure with adequate calibration statistics (Hosmer Lemeshow chi2 = 3.001; P = 0.557) and an area under the receiver operating characteristics curve of 82.0%. CONCLUSIONS: The CCF ileal pouch failure model is a simple and accurate way of predicting the risk of ileal pouch failure in clinical practice on a longitudinal basis. It may play an important role in providing risk estimates for patients wishing to make informed choices on the type of treatment offered to them.

Adenomatous Polyposis Coli↗

Long-term outcome of familial adenomatous polyposis patients after restorative coloproctectomy.

BACKGROUND: Restorative proctocolectomy (RPC) eliminates the risk of colorectal adenocarcinoma in familial adenomatous polyposis (FAP) patients, but desmoid tumors, duodenal, and ileal adenomas can still develop. Our aim was to assess the long-term outcome of FAP patients after RPC. PATIENTS AND METHODS: FAP patients who had RPC between 1983 and 1990 were contacted for interview and upper gastrointestinal (GI) and ileal pouch endoscopy. RESULTS: Sixty-two males and 48 females had undergone hand-sewn RPC during this period. One patient died postoperatively (0.9%). Among 96 patients available for a minimal follow-up of 11 years, 7 patients died: 3 from causes unrelated to FAP, 2 from metastatic colorectal cancer, and 2 from mesenteric desmoid tumor (MDT). Thirteen patients had a symptomatic MDT (13.5%). Of 73 patients who had an upper GI endoscopy, 52 developed duodenal and/or ampullary adenomas. Four patients required surgical treatment of their duodenal lesions. Among 54 patients who underwent ileal pouch endoscopy, pouch adenomas were noted in 29. No invasive duodenal or ileal pouch carcinoma were detected. Functional results of RPC were significantly worse in MDT patients. CONCLUSIONS: RPC eliminates the risk of colorectal cancer, and close upper GI surveillance may help prevent duodenal malignancy. MDTs are the principal cause of death, once colorectal cancer has been prevented, and the main reason for worsening functional results.

Adenomatous Polyposis Coli↗

Systematic review of surgical options for idiopathic megarectum and megacolon.

OBJECTIVE: A subgroup of patients with intractable constipation has persistent dilatation of the bowel, which in the absence of an organic cause is termed idiopathic megabowel (IMB). The aim of this systematic review was to evaluate the published outcome data of surgical procedures for IMB in adults. METHODS: Electronic searches of the MEDLINE (PubMed) database, Cochrane Library, EMBase, and Science Citation Index were performed. Only peer-reviewed articles of surgery for IMB published in the English language were evaluated. Studies of all surgical procedures were included, providing they were performed on 3 or more patients, and overall success rates were documented. Studies were critically appraised in terms of design and methodology, inclusion criteria, success, mortality and morbidity rates, and functional outcomes. RESULTS: A total of 27 suitable studies were identified, all evidence was low quality obtained from case series, and there were no comparative studies. The studies involved small numbers of patients (median 12, range 3-50), without long-term follow-up (median 3 years, range 0.5-7). Inclusion of subjects, methods of data acquisition, and reporting of outcomes were extremely variable. Subtotal colectomy was successful in 71.1% (0%-100%) but was associated with significant morbidity related to bowel obstruction (14.5%, range 0%-29%). Segmental resection was successful in 48.4% (12.5%-100%), and recurrent symptoms were common (23.8%). Rectal procedures achieved a successful outcome in 71% to 87% of patients. Proctectomy, the Duhamel, and pull-through procedures were associated with significant mortality (3%-25%) and morbidity (6%-29%). Vertical reduction rectoplasty (VRR) offered promising short-term success (83%). Pelvic-floor procedures were associated with poor outcomes. A stoma provided a safe alternative but was only effective in 65% of cases. CONCLUSIONS: Outcome data of surgery for IMB must be interpreted with extreme caution due to limitations of included studies. Recommendations based on firm evidence cannot be given, although colectomy appears to be the optimum procedure in patients with a nondilated rectum, restorative proctocolectomy the most suitable in those with dilatation of the colon and rectum, and VRR in those patients with dilatation confined to the rectum. Appropriately designed studies are required to make valid comparisons of the different procedures available.

Adolescent↗

What are the differences in body image between patients with a conventional stoma compared with those who have had a conventional stoma followed by a continent pouch?

This paper describes a qualitative study of seven patients which explored the perceptions of body image in patients with a conventional stoma, compared with those who have had a conventional stoma followed by a continent pouch. The operation of choice for patients with ulcerative colitis (the group of patients in the study) who wish to avoid permanent ileostomy is restorative proctocolectomy with ileal reservoir, more commonly known as the 'pouch' procedure. The outcome of this study, using a phenomonenological approach, reflects the problems faced by patients undergoing stoma surgery; that is, patients with a stoma, on the whole, expressed difficulties in coming to terms with an ostomy, with perceived negative feelings of body image. In contrast, patients with a continent pouch, provided they were not experiencing difficulties with the pouch, were of the opinion that such a procedure enhanced their lives over that of an ileostomy. However, in examining these patients' feelings, it was found that the majority still considered their experience traumatic. This implies that the benefits of a continent pouch were not so obvious, as body image changes of stoma surgery still had a perceived negative effect on the patients. This affected the outcome of the study so that the differences of the two groups of patients were not as significant as perceived by the writer.

Adaptation, Psychological↗

Prospective assessment of Cleveland Global Quality of Life (CGQL) as a novel marker of quality of life and disease activity in Crohn's disease.

OBJECTIVES: The Short Form 36 (SF-36) questionnaire is the most widely accepted measure of quality of life (QOL); however, it is cumbersome to use and requires complicated analysis. The Cleveland Global Quality of Life (CGQL) is a simpler tool, which has been validated in patients with ulcerative colitis undergoing restorative proctocolectomy. This study validates CGQL in patients with Crohn's disease and determines the correlation of QOL measurement systems with disease activity as measured by the Crohn's Disease Activity Index (CDAI). METHODS: All patients with Crohn's disease presenting to this department between September 2001 and February 2002 were prospectively entered into a database, including demographic data, disease manifestations, Vienna classification, age at diagnosis, and duration of Crohn's disease. SF-36, CGQL, and CDAI scores were entered concurrently. Cronbach's alpha was used to assess the internal consistency among the components of the CGQL score. Correlation among various QOL measures was assessed with Spearman's test. Relationships between QOL measures and various baseline factors were assessed by Wilcoxon rank sum test, Student's t test, Kruskal-Wallis test, and Pearson's test, depending on the type and distribution of data. Change from baseline in the CGQL was assessed with the Wilcoxon signed rank test. RESULTS: One hundred seventy-eight (178) patients were entered into the database, and 169 completed QOL and CDAI data. There was a significant correlation between SF-36 and CGQL. CGQL and SF-36 correlated similarly with CDAI. Neither patient age, gender, or smoking history were associated with CGQL. CONCLUSIONS: CGQL correlates with disease activity and is a simple measure of QOL in Crohn's disease.

Adult↗

Clinical approach to diseases of ileal pouch-anal anastomosis.

Restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the surgical treatment of choice for ulcerative colitis (UC) patients with medically refractory disease or dysplasia. IPAA significantly improves quality of life in UC patients who require surgery. However, certain inflammatory and noninflammatory diseases can develop after the surgery, including pouchitis, Crohn's disease of the pouch, cuffitis, and irritable pouch syndrome. The etiology and pathogenesis of these disease conditions of IPAA are largely unknown. Accurate diagnosis and classification are important for appropriate management. Endoscopic evaluation is the most important tool for the diagnosis and differential diagnosis.

Adrenal Cortex Hormones↗

Pouchitis--recurrence of the inflammatory bowel disease?

The incidence and characteristics of reservoir inflammation after restorative proctocolectomy for ulcerative colitis were studied in a series of 179 patients. The median follow up time was 27 months (range 6-80). Pouchitis occurred in 36 patients (20%) and nine of these (5%) developed a chronic, persisting pouchitis. There were no pouch failures as a result of pouchitis and no significant adverse effect on longterm functional outcome. The overall cumulative risk to develop pouchitis four years after surgery was 23%. The risk of pouchitis is unpredictable on clinical grounds except that there were significantly less patients with left sided colitis in the group who subsequently developed pouchitis. Morphological and histochemical studies showed a greater degree of colonic metaplasia in the pouch mucosa in patients with pouchitis and patients with a chronic pouchitis had the highest degree of changes. The results support the view that pouchitis is a novel manifestation of inflammatory bowel disease in ileal mucosa that has changed slowly to a colon like mucosa.

Adolescent↗

Breath hydrogen analysis in patients with ileoanal pouch anastomosis.

The possible influence on functional outcomes of hydrogen production in the ileoanal pouch after restorative proctocolectomy was investigated by means of lactulose H2 breath tests. Eight of 15 patients had significant increases in breath hydrogen after 10 g lactulose. One patient declined to participate in further investigations, the remaining seven responders had no evidence of small bowel bacterial overgrowth after glucose H2 breath tests. The ability to produce hydrogen by anaerobic fermentation of lactulose in the pouch was unrelated to the age of the patients or of the pouch. Seven of eight responders had successive breath tests after ingestion of lactulose 20 g and wheat starch 100 g. Five of seven had significant increases after lactulose but none after wheat starch. The overall function of the pouch continence, spontaneity of defecation, and 24 hour stool frequency was significantly better in responders than in non-responders. The absence of H2 production of 100 g wheat starch may indicate either increased absorption or defective fermentation.

Adolescent↗

Contribution of gastrointestinal transit and pouch characteristics in determining pouch function.

AIM: To determine the contributions of gastrointestinal transit and pouch characteristics to bowel frequency in patients with an ileoanal reservoir and no pouchitis. METHODS: Twenty one patients who had undergone restorative proctocolectomy, with ileostomy closure at least eight months previously, and who had no history of pouchitis were recruited. They were prospectively classified on the basis of their bowel frequency: 11 patients had good pouch function (bowel frequency less than six per day) and 10 had poor function (bowel frequency more than six per day). Gastrointestinal transit was studied using a dual isotope technique and anal and pouch physiological examination was performed on all patients. RESULTS: Lag phase, 25% and 50% gastric emptying, small bowel transit time, and 10% and 50% pouch filling times, all for solids and liquids, were not significantly different between patients with good and poor function. Anal manometry and pouch and anal electrical sensitivity were also similar in the two groups. The volume of air and water required to elicit an initial sensation and the urge to defaecate were similar in both groups, but the maximum tolerated volume to both air (525 v 245 ml, good v poor function, median values) and water (625 v 370 ml) infusion was significantly (both p < 0.02) lower in patients with poor function. CONCLUSION: Maximum tolerated volume in the pouch, which may reflect pouch size, sensitivity, compliance, or a combination of these is the major determinant of pouch function. Gastrointestinal transit does not seem to be an important determinant of function.

Adult↗

Pouchitis in ulcerative colitis: correlation between predictors from colectomy specimens and clinico-histological features.

Pouchitis after restorative proctocolectomy for ulcerative colitis is usually of ill-defined etiology and is encountered with sclerosing cholangitis, bacterial overgrowth, and ischemia. Recently, appendiceal involvement, ileitis, and fissures in the colectomy specimen have been associated with short- and long-term development of pouchitis. To corroborate these recent findings, the histology of 40 colectomies (70% males; mean age 46.3 years, age range 20-70 years; mean follow-up period 3.7 years, range 1-13 years) with yearly follow-up biopsies was correlated with pouchitis and clinical symptoms. Appendicitis, fissures, and ileitis were present in 47, 45 and 5% of the patients, respectively. Pouchitis in patients with appendicitis or with fissures was noted in 44 and 50% at first biopsy and in 70 and 58% during follow-up (p = NS). Of the patients without appendicitis or without fissures, 33 and 33% demonstrated pouchitis at the first biopsy and 30 and 55% during follow-up (p = NS). Clinico-histological correlation revealed normal/near-normal biopsies with the lowest clinical severity score in 77% and with the highest clinical score in 43% (p < 0.025). The histological findings of appendiceal involvement, fissuring ulcers, and ileitis in colectomies for ulcerative colitis do not correlate with the finding of pouchitis in early or late pouch biopsies. A high clinical suspicion score is frequently not correlated with significant inflammation of the pouch.

Adult↗

Pigmented mesenteric lymphadenopathy in familial adenomatous polyposis - an unusual cause of intraoperative abandonment of ileo-anal pouch.

Familial adenomatous polyposis (FAP) is an autosomal dominant condition with near complete penetrance, characterised by the presence of numerous adenomatous polyps of the colon and rectum. Melanosis coli describes the brownish-black discolouration of the colon resulting from the accumulation of a granular pigment in the phagosomes of macrophages in the colonic lamina propria. The presence of melanosis pigment in pericolonic lymph nodes has been reported in patients with coincidental melanosis coli, following segmental colonic resection. We report a unique case of FAP with melanosis pigment in lymph nodes in the small bowel mesentery that initially prevented a restorative proctocolectomy but that resolved following a colectomy, subsequently facilitating formation of an ileo-anal pouch.

Adenomatous Polyposis Coli↗

Pouchitis: extracolonic manifestation of ulcerative colitis?

Pouchitis is the most frequent complication of ileal pouch-anal anastomosis for treatment of ulcerative colitis. There are several possible explanations. Among them, we focus on the one that considers pouchitis as an extracolonic manifestation of ulcerative colitis. The aim of this study was to investigate the association between pouchitis and extra-intestinal manifestations (EIM), which are frequent in these patients. Sixty patients underwent restorative proctocolectomy with an ileal J pouch (IPAA) from September 1984 to December 1998. Pouchitis was defined by clinical, endoscopic, and histologic criteria. The following extra-intestinal manifestations were studied: articular, cutaneous, hepatobiliary, ocular, genitourinary, and growth failure. Thirteen patients, of which 10 were female (76.9%), developed one or more episodes of pouchitis. Twelve patients of this group (92.3%) presented some kind of extra-intestinal manifestation, 4 pre-operatively (exclusively), 2 post-operatively (exclusively), and 6 both pre- and post-operatively (1.7 per patient). Twenty patients (42.7%) of the 47 without pouchitis did not present extra-intestinal manifestations; 10/35 (28. 5%) of females had pouchitis, compared to 3/35 (12.0%) of men. Pouchitis was more frequent among females, though not statistically significant. EIM increases the risk of pouchitis. Pouchitis is related to EIM in 92.3 % of cases, corroborating the hypothesis that it could be an extracolonic manifestation of ulcerative colitis.

Colitis, Ulcerative↗

The influence of ileal pouch-anal anastomosis for ulcerative colitis on ileal glycoprotein synthesis.

Restorative proctocolectomy and ileal pouch-anal anastomosis are frequently associated with the onset of low-grade inflammation, colonic metaplasia, and pouchitis. The influence of intestinal adaptation and inflammatory activity on ileal glycoprotein synthesis (GS) has been assessed. Ileal GS activity in controls (n = 13, 28.9 +/- 3.6) was similar to that of patients with ulcerative colitis (n = 18, 26.4 +/- 3.6) irrespective of the presence of backwash ileitis (n = 6, 22.0 +/- 4.0). Ileoanal pouch construction was not associated with a significant change in ileal GS activity regardless of the presence of low-grade inflammation (n = 19, 25.9 +/- 2.2), frank pouchitis (n = 5, 24.4 +/- 4.6), or transformation to a colonic type of mucosa (n = 6, 25.4 +/- 5.2). The transformation of ileal mucosa from an absorptive to a storage function is associated with characteristic morphologic and inflammatory changes but does not produce a protective response mediated by increased GS.

Adaptation, Physiological↗

Intestinal intraluminal continuity is a prerequisite for the distal bowel motility response to feeding.

BACKGROUND: We wanted to elucidate further the regulation of the intestinal motility response to feeding. METHODS: After intraduodenal administration of an oleate solution, mimicking a meal, the distal bowel motility and the plasma levels of bile acids, cholecystokinin (CCK), and neurotensin were monitored in patients operated on with restorative proctocolectomy (n = 4) or low anterior resection of the rectum (n = 4). Investigations were performed both with and without a diverting loop ileostomy. RESULTS: Intraduodenal sodium oleate elicited a prompt and significant increase in distal bowel motility. The motility response failed to appear when the luminal flow was diverted by a loop ileostomy. An increase in plasma CCK preceded the motility increase, but CCK was increased also in patients with a loop ileostomy. Whereas plasma bile acid levels were significantly increased after 30-45 min (p < 0.05), both with and without a loop ileostomy, neurotensin levels were not affected. CONCLUSION: Intestinal continuity is a prerequisite for the distal bowel motility response, indicating that apart from other possible mechanisms, luminal factors are involved in the regulation of intestinal motility.

Adult↗

Gardner's syndrome: a case report and review of the literature.

Gardner's syndrome is an autosomal dominant disease characterized by the presence of colonic polyposis, osteomas and a multitude of soft tissue tumors. The syndrome may present at any age from 2 mo to 70 years with a variety of symptoms, either colonic or extracolonic. We present a case of a 11-year-old female patient with Gardner's syndrome who presented with a lumbar area desmoid tumor and treated with resection of the desmoid, restorative proctocolectomy and ileal pouch anal anastomosis, A review of the current literature has been performed.

Child↗