Preoperative Diagnosis Of Ulcerative Colitis Research Articles

P736 Class 4 Fistulizing Perineal Disease after Proctectomy for Crohn’s Disease: A Multicentre Study

Abstract Background The TOpCLASS International Consortium recently described a paradigm shifting classification scheme for perianal Crohn’s disease, based on the desired patient goals of care. Class 4 perineal fistulizing disease is a CD that persists after proctectomy, and there are limited data defining this entity. We sought to determine the rates of clinical perineal fistulizing disease after proctectomy and to secondarily define the risk factors for recurrence. Methods Adult patients with CD who underwent perineal resection with proctectomy or proctocolectomy at three hospitals within our institution’s health network between 2009 and 2021 were retrospectively reviewed. The exclusion criteria included a preoperative diagnosis of ulcerative colitis or indeterminate colitis, and patients who underwent restorative proctectomy. Extracted data included baseline demographic data, preoperative medical treatment for CD, proctectomy indication, clinical recurrence of perineal disease, and resolution of perineal disease. Results A total of 405 patients with Crohn’s disease were included, including 190 (46.9%) who underwent proctectomy and 215 (53.1%) who underwent proctocolectomy. The median age was 44 years (IQR 33-58) and 58.8% of the patients were female. Within three months prior to surgery, 36% were exposed to biologic therapy, 29.1% to immunomodulators, and 11.4% to corticosteroids. During a median follow-up of 4 years, clinical recurrence of fistulizing perineal CD occurred in 62 (15.3%) patients, including five patients with perineal-vaginal fistulas and one with adenocarcinoma in the fistula tract. The median time to the development of perineal disease was 6.5 months (IQR 3-23 months). Factors associated with perineal disease included younger age (38 vs. 46 years; p <0.01), female sex (71% female vs. 56.6% female; p=0.03), preoperative corticosteroid use (19.4% vs. 9.9%; p=0.031), and the presence of perianal CD, regardless of the indication for proctectomy (85.4% vs 60.1%; p<0.01). After treatment for class 4 disease, perineal symptom resolution was observed in 40 (62.9%) patients. There were 79 (19.5%) patients underwent examination under anesthesia (EUA) for perineal disease, with a median of 2 EUA’s (range 1-21). Preoperative biological therapy was not associated with recurrent perineal disease (p= 0.29). Conclusion Perineal CD after proctectomy is common and frequently requires multiple surgical interventions. Younger age, female sex, preoperative corticosteroid use, and the presence of perianal disease may benefit from a more intensive postoperative review and consideration of the continuation or early initiation of prophylactic post-proctectomy CD medical management with biologics.

High incidence of surgery and initiation of medical therapies after colectomy for ulcerative colitis or inflammatory bowel disease unclassified

Background and aimsDespite novel medical therapies, colectomy has a role in the management of patients with ulcerative colitis (UC) and inflammatory bowel disease unclassified (IBDU). This study aimed to determine the incidence of unplanned surgery and initiation of immunomodulatory or biologic therapy (IMBT) after colectomy in patients with UC or IBDU, and identify associated factors. MethodsData of patients with preoperative diagnosis of UC or IBDU who underwent colectomy and were followed up at a single tertiary centre was retrospectively collected. The primary outcome was the risk of unplanned surgery and initiation of IMBT during follow-up after colectomy. Secondary outcomes were development of Crohn's disease-like (CDL) complications and failure of reconstructive techniques. Results68 patients were included. After a median follow-up of 9.9 years, 32.4% of patients underwent unplanned surgery and IMBT was started in 38.2%. Unplanned surgery-free survival was 85% (95% confidence interval [CI] 73.8–91.6%) at 1 year, 76% (95% CI 63.2–84.9%) at 5 years and 69.1% (95% CI 55–79.6%) at 10 years. IMBT-free survival was 96.9% (95% CI 88.2–99.2%) at 1 year, 77.6% (95% CI 64.5–86.3%) at 5 years and 63.3% (95% CI 48.8–74.7%) at 10 years. 29.4% of patients met criteria for CDL complications. CDL complications were significantly associated to IMBT (hazard ratio 4.5, 95% CI 2–10.1). ConclusionIn a retrospective study, we found a high incidence of unplanned surgery and IMBT therapy initiation after colectomy among patients with UC or IBDU. These results further question the historical concept of surgery as a “definitive” treatment.

Controversies in Crohn's Disease Before and After Pouch Surgery.

IPAA is rarely performed in patients with a known diagnosis of Crohn's disease, although it may be done in highly selected patients with an absence of perianal disease or small bowel inflammation. Patients with a preoperative diagnosis of Crohn's disease are, however, at increased risk of developing Crohn's disease of the pouch. Crohn's disease of the pouch can also occur in people with a preoperative diagnosis of ulcerative colitis; however, it is unknown whether this phenotype is similar to classic Crohn's disease involving an intact bowel, or whether it is a distinct entity. This study aimed to describe Crohn's disease of the pouch, including luminal inflammation, and fibrostenotic and perforating phenotypes, as well as management of these conditions. Crohn's disease of the pouch can present in similar ways as classic Crohn's disease, classified as either inflammatory, fibrostenotic, or penetrating. Criteria involving the diagnosis of Crohn's disease of the pouch are stringent to prevent unnecessary treatments, because a diagnosis of Crohn's disease after IPAA for presumed ulcerative colitis can cause mental anguish. Treatment of Crohn's disease of the pouch depends on the presenting phenotype, although therapies are similar to classic Crohn's disease. Endoscopic, or even surgical treatment, may be required for fibrostenotic and fistulizing diseases. Crohn's disease of the pouch is a newly described disease of patients who undergo an ileoanal pouch anastomosis; and therefore, most of the evidence comes from retrospective trials in patients with an initial diagnosis of ulcerative colitis. While Crohn's disease of the pouch is controversial and perhaps a distinct disease within the spectrum of inflammatory bowel diseases, it nonetheless requires long-term medical therapy and close follow-up.

DOP29 The Normalization and Transition of J-Pouch Phenotype in the Chicago Classification of Pouchitis

Abstract Background Pouchitis can develop in up to 70% of patients with inflammatory bowel disease (IBD) treated by proctocolectomy with ileal pouch-anal anastomosis (IPAA). In our recent work, we proposed the “Chicago classification of pouchitis” describing 7 main pouch phenotypes with differing outcomes. In this study, we assessed the transition of pouch phenotypes in our classification. Methods This is a retrospective study of IBD patients with IPAA who subsequently underwent pouchoscopies at the University of Chicago between June 1997 and December 2019. We reviewed all available reports of pouchoscopies after ileostomy takedown and classified the pouches into 7 main phenotypes: (a) normal, (b) afferent limb (AL) involvement, (c) inlet (IL) involvement, (d) diffuse, (e) focal inflammation of the pouch body, (f) cuffitis, and (g) pouch fistulas (Figure 1). A normal pouch was defined as a pouch without any abnormal findings including fistulas. AL, IL involvement, and cuffitis were defined as any type of endoscopic inflammation in the AL, IL, and rectal cuff, respectively. Diffuse inflammation was defined as two or more endoscopic inflammatory findings in all anatomical locations of the pouch body (the tip, proximal, and distal pouch). Focal inflammation was defined as pouchitis that did not meet the criteria for diffuse inflammation. Pouch fistulas included pouches with any type of fistulas noted ≥6 months from ileostomy takedown. Using a Kaplan-Meier curve, event-free survival (EFS) was assessed from the scope date when a primary phenotype was first identified to the date when a subsequent phenotype was described. We excluded patients if a subsequent phenotype was identified before or at the scope date when a primary phenotype was initially noted. Logistic regression analysis was performed with variables with a P-value <0.05 in a Fisher’s exact test. Results We reviewed 1,359 pouchoscopies from 426 patients (90% preoperative diagnosis of ulcerative colitis). The most common subsequent inflammatory phenotype was focal inflammation (55%-88%), followed by IL involvement (31%-67%), cuffitis (33%-46%), diffuse inflammation (20%-38%), AL involvement (21%-32%), and pouch with fistulas (9%-20%). The rate of subsequent normal pouch was 6%-25% with pouch fistulas having the lowest normalization rate (6%, 5-year EFS 93%) (Table 1, Figure 2). On multivariable analysis, pouch fistulas were negatively associated with pouch normalization (OR 0.17, 95% CI 0.06–0.48) (Table 2). Conclusion We demonstrated that pouch phenotype can change over time and pouch normalization is possible. Further studies are needed to determine if pouch normalization should be a goal of treatment for IBD patients with IPAA.

P026 Assessment of Contributing Factors for Fistula Development in Patients with Inflammatory Bowel Disease Treated by Proctocolectomy with Ileal Pouch-Anal Anastomosis.

BACKGROUND: In patients with inflammatory bowel disease (IBD), surgical intervention is sometimes required due to medically refractory colitis or the development of neoplasia, and restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the most common procedure for those with colitis. However, despite a preoperative diagnosis of ulcerative colitis (UC), a phenotype of the J pouch which has features similar to Crohn’s disease (CD) may develop after the surgery. This study aims to assess the frequency and risk factors of fistula development in IBD patients with a J pouch. METHODS: This is a retrospective single-center study of IBD patients treated by total proctocolectomy with IPAA and who subsequently underwent pouchoscopies at the University of Chicago between January 2007 and December 2019. We reviewed the endoscopic findings in different anatomic areas of the pouch (the afferent limb, inlet, “tip of the J”, proximal and distal pouch, anastomosis, rectal cuff, anal canal, and perianal area). Endoscopic phenotypes were classified into 5 main categories: (1) afferent limb, (2) inlet involvement, (3) diffuse inflammation of the pouch body, (4) cuffitis, and (5) J pouch with fistulas. This analysis included pouches with any type of fistulas that developed ≥6 months after ileostomy takedown. We assessed clinical data and pathological findings of the resected colon including deep, focal inflammation, and granulomas generally thought to be unique features of CD. Deep inflammation included ulcerations, fissures, and lymphoid aggregates observed below the level of the mucosa. Fisher’s test was used for univariate analysis to assess factors contributing to fistula development. Logistic regression analysis was performed as a multivariable analysis including variables with a P-value < 0.10 in the univariate analysis. RESULTS: We reviewed 1,359 pouchoscopies from 426 IBD patients who underwent proctocolectomy with IPAA and identified 18.8% of patients (78/414) with a J pouch and fistulas ≥ 6 months from ileostomy takedown. Ninety-one percent of patients were preoperatively diagnosed with UC. The most frequent type of fistula was perianal fistula (60.3%), followed by enterovaginal or rectovaginal fistula (24.4%), and fistula from the pouch (7.7%). Among 325 IBD patients with available pathological reports of resected colon, 86 patients (26.5%) showed deep inflammation and 88.4% (76/86) of patients were diagnosed with UC rather than CD by our pathologists. On univariate analysis, we did not find any significant clinical factors for fistula development in patients with a J pouch. As for pathological data, our univariate and multivariable analyses showed that deep inflammation of the resected colon was significantly associated with fistula development (OR = 3.52; 95%CI = 1.84-6.72; P < 0.001), but focal inflammation and granulomas were not. Notably, this association was not observed in patients with other endoscopic phenotypes of the J pouch. CONCLUSION: In this tertiary cohort of patients with a preoperative diagnosis of UC, we found that deep inflammation of the resected colon is a specific predictor for fistula formation in the J pouch. These findings suggest that in such patients a careful monitoring and early treatment strategy may be beneficial.

Experience, Complications and Prognostic Factors of the Ileoanal Pouch in Ulcerative Colitis: An Observational Study

IntroductionIleoanal pouch following restorative proctocolectomy is the treatment for ulcerative colitis after failed medical treatment. Our main aim was to evaluate early and late morbidity associated with restorative proctocolectomy. The secondary aim was to assess risk factors for pouch failure. MethodsA retrospective, observational, single-center study was performed. Patients who had undergone restorative proctocolectomy for a preoperative diagnosis of ulcerative colitis from 1983 to 2015 were included. Early (<30 days) and late (>30 days) adverse events were analyzed. Pouch failure was defined as the need for pouch excision or when ileostomy closure could not be performed. Univariate and multivariate analyses were performed to assess pouch failure risk factors. ResultsThe study included 139 patients. One patient subsequently died in the early postoperative period. Mean follow-up was 23 years. Manual anastomoses were performed in 54 patients (39%). Early adverse events were found in 44 patients (32%), 15 of which (11%) had anastomotic fistula. Late adverse events were found in 90 patients (65%), and pouchrelated fistulae (29%) were the most commonly found in this group. Pouch failure was identified in 42 patients (32%). In the multivariate analysis, age >50 years ( P < .01; HR: 5.55), handsewn anastomosis ( P < .01; HR: 3.78), pouch-vaginal ( P = .02; HR: 2.86), pelvic ( P < .01; HR: 5.17) and cutaneous P = .01; HR: 3.01) fistulae were the main pouch failure risk factors. ConclusionRestorative proctocolectomy for a preoperative diagnosis of ulcerative colitis has high morbidity rates. Long-term outcomes could be improved if risk factors for failure are avoided.

Experiencia, complicaciones y factores pronósticos con el reservorio anal en la colitis ulcerosa. Estudio observacional

IntroductionIleoanal pouch following restorative proctocolectomy is the treatment for ulcerative colitis after failed medical treatment. Our main aim was to evaluate early and late morbidity associated with restorative proctocolectomy. The secondary aim was to assess risk factors for pouch failure. MethodsA retrospective, observational, single-center study was performed. Patients who had undergone restorative proctocolectomy for a preoperative diagnosis of ulcerative colitis from 1983-2015 were included. Early (<30 days) and late (>30 days) adverse events were analyzed. Pouch failure was defined as the need for pouch excision or when ileostomy closure could not be performed. Univariate and multivariate analyses were performed to assess pouch failure risk factors. ResultsThe study included 139 patients. One patient subsequently died in the early postoperative period. Mean follow-up was 23 years. Manual anastomoses were performed in 54 patients (39%). Early adverse events were found in 44 patients (32%), 15 of which (11%) had anastomotic fistula. Late adverse events were found in 90 patients (65%), and pouch-related fistulae (29%) were the most commonly found in this group. Pouch failure was identified in 42 patients (32%). In the multivariate analysis, age >50 years (p<0.01; HR: 5.55), handsewn anastomosis (p<0.01; HR: 3.78), pouch-vaginal (p=0.02; HR: 2.86), pelvic (p<0.01; HR: 5.17) and cutaneous p=0.01; HR: 3.01) fistulae were the main pouch failure risk factors. ConclusionRestorative proctocolectomy for a preoperative diagnosis of ulcerative colitis has high morbidity rates. Long-term outcomes could be improved if risk factors for failure are avoided.

High Incidence of Recurrent Crohn's Disease Following Colectomy for Ulcerative Colitis Revealed with Long Follow-Up.

Ulcerative colitis (UC) patients with progression of their disease despite optimized medical therapy may warrant "curative" proctocolectomy with end ileostomy or ileo-anal pouch (IPAA) anastomosis. The aim of our study was to assess the incidence of later recurrent ileitis that lead to altering the initial diagnosis to Crohn's disease (CD). A retrospective analysis was conducted on the inflammatory bowel disease database at Lenox Hill Hospital. The database consisted of patients that were diagnosed with UC or CD based on clinical assessment, endoscopic appearance, gross and histological examination, and imaging between 1960 and 2015. The post-colectomy follow-up period was at least 10years. Recurrent disease was classified by evidence of transmural inflammation in the distal ileum, fistulizing disease, or stricturing disease. From our IBD database, we identified 128 patients who underwent elective or urgent colectomy with the preoperative diagnosis of UC. Thirty-two (25%) had either an IPAA or end ileostomy with documented recurrence of inflammation in the small bowel mucosa consistent with CD. There was no significant difference between the type of surgical approach and the chance of recurrent disease (p=.20). The average time to clinically significant recurrence was 5years. The incidence of recurrent CD following colectomy for ulcerative colitis, when followed postoperatively for an average of 20years, was 25%, considerably more than previously reported. Patients who come to colectomy for ulcerative colitis and are followed for at least 10years show a high incidence of recurrent Crohn's disease in the ileostomy or ileo-anal pouch. Extended follow-up should be included in patients coming to colectomy for ulcerative colitis before they should be considered cured of their disease.

P644 Factors and salvage surgery for treatment of pouch failure after restorative proctocolectomy for ulcerative colitis – a single-institution study

Background: For many patients with ulcerative colitis (UC), total colectomy and pouch surgery (ileal pouch-anal canal anastomosis (IACA), ileal pouch-anal anastomosis (IAA)) are performed, improving the quality of life (QOL). However, pouch failure occurs in some patients, markedly reducing their QOL. In this study, we examined the course of patients with pouch failure. Methods: Of 1,156 patients who underwent total colectomy and pouch surgery under a preoperative diagnosis of UC between 1992 and 2015, the subjects were 26 patients (2.2%) in whom pouch-associated complications led to pouch failure (ileostomy state for 2 years or more or pouch resection). Total colectomy was indicated for 14 patients with refractory UC, 12 with severe UC, and 1 with cancer/dysplasia. Concerning techniques, IACA was selected for 21 patients, and IAA for 5. The median age at the time of pouch surgery was 31 years. The median interval from surgery until the appearance of complication-related symptoms was 19.5 months. The median follow-up period from pouch failure was 60 months. Results: Among complications causing pouch failure, fistulae from the anastomotic site accounted for 38% (10 patients), the highest percentage. In 4 of these, suture failure was noted as an early complication. In addition, anal canal-associated complications were observed in 34% (9 patients: 7 with anal fistulae, 1 with a vaginal fistula, and 1 with a pouch fistula (5%)). The final outcomes consisted of anal and pouch excision in 11 patients (after re-ileal pouch anal anastomosis in 2), pouch vacant in 14, and redo ileal pouch anal anastomosis in 1 (12%, 3/26). Of these, 1 required additional ileostomy. Finally, concerning the pouch function after stoma closure, the frequency of defecation was 10 to 15 times/day in both patients, facilitating rehabilitation. Conclusions: In UC patients with pouch failure, the incidence of complications related to fistulae from the anastomotic site was high, suggesting the possibility of early suture failure. For 92% of the subjects, permanent ileostomy was selected. However, redo ileal pouch anal anastomosis was possible in some patients, and closure was possible after temporary ileostomy in others. Treatment methods must be examined in accordance with individual patients.

Long-term Functional and Quality-of-Life Outcomes After IPAA in Children

The impact of IPAA on long-term outcomes in adults is favorable. Studies on functional outcome and quality of life in children are sparse. The aim of this study was to evaluate the functional and quality-of-life outcomes after IPAA in children and to investigate the possible correlations between the two. Standardized validated questionnaires of bowel/psychosocial functioning and quality of life were used to assess outcome. This study was conducted at a tertiary medical center. All children who had surgery at <18 years of age and were followed up at our institute were included. Global Assessment of Function Scale questionnaires were used to evaluate functional outcome. Quality of life in patients >18 years at the time of follow-up was scored by using the Short Form 36 questionnaire completed by the patients. Quality of life in the younger patients at the time of follow-up was scored using the Child Health Questionarie-28 filled by the parents. Data were obtained in 26 patients (25 with a preoperative diagnosis of ulcerative colitis) at a median follow-up of 6 years. The median age at surgery was 15 years. Quality of life was comparable to the general population for children >18 at the time of follow-up in 7 of 8 items, but was lower than in the general population in 7 of 12 items for those who were younger at the time of follow-up. The mean functional score was found to be negatively correlated to all 8 Short Form 36 parameters (r > 0.5; p < 0.005), but only to one of the Child Health Questionarie-28 parameters. This study was limited by its small sample size. The functional outcomes among pediatric patients undergoing IPAA are good. Parents' perception of their child's quality of life is poorer in comparison with the perception by patients themselves when older and with no correlation to the functional results.

Pre-Colectomy Appendectomy and Risk for Crohn’s Disease in Patients with Ileal Pouch-Anal Anastomosis

BackgroundA subset of patients with a pre-operative diagnosis of ulcerative colitis can develop Crohn’s disease (CD) of the pouch after restorative proctocolectomy. While appendectomy has been implicated to be associated with an increased risk for CD, its impact on the development of de novo CD of the pouch in patients’ ileal pouch-anal anastomosis (IPAA) has not been studied. The aims of the study were to assess the prevalence of CD of the pouch in patients with pre-colectomy appendectomy and to investigate the impact of appendectomy on the development of de novo CD of the pouch. MethodsAll eligible patients with restorative proctocolectomy and IPAA for IBD who had available information on pre-colectomy appendectomy were studied. Demographic and clinical characteristics were evaluated. Cox regression analysis was performed. ResultsThe study included 434 patients (44.9 % male) with a mean age of 45.2±14.4 years and follow-up of 4.6±2.3 years. Forty patients (9.2 %) had had appendectomy prior to colectomy. Appendectomy was not shown to be associated with CD of the pouch or its phenotypes in both univariable and multivariable analyses. In the Cox model, independent risk factors associated with CD of the pouch were active smoking (hazard ratio [HR] = 1.58; 95 % confidence interval [CI], 1.03–2.43) and family history of CD (HR = 1.82; 95 % CI, 0.99–3.32). ConclusionsWhile this study has shown no association between previous appendectomy and the development of CD of pouch, active smoking was an independent risk factor for development of CD of the pouch.

Serologic Profiles Change After Ileal Pouch-Anal Anastomosis in Patients with a Preoperative Diagnosis of Ulcerative Colitis

Serologic Profiles Change After Ileal Pouch-Anal Anastomosis in Patients with a Preoperative Diagnosis of Ulcerative Colitis

Diagnosis and Management of Postoperative Ileal Pouch Disorders

Although restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) has become the surgical treatment of choice for patients with refractory ulcerative colitis (UC) or UC with dysplasia, surgical, inflammatory, and noninflammatory adverse sequelae are common. Pouchitis, representing a spectrum of disease phenotypes, is the most common long-term complication of IPAA. De novo Crohn disease (CD) of the pouch can occur in patients with a preoperative diagnosis of UC. Differential diagnosis between fibrostenotic or fistulizing CD and surgery-associated strictures, sinuses, and fistulas often requires a combined assessment of symptom, endoscopy, histology, radiography, and examination under anesthesia. There is a role for endoscopic therapy for stricturing complications of IPAA. Chronic antibiotic-refractory pouchitis, refractory cuffitis, as well as fibrostenotic or fistulizing CD of the pouch are the leading late-onset causes for pouch failure.

Evaluation of endoscopic and imaging modalities in the diagnosis of structural disorders of the ileal pouch

Computerized tomography enterography (CTE), gastrograffin enema (GGE), magnetic resonance imaging (MRI), and pouch endoscopy (PES) have commonly been used to assess ileal pouch disorders. However, their diagnostic utility has not been systematically evaluated. The aims of this study were to compare these imaging techniques to each other and to optimize diagnosis of pouch disorders by using a combination of these diagnostic modalities. Clinical data of patients from the Pouchitis Clinic from 2003 to 2008 who had a PES and at least 1 additional imaging modalities (CTE, GGE, or MRI) used for evaluation of ileal pouch disorders were retrospectively evaluated. We analyzed the accuracy, sensitivity, specificity, negative predictive value (NPV), and positive predictive value (PPV) with which these tests were able diagnose pouch inlet and distal small bowel and pouch outlet strictures, pouch fistulas, sinuses, and leaks. Subsequently, accuracy was recalculated by combining 2 imaging modalities to see if this could enhance accuracy. A total of 66 patients underwent evaluation with PES and 1 other imaging modality as follows: PES + CTE (n = 23), PES + GGE (n = 34), and PES + MRI (n = 26). The mean age was 41.5 +/- 14.5 years, with 28 being female (42.4%). Sixty patients (90.9%) had J pouches and 59 (89.4%) had a preoperative diagnosis of ulcerative colitis. Overall, CTE, GGE, MRI, and PES all had reasonable accuracy for the diagnosis of small bowel and inlet strictures (73.9%-95.4%), outlet strictures (87.9%-92.3%), fistula (76.9%-84.8%), sinus (68.0%-93.9%), and pouch leak (83-93.9%). CTE had the lowest accuracy for small bowel and inlet strictures (73.9%) and MRI had the lowest accuracy for pouch sinus (68.0%). Combining 2 imaging tests can increase the accuracy of diagnosis to 100% for strictures, fistulas, sinus, and pouch leaks. CTE, GGE, MRI, and PES offer complementary information on disorders of the pouch and the combination of these tests increases diagnostic accuracy for complex cases.

Clinical Features and Outcomes of Crohnʼs Disease of the Ileal Pouch in Pediatric Patients

Purpose: Restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the surgical treatment of choice for children with ulcerative colitis (UC) and is frequently performed for patients with indeterminate colitis (IC) who require surgery. However, a subset of patients with an ileal pouch may develop Crohn's disease (CD) of the pouch. The aim of this study was to describe the clinical characteristics of pediatric patients with CD of the pouch and to compare these patients to adult patients with CD of the pouch. Methods: All pediatric patients with CD of the pouch were identified from our prospectively maintained Pouchitis Database between 2002 and 2008. Demographic, clinical, and endoscopic data were collected. A control group that consisted of 100 adult patients with CD of the pouch was used for comparison. CD of the pouch was classified into 3 phenotypes: inflammatory, fibrostenotic, and fistulizing. Results: A total of 25 pediatric patients with CD of the pouch were identified, predominantly Caucasian (96%) and female (56%) with a mean age of 11.2 (±4.4) years at the time of initial diagnosis with inflammatory bowel disease. Nineteen patients had a pre-operative diagnosis of UC and 6 patients had IC. Compared to adult patients, a diagnosis of toxic megacolon prior to IPAA was more common in the pediatric group (56% vs. 11%, p < 0.001), as well as the use of pre-operative biologics (44% vs. 10%, p < 0.001). CD of the pouch was inflammatory in 56%, fibrostenotic in 8% and fistulizing in 36%. The most common clinical presentation was diarrhea in 72%, followed by abdominal pain in 60% and hematochezia in 24% of the patients. Pediatric patients with CD of the pouch had a significantly shorter duration of inflammatory bowel disease prior to pouch creation (1 yr vs. 7 yrs, p < 0.001) and a shorter duration of their pouch prior to CD diagnosis (7 yrs vs. 10 yrs, p = 0.027). Children with CD of the pouch were more likely to be hospitalized than their adult counterparts (p < 0.001). Pouch failure, defined as the creation of a diverting ileostomy with or without pouch excision, occurred in 20% of pediatric patients with CD of the pouch compared to 7% of adult patients with a trend towards statistical significance (p = 0.06). Conclusion: In comparison to adult patients, CD of the pouch in children occurred earlier after IPAA and was associated with higher rates of hospitalization and pouch failure. A pre-operative diagnosis of toxic megacolon and use of biologics were more common in the pediatric population.

Outcomes in Patients with Ulcerative Colitis Undergoing Partial or Complete Reconstructive Surgery for Failing Ileal Pouch-Anal Anastomosis

Evaluate outcomes of patients with an original diagnosis of ulcerative colitis (UC) who required partial or complete ileal pouch reconstruction due to poor function or infectious complications. A prospectively collected ileal pouch-anal anastomosis (IPAA) database was reviewed retrospectively to identify UC patients undergoing major reconstructive revisions of their IPAA at our institution between 1981 and 2005. Functional results were derived from continued surveys of patients. Fifty-one UC patients were identified but 22 subsequently proved to have Crohns disease (CD). The initial IPAA was constructed at our institution in 32 patients and elsewhere in 19 patients. Indications for revision included infectious/inflammatory complications (65%) and mechanical difficulties (35%). Pouch revision was partial in 57% of patients and complete in 43%. There were no postoperative deaths. Following reconstruction, patients reported on average 5 daytime and 1 nighttime bowel movements. Daytime incontinence was occasional in 43% and frequent in 4%. Nighttime incontinence was occasional in 54% and frequent in 7%. The probability of pouch survival after reconstruction was 93% at 1 year and 89% at 5 years. Of the pouches that subsequently failed, 75% occurred in patients with a later diagnosis of CD. Postreconstruction abscess was a significant risk factor for ultimate pouch failure. In UC patients with failing IPAA, partial or complete pouch reconstruction can be done safely with good functional results, and may avoid pouch excision and permanent ileostomy in carefully selected patients, especially those with definite UC.

Family history of Crohnʼs disease is associated with an increased risk for Crohnʼs disease of the pouch

Crohn's disease (CD) of the pouch can occur in patients with restorative proctocolectomy and ileal pouch-anal anastomosis originally performed for a preoperative diagnosis of ulcerative colitis (UC). CD of the pouch was often observed in patients with a family history of CD. The purpose was to determine whether the family history of CD increased the risk for CD of the pouch in patients who underwent restorative proctocolectomy. A total of 558 eligible patients seen in the Pouchitis Clinic were enrolled, including 116 patients with CD of the pouch and 442 patients with a normal pouch or other pouch disorders. Demographic and clinical variables were included in the study. Multivariable logistic regression analyses were performed. The adjusted multivariate logistic analyses revealed that the risk for CD of the pouch was increased in patients with a family history of CD, with an odds ratio (OR) of 3.22 (95% confidence interval [CI] 1.56-6.67), or with a first-degree relative with CD (OR = 4.18, 95% CI, 1.48-11.8), or with a greater number of family members with CD (OR = 2.00 per family member, 95% CI, 1.19-3.37), adjusting for age, gender, smoking status, duration of IBD, duration of having a pouch, and a preoperation diagnosis of indeterminate colitis or CD. In addition, patients of younger age and longer duration of having a pouch had a higher risk for CD of the pouch. A diagnosis of CD of the pouch was associated with a poor outcome, with a greater than 5-fold estimated increased odds of pouch failure (OR = 5.58, 95% CI, 2.74-11.4). The presence of a family history of CD is associated with an increased risk for CD of the pouch, which in turn has a high risk for pouch failure.

Adenocarcinoma in Ileal Pouch-Anal Anastomosis: The Cleveland Clinic Experience

Purpose: Total proctocolectomy with ileal pouch-anal anastomosis substantially reduces the risk for dysplasia and cancer associated with ulcerative colitis (UC). Cancer in ileal pouch-anal anastmosis (IPAA) patients with underlying UC is a rare, which has only been reported as single cases. Natural history of IPAA cancer in these patients and its risk factors have not been investigated and there is lack of consensus for endoscopic cancer surveillance for these patients. The aim of the study was to characterize the natural history and clinical features, and histology in a group of patients with cancer of IPAA. Methods: Pouch database of 2,750 cases was searched. All patients had a preoperative diagnosis of UC or indeterminate colitis. Patients with cancer from the pouch and/or anal transitional zone (ATZ) were identified. Clinical features of these patients were characterized. Results: Eight patients (0.29%) with adenocarcinoma of the pouch and/or ATZ were identified from the database. Average age at cancer diagnosis was 56.8 ± 12.5 (SD) years. The mean duration from IBD diagnosis to cancer and from ileostomy take-down after IPAA to cancer diagnosis was 22.8 and 8.7 years, respectively. Most [5/8(62.5%)] had regular pouch endoscopic-surveillance after IPAA. Six cases had ATZ cancers and 2 had cancers of the pouch. Conclusion: The risk for cancer of IPAA is small but real, which can occur in UC patients without pre-colectomy diagnosis of dysplasia or cancer. A disproportional high number of these patients had post-operative Crohn's disease of the pouch. The majority of the patients had histologic poorly-differentiated cancer. Mucosectomy does not necessarily prevent cancers of the ATZ or pouch.Table: Demographic and Clinical Features of the 8 Patients with Cancer of IPAA

Risk Factors for Clinical Phenotypes of Crohn's Disease of the Ileal Pouch

Crohn's disease (CD) of the pouch can occur in patients with colectomy and ileal pouch-anal anastomosis (IPAA) originally performed for a preoperative diagnosis of ulcerative colitis. The clinical presentations of CD of the pouch are inflammatory, fibrostenotic, and fistulizing. Risk factors for clinical phenotypes of CD of the pouch have not been characterized. A total of 78 eligible patients with CD of the pouch together with 294 nonselected non-CD patients with IPAA seen in the Pouchitis Clinic were enrolled, including 28 with inflammatory CD, 18 with fibrostenotic CD, and 32 with fistulizing CD. The clinical phenotypes of CD were diagnosed based on a combined assessment of clinical, endoscopic, radiographic, and histologic features. Three separate analyses were performed, and for each analysis, the outcome of interest was having one of the phenotypes versus not having it. A stepwise selection multivariable logistic regression analysis was used. In the multivariable analysis, the risk factor for inflammatory CD was higher afferent-limb endoscopy scores (hazard ratio [HR] 1.87 95% confidence interval [CI] 1.54-2.27); the risk factors for fibrostenotic CD were higher afferent-limb (95% CI 1.81-3.48, HR 2.51) and higher cuff (95% CI 1.01-1.84, HR 1.36) endoscopy scores; and for fistulizing CD the risk factors were younger age (95% CI 0.93-0.99, HR 0.96), female gender (95% CI 1.35-6.97, HR 3.07), a preoperative diagnosis of indeterminate colitis (95% CI 1.72-9.34, HR 4.00), and no use of nonsteroidal antiinflammatory drugs (95% CI 1.31-8.25, HR 3.28). Each of the three phenotypes of CD of the pouch was associated with certain risk factors, suggesting that each of these diseases has a different etiology and disease process. The identification and management of some of the modifiable risk factors may reduce CD-related morbidity.

Anti– Saccharomyces cerevisiae Antibodies Are Associated With the Development of Postoperative Fistulas Following Ileal Pouch-Anal Anastomosis

Although serologic testing for perinuclear antineutrophil cytoplasmic antibodies (pANCA) and anti-Saccharomyces cerevisiae antibodies (ASCA) is reportedly useful in distinguishing ulcerative colitis (UC) from Crohn's disease (CD), there are few and conflicting reports assessing their utility in predicting postoperative complications after ileal pouch-anal anastomosis (IPAA). We examined the associations between postoperative complications such as pouchitis or fistulas and pANCA and ASCA antibodies in a group of patients who underwent IPAA for UC. We conducted a retrospective chart review of 34 patients initially diagnosed with UC (four of these patients had a diagnosis of indeterminate colitis) who underwent IPAA by a single surgeon, and who had pANCA and ASCA antibody levels measured during their clinical course. Study patients were assigned to four groups based on the pattern of antibody reactivity: pANCA+/ASCA- (16 patients), pANCA-/ASCA+ (nine patients), pANCA+/ASCA+ (five patients), and pANCA-/ASCA- (four patients). The median length of follow-up was 16 months (3-144 months). None of the patients (0 of 16) who were pANCA+/ASCA- had their preoperative diagnosis of UC changed after a median follow-up of 14 months (3-118 months). Of the nine patients with a preoperative diagnosis of UC who were pANCA-/ASCA+, four patients (44%) had their diagnosis changed postoperatively to CD based on clinical findings, with a median follow-up: 15 months (5-98 months). Of 16 patients who underwent IPAA and who were pANCA+/ASCA-, 15 of 16 (93.75%), were free of fistulas postoperatively, with a median follow-up of 14 months (3-118 months). Of nine patients with a preoperative diagnosis of UC who underwent IPAA and who were pANCA-/ASCA+, four of nine (44%; p = 0.04) developed fistulas postoperatively, with a median length of follow-up of 55 months (15-67 months). No relationship between serologic profiles or antibody titer levels and the development of pouchitis was identified. In a cohort of patients undergoing IPAA for UC, serologic profiles may be useful in identifying patients at risk of postoperative fistula formation. Patients who were pANCA-/ASCA+ were at increased risk for the development of fistulas postoperatively compared to patients who were pANCA+/ASCA-, and were also more likely to have their diagnosis changed postoperatively to CD. A larger study is needed to validate these observations.