Surgical Perspectives

Statement 1

Crohn’s Disease with DALMs, high grade dysplasia or multifocal low grade dysplasia of the colon and rectum should undergo surgical resection.

based on expert opinion

unifocal vs multifocal high grade dysplasia

DALM: development of dysplastic mass due to cancer potential of chronic colitis

DALM vs sporadic adenoma: endoscopic appearance, histo, pt age, duration of disease

Decision for colectomy affected by:

ongoing colitic symptoms

life expectancy

duration and extent of disease

primary sclerosing cholangitis

FHx CRC

shortcomings of colonoscopic surveillance

Zisman TL, Rubin DT. Colorectal cancer and dysplasia in inflammatory bowel disease. World J Gastroenterol 2008; 14: 2662-2669

Canavan C, Abrams KR, Mayberry J. Meta-analysis: colorectal and small bowel cancer risk in patients with Crohn’s disease. Aliment Pharmacol Ther 2006; 23: 1097-1104

Ekbom A, Helmick C, Zack M, Adami HO. Increased risk of large-bowel cancer in Crohn’s disease with colonic involvement. Lancet 1990; 336: 357-359

• Evidence: III, C

Statement 1 (amendments)

Crohn’s Disease with DALMs and high grade dysplasia of the colon and rectum should undergo surgical resection. [III-C]

LGD, options, independent two GI pathologist (to discuss in sub text)

Statement 2

Patients with long standing Crohn’s disease of the terminal ileum, ileocolon, or upper gastrointestinal locations should undergo biopsy of suspicious lesions at the time of stricturoplasty.

Statement 2

case reports

expert opinion

Statement 2Weedon DD, Shorter RG, Ilstrup DM, Huizenga KA, Taylor WF. Crohn's disease and cancer. N Engl J Med. 1973 Nov 22;289(21):1099–1103.

Fresko D, Lazarus SS, Dotan J, Reingold M. Early presentation of carcinoma of the small bowel in Crohn's disease ("Crohn's carcinoma"). Case reports and review of the literature. Gastroenterology. 1982 Apr;82(4):783–789.

Simpson S, Traube J, Riddell RH. The histologic appearance of dysplasia (precarcinomatous change) in Crohn's disease of the small and large intestine. Gastroenterology. 1981 Sep;81(3):492–501.

Newman RD, Bennett SJ, Pascal RR. Adenocarcinoma of the small intestine arising in Crohn's disease. Demonstration of a tumor-associated antigen in invasive and intraepithelial components. Cancer. 1975 Dec;36(6):2016–2019.

Perzin KH, Peterson M, Castiglione CL, Fenoglio CM, Wolff M. Intramucosal carcinoma of the small intestine arising in regional enteritis (Crohn's disease). Report of a case studied for carcinoembryonic antigen and review of the literature. Cancer. 1984 Jul 1;54(1):151–162.

Collier PE, Turowski P, Diamond DL. Small intestinal adenocarcinoma complicating regional enteritis. Cancer. 1985 Feb 1;55(3):516–521.

Fleming KA, Pollock AC. A case of 'Crohn's carcinoma'. Gut. 1975 Jul;16(7):533–537.

Riddell RH, Goldman H, Ransohoff DF, Appelman HD, Fenoglio CM, Haggitt RC, Ahren C, Correa P, Hamilton SR, Morson BC, et al. Dysplasia in inflammatory bowel disease: standardized classification with provisional clinical applications. Hum Pathol. 1983 Nov;14(11):931–968.

Intestinal adenocarcinoma in Crohn's disease: a report of 30 cases with a focus on coexisting dysplasia.Sigel JE, Petras RE, Lashner BA, Fazio VW, Goldblum JR. Am J Surg Pathol. 1999 Jun; 23(6):651-5.

• Evidence: III, C

Statement 2 (Amendments)

Patients with long standing small bowel Crohn’s disease should undergo biopsy of the stricture at the time of stricturoplasty.[III-C]

Statement 3

Patients who require surgery for disease of the rectum may undergo total proctocolectomy or proctectomy with creation of a stoma.

Restorative proctocolectomy with IPAA can be considered in this situation if the small bowel is unaffected and there is no perianal disease.

Phillips RKS. Ileal pouch-anal anastomosis for Crohn's disease. Gut. 1998;43:303-304.

Brown CJ, MacLean AR, Cohen Z, MacRae HM, O’Connor BI, McLeod RS. Crohn's disease and indeterminate colitis and the ileal pouch-anal anastomosis: outcomes and patterns of failure. Dis Colon Rectum. 2005;48:1542-1549.

Braveman JM, Schoetz DJ, Marcello PW, Roberts PL, Coller JA, Murray JJ. The fate of the ileal pouch in patients developing Crohn's disease. Dis Colon Rectum. 2004;47:1613-1619.

Hartley JE, Fazio VW, Remzi FH, Lavery IC, Church JM, Strong SA. Analysis of the outcomes of ileal pouch-anal anastomosis in patients with Crohn's disease. Dis Colon Rectum. 2004;47:1808-1815.

Hyman NH, Fazio VW, Tuckson WB, Lavery IC. Consequences of ileal pouch-anal anastomosis for Crohn's colitis. Dis Colon Rectum. 1991;34:653-657.

Tekkis PP, Heriot AG, Smith O, Smith JJ, Windsor AC.J, Nicholls RJ. Long-term outcomes of restorative proctocolectomy for Crohn's disease and indeterminate colitis. Colorectal Dis. 2005;7:218-223.

Sagar PM, Dozois RR, Wolff BG. Long-term results of ileal pouch-anal anastomosis in patients with Crohn's disease. Dis Colon Rectum. 1996;39:893-898.

Regimbeau JM, Panis Y, Pocard M, Bouhnik Y, Lavergne-Slove A, Rufat P. Long-term results of ileal pouch-anal anastomosis for colorectal Crohn's disease. Dis Colon Rectum. 2001;44:769-778.

Panis Y. Is there a place for ileal pouch-anal anastomosis in patients with Crohn's colitis?. Neth J Med. 1998;53:S47-S51.

Panis Y, Poupard B, Nemeth J, Lavergne A, Hautefeuille P, Valleur P. Ileal pouch/anal anastomosis for Crohn's disease. Lancet. 1996;347:854-857.

Shen B, Fazio VW, Remzi FH, Bennett AE, Lavery IC, Lopez R. Clinical features and quality of life in patients with different phenotypes of Crohn's disease of the ileal pouch. Dis Colon Rectum. 2007;50:1450-1459.

Peyrègne V, Francois Y, Gilly FN, Descos JL, Flourie B, Vignal J. Outcome of ileal pouch after secondary diagnosis of Crohn's disease. Int J Colorectal Dis. 2000;15:49-53.

De Oca J, Sánchez-Santos R, Martí Ragué J, Biondo S, Parés D, Osorio A. Long-term results of ileal-pouch anastomosis in Crohn's disease. Infl Bowel Dis. 2003;9:171-175.

Deutsch AA, McLeod RS, Cullen J, Cohen Z. Results of pelvic-pouch procedure in patients with Crohn's disease. Dis Colon Rectum. 1991;34:475-477.

Grobler SP, Hosie KB, Affie E, Thomson H, Keighley MRB. Outcomes of restorative proctocolectomy when the diagnosis is suggestive of Crohn's disease. Gut. 1993;34:1384-1388.

Longo WE, Oakley JR, Lavery IC, Church JM, Fazio VW. Outcome of ileorectal anastomosis for Crohn's colitis. Dis Colon Rectum. 1992;35:1066-1071.

Ann Surg. 2008 Oct;248(4):608-16.

Long-term outcomes with ileal pouch-anal anastomosis and Crohn's disease: pouch retention and implications of delayed diagnosis.Melton GB, Fazio VW, Kiran RP, He J, Lavery IC, Shen B, Achkar JP, Church JM, Remzi FH.Source

Digestive Disease Institute, Department of Colorectal Surgery, Cleveland Clinic Foundation, Cleveland, OH 44195, USA.

AbstractOBJECTIVE:To assess long-term outcomes after ileal pouch-anal anastomosis (IPAA) in Crohn's disease (CD).

SUMMARY BACKGROUND DATA:Although considered the procedure of choice in ulcerative colitis, performance of ileal pouch-anal anastomosis (IPAA) is controversial in CD.

METHODS:CD patients were identified from a prospectively maintained IPAA database. Time-to-diagnosis and pouch retention rates were analyzed using Kaplan-Meier curves. Demographic, clinical, and pathologic factors associated with pouch retention were evaluated with log-rank test and Cox proportional hazards model.

RESULTS:Two hundred and four CD patients (108 female, median age 33 years, and median follow-up 7.4 years) with primary IPAA were included. CD diagnosis was before IPAA (intentional) in 20(10%), from postoperative histopathology (incidental) in 97(47%) or made in a delayed fashion at median 36 months after IPAA in 87(43%). Overall 10-year pouch retention was 71%. On multivariate analysis, pouch loss was associated with delayed diagnosis (P = 0.03, hazard ratio [HR] 2.6 (95% confidence interval [CI] 1.1-6.5)), pouch-vaginal fistula (P = 0.01, HR 2.8 (95% CI 1.3-6.4)), and pelvic sepsis (P = 0.0001, HR 9.7(95% CI 3.4-27.3)). Patients with retained IPAA at follow-up had near-perfect/perfect continence (72%), rare/no urgency (68%) with median daily bowel movements 7 (range 2-20). Median overall quality of life, quality of health, level of energy, and happiness with surgery were 9, 9, 8, and 10 of 10, respectively.

CONCLUSIONS:For CD patients with IPAA, when the diagnosis is established preoperatively or immediately following surgery, pouch loss rates are low and functional results are favorable. Outcomes in patients with delayed diagnosis are worse but half retain their pouch at 10 years with good functional outcomes.

Dis Colon Rectum. 2004 Nov;47(11):1808-15.

Analysis of the outcome of ileal pouch-anal anastomosis in patients with Crohn's disease.Hartley JE, Fazio VW, Remzi FH, Lavery IC, Church JM, Strong SA, Hull TL, Senagore AJ, Delaney CP.Source

Department of Colorectal Surgery, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA. j.e.hartley@hull.ac.uk

AbstractPURPOSE:Ileal pouch-anal anastomosis has come to represent the procedure of choice for patients requiring surgery for mucosal ulcerative colitis. In contrast, a proven diagnosis of Crohn's disease is generally held to preclude ileal pouch-anal anastomosis. However, patients with ileal pouch-anal anastomosis for apparent mucosal ulcerative colitis who are subsequently found to have Crohn's disease have a variable course. We reviewed our experience in this scenario to determine whether selected patients with Crohn's disease may be candidates for ileal pouch-anal anastomosis.

METHODS:A retrospective review of the prospectively maintained ileal pouch-anal anastomosis database was undertaken to identify patients with a diagnosis of Crohn's disease after ileal pouch-anal anastomosis. Clinical outcome and quality-of-life data were obtained from the database and chart review. End points were the development of recrudescent Crohn's disease, pouch failure, and quality of life and functional outcome at the time of data collection. Differences between groups were calculated using the chi-squared test. Cumulative incidence of recrudescent Crohn's disease and pouch loss were calculated by the Kaplan-Meier method. Factors predictive of development of recrudescent Crohn's disease and pouch loss were examined by univariate analysis.

RESULTS:Sixty patients (32 females; median age, 33 (range, 15-74) years) who underwent ileal pouch-anal anastomosis for mucosal ulcerative colitis subsequently had that diagnosis revised to Crohn's disease. Median follow-up of all patients was 46 (range, 4-158) months at time of data collection by which time 21 patients (35 percent) had developed recrudescent Crohn's disease. No pre-ileal pouch-anal anastomosis factors examined were predictors of the development of recrudescent Crohn's disease on univariate analysis. Median follow-up of the latter group was 63 (range, 0-132) months from time of diagnosis, by which time six patients underwent pouch excision and another patient was permanently defunctioned. The overall pouch loss rate for the entire cohort was 12 percent and 33 percent for those with recrudescent Crohn's disease. Median daily bowel movements in those with ileal pouch-anal anastomosis in situ at the time of data collection was 7 (range, 3-20), with 50 percent of patients rarely or never experiencing urgency and 59 percent reporting perfect or near perfect continence. Median quality of life, health, and happiness scores were 9.9 and 10 of 10.

CONCLUSIONS:The secondary diagnosis of Crohn's disease after ileal pouch-anal anastomosis is associated with protracted freedom from clinically evident Crohn's disease, low pouch loss rate, and good functional outcome. Such results only can be improved by the continued development of medical strategies for the long-term suppression of Crohn's disease. These data support a prospective evaluation of ileal pouch-anal anastomosis in selected patients with Crohn's disease.

Adv Surg. 2009;43:111-37.

Can ileal pouch anal anastomosis be used in Crohn's disease?Joyce MR, Fazio VW.Source

Department of Colorectal Surgery, Digestive Disease Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue/A30, Cleveland, OH 44195, USA. joycem3@ccf.org

AbstractRestorative proctocolectomy with IPAA is now accepted as the standard of care in most patients with a diagnosis of mucosal ulcerative or IndC requiring surgery. In patients with Crohn's colitis needing intervention, proctocolectomy with end ileostomy or subtotal colectomy with ileorectal anastomosis is still the treatment of choice. In the authors' institution they consider performing an ileal pouch for a diagnosis of Crohn's colitis requiring rectal resection provided there is no existing or previous evidence of perianal or small bowel CD. They only perform this in a well-motivated patient who understands the potential sequelae, including an increased incidence of adverse effects and reduced functional outcome, if they develop CD of the pouch. A significant amount of time must be allocated to the patient and their family when counseling them with regards to the potential surgical options and outcomes associated with each. The development of CD of the pouch is associated with a considerable pouch failure rate requiring pouch excision or indefinite ileostomy formation. It does not always herald disaster, however, and a large percentage of patients can be salvaged using a combination of medical and surgical therapy. In this patient group the maintenance of intestinal continuity and avoidance of an ostomy is often the most important factor in their perception toward quality of life. The search for a serologic or genetic marker that will predict disease outcome in this select patient group and thereby direct surgical decision making should continue. It is recommended that in the presence of Crohn's colitis the decision to perform an ileal pouch should only be made in a tertiary center under the care of gastroenterologists, histopathologists, and colorectal surgeons with experience in the management of these complex cases.

• Evidence: II-3, C

Statement 3 (amendments)

Patients who require surgery for isolated rectal disease may undergo proctectomy with creation of a stoma. Those with concomitant colonic disease can be considered for procto-colectomy. [II-3,B]

Restorative proctocolectomy with IPAA can be considered in an experienced tertiary center if the small bowel is unaffected and there is no perianal disease provided patients are counseled on the long term risks of pouch failure.[II-3, B]

Statement 4

Complex Crohn’s perianal fistula may be treated by long-term draining setons.

Hepatogastroenterology. 2010 Jan-Feb;57(97):3-7.

Clinical advantages of combined seton placement and infliximab maintenance therapy for perianal fistulizing Crohn's disease: when and how were the seton drains removed?Tanaka S, Matsuo K, Sasaki T, Nakano M, Sakai K, Beppu R, Yamashita Y, Maeda K, Aoyagi K.Source

Department of Gastroenterological Surgery, Fukuoka University, School of Medicine Nanakuma 7-45-1, Jonan-ku, Fukuoka 814-0180, Japan. t-shin@fukuoka-u.ac.jp

AbstractBACKGROUND/AIMS:Perianal fistulas are often found in patients with Crohn's Disease (CD), however, the complete management of such fistulas tends to be difficult. The aim of this study is to critically evaluate the clinical advantages of combined seton placement and infliximab maintenance therapy for perianal fistulizing CD.

METHODOLOGY:Fourteen patients (9 males, 5 females) were evaluated for perianal fistulizing CD with the seton and infliximab therapy. Almost all patients were examined for the presence of either an abscess or fistulas by computed tomography (CT) and/or Magnetic Resonance Imaging (MRI) in addition to their physical findings. Seton placement was performed under general anesthesia, following the administration of inflixmab at a dose of 5 mg/kg for weeks 0, 2 and 6, and then about every 8 weeks as a maintenance therapy.

RESULTS:For all patients average number of inserted drains was 4.5 and the average number of infliximab infusions was 9.4 times. The mean follow-up period was 12.1 months. A redness and/or swelling in perianal lesion were seen in 12 patients, moreover, pus discharge was seen in 7 patients, and serous exudate was seen in 7 patients. After the administration of these treatments, a reversal of the redness and/or swelling was seen in the exudate and a wet-to-dry wound change was found in all patients. Furthermore, the seton drains were completely removed in 11 patients. In most patients, seton drains were completely removed after 5 rounds of infliximab infusion. Following the removal of the seton drains from all the patients, they reported their post-treatment health and well-being to be good while also reporting a good quality of life (QOL). In addition, no serious adverse events were observed.

CONCLUSIONS:The combined seton placement and infliximab maintenance therapy for perianal fistulizing CD was therefore found to be effective in terms of fistula closure and the removal of seton drains. This treatment modality is therefore considered to be a safe clinical procedure which improves the QOL in patients with CD.

Inflamm Bowel Dis. 2003 Mar;9(2):98-103.

Treatment of perianal fistulizing Crohn's disease with infliximab alone or as an adjunct to exam under anesthesia with seton placement.Regueiro M, Mardini H.Source

University of Pittsburgh School of Medicine, Presbyterian Hospital, Pittsburgh, Pennsylvania 15261, USA. regueirom@msx.dept-med.pitt.edu

AbstractPerianal fistulas occur in approximately 30% of patients with Crohn's disease (CD). Infliximab, a chimeric monoclonal antibody targeting human tumor necrosis factor alpha (TNF), is approved for the treatment of fistulizing CD. Although the initial response to infliximab is dramatic, the median duration of fistula closure is approximately 3 months, and repeated infusions are often required. An exam under anesthesia (EUA) by a surgeon allows for complete inspection of the fistula as well as incision and drainage of an abscess and placement of a seton. Our aim was to compare the rate of perianal fistula healing, relapse rate, and time to relapse in patients with fistulizing CD treated with infliximab alone or as an adjunct to surgical EUA with seton placement. Thirty-two consecutive patients with perianal fistulizing CD who completed at least 3 infusions with infliximab (5 mg/kg at 0, 2, 6 weeks) between October 1999 and October 2001 were analyzed. All patients had at least 3 months of follow-up after the third dose of infliximab. Response was defined as complete closure and cessation of drainage from the fistula. Patients with CD and perianal fistulas who had an EUA prior to infliximab infusions had a better initial response (100% vs. 82.6%, p = 0.014), lower recurrence rate (44% vs. 79%, p = 0.001), and longer time to recurrence (13.5 months vs. 3.6 months, p = 0.0001) compared with patients receiving infliximab alone. In conclusion, patients with fistulizing CD treated with infliximab are more likely to maintain fistula closure if treatment is preceded by EUA and seton placement.

Dis Colon Rectum. 2003 May;46(5):577-83.

Combined seton placement, infliximab infusion, and maintenance immunosuppressives improve healing rate in fistulizing anorectal Crohn's disease: a single center experience.Topstad DR, Panaccione R, Heine JA, Johnson DR, MacLean AR, Buie WD.Source

Department of Surgery, Foothills Hospital, University of Calgary, Calgary, Alberta, Canada.

AbstractPURPOSE:Infliximab (anti-TNF alpha) has been used for the treatment of fistulizing Crohn's disease with variable efficacy. The aim of this study was to evaluate the efficacy of infliximab combined with selective seton drainage in the healing of fistulizing anorectal Crohn's disease.

METHODS:This was a retrospective chart review of all patients with fistulizing Crohn's disease treated with infliximab between March 2000 and February 2002.

RESULTS:Twenty-nine patients (12 male; mean age, 31 years) received a mean of 3 (range, 1-5) doses of infliximab 5 mg/kg. Twenty-one patients had perianal fistulas; eight had rectovaginal fistulas, four with combined rectovaginal/perianal fistula. Fourteen of 21 patients (67 percent) with perianal fistula had a complete response (mean follow-up, 9 months), 4 of the 14 relapsed (mean, 6 months), but all had a complete response to retreatment (mean, 9 months). A partial response occurred in four patients (19 percent), defined by decreased drainage (2 patients) or infliximab dependence (2 patients) requiring repeated dosing every six to eight weeks. Three patients (14 percent) had no response. Seton drainage was used before infusion in 13 perianal patients for perianal infection and 17 were treated with maintenance azathioprine or methotrexate. Of eight patients with rectovaginal fistula, complete response occurred in one, partial response in five, and no response in two. Two partial responders became infliximab dependent. A complete response was observed in one patient with isolated rectovaginal fistula, a partial response in five. No patient with a combined rectovaginal/perianal fistula had a complete response. Five rectovaginal fistula patients were taking maintenance immunosuppressive agents and two had seton drainage before infusion.

CONCLUSIONS:Selective seton placement combined with infliximab infusion and maintenance immunosuppressives resulted in complete healing in 67 percent of Crohn's patients with perianal fistula and partial healing in 19 percent. Relapse was successfully treated with repeat infusion. Concomitant rectovaginal fistula was a poor prognostic indicator for successful infliximab therapy.

Dis Colon Rectum. 2005 Mar;48(3):459-63.

Long-term indwelling seton for complex anal fistulas in Crohn's disease.Thornton M, Solomon MJ.Source

Department of Colorectal Surgery, Royal Prince Alfred Medical Center, Newtown, NSW, Australia.

AbstractPURPOSE:This study was designed to review the results of long-term indwelling seton or depezzar catheter in the management of perianal Crohn's disease.

METHODS:A retrospective case review from data extracted from a prospective endorectal ultrasound database was performed. All patients underwent an intraoperative endorectal ultrasound to identify the extent of the fistulas and to assess anal wall thickness. Fistulas were classified by Parks' criteria. All patients then underwent insertion of a seton or depezzar catheter under ultrasound guidance. All patients were followed clinically and with endorectal ultrasound by the senior author. Outcome measures included symptom control, number of procedures required, fecal continence, and reduction in anal wall thickness.

RESULTS:Twenty-eight patients with 43 complex perianal Crohn's fistulas were identified. Median follow-up was 13 (range, 2-81) months. Twenty-one percent of patients developed recurrent or new perianal symptoms while the seton was in situ. Eleven percent of patients required further surgical intervention. The median anal wall thickness at the time of diagnosis was 18.5 mm reducing to a median of 14 mm after seton insertion and symptom control (P < 0.02). No patient reported a deterioration in fecal continence after seton insertion. In multivariate analysis, patient age (P < 0.005), reduction in anal wall thickness after seton insertion (P < 0.04), and length of follow-up (P < 0.03) were significant predictors of long-term symptom control.

CONCLUSIONS:Long-term indwelling seton is an effective management modality for complex perianal Crohn's fistulas, which does not negatively impact fecal continence. Clinical symptoms and course are associated with anal wall thickness as measured by endorectal ultrasound.

• Evidence: II-3, C

Statement 4 (amendments)

Complex Crohn’s perianal fistula may be treated by long-term draining setons. Combined treatment with biological therapy may allow removal of setons with healing in some cases. [II-2, B]

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