This is the current release of the guideline.

Crohn's disease of the terminal ileum, colon, ileocolon, and upper gastrointestinal tract

Note: The management of perianal Crohn's disease is not discussed in this guideline.

Management
Treatment

Colon and Rectal Surgery
Gastroenterology

Health Care Providers
Patients
Physicians

To provide practice parameters for the surgical management of Crohn's disease

Adults and children with Crohn's disease

Management/Treatment

1. Assessment of need for surgery over medication
2. Antibiotics and percutaneous drainage
3. Surgical drainage with or without resection
4. Computed tomography (CT) or magnetic resonance enterography
5. Interventional radiologic and/or endoscopic techniques
6. Endoscopic surveillance for dysplasia or neoplastic transformation
7. Resection with end stoma, diverted anastomosis, or nondiverted anastomosis
8. Strictureplasty with biopsy of lesions suspicious for cancer
9. Assessment of growth patterns in prepubertal children
10. Assessment of extraintestinal manifestations (EIMs)
11. Laparoscopic versus open surgery
12. Bypass surgery (gastrojejunostomy and duodenojejunostomy)
13. Endoscopic dilatation of affected area
14. Subtotal or total colectomy with end ileostomy
15. Segmental or total colectomy with or without a primary anastomosis
16. Total proctocolectomy or proctectomy with creation of a stoma

• Change in disease-related symptoms
• Morbidity and mortality from surgery
• Relapse rate
• Rate of progression to cancer
• Rate of involvement of contiguous organs
• Growth patterns of prepubertal patients

Searches of Electronic Databases

Not stated

Not stated

Weighting According to a Rating Scheme (Scheme Given)

Levels of Evidence

1. Meta-analysis of multiple well-designed, controlled studies, randomized trials with low false-positive and low false-negative errors (high power)
2. At least one well-designed experimental study; randomized trials with high false-positive or high false-negative errors or both (low power)
3. Well-designed, quasi-experimental studies, such as nonrandomized, controlled, single-group, preoperative-postoperative comparison, cohort, time, or matched case-control series
4. Well-designed, nonexperimental studies, such as comparative and correlational descriptive and case studies
5. Case reports and clinical examples

Adapted from Cook DJ, Guyatt GH, Laupacis A, Sackett DL. Rules of evidence and clinical recommendations on the use of antithrombotic agents. Chest 1992;102(4 Suppl):305S–11S. Sacker DL. Rules of evidence and clinical recommendations on the use of antithrombotic agents. Chest 1989;92(2 Suppl):2S–4S.

Review of Published Meta-Analyses
Systematic Review

Not stated

Not stated

Grades of Recommendations

1. Evidence of Type I or consistent findings from multiple studies of Type II, III, or IV
2. Evidence of Type II, III, or IV and generally consistent findings
3. Evidence of Type II, III, or IV but inconsistent findings
4. Little or no systematic empirical evidence

Adapted from Cook DJ, Guyatt GH, Laupacis A, Sackett DL. Rules of evidence and clinical recommendations on the use of antithrombotic agents. Chest 1992;102(4 Suppl):305S–11S. Sacker DL. Rules of evidence and clinical recommendations on the use of antithrombotic agents. Chest 1989;92(2 Suppl):2S–4S.

A formal cost analysis was not performed and published cost analyses were not reviewed.

Not stated

Not applicable

The levels of evidence (I-V) and the grades of recommendations (A-D) are defined at the end of the "Major Recommendations" field.

Operative Indications

Failed Medical Therapy

1. Patients with disease-related symptoms who suffer from medically unresponsive disease, demonstrate an inadequate response, manifest medication-related complications, or appear noncompliant with medication should be considered for operation. Level of Evidence: II; Grade of Recommendation: B.

   Surgical treatment is warranted if first-line and second-line therapies fail to induce remission safely in severe disease states, but surgery also should be considered before escalating medical therapy for patients with severe or steroid-dependent disease that is limited in disease extent, particularly in individuals with stricturing behavior or those with contraindications or risk factors for further medical therapy.

Perforation

1. Patients with symptoms and/or signs of free perforation should undergo operation. Level of Evidence: III; Grade of Recommendation: B.

   Immediate resection of the perforated segment is preferred over simple suture closure because of a relatively high mortality rate associated with the latter. After resection of a small-bowel perforation, an end stoma, diverted anastomosis, or nondiverted anastomosis can be performed depending on the presentation and operative findings. Colorectal perforations are more commonly resected in combination with a proximal stoma and mucous fistula or Hartmann's closure of the distal bowel; alternatively, a diverted anastomosis can be considered.

2. Patients with large anteroparietal, interloop, intramesenteric, or retroperitoneal abscesses may be managed by antibiotics and percutaneous drainage. If this approach is unavailable or unsuccessful, the patient should undergo surgical drainage with or without resection. Level of Evidence: III; Grade of Recommendation: B.
3. Patients with enteric fistulas and symptoms or signs of localized or systemic sepsis that persist despite appropriate medical therapy should be considered for operation. Asymptomatic patients with internal fistulas do not typically require surgery. Level of Evidence: III; Grade of Recommendation: B.

   Fistulas originating in diseased bowel and secondarily involving other intra-abdominal organs or the skin are not usually associated with localized or systemic sepsis. If sepsis is present, the patient should be started on broad-spectrum antibiotics and imaging studies should be performed to exclude a concomitant abscess, which should be drained. Regardless of whether an abscess is present, persistent sepsis usually warrants excision of the diseased bowel. Management of the target or "innocent bystander" organ is based on whether it is diseased bowel, noninflamed bowel, or another internal organ. Diseased bowel is generally resected, noninflamed bowel can be primarily closed, and other internal organs (e.g., bladder, vagina) can be primarily closed or left to heal by secondary intention. Surgery is usually not necessary if sepsis and symptoms are absent.

Obstruction

1. Patients with symptomatic strictures in any location that do not appear amenable or responsive to medical therapy should undergo operation. Level of Evidence: III; Grade of Recommendation: B.

   The incidence of fibrostenosing lesions in patients with Crohn's disease increases with longer disease duration, and this behavior is most commonly seen in patients with upper gastrointestinal disease involvement. Computed tomography and magnetic resonance enterography can sometimes help discern whether these areas of narrowing have an inflammatory component that might respond to medical therapy. If the patient is symptomatic and the stricture appears fibrotic with minimal inflammation, or if medical therapy fails to adequately improve the symptoms, surgery is generally warranted.

2. Patients with asymptomatic strictures of the colon that cannot be adequately surveyed by biopsy and/or cytology brushing should be considered for operation. Level of Evidence: III; Grade of Recommendation: B.

   Approximately 7 percent of large bowel strictures in patients with Crohn's disease are malignant and should be surveyed for neoplastic transformation. This is usually performed with multiple biopsies and cytologic brushing. If the stricture cannot be appropriately surveyed or if neoplastic changes are identified, resection is usually warranted.

Inflammation

1. Patients with acute colitis and symptoms or signs of impending or actual perforation should undergo operation. Level of Evidence: III; Grade of Recommendation: B.

   Although supporting evidence is limited, severe or fulminant colitis should be treated in the same way, regardless of the underlying inflammatory bowel disease. An abdominal film that reveals transverse colon distention >6 cm or persistent gaseous distension in a colonic segment indicates toxic megacolon and suggests that the patient is at risk for perforation. Persistent or progressing colonic dilatation, pneumatosis coli, evolving local peritonitis, and multiple organ failure also are evidence of impending or actual perforation and surgery is warranted.

2. Patients with acute colitis whose condition worsens despite appropriate medical therapy or fails to significantly improve after 48 to 96 hours of medical therapy should be considered for operation. Level of Evidence: III; Grade of Recommendation: B.

Hemorrhage

1. Patients with massive hemorrhage originating from any location may be managed by interventional radiologic and/or endoscopic techniques. If the patient is too unstable for this, or if this approach is unavailable or unsuccessful, the patient should undergo operation. Level of Evidence: III; Grade of Recommendation: B.

   Massive gastrointestinal hemorrhage is an uncommon event in patients with Crohn's disease. Other common etiologies of gastrointestinal hemorrhage, such as peptic ulcer disease and gastritis, should be excluded. If the hemorrhage is a direct consequence of Crohn's disease, it most commonly occurs from an ulcer that has eroded into a submucosal vessel. If the patient is stable and the source of bleeding can be endoscopically identified, local measures can be used to halt the bleeding. If the bleeding is too brisk to permit adequate endoscopic visualization or the source of hemorrhage cannot be identified, mesenteric angiography can be attempted and the bleeding site might be embolized. Patients who are hemodynamically unstable or fail to respond to these measures usually require laparotomy, sometimes with intraoperative endoscopy, and resection of the responsible bowel segment.

Neoplasia

1. Patients with long-standing Crohn's disease of the ileocolon or colon should undergo endoscopic surveillance. Level of Evidence: III; Grade of Recommendation: B.

   Patients with colitis often are advised to undergo a screening colonoscopy after eight to ten years of disease symptoms, and surveillance endoscopy every one to two years thereafter. Ideally, surveillance colonoscopy should be performed when the disease is in remission to minimize confusion interpreting neoplastic changes. Four quadrant random biopsies at 10-cm intervals along the colon and rectum may be obtained, as well as biopsies of any strictures, lesions, or mass; pseudopolyps that do not appear suspicious need not be sampled. Adenomatous-appearing polyps should be removed if possible, and the adjacent flat mucosa should be biopsied to exclude associated dysplasia.

2. Patients with carcinoma, dysplasia-associated lesion or mass (DALM), high-grade dysplasia, or multifocal, low-grade dysplasia of the colon or rectum should undergo resection. Level of Evidence: III; Grade of Recommendation: B.

   The appropriate extent of the resection is unclear and could range from a limited segment that includes only the inflamed bowel to the entire colon and rectum.

3. 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 strictureplasty. Level of Evidence: III; Grade of Recommendation: B.

   Small-bowel adenocarcinoma has been reported at the site of previous strictureplasty, and consequently the stricture site should be carefully inspected and any suspicious areas should be biopsied.

Growth Retardation and Extraintestinal Manifestations

1. Prepubertal patients with significant growth retardation despite appropriate medical therapy should be considered for operation. Level of Evidence: IV; Grade of Recommendation: C.
2. Patients with symptomatic disorders of the skin, mouth, eye, or joints who fail to respond to medical therapy should be considered for operation. Level of Evidence: IV; Grade of Recommendation: C.

   Extraintestinal manifestations (EIMs) of Crohn's disease occur in up to 30 percent of patients. The presence of one EIM sometimes predisposes an individual to the development of others. Some EIMs are temporally related to intestinal disease activity, whereas others occur independently. Erythema nodosum, oral aphthous ulcers, episcleritis, and some types of peripheral arthritis tend to parallel the intestinal disease activity, whereas pyoderma gangrenosum, uveitis, spondyloarthropathy, and primary sclerosing cholangitis do not. For the former group, therapy for the EIM focuses on treatment of the underlying intestinal disease, and medical management is usually sufficient. Nevertheless, if medical therapy fails to adequately control these manifestations, resection of the diseased intestine is warranted. For the group of EIMs that behave independently of the intestinal disease, surgery has no role in the management of the EIM.

Site-Specific Operations

Terminal Ileum, Ileocolon, and Upper Gastrointestinal Tract

1. Patients who require surgery for disease of the jejunum, proximal ileum, terminal ileum, or ileocolon without existing or impending short-bowel syndrome should usually undergo resection of the affected bowel. Level of Evidence: III; Grade of Recommendation: B.

   Most patients with penetrating or inflammatory disease of the terminal ileum or ileocolon can undergo resection of the offending bowel without significant risk to nondiseased bowel, superior mesenteric vessels, or retroperitoneal structures. Nevertheless, in rare cases when there is concern about damage to these structures, the diseased segment may be bypassed by using an ileocolostomy or proximal loop ileostomy. At the same time, any septic focus should be drained. Definitive resection at a later date is recommended, because the bypassed segment is at risk for recurrent disease and later adenocarcinoma. The subsequent operation should be delayed for several months to allow resolution of the inflammatory process and a safer excision.

2. Patients who require surgery for nonphlegmonous strictures of the jejunum, ileum, or ileocolon, and existing or impending short bowel syndrome should typically undergo strictureplasty. Level of Evidence: III; Grade of Recommendation: B.

   Strictureplasty should be strongly considered in patients with multiple strictures of the jejunum, proximal ileum, or terminal ileum.

3. Patients with symptomatic disease of the stomach or duodenum should be considered for bypass of the affected area or strictureplasty. Level of Evidence: III; Grade of Recommendation: C.

   Bypass options for refractory obstruction or pain secondary to gastroduodenal Crohn's disease include gastrojejunostomy and duodenojejunostomy. Truncal vagotomy with gastrojejunostomy may decrease the risk of marginal ulceration but increases the likelihood of diarrhea. Highly selective vagotomy may avoid these problems.

   Symptomatic strictures of the distal stomach or duodenum can be treated with bypass; nonperforated, nonphlegmonous stenotic lesions in this region also can be safely managed by strictureplasty.

4. Patients with symptomatic, accessible strictures of the intestinal tract can be considered for endoscopic dilatation of the affected area. Level of Evidence: II; Grade of Recommendation: C.

   Mild-to-moderate stricturing disease of the terminal ileum, colon, or duodenum can be safely and effectively treated by endoscopic dilatation with or without concomitant steroid injection. The procedure allows surgery to be postponed or avoided in many patients with Crohn's disease and short intestinal strictures. However, recurrent symptoms frequently necessitate a repeat procedure, and a dilatation should not be performed unless surgical services are available to intervene if perforation occurs.

Colon

1. Patients with disease of the colon that requires emergency or urgent surgery should typically undergo subtotal or total colectomy with end ileostomy. Level of Evidence: III; Grade of Recommendation: B.

   This approach removes most of the inflamed intestine with a relatively simple operation that avoids a pelvic dissection and an anastomosis and can be performed through a laparoscopic or open approach.

2. Patients with disease of the colon that requires elective surgery may undergo segmental or total colectomy with or without a primary anastomosis. Level of Evidence III; Grade of Recommendation: B

   Total colectomy with ileoproctostomy is preferable if two or more colonic segments are affected, because this subgroup has a higher recurrence rate when segmental resections are performed.

3. Patients who require surgery for disease of the rectum may undergo total proctocolectomy or proctectomy with creation of a stoma. Level of Evidence: III; Grade of Recommendation: C.

   Usually the entire rectum should be excised because cancer has been found in patients with even a short Hartmann's remnant.

Definitions:

Levels of Evidence

1. Meta-analysis of multiple well-designed, controlled studies, randomized trials with low false-positive and low false-negative errors (high power)
2. At least one well-designed experimental study; randomized trials with high false-positive or high false-negative errors or both (low power)
3. Well-designed, quasi-experimental studies, such as nonrandomized, controlled, single-group, preoperative-postoperative comparison, cohort, time, or matched case-control series
4. Well-designed, nonexperimental studies, such as comparative and correlational descriptive and case studies
5. Case reports and clinical examples

Grades of Recommendations

1. Evidence of Type I or consistent findings from multiple studies of Type II, III, or IV
2. Evidence of Type II, III, or IV and generally consistent findings
3. Evidence of Type II, III, or IV but inconsistent findings
4. Little or no systematic empirical evidence

None provided

The type of supporting evidence is identified and graded for each recommendation (see "Major Recommendations" field).

Appropriate surgical management of Crohn's disease

• Morbidity or mortality from surgery
• Requirement for a stoma

• These guidelines are inclusive and not prescriptive. Their purpose is to provide information on which decisions can be made, rather than dictate a specific form of treatment.
• It should be recognized that these guidelines should not be deemed inclusive of all proper methods of care or exclusive of methods of care reasonably directed to obtaining the same results. The ultimate judgment regarding the propriety of any specific procedure must be made by the physician in light of all of the circumstances presented by the individual patient.

An implementation strategy was not provided.

Getting Better
Living with Illness

Effectiveness
Timeliness

Not applicable: The guideline was not adapted from another source.

2007 Nov

American Society of Colon and Rectal Surgeons - Medical Specialty Society

American Society of Colon and Rectal Surgeons

Standards Practice Task Force of The American Society of Colon and Rectal Surgeons

Authors: Scott A. Strong, MD; Walter A. Koltun, MD; Neil H. Hyman, MD; W. Donald Buie, MD

Task Force Members: C. Neal Ellis, MD; Ravin Kumar, MD; Steven Mills, MD; Graham Newstead, MD; Paul Shellito, MD; Elin Sigurdson, MD; Scott Steele, MD; Joe Tjandra, MD

Not stated

This is the current release of the guideline.

None available

None available

This NGC summary was completed by ECRI Institute on July 7, 2008.

This NGC summary is based on the original guideline, which is subject to the guideline developer's copyright restrictions.