eMedicine Specialties > Pediatrics: Surgery > General Surgery

Ulcerative Colitis: Surgical Perspective

Author: E Stanton Adkins III, MD, Clinical Associate Professor, Departments of Pediatrics and Surgery, University of South Carolina School of Medicine
Coauthor(s): Kenneth Azarow, MD, Program Director, Pediatric Surgery, Children's Hospital and University of Nebraska Medical Center; Professor, Department of Surgery, Uniformed Services University of the Health Sciences
Contributor Information and Disclosures

Updated: Dec 4, 2008

Introduction

History of the Procedure

Historically, surgery has been viewed as definitive therapy for ulcerative colitis (UC). Total proctocolectomy is often curative, alleviating symptoms and removing the risk of colonic adenocarcinoma. Whereas, total proctocolectomy is palliative in patients with Crohn disease (CD) and pancolitis. Prior to 1980, total proctocolectomy with end ileostomy or continent (or Koch) ileostomy was the mainstay of therapy. However, in the late 1970s, reports of continence-preserving procedures involving ileal pouch–anal anastomosis began to surface.1 As experience amassed, the procedure was refined, and the ileal pouch–anal anastomosis has become the most common operation for patients with ulcerative colitis who wish to maintain anal continence.

Problem

Ulcerative colitis is an inflammatory condition of the colon. It must be differentiated from Crohn disease because, although both are inflammatory bowel diseases, each is managed differently by the surgeon (see Crohn Disease: Surgical Perspective). In general, the inflammatory changes in ulcerative colitis involve the rectum and extend proximally in a continuous fashion. The entire colon is involved in severe cases. It is the second most common cause of massive GI bleeding in children. In addition to the colonic involvement, extraintestinal manifestations include those of the eye, skin, and joints and biliary disease. These manifestations underscore the systemic nature of the inflammatory process. The disease has a chronic course, in which exacerbations are followed by periods of remission. Total proctocolectomy is curative; thus, it is the standard surgical treatment.

Frequency

About 1 million Americans are affected with ulcerative colitis.2 The incidence is higher in the northern hemisphere, with an incidence of 4-6 cases per 100,000 people in the United States, United Kingdom, and Scandinavia.3,4 Two of every 100,000 children are affected, and 20-25% of all cases of ulcerative colitis occur in persons aged 20 years or younger. Ulcerative colitis is more common in whites than in blacks and tends to occur in families; disease concordance is documented in monozygotic twins.5

Etiology

The etiology of ulcerative colitis is unknown. The cause appears to be multifactorial and follows a nonmendelian pattern, which suggests that more than one allele is involved.2 Environmental factors also play a role. For example, sulfate-reducing bacteria, which produce sulfides, are found in large numbers, and sulfide production is higher in patients with ulcerative colitis than in other people. Sulfide production is even higher in patients with active ulcerative colitis than in patients in remission.6 The bacterial microflora is altered in patients with active disease. A decrease in Klebsiella species is seen in the ileum of patients relative to controls.7  This difference disappears after proctocolectomy.

Nonsteroidal anti-inflammatory drug (NSAID) use is higher in patients with ulcerative colitis than in control subjects, and one third of patients with an exacerbation of ulcerative colitis report recent NSAID use. This finding leads some to recommend avoidance of NSAID use in patients with ulcerative colitis.8 Vitamins A and E, both considered antioxidants, are found in low levels in as many as 16% of children with ulcerative colitis exacerbation.9 Chromosomes are thought to be less stable in patients with ulcerative colitis, as measured with telomeric associations in peripheral leukocytes.10 This phenomenon may also contribute to their increased cancer risk. Whether these abnormalities are the cause or the result of the intense systemic inflammatory response in ulcerative colitis is unresolved.

An autoimmune phenomenon has been suggested as one possible etiologic factor in ulcerative colitis. The presence of antineutrophil cytoplasmic antibodies (ANCA) and anti– Saccharomyces cerevisiae antibodies (ASCA) is a well-known feature of inflammatory bowel disease.11,12,13,14,15 In addition, an immune modulatory abnormality has been assumed to be responsible for the lower incidence of ulcerative colitis in patients who have undergone previous appendectomy. The incidence of previous appendectomy is lower in patients with ulcerative colitis (4.5%) than in control subjects (19%), and a further protective effect is observed if the appendectomy was performed before the patient was aged 20 years.16

Also, patients in whom appendectomy was performed for inflammatory disorders (eg, appendicitis or mesenteric adenitis) seem to have a lower incidence of ulcerative colitis than patients who undergo appendectomy for other disorders such as nonspecific abdominal pain.17 Psychological and psychosocial stress factors can play a role in the presentation of ulcerative colitis and can precipitate exacerbations.18

Pathophysiology

Ulcerative colitis manifests as an intense inflammatory reaction in the large intestine. It involves the rectum and extends proximally to involve the entire colon (pancolitis) in severe cases. The terminal ileum is not primarily involved but may be secondarily inflamed because of backwash ileitis, or the reflux of noxious inflammatory mediators from the colon, in as many as 10% of patients.19,20 Rarely, patients have persistence of small intestinal inflammation following proctocolectomy and pullthrough.21,22

Grossly, the colonic mucosa appears hyperemic, with loss of the normal vascular pattern. The mucosa is granular and friable. Frequently, broad-based ulcerations cause islands of normal mucosa to appear polypoid, leading to the term pseudopolyp (see Media file 1). Involvement of the muscularis propria in the most severe cases can lead to damage to the nerve plexus, resulting in colonic dysmotility, dilation, and eventual infarction and gangrene, a condition termed toxic megacolon.4,23 Microscopically, acute and chronic inflammatory infiltrate of the lamina propria, crypt branching, and villous atrophy are present in ulcerative colitis (see Media file 1).

Differentiation between ulcerative colitis and Crohn disease is critical to developing a treatment plan. Grossly, Crohn disease is characteristically noncontiguous, with intervening, or skipped, areas of normal mucosa. The ulcerations in Crohn disease tend to be linear and often lead to the classic cobblestone appearance to the mucosa. Crohn disease may involve the entire GI tract, whereas ulcerative colitis involves only the large bowel.

Microscopically, the inflammation in both can appear the same, but noncaseating granulomas are present only in Crohn disease. Granulomas are present in 60% of Crohn disease specimens but are present in 0% of ulcerative colitis specimens; therefore, their presence is specific for Crohn disease.20,23 The inflammation of Crohn disease may be transmural, whereas it is confined to the mucosa and submucosa in ulcerative colitis. Unfortunately, the differentiation is not always possible preoperatively. All large series of proctocolectomies include a subset of patients (approximately 10%) who were preoperatively felt to have ulcerative colitis but were later diagnosed with Crohn disease. 

The traditional idea that ulcerative colitis involves only the large bowel has been challenged. Significant gastroduodenal inflammation in children with ulcerative colitis has been reported. However, aphthous ulceration is considered unique to Crohn disease.20 In addition, patchiness of the colonic mucosa suggestive of skip lesions may occur during the treatment phase of ulcerative colitis, leading one to question the diagnosis. These patchy areas may be seen endoscopically in as many as 38% of patients with ulcerative colitis who undergo medical therapy. Rectal sparing may also occur at some point during medical treatment of ulcerative colitis in as many as 44% of cases.24  Proximal disease may be seen even after proctocolectomy. Capsule endoscopy has demonstrated patchy inflammation in the proximal bowel in patients with chronic pouchitis following proctocolectomy with ileal pouch reconstruction.25  

Presentation

UC presents with cramplike abdominal pain, bloody diarrhea, and tenesmus. Some cases may present with a fulminant course marked by severe diarrhea, fever, leukocytosis, and abdominal distention. Fulminant disease occurs in children more often than adults.26 An estimated 15% of patients present with an attack severe enough to require hospitalization and steroid therapy.27,28 Children may also present with systemic complaints, including fatigue, arthritis, failure to gain weight, and delayed puberty. The differential diagnosis of these symptoms in the pediatric population includes many entities, and definitive diagnosis may be delayed.14

Ulcerative colitis is associated with various extracolonic manifestations. Primary sclerosing cholangitis (PSC) is a potentially severe associated condition, often resulting in cholestatic jaundice and liver failure that requires transplantation. Of patients with PSC, 75% have inflammatory bowel disease. Of patients with ulcerative colitis, 5% have cholestatic liver disease, and 40% of those have PSC. One interesting hypothesis about the etiology of PSC in patients with ulcerative colitis involves the release of proinflammatory agents in the colon that are absorbed; thus, they enter the enterohepatic circulation and are concentrated in the biliary system, leading to bile duct damage.29,30

Other extraintestinal manifestations include uveitis, pyoderma gangrenosum, pleuritis, erythema nodosum, ankylosing spondylitis, and spondyloarthropathies. Reportedly, 6.2% of patients with inflammatory bowel disease have a major extraintestinal manifestation. Uveitis is the most common, with an incidence of 3.8%, followed by PSC at 3%, ankylosing spondylitis at 2.7%, erythema nodosum at 1.9%, and pyoderma gangrenosum at 1.2%.31 However, reports vary, and some have stated that the incidence of ankylosing spondylitis is as high as 10%. Arthropathies occur in as many as 39% of patients with inflammatory bowel disease. About 30% of these have inflammatory back pain, 10% have synovitis, and as many as 40% have radiologic findings of sacroiliitis.32

Anecdotal reports of recurrent subcutaneous abscesses unrelated to pyoderma gangrenosum exist.33 Multiple sclerosis also has been weakly associated with ulcerative colitis.34 Immunobullous disease of the skin has been associated with ulcerative colitis. One theory regarding this association is the concept of epitope spread. Colonic inflammation leads to mucosal damage, which exposes otherwise hidden antigens. Antibodies to these antigens are then formed; these most likely are cell adhesion molecules, which cross-react with similar antigens in other tissues.35

Ulcerative Colitis vs Crohn Disease

Open table in new window

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Table

Ulcerative Colitis

Crohn Disease

Only colon involved

Panintestinal

Continuous inflammation extending proximally from rectum

Skip-lesions with intervening normal mucosa

Inflammation in mucosa and submucosa only

Transmural inflammation

No granulomas

Noncaseating granulomas

Perinuclear ANCA (pANCA) positive

ASCA positive

Bleeding (common)

Bleeding (uncommon)

Fistulae (rare)

Fistulae (common)

Ulcerative Colitis

Crohn Disease

Only colon involved

Panintestinal

Continuous inflammation extending proximally from rectum

Skip-lesions with intervening normal mucosa

Inflammation in mucosa and submucosa only

Transmural inflammation

No granulomas

Noncaseating granulomas

Perinuclear ANCA (pANCA) positive

ASCA positive

Bleeding (common)

Bleeding (uncommon)

Fistulae (rare)

Fistulae (common)



Indications

Indications for surgery in ulcerative colitis (UC) are varied. Failure of medical management is the most common indication for surgery. Classically, an acute attack of ulcerative colitis that fails to respond to intravenous steroid therapy within 10 days warrants surgical intervention.36 Steroid dependency is a predictor for the need for surgery and likely a marker for more severe disease.

In addition, the presence of pancolitis is the strongest predictor of the need for surgery in children; more than 80% of patients who require surgery have total colonic involvement.26 However, during a fulminant attack, the patient's condition is compromised, with a potentially poor nutritional status, low albumin level, low hematocrit level, and complications of high-dose corticosteroid use. As discussed later, the recent surge of cyclosporine use in patients with ulcerative colitis may be useful in inducing remission, providing a window to an improvement in the patient's overall health status prior to surgery and, hence, to minimizing complications. Other indications for surgical intervention include hemorrhage, intolerance to steroid therapy, and growth retardation. One major goal of management in children is the avoidance growth retardation caused by chronic steroid use.26

Many physicians use surveillance colonoscopy in following up patients with ulcerative colitis and determining the need for colectomy. This involves scheduled annual or biannual colonoscopy with multiple random biopsies. However, surveillance colonoscopy must be undertaken with caution because even low-grade dysplasia is associated with synchronous adenocarcinoma in as many as 42% of cases, and as many as 84% of neoplasms in ulcerative colitis are missed at random biopsy. Furthermore, 1% of colon cancers in patients with ulcerative colitis have no foci of preexisting dysplasia. Even in patients in whom the disease is medically controlled, the optimal time for colectomy may be 7-10 years after the onset of disease, to prevent colon cancer.37

The risk of developing colon adenocarcinoma must be addressed. Historically, patients with ulcerative colitis had a 1% risk of cancer per year after 8-10 years of disease. After 20 years of disease, the incidence of adenocarcinoma was as high as 25%.38 More recent data, after the advent of more effective medical therapy, suggest that the incidence of adenocarcinoma has somewhat decreased. Among 600 patients colonoscopically examined for 30 years, the cumulative risk of cancer was only 2.5% at 20 years, 7.6% at 30 years and 10.8% at 40 years.39  Colonic dysplasia is a precursor to adenocarcinoma and occurs in patients with ulcerative colitis. Backwash ileitis is an independent marker for the presence of dysplasia, as is age older than 45 years and the presence of disease for more than 10 years.19 Therefore, the patient with ulcerative colitis must be made aware of the significant risk of colon cancer, and surgical intervention in nonacute cases must be encouraged after10 years of disease.

  • Indications for urgent surgery in patients with ulcerative colitis include the following:
    • Toxic megacolon refractory to medical management
    • Fulminant attack refractory to medical management
    • Uncontrolled colonic bleeding
  • Indications for elective surgery in ulcerative colitis include the following:
    • Chronic steroid dependency
    • Dysplasia or adenocarcinoma found on screening biopsy
    • Disease present 7-10 years

Contraindications

Contraindications to ileal pouch–anal procedures in children with ulcerative colitis (UC) are few. The only absolute contraindication is anal sphincter dysfunction. Preexisting incontinence due to neurologic impairment or other causes makes reservoir construction unnecessary and makes ileoanal pull-through inadvisable.

Other contraindications include suspected Crohn disease (CD). As stressed below, the diagnosis of UC must be certain before an ileal pouch reservoir is created in a patient with inflammatory bowel disease. The need for pelvic radiation is also a contraindication to pelvic reservoir construction. For example, if rectal cancer is found at the time of exploration, end ileostomy should be performed in anticipation of postoperative pelvic irradiation. Radiation leads to pouch fibrosis and noncompliance, with resultant loss of reservoir function.

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References
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Further Reading

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Keywords

ulcerative colitis, UC, inflammatory bowel disease, colonic adenocarcinoma, Crohn disease, CD, pancolitis, proctocolectomy, biliary disease, Klebsiella species, nonsteroidal anti-inflammatory drug, NSAID, appendectomy, appendicitis, small intestinal inflammation, colonic dysmotility, colonic gangrene, toxic megacolon, abdominal pain, bloody diarrhea, tenesmus, Primary sclerosing cholangitis, PSC, uveitis, pyoderma gangrenosum, pleuritis, erythema nodosum, ankylosing spondylitis, spondyloarthropathies, multiple sclerosis, colon cancer

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Contributor Information and Disclosures

Author

E Stanton Adkins III, MD, Clinical Associate Professor, Departments of Pediatrics and Surgery, University of South Carolina School of Medicine
E Stanton Adkins III, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Medical Association, and American Pediatric Surgical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Kenneth Azarow, MD, Program Director, Pediatric Surgery, Children's Hospital and University of Nebraska Medical Center; Professor, Department of Surgery, Uniformed Services University of the Health Sciences
Kenneth Azarow, MD is a member of the following medical societies: American Pediatric Surgical Association
Disclosure: Nothing to disclose.

Medical Editor

Mary L Hilfiker, MD, PhD, Chief, Division of Pediatric Surgery, Assistant Professor, Department of Surgery, University of California at San Diego Medical Center
Mary L Hilfiker, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science and American College of Surgeons
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Andre Hebra, MD, Chief, Division of Pediatric Surgery, Medical University of South Carolina; Professor of Surgery and Pediatrics, Medical University of South Carolina
Andre Hebra, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, Association for Academic Surgery, Society of Laparoendoscopic Surgeons, South Carolina Medical Association, Southeastern Surgical Congress, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina
H Biemann Othersen Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American Cancer Society, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society for Parenteral and Enteral Nutrition, American Surgical Association, American Thoracic Society, British Association of Paediatric Surgeons, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, South Carolina Medical Association, Southeastern Surgical Congress, Southern Medical Association, Southern Society for Pediatric Research, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Chief Editor

Harsh Grewal, MD, FACS, FAAP, Professor of Surgery and Pediatrics, Temple University School of Medicine; Chief, Section of Pediatric Surgery, Temple University Children's Medical Center
Harsh Grewal, MD, FACS, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association for Surgical Education, Children's Oncology Group, Eastern Association for the Surgery of Trauma, International Pediatric Endosurgery Group, Society of American Gastrointestinal and Endoscopic Surgeons, Society of Laparoendoscopic Surgeons, and Southwestern Surgical Congress
Disclosure: Nothing to disclose.

 
 
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