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Ulcerative Colitis: Surgical Perspective

E Stanton Adkins III, MD, Clinical Associate Professor, Departments of Pediatrics and Surgery, University of South Carolina School of Medicine
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

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

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.

Workup

Laboratory Studies

  • Lab studies in children with suspected ulcerative colitis (UC) should begin with a CBC count, basic chemical analysis, liver function testing (LFT), and a determination of the albumin level.
    • The CBC count discloses the hematocrit level, which may be decreased in children with chronic disease or acute bleeding.
    • The platelet count may be elevated in inflammatory bowel disease, and the mean platelet volume is low in 18-30% of patients with active disease. This may be a response by the bone marrow megakaryocyte to the systemic inflammatory response in ulcerative colitis.40
  • Much of the work in the past decade has focused on the development of serologic markers for inflammatory bowel disease. Antineutrophil cytoplasmic antibodies (ANCA), antineutrophil cytoplasmic antibodies, and anti– Saccharomyces cerevisiae antibodies (ASCA) have been the most intensely studied.
    • ANCA is most commonly associated with ulcerative colitis. Specifically, perinuclear ANCA (pANCA), found on the inside of the nuclear membrane, is highly associated with ulcerative colitis. ANCA assay results are positive in 60-80% of patients with ulcerative colitis. The presence of pANCA is associated with an earlier need for surgery. The finding of ANCA is roughly 50% sensitive, is 94% specific, and has a 76% positive predictive value for ulcerative colitis.11,12,14
    • In children with ambiguous and mild complaints in whom ulcerative colitis is part of the differential diagnosis, algorithms have been proposed in which the presence of ANCA is used to identify those who require more invasive diagnostic tests.13
    • Attempts have been made to correlate ANCA titers with postoperative complications, although this association has not been proven.15 ANCA is present in only about 40% of patients with Crohn disease.
    • ASCA is more highly associated with Crohn disease and is present in 60% of cases. ASCA is present in only 12% of patients with ulcerative colitis.
    • ANCA and ASCA titers are not correlated with disease activity.

Imaging Studies

  • No imaging findings are definitive in the diagnosis of ulcerative colitis or in the differentiation of ulcerative colitis from Crohn disease (CD).
  • Contrast-enhanced small-bowel follow-through studies may show small-intestinal involvement that suggests Crohn disease.
  • Many children with a preliminary diagnosis of inflammatory bowel disease undergo CT scanning of the abdomen as part of the initial evaluation of abdominal pain. CT scan findings in ulcerative colitis are nonspecific and may indicate dilated and/or thickened colon and mesenteric inflammatory changes. On CT scans, small-bowel thickening suggests Crohn disease. In a child with signs of sepsis, CT is useful in identifying intra-abdominal abscesses.
  • MRI has been studied as a potential diagnostic aid in differentiating ulcerative colitis from Crohn disease. MRI correctly depicted ulcerative colitis with 100% accuracy in one small study but was only 40% effective in depicting Crohn disease. MRI can depict extracolonic (ie, small bowel) involvement. If Crohn disease is localized to only the colon, MRI offers no added diagnostic benefit because the appearance of the colon itself on MRIs cannot be used to differentiate Crohn disease and ulcerative colitis.41
  • Technetium 99m (99m Tc)–labeled WBC scans have also been used in the workup of ulcerative colitis. The labeled white cells concentrate in areas of inflammation. Thus,99m Tc imaging is reported to be 76% sensitive in ulcerative colitis, but the findings are nonspecific. False-positive results can occur in nearly any colonic inflammatory condition.23 However, once inflammatory bowel disease is diagnosed,99m Tc scanning may help in differentiating ulcerative colitis from Crohn disease. Inflammation of the colon may be classified as contiguous of noncontiguous on the basis of99m Tc findings. In one study,99m Tc imaging allowed correct differentiation of ulcerative colitis from Crohn disease in 100 of 106 cases.42
  • Transabdominal Doppler ultrasonography has been used to evaluate mesenteric blood flow in ulcerative colitis. Mesenteric blood flow is hyperdynamic in active ulcerative colitis, as measured with the Doppler velocity in the superior and inferior mesenteric arteries. Mesenteric blood flow increases 2-6 fold in inflammatory bowel disease. Failure of the Doppler velocity to normalize during clinical remission is predictive of disease recurrence.43
  • Endoscopy and biopsy are the criterion standards in diagnosing and differentiating inflammatory bowel diseases. In all children except those with sepsis, imaging studies should be undertaken only if they are deemed necessary after endoscopy is complete.

Diagnostic Procedures

  • Once ulcerative colitis is suspected, endoscopy must be performed. Flexible sigmoidoscopy may be performed if the symptoms are mild, and the physician is likely to initiate therapy on the basis of the results obtained with flexible sigmoidoscopy. However, most physicians are likely to perform full colonoscopy if inflammation is found with flexible sigmoidoscopy. Therefore, in most circumstances in which ulcerative colitis is suspected, directly proceeding to full colonoscopy is more cost effective.44 This practice may be especially applicable in young children, in whom flexible sigmoidoscopy is likely to require the same degree of sedation as that of colonoscopy.
  • Multiple biopsy samples should be obtained from both inflamed and normal-appearing mucosa. Despite reports that biopsy results are sensitive and specific in the diagnosis of ulcerative colitis, the inherent failure rates of rectal reconstruction in ulcerative colitis due to the late diagnosis of Crohn disease or indeterminate colitis indicate that biopsy results may not be as accurate as originally thought. However, diagnosis of Crohn disease on the basis of granuloma identification is reliable.45

Treatment

Medical Therapy

Medical treatment of ulcerative colitis (UC) varies depending on the severity of the disease and the portion of the colon involved. It consists of induction treatment followed by maintenance therapy.46 In mild disease confined to the rectum, topical mesalazine (Asacol) given by suppository is the preferred therapy. Enemas or foam are less effective because their concentration in the rectum rapidly diminishes. Left-sided colonic disease is best treated with a combination of mesalazine suppository and an oral aminosalicylate. Combined oral and topical therapy is better than either route alone. Of the oral amino salicylates, sulfasalazine has the longest history. Sulfasalazine is 5-aminosalicylate (5-ASA) coupled to a sulfapyridine. It is poorly absorbed in the proximal bowel, and the bacteria in the colon uncouple the 5-ASA from the sulfa moiety, allowing 5-ASA to exert its anti-inflammatory effect on the colonic mucosa by inhibiting prostaglandin synthesis.

Mesalazine, another 5-ASA containing molecule, is better tolerated orally than sulfasalazine and has become the preferred medication. Systemic steroids are indicated when disease fails to quickly respond to aminosalicylates.

Acute, severe ulcerative colitis (ie, >6 bloody bowel movements/d, with one of the following: fever >38ºC, hemoglobin level <10.5 g/dL, heart rate >90 beats per minute [bpm], erythrocyte sedimentation rate >30 mm/h, or C-reactive protein level >30) requires hospitalization with intravenous high-dose corticosteroids (hydrocortisone 400 mg/d or methylprednisolone 60 mg/d). Alternative induction medications have been evaluated. Cyclosporine, tacrolimus, and infliximab are often effective in bringing steroid resistant disease under control. They have not been compared in a randomized controlled fashion; thus, one cannot be recommended above the others. They have all been associated with overwhelming sepsis. Cyclosporine and tacrolimus are both nephrotoxic and should not be used for long-term therapy. Infliximab requires the coadministration of an antimetabolite to limit the development of human anti-mouse antibody (HAMA).

For induction, other treatments have been proposed but are not standard therapy. Antibiotics have been used as an adjunct to steroid therapy but have not altered outcomes. Helminth ova have not been shown to be superior to placebo. Heparin may promote epithelial repair but is not effective given current delivery systems. Leukocytapheresis has some promise and is quite popular in Japan, where it has been used to induce remission47 and prevent postoperative septic complications.48  Confirmatory studies in adults49 and pediatric patients50 are very promising. However, it remains a resource intensive treatment.

Once remission has been achieved, maintenance therapy is recommended for all patients to prevent relapse. Oral aminosalicylates are indicated for disease that responded to ASA or steroids. Failure to continue with suppressive therapy is associated with high rates of relapse.  Some patients are unable to maintain remission or are intolerant of 5-ASA. Azathioprine or 6-mercaptopurine are alternatives that have proven effectiveness. For patients who were induced with infliximab, maintenance therapy should continue with infliximab or azathioprine. 

Probiotics also appear to be effective at maintaining remission. Escherichia coli strain Nissle 1917 has been compared with mesalazine and proved to be similarly effective.51   Bio-Three, a commercially available probiotic supplement (Enterococcus T-110, C butyricum TO-A, B mesentericus TO-A) produced remission in 45% of patients tested with mild-to-moderate ulcerative colitis.52  Trials of fish oil to produce or maintain remission have shown no benefit over placebo.

Surgical Therapy

The underlying principle to the surgical management of ulcerative colitis is total removal of the colon and rectal mucosa. This is achieved by performing a total proctocolectomy. However, ulcerative colitis tends to occur in young individuals in whom the implications of permanent ileostomy may be psychologically damaging; therefore, over the past 20 years, numerous continence-restoring procedures have evolved.

The creation of an ileal pouch–anal anastomosis involves total proctocolectomy, with folding of the distal ileum into a J, S, or W formation to create a fecal reservoir. The anastomosis to the anus preserves continence function involving the internal and external anal sphincters. The S and W configurations have been associated with a failure rate as high as 66% and a need for revision. Conversely, the J configuration is associated with a need for revision in only 1-2% of cases.

Reasons for failure with S and W pouches include dilation of the reservoir that leads to stasis and elongation of the spout at the anal anastomosis that leads to stenosis.53 These technical points are all but alleviated with the current technique of J pouch construction. Transanal defecation is restored in 88% of children with J pouches, whereas 32% of those with S pouches and 32% of those undergoing straight ileoanal pull-through procedures require revision.54 Although most surgeons do not use the S pouch as the first option (because of its pouchitis rate), the spout created in its construction provide an additional 3-5 cm in length in the entire ileal reservoir, compared with the length of a J pouch.

Some still advocate straight ileoanal pull-through anastomosis without reservoir construction. Straight endorectal pull-through causes dilatation and compensation over time so that the pouch develops a reservoir function. In addition, length is generally not a problem with a straight pull-through. Thus, many pediatric surgeons perform this as their primary procedure. Good long-term outcomes and patient satisfaction are reported.55 However, others have noted a need for revision of the straight pull-through configuration in 70% of the cases.53 Construction of the ileal J pouch-anal anastomosis is described below. However, one should keep in mind that the straight ileoanal pull-through is performed in essentially the same manner and uses less total length of small bowel.

In summary, the choice of pouch size and type is a balance between increasing reservoir function to decrease stool frequency and the risk of developing pouchitis. All reservoirs have a tendency to enlarge over time. Consequently, most surgeons have opted for a smaller initial reservoir that depends on reservoir enlargement to gradually decrease stooling frequency while avoiding pouchitis.

Preoperative Details

Timing of the surgical intervention is the major preoperative concern in ulcerative colitis. If possible, emergency surgery should be avoided because of the considerations mentioned above. If emergency surgery is indicated, most advocate use of a staged procedure. Initially, emergency total colectomy with end ileostomy is performed to alleviate the major symptoms of the disease, including bleeding and pain, and allows the patient to be weaned from steroids. Later, an ileal pouch–anal anastomosis is created, if the patient desires it, with removal of the remaining rectum. Most advocate leaving the rectum in place during the initial emergency operation to prevent disrupting the pelvic tissue planes to make the subsequent pelvic dissection safer. If the patient has mild disease or disease in remission, total proctocolectomy with the creation of an ileal pouch–anal anastomosis may be performed as the initial definitive procedure.

Intraoperative Details

A total proctocolectomy is performed through a midline abdominal section. The ileum is divided close to the ileocecal valve with a stapler to save maximal ileal length. The ileal branch of the ileocolic artery is preserved, if possible, to provide optimal blood supply to the distal ileum. The rectum is stapled and divided, within 1 cm proximal to the dentate line. This procedure theoretically preserves the sensory nerve fibers in the anal transition zone that contribute to discrimination between gas and stool. Some refute the importance of retaining this zone, reporting no change in functional outcome when the anal transition zone is removed.56 However, rectal mucosectomy may be performed and the ileum brought through a short seromuscular sleeve of rectum.

The dimensions of the pouch depend on the size of the patent. In adolescents, as in adults, a 9-cm to 12-cm pouch is created by folding the distal ileum on itself in a J configuration and by using a linear cutting stapler to place staples longitudinally along the antimesenteric border between the two limbs of the J to create a reservoir (see Media file 2). Limb lengths of 8-10 cm are used in small children. The bowel at the lower end (ie, curve) of the J is then used to create an anastomosis to the anus with a circular stapling device or sutures. Because of the increased incidence of cancer in patients with ulcerative colitis and primary sclerosing cholangitis (PSC), complete mucosectomy to the dentate line and creation of a hand-sewn pouch-anal anastomosis has been recommended in these patients.30

To ensure a tension-free anastomosis at the anus, numerous techniques may be used to gain length in the small bowel. First, the ligament of Treitz may be mobilized to allow the proximal jejunum to turn toward the pelvis is a more gradual manner. The peritoneum overlying the small bowel mesentery may be sequentially opened in an orientation perpendicular to the superior mesenteric artery ("stair stepping") to release tension and provide length. The superior mesenteric artery may be divided just distal to the origin of the first or second arterial arcade. This proximal division preserves distal collateral flow and provides length. Finally, vein interposition grafts may be used as a last resort in the most extreme cases in which length is prohibitively short.57

The need for fecal diversion after ileal pouch–anal anastomosis is controversial in the adult patients. Usually, the need to operate on young patients is due to the severity of illness. Thus, most surgeons prefer to proximally divert the fecal stream in young patients. During the procedure, the distal vascular arcades of the ileum are often divided to gain length to reach the pelvis; this division predisposes the patient to ischemia. Therefore, many surgeons opt for an end ileostomy or loop ileostomy as the means of diversion. Many use loop ileostomy because of the widely held belief that takedown of a loop ileostomy is technically easier.

Recent data refute this assumption. On average, the operating time with loop ileostomy takedown is 54 minutes less than that of end ileostomy. However, loop ileostomy takedown lengthens the hospital stay, increases the time to oral feeding, and has a 2-fold higher wound infection rate compared with that of end-ileostomy takedown. In addition, loop ileostomy requires significantly more outpatient stoma care and is associated with more frequent anal complications.58

Finally, minimally invasive surgical technique has been used in total colectomy. Over the past 10 years, several centers have reported success with using laparoscopy to perform the total colectomy combined with transanal mucosectomy to completely remove the diseased colon and rectal mucosa. An ileo-anal anastomosis (with or without J Pouch) can be successfully performed, as described above. The main disadvantage of the laparoscopic approach is the increase in total operating time compared with open surgery. However, preliminary data suggest decreased length of hospital stay, shorter time to return to normal activities and school, and improved cosmetic results. This technically demanding laparoscopic operation has also been successfully performed using robotic-assisted technology.

Postoperative Details

After ileal pouch–anal anastomosis procedures, patients are treated as with any bowel procedure. Their diet is changed as bowel function returns. They are weaned from steroid use. At 6-12 weeks, diverting ileostomies are evaluated for closure. This evaluation usually involves contrast-enhanced imaging of the pouch to assess healing. Once the ostomy is taken down, stool frequency is evaluated, and the need for bulk-forming agents or motility agents determined.

Follow-up

Although the incidence of colon adenocarcinoma is greatly reduced with total proctocolectomy and ileal pouch–anal anastomosis, it is not zero. The residual colonic mucosa is at risk for dysplasia and neoplastic transformation.59  Moreover, in patients with chronic pouch inflammation, villous hypertrophy and dysplasia may occur. Although dysplasia has never been found within the pouch of a pediatric patient, chronic inflammatory changes have been found, leading to the supposition that dysplasia may develop. Yearly screening endoscopy has been recommended for the 5 years after the procedure. In children who have chronic inflammatory changes in the pouch reservoir, annual screening endoscopy should be performed. If no inflammation is present, screening endoscopy may be performed every 2 years.60

Complications

Several complications have been reported after ileal pouch–anal anastomosis procedures. The anastomotic leak rate is 7-9%.54,61 If this leak occurs, fecal diversion, percutaneous drain placement, or repeat surgery with removal or revision of the reservoir is required. Pelvic abscess, which frequently accompanies an anastomotic leak, occurs in about 5% of cases (reports vary from 0-25%). Among patients with a pelvic abscess, 26% require excision of the pouch. Only 5.9% of patients without an abscess have pouch failure that requires removal. If the abscess is managed with diversion and drainage, the pouch may be spared. However, these patients have higher rates of long-term incontinence and pain compared with those without abscesses.62 In patients who require pouch excision due to abscess, a gracilis muscle interposition flap has been used to maintain the anal canal and allow future attempts at pouch procedures.63

Pouch-vesicle, pouch-vaginal, pouch-anal, and enterocutaneous fistulas occur with a frequency of about 1% each.

Pouchitis is defined as a clinical syndrome in which the patient has increased stool frequency, malaise, fever, or incontinence that usually responds to antibiotic therapy. The most frequently used antibiotics are ciprofloxacin or metronidazole.61 The incidence is reported to be 40-60%. Risk increases with time; 18% have pouchitis at 1 year, and 48% have pouchitis at 10 years.54,61,64 Pouch dilatation and pouch-anal anastomotic stricture may lead to fecal stasis and predispose the patient to pouchitis. Patients without a pouch rarely develop pouchitis and have comparable stool frequency with time. Clostridium difficile and Clostridium perfringens have been disproportionately found in patients with ulcerative colitis after ileal pouch–anal anastomosis. Treatment of these infections has led to decreases in inflammation.65,66

Outcome and Prognosis

Outcomes of pouch procedures are classified as good to excellent in as many as 90% of patients. Stool frequency is less than 5 per day in as many as 74%. Difficulty with evacuation occurs in 20%. About 77% of patients require no dietary restrictions; the remaining patients have a lower stool frequency with a low-fat diet.67 Complete incontinence is reported in only 2%. Bulk-forming agents are required in as many as 30%.

Sexual dysfunction, manifest by retrograde ejaculation or impotence, occurs in 3% of males. Although Meagher et al (1998) reported sexual dysfunction to occur in only 6% of females,64 Ogilvie et al (2008) reported that 47% of females have low Female Sexual Function Index (FSFI) scores, indicating sexual dysfunction.68  Additionally, Cornish et al (2008) described a 25% incidence of sexual dysfunction manifested by dyspareunia or psychological aversion to intercourse for fear of stool leakage.69  The report also documented an increase of infertility from 8% preoperatively to 26% postoperatively. 

For excellent patient education resources, visit eMedicine's Crohn Disease Center and Esophagus, Stomach, and Intestine Center. Also, see eMedicine's patient education articles, Inflammatory Bowel Disease, Crohn Disease, and Crohn Disease FAQs.

Future and Controversies

Every patient who undergoes an ileal pouch–anal anastomotic procedure, as currently performed, must be able to accept the possibilities of stool seepage or incontinence and frequent bowel movements, with a minimum of 4-6 per day. Although the procedure results in removal of the diseased organ and although it is more technically advanced than end ileostomy, it is not a perfect solution. Surgical techniques can be improved to ensure better postoperative functional outcomes.

Additional research may show that genetic predispositions, errors in oxidative metabolism, infectious causes, or autoimmune abnormalities are the primary causes of ulcerative colitis. If so, directed therapies could be pursued.

Present research is focused on the potential benefits of the use of additional immunosuppressive agents such as Methotrexate or cyclosporine alone or in combination with mannose-6-phosphate (6-MP), prednisone, or azathioprine. Finally, the use of directed antibody therapy is in its infancy. Indications and contraindications to these therapies continue to evolve.

Multimedia

Inflamed colonic mucosa demonstrating pseudopolyp...

Media file 1: Inflamed colonic mucosa demonstrating pseudopolyps.

A "J" pouch reservoir is created by placing linea...

Media file 2: A "J" pouch reservoir is created by placing linear cutting staples in a longitudinal orientation between the limbs of the J.

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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

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.

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Ronald Place, MD, to the development and writing of this article.

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