General Considerations

The term inflammatory bowel disease (IBD) encompasses a collection of gastrointestinal diseases that medical and surgical specialists treat in a collaborative fashion. Chapter 2 discusses genetic and immunologic factors influencing the development of IBD. Chapter 3 describes medical therapy for IBD. This chapter discusses the indications and types of surgery used for ulcerative colitis and Crohn disease.

In general, these diseases are first treated with medications, and surgery is recommended after medical therapy has been exhausted. However, with certain presentations (eg, anal abscess, toxic colitis), surgery is the first line of therapy, with medication often given after a procedure. Our purpose in this chapter is to survey the surgical procedures used in the treatment of IBD, and to discuss indications and timings relating to their use.

Ulcerative colitis is usually successfully treated medically; however, it is estimated that between 25% and 45% of patients with ulcerative colitis will ultimately require surgery. Surgical therapy is indicated in the following three cases: (1) in patients in whom medication is not controlling the disease, or side effects of the medication are too severe to be endured; (2) in patients with severe acute colitis requiring an urgent procedure; and (3) in patients with either cancer or dysplasia of the colorectal mucosa. Given that ulcerative colitis is a disease limited to the colon and rectum, one needs to remember that it is possible to “cure” this disease with surgical intervention. Further, in most patients, success rates of surgical therapy are high, and sphincter-sparing options exist. Despite the use of new therapies such as immunomodulators, the incidence of surgery has not significantly changed in the past few years.

Before discussing the specific indications for surgery, several general issues relating to the patient with colitis need to be reviewed.

1. IPAA or ileostomy?

When surgery is recommended for a patient with colitis, the surgeon needs to assess whether the patient is ultimately a candidate for a sphincter-sparing surgery that avoids a permanent ileostomy. Most patients are candidates for an ileoanal procedure (see later discussion), but some are not. The main considerations in determining whether a patient should have an ileostomy or a sphincter-sparing procedure include age, gender, type of job, and lifestyle. For example, an older woman who has had several children may at presentation to the surgeon already have some fecal incontinence issues. As difficult as it may be for the patient’s body image, recommending an ileoanal procedure in this patient would do her a disservice because it is highly likely that there would be significant fecal leakage and hygiene issues. Further, if a patient has a job or hobby that does not allow for access to a bathroom, an ileostomy may provide for a better quality of life than an ileoanal procedure.

2. Emotional support and education

When surgery is recommended, the patient—and on occasion the medical team—may feel a sense of failure. In addition, the patient often has a significant fear of the procedure and the possible need for a permanent ostomy. The medical team, surgeon, and support staff need to be cognizant of this perception. Reassurance and education about the upcoming process is the best approach to the patient at this time. Availability of support staff, such as an ostomy nurse, and discussions with previous patients who have undergone the procedure are extremely helpful in improving the patient’s outlook on the surgery.

3. Immunosuppression

The ill colitis patient is often referred to the surgeon after all immunosuppressive options have been exhausted. Surgical outcomes in avoiding infection and with wound healing can be significantly affected by immunosuppressive medications. The gastroenterologist, surgeon, and patient should discuss whether any medications can be stopped before the procedure without risk of a significant disease flare. It is not realistic to wean a patient off all immunosuppressive medications prior to surgery. In particular, many patients require and should remain on corticosteroids if these medications are necessary to avoid a significant exacerbation of a flare. Although patients undergoing surgery for inflammatory bowel disease are at increased risk of developing surgical site infections, recent studies have shown that the addition of biologics to the treatment of ulcerative colitis has not increased the risks of postoperative complications.

4. Nutrition

IBD and especially severe colitis can lead to weight loss, protein loss, and malnutrition. Intravenous nutrition preoperatively and perioperatively is essential in the most severe cases. Choice of operation is also affected by the nutritional status of the patient.

5. Medical comorbidities

Although colitis usually affects younger patients, the medical comorbidities of the patient need to be reviewed because they may affect the type of procedure recommended. In particular, patients with colitis have an increased incidence of thrombotic complications in the postoperative period; therefore, a low threshold should be set for investigating any preoperative risk or postoperative symptoms for venous thrombosis either of the extremities or the intestinal venous system.

6. Bowel preparation

Bowel preparation is recommended in any patient undergoing a colon resection. Recent studies have shown that mechanical preparation is not necessarily associated with a decreased risk of infectious complications. These studies, however, have been performed on the “average” colon patient and not on the subset of colitis patients with the issues already noted. We therefore recommend, when possible, that colitis patients undergo mechanical bowel preparation followed by administration of perioperative antibiotics to cover aerobic and anaerobic bacteria.

Indications for Surgery

A. Acute Colitis

Ideally operations for ulcerative colitis should be performed in a staged and deliberate manner. However, some patients develop severe acute colitis, requiring emergent surgical intervention.

Acute colitis is a term used to describe a series of signs and symptoms that include rapid onset of abdominal pain, bloody diarrhea, abdominal distention and tenderness, anorexia, fever, tachycardia, leukocytosis, and low urine output. It is important to remember that most of these patients use corticosteroids and other immunosuppressive agents that may mask the severity of many clinical symptoms. Severe colonic dilation or toxic megacolon is a feared complication of therapy that can lead to perforation, stool spillage, sepsis, and even death. Although acute colitis may occur in patients with known ulcerative colitis, it can also present as the heralding sign of colonic pathology in a patient not previously diagnosed with ulcerative colitis. Surgery is indicated for patients who exhibit signs of visceral perforation, generalized peritonitis, sepsis, or massive gastrointestinal bleeding. In addition, patients who do not improve despite maximized medical therapy after approximately 72 hours often require emergent operation. Other patients, who improve with medical treatment while NPO but who then deteriorate once an oral diet is resumed, often require surgery. The differential diagnosis of acute colitis includes Clostridium difficile infection, infectious diarrhea, and cytomegalovirus colitis. These conditions can coexist with and complicate ulcerative colitis. It is important to rule out these diseases, when possible, because their successful medical treatment can avoid an urgent or emergent surgical procedure. At times, however, the combination of baseline colitis and these secondary colitides necessitates an emergency procedure.

The choice of operation for an emergent procedure is discussed later in this chapter. In most cases, the goal of the surgeon is to “get in and get out.” Typically, a total colectomy with ileostomy and Hartmann pouch (the oversewn rectum) is performed. This procedure can be completed quickly, removes about 90% of the large intestine, and avoids a complex pelvic dissection and anastomosis. The procedure also does not eliminate further treatment options for the patient; for instance, a sphincter-sparing ileoanal anastomosis can still be performed at a later date.

B. “Subacute” Colitis: Failure of Medical Therapy

Subacute colitis describes a disease pattern in which the patient is neither acutely ill with an unmistakable indication for resection, nor in remission and symptom free. Patients who experience symptoms of chronic intractable disease such as recurring acute colitis, steroid dependence, chronic fecal urgency, growth retardation, persistent active disease, or complications of medical therapy (eg, diabetes, hypertension, peptic ulcer disease, psychosis, myopathy, osteonecrosis, and cataracts) often benefit from surgical intervention. The long-term side effects of some of the new biologic medications have not yet been determined, and the patient, gastroenterologist, and surgeon need to balance the risks of long-term medical therapy against those of surgery. Again, it should be emphasized that one can “cure” ulcerative colitis with surgery, and most patients have an excellent quality of life without the need for medications. However, a small percentage of patients fail surgical therapy with a sphincter-sparing procedure and require an ileostomy, and some develop pouchitis, a postoperative condition that is rarely debilitating but can require chronic treatment.

C. Dysplasia or Cancer

In addition to failure of medical management, dysplasia or cancer is an indication for surgery in patients with ulcerative colitis. Patients with IBD are at increased risk of colorectal cancer (CRC). The risk of CRC in ulcerative colitis depends on the duration and extent of disease. A population-based study in the United States estimated the risk was significantly increased in those with extensive disease or pancolitis (standardized incidence ratio [SIR] 2.4, 95% CI 0.6–6.0). In addition, patients with ulcerative colitis complicating primary sclerosing cholangitis (PSC) may be at increased risk for CRC compared with those without PSC. A case-control study in which cases and controls were matched for the extent and duration of disease found that the risk of CRC was reduced with use of anti-inflammatory agents (including aspirin, nonsteroidal anti-inflammatory drugs, and 5-aminosalicylic acid [5-ASA] agents) and by surveillance colonoscopy, while it was increased in patients with a history of postinflammatory pseudopolyps.

Patients with disease extending to the hepatic flexure or more proximally have the greatest risk of CRC. Compared with an age-matched population, the risk begins to increase 8–10 years after the onset of symptoms. The approximate cumulative incidence of CRC is 5–10% after 20 years and 12–20% after 30 years of disease. Lower rates of CRC have also been found. In a population-based study from Copenhagen, the risk of CRC was not different from the general population, a finding that the authors hypothesize may have been due to an active surgical approach in medical treatment failures and long-term use of 5-ASA drugs. The highest cancer risks have been reported by medical centers that predominantly receive referral patients who may have more severe or long-standing disease.

In one series, the absolute risk of CRC in patients with pancolitis was 30% after 35 years of disease. The risk was increased in those with the onset of symptoms prior to age 15. However, in other reports, the age of onset of colitis did not increase the risk of CRC after adjusting for the longer period of time that young patients were at risk and the extent of the disease.

Most studies have found that the risk of CRC increases after 15–20 years (~ one decade later than in pancolitis) in patients with colitis confined to the left colon (ie, distal to the splenic flexure). However, rates of CRC and dysplasia similar to those seen in patients with pancolitis have been described. Patients with ulcerative proctitis and proctosigmoiditis are probably not at increased risk for CRC. Although optimal surveillance strategies for colon cancer in patients with IBD have not been established, the following recommendations for patients with ulcerative colitis have been issued by major national medical societies in the United States.

1. American Gastroenterological Association (AGA)

The AGA recommends that colonoscopic surveillance begin after 8 years in patients with pancolitis, and after 15 years in patients with colitis involving the left colon. Colonoscopy should be repeated every 1–2 years.

2. American College of Gastroenterology (ACG)

The ACG recommends annual surveillance colonoscopy beginning after 8–10 years of disease in patients who are surgical candidates. Multiple biopsies should be performed at regular intervals. The finding of definite dysplasia (of any grade) should be confirmed by an expert pathologist and is an indication for colectomy. Patients whose biopsy specimens are indefinite for dysplasia after review by an expert pathologist should undergo repeat surveillance colonoscopy at a shorter interval. The ACG recommendations do not specify whether surveillance can begin later in patients with disease limited to the left colon.

Surgical Options

The choice of operation(s) for a patient with ulcerative colitis is dependent on the presenting condition (emergent vs urgent vs elective), general issues previously discussed, and the experience of the surgeon. There are three eventual choices for the patient: (1) proctocolectomy and creation of an ileoanal anastomosis with a pouch (IPAA), (2) proctocolectomy with end ileostomy, and (3) continent ileostomy (Koch pouch or variation).

Another surgical issue is how to stage these procedures. Can the procedure be completed in one operation, or does it require a two-stage or three-stage approach? Finally, in patients undergoing IPAA, should the anorectal mucosa that lines the sphincter complex and distal rectal wall just proximal to the dentate line be “stripped,” or should 0–3 cm of this mucosa be left intact and the pouch stapled to this anorectal cuff?

Many factors are taken into account when the surgeon and patient choose an operation, including age, comorbidities, patient size, extent of disease, and patient preference. Ultimately, however, surgery results in complete removal of the colon and rectum, and near complete removal of the proximal anal mucosa, which effectively eliminates the disease.

A. Emergency Surgery

When an emergency operation is indicated, the best surgical option consists of total colectomy, end ileostomy, and the Hartmann pouch (stapling or sewing off the stump of the rectum). As mentioned, this procedure avoids a pelvic dissection, removes most of the large intestine, avoids an anastomosis, and does not eliminate any future options. The procedure also is used in patients in whom the diagnosis of ulcerative colitis is not clear. If the differential diagnosis includes Crohn disease, indeterminate colitis, or C difficile or other infectious colitis, this procedure should be performed; the eventual surgical choice can then be discussed after the pathologist has been able to examine the whole specimen. On extremely rare occasions, the surgeon’s only option is a diverting ileostomy. The only indication for this procedure is a patient who is hemodynamically unstable on the operating room table.

B. Ileoanal J Pouch Procedure