Ulcerative Colitis Ulcerative colitis (UC) is a disease that involves the inner lining (mucosa) of the colon and rectum (Fig. 1). While the cause remains elusive, surgical removal of the entire colon and rectum is curative. Patients and their physicians need to weigh the therapeutic options available and decide when surgical management is indicated. The ideal operation for ulcerative colitis would remove the diseased bowel and leave the patient with normal bowel function and free from medication. Unfortunately, the ideal operation does not exist making it vital that each patient consult with their physician in order to choose an operation that will best treat their disease and fit their lifestyle. To aid in these decisions, this paper discusses the surgical alternatives in the management of ulcerative colitis. Normal preoperative anatomy is schematically demonstrated in Figure 1 and can be used as a reference for the subsequent operative drawings.
Indications for Surgery Indications for surgery can be arbitrarily divided into three categories: 1) immediate or acute life threatening; 2), chronic debilitating; and cancer or cancer risk.
Acute Life-threatening Conditions Approximately 20 % of patients require urgent or emergency surgery for acute problems1. These acute conditions include toxic colitis, perforation, and massive bleeding (hemorrhage). Fortunately with advances in patient care, such as avoidance of barium enemas and antidiarrheal medication (eg. Immodium) during acute disease flares, the frequency of surgery for toxic colitis has decreased. Patients with severe acute manifestations of ulcerative colitis are initially managed with supportive measures to include intravenous fluids and medications such as steroids. The patient's intestinal tract is placed at rest by avoiding oral intake and in patients with nausea or vomiting, nasogastric suction is added. Anemia patients age given blood transfusions and nutritional supplementation may also be given intravenously. Surgery is recommended if the patient develops an intestinal perforation, has an overwhelming abdominal infection (peritonitis), or is not responding to medical treatment. If surgery is required, these acutely ill patients receive a subtotal colectomy and ileostomy (Figure 2). This procedure, which is described in greater detail later in this article, removes the majority of diseased bowel, avoids an anastomosis which has an unacceptablly high leak rate in these very sick patients, and preserves future options. An unusual problem is the patient with toxic colonic dilatation (toxic megacolon) who has no obvious perforation. These patients are so sick and their colon is so weakened that attempts to remove the colon would lead to it rupturing and spilling stool throughout the abdomen. These patients are best managed with a special type of transverse loop colostomy and a diverting loop ileostomy. This procedure safely decompresses the colon, allows the patient to recover from their illness, and serves as a bridge until a more definitive procedure can be performed. Hemorrhage is currently the most common cause for urgent or emergency surgery in UC patients. These patients are sick, often malnourished, and are also managed with a Sub-total colectomy and an end ileostomy.
Chronic Debilitating Conditions The majority of UC patients (approximately 70%) undergo surgery for chronic problems such as crampy abdominal pain, the urge for frequent bowel movements, or chronic diarrhea that significantly impacts their life style. Additional chronic problems include malnourishment, and failure to grow in children, infertility in women, and effects of medication. Side effects of long term steroid use (Table 1) are profound and can be devasting. 2 Many of these conditions are related to both the dose duration of steroid treatment. Some, such as weight gain, skin changes and fat deposition, are reversible after the steroids are stopped; however, others, most notably the development of diabetes and kidney failure, are irreversible. Since effective surgical options are available to cure ulcerative colitis, it is unacceptable to excessively delay surgical therapy and allow these significant problems to develop. Additional side effects which may limit patient's ability to use medication include low sperm counts for males (associated with asulfaduine) and peripheral neuropathy (nerve pain or numbness in fingers or toes) related to the use of metronidazole. Uncontrolable medication side effects or failure of medical therapy to adequately control a patient's symptoms are the most frequent indication for surgery.
Cancer Risk Ten percent of patients with UC undergo surgery for cancer or the risk of cancer. When the entire colon is affected by colitis (pan colitis) , the risk of developing cancer has been estimated as 3% at 15 years, 5% at 20 years and 9% at 25 years. The development of atypical premalignant cells (dysplasia) appears to precede development of cancer. However, colorectal cancer has been reported to occur in the absence of dysplasia and not all patients with dysplasia develop cancer. Due to the very real risk of cancer, it is reasonable for patients with pan ulcerative colitis of greater than eight years duration to undergo annual colonoscopy with random biopsies. In this procedure, 1-2 mm colonic mucosal biopsies are obtained from each portion of the colon (for a total of at least 10 biopsies). Patients with dysplasia on pathologic review or those who are unable or unwilling to participate in surveillance should be considered for surgical therapy. When cancer is diagnosed in a colitic patient, surgical therapy is indicated. The section of bowel containing the tumor as well as the surrounding lymph nodes should be removed, as would be done for a person without UC. Unlike patients not affected by UC, in patients with UC the remaining colon and rectal mucosa is removed as described below. As in all operations, the risks of the surgical therapy must be balanced against the benefits to the patient. Risks of surgery include the morbidity and mortality associated with the procedure itself and the alteration in life style resulting from the removal and reconstruction of the bowel. The benefits include a cure of UC, if the entire colon and rectal mucosa is removed, removing the risk of developing cancer, and improving an individual's lifestyle. After surgical therapy, the patient's symptoms should be eliminated or significantly reduced. In addition, requirements for immunosupressive medication are reduced or eliminated. Many people are often unaware of the extent to which their lives are or have been controlled by their disease. Patients know the exact location of each bathroom in their area. Limitations in social activities and work, as well as the lack of well being and a reduced energy level, are often significantly improved after surgery. Balancing these complex risks and benefits for each individual patient helps select the appropriate surgical option. Operative procedures are expensive. However, operative costs must be compared to the loss of employment with disease execrations, the cost of medications, medical therapy and surveillance, and the threat of cancer.
Five surgical alternatives for managing ulcerative colitis are listed in Table 2. The following paragraphs briefly describe each procedure and summarize the advantages and disadvantages of each.
Subtotal Colectomy with Ileostomy This is a smaller operation that is performed through an abdominal incision. The majority of the colon is removed and an end-ileostomy is created in the right lower abdomen (Fig 2 A & B) . The rectum is left in place to drain out the anus. The proximal end of the rectum may be closed as a Hartman's pouch or closed at the distal sigmoid level and brought up the skin of the lower abdomen as a mucous fistula. As described earlier, a subtotal colectomy is used in acutely ill patients. It leaves the patient with an ileostomy which may be temporary or permanent and maintains future options for the patient. Disadvantages of this procedure include the requirement for an external appliance (ileostomy pouch) which the patient must wear continuosly and the risks associated with a retained diseased rectum. Although it has disadvantages, overall patient acceptance of a conventional ileostomy is quite high. Long term, almost all patients will require another operation after a subtotal colectomy and ileostomy to takedown (close) the ileostomy or to remove the patient's remaining rectum. This later option can be accomplishedwith or without reconstruction (pouch-anal anastomosis).
Colectomy with Ileorectal Anastomosis This operation is performed in one stage through the abdomen. The entire colon is removed and the distal end of the ileum (small bowel) is attached to the upper rectum (Fig 3). The anastomosis is performed in an end-to-end fashion with an intraluminal circular stapling devise or sutures. This is a safe operation with a reported anastomotic leak rate of < 2 % and a mortality rate of 1.4 %. With this operation there is no pelvic dissection and the patient is spared an ileostomy and a perineal wound. The retained rectum, however, is at risk for progression or recurrence of the inflammatory disease and the development of cancer. The risk of cancer in the retained segment is related to the duration of the patient's disease. In reviewing several series of patients after a subtotal colectomy, cancer occured in no patients at less than 10 years, in 2-6% of patients at 10-19 years, and 13-15% of patients at 20-30 years. This is an excellent operation for patients with minimal rectal disease (a distensible rectum) and good sphincter tone; especially young patients. If the rectal disease becomes a problem, (reported to occur in 50% of patients) additional procedures (ie. protectomy or pouch anal anastomosis) are performed. Bowel function after subtotal colectomy and ileorectal anastomosis is related to the age of the patient and the length and status of the reatined rectum. Younger patients with adaptable small bowel can be expected to average 2-3 bowel movements per day. Older patients routinely average 3-4 bowel movements per day and may require medication such as loperamide hydrochloride (Immodium) to reduce urgency.
Proctocolectomy with Brooke Ileostomy This large operation cures the patient of ulcerative colitis as the entire colon and rectum are removed, but leaves the patient with a perminent ileostomy (Fig 2 A & C). As one of the earliest operations performed to cure ulcerative colitis, this operation has the longest patient follow-up. Disadvantages of this operation, include the pelvic dissection associated with the proctectomy and the need to wear a permanent external appliance. The operation for inflammatory bowel disease differs from that of cancer in that the dissection is performed close to the bowel wall. However, a small but significant incidence of bladder or sexual dysfunction (less than 1%) persists. Most experienced surgeons perform an intersphincteric protectomy which leaves a smaller perineal wound which is closed primarily. The majority of patients heal this wound without difficulty. Unfortunately, a small number of patients develop a non healing perineal wound which can be a difficult problem to resolve. Multiple procedures varying from curretage to placement of a vascularized graft may be required to obtain complete healing.
Proctocolectomy with Continent Ileostomy (Kock Pouch) To reduce some of the problems with a conventional ileostomy, a continent ileostomy was developed (Fig 4). This intrabdominal pouch is comprised of 2 - 3 loops of small bowel and an intuscepted valve which provides internal storage for the patient's intestinal contents. The pouch is attached to the abdominal wall with a flush ostomy opening. The patient empties the pouch 4-6 times per day by inserting a silastic or plastic catheter through the stoma into the pouch. A continent ileostomy eliminates the requirement for an external appliance which simplifies social and recreational activities. Thus this procedure offers the patient a cosmetic option. Despite multiple technical modifications, valve slippage remains a significant problem with this operation and is reported to occur in 5-15% of patient followed long term. If significant valve slippage occurs (usually manifested by pouch incontinence or difficulty with pouch intubation) surgical correction is required. If a patient develops difficulty in intubating their pouch, they must seek medical attention. A continent pouch which can not be intubated results in a complete small bowel obstruction. If the pouch can not be intubated with a catheter, endoscopic intubation may be required. Development of pouch anal procedures (discussed later) has significantly reduced the demand for continent ileostomies. However, a small select group of patients are best served with this option. It is also a consideration in the unfortunate patient that looses their ileoanal pouch due to septic complications.
Restorative Proctocolectomy with Ileal Pouch Anal Anastomosis This operation removes the entire colon, upper rectum, and anal mucosa. The distal ileum is then fashioned into a pouch or reservoir and connected to the anus (Fig 5). Several types of pouch construction have been described (eg. S, J, W, H). A "J" pouch configuration is the easiest to construct and is the most common type of pouch in current clinical use. A temporary ileostomy is often constructed during the first stage of the procedure (Fig. 6). Advantages of this operation include the elimination of all colorectal mucosa and preservation of reasonable postoperative bowel function via an anal route. The complicated technical requirements of the surgery lead to several disadvantages. Most patients need a temporary stoma (loop ileostomy) for 6-8 weeks to allow complete healing. Thus two operations and hospitalizations are required. Postoperative bowel function is variable but patients average 8-10 movements per day in the initial postoperative period and 4-6 movements per day at one year after surgery. Anal seepage or soilage occurs in some patients which necessitates wearing a protective pad. Some antidiarrheal medication (loperamide hydrochloride, diphenoxylate hydrochloride with atropine sulfate), and or bulking agents to control the frequency of bowel movements for the first 6 to 12 months after surgery. An occasional patient may require medication long term. An additional potential risk after pouch construction is pouchitis. This condition often presents as urgency, increased frequency, and loose bloody stools. The etiology of this condition is unclear but patients usually respond rapidly to treatment with antibiotics (eg. metronidazole). An occasional patient requires removal of the pouch to resolve excessive symptoms. Despite these limitations a colectomy with an ileal pouch anal reconstruction is currently the procedure of choice for ulcerative colitis.
Proctocolectomy with Ileal Pouch Anal Transitional Zone Anastomosis (IPATZa, Double Staple) This procedure is a modification of the ileal pouch anal procedure. It is technically simpler to perform and the operation can usually be accomplished in 45-60 minutes less time than traditional methods (Fig 5B). In good risk patients (no steroids, well nourished, easy operation) some surgeons avoid using a temporary stoma with this procedure. This eliminates the poptential risks and difficulties asociated with a stoma and the second operation required to close the stoma. However, this option places the patient at greater risk if a septic pouch complication occurs. A potential limitation of IPATZA is the retention of one to two cm of anal transition zone. The long term results of retaining the anal transition zone are not presently known. Although proponents of this procedure claim a better functional result two prospectivew trials using this technique with short term follow-up have failed to demonstrate a significant clinical improvement with the double staple technique. The author currently performs both versions of the pouch operation in a selective manner.
Summary Many surgical options are available for patients with ulcerative colitis. These options can be individually tailored to meet the need of treatment and best accommodate an individual's lifestyle. Consultation and communication between patients and their surgeon remains the best chance for a successful outcome
Additional Readings 1. Beck DE, Whitlow CB. Surgical treatment of ulcerative colitis. Ostomy Quaterly 1997; 34:36-40. 2. Beck DE. Steroids: The good and the bad. Ostomy Quarterly. 2004;41:64-67.