41 Surgical Management of Ulcerative Colitis

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41

Surgical Management of Ulcerative Colitis

Phillip R. Fleshner and David J. Schoetz, Jr.

567 Ulcerative colitis (UC) is a diffuse inflammatory disease of

the mucosal lining of the colon and rectum that manifests clinically as diarrhea, abdominal pain, fever, weight loss, and rectal bleeding. Because removal of the affected organ is cur- ative, surgery has assumed a pivotal position in the management of these patients. Although removal of the entire colorectum and permanent ileostomy had been the standard operation for decades, increased experience with anal sphincter preservation has demonstrated the feasibility of performing surgical procedures that spare sphincter function while still removing all disease. This chapter considers the surgical alternatives, decision making, and techniques surrounding these procedures.

Indications for Surgery

The overall incidence of colectomy in a UC patient ranges from 23% to 45%.^1–3This risk is higher in patients with pan- colitis than in patients with left-sided disease.^1,2Indications for colectomy include an acute flare unresponsive to medical measures, development of a life-threatening complication (e.g., toxic colitis, perforation, or hemorrhage), medical intractability, risk of malignancy, disabling extracolonic disease, and growth retardation in children. During an episode of acute colitis, the patient should be aggressively treated with intravenous steroids and bowel rest. The role of parenteral hyperalimentation in this situation is controversial.

Encouraging results have been reported with the use of cyclosporine in acute colitis⁴yet long-term effectiveness of this particular treatment modality remains undefined.

However, there is no reported increase in the incidence of perioperative complications after subtotal colectomy in patients treated before surgery with cyclosporine.^5,6

Patients with life-threatening complications are generally easy to recognize and define. Nevertheless, these patients are frequently taking large doses of steroids and may appear deceptively well; consequently, appreciation of the severity of the disease and the timing of operation are of paramount

importance. Medical intractability is the most common indication for operation and may seem difficult to define. In fact, there is probably no strict definition that a physician can uni- formly apply. It is important to recognize that medical intractability is a problem the patient identifies in conjunction with the physician. Although a physician may believe that 12 months of steroids or other immunosuppressive management without complete resolution of symptoms is an adequate medical trial, the patient must be convinced that surgery is indicated. Only the patient can decide he or she feels fatigued, has missed much work or school, or is unable to do things he or she would like to do because of the systemic effects of active colitis and its treatment. If the surgeon waits until the patient has arrived at the conclusion that the disease is not sat- isfactorily controlled medically, the patient will graciously accept alternatives the surgeon has to offer. We believe this is a particularly important strategy for the surgeon to use if the patient is to be satisfied.

Patients with UC are also prone to the development of colorectal cancer. The risk of cancer is relatively low for the first 10 years after disease onset but then begins to increase at a rate of 1%–2% per year.⁷Thus, by the time the patients have had the disease for 20 years, the cumulative risk of colorectal cancer may be as high as 20%. The question of timing of surgery for cancer prophylaxis remains controversial. Certainly, with an established carcinoma, surgical treatment is mandatory. More controversial, however, is the management of patients with dysplasia. Most surgeons contend that during a surveillance biopsy program, identification of high-grade dysplasia by an experienced pathologist is an indication for colectomy. Patients with low-grade dysplasia should also be offered colectomy, although nonoperative management of these patients has been suggested by some because the natural history of low-grade dysplasia has not been well established.⁸ Elective colectomy may be indicated for some categories of severe extraintestinal manifestations of the disease. Persistent or recurrent monoarticular arthritis, uveitis, or iritis all respond favorably to colectomy. However, primary sclerosing cholangitis, ankylosing spondylitis, and sacroiliitis are not

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improved by colectomy. The response of pyoderma gan- grenosum to colectomy is unpredictable.

Growth retardation is a common feature in children with UC. Contrary to popular belief, steroid therapy cannot be entirely blamed for delayed growth. Inadequate protein intake and excess loss in the colon are also contributory.⁹ A rapid growth spurt is often observed after definitive surgery.

Emergency Versus Elective Procedures

Operative management of UC largely depends on whether the surgery is elective or emergent. Under elective conditions, the four available surgical options are: 1) total proctocolectomy and Brooke ileostomy, 2) total proctocolectomy and continent ileostomy, 3) abdominal colectomy with ileorectal anastomosis (IRA), and 4) ileal pouch–anal anastomosis (IPAA). Total proctocolectomy and Brooke ileostomy has been traditionally regarded as the optimal surgical approach and remains the operation with which alternative procedures should be com- pared. The technique has been well described and the immediate and late results are very satisfactory. Furthermore, patients avoid any risk for cancer, steroid medications are eliminated, and physician visits and reoperations are kept to a minimum. Although quality-of-life studies¹⁰ have demonstrated excellent results, the loss of fecal continence and its attendant physical and psychologic sequelae continue to be significant drawbacks of the procedure. In addition, problems with nonhealing of the perineal wound, and the high incidence of small bowel obstruction and ileostomy revision, are not to be minimized.

Total proctocolectomy and continent ileostomy couples the benefit of complete large bowel excision with a reduction in some of the untoward aspects of an ileostomy, because no external appliance is needed and the stoma can be placed in a less conspicuous position on the abdominal wall. Continent ileostomy can be performed at anytime in UC patients having previously undergone total proctocolectomy and Brooke ileostomy if they find a standard ileostomy unsatisfactory.

Because of increased surgical experience and improved surgical techniques, continent pouch morbidity has decreased since its initial clinical description. Most patients are ultimately happy with the results of the operation. Nonetheless, troublesome complications leading to incontinence continue to plague the postoperative course of a substantial number of patients.¹¹

There are many attractive features of colectomy and IRA.

The procedure avoids the perineal complications of total proctocolectomy, the risk of sexual dysfunction is minimal, is technically easy to perform, may provide perfect control of feces and flatus, and is well accepted by most patients.

However, unlike the three other surgical options, ileorec- tostomy does not achieve total excision of colorectal mucosa.

Many surgeons have not used this operation for UC, arguing that more than 25% of patients will require subsequent rectal

excision for persistent proctitis,^12,13 a small percentage of patients will develop cancer in the rectal remnant, and only half of the patients have satisfactory long-term functional results. Although we concur that this operation should not be advised in most UC patients, IRA does have a role in certain clinical situations. For example, an elderly patient with a long history of UC who develops a transverse colon cancer may be well served with an IRA in lieu of total proctocolectomy.

Decisions must be made on an individualized basis, taking into account the compliance of the rectum and the integrity of the sphincter mechanism.

IPAA has the attractive features of complete excision of the colorectal mucosa, avoidance of a permanent intestinal stoma, continence via a normal route of defecation, and no prospect for a troublesome nonhealing perineal wound. Continence is usually preserved and the frequency of defecation is diminished with incorporation of a pelvic pouch into the operative procedure. Although the operation is associated with minimal mortality, the morbidity of this complex procedure is relatively high, and problems such as small bowel obstruction and pouchitis continue to be a cause for concern.

Under emergent conditions, surgical alternatives are limited. If the patient is septic, the diseased or perforated bowel should be removed. If the colon is bleeding, the colon should be removed. Traditionally, it has been taught that the rectum should also be removed. However, with the sphincter-saving alternatives that are currently available, careful preoperative proctoscopic evaluation to exclude a rectal etiology for the bleeding and a subsequent abdominal colectomy with end ileostomy can be safely performed. A subsequent procedure can then restore intestinal continuity.

Similarly, with toxic colitis, it is seldom necessary to perform a proctectomy at the time of colectomy. In general, concerns over healing of the perineal wound in these frequently malnourished patients who are taking high-dose steroids should deter surgeons from doing proctectomy in the emergent setting. The authors have not found it necessary to use the blow-hole technique of Turnbull, but this is a philosophically acceptable approach in that it does not preclude subsequent continence-preserving alternatives. This technique is mainly of historical significance because most UC patients currently present for colectomy earlier and are not as nutritionally depleted as when this technique was frequently used.¹⁴

A few technical issues regarding subtotal colectomy in these patients must be stressed. Mesenteric dissection in the vicinity of the ileocecal valve should be flush with the colon to preserve ileal branches of the ileocolic artery and vein.

These branches are necessary to facilitate subsequent construction of an ileal pouch. Distally, it is unnecessary to mobi- lize the rectum within the pelvis. In fact, dissection of the sigmoid to the sacral promontory, without violation of presacral planes, and a Hartmann procedure are recommended.

This has shown to decrease the incidence of pelvic sepsis and facilitate subsequent pelvic surgery. The colon should be

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transected at the rectosigmoid junction and an intraperitoneal rectal stump created. Technically, closure of the rectal stump can be hazardous because the bowel is markedly inflamed and does not hold sutures or staples well. Bringing the distal site of transaction out as a mucous fistula, which can be either primarily matured or buried within the abdominal incision¹⁵or the subcutaneous tissue,¹⁶are alternative techniques to safely manage the very diseased rectal stump. A transanal rectal drain may prevent leakage from the diseased Hartmann pouch closure site.

There is a trend to avoid subjecting patients to multiple surgical procedures and to perform a definitive procedure at the time of emergent surgery. Although an IPAA can be success- fully performed in patients undergoing surgery for emergent complications, the authors believe this generally is not a safe approach. These patients are usually on high doses of steroids and are nutritionally depleted. Patients with UC receiving high-dose steroids (more than 40 mg/day) have a significantly greater risk of developing pouch-related complications after colectomy than patients with UC receiving 1–40 mg/day and patients with UC who are not receiving corticosteroids.⁸From a practical standpoint, surgical options are limited in emergent situations. Salvage of the patient should be the primary concern. Abdominal colectomy is safe in these very ill, nutritionally depleted patients^5,15–18 and the procedure does not preclude any of the other surgical alternatives in the future. Additionally, the patient is able to live with an ileostomy and assess its impact on his or her life, thus allowing for an informed decision regarding subsequent continence-restoring surgery.

Brooke Ileostomy

The preoperative period should include effective patient education. A patient must be fully informed of the effects of an ileostomy on his or her quality of life. An ileostomy visitor, preferably age and sex matched and who has completely recovered from surgery, is invaluable during this period.

Resistance to a permanent ileostomy can be tempered by stressing the beneficial aspects of this operation (e.g., curing the disease). It is also essential, when possible, to select the stoma site preoperatively with the help of an enterostomal therapist. As discussed in Chapter 44, the stoma should be placed in a flat area away from bony prominences, scars, and significant skin creases. Attention to these details will ensure a well-functioning ileostomy.

Operative Technique

A colectomy is performed in the standard manner with the patient in a modified lithotomy Trendelenburg position. The proctectomy phase of the procedure is remarkable for keep- ing the dissection close to the rectal wall, especially anteri- orly in the area of Denonvilliers’ fascia. Meticulous

dissection to minimize the risk of injury to pelvic autonomic nerves is essential. Perineal dissection should be performed in the intersphincteric plane. After the colorectum is removed, a Brooke ileostomy is constructed.¹⁹An appliance is then placed over the stoma. Bowel function is expected in 3–6 days.

In some situations, the end of the ileum does not reach far enough through the abdominal wall to allow primary maturation. In these situations, the mesentery is usually a limiting factor and selection of a more proximal site in the bowel may allow better mobilization. Alternatively, a loop-end ileostomy rather than an end ileostomy may reach more easily.

Postoperative Complications

A proctocolectomy with a Brooke ileostomy is a safe procedure with a predictable long-term outcome. It is, however, not entirely free of complications. Delayed healing of the perineal wound is not uncommon and can be quite problematic.²⁰ Failure of the wound to close should prompt investigation to exclude the presence of retained mucosa, foreign material, or Crohn’s disease (CD). Sexual complications of proctocolectomy in men are much less common than in patients having a radical resection for cancer, yet permanent impotence or ret- rograde ejaculation can occur. Almost 30% of women com- plain of dyspareunia after this operation, presumably as a result of perineal scarring.²¹Intestinal obstruction is a troublesome complication that can be managed conservatively in most patients. Gentle irrigation of the stoma is an important therapeutic maneuver. Prolonged nonoperative treatment should not be pursued for fear of infarction. Although problems from the ileostomy have diminished markedly with the use of modern appliances and the Brooke modification, skin irritation, stomal stenosis, prolapse, and herniation remain significant causes of postoperative morbidity. Treatment of these problems can be as simple as reeducating a patient about the proper maintenance of the ileostomy. However, up to one-third of these patients ultimately require operative revision.²² Despite the fact that these patients have undergone major abdominal surgery and have a permanent stoma, their quality of life as measured by validated questionnaires is very good and similar to that of the general population.¹⁰ More than 90% of patients are happy with their current lifestyle.

However, significant problems do remain. Almost 25% of patients are restricted in their social and recreation activities, and nearly 15% of patients who are knowledgeable of alternative procedures would consider conversion. In short, the Brooke ileostomy is generally well accepted, although a number of patients experience significant psychosocial and mechanical difficulties.

Current indications for the procedure include elderly patients, individuals with distal rectal cancer, patients with severely compromised anal function, and patients who choose this operation after appropriate education.

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Continent Ileostomy

Physicians involved with patients requiring an ileostomy should be aware of the continent ileostomy. Although this procedure is less often performed today, it remains a viable alternative in patients who have discrete problems with an appliance. A continent ileostomy is usually reserved for patients who have failed Brooke ileostomy or those who are candidates for an IPAA but cannot have a pouch because of rectal cancer, perianal fistulas, poor anal sphincter function, or occupations that may preclude frequent visits to the toilet.

Preoperatively, a search for CD using barium examination of the stomach and small intestine is important. Suspicion of CD contraindicates construction of a continent ileostomy, because the risk of recurrent disease in the pouch is increased;

this could necessitate resection of 45 cm of valuable small bowel and render the patient unable to maintain nutrition.

Obesity and age over 40 years are associated with an increased risk of pouch dysfunction and represent relative contraindications to the continent ileostomy.²³

The period before surgery must also include an open dis- cussion with the patient, stressing that although continence is likely, major complications often occur. These setbacks generally must be corrected surgically, sometimes leading to pouch excision and creation of a standard Brooke ileostomy.

The patient must comprehend that by learning to care for and intubate the reservoir, he or she has an important role in its functional outcome. Only highly motivated, emotionally stable individuals should consider this procedure.

Operative Technique

Patients undergoing combined total proctocolectomy/continent ileostomy have a proctocolectomy performed in the usual manner. Excision of a very short segment of terminal ileum and a diligent search for CD during the procedure are essential. In patients with a standard ileostomy undergoing conversion to continent ileostomy, the stoma is mobilized from the abdominal wall. Construction of the reservoir in these two patient groups is then performed in an identical manner.

The technique of constructing a continent ileostomy is conceptually difficult (Figure 41-1). Using the terminal 45–60 cm of the ileum, an aperistaltic reservoir is created by making an S-pouch or a folded two-limb pouch originally described by Kock et al.²³In the classic technique, two 15-cm limbs of ileum are sutured together with continuous absorbable sutures to form a pouch. The antimesenteric bor- der is incised and then folded over to form a reservoir. The ileum immediately distal to the reservoir is then scarified with electrocautery and 5 cm of adjacent mesentery is removed or thinned of fat and intussusception of this terminal 15 cm of ileum into the pouch is performed. The intussusception is secured with multiple nonabsorbable sutures and staples. The end of the ileum is then brought through the abdominal wall at the preoperatively identified site. Because

no external appliance is required, a continent ileostomy can be located lower on the abdominal wall for cosmetic reasons.

The stoma is sutured flush with the skin and the pouch firmly anchored to the posterior rectus sheath. A wide plastic tube with large openings (i.e., Madina catheter; AStra Tech, Molndal, Sweden) is placed into the pouch to allow gravity drainage of the pouch in the early postoperative period. This tube is occluded for progressively longer periods beginning 10 days after surgery until it can be removed for 8 hours without distress. At this point, the pouch is significantly expanded, the tube is removed, and drainage is achieved by intubating the pouch three times a day.

FIGURE 41-1. Continent ileostomy. A Three limbs of small bowel are measured and the bowel wall is sutured together. B After opening the bowel (see the dotted lines in A), the edges are sewn together to form a two-layered closure. C A valve is created by intussuscepting the efferent limb into the pouch and fixing it in place with a linear non- cutting stapler. (Inset: staples in place on valve). D The valve is attached to the pouch sidewall with the linear noncutting stapler (a cross-section of the finished pouch is shown). E After closure of the last suture line, the pouch is attached to the abdominal wall and a catheter is inserted to keep the pouch decompressed during healing.

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Postoperative complications that occur with sufficient frequency are nipple valve slippage, pouchitis, intestinal obstruction, and fistula. Nipple valve slippage^24,25occurs because of the tendency of the intussuscepted segment to slide and extrude on its mesenteric aspect. Difficult pouch catheterization, chronic outflow tract obstruction, and incontinence ensue. Because of the frequency of this problem, many techniques other than simple surgical stapling have been described to stabilize the valve. Wrapping the valve with prosthetic materials does prevent valve slippage but also is accompanied by a potentially unacceptably high incidence of parastomal abscess and fistula formation.¹¹Despite these technical modifications, nipple valve slippage remains the most common complication after continent ileostomy, occur- ring in almost 30% of patients.^11,24,25Although nonoperative approaches have been attempted to correct this problem, surgical correction is virtually inevitable. Repair of the existing malfunctioning valve or creation of a new valve from the afferent ileal limb is performed.

Pouchitis is recognized in 25% of patients, making this the second most common postoperative complication after continent ileostomy.^11,23,24Pouchitis refers to nonspecific inflammation that develops in the reservoir, and is thought to result from stasis and overgrowth of anaerobic bacteria. Patients present with a combination of increased ileostomy output, fever, weight loss, and stomal bleeding. The diagnosis is made by history and confirmed by pouch endoscopy. Pouchitis usually responds to a course of antibiotics and continuous pouch drainage.

Other complications include an incidence of intestinal obstruction after continent ileostomy of about 5%. Surgical intervention is mandatory when nonoperative therapy has been unsuccessful. The incidence of fistulas after creation of a continent ileostomy is approximately 10%. Fistulas usually originate in the pouch itself or at the base of the nipple valve.

Pouch fistulas result from dehiscence of suture lines or, rarely, ileostomy tube erosion. These tracts may close with bowel rest, parenteral nutrition, and continuous pouch drainage.

Fistulas from the base of the valve lead to incontinence, because ileal contents bypass the high-pressure zone of the nipple valve. These fistulas usually arise with tearing of the sutures anchoring the pouch to the anterior abdominal wall. Valve fistulas rarely heal without operation. At laparo- tomy, the valve is excised, the pouch rotated, and a new continent valve constructed from the afferent tract.

Patient satisfaction with a continent ileostomy is excellent.²⁶ Most patients note a marked improvement in their lifestyle, and almost all patients work and participate in social and recreational activities without restriction.²⁴These observations are understandable in that 90% of patients eventually have total continence after one or more procedures. However, their enthu- siasm is surprising considering that complications are quite frequent and often require major surgical intervention.^23,24 The often advertised Barnett modification of the Kock pouch

(Figure 41-2) uses the afferent limb of small bowel to construct the nipple valve and wraps a portion of the residual efferent limb around the nipple valve.²⁷This modification was designed to reduce the incidence of valve slippage and fistula formation.

Another recently described variation²⁸is the “T-pouch” in which a portion of ileum is folded into the side of the pouch rather than being intussuscepted (Figure 41-3). Theoretically, this eliminates valve slippage. Unfortunately, there are no controlled data to suggest that either of these modifications is any better than the standard procedure most centers are using.

Ileorectal Anastomosis

Before the advent of IPAA, abdominal colectomy with IRA was performed in UC patients who might otherwise have been offered a permanent ileostomy. Currently, IRA is mainly considered in patients with indeterminate colitis (IC), in

FIGURE 41-2. Barnett continent ileostomy reservoir (BCIR). A Two limbs of small intestine are sewn together and opened. B The affer- ent limb is intussuscepted to form a valve and the valve is stapled to the side of the reservoir. C The pouch is folded back and sutured closed. Insert shows cross-section of pouch. D Completed BCIR.

The afferent limb of bowel has been divided and reattached to the apex of the pouch and the efferent limb is wrapped around the valve to form a collar.

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high-risk or older patients who are not good candidates for IPAA, or if there is mild rectal disease in which rectal compliance remains adequate. The use of the operation may also be indicated in the teenager or young adult to rapidly regain good health, avoid a stoma, and return to school or work quickly. Functional results depend on the level of the anastomosis as well as the state of the rectum. Contraindications to IRA include a very diseased and noncompliant rectum, dysplasia or nonmetastatic cancer, perianal disease, and a severely compromised anal sphincter.

IRA is a safe operation; mortality is low, particularly when it is performed as an elective procedure. The early morbidity of IRA is low, with the incidence of anastomotic leak being less than 10%, and major sepsis is very uncommon. Sexual

function is well preserved. The overall complication rate is much lower than that of an IPAA.¹²Although the frequency of defecation after IRA is variable, most patients pass between two and four semiliquid stools a day. Nocturnal defecation is quite common, but true incontinence is rare.²⁹

The main concerns surrounding IRA for UC are the long- term issues regarding cancer risk in the retained rectum and the incidence of persistent rectal inflammation. The overall risk of cancer developing in the rectum after IRA approxi- mates 6%, but this depends on the duration of follow-up.³⁰ Few of these cancers develop less than 10 years after operation, with most cancers appearing 15–20 years after operation. Cancer in the rectum after IRA produces few symptoms and early lesions are not always easily identified at sigmoidoscopy. Patients being offered IRA must realize the need for semiannual sigmoidoscopy with multiple biopsies to detect dysplasia, polyps, or invasive cancer. This recommen- dation is particularly important in young adults or children because these patients have the highest risk of developing cancer and are much more likely to be lost to follow-up.

The rectal stump may be the site of recurrent or persistent inflammation in 20%–45% of patients. Clinical features include severe diarrhea, tenesmus, bleeding, and urgency.

Rectal excision is needed in those cases that do not respond to topical or systemic therapies. About one-quarter of patients require proctectomy after IRA for severe proctitis.^12,13 The only clinical factor that predicts a successful outcome is the degree of inflammation in the rectum preoperatively, minimal proctitis being associated with an excellent prognosis.¹² A great advantage of the IRA is that should a failure occur, other options remain. Conversion from an IRA to an IPAA may be required when there is a poor functional outcome because of poor rectal compliance, persistent and disabling proctitis, and with development of an upper rectal cancer. If conversion to IPAA is required, it can be performed safely, although poorer bowel function may be expected. However, quality of life is similar before and after conversion in these patients.³⁰

Ileal Pouch–Anal Anastomosis

The most attractive of the continence-preserving alternatives is the IPAA, which consists of near total proctocolectomy, creation of an ileal reservoir, and preservation of the anal sphincter complex. The original operation as described by Sir Alan Parks included a complete stripping of the anal mucosa of the anal canal.³¹In an attempt to improve functional outcome, some surgeons^32,33preserve the anal transition zone and perform a stapled anastomosis between the ileal pouch and the anal canal immediately cephalad to the dentate line (“double-stapled” technique). Both of these techniques remove the colorectum without creating a perianal wound, preserve innervation to the anus, bladder, and genitals, and retain the usual pathway for defecation. Preoperatively, the rectum should be evaluated sigmoidoscopically. Active rectal FIGURE 41-3. T-pouch. A Seromuscular sutures approximate the

back wall of the pouch and fix the valve segment to the pouch through mesenteric windows. B The bowel is opened. C Edges of the bowel are closed over the valve segment. D The reservoir is folded in half and closed.

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disease requires topical 5-aminosalicylic acid or steroid enemas to minimize rectal inflammation and facilitate mucosectomy. The anorectal sphincter mechanism must be intact to prevent leakage of watery ileal contents. Use of this procedure in patients with poor sphincter function or fecal incontinence must be carefully individualized. Preoperative evaluation also allows the surgeon to be certain that patients undergoing this operation are highly motivated and willing to cope with potential postoperative complications.

Operative Technique

After appropriate bowel preparation, the patient is brought to the operating room and placed in the modified lithotomy position. A midline incision is made and the abdomen explored to rule out evidence of CD. The colon is mobilized in the usual manner. A few technical points should be stressed. Omen- tectomy may be inappropriate, because there is a lower incidence of postoperative sepsis when the omentum is preserved.³⁴ Stapling of the distal ileum flush with the cecum is very important, as is preservation of the ileal branches of the ileocolic artery and vein. These vessels provide perfusion of the pouch after mesenteric division. The pelvic peritoneum is incised and rectal mobilization begun. Dissection is carried ventrally to the level of the prostate in men and the mid-portion of the vagina in women. Posteriorly, the dissection is carried past the end of the coccyx. Mobilization of the rectum should be flush with

fascia propria to minimize damage to nearby autonomic nerves traveling to urinary bladder and sexual organs.

Mucosal stripping is performed from a perineal approach.

The use of a Lone Star™ retractor facilitates exposure and minimizes damage to the sphincter mechanism (Figure 41-4).

A solution of dilute epinephrine is injected into the submu- cosal plane to facilitate mucosectomy and minimize bleeding (Figure 41-5). The excised mucosa and remaining proximal rectum are removed, leaving a short cuff of denuded rectal muscle distally for about 4 cm above the dentate line.

Attention is then directed toward creation of the ileal reservoir. The terminal ileum is aligned in a J configuration and the pouch constructed with either a continuous absorbable suture or stapling device (Figures 41-6 to 41-9). Both limbs of the J are approximately 15–25 cm in length, the exact length guided by where the pouch reaches deepest into the pelvis.

The prospective apex of the pouch must reach beyond the symphysis pubis to accomplish a tension-free ileoanal anastomosis. Selective division of mesenteric vessels to the apex of a proposed J-pouch will allow for more length (Figure 41-10).

Superficial incision on the anterior and posterior aspects of the small bowel mesentery along the course of the superior mesenteric artery, and mobilization of the small bowel mesentery up to and anterior to the duodenum, are two additional important lengthening maneuvers. The pouch is then pulled into the pelvis and the anastomosis performed between the apex of the pouch and the dentate line, approximating full thickness of the pouch wall to the internal sphincter and anal mucosa (Figure 41-11). A proximal defunctioning loop ileostomy is created. One or two suction drains are placed in the presacral space and brought out through the left lower quadrant of the abdomen away from the ileostomy site.

In the double-stapled technique, the anorectum is divided by the abdominal operator approximately 2 cm above the dentate line using a right-angle linear stapler (Figure 41-12). After the pouch is created, the anvil of the mid-sized circular stapler FIGURE 41-4. Lone Star™ retractor.

FIGURE 41-5. Mucosectomy. A A spinal needle is used to inject saline solution with epinephrine (1:200,000) into the submucosa from the dentate line to the levators. A circumferential incision through the mucosa is made at the dentate line. B A sleeve of mucosa is dissected free from the internal sphincter using sharp dissection.

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device is tied in to the apex of the ileal pouch. Before pro- ceeding with the anastomosis, integrity of the rectal staple line is tested using air insufflation. The stapler is placed transanally and the trocar advanced through the transverse staple line. The stapler is then closed as the abdominal surgeon ensures that no extraneous tissues are trapped within the stapling device.

Postoperative management is similar to that in patients who have had an ultra-low anterior resection or coloanal procedure protected by a loop ileostomy. Ileostomy output can be quite

FIGURE41-7. Ileal J-pouch. Intraoperative photograph showing the two limbs of the ileum properly oriented using stay sutures.

FIGURE41-8. Ileal J-pouch. Intraoperative photograph showing appli- cation of the linear stapler through the apical enterotomy. Note how the stay sutures are helpful in advancing the bowel over the stapler.

FIGURE41-9. Ileal J-pouch. Intraoperative photograph showing the completed J-pouch.

FIGURE41-6. Ileal J-pouch creation. A The limbs of the ileum are oriented using stay sutures. B The common wall of the two limbs is then divided using a linear cutting stapler placed through an apical antimesenteric enterotomy. C The J-reservoir is then placed within the rectal muscular sleeve and sutured to the dentate line. (Reprinted from Veidenheimer MC. Mucosal proctectomy, ileal J-reservoir, and ileoanal anastomosis. In: Braasch JW, Sedgwick CE, Veidenheimer MC, Ellis FH Jr, eds. Atlas of Abdominal Surgery. Philadelphia: WB Saunders; copyright 1991, with permission from Elsevier).

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high, because the stoma is more proximal than a traditional Brooke ileostomy. Patients should be encouraged to keep themselves well hydrated. In some instances, antidiarrheal medication is prescribed.

Patients are usually discharged after 7–10 days in the hos- pital and return 6–8 weeks later to have the temporary ileostomy closed. Before closure, however, the pouch is thor- oughly investigated. Digital rectal examination is used to assess anal sphincter tone and detect anastomotic strictures or defects. The pouch is examined endoscopically to ensure that the suture lines are healed, and a contrast study is performed to detect pouch leaks, fistulas, and sinus tracts. Only after confirmation that pouch abnormalities are not present is the ileostomy closed. Sphincter strengthening exercises should be encouraged in the period leading up to ileostomy closure, because they seem to improve functional results. In more than 90% of patients, the ileostomy can be closed through a peris- tomal incision. However, in the remainder, the midline abdominal incision must be reopened.

Performing an ileoanal anastomosis is safe, with reported mortality rates ranging from 0% to 1%.³⁵In distinct contrast to mortality, however, morbidity after IPAA remains consid- erable. Small bowel obstruction occurs in 20% of patients and results from adhesion formation to the large number of raw surfaces after colectomy and from kinking at the ileostomy FIGURE41-10. Ileal J-pouch. The peritoneum is scored to lengthen

the mesentery. Selective division of mesenteric arcades is used to produce additional length (Reprinted from Veidenheimer MC.

Mucosal proctectomy, ileal J-reservoir, and ileoanal anastomosis. In:

Braasch JW, Sedgwick CE, Veidenheimer MC, Ellis FH Jr, eds.

FIGURE41-11. Hand-sewn ileoanal anastomosis. A After the pouch is gently pulled through the anal canal by the perineal surgeon, four sutures incorporating full thickness of the pouch and a generous bite of the internal sphincter are placed at right angles to anchor the effer- ent limb within the anal canal. B The anastomosis is completed by placing sutures between each anchoring suture. C The mucosally intact anastomosis is completed.

FIGURE41-12. Double-stapled J-pouch anastomosis. A The anvil of a mid-sized circular stapler is tied into the apex of the J-pouch.

B The anorectum is divided with a stapler within the levator muscles about 1–2 cm above the dentate line. Adjacent tissue such as the bladder or vagina must be excluded from incorporation in the staple line. The integrity of the staple line should be tested with air insuf- flation through an anoscope. C The perineal operator advances the mid-sized circular stapler against the anorectal transaction site and advances the trocar through the transverse staple line. D The anvil mechanism is positioned onto the rod of the circular stapler. Before completing the anastomosis, the abdominal operator must prevent extraneous tissue from being trapped into the stapling device.

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site. Most of the obstructive episodes occur in the immediate period after either procedure. The most important factor pre- dictive of small bowel obstruction is rotating the ileostomy 180 degrees (as some authors promote to reduce fecal spillage into the defunctionalized pouch).³⁶Although an initial trial of nonoperative therapy is appropriate, surgical intervention may ultimately be required.

Although the incidence has steadily decreased with increasing surgical experience, pelvic sepsis still occurs in 5% of patients after IPAA. Pelvic sepsis may present in the immediate postoperative period or it may be delayed, mani- festing as abscess formation (usually presacral) or a perineal fistula. The symptoms suggestive of early pelvic sepsis are fever, anal pain, tenesmus, and discharge of pus or secondary hemorrhage through the anus. Diagnosis is confirmed using computed tomography or magnetic resonance imaging which demonstrates the presence of an abscess or of edematous tissues. Because patients who develop sepsis in the early postoperative period have a higher likelihood of subsequent pouch failure,³⁷ an aggressive therapeutic approach should be adopted in these patients. Although most patients respond to intravenous antibiotics within 24–36 hours, patients with ongoing sepsis and an organized abscess should undergo early operative endoanal or imaging-guided percutaneous drainage.

If drainage of the cavity is unsatisfactory, an attempt should be made to deroof the abscess and curette the cavity through the anus, creating a large communication between the abscess and the reservoir. Sometimes several local procedures are needed to eradicate sepsis. Rarely, an abdominal approach is needed.

The reported incidence of ileoanal anastomotic stricture has varied between 5% and 38%.^38–41This difference depends in part on the definition of stricture used by different authors. For some, a stricture is a narrowing of the anastomosis that requires at least two dilations^39,40whereas, for others, a stricture is narrowing associated with pouch-outlet obstruction and poor evacuation that requires repeated dilations. The etiology is usually anastomotic tension that also predisposes to infection from leakage. Full mobilization of the mesentery and avoidance of traction on the reservoir are key technical maneuvers to avoid stricture formation. Anchoring the pouch to surrounding tissues may prevent direct tension on the anastomosis itself. Avoidance of sepsis is paramount to a successful outcome. An apparent stricture may be noted when digital examination is performed for the first time after the operation. These asymptomatic, web- like strictures can be easily disrupted by gentle passage of the finger. More fibrotic strictures can usually be fractured digitally but occasionally the insertion of graded dilators under anesthe- sia is necessary. Operative management usually requires repeated dilatations yet reasonable function can be expected in more than 50% of patients.^39,40,42Rarely, a transanal approach involving excision of the stricture and pouch advancement distally is necessary.^43,44

Anastomotic separation is seen in approximately 10% of patients. If this complication is recognized during

preileostomy closure contrast studies or as a defect on digital examination, ileostomy closure should be delayed until complete clinical and radiographic evidence of healing. Local drainage procedures for an associated abscess or a direct repair of the separation are sometimes necessary.³⁵ This aggressive approach will almost always be successful, allowing ileostomy closure.

The reported incidence of pouch-vaginal fistula ranges from 3% to 16%.^45–49The patient complains of a vaginal discharge and clinical examination usually demonstrates the fistula. Occasionally, it is only detected by radiologic contrast enema (pouchogram). It is important to exclude a pouch- vaginal fistula by careful operative examination of the vagina as well as the anal canal before closing the defunctioning ileostomy. The fistula may present before ileostomy closure or after stoma closure.⁴⁶ The internal opening is usually located at the ileoanal anastomosis, but less often it may arise at the dentate line, perhaps as a form of cryptoglandular sepsis. Causative factors may include injury to the vagina or rectovaginal septum during the rectal dissection or anastomotic dehiscence with pelvic sepsis. The latter is probably the major predisposing factor because pelvic sepsis rates are significantly higher in patients with pouch-vaginal fistula than in those without.⁵⁰CD has been reported to be more common in patients with pouch-vaginal fistula, yet is difficult to prove in the majority of cases. Management depends on the severity of symptoms. When these are minimal and acceptable to the patient, no action or the placement of a seton may be all that is necessary.⁴⁷In those with a clinically significant degree of incontinence, a diverting ileostomy should be established if not already present. On defunctioning, sepsis is drained with or without placement of a seton suture and, once it has settled, repair is indicated. Simple defunctioning alone does not often lead to fistula closure.⁵¹Medical therapy is not indicated in managing these fistulas, although one recent series showed efficacy of infliximab.⁵² Surgical options are divided into abdominal and local procedures. The former includes abdominal revision with advancement of the ileoanal anastomosis, and the latter fistulectomy with or without sphincter repair, endoanal advancement flap repair, and endovaginal or transvaginal repair. The height of the ileoanal anastomosis is the essential feature that influences the choice of operative approach. Pouch-vaginal fistula from an anastomosis at or above the anorectal junction should be approached abdomi- nally with pouch dissection, repair of the vaginal defect, and creation of a new ileoanal anastomosis. Several authors have reported an approximately 80% success rate using this approach.^51,53,54A fistula arising from an anastomosis within the anal canal should not be treated with an abdominal procedure because there is not sufficient distal anal canal length to be clear of the fistula. A local procedure is necessary in such circumstances and most surgeons have used either an endoanal ileal advancement flap procedure45,46,48–50or a transvaginal technique.^42,50,55 Although both approaches result in fistula closure in 50%–60% of cases, the transvaginal repair

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may have an advantage over the endoanal technique because it allows a direct approach to the fistula without the possibil- ity of sphincter damage. Pouch-vaginal fistulas complicating CD are often difficult to treat, recurrence is common, and they frequently lead to pouch excision.^49–51

The most frequent long-term complication after IPAA for UC is a nonspecific inflammation of the ileal pouch commonly known as pouchitis.^35,56–58 The presence of extraintestinal manifestations of UC before colectomy, especially primary sclerosing cholangitis, has been associated with the development of pouchitis.^56,59 Backwash ileitis does not predict the ultimate development of pouchitis. High-level expression of the serologic factor perinuclear antineutrophil cytoplasmic antibody before colectomy predicts the development of chronic pouchitis after IPAA.⁶⁰ The etiology of this nonspecific inflammation is unclear but, as with the continent ileostomy, may be attributable to an overgrowth of anaerobic bacteria. Presenting symptoms include abdominal cramps, fever, pelvic pain, and sudden increase in stool frequency.

Treatment of pelvic reservoir pouchitis relies primarily on the use of antibiotics such as metronidazole and ciprofloxacin.^61,62 A mixture of probiotics can also be used in IPAA patients after resolution of the acute symptoms to prevent recurrence of pouchitis.⁶³Although these regimens are almost always successful, occasionally steroid enemas or 5-aminosalicylates will be necessary. Patients with chronic pouchitis should be suspected of having CD. Uncommonly, an ileostomy with or without pouch excision is required for severe refractory pouchitis.

The number of bowel movements after successful ileoanal pouch procedures averages six per 24 hours. It should be pointed out that most patients are not particularly concerned with how often they defecate, because most can postpone defecation to accommodate social and recreational activities.

Major incontinence is very unusual, although minor incontinence to mucus or stool, particularly at night, is observed in approximately 30% of patients. These patients are managed effectively with good perianal hygiene and the occasional use of a perineal pad. Although continence is clearly altered after pelvic pouch surgery, quality of life is extremely well preserved.^35,64 To obtain these results, however, approximately half of these patients regularly take a bulking agent or antidiarrheal medication to help regulate their bowels. Many patients also tend to eat less in the evening than at midday to minimize bowel movements when they are going out or while sleeping. Total failure, defined as removal of the pouch, occurs in only 5%–8% of cases and is usually caused by pelvic sepsis, undiagnosed CD, or an unacceptable functional outcome. Quality-of-life studies have disclosed that more than 95% of patients are satisfied with their pouch^35,65 and would not go back to an ileostomy.

Issues related to fertility, pregnancy, and the preferred method of delivery are of great importance in the female IPAA patient, many of whom are young and within their reproductive years. The ability of women desiring pregnancy to conceive after IPAA has been evaluated by several

investigators. Most reports are characterized by small num- bers of subjects attempting conception after surgery, and therefore do not permit any conclusions about fertility.^66–69 However, two larger studies have shown decreased postoperative fertility.^70–72 The severe decrease in postoperative fertility was attributed to probable tubal occlusion from adhe- sions, a phenomenon observed in another study.⁶⁷However, physician recommendations against conception and patient concerns about having children affected with UC could not be discounted.⁷⁰ A second report from the same group on a different patient cohort found normal fecundity before UC diagnosis and from UC diagnosis to colectomy. However, fecundity decreased 80% after IPAA.⁷¹Placement of an anti- adhesion membrane around the fallopian tubes and ovaries during surgery may be useful in an attempt to reduce the incidence of these complications.

The optimal method of delivery remains controversial.

Cesarean delivery decreases the risk of incontinence resulting from damage to the anal sphincters and yet is associated with complications inherent to abdominal surgery, including injury to the pelvic pouch and adhesion formation. Vaginal delivery may damage the pudendal nerve and the anal sphincter mechanism, but it reduces the problems associated with abdominal surgery and recovery is more rapid. Some short-term studies have shown the safety of vaginal delivery after IPAA.⁷³ However, vaginal delivery has been shown to cause occult sphincter damage⁷⁴and injury to the innervation of the pelvic floor in normal females.⁷⁵ These factors could lead to an increased risk of fecal incontinence with age, which would be particularly devastating in a patient with a pelvic pouch.

Controversies

In approximately 10% of colitis patients, there are inadequate diagnostic criteria to make a definite distinction between UC and CD, especially in the setting of fulminant colitis.^76,77 These patients are labeled as having IC. Several major clinical concerns remain regarding performance of IPAA for IC, including a higher rate of perineal complications, development of CD, and eventual pouch loss.^78,79Other investigators, however, have demonstrated acceptable outcomes of this procedure in IC.^80,81 Until the reasons underlying these discrepant data are uncovered, patients with IC should be counseled that undergoing IPAA may predispose them to a higher incidence of pouch-related complications. Although preoperative clinical factors that can predict those IC patients at risk for developing pouch complications or CD after IPAA have yet to be identified, a recent prospective study suggests that IC patients who express specific inflammatory bowel disease serologic markers before surgery have a significantly higher incidence of chronic pouchitis and CD after IPAA than IC patients who have a serologically negative profile.⁸²

Another debated issue is whether IPAA should be offered to elderly patients. Two reasons to avoid these procedures in older patients relate to the higher incidence of anal sphincter

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dysfunction with increasing age and the morbidity of reoperations in these potentially medically more compromised patients. However, operations for rectal cancer with anastomosis to the anal sphincter are regularly performed in patients in their seventh and eighth decades, and thus many surgeons contend that an IPAA should also be made available. Many groups have demonstrated that IPAA in the elderly patient is safe and feasible.^83,84It seems that chronologic age should not itself be used as an exclusion criterion. Pouch procedures are feasible in suitably motivated elderly individuals who under- stand the risks and problems of this procedure. Although bowel frequency remains constant in the first decade after the surgical procedure,^35,85,86it is unclear what will occur as the patient continues to age. Perhaps the use of a double-stapled technique with preservation of the anal transition zone might improve function over time, but this remains unproven.

Another controversy relates to the use of the IPAA in UC patients who have an established colorectal cancer. The presence of distant metastatic disease is generally a contraindica- tion to IPAA. These unfortunate patients should be managed with segmental colectomy or abdominal colectomy with IRA to facilitate early discharge and allow them to spend the rest of their lives relatively free of complications. Patients with middle and low rectal tumors, in accordance with basic prin- ciples of cancer surgery, may not be eligible for this procedure. Radiation therapy, if indicated, should be performed preoperatively; a pelvic pouch should not be subjected to radiation because of a high incidence of pouch loss. UC patients with cecal cancers represent another unique subgroup of patients. The sacrifice of a long segment of adjacent distal ileum with its mesenteric vessels may limit positioning of the reservoir into the pelvis. If a tension-free anastomosis cannot be ensured, a Brooke ileostomy may be necessary. Studies examining the use of the ileoanal pouch in patients with locally invasive cancers of the colon and upper rectum have been conflicting. In one series,⁸⁷UC patients with a carcinoma had postoperative complications and functional results identical to UC patients without cancer. Metastatic disease developed in a small number of patients. In contrast, another study revealed that almost 20% of UC patients with cancer who had an IPAA died of metastatic disease.⁸⁸Because both of these patients had T3 cancers at surgery, it is unclear that their course was adversely influenced by performing IPAA.

This conservative management approach is also encouraged by surgeons at the Lahey Clinic,⁸⁹where UC patients with a T3 cancer initially undergo an abdominal colectomy with ileostomy. An observation period of at least 12 months is recommended to ensure that no recurrent disease develops.

Another reason to postpone IPAA in these patients is to allow adjuvant chemoradiation therapy to proceed unhindered without any added morbidity from a pouch–anal anastomosis and a relatively proximal ileostomy.

A number of innovations of the IPAA operation spurred by a desire to decrease complications and improve function have led to a series of technical controversies. Some authors

believe that the entire rectal mucosa does not need to be removed. They favor leaving 1–2 cm of distal mucosa behind, transecting the rectum just above the puborectalis muscle and stapling the pouch to the rectal remnant. The potential advan- tages of the double-stapled approach include technical ease because it avoids a mucosectomy and the perineal phase of the operation, less tension on the anastomotic line, and improved functional results because sphincter injury is minimized and the anal transition zone with its abundant supply of sensory nerve endings is preserved.⁹⁰However, surgeons who oppose this operative approach contend that residual diseased mucosa is at risk of malignancy. There have been nine reports of cancer developing after IPAA, eight in patients who under- went the procedure for dysplasia or colorectal cancer.^91–98 Two of these cases occurred in the preserved mucosa within the anal transition zone.^94,98Although the origin of adenocar- cinoma in these cases is a subject of debate, it is reasonably clear that a double-stapled technique should not be performed in the UC patient who is a high cancer risk (i.e., dysplasia or established cancer) at the time of IPAA. In addition, the potential for continuing colitis in this residual mucosa is another concern. Rauh and coworkers⁹⁹have described a

“short-strip pouchitis” that manifests as inflammation at the pouch anal anastomosis thought secondary to residual colitic mucosa. In an effort to resolve these issues, three prospective, randomized trials have demonstrated no significant differ- ences in perioperative complications or functional results in those patients where a mucosectomy was done versus those patients where the distal rectal mucosa was preserved.^100–102It is important that the surgeon performing an IPAA be familiar with both techniques in the event of failure or inability to use the stapler or when a hand-sewn anastomosis is contemplated but where anastomotic tension is excessive. It must be stressed that if a stapled technique is used, care should be taken to create an ileal pouch to anal anastomosis and not an ileal-to-rectum anastomosis.

Another technical controversial issue is the shape and size of the reservoir. Although the initial ileal reservoir created by Parks in the late 1970s was a triple-loop S pouch,³¹other pouch configurations have been described in an attempt to reduce pouch complications and improve functional outcome (Figure 41-13). Three other configurations that have been described are the double-loop J-pouch, the quadruple-loop W-pouch, and the lateral isoperistaltic H-pouch.^103–105 S-pouches were initially plagued with evacuation problems associated with a long (5-cm or more) exit conduit, frequently requiring pouch catheterization.³¹With shortening of the exit conduit to 2 cm or less, mandatory catheterization has been substantially reduced.¹⁰⁶The long outlet tract formed in the H-pouch was also associated with pouch distention, stasis, and pouchitis.¹⁰⁷The W-pouch has been favored by some surgeons¹⁰⁵because its theoretically greater capacity may lead to fewer daily bowel movements. However, two randomized trials comparing the W- and J-pouch did not confirm this hypothesis.^108,109In one study,¹⁰⁸the median number of stools