Surgery for ulcerative colitis Patricia L Roberts CLINICAL VIGNETTE Challenging Case A 35-year-old male is undergoing an ileoanal pouch procedure for ulcerative colitis. Following transection of the ileum flush with the cecum, the surgeon notes that it will be difficult for the pouch to reach the anus. Challenging Case Management Difficulties with the ileoanal pouch reaching the anus occur for two main reasons: failure to mobilize the small bowel, or patient-related factors such as obesity or a long narrow anal canal. Difficulty with reach is more common if a mucosectomy is performed rather than a double-stapled anastomosis. An S pouch may reach the anus eas- ier than a J pouch. If the main reason for the pouch not reaching is patient obesity and a thickened mesentery, an initial total abdomi- nal colectomy, ileostomy, and Hartmann closure of the rectum may be performed. Following weight reduction, an ileoanal pouch pro- cedure can be performed. A series of technical maneuvers includ- ing mobilization of the small bowel up to the duodenum, scoring the peritoneum over the superior mesenteric artery, and the crea- tion of mesenteric windows can facilitate pouch reach. If, despite these maneuvers, the pouch does not reach, the pouch can be left in the pelvis, a loop ileostomy created, and, after a period of sev- eral months, the pouch can then be joined to the anus. Additional details of these technical maneuvers are described in the text. INTRODUCTION Ulcerative colitis is an inflammatory condition involving the colon and rectum. The incidence in the United States is 8.8 cases per 100,000 person years.(1) Thus, in this country, there are approxi- mately 26,000 new cases of ulcerative colitis diagnosed annually and 730,000 people with ulcerative colitis.(1, 2) Although many patients are treated effectively with medical therapy, approximately 23–45% of patients require colectomy. The risk of requiring colectomy is higher in patients with pancolitis than patients with left sided disease.(3, 4) This chapter concentrates on the indications for surgery, the opera- tive options, and the outcome of surgery for ulcerative colitis. INDICATIONS FOR SURGERY Surgery for ulcerative colitis is divided into two categories: urgent or emergency surgery, and elective surgery. Acute Colitis Urgent or emergent surgery is indicated for patients with acute unresolving colitis or life-threatening complications associated with colitis, including fulminant or toxic colitis, hemorrhage, colonic perforation, or obstruction. Severe acute colitis may occur in 5 to 15% of patients with ulcerative colitis. The classification system of Truelove and Witts is most commonly used and iden- tifies clinical parameters by which colitis is categorized as mild, moderate, and severe (5, 6) (Table 31.1). For patients with acute colitis, stool studies should be done to rule out superinfection with clostridium difficile, bacteria, or ova and other parasites. A flexible sigmoidoscopy without bowel preparation with mini- mal insufflation of air is helpful to biopsy the rectum to exclude cytomegalovirus (CMV). In one series of patients with steroid resistant acute ulcerative colitis, the incidence of associated cytomegalovirus was 36%.(7) The majority of patients diagnosed with CMV responded to administration of foscarnet or ganciclo- vir. After exclusion of an infectious etiology, patients are treated with intravenous steroids for 5–7 days. If there is no clinical response, cyclosporine or infliximab is considered. Patients who are reluctant to use cyclosporine or infliximab, or patients who do not respond, should undergo colectomy. While administra- tion of steroids is associated with an increase in postoperative complications, immunosuppressives do not appear to increase the incidence of postoperative complications.(8) A small subset of patients may develop fulminant colitis. The classification system of Truelove and Witts does not define ful- minant colitis, but Hanauer (9) has modified the classification system to define patients with fulminant colitis. In the classifica- tion system of Truelove and Witts, severe disease is defined as >6 stools per day, a temperature >37.5 degree Celsius, a pulse of >90 beats per minute, hemoglobin <75% of normal, an erythrocyte sedimentation rate of >30 mm/hr, the presence of air, edema- tous wall, or thumbprinting on x-ray and abdominal tenderness. Fulminant colitis is defined as >10 stools per day, continuous Table 31.1 Truelove and Witts Criteria for Evaluating the Severity of Ulcerative Colitis. Variable Mild disease Severe Disease Fulminant Disease Stools (Number/day) <4 >6 >10 Blood in stool Intermittent Frequent Continuous Temperature (ºC) Normal >37.5 >37.5 Pulse (beats/min) Normal >90 >90 Hemoglobin Normal <75% of normal value Transfusion required Erythrocyte sedimentation rate <30 >30 >30 Colonic features on x-ray Air, edematous wall, thumb- printing Dilatation Clinical Signs Abdominal tenderness Abdominal distention and tenderness Source: After Truelove and Witts. BMJ 1955; 2: 1041–45. Reprinted with permission from Clinics in Colon and Rectal Surgery. Volume 17, Number 1, 2004, page 8. surgery for ulcerative colitis bloody bowel movements, a temperature of >37.5 degree Celsius, a pulse of >90 beats per minute, transfusion requirement, an erythrocyte sedimentation rate of >30 mm/hr, dilatation of the colon and abdominal distention and tenderness. The term toxic megacolon has been used when the colonic distention of the transverse colon exceeds 6 cm, but relying on this finding to diagnose toxic colitis is not necessary, as some patients will have “toxicity” in the absence of colonic distention. Prompt treatment and diagnosis of toxic colitis is needed to avoid progression to perforation. Approximately 20–30% of patients with toxic colitis require emergency surgery. Perforation in the setting of toxic or fulminant colitis substan- tially increases the mortality rate. Patients whose condition wors- ens or who fail to make substantial improvement after a period of 48–96 hours should be considered for surgery to avoid this complication.(10) Massive hemorrhage in patients with ulcera- tive colitis is uncommon, accounting for <10% of emergency colectomies performed for ulcerative colitis, and raises the pos- sibility of Crohn’s disease.(11) Emergency vs. Elective Procedures The surgical options for patients who require emergency surgery for acute colitis are aimed at restoring the patient back to a gen- eral state of health and preserving reconstructive options for sub- sequent surgery. The most common operation performed is total abdominal colectomy with ileostomy, and either Hartmann closure of the rectum or creation of a mucous fistula. Preoperative coun- seling and marking by an enterostomal therapist is optimal. This procedure removes the majority of the diseased bowel, avoids an intestinal anastomosis in an ill patient, and preserves the option for an ileoanal pouch procedure in the future. The colon is transected at the level of the sacral promontory avoiding the need for a pel- vic dissection. If the severity of disease as demonstrated by severe ulcerations and friability of the bowel precludes safe closure of the stump, a variety of other options may be employed. The stump may be exteriorized as a mucous fistula. This requires a longer segment of bowel and is associated with bleeding and mucus from an addi- tional stoma. Alternatively, it has been suggested that extrafascial placement compared with intraperitoneal closure of the Hartmann stump may be associated with fewer infectious complications.(12) Transanal drainage has also been suggested to decrease the inci- dence of infectious complications associated with the Hartmann stump.(13) Pelvic dissection and creation of a relatively short Hartmann pouch should be avoided as this makes dissection and subsequent ileoanal pouch creation more difficult. A laparoscopic or open approach may be used for performance of total abdominal colectomy and ileostomy in patients with acute colitis.(14) Elective Procedures The most common indication for elective surgery is intractabil- ity to medical management defined as failure of medical ther- apy. Intractability includes insufficient symptom control despite intensive medical therapy. Due to loss of time from work, school or activities in general, the patient may not have an acceptable quality of life. The risks of medical therapy may be substantial including potential complications from long-term steroid ther- apy or complications of the side effects of medical therapy. In children, growth retardation can result from poorly controlled ulcerative colitis and is an indication for colectomy. Patients with longstanding ulcerative colitis are at an increased risk for the development of colorectal cancer. The exact risk is dif- ficult to determine since many series have lacked longitudinal fol- low-up or have included patients seen at tertiary referral facilities. Surveillance colonoscopy with biopsy has been recommended in patients with left-sided or pan colitis (defined as microscopic dis- ease proximal to the splenic flexure) after 8 years of disease symp- toms. At least 33 biopsies are necessary to obtain a sensitivity of 90%, and four quadrant biopsies are recommended every 10 cm along the colon and in any abnormal appearing area. A recent meta-analysis has estimated the risk of the development of color- ectal cancer in patients with long-standing ulcerative colitis to be 2% at 10 years, 8% at 20 years, and 18% after 30 years of disease. (15) There is no evidence to show that surveillance prolongs sur- vival in such patients, although patients who develop cancers in a surveillance program tend to have earlier stage cancers.(16, 17) Proctocolectomy is indicated for patients with carcinoma, nonadenoma-like dysplasia associated lesion or mass (DALM), and patients with high grade dysplasia.(10) The presence of high grade dysplasia should ideally be confirmed by two independent expert pathologists. For those patients who underwent immedi- ate colectomy, cancer was detected in 42% of patients with high- grade dysplasia and 19% with low-grade dysplasia.(18) Although patients with low-grade dysplasia should be offered colectomy, the natural history of low-grade dysplasia is not as well defined. The interobserver variation between pathologists confounds the recommendations about low-grade dysplasia. Studies are con- flicting, with one study of a surveillance program showing that in patients with low-grade dysplasia the 5-year predictive value for the development of cancer or high-grade dysplasia was 54%.(19) Another study showed that only 18% of patients with low-grade dysplasia progressed to high-grade dysplasia or a dysplasia associ- ated lesion/mass.(20) Strictures may also develop in 10–25% of patients with ulcera- tive colitis, and while the majority are benign up to 25% are malignant. Strictures which cause obstruction, develop in long- standing disease, and are found proximal to the splenic flexure, are most likely to be malignant and are another indication for colectomy.(21) PROCTOCOLECTOMY WITH BROOKE ILEOSTOMY Proctocolectomy with ileostomy has previously been the “gold stand- ard” operation for ulcerative colitis against which other operations have been compared. This operation essentially cures the disease and restores patients back to health and to a relatively normal life. It is a one-stage procedure which removes the diseased mucosa and has fewer potential complications than the ileoanal pouch proce- dure. The main drawback is the presence of a permanent ileostomy, something which most patients wish to avoid. Indications This operation is indicated in those patients who require surgery for ulcerative colitis, but are not candidates for the ileoanal pouch procedure. These patients include those who are elderly, have fecal incontinence or an inadequate sphincter, patients with low rectal improved outcomes in colon and rectal surgery cancers in association with ulcerative colitis who require proc- tectomy and possibly pelvic radiation, and those patients who opt for a permanent Brook ileostomy for personal preferences. Furthermore, patients who develop pouch failure and require pouch excision essentially have a completion proctectomy. Operative Technique The preoperative period includes patient education about the procedure and the effects of an ileostomy. Preoperative consulta- tion with an enterostomal nurse is helpful. The stoma site selected should be a flat area, away from bony prominences and creases. Proctocolectomy is performed through either an open or laparoscopic approach. Following mechanical bowel preparation the day before surgery, the patient is administered preoperative intravenous antibiotics and positioned in lithotomy position. After performance of a standard colectomy, pelvic dissection is performed. The retrorectal space is entered sharply and the pelvic dissection is undertaken with careful attention to the ureters and identification of the hypogastric nerves. The dissection is carried out to the pelvic floor. A pack is placed posterior to the rectum and the perineal dissection is performed. An intersphincteric dis- section allows for a smaller wound, a relatively bloodless dissec- tion, and presumably better healing. The perineal dissection is carried out to the level of the pelvic dissection. After excision of the colon and rectum, the wound is closed in layers and a Brooke ileostomy constructed. A foley catheter is left for several days in addition to a closed suction drain. Outcome Proctocolectomy with ileostomy is associated with fewer poten- tial complications than ileoanal pouch procedure. In one series, the long-term complication rate in patients undergoing procto- colectomy with ileostomy compared to ileoanal pouch procedure was 26% vs. 52%.(22) The most common long-term complica- tions include stoma related complications. From a physiologic standpoint, patients with an ileostomy are more prone to dehy- dration, electrolyte abnormalities, and kidney stone formation. Patients should be counseled to be aware of signs and symptoms of dehydration. Although problems have decreased substantially with modern pouching systems, preoperative stoma marking, and the expertise of enterostomal nurses, patients may experience peristomal skin irritation, parastomal hernia formation, stomal retraction, fistula, and stomal stenosis. In the long-term, up to one third of patients require operative revision.(23) Slow or delayed perineal wound healing occurs in up to 25% of patients after proctocolectomy with ileostomy. An intersphinc- teric dissection may decrease the size of the perineal wound and improve wound-related complications.(24) If infection or delayed wound healing occurs, local wound care with examination under anesthesia, debridement, and curettage is performed. The vacuum assisted closure device has been helpful to treat persistent perineal wounds.(25) In some cases, muscle transposition, such as gracilis muscle transposition is necessary to heal persistent wounds. As with any operation involving a pelvic dissection, sexual and uri- nary dysfunction may occur from injury to the sympathetic and para- sympathetic nerves. The incidence of sexual dysfunction is felt to be less than that occurring in those patients who undergo proctectomy for malignant disease. However, this may reflect the younger age of patients undergoing proctocolectomy for ulcerative colitis. Impotence occurs in 1–2% of patients and retrograde ejaculation may occur in up to 5% of patients.(26) Dysparuenia and increase in vaginal dis- charge occur in up to 30% of women from scarring and change in the in-axis of the vagina.(27) Women must also be counseled about the potential for infertility because of scarring pelvic adhesions. Despite the fact that patients have undergone a major surgical procedure, the quality of life remains high after proctocolectomy with ileostomy. Overall 90–93% of patients are satisfied with their quality of life.(28, 29) Despite the satisfaction, a number of difficul- ties exist, including restriction of social and recreational activities in up to 25%, and dietary restrictions in almost 30%. PROCTOCOLECTOMY WITH CONTINENT ILEOSTOMY Another option for patients who require surgery for ulcerative colitis is a continent ileostomy, introduced by Nils Kock in 1969. (30) Despite initial enthusiasm, this operation is infrequently performed today because of the appreciable number of complica- tions associated with the procedure, in addition to the fact that it has been largely supplanted by the ileal pouch anal anastomosis. Indications for a continent ileostomy include those patients who have undergone prior proctocolectomy with ileostomy and desire a continent stoma, selected patients who have a failed ileoanal pouch procedure, patients with ulcerative colitis and rectal cancer who could not undergo an ileal pouch anal anastomosis (IPAA) and patients with poor sphincter tone in whom the functional results would be quite poor. Advanced age and obesity are relative contraindications to per- formance of the procedure. As with the ileoanal pouch procedure, Crohn’s disease is a general contraindication to the procedure because of the risk of recurrent disease which could necessitate resection of the continent ileostomy. Operative Technique The operative technique involves initial performance of a proctocolec- tomy. The continent ileostomy is then constructed using the terminal 40–60 cm of the ileum. A three limb pouch with an intussuscepted nipple valve is used (Figure 31.1). The valve is created by intussuscept- ing the efferent loop. After being tested for integrity and continence, the exit conduit is brought through the abdominal wall. The site of continent ileostomy is generally determined preoperatively with an enterostomal therapist and is lower in the abdomen than a standard ileostomy. Catheter drainage is maintained for approximately 4 weeks to allow complete healing of the pouch.(31, 32) Guidelines for catheter management have been outlined by Beck.(33) A number of technical modifications have been made over the years to prevent nipple valve complications. Mesh was initially used to stabilize the valve, but the technique was abandoned because of a high incidence (42.5%) of fistula formation.(31) A recently described modification to avoid slippage of the nipple valve is the “T-pouch” in which a portion of the ileum is folded into the side of the pouch.(33, 34) Outcome In a large series of patients undergoing continent ileostomy with a median follow-up of 11 years, 16.6% of patients required Kock surgery for ulcerative colitis Figure 31.1 Continent ileostomy (A) Three limbs of small bowel are measured and the bowel wall is sutured together. (B) After opening the bowel along the dotted lines in (A), the edges are sewn together to form a two-layered closure. (C) A valve is created intussuscepting the efferent limb into the pouch and fixing it in place with a linear noncutting stapler. (Inset: staples in place on valve.) (D) The valve is attached to the pouch side-wall 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 (Reprinted with permission). (A) (B) (C) (D) (E) improved outcomes in colon and rectal surgery pouch excision.(31) The number of complications associated with the procedure was high with an average of 3.7 (range 1–28) complications per patient. Some of the most significant complications are associated with nipple valve slippage which occurs because of the tendency of the intussuscepted segment to slide and evert on the mesenteric aspect. Manifestations of nipple valve slippage include difficult catheteriza- tion, incontinence, and obstructive symptoms from obstruction of the outflow tract. The incidence of nipple valve slippage is approxi- mately 30%. A variety of technical modifications have been devised to reduce the incidence of this complication. Use of prosthetic mate- rials to wrap the valve reduces the incidence of nipple valve slippage but is associated with abscess and fistula formation.(35) The T-pouch modification (34) has been advocated to avoid this complication, but there is currently no controlled data available. Pouchitis is a well recognized complication of the Kock pouch occurring in up to 25% of patients. It is manifested by increased bowel frequency, often associated with blood and mucus and at times, incontinence. The etiology of pouchitis is unknown, but the majority of patients are treated effectively with antibiotics and continuous pouch drainage. Other complications associated with the procedure include the development of fistula, parastomal hernia, and small bowel obstruction. Long-term results of patients with continent ilesotomies reveal a cumulative success rate of 71% at 29 years in 96 patients followed from 1972 to 2000.(36) The success rate with continent ileostomy is appreciably less than with the ileoanal pouch procedure. TOTAL ABDOMINAL COLECTOMY WITH ILEORECTAL ANASTOMOSIS Although the majority of patients with ulcerative colitis have rectal involvement, a small number of patients with rectal sparing may be treated with total abdominal colectomy and ileorectal anasto- mosis. Such patients may subsequently require rectal excision for diarrhea and poor functional results, ongoing proctitis, and malig- nant transformation. Surveillance for the development of dysplasia is recommended. Recent series have shown an average number of bowel movements of 3–6/day after the procedure with a failure rate of 11–57% (37, 38, 39). The incidence of developing cancer with long-term follow-up ranges from 0–6% (40, 41, 42). RESTORATIVE PROCTOCOLECTOMY WITH ILEOANAL POUCH Since its introduction in 1978, the ileoanal pouch procedure has become the procedure of choice for patients who require surgery for ulcerative colitis and familial adenomatous polyposis. Over the years, the operation has undergone a series of technical modi- fications and it can be performed with essentially no mortality and good long-term outcomes. The procedure avoids the need for a permanent stoma and removes the diseased bowel. Indications The most common indication for the ileoanal pouch procedure is failure of medical therapy for ulcerative colitis or development of complications from medical therapy which outweigh the benefit. Additional indications include the development of dysplasia and certain extraintestinal manifestations. Colon cancer is not a con- traindication to the procedure, but performance of an ileoanal pouch must not compromise the oncologic resection. IPAA is usually not advisable in a low- or mid-rectal cancer because of the need for chemoradiation therapy and the potential effects on the pouch and the anal sphincter. Although the majority of patients who undergo pouch surgery are young, age is not a contraindica- tion to the performance of the procedure. We advise patients on a case by case basis over the age of 65. Nocturnal leakage and incon- tinence is more common in older patients who undergo pouch surgery and preoperative assessment should include assessment of anal sphincter function and extensive discussion about the potential functional outcome. Operative Technique Preoperatively, the risks and benefits of the procedure are dis- cussed with the patient, and consultation with an enterostomal therapist is beneficial. An appropriate site for the intended stoma is marked in the right lower quadrant. The procedure is perform- ance after mechanical and antibiotic bowel preparation. Although the procedure may be performed with an open or laparoscopic approach, pouch surgery is increasingly being performed by a laparoscopic approach. Retrospective case-matched comparative studies have shown a longer operative time (median 330 min vs. 230 min), but a quicker return of bowel function (2 days vs. 4 days) and a shorter hospital stay (7 days vs. 8 days) with laparo- scopic pouch procedures (43). A recent meta- analysis of 10 stud- ies with 329 patients confirmed that despite a longer operative time, patients had a lower blood loss, shorter hospital stay, and smoother recovery compared to open surgery.(44) In a review of 100 laparoscopic and 189 open ileoanal pouch procedures for ulcerative colitis, patients reported excellent body image and quality of life scores regardless of open or laparoscopic approach. (45) In the past 5 years, the majority of the ileoanal pouch proce- dures have been performed at our institution with a laparoscopic hand-assisted approach. The technical details of the procedure are outlined in videos (CineMed-American College of Surgeons). One of the critical maneuvers during the performance of ile- oanal pouch surgery is the creation of a tension-free anastomosis between the pouch and the anus. Undue tension on the anasto- mosis leads to stricture formation, anastomotic leakage, potential pelvic sepsis, and poor function. To perform a tension-free anas- tomosis, the apex of the pouch should reach the inferior border of the symphysis pubis. Assessment of potential pouch reach to the anus is performed before pouch creation. In obese patients, it may be necessary to perform an initial total abdominal colec- tomy, ileostomy and Hartman closure of the rectum in anticipa- tion of significant weight reduction and then pouch creation.(46) An S-pouch may afford an additional 2 cm of length compared to a J-pouch but it is more difficult to construct and has potential efferent limb problems.(47) A tension-free anastomosis is more difficult to achieve in male patients with a narrow pelvis, patients with a long anal canal, obese patients, and patients who undergo mucosectomy with handsewn anastomosis. To achieve adequate length on the mesentery, a series of technical maneuvers is performed, including mobilization of the posterior attachment surgery for ulcerative colitis of the small bowel mesentery, exposing the inferior portion of the head of the pancreas, and scoring the peritoneum of the small bowel mesentery serially on the anterior and posterior surfaces. (48) Each of these relaxing incisions confers an additional 1 cm of distal reach. At least two relaxing incisions are made along the course of the superior mesenteric artery. If additional length is required, the mesentery of the small bowel is transilluminated to delineate the loop formed by the ileocolic artery and the terminal ileal branch of the superior mesenteric artery. Traction is placed on the small bowel by grasping the intended apex of the pouch, and vessels between the primary and secondary arcades that are under tension are identified and ligated. This maneuver adds 2–5 cm of additional length. The terminal branches of the superior mesenteric artery of the ileocolic artery can be divided for addi- tional length. These vessels are clamped for 10–15 minutes before ligation to confirm adequate vascularity of the ileum before divi- sion. In selected cases, interposition vein grafts have been used to obtain adequate mesenteric length.(49) If there is inadequate length despite these maneuvers, the pouch may be left in the pel- vis, and not anastomosed to the anal canal with plans to return at a subsequent date for anastomosis. The weight of the pouch and the dependent portion of it with the aid of gravity may facilitate reach to the anus at a later date. Outcome The mortality after ileoanal pouch surgery is <1%. The major- ity of the patients undergoing the procedure are young and oth- erwise in good health, with the exception of ulcerative colitis or familial adenomatous polyposis. Despite refinements in surgi- cal technique, the operation is associated with an appreciable number of complications. A recent meta-analysis with a review of 5,215 patients who underwent ileoanal pouch surgery between 1988 through 2000 revealed a preoperative diagnosis of ulcerative colitis in 87.5%, indeterminate colitis in 2%, Crohn’s disease in 0.8%, familial ade- nomatous polyposis in 8.9%, and other diagnoses in 0.7%.(50) A diverting ileostomy was performed in 81.6%. FUNCTIONAL RESULTS (BOWEL, URINARY, GYNECOLOGIC AND SEXUAL FUNCTION) At a median follow-up of 37.2 mos after ileoanal pouch surgery and ileostomy reversal, the mean defecation frequency was 5.2 during the day with a mean night-time frequency of 1.0.(50) Mild fecal incontinence during the day occurred in 17%, while 3.7% had severe fecal incontinence during the day and 7.3% had urge incontinence. Bowel function deteriorates with advancing age. (51) Prospective evaluation of long-term function reveals that especially 12 years or more after surgery, major and minor incon- tinence are worse. Twelve years following surgery, 27% of patients vs. 9% (<12 years) had major daytime incontinence and 33% vs. 10% reported more major night time incontinence. Furthermore, minor incontinence was seen in 48% of patients after 12 years vs. 16% of patients followed for under 12 years.(51) The reported incidence of sexual dysfunction in a meta-analysis of 21 studies including 5,112 patients was 3.6%.(50) The authors point out the risk of underestimating complications due to a posi- tive publication bias, and thus studies with negative results may be less likely to be submitted and published. Indeed, a more recent review has further quantified the impact of the ileoanal pouch procedure on sexual and gynecologic function in women. A sys- tematic review of 22 in 1,852 women who underwent restorative proctocolectomy from 1980 to 2005 revealed a much more sig- nificant impact on function.(52) The incidence of infertility was 12% before restorative proctocolectomy and 26% after (n = 945 women, 7 studies). Sexual dysfunction occurred in 8% preopera- tively and 25% postoperatively (n = 419 women, 7 studies). More Cesarean sections were performed after restorative proctocolec- tomy, although no significant differences in pouch function and no significant perineal trauma was seen after vaginal delivery, thus suggesting that the mode of delivery should be based on obstet- ric considerations. An increase in bowel actions was noted during the third trimester but bowel activity returned to normal within 6 months of delivery. Peritoneal inclusion cysts which are associated with pelvic sepsis and adhesions are an additional underreported consequence of the ileoanal pouch procedure.(53, 54) COMPLICATIONS Despite refinements in surgical technique, restorative proctocolec- tomy is associated with an appreciable number of complications including pelvic sepsis, fistulas, strictures, fecal incontinence, pouch failure, and sexual dysfunction. A recent meta-analysis on pooled data of observational studies has been performed on 43 studies com- prising 9,317 patients detailing the results and complications.(50) Small Bowel Obstruction Small bowel obstruction is a common complication after restora- tive proctocolectomy ranging from 15–44% of patients, with approximately half of patients requiring operation for treatment of obstruction.(55) Small bowel obstruction occurs more commonly after restorative proctocolectomy than after Brook ileostomy, pre- sumably because of the cumulative increase in obstruction after multiple procedures. Patients who develop early postoperative small bowel obstruction are more likely to resolve with conserva- tive measures than those patients diagnosed in later follow-up.(56) In a series of 1,178 patients who underwent IPAA, the cumulative risk of small bowel obstruction was 9% at 30 days, 18% at 1 year, 27% at 5 years and 31% at 10 years.(57) The most common site of adhesions were pelvic adhesions (32%) and adhesions at the ileos- tomy closure site (21%). Recent strategies to decrease the risk of adhesions have focused on the use of a bioresorbable membrane which has reduced the incidence, extent, and severity of adhesions (58), as well as the use of laparoscopic surgery (which results in less adhesions). Postoperative Hemorrhage Intraabdominal hemorrhage may occur from failure to secure the vascular pedicles and from pelvic bleeding, in addition to bleed- ing of the pouch suture or staple line. Pouch ischemia may also be associated with bleeding. Pouch bleeding noted intraoperatively is best treated by eversion of the pouch to expose the mucosa and cauterization or suture ligation as needed. Postoperative bleeding may require examination under anesthesia and/or pouch endos- copy with suture or endoscopic clipping of the bleeding point. Bleeding, especially 5–7 days after operation, may be associated improved outcomes in colon and rectal surgery with anastomotic dehiscence. In a series of 1,005 patients, pouch bleeding occurred in 38 patients (3.8%) and was treated with local irrigation with saline and adrenaline in 30 patients and transanal suture ligation in 8.(59) Pelvic sepsis Pelvic sepsis is defined as pelvic abscess, anastomotic leakage or dehiscence, or any pelvic or perineal infection. Some series distin- guish between pelvic sepsis and anastomotic leak; pelvic sepsis gen- erally results from a defect in the ileoanal anastomosis, anastomotic leak, or defect of the other staple or suture lines. A meta-analysis noted the incidence of pelvic sepsis to be 9.8%.(50) Manifestations of pelvic sepsis include fever, leukocytosis, perineal pain, purulent drain output, and prolonged ileus. As pelvic sepsis is a significant cause of pouch failure and since those patients with sepsis are more likely to have compromise of pouch function, any patient suspected of having pelvic sepsis, should be evaluated and treated expeditiously. CT scan confirms the diagnosis of pelvic sepsis, and contrast in the pouch (either by instilling rectal contrast or contrast through the efferent limb of the ileostomy) is useful in assessing the integrity of the anastomosis. Alternatively, a pouchogram and examination under anesthesia may be necessary. Intraabdominal or pelvic abscess requires percutaneous or operative drainage in addition to broad spectrum antibiotics (Figure 31.2). For patients with leakage from the anastomotic suture or staple line the abscess can be drained into the pouch. This potentially avoids the develop- ment of a complex fistula. Untreated pelvic sepsis results in fibrosis, a stiff, non-compliant reservoir, and a higher incidence of ultimate pouch failure.(60) Anastomotic leak or dehiscence Anastomotic leak after the ileoanal pouch procedure occurs between 5–18% of patients. In a recent meta-analysis, the incidence of anastomotic leakage from either the pouch-anal anastomosis or the pouch itself was 7.1%.(61) The incidence of anastomotic leakage was more common in patients who did not have a stoma at the time of pouch surgery. The presence of a stoma may help to ameliorate the clinical manifestations of a leak. A leak may occur at the pouch anal anastomosis or along any (A) (B) Figure 31.2 Retrograde pouch study shows a presacral collection (A) confirmed on CT scan (B). Collections arising from the anastomosis are preferably drained into the pouch to avoid a complex fistula. Figure 31.3 Asymptomatic anastomotic sinus in patient before ileostomy closure often requires no further treatment. Delay in ileostomy closure and repeat pouch study generally shows healing. surgery for ulcerative colitis of the staple or suture lines including the top of the J-pouch, the ileoanal anastomosis, or the pouch itself. Manifestations of a leak include the development of an abscess, fistula, or symptoms of pelvic pain, diarrhea, and fever. Risk factors associated with leak include tension on the anastomosis and ischemia resulting from tension on the anastomosis. One study suggested a lower inci- dence of pelvic sepsis associated with a double-stapled anastomo- sis compared with a mucosectomy and hand sewn anastomosis. (62) Management of anastomotic leak is individualized; patients who have an asymptomatic sinus before ileostomy closure with- out associated sepsis can be treated by delay in ileostomy closure and in most cases, ultimate healing of the tract.(Figure 31.3) Patients with peritonitis who have undergone restorative proc- tocolectomy without diverting ileostomy require diversion and drainage. Leaks from the tip of the J pouch are challenging both to diagnose and treat and developed in 14 out of 1,309 patients; all required surgical repair and none healed with conservative treatment (63) (Figure 31.4). With expertise and individual- ized management, pouch salvage can be was achieved in 88% of patients who developed anastomotic leak.(64) Stricture at the ileal pouch anal anastomosis Strictures at the ileal pouch anal anastomosis occur in approxi- mately 10% of patients, and are more common after mucosec- tomy and handsewn anastomosis than after double-stapled anastomosis.(65, 66) Tension on the anastomosis and ischemia are associated with stricture formation. A lumen which admits the DIP joint of the index finger is generally satisfactory for good bowel function. Soft strictures are treated with gentle finger dila- tion or with balloon dilators. Long fibrotic strictures are more challenging to treat and pouch advancement and neoileoanal anastomosis may be necessary to treat such patients.(67) Pouch vaginal fistula The incidence of pouch-vaginal fistulas ranges from 3–16%.(68) Pouch-vaginal fistulas are a major potential cause of pouch fail- ure. Fistulas which occur in the early postoperative period are most commonly a manifestation of sepsis, and can occur from anasto- motic leak and necessitation through the vaginal wall, or may result from technical factors including entrapment of the perivaginal tis- sue in the staple line (Figure 31.5). An important part of the ileoanal pouch procedure is to ensure that the vagina is not incorporated within the stapler. Late pouch-vaginal fistulas are more commonly associated with unsuspected Crohn’s disease.(69) Pouch-vaginal fistulas may manifest as pelvic pain, fever, a “Bartholin’s abscess” which when drained has fecalent mate- rial, or passage of gas. Fistulas which occur before ileostomy takedown are treated by management of infection, delayed ile- ostomy closure, and local repair. A number of procedures have been described for treatment of pouch vaginal fistulas. Ultimate success may be achieved in over 50% (70) but often requires mul- tiple procedures. For patients with Crohn’s disease, the use of infliximab and other biologics may be helpful. Pouch anal fistulas Early fistulas are generally a manifestation of sepsis and leakage at the ileoanal anastomosis. Late fistulas may be crytoglandular in origin and may also be a manifestation of Crohn’s disease. Our preference is for liberal use of draining setons and avoidance of fistulotomy. Figure 31.4 A leak from the efferent limb of the pouch may be difficult to diagnose. Such patients rarely heal with antibiotics and drainage alone and often require exploration and repair. Figure 31.5 A pouch vaginal fistula is seen on retrograde study. Early fistulas are due to infection and leak at the anastomosis while late fistulas often herald unsuspected Crohn’s disease. improved outcomes in colon and rectal surgery Pouchitis The most frequent long-term complication of the ileoanal pouch procedure is the development of pouchitis, a nonspecific inflam- mation of the ileal pouch mucosa. The precise etiology of pouchi- tis has not been elucidated but it is believed to potentially result from an overgrowth of anaerobic bacteria. It is disease specific and more commonly seen in patients with ulcerative colitis; it is rarely encountered in patients with familial adenomatous polyposis. Patients with ulcerative colitis associated with extraintestinal man- ifestations and patients with sclerosing cholangitis have a higher incidence of pouchitis.(71, 72) Presenting signs and symptoms of pouchitis include abdominal cramps, abdominal tenderness, fever, and increase in stool frequency, often associated with blood or mucus. The diagnosis may be made clinically, on the basis of endo- scopic examination in addition to clinical findings, or on the basis of histologic examination of the pouch mucosa; the lack of uni- form criteria to make such a diagnosis accounts for the variation in the incidence of pouchitis in many series. A pouchitis disease activ- ity index has been devised which includes clinical, endoscopic, and histologic features.(73) Pouchitis is generally treated with antibiotic therapy and the most commonly used agents include metronida- zole or ciprofloxacin. Some patients with pouchitis develop ongo- ing symptoms, and for patients with refractory pouchitis or rapidly relapsing symptoms, the use of probiotics appears to be helpful. Probiotics may suppress the resident pathogenic bacteria, stimu- late mucin glyocoprotein, prevent adhesion of pathogenic strains to epithelial cells, and reduce host immune responses. Probiotics may also be helpful in preventing recurrent pouchitis. A diagnosis of Crohn’s disease should be considered in patients with chronic pouchitis. In some cases, pouchitis is a cause of pouch failure. Pouchitis has been termed by some as “the Achilles heel” of the ileoanal pouch procedure. It is a cause of significant long-term morbidity; elucidation of the cause of pouchitis would likely ben- efit a large number of patients. Dysplasia and Malignancy Following construction, the ileoanal pouch undergoes a number of histologic changes, and with time, the metaplastic changes result in the ileal mucosa resembling colonic mucosa. These changes may also occur because of inflammation in the pouch and raise concerns of malignant transformation and the development of dysplasia. Neoplastic changes appear to be extremely rare. The majority of ile- oanal pouch patients who develop cancer had a prior cancer at the time of pouch construction. The recent ASCRS guidelines do not endorse routine surveillance of ileal pouches for dysplasia.(10) Pouch Failure Pouch failure defined as pouch excision or a nonfunctioning pouch at 12 months after the ileoanal pouch procedure occurs in 5 to 15%. While the majority of pouch failures occur within 2 years of pouch construction, late pouch failures also occur. The common cause of pouch failure include unsuspected Crohn’s disease, chronic pouchitis, poor function with incontinence, persistent fistula, and other pouch related complications such as stenosis with outlet obstruction. Reoperative pouch surgery with an attempt to salvage the pouch is challenging; pouch salvage is higher in patients with ulcerative colitis than Crohn’s disease.(74) CONTROVERSIES Reservoir Design While the original report by Parks used an S-pouch configu- ration, a number of other pouch configurations have been described, including J-pouch, lateral isoperistaltic H-pouch, and quadruple-loop W pouch. S pouches were initially asso- ciated with an increased need for catheterization because of a long distal ileal conduit. Shortening of the ileal conduit helps to initially avoid this complication, however, with time, the exit conduit of the S-pouch seems to elongate and obstructive defecation can occur. An S pouch may confer additional length compared to a J-pouch and may be the preferred configuration if achieving adequate length to performed a tension-free anas- tomosis in selected cases. The long outlet tract associated with an H pouch has been associated with stasis, pouch distention, and pouchitis. There have been no significant differences in pouch func- tion based on the configuration of the pouch. Due to the ease of construction and the lack of compelling data favoring a specific pouch design, J pouches are most frequently performed. Use of an S or W pouch adds about 45 minutes to the time of the opera- tive procedure. A recent meta-analysis examined the short and long-term out- come of J-, S- and W- reservoirs in patients undergoing restora- tive proctocolectomy.(75) A total of 18 studies of 1,519 patients (689 J, 306 W, and 524 S pouches) were reviewed. There was no difference in the incidence of early complications among the 3 types of pouch design. The frequency of defecation favored an S- or W- pouch design over a J pouch, although in practical terms the difference of 1–1.5 stools in a 24 hour period is unlikely to be of clinical significance to the patient. Night evacuation was significantly lower for a W than a J pouch. S pouches were associ- ated with a greater need for pouch intubation due to a long distal conduit; W pouches also required intubation more often than J pouches. Mucosectomy vs. Double-Stapled Technique The ileoanal anastomosis may be performed with a handsewn technique after mucosal stripping (mucosectomy) or with a dou- ble-stapled technique. The initial technique reported by Parks was mucosal strip- ping commencing at the dentate line and removing all diseased mucosa, thus eliminating the risk of recurrent proctitis or neo- plastic transformation. A potential advantage of the double- stapled technique is greater technical ease, and potentially less tension on the anastomosis. Preservation of the anal transitional zone may minimize sphincter damage and improve functional results. Three prospective randomized trials have not shown an advantage for the double-stapled technique vs. the muco- sectomy technique.(76, 77, 78) These trials have all been small and are underpowered to demonstrate a difference. A meta- analysis of 4,183 patients (2,699 hand-sewn vs. 1,488 stapled IPAA) found similar early postoperative outcomes; however, stapled IPAA patients had improved nocturnal continence and had higher resting and squeeze pressures on anorectal physi- ologic testing.(79) surgery for ulcerative colitis Preservation of the anal transitional zone and performance of a double-stapled technique leaves a residual 1–2 cm of dis- eased rectal mucosa, which may be at risk for the development of dysplasia and subsequent malignant transformation. It has been suggested that patients who have had a double-stapled tech- nique be followed in a surveillance program, with biopsies of the retained columnar mucosa at least every 2 years beginning 8 to 10 years after the onset of symptoms of disease.(80) The recommen- dations for biopsy are controversial and is an area where further study is needed to define the natural history of the retained 1–2 cm of columnar mucosa. Other authors have not found the devel- opment of dysplasia with long-term follow-up.(81) Omission of ileostomy Restorative proctocolectomy is most commonly performed in two stages with an initial proctocolectomy, pouch construction, and diverting ileostomy, followed by ileostomy takedown after demonstration of satisfactory pouch healing. However, construc- tion of a loop ileostomy may be associated with excessive stoma output, dehydration, hernia, bowel obstruction, and subsequent anastomotic complications associated with ileostomy takedown; these have been cited as a reason to potentially avoid diverting ileostomy in selected patients after ileoanal pouch construction. Conversely, many feel that loop ileostomy construction will mini- mize the potential consequences of pelvic sepsis (and potentially reduce the chance of pouch failure). This issue has been characterized by a great deal of passion and no randomized controlled studies. A one stage procedure without loop ileostomy is associated with a more difficult initial recovery and most likely a slight increased rate of anastomotic disruption and pelvic sepsis. An alternate view of this is that with fecal diver- sion, some patients with minor leaks and sepsis may not be clini- cally detected. Loop ileostomy avoids some of the consequences of pelvic sep- sis, which is a major cause of pouch failure. Despite aggressive treatment the risk of pouch failure after pelvic sepsis is 20%, 31% and 35% at 3, 5 and 10 years respectively.(61) A single stage IPAA without loop ileostomy decreases the risk of ileostomy related complications, and complications including small bowel obstruc- tion associated with an additional operative procedure. A recent review compared 17 studies with 1,486 patients (765 without ileostomy and 721 with ileostomy).(61) While there was no significant difference in the functional outcome of the two groups, those patients without an ileostomy had a higher inci- dence of pouch related leak and stricture formation. Selective omission of an ileostomy may be considered when an anastomosis is intact and under no tension, the procedure is not complicated by excessive bleeding or other technical difficulties and the patient is not on high dose steroids before the procedure. (10) The patient should be adequately counseled preoperatively concerning the pros and cons of ileostomy omission. Crohn’s Disease and Indeterminate Colitis Crohn’s disease has been considered to be a contraindication to the performance of an ileoanal pouch procedure because of the risks of recurrent disease and the potential need for pouch exci- sion with subsequent loss of substantial amounts of bowel. However, there are some patients who undergo the procedure for ulcerative colitis and an ultimate diagnosis of Crohn’s disease is made. In general these patients are found to have a higher risk of pouch failure from 28–52% (82, 83, 84, 85) compared to patients with ulcerative colitis or familial adenomatous polyposis. In a cohort of 32 patients out of 790 patients with an ultimate diagnosis of Crohn’s disease, 93% had complications including perineal abscess/ fistula (63%), pouchitis (50%), and anal stricture (38%) (85). It is not known whether administration of agents such as infliximab to such patients will ultimately impact the incidence of pouch failure, or whether it will delay the diagnosis or pouch failure. All efforts should be made to confirm a diagnosis of ulcerative colitis and exclude a diagnosis of Crohn’s disease preoperatively. In addition to a thorough history and examination, a recent study suggested that a family history of Crohn’s disease and serology positive for anti- Saccharomyces cerevisiae immunoglobulin-A were more likely to be diagnosed with Crohn’s s after IPAA (67%) than patients with either risk factor (18%) or neither risk factor (4%) (86). While techniques of restorative surgery for ulcerative colitis have shown substantial advances over the past several decades, further study focusing on improvements in complications and functional outcomes will ultimately further improve a patient’s quality of life. REFERENCES 1. Loftus CG, Loftus EV, Sandborn WJ et al. Update on inci- dence and prevalence of Crohn’s disease (CD) and ulcer- ative colitis (UC) in Olmsted County, Minnesota [abstract]. Gastroenterology 2003; 124: A36. 2. Loftus EV Jr. Clinical epidemiology of inflammatory bowel disease: incidence, prevalence, and environmental influences. Gastroenterology 2004; 126: 1504–7. 3. Leijonmarck CE. Surgical treatment of ulcerative colitis in Stockholm county. Acta Chir Scand Suppl 1990; 554: 1–56. 4. Wexner SD, Rosen L, Lowry A et al. Practice parameters for the treatment of mucosal ulcerative colitis. Dis Colon Rectum 1997; 40: 1277–85. 5. Truelove SC, Witts L. Cortisone in ulcerative colitis: final report on a therapeutic trial. BMJ 1955; 2: 1041–8. 6. Mahadevan U. Medical treatment of ulcerative colitis. Clin Colon Rectal Surg 2004; 17: 7–19. 7. Cottone M, Pietrosi G, Martorana G et al. Prevalence of cytomegalovirus infection in severe refractory ulcerative and Crohn’s colitis. Am J Gastroenterol 2001; 96: 773–5. 8. Mahadevan U, Loftus EV Jr, Tremaine WJ et al. Azathioprine or 6-mercaptopurine before colectomy for ulcerative colitis is not associated with increased postoperative complications. Inflamm Bowel Dis 2002; 8: 311–6. 9. Hanauer SB. Drug therapy: inflammatory bowel disease. N Engl J Med 1996; 334: 841–8. 10. Cohen JL, Strong SA, Hyman NH et al. Practice parameters for the surgical treatment of ulcerative colitis. Dis Colon Rectum 2005; 48: 1979–2009. 11. Robert JH, Sachar DB, Aufses A et al. Management of severe hemorrhage in ulcerative colitis. Am J Surg 1990; 159: 550–5. . incontinence or an inadequate sphincter, patients with low rectal improved outcomes in colon and rectal surgery cancers in association with ulcerative colitis who require proc- tectomy and. Signs Abdominal tenderness Abdominal distention and tenderness Source: After Truelove and Witts. BMJ 1955; 2: 1041–45. Reprinted with permission from Clinics in Colon and Rectal Surgery. Volume. of the colon and abdominal distention and tenderness. The term toxic megacolon has been used when the colonic distention of the transverse colon exceeds 6 cm, but relying on this finding to