Fulminant Ulcerative Colitis — 1 Fulminant Ulcerative Colitis

Roger Hurst, MD, Sharon L. Stein, MD, and Fabrizio Michelassi, MD

Fulminant ulcerative colitis is a potentially life-threatening terms “severe” and “fulminant” interchangeably, whereas disorder that requires expert management to allow for opti- others, concerned over the lack of a clear defi nition, recom- mal outcomes. Once associated with very high mortality,1 the mend that the term “fulminant ulcerative colitis” be avoided.7 medical and surgical treatment of fulminate ulcerative colitis This recommendation aside, the term “fulminant ulcerative has greatly improved such that mortality from fulminant colitis” is an established component of the medical vernacular ulcerative colitis currently is less than 3%.2 , 3 Optimal even if the term itself is not clearly defi ned. 8– 10 Fulminant management necessitates coordination between medical and ulcerative colitis is certainly a severe condition associated surgical therapy; hence, multidisciplinary strategies are with systemic deterioration related to progressive ulcerative required. colitis. Most would agree that a fl are of ulcerative colitis can be considered fulminant if it is associated with one or more of the following: high fever, tachycardia, profound anemia Disease Defi nition requiring transfusion, dehydration, low urine output, abdom- The most commonly applied classifi cation for the severity inal tenderness with distention, and profound leukocytosis of ulcerative colitis was described by Truelove and Witts, who with left shift, severe malaise, or prostration. Patients with identifi ed clinical parameters to categorize mild, moderate, these symptoms should be hospitalized for aggressive resusci- and severe colitis. 4 The Truelove-Witts classifi cation, how- tation while clinical assessment and treatment are initiated. 11 ever, does not specify a unique category for fulminant disease. Hanauer modifi ed this classifi cation scheme to include the designation of fulminant colitis [see Table 1 ].5 There is, Clinical Assessment however, no universally agreed-upon distinction between Patients admitted with severe or severe and fulminant ulcerative colitis.6 Some authors use the fulminant ulcerative colitis require a complete history and physical Table 1 Criteria for Evaluating the Severity examination. Fulminant ulcerative of Ulcerative Colitis colitis is rarely the initial presenta- tion of ulcerative colitis, and most Mild Severe Fulminant Variable Disease Disease Disease patients will have a prior diagnosis. The abdominal examination Stools < 4 > 6 > 10 should focus on signs of perito- (no./day) neal irritation that may suggest Blood in stool Intermittent Frequent Continuous perforation or abscess formation. Any patients admitted with Temperature Normal > 37.5 > 37.5 severe ulcerative colitis may have already received substantial (°C) doses of corticosteroids, which can mask the physical fi ndings Pulse (beats/ Normal > 90 > 90 of peritonitis. Initial laboratory studies should include a com- min) plete blood count with differential, a coagulation profi le, and Hemoglobin Normal < 75% of Transfusion a complete metabolic profi le with assessment of nutritional normal value required parameters such as the serum albumin. Abdominal fi lms and Erythrocyte £ 30 > 30 > 30 an upright chest x-ray should be obtained to assess for colonic sedimenta- distention indicating toxic megacolon and to assess for the tion rate presence of pneumoperitoneum indicating perforation. Infec- (mm/hr) tious agents should be ruled out by multiple stool specimens Colonic __ Air, edematous Dilatation sent for Clostridium diffi cile, cytomegalovirus, and Escherichia features on wall, thumb coli O157:H7.12, 13 It is important to identify the presence of radiography printing opportunistic infections, particularly C. diffi cile, even in Clinical signs __ Abdominal Abdominal patients with an established diagnosis of ulcerative colitis, as tenderness distention and superinfection with C. diffi cile in ulcerative colitis patients is tenderness common. Assessment with endoscopic examination of the

Red text is tied to a SCORE learning objective. DOI 10.2310/7800.S05C13

Management of Fulminant Ulcerative Colitis

Patient has severe or fulminant Hospitalize patient. ulcerative colitis Give blood products to treat anemia or coagulopathy. Correct metabolic derangements. Perform history and physical examination. Abdominal examination focuses on peritoneal Optimize nutritional status (e.g., via bowel rest and signs (sometimes masked by corticosteroid therapy). total parenteral nutrition). Order investigative studies: • Laboratory tests: Complete blood count with differential, coagulation profile, metabolic profile, Patient is stable and has no Patient is unstable or has stool testing (for Clostridium difficile, Cytomegalovirus, indications for emergency surgery indication for emergency Escherichia coli). surgery (e.g., findings • Imaging: Abdominal films, chest x-ray, colonoscopy Initiate intravenous (IV) corticosteroid suggestive of perforation, (for minimum necessary distance). therapy (e.g., methylprednisolone, massive gastrointestinal 40–60 mg/day IV). bleeding, or toxic megacolon).

Colitis responds to IV corticosteroid Colitis does not respond to IV therapy corticosteroid therapy within 5–7 days

Switch to oral regimen, then gradually wean patient from steroids. Initiate maintenance therapy with purine analogues or immunosalicylates.

Immunosuppressive therapy Further immunosuppressive therapy is is not contraindicated contraindicated (e.g., because of renal insufficiency, hypocholesterolemia, sepsis, or patient refusal) Infliximab 5 mg/kg IV infusion. Cyclosporine Induction dose at 0, 2, Initiate IV therapy, and 6 weeks with 5 mg/kg initially 4 (or 2) mg/kg/day IV, every 8 weeks afterwards. adjusted as necessary.

Colitis responds to IV Colitis does not respond to IV immunosuppressive therapy immunosuppressive therapy within 4–5 days or complete remission is Continue current treatment. not achieved within 10–14 days Consider maintenance therapy with 6-mercaptopurine or azathioprine. Initiate surgical treatment. Consider laparoscopic-assisted approach as an option (except in cases of toxic megacolon).

Patient is healthy enough to Patient has perforation, Patient does not have obvious undergo full procedure at once peritonitis, or sepsis perforation, peritonitis, or sepsis but may not be healthy enough to undergo full Perform proctocolectomy with Perform a staged procedure procedure at once ileoanal anastomosis. (abdominal colectomy with ileostomy, followed later by proctectomy with Choose all-at-once or staged approach on the ileoanal anastomosis). basis of experience and clinical judgment. Most patients who do not respond to maximal medical therapy are probably best treated with a staged procedure.

medical therapy. Narcotics, antidi- arrheal agents, and other anticholinergic medications should be avoided as they can precipitate toxic dilation of the colon. Bowel rest typically reduces the volume of diarrhea, but it is not yet clearly established if bowel rest affects the clinical course of the fulminant colitis.18, 19 McIntyre and colleagues reported no signifi cant change in outcome in patients with acute fl ares of ulcerative colitis managed with total parenteral nutrition (TPN) and bowel rest compared to patients taking enteral nutrition.19 This study, however, involved varying degrees of severity of colitis such that only a small number of patients with fulminant ulcerative colitis appear to have been included in the study. Conversely, Mikkola and Jarvinen reported a potential clinical advantage to bowel rest and TPN in patients suffering from fulminant ulcerative colitis.18 The most common approach is to initially place these patients on Figure 1 Sigmoidoscopy demonstrating deep ulcerations bowel rest with hyperalimentation. Oral feedings are initiated in a patient suffering from fulminant ulcerative colitis. once symptoms of the fulminant attack begin to improve. Whether patients are maintained on bowel rest or given oral colon and rectum in the face of fulminant ulcerative colitis is feeds, each patient should always receive adequate nutritional controversial.14– 16 Colonoscopy with biopsy can provide useful support; hence, TPN should be maintained until the patient diagnostic information. Reports indicate that in experienced is tolerating full enteral feedings. hands, colonoscopy can be performed in patients with severe colitis with little risk. 14, 15 In general, however, it is recommended that endoscopic examination be limited to the mini- Medical Therapy mum distance necessary to confi rm severe colitis. If an The main standard medical therapy for fulminant ulcer- endoscopic examination is to be performed, it is important to ative colitis involves the induction of remission with intrave- minimize the amount of air insuffl ation as overdistention of nous (IV) corticosteroids or biologics followed by long-term the colon may lead to perforation or the development of maintenance treatment in the form of purine analogues or megacolon. Typical endoscopic fi ndings in fulminant biologics for those patients who achieve remission. Cases that ulcerative colitis are severe infl ammation, ulcerations, and are unresponsive to IV steroids are considered for IV mucosal sloughing. Colitis typically is worst in the rectum cyclosporine and, more recently, infl iximab. and continues proximally in a contiguous fashion. Occasion- ally, the distal rectum may be spared secondary to the use of steroids topical medications such as steroid suppositories. Although these fi ndings help differentiate ulcerative colitis from Crohn Steroid treatment has been the disease and indeterminate colitis, the differentiation is frontline therapy for acute fl ares of probably not important in the setting of fulminant disease, ulcerative colitis for almost 50 years. where maximal medical therapy or surgical treatment with Response rates for cases of fulmi- subtotal colectomy will be the same regardless of diagnosis. nant ulcerative colitis fall in the Beyond the diagnostic information, endoscopy can provide range of 50 to 60% when steroids useful prognostic information. Carbonell and colleagues are given over a 5- to 10-day course noted that the presence of deep extensive colonic ulcerations of treatment.20, 21 Methylpredniso- indicates a low probability for successful medical treatment of lone in a dose of 40 to 60 mg per fulminant ulcerative colitis, with less than 10% of patients day, given as a continuous IV infu- with deep ulcers responding to medical treatment. 14 Such an sion, is a common regimen.5, 22– 24 The duration of treatment endoscopic fi nding thus may assist in the decision to proceed to allow for response from IV steroid therapy has been con- with early surgery if medical therapy does not show rapid and troversial. Truelove and Witts in 1955 recommended urgent signifi cant improvement [ see Figure 1 ]. surgery after 5 days if the patient has not responded to IV steroid therapy. 4 This 5-day rule has been widely adopted, but experience suggests that courses up to 7 to 10 days can General Care be safely administered under careful observation to allow for All patients with fulminant ulcerative colitis require hospi- further time for response. 12 Patients who respond to IV steroid talization. Blood products should be administered to treat therapy are converted to oral steroids, typically prednisone. signifi cant anemia or coagulopathy. Metabolic derangements Corticosteroids, however, should never be used as a long- should be corrected.17 Patients with perforation or massive term maintenance therapy.5, 25 The toxic effects of corticoster- lower gastrointestinal hemorrhage need emergent operative oids are related to both the dose and duration of treatment. treatment. More stable patients are initially managed with Severe complications, including diabetes, osteoporosis, mood

11/09 © 2009 Decker Intellectual Properties Inc Scientific American Surgery GASTROINTESTINAL TRACT AND ABDOMEN FULMINANT ULCERATIVE COLITIS — 4 disturbances, and weight gain, are common with extended cyclosporine treatment; hence, serum magnesium levels use of even modest doses of steroids. Patients should be should be closely followed. slowly but completely weaned from steroid therapy. Because Dosing regimens for cyclosporine vary, but patients are typ- recurrence of symptomatic colitis occurs in between 40 and ically started on 4 mg/kg per day of IV cyclosporine, with the 50% of initial responders to IV therapy, maintenance therapy dose then adjusted to achieve a whole blood level between 150 with either purine analogues, immunosalicylates, or biologics and 400 ng/mL as measured by high-performance liquid chro- should be administered.5, 20 Unfortunately, dependency on matography or radioimmunoassay.36, 37 Higher levels up to corticosteroids is often encountered in patients with ulcerative 800 ng/mL in whole blood have been cited as acceptable by colitis. In these cases, the dose of steroids cannot be tapered some investigators. 28 If the patient does not show any improve- without an increase in disease activity and symptoms. When ment within 4 to 5 days or if complete remission is not achieved such patients cannot be taken off steroids within 3 to 6 by 10 to 14 days, surgery is then advised.12 Most of the side months, then surgical treatment is indicated. effects from cyclosporine are dose dependent, and some studies have shown that an initial dose of 2 mg/kg per day of IV cyclosporine cyclosporine can also be effective in achieving a remission.38– 40 Nonresponders to IV steroid treatment, once universally referred infliximab for surgery, may be treated with IV Infl iximab is a chimeric monoclonal antibody directed cyclosporine. Cyclosporine is an against human tumor necrosis factor (TNF). Infl iximab has immunosuppressant macrolide that been used to treat Crohn disease for over 10 years. It was not suppresses the production of until 2005, however, that infl iximab was approved for use in interleukin-2 by activated T cells patients with ulcerative colitis. Potential adverse effects from through a calcineurin-dependent infl iximab include activation of tuberculosis, infusion reac- pathway. 26 Originally applied as a tions, hypersensitivity reactions, the development of lym- means of preventing tissue rejec- phoma, and infectious complications. 41 Additionally, recent tion following transplantation, accumulating data suggest that infl iximab may signifi cantly cyclosporine has become the standard for the treatment of increase the risk for postoperative infection and healing com- steroid-refractory severe ulcerative colitis. The fi rst report of plications following surgery for ulcerative colitis.42, 43 the use of cyclosporine for the treatment of ulcerative colitis The use of infl iximab therapy in the setting of fulminant was by Gupta and colleagues in 1984.27 It was not until colitis is controversial. Whereas the data supporting infl ix- 10 years later that a randomized placebo-controlled trial of imab in the treatment of moderate to moderately severe dis- cyclosporine for steroid-refractory ulcerative colitis by Lichti- ease are convincing, the data demonstrating safety and ger and colleagues demonstrated the effectiveness of this effi cacy in the setting of fulminant colitis are limited. Based agent. 28 This revealed a response rate of 82% with 4 mg/kg on the current data, some experts have supported the initia- of cyclosporine in patients with steroid-refractory ulcerative tion of infl iximab treatment in patients with fulminant colitis colitis compared to zero response with continued IV steroid who have failed IV steroid therapy.44 On the other hand, therapy alone. Since this initial report, response rates of 56 to others have advocated the avoidance of infl iximab therapy in 91% have been reported in the medical literature, confi rming patients with fulminant colitis. 41 With wider use of anti-TNF cyclosporine as a major advance in the treatment of severe and therapy for ulcerative colitis, a greater percentage of patients fulminate ulcerative colitis.29– 31 Dosing and monitoring of admitted to the hospital with fulminant colitis are likely to cyclosporine are complicated and cumbersome for both physi- already be on infl iximab therapy; hence, the decision whether cians and patients, limiting its use. In addition, recurrence of or not to initiate therapy is often moot. Because of the risk of disease after initial remission with cyclosporine is high, with as severe complications, infl iximab therapy should not be given many as 60% of patients developing recurrent disease.32 Recur- in combination with cyclosporine therapy.45 rence rates can be substantially lowered with maintenance Because of the increased risk for anastomotic leak and therapy with mercaptopurine or azathioprine. With appropri- pelvic sepsis in patients undergoing the ileoanal procedure ate maintenance therapy, early recurrence of symptoms after while on anti-TNF therapy, patients with fulminant or severe successful IV cyclosporine treatment have been recorded as ulcerative colitis should have a staged abdominal colectomy low as 22%. 30, 33 Even if disease activity recurs and surgery is prior to the ileoanal procedure if they are being treated with necessary, cyclosporine therapy can allow for elective surgical infl iximab. The negative effect of infl iximab on wound heal- management when the patient is in better general health. This ing and infection is prolonged and appears to persist beyond is a clear advantage as urgent surgery for ulcerative colitis car- 3 to 4 months after the last dose given.42, 43 ries a much higher risk for complications when compared with The prolongation of medical therapy in patients with severe surgery performed in a more elective setting. 18, 34, 35 disease who have already received high doses of corticoster- Major side effects associated with cyclosporine treatment oids has caused concerns that those patients who fail both include renal insuffi ciency, opportunistic infections, and sei- steroid and subsequent cyclosporine or infl iximab therapy zures. The risk for seizures appears to be highest in patients may be at high risk for perioperative morbidity and mortality. with hypocholesterolemia. As such, patients with signifi cant Current experience, however, has not identifi ed an increased (less than 100 mg/dL) hypocholesterolemia should not receive risk for perioperative complications in patients who fail to cyclosporine treatment. Hypomagnesemia is commonly seen respond to cyclosporine therapy and thus does not appear to in patients with fulminant ulcerative colitis undergoing compromise surgical results.46 Yet recent data on infl iximab

11/09 © 2009 Decker Intellectual Properties Inc Scientific American Surgery GASTROINTESTINAL TRACT AND ABDOMEN FULMINANT ULCERATIVE COLITIS — 5 in ulcerative colitis do demonstrate a trend toward increased be referred for early surgery, independent of other indications number and severity of septic complications.42, 43, 46 of perforation or peritonitis. Toxic megacolon is an uncommon complication of severe ulcerative colitis. Associated with impending colonic perfora- Surgical Therapy tion, toxic megacolon requires aggressive management. Two indications for surgery specifi c parameters are required to confer the diagnosis of 49 The indications for surgery in a toxic megacolon. First, there must be colonic dilatation. patient suffering from fulminant Second, the patient should appear “toxic.” Patients with mild ulcerative colitis are listed in symptoms of ulcerative colitis can experience colonic dilatation, Table 2 . When the options for perhaps associated with a colonic ileus. This is distinctly dif- appropriate medical treatment have ferent from patients with fulminant ulcerative colitis, some been exhausted, surgery will, of degree of generalized toxicity, and colonic dilatation. Patients course, be required. Because most with fulminant ulcerative colitis should have an abdominal patients suffering from fulminant x-ray to assess for colonic dilatation [see Figure 2 ]. Addition- ulcerative colitis will respond to ally, patients with fulminant ulcerative colitis who develop aggressive medical therapy, an abdominal distention or have a sudden decrease in the attempt at medical treatment is number of bowel movements without signs of signifi cant warranted in almost all cases. Care must be exercised, how- clinical improvement should also be assessed radiographically ever, not to overtreat the patient with fulminant ulcerative for colonic dilatation. colitis who is unresponsive or shows minimal response to As noted, patients with toxic megacolon should be treated medical treatment. The immunosuppressive effects of high- aggressively. Individuals who are otherwise stable may undergo dose corticosteroids and IV cyclosporine or infl iximab, along a brief trial of conservative management consisting of elimi- with the debilitation of prolonged severe disease, can place nating narcotics and anticholinergic agents. Changing the the patient at high risk for perioperative complications. If the patient’s position from side to side, supine to prone, and into patient fails to show signifi cant improvement with IV steroids the knee-elbow prone position is thought to assist in the expul- 50 in 5 to 7 days, then the patient should be started on IV sion of colonic gas. Patients should be kept NPO, and broad- cyclosporine or infl iximab or referred for surgery. 12 For spectrum IV antibiotics are advocated. Attempts at endoscopic patients who fail to show improvement on second-line medi- decompression are to be avoided, and blind placement of cations within 4 days or fail to achieve remission of major rectal tubes is ineffective and may be harmful. Patients with symptoms by 2 weeks, surgery should be undertaken. If symptoms progress during the course of IV therapy or if no sign of improvement occurs, then the patient should be considered for early surgery. Additionally, patients known to have deep longitudinal ulcerations may also be referred for early surgery, given that these patients are more likely to fail IV medical therapy. The decision when best to abandon medical therapy in favor of surgery for patients with fulminant ulcerative colitis is diffi cult and requires experience and spe- cial expertise. Thus, patients with fulminant ulcerative colitis are best managed in a center specializing in infl ammatory bowel disease. Patients with perforation or severe bleeding require urgent surgery. 47 Debilitation from disease and immunosuppression from intensive medical therapy can mask the signs and symptoms of sepsis and peritonitis associated with perforation. When perforation occurs, the risk for perioperative mortality is up to 10 times greater compared to cases of fulminant colitis without perforation. 48 For these reasons, patients with high fever, marked leukocytosis, and persistent tachycardia should

Table 2 Indications for Operation Perforation Peritonitis Progressive signs of sepsis Failure to respond to medical treatment Inability of tolerate medical treatment Severe hemorrhage Figure 2 Abdominal radiograph of a patient with toxic megacolon. Printed with permission of University of Chicago Toxic megacolon Department of Surgery Archives.

11/09 © 2009 Decker Intellectual Properties Inc Scientific American Surgery GASTROINTESTINAL TRACT AND ABDOMEN FULMINANT ULCERATIVE COLITIS — 6 toxic megacolon who do not rapidly respond to conservative reported on 12 patients also undergoing urgent restorative management and those who show signs of peritonitis are oth- proctocolectomy with ileoanal procedure for ulcerative colitis erwise unstable and should undergo urgent surgery. 51 and noted a 41% anastomotic leak rate compared to an 11% leak rate in patients undergoing ileoanal anastomosis under preparation for surgery more controlled conditions.54 Based on these results, Hey- Patients with fulminant ulcerative colitis who are stable but vaert and colleagues counseled against ileoanal anastomosis not responding to medical therapy may have time to prepare in the urgent setting. Fukushima and colleagues also noted a for surgery. Patients who are not NPO should be maintained higher risk for anastomotic leak (36%) in patients undergoing on clear liquids and then kept NPO for 6 to 8 hours prior to urgent restorative proctocolectomy with ileoanal anastomo- surgery. Usually, these patients do not require any form of sis. 55 This group likewise advised against performing an ileo- bowel preparation as they have multiple bloody, liquid bowel anal anastomosis in the urgent setting. movements and will require an emergent colectomy with Precise parameters under which it is best to stage the proce- diversion. If used, bowel preparations may be limited to a dure with an initial abdominal colectomy have not been clearly rectal washout at the time of surgery. If time allows, defi ned. It is universally accepted that patients with perfora- patients should be provided with a consultation with an expe- tion, peritonitis, or sepsis require a staged procedure. Any rienced enterostomal therapist and an optimal site for the patient with suspicion of Crohn colitis or indeterminate colitis ostomy should be marked on the abdomen. Prophylactic should undergo subtotal colectomy to allow for further diag- antibiotics should be given prior to the creation of the surgical nostic evaluation prior to creation of ileal pouch-anal anasto- incision, and appropriate stress-dose steroids should also be mosis. Beyond this, there is no clear consensus. The available administered. studies addressing this issue unfortunately involve a small number of patients, do not clearly defi ne what is meant by surgical strategies “fulminant” colitis, or do not directly compare results between The operative strategies for the the two alternative strategies of staged colectomy versus imme- treatment of fulminant ulcerative diate ileoanal anastomosis. Clearly, there is a small subset of colitis are controversial. Ultimately, patients with symptoms severe enough to require hospitaliza- almost all patients will end up with tion who are healthy enough to safely undergo a primary ileo- a restorative proctocolectomy and anal anastomosis. On the other end of the spectrum, severely an ileoanal anastomosis. Most ill patients, that is, most patients with fulminant colitis, should patients with fulminant ulcerative have a staged procedure. Because specifi c criteria to quantify colitis, however, will have the fi nal the risk have not been defi ned, the decision to stage or not to surgical goal achieved in multiple stage ultimately rests with the clinical judgment of the experi- steps. The safety of performing an enced surgeon. It has been the author’s experience, however, extensive resection with a prolonged and delicate reconstruc- that a large majority of patients who fi t the criteria of fulminant tive procedure in an acutely ill patient is questionable. It has colitis as noted in Table 1 and have failed maximal medical thus been the practice of many to fi rst perform a total abdom- therapy are best managed with a staged approach. inal colectomy with an ileostomy, leaving the rectum as a If the procedure is staged and the proctectomy delayed, stapled stump or a mucous fi stula.47, 48 This approach allows there are several advantages to the patient in terms of having the patient to recover from the acute illness, to wean off the time to fully consider lifestyle and reproductive options. immunosuppressive agents, and to improve the nutritional Living with an ileostomy for 2 to 4 months ensures that the status. Although the remaining rectal stump will be affected patient understands the relationship between the timing and by ulcerative colitis, the activity of disease is greatly dimin- quality of oral intake and the frequency and consistency of ished with the fecal diversion, and almost all patients can be ileostomy output. This knowledge is extremely helpful to the completely weaned from steroids and other immunosuppres- patient in affecting the frequency, timing, and consistency of sive medications. A subsequent restorative proctectomy with bowel movements after completion of all stages associated ileoanal anastomosis can then be performed in a more con- with a restorative proctectomy and ileoanal pouch procedure. trolled situation. Further, it gives the patient confi dence that life with an ileo- The benefi ts of undergoing a staged procedure may be stomy is manageable, a notion that may be important if com- multiple; some studies have demonstrated a reduced risk of plications of an ileoanal procedure escalate to the point of anastomotic leak, but fi ndings are not universal. Ziv and col- considering reversal. leagues reported excellent long-term results and acceptable All patients who undergo proctectomy face the risk of short-term morbidity in 12 patients undergoing immediate decreased fertility and sexual function postoperatively, and restorative proctocolectomy with ileal pouch-anal anastomo- consideration of timing of surgery is appropriate. If the rectum sis.52 The authors used a liberal defi nition for fulminant coli- is acutely infl amed, dissection may be more diffi cult and inju- tis that included patients who may not have been as acutely ries to pelvic nerves and formation of adhesions and abscesses ill and represent an extraordinarily small proportion of the may be greater. As a large percentage of the population with total number of ileoanal procedures performed at their insti- ulcerative colitis are in their reproductive prime, preoperative tution. Harms and colleagues reported on 20 patients under- consideration of issues is appropriate. going restorative proctocolectomy with ileal pouch-anal In males, erectile dysfunction appears to be altered after anastomosis for the urgent treatment of ulcerative colitis and damage to the parasympathetic nerves, whereas ejaculatory also reported excellent long-term results and exceptional dysfunction results from sympathetic nerve injury. It is esti- perioperative morbidity. 53 However, Heyvaert and colleagues mated that between 0 and 10% of males experience some

11/09 © 2009 Decker Intellectual Properties Inc Scientific American Surgery GASTROINTESTINAL TRACT AND ABDOMEN FULMINANT ULCERATIVE COLITIS — 7 degree of sexual dysfunction postoperatively.56, 57 Although to a very diffi cult proctectomy at the next stage of the proce- studies demonstrate that dysfunction may be transient and dure sequence. Too long of a stump can run the risk of com- relieved with pharmaceutical agents such as sildenafi l, this is plications related to persistent disease in the rectum, including a signifi cant consideration for males in the peak of their repro- bleeding, discharge, and tenesmus. In most cases, the rectum ductive years. 58 Subtotal colectomy has not been associated can be safely stapled at the level of the sacral promontory [ see with decreased function; therefore, males in need of an urgent Figure 4]. When performing the colectomy, the sigmoid procedure may defer the proctectomy to a later time and con- branches of the inferior mesenteric artery are divided, whereas sider cryopreservation prior to the removal of the rectum. the terminal branches of the inferior mesenteric artery are pre- In women, causes of sexual dysfunction are more diffi cult served. This will ensure a good blood supply to the remaining to determine, but infertility rates are signifi cantly elevated rectal stump and aid in the healing of the stapled closure. following proctocolectomy. Fecundity rates, or the percent- Preservation of the terminal branches of the inferior mesen- age of women who become pregnant per unit time, are teric artery and the superior rectal artery also simplifi es the reduced to one third of baseline populations after proctec- subsequent proctectomy by keeping the pelvic sympathetic tomy for ulcerative colitis.59, 60 Adhesions and occlusion of nerves free of surrounding scar tissue and by providing a key fallopian tubes have been noted in a large number of postop- anatomic landmark that will assist in the location of the appro- erative patients obstructing normal ovulation and ferti- priate presacral dissection plane at the time of the proctec- lization.61, 62 Although it would be simple to blame surgery tomy. To staple the proximal rectum safely, the mesenteric alone, patients requiring ileal pouch-anal anastomosis for and pericolonic fat are removed from the bowel wall. Approx- familial adenomatous polyposis do not experience this radical imately 2 cm of bowel is prepared in such a manner, and the decrease in fertility postopertively.63 Although in vitro fertil- bowel is then closed with a transverse anastomosis stapler ization has been highly successful in this group, consideration (TIA stapler) using 4.8 mm staples. The bowel is then divided of postponement of proctectomy for family planning may be proximal to the staple line. It is important to closely examine reasonable in these patients.64 the staple line to ensure that the staples are formed properly into two rows of well-formed “B’s.” The staple line should surgical technique also be examined to make sure that individual staples are not Surgical exploration can be performed with a midline or cutting into the muscularis propria of the bowel. To provide transverse incision. The abdomen should be carefully exam- extra assurance against dehiscence, the staple line can be over- ined with particular attention given to the small intestine sewn with interrupted Lembert sutures [ see Figure 4 ]. If used, looking for signs of Crohn disease. The colon often shows the these sutures should be carefully placed so that the anterior changes of colitis with serosal hyperemia, corkscrew vessels, and posterior serosal surfaces are approximated without undue and edema [ see Figure 3 ]. Colectomy can be performed in the tension. In a well-constructed rectal pouch, placement of standard fashion with mesenteric division occurring at a con- pelvic drains is not necessary and can be harmful as their venient distance from the bowel. Wide mesenteric resection placement close to the suture line may promote dehiscence. is not necessary. In some cases, the colon at the level of the sacral promon- If a staged colectomy is performed, an ileostomy is created tory will be affected by deep ulcerations and severe infl amma- in the standard fashion at the site selected as least inconve- tion such that the closure of the rectum at this level may be nient for the patient preoperatively, and the rectum is left at high risk for dehiscence [see Figure 5 ]. If the severity of the behind either stapled or brought up to the abdominal wall as disease precludes safe closure of the rectal stump, then cre- a mucous fi stula. When stapled, it is important that the stump ation of a mucous fi stula should be considered. The mucous be of the appropriate length. Too short of a pouch can lead fi stula does require a longer segment of bowel and thus is

Figure 3 Intraoperative photograph of colon affected by fulminant ulcerative colitis. Changes on the serosal aspect Figure 4 Hartmann pouch constructed at the level of the are typically subtle. Serosal hyperemia with small sacral promontory. The transverse anastomosis stapler line “corkscrew” vessels is present. is reinforced with interrupted silk Lembert sutures.

Figure 5 Surgical specimen showing severe ulceration and infl ammation seen with fulminate ulcerative colitis. Figure 6 Laparoscopic colectomy for fulminant ulcerative colitis. Mobilization of the splenic fl exure. associated with a greater risk of bleeding from the retained segment. Additionally, a mucous fi stula is unsightly and often division of the mesentery can be accomplished laparoscopi- generates a very foul odor. As a compromise approach, some cally with the specimen being removed through a small Pfan- surgeons have advocated stapling the rectosigmoid and plac- nenstiel incision [ see Figure 6 ]. An end ileostomy is also ing the proximal end of the stump through the fascia at the fashioned with the aid of inspection through the Pfannenstiel lower edge of the midline incision. The end of the stump is incision. Alternatively, the Pfannenstiel incision can be made then left buried in the subcutaneous tissue. The benefi t of early on in the procedure and used as a hand assist port and this approach is that should dehiscence of the staple line the colon removed using a hand-assisted laparoscopic occur, then sepsis should be limited to the subcutaneous approach. The clinical advantages of a laparoscopic-assisted space rather than result in an intra-abdominal or pelvic approach in the management of fulminate ulcerative colitis have not been fully defi ned. However, increasing experience abscess. with this approach indicates that laparoscopic-assisted If attempts to fashion a secure rectal closure fail, and the colectomy is a safe and reasonable alternative that may well remaining rectal stump is too short to bring out as a mucous result in shorter hospital stays, decreased postoperative pain, fi stula, then two options remain: an additional inch or two of decreased complication rates, and possible reduced proximal rectum can be resected and closure of the rectal adhesions.68, 69 The laparoscopic-assisted approach thus stump performed lower, sometimes just below the peritoneal appears to be a reasonable option for most patients suffering refl ection. In this situation, closed suction drains should be from fulminant ulcerative colitis. placed in the deep pelvis and, if possible, the peritoneum Patients suffering from toxic megacolon, however, should closed over the rectal stump. Such a short rectal stump, how- be managed with an open surgical approach as the laparo- ever, will make fi nding it during subsequent completion scopic instruments used to grasp the bowel are likely to cause restorative proctectomy and ileoanal anastomosis more diffi - perforation in the severely thinned walls of the dilated cult. Alternatively, a large Malecott drain, inserted through colon. the lower abdominal wall, can be placed in the proximal rectum and the opening of the rectum can be synched around it with a purse-string suture. In this case, as well as in any Summary case where the closure of the rectal stump seems precarious, The optimal management of fulminate ulcerative colitis is transanal placement of a rectal tube to drain rectal secretions challenging. Most patients will respond to medical therapy and blood may be benefi cial in reducing the risk of intra- such that long-term control of disease can be achieved or at abdominal spillage of rectal contents or of dehiscence of the least surgery can be undertaken at later, safer, elective stapled rectal stump. conditions. Surgical strategies must be tailored to account for each individual patient’s overall physical condition, with most patients who fail medical therapy requiring an laparoscopy abdominal colectomy as the fi rst step in a staged surgical Experience with laparoscopic-assisted approaches has dem- approach. onstrated that abdominal colectomy can be performed safely in patients suffering from ulcerative colitis using these mini- mally invasive approaches.65– 67 Mobilization of the colon and Financial Disclosures: None Reported

References

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