42 for Crohn’s Disease

Scott A. Strong

Crohn’s disease is a chronic, unremitting, incurable, inflam- cellular function, nonimmune cell activity, protein expression, matory disorder that can affect the entire intestinal tract. and cellular apoptosis hint as to the role of the dysregulated Although the etiology remains uncertain, the distribution and effector mechanisms in the pathogenesis of Crohn’s disease.9 behavior of the disease can be generally characterized. The prevalence of Crohn’s disease in the United States is The presenting symptoms and signs, medical and operative approximately seven cases per 100,000 persons, and the inci- options, and outcome likely depend on the disease genotype dence has steadily increased over the past 5 decades.10,11 and phenotype. Specifically, the surgical procedures typically Internationally, the prevalence is relatively high in northern utilized in the operative management of Crohn’s disease Europe, significantly lower in southern Europe and Australia, include nonresectional techniques such as internal bypass, and the lowest in South America, Asia, and upper Africa. This fecal diversion, and strictureplasty as well as resectional pro- discrepancy in prevalence is likely multifactorial in nature, but cedures with or without concomitant anastomoses. The mor- it is recognized that the incidence and prevalence of Crohn’s bidity and risk for disease recurrence varies between the disease increases as a region becomes more urbanized. different procedures depending on myriad factors such as The male-to-female ratio of the disease is 1.1Ð1.8:1 and the preoperative variables, site and behavior of disease, and disease has a bimodal age distribution with the first peak postoperative influences. occurring between the ages of 15Ð30 years and the second between 60Ð80 years; most persons experience the onset of disease symptoms before 30 years of age. The disorder is Etiology and Incidence more common in whites than in blacks, Hispanics, or Asians, and a two- to fourfold increase in the prevalence has been The cause of Crohn’s disease is unclear but recent investiga- found among the Jewish population in the United States, tions continue to provide insight into the etiology and patho- Europe, and South Africa compared with other ethnic groups. genesis of this inflammatory disorder that can affect any portion of the intestinal tract through a complex interplay between conditioning factors and effector mechanisms. The Disease Classification conditioning factors include genetic influences and triggering events that create a permissive host, whereas the effector The original classification of Crohn’s disease was described mechanisms mediate tissue damage through dysregulation of nearly three decades ago,12 but inaccuracies associated with the intestinal immune and nonimmune functions. Patients this and subsequent systems led to the most recent refinement, afflicted with symptomatic disease are likely genetically sus- the Vienna Classification.13 This scheme was generated by a ceptible because abnormalities in seven loci on chromosomes World Congress of Gastroenterology Working Party that 16q, 12, 6p, 14q, 5q, 19, and 1p have been identified in prospectively designed a simple phenotypic classification sys- selected populations,1 but there must be other factors at play tem based on objective and reproducible clinical variables because these variations have not been replicated in all popu- that include age at diagnosis, anatomic location, and disease lations and the proband concordance rate among monozygotic behavior. The age at diagnosis is stratified into patients <40 twins is only 50%Ð60%.2,3 Initiating or triggering events such years and those ≥40 years. The anatomic location is classified as environmental factors and microbial agents also probably as terminal ileum, colon, ileocolon, and upper gastrointesti- contribute to disease susceptibility as evidenced by reports nal. Terminal ileal disease is defined as disease limited to the that describe the effects of tobacco usage4Ð7 and fecal flora8 lower third of the small bowel with or without cecal involve- on disease activity. In addition, abnormalities in immune ment. Colon disease is any colonic involvement between the

584 42. Surgery for Crohn’s Disease 585 cecum and without small bowel or upper gastroin- increasing colonic dilatation, massive hemorrhage, peritonitis, testinal disease. Ileocolon disease is disease of the terminal and septic shock are indications for emergent operation after ileum with colonic involvement noted between the cecum and the patient has been adequately resuscitated. In the absence of rectum. And, upper gastrointestinal disease is defined as any these features, medical therapy is initiated with high dosages disease location proximal to the terminal ileum regardless of of intravenous corticosteroids, immunomodulators, and/or involvement in other areas. The disease behavior is grouped biologic agents.19 Broad-spectrum antibiotics directed against as nonstricturing nonpenetrating (inflammatory), stricturing, intestinal flora are prescribed to minimize the risk of sepsis and penetrating. Subsequent application of the Vienna secondary to transmural inflammation or microperforation. Classification to clinical practice has demonstrated that the Anticholinergics, antidiarrheals, and narcotics are avoided Crohn’s disease phenotype markedly changes for a given because they may worsen already impaired colonic motility or patient over time14Ð17 with 15% of patients experiencing a conceal ominous symptoms. Hyperalimentation may be change in anatomic location and 80% of individuals with started and the patient is closely observed with serial exami- inflammatory disease ultimately demonstrating a stricturing nations and abdominal roentgenograms. Any worsening of the or penetrating behavior. Moreover, the ability of experts to clinical course over the ensuing 24Ð72 hours mandates urgent independently agree on disease phenotype using the Vienna . If the patient improves minimally after 5Ð7 days Classification in controlled trials ranges from poor to fair.18 It of conventional therapy, the medical therapy should be altered is unclear whether the varied classification systems fail or surgery should be advised. Experience with cyclosporine or because of the heterogeneity of the disease or inherent short- infliximab in this setting is anecdotal, and should be weighed comings of the classification schemes. Although these fail- against operative therapy while understanding that surgery in ings limit the utility of the Vienna Classification in clinical this setting often relegates the patient to a life-long trials and disease management, recent advances in determin- .20,21 ing the genetic linkages associated with Crohn’s disease will The principal operative options in patients with toxic coli- likely lead to a revised Crohn’s disease classification system tis complicating Crohn’s disease include subtotal that combines genotype and phenotype characteristics. with end ileostomy, total proctocolectomy with end ileostomy, and loop ileostomy with decompressive blowhole . Of these alternatives, subtotal colectomy with end Operative Indications ileostomy is the most widely practiced procedure. The most difficult aspect of the operation is managing the distal bowel The indications for operative management of Crohn’s disease stump. The distal limb may be closed with sutures or staples include acute disease complications, chronic disease compli- and then delivered to the anterior abdominal wall where it can cations, and failed medical therapy. The acute complications lie without tension in the subcutaneous fat of the lower mid- are toxic colitis with or without associated megacolon, line wound. Dehiscence of the closure during the postopera- hemorrhage, and perforation, whereas the chronic disease tive period results in a mucous fistula instead of a pelvic complications include neoplasia, growth retardation, and abscess as witnessed when the closed stump is left within the extraintestinal manifestations. Failed medical therapy can peritoneal cavity. If the bowel wall is too friable to hold take several forms including unresponsive disease, incom- sutures or staples, a mucous fistula is primarily created. plete response, medication-related complications, and non- Rarely, instead of creating the fistula, the rectosigmoid stump compliance with medication. must be exteriorized and wrapped in gauze to prevent retrac- tion with a mucous fistula safely fashioned 7Ð10 days later. Toxic Colitis The patient typically improves over the ensuing few days and can be typically discharged within a week of the opera- Toxic colitis is a potentially fatal complication of Crohn’s dis- tion. An ileoproctostomy can be recommended 6 months later ease, particularly if accompanied by megacolon. Although in selected persons who demonstrate minimal mucosal several schemes exist to accurately identify toxic colitis, one inflammation, adequate rectal compliance, absence of signif- reasonably simple system uses a definition that includes a dis- icant anoperineal disease, and sufficient sphincter strength. ease flare accompanied by two of the following criteria: Otherwise, the diseased rectum is left in place and the patient hypoalbuminemia (<3.0 g/dL), leukocytosis (>10.5 × 109 is counseled about the risk of neoplasia and the need for cells/L), tachycardia (>100 beats/minute), temperature appropriate surveillance .22 In these individuals, increase (>38.6°C). Use of this relatively objective definition proctectomy is usually recommended if disease-related symp- may aid in the diagnosis and care of these patients whose toms prove to be too bothersome, neoplasia is identified, sur- severe condition can be under-appreciated because of high veillance is limited because of stricturing, or laparotomy is dosages of steroids, immunomodulators, or biologic agents. warranted for other reasons. Disease-related symptoms are The initial management is directed at reversing physiologic likely to occur in patients with prior anoperineal disease and deficits with intravenous hydration, correction of electrolyte proctectomy is often required within the first few postopera- imbalances, and blood product transfusions. Free perforation, tive years.21,23 586 S.A. Strong

Proctocolectomy with end ileostomy is rarely performed in modalities, the catheter is left in position and intraoperative the severely ill patient with toxic colitis because of the exces- angiography is performed to accurately identify the bleeding sive rates of morbidity and mortality.24Ð26 Proctectomy site and guide a limited .31 Otherwise, wide increases the difficulty of the procedure and risks pelvic resection might be necessary to manage hemorrhage from bleeding as well as autonomic nerve damage. In rare instances a small ulcerated area within an extensive segment of of rectal perforation or profuse colorectal hemorrhage, or affected bowel. in the less severely ill patient who would not be a candidate Laparotomy and resection with or without anastomosis are for future ileoproctostomy, proctocolectomy may be a viable required if the patient’s hemodynamic state cannot be sus- option. The surgeon must be cautioned, however, that tained, bleeding persists despite 6 units of transfused blood, the macroscopic and microscopic differentiation of ulcerative hemorrhage recurs, or another indication for surgery exists. colitis from Crohn’s proctocolitis is especially difficult in severe colitis, and primary proctocolectomy would nullify Perforation the future option of a restorative procedure in a patient with ulcerative colitis. Free perforation of the small bowel is also unusual and typi- The need for loop ileostomy combined with decompression cally occurs at or just proximal to a strictured site.32,33 The blowhole colostomy has virtually disappeared with improved most appropriate treatment is resection of the involved bowel medical recognition and more sophisticated management of with immediate or delayed anastomosis. A nondiverted toxic colitis. The operation is still useful in extremely ill anastomosis should be avoided in the setting of delayed patients or those in whom colectomy would be especially treatment, malnutrition, significant comorbidity, or severe hazardous (e.g., contained perforation, high-lying splenic sepsis. Resection with proximal ileostomy has an associated flexure, pregnancy). Contraindications to the procedure mortality rate of 4% compared with 41% with simple suture include colorectal hemorrhage, free perforation, and intraab- closure alone.34 Perforation of the colon in patients with dominal abscess. The operation is considered only a tempo- Crohn’s disease is also rare and typically requires subtotal rizing procedure, and a definitive operation is usually colectomy for optimal management because these cases often performed approximately 6 months later. occur in the setting of severe colitis or steroid usage.35

Hemorrhage Neoplasia Crohn’s disease may be responsible for life-threatening lower Overall, persons with Crohn’s disease are at increased risk for gastrointestinal hemorrhage and even exsanguination, but for- developing cancer compared with the general population. In a tunately this is an infrequent complication.27Ð30 More fre- population-based study from Canada,36 these patients had an quently, entities unrelated to disease involvement, including increased relative risk of developing carcinoma of the small peptic ulcer disease and gastritis, may precipitate intestinal intestine [17.4; 95% confidence interval (CI), 4.16Ð72.9] as bleeding. Accordingly, gastric aspiration and possibly esoph- well as malignancies of the and biliary tract (5.22; 95% agogastroduodenoscopy are required to exclude sources of CI, 0.96Ð28.5), and males were at a particular risk for lym- hemorrhage indirectly associated with Crohn’s disease. The phoma (3.63; 95% CI, 1.53Ð8.62). Their patients with principal management of disease-related hemorrhage is deter- Crohn’s disease were also at increased risk for the develop- mined by the severity and persistence of bleeding as well as ment of colon cancer (2.64; 95% CI, 1.69Ð4.12) and the risk the risk for recurrence. Localization of the bleeding site is was similar to that seen in persons with ulcerative colitis essential regardless of the planned therapy. In a stable patient (2.75; 95% CI, 1.91Ð3.97); the risk for rectal carcinoma, how- with colonic disease, endoscopic evaluation is preferred ever, was similar to that demonstrated by a demographically because this approach allows for disease assessment and ther- matched cohort without inflammatory bowel disease.36 Other apeutic attempts at control of the identified bleeding site. population-based studies have supported the notion that However, indiscriminate usage of for bleeding Crohn’s disease of the colon is associated with an increased colitis should be discouraged because this form of hemor- risk of colorectal cancer,37Ð39 whereas reports from some rhage typically accompanies severe colitis, and colectomy centers40Ð42 have not noted the same association. with ileostomy is advised in this instance, regardless of the In a series of 22 patients with colorectal carcinoma com- endoscopic findings. plicating their Crohn’s disease, 19 (86%) and 9 (41%) had A patient who requires ongoing resuscitation to maintain adjacent or distant dysplasia, respectively, supporting a dys- hemodynamic stability or in whom a small bowel source of plasia-carcinoma sequence in Crohn’s disease.43 When a active bleeding is suspected should undergo emergent mesen- screening and surveillance program was adopted, dysplasia or teric angiography to localize the source of hemorrhage and cancer was detected in 16% of patients, and the probability of arrest ongoing bleeding through selective angiographic infu- detecting dysplasia or cancer after a negative screening sion of vasopressin or embolization. If the hemorrhage is colonoscopy was 22% by the fourth surveillance examination. localized but cannot be controlled by these interventional Accordingly, despite some controversy, many pundits44,45 42. Surgery for Crohn’s Disease 587 advocate that the endoscopic screening and surveillance pro- medical therapy has failed, operative intervention is warranted. tocols used for patients with ulcerative colitis should also be The continuation of ineffective medical management risks the recommended for individuals with Crohn’s disease of the development of further disease complications that may detri- large bowel. Specifically, a screening endoscopy should be mentally impact surgical outcome. performed 8Ð10 years after the onset of disease symptoms and four-quadrant random biopsies should be obtained every 10 cm along the length of the large bowel and directed biop- Operative Considerations sies should be procured from any strictures, lesions, or masses; subsequent surveillance endoscopy with similar biop- Some fundamental observations that must be considered sies should then be performed every 1Ð2 years. The finding when operating for Crohn’s disease are as follows: of multifocal low-grade dysplasia, high-grade dysplasia, or ● Crohn’s disease is incurable invasive cancer would likely warrant review by a second ● Intestinal complications are the most common operative experienced pathologist and confirmation would prompt a indication colectomy. ● Operative options are influenced by myriad factors ● Asymptomatic disease should be ignored Growth Retardation ● Nondiseased bowel can be involved by inflammatory adhe- sions or internal fistulas Abnormal linear growth secondary to delayed skeletal matu- ● Mesenteric division can be difficult ration is frequently encountered in children and adolescents ● Resection margins should be conservative (2 cm) with Crohn’s disease. Specifically, more than half of children may have a subnormal height velocity and approximately Crohn’s disease is a chronic inflammatory disorder that can- one-quarter will have short stature.46 Fortunately, surgical not be cured by medical therapy or operative intervention. resection is often accompanied by growth response and asso- Accordingly, treatment focuses on safely alleviating disease ciated psychologic benefit. symptoms and restoring quality of life while attempting to maintain continuity of the intestinal tract. Of the various oper- Extraintestinal Manifestations ative indications, intestinal complications including strictur- ing or penetrating disease that are unresponsive to medical Extraintestinal manifestations of Crohn’s disease occur in therapy constitute the bulk of the indications, and the opera- nearly one-quarter of patients with Crohn’s disease and can tive options depend on the multiple variables including involve most organ systems.47Ð49 Disorders of the skin, mouth, patient age, anatomic location, disease behavior, symptoms, eye, and joints occur frequently with large bowel disease and prior therapies, nutritional status, comorbid conditions, and their activity typically parallels the degree of intestinal associated sepsis. The patient’s symptoms are especially inflammation. Operative management of the intestinal disease important because the disease encountered at the time of sur- can provide beneficial control of the extraintestinal manifes- gery is often unanticipated despite preoperative evaluation.50 tation. Conversely, abnormalities affecting the hepatic, vascu- In these instances, the findings must be compared with the lar, hematologic, pulmonary, cardiac, or neurologic systems presenting symptoms and signs, and any extensive disease behave independent of the intestinal disease. Other disorders that does not appear to be contributing to symptoms should be such as nephrolithiasis and cholelithiasis are disease compli- typically ignored. Exceptions to this axiom include the man- cations that likely arise from altered intestinal absorption. agement of out-of-circuit bowel and short, uncomplicated strictures, which should be addressed in most Failed Medical Therapy patients. Nondiseased bowel can be affected through inflammatory Antibiotics, probiotics, 5-aminosalicylate compounds, steroids, adhesions or internal fistulas. With adhesions, every attempt immunomodulators, and biologic agents all have a potential should be made to conserve the nondiseased bowel, although role in the management of Crohn’s disease depending on the this can be especially difficult when managing enteroparietal clinical presentation. Each medication within these therapeutic or interloop abscesses. Most internal fistulas are best managed groups possesses appropriate dosing parameters, associated by wedge excision and primary closure of the fistula site in the side effects, and an optimal time interval during which benefi- secondarily affected small bowel. However, a short segmental cial effects should appear. Before initiating treatment with any resection with primary anastomosis may be required for fistu- medication, the patient should be counseled about these fea- las targeting the rectosigmoid region because these often enter tures and objective criteria for disease response should be dis- the bowel at the mesenteric margin and simple wedge excision cussed and then sought after an established time interval. If the may be vulnerable to breakdown of the suture line. desired response is not achieved, prohibitive side effects arise, The mesentery of the diseased bowel is usually thickened or noncompliance is problematic, the medication has failed and because of fat deposition and enlarged mesenteric lymph another medication should be trialed. When all appropriate nodes that straddle the ileocolic and sometimes superior 588 S.A. Strong mesenteric vessels. Attempts at simple division and ligation sometimes the preferred method of managing symptomatic of the vessels may injure the remaining vascular pedicle lead- gastroduodenal Crohn’s disease that is refractory to medical ing to a rapidly spreading mesenteric hematoma that risks dis- treatment where resection would entail extensive reconstruc- tal bowel ischemia. Instead, serial overlapping clamps applied tion of the upper intestinal tract or pancreaticobiliary system. to both sides of the intended transection line provide an ample margin or cuff on the mesenteric edge. Heavy, interlocking Fecal Diversion suture ligatures can then be used to under-run each pedicle caught within the clamps, eliminating concern for a spreading Fecal diversion can be permanent or temporary. Many of the hematoma. Conservative (2 cm), macroscopically normal stomas created to permanently bypass unresected disease fail resection margins are associated with the same rate of opera- to control symptoms secondary to the out-of-circuit bowel, tive morbidity and disease recurrence as extensive (12 cm), and resection is ultimately warranted. High complex fistulas microscopically normal margins.51 However, the luminal dis- and deep ulcerations are among the disease characteristics ease margin can be difficult to judge by inspecting the exterior likely to mandate proctectomy with permanent ostomy for of the bowel. Whereas inspection of the diseased bowel may persistent disease symptoms despite fecal diversion.52 reveal lymphadenopathy, creeping mesenteric fat, and cork- Similarly, temporary diversion intended to heal distal disease screwing of the serosal vessels, the nondiseased bowel may or its sequelae is usually unsuccessful unless combined with appear dilated with muscular hypertrophy and bowel wall a secondary procedure such as a rectal mucosal advancement edema. The key to discriminating between diseased and flap that directly addresses the problem.53 Even for free per- nondiseased bowel lies with palpation of the mesenteric mar- foration of the small bowel, exteriorization of the proximal gin of the bowel wall. A mesenteric ulcer will obscure the pal- bowel alone is rarely the procedure of choice. pable transition from the mesentery to the bowel wall because of fat deposition between the terminal branches of the mar- Strictureplasty ginal vessels. If the surgeon’s fingers passing from the mesen- tery onto the bowel can readily identify the edge of the bowel The incurable and pan-intestinal nature of Crohn’s disease has wall, the luminal mucosa will be macroscopically normal. led to a more conservative operative approach. For patients with multiple strictures of the small bowel, intestinal conser- vation may be maximally achieved by surgically widening the Operative Options narrowed segment by performing a strictureplasty. This tech- nique was initially described by Katariya et al.54 for the suc- The surgical procedures performed for intestinal Crohn’s dis- cessful treatment of tubercular small bowel strictures, and ease can be divided into groups depending on whether resec- later used in strictures secondary to Crohn’s disease.55 The tion of an intestinal segment is performed. The nonresectional procedure safely relieves obstructive symptoms56Ð58 with the procedures include internal bypass, fecal diversion, and stric- operated patients demonstrating weight gain accompanied by tureplasty, whereas the resectional procedures include improved food tolerance as well as discontinuation or resected bowel. Patients often undergo multiple procedures at reduction of steroid usage.59 Moreover, patients undergoing the time of their single operation and these can be a combina- strictureplasty alone are no more likely to require reoperation tion of nonresectional as well as resectional procedures. than those who undergo a concomitant resection,60 and reop- eration rates after first and second operations are also similar.61 Internal Bypass The situations for which strictureplasty is considered are as follows: Internal bypass was the procedure of choice in the early days of surgery for Crohn’s disease when mortality rates associated ● Diffuse involvement of the small bowel with multiple stric- with resection were high because of lack of transfusion tech- tures nology, antimicrobial medications, adequate anesthetic ● Stricture(s) in a patient who has undergone previous major agents, and nutritional support services. However, with the resection(s) of small bowel (>100 cm) advent of these modalities and recognition of complications ● Rapid recurrence of Crohn’s disease manifested as obstruc- such as recrudescent disease, mucoceles, and malignancy tion arising in diverted segments, this procedure was largely aban- ● Stricture in a patient with short bowel syndrome doned. However, bypass operations are still considered rea- ● Nonphlegmonous fibrotic stricture sonable or desirable in specific circumstances. A complicated The contraindications to strictureplasty are as follows: ileocecal phlegmon with dense attachment to the iliac vessels or retroperitoneum can be aptly managed by an exclusion ● Free or contained perforation of the small bowel bypass if the proximal end of the excluded ileal segment is ● Phlegmonous inflammation, internal fistula, or external fis- exteriorized as a small mucus fistula and definitive resection tula involving the affected site is planned to occur in later months. Continuity bypass is ● Multiple strictures within a short segment 42. Surgery for Crohn’s Disease 589

● Stricture in close proximity to a site chosen for resection anterior abdominal wall enables separation of the involved ● Hypoalbuminemia (<2.0 g/dL) intestinal loops and permits closer inspection to determine which segments require resection. Enteric fistulas often Multiple strictures in a patient with an albumin value <2.5 originate from diseased bowel that communicates with g/dL, preoperative weight loss, or advanced age may be nondiseased intestine. Whereas the primary site usually regarded by some as a situation in which strictureplasty requires resection, the secondarily affected bowel segments should be avoided because of concerns of sepsis, but a proxi- are typically treated by conservative wedge excision and mal diverting stoma with multiple strictureplasties should be simple closure of the resultant defect. The diseased bowel considered in this instance.57 Factors that do not seem to be should be resected with conservative margins and the associated with increased operative risk include perforative or mesentery divided using the previously described methods. phlegmonous disease remote from the strictureplasty site, Removal of enlarged mesenteric lymph nodes is not a goal steroid dosage, synchronous resection, number of stricture- of resectional surgery because this practice risks vascular plasties, and length of stricture. injury without reducing the likelihood of recurrent disease. The length of the strictured segment dictates the type of The specimen should be opened after it has been delivered strictureplasty technique used. Short (<10 cm) strictures are from the operative field to assure macroscopic disease-free best managed by a Heineke-Mikulicz type of strictureplasty, resection margins. whereas medium length (10Ð20 cm) strictures can be cor- A laparoscopic approach can be used for a variety of rected by a Finney-type strictureplasty. Long (>20 cm) stric- resectional procedures and is typically associated with tures are best managed by a side-to-side isoperistaltic longer procedure times, but shorter lengths of stay and strictureplasty.62 Regardless the technique, the bowel is briefer periods of recovery.72Ð79 Although disease compli- incised along its antimesenteric margin extending 1Ð2 cm cated by fistulas or phlegmons can prove challenging, expe- beyond the diseased segment, which is identified by the pres- rienced laparoscopic surgeons have been able to safely ence of mesenteric ulceration. Biopsy of any suspicious complete procedures in these instances without converting mucosa is performed to exclude carcinoma63Ð65 and closure is to a laparotomy.80,81 achieved using an absorbable suture in a one- or two-layer After the diseased bowel has been resected, the surgeon manner. The mesentery at each of the strictureplasty sites is must decide whether to create an end stoma, an anastomosis, then labeled with metallic clips to allow discrimination or a diverted anastomosis. In general, an end stoma is desir- between the multiple sites in the unlikely event that postoper- able in patients who are critically ill, demonstrate fecal peri- ative hemorrhage occurs. Selective mesenteric angiography tonitis, or have coagulopathy. An anastomosis can be safely with intraarterial vasopressin infusion will control most created in most other instances assuming a few general prin- bleeding episodes, but the radio-opaque metal clips will help ciples are respected that include the following: avoid the need to open each of the strictureplasty sites to ● Adequate blood supply must be assured localize the bleeding site if reoperation is required.66 ● Tension or torsion are unacceptable Many centers have used a Finney-type strictureplasty for ● Luminal size needs to be equivalent recurrent terminal ileal disease with the anastomosis created ● The mesenteric defect should be closed between the terminal ileum and proximal colon.67Ð70 Others have extrapolated this experience into patients undergoing A temporary diverting stoma should be considered to protect their first operation for terminal ileal Crohn’s disease.71 A long the anastomosis in instances of incompletely drained sepsis, ileocolostomy is constructed encompassing the entirety of the excessive blood loss during a long operation, or severe diseased bowel. Interestingly, subsequent endoscopic and hypoalbuminemia (<2.5 g/dL). imaging studies have revealed complete morphologic disease In operations for terminal ileal disease, the neoterminal regression.71 ileum tends to be the usual site of disease recurrence. Accordingly, the optimal anastomotic configuration and pre- 82Ð84 Resection ferred materials are subject to debate. Some recent retro- spective studies85Ð88 and one prospective, randomized trial89 The basic principles of resection should be followed suggest that larger side-to-side anastomoses are associated whether an open or laparoscopic approach is used, and with a reduced risk for disease recurrence. Although many include mobilization of both diseased intestine as well as investigators have found no association between the materials sufficient nondiseased bowel to facilitate the subsequent used to create the anastomosis and morbidity rates,86,87 at least creation of a tension-free anastomosis or construction of an three studies85,90,91 have reported that a stapled anastomosis is ostomy. Extensive mobilization may facilitate operations for safer than a hand-sewn anastomosis. Regardless, it is impor- terminal ileal disease complicated by fused ileal loops or a tant to use a hand-sewn technique when the bowel wall is phlegmonous mass adherent to matted loops of small bowel, abnormally thickened because the stapling instruments are omentum, or retroperitoneal structures. Delivery of the not designed to safely construct an anastomosis under these ascending colon and terminal ileum into the wound or to the conditions. 590 S.A. Strong

Specific Anatomic Locations resection is particularly ideal for older individuals (>50 years) and patients with colitis who have previously under- Terminal Ileum gone significant small bowel resection (>30 cm). In both instances, preservation of the ileocecal valve and colonic Terminal ileal disease is defined as disease limited to the absorptive surface may protect against diarrhea and dramati- lower third of the small bowel with or without cecal involve- cally improve the functional outcome. Resection with colo- ment. Approximately 20% of patients with Crohn’s disease proctostomy is used for these selected patients with left-sided will express this phenotype, and usually present with symp- disease, and a cecorectal anastomosis is constructed if the toms suggestive of inflammation or obstruction. In the major- transverse colon is also involved. In younger patients and ity of cases, resection with construction of an ileal-ascending those without prior small bowel resection, the diseased seg- colon anastomosis is feasible and desirable. All nondiseased ment and uninvolved proximal colon are resected and an ascending colon should be preserved to provide the largest ileosigmoid or ileorectal anastomosis is constructed. possible surface area for water absorption and to avoid a com- Colonic strictureplasty has been described for short stric- plex fistula involving retroperitoneal structures associated tures and seems to be associated with a morbidity rate, risk for with recurrent disease involving an anastomosis that overlies surgical recurrence, and postoperative quality of life compa- the second portion of the duodenum. Alternatively, this is the rable to that seen with resection.92 However, given the 7% situation in which some centers avoid bowel resection by incidence of malignancy arising in a colonic stricture,93 some creating a large Finney-type ileocolostomy.71 surgeons argue that resection should be exclusively encour- Terminal ileal disease with sparing of the ileocecal valve aged if all of the outcome measures are comparable. and cecum is ideally treated with resection and enteroenteros- Patients with extensive colonic involvement, relative rectal tomy provided there is sufficient length (5Ð7 cm) of normal- sparing, and adequate fecal continence without active anoper- appearing distal ileum after definitive ileal resection. ineal sepsis or compromised rectal compliance are candidates Preservation of the ileocecal valve helps to minimize the risk for colectomy with ileoproctostomy. Rectal compliance can be of postoperative diarrhea. In many instances, a hand-sewn subjectively judged by distending the rectum during proc- anastomosis is preferred because the distal segment may be toscopy or objectively quantified with anorectal physiology too short to accommodate a stapled anastomosis. testing; patients whose maximum tolerated rectal volume measures <150 mL will do poorly with an ileoproctostomy.94 Colon A rare patient presents with pan-colonic disease, significant upper rectal involvement, and sparing of the mid- and distal- Colon disease is any colonic involvement between the cecum rectum. Resection of all disease in this setting leaves an anas- and rectum without small bowel or upper gastrointestinal dis- tomosis only 6Ð8 cm above the anal verge, and is often ease. Nearly 40% of patients have this disease distribution, associated with impaired function secondary to compromised and often complain of inflammatory disease symptoms compliance. Instead, an ileal J-pouch can be configured with including abdominal cramping, bloody diarrhea, and urgency. 10-cm limbs and joined to the spared mid-rectum after subtotal Persons presenting with segmental disease are best treated proctocolectomy. Despite a likely increased disease recurrence with segmental resection to protect against dehydration and compared with that seen with total proctocolectomy and electrolyte imbalances associated with loss of the large intes- ileostomy, the patient may enjoy several years without a stoma. tine’s physiologic role. In patients with disease limited to the Patients with proctocolitis that warrants operative treatment ascending colon, the transverse colon is divided at the level of usually require a total proctocolectomy with creation of an the middle colic vessels so that the mesenteric root naturally end ileostomy, especially those persons with colitis whose separates the anastomosis from the retroperitoneum, mini- proctitis, sphincter dysfunction, or anoperineal sepsis is too mizing the risk for recurrent disease complicated by complex severe for rectal preservation and ileoproctostomy. If proctec- fistulas. Alternatively, a more proximal anastomosis may be tomy is required, the entirety of the rectum should be excised wrapped with a pedicle of omentum, thereby preventing the in a single or staged procedure because of the significant risk anastomosis from lying in direct contact with the retroperi- of cancer developing in the defunctioned rectal stump despite toneum. Disease involving the ascending and transverse surveillance .95 An unhealed perineal wound that colons is treated in a similar manner except an extended right persists 6Ð12 months after endoanal proctectomy should be colectomy is recommended because the mesentery of the evaluated to exclude concomitant pyoderma gangrenosum, ileum is more easily approximated to the mesentery of the sig- perineal sinus, enteroperineal fistula, and malignancy. A sim- moid colon than the descending colon. Resection of the ple shallow wound will usually respond to repeated wound additional colonic segment avoids an internal and does debridements and diligent wound care with vacuum-assisted not adversely affect the functional outcome. Crohn’s disease closure system and split-thickness skin grafts providing addi- of the transverse, descending, and sigmoid colons presents a tional benefit. Wounds complicated by a perineal sinus or situation in which segmental resection and colocolic or enteroperineal fistula require more extensive procedures that colorectal anastomosis is most frequently used. Segmental often include omental, muscle, or myocutaneous flaps.96Ð98 42. Surgery for Crohn’s Disease 591

One center has chosen to offer patients with Crohn’s dis- demonstrate macroscopic abnormalities in the majority of ease isolated to the colon and rectum a total proctocolectomy patients with the antrum most frequently involved.105 Isolated with ileal pouchÐanal anastomosis.99 They have reported that gastric disease is exceedingly rare and any reports of success- the rates of Crohn’s diseaseÐrelated complications and pouch ful treatment are purely anecdotal.106 For duodenal disease, excision are 35% and 10%, respectively, after 10 years of fol- medical therapy is the mainstay of treatment for inflammatory low-up. However, other reports suggest that 12%Ð29% and and penetrating disease, whereas strictures present a different 45%–52% of patients with Crohn’s disease subsequently challenge.107 Ulcer-like lesions are nonspecific, rarely cause require pouch excision 5 and 10 years after restorative proc- stenosis, spontaneously regress, and are usually associated tocolectomy, respectively.100Ð103 Consequently, a restorative with other diseased sites. Contrarily, stenotic duodenal seg- proctocolectomy is usually avoided in the setting of recog- ments are typically unifocal and often respond poorly to med- nized Crohn’s disease, and is typically performed only as part ical management. Endoscopic balloon dilatation has been of a controlled trial. safely used to treat short duodenal strictures, and the proce- dure seems to be well tolerated while providing marked 108,109 Ileocolon symptom relief. In the past, the operative management of duodenal strictures was restricted to gastrojejunostomy Ileocolon disease is disease of the terminal ileum with colonic with or without concomitant .110,111 Protagonists of involvement noted distal to the cecum and proximal to the truncal vagotomy cited the high risk for marginal ulceration rectum. This disease phenotype occurs as often as terminal whereas antagonists raised concerns about postoperative diar- ileal disease, and the operative approach to these patients is rhea. Recently, success with duodenal strictureplasty has been similar to that already outlined for individuals with terminal reported by several centers, and the technique seems to be the ileal or colon disease. Specifically, the surgeon must conserve procedure of choice if the affected bowel is sufficiently supple as much of the nondiseased colon as possible and avoid large and devoid of associated sepsis.112Ð115 mesenteric defects. This often requires the construction of two anastomoses, which does not seem to significantly Anoperineum increase operative morbidity. Crohn’s disease will affect the anus or perineum in as many Upper Gastrointestinal as 61%Ð80% of patients, and typically occurs with or follow- ing the onset of disease in other anatomic locations.116 Upper gastrointestinal disease is defined as any disease loca- Involvement of this area can manifest itself as a fissure, skin tion proximal to the terminal ileum regardless of involvement tag or hemorrhoid, cavitating ulcer, abscess or fistula, in other areas, and represents the phenotype that is often the anovaginal fistula, anorectal stricture, or carcinoma. These most difficult to manage because of its predilection for exten- comprise the basis of the accurately descriptive and compre- sive disease and predominantly stricturing or penetrating hensive Cardiff classification of anal Crohn’s disease,117 behavior. which has not been widely accepted by clinicians because it Small bowel disease proximal to the terminal ileum is often is perceived to be of minimal clinical relevance.118,119 typified by several stenotic segments separated from one Whereas this classification system is solely based on the another by noninvolved bowel. These diseased segments anatomic and pathologic features, scoring systems of disease range in length and can measure >50 cm. The prognosis for activity have been proposed to complement this scheme. Crohn’s disease diffusely involving the small bowel is signif- These include the Perianal Crohn’s Disease Activity Index120 icantly worse than that of localized disease.104 The operative and a newer system intended to evaluate and predict the options in a symptomatic patient with diffuse jejunoileitis outcome of operative management.121 include internal intestinal bypass, strictureplasty, and resec- The evaluation of anoperineal Crohn’s disease should tion. Intestinal bypass is reproved by most clinicians because include a regional examination as well as investigations to of concerns about bacterial overgrowth and malignant degen- determine the extent and activity of disease located elsewhere eration. Resection risks immediate or future short bowel syn- through varied imaging and endoscopic studies. The regional drome and is not generally recommended. An operation that examination may be significantly enhanced by assessment consists of multiple strictureplasties is the procedure of with fistulography,122,123 endoanal ultrasonography,124Ð126 choice using the previously discussed techniques to safely magnetic resonance imaging,127Ð130 or examination under conserve small bowel and relieve symptoms secondary to anesthesia. Comparative reports suggest that these modalities luminal stenosis. The involved segments can be ignored only are associated with comparable accuracy,131,132 and overall in the rare instance in which the diseased intestine appears to accuracy might be best enhanced by combining the results of be inflamed without evidence of stricture or penetration. any two modalities.131 Gastroduodenal Crohn’s disease is relatively rare, and the The first priority of therapy is to drain any associated sep- most common presenting complaints are upper abdominal sis through the insertion of drainage catheters with or without pain and symptoms of duodenal obstruction. Endoscopy will placement of noncutting setons. The second priority focuses 592 S.A. Strong on stabilizing the infectious component using antibiotic ther- from 50% to 80% in series containing at least 20 apy such as metronidazole or ciprofloxacin. In addition, patients,143Ð145 and a history of non-colon Crohn’s disease is a attempts at medical management of the disease process predictor of failure.144 Alternatively, a chronic indwelling are initiated with immunomodulators and biologic agents; noncutting seton can be used in this setting. These setons are 5-aminosalicylic acid compounds and steroids provide little more ideally suited for chronic drainage of a fistula compli- benefit. The third priority is optimization of quality of life cated by rectal inflammation, with proctectomy required in through continued medical therapy or operative intervention 0%Ð33% of patients reported in series composed of at least used individually or in combination. Asymptomatic fissures, 20 patients.146Ð150 Fibrin sealant has also been used in these skin tags, or hemorrhoids are best ignored because surgical situations to obliterate the fistula tract, but success has been treatment may escalate the disease to a point in which proc- limited.151Ð153 In some patients, the severity of rectal inflam- tectomy is eventually required.52,133,134 Cavitating ulcers may mation or extent of perineal sepsis mandates endoanal proc- dramatically improve with operative debridement and intrale- tectomy and permanent fecal diversion.116,142 sional steroid injection in combination with appropriately Anovaginal fistulas are more difficult to manage than aggressive medical therapy. anoperineal fistulas because they often originate from an anal Medical management typically includes antibiotics, ulcer and traverse a short distance through sometimes attenu- immunomodulators, and biologic agents used individually or ated muscle. Fistulas that are not associated with an in combination. Metronidazole (20 mg/kg/day) prescribed for ulcer are usually managed with a rectal mucosal advancement 6Ð8 weeks is associated with a 50%Ð56% healing rate, but flap if the rectal mucosa is noninflamed,145,154 or an anocuta- nearly half of patients will experience disease exacerbation neous flap if the rectum is moderately diseased.155 For women and paresthesias with dosage reduction.135,136 Azathioprine with an anovaginal fistula and anal canal ulceration or severe (2Ð3 mg/kg/day) or 6-mercaptopurine (1.5 mg/kg/day) used proctitis, proctectomy is often required. alone heals 54% of fistulas compared with a 21% healing rate Many clinicians are beginning to use medical therapy in with placebo.137 Three doses of infliximab (5 mg/kg) deliv- combination with operative treatment. They are reporting that ered at 0, 2, and 6 weeks can promote fistula closure in 55% an examination under anesthesia before infliximab treatment of the patients, as compared with 13% of the patients treated accelerates healing156 and infliximab treatment followed by with placebo, and the median length of time during which the definitive surgery has a beneficial additive effect in a multi- fistula remains closed is 3 months.138 However, ciprofloxacin step treatment regimen for the management of complex anal (1000 mg/day) in combination with infliximab tends to be fistulas arising in patients with active proctitis.157 more effective than infliximab alone,132,139 and re-treatment Strictures, which are typically situated at the top of the with infliximab every 8 weeks is more effective than placebo anorectal ring, should be ignored if asymptomatic or gently in maintaining fistula closure.140,141 Lastly, concomitant dilated if associated with complaints suggestive of outlet immunosuppressive therapy with azathioprine, 6-mercaptop- obstruction.158,159 In selected patients with nondiseased rec- urine, or methotrexate may result in improved outcomes tums, a rectal sleeve advancement may be attempted.160 Both because of a reduction in the frequency of human anti- squamous cell carcinoma and adenocarcinoma can compli- chimeric antibody formation, acute infusion reactions, and a cate preexisting anoperineal Crohn’s disease and persons with reduced risk of delayed hypersensitivity-like reactions and chronic involvement may require examinations under anes- formation of antinuclear antibodies. thesia with both directed and random biopsies of chronically The operative management of a perineal abscess or anoper- indurated areas to exclude the possibility of malignant degen- ineal fistula is predicated upon the patient’s baseline conti- eration.95,161Ð164 If a cancer is identified, the lymph node nence, complexity of the fistula, amount of sphincter drainage basin should be closely examined and an oncologic encompassed by the fistula, and severity of rectal involve- resection planned with or without perioperative adjuvant ment. In a review of 21 retrospective studies that focused on therapy depending on the histology and stage of the tumor. fistulotomy for a low-lying fistula, the postoperative inconti- nence rates ranged from 0% to 50%, and 6% to 60% ulti- 142 mately required a stoma. The initial healing rates in these Special Circumstances studies ranged from 8% to 100%, with rates of 80%Ð100% in 13 of 21 studies, 60%Ð79% in five of 13 studies, and <60% Enteroparietal Abscess in three of 21 studies. The clinical scenario best suited for fistulotomy is the continent patient with a simple, low-lying, An enteroparietal abscess is likely best treated by initial exter- posterior fistula without associated rectal disease. nal drainage using a computed tomography (CT)-guided Fistulotomy for an anterior fistula in this setting, especially in catheter if the cavity is accessible or, otherwise, by surgical a woman, may risk incontinence. If fistulotomy is likely to drainage. Conversely, some surgeons suggest that the abscess cause a disturbance in fecal continence in a patient with min- is best managed by laparotomy, resection, and occasional imal rectal inflammation, a rectal mucosal advancement flap anastomosis.165 Antagonists of this approach cite their con- is recommended. The healing rates with this procedure range cerns about short bowel syndrome after laparotomy because 42. Surgery for Crohn’s Disease 593 nondiseased bowel involved by the abscess often requires and omentum is interposed between the bowel and the resid- concomitant resection to manage the abscess.166 Furthermore, ual cavity. Sinography is completed immediately before successful CT-guided drainage procedures obviate the need catheter removal to assure collapse of the cavity. If the cavity for early as well as late operative intervention in nearly half of persists, longer drainage is recommended. patients.166 Patients with large, recurrent abscesses or fistulas after drainage are more likely to require subsequent laparo- Enterocutaneous Fistula tomy, but a fistula does not pose much operative difficulty if resection has been deferred for approximately 6 weeks. Enterocutaneous fistulas can develop before any surgical ther- apy, during the immediate postoperative period, or several 167 Interloop Abscess weeks after an operation. Early postoperative fistulas most likely represent breakdown of an anastomosis or an unrecog- Interloop abscesses, which are considerably smaller than the nized enterotomy. Otherwise, they are the result of active pen- enteroparietal form, are often occult or subtle in presentation etrating disease. This latter presentation is best evaluated by and are usually identified only at the time of resection when imaging studies and endoscopy to determine the extent of separating loops of matted bowel. disease and exclude possible septic foci that would require drainage. Medical therapy is then usually initiated with oper- Intramesenteric Abscess ative management warranted for significantly symptomatic fistulas that are unlikely to heal or fail to heal with medical Intramesenteric abscesses arise from penetrating disease treatment. Low-output fistulas that minimally soak a gauze eroding into the mesentery of the small bowel, colon, or rec- dressing may be managed by nonoperative techniques, espe- tum. In the small bowel, the abscess often dissects between cially in patients with significant operative risk. If an opera- the mesenteric leaves, extending sometimes to the origins of tion is required, the fistulizing segment of bowel is typically the superior mesenteric vessels. Resection of the bowel with diseased or situated proximal to an obstructed segment. a cuff of mesentery carries a particular risk for vascular injury Regardless, the fistulizing bowel and any other disease sites or secondary hemorrhage. Instead, the abscess is identified by are addressed. Wedge excision or strictureplasty of the fistula intraoperative needle aspiration, and the purulent fluid is site is not recommended because of the associated risk for drained back into the small bowel lumen by compression of postoperative leak and recurrent fistula.168 However, stricture- the mesenteric leaves and needle aspiration. An exclusion plasty may be performed in other diseased areas provided the bypass of the involved bowel is then performed by creating previously mentioned guidelines are followed. In a patient proximal and distal mucus fistulas, and constructing an with a complex fistula requiring a prolonged operation with enteroenterostomy above and below the diseased segment to extensive enterolysis, multiple anastomoses, enterotomy restore bowel continuity. The excluded segment is then closures, or strictureplasties, a diverting stoma proximal to all resected 6 months later. Intramesenteric abscesses of the sig- procedure sites is often prudent to allow healing before moid colon or rectum are best managed by external drainage restoration of the normal fecal flow. These persons often combined with diverting end colostomy proximal to the site require home hyperalimentation for 3Ð6 months followed of disease. Resection with anastomosis to the normal rectum by stoma closure after preoperative imaging confirms is performed 6 months later. complete healing of all suture lines and no areas of distal obstruction. Retroperitoneal and Psoas Abscess The management of a fistula developing during the early postoperative period depends on the timing of the presentation Abscesses arising in this anatomic location may be large and and other variables. If the operation was relatively straight- well-circumscribed or poorly localized with the infectious forward and the fistula presents in the first 7Ð10 postoperative process extending deep to the psoas fascia in both caudad and days, re-laparotomy, resection, or fistula repair, and probable cephalad directions. CT-guided drainage is usually first used proximal fecal diversion is warranted. Beyond that time inter- followed by elective resection of the diseased segment. If a val or after a difficult operation, re-laparotomy may be associ- CT-guided approach fails, surgical drainage is warranted. A ated with more harm than benefit because of formidable large, multilocular abscess is best treated by incising over the adhesions and the risk of iatrogenic bowel injury. An operation appropriate site, separating the oblique muscles, localizing is indicated in these patients if they have evidence of sepsis or the abscess by needle aspiration, incising the pyogenic mem- hemorrhage that cannot be managed by interventional radiol- brane, and digitally disrupting any septations. A drainage ogy techniques or potentially life-threatening ischemic bowel. catheter is inserted and continued until the cavity has col- In addition, some individuals with fistulas that are particularly lapsed after 4Ð6 weeks. If the abscess is initially identified at difficult to manage can be aided by a laparotomy whereby the laparotomy, the diseased segment is mobilized, the involved upper abdomen is entered and a segment of jejunum proximal bowel resected, and an anastomosis is usually constructed. to the fistula site is brought out as a diverting stoma. These The abscess is then unroofed and extraperitoneally drained, patients with postoperative fistulas treated nonoperatively will 594 S.A. Strong usually require home hyperalimentation, somatostatin, and ease treated by limited resection with colocolonic or colorec- possible gastric decompression until the fistula has healed.169 tal anastomosis for Crohn’s disease of the large bowel has If the fistula persists despite 6Ð12 months of management, been described by a number of institutions over the past operative intervention is planned after extensive evaluation of 3 decades193Ð201 (Table 42-1). Although the majority of the fistula and intestinal tract. patients will experience symptomatic recurrence, more than 75% will maintain intestinal continuity for more than a Enteroenteric Fistulas decade after their initial resection with anastomosis. Despite the symptomatic recurrence rate, it is important to recall that Enteroenteric fistulas are the most common type of internal segmental colonic resection delays the need for permanent fistula arising in people with Crohn’s disease, and they have ileostomy and partially conserves a portion of the large intes- been reported to occur in 33% of patients whereas external tine’s functional absorbing surface. Crohn’s disease of the fistulas affect 15% of people.170 Isolated enteroenteric fistulas colon with relative rectal sparing can be adequately treated by usually cause few symptoms unless obstructive or septic com- colectomy with ileoproctostomy as described earlier. Longo plications dominate the clinical presentation. However, nearly and colleagues202 reviewed the Cleveland Clinic’s experience 40% of patients with internal fistulas initially managed by using this technique. The procedure was safely performed in nonoperative methods will require surgery within 1 year, 118 patients over a 26-year period. After an average 10 years mainly because of disease intractability.171 The principles of of follow-up, 61% of patients maintained intestinal continuity surgical management include resection of the fistula source, with a functioning ileoproctostomy. The success of this oper- freshening of the defect in the adjacent bowel loop by wedge ation is independent of patient age and duration of symptoms, excision, and transverse closure of the defect. Primary bowel but inversely linked, in part, to the presence of concomitant anastomosis can usually be safely performed after resection small bowel disease at the time of anastomosis. Many other of the diseased segment is completed. However, certain clini- authors have reported similar favorable find- cal situations may pose particular difficulties.172 If a phleg- ings.197,199,200,202Ð207 (Table 42-2) One of the most common monous reaction involving the rectosigmoid region is part of components of Crohn’s disease that manifests itself after an ileosigmoid fistula, suture closure of the sigmoid defect proctocolectomy is recurrence of disease in the ileostomy or may be vulnerable to breakdown. Instead, a limited sigmoid remaining small bowel. Scammell and associates reported a resection with primary anastomosis should be performed 24% and 35% cumulative reoperative rate for recurrence at because the likelihood of anastomotic dehiscence is negligi- 5 and 10 years, respectively.208 The majority of recurrences ble.173 In these cases, the sigmoid colon is diseased in nearly occurred within 25 cm of the stoma. Although the rates vary, 40% of patients and the reoperative recurrence rate is signifi- these values largely agree with the experience of others.199,206 cantly increased when preoperative endoscopy is omitted.174 Various forms of medical therapy have been trialed to prevent the likelihood of recurrent Crohn’s disease after operative management, but no clear prophylactic drug Recurrence regime has emerged. First-line therapy generally consists of the 5-aminosalicylic acid compounds, which are only mildly Within a few decades of the first description of regional ileitis, protective and a recent metaanalysis of studies addressing this the recurrent nature of the disease was recognized.175 One year indication suggested that they were no better than placebo.209 after an initial resection, 60%Ð80% of patients possess endo- Second-line treatment includes immunomodulator medica- scopic recurrence, 10%Ð20% experience clinical relapse, and tions that are potentially beneficial in postoperative patients 5% demonstrate operative recurrence.176 Physicians from with high risk for recurrence, endoscopic lesions noted in many centers have tried to elucidate those factors responsible the neoterminal ileum, or disease-related symptoms.210,211 for its recurrent nature, but several of the parameters that ini- tially were thought important in predicting disease recurrence have ultimately proved to be unrelated including age of disease TABLE 42-1. Recurrence after segmental colonic resection onset,177Ð185 gender,177Ð185 anatomic location,177Ð179,181,182,185 duration of preoperative symptoms,180,183,186Ð188 previous resec- Recurrence Follow-up tion,189,190 operative indication,178,179,185 and blood transfu- Author No. of patients (%) (y) sion.187,191,192 Although disease behavior may impact the de Dombal et al.193 42 37 15 194 likelihood of recurrence,182 tobacco usage has been almost uni- Sanfey et al. 13 8 7 Stern et al.195 5205 formly linked to recurrence. Specifically, smoking is an inde- Longo et al.196 18 62 5 pendent risk factor for symptomatic, endoscopic, and Allan et al.197 36 66 15 operative recurrence.4Ð7 Prabhakar et al.198 33 42 14 The choice of operation might also impact the likelihood Bernell et al.199 134 49 10 200 of recurrent disease, and various operative options may Andersson et al. 31 39 11 Martel et al.201 84 43 9 potentially affect the recurrence rate. Segmental colonic dis- 42. Surgery for Crohn’s Disease 595

TABLE 42-2. Recurrence after total colectomy and ileoproctostomy Canadian Mesalamine for Remission of Crohn’s Disease Study Recurrence Follow-up Group. Gastroenterology 1998;114:1143Ð1150. 6. Ryan WR, Allan RN, Yamamoto T, Keighley MR. Crohn’s dis- Author No. of patients (%) (y) ease patients who quit smoking have a reduced risk of reoper- 197 Allan et al. 63 53 15 ation for recurrence. Am J Surg 2004;187:219Ð225. 202 Longo et al. 131 65 10 7. Kane SV, Flicker M, Katz-Nelson F. Tobacco use is associated Flint et al.203 37 41 6 with accelerated clinical recurrence of Crohn’s disease after Buchmann et al.204 105 30 8 Ambrose et al.205 63 48 10 surgically induced remission. J Clin Gastroenterol 2005;39: Goligher206 47 49 15 32Ð35. Martel et al.207 39 41 10 8. Rutgeerts P, Goboes K, Peeters M, et al. Effect of fecal diver- Bernell et al.199 106 53 15 sion on recurrence of Crohn’s disease in the neoterminal ileum. Andersson et al.200 26 46 9 Lancet 1991;338:771Ð774. 9. Gordon JN, Sabatino AD, Macdonald TT. The pathophysio- logic rationale for biological therapies in inflammatory bowel Third-line therapy would likely include nitroimidazole antibi- disease. Curr Opin Gastroenterol 2005;21:431Ð437. otics that prevent early endoscopic recurrence and postpone 10. Binder V. Epidemiology of IBD during the twentieth century: symptomatic relapse, but are not well tolerated.212 an integrated view. Best Pract Res Clin Gastroenterol 2004; 18:463Ð479. Conventional corticosteroids, budesonide, and probiotics 11. Loftus EV Jr. Clinical epidemiology of inflammatory bowel have been shown to ineffectively protect against postoperative disease: incidence, prevalence, and environmental influences. disease recurrence, and the biologic agents have not been Gastroenterology 2004;126:1504Ð1517. appropriately trialed. 12. Farmer RG, Hawk WA, Turnbull RB. Clinical patterns in Crohn’s disease: a statistical study of 615 cases. Gastroentero- logy 1975;68:627Ð635. Summary 13. Gasche C, Scholmerich J, Brynskov J, et al. A simple classifi- cation of Crohn’s disease: report of the Working Party for the Crohn’s disease remains a chronic, incurable disorder that World Congresses of Gastroenterology, Vienna 1998. Inflamm presents unique challenges to the surgeon. The proper care of Bowel Dis 2000;6:8Ð15. these patients requires a thorough interview, examination, and 14. Louis E, Collard A, Oger AF, Degroote E, Aboul Nasr El Yafi FA, Belaiche J. Behaviour of Crohn’s disease according to the evaluation because the presenting symptoms and signs can be Vienna classification: changing pattern over the course of the quite subtle yet profoundly significant. Multiple factors must disease. Gut 2001;49:777Ð782. be considered to allow development of an appropriate treat- 15. Cosnes J, Cattan S, Blain A, et al. Long-term evolution of dis- ment plan. Medical therapy often precedes or complements ease behavior of Crohn’s disease. Inflamm Bowel Dis operative management, and although resection remains the 2002;8:244Ð250. principle operation of choice, the nonresectional techniques 16. Freeman HJ. Natural history and clinical behavior of Crohn’s are often required to allow bowel conservation. The recurrent disease extending beyond two decades. J Clin Gastroenterol nature of the disease mandates that we continue to search for 2003;37:216Ð219. alterations in operative techniques and innovative medical 17. Papi C, Festa V, Fagnani C, et al. Evolution of clinical behav- therapies that reduce the need for repeat operations. iour in Crohn’s disease: predictive factors of penetrating com- plications. Dig Liver Dis 2005;37:247Ð253. 18. Fedorak RN. Is it time to re-classify Crohn’s disease? Best References Pract Res Clin Gastroenterol 2004;18(suppl):99Ð106. 19. Santos JV, Baudet JA, Casellas FJ, Guarner LA, Vilaseca JM, 1. Brant SR, Shugart YY. Inflammatory bowel disease gene hunt- Malagelada JR. 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