© 2009 Decker Intellectual Properties Inc Scientific American Surgery GASTROINTESTINAL TRACT AND ABDOMEN 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 dis- ease. 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 diagno- sis. 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 11/09 © 2009 Decker Intellectual Properties Inc Scientific American Surgery GASTROINTESTINAL TRACT AND ABDOMEN FULMINANT ULCERATIVE COLITIS — 2 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. 11/09 © 2009 Decker Intellectual Properties Inc Scientific American Surgery GASTROINTESTINAL TRACT AND ABDOMEN FULMINANT ULCERATIVE COLITIS — 3 medical therapy. Narcotics, antidi- arrheal agents, and other anticho- linergic 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 recom- mended 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