91 Ulcerative

Ulcerative colitis (UC) is an inflammatory disease of the UC, it must be assumed that the cause is malignant until and colon. The disease is characterized by proven otherwise. The risk of developing colon cancer mucosal ulcerations beginning in the rectum and extend- increases with the extent of disease, disease duration, ing proximally to the colon. A continuous pattern of presence of dysplasia, and presence of primary sclerosing inflammation is present in contrast to the skip lesions cholangitis. The risk for colon cancer increases by 1% to found in Crohn’s disease. The etiology has been attrib- 2% each 10 years with pancolitis. The degree of dyspla- uted to dietary factors, immune dysfunction, and genet- sia also has a directly proportional increased risk of ics, and patients with UC have elevated levels of cancer (i.e., low grade ∼10%, high grade ∼40%). Fibrosis inflammatory cytokines. The disease occurs equally in of the extrahepatic and intrahepatic biliary ducts results both sexes and can appear in any age group. However, in primary sclerosing cholangitis. It is associated more so there are peak onsets occurring during the second and with UC than Crohn’s disease; furthermore, 70% of fourth decades. patients with primary sclerosing cholangitis also have The disease pattern of UC is quite characteristic. UC. The presence of primary sclerosing cholangitis Rectal involvement is nearly always present, with vari- indicates a fivefold greater risk of cancer than UC alone. able extension into the colon.The inflammation is limited Toxic is a potentially life-threatening compli- to the mucosal and submucosal layers, which is in con- cation of UC. Patients generally present with a septic trast to the transmural nature of Crohn’s disease. The picture, including abdominal distension, tenderness, mucosa takes on a granular and swollen appearance or , , and leukocytosis. Extraintestinal man- can appear denuded in the case of long-standing disease. ifestations consist of peripheral arthritis affecting the At times pseudopolyps may be seen, which represent knees and ankles and ankylosing spondylitis. These areas of regenerated inflamed mucosa. Microscopic conditions tend to dissipate after . features include polymorphonuclear infiltration into the Complete blood cell count, erythrocyte sedimentation crypts of Lieberkühn, multiple crypt abscesses, and rate, and stool studies are ordered to rule out other mucosal ulcerations. causes. Upper gastrointestinal imaging with small bowel and rectal bleeding are the most common follow-through should be completed to help differentiate presenting symptoms. Stools occur frequently and tend to between Crohn’s disease and UC. Perinuclear antineu- be bloody and mucoid in nature. Nocturnal diarrhea is trophil cytoplasmic antibodies are serologic markers that associated with extensive disease. Other symptoms have shown to be specific for UC. The diagnosis of UC is include left lower quadrant (caused by confirmed by colonoscopy. Endoscopic findings include involvement of the left colon), fever, weight loss, fecal friable-appearing mucosa to complete ulceration with urgency, and tenesmus. edema and bleeding. Multiple sequential biopsy speci- Complications of the disease include strictures, toxic mens are taken every 10cm to inspect for dysplasia. megacolon, colon cancer, and extraintestinal manifesta- Surgical management has offered the only cure for UC. tions. Strictures can have two etiologies, benign and This is in contrast to Crohn’s disease, which cannot be malignant. Benign strictures are the result of muscularis cured surgically. Medical therapies generally consist of mucosa hypertrophy. Features suggestive of malignant mesalamine, , , and immunosup- strictures include occurrence late in the disease, proximal pressants. Routine surveillance colonoscopy is recom- location to the splenic flexure, and associated large bowel mended every 1 to 2 years for pancolitis with duration obstruction. Because of the increased risk of cancer with greater than 8 years. For patients with disease not extend-

217 218 Part XII. Gastrointestinal Disorders ing beyond the left colon, surveillance may be delayed The basic approach is a staged procedure beginning with until 15 to 20 years after presentation. Indications for abdominocolectomy, , and, if possible, rectal surgery include disease refractory to medical therapy, mucous fistula. The mucous fistula decompresses the toxic megacolon, massive hemorrhage, the presence of rectum and makes identification of the rectum easier at dysplasia, and cancer. a later time. After recovery, the proctocolectomy can be The choice of surgical procedure is determined by the completed with removal of the rectum along with extent of disease and by the presence of complications. ileostomy take down and ileal pouch anal anastomosis. The most common indication for surgery is intractable The delayed procedure reduces the risk of pelvic disease. In this case, a total proctocolectomy with ileal that may occur if proctectomy is completed in the pres- pouch anal anastomosis is the gold standard and offers ence of toxic megacolon. The confirmed discovery of the advantage of avoiding a permanent stoma. In the case high-grade dysplasia in biopsy material is an absolute of toxic megacolon, surgery is undertaken only after indication for total proctocolectomy. It remains contro- failure of medical therapy (intravenous hydration, broad- versial whether to recommend surgery in the presence of spectrum antibiotics, steroids, and immunosuppressants). low-grade dysplasia.