A 45-year-old man with a 15-year history of ulcerative colitis (UC) is evaluated in the outpatient office with chronic bloody diarrhea over the past 6 weeks. The patient’s vital signs are unremarkable. His hemoglobin level is 11.0 g/dL. His current medications consist of prednisone and mesalamine (a 5-aminosalicylate derivative), and he recently completed a course of cyclosporine therapy 2 months ago for another bout of disease flare-up. The patient has been unable to maintain full-time employment over the past year because of UC exacerbations. Previous colonoscopy has shown that his disease extends from the rectum to the cecum.
What should be your next step?
What is the best therapy?
ANSWERS TO CASE 51: Ulcerative Colitis
Summary: A 45-year-old man has pancolonic chronic UC that is refractory to medical management and causes significant disability.
• Next step: The option of surgical therapy should be presented to this patient. The discussion should explain the benefits, risks, and limitations of surgery versus those of continued medical therapy.
• Best therapy: Proctocolectomy with ileal pouch-anal anastomosis.
1. Become familiar with the clinical presentation, natural history, medical management, and complications of UC.
2. Become familiar with the indications for urgent and elective operations for the treatment of UC.
3. Be aware of the surgical options and their outcomes for the treatment of UC.
Ulcerative colitis is a chronic disease with variable disease severity and variable involvement of the different colonic segments. The symptoms associated with this disease generally respond to medicated enemas or systemic therapy. When a 45-year-old man presents with a 15-year history of pancolitis and disabling symptoms that have been refractory to medical management, the discussion regarding treatment should explain medical as well as surgical options. Surgical excision of the diseased colon and rectum would lead to resolution of the gastrointestinal (GI) symptoms associated with UC. However, the operation would result in permanent changes in bowel function and body image. It is essential to convey to the patient that surgical excision will not resolve the extraintestinal manifestations of UC. Ongoing medical therapy for these symptoms will more than likely be required. Another important consideration for this patient is cancer risk in the setting of chronic UC. This risk is increased with disease extent and duration. Proctocolectomy with ileal reservoir reconstruction can improve the quality of life and virtually eliminate the colorectal cancer risk in properly selected patients.
APPROACH TO: Ulcerative Colitis
FULMINANT COLITIS AND TOXIC MEGACOLON: Fulminant colitis is a condition characterized by abdominal pain, fever, and sepsis that most commonly develops in the setting of UC but occasionally occurs in the settings of Crohn colitis and pseudomembranous colitis. Toxic megacolon occurs when the preceding findings are associated with radiographic evidence of colonic distension (>6 cm). The cecum is the most frequent site of distension. Patients can become extremely ill with clinical signs of sepsis, and this clinical entity can be highly lethal if not promptly recognized and treated. When identified with either condition, patients require prompt fluid resuscitation and the initiation of broad-spectrum antibiotic therapy, with maximal medical treatment. Colectomy is indicated if the patient fails to respond to medical therapy. A third of patients generally go on to require colectomy for this complication.
DYSPLASIA: Premalignant transformation of the mucosa caused by chronic UC. The risk of cancer associated with dysplasia varies depending on the severity of the dysplastic changes. Roughly 40% of patients with high-grade dysplasia harbor synchronous cancer, and 20% of patients with low-grade dysplasia harbor synchronous cancer.
DYSPLASIA-ASSOCIATED LESION OR MASS: A sessile pseudopolyp arising from dysplastic mucosa affected by chronic UC. Fifty percent of patients with these lesions have carcinoma. Patients with this finding should undergo colorectal resection.
PANCOLITIS: Ulcerative colitis that involves the rectum and the entire colon. Patients with this pattern of disease have a significant risk for the development of subsequent colorectal cancers.
ILEAL J-POUCH: A neorectum created with the terminal ileum in the shape of a J. This is then anastomosed to the anus to form the ileal pouch-anal anastomosis.
POUCHITIS: Idiopathic inflammation of the ileal pouch that can develop following ileal reservoir reconstruction. Patients can present with any number of symptoms, including increased stool frequency, fecal urgency, incontinence, watery diarrhea, bleeding, abdominal cramps, fever, and malaise. Bacterial overgrowth can be a contributing factor for pouchitis, and therefore some patients respond to antibiotic therapy.
Ulcerative colitis is an inflammatory condition of unknown etiology. Unlike Crohn disease, which is a transmural process, the disease involvement in UC is limited to the mucosa. The distribution begins in the rectum and extends to the proximal colon, with occasional extension to the terminal ileum (backwash ileitis). With chronic inflammation of the colonic mucosa, there is loss of water absorption and normal motility, leading to watery diarrhea, crampy abdominal pain, tenesmus, and urgency. A number of extraintestinal manifestations are associated with UC, including ankylosing spondylitis, uveitis, scleroderma, sclerosing cholangitis, arthritis, dermatomyositis, and hypercoagulable states. Surgical therapy virtually eliminates the symptoms related to the diseased colon and rectum; however, the benefits of surgery for the extraintestinal manifestations have not been established (Table 51–1). In fact, some reports have suggested that extraintestinal disease can be aggravated by removal of the colon and rectum.
Table 51–1 • SURGICAL OPTIONS FOR ULCERATIVE COLITIS
The medical management of UC consists of anti-inflammatory therapies of escalating intensity coupled with the administration of antibiotics. When antibiotics and anti-inflammatory agents fail, steroid agents are the next line of therapy. The long-term use of steroids can be effective in reducing the symptoms associated with UC but can lead to immunosuppression, accelerated bone loss, hirsutism, masculinization, osteoporosis, aseptic necrosis, glucose intolerance, and loss of muscle mass. Short- and long-term steroid use increases the morbidity associated with surgical treatment. Newer studies are examining the role of infliximab, the monoclonal anti-TNF (tumor necrosis factor) antibody, in treating UC.
The main indications for surgical therapy in UC are fulminant colitis or toxic megacolon, dysplasia or cancer, and intractable disease. The most commonly performed operation for fulminant colitis is total abdominal colectomy with end ileostomy. Because the colorectal cancer risk increases with chronic UC, patients with duration of disease of more than 7 to 9 years should undergo annual or biannual surveillance colonoscopy with biopsies. If a surveillance program is not instituted, they should be considered for total proctocolectomy. The majority of patients with UC undergoing surgery do so because of disease intractability. This is determined on the basis of disease symptomatology and tolerance to medical therapy. In the elective setting, surgical options include total proctocolectomy with permanent end ileostomy, total proctocolectomy with continent ileostomy, or total proctocolectomy with ileal pouch-anal anastomosis. The latter two operations, although restoring continence, are associated with many more complications than the end ileostomy. The decision as to which procedure to perform is ultimately up to the patient after receiving extensive preoperative counseling.
51.1 A 35-year-old woman with ulcerative colitis for approximately 15 years underwent a colonoscopy revealing an area of colonic dysplasia described as high grade. Which of the following is the best management for this patient?
A. Surgical resection of colon and rectum.
B. Intensive medical therapy and reevaluation with colonoscopy in 3 months.
C. Increase surveillance to every 6 months.
D. Add an immunosuppressive agent to the medical therapy.
E. Initiate chemoprevention therapy with COX2 inhibitor.
51.2 A 40-year-old woman with a 15-year history of chronic diarrhea and a diagnosis of UC is referred for consideration for total proctocolectomy with ileal pouch-anal anastomosis to eliminate future cancer risks. During the colonoscopy, you notice that the disease involves the entire colon and terminal ileum, with sparing of the rectum. Which of the following is the most appropriate treatment?
A. Proctocolectomy with ileal pouch-anal anastomosis
B. Total abdominal colectomy with ileal-rectal anastomosis
C. Repeated biopsy of the rectum and involved portions of the colon and ileum
D. Total proctocolectomy with the construction of continent ileostomy
E. Total colectomy and ileal pouch-rectal anastomosis
51.3 A 46-year-old woman underwent total abdominal colectomy and ileostomy for a severe bout of colitis and sepsis 1 year ago. She has recovered and now desires restoration of GI tract continuity. Which of the following findings would be considered a contraindication for completion proctectomy and ileal pouch-anal anastomosis?
A. The finding of high-grade dysplasia in the rectal segment at 10 cm from the anal verge
B. The finding of high-grade dysplasia with carcinoma in situ in the previously resected colon
C. The finding of granulomatous changes and transmural inflammation in the previously resected colon
D. A prior history of alcohol-induced acute pancreatitis
E. Presence of mucosal ulceration without dysplasia in the remaining rectum
51.1 A. High-grade dysplasia found on colonic surveillance in a patient with ulcerative colitis is usually treated with total proctocolectomy because of the increased frequency of subclinical cancers or to avoid the future development of cancer.
51.2 C. Noninvolvement of the rectum should raise a suspicion of possible Crohn disease, which is a contraindication to performing total proctocolectomy and ileal pouch-anal reconstruction. Repeated colonoscopy and biopsy are indicated in this case.
51.3 C. Ulcerative colitis is a mucosal-bound disease, and these pathological changes described are more compatible with Crohn disease. Patients with Crohn colitis are not candidates for ileal pouch reconstruction because of the likelihood of disease development in the ileal pouches. The presence of cancer or dysplasia in the rectum away from the planned area of resection is not a contraindication to ileal pouch-anal reconstruction.
The main indications for surgical therapy in UC are fulminant colitis or toxic megacolon, dysplasia or cancer, and intractable disease.
Extraintestinal manifestations of UC include ankylosing spondylitis, uveitis, scleroderma, sclerosing cholangitis, arthritis, dermatomyositis, and hypercoagulable states for which surgery is not effective.
Surgical and medical treatments for UC are complementary, not competing, modalities.
The term “refractory to medical therapy” is not strictly defined and should also refer to the failure of appropriate medical therapy as well as patient intolerance to the adverse effects of medical therapy.
Transmural processes or sparing of the rectum should make one suspicious of Crohn disease rather than UC.
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Spencer MP, Melton GB. Management of chronic ulcerative colitis. In: Cameron JL, Cameron AM, eds. Current Surgical Therapy. 10th ed. Philadelphia, PA: Elsevier Saunders; 2011:136-139.