Samantha Hendren
Presentation
A 28-year-old man with a history of ulcerative colitis (UC) diagnosed 13 months ago presents to the emergency department with bloody diarrhea and abdominal pain. His symptoms worsened 2 weeks ago, and his outpatient gastroenterologist started him on Prednisone 40 mg daily, in addition to his usual medications, pentasa and azathioprine. He is now having about 12 to 15 bloody bowel movements every 24 hours. This is his third hospital admission since diagnosis, with the most recent 3 months ago. Laboratory testing reveals hemoglobin of 9.8 g/dL, erythrocyte sedimentation rate (Westergren) of 43 mm/h, C-reactive protein of 12.06 mg/dL, and albumin of 2.8 g/dL.
Differential Diagnosis
This patient presents with an exacerbation of UC, superimposed on a relatively aggressive disease course since diagnosis. While medically refractory disease is the most likely diagnosis, it is important to remember that superimposed infectious colitis including Clostridium difficile colitis affects up to 30% of UC patients presenting with acute exacerbations of their disease. As such, ruling out superimposed infection and optimizing medical therapy is an essential step prior to proceeding with surgical therapy. A misconception among surgeons is that surgical therapy is inevitable for severe UC. On the contrary, recent case series have shown colectomy rates as low as 20% at 2 years for patients with severe UC. Long term, about 30% of all UC patients undergo colectomy, although this is likely decreasing over time due to improvements in medical therapy.
The consulting surgeon has a responsibility to recognize and offer immediate surgical treatment for toxic megacolon, characterized by the following signs and symptoms: abdominal distention, tenderness, fever, leukocytosis, more than 10 bowel movements per day, continuous bleeding, transfusion requirement, hypoalbuminemia, radiologic evidence of colonic wall thickening, and possible dilatation (not always present).
Workup
The patient is admitted to the medicine service, and the gastroenterologists perform a computed tomography scan (CT scan, Figure 1) and a colonoscopy. The colonoscopy reveals pancolitis with ulcerations, granularity, and distorted vascular pattern.
FIGURE 1 • Radiologic Appearance of Severe Ulcerative Colitis.
Stool samples were sent for C. difficile toxin and antigen, and biopsies for cytomegalovirus infection were obtained at colonoscopy; both these were negative. The patient was offered treatment with the anti-TNF agent, infliximab, and surgery was also consulted to introduce the principles of surgical treatment to the patient. He did not have signs or symptoms of toxic megacolon.
As the surgical consultant, important features of the history and physical examination include the following: reviewing documentation of all prior colonoscopy, pathology, and radiologic testing to insure there is no evidence of Crohn’s disease (such as small bowel disease), or dysplasia/malignancy that might alter the surgical approach. It is also essential to ask about any history of anorectal surgery or abscess-fistula disease, assessing continence (keeping in mind that some fecal incontinence during a severe flare is common), and quality of life related to disease activity and medical therapy. A patient who is unable to work due to frequent disease flares or toxic effects of medical treatments should be considered for surgery. Physical exam for signs of toxic colitis and for anal sphincter integrity and signs of any current or prior abscess-fistula disease are also essential.
Diagnosis and Treatment
Based on the workup, this patient has medically refractory UC. The principles of treatment include consideration of salvage medical therapy versus surgical treatment. The decision making between these two options should include a multidisciplinary approach, and the patient should always be introduced to the option of surgery at this point. The patient elected to proceed with infliximab treatment and was discharged after an improvement but not resolution of his symptoms on infliximab and high-dose steroids. Unfortunately his symptoms persisted at home, with bloody bowel movements, abdominal pain, and malaise. His gastroenterologist calls you and asks you to consider surgical treatment at this point.
Surgical Approach
The goal of surgery for UC is removal of the entire colon and rectum, which can be immediately performed, or performed in a staged fashion. Both a total proctocolectomy with end ileostomy and reconstructive surgery with a pelvic pouch provide good quality of life, but most patients prefer reconstructive surgery to avoid a permanent ostomy. Since the 1980s, the most popular operation for medically refractory UC has been the ileal pouch anal anastomosis operation (IPAA, also called restorative proctocolectomy), usually with a J-pouch. This operation is associated with high but acceptable complication rates, good quality of life, and an overall success rate of about 90% long term (Figure 2).
FIGURE 2 • Ileal pouch configurations
The decision of whether to perform UC surgery in 1-stage, 2 stages, or 3 stages is influenced by several factors: patient presentation, medications (especially anti-TNF agents and high-dose steroids, which may be associated with higher complication rates), nutritional status, comorbidities, and technical issues encountered at the time of surgery (such as the degree of tension on the pouch-anal anastomosis). For the patient in this scenario, a 2- or 3-stage approach beginning with a subtotal colectomy is probably the safest option, given recent administration of infliximab, malnutrition, and steroids—all of which may increase anastomotic leak rate if IPAA is performed immediately. An alternative of IPAA with diverting ileostomy could be considered.
A subtotal colectomy for UC can be performed laparoscopically or open. The key technical features include preoperative marking of the ileostomy site with the patient sitting and supine (avoiding folds and scars), and avoiding pelvic dissection to maintain the virgin tissue planes for the next stage of surgery. For severe colitis in which rectal stump leak is a concern, consider a mucus fistula of Hartmann’s pouch, suturing the pouch to the abdominal wound so a leak can drain via the wound, or placing a temporary draining rectal tube to minimize pressure in the stump.
The steps of the routine IPAA procedure are outlined in Table 1. These include resection of the entire or the remaining colon and rectum, mobilization of the small bowel and its mesentery, creation of a 15- to 20-cm long J-pouch, suturing or stapling the pouch to the anus, and performing a diverting loop ileostomy in most cases. In the case of a double-stapled technique, the rectum is stapled and divided at the level of the levator ani muscles, and then the pouch is stapled to the anal canal using the EEA stapler. In the case of the hand-sewn pouch-anal anastomosis, the distal rectum is divided, the mucosa above the dentate line is stripped transanally, and the pouch is pulled through the rectal cuff and hand sutured at the dentate line (Figure 3).
TABLE 1. Key Technical Steps and Potential Pitfalls in lleal Pouch Anal Anastomosis (IPAA)
FIGURE 3 • Ileal pouch–anal anastomosis. (A) Sutured anastomosis at dentate line after mucosectomy. (B) Double-stapled anastomosis. There is a residual 1- to 2-cm cuff of rectal mucosa.
Special Intraoperative Considerations
The key intraoperative problem for which the surgeon must be prepared is the possibility that an ileal J-pouch will not reach to the anal canal for anastomosis. This is a particular problem for tall or obese male patients. In these cases, maneuvers to create length must be performed, including division of peritoneum overlying the mesentery and selective ligation of the mesenteric vessels with transillumination to ensure there are collateral pathways for blood flow to the entire small bowel. Usually, ileocolic artery ligation will result in sufficient length, but if not consider temporary occlusion of vessels (e.g., with a bulldog clamp) prior to selective ligation. If reach is still a problem an S-shaped or W-shaped pouch may be considered, as these may reach better depending on the individual’s mesenteric vascular anatomy.
Another pitfall is the finding of evidence of Crohn’s disease at the time of surgery. While IPAA for highly selected Crohn’s colitis patients is performed in specialty centers, small bowel or anal Crohn’s disease is a contraindication to IPAA. Ileorectal anastomosis or ileostomy should be performed. Patients must be informed about these potential pitfalls prior to surgery.
Postoperative Management
Complications are common after IPAA. The ACS-NSQIP database shows that 24% of patients had major complications and 17% had minor complications within 30 days after IPAA, most commonly superficial and organ space surgical site infections. The most feared early complication is anastomotic leak, for which management depends on the severity of presentation and whether or not ileostomy was performed at the original operation. Some combination of broad-spectrum antibiotics, percutaneous drainage, diverting ileostomy, and open washout may be required depending on the situation. While there is controversy about the value of diverting ileostomy with IPAA, most procedures are performed with diversion due to concern for increased risk of pelvic sepsis without diversion, and possible poor functional outcome after pelvic sepsis.
Long-term complications after IPAA include pouchitis, bowel obstruction, and female infertility. Long-term septic complications include anastomotic strictures and fistulas. Pouch failure occurs in approximately 10% of patients, due to septic complications, chronic pouchitis, or other complications. Frequent bowel movements are not a complication of IPAA, but rather the expected functional outcome. Five to seven loose bowel movements per day with at least one at night and some nighttime leakage is a reasonable functional result, and patients must be well informed preoperatively. Follow-up for dysplasia of the pouch-anal anastomosis is usually recommended beginning 8 to 10 years after onset of UC.
TAKE HOME POINTS
· Surgical treatment should be considered for medically refractory UC, and decision making is multidisciplinary.
· Prior to surgery, medical treatment should be optimized, including ruling out superimposed infectious colitis.
· Evaluate for signs and symptoms of toxic colitis and Crohn’s disease.
· Consider a 3-stage approach with initial subtotal colectomy for ill patients.
· Ileal pouch anal anastomosis is the most common surgical treatment for UC.
· Be prepared with lengthening procedures and alternative pouch configurations for cases in which the pouch will not reach the anus.
· Diverting ileostomy is usually performed.
· Complications are common after IPAA, but there are 90% long-term success rates.
SUGGESTED READINGS
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