Proctocolectomy with Ileal Pouch-anal Anastomosis (IPAA): Complications, Pouchitis Management, Psychosocial Issues, and Surveillance

A restorative proctocolectomy with ileal pouch-anal anastomosis (RPC-IPAA,) also called a J-pouch as discussed in previous issues, is often the procedure of choice for patients with ulcerative colitis (UC). The procedure removes the entire colon and rectum while preserving the anal sphincter and, hence, normal bowel function and fecal continence.^1-3 The major benefit is that the patient does not have a permanent ileostomy; the J-pouch serves as an internal pelvic reservoir for intestinal contents. 

The techniques for performing a laparoscopic IPAA procedure have been refined and simplified since the procedure was first described. The in-hospital and 30-day mortality rates are low (<1%); however, overall morbidity rates approach 65%.^3-8 It is important for providers and patients to understand the potential risks and complications that can occur so problems can be addressed early. Ongoing J-pouch surveillance, as outlined in this article, will help patients recognize signs of complications such as pouchitis, self-manage their J-pouch function, and ensure a better quality of life in living with a J-pouch. This article reviews some of the potential postoperative complications, pouchitis management, and ongoing surveillance of the J-pouch that are key to achieving the best possible short- and long-term outcomes for patients with a J-pouch.

IPAA Complications

Complications of mechanical, inflammatory, functional, neoplastic, and metabolic conditions related to the pouch can occur postoperatively (see Table⁹). 

Acute and chronic complications can lead to J-pouch failure such as recurrent pelvic sepsis, Crohn’s disease (CD) of the pouch with fistulas, chronic unrelenting pouchitis, or poor J-pouch function. CD of the J-pouch can be one or all three phenotypes (ie, inflammatory, fibrostenotic, and fistulizing), which parallel non-J-pouch CD.^9,10 Patients with CD of the J-pouch usually have segmental inflammation of the J-pouch body and/or afferent limb. CD involving the ileal J-pouch is generally associated with a high failure rate and J-pouch-related fistulas. The complications associated with failure are extensive, and the options for reconstructive surgery in patients with CD should be assessed.^11,12 

Pelvic sepsis is a common early complication of IPAA. It occurs in 6% to 16% of patients; postoperative anastomotic leak with pelvic sepsis also is associated with poor pouch function. Acute and chronic complications can necessitate removal of the ileal reservoir and construction of a permanent ileostomy.^3,13-16

Sexual dysfunction, such as postoperative impotence and retrograde ejaculation, also can occur and have been observed in approximately 1.5% and 4% of men, respectively.¹⁷ Transient dyspareunia occurs in approximately 7% of women, although coital frequency and the ability to experience orgasm remain unchanged. In a study of 200 women who underwent IPAA <20 years of age, only 5% reported severely restricted sexual function.¹⁸ Female infertility may decrease after IPAA, but the laparoscopic approach has significantly lessened the risk. Successful pregnancies occur regularly.^19-22 

Patients who have had an IPAA procedure should not be discouraged from childbearing because of the pouch. They often are concerned that they may not be able to get pregnant or that the pregnancy will cause problems with the J-pouch. Patients with IPAA need to be informed that pregnancy and delivery are safe. Patients may experience a transient increase in stool frequency or incontinence during pregnancy, most of which resolve after delivery.^23,24 It remains controversial as to whether vaginal or cesarean delivery is better for women with a pelvic pouch, because vaginal delivery has the potential to disrupt the anal sphincter, which could result in incontinence of stool. The mode of delivery should be dictated primarily by specialty obstetricians who can assess the delivery risk in terms of anal sphincter injury.²⁵ Most patients will choose to have a cesarean delivery just to be safe. In my practice, I have had a few patients who had a spontaneous vaginal delivery, and they had no problems. 

Evaluation. Ongoing symptoms need to be evaluated when they occur to assess for J-pouch disorders and associated complications¹⁰ (see Figure 1). Pelvic contrast magnetic resonance imaging and dynamic proctography have proven valuable for the diagnostic assessment of patients with clinically suspected J-pouch-related complications, including leaks and fistulas. Pelvic sepsis is treated with antibiotics; in some cases, the fluid collection may require drainage using interventional radiology drain placement.

Anal transition zone (ATZ) inflammation or cuffitis is an acute and chronic inflammation of the 1 cm to 2 cm of retained rectal mucosa in a stapled anastomosis.^24,26 Symptoms are severe tenesmus, urgency, bleeding, and frequent number of bowel movements often with minimal stool. This can occur in a small number of patients and is treated with 5- aminosalicyclic acid (5-ASA) or steroid suppository or foam enema preparations. 

It is important to note that pouchitis and cuffitis can coexist. Based on the response to topical medical therapy, cuffitis can be classified into steroid/mesalamine-responsive, steroid/mesalamine-dependent, and steroid/mesalamine-refractory types. It is important to tell patients that the development of dysplasia in the ATZ is infrequent and has not led to the development of cancer in a minimum of up to 10 years of follow-up, but that it still needs to be assessed with a pouchoscopy and biopsy based on individual risk. Pouchoscopy with biopsy of the ATZ is recommended every 3 to 5 years after an IPAA procedure unless the patient experiences multiple episodes of pouchitis, dysplasia, or ongoing inflammation. In such cases, surveillance of the pouch with a pouchoscopy and biopsies should occur yearly.^24,27 

To effectively diagnose pouchitis, cuffitis, or CD of the J-pouch, a pouchoscopy with biopsies taken in the terminal ileum, in the body of the J-pouch, and in the 1 cm to 2 cm rectal cuff in a stapled ileoanal J-pouch anastomosis is recommended. A stool fecal calprotectin test with a level of >56 µg/g has been found to correlate with inflammation in the J-pouch and can be utilized between recommended surveillance pouchoscopy screenings.¹⁰ J-pouch removal with an ileostomy may eventually be required if the J-pouch fails due to anastomotic complications, infection, fistulization, development of CD, or poor J-pouch function. I always remind patients that if your quality of life is affected due to poor J-pouch function that cannot be treated, then “Get it out!” The decision to have the J-pouch in the first place was to provide a good quality of life. An end ileostomy also can provide a good quality of life, and that is what it is all about. Clinicians should remember to always reinforce with the patient the concept of what a good quality of life is with a J-pouch.

Pouchitis: Most Common J-pouch Complication 

The most frequently observed long-term complication of IPAA is pouchitis, an acute and/or chronic inflammation of the ileal reservoir. This process of the pouch occurs in 40% to 50% of patients; in a minority of these patients, pouchitis can become chronic.^28,29 Potential risk factors include extraintestinal manifestations such as primary sclerosing cholangitis, backwash ileitis, and extensive UC; the use of nonsteroidal anti-inflammatory drugs (NSAIDS) over an extended period of days; and smoking.²⁹ Most patients with primary sclerosing cholangitis (PSC) will develop chronic pouchitis and require ongoing treatment and surveillance. Therefore, it is important that patients with PSC understand that chronic pouchitis is common and may need lifelong medical treatment. In addition, an ileoanal stricture with incomplete emptying over time may initiate pouchitis due to holding of stool in the J-pouch.³⁰ Patients need to be aware that if they have to strain to pass stool, they may have a stricture. At every visit, patients should be instructed on the importance of an anal digital exam to assess for a possible stricture at the ileal pouch anal anastomosis.  

Pouchitis should be expected in any patients who experience abdominal cramps, increased stool frequency, watery diarrhea, urgency, and fatigue. Patients may or may not report blood in the stool, fever, leakage of stool, or a flare of joint or body aches. Patients commonly state, “It feels like UC all over again or flu-like symptoms,” which is what I tell all patients early on so they can then identify the symptoms of pouchitis when they occur. In addition, pouchitis symptoms usually do not occur within the first 6 months after closure of the ileostomy.²⁸ Often, increased bowel function of 10 to 15 watery bowel movements a day, abdominal cramping with gas, and dehydration initially after surgery are more due to diet and drinking on an empty stomach; patients should eat first, then drink liquids. It is important to stress that the J-pouch needs time to adapt, which can take up to 1 year, to accommodate holding more stool. Eating foods to thicken stool such as soluble fiber and starches to decrease the number of stools a day and trying to delay bowel movements can increase J-pouch capacity to hold more stool. 

Diagnosis and Management of Pouchitis

The exact cause of pouchitis is unclear, but it is often successfully treated with a 2-week course of antibiotics, particularly metronidazole or ciprofloxacin. If the patient is intolerant of these substances, other antibiotics such as augmentin, levofloxacin, or sulfamethoxazole and trimethoprim also can be considered. Symptoms of pouchitis usually resolve within 24 to 48 hours, but the patient needs to be reminded to complete the full 2-week course. Patients often stop the antibiotic once they feel the symptoms have resolved and they feel better, making it imperative to reinforce completion of the 2-week course to treat pouchitis effectively. Pouchitis affects approximately 2% of UC patients in the first year and 50% 5 years post-creation of the IPAA. Approximately 30% of pouchitis patients experience a single episode, 60% have two or more episodes, and approximately 10% develop chronic pouchitis.^28,29 In chronic pouchitis, if the patient develops a prompt recurrence of symptoms within a week or 2 after stopping the antibiotic, the patient needs to go back on a chronic antibiotic regimen and possibly a maintenance probiotic (VSL#3 DS or Visbiome DS). 

Changes in the gut microbiota in the ileal pouch most likely occur due to fecal stasis with bacterial overload and mucosal adaptive changes from small bowel mucosa to colon-like mucosa of the pouch body and inflammation. Detailed studies of the microbiota in patients with pouchitis have demonstrated unique patterns, including the persistence of Fusobacteria and enteric species, increased Clostridium perfringens, and the absence of Streptococcus and Faecalibacterium species in the inflamed pouch.^31,32 These observations and a high rate of response to various antibiotics support an important role for bacteria in the pathogenesis of pouchitis and provide a rationale for clinical trials aimed at altering the microbiota with probiotics. However, attempts should be made to taper the antibiotics when possible. Probiotics such as VSL#3 DS or Visbiome DS contain 900 billion bacteria and are most effective in mild pouchitis symptoms but are not usually as effective for acute pouchitis. They often are used as maintenance treatment.²⁹ It is important to tell patients that probiotics take a while (sometimes up to a year) to ultimately change the microbiome. Sometimes, a combination of antibiotics or cycling antibiotics may be effective when loss of response occurs. When antibiotics are no longer effective, topical steroids, 5-ASA enemas, budesonide, and immunomodulators may be utilized. 

In patients with concurrent pouchitis and enteritis above the J-pouch, oral budesonide may be effective.¹⁰ Treatment for chronic pouchitis involves induction therapy as well as maintenance therapy, similar to IBD treatment. Chronic pouchitis may eventually turn into CD. In patients with underlying CD or concurrent autoimmune disorders (eg, rheumatoid arthritis, psoriasis), anti-tumor necrosis factor (TNF) agents (infliximab, adalimumab, usekinumab) are given. Patients with chronic antibiotic-resistant pouchitis or CD of the pouch may benefit from induction therapy as well as maintenance therapy from infliximab or adalimumab.^33,34 Other agents have been used for treating chronic antibiotic refractory pouchitis. Vedolizumab, an anti-integrin antibody, and ustekinumab, an anti-interleukin 12/23 antibody, appear to be effective in some cases.^35,37 For an algorithm¹⁰ of the treatment of pouchitis, see Figure 2.

In patients with primary sclerosing cholangitis (PSC) or IgG4-associated pouchitis, oral budesonide or a budesonide enema is used.³⁰ The use of low-dose mercaptopurine (50 mg daily) or methotrexate (7.5 to 12.5 mg daily, either oral or intramuscular) may be used in patients who fail to respond to budesonide and in patients with multiple extraintestinal manifestations of autoimmune pouchitis.¹⁰ 

Patients who develop chronic antibiotic-resistant pouchitis or CD of the pouch have an elevated risk for needing pouch excision. Patients who fail immunomodulator and biologic therapy after the exclusion of secondary causes should be referred to a colorectal surgeon for consideration of pouch excision and permanent ileostomy. Although many patients are treated just in the presence of clinical symptoms, an accurate diagnosis requires endoscopic visualization of the pouch and histologic evaluation to determine whether pouchitis or CD is the culprit.^10,29

Psychosocial Assessment/Issues 

After surgery, the quality of life and defecatory and bowel function plateau at approximately 1 year and remain constant, with more than 90% of patients satisfied with the outcome of the operation and quality of life.⁴ Two large studies looked at health-related quality of life after pouch failure; overall, patients with a failed pouch reported significantly worse physical function, social functioning, energy level, and physical role function compared with the group with a functioning pouch.³⁸ Long-term functional results and quality of life with a pelvic ileal reservoir are typically superior to that of patients with a Brooke ileostomy or medically treated colitis.^26,39-42 However, it is still unclear whether this complex procedure results in an improved quality of life because the disease was removed or because the patients could control their stools. Other authors have shown that quality of life improves no matter what procedure is performed and is due to eradication of the disease.^26,41 In one study that ranked the role of altered bowel emptying on quality of life and disability, the patients with pelvic J-pouches were found to rank altered bowel emptying as a significantly worse concern than did those with stomas.⁴³ Although it is accepted that avoiding an abdominal stoma will improve quality of life by maintaining body image, it is unclear if the relative change in bowel emptying with a J-pouch causes enhanced quality of life relative to a stoma. Although functional outcomes and health-related quality of life have been shown to be similar after laparoscopic versus open IPAA, better patient acceptance and improved cosmesis, combined with a decreased risk of hernia formation in previous incisions, make laparoscopic IPAA the preferred surgical approach for UC.⁴⁴ 

In addition, patients with UC often experience mental health issues related to depression, anxiety, and post-traumatic stress syndrome.⁴⁵ These issues do not necessarily go away once J-pouch surgery is performed. Some issues are due to not understanding that having a J-pouch does not mean life is normal right away and that it takes patience and resilience to manage pouch function, particularly if patients have frequent stools per day, stool leakage, perianal irritation, or sexual dysfunction that may take up to a year to improve. Some patients may have complications such as recurring pouchitis symptoms and need treatment. The key point is that it is important to ask the patient about their mental health at each visit and offer a referral to a gastrointestinal psychologist or psychosocial counseling as needed. Anti-depressant, anti-anxiety medications, or “brain-gut” behavioral therapies can make a tremendous difference in their quality of life and living with a J-pouch.⁴⁶ My mantra is “Just ASK” at every visit! 

IPAA Patient Follow Up 

J-pouch patients need ongoing follow up to assess pouch function, reaffirm what are considered good functional results, and assess for anemia, iron, folate, vitamin D, vitamin B12, zinc, and other nutritional deficiencies that can occur when the pouch is functioning like a rectum to hold stool.⁴⁷ It is important for the patient and the provider to recognize symptoms of pouchitis and to assess the pouch endoscopically with biopsies or with a stool fecal calprotectin test to confirm the presence of inflammation and that the problem is pouchitis versus CD or cuffitis. This confirmation is key to J-pouch treatment and management.¹⁰ Below is the recommended follow-up plan used in my J-pouch clinic to ensure these patients are continually monitored, assessed, educated, and treated if issues develop or complications occur. Complications can occur years after the procedure, so it is important that patients understand that they need to be assessed not only often in the beginning, but also annually for the rest of their lives. Early recognition of problems is critical to a satisfactory outcome with a J-pouch. 

In addition, I have learned that patients forget what “normal” pouch function is and start accepting poor pouch function when symptoms gradually develop over time. They start accepting symptoms like they did when they had symptoms of UC. Initial frequent visits focus on pouch adaptation over time, educating patients on what is good pouch function lifelong as well as recognizing symptoms of pouchitis. This is crucial for patients to understand how important it is that they follow-up regularly to ensure good pouch function and reduce any possible short- or long-term complications. The following is the IPAA surveillance protocol that I use to monitor patients who had the procedure for UC.

Recommended IPAA Patient Surveillance 

Visits 

• Initially in the adaptation phase, the patient should be seen every 3 months, then at 18 and 24 months, then yearly thereafter to assess pouch function and potential complications. 

At each visit:

• Educate on what good pouch function is as discussed.

• Assess bowel function at each visit with a weekly diet and stool diary noting stool consistency and if leaks of stool occur. 

• Assess mental health—“Just Ask.”

• Perform an anal digital exam to assess for an ileal pouch anal anastomotic stricture.

Yearly 

• Perform blood workup to assess for anemia, vitamin, mineral, and nutritional deficiencies. 

• Perform pouchoscopy with biopsies every year or 2 as indicated.

• Assess for inflammation or dysplastic tissue. 

• Biopsy above the pouch, in the pouch, and cuff (ATZ) if present.

• If inflammation/dysplasia are noted, perform yearly pouchoscopy with biopsies.

- For a normal pouch bopsy, a pouchoscopy is recommended every other year to 3 years based on risk. 

• Check fecal calprotectin to assess for inflammation as a quick assessment between pouchoscopies as needed.

• Schedule additional visits if problems develop and perform more frequent monitoring.

Conclusion

A proctocolectomy with ileoanal pouch anastomosis procedure is the surgical procedure of choice for most patients with refractory UC; the benefit is that a permanent ileostomy is not needed. However, the IPAA procedure has potential risks and complications that can develop early on or later. Pouchitis is the most common complication; early recognition of symptoms and treatment are key. It is important to note that 90% of patients with the J-pouch are happy with the results, but they need to be aware that patience and resilience are qualities needed in the first year or so with J-pouch adaptation. Complications such as multiple stools a day, dehydration, perianal irritation, leakage of stool, and possible pouchitis are the most common and need to be assessed and treated. Patients should be followed up for pouch surveillance once a year after the initial frequent visits in order to achieve short- and long-term success living with a J-pouch. As I always say, “Respect the Pouch!” 

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Michele Rubin is an Advanced Practice Nurse certified as a Clinical Nurse Specialist in Gastroenterology Nursing. She has 45 years of experience in Inflammatory Bowel Disease (IBD) at the University of Chicago Medicine (UCM) ranging from positions in nursing leadership, IBD research, to IBD Clinical Nurse Specialist in Colorectal surgery. She is also the Associate Director of the IBD Center. Michele works closely with the UCM multidisciplinary IBD patient management team with the IBD surgeons and gastroenterologists in managing the care of IBD patients and families. She works in colorectal surgery with IBD patients and initiated the J-Pouch clinic to ensure patients adapt successfully and get ongoing surveillance. Michele is a known leader and lecturer on IBD across the country for various organizations.  She has authored and co-authored numerous articles and participates in research focusing on IBD. She is a professional member of the Crohn’s and Colitis Foundation and is a past honoree of the National Crohn’s and Colitis Foundation’s Uniting Care & Cure Award. She participates on the National Crohn’s and Colitis Nurse and Advanced Practice Provider Committee and is Director of the APP Preceptorship program focused on IBD specialty training for new APPs. Michele was also a past Midwest Regional and National GEM (Giving Excellence Meaning) Awardee for “Excellence in Community Care” by Nurse.com. Please send inquiries to advocacy@ostomy.org. Information in this article was provided by UOAA. UOAA does not endorse particular products, manufacturers, providers, or other sellers of ostomy products. This column was not subject to the Wound Management & Prevention peer-review process.