When a total proctocolectomy is required, the ileal pouch–anal anastomosis (IPAA) procedure is favored over an end ileostomy because the IPAA allows continence with defecation by the normal route. The procedure can be performed with favorable results for patients with ulcerative colitis and familial adenomatous polyposis, as well as in some patients with indeterminate colitis and highly selectively in patients with Crohn disease that is confined to the large intestine. Good early and long-term function and quality of life can be expected; however, because complications influence long-term results and hence pouch retention, sound surgical decision making and use of sound technique are critical.
Use of strategies that can target modifiable risk factors for complications and reduce risk in persons most likely to have complications optimizes good long-term outcomes. A staged procedure with an initial subtotal colectomy reduces risk of complications in high-risk patients with poor general health or malnutrition, in patients receiving high doses of immunosuppressive drugs or who are being treated with steroids, and in patients who have severe acute colitis. Similarly, an awareness of the perioperative complications that can occur facilitates their identification and prompt management, which affects both short-term results and long-term pouch function and retention.
For certain pouch-related problems, correction by both perineal and abdominal approaches is feasible, allowing the pouch to be salvaged. Pouch failure is generally defined as the need for the creation of a permanent ostomy with or without excision of the pouch. Factors shown to be associated with pouch failure include certain diseases (e.g., Crohn disease), prior anal disease, abnormal anal manometry findings, comorbid conditions, pouch-perineal or pouch-vaginal fistulae, pelvic sepsis, and anastomotic stricture and separation. The following preoperative factors have the strongest influence on pouch survival: extent of resection (total proctocolectomy vs. completion proctectomy), type of anastomosis (stapled vs. hand sewn), patient diagnosis (mucosal ulcerative colitis and others vs. Crohn disease), and comorbidity. In this chapter, various complications related to the ileoanal pouch—several of which may lead to pouch failure—and their management are discussed.
Patients with a well-constructed, uncomplicated ileoanal pouch typically have six to eight bowel movements over a 24-hour period. Most patients do not need to wear pads, although some patients do so for peace of mind. Patients can defer defecation for at least an hour, and incontinence is rare. Quality of life is high, and most patients deny having physical, social, work-related, or sexual restrictions.
When patients experience pouch dysfunction, a detailed history of symptoms, function of the pouch, and the timing of the onset of symptoms need to be obtained. The state of the anoperineum and the integrity of the sphincter mechanism are assessed by examination, followed by pouchoscopy with biopsies of the pouch, residual anal canal, and afferent limb. Biopsies help evaluate the presence of inflammation, suggesting pouchitis or cuffitis, Crohn disease, or other inflammatory and infectious complications. Stool and blood tests help exclude an infection as the cause of the changes in pouch function. The potential diagnosis of Crohn disease needs to be considered in any patient who experiences fistulous and septic complications after creation of an ileoanal pouch. However, septic complications after ileoanal pouch creation may mimic Crohn disease, and the distinction may be challenging. In general, septic complications that occur within 1 year of pouch creation or closure of a defunctioning ostomy above a pouch are likely to be due to perioperative anastomotic or pelvic infections. If complications occur more than 1 year after creation of the pouch, the degree of suspicion for Crohn disease should increase.
A Gastrografin enema helps evaluate pouch structure and capacity and identify pouch-perineal or pouch-vaginal fistulae, narrowing of the afferent limb, inlet, or outlet, and any posterior sinus tract leading from the anastomosis. Magnetic resonance imaging (MRI) of the pelvis helps detect any persistent presacral collections, abscesses, or fistulae that could be contributing to the patient’s symptoms. Computed tomography (CT) enterography is useful in the evaluation of the small intestine proximal to the pouch; inflammation indicates the possibility of Crohn disease. Anorectal manometry and endoanal ultrasound are used to assess the sphincter tone, anatomic integrity, and presence of paradoxical pressures, which, when correlated with difficulties with evacuation, suggest outlet obstruction. A defecating pouchogram or magnetic resonance defecography identifies problems with evacuation.
Intraoperative complications specifically related to pouch creation include bleeding, difficulty with reach of the pouch, and pouch ischemia resulting either from tension or extrazealous attempts at vascular division to allow an ileoanal pouch to reach to the residual anal canal.
Postoperative complications include pelvic sepsis, anastomotic dehiscence, pouch bleeding, pouch-perineal and pouch-vaginal fistulae, pouch sinus, leak from the tip of the J limb, pouch prolapse, pouchitis and cuffitis, Crohn disease of the pouch, outlet dysfunction, pouch cancer, and pouch failure. Late complications typically occur 3 months after creation of the pouch or after takedown of the ileostomy when the pouch has been defunctioned.
Pelvis Sepsis and Anastomotic Leak
Ileoanal pouch–related pelvic sepsis is a complication of varying degrees of severity, and apart from leading to poor outcomes over the short and intermediate term, it may predispose to pouch failure. Even when the sepsis is successfully treated, patients may have impaired long-term function and a reduced quality of life. A careful assessment of potentially predisposing factors, together with preoperative and intraoperative planning to minimize their affect, is important. A high body mass index, a final pathologic diagnosis of ulcerative/indeterminate colitis or Crohn disease, and use of intraoperative and postoperative transfusions are associated with pelvic sepsis.
Pelvic sepsis may occur as a result of, or separate from, an anastomotic dehiscence. Pelvic sepsis presents with fever, leukocytosis, tachycardia, and pelvic or lower back pain, although the symptoms can be nonspecific, such as an ileus or a delayed recovery. A CT scan of the abdomen and pelvis with use of oral, intravenous, and rectal contrast material is useful in identifying a peripouch abscess and its communication with any anastomotic leak. Unstable patients require an emergency exploratory laparotomy with peritoneal washout and an ostomy if the pouch has not already been defunctioned. In some cases, a loop ileostomy above a pouch may need to be converted to an end ostomy. In stable patients, percutaneous or transanal drainage of the abscess in conjunction with administration of antibiotics is required for prompt control of sepsis, which may allow preservation of the pouch. For abscesses communicating with the anastomosis, drainage can be transanal or percutaneous, and if percutaneous, drainage can occur by transabdominal or transgluteal routes.
Postoperative Bleeding from the Pouch
The long suture or staple line within the pouch predisposes patients to perioperative bleeding, which usually presents as anal bleeding or blood from the ostomy. Although severe bleeding is uncommon, it may be troublesome. Oversewing of the back row of staples along the mesentery of the small bowel after the pouch is created may minimize the chances of postoperative bleeding. Pouchoscopy with cautery, application of hemostatic clips, or injection of epinephrine usually controls bleeding. When diffuse oozing occurs, a continuous irrigation of the pouch with saline solution/epinephrine through the downstream end of the ileostomy with recovery of the fluid through a catheter placed in the anus usually facilitates control.
Pouch-Perineal and Pouch-Vaginal Fistulae
A fistula from the pouch or the pouch anal anastomosis to the vagina or the perineal skin presents with increasing pain and local sepsis that ultimately leads to discharge of pus or stool and gas. A fistula may occur after drainage of a pouch or an anastomotic leak. The patient also may experience discomfort, irritation, and incontinence, as well as recurrent vaginal and urinary infections. Fistulas may present soon after ileostomy closure or construction of an undiverted pouch or some months later. A thorough review of the history and medical records relating to the ileoanal pouch surgery, the postoperative course after the procedure, and a pathology review of biopsies from prior to surgery, as well as of the colectomy or proctocolectomy specimen, may help achieve a better assessment of the pathologic diagnosis and particularly establish the possibility of Crohn disease. General, abdominal, and perineal examination for clinical indications of Crohn disease, together with the function of the anal sphincter, provides useful information.
Vaginoscopy and pouchoscopy may allow for both the identification of the fistula and a determination of the location, number, nature, and size of the fistulous openings, together with an assessment of the physical state of the pouch, anal canal, and vagina. This examination is best performed after induction of anesthesia, which allows the most thorough evaluation of the pouch and perineum and provides the best information. Other tests that should be considered include a Gastrografin enema, vaginography, and MRI of the pelvis because these tests help define the presence and anatomy of a fistula. CT or magnetic resonance enterography show both the anatomy of the pouch and the state of the small bowel above the pouch.
When active sepsis is present, the first priority is to obtain control by adequate drainage of any abscess cavity or infected track, which helps resolve symptoms and limits further tissue damage. If local drainage is unsuccessful in controlling the sepsis, then antibiotics, antiinflammatory agents, or specific Crohn disease medications may help. The final step in controlling the sepsis is fecal diversion. Once the sepsis is controlled and has settled, options for repair of the fistula can be entertained.
Local procedures may be considered for low, simple, pouch-related fistulae and include pouch or vaginal advancement flap repairs, perineal pouch advancement, fistula glue or plugs, and gracilis flap repair or interposition of a biologic mesh. If the perineal, local, or pouch advancement repair fails, subsequent attempts at local flap repair may be made. When perineal pouch advancement is undertaken, half of the pouch–anal anastomosis adjoining the location of the fistula is disconnected and the entire pouch wall is mobilized transanally prior to approximation to the anal canal with or without mucosectomy. A repeat IPAA is also an option for patients in whom repair by the perineal approach is unlikely to be successful and when local procedures have definitively failed. Crohn disease must first be excluded to the extent possible. The pouch is disconnected from the anus by combined abdominoperineal dissection and is then revised with excision and debridement of the portion involved in the fistula tract. Any defect in the rectovaginal septum is repaired, and a mucosectomy and a hand-sewn anastomosis is performed. The previous pouch may be reused, refashioned, or augmented as needed, or a new pouch may be created after resection of the previous pouch. An omental pedicle flap can be used to separate the pouch from the vagina.
Up to 85% of pouch-vaginal fistulae can be healed using these approaches, resulting in a pouch failure rate of 15%. For the 15% of persons who experience pouch failure, pouch excision with an end ileostomy or conversion of the J pouch to a K pouch is performed.
A pouch sinus is a blind track that comes from an opening at the anastomosis or in the pouch itself. This complication occurs in 2.8% to 8% of patients after an IPAA procedure. The sinus track may be of variable size, its opening may be narrow or wide-necked, and it may be either incidental or symptomatic. When symptoms occur, they may either be minor or major, such as sepsis, pelvic pain, pouch dysfunction, and even pouch failure. Presentation with symptoms is a significant predictor for low healing rates and pouch failure.
Therapeutic options include debridement, unroofing, use of fibrin glue, pouch revision, and creation of a new pouch. The choice of treatments depends on the presentation, size, location, and other factors, such as whether the pouch is defunctioned. In asymptomatic patients and in patients with a proximal ostomy in whom the condition is detected incidentally, spontaneous healing occurs with time. For a symptomatic sinus, transanal drain placement, deroofing, or sinusotomy may be an option, with the creation of an ostomy to cover a pouch that is not defunctioned. For an incidental sinus in a patient with a diverted pouch, the sinus may be opened or repaired prior to closure. Sometimes closure can proceed with no treatment if the sinus appears harmless. When healing does not occur in symptomatic patients, a repeat IPAA or pouch excision with an ileostomy may be considered.
Leak from the Tip of the “J”
Leaks from suture and staple lines in the pouch that are not located at the ileal anal pouch anastomosis are rare. A leak from the tip of the J pouch is uncommon and difficult to detect because the hole in the pouch is not in the direct flow of the fecal stream; thus symptoms are subtle and the presentation is indolent. Patients present with nonspecific and usually subacute symptoms such as abdominal pain, fever, or diarrhea. In some patients, leaks may present with a fistula rather than a pelvic abscess, a presentation usually suggestive of an anastomotic leak or Crohn disease. Pouchoscopy, a Gastrografin enema, a CT scan with use of a rectal contrast agent, or an MRI scan of the pelvis may detect the site of the leak, although sometimes detection may only be possible upon laparotomy. A high degree of suspicion is required for its diagnosis, and when detected, management depends upon the nature and degree of the defect and associated findings in the pouch and pelvis. Salvage surgery may require pouch repair of the leak site, a pouch revision, or pouch resection with a repeat IPAA with or without a proximal ostomy.
Pouch prolapse is a rare complication of the ileoanal pouch that occurs in 0.3% of patients in whom a pouch is created. Patients may present with tissue prolapse or outlet dysfunction, and clinical examination, manometry, and pouchoscopy may suggest the diagnosis. In patients with mucosal prolapse, stool bulking agents and biofeedback are initially utilized in an attempt to avoid excessive straining. When symptoms persist, a local perineal procedure with pouch advancement after the excision of redundant mucosal tissue can be considered. For patients with full-thickness pouch prolapse, definitive transabdominal surgery may be the best treatment. Pouchpexy using a transabdominal approach, with fixation of pouch to the sacrum with nonabsorbable sutures, is a reasonable option.
Pouchitis and Cuffitis
Pouchitis and cuffitis relate to inflammation of the pouch or the lining of the residual anal canal and are diagnosed at pouchoscopy and biopsy. Treatment is primarily medical. Small areas of cuff inflammation may be approached through ablative or excisional means. However, a defunctioning ostomy or pouch excision with an end ileostomy may be required for recalcitrant pouchitis or cuffitis that is unresponsive to medical treatment. Corrective surgery or a repeat IPAA also may be required for the management of pouchitis resulting from complications of the pouch such as a chronic presacral abscess, a pouch sinus, small pouch size, or obstruction resulting from a stricture or pouch prolapse. A repeat IPAA is also an option for proctitis resulting from a long rectal remnant retained at the time of IPAA. Recurrent cuffitis occasionally can be managed with a mucosectomy and perineal pouch advancement.
Crohn Disease of the Pouch
Crohn disease may affect the body of the pouch, the afferent limb, the pouch-anal anastomosis, the perineum, or the small intestine proximal to the pouch. Medical management includes steroids, immunosuppressive medication, and/or biologic agents. Endoscopic intervention, including dilatation, may be used for isolated short-segment strictures of the pouch-anal anastomosis, pouch body, or afferent limb. Surgical treatment may be required for strictures that are not amenable to endoscopic therapy and when endoscopic therapy fails. Strictures of the afferent limb may require small bowel resection or stricturoplasty or rarely a side-to-side anastomosis between the strictured segment and the top of the pouch. Strictures of the pouch body similarly can be managed with a stricturoplasty. Such treatment is usually combined with a defunctioning ostomy above the pouch and medical treatment of the Crohn disease. More extensive disease involving the pouch may lead to pouch excision or permanent defunction. Surgical management of strictures of the pouch–anal anastomosis may include dilatation, stricturoplasty, diversion, or a repeat IPAA. Perianal disease may be managed with drain or seton placement to allow drainage of loculated abscesses or fistulae in combination with medical treatment.
Problems with pouch evacuation may develop as a result of a stricture at the anastomosis or pouch prolapse. Strictures resulting from scar tissue or Crohn disease can be managed by periodic self-dilatation, endoscopic balloon dilatation, serial bougie dilatation, or a stricturoplasty. For outlet dysfunction that occurs in the absence of an anatomic cause, evaluation of the pelvic floor may reveal paradoxical contraction of the puborectalis. Biofeedback is an option for these patients, and injection of the pelvic floor with botulinum toxin A (Botox) can be attempted. Enemas and intermittent self-intubation of the pouch with irrigation may be useful in both organic and functional obstructive disorders.
In patients with an S pouch, a long spout may cause problems with defecation. The spout should be no longer than 2 cm. Transanal shortening of the spout may help.
Cancer of the Pouch
Cancer of the pouch itself is rare, even in patients with familial adenomatous polyposis. Cancer in the anal transition zone arising from residual rectal mucosa is more common, although still rare. Mucosectomy with a hand-sewn anastomosis does not eliminate the risk of cancer, but the risk is less than that of a stapled IPAA. A stapled ileal pouch anal anastomosis may facilitate surveillance of the pouch and anal transitional zone, but patients are at risk for cancer after both types of anastomoses.
When pouch failure develops, surgical options include pouch excision with a temporary or permanent end ileostomy, pouch revision or creation of a new pouch, conversion of the J pouch to a K pouch, and the creation of an ostomy above a pouch left in situ. In the latter instance, provided symptoms from the retained pouch are not unmanageable and do not impinge on quality of life, the pouch can be retained for an extended period. In these circumstances, periodic surveillance of the pouch with surveillance biopsies is necessary.