RESIDENT S CORNER Prevention, Diagnosis, and Treatment of Complications of the IPAA for Ulcerative Colitis Stefan D. Holubar, M.D., M.S. Department of Colon & Rectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio CASE SUMMARY: A 60-year-old man with a history of an IPAA for ulcerative colitis (UC) presented with a pouch-anal anastomotic (PAA) stricture (Fig. 1) refractory to endoscopic balloon dilation and needleknife stricturotomy. After extensive counseling regarding surgical options, he declined operative intervention and was taught manual self-dilation; his obstructive symptoms remain improved to date. CLINICAL QUESTIONS How are complications of IPAA for UC classified? How are IPAA complications prevented? What medical, endoscopic, and surgical options are available to treat ileal pouch complications? BACKGROUND Restorative total proctocolectomy with IPAA is the procedure of choice for most patients with UC, IBD unclassified (formerly indeterminate colitis), familial adenomatous polyposis, and, less commonly, isolated Crohn s colitis. Complications after IPAA are common and significantly impact patient quality of life. Successful management requires a multidisciplinary approach including medical, endoscopic, and surgical interventions. Earn Continuing Education (CME) credit online at cme.lww.com. This activity has been approved for AMA PRA Category 1 Credit. TM Funding/Support: None reported. Financial Disclosures: None reported. Correspondence: Stefan D. Holubar, M.D., M.S., Department of Colon & Rectal Surgery, Cleveland Clinic, 9500 Euclid Ave, A30, Cleveland OH 44195-0001. E-mail: holubas@ccf.org Dis Colon Rectum 2018; 61: 532 536 DOI: 10.1097/DCR.0000000000001094 The ASCRS 2018 PRESENTATION AND DIAGNOSIS Complications of IPAA for UC can be classified early vs late relative to pouch construction. Early complications are usually technical and, if a patient is still diverted, may be occult. Early complications include small-bowel obstruction (SBO), portomesenteric vein thrombosis, and pelvic sepsis from leaks from the tip or body of the J-pouch, or from the PAA with presacral sinus/abscess or pouch vaginal fistula. Late complications are further classified as obstructive/mechanical (adhesive SBO, strictures, malrotated (twisted) pouch, pouch prolapse, megapouch, afferent limb syndrome, and S-pouch efferent limb syndrome), functional (pelvic floor dysfunction with outlet obstruction, fecal incontinence), inflammatory (pouchitis/ cuffitis with tenesmus, urgency, and nonbloody diarrhea), or penetrating (perianal fistulas, anovaginal and pouch-vaginal fistulas). Fistulas and strictures may be either delayed technical complications or manifestations of phenotypic switching to Crohn s disease (CD). Finally, pouch neoplasia is a rare but dread late complication. Many IPAA complications (pouchitis, fistulas, strictures) are diagnosed by thorough history (symptoms, bowel habits), physical examination, digital rectal examination, and liberal use of examination under anesthesia (EUA). Other tests include the pouchogram which is a gastrografin enema using a Christmas tree-tip catheter in the distal anal canal as if the tip is above the PAA, a presacral sinus may be missed. Cross-sectional imaging includes CT or MR enterography to assess for proximal disease, and pelvic MRI to provide a preoperative roadmap in cases of penetrating complications, as well as local staging of cancers. Pouchoscopy is the mainstay in diagnosis of inflammatory and neoplastic complications of the pouch, but may also identify presacral sinus and stricture, and may help diagnose otherwise occult malrotation of the pouch and tip of the J-pouch leaks. The former may result in abdominal pain with or without obstruction, whereas the latter requires a high index of suspicion in cases of recurrent abdominopelvic abscesses and a nondiagnostic gastrografin enema. Anorectal manometry and defecating pouchograms may aid in cases of functional complications. 532 DISEASES OF THE COLON & RECTUM VOLUME 61: 5 (2018)
DISEASES OF THE COLON & RECTUM VOLUME 61: 5 (2018) 533 FIGURE 1. Endoscopic view of a severe pouch-anal anastomotic stricture before, during, and after endoscopic balloon dilation (serial 16-17- 18 mm balloons) with the resultant view of a normal stool-filled pouch. Upper panels show before dilation, lower left panel during pneumatic balloon dilation, and right lower panel after dilation. CCF 2018. Used with permission of Cleveland Clinic Foundation, Cleveland Clinic OH. MANAGEMENT Prevention The most important principle in preventing IPAA complications is patient selection, with minimization of malnutrition, anemia, and immunosuppression at the time of pouch construction. The impact of biologic agents on postoperative complications is controversial, 1 but, at present, the majority of IPAAs in the United States are performed in a 3-stage manner. 2 Patient selection vis-à-vis sphincter function is crucial, because UC patients may have urgency from proctitis as opposed to weak sphincters; patients with true fecal incontinence may benefit from permanent ileostomy instead of IPAA. Finally, although colonic strictures may be seen in long-standing UC (lead-pipe colon), colitis with skip areas, enteritis, granulomas, or fistulas likely have CD and should not be offered IPAA. Several complications are purely technical and may be prevented during IPAA construction. Minimizing PAA tension, assurance of good blood supply and of a nonrotated mesentery, mobilization of the rectovaginal septum with protection of the vagina to avoid incorporating it into the anastomosis, and intraoperative pouchoscopy with water- (ie, filling the pouch up with saline/betadine) and/or air-leak testing (as after any low pelvic anastomosis) are critical. When diverting an IPAA, the afferent limb of the loop ileostomy should always be cephalad because intentional rotation of the ileostomy, which may be more fully diverting in other conditions, is associated with SBO in IPAA because of mesenteric tension. 3 Finally, the PAA should be 2 cm proximal to the dentate line, often corresponding to the surgeons proximal interphalangeal joint on digital rectal examination. An IPAA too close to the dentate line may result in fecal incontinence; one too high may lead to difficulty to treat cuffitis (ie, proctitis). Treatment of Specific Complications Anastomotic leaks (Fig. 2, left) are managed by a combination of drainage of abscesses, delaying ileostomy closure (or rediversion if highly symptomatic), serial EUAs, and patience on both the surgeon s and patient s part because many will heal with time. Presacral sinus is the result of a posterior PAA leak and is managed similarly but also with serial mushroom catheter downsizing and surgical unroofing (laying open) of the sinus. Endoscopic needleknife sinusotomy is also an option. 4 Leaks from the tip of the J-pouch may prevented, and treated, by staple line angulation so the antimesenteric tip is more proximal on the bowel and thus better vascularized. Perianal fistulas follow the usual tenets of a staged approach with serial EUAs, abscess drainage, draining seton(s), and sphincter-preserving surgery. Pouch vaginal
534 HOLUBAR: COMPLICATIONS OF IPAA FOR ULCERATIVE COLITIS FIGURE 2. Illustration of common IPAA complications. Left: 1 = presacral sinus; 2 = leak from tip of the J-pouch; 3 = leak from body of the J-pouch; 4 = pouch-anal anastomotic leak with transphincteric fistula-in-ano; 5 = pouch-vaginal fistula. Right panel illustrates, from top to bottom, afferent limb stricture, pouchitis with aphthous ulcerations, and a pouch-anal anastomosis stricture. CCF 2018. Used with permission of Cleveland Clinic Foundation, Cleveland Clinic OH. fistula is treated by advancement flap with or without rediversion. For fistulous disease, medical therapy for underlying CD with an immune modulator and/or biologic therapy may be required. Inflammatory complications include acute pouchitis (Fig. 2, right), which, after confirmatory pouchoscopy, usually responds to a short course of oral antibiotics. These include metronidazole or ciprofloxacin, although FIGURE 3. Illustration of redo IPAA operations. Left panel illustrates perineal pouch advancement procedure with anastomotic stricture and fistula (A), mobilization of the pouch into the levator hiatus (B), advancement of the pouch transanally with resection of the stricture (C), handsewn reanastomosis (D). Right panel illustrates an abdominoperineal redo pouch procedure with presacral sinus (A), curettage of the presacral sinus (B), handsewn reanastomosis (C and D). CCF 2018. Used with permission of Cleveland Clinic Foundation, Cleveland Clinic OH.
DISEASES OF THE COLON & RECTUM VOLUME 61: 5 (2018) 535 the latter has a black-box Food and Drug Administration warning for tendon rupture. Other oral antibiotics such as sulfamethoxazole/trimethoprim are also effective. 5 Although chronic pouchitis may be antibiotic dependent, probiotics may help maintain remission; antibiotic-resistant pouchitis may or may not respond to biologics. 6 Severe pouchitis may require rediversion or pouch excision as the inflammatory condition is likely recur after a neo- IPAA. Cuffitis often responds to mesalamine or hydrocortisone suppositories. 7 Obstructive complications are classified as functional or mechanical. Functional outlet obstruction is treated by lifestyle modification and physical therapy/biofeedback. Surgeons should be wary of operating for presumed mechanical SBO when a nonrelaxing pelvic floor is the true etiology, with megapouch, dilated prepouch ileum with no transition point, and a nonrelaxing pelvic floor. 8 Nonrelaxing pelvic floor may also be associated with pouch mucosal prolapse and even frank incarceration. 9,10 Laparoscopic or open suture or mesh pouch pexy procedures are indicated for pouch prolapse. Obstruction from PAA stricture are common and requires surgical dilation with Hegar dilators, endoscopic balloon dilation (Fig. 1), needle-knife stricturotomy, chronic self-dilation at home, and, in refractory cases, pouch advancement (Fig. 3, left). A malrotated pouch requires detorsion and a redo PAA. Classic afferent limb syndrome is when a loop of bowel becomes adhesed to the sacrum behind the pouch and given the risk of IPAA devascularization by posterior mobilization, enteropouch bypass may be indicated; however, afferent limb syndrome may also be caused by upstream mechanical narrowing, kinking, or strictures. 11 In the latter, surgical strictureplasty is an option. The efferent limb of an S-pouch is prone to kinking as the pouch enlarges over time and may require efferent limb resection and handsewn reanastomosis (similar to Fig. 3, right). Finally, adhesions are the most common cause of SBO after IPAA and can be prevented by laparoscopy; laparoscopy also reduces adnexal adhesions and maintains fertility. Rarely, pelvic nerve damage results in retrograde ejaculation, but sperm may be harvested from the urine after orgasm. Erectile dysfunction may respond to phosphodiesterase inhibitors, which can also be used in women to aid in lubrication and orgasm; lubricants and estrogen gel are recommended for dyspareunia from vaginal dryness. Importantly, fecundity is normal in women after IPAA, and in vitro fertilization may overcome infertility. Cesarean delivery is generally recommended after IPAA to avoid rare albeit disastrous sphincter damage. Fecal incontinence may be managed by the addition of fiber, bowel stoppers, Kegel exercises, physical therapy/biofeedback, and sacral nerve stimulation. Neoplasia after IPAA is rare. Low- and high-grade dysplasia, after multidisciplinary discussion, may be treated endoscopically followed by close surveillance, but in young or fit patients, excision must be considered. For cancers of the anus (squamous or adenocarcinoma), rectal cuff, retained mucosa after mucosectomy, or pouch itself (ie, rectal or small-bowel adenocarcinoma), a multidisciplinary team approach and radical surgical extirpation is indicated. Definitive Surgical Options When a combination of medical, endoscopic, and local/ minor surgical repairs is not an option or fails, the patient is left with one of several options. First is permanent rediversion, which can be done laparoscopically in many cases; however, pouch surveillance is still needed. If rediversion does not suffice, then options are to salvage the pouch by pouch advancement (Fig. 3, left), PAA revision (Fig. 3, right), or neo-ipaa construction, all with acceptable continence and quality of life. 12 Finally, pouch excision with conversion to a permanent end or continent ileostomy (Kock pouch) may be required. When patients and surgeons are faced with these complex cases, high-volume IBD-specialty center referral may help salvage a patient s failing pouch.
536 HOLUBAR: COMPLICATIONS OF IPAA FOR ULCERATIVE COLITIS EVALUATION AND TREATMENT ALGORITHM Pouchitis Pouchoscopy with biopsy Combined medical (biologics) +/- surgical approach Pouchitis confirmed oral antibiotics If medically-refractory excision Inflammatory Fistula Leaks Yes Crohn s suspected Leak management Delay reversal Drain abscess Serial pouchograms No Body or tip of J leaks suture/stapled repair Fistula, Crohn s not suspected Delay of ileostomy closure, draining seton Advancement flap, LIFT etc. Ileoanal pouch dysfunction Anatomic Presacral sinus IPAA stenosis Biopsy Presacral sinus management Serial EUA s, mushroom drains Sinusotomy (un-roof/lay-open) Stable sinus close ileostomy Benign, persistent stricture Self-dilation Stricturotomy Persistent dysfunction/symptoms Re-diversion Pouch advancement Pouch revision/redo IPAA Neo-IPAA Convert to continent ileostomy Pouch excision Functional Functional obstruction Prolapse Mechanical obstruction Manometry, defecography Normal manometry Abnormal manometry Obstruction management Adhesive SBO: adhesiolysis Twisted pouch: redo IPAA Afferent-limb syndrome: entero-pouch bypass S-pouch efferent limb syndrome: revision Lifestyle modification, physical therapy + biofeedback Prolapse management Suture or mesh pouch-pexy Re-diversion as a pouch-pexy Functional obstruction adjuncts Alpha galactosidase, simethicone Self-intubation (Waters tube) Cognitive behavioral therapy Re-diversion Evaluation and treatment algorithm of IPAA complications after IPAA for UC. EUA = examination under anesthesia; LIFT = ligation of intersphincteric fistula tract; SBO = small-bowel obstruction; UC = ulcerative colitis. CCF 2018. Used with permission of Cleveland Clinic Foundation, Cleveland Clinic OH. Expert Commentary on Prevention, Diagnosis, and Treatment of Complications of the IPAA for Ulcerative Colitis Scott A. Strong, M.D. Chicago, Illinois The IPAA procedure has become the preferred operation for patients requiring proctocolectomy despite its greater risk for complications without an improved quality of life compared with ileostomy. The complications present soon after IPAA creation (eg, anastomotic dehiscence/leak, autonomic nerve injury, hemorrhage, pelvic abscess, portal vein thrombosis), around the time of planned/actual ileostomy closure (eg, anastomotic fistula/sinus, anastomotic stricture, ileal pouch body/j-tip leak), or months/years after restoration of intestinal continuity (eg, anal fistula, bowel obstruction, cuffitis, functional disorder, infecundity, neoplasia, outlet obstruction,
DISEASES OF THE COLON & RECTUM VOLUME 61: 5 (2018) 537 pouchitis, pouch prolapse/torsion). Dr Holubar has provided an insightful guide to the diagnosis and management of IPAA complications, and a few pearls learned over time may help highlight some of its critical components. The risk for many of these complications can be reduced by avoiding construction of the IPAA when the patient is immunosuppressed, malnourished, obese, or severely ill. This calls for the surgeon to use a 3-stage approach to allow correction of the compromising condition(s) or offer an ileostomy as the only option if the condition(s) cannot be ultimately altered. Most anastomotic leaks and pelvic abscesses diagnosed shortly after IPAA construction will remedy themselves with adequate drainage that is progressively downsized over 3 to 6 months. An anastomotic leak with associated abscess is best treated by drainage through the defect, as opposed to placement of a transgluteal drain that is often painful and can lead to a troublesome extrasphincteric fistula. Many of the smaller anovaginal fistulas will spontaneously heal with removal of foreign bodies (eg, staples) and prolonged fecal diversion. A sinus tract noted on imaging before ileostomy closure is also managed by repeated procedures scheduled every 4 to 6 weeks to minimize the tract s length and size. Once the tract is 2 to 3 cm long, the defect can be saucerized and the ileostomy closed. If a 4- to 6-cm tract persists despite the above approach, the wall of the ileal pouch overlying the tract can be divided with an energy device. The ileostomy is reversed when imaging shows any residual tract readily empties its contents. Longer tracts unresponsive to local measures are best treated by neo-ipaa, because pouch wall division can disrupt the posteriorly situated pouch mesentery. Leaks from the tip of the J-pouch are frequently undetected by preoperative imaging and should be suspected when an abscess of the upper pelvis develops after ileostomy closure. Patients with complications presenting long after ileostomy closure are the most challenging because issues such as cryptoglandular fistulas, chronic parapouch sepsis, pouchitis, and Crohn s disease must be distinguished from one another. The appearance, location, and number of internal openings commonly provide clarity, whereas MRI often identifies an occult anastomotic complication as the underlying cause of chronic/refractory pouchitis. Noncutting setons are a useful long-term solution for complex fistulas, but simpler cryptoglandular fistulas can be managed with transanal pouch advancement. The ileal pouch can be advanced for part (anorectal fistula) or all (cuff inflammation/ dysplasia, outlet elongation/stricture) of its circumference. Correspondence: Scott A. Strong, M.D., Northwestern University Feinberg School of Medicine, 676 North St. Clair St., Arkes Family Pavilion, Suite 650, Chicago, IL 60611. E-mail: sstrong@nm.org. Dis Colon Rectum 2018; 61: 536 537 DOI: 10.1097/DCR.0000000000001095 The ASCRS 2018 The procedure is facilitated by using a prone jackknife position and lighted retractors (eg, Hill Ferguson, Sauerbruch). A mucosectomy is initiated at the dentate line and carried cephalad to the anastomosis. The bowel wall is breached, and the dissection is carried 2 to 5 cm into the peripouch space. The diseased area is excised, any fistula tracts are closed as they enter the sphincter, and the pouch is advanced to the dentate line where it is secured using interrupted polyglycolic acid sutures incorporating the underlying internal sphincter and full thickness of the pouch wall. An intact sphincter must be ensured before using this approach for anovaginal fistulas. Some patients will ultimately need a permanent ileostomy, in which case it is important to recognize that the quality of life is better with pouch excision than a permanently diverted pouch, but excision is associated with poor perineal healing that can be improved by using a staged approach and flap closure of large defects. REFERENCES 1. Holubar SD, Holder-Murray J, Flasar M, Lazarev M. Anti-tumor necrosis factor-α antibody therapy management before and after intestinal surgery for inflammatory bowel disease: a CCFA position paper. Inflamm Bowel Dis. 2015;21:2658 2672. 2. Geltzeiler CB, Lu KC, Diggs BS, et al. 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