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 PRESENTATION AND DIAGNOSIS of an IPAA for ulcerative colitis (UC) presented with Complications of IPAA for UC can be classified early vs late a pouch-anal anastomotic (PAA) stricture (Fig. 1) relative to pouch construction. Early complications are usu- refractory to endoscopic balloon dilation and needle- ally technical and, if a patient is still diverted, may be oc- knife stricturotomy. After extensive counseling regarding cult. Early complications include small-bowel obstruction surgical options, he declined operative intervention (SBO), portomesenteric vein thrombosis, and pelvic sepsis and was taught manual self-dilation; his obstructive from leaks from the tip or body of the J-pouch, or from the symptoms remain improved to date. PAA with presacral sinus/abscess or pouch vaginal fistula. Late complications are further classified as obstruc- tive/mechanical (adhesive SBO, strictures, malrotated CLINICAL QUESTIONS (twisted) pouch, pouch prolapse, megapouch, afferent limb syndrome, and S-pouch efferent limb syndrome), •• How are complications of IPAA for UC classified? functional (pelvic floor dysfunction with outlet ob- •• How are IPAA complications prevented? struction, fecal incontinence), inflammatory (pouchitis/ •• What medical, endoscopic, and surgical options are cuffitis with tenesmus, urgency, and nonbloody diar- available to treat ileal pouch complications? rhea), or penetrating (perianal fistulas, anovaginal and pouch-vaginal fistulas). Fistulas and strictures may be ei- ther delayed technical complications or manifestations of BACKGROUND “phenotypic switching” to Crohn’s disease (CD). Finally, Restorative total proctocolectomy with IPAA is the proce- pouch neoplasia is a rare but dread late complication. dure of choice for most patients with UC, IBD unclassified Many IPAA complications (pouchitis, fistulas, stric- (formerly indeterminate colitis), familial adenomatous tures) are diagnosed by thorough history (symptoms, bow- polyposis, and, less commonly, isolated Crohn’s colitis. el habits), physical examination, digital rectal examination, Complications after IPAA are common and significantly and liberal use of examination under anesthesia (EUA). impact patient quality of life. Successful management re- Other tests include the “pouchogram” which is a gastrogra- quires a multidisciplinary approach including medical, fin enema using a “Christmas tree-tip” catheter in the distal endoscopic, and surgical interventions. 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 Earn Continuing Education (CME) credit online at cme.lww.com. This MRI to provide a preoperative “roadmap” in cases of pen- activity has been approved for AMA PRA Category 1 Credit.TM etrating complications, as well as local staging of cancers. Pouchoscopy is the mainstay in diagnosis of inflam- Funding/Support: None reported. matory and neoplastic complications of the pouch, but may also identify presacral sinus and stricture, and may Financial Disclosures: None reported. help diagnose otherwise occult malrotation of the pouch Correspondence: Stefan D. Holubar, M.D., M.S., Department of ­Colon and tip of the J-pouch leaks. The former may result in ab- & Rectal Surgery, Cleveland Clinic, 9500 Euclid Ave, A30, ­Cleveland OH dominal pain with or without obstruction, whereas the lat- 44195-0001. E-mail: [email protected] ter requires a high index of suspicion in cases of recurrent Dis Colon Rectum 2018; 61: 532–536 abdominopelvic abscesses and a nondiagnostic gastrogra- DOI: 10.1097/DCR.0000000000001094 fin enema. Anorectal manometry and defecating poucho- © The ASCRS 2018 grams 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 the afferent limb of the loop ileostomy should always be cephalad because intentional rotation of the ileostomy, Prevention which may be more fully diverting in other conditions, is The most important principle in preventing IPAA com- associated with SBO in IPAA because of mesenteric ten- plications is patient selection, with minimization of mal- sion.3 Finally, the PAA should be 2 cm proximal to the nutrition, anemia, and immunosuppression at the time dentate line, often corresponding to the surgeons proxi- of pouch construction. The impact of biologic agents on mal interphalangeal joint on digital rectal examination. 1 postoperative complications is controversial, but, at pres- An IPAA too close to the dentate line may result in fecal ent, the majority of IPAAs in the United States are per- incontinence; one too high may lead to difficulty to treat formed in a 3-stage manner.2 cuffitis (ie, proctitis). Patient selection vis-à-vis sphincter function is cru- cial, because UC patients may have urgency from proctitis Treatment of Specific Complications as opposed to weak sphincters; patients with true fecal in- Anastomotic leaks (Fig. 2, left) are managed by a combina- continence may benefit from permanent ileostomy instead tion of drainage of abscesses, delaying ileostomy closure of IPAA. Finally, although colonic strictures may be seen (or rediversion if highly symptomatic), serial EUAs, and in long-standing UC (lead-pipe colon), colitis with skip patience on both the surgeon’s and patient’s part because areas, enteritis, granulomas, or fistulas likely have CD and many will heal with time. Presacral sinus is the result of should not be offered IPAA. a posterior PAA leak and is managed similarly but also Several complications are purely technical and may with serial mushroom catheter downsizing and surgical be prevented during IPAA construction. Minimizing unroofing (laying open) of the sinus. Endoscopic needle- PAA tension, assurance of good blood supply and of a knife sinusotomy is also an option.4 Leaks from the tip nonrotated mesentery, mobilization of the rectovagi- of the J-pouch may prevented, and treated, by staple line nal septum with protection of the vagina to avoid in- ­angulation so the antimesenteric tip is more proximal on corporating it into the anastomosis, and intraoperative the bowel and thus better vascularized. pouchoscopy with water- (ie, filling the pouch up with Perianal fistulas follow the usual tenets of a staged saline/betadine) and/or air-leak testing (as after any low approach with serial EUAs, abscess drainage, draining pelvic anastomosis) are critical. When diverting an IPAA, 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 re- Inflammatory complications include acute pouchitis diversion. For fistulous disease, medical therapy for un- (Fig. 2, right), which, after confirmatory pouchoscopy, derlying CD with an immune modulator and/or biologic usually responds to a short course of oral antibiotics. therapy may be required. 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 laparoscopy; laparoscopy also reduces adnexal adhesions warning for tendon rupture. Other oral antibiotics such and maintains fertility. as sulfamethoxazole/trimethoprim are also effective.5 Rarely, pelvic nerve damage results in retrograde ­Although chronic pouchitis may be antibiotic dependent, ejaculation, but sperm may be harvested from the urine probiotics may help maintain remission; antibiotic-resis- after orgasm. Erectile dysfunction may respond to phos- tant pouchitis may or may not respond to biologics.6 Se- phodiesterase inhibitors, which can also be used in women vere pouchitis may require rediversion or pouch excision to aid in lubrication and orgasm; lubricants and estrogen as the inflammatory condition is likely recur after a neo- gel are recommended for dyspareunia from vaginal dry- IPAA. Cuffitis often responds to mesalamine or hydrocor- ness. Importantly, fecundity is normal in women after tisone suppositories.7 IPAA, and in vitro fertilization may overcome infertility. Obstructive complications are classified as functional Cesarean delivery is generally recommended after IPAA to or mechanical. Functional outlet obstruction is treated by avoid rare albeit disastrous sphincter damage. Fecal incon- lifestyle modification and physical therapy/biofeedback. tinence may be managed by the addition of fiber, bowel Surgeons should be wary of operating for presumed me- stoppers, Kegel exercises, physical therapy/biofeedback, chanical SBO when a nonrelaxing pelvic floor is the true and sacral nerve stimulation. etiology, with megapouch, dilated prepouch ileum with Neoplasia after IPAA is rare. Low- and high-grade dys- no transition point, and a nonrelaxing pelvic floor.8 Non- plasia, after multidisciplinary discussion, may be treated relaxing pelvic floor may also be associated with pouch endoscopically followed by close surveillance, but in young mucosal prolapse and even frank incarceration.9,10 Lapa- or fit patients, excision must be considered. For cancers of roscopic or open suture or mesh pouch pexy procedures the anus (squamous or adenocarcinoma), rectal cuff, re- are indicated for pouch prolapse. tained mucosa after mucosectomy, or pouch itself (ie, rectal Obstruction from PAA stricture are common and or small-bowel adenocarcinoma), a multidisciplinary team requires surgical dilation with Hegar dilators, endoscop- approach and radical surgical extirpation is indicated. ic balloon dilation (Fig. 1), needle-knife stricturotomy, chronic self-dilation at home, and, in refractory cases, Definitive Surgical Options pouch advancement (Fig. 3, left). A malrotated pouch When a combination of medical, endoscopic, and local/ requires detorsion­ and a redo PAA. Classic afferent limb minor surgical repairs is not an option or fails, the patient syndrome is when a loop of bowel becomes adhesed to is left with one of several options. First is permanent redi- the sacrum behind the pouch and given the risk of IPAA version, which can be done laparoscopically in many cases; devascularization by posterior mobilization, enteropouch however, pouch surveillance is still needed. If rediversion bypass may be indicated; however, afferent limb syndrome does not suffice, then options are to salvage the pouch may also be caused by upstream mechanical narrowing, by pouch advancement (Fig. 3, left), PAA revision (Fig. 3, kinking, or strictures.11 In the latter, surgical strictureplas- right), or neo-IPAA construction, all with acceptable conti- ty is an option. The ­efferent limb of an S-pouch is prone nence and quality of life.12 Finally, pouch excision with con- to kinking as the pouch enlarges over time and may re- version to a permanent end or continent ileostomy (Kock quire efferent limb resection and handsewn reanastomosis pouch) may be required. When patients and surgeons are (similar to Fig. 3, right). Finally, adhesions are the most faced with these complex cases, high-volume IBD-specialty common cause of SBO after IPAA and can be prevented by center referral may help salvage a patient’s failing pouch. 536 HOLUBAR: COMPLICATIONS OF IPAA FOR ULCERATIVE COLITIS

EVALUATION AND TREATMENT ALGORITHM

Pouchitis confirmed oral antibiotics Combined medical (biologics) • If medically-refractory excision Pouchoscopy Pouchitis +/- surgical approach with biopsy

Yes No Fistula Crohn’s suspected Fistula, Crohn’s not suspected • Delay of ileostomy closure, draining seton • Advancement flap, LIFT etc. Inflammatory Leaks Leak management • Delay reversal • Drain abscess Body or tip of J leaks • Serial pouchograms suture/stapled repair

Presacral sinus management Presacral Persistent dysfunction/symptoms • Serial EUA’s, mushroom drains sinus • Re-diversion • Sinusotomy (un-roof/lay-open) Ileoanal pouch Anatomic • Pouch advancement dysfunction • Stable sinus close ileostomy • Pouch revision/redo IPAA • Neo-IPAA • Convert to continent ileostomy IPAA Biopsy Benign, persistent stricture • Pouch excision stenosis • Self-dilation • Stricturotomy

Obstruction management Functional Mechanical • Adhesive SBO: adhesiolysis obstruction • Twisted pouch: redo IPAA • Afferent-limb syndrome: entero-pouch bypass • S-pouch efferent limb syndrome: revision Prolapse Prolapse management xxxxxxXXX • Suture or mesh pouch-pexy Normal • Re-diversion as a pouch-pexy manometry Functional Manometry, Functional obstruction adjuncts obstruction defecography • Alpha galactosidase, simethicone Lifestyle modification, physical • Self-intubation (Waters tube) Abnormal therapy + biofeedback • Cognitive behavioral therapy manometry • 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

he IPAA procedure has become the preferred rhage, pelvic abscess, portal vein thrombosis), around the ­operation for patients requiring proctocolectomy time of planned/actual ileostomy closure (eg, anastomotic Tdespite its greater risk for complications without fistula/sinus, anastomotic stricture, ileal pouch body/J-tip an improved quality of life compared with ileostomy. The leak), or months/years after restoration of intestinal con- complications present soon after IPAA creation (eg, anas- tinuity (eg, anal fistula, bowel obstruction, cuffitis, func- tomotic dehiscence/leak, autonomic nerve injury, hemor- tional disorder, infecundity, neoplasia, outlet obstruction, RESIDENT’S CORNER DISEASES OF THE COLON & RECTUM VOLUME 61: 5 (2018) 537 pouchitis, pouch prolapse/torsion). Dr Holubar has pro- The procedure is facilitated by using a prone jackknife posi- vided an insightful guide to the diagnosis and management tion and lighted retractors (eg, Hill Ferguson, Sauerbruch). of IPAA complications, and a few “pearls” learned over A mucosectomy is initiated at the dentate line and carried time may help highlight some of its critical components. cephalad to the anastomosis. The bowel wall is breached, and The risk for many of these complications can be re- the dissection is carried 2 to 5 cm into the peripouch space. duced by avoiding construction of the IPAA when the The diseased area is excised, any fistula tracts are closed as patient is immunosuppressed, malnourished, obese, they enter the sphincter, and the pouch is advanced to the or severely ill. This calls for the surgeon to use a 3-stage dentate line where it is secured using interrupted polyglycolic approach to allow correction of the compromising acid sutures incorporating the underlying internal sphincter condition(s) or offer an ileostomy as the only option if the and full thickness of the pouch wall. An intact sphincter must condition(s) cannot be ultimately altered. be ensured before using this approach for anovaginal fistulas. Most anastomotic leaks and pelvic abscesses diagnosed Some patients will ultimately need a permanent ileos- shortly after IPAA construction will remedy themselves with tomy, in which case it is important to recognize that the adequate drainage that is progressively downsized over 3 to 6 quality of life is better with pouch excision than a perma- months. An anastomotic leak with associated abscess is best nently diverted pouch, but excision is associated with poor treated by drainage through the defect, as opposed to place- perineal healing that can be improved by using a staged ment of a transgluteal drain that is often painful and can lead approach and flap closure of large defects. to a troublesome extrasphincteric fistula. Many of the small- er anovaginal fistulas will spontaneously heal with removal REFERENCES of foreign bodies (eg, staples) and prolonged fecal diversion. A sinus tract noted on imaging before ileostomy closure 1. Holubar SD, Holder-Murray J, Flasar M, Lazarev M. Anti-tu- is also managed by repeated procedures scheduled every 4 to mor necrosis factor-α antibody therapy management before 6 weeks to minimize the tract’s length and size. Once the tract and after intestinal surgery for inflammatory bowel disease: a is 2 to 3 cm long, the defect can be saucerized and the ileos- CCFA position paper. 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