1. Perianal Fistulizing Crohn’s Disease Simple, Complex, Incision and Drainage, Fistulotomy, Setons, Diversion or Proctectomy ? OH MY!

2. 24 yr old male with a 1 ½ yr. h/o stricturing ileal disease s/p ileocecetomy and rectosigmoid Crohn’s disease. He is currently on Infliximab and Azathioprine but continues with recurrent and now worsening perianal pain with severe throbbing discomfort and swelling upon sitting especially for extended periods. Past MRI had revealed incidentally a very small fluid collection. He has had perianal discomfort on and off in the past treated with short courses of antibiotics resulting in drainage with resolution of swelling. He has discomfort on walking and especially sitting for extended periods. Now referred to colorectal clinic for surgical evaluation of persistent perirectal abscess/fistula. Case Study Perianal Fistulizing Crohn’s

3. What do we know? History – h/o rectosigmoid disease and recurring abscess. Symptoms – throbbing pain, periodically drains purulence, smells Testing – MRI showing fluid collection Treatments – Azathioprine and Infliximab – Cipro/flagyl QoL – constant annoyance – Difficulty sitting, sometimes walking, recurring abscess, difficult when job requires sitting in multiple meetings a day

4. Perianal Abscess abscess

5. What testing might be considered to evaluate abscess? Digital exam – feel fistula, induration, swelling - may be too painful MRI – helps delineate tracts and fluid collection, helps evaluate healing – may over report in some cases Anal Endosongraphy – good to evaluate tracts close to the anal canal but loss of resolution further away Exam under anesthesia – Very good to evaluate the area as usually painful and note presence of fluid collections Kamm, M, NG.S 2008 Clin Gastroenterol and Hepatol 6:7-10

6. Recent testing - MRI abd/pelvis Thickening rectal- sigmoid area with left lateral rectal area with small fluid collection

7. Exam Under Anesthesia Complex fistula opening was found in the rectum on the left lateral aspect. Wire probe placed through the opening and followed tract to an abscess cavity within the sphincter muscles to the outside of the perineum. The abscess was opened and purulent material drained through to the outside opening.

8. fistula Abscess

9. What surgical procedure would you recommend for this perianal abscess? Incision and drainage Fistulotomy Seton placement Diverting stoma

10. Abscess is superficial outside the sphincter muscles! Schwartz, D.A., J.H. Pemberton, and W.J. Sandborn, Diagnosis and treatment of perianal fistulas in Crohn disease. Ann Intern Med, 2001. 135(10): p. 906-18.

12. Seton keeps the fistula track open so abscess drains Abscess is within the sphincter muscles

13. Preoperative discussions? What major risk should be discussed related to perianal procedures? – Incontinence - damage to sphincters – Perineal tissue destruction - scarring What postoperative recommendations and patient education should you discuss for this case? – Hot sitz baths several times per day – Use of butterfly or anal incontinence pads to wick away moisture – For a seton left in with a circle tie: Move seton from side to side Sutured connection needs to be outside tract Recommend leaving a tail on the seton so cannot rotate inside the tract

14. In Perineal Crohn’s Evolution toward upfront aggressive COMBINED medical AND surgical therapy

15. Surgery for Perianal Crohn’s Disease Combination Therapy Initial Response (%) Recurrence (%) Time to Recurrence (Months) Infliximab 82793.6 EUA + Seton+ Infliximab 1004413 Regueiro M & Mardine H. Inflammatory Bowel Diseases, March 2003, 9(2):98-103 ACCENT 2 trial extended trial

16. Antibiotics often help to decrease inflammation in the short term – Metronidazole and/or Ciprofloxacin Patients generally start infliximab therapy after sepsis has been drained Often a seton is left in to control drainage, prevent recurrent sepsis and allow inflammation to resolve. Removal of a seton within a few weeks of starting therapy is necessary to facilitate track healing! In extensive complex fistulae may be longer Treatment of Perianal Fistulae Causey, Marlin et al Gastroenterol April 5 ( 2013) 58-63

17. Surgery for Crohn’s Disease Infliximab & Perineal Fistula For perianal fistulizing CD, repeat doses of Infliximab improves clinical and radiological outcomes, although complete radiologic healing occurs in a minority of patients! Rasul I et al. Am J Gastro2004:99:82-88

18. Surgery for Perineal Crohn’s Disease Summary Setons prevent sphincter damage by preventing recurrent abscess formation Presence of active proctitis reduces chance of the fistula healing, thus proctitis needs to be aggressively treated! Setons can be removed after a couple doses of a biologic when the tract is healing or left in long term if healing does not occur. If the tract appears healed or dry the seton can be removed. The perineum is re-examined regularly – MRI If fecal incontinence develops, proctectomy is discussed Kamm, M, NG.S 2008 Clin Gastroenterol and Hepatol 6:7-10

19. Cutting Seton – elastic band Tightening can occur every 2-3 weeks as tolerated. With each tightening, the seton cuts slowly through the sphincter tissue and heals the tissue behind. Eventually the seton falls out and has done it’s job! Chuang-Wei,Surgeon,1 June 2008 185-88

20. Healed Fistula tracts COMBINATION TREATMENT EUA, seton, Infliximab,AZA

21. Case Study #2 25 y/o gentleman who was referred for evaluation of uncontrolled perianal Crohn's disease originally noted symptoms in high school of increased stools and progressed to LLQ abdominal pain and diarrhea Colonoscopy in August 2011 which was notable for perianal CD with multiple fissures, deep ulcerations in the distal rectum as well as severe inflammation with ulceration extending up to the splenic flexure. TI was normal. Initiated on prednisone, mesalamine, and metronidazole. He was then started on adalimumab in late 2011, but no loading doses. SHx: Works in a factory building doors. States that normally he 'just comes home and goes to his room' after work, does not socialize much. FHx: Denies family history of IBD or CRC ROS: No F/C, No N/V. No SOB/CP. No HA. Denies dysphoria. No abdominal pain, + perianal discomfort + diarrhea + perianal skin wetness and lots of milky drainage. Patient smells of sickness and clothes wet.

22. He is notably despondent in the office and is not forthcoming about how he is feeling, wearing a hat pulled over eyes, poor eye contact and soft voice with paucity of words. He appears to be very uncomfortable sitting but states he is definitely sitting fine! Father does most of the talking.

23. Perineal Fistulizing Disease Perianal fistulizing disease can lead to substantial physical and emotional disease: – Pain – Discharge – Incontinence – Perineal and genital disfigurement – Slow resolution even with treatment  Patients often reluctant to seek medical care  Providers unfamiliar with nuances to manage the disease Causey, Marlin et al Gastroenterol April 5 ( 2013) 58-63

24. Complex fistular network 24

25. Evaluation/Testing/Surgical Procedure? –MRI or US? – EUA, I&D, setons, fistulotomy? –Stoma? Diverting/permanent?

26. MRI MRI : Changes in the descending colon and sigmoid colon compatible with CD. Extensive perianal and rectal fistulas and abscess collection extending into the gluteal soft tissues, perineum and scrotal sac.

27. Exam under anesthesia and setons

28. Returns now for follow up visit: Since his EUA he has remained on adalimumab, Ciprofloxacin, and metronidazole States he is feeling 'better' with setons but has had persistent daily perianal drainage, leakage/accidents of stool and has 5 BMs during day and 1-2 BMs at night. Still despondent, not sitting comfortably, not eating as fear of bowel movements Clearly patient is not doing well Perianal area will not heal with continued stool flow!

29. Chance of fistulae and rectum healing given his severe proctitis Diversion – Stoma Need for removal of colon/rectum Quality of life Depression Patient support network/education Patient Discussion and Education

30. Risk factors for Proctectomy 5 yr. Extensive fistula/abscess vs simple – 26% vs. 6% Severe Proctitis vs none or mild – 37% vs. 10% Severe proctitis and extensive fistula/abscess – 46% proctectomy rate Often Proctectomy performed only after a patient has “had enough” of their disease! Causey, Marlin et al Gastroenterol April 5 ( 2013) 58-63

31. Diversion does not alter the course of the disease! Patients undergoing diversion for perineal CD have <20% chance of successful restoration of intestinal continuity which is NOT improved with biologic therapy Hong MK et al Colorectal Dis 2011 13 (2); 171-6 However…..Fecal diversion is useful to quiet the perineum and promote healing: Prior to repairing an RV fistula Gives patients time to often realize a better quality of life with a stoma Staging prior to a completion proctectomy Fecal Diversion Causey, Marlin et al Gastroenterol April 5 ( 2013) 58-63

32. Option: Proctectomy? Patient: No way!!!! Provider: Pelvis too septic!

33. Option: Diverting stoma? Patient:Yes as a temporary step! Leaves door open for reconnection!

34. Takedown stoma and reconnect bowel? Keep stoma? Proctectomy? –risk of malignancy Often patients do not want to risk return of severe perianal disease and recognize life with the ileostomy is better!! Have him return in 6-12 months Now What? Kamm, M, NG.S 2008 Clin Gastroenterol and Hepatol 6:7-10

35. It is all about QUALITY OF LIFE! Staging procedures to get patient acceptance! Patient/family engagement at every step with shared decision-making!!