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Anorectal stenosis (ARS) is a rare complication of Crohn's disease (CD) and a risk factor for both fecal diversion (FD) and proctectomy,[1,2] especially in patients who failed anti-tumor necrosis factor-¦Á (anti-TNF) therapy.[3,4] Herein, we presented a case of a 17-year-old female diagnosed with CD at age 7, with inflammation located in the small bowel and colon (Paris classification: L3L4b). Due to corticosteroid dependency, infl iximab with concomitant azathioprine was introduced at age 9 and discontinued two years after, because of the appearance of a 3 cm tumor-mass, which resembled a lymphoma, in the oral cavity. The histopathologic examination confirmed orofacial granulomatosis. The therapy with anti-TNF agent (adalimumab) was continued. While treated with adalimumab and azathioprine, she presented with symptoms of ileus at the age of 14. The cause of the obstruction was ARS (4 cm in length), impassable by the coloscope, which was resolved by endoscopic balloon dilatation (EBD). The dose of adalimumab was increased to 80 mg per week and azathioprine was continued. Despite this therapy, she needed EBD every 3-5 months because of repeated obstructive ARS. She suffered from extreme fatigue and was not able to attend school. Pediatric Crohn's disease activity index (PCDAI) was 45. At that time, surgical therapy with FD was planned. Colonoscopy revealed severe proctitis with ARS and a simple endoscopic score for CD (SES-CD) of 11 in the rectum (Fig. A). However, a trial with vedolizumab (VDZ) was started prior to surgical therapy at age 16. The response to VDZ was assessed at week 10; she was in clinical remission (PCDAI=5), feeling energetic and again able to attend school. Colonoscopy revealed a dramatic endoscopic improvement (Fig. B) with SES-CD of 3.
The management of CD with ARS is challenging. Our patient had failed anti-TNF therapy, therefore a trial involving the biologic agent VDZ with a different mechanism of action was a logical treatment option. VDZ is a selective monoclonal antibody against ¦Á4¦Â7-integrin, which targets leukocyte trafficking in the gastrointestinal tract (GIT).[5] VDZ has not been approved for use in pediatric CD, although there are clinical trials that report its effectiveness and safety in adult CD.[6] Our case demonstrates that VDZ may represent a viable treatment option in severe pediatric CD patients with refractory ARS intended for surgical therapy. After one year of the VDZ therapy, our patient remains in clinical and endoscopic remission without the need for EBD or surgical treatment.
Darja Urlep, Rok Orel Department of Gastroenterology, Hepatology and Nutrition, University Children's Hospital Ljubljana, University Medical Center, Bohoričeva 20, 1000 Ljubljana, Slovenia Email: darja.urlep@kclj.si
References
1 Galandiuk S, Kimberling J, Al-Mishlab TG, Stromberg AJ. Perianal Crohn disease: predictors of need for permanent diversion. Ann Surg 2005;241:796-801.
2 Brochard C, Siproudhis L, Wallenhorst T, Cuen D, d'Halluin PN, Garros A, et al. Anorectal stricture in 102 patients with Crohn's disease: natural history in the era of biologics. Aliment Pharmacol Ther 2014;40:796-803.
3 Singh S, Ding NS, Mathis KL, Dulai PS, Farrell AM, Pemberton JH, et al. Systematic review with meta-analysis: faecal diversion for management of perianal Crohn's disease. Aliment Pharmacol Ther 2015;42:783-792.
4 Strong S, Steele SR, Boutrous M, Bordineau L, Chun J, Stewart DB, et al. Clinical practice guideline for the surgical management of Crohn's disease. Dis Colon Rectum 2015;58:1021-1036.
5 Bryant RV, Sandborn WJ, Travis SP. Introducing vedolizumab to clinical practice: who, when, and how? J Crohns Colitis 2015;9:356-366.
6 Sandborn WJ, Feagan BG, Rutgeerts P, Hanauer S, Colombel JF, Sands BE, et al. Vedolizumab as induction and maintenance therapy for Crohn's disease. N Engl J Med 2013;369:711-721. doi: 10.1007/s12519-017-0038-1
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