Clinical Features and Outcomes of Crohnʼs Disease of the Ileal Pouch in Pediatric Patients

Abstract

Purpose: Restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the surgical treatment of choice for children with ulcerative colitis (UC) and is frequently performed for patients with indeterminate colitis (IC) who require surgery. However, a subset of patients with an ileal pouch may develop Crohn's disease (CD) of the pouch. The aim of this study was to describe the clinical characteristics of pediatric patients with CD of the pouch and to compare these patients to adult patients with CD of the pouch. Methods: All pediatric patients with CD of the pouch were identified from our prospectively maintained Pouchitis Database between 2002 and 2008. Demographic, clinical, and endoscopic data were collected. A control group that consisted of 100 adult patients with CD of the pouch was used for comparison. CD of the pouch was classified into 3 phenotypes: inflammatory, fibrostenotic, and fistulizing. Results: A total of 25 pediatric patients with CD of the pouch were identified, predominantly Caucasian (96%) and female (56%) with a mean age of 11.2 (±4.4) years at the time of initial diagnosis with inflammatory bowel disease. Nineteen patients had a pre-operative diagnosis of UC and 6 patients had IC. Compared to adult patients, a diagnosis of toxic megacolon prior to IPAA was more common in the pediatric group (56% vs. 11%, p < 0.001), as well as the use of pre-operative biologics (44% vs. 10%, p < 0.001). CD of the pouch was inflammatory in 56%, fibrostenotic in 8% and fistulizing in 36%. The most common clinical presentation was diarrhea in 72%, followed by abdominal pain in 60% and hematochezia in 24% of the patients. Pediatric patients with CD of the pouch had a significantly shorter duration of inflammatory bowel disease prior to pouch creation (1 yr vs. 7 yrs, p < 0.001) and a shorter duration of their pouch prior to CD diagnosis (7 yrs vs. 10 yrs, p = 0.027). Children with CD of the pouch were more likely to be hospitalized than their adult counterparts (p < 0.001). Pouch failure, defined as the creation of a diverting ileostomy with or without pouch excision, occurred in 20% of pediatric patients with CD of the pouch compared to 7% of adult patients with a trend towards statistical significance (p = 0.06). Conclusion: In comparison to adult patients, CD of the pouch in children occurred earlier after IPAA and was associated with higher rates of hospitalization and pouch failure. A pre-operative diagnosis of toxic megacolon and use of biologics were more common in the pediatric population.