Distinct phenotypes of children with perianal perforating Crohn’s disease

Abstract

Purpose Perianal perforating disease (PF) has been reported in approximately 15% of children with Crohn’s disease (CD). It is unknown whether children who present with PF at the time of diagnosis have a different course than those that develop PF while on therapy. Methods From a prospective, single institution observational registry of children diagnosed with CD, we identified children with perianal perforating CD, defined as perianal abscesses and/or fistulae. Patients who presented with perianal perforating CD (PF-CD0) were compared to those who developed perianal perforating CD (PF-CD1) after initial diagnosis. Results Thirty-eight of 215 (18%) children with CD had PF-CD during a median follow up of 4.5 years. Patients with PF-CD0 (n = 26) tended to be more likely male (81% vs. 50%, p = 0.07) and younger (9.3 yrs vs. 12.5 yrs, p = 0.02). PF-CD1 (n = 12) patients were more likely to require diverting ileostomy (42% vs. 8%, p = 0.02) and colectomy (33% vs. 4%, p = 0.03). Multivariable analysis predicted increased rate of diverting ileostomy in the PF-CD1 group (p = 0.007, OR 19.1, 95% CI 1.6–234.8). Conclusion Pediatric CD patients who develop PF while on therapy for CD have a more severe phenotype and are more likely to require diverting ileostomy or colectomy compared to those who present with PF-CD.

Variable outcome in infantile-onset inflammatory bowel disease in an Asian cohort

“…A review in the literature did not reveal any cases of CD with spontaneous remission without immunosuppression or other medical therapy[31,32]. Thus even though the clinical, colonoscopic and histological features in this patient closely resembled that of CD, the child was classified as having IBD-U.…”

Perianal Crohn's Disease in Children and Adolescents

“…Children with perianal disease have worse clinical outcomes, greater inflammatory activity, more frequent rectal and jejunal involvement, higher prevalence of inflamed skin tags and granulomas, need for more aggressive treatment at diagnosis, impaired growth, and they are more likely to require a diverting enterostomy compared with patients without perianal disease. [15][16][17] Patients presenting with noninflamed skin tags and/or fissures without perianal sepsis seem to have similar clinical, endoscopic, and imaging findings than patients without perianal disease, suggesting that isolated skin tags and/or fissures are not signs of the severe phenotype. 15 In large cohort studies, male sex and initial corticosteroid therapy has been shown to associate with an increased risk to develop perianal disease after Crohn's diagnosis.…”

Cone-like resection, fistulectomy and mucosal rectal sleeve partial endorectal pull-through in paediatric Crohn's disease with perianal complex fistula

“…One girl, previously followed for syndromic diarrhoea by home parenteral nutrition, presented with perianal rectal Crohn's-like disease involvement. Median Perineal Disease Activity Index (PDAI) was 5 (range: [3][4][5][6][7][8][9][10][11][12].…”

“…Different approaches have been described for the treatment of complex fistula in children, including simple drainage, mobilization of tissue flaps, seton placement, fistulotomy, anus-sparing proctocolectomy, and defunctioning ileostomy [6][7][8]. However, the risk of complications remained high, with more than one procedure for recurrence in 29-50% of cases [8][9][10].…”