title: Distinct phenotypes of children with perianal perforating Crohn's disease.
abstract: OBJECTIVE
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA.
authors.forename: Scott S
authors.initials: SS
authors.lastname: Short
authors.forename: Marla C
authors.initials: MC
authors.lastname: Dubinsky
authors.forename: Shervin
authors.initials: S
authors.lastname: Rabizadeh
authors.forename: Sharmayne
authors.initials: S
authors.lastname: Farrior
authors.forename: Dror
authors.initials: D
authors.lastname: Berel
authors.forename: Philip K
authors.initials: PK
authors.lastname: Frykman
mesh_terms: D000038:Abscess / Q000209:etiology* / Q000601:surgery; D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001004:Anus Diseases / Q000209:etiology* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D003082:Colectomy / Q000706:statistics & numerical data; D003131:Combined Modality Therapy; D003424:Crohn Disease / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000601:surgery; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007081:Ileostomy / Q000706:statistics & numerical data; D016015:Logistic Models; D008297:Male; D015999:Multivariate Analysis; D010641:Phenotype; D011379:Prognosis; D011446:Prospective Studies; D012003:Rectal Fistula / Q000209:etiology* / Q000601:surgery; D012042:Registries; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents
keywords: Crohn’s disease; Inflammatory bowel disease; Perforating; Perianal Crohn’s disease; Seton