title: Significance of esophageal Crohn disease in children.
abstract: OBJECTIVE
  Esophageal Crohn disease (ECD) is more common than it was originally thought to be. Only limited information, however, is available regarding its significance and effect on clinical course in the pediatric population. The aim of the study was to determine the prevalence of ECD in our patient population and compare clinical features and severity of disease among patients with ECD and nonesophageal Crohn disease (NECD).
  
  
  METHODS
  Medical records of all patients with ECD diagnosed during a 12-year period based on specific endoscopic and histological criteria were reviewed and compared with a random group of patients with NECD.
  
  
  RESULTS
  During the study period, 81 (20%) patients with ECD were identified. Mean age at diagnosis was 12 (range 4-19 years) with a male predominance of 63%. Only 29 (36%) patients had symptoms suggestive of upper gastrointestinal involvement. Endoscopic ulcers were present in 45 (56%) of patients with ECD, whereas noncaseating granulomas were found in 10 (12%) of those patients. The majority (89%) of these patients had concomitant gastric and/or duodenal involvement. When compared with 160 random patients with NECD, patients with ECD had higher mean Pediatric Crohn Disease Activity Index scores (40.2 vs 23.9; P < 0.001), more penetrating-type disease (12% vs 2%; P = 0.001), and a greater frequency of perianal involvement (51% vs 33%; P = 0.005) at diagnosis. No differences, however, were noted between the 2 groups in terms of need for surgical resection throughout duration of follow-up.
  
  
  CONCLUSIONS
  Patients with ECD may represent a phenotype of Crohn disease with a more severe presentation. Patients with perianal disease at the time of initial physical examination should be considered for an upper endoscopy in addition to the colonoscopy to exclude esophageal involvement despite the absence of specific upper gastrointestinal symptoms. These observations should foster additional investigation into ECD phenotype to determine appropriate treatment and prognosis.
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Indiana University School of Medicine, Indianapolis, IN 46202, USA. rammoury@iupui.edu
authors.forename: Rana F
authors.initials: RF
authors.lastname: Ammoury
authors.forename: Marian D
authors.initials: MD
authors.lastname: Pfefferkorn
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003424:Crohn Disease / Q000150:complications*; D004724:Endoscopy; D004935:Esophageal Diseases / Q000150:complications* / Q000453:epidemiology; D005260:Female; D005767:Gastrointestinal Diseases / Q000150:complications* / Q000453:epidemiology; D006099:Granuloma / Q000150:complications / Q000453:epidemiology; D006801:Humans; D008297:Male; D015995:Prevalence; D012189:Retrospective Studies; D012720:Severity of Illness Index; D012737:Sex Factors; D055815:Young Adult