title: A staging system for congenital cholesteatoma.
abstract: OBJECTIVE
  To develop a staging system for congenital cholesteatoma in predicting the likelihood of residual disease.
  
  
  METHODS
  Retrospective analysis of data from a case series, to identify predictors of residual disease.
  
  
  METHODS
  Tertiary care pediatric hospital.
  
  
  METHODS
  Children undergoing surgical removal of congenital cholesteatoma. There were 156 patients, with 160 cholesteatomas; 4 children had bilateral disease.
  
  
  METHODS
  Each case was scored as to quadrants of the middle ear involved, ossicular involvement, and mastoid extension.
  
  
  METHODS
  Surgically confirmed residual disease at any time after the initial procedure.
  
  
  RESULTS
  Four stages were defined as follows: stage I, disease confined to a single quadrant; stage II, cholesteatoma in multiple quadrants, but without ossicular involvement or mastoid extension; stage III, ossicular involvement without mastoid extension; and stage IV, mastoid disease. There was a strong association between stage and residual disease, ranging from a 13% risk in stage I to 67% in stage IV.
  
  
  CONCLUSIONS
  This simple staging system may be particularly useful in standardizing the reporting of congenital cholesteatoma and in adjusting for severity in evaluating outcomes. It also provides information that is useful in counseling parents.
authors.affiliation: Division of Pediatric Otolaryngology, 1 Wood Bldg, The Children's Hospital of Philadelphia, 324 S 34th St, Philadelphia, PA 19104, USA. potsic@email.chop.edu
authors.forename: William P
authors.initials: WP
authors.lastname: Potsic
authors.forename: Daniel S
authors.initials: DS
authors.lastname: Samadi
authors.forename: Roger R
authors.initials: RR
authors.lastname: Marsh
authors.forename: Ralph F
authors.initials: RF
authors.lastname: Wetmore
mesh_terms: D002648:Child; D002675:Child, Preschool; D002781:Cholesteatoma / Q000151:congenital* / Q000473:pathology* / Q000601:surgery; D004428:Ear Neoplasms / Q000151:congenital* / Q000473:pathology* / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D009367:Neoplasm Staging / Q000379:methods*; D018365:Neoplasm, Residual; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012720:Severity of Illness Index; D016896:Treatment Outcome