title: Acquired middle ear cholesteatoma in children with cleft palate: experience from 18 surgical cases.
abstract: OBJECTIVE
  To review an institutional experience with the surgical management of middle ear cholesteatoma in children with cleft palate.
  
  
  METHODS
  We analyzed retrospectively 18 children diagnosed with cleft palate who underwent surgery for acquired middle ear cholesteatoma between 2000 and 2007. The following data were recorded: age, sex, history of ventilation tube insertion, status of the contralateral ear, cholesteatoma location and extension, and surgical technique involved. Cholesteatoma recidivism, stable mastoid cavity and hearing levels were the main outcomes measured.
  
  
  RESULTS
  Follow-up ranged from 5 to 12 years (mean 8 years). Twelve children underwent planned staged canal wall up mastoidectomy: a residual cholesteatoma was found and removed during the second-look procedure in 2 ears (16.6%); two children (16.6%) showed a recurrent cholesteatoma and required conversion to canal wall down mastoidectomy. A modified Bondy technique was chosen in two children with an epitympanic cholesteatoma with an intact tympano-ossicular system, while in the remaining four subjects a canal wall down mastoidectomy was performed because of an irreparable erosion of the postero-superior canal wall: no cases of recurrent cholesteatoma were observed in these 6 children; revision mastoidectomy was needed in one patient for cavity granulation. A postoperative air-bone gap result of 0-20dB was achieved in 11 children (61.1%); in 5 cases (27.7%) postoperative air-bone gap was between 21 and 30dB, while in 2 (11.1%) was >30dB. Bone conduction thresholds remained unaffected in all cases.
  
  
  CONCLUSIONS
  Our results indicate that most cleft palate children with cholesteatoma can be managed with a canal wall up mastoidectomy with low complication rates. In extensive disease with large erosion of the canal wall as well in presence of a retraction pocket in the contralateral ear, a canal wall down mastoidectomy should be considered. In epitympanic cholesteatomas with an intact tympano-ossicular system and mesotympanum free of disease, the modified Bondy procedure is an effective surgical option. As in the general pediatric population, improvement or preservation of hearing can be obtained in most patients.
authors.affiliation: Department of Clinical and Experimental Medicine, Unit of Audiology and Pediatric Otorhinolaryngology, University of Parma, Italy. Electronic address: vincenzo.vincenti@unipr.it.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Vincenti
authors.affiliation: Department of Clinical and Experimental Medicine, Unit of Audiology and Pediatric Otorhinolaryngology, University of Parma, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Marra
authors.affiliation: Department of Clinical and Experimental Medicine, Unit of Audiology and Pediatric Otorhinolaryngology, University of Parma, Italy.
authors.forename: Barbara
authors.initials: B
authors.lastname: Bertoldi
authors.affiliation: Department of Clinical and Experimental Medicine, Unit of Audiology and Pediatric Otorhinolaryngology, University of Parma, Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Tonni
authors.affiliation: Department of Clinical and Experimental Medicine, Unit of Audiology and Pediatric Otorhinolaryngology, University of Parma, Italy.
authors.forename: Maria Silvia
authors.initials: MS
authors.lastname: Saccardi
authors.affiliation: Department of Clinical and Experimental Medicine, Unit of Audiology and Pediatric Otorhinolaryngology, University of Parma, Italy.
authors.forename: Salvatore
authors.initials: S
authors.lastname: Bacciu
authors.affiliation: Department of Clinical and Experimental Medicine, Unit of Audiology and Pediatric Otorhinolaryngology, University of Parma, Italy.
authors.forename: Enrico
authors.initials: E
authors.lastname: Pasanisi
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D018424:Cholesteatoma, Middle Ear / Q000150:complications / Q000601:surgery*; D002972:Cleft Palate / Q000150:complications* / Q000601:surgery; D004432:Ear, Middle / Q000473:pathology* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006309:Hearing; D006801:Humans; D008297:Male; D008416:Mastoid / Q000601:surgery*; D013517:Otorhinolaryngologic Surgical Procedures / Q000009:adverse effects / Q000379:methods*; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Cholesteatoma; Cleft palate; Hearing results; Mastoidectomy; Middle ear surgery; Tympanoplasty