title: Clinical Aspects and Surgical Outcomes of Congenital Cholesteatoma in 93 Children: Increasing Trends of Congenital Cholesteatoma from 1997 through 2012.
abstract: OBJECTIVE
  The recent increase in the reported incidence of congenital cholesteatoma (CC) may be secondary to the widespread use of otoendoscopy as well as an increased awareness of these lesions among primary care physicians. However, little research about CC has been conducted in a large group of patients. This study aimed to analyze the clinical characteristics of CC including the annual number of patients, symptoms, age at diagnosis, stage and type of disease, surgical techniques, recurrence, and postoperative complications.
  
  
  METHODS
  Retrospective chart review was performed for patients who met the inclusion criteria between January 1997 and June 2012.
  
  
  RESULTS
  Ninety-three patients underwent surgery for CC. The age at operation ranged from 12 months to 17 years (mean age, 6.1 years). The number of patients was less than 4 per year until 2005, but increased to more than 10 per year since 2008. CC was most commonly reported as an incidental finding (58.1%). The operative procedures included the transcanal myringotomy approach (46.2%), canal wall up mastoidectomy (37.6%), tympanoplasty (8.6%), and canal wall down mastoidectomy (7.5%). The recurrence rate was 20.4% and the complication rate was 12.9%. No patients with stage I CC had complications.
  
  
  CONCLUSIONS
  This study showed that the incidence of CC has recently increased notably. Most patients with stage I and II CC were completely cured by transtympanic surgery, and complication and recurrence rates increased according to the extent of disease. Early detection of CC is important to facilitate minimally invasive surgery and to reduce complication and recurrence rates.
authors.affiliation: Department of Otorhinolaryngology-Head and Neck Surgery, School of Medicine, Kyungpook National University, Daegu, Korea.
authors.forename: Hyun Soo
authors.initials: HS
authors.lastname: Cho
authors.affiliation: Department of Otorhinolaryngology-Head and Neck Surgery, School of Medicine, Kyungpook National University, Daegu, Korea.
authors.forename: Hak Geon
authors.initials: HG
authors.lastname: Kim
authors.affiliation: Department of Otorhinolaryngology-Head and Neck Surgery, School of Medicine, Kyungpook National University, Daegu, Korea.
authors.forename: Da Jung
authors.initials: DJ
authors.lastname: Jung
authors.affiliation: Department of Otorhinolaryngology-Head and Neck Surgery, School of Medicine, Kyungpook National University, Daegu, Korea.
authors.forename: Jeong Hun
authors.initials: JH
authors.lastname: Jang
authors.affiliation: Department of Otorhinolaryngology-Head and Neck Surgery, Daegu Veterans Hospital, Daegu, Korea.
authors.forename: Sang Heun
authors.initials: SH
authors.lastname: Lee
authors.affiliation: Department of Otorhinolaryngology-Head and Neck Surgery, School of Medicine, Kyungpook National University, Daegu, Korea.
authors.forename: Kyu-Yup
authors.initials: KY
authors.lastname: Lee
keywords: Congenital cholesteatoma; Incidence; Staging; Tympanoplasty