Clinical Aspects and Surgical Outcomes of Congenital Cholesteatoma in 93 Children: Increasing Trends of Congenital Cholesteatoma from 1997 through 2012

Abstract

Background and ObjectivesThe 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.Subjects and MethodsRetrospective chart review was performed for patients who met the inclusion criteria between January 1997 and June 2012.ResultsNinety-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.ConclusionsThis 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.

Concurrent Occurrence of Congenital Ossicular Anomaly and Localized Cholesteatoma: Series of 10 Cases

“…Additionally, several features of the CCs in this study were consistent with the findings from previous reports on this disorder [6][7][8][9][10][11]. For instance, unilateral conductive hearing loss was the most frequent presentation.…”

“…The incidence of CC is reportedly less than 1 per 100,000 children [4,5]. It mostly occurs in boys, with average ages of 4-14 years at diagnosis [6][7][8][9][10][11].…”

Clinical aspects and surgical outcomes of pediatric cholesteatoma in a tertiary care teaching hospital

“…17 The study done by Cho et al also shown similar mean age of 6.1 years. 13 Though this condition is congenital, often the diagnosis is delayed because CC may grow for years without causing signs or symptoms and, having grown without early detection. 18 In the present study, all the cases have unilateral ear involvement with right ear more involved.…”

“…In a study done by Cho et al, all 93 patients had unilateral ear involvement. 13 Particular attention must be paid to the status of the ears of children to complain about symptoms associated with cholesteatoma, especially when presentations are subtle or unilateral. 19 Out of 46 patients, 14 (30.43%) patients were asymptomatic and CC were found incidental in the present study.…”

“…Again, the type and approach of surgical intervention is determined by the location, type, and stage of the lesion as well as by the surgeon's preference and experience. 13 Cholesteatoma in children is customarily viewed as a separate and special entity as it presents the otolaryngologist with a verity of diagnostic and therapeutic challenges. So, this was planned with objective of studying the clinical aspects and surgical outcomes of pediatric cholesteatoma.…”