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There are two types of cholesteatoma in children, congenital and acquired. The pathogenesis of each type is thought to be different. The pathogenesis of congenital cholesteatoma is thought to be the embryonic inclusion of squamous epithelium, whereas acquired cholesteatoma is thought to develop from various factors including recurrent otitis media and tubal insufficiency. We reviewed a series of 23 patients with congenital cholesteatoma and 27 patients with acquired cholesteatoma, aged 2-15 years, who were operated on at our institution between January 1981 and December 1990. Planned staged surgery was performed in all patients. In the first-stage operation, the canal-wall-up technique was used in all cases. About 12 months after the first operation, the second-stage operation was performed to investigate the course of the disease. The presence of recurrent and residual cholesteatoma was evaluated at this time. In the congenital cholesteatoma series, 11 cases of residual cholesteatoma (48%) were detected at the second-stage operation. Since recurrent cholesteatoma was not observed in the series of congenital cholesteatoma cases, the prognosis of these cases was considered to be good. On the other hand, in the acquired cholesteatoma series, 9 cases (33%) of recurrent cholesteatoma and 13 (48%) of residual cholesteatoma were detected at the second-stage operation. For the second-stage operation, the canal-wall-up technique was performed on 18 patients with no recurrence, whereas the canal-wall-down technique was used on all 9 patients with recurrence. Additional mastoid obliteration was performed on 7 of these 9 patients. The prognosis of the patients who underwent obliteration was good, and there was no recurrence of cholesteatoma after the second operation. From these observations, it was concluded that planned staged tympanoplasty was useful for eliminating recurrent and residual cholesteatoma. It is also apparent that, in order to prevent recurrent cholesteatoma, it is necessary to reduce the air space to compensate for poor eustachian tube ventilation.

Surgical Evaluation and Clinical Features of Cholesteatoma in Children.