The role of planned two-stage surgery in the management of congenital cholesteatoma

Objective: The purpose of this study is to verify the role of "planned two-stage surgery" in the management of advanced congenital cholesteatoma regarding disease recurrence rates, complications and the need for salvage surgery. Method: Retrospective review of all congenital cholesteatoma under the age of 18 years underwent surgery from October 2007 to December 2021 in a single tertiary referral center. Patients with Potsic stage I/II who had closed-type congenital cholesteatoma received one-stage surgery. Advanced cases or those with open-type infiltrative congenital cholesteatomas underwent planned two-stage surgery. The second stage of surgery was performed 6-10 months after the first stage of surgery. Ossiculoplasty would be performed in the second operation if a significant air-bone gap was detected in the preoperative pure-tone audiometry test. Results: Twenty-four patients were included in the series. Six patients received one-stage surgery and no recurrence was noted in this group. The remaining 18 underwent planned two-stage surgery. Residual lesions found in the second operative phase were observed in 39% of patients who received planned two-stage surgery. Except for one patient whose ossicular replacement prosthesis protruded and two patients who had perforated tympanic membranes, none of the 24 patients required salvage surgery during follow-up (mean, 77 months after surgery), and no major complications occurred. Conclusions: Planned two-stage surgery for advanced-stage or open infiltrative congenital cholesteatoma could timely detect residual lesions to avoid extensive surgery and reduce complications.