Management of acquired cholesteatoma in patients with craniofacial anomalies: an institutional experience

Purpose To analyze the surgical management of cholesteatoma in patients with craniofacial abnormalities, cleft lip/palate by reviewing the institutional experience. The secondary aim was to identify and describe the epidemiological profile of the collected data, and to relate the cleft palate and cholesteatoma. Design and methods This retrospective chart review includes 97 patients with craniofacial abnormalities and acquired cholesteatoma with anatomopathological proven in 118 ears. The following data were collected from the medical records between 1994 and 2018. Results The first surgery performed on 76 of the 118 ears (64.4%) was the wall up mastoidectomy, while 42 of the 118 ears (35.5%) received the wall down technique. During the follow-up period of these patients, which ranged from 2 to 29 years, with an average of 13.4 years (±5.88), 77 wall up (40.3%) and 114 wall down (59.6%) mastoidectomies were performed. This brought the total to 191 mastoidectomy surgeries in 118 ears of 97 patients. Of the wall up mastoidectomies, 65 of the 77 (84.4%) presented with cholesteatoma recurrence. In the wall down mastoidectomies follow up, there were new surgical approaches in 15 of the 114 procedures (13.1%), with 6 patients (5.2%) having anatomopathologically proven cholesteatoma recurrences and 9 (7.8%) having clinical instability for cavity cleaning without identification of disease recurrence. Conclusions Early approach with wall down/modified techniques guided by specific indication criteria may be more resolute, prevent multiple procedures, and preserve the bone pathway to facilitate possible future hearing rehabilitation in these patients.