Malformations Associated With Pediatric Congenital Cholesteatomas

OBJECTIVE To describe malformations associated with pediatric congenital cholesteatomas of the middle ear. STUDY DESIGN Retrospective study. SETTING Tertiary referral center. PATIENTS One hundred and seventy-three cases of middle ear congenital cholesteatoma (CC) in 171 children operated between 2007 and 2017. INTERVENTIONS Demographic, clinical, and surgical data were collected from operative reports. MAIN OUTCOME MEASURES We first described the type and rate of malformations associated with CC. Secondly, we compared cholesteatoma features in two subgroups: anterior superior (AS) versus posterior superior (PS) starting point. Third, we compared demographic, clinical, and surgical data between patients with and without malformation. RESULTS CC was associated with malformations in 17 cases (17/173; 9.8%). The main malformation was preauricular fistula (8/173; 4.6%). Other malformations were: one first branchial cleft, two labio palatine cleft, one nasal cyst, two preauricular fibrochondroma, and five other malformations. PS congenital cholesteatomas were diagnosed in older children (4.6 versus 8.6 years, p