Congenital mastoid cholesteatoma: case series, definition, surgical key points, and literature review

Abstract

Objectives: We evaluate 3 new cases of congenital cholesteatoma confined to the mastoid process, and compare them with cases presented in the literature in order to better define this rare lesion.

Methods: We performed a retrospective chart analysis of all congenital cholesteatomas treated surgically in a tertiary referral and skull base center. We performed a complete analysis (history, radiologic, and surgical) of all patients with congenital cholesteatoma confined to the mastoid process; we then performed a literature review and compared our findings with the presented cases.

Results: The results of preoperative imaging were in line with the surgical findings. The most important surgical issue in this type of lesion was the management of the sigmoid sinus and the jugular bulb. Half of the cases previously reported in the literature appeared not to fulfill the definition criteria of a congenital cholesteatoma of the mastoid process.

Conclusions: Congenital cholesteatoma confined to the mastoid process is a rare lesion, and is even more exceptional upon critical review of the literature. Symptoms are often lacking or nonspecific, and although cases have a congenital origin, the diagnosis often is not made until adulthood. A combined congenital cholesteatoma group with middle ear and mastoid features seems to fill in the gap in the definition. Management of the sigmoid sinus and the jugular bulb is the most demanding surgical key point.