Bilateral congenital cholesteatoma: A case report and review of the literature

Objectives: Congenital cholesteatoma (CC) occurs less commonly than acquired cholesteatoma (AC), and bilateral CC (BCC) is even more rare with only 38 such cases having been reported in the past 42 years. Because of the rarity of this condition, providers confronted with cases of BCC may find it difficult to treat while balancing complete removal of disease, optimal hearing outcomes, and minimized surgical burden in the pediatric patient. This review alerts physicians that BCC occurs, highlights past presentations and management strategies, describes the considerations in treatment and offers an algorithm helpful in the management of BCC.

Methods: Review of a single case with extensive review of published reports from 1975 to 2017 pertaining to management of BCC.

Results: A five-year-old boy presented with bilateral congenital cholesteatoma. Tympanomastoidectomy was performed to remove cholesteatoma in the left ear then in the right ear months later. Ossicular chain reconstruction was deferred in both cases. Second look procedures revealed persistent cholesteatoma in both ears. In the descriptions of the 38 published BCC cases, the extent and location of the CC varied widely as did the approach to management. In the 18 cases that had descriptions of surgical management, four had second look procedures. In the 16 reports that described extent of cholesteatoma, 12 had the first of two or more operations on the ear with more extensive cholesteatoma.

Conclusions: Risks are increased for recidivism/recurrence and hearing impairment in children with BCC compared to children with unilateral CC. We present a novel algorithm for management of BCC that recommends surgery for cholesteatoma removal first in the more severely affected ear and delayed OCR for both ears. Simultaneous surgery may be considered in certain cases.