Objective: The study compares the clinical features of patients with congenital cholesteatoma seen in our institution over the most recent 10 years versus those seen in the previous decade. Furthermore, this study includes a systematic review and meta-analysis investigating whether differences in site predilection of congenital cholesteatoma exist between patients from Asian and Western countries.
Data sources: The retrospective review consisted of 31 cases encountered from 2001 to 2011 as compared with 40 cases previously described. For systematic review, data were retrieved from PubMed, EmBase, and the Japan Medical Abstracts Society Database (January 1995 through May 2012).
Study selections: Articles addressing site predilection of congenital cholesteatoma were selected with no language preference.
Data extraction: The database was searched using the keywords "congenital cholesteatoma." After critical review of 408 studies, 27 studies were eligible for quantitative synthesis.
Data synthesis: A retrospective analysis of our departmental cases showed that more than 70% (8/11) of the Potsic's stage I or II cases had a predilection for the anterior-superior quadrant (ASQ). None of the Stage III (17) or IV (3) cases had a similar site predilection. Consistent with our previous chart review, Stage III was the most common, comprising more than 60% of cases. Meta-analysis of the 27 eligible studies provided sufficient information regarding locations of congenital cholesteatoma, and Asian studies showed less frequent ASQ involvement (overall estimate, 0.54; 95% confident interval [CI], 0.49-0.59) when compared with Western studies (overall estimate, 0.76; 95% CI, 0.73-0.80). Moreover, the involvement of posterior-superior quadrant (PSQ) in Asian studies was estimated as 0.69 (95% CI, 0.64-0.73), which was higher than that associated with involvement of ASQ.
Conclusion: In contrast to Western studies, PSQ was more frequently involved than ASQ in Asian studies of congenital cholesteatoma. The posteriorly located lesions might have a completely distinct pathophysiologic origin when compared with lesions originating from the ASQ.