Chen J M, Schloss M D, Manoukian J J, Shapiro R S
Department of Otolaryngology, McGill University, Montreal, Quebec, Canada.
J Otolaryngol. 1989 Feb;18(1):44-8.
Congenital cholesteatoma, once considered to be a rarity, has shown an exponential increase in its incidence as evidenced by numerous clinical studies over the past decade. No explanation has been offered for this phenomenon. A review of the literature indicates that the definition of "congenital cholesteatoma" has strayed from the original one formulated by Derlacki and Clemis, which included a cholesteatoma medial to an intact tympanic membrane, without prior history of aural infections. Allowing patients with recurrent otitis media into studies may have contributed to a "false positive" rise in the incidence of this condition. This article reviews 19 cases of cholesteatomas behind intact tympanic membranes, treated at the Montreal Children's Hospital over a 10-year period. They represent 22% of the entire cholesteatoma population. There appears to be two groups of patients, each with a distinct clinical presentation that correlates well with the surgical outcome. Whether this implies different pathogenetic mechanisms remains unclear.
先天性胆脂瘤，曾被认为是一种罕见病，但过去十年众多临床研究表明其发病率呈指数级增长。目前尚未对这一现象作出解释。文献综述显示，“先天性胆脂瘤”的定义已偏离Derlacki和Clemis最初制定的定义，即完整鼓膜内侧的胆脂瘤，且无耳部感染病史。将复发性中耳炎患者纳入研究可能导致了这种疾病发病率的“假阳性”上升。本文回顾了蒙特利尔儿童医院在10年期间治疗的19例完整鼓膜后胆脂瘤病例。它们占整个胆脂瘤患者群体的22%。似乎有两组患者，每组都有独特的临床表现，且与手术结果密切相关。这是否意味着不同的发病机制尚不清楚。
