先天性胆脂瘤(Congenital cholesteatoma)亦名原发性或真性胆脂瘤。多认为胚胎期外胚层上皮始基移位生长于颅腔颞骨等处发展而成。其机转与皮样囊肿生长相类似。可发生在颅骨、颌骨、鼻窦、扁桃体、咽侧、中耳、肝、脾、肾、膀胱、子宫、乳腺和中枢神经系统。通常所谓颞骨胆脂瘤系指慢性中耳炎病理发展过程上皮过度增生所致,而颞骨先天性胆脂瘤则甚为少见。本院自1979年以来共遇到两例,特报告如下。 Congenital cholesteatoma also known as primary or true cholesteatoma. More that the embryonic ectoderm epithelial base shift shift growth in the cranial cavity and other temporal bone development. Its rotation and dermoid cyst growth are similar. Can occur in the skull, jaw, sinuses, tonsils, pharynx, middle ear, liver, spleen, kidney, bladder, uterus, breast and central nervous system. The so-called temporal bone cholesteatoma refers to the pathogenesis of chronic otitis media epithelial hyperplasia, while the temporal bone congenital cholesteatoma is very rare. Our hospital has encountered two cases since 1979, the special report is as follows.