Area of Otolaryngology, Interdisciplinary Center for Biomedical Research (CIR), University Campus Bio-Medico, Rome, Italy.
Eur Rev Med Pharmacol Sci. 2012 May;16(5):699-700.
We present an additional very rare case of a congenital tympanic membrane cholesteatoma (CTMC) in the adult.
Case report and literature review of CTMC.
A 54-year old man was referred to us by his primary care physician who noted a white mass on the right tympanic membrane without prior history of otorrhea, tympanic perforations or previous otologic procedures. The pearl was about 5 mm diameter, centered on the umbo of a normal tympanic membrane (TM). The audiogram and the tympanogram was absolutely normal. CT confirmed a soft round shape tissue mass, located in the centre of the TM near umbo. The mass protruded both in the auditory canal and in the middle ear space, touching the malleus extremity, without any relationship with medial wall of the cavum tympani. A surgical excision was performed using a "minimal" retroauricolar transcanalar approach: the CTMC was located into the thickness of the TM, between epidermic and mucous layers. The ossicular chain was preserved intact. A partial myringoplasty (underlay technique) using a temporalis fascia graft was necessary. Histopathology confirmed a cystic cholesteatoma. After two months and one year follow-up, otoendoscopy showed a well-healed TM with a preserved normal audiogram and tympanogram.
This exceptional (probably the first reported) case showed the possible localization of the CC in the TM, also in the adult. Criteria for classification of a TM cholesteatoma as congenital and possible pathogenetic mechanisms are discussed.
我们报告一例成人先天性鼓膜胆脂瘤（CTMC）的罕见病例。
对 CTMC 进行病例报告和文献复习。
一名 54 岁男性因初级保健医生发现右侧鼓膜上有一个白色肿块而被转介给我们，该患者既往无耳漏、鼓膜穿孔或先前的耳科手术史。珍珠样物直径约 5 毫米，位于正常鼓膜（TM）的耳屏处。听力图和鼓室图完全正常。CT 证实了一个质地柔软的圆形组织肿块，位于 TM 中心靠近耳屏处。该肿块向耳道和中耳腔突出，触及锤骨末端，与鼓室内侧壁无任何关系。采用“最小”耳后经耳道入路行手术切除：CTMC 位于 TM 厚度内，位于表皮和黏膜层之间。听骨链保持完整。需要进行部分鼓膜成形术（下置法），使用颞肌筋膜移植物。组织病理学证实为囊性胆脂瘤。随访 2 个月和 1 年后，耳内镜检查显示 TM 愈合良好，听力图和鼓室图正常。
这个特殊的（可能是首例报道）病例显示了 CC 可能定位于成人的 TM 中。讨论了将 TM 胆脂瘤分类为先天性的标准和可能的发病机制。
