Liang W Q, Wang L, Gong S S, Wang W
Department of Otorhinolaryngology Head and Neck Surgery，Beijing Friendship Hospital，Capital Medical University，Beijing，100032，China.
Lin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2020 Jan;34(1):45-48. doi: 10.13201/j.issn.1001-1781.2020.01.011.
The clinical symptoms, imaging features and surgical treatment of congenital cholesteatoma are reported. The clinical data of 20 patients with congenital cholesteatoma diagnosed and treated in our hospital from January 2016 to May 2018 were retrospectively analyzed, including the age of onset, clinical symptoms, signs, audiology, HRCT of temporal bone, surgical methods and so on. In 20 patients with congenital cholesteatoma, the clinical manifestations were hearing loss in the affected ears, including 5 cases with ear fullness and 4 cases with tinnitus. There were 13 cases of local yellow-white shadow of tympanic membrane, 5 cases of normal tympanic membrane and 2 cases of tympanic membrane bombe. Pure tone audiometry showed conductive deafness in 14 cases and mixed deafness in 6 cases. CT scan of temporal bone showed that 15 cases had pneumatic type of mastoid, 4 cases had diploectic type and 1 case had sclerostic type. There were 15 cases of interspersed flocculent shadow and 5 cases of mass shadow. Among the 20 cases of chronic otitis media with cholesteatoma, 6 have cholesteatoma in epitympanum, 4 in tympanic cavity, 3 in tympanic cavity and epitympanum, 2 in mastoid and tympanic antrum, 1 in mastoid, tympanic antrum and tympanic cavity, 1 in tympanic antrum, tympanic cavity and petrous apex, 1 in tympanic cavity, hypotympanum, posterior tympanum and ostium tympanicum tubae auditivae, 1 in aditus ad antrum, epitympanum and posterior tympanum, 1 in tympanic cavity, epitympanum and posterior tympanum. Intact canal wall mastoidectomy and tympanoplasty were performed in 7 cases, open mastoidectomy and tympanoplasty in 5 cases, middle ear exploration and tympanoplasty in 4 cases, atticotomy with reconstruction and tympanoplasty in 3 cases and subtotal temporal bone resection in 1 case. For patients with intact tympanic membrane presenting with hearing loss, the tympanic membranes should be carefully examined, and thin-section CT and MRI of temporal bone should be performed in time to avoid missed diagnosis of congenital cholesteatoma. Once diagnosed, surgery should be performed as soon as possible.
报道了先天性胆脂瘤的临床症状、影像学特征及手术治疗方法。回顾性分析了2016年1月至2018年5月在我院确诊并治疗的20例先天性胆脂瘤患者的临床资料，包括发病年龄、临床症状、体征、听力学检查、颞骨高分辨率CT、手术方式等。20例先天性胆脂瘤患者中，患耳临床表现为听力下降，其中5例有耳闷，4例有耳鸣。鼓膜局部黄白色影13例，鼓膜正常5例，鼓膜膨隆2例。纯音听力测试显示传导性聋14例，混合性聋6例。颞骨CT扫描显示乳突气化型15例，板障型4例，硬化型1例。有散在絮状影15例，团块状影5例。20例慢性胆脂瘤型中耳炎中，上鼓室胆脂瘤6例，鼓室胆脂瘤4例，鼓室及上鼓室胆脂瘤3例，乳突及鼓窦胆脂瘤2例，乳突、鼓窦及鼓室胆脂瘤1例，鼓窦、鼓室及岩尖胆脂瘤1例，鼓室、下鼓室、后鼓室及咽鼓管鼓口胆脂瘤1例，鼓窦入口、上鼓室及后鼓室胆脂瘤1例，鼓室、上鼓室及后鼓室胆脂瘤1例。行完壁式乳突根治术及鼓室成形术7例，开放式乳突根治术及鼓室成形术5例，中耳探查及鼓室成形术4例，上鼓室切开重建及鼓室成形术3例，颞骨次全切除术1例。对于鼓膜完整而出现听力下降的患者，应仔细检查鼓膜，及时行颞骨薄层CT及MRI检查，以免漏诊先天性胆脂瘤。一旦确诊，应尽早手术。
