【摘要】 目的分析总结颞骨先天性胆脂瘤的病变部位、范围、大小、临床表现、影像学资料、诊断、鉴别诊断及手术经验,包括受损面神经恢复情况。方法回顾性分析1999年1月至2013年4月诊治的10例颞骨先天性胆脂瘤患者的临床资料和诊治规律。结果 10例颞骨先天性胆脂瘤患者中,年龄为1~57岁,男7例,女3例,其中患耳为左侧5例,右侧5例。病变位于乳突区6例,其中1例患者外耳道后壁受累破坏;病变累及乳突区、岩尖4例,术前出现面瘫,面神经骨管均已破坏、裸露,其中1例术中出现脑脊液漏,予同期行脑脊液漏修补术。本组均采取手术治疗的方式,9例行改良开放式乳突根治术,其中1例术中同期行面神经减压+鼓室成形术;1例为人工耳蜗植入患者同期行闭合式乳突凿开鼓室成形术,同期植入人工耳蜗电极,术后语训恢复良好。所有病例术后1年复查CT等没有发现胆脂瘤残留或复发。结论对先天性胆脂瘤通过病史、听力学、影像学资料等进行鉴别诊断,可以做到早期诊断,并选择合适的手术入路。特别是对于岩部先天性胆脂瘤,在出现面瘫等并发症,及时通过CT、MRI等影像学资料,做到早期诊断、早期治疗,可能会避免误诊和漏诊。更多还原

【Abstract】 Objective To investigate the location, range of involvement,size,clinicial symptoms and imaging data of congenital cholesteatoma in temporal bone, to report authors’ experiences with its differential diagnosis and surgical treatment, as well as complications including damage to function of the facial nerve. Methods This was a retrospective review of ten cases of congenital temporal bone cholesteatoma treated from January 1999 to April 2013. Clinical data and treatment evaluate were evaluationed. Results The age of the 10 patients(7 males and 3 females) ranged from 1 to 57 years. The lesion involved the left ear in 5 cases and right ear in 5 cases. The lesion was located in the mastoid area in 6 cases(including 1 showing external auditory canal wall damage), and the petrous bone in 4 cases(with also facial paralysis). Intraoperative cerebrospinal fluid leakage in 1 case, which was repaired during surgery. Modified canal wall down tympanomastoidectomy was performed in 9 cases, including 1 with simultaneous facial nerve decompression and tympanoplasty. Canal wall up tympanomastoidectomy with simultaneous cochlear implantation was performed in 1 case, with satisfactory postoperative training outcomes. No residual or recurrent cholesteatoma was found on CT scan 1 year after operation. Conclusions For congenital cholesteatoma, early detection through history, audiology and imaging improves differential diagnosis and makes early diagnosis and selection of appropriate surgical approach possible. Particularly in congenital petrous bone cholesteatoma with facial paralysis or other complications,CT,MRI and other imaging data improve early diagnosis and treatment, and help the surgeon avoid wrong or missed diagnosis.更多还原