CAJ | 학술논문

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。
목적：분석총결섭골선천성담지류적병변부위、범위、대소、림상표현、영상학자료、진단、감별진단급수술경험，포괄수손면신경회복정황。방법회고성분석1999년1월지2013년4월진치적10례섭골선천성담지류환자적림상자료화진치규률。결과10례섭골선천성담지류환자중，년령위1～57세，남7례，녀3례，기중환이위좌측5례，우측5례。병변위우유돌구6례，기중1례환자외이도후벽수루파배；병변루급유돌구、암첨4례，술전출현면탄，면신경골관균이파배、라로，기중1례술중출현뇌척액루，여동기행뇌척액루수보술。본조균채취수술치료적방식，9례행개량개방식유돌근치술，기중1례술중동기행면신경감압+고실성형술；1례위인공이와식입환자동기행폐합식유돌착개고실성형술，동기식입인공이와전겁，술후어훈회복량호。소유병례술후1년복사CT등몰유발현담지류잔류혹복발。결론대선천성담지류통과병사、은역학、영상학자료등진행감별진단，가이주도조기진단，병선택합괄적수술입로。특별시대우암부선천성담지류，재출현면탄등병발증，급시통과CT、MRI등영상학자료，주도조기진단、조기치료，가능회피면오진화루진。
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 treat-ment, 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 cere-brospinal 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 tympano-mastoidectomy with simultaneous cochlear implantation was performed in 1 case, with satisfactory postoperative training out-comes. No residual or recurrent cholesteatoma was found on CT scan 1 year after operation. Conclusions For congenital cho-lesteatoma, 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 pa-ralysis or other complications,CT,MRI and other imaging data improve early diagnosis and treatment, and help the surgeon avoid wrong or missed diagnosis.