先天性膽脂瘤：17病例分析

Congenital Cholesteatoma: A Review of Seventeen Cases

方德詠(Te-Yung Fang) ； 洪朝明(Chao-Ming Hung)

《台灣耳鼻喉頭頸外科雜誌》 44卷4期 (2009/08) Pp. 95-104

https://doi.org/10.6286/2009.44.4.95

摘要

背景：先天性膽脂瘤很少見，常被診斷爲中耳其他疾病，直到手術時才被發現。但也偶爾被細心的醫師在診察聽力正常或傳音性聽障的病例時，從外觀正常的鼓膜發現而早期診斷。本文試從回溯性統計分析病例，來探討其特性及可行的早期診斷方法。
方法：國泰醫院自1992至2008年間有17例先天性膽脂瘤，回溯性瀏覽其病例，統計術中發現、手術方式、聽力變化及追蹤結果。
結果：17例包括5名男性（1名爲雙耳）11名女性；年齡2至38歲，中位數16歲。臨床表現以中至重度以上傳導性聽障爲主。局部檢查所見，15例鼓膜完整，其中8例可見中耳腔有白色腫塊，1例（2歲兒童）鼓膜已破損，1例（岩部膽脂瘤）鼓膜有針孔大小穿孔合併鼓室及上鼓室肉芽腫。術前17例聽力氣骨導差平均42 dB，膽脂瘤位置侷限在中鼓室前上象限1例，超過2象限並侵犯聽小骨7例，範圍廣泛已侵犯至乳突8例，位於顳骨岩部1例；其中2例有水平半規管瘻管，1例有前半規管壺腹部骨性缺損，5例已併發感染。術中所見有13例鉆骨缺損，10例鐙骨缺損，乳突氣化多良好。膽脂瘤外觀無明顯包膜，呈棉絮狀，且常深入乳突氣室。手術方式視膽脂瘤範圍及耳咽管功能而定，1例做鼓室成形術，3例做鼓室成形術併上鼓室鑿開術，8例做完壁式乳突切除併鼓室成形術，5例做開放式乳突切除術；其中7例手術分成兩階段，探查並做聽力重建，結果其中2例有殘餘膽脂瘤。術後聽力進步11例，退步1例，3例成感音性聽障，2例無術後聽力資料。
結論：對於不明原因、鼓膜完整的傳導性聽障兒童或年輕人，需懷疑先天性膽脂瘤的可能性，及安排電腦斷層或磁振造影檢查。治療以手術為主，如果膽脂瘤已擴及乳突，通常需要合併乳突切除術及聽力重建，且需建議定期影像追蹤。若病變範圍較廣，或鐙骨缺損，則需實施階段性手術。早期診斷可避免併發症產生及範圍大的手術，且有助聽力結果。

關鍵字

膽脂瘤 ； 先天性膽脂瘤

並列摘要

BACKGROUND: Congenital cholesteatoma is a rare disorder and is usually diagnosed as other middle ear disease initially until it is identified during an operation. Early diagnosis may occur when a physician carefully examines the appearance of the ear drum when there is normal or conductive hearing loss. Here we carry out a retrospective review of congenital cholesteatoma cases, analyzing and discussing their characteristics in order to identify possible approaches to early diagnosis.
METHODS: This is a retrospective review from 1992 to 2008. We have summarized the surgical findings, the surgical approaches, the results in terms of hearing and the postoperative follow up.
RESULTS: Seventeen congenital cases, made up of five males and eleven females having an median age of sixteen years, were identified. One boy had bilateral disease. The presenting symptoms were mainly moderate to severe hearing loss. Local findings revealed fifteen ears that had intact tympanic membranes; among these eight had white masses that could be seen through the intact drum. Two ears had a perforated drum. One was a two year-old girl and the other was petrous cholesteatoma with a pinpoint drum perforation and granulation at tympanum and epitympanum. The preoperative air-bone gap was 42 dB on average. According to Potsic's classification, there were cholesteatomas located within one quadrant of the mesotympanum only in one case, there were two or more quadrants with ossicular erosion in seven cases, and there was mastoid extension in eight cases. The cholesteatomas were located at petrous in one case. A lateral semicircular canal fistula was found in two cases. Ampulla of the superior semicircular canal bony defect was noted in one case. Mastoiditis was identified in five cases. Ossicular defects including the incus were present in thirteen cases and including the stapes in ten cases. The characteristics of congenital cholesteatoma that were found during the various operations were an absence of an obvious capsule, a lobulated appearance, seating was deep into well pneumatized mastoid air cells, and these was coexistence with rather healthy looking cells. The surgical approach used depended on the extent of disease and the functioning of the Eustachian tube. We performed intact canal wall tympanoplasty in eight cases, open cavity mastoidectomy in five cases, tympanoplasty with atticotomy in three cases, and tympanoplasty in one case. Staged operations were performed in seven cases for exploration of any residual disease and for hearing reconstruction. Two of the patients had residual disease. The postoperative air-bone gap improved in eleven cases, and worsened in one case. Three cases showed sensorineural hearing loss postoperatively. Two cases lacked a postoperative hearing result.
CONCLUSIONS: For children and young adults who have conductive hearing impairment and an intact tympanic membrane, we should consider the possibility of congenital cholesteatoma and arrange either computerized tomography or magnetic resonance imaging if necessary. Surgery is the primary treatment for congenital cholesteatoma. Postoperative follow up by imaging at regular intervals is suggested. If the disease is extensive or the stapes is defective, a staged operation should be scheduled. Early diagnosis can prevent complications and improve patient hearing.

並列關鍵字

cholesteatoma ； congenital cholesteatoma