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摘要


背景:耳部先天性膽脂瘤目前病例報告有逐漸增加的趨勢,尤其是小兒中耳腔先天性膽脂瘤。先天性膽脂瘤在中耳好發部位,考察國外文獻,不同作者有不同統計。本文將分析所經歷之病例,報告此病之臨床表現。方法:從1984至2011年,在台北醫學大學附設醫院及萬芳醫院耳鼻喉科共診斷出12例小兒先天性中耳腔膽脂瘤。診斷年齡從1歲到14歲5個月,平均4歲10月大。其中有8名男性,4名女性。所有病例皆為單側,8例位於左耳,4例在右耳。12例中有11例接受高解析度電腦斷層評估其範圍,且膽脂瘤在中耳的位置皆用硬式耳用內視鏡記錄。其中7例可見耳膜後之白色腫塊主要位於前上象限,4例在後上象限,1例位於中耳腔下半部。依Potsic等人在2002年提出先天性膽脂瘤分期之標準,本系列12例中,stage I者7例,stage II者2例,stage III者3例。其中12例中有11例接受手術的治療。結果:經過5月至15年,平均6.5年的追蹤,有1例在5年6月後發現膽脂瘤的復發,接受開放腔第3型鼓室成形術;有1例在4年2月後乳突及頂鼓室出現膽脂肉芽腫,引起外耳道狹窄,而再次接受手術,並以PORP重建聽小骨鏈;1例在8年後頂鼓室一直凹陷形成後天性頂鼓室乳突膽脂瘤,亦再次接受修正式乳突切除術。其餘8例術後皆順利並未復發。接受手術的11例中有6例術後聽力正常,4例氣骨導差20至25 dB,1例有35 dB的傳導性聽力障礙。結論:在本研究中發現小兒先天性中耳腔膽脂瘤好發於左側及男性,左右及男女比例皆為2:1。若能早期診斷,則完整將病灶切除及保存患者聽力是可以預期的。

關鍵字

膽脂瘤 先天性膽脂瘤 中耳

並列摘要


BACKGROUND: The incidence of congenital cholesteatoma of the middle ear in children has an increasing trend of reported cases in the literature. There is racial difference of the most common locations of this lesion in the middle ear in the literature.METHODS: From 1984 to 2011, a total of 12 cases of pediatric congenital middle ear cholesteatoma were treated at Department of Otolaryngology, Taipei Medical University hospital and Taipei Medical University-Wan Fang hospital. The age of the patient at diagnosis ranged from 1 year to 14 years and 5 months, with a median age of 4 years and 10 months. There were 8 boys and 4 girls. All patients had unilateral involvement: 8 cases involved the left ear and 4 involved the right. Of all patients, the location of the cholesteatoma was recorded by otoscopic photography. The extent of the cholesteatoma was evaluated with high resolution CT of the temporal bone in 11 of 12 cases. In 7 of 12 cases, the cholesteatoma was manifested as a retrotympanic white mass at anteriorsuperior middle ear quadrant, 4 cases at posterior-superior quadrant and 1 case at inferior middle ear cavity. Eleven of the 12 cases undergone surgical treatment.RESULTS: The extent of follow ups ranged from 5 months to 15 years with an average of 6.5 years. There were no recurrence of the cholesteatomas in 8 cases, however, there was 1 case with recurrence of cholesteatoma, 1 case with acquired attic retraction cholesteatoma and 1 case with occurrence of large cholesterol granuloma at atticomastoid area; and these 3 cases received revised operation. The postoperative hearing was normal in 6 of 11 operated cases; 4 cases had air-bone gap between 20 and 25 dB and 1 case with 35 dB conductive hearing loss.CONCLUSIONS: A male and left side preponderance of 2:1 were noted in our series of congenital cholesteatoma of the middle ear in children from Taiwan. Curative removal and preservation of normal hearing can be expected when early diagnosis of congenital cholesteatoma of the middle ear is made.

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