先天性耳道狹窄併膽脂瘤及耳後膿瘍—病例報告

Congenital Aural Stenosis with Cholesteatoma and Retroauricular Abscess-Case Report

賴政光(Chen-Kwang Lai) ； 陳祖傑(Chu-Jei Chen)

《中華民國耳鼻喉科醫學雜誌》 37卷6期 (2002/10) Pp. 465-469

https://doi.org/10.6286/2002.37.6.465

摘要

外耳道約在胚胎第21週開始形成，這期間有任何發展停頓，便會有耳道閉鎖或狹窄，這情形易併有小耳症和中耳腔發育不全。耳道的狹窄易使剝落之表皮角質積聚而有形成膽脂瘤的可能，有時會以狹窄耳道及畸形耳郭周圍反覆性發作之膿瘍來表現，但臨床上易被忽略。彰化秀傳紀念醫院於2000年5月經歷1名9歲女孩，主訴左側耳郭從小就略小，長期有惡臭性耳漏及耳痛，且耳後有一持續分泌惡臭膿液之病兆多年，曾至外科診所診治經反覆切開引流但無改善。理學檢查發現左側耳道狹窄且為Gr I之小耳症，耳道有膿液釋出，耳後有一膿瘍，並持續有膿液釋出；顳骨電腦斷層掃描顯示狹窄之耳道內有一擴大並充滿軟組織之空腔；左耳純音聽力檢查為70dB，氣骨導差間距為48dB，經診斷為小耳症及先天性耳道狹窄併有外耳道膽脂瘤及耳後膿瘍。於是為她安排外耳道重建手術，術中發現外耳道有一惡臭之銀白碎屑堆積，將之移除後發現鼓環形成萎縮板，中耳腔狹窄，鎚、砧骨黏連成一塊，砧、鐙骨分離，鐙骨足板活動正常。術後24個月，不再有耳漏、耳痛及耳後膿瘍之形成。由此病例得知，在畸型的耳郭周圍發現膿瘍，應考慮是否有膽脂瘤的形成。

關鍵字

膽脂瘤 ； 小耳症 ； 先天性外耳道狹窄 ； 耳後膿瘍

並列摘要

Formation of the external auditory canal typically commences around the 21st week of fetal development. Failure of meatal-plug reabsorption during this developmental period results in congenital aural atresia or stenosis, and this lesion can be combined with microtia and hypogenesis of middle ear cavity. Aural stenosis with intractable retroauricular abscess indicates the possibility of epithelial cyst or cholesteatoma, however, it is easily overlooked in a clinical setting. In May 2000, a nine-year-old girl presented at our outpatient department with left microtia. Her medical history included intermittent foul otorrhea, otalgia and tender erythematous swelling over the left retroauricular region for a number of years. The tender swelling was incised and drained by a general practitioner, to no avail. Microtia (Grade I), left EAC stenosis with purulent otorrhea, and retroauricular erythematous swelling with purulent discharge were noted. A pure tone audiogram of left ear revealed an average conductive hearing loss of 70 dB, with an air-bone gap of 48 dB. High-resolution computed tomography of the temporal bone revealed a cloudy shadow in the stenotic canal. Under the impression of congenital aural stenosis with EAC cholesteatoma and retroauricular abscess, canaloplasty and tympanoplasty were performed. A foul whitish debris was noted in the stenotic meatus. After debris was removed, a bony atresic plate was noted. Malleus-incus fusion and incus-stapes joint dislocation were noted in a narrow middle-ear cavity. After removal of the bony mass, a PORP was inserted. Twenty-narrow middle-ear cavity. After removal of the bony mass, A PORP was inserted. Twenty-four months after the operation, the otorrhea, otalgia and retroauricular abscess had subsided. Hence, recurrent painful swelling near the congenital anomaly of the ear despite conservative treatment indicates the possibility of epithelial cyst or cholesteatoma, and further otological radiographys should be arranged.

並列關鍵字

cholesteatoma ； microtia ； congenital aural stenosis ； retroauricular abscess