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I.はじめに
レックリングハウゼン病は,皮膚のカフェオレ斑や神経線維腫を主徴とし,骨病変,眼病変,脳脊髄腫瘍など多彩な症状を呈する全身性母斑症である。比較的稀な疾患ではあるが,約0.25%の頻度で生じるとされている1,2)。頭頸部病変も高頻度に認め,多彩な症状を示す。外耳道病変は比較的稀ではあるが,周囲に重要な組織が隣接しているため,特有の問題を生じる1~3)。
われわれは,レックリングハウゼン病における外耳道狭窄症例を経験し,その病理学的特性と対処法について文献的考察を加え報告する。
An 8-year-old boy with von Recklinghausen's disease(vRD) presented with a periauricular mass,external auditory canal stenosis,hearing loss and otalgia. Subtotal tumor resection was performed to reduce the mass volume around the external ear canal,because the mass was non-encapsuled and too diffuse to be enucleated. Its histopathology was plexiform neurofibroma. Periauricular toumor caused by vRD is often plexiform neurofibroma,which is too diffuse to be enucleated. In case of having complications,surgery should be considered. Total tumor resection is recommended when small enough to be removed safely. However,subtotal tumor resection is recommended when not small enough to be enucleated.
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