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題名 | 多發齒源性角化囊腫--病例報告=Multiple Odontogenic Keratocysts--Case Report |
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作 者 | 黃威雄; 沈宗憲; 饒宇東; | 書刊名 | 中華民國耳鼻喉科醫學會雜誌 |
卷期 | 33:4 1998.08[民87.08] |
頁次 | 頁96-101 |
分類號 | 416.94 |
關鍵詞 | 多發齒源性角化囊腫; 痣樣基底細胞癌症候群; 哥林氏症候群; Multiple odontogenic keratocysts; Nevoid basal-cell carcinoma syndrome; Gorlin syndrome; |
語文 | 中文(Chinese) |
中文摘要 | 多發齒源性化囊腫(multiple odontogenic keratocysts)相當少見,臨床上 它常以某候群的諸多病變之一而表現, 這些症候群包括: 痣樣基底細胞癌症候群 (nevoid basal-cell carcinoma syndrome)、 Noonan 氏症候群、 Hunter 氏症候群及黏多糖貯積症 (muco-polysaccharidosis)。 當然它也可以是獨立的病變。在此提出 1 例 14 歲女性病人 , 有多發齒源性角化囊腫、 多處分歧肋骨 (bifid ribs)、 大腦鐮鈣化(calcification of falx cerbri)、蝶鞍橋 (bridging of sella turcica)、 脊柱前曲側彎 (kyphoscoliosis)、 雞胸 (pigeon chest)、腦性麻痺、智能不足及眼距過大 (hypertelorism) 等等問題,但皮 膚並無痣樣的病灶出現。此病例疑似痣樣基底細胞癌症候群,可能皮膚病變尚未出現。 |
英文摘要 | The disease of multiple odontogenic keratocysis is rare. It often presents as one manifestation in some syndromes such as nevoid basal cell carcinoma syndrome, Noonan syndrome, Hunter syndrome and mucopolysaccharidosis. However, sometimes, it is not associated with any syndrome and exists independently. This 14 year-old girl presented with multiple odontogenic keratocysts, bifid ribs, calcification of falx cerebri, bridging of sella turcica, kyphoscoliosis, pigeon chest, cerebral palsy, mental retardation and hypertelorism. No skin lesions such as basal cell carcinoma were found on presentation. She might be a victim of nevoid basal cell carcinoma syndrome. |
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