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Enophthalmos Associated with the Absence of the Greater Sphenoid Wing and Concurrent with Retinitis Pigmentosa: A Case Report
蝶骨大翼缺損致眼球內陷合併視網膜色素變性－病例報告
摘要
目的：報告一罕見病例，病患因蝶骨大翼缺損而導致眼球內陷，且合併有視網膜色素變性。 方法：個案報告 結果：一位23歲女性主訴左眼球下陷及雙眼漸近性視力下降。理學檢查發現左眼球下陷並有自發性眼球搏動。眼底鏡檢查發現雙眼有廣泛性視網膜色素變性，左眼視網膜呈現高度近視的眼底變化，電腦斷層掃描發現病患左側蝶骨大翼缺損，且左眼球軸明顯變長。 結論：蝶骨大翼缺損是一種罕見的疾病，可能導致眼球內陷或外凸，它常與第一型神經纖維瘤病相關。本病例普未發現有第一型神經纖維瘤病的臨床表徵或家族史，但發現有雙眼視網膜色素變性。以往的文獻並未報告過蝶骨缺損與視網膜色素變性之間的關聯性。此外，施行球後注射時必須留意眼窩骨骼是否有缺損，以免注射入顱內。
關鍵字
無資料並列摘要
Purpose: To report a rare case of enophthalmos associated with the absence of the greater sphenoid wing simultaneous with retinitis pigmentose (RP). Method: An observational case report Result: A 23-year-old woman suffered from sunken globe in the left eye and progressively blurred visio in both eyes. Physical examination revealed enopthalmos with spontaneous pulsation in the left globe. Ophthalmoscopy examination revealed diffuse retinal pigmentary changes in both eyes and a myopic retinal change in the left eye. CT scan showed left greater shhenoid wing dysplasia and left globe elongation. Conclusions: Enophthalmos with dysplasia of sphenoid greater wing is a rare condition, which is usually accociated with type I neurofibromatosis. Wereport on a patient with enophthalmos caused by the greater sphenoid wing dyspiasia with simultaneous retinitis pigmentosa, but without stogmata or family history of neurofibromatosis. To our knowledge, there is no direct correlation between enophthalmos caused by the absence of the greater sphenoid wing and RP, and this case may be either coincidental or a new entity. When performing retrobulbaranesthesia for ocular surgery on patients with enophthalmos, care must be taken to identify any bony structural defect of the orbit to avoid care must be taken to identify any bony structural defect of the orbit to avoid penetration of anesthetic agents into the brain tissue.