Fibrous Dysplasia Associated with Optic Atrophy-A Case Report

纖維性發育不良合併視神經萎縮－病例報告

丁佩卿(Pei-Ching Ding) ； 蔡榮坤(Rong-Kung Tsai) ； 孫仲毅(Chung-Yi Sun)

《中華民國眼科醫學會雜誌》 38卷2期 (1999/06) Pp. 268-272

https://doi.org/10.30048/ACTASOS.199906.0010

摘要

本文報告一位十三歲女性罹患纖維性發育不良合併視神經萎縮的臨床和影像學上的表現。她在最近的三到四個月內逐漸喪失右眼視力，而且發現她的雙側瞼頰不對稱。眼科檢查顯示她的右眼視力僅剩下在眼前30cm處可辨手指數，右眼有凸眼，瞳孔光反射異常，及視神經萎縮。她的過去史中沒有皮膚色素沈着，性早熟，或其他內分泌異常。電腦斷層顯示右側顏面骨有散在性的增厚及右側視神經管狹窄的現象。較晚作的磁振掃描發現其病灶仍然在惡化。我們提出此罕見的病例並討論其病理生理學。

並列摘要

We report the clinical and radiological features of a 13-year-old girl with fibrous dysplasia. She has suffered from progressive loss of vision in the right eye for 3 or 4 months and facial asymmetry for many years. Ophthalmological examination revealed reduced visual acuity to finger counting at 30cm, proptosis, Marcus Gunn pupil, and optic atrophy in the right eye. She had no history of skin pigmentation, precocious puberty, or other endocrine abnormalities. CT scan showed the diffuse thickening of right facial bone with ground-glass appearance and narrowed right optic canal. Later, magnetic resonance imaging revealed a progression of the lesion. We report this rare case and pathophysiology of fibrous dysplasia.

並列關鍵字

fibrous dysplasia ； craniofacial bones ； optic atrophy