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Familial Exudative Vitreoretinopathy-A Case Report

病例報告-家族滲出性玻璃體視網膜病變

摘要


目的:報告一個家族滲出性玻璃體視網膜病變的疾病,探討此疾病的臨床特徵、眼底螢光血管攝影與治療。 方法:病例報告 結果:一位9歲的男性病患主訴雙眼視力模糊6個月,最佳矯正視力右眼為6/10左眼為6/20。眼底檢查發現雙眼非裂孔性視網膜剝離合併視網膜下纖維化。眼底螢光血管攝影發現周邊血管呈貝扇狀,雙眼顳側部顯示無血管灌流區域且有新生血管。此病患無早產兒病史且出生體重為正常,出生後並無使用氧氣治療。經手術治療後,雙眼視網膜均已復位。追蹤兩年後最佳矯正視力右眼為6/60左眼為6/10。 結論:視網膜下纖維化並非是家族滲出性玻璃體視網膜病變之常見特徵。早期的手術治療對於此疾病有較佳之癒後。

關鍵字

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並列摘要


Purpose: To report a case of familial exudative vitreoretinopathy (FEVR) and discuss the clinical manifestation, angiographic features and treatment of this disease. Method: Interventional case report Result: A healthy 9-year-old boy complained of progressive blurred vision in both eyes for 6 months. The presenting best-corrected visual acuity (BCVA) was 6/10 in the right eye and 6/20 in the left eye. Fundoscopy disclosed bilateral non-rhegmatogenous retinal detachment and subretinal fibrosis. Fluorescein angiography revealed avascular area with neovascularization at temporal periphery in both eyes. The temporal retinal vasculature terminated abruptly with scalloped borders adjacent to the avascular zone. He was born in full term without previous exposure to oxygen therapy. After surgical intervention, retina was attached in both eyes. Final BCVA was 6/60 in the right eye and 6/10 in the left eye. Conclusion: Subretinal fibrosis is an unusual feature in FEVR. Early surgical intervention is beneficial to this disease.

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