Watanabe Akira, Gekka Tamaki, Arai Kota, Tsuneoka Hiroshi
a Department of Ophthalmology , The Jikei University School of Medicine , Tokyo , Japan.
Ophthalmic Genet. 2017 May-Jun;38(3):273-276. doi: 10.1080/13816810.2016.1193878. Epub 2016 Jul 18.
This is the first report of vitreous surgery for traction retinal detachment in a patient with type III Gaucher disease with multiple vitreous opacities.
A 16-year-old boy who was diagnosed with Gaucher disease at age two and was undergoing enzyme replacement therapy presented with numerous white opacities of varying sizes in the vitreous bodies of both eyes. Visual acuity was 20/40 in the right eye and 20/2000 in the left eye. The retina of the left eye was completely detached, and vitreous surgery was performed.
Liquefaction of the vitreous body was advanced, and the central part of the vitreous cavity contained almost no vitreous humor. The macular region was successfully aspirated with a vitreous cutter to form a posterior vitreous detachment. From the optic disk to the nasal side, however, posterior vitreous detachment formation was prevented by strong adhesions between the retina and the vitreous body. The traction retinal detachment of the posterior fundus improved after vitreous body resection alone.
Traction retinal detachment may occur as a result of severe vitreous liquefaction in cases of Gaucher disease with numerous vitreous opacities.
本文是关于III型戈谢病合并多发性玻璃体混浊患者行玻璃体手术治疗牵引性视网膜脱离的首例报告。
一名16岁男孩，两岁时被诊断为戈谢病，正在接受酶替代治疗，双眼玻璃体内出现大量大小不一的白色混浊物。右眼视力为20/40，左眼视力为20/2000。左眼视网膜完全脱离，遂行玻璃体手术。
玻璃体液化进展明显，玻璃体腔中央几乎没有玻璃体。用玻璃体切割器成功抽吸黄斑区，形成玻璃体后脱离。然而，从视盘到鼻侧，视网膜与玻璃体之间的强烈粘连阻止了玻璃体后脱离的形成。仅行玻璃体切除术后，眼底后部的牵引性视网膜脱离有所改善。
在有大量玻璃体混浊的戈谢病病例中，严重的玻璃体液化可能导致牵引性视网膜脱离。
