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目的分析结晶样视网膜色素变性的荧光素眼底血管造影(FFA)特点,进一步探索其发病机制。设计回顾性病例系列。研究对象结晶样视网膜色素变性患者。方法北京同仁医院眼科中心2004～2006年门诊诊断的结晶样视网膜色素变性患者32例。所有患者均行FFA检查。主要指标FFA表现。结果全部患者FFA显示后极部斑驳状、点状透见荧光。背景荧光部分增高,部分减弱。视盘高荧光或部分高荧光8例。2例环以低荧光。动脉血管变细18例,但其中仅3例出现血管充盈迟缓。4例视网膜血管闭塞,且全部位于周边部。3例视网膜后极部血管渗漏。仅1例出现无灌注区。6例黄斑拱环结构破坏或结构不清。结论结晶样视网膜色素变性的FFA特点显示:其病变主要是视网膜色素上皮细胞改变和脉络膜毛细血管的萎缩,同时累及神经视网膜及其视网膜血管组织。
Objective To analyze the characteristics of fluorescein fundus angiography (FFA) of crystalline retinitis pigmentosa and further explore its pathogenesis. Design retrospective case series. Study object Crystal-like retinitis pigmentosa patients. Methods 32 cases of patients with crystalline retinitis pigmentosa diagnosed by out-patient department of Beijing Tongren Hospital Eye Center from 2004 to 2006 were enrolled. All patients underwent FFA examination. FFA performance of the main indicators. Results All patients with FFA showed mottled mottled, punctate see through fluorescence. Part of the background fluorescence increased, partially weakened. 8 cases of high fluorescence or partial high fluorescence. 2 cases with low fluorescence. 18 cases of arterial thinning, but only 3 cases of vascular filling slow. 4 cases of retinal vascular occlusion, and all located in the periphery. Three cases of posterior retina vascular leakage. Only one case showed no perfusion area. Six cases of macular arch ring structure or unclear structure. Conclusion Crystalline-like retinitis pigmentosa of FFA features: the main pathological changes of the retinal pigment epithelium and choroidal capillary atrophy, involving both the retina and retinal vascular tissue.