结晶样视网膜色素变性的荧光素眼底血管造影特点分析

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。

Characteristics of fundus fluorescent angiography in patients with crystalline retinitis pigmentosa

Objective To analyse the characteristics of fundus fluorescent angiography in patients with crystalline retinitis pigmentosa and to explore its pathogenesy further.Design Retrospective case series.Participants Patients with retinitis pigmentosa. Methods All 32 patients with crystalline retinitis pigmentosa diagnosed in Tong Ren Hospital from the year 2004 to 2006 were examined with FFA.Main Outcome Measures Manifestation of FFA.Results FFA of all the eyes showed mottling in the post pole. Some eyes had weaker background fluorescence than normal,but some had stronger ones.8 patients (16 eyes) had high fluorescent optic disk,2 of them were circled by weak fluorescence.18 patients(36 eyes)had thin retinal arteries,in which there were only 3 eyes had delayed filling. Vascular occlusion happened in four eyes, which all located in the peripheral fundus.Fluorescein dye leakage from retinal vascular happened in 3 patients,which all located in the post pole area.Nonperfusion of the retinal vasculars was only found in one patient.Damaged or unclear macular structure was found in 6 patients.Conclusion The characteristics of FFA shows that the damage of the fundus in patients with crystalline retinitis pigmentosa are mainly the alternation of retinal pigment epithelium and the atrophy of ehoroidal capillaries,the neural retina and retinal vessel are also damaged.