目的：探讨不同病变阶段视网膜色素变性患者的脉络膜血管状态。方法：回顾性分析云南省第二人民医院眼科2000年1月至2015年4月诊断为原发性视网膜色素变性的患者226例(452眼)的眼底特征，并复习相关文献，重点分析总结脉络膜血管情况。结果：31例(62眼)病变前期患者，荧光素眼底血管造影显示动脉期脉络膜血管及视网膜血管充盈正常，未出现充盈延迟或缺损现象。25例(50眼)病变早期患者，荧光素眼底血管造影显示动脉前期可见脉络膜背景荧光显示，部分脉络膜毛细血管未同时充盈，动脉期时上述部分完成充盈。106例(112眼)病变中期患者，荧光素眼底血管造影显示动脉期出现部分脉络膜毛细血管萎缩区，仅能看到残存的粗大脉络膜血管，随造影过程的进展，此区域并未出现充盈，即呈现永久的脉络膜毛细血管充盈缺损。64例(128眼)病变晚 期患者荧光素眼底血管造影显示，广泛的脉络膜毛细血管萎缩区，其间可见残存的脉络膜粗大血管，至造影晚期均呈现充盈缺损，萎缩区边缘随造影过程呈强荧光表现。结论：荧光素眼底血管造影可显示脉络膜血管萎缩变化情况，这一指标可作为反映不同病变阶段视网膜色素变性患者病情进展变化的重要依据。

Objective: To investigate clinical characteristics of choroid in different stages of retinitis pigmentosa. Methods: The characteristics of fundus, visual conditions and characters of choroid of 226 cases (452 eyes) patients with retinitis pigmentosa in No. 2 People’s Hospital of Yunnan Province from Jan.2000 to Apr.2015 were retrospectively analyzed. Results: Fundus fluorescein angiography of 31 cases (62 eyes) before early stage showed:  arterial choroidal and retinal vascular filling normal, filling delay or defect phenomenon does not be observed. Fundus fluorescein angiography 25 cases (50 eyes) patients in early disease showed: preliminary choroidal artery background fluorescence was displayed, at the same time, part of the choriocapillaris was not filling, the filling was completed in arterial stage. Fundus fluorescein angiography of 106 cases (112 eyes) patients in the medium-stage showed: arterial phase appears part choriocapillaris atrophy area, thick choroidal vessels can be seen, with the progress angiography procedure, filling was not be observed in this area, which presents permanent choriocapillaris filling defect. Fluorescein angiography of 64 cases (128 eyes) in patients in advanced stage showed widespread choriocapillaris atrophy area, during which thick choroidal vessels remaining filling defect in late stage, atrophic area with a contrast edge high fluorescence performance. Conclusion: Fluorescein angiography can show choroidal atrophy changes, it can be used as an indicator to assess the progression of retinal changes in patients with retinitis pigmentosa.

报告1例2019年1月因无色素性视网膜色素变性而就诊的病例。患者因发现视力差，常规眼科检查及全身的检查未发现异常，给予眼底荧光造影后确诊。给予眼底荧光造影后最终确诊为罕见的无色素性视网膜色素变性，防止了疾病的漏诊和误诊。对于缺乏视网膜色素变性典型的三联征的无色素性视网膜色素变性患者，临床要谨防漏诊，眼底荧光血管造影(fundus fluorescein angiography，FFA)可明确诊断。

We reported a case of achromatic retinitis pigmentosa admitted to the hospital in January 2019. The patient was diagnosed by fundus fluorescein angiography because of poor vision, and no abnormality was found by routine ophthalmological examination and general examination. After fundus fluorescein angiography (FFA), a rare non-pigmented retinitis pigmentosa was finally diagnosed, which prevented misdiagnosis and missed diagnosis of the disease. For the patients without typical triad of retinitis pigmentosa, we should pay attention to missing the diagnosis in clinical practice. FFA can make a definite diagnosis of retinitis pigmentosa.