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.

目的：探讨中心性晕轮状视网膜脉络膜萎缩不同病变阶段的眼底影像学特征。方法：回顾分析中心性晕轮状视网膜脉络膜萎缩患者眼部检查及眼底荧光血管造影(fundus fluorescein angiography，FFA)检查，分析不同病变阶段荧光素眼底血管造影的影像学特征，总结该病发展转归的临床规律。结果：I期：眼底彩色照相：黄斑区轻度色素紊乱，可累及或未累及中心凹。FFA示：中心凹附近高荧光，RPE和脉络膜毛细血管的萎缩面积及程度尚不足以透见下方粗大的脉络膜血管。II期：眼底可见一类圆形的低色素区域，荧光造影可见与眼底彩照对应的高荧光区，随造影过程延长可见萎缩区荧光素渗漏。III期：黄斑区萎缩灶外围边界模糊，其中可见一部分边界清晰、稳定的完全萎缩灶。FFA：萎缩灶内边界清晰的部分视网膜色素上皮细胞(retinal pigment epithelium，RPE)及脉络膜完全萎缩，周边在造影晚期可见未完全萎缩的脉络膜毛细血管渗漏区域。IV期：黄斑区边界清晰的视网膜脉络膜萎缩灶，黄斑中心凹累及。FFA可见与眼底像相对应的脉络膜萎缩灶，其中可透见粗大的脉络膜血管。周围部分未见活动性荧光素渗漏，病变稳定。结论：荧光素眼底血管造影能反映的脉络膜萎缩程度是不同阶段病变阶段的主要指标。

Objective: To investigate the imaging features of fundus fluorescein angiography in central areolar choroidal dystrophy at different stages. Methods: Ocular examination and fundus fluorescein angiography were carried out, imaging features of fundus ffuorescein angiography were retrospectively analyzed. Results: Stage I: fundus photography showed mild macular pigment disorders, which was involved with the fovea. Fundus fluorescein angiography (FFA): high fluorescence was close to the fovea, the area and the extent of RPE and choroidal capillaries atrophy were insufffcient to see through the thick choroidal vessels beneath. Stage II: fundus showed a round of low pigment area, fluorescein angiography showed high fluorescence corresponding region, with the angiography procedure the ffuorescein leakage could be observed. Stage III: boundaries of macular atrophy lesions were unclear which showed part of a clear boundary, stable completely atrophy lesions. FFA: part of retinal pigment epithelium (RPE) and choroidal uncompletely atrophy surrounded was visible, in the late of angiography choroidal atrophy capillary leakage could be seen. Stage IV: boundary of macular retinal choroidal atrophy was clear, foveal involvement. FFA: choroidal atrophy lesions were clear which could be seen through the thick choroidal vessels. Fluorescein leakage disappeared and the disease was stable. Conclusion: The extend of choroidal atrophy in fundus ffuorescein angiography is an important indictor reflecting the progression of different stages. 

To report the case of a patient who presented with idiopathic choroidal neovascularization (CNV) as the first sign of multiple evanescent white dot syndrome (MEWDS). A 25-year-old woman presented with recent onset of decreased vision and metamorphopsia in the right eye. The results of fundoscopic examination, fluorescein angiography, and optical coherence tomography (OCT) were compatible with a diagnosis of idiopathic CNV, which was treated with one intravitreal injection of bevacizumab. Five years later, the patient returned complaining of photopsia and decreased vision in the same eye. The fundoscopic examination showed typical signs of MEWDS. After 3 months, recurrence of CNV was observed in the same eye. In conclusion, idiopathic CNV might be the only manifestation of a subclinical occurrence of MEWDS. In this case, it was followed by a recurrence of MEWDS and subsequent reactivation of CNV. 

To report the case of a patient who presented with idiopathic choroidal neovascularization (CNV) as the first sign of multiple evanescent white dot syndrome (MEWDS). A 25-year-old woman presented with recent onset of decreased vision and metamorphopsia in the right eye. The results of fundoscopic examination, fluorescein angiography, and optical coherence tomography (OCT) were compatible with a diagnosis of idiopathic CNV, which was treated with one intravitreal injection of bevacizumab. Five years later, the patient returned complaining of photopsia and decreased vision in the same eye. The fundoscopic examination showed typical signs of MEWDS. After 3 months, recurrence of CNV was observed in the same eye. In conclusion, idiopathic CNV might be the only manifestation of a subclinical occurrence of MEWDS. In this case, it was followed by a recurrence of MEWDS and subsequent reactivation of CNV. 

The present study reports a case of a patient with choroidal neovascularization (CNV) associated with pseudoxanthoma elasticum (PXE). We observed the functional and anatomical improvement of the patient treated with intravitreal vascular endothelial growth factor (VEGF) inhibitor bevacizumab. The study also systematically searched the database for similar cases to provide a literature review. Data concerning the clinical features, treatment strategies and outcomes were extracted and analyzed. Retrospective interventional case report and systematic literature review. A 56-year-old healthy Chinese woman with CNV secondary to PXE was reported. Examinations included best corrected visual acuity (BCVA), biomicroscopy, optical coherence tomography (OCT), fluorescein and indocyanine green angiography and digital fundus photography. The patient managed with intravitreal anti-vascular endothelial growth factor (anti-VEGF) injections (bevacizumab 1.25 mg/0.05 mL). The Cochrane Library, PubMed, OVID, and UpToDate databases were searched using the term pseudoxanthoma elasticum or Gr?nblad-Strandberg syndrome with the limits English. Articles that predated the databases were gathered from current references. Fundus examination revealed angioid streaks bilaterally and CNV in left eye (LE). After the patient underwent three intravitreal injections of bevacizumab, the LE showed absorption of the subretinal fluid and shrinkage of the CNV. Visual acuity (VA) was improved in her treated LE. Bevacizumab treatment was well tolerated with no adverse events reported. Approximately ten articles about 45 patients (49 eyes) describing CNV secondary to angioid streaks in PXE treated with anti-VEGF were found in the literature search. In the present case, bevacizumab of an initial three injection loading dose, achieved maintenance of visual function in the treatment of CNV associated with angioid streaks in PXE. Literature articles concluded that the intravitreal application of anti-VEGF is highly efficient for improving and stabilizing the lesion as well as the eyesight. So we believe that anti-VEGF therapy can be a great choice of treatment for CNV secondary to angioid streaks related PXE.

The present study reports a case of a patient with choroidal neovascularization (CNV) associated with pseudoxanthoma elasticum (PXE). We observed the functional and anatomical improvement of the patient treated with intravitreal vascular endothelial growth factor (VEGF) inhibitor bevacizumab. The study also systematically searched the database for similar cases to provide a literature review. Data concerning the clinical features, treatment strategies and outcomes were extracted and analyzed. Retrospective interventional case report and systematic literature review. A 56-year-old healthy Chinese woman with CNV secondary to PXE was reported. Examinations included best corrected visual acuity (BCVA), biomicroscopy, optical coherence tomography (OCT), fluorescein and indocyanine green angiography and digital fundus photography. The patient managed with intravitreal anti-vascular endothelial growth factor (anti-VEGF) injections (bevacizumab 1.25 mg/0.05 mL). The Cochrane Library, PubMed, OVID, and UpToDate databases were searched using the term pseudoxanthoma elasticum or Gr?nblad-Strandberg syndrome with the limits English. Articles that predated the databases were gathered from current references. Fundus examination revealed angioid streaks bilaterally and CNV in left eye (LE). After the patient underwent three intravitreal injections of bevacizumab, the LE showed absorption of the subretinal fluid and shrinkage of the CNV. Visual acuity (VA) was improved in her treated LE. Bevacizumab treatment was well tolerated with no adverse events reported. Approximately ten articles about 45 patients (49 eyes) describing CNV secondary to angioid streaks in PXE treated with anti-VEGF were found in the literature search. In the present case, bevacizumab of an initial three injection loading dose, achieved maintenance of visual function in the treatment of CNV associated with angioid streaks in PXE. Literature articles concluded that the intravitreal application of anti-VEGF is highly efficient for improving and stabilizing the lesion as well as the eyesight. So we believe that anti-VEGF therapy can be a great choice of treatment for CNV secondary to angioid streaks related PXE.

A 45-year-old female presented with typical features of posterior scleritis in her left eye with visual acuity of 20/252. After treatment with oral steroids and immunosuppressive drugs, at 2 months follow-up, posterior scleritis resolved and visual acuity improved to 20/50. Five months later she presented with vision loss (20/160) associated with active choroidal neovascular membrane (CNVM) close to scar. Significant choroidal thinning (subfoveal choroidal thickness =137 microns), compared to fellow eye (subfoveal choroidal thickness =247 microns) was noted. Two doses of intravitreal bevacizumab (IVB) were given at 1 month interval. At 9 months follow-up, her visual acuity was maintained at 20/160 with scarred CNVM. In conclusion, IVB is safe and efficacious in treatment of inflammatory CNVM secondary to posterior scleritis. Choroidal changes after posterior scleritis could be contributory factor for formation of CNVM.

A 45-year-old female presented with typical features of posterior scleritis in her left eye with visual acuity of 20/252. After treatment with oral steroids and immunosuppressive drugs, at 2 months follow-up, posterior scleritis resolved and visual acuity improved to 20/50. Five months later she presented with vision loss (20/160) associated with active choroidal neovascular membrane (CNVM) close to scar. Significant choroidal thinning (subfoveal choroidal thickness =137 microns), compared to fellow eye (subfoveal choroidal thickness =247 microns) was noted. Two doses of intravitreal bevacizumab (IVB) were given at 1 month interval. At 9 months follow-up, her visual acuity was maintained at 20/160 with scarred CNVM. In conclusion, IVB is safe and efficacious in treatment of inflammatory CNVM secondary to posterior scleritis. Choroidal changes after posterior scleritis could be contributory factor for formation of CNVM.

暴发性脉络膜上腔出血(subchoroidal expulsive hemorrhage，SEH)是内眼手术中罕见且严重的并发症，广东省人民医院眼科收治1名因晶状体完全脱位继发青光眼的女性患者，73岁，其手术过程中发生SEH，现报告如下。通过回顾病例，讨论及分析SEH的原因、危险因素及治疗。

Subchoroidal expulsive hemorrhage (SEH) is one of the rarest and worst complications of intraocular surgery. We treated one patient with secondary glaucoma due to complete dislocation of the lens, who developed SEH during the surgery. In this case report, the causes, risk factors and treatment of subchoroidal expulsive were discussed and analyzed by reviewing the case.

      息肉状脉络膜血管病变(polypoidal choroidal vasculopathy,PCV)是亚洲人群中常见的致盲性眼病，发生大出血并发症后严重危害视力且预后差。PCV大出血包括视网膜下出血(subretinal hemorrhage,SRH)和玻璃体积血(vitreous hemorrhage,VH)。SRH的危险因素包括较长病程、簇型PCV、息肉状病灶不消退、合并视网膜色素上皮脱离；其治疗方式包括抗血管内皮生长因子药物、光动力疗法、激光、玻璃体腔注气、眼内注射组织纤溶酶原激活剂、玻璃体切割术或联合治疗等方式，其中，黄斑中心凹是否受累和出血时间是影响治疗方式选择的主要因素。发病年龄较大、白细胞计数较高、天门氨酸转移酶和丙氨酸转氨酶的比值较高、活化部分凝血活酶时间较长、曾行光动力疗法、有玻璃体腔注药治疗史、SRH面积大、出现视网膜色素上皮脱离的PCV患者发生VH的风险高。浓厚的VH通常需行玻璃体切割术，其手术时机和手术方式的选择是临床关注的焦点。鉴于目前PCV大出血的危险因素尚不完全明确、治疗方面也尚未达成共识，需要开展相关临床研究，提供更多依据。

      息肉状脉络膜血管病变(polypoidal choroidal vasculopathy,PCV)是亚洲人群中常见的致盲性眼病，发生大出血并发症后严重危害视力且预后差。PCV大出血包括视网膜下出血(subretinal hemorrhage,SRH)和玻璃体积血(vitreous hemorrhage,VH)。SRH的危险因素包括较长病程、簇型PCV、息肉状病灶不消退、合并视网膜色素上皮脱离；其治疗方式包括抗血管内皮生长因子药物、光动力疗法、激光、玻璃体腔注气、眼内注射组织纤溶酶原激活剂、玻璃体切割术或联合治疗等方式，其中，黄斑中心凹是否受累和出血时间是影响治疗方式选择的主要因素。发病年龄较大、白细胞计数较高、天门氨酸转移酶和丙氨酸转氨酶的比值较高、活化部分凝血活酶时间较长、曾行光动力疗法、有玻璃体腔注药治疗史、SRH面积大、出现视网膜色素上皮脱离的PCV患者发生VH的风险高。浓厚的VH通常需行玻璃体切割术，其手术时机和手术方式的选择是临床关注的焦点。鉴于目前PCV大出血的危险因素尚不完全明确、治疗方面也尚未达成共识，需要开展相关临床研究，提供更多依据。

息肉样脉络膜血管病变(polypoidal choroidal vasculopathy,PCV)是一种以脉络膜异常分支血管网和血管末端息肉状脉络膜血管扩张灶为特征的眼底疾病。(indocyanine green angiography,ICGA)是诊断PCV的金标准，可见异常分支血管网、息肉样高荧光。(optical coherence tomography,OCT)可见色素上皮脱离(pigment epithelial detachments,PED)、双层征、指状突起、PED切迹、脉络膜增厚等。基于 OCT 的影像学研究表明，PCV属于肥厚型脉络膜谱系疾病(pachychoroid disease spectrum,PCD)，其特征是脉络膜增厚、脉络膜大血管层(Haller层)扩张，伴有毛细血管层(Ruysch层)和中血管层(Sattler层)变薄，其发病机制可能涉及脉络膜、涡静脉、巩膜等相关改变。

局限性脉络膜凹陷(focal choroidal excavation,FCE)是表现为光感受器细胞层、视网膜色素上皮层向脉络膜层凹陷的眼底病变。发病机制多认为是先天脉络膜发育异常、炎症或感染。基于光学相干断层扫描(optical coherence tomography,OCT)，可将FCE从光感受器尖端与视网膜色素上皮层是否分离、形态特征或中央脉络膜厚度三种方式对其进行分类。FCE常合并脉络膜新生血管等疾病。该文对FCE的临床研究现状做一综述。

Focal choroidal excavation (FCE) is a retinopathy, expressing with a depression in photoreceptor cell layer and retinal pigment layer towards the choroidal layer. The pathogenesis is recongized as congenital choroidal developmental abnormalities, inflammation, or infection. Based on optical coherence tomography (OCT), FCE can be classified by three methods: the situation of photoreceptor separating from the retinal pigment upper cortex, morphological features, or central choroidal thickness. FCE is usually associated with other diseases such as choroidal neovascularization. This article reviews the current clinical research on FCE.