息肉状脉络膜血管病变（polypoidal choroidal vasculopathy, PCV）是中国人新生血管性年龄相关性黄斑变性的(age-related macular degeneration, AMD)主要亚型。PCV与典型的新生血管性AMD在流行病学、临床表现、影像学特征和自然病程方面存在一定差异。近年来的研究表明，除了传统的玻璃膜疣驱动机制外，PCV可能与肥厚脉络膜机制相关，后者在亚洲人群中更为常见。深入的病理学探索将有助于揭示PCV的发病机制，并探索PCV与其他脉络膜疾病之间的内在联系。由于PCV患者眼球标本的稀缺，现有的病理学研究较少，且结果之间存在一定差异。文章通过介绍笔者最新的临床病理研究结果，并结合历年来国内外的研究，总结了关于PCV病灶所在的层次、起源及血管内皮生长因子（vascular endothelial growth factor, VEGF）表达水平的争议问题，阐明了PCV的临床病理研究现状。第一，PCV病灶的层次。临床上，OCT成像显示PCV病灶位于视网膜色素上皮（retinal pigment epithelium, RPE）与Bruch膜的高反射线之间，属于I型脉络膜新生血管的特殊亚型。部分病理学研究认为PCV病灶位于Bruch膜内，但实际上PCV病灶更准确地位于RPE基底膜下。第二，异常分支血管网（branching vascular networks, BVN）的起源。尸体眼标本的病理分析表明，BVN起源于脉络膜动脉，且动脉穿过Bruch膜后，转变为薄壁毛细血管形成I型脉络膜新生血管。少数研究指出PCV可能由静脉扩张形成，并存在脉络膜静脉的淤滞。第三，VEGF在PCV病灶中的表达。VEGF是新生血管性AMD的关键致病因子，一些研究表明PCV病灶中VEGF表达升高，提示PCV可能与新生血管性AMD具有相似的发病机制，但也有研究发现PCV病灶中的VEGF表达为阴性，提示PCV的机制可能不完全依赖于VEGF。综上，PCV的病理特征具有复杂性，既有与新生血管性AMD相似的表现，也有肥厚脉络膜的特征。随着眼球捐献意识的提高，未来有望获得更多宝贵的眼球标本，为进一步探索PCV的发病机制提供支持,并为其临床诊断和治疗提供更有效的策略。
Polypoidal choroidal vasculopathy (PCV) is the main subtype of neovascular age-related macular degeneration (AMD) in China. PCV differs from typical neovascular AMD in terms of epidemiology, clinical presentation, imaging features, and natural disease course. Recent studies suggest that, in addition to the traditional drusen-driven mechanism, PCV may also be associated with pachychoroid mechanism, which is particularly more common in Asian populations. In-depth pathological research will help uncover the pathogenesis of PCV and explore the intrinsic connections between PCV and other choroidal diseases. Due to the rarity of eye specimens from PCV patients, there is limited pathological research, and results can vary. Herein, this article summarize the controversial issues regarding the location level, origin, and the vascular endothelial growth factor (VEGF) expression of PCV lesions by introducing our latest clinicopathologic study on PCV and combining with previous studies in China and worldwide. First, the layer of PCV lesions. Clinically, OCT imaging shows that PCV lesions are located between the retinal pigment epithelium (RPE) and the hyperreflective line of Bruch membrane, making them a special subtype of type I choroidal neovascularization. Some pathological studies suggest that PCV lesions are located within Bruch membrane, but in fact, PCV lesions are more accurately located beneath the RPE basement membrane. Second, the origin of the branching vascular networks (BVN). Pathological analysis of postmortem eye specimens indicates that BVN originates from choroidal arteries, and after passing through Bruch membrane, they transform into thin-walled capillaries, forming type I choroidal neovascularization. A few studies suggest that PCV may result from dilation of choroidal vein, accompanied with vein stasis. Third, VEGF expression in PCV lesions. VEGF is a key pathogenic factor in neovascular AMD. Some studies show increased VEGF expression in PCV lesions, suggesting that PCV may share a similar pathogenic mechanism with neovascular AMD. However, other studies have found negative VEGF expression in PCV lesions, indicating that the mechanism of PCV may not be entirely dependent on VEGF. In conclusion, the pathological features of PCV are complex, showing both similarities to neovascular AMD and characteristics of pachychoroid. With the increasing awareness of eye donation, more valuable eye specimens are expected to be obtained in the future, providing support for further ex;ploration of the pathogenesis of PCV and offering more effective strategies for its clinical diagnosis and treatment.
肥厚型脉络膜谱系疾病(pachychoroid disease, PCD)是一组以病理性脉络膜增厚为共同特征的疾病谱系。其特征性改变包括Haller层脉络膜血管扩张，脉络膜毛细血管层和Sattler层变薄，以及肥厚血管(pachyvessels)上视网膜色素上皮(retinal pigment epithelium, RPE)的异常。PCD主要包括单纯肥厚型脉络膜病变(uncomplicated pachychoroid, UCP)、肥厚型脉络膜色素上皮病变(pachychoroid pigment epitheliopathy, PPE)、中心性浆液性脉络膜视网膜病变(central serous chorioretinopathy, CSC)、肥厚型脉络膜新生血管病变(pachychoroid neovasculopathy, PNV)和息肉状脉络膜血管病变(polypoidal choroidal vasculopathy, PCV)。传统眼底检查因单张成像局限于后极部，难以全面评估病变范围。广角影像技术突破了这一局限，其成像范围覆盖后极部至赤道部涡静脉壶腹部(约60°~100°)，而超广角成像更可达后极部至锯齿缘(约 110°~220°)。这一技术的进步不仅扩大了PCD眼底病灶的观察范围，更提升了对脉络膜结构和功能的评估能力，为深化研究PCD的发病机制提供了新的视角。近年来，基于深度学习的人工智能技术在PCD辅助诊断方面取得重要突破，展现出优异的PCD相关疾病识别和分类能力，有助于显著提升基层医疗机构诊断效率，并推动医疗资源优化配置。文章综述了广角眼底影像技术在PCD评估与诊断中的研究进展，旨在为眼科临床工作者和研究者提供最新的技术应用视角，并为进一步探索PCD的病理机制和诊疗方法奠定科学基础。
Pachychoroid disease (PCD) represents a group of disorders characterized by pathological choroidal thickening. The characteristic changes include dilated choroidal vessels in Haller's layer, thinning of the choriocapillaris and Sattler's layer, and retinal pigment epithelium (RPE) abnormalities overlying the pachyvessels. The PCD primarily encompasses uncomplicated pachychoroid (UCP), pachychoroid pigment epitheliopathy (PPE), central serous chorioretinopathy (CSC), pachychoroid neovasculopathy (PNV), and polypoidal choroidal vasculopathy (PCV). Traditional fundus examination is limited to the posterior pole in single-frame imaging, making it challenging to comprehensively evaluate the extent of lesions. Wide-field imaging technology has overcome this limitation, with its imaging range covering from the posterior pole to the ampulla of vortex veins at the equator (approximately 60-100°), while ultra-wide-field imaging can extend from the posterior pole to the pars plana (approximately 110-220°). This technological advancement has not only expanded the observation range of PCD fundus lesions but also enhanced the assessment capabilities of choroidal structure and function, providing new perspectives for investigating PCD pathogenesis. In recent years, deep learning-based artificial intelligence technology has achieved significant breakthroughs in PCD-assisted diagnosis, demonstrating excellent capability in identifying and classifying PCD-related diseases. This has contributed to significantly improving diagnostic efficiency in primary healthcare institutions and optimizing medical resource allocation. This review summarizes the advances in wide-field fundus imaging technologies for the assessment and diagnosis of PCD.
Myopic choroidal neovascularization (mCNV) can cause severe visual impairment in highly myopic patients. We review the randomized trials of two approved pharmacotherapy for treating mCNV, including intravitreal injections of ranibizumab and afl ibercept. These two vascular endothelial growth factor (VEGF) antagonists show superior ability to improve vision and reduce macular thickness, comparing with sham injections or verteporfin photodynamic therapy (vPDT). There is no severe ocular or systemic adverse reaction reported in studies associated with ranibizumab and afl ibercept for mCNV. Prompt treatment with these agents can lead to a better outcome.
Myopic choroidal neovascularization (mCNV) can cause severe visual impairment in highly myopic patients. We review the randomized trials of two approved pharmacotherapy for treating mCNV, including intravitreal injections of ranibizumab and afl ibercept. These two vascular endothelial growth factor (VEGF) antagonists show superior ability to improve vision and reduce macular thickness, comparing with sham injections or verteporfin photodynamic therapy (vPDT). There is no severe ocular or systemic adverse reaction reported in studies associated with ranibizumab and afl ibercept for mCNV. Prompt treatment with these agents can lead to a better outcome.
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.
