Purpose: To develop a survival prediction model for primary conjunctival malignant tumors.

Methods: Detailed information on cases diagnosed with primary conjunctival malignant tumors from 2000 to 2019 was collected from SEER database.Subsequently,  cases meeting the inclusion criteria were randomly assigned to either the development group (1,216 cases) or validation group (608 cases). Relevant risk factors affecting overall survival (OS) were identified using Cox proportional hazards regression analysis. A nomogram was constructed to predict the 1-year, 3-year, and 5-year survival rates. The concordance index (C index) was calculated to assess the predictive power of the model. Receiver operating characteristic curves (ROC curves) and calibration curves were plotted. The area under the curve (AUC) was measured. Decision curve analysis (DCA) was also applied.

Results: The overall survival rate was 77.8%. Statistically significant differences in the survival time distribution were observed among groups based on age (P < 0.001), histology (P < 0.001), and stage (P = 0.01). According to the multivariate analysis, patients with lymphoma, younger age, and localized lesions exhibited better survival outcomes. The C-index of the constructed model was 0.79. In the training group, the AUC values for predicting 1-year, 3-year, and 5-year mortality were 0.824, 0.796, and 0.815, respectively. In the validation group, tge corresponding AU values were 0.750, 0.820, and 0.838. The DCA results demonstrated a significant advantage of the model, while the calibration plots indicated that the predicted OS was in good agreement with the actual OS in both groups. 

Conclusions: This study presents a satisfying survival prediction model for malignant conjunctival tumors.

Purpose: To develop a survival prediction model for primary conjunctival malignant tumors.

Methods: Detailed information on cases diagnosed with primary conjunctival malignant tumors from 2000 to 2019 was collected from SEER database.Subsequently,  cases meeting the inclusion criteria were randomly assigned to either the development group (1,216 cases) or validation group (608 cases). Relevant risk factors affecting overall survival (OS) were identified using Cox proportional hazards regression analysis. A nomogram was constructed to predict the 1-year, 3-year, and 5-year survival rates. The concordance index (C index) was calculated to assess the predictive power of the model. Receiver operating characteristic curves (ROC curves) and calibration curves were plotted. The area under the curve (AUC) was measured. Decision curve analysis (DCA) was also applied.

Results: The overall survival rate was 77.8%. Statistically significant differences in the survival time distribution were observed among groups based on age (P < 0.001), histology (P < 0.001), and stage (P = 0.01). According to the multivariate analysis, patients with lymphoma, younger age, and localized lesions exhibited better survival outcomes. The C-index of the constructed model was 0.79. In the training group, the AUC values for predicting 1-year, 3-year, and 5-year mortality were 0.824, 0.796, and 0.815, respectively. In the validation group, tge corresponding AU values were 0.750, 0.820, and 0.838. The DCA results demonstrated a significant advantage of the model, while the calibration plots indicated that the predicted OS was in good agreement with the actual OS in both groups. 

Conclusions: This study presents a satisfying survival prediction model for malignant conjunctival tumors.

年龄相关性黄斑变性(age-related macular degeneration, AMD)是老年人视力丧失的主要原因之一，其中新生血管性AMD (neovascular AMD, nAMD)以其进展迅速、严重损伤视力的特点，成为全球眼科研究的焦点。随着人口老龄化加剧，nAMD的疾病负担日益沉重，对其发病机制的深入研究和有效治疗策略的探 索迫在眉睫。近年来，高通量组学技术的蓬勃发展为解析nAMD复杂的分子病理机制提供了前所未有的机遇。基因组学、转录组学、蛋白质组学、代谢组学以及多组学整合分析，不仅有助于深入挖掘疾病相关的关键分子、通路和网络，也为发现新的生物标志物和潜在治疗靶点提供了新的视角。文章系统综述了近年来分子组学技术在nAMD研究中的最新进展，重点关注不同组学方法在各类生物样本研究中 的发现，分析多组学整合在揭示疾病机制和筛选生物标志物方面的优势，以期为该领域的未来研究提供参考。

Age-related macular degeneration (AMD) is one of the leading causes of vision loss in elderly population. Among its subtypes, neovascular AMD (nAMD) has become a global focus in ophthalmological research due to its rapid progression and severe vision impairment. With the acceleration of population aging, the disease burden of nAMD is increasingly heavy, making it urgent to conduct in-depth research on its pathogenesis and explore effective therapeutic strategies. In recent years, the rapid development of high-throughput omics technologies has provided unprecedented opportunities to decipher the complex molecular pathological mechanisms of nAMD. Genomics, transcriptomics, proteomics, metabolomics, and multi-omics integration analyses have not only helped to deeply explore disease-related key molecules, pathways, and networks but also provided new perspectives for discovering novel biomarkers and potential therapeutic targets. This review systematically summarizes the recent advances in molecular omics technologies in nAMD research, focusing on findings from different omics approaches across various biological samples, and analyzes the advantages of multi-omics integration in revealing disease mechanisms and screening biomarkers, aiming to provide references for future research in this field.

随着多模式影像技术的应用，年龄相关性黄斑变性(age related macular degeneration, AMD)的一种特殊亚型：表皮玻璃疣(cuticular drusen)被发现，其与AMD尤其是非渗出性AMD又称干性(dry)AMD进展密切相关。但目前聚焦于AMD合并表皮玻璃疣的多模式影像特点等的相关文献较少，因此研究和阐明表皮玻璃疣的多模式影像特点、与其他AMD亚型玻璃疣的鉴别诊断、病理生理机制、治疗方法等具有重要的临床意义。运用多模式影像可以看到表皮玻璃疣呈双眼对称的无数散在的大小均匀的黄色视网膜下结节，典型的“星空状”荧光素钠眼底血管造影(fundus fluorescein angiography, FFA)表现， 以及光学相干断 层成像(optical coherence tomography, OCT)中视网膜色素上皮(retinal pigment epithelium, RPE)和Bruch膜之 间的锯齿状隆起。AMD合并表皮玻璃疣患者的分布特点个体差异很大，容易与AMD其他亚型的玻璃疣混淆，需要与硬性玻璃疣、软性玻璃疣、大胶质玻璃疣、Sorsby眼底营养不良(Sorsby's fundus dystrophy , SFD) 等相鉴别。研究表明表皮玻璃疣具有遗传性的类似动脉粥样硬化的发病机制，与RPE分泌的大型脂蛋白颗粒的堆积密切相关。未来可能会有针对补体系统的药物用于延缓表皮玻璃疣相关病变的进展。文章就表皮玻璃疣的临床表现、多模式影像的典型特点、鉴别诊断、遗传学基础、病理生理学发病机制、 并发症以及临床诊疗策略进行综述。

With the application of multimodal imaging techniques, a specific subtype of age-related macular degeneration (AMD),
known as cuticular drusen, has been identified. This subtype is closely associated with the progression of AMD, particularly non-exudative AMD, also referred to as dry AMD. However, there is a scarcity of literature focusing on the multimodal imaging characteristics of AMD combined with cuticular drusen. Therefore, studying and elucidating the multimodal imaging features of cuticular drusen, its differential diagnosis from other AMD subtypes of drusen, pathophysiological mechanisms, and treatment methods holds significant clinical importance. Multimodal imaging reveals cuticular drusenas numerous, symmetrical, evenly sized, yellow subretinal nodules in both eyes, exhibiting a typical "stars-in-the-sky" appearance on fluorescein angiography (FFA) and zigzag elevations between the retinal pigment epithelium (RPE) and Bruch's membrane on optical coherence tomography (OCT). The distribution characteristics of AMD patients with cuticular drusen vary greatly among individuals and can easily be confused with drusen from other AMD subtypes, so the differentiation from hard drusen, soft drusen, large colloidal drusen, and Sorsby's fundus dystrophy (SFD) is necessary. Studies suggest that cuticular drusen have a genetic, atherosclerosis- like pathogenesis which closely related to the accumulation of large lipoprotein particles secreted by the RPE. Future therapies targeting the complement system maybe employed to delay the progression of cuticulardrusen-related lesions. This article reviews the clinical manifestations, typical multimodal imaging features, differential diagnosis, genetic basis, pathophysiological mechanisms, complications, and clinical management strategies of cuticulardrusen.

巨噬细胞样细胞(macrophage-like cells, MLC)指起源、功能与巨噬细胞类似的免疫细胞，包括小胶质细胞、玻璃体细胞及巨噬细胞。将en face OCT显示层面设置在视网膜表明即可观测到视网膜表明的 MLC(epiretinal MLC, eMLC)，随后利用ImageJ软件即可对细胞进行提取和量化。研究表明，eMLC在炎症情况下均可出现细胞募集及活化现象，但在不同眼底病中各具特点。在糖尿病视网膜病变、视网膜静脉阻塞等视网膜缺血缺氧性疾病中，eMLC密度越高，黄斑水肿可能越严重。此外，eMLC密度更高的视网膜静脉阻塞患者抗VEGF疗效更差，视力预后不佳，提示基于en face OCT的eMLC不仅可用于评估视网膜炎情况，而且还能充当提示疾病疗效及预后的标志物。在葡萄膜炎等免疫炎症性疾病中，en face OCT亦可观测到eMLC密度、形态等改变。白塞病葡萄膜炎患者视网膜血管渗漏程度与eMLC密度相关性强，故eMLC密度可充当无创评估视网膜血管渗漏程度的新指标。然而，目前提取和量化eMLC的方法及标准不统一，降低了各研究间的可比性。因此，亟需制定统一的操作规范和评估标准。此外eMLC 所代表的具体细胞类型及功能仍需进一步探究。未来，研究者可以利用en face OCT对眼底炎症地进行无创评估。基于en face OCT的eMLC还能作为基础研究与临床研究之间的桥梁，为揭示疾病的致病机制提供重要参考。

Macrophage-like cells (MLC) refer to immune cells that originate from and function similarly to macrophages, including microglia, hyalocytes, and macrophages themselves. By setting the display level of en face OCT to the retinal surface, epiretinal MLC (eMLC) can be observed and subsequently extracted and quantified using ImageJ software. Studies indicate that eMLC can exhibit cell recruitment and activation in inflammatory conditions, each displaying distinct characteristics in different retinal diseases. In ischemic and hypoxic retinal conditions such as diabetic retinopathy and retinal vein occlusion, higher densities of eMLC are associated with more severe macularedema. Moreover, patients with retinal vein occlusion showing higher eMLC densities tend to have poorer responses to anti-VEGF treatments and worse visual prognoses, suggesting that eMLC identified via en face OCT can be used not only to assess retinal inflammation but also as biomarkers for disease efficacy and prognosis. In immune-inflammatory diseases like uveitis, changes in eMLC density and morphology can also be observed through en face OCT. Inpatients with Beh?et's disease, a strong correlation exists between the degree of retinal vascular leakage and eMLC density, making eMLC density a potential non-invasive marker for assessing retinal vascular leakage. However, the current methods and standards for extracting and quantifying eMLC are not unified, significantly reducing comparability between studies. Therefore, there is an urgent need to establish uniform operational protocols and assessment standards. Furthermore, the specific cell types and functions represented by eMLC observed via en face OCT require further investigation. In the future, en face OCT could be utilized for non-invasive assessment of retinal inflammation. eMLC based onen face OCT could also serve as a bridge between basic research and clinical studies, providing valuable insights into the pathogenic mechanisms of diseases.

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.

糖尿病视网膜病变(diabetic retinopathy, DR)作为糖尿病的一种常见并发症是导致工作年龄人群失明的主要原因。血糖变异性(glycemic variability, GV)指血糖波动的程度。最新研究表明，GV与糖尿病患者的代谢状况和微血管病变密切相关。该文综述了GV对DR的影响及其研究进展。GV是指血糖水平在高点和低点之间波动的不稳定状态，分为长期GV和短期GV。长期GV主要通过空腹血糖(fasting plasma glucose, FPG)、餐后血糖(postprandial plasma glucose, PPG)和糖化血红蛋白(glycated hemoglobin, HbA1c)评估，短期GV则通过血糖标准差(standard deviation, SD)、变异系数(coefficient of variation, CV)、低血糖指数(low blood glucose index, LBGI)等指标量化。研究表明，GV是糖尿病大管和微血管并发症的重要风险预测因子，与冠状动脉综合征、心肌梗死、脑卒中、糖尿病肾病、周围神经病变等密切相关。在DR方面，GV可能是其进展的风险因素，高GV会加剧氧化应激、炎症反应、内皮功能障碍和新生血管生成，从而促进DR的发展。治疗策略包括动态血糖监测系统、药物干预(如基础胰岛素、阿格列汀等)、合理饮食和运动等，这些方法可改善GV，降低并发症风险，提高患者预后和生活质量。

Diabetic retinopathy (DR) is a common complication of diabetes and is a leading cause of blindness in the working-age population. Glycemic variability (GV) refers to the degree of fluctuation in blood glucose levels. Recent studies have shown that GV is closely related to the metabolic status and microvascular complications in patients with diabetes. This article reviews the impact of glycemic variability (GV) on diabetic retinopathy (DR) and the latest research progress.GV is defined as the unstable state of blood glucose levels fluctuating between highs and lows, which is categorized into long-term GV and short-term GV. Long-term GV is mainly assessed through fasting plasma glucose (FPG), postprandial plasma glucose (PPG), and glycated hemoglobin (HbA1c). Short-term GV is quantified by indicators such as the standard deviation of blood glucose (SD), coefficient of variation (CV), and low blood glucose index (LBGI).Studies have shown that GV is an important risk predictor for both macrovascular and microvascular complications in diabetic patients, being closely associated with conditions such as coronary artery syndrome, myocardial infarction, stroke, diabetic nephropathy, and peripheral neuropathy. Regarding DR, GV is likely a risk factor for its progression. High GV can exacerbate oxidative stress, inflammatory responses, endothelial dysfunction, and neovascularization, thereby promoting the development of DR.Treatment strategies include continuous glucose monitoring systems, pharmacological interventions (such as basal insulin, alogliptin, etc.), as well as proper diet and exercise. These approaches can improve GV, reduce the risk of complications, and enhance patients' prognosis and quality of life.

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.

急性渗出性多形性卵黄样黄斑病变(acute exudative polymorphous vitelliform maculopathy, AEPVM)是一种在临床较为罕见的眼底疾病，主要表现为黄斑区神经上皮脱离以及后极部卵黄样物质的沉积。至今，其具体发病机制仍未完全明确，但可能与全身感染、自身免疫反应或副肿瘤综合征等因素密切相关。在临床表现上，患者往往会出现轻微视力减退或畏光等症状，眼底后极部可见斑点状黄白色病灶，这些病灶在自发荧光中呈现相对较高的自发荧光。荧光素眼底血管造影时，病灶区域通常不会出现荧光素渗漏现象。OCT检查能够揭示椭圆体带的显著增厚以及神经上皮层内囊腔样改变。迄今为止，对于这一疾病的治疗尚未有确立的方案。文章综述了AEPVM的临床表现、影像学特征、诊断及鉴别诊断、发病机制以及治疗方面的最新研究进展。急性渗出性多形性卵黄样黄斑病变的自然病程复杂多样，其诊断与鉴别诊断仍面临重大挑战，深入掌握该疾病的临床表现与影像学特征，对于未来深化对其发病机制的理解及开发有效治疗策略具有重要意义。

Acute Exudative Polymorphous Vitelliform Maculopathy (AEPVM) is a clinically rare fundus disease characterized primarily by neurosensory detachment in the macular area and accumulation of vitelliform material in the posterior pole. The exact cause is unclear and may be related to systemic infections, autoimmune responses, or paraneoplastic syndromes. Clinical manifestations usually include mild vision loss or photophobia, and yellow-white deposits can be seen in the posterior pole of the fundus, exhibiting relatively higher autofluorescence in spontaneous fluorescence. Fundus fluorescein angiography typically does not show leakage of fluorescein in the lesion area. Optical coherence tomography (OCT)examination can reveal thickening of the ellipsoid zone and cystic changes within the neurosensory layer. Currently, there is no explicit treatment plan. This article reviews the clinical presentation, imaging characteristics, diagnosis and differential diagnosis, pathogenesis, and treatment-related research progress of acute exudative polymorphous vitelliform maculopathy. In summary, the natural course of acute exudative polymorphous vitelliform maculopathy is complex and diverse, and its diagnosis and differential diagnosis remain challenging. A deeper understanding of the clinical presentations and imaging characteristics of acute exudative polymorphous vitelliform maculopathy will facilitate further research and exploration to clarify its pathological mechanisms and identify effective treatment methods in the future.

眼底老化是年龄相关性黄斑变性(age-related macular degeneration, AMD)发生和进展的关键因素及病理基础，在组织病理学上主要表现为脉络膜毛细血管萎缩、布鲁赫膜(Bruch's membrane, BrM)增厚以及视网膜色素上皮(retinal pigment epithelium, RPE)异常。BrM增厚可由多种眼底老化沉积物聚集引起，在AMD的病理机制中具有重要作用。其中，基底薄层沉积物(basal laminar deposit, BLamD)代表了RPE基底膜的弥漫性增厚，通常作为一种正常眼底老化改变。而以酯化和未酯化胆固醇等中性脂质为主的RPE基底膜下沉积物，即基底线性沉积物(basal linear deposits, BLinD)和软性玻璃膜疣，均可参与破坏脉络膜与外层视网膜间物质交换稳态，造成外层视网膜缺血、缺氧及氧化应激，是AMD早期重要病理改变。硬性玻璃膜疣主要分布于周边视网膜，多见于正常老化眼底；表皮玻璃膜疣是RPE基底膜局灶性结节状增厚的结果，眼底表现与硬性玻璃膜疣相似，但其主要分布于后极部，数量更多且密度更高。近年来逐渐加深了对视网膜下玻璃膜疣样沉积物(subretinal drusenoid deposit, SDD)的认识和研究，其是位于RPE上方的沉积物，在AMD发病机制中亦具有深刻意义。文章就几种眼底老化相关沉积物(包括硬性玻璃膜及表皮玻璃膜疣)的病理特征和多模态影像学表现进行综述，旨在帮助认识和理解这些沉积物的眼底表现、病理特征和形成机制，以及在AMD发生及进展中的临床意义。

Fundus aging is a key factor and pathological basis for the development and progression of age-related macular degeneration (AMD), which is histopathologically characterized by choroidal capillary atrophy, Bruch’s membrane (BrM) thickening, and abnormalities of retinal pigment epithelium (RPE). BrM thickening can be induced by the aggregation of age-related fundus deposits and plays an essential role in the pathogenesis of AMD. Basal laminar deposits (BLamD) represent diffuse thickening of the basement membrane of RPE, usually considered to be a normal fundus aging. Basal linear deposits (BLinD) and soft drusen, mainly composed of neutral lipids such as esterified and unesterified cholesterol, can disrupt the homeostasis of material exchange between the choroid and the outer retina, resulting in ischemia, hypoxia, and oxidative stress in the outer retina, which are important pathological changes in the early AMD. Hard drusen is mainly distributed in the peripheral retina and is very common in aging fundus; Cuticular drusen are resulted from focal nodular thickening of the basement membrane of RPE, with similar appearance as hard drusen, but mainly distributed in the posterior pole and more numerous and denser. Recently, our understanding of subretinal drusenoid deposits (SDD), deposits above the RPE, has been gradually deepened and it also has profound significance in the pathogenesis of AMD. In this review, we presented the pathologic features and multimodal imaging of age-related fundus deposits (including hard drusen and cuticular drusen), aiming to help recognize and understand the manifestations, pathologic features, and formation mechanisms of these deposits, as well as their clinical significance in the development and progression of AMD.

CSNB是一组高度异质的遗传性视网膜疾病(inherited retinal disease, IRD)，主要由视网膜光感受器细胞和双极细胞间的信号传导障碍引发。其主要临床特征为静止性夜盲和暗适应功能障碍，常伴有早发性近视、眼球震颤、斜视和远视等症状，ERG在CSNB的诊断、分型及治疗指导中起着至关重要的作用。尽管CSNB发病率低，属于罕见病，但其真实发病率可能被低估，部分原因在于其症状轻微、眼底表现多不明显，且临床常忽视视网膜功能检查，导致较高的漏诊和误诊率。随着分子遗传学技术的进步，大量研究揭示了CSNB不同基因缺陷的致病机制，特别是与早发近视的关联机制，这些研究同也增加了对视网膜信号传导和近视发病机制的理解。然而，CSNB的基因治疗仍处于早期阶段。本综述旨在全面探讨CSNB的疾病谱，包括不同类型患者的临床表现、影像学和功能学表型特征，以及相关遗传学致病机制，并总结基因型与表型的关联。同时，综述最新研究成果与未来发展方向，旨在提高国内学者对CSNB的认识，为临床诊断和治疗提供参考，并为后续研究提供新思路。

Congenital Stationary Night Blindness (CSNB) represents a group of highly heterogeneous inherited retinal diseases (IRDs) primarily caused by impaired signal transmission between photoreceptor cells and bipolar cells in the retina. The main clinical features include stationary night blindness and dark adaptation dysfunction, often accompanied by early-onset myopia, nystagmus, strabismus, and hyperopia. Electroretinography (ERG) plays a crucial role in the diagnosis, classification, and therapeutic management of CSNB. Although CSNB is classified as a rare disease due to its low incidence, its true prevalence is likely underestimated, partly because of its mild symptoms, inconspicuous fundus manifestations, and frequent oversight of retinal function tests in clinical practice, leading to high rates of underdiagnosis and misdiagnosis. With advances in molecular genetics, extensive research has elucidated the pathogenic mechanisms of various genetic defects in CSNB, particularly those associated with early-onset myopia. These studies have also enhanced our understanding of retinal signal transduction and the pathogenesis of myopia. However, gene therapy for CSNB remains in its early stages. This review aims to comprehensively explore the disease spectrum of CSNB, including clinical manifestations, imaging and functional phenotypic characteristics across different subtypes, and associated genetic pathogenic mechanisms. We also summarize genotype-phenotype correlations, review the latest research advancements, and discuss future directions. By doing so, this review seeks to improve the understanding of CSNB among domestic researchers, provide guidance for clinical diagnosis and treatment, and offer new insights for future research.