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.

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.

“六要素，三个一”是眼底影像基础阅片工作中进行眼底疾病临床创新性研究的模式要点，即在眼底阅片过程中捕捉到1个异常的病例后，通过积累病例、提炼特征、文献检索、寻同查异，进而扩展到1组病例，最后通过思辨创新，提出或完善1种新的疾病或疾病表征。近二十年来，在此模式的指导下，团队在眼底疾病研究工作中取得了一些原创性的成果：比如息肉状脉络膜血管病变的认识及其在国人新生血管性年龄相关性黄斑变性中发病比例第一，提出点状内层脉络膜病变病灶国际分期和命名新亚型，年龄相关的吲哚菁绿血管造影晚期散在弱荧光点揭示潴留性视网膜色素上皮脱离的发病机制，发现急性黄斑神经视网膜病变是登革热患者视力下降的主要原因，在全球最大的持续性鳞状黄斑病变的病例系列中明确病灶层次等创新性成果。“六要素”框架规范眼底影像研究流程，强调研究过程的严谨性与渐进性，且多次循环后衍生发散出更多研究线索和思路，极大拓展研究深度和广度。“三个一”路径体现了研究的层次性，从个体现象(点)到群体规律(线)，最终构建疾病认知的立体网络(面)；指导眼底异常影像征象、罕见病、新病种研究，加速疾病谱系完善。以“六要素”为纲，以“三个一”为略，将继续推动眼底疾病临床研究的创新与突破。

The "6 Elements, 3 Ones" constitutes a methodological framework for conducting innovative clinical research of ocular fundus diseases in foundational fundus imaging interpretation. This model emphasizes: 1) identifying a single abnormal case during routine fundus evaluation; 2) systematically expanding this observation into a case series through case accumulation, feature extraction, literature review, and comparative analysis; and 3) ultimately proposing or refining novel disease entities or manifestations through critical thinking and innovation. Over the past two decades, guided by this paradigm, our research team has achieved several original breakthroughs in fundus imaging studies, including: establishing polypoidal choroidal vasculopathy as the predominant subtype of neovascular age-related macular degeneration in Chinese populations; proposing an international staging system and novel subtypes for punctate inner choroidopathy; elucidating the pathogenesis of retentional retinal pigment epithelial detachment through the sign of age-related scattered hypofluorescent spots on late-phase indocyanine green angiography; identifying acute macular neuroretinopathy as the primary cause of vision loss in dengue fever patients; and precisely localizing lesion in the world's largest case series of persistent placoid maculopathy. The "6 Elements" framework standardizes fundus disease research protocols, emphasizing methodological rigor and progressive investigation while generating multiple research trajectories through iterative cycles, thereby expanding both the depth and breadth of scientific inquiry. The "3 Ones" pathway embodies hierarchical research progression - transitioning from individual phenomena (point observations) to population-level patterns (linear correlations), ultimately constructing a multidimensional disease cognition network (planar integration). This approach guides investigations ranging from signs of common disease to rare disorders and novel disease entities, accelerating the refinement of disease taxonomies. By adhering to the "6 Elements" as the structural framework and implementing the "3 Ones" as the strategic pathway, we will continue to advance innovation and achieve breakthroughs in clinical fundus disease researches.

目的：研究IgG4相关性眼病(IgG4-related ophthalmic disease, IgG4-ROD)患者的影像学特征与外周血免疫球蛋白G4(IgG4)水平之间的相关性，为评估IgG4相关性疾病全身性严重程度提供新思路。方法：收集2023年8月—2024年9月在吉林大学第二医院眼科医院经术后组织标本病理确诊的29例IgG4-ROD阳性患者。回顾性分析患者眼眶影像学特点与血清IgG4水平相关性，探讨影像学中特征性表现包括泪腺肿大、三叉神经分支增粗、眼外肌增粗、鼻黏膜类炎症改变、眼睑软组织肥厚，以及其他眶内软组织增生等特征性影像学改变出现比例，并按照累及组织结构情况分级评分，评估特征性影像学改变与血清IgG4水平之间的相关性。结果：29例病理确诊IgG4-ROD患者中，泪腺均受累，占比100%；眼外肌受累17例，占比58.62%；三叉神经分支受累5例(4例眶下神经受累，3例额神经病受累，2例眶下神经与额神经同时受累)，占比17.24%眼睑软组织肥厚24例，占比82.76%鼻黏膜出现类炎症反应15例，占比51.72%；合并眶内其他软组织增生性病变2例，占比6.90%。影像学中特征性受累组织结构分级评分与血清IgG4水平呈正相关(P < 0.05)。结论：IgG4-ROD影像学中特征性组织结构受累及范围与血清IgG4水平明显相关，可以辅助评估IgG4相关性疾病全身性严重程度。

Objective: To investigate the correlation between the imaging characteristics of patients with IgG4-related ophthalmic disease (IgG4-ROD) and the serum immunoglobulin G4 (IgG4) levels, providing new insights for assessing the systemic severity of IgG4-related diseases. Methods: This study collected postoperative tissue samples from 29 patients with histopathologically conffrmed IgG4-ROD at the Ophthalmology Department of Jilin University Second Hospital from August 2023 to September 2024. TTis study retrospectively analyzed the correlation between patients' orbital imaging features and serum IgG4 levels, and explored the proportion of characteristic imaging changes including enlargement of the lacrimal gland, thickening of the trigeminal nerve branches, thickening of the extraocular muscles, inffammatory like changes of the nasal mucous membranes, hypertrophy of the eyelid soft tissues, as well as hyperplasia of other intraorbital soft tissues in the imaging. A grading score for affected tissue structures was established to evaluate the correlation between characteristic imaging changes and serum IgG4 levels. Results: Among the 29 patients diagnosed with IgG4-ROD, lacrimal gland involvement was observed in all patients (100%). Extraocular muscle involvement was present in 17 patients (58.62%). Five patients had involvement of the trigeminal nerve branches (including 4 with infraorbital nerve involvement and 3 with frontal nerve involvement, with 2 patients having simultaneous involvement of both nerves), accounting for 17.24% of the cases. Eyelid soff tissue hypertrophy was observed in 24 patients (82.76%), and nasal mucosal inflammatory responses were noted in 15 patients (51.72%). Additionally, two patients (6.90%) presented with other proliferative lesions within the orbit. The correlation analysis between the grading scores for imaging features and serum IgG4 levels demonstrated a significant positive correlation. Conclusions: The extent of characteristic structural involvement observed in the imaging features of IgG4-ROD is significantly correlated with serum IgG4 levels. TTis correlation can assist in evaluating the systemic severity of IgG4-related diseases and provides clinical evidence supporting the need for comprehensive systemic evaluations, such as PET-CT, in patients whose initial presentation is IgG4-related ophthalmic disease.

目的：了解中山大学中山眼科医院门诊重症疑难眼病会诊的现状，探讨门诊重症疑难眼病会诊亚专科的分布情况及会诊效果，为建立科学的门诊重症疑难眼病会诊体系提供支持和依据，同时也为合理安排会诊流程提供依据。方法：对中山大学中山眼科医院2016年3月至2017年4月收集的265例门诊重症疑难病例，通过时间、地区、性别、年龄、会诊原因、会诊亚专科、会诊后的转归及效果等方面进行统计分析，所有数据均通过Excel软件进行录入，根据分析目的和资料的类型，采用相应的统计指标进行描述，并进行统计推断，检验水准为α=0.05。结果：2016年3月至2017年4月期间中山大学中山眼科医院门诊重症疑难眼病会诊人数265例。在地区分布中，广东省地区最多，为145例，外国最少，为3例。人群分布中，男167例，女98例，男女比例为1.70:1。年龄分布中，30~44岁及45~59岁的病例数最多，均为53例(20%)，70岁以上老年病例最少，为13例(4.9%)。中山大学中山眼科医院门诊重症疑难眼病会诊中，2016年3月至2017年4月期间，各眼科亚专科共参加会诊714次，眼底外科参加会诊次数最多，为147次(20.5%)，结膜科参加会诊的次数最少，为1次(0.1%)。在分析会诊原因方面，因诊治涉及多个专科而会诊的病例数最多，为161例，因病因不明会会诊的病例最少，为20例；会诊后比会诊前明确治疗方案的病例数增加190例。结论：中山大学中山眼科医院门诊组织实施重症疑难眼病会诊使明确治疗方案率大幅提高，在提高疑难眼病治疗效果方面起到一定的促进作用。

Objective: To understand the current situation of the consultation for outpatients with severe eye disease in Zhongshan Ophthalmic Center, and to explore the distribution and effects of sub-specialty consultation for outpatients with severe eye disease. To set up a scientific system of the consultation for outpatients with severe eye disease, and also provide a basis for reasonable arrangement of consultation process. Methods: We analyzed 265 severe cases from March 2016 to April 2017 in Zhongshan Ophthalmic Center by time, region, gender, age, cause of consultation, sub-specialty, and outcomes after consultation. And all data was analyzed by Excel software, according to the purpose and the type of data, and the corresponding statistical index and statistical inference, inspection level for α=0.05. Results: There were 265 cases with severe eye disease from March 2016 to April 2017. As for the regional distribution, Guangdong province was the largest, with 145 cases, while the lowest number was from foreign countries, which was 3 cases. In the population distribution, 167 cases were males, 98 were females and the ratio was 1.70. In age distribution, patients aged from 30 to 44 years and 45 to 59 years predominated and the number of cases were both 53, accounting for 20% of the total number. Patients over 70 were the least which were 13 cases, accounting for 4.9% of the total number. From March 2016 to April 2017, all sub-specialties attended consultation for 714 times, as Ocular Fundus Department attended the most, accounting for 20.5% of the total number while Conjunctive Department attended the least, for only one, accounting for 0.1% of the total number. As for the reason of the consultation, the number of cases involving multiple specialties was the largest, with 161 cases, while the lowest number of cases due to unexplained causes, which were 20 cases. The number of cases of explicit treatment was increased by 190 after consultation. Conclusion: Consultations organized by Zhongshan Ophthalmic Center improves the treatment for outpatients with severe eye disease.

原发性干燥综合征 (primary Sjogren' s syndrome，SS) 是一种主要累及外分泌腺体的自身免疫性疾病，患者通常因为严重的干眼症状首先就诊于眼科，大多数临床医师对原发性干燥综合征相关性干眼 (Sjogren' s syndrome dry eye disease，SS-DED) 认识不足，可能导致漏诊和误诊。侵入性极小的客观检查及生物标志物的发展，将有助于发现 SS-DED 的真面目，并可能从新的角度阐释其发病机制，为其诊断、分类及治疗提供新的思路。SS-DED 的治疗没有特效的药物，大多数患者需接受多种方法的治疗，以了解哪些方法最有效。

Primary Sjogren' s syndrome is an autoimmune disease that mainly affects exocrine glands. Patients usually refer to ophthalmologists because of severe dry eye symptoms. Most clinicians have insufficient knowledge with dry eye disease associated with primary Sjogren' s syndrome probably leading to misdiagnosis or missing the diagnosis. The diagnosis of Sjogren' s syndrome dry eye disease (SS-DED) is difficult, but the extremely invasive objective examination and the development of biomarkers will help to understand this disease and explain its pathogenesis from a new perspective. There is no specific treatment for the SS-DED, and most patients should receive multiple treatments to select the optimal treatment. 

教学查房是手把手教学的重要手段和具体表现，是让同学们把书本知识应用到医疗诊疗中的重要桥梁。眼科学具有形象生动、结构复杂、直观性和操作性强等特点。外眼疾病的特征更是显而易见，针对外眼疾病诊疗中的一些不规范的操作也更易暴露出来。本文以上睑下垂为例，旨在阐明教学查房在外眼疾病教学中的重要性。

Teaching rounds are an important means and concrete manifestation of hands-on teaching, and an important bridge for students to apply book knowledge to medical diagnosis and treatment. Ophthalmology has the characteristics of vivid image, complex structure, intuition and strong operability. The characteristics of external eye diseases are more obvious, and some non-standard operations in the diagnosis and treatment of external eye diseases are easier to expose. This article takes blepharoptosis as an example to illustrate the importance of teaching rounds in the teaching of external eye diseases.

目的：探讨推广新生儿眼病筛查以及婴儿期定期眼保健的必要性。方法：回顾分析 2014 年 1 月至 2016 年 12 月 20800 例惠州市妇幼保健计划生育服务中心门诊收集的婴儿眼保健资料。结果：共检出致盲性眼病 7 种 158 例，其中满 42 d 内检出 147 例，3 个月内检出 8 例，6 个月内检出 2 例，12 个月内检出 1 例。检出时已合并眼位、眼前节异常、影响视功能者 8 例。不同出生胎龄致盲性眼病检出率：≤ 34 周为 11.64％，34～37 周为 1.24％，≥ 37 周为 1.29％。相对于纸质宣传材料，开展家长学校讲课，利用 PPT 结合实际病例讲解，更有助于普及眼保健。相对于眼底检查，眼前节的方式更容易被接受、被推广。结论：致盲性眼病婴儿期发病在临床中并不少见，并且部分是可以早期筛查、早期治疗以避免视功能损害的。临床中需要大力普及眼保健知识，并可以在具备条件的医院，适当利用新生儿广域成像系统推广新生儿眼病普筛。

Objective: To discuss the necessity of screening for neonatal eye disease and regular eye care in infancy. Methods: A retrospective analysis of 20 800 infants in our hospital from January 2014 to December 2016 was conducted. From January 2014 to December 2016. Results: Seven types of blindness eye diseases were detected in 158 cases, including 147 cases at 42 days, 8 cases at 3 months, 2 cases at 6 months and 1 case at 12 months after birth. Eight cases were supervened with abnormal eye position, anterior segment abnormality and poor visual function. The detection rate of blindness-inducing diseases was 11.64% for infants aged ≤ 34 weeks, 1.24% for 34–37 weeks and 1.29% for ≥ 37 weeks. Compared with the paper propaganda materials, the introduction of parental school lectures and PPT combined with explanation of actual cases was more conducive to popularize eye care. Compared with fundus examination, anterior segment examination was more likely to be accepted and popularized. Conclusion: Eye disease inducing blindness in infancy are commonly encountered in clinical practice, which can be screened and treated early to avoid visual impairment. It is necessary to popularize the knowledge of eye care, and promote the screening of newborn eye disease in qualified hospitals.