Aims: This study describes vascular abnormalities in X-linked retinoschisis (XLRS) using fundus fluorescein angiography (FFA) and ultra-widefield swept-source optical coherence tomography angiography (UWF SS-OCTA) to better understand the disease's vascular features and impact. Methods: A retrospective cross-sectional study was conducted on 26 XLRS patients (46 eyes). A comprehensive ophthalmic examination was performed, including FFA and UWF SS-OCTA. FFA abnormalities were divided into peripheral schisis-associated and optic disc-associated types. Results: The mean age of patients was 11.3±6.5 years. Macular schisis appeared in 97.8% of eyes, peripheral schisis in 89.1%, and peripheral bullous schisis (PBS) in 67.39%. Major vascular changes identified by FFA included dendritic capillary dilation/leakage (91.3%), internal residual vessel leakage (78.3%), and capillary dropout/ischemia (71.7%). Minor changes included zonal retinal pigment epithelium (RPE) proliferation (6.5%), bridging vessels (4.4%), and capillary sheathing (4.4%). peripapillary choroidal neovascularization (PPCNV) was noted in 10.9% and situs inversus of optic disc in 13.0% of eyes. Additionally, situs inversusof optic disc and zonal RPE proliferation were novel findings. Major FFA changes correlated with broader PBS (P = 0.045) (P ＜ 0.001) (P = 0.003). Clock hours of PBS were significant predictors for internal residual vessel leakage (OR = 0.30, P = 0.03). No significant correlation was found between gene mutation type and FFA abnormalities(P = 1.000)(P = 0.539). Conclusions: This study highlighted the significant prevalence (95.7%) of vascular abnormalities in XLRS and emphasized the importance of combining FFA with UWF SS-OCTA for comprehensive evaluation, enhancing the understanding of XLRS pathophysiology and aiding in targeted treatment approaches.
Aims: This study describes vascular abnormalities in X-linked retinoschisis (XLRS) using fundus fluorescein angiography (FFA) and ultra-widefield swept-source optical coherence tomography angiography (UWF SS-OCTA) to better understand the disease's vascular features and impact. Methods: A retrospective cross-sectional study was conducted on 26 XLRS patients (46 eyes). A comprehensive ophthalmic examination was performed, including FFA and UWF SS-OCTA. FFA abnormalities were divided into peripheral schisis-associated and optic disc-associated types. Results: The mean age of patients was 11.3±6.5 years. Macular schisis appeared in 97.8% of eyes, peripheral schisis in 89.1%, and peripheral bullous schisis (PBS) in 67.39%. Major vascular changes identified by FFA included dendritic capillary dilation/leakage (91.3%), internal residual vessel leakage (78.3%), and capillary dropout/ischemia (71.7%). Minor changes included zonal retinal pigment epithelium (RPE) proliferation (6.5%), bridging vessels (4.4%), and capillary sheathing (4.4%). peripapillary choroidal neovascularization (PPCNV) was noted in 10.9% and situs inversus of optic disc in 13.0% of eyes. Additionally, situs inversusof optic disc and zonal RPE proliferation were novel findings. Major FFA changes correlated with broader PBS (P = 0.045) (P ＜ 0.001) (P = 0.003). Clock hours of PBS were significant predictors for internal residual vessel leakage (OR = 0.30, P = 0.03). No significant correlation was found between gene mutation type and FFA abnormalities(P = 0.539). Conclusions: This study highlighted the significant prevalence (95.7%) of vascular abnormalities in XLRS and emphasized the importance of combining FFA with UWF SS-OCTA for comprehensive evaluation, enhancing the understanding of XLRS pathophysiology and aiding in targeted treatment approaches.
目的：评估炎非感染性葡萄膜炎继发炎性脉络膜新生血管(inflammatory choroidal neovascularization, iCNV)的临床特征及眼底多模式影像表现。方法：采用回顾性观察性研究，采用眼底荧光素血管造影(fundus fluorescein angiography, FFA)、吲哚菁绿血管造影(Indocyanine green angiography, ICGA)、谱域相干光断层扫描(spectral domain optical coherence tomography, SD-OCT)联合光学相干断层扫描血管成像(optical coherence tomography angiography, OCTA)等多种眼底影像学方法，对纳入患者的眼底进行检查，分析非感染性iCNV的面积、分型、位置及形态等影像学特征与临床特征的关系。结果：研究共纳入39例患者，对48只患眼中的51处iCNV病灶进行了评估。纳入患者年龄为(35.28±13.62)岁。其中3例患眼出现多灶性CNV。SD-OCT显示92.16%(47/51)的iCNV为2型CNV，17.65%(9/51)的iCNV出现海绵征，13.72%(7/51)的iCNV伴有局灶脉络膜凹陷。ICGA造影期间，74.50%的iCNV病灶(38/51)伴有弱荧光病灶，25.49%的病例(13/51)显示脉络膜高通透性表现。OCTA enface图像显示iCNV形态多样，包括焦点状(15例，29.41%)、盘状/海扇状(16例，31.37%)、枯树状(9例，17.65%)、星状(9例，17.65%)及弥漫网状(2例，3.92%)。其中，枯树状及星状iCNV提示iCNV为非活动性(P＜0.01)。结论：非感染性iCNV与炎性病灶关系密切，在SD-OCT，ICGA上皆具特征性的影像表现。OCTA能直观地观察到iCNV的形态。这些多模式影像特征为临床医生提供了对于非感染性iCNV重要的鉴别诊断依据，有助于制定有效的诊疗方案。
Objective: To evaluate the clinical characteristics and multimodal imaging features of non-infectious inflammatory choroidal neovascularization (iCNV). Methods: In this study retrospective, observational study, multimodal imaging examinations, including fluorescein angiography (FFA), indocyanine green angiography (ICGA), spectral-domain optical coherence tomography (SD-OCT), and optical coherence tomography angiography (OCTA), were used to observe the morphology of non-infectious iCNV in patients diagnosed with uveitis. The area of iCNV, CNV types, CNV morphology and other imaging characteristics were further analyzed. Results: A total of 39 patients were included, with 48 affected eyes and 51 iCNV were identified. The average age of the included patients was 35.28±13.62 years. Among the affected eyes, 3 presented with multifocal CNV, and 92.16% of iCNV were classified as type 2 CNV. iCNV exhibited diverse morphologies, including focal-like pattern (15 cases, 29.41%),sea-fan pattern(16 cases, 31.37%), dead-tree pattern CNV(9 cases, 17.65%), stellar pattern (9 cases, 17.65%), and diffuse reticular (2 cases, 3.92%). Notably, tree-like and stellar pattern iCNV showed a significant correlation with non-active CNV (P < 0.01). Conclusions: Non-infectious iCNV is closely related to inflammatory lesions, exhibiting characteristic imaging features on SD-OCT and ICGA. OCTA allows for direct observation of the morphology of iCNV. These multimodal imaging characteristics provide important diagnostic criteria for clinicians, aiding in the formulation of effective treatment plans.
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.
目的：探讨白塞病性葡萄膜炎(Behçet's uveitis, BU)并发视盘新生血管(neovascularization of the optic disc, NVD)和(或)视盘以外视网膜新生血管(retinal neovascularization elsewhere, NVE)的临床及影像特征。方法：回顾性分析2022年1月—2024年9月就诊的BU并发NVD和(或)NVE患者的临床资料和眼底影像学检查结果，包括眼底彩照、荧光素眼底血管造影(fluorescein fundus angiography, FFA)、光学相干断层成像(optical coherence tomography, OCT)和光学相干断层血管成像(OCT angiography, OCTA)。并分析NVD和(或)NVE面积与患眼的改良的眼后段炎症评分以及视网膜血管渗漏评分的相关性。结果：共纳入27例患者(30只眼)，年龄为(27.70±12.58)岁，男女比例约为1:1。3例(11%)患者双眼并发NVD和(或)NVE；25只眼(83%)存在NVD：17只眼(57%)仅有NVD；8只眼(27%)存在NVD和NVE。仅2只眼(7%)存在视网膜无灌注区，7只眼(23%)同时发生玻璃体积血。FFA眼后段炎症评分为(20.93±4.37)分。FFA血管渗漏评分为(7.57±1.25)分。NVD和(或)NVE面积与眼后段炎症评分(rs = 0.403，P = 0.027)及视网膜血管渗漏评分(rs = 0.518，P = 0.003)均呈正相关。FFA与OCTA在检测NVD和NVE上表现完全一致(κ= 1.0)。结论：BU并发NVD和(或)NVE并不罕见，以NVD为主，绝大多数与视网膜无灌注不相关，可能由BU炎症诱发。
Purpose: To investigate the clinical and imaging characteristics of retinal neovascularization of the optic disc (NVD) and/or elsewhere in the retina (retinal neovascularization elsewhere, NVE) in eyes with Behçet's uveitis (BU). Methods: This retrospective analysis reviewed the clinical data and fundus imaging findings of patients diagnosed with BU complicated by NVD and/or NVE from January 2022 to September 2024. Imaging modalities included fundus photography, fluorescein fundus angiography (FFA), optical coherence tomography (OCT), and OCT angiography (OCTA). The study analyzed the correlation between the areas of NVD and/or NVE and the modified posterior segment inflammation scores, as well as retinal vascular leakage scores of the affected eyes. Results: The study included 27 patients (30 eyes) with an average age of (27.70 ± 12.58) years and a gender ratio of approximately 1:1. Bilateral NVD and/or NVE was observed in three patients (11%); 25 eyes (83%) had NVD, of which 17 eyes (57%) had only NVD and 8 eyes (27%) had both NVD and NVE. Two eyes (7%) showed areas of retinal non-perfusion, and seven eyes (23%) had concurrent vitreous hemorrhage. The average score for posterior segment inflammation on FFA was 20.93 ± 4.37, and the average score for vascular leakage was 7.57 ± 1.25. There was a significant positive correlation between the area of NVD and/or NVE and both the inflammation score (rs = 0.403, P = 0.027) and the vascular leakage score (rs = 0.518, P = 0.003). FFA and OCTA showed perfect agreement in detecting NVD and NVE (κ = 1.0). Conclusion: NVD and/or NVE in BU are not uncommon and are predominantly presented as NVD, mostly not associated with retinal nonperfusion, likely induced by inflammatory factors related to BU.
“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.
眼结核的表现形式多样，有时会被误诊漏诊。本病例为一年轻男性，以右眼视力突发下降为主诉就诊，眼部表现可见黄斑区脉络膜占位病灶及视网膜内小病灶，经γ-干扰素释放试验、胸部CT等检查，确诊为血型播散性肺结核，眼部诊断为结核性脉络膜结节联合视网膜结节，全身接受抗结核治疗后，眼部病灶消退。
Ocular tuberculosis encompasses a variety of clinical manifestations, which is easily misdiagnosed sometimes. In this article, a young male was admitted to our hospital with a chief complaint of sudden blurred vision in his right eye. His ocular examination detected a tiny mass in the choroid and a small lesion in the retina. After interferon-gamma release assay (IGRA) and chest CT scan, the patient was diagnosed with military tuberculosis. The ocular examination confirmed the diagnosis of choroidal tubercle complicated with intrarentinal tubercle, which were healed after systemic anti-tuberculosis therapy.
目的：观察急性中心性浆液性脉络膜视网膜病变(central serous chorioretinopathy，CSC)的自然病程中渗漏点的形态及变化。方法：本研究为前瞻性研究，使用光学相干断层扫描(optical coherence tomography，OCT)观察从发病到发病后5~13个月的CSC患者的渗漏点的OCT形态，测量并计算Hall层、脉络膜全层各自厚度及比值，并进行比较。结果：共20例患者[男14例，女6例，年龄33~59(中位数41)岁]纳入研究。随访时间为5~13个月。在19例患者中观察到微小视网膜色素上皮脱离(pigment epithelium detachment，PED)。1例患者可见视网膜色素上皮(retinal pigment epithelium， RPE)小凸起。在随访期间，仅1例患者的PED完全恢复，其他19例患者在视网膜下液被完全吸收时，RPE和Bruch膜之间仍存在微小分离。渗漏点处的Haller层/脉络膜厚度显著高于中央凹处(初诊时0.806±0.08 vs 0.863±0.06，P=0.003；最后1次随访时为0.801±0.07 vs 0.851±0.06，P=0.004)。结论：本研究观察到在急性CSC患者自然病程中，即使视网膜下液吸收，OCT显示渗漏点处仍存在持续的PED，更厚的Haller层及更薄的内层脉络膜，这些发现为CSC的发病机制提供了更多线索。
Objective: To observe the morphology and changes of leakage points in the natural course of acute central serous chorioretinopathy (CSC). Methods: This study was a prospective study, using optical coherence tomography to observe the OCT morphology of leakage points in CSC patients from onset to 5 to 13 months after onset, measuring the thickness and ratio of Hall layer and the whole choroid, and then compare them. Results: A total of 20 patients were included in the study, including 14 males and 6 females, aged from 33 to 59, with the median being 41 years old. The follow-up time ranged from 5 months to 13 months. Minute retinal pigment epithelial detachments (PED) were observed in 19 patients. A small bulge of retinal pigment epithelium (RPE) was observed in 1 patient. During the follow-up, only one patient totally recovered. Small separation between RPE and Bruch membrane still exit even subretinal fluid were absorbed completely in the other 19 patients. The thickness of Haller layer or choroid at the leakage point was significantly higher than that of the fovea (0.806±0.08 vs 0.863±0.06, P=0.003, at the first visit; 0.801±0.07 vs 0.851±0.06, P=0.004, at the last follow-up). Conclusion: This study observed that in the natural course of acute CSC patients, even if the subretinal fluid was absorbed, OCT still showed that there was persistent PED at the leakage point, thicker Haller layer and thinner inner choroid layer. These findings provided more clues to the pathogenesis of CSC.
目的：分析医学人工智能通识课程“眼科人工智能的研发与应用”的开展效果，为相关医学人工智能通识课程的开展提供参考和借鉴。方法：纵向观察性研究。观察分析2020年秋季学期眼科人工智能的研发与应用通识课程学生人群，课程考核结果以及学生对课程的整体评价。结果：共有118名本科生同学参与了课程学习。其中大部分为低年级临床医学专业本科生。期中考核得分为77.21±10.07，有56位同学(47.46%)达到80分以上。期末考核得分为82.24±6.77，有91位同学(77.12%)达到80分以上。同学对课程的评分为98.76±3.55，超过90%的同学表示课程备课认真、授课条理清晰、表达准确。结论：本课程的顺利进展证明医学人工智能联合教学模式的可行性，理论和实践穿插的教学设置帮助同学们更好地掌握知识技术，完成教学目标。
Objective: To analyze the effectiveness of medical education curriculum named “Development and Application of Ophthalmic Artificial Intelligence”, and provide reference for the development of other related curriculums. Methods: Longitudinal observational study method was adopted. During the fall semester of 2020, we conducted an education curriculum named “Development and Application of Ophthalmic Artificial Intelligence” and analyzed the results of mid-term and final examinations, and curriculum evaluation of students. Results: There were 118 undergraduate students taking the course and most of them were junior students majoring in clinical medicine. The score of the mid-term examination was in the range of 77.2±10.07, and 56 students (47.46%) got more than 80 points. The score of the final examination was in the range of 82.24±6.77, and 91 students (77.12%) got more than 80 points. The score of course evaluation of students was in the range of 98.76±3.55, and more than 90% of the students thought that teachers have made full preparations before class, together with clear teaching logic and accurate expressions in class. Conclusion: The smooth progress of our course proved the feasibility of medical artificial intelligence teaching. The teaching setting interspersed with theory and practice could help students to master knowledge and technology better, so as to achieve the teaching objectives.
