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Peripheral Retinal Angiopathy Associated with COL4A1 Mutation. 与COL4A1突变相关的视网膜周围血管病变。
IF 5.7 Q1 OPHTHALMOLOGY Pub Date : 2026-01-31 DOI: 10.1016/j.oret.2026.01.007
Prithvi Ramtohul, Kevin Mairot, Thierry David
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引用次数: 0
Spontaneously Resolved Lipemia Retinalis. 自发解决视网膜脂血症。
IF 5.7 Q1 OPHTHALMOLOGY Pub Date : 2026-01-31 DOI: 10.1016/j.oret.2026.01.005
Manu Sharma, Nitin Gautam, Reema Bansal
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引用次数: 0
Global Validation of the Postnatal Growth and Retinopathy of Prematurity Screening Model: A Systematic Review and Meta-analysis. 早产儿筛选模型的出生后生长和视网膜病变的全球验证:系统回顾和荟萃分析。
IF 5.7 Q1 OPHTHALMOLOGY Pub Date : 2026-01-30 DOI: 10.1016/j.oret.2026.01.020
Sheng-Chu Chi, Hsin-Ho Chang, Tai-Chi Lin, Shih-Jen Chen, Jin-Hua Chen

Topic: The G-ROP criteria, incorporating postnatal weight gain, are widely validated for retinopathy of prematurity (ROP) screening. We assessed the G-ROP algorithm's diagnostic accuracy across various outcomes and explored heterogeneity sources.

Clinical relevance: If G-ROP maintains sensitivity with fewer exams, it would optimize screening for this blinding disease.

Method: We searched PubMed, EMBASE, and Cochrane databases (through September 2024) for studies using G-ROP to predict type 1, type 2, treated, or any-stage ROP. We assessed risk of bias using QUADAS-2 and certainty of evidence using GRADE approach. The protocol was prospectively registered with PROSPERO (CRD42024571794).

Results: Twenty-three studies were included. For type 1 ROP (17 cohorts; 1,406 infants), sensitivity was 0.99 (95% CI: 0.99-1.00; I2=44.5%) and specificity (9 cohorts; 2,522 infants) was 0.34 (95% CI: 0.32-0.36; I2=96.3%).The diagnostic odds ratio (DOR) was 6.22 (95% CI: 2.34-16.53; I2=40.7%) ; AUC was 0.87 (95% CI: 0.83-0.90). Certainty of evidence was high for sensitivity and low for specificity and DOR. For type 2 ROP, pooled sensitivity from 9 cohorts (871 infants) was 0.98 (95% CI: 0.97-0.99; I2=41.4%), while specificity from 3 cohorts (621 infants) was 0.25 (95% CI: 0.22-0.29; I2=92.2%). The DOR was 3.21 (95% CI: 1.24-8.28; I2=0%); AUC was 0.76 (95% CI: 0.72-0.80). Certainty was high for sensitivity, very low for specificity, and moderate for DOR. For any-stage ROP, pooled sensitivity from 16 cohorts (3,674 infants) was 0.87 (95% CI: 0.86-0.88; I2=90.2%), while specificity from 13 cohorts (2,846 infants) was 0.45 (95% CI: 0.43-0.47; I2=95.8%). The DOR was 5.88 (95% CI: 3.89-8.89; I2=75.3%), and the AUC was 0.78 (95% CI: 0.74-0.81). Certainty was low for sensitivity, low for specificity, and moderate for DOR.

Conclusions: The G-ROP algorithm demonstrates consistently high sensitivity, particularly for type 1 and treated ROP, supporting its use as a screening tool for severe disease. While the model reduces examinations compared with current standards, its low specificity may limit its usefulness in low-resource settings, where minimizing unnecessary screening is essential.

G-ROP标准,包括出生后体重增加,被广泛证实用于早产儿视网膜病变(ROP)筛查。我们评估了G-ROP算法在各种结果中的诊断准确性,并探索了异质性来源。临床意义:如果G-ROP在较少检查的情况下保持敏感性,将优化对这种致盲疾病的筛查。方法:我们检索PubMed、EMBASE和Cochrane数据库(截至2024年9月),查找使用G-ROP预测1型、2型、治疗期或任何阶段ROP的研究。我们使用QUADAS-2评估偏倚风险,使用GRADE方法评估证据的确定性。该方案在PROSPERO进行前瞻性注册(CRD42024571794)。结果:纳入23项研究。对于1型ROP(17个队列,1,406名婴儿),敏感性为0.99 (95% CI: 0.99-1.00; I2=44.5%),特异性(9个队列,2,522名婴儿)为0.34 (95% CI: 0.32-0.36; I2=96.3%)。诊断优势比(DOR)为6.22 (95% CI: 2.34-16.53; I2=40.7%);AUC为0.87 (95% CI: 0.83-0.90)。证据的确定性在敏感性方面高,在特异性和DOR方面低。对于2型ROP,来自9个队列(871名婴儿)的合并敏感性为0.98 (95% CI: 0.97-0.99; I2=41.4%),而来自3个队列(621名婴儿)的特异性为0.25 (95% CI: 0.22-0.29; I2=92.2%)。DOR为3.21 (95% CI: 1.24-8.28; I2=0%);AUC为0.76 (95% CI: 0.72-0.80)。灵敏度高,特异性低,DOR为中等。对于任何阶段的ROP, 16个队列(3,674名婴儿)的合并敏感性为0.87 (95% CI: 0.86-0.88; I2=90.2%),而13个队列(2,846名婴儿)的特异性为0.45 (95% CI: 0.43-0.47; I2=95.8%)。DOR为5.88 (95% CI: 3.89-8.89; I2=75.3%), AUC为0.78 (95% CI: 0.74-0.81)。确定性的敏感性低,特异性低,DOR中等。结论:G-ROP算法表现出一贯的高灵敏度,特别是对于1型和治疗型ROP,支持其作为严重疾病的筛查工具。虽然与目前的标准相比,该模型减少了检查,但其低特异性可能会限制其在低资源环境中的实用性,在这些环境中,最大限度地减少不必要的筛查是至关重要的。
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引用次数: 0
Association of metformin use and new-onset ICD coding of neovascular age-related macular degeneration. 二甲双胍使用与新生血管性年龄相关性黄斑变性新发ICD编码的关系。
IF 5.7 Q1 OPHTHALMOLOGY Pub Date : 2026-01-30 DOI: 10.1016/j.oret.2026.01.019
Jason F Xiao, Max J Hyman, John Moir, Madeleine Yehia, Seenu Hariprasad, Andrea Flores, Dimitra Skondra

Purpose: To investigate whether metformin use is associated with decreased odds of new-onset International Classification of Disease (ICD) coding of neovascular age-related macular degeneration (nAMD).

Design: Case-control study.

Participants: 22,205 cases with new-onset ICD coding of nAMD and 22,126 matched controls without AMD were identified in the Merative™ MarketScan® Research Databases between 2008 and 2017. A subgroup of patients diagnosed with diabetes included 6,664 cases and 5,513 controls.

Methods: Cases with new-onset ICD coding of nAMD were propensity score matched to controls without AMD. Multivariable conditional logistic regression analyzed the association between new-onset ICD coding of nAMD and metformin use controlling for (1) AMD risk factors, including diabetes, diabetic retinopathy, hyperlipidemia, obesity, and smoking, (2) exposures to other antidiabetic medications, including insulin, sulfonylureas, glitazones, meglitinides, other diabetic medications, and statins, and (3) the number of antidiabetic medications.

Main outcome measures: Adjusted odds ratios of new-onset ICD coding of nAMD for any metformin use and cumulative two-year metformin dose in grams (g).

Results: Any metformin use was associated with decreased adjusted odds of new-onset nAMD (aOR 0.84, 95% CI 0.74-0.95). In an exploratory dosing analysis, associations across cumulative-dose categories were heterogeneous, with the largest magnitude in the mid-dose range (between 271 and 600 g; aOR 0.73, 95% CI 0.63-0.85); because adherence cannot be verified from claims, dose-response findings are interpreted as hypothesis-generating. These associations persisted among diabetic patients (Any metformin use: aOR 0.83, 95% CI 0.72-0.94; 271 to 600 g: aOR 0.72, 95% CI 0.61-0.85; >1080 g: aOR 0.85, 95% CI 0.72-0.999). Any metformin use in diabetic patients without retinopathy was associated with decreased odds of new-onset ICD nAMD (aOR 0.85, 95% CI 0.78-0.93); however, this association was absent in diabetic patients with retinopathy.

Conclusions: In this claims-based analysis, metformin use was associated with lower odds of incident ICD-coded nAMD, and an exploratory dosing analysis suggests a low to moderate cumulative dose may be associated with the greatest decrease. These associations persisted among diabetic patients without retinopathy. Causal inference is limited by the observational design, potential diagnostic misclassification, imperfect exposure measurement, and residual confounding. Replication with validated case definitions, active-comparator new-user designs, and adjudicated outcomes is warranted.

目的:研究使用二甲双胍是否与新发血管性年龄相关性黄斑变性(nAMD)国际疾病分类(ICD)编码的几率降低有关。设计:病例对照研究。参与者:在2008年至2017年期间,在Merative™MarketScan®研究数据库中确定了22205例新发ICD编码为nAMD的病例和22126例无AMD的匹配对照。诊断为糖尿病的患者亚组包括6664例和5513例对照。方法:将新发ICD编码为nAMD的病例与无AMD的对照组进行倾向评分匹配。多变量条件logistic回归分析了新发ICD编码nAMD与二甲双胍使用控制之间的关系:(1)AMD危险因素,包括糖尿病、糖尿病视网膜病变、高脂血症、肥胖和吸烟;(2)其他降糖药物暴露,包括胰岛素、磺脲类、格列酮、美格列酮、其他降糖药物和他汀类药物;(3)降糖药物的数量。主要结局指标:任何二甲双胍使用的新发ICD编码nAMD的调整优势比和以克(g)为单位的两年二甲双胍累积剂量。结果:任何二甲双胍的使用都与新发nAMD的调整后几率降低相关(aOR 0.84, 95% CI 0.74-0.95)。在一项探索性剂量分析中,累积剂量类别之间的相关性是不均匀的,在中剂量范围内的相关性最大(271至600 g; aOR 0.73, 95% CI 0.63-0.85);由于依从性不能从声明中得到证实,剂量-反应结果被解释为产生假设。这些相关性在糖尿病患者中持续存在(任何二甲双胍使用:aOR 0.83, 95% CI 0.72-0.94; 271 - 600 g: aOR 0.72, 95% CI 0.61-0.85; bbb1080 g: aOR 0.85, 95% CI 0.72-0.999)。无视网膜病变的糖尿病患者使用二甲双胍与新发ICD nAMD的几率降低相关(aOR 0.85, 95% CI 0.78-0.93);然而,这种关联在糖尿病视网膜病变患者中不存在。结论:在这项基于索赔的分析中,使用二甲双胍与icd编码的nAMD发生率较低相关,探索性剂量分析表明,低至中等累积剂量可能与最大的降低相关。这些关联在没有视网膜病变的糖尿病患者中持续存在。因果推断受到观察设计、潜在的诊断错误分类、不完善的暴露测量和残留混淆的限制。通过验证案例定义、主动比较器新用户设计和裁决结果进行复制是有保证的。
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引用次数: 0
Standardizing Measurement of cRORA (Complete Retinal Pigment Epithelial and Outer Retinal Atrophy)- CAM Report 8. cRORA(完全视网膜色素上皮和外视网膜萎缩)的标准化测量- CAM报告
IF 5.7 Q1 OPHTHALMOLOGY Pub Date : 2026-01-30 DOI: 10.1016/j.oret.2026.01.017
David Sarraf, Federico Corvi, Philip J Rosenfeld, Thomas Ach, Barbara Blodi, Ferdinando Bottoni, Mariano Cozzi, Usha Chakravarthy, Emily Y Chew, Karl Csaky, Cathy Cukras, Christine A Curcio, Frederick L Ferris, K Bailey Freund, Juan Grunwald, Robyn H Guymer, Frank G Holz, Glenn J Jaffe, Sandra Liakopoulos, Tock Han Lim, Jordi M Monés, Cynthia Owsley, Sergio Pagliarini, Daniel Pauleikhoff, Steffen Schmitz-Valckenberg, Richard F Spaide, Janet Sparrow, Ramin Tadayoni, Adnan Tufail, Francesco Viola, Scott Fraser, Giovanni Staurenghi, Srinivas R Sadda

Purpose: To highlight the role of en face OCT in the measurement of choroidal hypertransmission defects (hyperTDs) in complete retinal pigment epithelial and outer retinal atrophy (cRORA), and to refine the definition of retinal pigment epithelium (RPE)-related alterations associated with cRORA.

Design: Consensus meeting.

Participants: Panel of retina specialists, including retinal imaging experts, reading center leaders, and retinal histologists.

Methods: As part of the Classification of Atrophy Meeting (CAM) program, an international group of experts analyzed and discussed the role of en face OCT in the assessment of cRORA. A structured study was conducted, consisting of an exercise to assess en face OCT cases, reviewed jointly during the 8th CAM meeting, and to explore the utility of this advanced tool for disease staging and progression. Additionally, definitions previously applied to RPE abnormalities were discussed and refined leading to modifications. The current report summarizes the methods employed during the consensus meeting and the outcomes achieved as pertains to the application of en face OCT for cRORA grading and simplification of the RPE assessment criteria.

Main outcome measure: Defining the role of en face OCT in the detection and quantification of hyperTDs, improving classification of cRORA, and refining the terminology related to RPE alterations to enhance grading consistency.

Results: During the consensus case discussions, high levels of agreement were achieved for hyperTD detection using en face OCT. The CAM group affirmed the role of en face OCT as a critical adjunct to traditional B-scan analysis, for more precise measurement of the area of cRORA lesions and for distinguishing threshold cRORA from smaller incomplete (iRORA) lesions. Additionally, merger of RPE attenuation and RPE disruption into a unified category termed "abnormal RPE band" significantly reduced inter-reader variability, leading to greater consistency among graders.

Conclusions: The integration of en face OCT with cross-sectional B-scan imaging enhances the accurate classification and area measurement of early atrophic alterations in AMD, improving diagnostic consistency and lesion assessment. The consensus panel's adoption of the abnormal RPE band term as a unified category for RPE abnormalities may reduce confusion regarding the definition of RPE degeneration and has the potential to improve inter-reader variability.

目的:强调表面OCT在测量完全视网膜色素上皮和视网膜外萎缩(cRORA)的脉络膜超透射缺陷(hypertd)中的作用,并完善cRORA相关视网膜色素上皮(RPE)相关改变的定义。设计:共识会议。参与者:视网膜专家小组,包括视网膜成像专家、阅读中心领导和视网膜组织学家。方法:作为萎缩分类会议(CAM)计划的一部分,一个国际专家小组分析和讨论了正面OCT在cRORA评估中的作用。我们进行了一项结构化的研究,包括评估面对面OCT病例的练习,在第8届CAM会议期间进行了联合审查,并探讨了这种先进工具在疾病分期和进展方面的应用。此外,对先前应用于RPE异常的定义进行了讨论和改进,从而进行了修改。本报告总结了共识会议期间采用的方法以及在应用面对面OCT进行cRORA分级和简化RPE评估标准方面取得的成果。主要结果测量:确定正面OCT在hypertd检测和定量中的作用,改进cRORA分类,细化与RPE改变相关的术语以提高分级一致性。结果:在一致的病例讨论中,使用正面OCT检测超td达到了高度的一致性。CAM组肯定了正面OCT作为传统b扫描分析的关键辅助手段的作用,可以更精确地测量cRORA病变的面积,并将阈值cRORA与较小的不完全(iRORA)病变区分出来。此外,将RPE衰减和RPE中断合并为一个统一的类别,称为“异常RPE带”,显著降低了阅读器之间的变异性,从而提高了评分者之间的一致性。结论:正面OCT与横断b扫描影像的结合可提高AMD早期萎缩性病变的准确分类和面积测量,提高诊断一致性和病变评估。共识小组采用异常RPE波段术语作为RPE异常的统一类别,可能会减少关于RPE变性定义的混淆,并有可能改善读者之间的差异。
{"title":"Standardizing Measurement of cRORA (Complete Retinal Pigment Epithelial and Outer Retinal Atrophy)- CAM Report 8.","authors":"David Sarraf, Federico Corvi, Philip J Rosenfeld, Thomas Ach, Barbara Blodi, Ferdinando Bottoni, Mariano Cozzi, Usha Chakravarthy, Emily Y Chew, Karl Csaky, Cathy Cukras, Christine A Curcio, Frederick L Ferris, K Bailey Freund, Juan Grunwald, Robyn H Guymer, Frank G Holz, Glenn J Jaffe, Sandra Liakopoulos, Tock Han Lim, Jordi M Monés, Cynthia Owsley, Sergio Pagliarini, Daniel Pauleikhoff, Steffen Schmitz-Valckenberg, Richard F Spaide, Janet Sparrow, Ramin Tadayoni, Adnan Tufail, Francesco Viola, Scott Fraser, Giovanni Staurenghi, Srinivas R Sadda","doi":"10.1016/j.oret.2026.01.017","DOIUrl":"https://doi.org/10.1016/j.oret.2026.01.017","url":null,"abstract":"<p><strong>Purpose: </strong>To highlight the role of en face OCT in the measurement of choroidal hypertransmission defects (hyperTDs) in complete retinal pigment epithelial and outer retinal atrophy (cRORA), and to refine the definition of retinal pigment epithelium (RPE)-related alterations associated with cRORA.</p><p><strong>Design: </strong>Consensus meeting.</p><p><strong>Participants: </strong>Panel of retina specialists, including retinal imaging experts, reading center leaders, and retinal histologists.</p><p><strong>Methods: </strong>As part of the Classification of Atrophy Meeting (CAM) program, an international group of experts analyzed and discussed the role of en face OCT in the assessment of cRORA. A structured study was conducted, consisting of an exercise to assess en face OCT cases, reviewed jointly during the 8<sup>th</sup> CAM meeting, and to explore the utility of this advanced tool for disease staging and progression. Additionally, definitions previously applied to RPE abnormalities were discussed and refined leading to modifications. The current report summarizes the methods employed during the consensus meeting and the outcomes achieved as pertains to the application of en face OCT for cRORA grading and simplification of the RPE assessment criteria.</p><p><strong>Main outcome measure: </strong>Defining the role of en face OCT in the detection and quantification of hyperTDs, improving classification of cRORA, and refining the terminology related to RPE alterations to enhance grading consistency.</p><p><strong>Results: </strong>During the consensus case discussions, high levels of agreement were achieved for hyperTD detection using en face OCT. The CAM group affirmed the role of en face OCT as a critical adjunct to traditional B-scan analysis, for more precise measurement of the area of cRORA lesions and for distinguishing threshold cRORA from smaller incomplete (iRORA) lesions. Additionally, merger of RPE attenuation and RPE disruption into a unified category termed \"abnormal RPE band\" significantly reduced inter-reader variability, leading to greater consistency among graders.</p><p><strong>Conclusions: </strong>The integration of en face OCT with cross-sectional B-scan imaging enhances the accurate classification and area measurement of early atrophic alterations in AMD, improving diagnostic consistency and lesion assessment. The consensus panel's adoption of the abnormal RPE band term as a unified category for RPE abnormalities may reduce confusion regarding the definition of RPE degeneration and has the potential to improve inter-reader variability.</p>","PeriodicalId":19501,"journal":{"name":"Ophthalmology. Retina","volume":" ","pages":""},"PeriodicalIF":5.7,"publicationDate":"2026-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146100456","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Retinal Pigment Epithelial Hamartomas in Familial Adenomatous Polyposis: New Insights Based on Multimodal Imaging of 233 Lesions. 家族性腺瘤性息肉病中的视网膜色素上皮错构瘤:基于233个病变的多模态成像的新见解。
IF 5.7 Q1 OPHTHALMOLOGY Pub Date : 2026-01-28 DOI: 10.1016/j.oret.2026.01.012
Cheng Fei Kong, Christopher Go, Stephanie Oi-Lam Wu, Marion R Munk, Adrian T Fung

Objective: To describe the multimodal imaging characteristics of retinal pigment epithelial hamartomas associated with familial adenomatous polyposis (RPEH-FAP).

Design: Prospective, observational case series.

Participants: Twenty-four eyes of 12 patients diagnosed with FAP based on systemic clinical findings.

Methods: Multimodal imaging of RPEH-FAP lesions included: Ultra-wide field fundus photography, ultra-wide field fundus autofluorescence, enhanced-depth imaging optical coherence tomography (EDI-OCT) and angiography (OCT-A).

Main outcomes measured: Features of RPEH-FAP on multimodal imaging.

Results: The mean age of patient was 46.8 years (range 20-72 years). There were 8 females (66.7%), and all patients were Caucasian. Two hundred and thirty-three RPEH-FAP lesions were identified (mean of 9.7 lesions per eye, range 1-31). The superotemporal quadrant was the most commonly involved quadrant (36.1%). Most were outside the posterior pole (92.7%). The most common appearance were small circular pigmented dots (42.9%). Depigmented margins were seen in 113 (48.5%) lesions. The pigmented portion of the lesion was hypoautofluorescent in 93.3% of cases, and when present, the depigmented halos/fish tails were either isoautofluorescent (46.8%) or hyperautofluorescent (35.1%). OCT was obtained for 49 lesions. Mean sub-lesional choroidal thickness was not different compared with choroidal thickness 50μm outside the margin. The RPE was relatively thickened in 67.3% of cases. Retinal thinning occurs in the outer retina, with thinning or absence of the outer nuclear layer in 83.7% of cases and of the ellipsoid zone in 93.9% of cases. Hyperreflective retinal spots were seen in 14 cases (28.6%). Eleven lesions (22.4%) had an associated pigment epithelial detachment (PED). One lesion demonstrated a subretinal cleft. No lesion demonstrated cystoid edema or subretinal fluid. No OCTA signal was observed on OCT-A.

Conclusions: In this series, a mean of about 10 RPEH-FAP lesions were identified in each eye. RPEH-FAP are usually hypoautofluorescent with outer retinal loss on OCT and as such are similar to typical solitary Congenital Hypertrophy of the Retinal Pigment Epithelium (CHRPE) lesions not associated with FAP. However, key features differentiating RPEH-FAP from typical solitary CHRPE are the higher number of lesions involving both eyes, bilateral involvement, presence of an iso- or hyperautofluorescent depigmented halo or fish tail and the absence of lacunae or cystoid edema. The presence of PEDs is a new finding.

目的:探讨视网膜色素上皮错构瘤合并家族性腺瘤性息肉病(RPEH-FAP)的多模态影像学特征。设计:前瞻性观察性病例系列。参与者:根据系统临床表现诊断为FAP的12例患者24只眼。方法:RPEH-FAP病变的多模态成像包括:超宽视场眼底摄影、超宽视场眼底自身荧光、增强深度成像光学相干断层扫描(edii - oct)和血管造影(OCT-A)。测量的主要结果:RPEH-FAP在多模态成像上的特征。结果:患者平均年龄46.8岁(20 ~ 72岁)。女性8例(66.7%),均为白种人。共发现233个RPEH-FAP病变(平均每只眼9.7个病变,范围1-31)。颞上象限是最常见的象限(36.1%)。大多数位于后极外(92.7%)。最常见的外观是小的圆形色素点(42.9%)。113例(48.5%)病灶边缘有脱色。在93.3%的病例中,色素沉着的病变部分是低自荧光的,当出现色素沉着的晕/鱼尾时,要么是等自荧光的(46.8%),要么是超自荧光的(35.1%)。49个病灶行OCT检查。亚病变脉络膜的平均厚度与边缘外50μm的脉络膜厚度没有差异。67.3%的病例RPE相对增厚。视网膜变薄发生在外视网膜,83.7%的病例外核层变薄或缺失,93.9%的病例椭球区变薄或缺失。高反射性视网膜斑点14例(28.6%)。11个病变(22.4%)伴有色素上皮脱离(PED)。一处病变表现为视网膜下裂。没有病变显示囊样水肿或视网膜下积液。OCT-A未见OCTA信号。结论:在本研究中,每只眼平均发现约10个RPEH-FAP病变。RPEH-FAP通常在OCT上表现为低自身荧光并伴有外视网膜缺失,因此与典型的与FAP无关的先天性视网膜色素上皮肥厚(CHRPE)病变相似。然而,区分RPEH-FAP与典型孤立性CHRPE的关键特征是累及双眼的病变数量较多,双侧受累,存在等荧光或高自荧光脱色晕或鱼尾,没有腔窝或囊样水肿。儿科医生的出现是一个新发现。
{"title":"Retinal Pigment Epithelial Hamartomas in Familial Adenomatous Polyposis: New Insights Based on Multimodal Imaging of 233 Lesions.","authors":"Cheng Fei Kong, Christopher Go, Stephanie Oi-Lam Wu, Marion R Munk, Adrian T Fung","doi":"10.1016/j.oret.2026.01.012","DOIUrl":"https://doi.org/10.1016/j.oret.2026.01.012","url":null,"abstract":"<p><strong>Objective: </strong>To describe the multimodal imaging characteristics of retinal pigment epithelial hamartomas associated with familial adenomatous polyposis (RPEH-FAP).</p><p><strong>Design: </strong>Prospective, observational case series.</p><p><strong>Participants: </strong>Twenty-four eyes of 12 patients diagnosed with FAP based on systemic clinical findings.</p><p><strong>Methods: </strong>Multimodal imaging of RPEH-FAP lesions included: Ultra-wide field fundus photography, ultra-wide field fundus autofluorescence, enhanced-depth imaging optical coherence tomography (EDI-OCT) and angiography (OCT-A).</p><p><strong>Main outcomes measured: </strong>Features of RPEH-FAP on multimodal imaging.</p><p><strong>Results: </strong>The mean age of patient was 46.8 years (range 20-72 years). There were 8 females (66.7%), and all patients were Caucasian. Two hundred and thirty-three RPEH-FAP lesions were identified (mean of 9.7 lesions per eye, range 1-31). The superotemporal quadrant was the most commonly involved quadrant (36.1%). Most were outside the posterior pole (92.7%). The most common appearance were small circular pigmented dots (42.9%). Depigmented margins were seen in 113 (48.5%) lesions. The pigmented portion of the lesion was hypoautofluorescent in 93.3% of cases, and when present, the depigmented halos/fish tails were either isoautofluorescent (46.8%) or hyperautofluorescent (35.1%). OCT was obtained for 49 lesions. Mean sub-lesional choroidal thickness was not different compared with choroidal thickness 50μm outside the margin. The RPE was relatively thickened in 67.3% of cases. Retinal thinning occurs in the outer retina, with thinning or absence of the outer nuclear layer in 83.7% of cases and of the ellipsoid zone in 93.9% of cases. Hyperreflective retinal spots were seen in 14 cases (28.6%). Eleven lesions (22.4%) had an associated pigment epithelial detachment (PED). One lesion demonstrated a subretinal cleft. No lesion demonstrated cystoid edema or subretinal fluid. No OCTA signal was observed on OCT-A.</p><p><strong>Conclusions: </strong>In this series, a mean of about 10 RPEH-FAP lesions were identified in each eye. RPEH-FAP are usually hypoautofluorescent with outer retinal loss on OCT and as such are similar to typical solitary Congenital Hypertrophy of the Retinal Pigment Epithelium (CHRPE) lesions not associated with FAP. However, key features differentiating RPEH-FAP from typical solitary CHRPE are the higher number of lesions involving both eyes, bilateral involvement, presence of an iso- or hyperautofluorescent depigmented halo or fish tail and the absence of lacunae or cystoid edema. The presence of PEDs is a new finding.</p>","PeriodicalId":19501,"journal":{"name":"Ophthalmology. Retina","volume":" ","pages":""},"PeriodicalIF":5.7,"publicationDate":"2026-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146092901","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Diagnostic Pars Plana Vitrectomy in Undifferentiated Uveitis: Systematic Review and Meta-Analysis. 诊断性玻璃体切除术治疗未分化葡萄膜炎:系统回顾和荟萃分析。
IF 5.7 Q1 OPHTHALMOLOGY Pub Date : 2026-01-28 DOI: 10.1016/j.oret.2026.01.011
Charles Zhang, Georges AbouKasm, Danial A Lai, Sinan Ersan, Nicholas Leung, Daniel Zhu, Nicolas A Yannuzzi, Steven Yeh, Thomas A Albini

Topic: With advancements in surgical techniques and diagnostic methodologies, this meta-analysis aims to assess the diagnostic yield of pars plana vitrectomy (PPV) in undifferentiated uveitis.

Clinical relevance: Undifferentiated uveitis poses significant diagnostic challenges in ophthalmology. While diagnostic PPV has been employed to identify underlying etiologies, the diagnostic utility of this procedure is still unclear.

Methods: A comprehensive literature search was conducted on March 24th, 2025 using PubMed, Embase, and Scopus to identify studies evaluating the diagnostic yield of PPV in undifferentiated uveitis. Studies were included if they evaluated the diagnostic yield of PPV in undifferentiated uveitis and contained various outcome measures: identification rates of infectious and malignant etiologies, confirmation of pre-operative suspicion, changes in management, and rates of retinal detachment. A random-effects model was used to compute pooled estimates, and heterogeneity was assessed using the I2 statistic. ROBINS-I, NIH Quality Assessment Tool, and GRADE were used to assess risk of bias and strength of certainty.

Results: Seventeen studies (1,032 eyes) met inclusion criteria. The pooled diagnostic yield of PPV was 45.5% [95% CI: 37.4-53.6%], with significant heterogeneity (I2=84.5%). Subgroup analysis indicated a higher diagnostic yield in studies (10 studies, 521 eyes) performed after 2010 (53.4%) [95% CI: 45.0-61.6%] compared to those (7 studies, 511 eyes) before 2010 (35.2%) [95% CI: 23.0-48.3%, p=0.0217]. Infectious etiologies were identified in 60.9% [95% CI: 50.8-70.5%] of cases, while malignant causes accounted for 33.2% [95% CI: 23.6-44.4%]. Pre-operative clinical suspicion for infection or malignancy was confirmed in 70.2% [95% CI: 57.1-82.0%] and 51.7% [95% CI: 34.6-68.6%] of cases, respectively. Conversely, cases without a clear pre-operative suspicion resulted in a diagnostic yield in only 25.2% [95% CI: 12.8-39.7%]. A change in management was observed in 21.6% [95% CI: 12.6-32.1%] of cases following PPV. Post-operative retinal detachment occurred in 3.9% [95% CI: 1.5-7.1%] of eyes. Using GRADE tool, two outcomes were graded low and four were graded very low due to the retrospective nature of all studies included for analysis.

Conclusions: Diagnostic PPV may serve as a useful adjunct in the evaluation of undifferentiated uveitis; however, its diagnostic yield is greater when guided by a specific pre-operative diagnostic hypothesis, particularly for suspected infectious or malignant etiologies.

主题:随着手术技术和诊断方法的进步,本荟萃分析旨在评估未分化葡萄膜炎的玻璃体切除(PPV)的诊断率。临床相关性:未分化的葡萄膜炎对眼科的诊断提出了重大挑战。虽然诊断性PPV已被用于确定潜在的病因,但该程序的诊断效用仍不清楚。方法:于2025年3月24日使用PubMed、Embase和Scopus进行全面的文献检索,以确定评估PPV在未分化葡萄膜炎诊断率的研究。如果研究评估了未分化性葡萄膜炎中PPV的诊断率,并包含各种结果测量:感染性和恶性病因的识别率、术前怀疑的确认、管理的改变和视网膜脱离的发生率,则纳入研究。随机效应模型用于计算汇总估计值,并使用I2统计量评估异质性。采用ROBINS-I、NIH质量评估工具和GRADE来评估偏倚风险和确定性强度。结果:17项研究(1032只眼)符合纳入标准。PPV的综合诊断率为45.5% [95% CI: 37.4-53.6%],异质性显著(I2=84.5%)。亚组分析显示,2010年后进行的研究(10项研究,521只眼)的诊断率(53.4%)[95% CI: 45.0 ~ 61.6%]高于2010年前的研究(7项研究,511只眼)(35.2%)[95% CI: 23.0 ~ 48.3%, p=0.0217]。60.9% [95% CI: 50.8 ~ 70.5%]的病例为感染性病因,33.2% [95% CI: 23.6 ~ 44.4%]的病例为恶性病因。70.2% [95% CI: 57.1-82.0%]和51.7% [95% CI: 34.6-68.6%]的患者术前临床怀疑有感染或恶性肿瘤。相反,术前未明确怀疑的病例的诊断率仅为25.2% [95% CI: 12.8-39.7%]。21.6% [95% CI: 12.6-32.1%]的PPV患者出现管理改变。术后视网膜脱离发生率为3.9% [95% CI: 1.5-7.1%]。使用GRADE工具,由于纳入分析的所有研究的回顾性性质,两个结果被评为低,四个结果被评为非常低。结论:诊断性PPV可作为评价未分化葡萄膜炎的有用辅助手段;然而,在特定的术前诊断假设的指导下,特别是对于疑似感染或恶性病因,其诊断率更高。
{"title":"Diagnostic Pars Plana Vitrectomy in Undifferentiated Uveitis: Systematic Review and Meta-Analysis.","authors":"Charles Zhang, Georges AbouKasm, Danial A Lai, Sinan Ersan, Nicholas Leung, Daniel Zhu, Nicolas A Yannuzzi, Steven Yeh, Thomas A Albini","doi":"10.1016/j.oret.2026.01.011","DOIUrl":"https://doi.org/10.1016/j.oret.2026.01.011","url":null,"abstract":"<p><strong>Topic: </strong>With advancements in surgical techniques and diagnostic methodologies, this meta-analysis aims to assess the diagnostic yield of pars plana vitrectomy (PPV) in undifferentiated uveitis.</p><p><strong>Clinical relevance: </strong>Undifferentiated uveitis poses significant diagnostic challenges in ophthalmology. While diagnostic PPV has been employed to identify underlying etiologies, the diagnostic utility of this procedure is still unclear.</p><p><strong>Methods: </strong>A comprehensive literature search was conducted on March 24<sup>th</sup>, 2025 using PubMed, Embase, and Scopus to identify studies evaluating the diagnostic yield of PPV in undifferentiated uveitis. Studies were included if they evaluated the diagnostic yield of PPV in undifferentiated uveitis and contained various outcome measures: identification rates of infectious and malignant etiologies, confirmation of pre-operative suspicion, changes in management, and rates of retinal detachment. A random-effects model was used to compute pooled estimates, and heterogeneity was assessed using the I<sup>2</sup> statistic. ROBINS-I, NIH Quality Assessment Tool, and GRADE were used to assess risk of bias and strength of certainty.</p><p><strong>Results: </strong>Seventeen studies (1,032 eyes) met inclusion criteria. The pooled diagnostic yield of PPV was 45.5% [95% CI: 37.4-53.6%], with significant heterogeneity (I<sup>2</sup>=84.5%). Subgroup analysis indicated a higher diagnostic yield in studies (10 studies, 521 eyes) performed after 2010 (53.4%) [95% CI: 45.0-61.6%] compared to those (7 studies, 511 eyes) before 2010 (35.2%) [95% CI: 23.0-48.3%, p=0.0217]. Infectious etiologies were identified in 60.9% [95% CI: 50.8-70.5%] of cases, while malignant causes accounted for 33.2% [95% CI: 23.6-44.4%]. Pre-operative clinical suspicion for infection or malignancy was confirmed in 70.2% [95% CI: 57.1-82.0%] and 51.7% [95% CI: 34.6-68.6%] of cases, respectively. Conversely, cases without a clear pre-operative suspicion resulted in a diagnostic yield in only 25.2% [95% CI: 12.8-39.7%]. A change in management was observed in 21.6% [95% CI: 12.6-32.1%] of cases following PPV. Post-operative retinal detachment occurred in 3.9% [95% CI: 1.5-7.1%] of eyes. Using GRADE tool, two outcomes were graded low and four were graded very low due to the retrospective nature of all studies included for analysis.</p><p><strong>Conclusions: </strong>Diagnostic PPV may serve as a useful adjunct in the evaluation of undifferentiated uveitis; however, its diagnostic yield is greater when guided by a specific pre-operative diagnostic hypothesis, particularly for suspected infectious or malignant etiologies.</p>","PeriodicalId":19501,"journal":{"name":"Ophthalmology. Retina","volume":" ","pages":""},"PeriodicalIF":5.7,"publicationDate":"2026-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146093635","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Outcomes and Complications of Pars Plana Vitrectomy for Non-clearing Vitreous Hemorrhage without Tractional Elements. 玻璃体平面体切除术治疗无牵引性玻璃体出血的疗效及并发症。
IF 5.7 Q1 OPHTHALMOLOGY Pub Date : 2026-01-28 DOI: 10.1016/j.oret.2026.01.013
Justin C Muste, Matthew J Schulgit, Scott Perkins, Matthew Russell, Saam Mojtahed, Alex Yuan, Jonathan E Sears, Phoebe Lin, Aleksandra Rachitskaya, Sunil K Srivastava, Katherine E Talcott, Peter Kaiser, Justis P Ehlers, Danny A Mammo, Rishi P Singh, Daniel F Martin, Andrew P Schachat, Sumit Sharma

Objective: To evaluate outcomes and complications of pars plana vitrectomy (PPV) for nonclearing vitreous hemorrhage (NCVH) secondary to proliferative diabetic retinopathy (PDR) in the small gauge, anti-vascular endothelial growth factor (anti-VEGF) era.

Design: Retrospective consecutive case series of patients seen at Cole Eye Institute from 2014-2022 SUBJECTS: 306 eyes undergoing PPV for NCVH.

Methods: Key inclusion criteria included PPV specifically indicated for NCVH without tractional retinal detachment secondary to diagnosis of DR of any severity. Patients without preoperative documentation or 6 - and 12 -month follow-up visit were excluded. Pertinent data was then manually extracted from the electronic medical record. Descriptive statistics, univariate analysis, and propensity score matched regression analysis was performed.

Main outcome measures: Post-operative vitreous hemorrhage, repeated vitrectomy, and best corrected visual acuity (BCVA) at 12 months.

Results (short): Anti-VEGF was used in 199 of 306 eyes (65.0%) preoperatively with an average number of 1.67 ± 0.8 injections prior to vitrectomy. At 12 months after surgery 94.8% of patients gained or maintained their BCVA. Seventy-five of 306 (24.5%) of patients had a postoperative vitreous hemorrhage with only 48 of 306 (15.6%) requiring a second vitrectomy. Preoperative anti-VEGF injections was not associated with reduced odds of recurrent vitreous hemorrhage, improved 12-month BCVA, nor did it protect against repeat vitrectomy.

Conclusions: This study did not find evidence that preoperative anti-VEGF provided significant benefit in visual rehabilitation, reoperation or overall postoperative vitreous hemorrhage in NCVH cases without preoperative evidence of tractional detachment.

目的:探讨小尺度、抗血管内皮生长因子(anti-VEGF)时代增生性糖尿病视网膜病变(PDR)继发非清性玻璃体出血(NCVH)的玻璃体切割(PPV)治疗效果及并发症。设计:2014-2022年在科尔眼科研究所观察的连续病例系列回顾性研究对象:306只眼睛因NCVH接受PPV治疗。方法:主要纳入标准包括专门用于无牵引性视网膜脱离的NCVH的PPV,继发于任何严重程度的DR诊断。没有术前记录或6个月和12个月随访的患者被排除在外。然后人工从电子病历中提取相关数据。进行描述性统计、单变量分析和倾向评分匹配回归分析。主要观察指标:术后玻璃体出血,反复玻璃体切除术,12个月最佳矫正视力(BCVA)。结果(短):术前306只眼中199只(65.0%)使用抗vegf,玻璃体切割前平均注射次数1.67±0.8次。术后12个月,94.8%的患者BCVA增加或维持。306例患者中75例(24.5%)术后发生玻璃体出血,只有48例(15.6%)需要第二次玻璃体切除术。术前注射抗vegf与降低玻璃体出血复发几率、改善12个月BCVA无关,也不能防止重复玻璃体切除术。结论:本研究未发现证据表明术前抗vegf对术前无牵引性脱离的NCVH患者的视力康复、再手术或术后整体玻璃体出血有显著益处。
{"title":"Outcomes and Complications of Pars Plana Vitrectomy for Non-clearing Vitreous Hemorrhage without Tractional Elements.","authors":"Justin C Muste, Matthew J Schulgit, Scott Perkins, Matthew Russell, Saam Mojtahed, Alex Yuan, Jonathan E Sears, Phoebe Lin, Aleksandra Rachitskaya, Sunil K Srivastava, Katherine E Talcott, Peter Kaiser, Justis P Ehlers, Danny A Mammo, Rishi P Singh, Daniel F Martin, Andrew P Schachat, Sumit Sharma","doi":"10.1016/j.oret.2026.01.013","DOIUrl":"https://doi.org/10.1016/j.oret.2026.01.013","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate outcomes and complications of pars plana vitrectomy (PPV) for nonclearing vitreous hemorrhage (NCVH) secondary to proliferative diabetic retinopathy (PDR) in the small gauge, anti-vascular endothelial growth factor (anti-VEGF) era.</p><p><strong>Design: </strong>Retrospective consecutive case series of patients seen at Cole Eye Institute from 2014-2022 SUBJECTS: 306 eyes undergoing PPV for NCVH.</p><p><strong>Methods: </strong>Key inclusion criteria included PPV specifically indicated for NCVH without tractional retinal detachment secondary to diagnosis of DR of any severity. Patients without preoperative documentation or 6 - and 12 -month follow-up visit were excluded. Pertinent data was then manually extracted from the electronic medical record. Descriptive statistics, univariate analysis, and propensity score matched regression analysis was performed.</p><p><strong>Main outcome measures: </strong>Post-operative vitreous hemorrhage, repeated vitrectomy, and best corrected visual acuity (BCVA) at 12 months.</p><p><strong>Results (short): </strong>Anti-VEGF was used in 199 of 306 eyes (65.0%) preoperatively with an average number of 1.67 ± 0.8 injections prior to vitrectomy. At 12 months after surgery 94.8% of patients gained or maintained their BCVA. Seventy-five of 306 (24.5%) of patients had a postoperative vitreous hemorrhage with only 48 of 306 (15.6%) requiring a second vitrectomy. Preoperative anti-VEGF injections was not associated with reduced odds of recurrent vitreous hemorrhage, improved 12-month BCVA, nor did it protect against repeat vitrectomy.</p><p><strong>Conclusions: </strong>This study did not find evidence that preoperative anti-VEGF provided significant benefit in visual rehabilitation, reoperation or overall postoperative vitreous hemorrhage in NCVH cases without preoperative evidence of tractional detachment.</p>","PeriodicalId":19501,"journal":{"name":"Ophthalmology. Retina","volume":" ","pages":""},"PeriodicalIF":5.7,"publicationDate":"2026-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146093564","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Familial Chylomicronemia Syndrome-Lipemia Retinalis. 家族性乳糜微粒血症综合征-视网膜脂血症。
IF 5.7 Q1 OPHTHALMOLOGY Pub Date : 2026-01-23 DOI: 10.1016/j.oret.2025.12.022
Zu Jie, Ma Qingmin
{"title":"Familial Chylomicronemia Syndrome-Lipemia Retinalis.","authors":"Zu Jie, Ma Qingmin","doi":"10.1016/j.oret.2025.12.022","DOIUrl":"https://doi.org/10.1016/j.oret.2025.12.022","url":null,"abstract":"","PeriodicalId":19501,"journal":{"name":"Ophthalmology. Retina","volume":" ","pages":""},"PeriodicalIF":5.7,"publicationDate":"2026-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146041486","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
When the Retina Crystallizes: Retinal Oxalosis with Neovascular Sequelae. 视网膜结晶:视网膜草化症伴新血管后遗症。
IF 5.7 Q1 OPHTHALMOLOGY Pub Date : 2026-01-23 DOI: 10.1016/j.oret.2026.01.001
Shweta Jagdeesh, Anuradha Vadakke Kanakath, Palmeera D Souza
{"title":"When the Retina Crystallizes: Retinal Oxalosis with Neovascular Sequelae.","authors":"Shweta Jagdeesh, Anuradha Vadakke Kanakath, Palmeera D Souza","doi":"10.1016/j.oret.2026.01.001","DOIUrl":"https://doi.org/10.1016/j.oret.2026.01.001","url":null,"abstract":"","PeriodicalId":19501,"journal":{"name":"Ophthalmology. Retina","volume":" ","pages":""},"PeriodicalIF":5.7,"publicationDate":"2026-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146030427","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
Ophthalmology. Retina
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