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Adaptive optics retinal imaging in patients with usher syndrome. 厄舍综合征患者的自适应光学视网膜成像。
Pub Date : 2024-05-28 eCollection Date: 2024-01-01 DOI: 10.3389/fopht.2024.1349234
Melanie Kempf, Susanne Kohl, Krunoslav Stingl, Fadi Nasser, Katarina Stingl, Friederike C Kortuem

Purpose: To determine the structure of the cone photoreceptor mosaic in the macula in eyes with retinitis pigmentosa related to Usher syndrome using adaptive optics fundus (AO) imaging and to correlate these findings with those of the standard clinical diagnostics.

Methods: Ten patients with a genetically confirmed retinitis pigmentosa in Usher syndrome due to biallelic variants in MYO7A or USH2A were enrolled in the study. All patients underwent a complete ophthalmological examination including best corrected visual acuity (BCVA), spectral-domain optical coherence tomography (SD-OCT) with fundus autofluorescence photography (FAF), full-field (ffERG) and multifocal electroretinography (mfERG) and Adaptive Optics Flood Illuminated Ophthalmoscopy (AO, rtx1™, Imagine Eyes, Orsay, France). The cone density was assessed centrally and at each 0.5 degree horizontally and vertically from 1-4 degree of eccentricity.

Results: In the AO images, photoreceptor cell death was visualized as a disruption of the cone mosaic and low cone density. In the early stage of the disease, cones were still visible in the fovea, whereas outside the fovea a loss of cones was recognizable by blurry, dark patches. The blurry patches corresponded to the parafoveal hypofluorescent ring in the FAF images and the beginning loss of the IS/OS line and external limiting membrane in the SD-OCT images. FfERGs were non-recordable in 7 patients and reduced in 3. The mfERG was reduced in all patients and correlated significantly (p <0.001) with the cone density. The kinetic visual field area, measured with III4e and I4e, did not correlate with the cone density.

Conclusion: The structure of the photoreceptors in Usher syndrome patients were detectable by AO fundus imaging. The approach of using high-resolution technique to assess the photoreceptor structure complements the established clinical examinations and allows a more sensitive monitoring of early stages of retinitis pigmentosa in Usher syndrome.

目的:利用自适应光学眼底(AO)成像技术确定与乌谢尔综合征相关的视网膜色素变性患者黄斑中锥体感光器镶嵌的结构,并将这些结果与标准临床诊断结果进行对比:本研究共招募了十名经遗传学证实患有视网膜色素变性的乌谢尔综合征患者,这些患者都是因 MYO7A 或 USH2A 双重变异所致。所有患者都接受了全面的眼科检查,包括最佳矫正视力(BCVA)、光谱域光学相干断层扫描(SD-OCT)和眼底自动荧光摄影(FAF)、全视野(ffERG)和多焦点视网膜电图(mfERG)以及自适应光学泛光照明眼底镜(AO,rtx1™,Imagine Eyes,法国奥赛公司)。锥体密度在中心和偏心 1-4 度的水平和垂直方向各 0.5 度进行评估:结果:在 AO 图像中,感光细胞的死亡表现为视锥镶嵌的破坏和视锥密度的降低。在疾病的早期阶段,视锥在眼窝内仍然可见,而在眼窝外,视锥的消失则表现为模糊的黑斑。这些模糊斑块与 FAF 图像中的眼窝旁低荧光环以及 SD-OCT 图像中的 IS/OS 线和外缘膜开始丧失相对应。7 名患者的 FfERG 无法记录,3 名患者的 FfERG 减低:通过 AO 眼底成像可以检测到乌谢尔综合征患者感光器的结构。使用高分辨率技术评估光感受器结构的方法与既有的临床检查方法相辅相成,可以更灵敏地监测乌谢尔综合征视网膜色素变性的早期阶段。
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引用次数: 0
Utility of artificial intelligence in the diagnosis and management of keratoconus: a systematic review 人工智能在角膜病诊断和管理中的应用:系统综述
Pub Date : 2024-05-17 DOI: 10.3389/fopht.2024.1380701
Deniz Goodman, Angela Y. Zhu
The application of artificial intelligence (AI) systems in ophthalmology is rapidly expanding. Early detection and management of keratoconus is important for preventing disease progression and the need for corneal transplant. We review studies regarding the utility of AI in the diagnosis and management of keratoconus and other corneal ectasias.We conducted a systematic search for relevant original, English-language research studies in the PubMed, Web of Science, Embase, and Cochrane databases from inception to October 31, 2023, using a combination of the following keywords: artificial intelligence, deep learning, machine learning, keratoconus, and corneal ectasia. Case reports, literature reviews, conference proceedings, and editorials were excluded. We extracted the following data from each eligible study: type of AI, input used for training, output, ground truth or reference, dataset size, availability of algorithm/model, availability of dataset, and major study findings.Ninety-three original research studies were included in this review, with the date of publication ranging from 1994 to 2023. The majority of studies were regarding the use of AI in detecting keratoconus or subclinical keratoconus (n=61). Among studies regarding keratoconus diagnosis, the most common inputs were corneal topography, Scheimpflug-based corneal tomography, and anterior segment-optical coherence tomography. This review also summarized 16 original research studies regarding AI-based assessment of severity and clinical features, 7 studies regarding the prediction of disease progression, and 6 studies regarding the characterization of treatment response. There were only three studies regarding the use of AI in identifying susceptibility genes involved in the etiology and pathogenesis of keratoconus.Algorithms trained on Scheimpflug-based tomography seem promising tools for the early diagnosis of keratoconus that can be particularly applied in low-resource communities. Future studies could investigate the application of AI models trained on multimodal patient information for staging keratoconus severity and tracking disease progression.
人工智能(AI)系统在眼科领域的应用正在迅速扩大。早期发现和治疗角膜塑形镜对于防止疾病恶化和角膜移植的需要非常重要。我们对 PubMed、Web of Science、Embase 和 Cochrane 数据库中从开始到 2023 年 10 月 31 日的相关原始英文研究进行了系统检索,检索时使用了以下关键词:人工智能、深度学习、机器学习、角膜病和角膜异位症。病例报告、文献综述、会议论文集和社论均被排除在外。我们从每项符合条件的研究中提取了以下数据:人工智能的类型、用于训练的输入、输出、地面实况或参考、数据集大小、算法/模型的可用性、数据集的可用性以及主要研究结果。大多数研究涉及人工智能在检测角膜病或亚临床角膜病中的应用(n=61)。在有关角膜炎诊断的研究中,最常见的输入是角膜地形图、基于 Scheimpflug 的角膜断层扫描和前段光学相干断层扫描。本综述还总结了 16 项关于基于人工智能的严重程度和临床特征评估的原创性研究、7 项关于疾病进展预测的研究和 6 项关于治疗反应特征的研究。在基于 Scheimpflug 的层析成像上训练的算法似乎是角膜病早期诊断的有前途的工具,尤其适用于资源匮乏的社区。未来的研究可以探讨如何应用根据患者多模态信息训练的人工智能模型来分期角膜病的严重程度并跟踪疾病的进展。
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引用次数: 0
Editorial: Insights in pediatric ophthalmology and strabismus: 2022 社论:小儿眼科和斜视的启示:2022 年
Pub Date : 2024-05-15 DOI: 10.3389/fopht.2024.1382073
Dominique Bremond-Gignac
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引用次数: 0
Improved MRI methods to quantify retinal and choroidal blood flow applied to a model of glaucoma 应用于青光眼模型的视网膜和脉络膜血流量化磁共振成像改进方法
Pub Date : 2024-05-13 DOI: 10.3389/fopht.2024.1385495
Zhao Jiang, Diane Chernoff, Andre Galenchik-Chan, David Tomorri, Robert A. Honkanen, Timothy Q. Duong, E. Muir
Blood flow (BF) of the retinal and choroidal vasculatures can be quantitatively imaged using MRI. This study sought to improve methods of data acquisition and analysis for MRI of layer-specific retinal and choroidal BF and then applied this approach to detect reduced ocular BF in a well-established mouse model of glaucoma from both eyes.Quantitative BF magnetic resonance imaging (MRI) was performed on glaucomatous DBA/2J and normal C57BL/6J mice. Arterial spin labeling MRI was applied to image retinal and choroidal BF using custom-made dual eye coils that could image both eyes during the same scan. Statistics using data from a single eye or two eyes were compared. BF values were calculated using two approaches. The BF rate per quantity of tissue was calculated as commonly done, and the peak BF values of the retinal and choroidal vasculatures were taken. Additionally, the BF rate per retinal surface area was calculated using a new analysis approach to attempt to reduce partial volume and variability by integrating BF over the retinal and choroidal depths.Ocular BF of both eyes could be imaged using the dual coil setup without effecting scan time. Intraocular pressure was significantly elevated in DBA/2J mice compared to C57BL/6J mice (P<0.01). Both retinal and choroidal BF were significantly decreased in DBA/2J mice in comparison to the age-matched normal C57BL/6J mice across all measurements (P < 0.01). From simulations, the values from the integrated BF analysis method had less partial volume effect, and from in vivo scans, this analysis approach also improved power.The dual eye coil setup allows bilateral eye data acquisition, increasing the amount of data acquired without increasing acquisition times in vivo. The reduced ocular BF found using the improved acquisition and analysis approaches replicated the results of previous studies on DBA/2J mice. The ocular hypertensive stress-induced BF reduction found within these mice may represent changes associated with glaucomatous progression.
视网膜和脉络膜血管的血流(BF)可通过核磁共振成像进行定量成像。本研究试图改进视网膜和脉络膜特异层血流磁共振成像的数据采集和分析方法,然后应用这种方法检测一种成熟的青光眼小鼠模型中双眼眼部血流减少的情况。动脉自旋标记核磁共振成像应用于视网膜和脉络膜BF成像,使用的是定制的双眼线圈,可在同一次扫描中对双眼进行成像。使用单眼或双眼数据进行统计比较。采用两种方法计算 BF 值。按照通常的方法计算每量组织的 BF 率,并得出视网膜和脉络膜血管的峰值 BF 值。此外,还使用一种新的分析方法计算每视网膜表面积的 BF 率,试图通过整合视网膜和脉络膜深度的 BF 来减少部分体积和可变性。与 C57BL/6J 小鼠相比,DBA/2J 小鼠的眼压明显升高(P<0.01)。在所有测量中,与年龄匹配的正常 C57BL/6J 小鼠相比,DBA/2J 小鼠的视网膜和脉络膜 BF 都明显下降(P < 0.01)。从模拟结果来看,综合 BF 分析方法得出的数值具有较小的部分体积效应,而从体内扫描结果来看,这种分析方法也提高了功率。双眼线圈设置允许采集双眼数据,在不增加体内采集时间的情况下增加了采集的数据量。使用改进后的采集和分析方法发现,眼压降低了,这与之前对 DBA/2J 小鼠的研究结果相吻合。在这些小鼠体内发现的眼压应激引起的眼压降低可能代表了与青光眼进展相关的变化。
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引用次数: 0
Case report: Bilateral optic nerve sheath meningocele: clinical aspects 病例报告:双侧视神经鞘膜积液:临床方面
Pub Date : 2024-05-08 DOI: 10.3389/fopht.2024.1385485
Saray Catalán-Coronado, A. Parrado-Carrillo, Javier Nogués-Castell, Josep Rosinés-Fonoll, A. Camós-Carreras, Rafel Alcubierre, M. Carrión-Donderis, C. Bernal-Morales, Bernardo Sánchez-Dalmau
Optic nerve sheath meningocele is an enlargement of the sheath itself, consisting of a collection of cerebrospinal fluid along the perineural space. It should be considered primary if it is not associated with orbital–cerebral neoplasm or with cranio-orbital junction malformations. We report three cases of bilateral primary idiopathic optic nerve sheath meningocele, two of them with gradual vision loss. The first case presented a history of monocular blurred vision of the right eye and headache. It was initially treated with acetazolamide without any improvement, after which optic nerve sheath fenestration was required. The second case showed intermittent binocular diplopia with central 24-2 perimetry defects in the left eye. The third case was first presented as a subacute bilateral conjunctivitis with a suspected orbital pseudotumor. An incidental bilateral optic nerve sheath meningocele was found in the orbital imaging, being totally asymptomatic. In all the cases, orbital and cranial magnetic resonance with contrast and fat suppression was crucial in the diagnosis.
视神经鞘膜积液是视神经鞘本身的肿大,由沿神经周围间隙的脑脊液聚集组成。如果不伴有眶脑肿瘤或颅眶交界处畸形,则应视为原发性。我们报告了三例双侧原发性特发性视神经鞘膜积液,其中两例患者视力逐渐下降。第一个病例的病史是右眼单眼视力模糊和头痛。起初使用乙酰唑胺治疗,但没有任何改善,之后需要进行视神经鞘膜切开术。第二个病例表现为间歇性双眼复视,左眼有中心 24-2 光度计缺陷。第三个病例最初表现为亚急性双侧结膜炎,疑似眼眶假瘤。在眼眶造影中偶然发现双侧视神经鞘膜积液,但完全没有症状。在所有病例中,使用造影剂和脂肪抑制的眼眶和头颅磁共振对诊断至关重要。
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引用次数: 0
Mechanism and treatment of secondary glaucoma after corneal transplantation: a review 角膜移植后继发性青光眼的机制和治疗:综述
Pub Date : 2024-05-08 DOI: 10.3389/fopht.2024.1361704
Yumeng Lin, Qiaoyin Gou, Ping Yu, Zhengfang Wu, Liuzhi Zeng, Haoran Chen
Corneal transplantation is a common treatment for corneal diseases. Secondary glaucoma after corneal transplantation is the second leading cause of failure of keratoplasty. This article reviews the mechanism and treatment of secondary glaucoma after corneal transplantation.
角膜移植是一种常见的角膜疾病治疗方法。角膜移植术后继发性青光眼是角膜移植术失败的第二大原因。本文回顾了角膜移植术后继发性青光眼的发病机制和治疗方法。
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引用次数: 0
Editorial: Glial interactions in glaucoma 社论:青光眼中的神经胶质相互作用
Pub Date : 2024-05-06 DOI: 10.3389/fopht.2024.1393555
Youichi Shinozaki
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引用次数: 0
Corrigendum: Pilot report: objective quantification of trabecular meshwork pigmentation using densitometry and the NIDEK GS-1 gonioscope in glaucoma patients. 更正:试验报告:使用密度计和 NIDEK GS-1 光学显微镜客观量化青光眼患者小梁网色素沉着。
Pub Date : 2024-04-17 eCollection Date: 2024-01-01 DOI: 10.3389/fopht.2024.1382567
Daniel Laroche, Aaron Brown, Jose Sinon, Alexander Martin, Chester Ng, Sohail Sakkari

[This corrects the article DOI: 10.3389/fopht.2023.1322178.].

[此处更正了文章 DOI:10.3389/fopht.2023.1322178]。
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引用次数: 0
Defining reference values of arterioles in healthy individuals for studies with adaptive optics imaging 为自适应光学成像研究确定健康人动脉血管的参考值
Pub Date : 2024-03-22 DOI: 10.3389/fopht.2024.1348900
F. Kortuem, David A Merle, Milda Reith, L. Kuehlewein, Ronja Jung, Saskia Holocher, K. Stingl, Katarina Stingl, Melanie Kempf
To investigate age-dependent wall to lumen ratio (WLR) reference values for healthy individuals in adaptive optics imaging (AO). WLR serves as an objective, dimensionless parameter for the evaluation of structural changes in vessels caused by conditions like arterial hypertension, diabetes or vascular stenosis.50 right eyes of healthy individuals were examined by adaptive optics imaging. The central big arterioles and smaller arterial branches at least one disc diameter away from the optic disc, approximately above or below the macula were measured by the manufacturer’s software. The wall-lumen-ratio (WLR), the wall cross-sectional area (WCSA) and lumen diameter (LD) were assessed. Subsequent data analysis was performed with a focus on variables including age, gender and blood pressure.Normative values for WLR, WCSA and LD in 5 different age groups could be established. However, no significant differences between the age groups were found. Intra-subject comparisons revealed significantly higher WLRs on peripheral branches when compared to central arterioles. WLR showed in this normotensive cohort no relevant correlation with the systolic, diastolic and mean blood pressure. Gender and intraocular pressure had no influence on the vascular parameters.AO is capable of examining vascular alterations in arterioles at an almost microscopic level. Age did not seem to alter WLR, normotensive blood pressure parameters showed also no significant impact. AO-based vessel analysis may provide clinically useful biomarkers for cardiovascular health and should be tested in future studies.
研究健康人在自适应光学成像(AO)中与年龄有关的管壁与管腔比(WLR)参考值。WLR 是一个客观、无量纲的参数,可用于评估动脉高血压、糖尿病或血管狭窄等情况引起的血管结构变化。50 健康人的右眼通过自适应光学成像进行检查。50 健康人的右眼通过自适应光学成像进行检查,使用制造商提供的软件测量中央大动脉和距离视盘至少一个视盘直径的较小动脉分支,大约在黄斑上方或下方。评估了管壁-管腔比率(WLR)、管壁横截面积(WCSA)和管腔直径(LD)。随后进行了数据分析,重点分析了年龄、性别和血压等变量。然而,各年龄组之间没有发现明显差异。受试者内部比较显示,与中心动脉血管相比,外周分支的 WLR 明显更高。在正常血压人群中,WLR 与收缩压、舒张压和平均血压没有相关性。性别和眼压对血管参数没有影响。年龄似乎不会改变 WLR,正常血压参数也没有明显影响。基于光学视网膜的血管分析可为心血管健康提供临床有用的生物标记,应在未来的研究中进行测试。
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引用次数: 0
G protein-coupled estrogen receptor expression in postnatal developing mouse retina 小鼠出生后视网膜发育过程中 G 蛋白偶联雌激素受体的表达
Pub Date : 2024-03-15 DOI: 10.3389/fopht.2024.1331298
Wendy L. Piñon-Teal, Judith Mosinger Ogilvie
Estrogen has emerged as a multifaceted signaling molecule in the retina, playing an important role in neural development and providing neuroprotection in adults. It interacts with two receptor types: classical estrogen receptors (ERs) alpha and beta, and G protein-coupled estrogen receptor (Gper). Gper differs from classical ERs in structure, localization, and signaling. Here we provide the first report of the temporal and spatial properties of Gper transcript and protein expression in the developing and mature mouse retina.We applied qRT-PCR to determine Gper transcript expression in wild type mouse retina from P0-P21. Immunohistochemistry and Western blot were used to determine Gper protein expression and localization at the same time points.Gper expression showed a 6-fold increase during postnatal development, peaking at P14. Relative total Gper expression exhibited a significant decrease during retinal development, although variations emerged in the timing of changes among different forms of the protein. Gper immunoreactivity was seen in retinal ganglion cells (RGCs) throughout development and also in somas in the position of horizontal cells at early time points. Immunoreactivity was observed in the cytoplasm and Golgi at all time points, in the nucleus at early time points, and in RGC axons as the retina matured.In conclusion, our study illuminates the spatial and temporal expression patterns of Gper in the developing mouse retina and provides a vital foundation for further investigations into the role of Gper in retinal development and degeneration.
雌激素已成为视网膜中的一种多层面信号分子,在神经发育和成人神经保护中发挥着重要作用。它与两种类型的受体相互作用:经典的雌激素受体(ER)α 和β,以及 G 蛋白偶联雌激素受体(Gper)。Gper 在结构、定位和信号传导方面与传统雌激素受体不同。我们应用 qRT-PCR 技术测定了 P0-P21 期野生型小鼠视网膜中 Gper 转录本的表达。在出生后的发育过程中,Gper的表达量增加了6倍,在P14达到高峰。Gper的相对总表达量在视网膜发育过程中出现了显著下降,但不同形式的蛋白质在变化时间上出现了差异。Gper免疫反应可见于视网膜神经节细胞(RGC)的整个发育过程,也可见于早期水平细胞位置的体节。总之,我们的研究阐明了Gper在小鼠视网膜发育过程中的空间和时间表达模式,为进一步研究Gper在视网膜发育和退化过程中的作用奠定了重要基础。
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引用次数: 0
期刊
Frontiers in ophthalmology
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