Optical coherence tomography angiography features in Waldenström macroglobulinemia patients without Hyperviscosity syndrome: A pilot prospective study.

PURPOSE: To evaluate the retinal vessel density (VD) with optical coherence tomography angiography (OCTA) in asymptomatic patients affected by Waldenström macroglobulinemia (WM) without hyperviscosity syndrome (HVS) and to highlight the presence of microvascular damage in theese clinically asymptomatic WD patients. DESIGN: Prospective study. METHODS: A total of 43 eyes from 43 WM patients (24 females, 19 males, mean age 55.1 ± 13.6 years) were enrolled from January 2023 to December 2023 in the Eye Clinic of the University of Naples Federico II. Along with WM patients, 40 healthy subjects (HS) (20 females, 20 males, mean age 52.3 ± 15.6 years) with a normal ophthalmic examination and no history of intraocular surgery or retinal pathologic features were included as control group All patients and controls underwent OCTA RESULTS: The two groups were not significantly different for age and sex Visual acuity examination showed no statistically significant difference in BCVA between controls and patients Compared to HS, WD patients showed lower VD values in the SCP in the whole image (47.95 ± 5.17% vs. 52.99 ± 2.52 %; p < 0.001), as well as in the parafovea (53.01 ± 6.69% vs. 55.30 ± 2.61 %; p = 0.002), and fovea (21.38 ± 9.01% vs. 30.31 ± 5.84 %; p < 0.0001). On the other hand, in the DCP VD values were significantly higher in patients compared to controls in the whole image (55.82 ± 8.07% vs. 50.83 ± 5.46 %; p = 0.005), as well as in the parafovea (56.76 ± 6.26% vs. 52.59 ± 5.46 %; p = 0.0001), and fovea (38.75 ± 8.59% vs. 33.43 ± 8.68 %; p < 0.0001). CONCLUSION: The finding that OCTA confirmed the presence of widespread microvascular damage in WD patients clinically silent. Thus, OCTA is a safe rapid imaging technique that could represent a valid biomarker of systemic vascular dysfunction.

Advanced OCTA imaging segmentation: Unsupervised, non-linear retinal vessel detection using modified self-organizing maps and joint MGRF modeling.

BACKGROUND AND OBJECTIVE: This paper proposes a fully automated and unsupervised stochastic segmentation approach using two-level joint Markov-Gibbs Random Field (MGRF) to detect the vascular system from retinal Optical Coherence Tomography Angiography (OCTA) images, which is a critical step in developing Computer-Aided Diagnosis (CAD) systems for detecting retinal diseases. METHODS: Using a new probabilistic model based on a Linear Combination of Discrete Gaussian (LCDG), the first level models the appearance of OCTA images and their spatially smoothed images. The parameters of the LCDG model are estimated using a modified Expectation Maximization (EM) algorithm. The second level models the maps of OCTA images, including the vascular system and other retina tissues, using MGRF with analytically estimated parameters from the input images. The proposed segmentation approach employs modified self-organizing maps as a MAP-based optimizer maximizing the joint likelihood and handles the Joint MGRF model in a new, unsupervised way. This approach deviates from traditional stochastic optimization approaches and leverages non-linear optimization to achieve more accurate segmentation results. RESULTS: The proposed segmentation framework is evaluated quantitatively on a dataset of 204 subjects. Achieving 0.92 ± 0.03 Dice similarity coefficient, 0.69 ± 0.25 95-percentile bidirectional Hausdorff distance, and 0.93 ± 0.03 accuracy, confirms the superior performance of the proposed approach. CONCLUSIONS: The conclusions drawn from the study highlight the superior performance of the proposed unsupervised and fully automated segmentation approach in detecting the vascular system from OCTA images. This approach not only deviates from traditional methods but also achieves more accurate segmentation results, demonstrating its potential in aiding the development of CAD systems for detecting retinal diseases.

OCTA-based research on changes of retinal microcirculation in digestive tract malignancy.

PURPOSE: This cross-sectional study measured retinal vessel density (VD) in patients with digestive tract malignancy by optical coherence tomography angiography (OCTA), and compared them with healthy controls to explore the retinal microcirculation changes in patients with digestive tract malignancy. METHODS: 106 eligible participants were divided into three groups: gastric cancer (GC) group (36 individuals), colorectal cancer (CRC) group (34 individuals), and healthy control group (36 individuals). Angio 6*6 512*512 R4 and ONH Angio 6*6 512*512 R4 modes were performed to collect retinal vessel density data centered on fovea and papillary, respectively. The retina was automatically segmented into different layers (superficial vascular plexus (SVP), the inner retinal layer, radial peripapillary capillary plexus (RPCP), deep vascular plexus (DVP)) and areas to analyze. RESULTS: At the optic nerve head (ONH) region, the VD of the inner retinal layer increased in both GC and CRC groups in all quadrants and areas. In the papillary area, VD in the inner retinal layer, SVP, and RPCP increased in the GC and CRC groups. In the parapapillary area, VD in the inner retinal layer increased in the GC and the CRC groups. Significant increase in the global VD were found in the GC group of the RPCP and SVP. Regarding the macular region, no statistical differences were observed in each layer. CONCLUSIONS: The study suggested that retinal vessel density changed in patients with digestive tract malignancy, especially in the inner retinal layer of the ONH region, revealing the potential relevance of the relation between gastrointestinal cancer and retinal microcirculation.

Neurovascular retinal impairment in early-treated adults with phenylketonuria.

Purpose: To compare radial peripapillary capillary (RPC) vascular plexus parameters and peripapillary retinal nerve fiber layer (pRNFL) thickness between Early-Treated Adults with Phenylketonuria (ETPKU) and controls. Methods: This observational study was a monocentric, case control study including 36 eyes of 36 participants. Among these, 18 were early-treated PKU (ETPKU) and 18 were controls. A SD-OCTA (XR Avanti AngioVue OCTA; Optovue Inc., Fremont, CA) was employed to assess the OCT and OCTA parameters of all the participants. The main outcome measures were the RPC vessels density (VD) %, and the pRNFL thickness. Results: The average pRNFL thickness was significantly reduced in ETPKU (110.78 ± 12.48 µm) compared to controls (113.22 ± 13.95 µm), p = 0.046. The mean VD% of the small vessels of the RPC plexus was 52.31 ± 2.2 in ETPKU and 50.71 ± 3.2 in controls (p = 0.049), while the VD% of all the radial peripapillary capillary plexus (RPCP) was 58.5 ± 2.2 in ETPKU and 55.08 ± 3.4 in controls (p < 0.001). By contrast, there were no differences in age, sex, and IOP between the two groups. Conclusion: Through structural OCT and OCTA, we observed thinning of the nerve fibers accompanied by an increase in perfusion of the RPC plexus. Thus, our conclusions suggest that OCTA may serve as a noninvasive method to identify novel retinal biomarkers in ETPKU.

Triaceto-nitrile-(1,4,7-trimethyl-1,4,7-tri-aza-cyclonona-ne)cobalt(II) bis-(tetra-phenyl-borate).

The title cobalt(II) complex, [Co(C2H3N)3(C9H21N3)](C24H20B)2 or [(tacn)Co(NCMe)3][BPh4]2, has been characterized by single-crystal X-ray diffraction. It incorporates the well-known macrocyclic tacn (1,4,7-trimethyl-1,4,7-tri-aza-cyclo-nona-ne) ligand, which is coordinated facially to the metal center. The complex crystallizes in space group P21/c with Z = 4. The divalent cobalt ion exhibits a six-coordinate octa-hedral geometry by one tacn and three aceto-nitrile ligands. Two non-coordinating tetra-phenyl-borate (BPh4 -) anions are also present.

Multimodal phenotyping of foveal hypoplasia in albinism and albino-like conditions: a pediatric case series with adaptive optics insights.

Aim of the present study is to evaluate the relationship between genetic and phenotypic data in a series of patients affected by grade I and II of foveal hypoplasia with stable fixation and good visual acuity using multimodal imaging techniques. All patients underwent complete clinical and instrumental assessment including structural Optical Coherence Tomography (OCT), OCT Angiography and Adaptive Optics (AO) imaging. Central macular thickness (CMT), inner nuclear layer (INL), vessel density in superficial capillary plexus were the main variables evaluated with OCT technology. Cone density, cone spacing, cone regularity, cone dispersion and angular density were the parameters evaluated with AO. Genetic evaluation and trio exome sequencing were performed in all affected individuals. Eight patients (3 males and 5 females) with a mean age of 12.62 years (range 8-18) were enrolled. The mean best corrected visual acuity (BCVA) was 0.18 ± 0.13 logMAR, mean CMT was 291.9 ± 16.6 µm and INL was 26.2 ± 4.6 µm. The absence of a foveal avascular zone (FAZ) was documented by examination of OCT-A in seven patients in the superficial capillary plexus. However, there was a partial FAZ in the deep plexus in patients P5 and P8. Of note, all the patients presented with major retinal vessels clearly crossing the foveal center. All individuals exhibited a grade I or II of foveal hypoplasia. In 5 patients molecular analyses showed an extremely mild form of albinism caused by compound heterozygosity of a TYR pathogenic variant and the hypomorphic p.[Ser192Tyr;Arg402Gln] haplotype. One patient had Waardenburg syndrome type 2A caused by a de novo variant in MITF. Two patients had inconclusive molecular analyses. All the patients displayed abnormalities on OCT-A. Photoreceptor count did not differ from normal subjects according to the current literature, but qualitative analysis of AO imaging showed distinctive features likely related to an abnormal pigment distribution in this subset of individuals. In patients with foveal hypoplasia, genetic and multimodal imaging data, including AO findings, can help understand the physiopathology of the foveal hypoplasia phenotype. This study confirms that cone density and visual function can both be preserved despite the absence of a pit.

Retinal thickness and microvascular alterations observed by optical coherence tomography in antineutrophil cytoplasmic antibody-associated vasculitis: a cross-sectional study.

Background: As an autoimmune disease, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) often affects multiple organs, including the ocular system. This study aims to investigate differences in retinal thickness (RT) and retinal superficial vascular density (SVD) between patients with AAV and healthy controls (HCs) using optical coherence tomography angiography (OCTA). Currently, these differences are not clear. Methods: A total of 16 AAV individuals (32 eyes) and 16 HCs (32 eyes) were recruited to this cross-sectional study conducted in the First Affiliated Hospital of Nanchang University from June 2023 to September 2023. The study protocol conformed with the tenets of the Declaration of Helsinki (as revised in 2013). Each image observed by OCTA was divided into 9 regions using the Early Treatment Diabetic Retinopathy Study (ETDRS) subzones as a guide. Results: In the full layer, the RT of AAV patients was found to be significantly reduced in the inner superior (IS, P<0.001), outer superior (OS, P=0.003), inner temporal (IT, P=0.003), and outer temporal (OT, P<0.001) regions; inner RT was significantly lower in the IS (P=0.006), OS (P<0.001), inner nasal (IN, P=0.005), outer nasal (ON, P<0.001), and center (C, P=0.01) regions than that in HCs. Outer RT of AAV patients showed a reduction in the IS (P<0.001), as well as IT (P=0.008), and OT (P<0.001) regions. No statistically significant differences were seen in the different subregions in other different layers (P>0.05). Only the inner inferior (II) and outer inferior (OI) regions of SVD in AAV patients did not differ significantly from controls. All other regions showed a reduction in SVD. The details are as follows: IS (P<0.001), OS (P<0.001), IT (P=0.005), OT (P<0.001), IN (P<0.001), ON (P<0.001), and C (P=0.003). According to receiver operating characteristic (ROC) curve analysis, the full IS region [area under the curve (AUC): 0.8892, 95% confidence interval (CI): 0.8041-0.9742, P<0.001] had the highest diagnostic value for AAV-induced reduction in RT. The IS (AUC: 0.9121, 95% CI: 0.8322-0.9920, P<0.001) region was also the most sensitive to changes in SVD of AAV individuals. In addition, we found that SVD in the IN region (r=-0.4224, 95% CI: -0.6779 to -0.0757, P=0.02) as well as mean visual acuity (r=-0.3922, 95% CI: -0.6579 to -0.0397, P=0.03) of AAV patients were negatively correlated with disease duration. However, we did not find an association between SVD and RT in this study. Conclusions: The findings from OCTA indicated a reduction in RT and SVD among patients with AAV. OCTA allows for the evaluation of AAV-related ocular lesions and holds promise for monitoring of disease progression through regular evaluations.

Quantitative optical coherence tomography angiography assessments in the fellow eyes of those with central serous chorioretinopathy.

BACKGROUND: To analyze the vessel density (VD) of the retina and choriocapillaris (CC) layer and the structure of the foveal avascular zone (FAZ) in the fellow eyes of central serous chorioretinopathy (CSC) patients by using optical coherence tomography angiography (OCTA). METHODS: This was a case-control study. Unilateral CSC patients and age-matched healthy subjects were recruited from the Affiliated Eye Hospital of Wenzhou Medical University between July 2016 and July 2021. All eyes were divided into three groups: acute CSC (aCSC), chronic CSC (cCSC), and healthy controls. Both aCSC and cCSC were again divided into two subgroups: the affected eyes and the fellow eyes. In this study, all parameters of VD and FAZ were measured by self-software of OCTA. RESULTS: A total of 231 eyes of 137 subjects were included, with 47 aCSC patients, 47 cCSC patients, and 43 healthy controls. In the fellow eyes of CSC, the retinal VD was significantly lower (all P < 0.05), and the FAZ was significantly larger (all P < 0.05) in the cCSC group than in healthy controls, while no difference was detected in the CC layer. There was no significant difference between the aCSC group and healthy controls in all OCTA parameters. In the affected eyes of CSC, the superficial retinal vessel density (SRVD) was significantly higher (all P < 0.05) in healthy controls than in the aCSC and cCSC groups, while the deep retinal vessel density (DRVD) was significantly lower (all P < 0.05) and the FAZ was larger (all P < 0.05) in the cCSC group than in the aCSC group and healthy controls. A liner regression equation was established: Y (BCVA, best corrected visual acuity) = 3.692-0.036✱X1 (DRVD-Fovea)-0.031✱X2 (FD-300, vessel density around the 300 µm width of the FAZ), R2 = 0.427. CONCLUSION: Based on OCTA measurements, this study revealed that the retinal microvascular network was impaired even in the fellow eyes of those with cCSC, which should arouse attention to the observation of unilateral CSC.

Intralesional Vessel Diameter Measured by Optical Coherence Tomography Angiography Could Improve the Differential Diagnosis of Small Melanocytic Choroidal Lesions.

In this study, we aimed to identify the features of indeterminate choroidal melanocytic lesions visualized on optical coherence tomography angiography (OCTA) and to identify the predictors of growth. We retrospectively evaluated 86 patients with indeterminate lesions treated at our centre from 2016 to 2021. Clinical management involved active surveillance followed by brachytherapy if growth was detected. The lesions were classified into two groups according to whether they grew (small melanomas) or remained stable (choroidal nevi). Growth was detected in 19 (22.1%) lesions. All patients underwent OCTA at baseline. These images were compared to identify the possible predictors of growth. Significant between-group differences were observed in thickness (p = 0.00), greatest basal diameter (p = 0.00), number of risk factors (p = 0.00), symptoms (p = 0.001; relative risk [RR]: 4.3), orange pigment (p = 0.00; RR: 6.02), and ultrasonographic hollowness (Kappa sign); p = 0.000; RR: 5.3). The melanomas had significantly more vessels with a diameter ≥ 76.3 µm (p = 0.02; RR: 2.46). The time to growth in these lesions was significantly shorter (p = 0.05) than in lesions with smaller vessels. These findings show that vessel diameter quantified by OCTA can help differentiate between choroidal nevi and small melanomas, when considered together with clinical risk factors.

Multimodal imaging in diabetic retinopathy and macular edema: An update about biomarkers.

Diabetic macular edema (DME), defined as retinal thickening near, or involving the fovea caused by fluid accumulation in the retina, can lead to vision impairment and blindness in patients with diabetes. Current knowledge of retina anatomy and function and DME pathophysiology has taken great advantage of the availability of several techniques for visualizing the retina. Combining these techniques in a multimodal imaging approach to DME is recommended to improve diagnosis and to guide treatment decisions. We review the recent literature about the following retinal imaging technologies: optical coherence tomography (OCT), OCT angiography (OCTA), wide-field and ultrawide-field techniques applied to fundus photography, fluorescein angiography, and OCTA. The emphasis will be on characteristic DME features identified by these imaging technologies and their potential or established role as diagnostic, prognostic, or predictive biomarkers. The role of artificial intelligence in the assessment and interpretation of retina images is also discussed.

Diagnostic ability of the combination of retinal microvasculature evaluation and static automated perimetry for early primary open-angle glaucoma.

PURPOSE: To investigate the diagnostic ability of retinal superficial vasculature evaluation by optic coherence tomography angiography (OCTA) combined with visual field (VF) testing for early primary open-angle glaucoma (POAG). PATIENTS AND METHODS: In this cross-sectional study, 84 participants were included, including 11 in the ocular hypertension (OHT) group, 11 in the preperimetric POAG (pre-POAG) group, 29 in the early POAG group and 33 in the control group. All participants underwent 6 × 6 mm2 scans of macula and optic nerved head by optic coherence tomography (OCT) and OCTA, along with white-on-white and blue-on-yellow VF testing by static automated perimetry. The ability of diagnosing early glaucoma by either various examinations separately or combination of examinations in both terms of function and structure was studied using the receiver operating characteristic (ROC) curve and the area under the curve (AUC). RESULTS: The superficial retinal vessel densities (VD) in peri-nasal, para-temporal, peri-temporal and peri-inferior regions around the macula, as well as vessel area densities (VAD) in all peripapillary regions, were significantly different among the four groups, with lower VD or VAD in the early POAG patients compared to the normal individuals. The diagnostic ability of peripapillary superficial retinal VAD alone or VF testing alone was limited for early POAG only. However, the combination of these two was more effective in distinguishing normal individuals from OHT subjects or pre-POAG patients without VF defects, with better performance than the combination of peripapillary retinal nerve fiber layer (RNFL) thickness and VF indicators. CONCLUSIONS: Peripapillary retinal vessel densities were generally lower in early POAG patients compared to normal individuals. The combination of peripapillary superficial retinal VAD by OCTA with white-on-white VF testing improved the ability to distinguish POAG patients at early stage without function impairment, which may help in providing reference and guidance for the following-up and treatment of suspected POAG patients.

Longitudinal evaluation of macular vascular density alterations in unilateral amblyopic children undergoing therapy: An optical coherence tomographic angiography study.

Importance: Understanding the pathophysiology of the macula in amblyopic eyes is an active research area. Objective: To correlate macular retinal vascular density changes with best-corrected visual acuity changes following occlusion therapy for amblyopia in children. Design: A prospective cohort study of children visiting the Pediatric Ophthalmology Division of our institution between January 2020 and January 2022 was conducted. Setting: A specialist eye hospital in Saudi Arabia. Participants: Thirty children with unilateral amblyopia. Exposure: Occlusion therapy for amblyopia.Main Outcome and Measures: Best corrected visual acuity (logMAR) before and at each of the four optical coherence tomographic angiographies was compared in amblyopic and fellow eyes. The effect of pretreatment determinants on the correlation between best-corrected visual acuity and retinal vascular density changes was reviewed. Results: In this cohort of 30 amblyopic and 30 fellow eyes from 30 children (mean age 8.7 ± 1.4 years; male: female 18:12. The best-corrected visual acuity improved from a median of 0.6 (interquartile range 0.5; 1.1) pretreatment to a median of 0.4 (interquartile range 0.2; 0.6) posttreatment in amblyopic eyes, and from a median of 0.1 to 0.05 in the fellow eyes. The total percentage change in retinal vascular density in the amblyopic eye was significantly higher than that in the fellow eye (Z = -1.92, P = 0.05). The change in best-corrected visual acuity in the amblyopic eye after a median of 98 months (interquartile range, 69-126 months) of intervention was significantly correlated with the refraction-adjusted change in retinal vascular density (B = -0.03, 95 % confidence interval -0.04, -0.02, P < 0.001) and was influenced by strabismus (B = -0.46, 95 % confidence interval -0.59, -0.34, P < 0.001), type of amblyopia (B = 0.24, 95 % confidence interval 0.12, 0.36, P < 0.001), duration of occlusion (B = -0.43, 95 % confidence interval -0.65, -0.22, P < 0.001), and occlusion compliance (B = 0.24, 95 % confidence interval 0.11, 0.36, P < 0.001). Conclusions: and Relevance: The RVD in amblyopic eyes in the first six months of therapy was significantly lower than that in fellow eyes, but not in subsequent assessments.

Octa-kis(di-butyl-ammonium) deca-molybdate(VI).

In the title salt, (C8H20N)8[Mo10O34], the [Mo10O34]8- polyanion is located about an inversion centre and can be considered as a ß-type octa-molybdate anion to which two additional MoO4 tetra-hedra are linked via common corners. The [Mo10O34]8- polyanions are packed in rows extending parallel to [001] and are connected to the di-butyl-ammonium counter-cations through N-H⋯O hydrogen-bonding inter-actions.

Associations of retinal microvascular alterations with diabetes mellitus: an OCTA-based cross-sectional study.

BACKGROUND: Diabetes, a health crisis afflicting millions worldwide, is increasing rapidly in prevalence. The microvascular complications triggered by diabetes have emerged as the principal cause of renal disease and blindness. The retinal microvascular network may be sensitive to early systemic vascular structural and functional changes. Therefore, this research endeavored to discern the systemic determinants influencing the retinal microvascular network in patients with and without diabetes. METHODS: The Kailuan Eye Study is a cross-sectional study based on the community-based cohort Kailuan Study. Participants underwent optical coherence tomography angiography (OCTA) (Zeiss Cirrus 5000; Carl Zeiss Meditec) and comprehensive systemic examination. Metrics such as perfusion density (PD), vascular density (VD), foveal avascular zone (FAZ) parameters of the superficial capillary plexus (SCP) in the macula were assessed. RESULTS: This study included 860 eligible participants (average age = 62.75 ± 6.52 years; 21.9% female), of which 449 were diabetics. People with diabetes had diminished PD and VD in the entire macular and parafoveal regions compared to people without diabetes. Reduced PD in the whole macular region was correlated with higher fasting plasma glucose (FPG, mmol/L) concentration (Beta = -0.19, 95% CI = -0.42 to -0.36, P < 0.001), longer axial length (AL, mm) (Beta = -0.13, 95%CI = -0.48 to -0.25, P = 0.002), and elevated heart rate (Beta = -0.10, 95%CI = -0.14 to -0.19, P = 0.014), after adjusting for younger age (Beta = -0.18, 95%CI = -0.24 to -0.35, P < 0.001), consistent with VD of the whole macular region. A higher FPG level was significantly correlated with lower SCP density of both PD and VD in the macular and parafoveal region (P < 0.05 for all), as well as increased systolic blood pressure and low-density lipoprotein cholesterol concentration (P < 0.01 for all). CONCLUSIONS: In this large-sample cross-sectional study, OCTA evaluation revealed that high prevalence of diabetes and elevated FPG levels were correlated with reduced retinal VD and PD. Hypertension and hyperlipidemia are important risk factors for the development of atherosclerotic cardiovascular disease but have no significant effect on retinal microvascular abnormalities.

Wide-field swept-source optical coherence tomography angiography (SS-OCTA) in the assessment of retinal vessel density and thickness in 4-to 16-year-old myopic children.

PURPOSE: To analyze the characteristics of macular retinal vessel density and thickness in children with myopia. METHODS: A cross-sectional study was conducted. A total of 228 children aged 4-16 years who visited the Ineye Hospital of Chengdu University of Traditional Chinese Medicine from September 2022 to November 2023 were included. Those with -0.5D < spherical equivalent (SE) < +2.0D were included in the non-myopia group (150 eyes), those with -3.0D < SE ≤ -0.5D were included in the low myopia group (246 eyes), and those with SE ≤ -3.0D were included in the moderate-to-high myopia group (60 eyes). All subjects underwent cycloplegic refraction, IOLmaster500, and Wide-field SS-OCTA (to exclude some peripheral retinal degeneration). Multiple linear regression analysis was used to analyze the correlation between macular ETDRS subfield of full retinal thickness (FRT), outer, inner retinal thickness (ORT, IRT), retinal vessel density (VD), deep and superficial retinal vessel density (DVD, SVD), and SE, axial length (AL). RESULTS: There were statistically significant differences (P < 0.05) in FRT in the central fovea (1 mm diameter)and perifovea (Diameter 3 to 6 mm) among the non-myopia group, low myopia group, and moderate-to-high myopia group. The three groups also showed statistically significant differences (P < 0.05) in VD in the central fovea and parafovea with a diameter of 1 to 3 mm (except the lower part). In multiple linear regression analysis adjusted for gender and age, SE and AL were found to be correlated with FRT in all ETDRS regions (except the central fovea) (P < 0.01), and SE and AL were correlated with IRT in the central fovea and perifovea, respectively (ß range -2.302 to 1.652; P < 0.05). SE and AL were also correlated with ORT in the parafovea and perifovea, respectively (ß range -4.371 to -2.344; P < 0.05). AL was negatively correlated with VD in the central fovea and parafovea (except the inferior region) (P < 0.05), as well as with DVD in all ETDRS regions (ß range -1.314 to -1.031; P < 0.05). AL was only negatively correlated with SVD in the parafoveal nasal region (ß = -0.633, P < 0.05). Additionally, the correlation between AL and DVD, ORT was higher than that with SVD, IRT. CONCLUSION: The more severe the myopia, the longer the AL, the thinner the FRT in the perifovea, and the lower the VD in both the fovea and parafovea in children. In addition, DVD and ORT were more significantly correlated with AL, suggesting that they may be more closely related to the growth of AL.

Genetic Linkage between CAPN5 and TYR Variants in the Context of Albinism and Autosomal Dominant Neovascular Inflammatory Vitreoretinopathy Absence: A Case Report.

We present a case involving a patient whose clinical phenotype aligns with oculocutaneous albinism (OCA), yet exhibits a complex genotype primarily characterized by variants of unknown significance (VUS). An 11-year-old boy manifested iris hypopigmentation and translucency, pronounced photophobia, diminished visual acuity and stereopsis, nystagmus, reduced pigmentation of the retina, and foveal hypoplasia. Genetic testing was performed. A heterozygous missense VUS CAPN5 c.230A>G, p.(Gln77Arg), a heterozygous missense VUS TYR c.1307G>C, p.(Gly436Ala), and a heterozygous missense variant TYR c.1205G>A, p.(Arg402Gln) which was classified as a risk factor, were identified. We hypothesized that the TYR c.1307G>C, p.(Gly436Ala) variant is in genetic disequilibrium with the TYR c.1205G>A, p.(Arg402Gln) variant leading to deficient expression of melanogenic enzymes in retinal cells, resulting in the manifestation of mild OCA. Additionally, this study represents the case where we did not detect chiasmal misrouting in visual evoked potentials, nor did we observe a shift in the distribution of ganglion cell thickness from a temporal to a central position. Moreover, our patient's case supports the probable benign nature of the CAPN5 c.230A>G, p.(Gln77Arg) variant.

Quantitative Multimodal Imaging Characterization of Intraretinal Cysts versus Degenerative Pseudocysts in Neovascular Age-Related Macular Degeneration.

OBJECTIVE: To differentiate intraretinal fluid (IRF) cysts from degenerative pseudocysts in neovascular age-related macular degeneration (AMD) by quantitative multimodal imaging. DESIGN: Observational, cross-sectional. PARTICIPANTS: Patients affected by macular neovascularization secondary to AMD. METHODS: All patients were analyzed by OCT, OCT angiography (OCTA), and dense automatic real-time (ART) OCTA. New-onset cysts were considered IRF, whereas those cysts that were found to be persistent for at least 3 months were categorized as degenerative pseudocysts. Intraretinal cysts were automatically segmented to calculate cyst circularity. Peri-cyst space was quantitatively analyzed to assess the presence of perfusion signal and hyperreflective foci (HF). MAIN OUTCOME MEASURES: Best-corrected visual acuity, cyst circularity, peri-cyst perfusion, peri-cyst HF, fibrosis, and outer retinal atrophy. RESULTS: We analyzed 387 cysts collected from 35 eyes of 35 patients with neovascular AMD (14 men; mean age, 80 ± 5 years). We classified 302 IRF cysts and 85 degenerative pseudocysts. Intraretinal fluid cysts were characterized by significantly higher circularity (0.86; range, 0.81-0.91), perfusion signal in the peri-cyst space, and peri-cyst HF in 89% of cases (all P < 0.05). Degenerative pseudocysts showed significantly lower circularity (0.68; range, 0.64-0.76), no perfusion signal in the peri-cyst space, and peri-cyst HF in only 29% of cases (all P < 0.05). The adopted quantitative metrics significantly correlated with disease duration, number of injections, fibrosis, and outer retinal atrophy. CONCLUSIONS: Intraretinal fluid cysts can be discriminated from degenerative pseudocysts using a quantitative multimodal imaging approach. These findings are clinically relevant and should be included in future training models for artificial intelligence algorithms to improve the diagnostic power and fluid monitoring in neovascular AMD. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Repeatability of retinal and choroidal optical coherence tomography angiography indices in healthy children and young adults.

PURPOSE: To evaluate the repeatability of retinal and choroidal optical coherence tomography angiography (OCT-A) indices among healthy children and compare it to healthy young adults. METHODS: This prospective study captured 3 mm × 3 mm and 6 mm × 6 mm macular OCT-A scans including superficial and deep retinal layers, choriocapillaris and deep choroid over two visits, 1 week apart at approximately the same time of day, for 22 healthy adults (18-30 years) and 21 children (6-15 years). Magnification and projection-artefact corrected indices extracted using a custom image analysis program and individual biometry were compared between visits using Bland-Altman analysis and intraclass correlation (ICC). Retinal indices included foveal avascular zone metrics, perfusion and vessel density and choroidal indices included choriocapillaris flow deficit metrics and deep choroid perfusion density, in the foveal, parafoveal and perifoveal regions. Repeatability between adults and children was compared with F-test. RESULTS: Bland-Altman analysis showed that the mean differences between repeated OCT-A indices were not significantly different from zero for either of the zones, layers and scan sizes in the two age groups (p > 0.05) except for foveal vessel density and foveal avascular zone perimeter (p = 0.04 for both) of 6-mm-deep retinal layer scans. The ICC ranged between 0.67 and 0.99. Significantly higher variability between visits (p < 0.05) in the indices was noted among adults than children, especially for choroidal indices of larger scan size. CONCLUSION: The retinal and choroidal OCT-A indices in the foveal, parafoveal and perifoveal zones were repeatable in healthy children except for the foveal vessel density and foveal avascular zone perimeter of the 6-mm-deep retinal layer, which exhibited statistically borderline differences between visits. The adult group showed more variability between visits compared to children, especially in the larger scan size for choroidal OCT-A indices.

Optical coherence tomography angiography suggests different retinal pathologies in multiple sclerosis and Sjögren's syndrome.

BACKGROUND: While retinal vessel changes are evident in the eyes of patients with relapsing-remitting multiple sclerosis (RRMS), changes in the vasculature of possible MS mimics such as primary Sjögren's syndrome (pSS) remain to be determined. We investigated the potential of retinal optical coherence tomography (OCT) angiography (OCTA) as diagnostic tool to differentiate between patients with RRMS and pSS. METHODS: This cross-sectional study included patients with RRMS (n = 36), pSS (n = 36) and healthy controls (n = 30). Participants underwent clinical examination, assessment of visual acuity, retinal OCT, OCTA, and serum markers of glial and neuronal damage. We investigated the associations between OCTA parameters, visual functions, and serum markers. Eyes with a history of optic neuritis (ON) were excluded from analysis. RESULTS: We observed a significant thinning of the combined ganglion cell and inner plexiform layer in the eyes of patients with RRMS but not with pSS, when compared to healthy controls. Retinal vessel densities of the superficial vascular complex (SVC) were reduced in both patients with RRMS and pSS. However, retinal vessel rarefication of the deep vascular complex (DVC) was only evident in patients with pSS but not RRMS. Using multivariate regression analysis, we found that DVC vessel loss in pSS patients was associated with worse visual acuity. CONCLUSIONS: Compared to patients with RRMS, rarefication of deep retinal vessels is a unique characteristic of pSS and associated with worse visual function. Assuming a disease-specific retinal vessel pathology, these data are indicative of a differential affliction of the gliovascular complex in the retina of RRMS and pSS patients.