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Purpose: To report on the venous abnormalities of a patient with Sturge-Weber syndrome., Method: Case report., Patient: A 29-year-old woman with a history of Sturge-Weber syndrome since infancy was referred for evaluation of possible diffuse choroidal hemangioma. Multimodal imaging, including ultra-widefield fluorescein, indocyanine green, and optical coherence tomography angiography, was performed., Results: Dilated fundus examination was remarkable for increased cupping of the optic disk in the right eye, venous tortuosity, and marked dilation of the choroidal vessels. Ultra-widefield fluorescein angiography confirmed marked venous tortuosity and dilation, as well as anastomoses of the retinal veins ipsilateral to the port wine stain. Indocyanine green angiography revealed marked engorgement of the vortex veins and choroidal vasculature. Optical coherence tomography angiography revealed dilated vascular channels in the deep capillary plexus that were directly anastomosing to the superficial capillary plexus, but not the intermediate capillary plexus. Engorgement of the ampullae of the deep capillary plexus vortex system was also observed. The normal contralateral eye was used as comparison for all imaging studies., Conclusion: These findings support the notion of generalized venous hypertension state in adult eyes with Sturge-Weber syndrome and corroborate previous evidence that the deep capillary plexus acts as a venous outflow system., Competing Interests: B. Fortune: Heidelberg Engineering, GmbH (equipment support). The remaining authors have no conflicts of interest.

Objectives: A third of asymptomatic individuals with type 1 diabetes (T1D) show signs of cerebrovascular disease in brain MRI. These signs associate with advanced stages of diabetic retinal disease, but not in mild or moderate retinopathy. We aimed to evaluate a wider spectrum of retinal changes by exploring the relationship between quantitative measures of retinal vessel parameters (RVP) and cerebrovascular changes in T1D., Methods: We included 146 neurologically asymptomatic individuals with T1D [51% women, median age 40 (33.0-45.1) years] and 24 healthy, sex-matched and age-matched controls. All individuals underwent a clinical and biochemical work-up and brain MRI, which was evaluated for cerebral microbleeds (CMBs), white matter hyperintensities, and lacunar infarcts. RVPs, including central retinal arteriole (CRAE) and central retinal vein (CRVE) equivalents and the ratio of the two variables (arteriovenous ratio, AVR) were assessed quantitatively by a computer-assisted method (IVAN software, version 3.2.6) from fundus images., Results: Among T1D participants, those with CMBs had a lower arteriovenous ratio (AVR) compared with those without CMBs ( P  = 0.023). AVR was inversely associated with the amount of CMBs ( r  = -0.063, P  = 0.035). CMB prevalence was higher in those with AVR below the median (31%) compared with above the median (16%, P  < 0.001), and this difference was significant also after individuals with only no-to-mild retinopathy were included (28 vs. 16%, P  = 0.005). A correlation between blood pressure and CRAE ( r  = -0.19, P  = 0.025) appeared among those with T1D., Conclusion: Regardless of the severity of diabetic retinopathy, AVR is associated with the existence of CMBs in T1D., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Cerebral vascular malformations (CVMs) may result in hemorrhage, seizure, neurologic dysfunction, and death. CVMs include capillary telangiectasias, venous malformations, cavernous malformations, and arteriovenous malformations. Cavernous and arteriovenous malformations carry the highest risk of complications. Retinal venous malformations (RVMs) have been proposed as an associated finding. Our objective was to determine the prevalence of RVMs in patients with high-risk CVMs., Methods: We retrospectively reviewed patients diagnosed with cerebral cavernous or arteriovenous malformations (high-risk CVMs) who were evaluated by the ophthalmology service at Northwestern University between 2017 and 2020. Patients were stratified into 3 cohorts based on level of certainty: dilated funduscopic examination (DFE), DFE with any form of ocular imaging, and DFE with complete imaging of the macula. We recorded ophthalmic examination abnormalities, ocular imaging findings, and major CVM complications., Results: We evaluated 156 patients with high-risk CVMs who had undergone DFE. Ocular imaging of any type was performed in 56 patients, of whom 46 had complete imaging of the macula. Zero RVMs were identified in any cohort (95% confidence interval: 0%-1.9% for the entire cohort, 0%-5.4% for any ocular imaging cohort, and 0%-6.5% for the complete macular imaging cohort). Cerebral hemorrhage or seizure occurred in 15%-33% of patients. Associated visual field defects or cranial nerve palsies were found in 14%-20% of patients., Conclusions: Zero RVMs were identified in patients with high-risk CVMs. However, neuro-ophthalmic findings were common. Therefore, we recommend neuroimaging for patients with RVMs and neuro-ophthalmic signs or symptoms. In asymptomatic patients with RVMs, a potential algorithm for neuroimaging is proposed., Competing Interests: J. A. Lavine is a consultant for Genentech for work unrelated to this study. All other authors report no conflicts of interest., (Copyright © 2023 by North American Neuro-Ophthalmology Society.)

Purpose: To measure non-invasively retinal venous blood flow (RBF) in healthy subjects and patients with retinal venous occlusion (RVO)., Methods: The prototype named AO-LDV (Adaptive Optics Laser Doppler Velocimeter), which combines a new absolute laser Doppler velocimeter with an adaptive optics fundus camera (rtx1, Imagine Eyes®, Orsay, France), was studied for the measurement of absolute RBF as a function of retinal vessel diameters and simultaneous measurement of red blood cell velocity. RBF was measured in healthy subjects (n = 15) and patients with retinal venous occlusion (RVO, n = 6). We also evaluated two softwares for the measurement of retinal vessel diameters: software 1 (automatic vessel detection, profile analysis) and software 2 (based on the use of deep neural networks for semantic segmentation of vessels, using a M2u-Net architecture)., Results: Software 2 provided a higher rate of automatic retinal vessel measurement (99.5 % of 12,320 AO images) than software 1 (64.9 %) and wider measurements (75.5 ± 15.7 μm vs 70.9 ± 19.8 μm, p < 0.001). For healthy subjects (n = 15), all the retinal veins in one eye were measured to obtain the total RBF. In healthy subjects, the total RBF was 37.8 ± 6.8 μl/min. There was a significant linear correlation between retinal vessel diameter and maximal velocity (slope = 0.1016; p < 0.001; r 2  = 0.8597) and a significant power curve correlation between retinal vessel diameter and blood flow (3.63 × 10 -5  × D 2.54 ; p < 0.001; r 2  = 0.7287). No significant relationship was found between total RBF and systolic and diastolic blood pressure, ocular perfusion pressure, heart rate, or hematocrit. For RVO patients (n = 6), a significant decrease in RBF was noted in occluded veins (3.51 ± 2.25 μl/min) compared with the contralateral healthy eye (11.07 ± 4.53 μl/min). For occluded vessels, the slope between diameter and velocity was 0.0195 (p < 0.001; r 2  = 0.6068) and the relation between diameter and flow was Q = 9.91 × 10 -6  × D 2.41 (p < 0.01; r 2  = 0.2526)., Conclusion: This AO-LDV prototype offers new opportunity to study RBF in humans and to evaluate treatment in retinal vein diseases., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Older individuals experience cardiovascular dysfunction during extended bedridden hospital or care home stays. Bed rest is also used as a model to simulate accelerated vascular deconditioning occurring during spaceflight. This study investigates changes in retinal microcirculation during a ten-day bed rest protocol. Ten healthy young males (22.9 ± 4.7 years; body mass index: 23.6 ± 2.5 kg·m -2 ) participated in a strictly controlled repeated-measures bed rest study lasting ten days. High-resolution images were obtained using a hand-held fundus camera at baseline, daily during the 10 days of bed rest, and 1 day after re-ambulation. Retinal vessel analysis was performed using a semi-automated software system to obtain metrics for retinal arteriolar and venular diameters, central retinal artery equivalent and central retinal vein equivalent, respectively. Data analysis employed a mixed linear model. At the end of the bed rest period, a significant decrease in retinal venular diameter was observed, indicated by a significantly lower central retinal vein equivalent (from 226.1 µm, CI 8.90, to 211.4 µm, CI 8.28, p = .026), while no significant changes in central retinal artery equivalent were noted. Prolonged bed rest confinement resulted in a significant (up to 6.5%) reduction in retinal venular diameter. These findings suggest that the changes in retinal venular diameter during bedrest may be attributed to plasma volume losses and reflect overall (cardio)-vascular deconditioning., (© 2023. The Author(s).)

Background Advanced diagnostics are not easily accessible in austere topographical locations. We documented retinal changes in patients with acute mountain sickness (AMS+) and compared these with asymptomatic individuals (AMS-) with recent induction into high altitude using direct ophthalmoscopy as a screening tool. Methods We evaluated 97 individuals (43 AMS- and 54 AMS+) who were inducted to an altitude 3800 m above sea level by direct ophthalmoscopy after pupillary dilatation, on day 2 of arrival. Results Retinal vein dilatation was seen in 36 (66.7%) AMS+ v. 14 (32.6%) AMS- (p<0.01), hyperaemia of the optic disc in 30 (55.6%) AMS+ v. 14 (32.6%) AMS- (p<0.05), hyperaemia of the optic disc along with retinal vein dilatation in 27 (50%) AMS+ v. 9 (20.9%) AMS- (p<0.01), retinal vein tortuosity in 12 (22.2%) AMS+ v. 3 (7%) AMS- (p<0.02). In AMS+ with retinal vein dilatation 17 (50%) had SpO2 >91% and 19 (79.2%) had SpO2 <91% (p<0.01). An AMS score of >5 was recorded in 25 (69.4%; p<0.001) with venular dilatation and in 19 (52.8%; p<0.001) who were AMS+ with an induction number ≥3 had retinal dilatation. Conclusion Acute hypobaric hypoxia causes retinal venous dilatation, tortuosity and hyperaemia of the optic disc in those with AMS and correlates directly with SpO2 levels. The incidence of retinal vein dilatation increases with frequent re-entry into high altitude and more severe symptoms of AMS. Hence, all those being inducted to high altitude should be screened for retinal vascular changes.

Objective: To study aberrant filling of the retinal vessels on fluorescein angiography (FA) images in patients with branch (BRAO) or hemi-central retinal artery occlusions (hemi-CRAO) and correlate with associated changes on optical coherence tomography (OCT)., Methods: Cases of acute BRAO or hemi-CRAO having FA and OCT images at presentation between June 2017 to May 2022 were included. Comparisons were made between the cases with and without aberrant filling of the retinal vessel., Results: Thirty-nine eyes of 39 patients with acute retinal artery occlusions underwent FA and OCT imaging. Seven of the ten cases with either BRAO or hemi-CRAO showed aberrant filling of the retinal vein on FA. No case with CRAO showed aberrant filling on FA. The cases with aberrant filling showed a better presenting visual acuity and lesser severity of retinal ischemia on OCT. Shunts between the retinal artery in the unaffected region and the retinal vein in the affected segment was seen in two eyes. The remaining five eyes showed no arteriovenous anastomosis. Cases with aberrant filling had a better visual function even at the last visit., Conclusion: Aberrant retinal vein filling across the horizontal median raphe in the affected region in BRAO or hemi-CRAO eyes had less severity of retinal ischemia and better presenting and final visual acuities. Reduced perfusion in retinal capillaries with altered retinal vascular anatomy or arteriovenous shunts could be probable reasons for the development of this FA finding. Presence of aberrant filling of the retinal vein could indicate better final visual prognosis., (© 2022. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Spontaneous retinal Venous Pulsations (SVP) are rhythmic changes in the caliber of the central retinal vein and are observed in the optic disc region (ODR) of the retina. Its absence is a critical indicator of various ocular or neurological abnormalities. Recent advances in imaging technology have enabled the development of portable smartphone-based devices for observing the retina and assessment of SVPs. However, the quality of smartphone-based retinal videos is often poor due to noise and image jitting, which in return, can severely obstruct the observation of SVPs. In this work, we developed a fully automated retinal video stabilization method that enables the examination of SVPs captured by various mobile devices. Specifically, we first propose an ODR Spatio-Temporal Localization (ODR-STL) module to localize visible ODR and remove noisy and jittering frames. Then, we introduce a Noise-Aware Template Matching (NATM) module to stabilize high-quality video segments at a fixed position in the field of view. After the processing, the SVPs can be easily observed in the stabilized videos, significantly facilitating user observations. Furthermore, our method is cost-effective and has been tested in both subjective and objective evaluations. Both of the evaluations support its effectiveness in facilitating the observation of SVPs. This can improve the timely diagnosis and treatment of associated diseases, making it a valuable tool for eye health professionals.

Objective: Retinal vein cannulation is a technically demanding surgical procedure and its feasibility may rely on using advanced surgical robots equipped with force-sensing microneedles. Reliable detection of the moment of venous puncture is important, to either alert or prevent the clinician from double puncturing the vessel and damaging the retinal surface beneath. This paper reports the first in-vivo retinal vein cannulation trial on rabbit eyes, using sensorized metal needles, and investigates puncture detection., Methods: We utilized total of four indices including two previously demonstrated ones and two new indices, based on the velocity and force of the needle tip and the correlation between the needle-tissue and tool-sclera interaction forces. We also studied the effect of detection timespan on the performance of detecting actual punctures., Results: The new indices, when used in conjunction with the previous algorithm, improved the detection rate form 75% to 92%, but slightly increased the number of false detections from 37 to 43. Increasing the detection window improved the detection performance, at the cost of adding to the delay., Conclusion: The current algorithm can supplement the surgeons' visual feedback and surgical judgment. To achieve automatic puncture detection, more measurements and further analysis are required. Subsequent in-vivo studies in other animals, such as pigs with their more human like eye anatomy, are required, before clinical trials., Significance: The study provides promising results and the criteria developed may serve as guidelines for further investigation into puncture detection in in-vivo retinal vein cannulation.

Purpose: To compare patterns of choroidal venous drainage in eyes with pachychoroid disease to those of healthy subjects using ultra-widefield indocyanine green angiography (UWF ICGA)., Methods: Patients with pachychoroid disease and healthy controls were recruited at two referral centers. UWF ICGA images were used to evaluate the proportion of the postequatorial fundus drained by major vortex vein systems in each quadrant and to study the incidence and topography of choroidal vascular hyperpermeability (CVH) and intervortex venous anastomoses. Widefield swept-source optical coherence tomography (SS-OCT) was used to evaluate choroidal thickness at the posterior pole in eyes with pachychoroid disease., Results: Fifty-two pachychoroid eyes and 26 healthy eyes were evaluated. Eyes with pachychoroid disease showed a significant within-subject variance in the proportion of the postequatorial fundus drained by each vortex vein system (range, 4.1%-48.1%; P < 0.0001) that was not seen in controls (range, 17.3%-31.7%; P = 0.11). CVH was present in all pachychoroid disease eyes and three of 26 controls. Intervortex venous anastomoses were present in 46 of 52 pachychoroid disease eyes and nine of 26 control eyes. Vortex vein systems with large drainage areas showed greater density of CVH spots. SS-OCT demonstrated asymmetric choroidal drainage in the macula of 59% of pachychoroid eyes. CVH and intervortex venous anastomoses were more prominent in areas showing maximal choroidal thickness., Conclusions: In eyes with pachychoroid disease, imbalanced choroidal venous drainage with congestion of specific vortex vein systems may contribute to a state of choroidal venous insufficiency characterized by regional choroidal thickening, CVH and remodeling of venous drainage routes.

Purpose: To develop methods to quantitatively measure retinal vessel tortuosity from fundus images acquired in subjects with papilledema and assess sources of variability in these measurements., Methods: Digital fundus images from 30 eyes of subjects with untreated idiopathic intracranial hypertension and papilledema were analyzed. Retinal vein and artery tortuosities for three to four vessels of each type were measured in a region of interest 1.8 to 2.7 mm from the center of the optic nerve head. Measurements were averaged to generate a venous tortuosity index (VTI) and arterial tortuosity index (ATI) for each eye. One image of each eye was analyzed two times by the same rater. Two images of each eye, differing by focal depth, were analyzed by the same rater. Correlations between VTI and ATI for the same image and different images were calculated., Results: Intrarater Pearson correlations (r) were 0.8 (95% confidence interval [CI], 0.59-0.9) and 0.90 (95% CI, 0.73-0.96) for VTI and ATI, respectively, with one outlier removed. Interimage r values were 0.72 (95% CI, 0.48-0.87) and 0.96 (95% CI, 0.89-0.99) for VTI and ATI, respectively, with one outlier removed. The intraclass correlation coefficients for agreement and consistency were similar, suggesting that the discrepancy between measurements was due to residual random error., Conclusions: The finding of similar intrarater and interimage variability suggests that intrarater variability may be a more dominant source than physiology and image acquisition., Translational Relevance: Standardizing rater procedures and averaging multiple measuring sessions are strategies to reduce variability and improve reliability of detecting retinal vessel tortuosity changes in images of eyes with papilledema.

Purpose/Aim of this study : Retinal vessel caliber is an independent risk marker of cardiovascular disease risk. However, variable mechanical delays in capturing retinal photographs and cardiac cycle-induced retinal vascular changes have been shown to reduce the accuracy of retinal vessel caliber measurements, but this has only ever been investigated in healthy subjects. This cross-sectional study is the first study to investigate this issue in type 2 diabetes. The aim of this study was to determine whether ECG-gating retinal photographs reduce the variability in retinal arteriolar and venular caliber measurements in controls and type 2 diabetes. Materials and Methods : Fifteen controls and 15 patients with type 2 diabetes were arbitrarily recruited from Westmead Hospital, Sydney, Australia. A mydriatic fundoscope connected to our novel ECG synchronization unit captured 10 ECG-gated (at the QRS) and 10 ungated digital retinal photographs of the left eye in a randomized fashion, blinded to study participants. Two independent reviewers used an in-house semi-automated software to grade single cross-sectional vessel diameters across photographs, between 900 and 1800 microns from the optic disc edge. The coefficient of variation compared caliber variability between retinal arterioles and venules. Results : Our ECG synchronization unit reported the smallest time delay (33.1 ± 48.4 ms) in image capture known in the literature. All 30 participants demonstrated a higher reduction in retinal arteriolar (ungated: 1.02, 95%CI 0.88-1.17% vs ECG-gated: 0.39, 95%CI 0.29-0.49%, p < .0001) than venular (ungated 0.62, 95%CI 0.53-0.73% vs ECG-gated: 0.26, 95%CI 0.19-0.35%, p < .0001) coefficient of variation by ECG-gating photographs. Intra-observer repeatability and inter-observer reproducibility analysis reported high interclass correlation coefficients ranging from 0.80 to 0.86 and 0.80 to 0.93 respectively. Conclusion : ECG-gating photographs at the QRS are recommended for retinal vessel caliber analysis in controls and patients with type 2 diabetes as they refine measurements.

This study aimed to theoretically identify the vascular nature of the deep capillary plexus (DCP) by examining patients presenting with both paracentral acute middle maculopathy (PAMM) and prominent middle limiting membrane (p-MLM) sign and p-MLM sign alone in spectral-domain optical coherence tomography (SD-OCT). A retrospective review of the medical records of patients with retinal vein or artery occlusion from two tertiary medical centers was performed. Consecutive patients with a clinical diagnosis of all categories of retinal artery occlusion (RAO) and retinal vein occlusion (RVO) (branch or central and ischemic or non-ischemic) who had undergone SD-OCT imaging from January 2015 to May 2020 were recruited and their p-MLM signs and PAMM lesions were assessed. We included 118 patients who presented with p-MLM sign with or without PAMM lesions. Amon them, 40 were female and 78 were male, with a mean age of 61.1 years. Of the 109 patients with both p-MLM sign and PAMM lesions, 23 had branch RAO, two had branch RVO, 67 had central RAO, 13 had central RVO, and four had a combination of central RAO and central RVO. All nine patients with the p-MLM sign alone had central RVO accompanied by cystoid macular edema. In all the enrolled patients, the hyperreflective lines of the p-MLM sign were continuous, regardless of the type of PAMM lesions. In conclusion, when PAMM and p-MLM sign are examined together, further proof regarding the possible complete venous nature of the vasculature of the retinal DCP might be speculated., Competing Interests: The authors have declared that no competing interests exist.

Significance: Commercially available platforms show good agreement in clinical outcomes for retinal vessel caliber measurements, despite differing absolute values. Tighter agreement is observed when right and left eye data are averaged, suggesting an approach suitable for clinical practice., Purpose: The purpose of this study was to compare the retinal vessel caliber measurements generated by different commercially available platforms and their associations with systemic blood pressure and age., Methods: A total of 209 participants were recruited from a UK optometric practice. After a routine eye examination, participants had disc-centered retinal photographs and systemic blood pressure taken. Retinal vessel calibers (central retinal artery equivalent, central retinal vein equivalent, and arteriovenous ratio) were calculated using both MONA REVA and VesselMap., Results: An inverse Pearson correlation was observed between central retinal artery equivalent and mean arterial blood pressure on both platforms (r = -0.275 [P ≤ .001] and r = -0.388 [P ≤ .001] for MONA REVA and VesselMap, respectively); this correlation was also observed with arteriovenous ratio and blood pressure. An inverse correlation was observed between central retinal artery equivalent and age (r = -0.362 [P ≤ .001] and r = -0.404 [P ≤ .001] for MONA REVA and VesselMap, respectively); this was also seen between central retinal vein equivalent and age (r = -0.322 [P ≤ .001] and r = -0.369 [P ≤ .001]). Arteriovenous ratio remained independent from age for both platforms. Bland-Altman plots demonstrated good agreement between the platforms for all three variables., Conclusions: Although absolute caliber measurements differed between the platforms, the correlations observed were of similar magnitudes, with good agreement between the two platforms. Tighter spaced limits of agreement were observed when right and left eye data were averaged for each subject. In the absence of localized ocular pathology, this approach should be used., Competing Interests: Conflict of Interest Disclosure: None of the authors have reported a financial conflict of interest., (Copyright © 2021 American Academy of Optometry.)

Accumulating evidence points to pachychoroid possibly being caused by vortex vein congestion which results in remodeling of choroidal drainage routes via intervortex vein anastomosis. This hypothesis prompted us to investigate vortex vein hemodynamics by studying videos of indocyanine green angiography (ICGA) in a retrospective case series of 295 eyes with pachychoroid spectrum diseases. In the early phase of the video-ICGA, pulsatile vortex venous flow was observed in 76 eyes (25.8%) at the vortex veins connected with anastomosis between superior and inferior vortex veins. The patients with pulsatile vortex venous flow were significantly older than those without pulsatile vortex venous flow (67.8 ± 13.2 vs. 63.9 ± 14.5 years, P < 0.05). Pulsatile vortex venous flow was 1.84 times more common in the inferior quadrants than in the superior quadrants. Interestingly, 14 of 76 eyes (18.4%) with pulsatile vortex venous flow showed retrograde pulsatile blood flow in the vortex veins. This retrograde pulsatile blood flow was 2.50 times more common in the inferior than in the superior quadrants. These findings indicate altered vortex vein hemodynamics due to vortex vein congestion in pachychoroid spectrum diseases., (© 2021. The Author(s).)

Purpose : To assess the agreement between Line 3-5 raster scan mode and circular scan mode for measuring retinal vessel diameter, and to analyze the influence of scanning distance on retinal vessel diameter and agreement. Methods : 79 healthy participants (79 eyes) were scanned with two modes. The scanning distance was defined as the distance from the center of the optic disc to the intersection of the blood vessel and the scan line on the raster image. The large superior temporal vessel was measured, with the distance between vascular wall hyperreflectivities measured to obtain vessel diameters. The degree of agreement between the line 3-5 raster scan and circular scan modes, and the effect of scanning distance on agreement and vascular diameter were assessed. Results : There was good agreement between line 3 subgroup and the circular scan for measuring venous and arterial diameter (venous: intraclass correlation coefficients (ICCs) = 0.87, p < .001; arterial: ICCs = 0.84, p < .001). Unexpectedly, diameters from the fourth raster scan were only comparable to the circular scan in measuring venous diameter (ICCs = 0.86, p < .001), despite the same scanning distance between the fourth raster line and circular scan. Vessels with a scanning distance between 1400 μm - 1799 μm showed good agreement with the circular scan (venous and arterial: all ICCs ≥ 0.84, p < .001). In addition, venous diameter and arterial diameter decreased with increasing distance from the optic disc center, with venous and arterial diameter decreasing by 0.02 μm/μm ( p < .001) and 0.007 μm/μm ( p = .02), respectively. Conclusion : Arterial and venous diameter measured by the circular mode was comparable to only one scan line and two scan lines of the raster scan mode, respectively. Our study identified a difference between the two scan modes, with the difference not fully attributable to differences in scanning distance. Prospective studies reporting vascular diameter as a primary outcome should report the scan mode used.

Purpose: To compare widefield optical coherence tomography angiography (OCTA) to ultra-widefield fluorescein angiography (UWFA) in the assessment of nonperfusion in retinal vein occlusion (RVO)., Methods: A cross-sectional study of 43 eyes of 43 patients with RVO examined using both widefield OCTA (PLEX Elite, Carl Zeiss Meditec, Dublin, CA) with a panoramic montage of five 12 × 12-mm images and UWFA (Optos, 200°). Qualitative analysis was performed according to nonperfusion areas (cutoff: three disk areas) on widefield OCTA. The quantitative analysis assessed the vascular density on the widefield OCTA and ischemic index on UWFA., Results: The ischemic index on UWFA and vascular density in the superficial and deep plexus correlated significantly (P = 0.019, r = 0.357 and P < 0.013, r = 0.375, respectively). The qualitative classification on widefield OCTA and ischemic index on UWFA correlated significantly (P < 0.001, r = 0.618). For the detection of marked nonperfusion (ischemic index ≥ 25%), widefield OCTA had a sensitivity of 100% and a specificity of 64.9%., Conclusion: The presence of nonperfusion on UWFA correlated with widefield OCTA. Optical coherence tomography angiography could help to identify high-risk RVO patients who might benefit from a further evaluation using fluorescein angiography.

Purpose: To precisely quantify retinal nonperfusion areas (NPAs) in branch retinal vein occlusion using widefield optical coherence tomography angiography (OCTA) and examine their association with neovascular complications., Methods: We enrolled 26 patients with treatment-naïve branch retinal vein occlusion and prospectively examined them for 12 months. After 3 monthly ranibizumab injections to treat macular edema, each patient underwent ultra-widefield (UWF) fluorescein angiography (FA) and OCTA. Ultra-widefield FA was additionally performed at Month 12. For UWF FA, the retinal NPA was measured using the equipment's built-in software. For OCTA, we used panoramic image montaged from 5 single 12 × 12 mm2 images and quantified the retinal NPA using a Gullstrand eye with a grid scale at each patient. Measurements were expressed in terms of actual values and disc area units., Results: The retinal NPAs as measured using single OCTA and panoramic OCTA were significantly associated with that measured using UWF FA (P < 0.001 for both). Retinal neovascularization lesions were observed in 4 (15.4%) of 26 eyes. For patients with accompanying neovascularization, the retinal NPA measured using UWF FA, single OCTA, and panoramic OCTA were 187.9 ± 39.5 mm2 (109.9 ± 21.4 disc area), 34.3 ± 13.7 mm2 (19.9 ± 7.7 disc area), and 106.6 ± 24.5 mm2 (62.4 ± 13.6 disc area), respectively, which were larger than for those without neovascularization (P < 0.001, 0.014, and <0.001, respectively)., Conclusion: Using widefield OCTA, we could quantify the retinal NPA of eyes with branch retinal vein occlusion. These could serve as valid references to assess the risk of neovascular complications.

Purpose: To evaluate the choroidal vascular patterns of patients with pachychoroid-related diseases in eyes images with wide-field indocyanine green angiography., Methods: Retrospective study of wide-field indocyanine green angiographic images of patients with pachychoroid, peripapillary pachychoroid syndrome, central serous chorioretinopathy, and pachychoroid-associated neovascularization that were evaluated for anastomoses between vortex vein systems, which are ordinarily separated by a watershed zone., Results: There were 21 subjects with a mean age of 57.4 years and 15 were male. Among the 42 eyes evaluated, central serous chorioretinopathy was found in 24 eyes (57.1%), peripapillary pachychoroid syndrome in 5 (11.9%), pachychoroid associated neovascularization in 7 (16.7%), and pachychoroid in 6 (14.3%). Every eye showed anastomosis between the superonasal, superotemporal, and inferotemporal vortex vein systems. The inferonasal vortex vein system was less likely to demonstrate anastomosis except for peripapillary pachychoroid syndrome, which showed anastomosis in all eyes. The anastomotic connections were prominent in the central macula in the central serous chorioretinopathy and pachychoroid-associated neovascularization cases, and around the nerve in the peripapillary pachychoroid syndrome cases. Although the large choroidal veins were particularly prominent in the neovascular cases, the number was fewer in the macular region than in other pachychoroid-related diseases in this series. Compared with a control group of nine eyes, the inferotemporal-superotemporal-superonasal anastomotic connections were more common in the case group (P < 0.001) and inferonasal quadrant (P = 0.023 right eye; P = 0.01, left eye)., Conclusion: Intervortex venous anastomosis is common in pachychoroid, central serous chorioretinopathy, peripapillary pachychoroid syndrome, and pachychoroid-associated neovascularization. This finding has important implications concerning pathogenesis and classification of disease.

Purpose: To investigate the dilated choroidal veins (DCVs) at or around myopic macular neovascularizations (MNVs) and to determine whether there is a hemodynamic relationship between them., Methods: Fifty-eight eyes of 57 patients with myopic MNVs were examined. Dilated choroidal veins were defined as choroidal veins whose diameter was 2X larger than adjacent veins. Indocyanine green angiography and swept-source optical coherence tomography images were reviewed to detect DCVs that crossed the subfoveal area. The filling sequence of the DCVs and MNVs was determined., Results: Patients' mean age was 71.4 ± 10.6 years. The mean axial length was 29.3 ± 1.8 mm. Dilated choroidal veins below or around the MNV were found in 17 eyes (29.3%). Emissaries of the short posterior ciliary arteries were seen at or around MNVs in 8 of the 17 eyes. In these eyes, the short posterior ciliary artery was filled first or almost simultaneously with the filling of the MNV, followed by a laminar filling of the DCVs. In one eye, afferent arterioles from the short posterior ciliary arteries and efferent venules connected to DCVs were seen., Conclusion: Dilated choroidal veins are present below or around MNVs in about 30% of eyes with myopic MNVs. Our findings suggest that an MNV might be a vascular unit consisting of short posterior ciliary arteries, afferent arterioles, efferent venules, and DCVs.

The purpose of this study was to determine the number and location of vortex vein ampullae (VVA) in normal eyes. This was an observational retrospective study. Montage images of one on-axis and two off-axis ultra-widefield images of 74 healthy eyes were enhanced, and reverse projected onto a 3D model eye. The number and distance between the optic disc to each VVA in the four sectors were compared. The significance of correlations between these values and age, sex, visual acuity, refractive error, and axial length was determined. The mean number of VVA was 8.10/eye with 1.84, 2.12, 2.19 and 1.95 in upper lateral, lower lateral, upper nasal, and lower nasal sectors, respectively. The mean number of VVA/eye was significantly greater in men at 8.43 than women at 7.76 (P = 0.025). The mean distance between the optic disc and VVA was 14.15 mm, and it was 14.04, 15.55, 13.29 and 13.66 mm in the upper lateral, lower lateral, upper nasal and lower nasal sectors, respectively (all P < 0.05). The number and location of VVA can be obtained non-invasively, and the number was significantly higher in men than women. This technique can be used to determine whether these values are altered in a retinochoroidal disease.

Purpose: Orbital veins such as the retinal veins and episcleral veins drain into the cavernous sinus, an intracranial venous structure. We studied the effects of acute intracranial pressure (ICP) elevation on episcleral venous pressure, intraocular pressure and retinal vein diameter in an established non-survival pig model., Methods: In six adult female domestic pigs, we increased ICP in 5 mm Hg increments using saline infusion through a lumbar drain. We measured ICP (using parenchymal pressure monitor), intraocular pressure (using pneumatonometer), episcleral venous pressure (using venomanometer), retinal vein diameter (using OCT images) and arterial blood pressure at each stable ICP increment. The average baseline ICP was 5.4 mm Hg (range 1.5-9 mm Hg) and the maximum stable ICP ranged from 18 to 40 mm Hg. Linear mixed models with random intercepts were used to evaluate the effect of acute ICP increase on outcome variables., Results: With acute ICP elevation, we found loss of retinal venous pulsation and increased episcleral venous pressure, intraocular pressure and retinal vein pressure in all animals. Specifically, acute ICP increase was significantly associated with episcleral venous pressure (β = 0.31; 95% CI 0.14-0.48, p < .001), intraocular pressure (β = 0.37, 95%CI 0.24-0.50; p < .001) and retinal vein diameter (β = 11.29, 95%CI 1.57-21.00; p = .03) after controlling for the effects of arterial blood pressure., Conclusion: We believe that the ophthalmic effects of acute ICP elevation are mediated by increased intracranial venous pressure producing upstream pressure changes within the orbital and retinal veins. These results offer exciting possibilities for the development of non-invasive ophthalmic biomarkers to estimate acute ICP elevations following significant neuro-trauma.

Importance: Individuals with autosomal dominant mutations for Alzheimer disease are valuable in determining biomarkers present prior to the onset of cognitive decline, improving the ability to diagnose Alzheimer disease as early as possible. Optical coherence tomography (OCT) has surfaced as a potential noninvasive technique capable of analyzing central nervous system tissues for biomarkers of Alzheimer disease., Objective: To evaluate whether OCT can detect early retinal alterations in carriers of the presenilin 1 (PSEN1 [OMIM 104311]) E280A mutation who are cognitively unimpaired., Design, Setting, and Participants: A cross-sectional imaging study conducted from July 13, 2015, to September 16, 2020, included 10 carriers of the PSEN1 E280A mutation who were cognitively unimpaired and 10 healthy noncarrier family members, all leveraged from a homogenous Colombian kindred. Statistical analysis was conducted from September 9, 2017, to September 16, 2020., Main Outcomes and Measures: Mixed-effects multiple linear regression was performed to compare the thickness values of the whole retina and individual retinal layers on OCT scans between mutation carriers and noncarriers. Simple linear-effects and mixed-effects multiple linear regression models were used to assess whether age was an effect modifier for PSEN1 mutation of amyloid β levels and retinal thickness, respectively. Fundus photographs were used to compare the number of arterial and venous branch points, arterial and venous tortuosity, and fractal dimension., Results: This study included 10 carriers of the PSEN1 E280A mutation who were cognitively unimpaired (7 women [70%]; mean [SD] age, 36.3 [8.1] years) and 10 healthy noncarrier family members (7 women [70%]; mean [SD] age, 36.4 [8.2] years). Compared with noncarrier controls, PSEN1 mutation carriers who were cognitively unimpaired had a generalized decrease in thickness of the whole retina as well as individual layers detected on OCT scans, with the inner nuclear layer (outer superior quadrant, β = -3.06; P = .007; outer inferior quadrant, β = -2.60; P = .02), outer plexiform layer (outer superior quadrant, β = -3.44; P = .03), and outer nuclear layer (central quadrant, β = -8.61; P = .03; inner nasal quadrant, β = -8.39; P = .04; inner temporal quadrant, β = -9.39; P = .02) showing the greatest amount of statistically significant thinning. Age was a significant effect modifier for the association between PSEN1 mutation and amyloid β levels in cortical regions (β = 0.03; P = .001) but not for the association between PSEN1 mutation and retinal thickness. No statistical difference was detected in any of the vascular parameters studied., Conclusions and Relevance: These findings suggest that OCT can detect functional and morphologic changes in the retina of carriers of familial Alzheimer disease who are cognitively unimpaired several years before clinical onset, suggesting that OCT findings and retinal vascular parameters may be biomarkers prior to the onset of cognitive decline.

Purpose: To study the characteristics of venous circulation in the eyes with retinopathy of prematurity (ROP) observed in examination of blood flow in the central retinal vein and superior orbital vein in patients with different forms, stages and state of the disease., Material and Methods: Color duplex scanning of the central retinal vein and superior orbital vein was performed using color Doppler imaging and pulsed Doppler sonography in 55 premature babies (55 eyes) with active ROP and 8 premature babies (8 eyes) with no signs of ROP. All babies included in the study were born at 25-32 weeks of gestation, their birth weight was 680-1760 g., Results: A characteristic feature of hemodynamic parameters in the central retinal vein at stages 1-3 of active ROP was a decrease in both the maximum and minimum blood flow velocities (V max and V min ), indicating a serious disorder of ocular hemodynamics in this category of patients. An increase in V max in the central retinal vein was recorded for patients with aggressive posterior ROP, which occurs in response to venous stasis that is common in this form of the disease. The performed statistical assessment of the prognostic significance of hemodynamic parameters of the central retinal vein in relation to the type of active ROP revealed a high information content of V min , which is promising for early detection of unfavorable course of the disease helping to ensure timely treatment. The blood flow in the superior orbital vein showed distinctive absence of any correlations with the course of the disease, disallowing any immediate conclusions on the informativeness and predictive value of its parameters., Conclusion: The revealed features of venous blood flow in patients with active ROP help expand the understanding of vascular changes in this pathology, and can also be applied in clinical practice to improve the accuracy of predicting the course of the disease.

Purpose: Ocular structural and functional changes, collectively termed spaceflight-associated neuro-ocular syndrome (SANS), have been described in astronauts undergoing long-duration missions in the microgravity environment of the International Space Station. We tested the hypothesis that retinal vascular remodeling, particularly by smaller vessels, mediates the chronic headward fluid shifts associated with SANS., Methods: As a retrospective study, arterial and venous patterns extracted from 30° infrared Heidelberg Spectralis retinal images of eight crew members acquired before and after six-month missions were analyzed with NASA's recently released VESsel GENeration Analysis (VESGEN) software. Output parameters included the fractal dimension and overall vessel length density that was further classified into large and small vascular branching generations. Vascular results were compared with SANS-associated clinical ocular measures., Results: Significant postflight decreases in Df, Lv, and in smaller but not larger vessels were quantified in 11 of 16 retinas for arteries and veins (P value for Df, Lv, and smaller vessels in all 16 retinas were ≤0.033). The greatest vascular decreases occurred in the only retina displaying clinical evidence of SANS by choroidal folds and optic disc edema. In the remaining 15 retinas, decreases in vascular density from Df and Lv ranged from minimal to high by a custom Subclinical Vascular Pathology Index., Conclusions: Together with VESGEN, the Subclinical Vascular Pathology Index may represent a new, useful SANS biomarker for advancing the understanding of SANS etiology and developing successful countermeasures for long duration space exploration in microgravity, although further research is required to better characterize retinal microvascular adaptations.

Pachychoroid spectrum diseases have attracted increasing attention, though their pathophysiology has yet to be fully elucidated. In this study, we assessed the vascular diameters of vortex veins in pachychoroid spectrum diseases such as central serous chorioretinopathy (CSC), pachychoroid neovasculopathy without polypoidal lesions (PNV), and pachychoroid neovasculopathy with polypoidal lesions (polypoidal choroidal vasculopathy: PCV). In a retrospective case series of 94 eyes with CSC, 60 eyes with PNV and 57 with PCV, we binarized en face optical coherence tomography (OCT) images of choroidal vortex veins and analyzed the mean diameter of vortex veins. The presence of anastomosis between the superior and inferior vortex veins and central choroidal thickness (CCT) were also evaluated using OCT images. CSC showed significantly larger mean diameter of vortex veins than PCV (P < 0.05). Anastomosis between superior and inferior vortex veins was observed in over 90% of eyes with each pachychoroid spectrum disease. The patients with CSC were the youngest, followed by PNV patients, and then patients with PCV. The largest CCT values were observed in CSC eyes, followed by PNV eyes, and then PCV eyes. CCT correlated with the mean diameter of vortex veins (rs = 0.51, P < 0.01). These findings suggest that congestion of vortex veins might show gradual amelioration corresponding to the development of anastomosis between the superior and inferior vortex veins during the course of progression of pachychoroid spectrum diseases. Moreover, the mean diameter of vortex veins can be used as a parameter indicating choroidal congestion.

Competing Interests: None

Purpose: To evaluate the vascular changes in vortex veins at the posterior pole in pachychoroid spectrum diseases, including central serous chorioretinopathy (CSC), pachychoroid neovasculopathy without polypoidal lesions (PNV), and pachychoroid neovasculopathy with polypoidal lesions (polypoidal choroidal vasculopathy [PCV])., Design: Retrospective case-control study., Participants: Ninety-two eyes of 89 patients with CSC, 61 eyes of 59 patients with PNV, 63 eyes of 61 patients with PCV, and 25 healthy control eyes of 25 age- and gender-matched participants for each pachychoroid spectrum disease., Methods: Clinical records of patients with pachychoroid spectrum diseases and healthy controls were reviewed. Multimodal images of each group were analyzed. Swept-source OCT was performed to obtain B-mode and en face images in patients with pachychoroid spectrum diseases and healthy controls. All patients underwent fluorescein angiography, indocyanine green angiography, and OCT., Main Outcome Measures: Vortex vein anastomosis at the watershed, determined using en face OCT, and central choroidal thickness (CCT), measured using B-mode OCT, were examined in patients and healthy controls. Patient ages also were taken into consideration., Results: Patients with CSC were the youngest, followed by patients with PNV, and then those with PCV (P < 0.01, CSC vs. PNV and PNV vs. PCV), whereas CSC eyes showed the highest CCT values, followed by the PNV and then the PCV eyes (P < 0.01, CSC vs. PNV; P < 0.05, PNV vs. PCV). Central choroidal thickness was significantly greater in pachychoroid spectrum diseases than in healthy controls. No significant CCT differences were found among healthy controls. Anastomosis between superior and inferior vortex veins was observed in more than 90% of eyes with pachychoroid spectrum diseases, making this finding significantly more frequent than in healthy controls (P < 0.01, each pachychoroid spectrum disease vs. control). Polypoidal choroidal vasculopathy showed a significantly higher rate of anastomosis than CSC (P < 0.05)., Conclusions: Anastomosis between superior and inferior vortex veins was found to be a common feature in pachychoroid spectrum diseases. Longstanding vortex vein congestion may lead to the development of pachychoroid spectrum diseases. Choroidal congestion may be compensated for by new drainage routes formed via vortex vein anastomosis., (Copyright © 2020 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Vascular tree disentanglement and vessel type classification are two crucial steps of the graph-based method for retinal artery-vein (A/V) separation. Existing approaches treat them as two independent tasks and mostly rely on ad hoc rules (e.g. change of vessel directions) and hand-crafted features (e.g. color, thickness) to handle them respectively. However, we argue that the two tasks are highly correlated and should be handled jointly since knowing the A/V type can unravel those highly entangled vascular trees, which in turn helps to infer the types of connected vessels that are hard to classify based on only appearance. Therefore, designing features and models isolatedly for the two tasks often leads to a suboptimal solution of A/V separation. In view of this, this paper proposes a multi-task siamese network which aims to learn the two tasks jointly and thus yields more robust deep features for accurate A/V separation. Specifically, we first introduce Convolution Along Vessel (CAV) to extract the visual features by convolving a fundus image along vessel segments, and the geometric features by tracking the directions of blood flow in vessels. The siamese network is then trained to learn multiple tasks: i) classifying A/V types of vessel segments using visual features only, and ii) estimating the similarity of every two connected segments by comparing their visual and geometric features in order to disentangle the vasculature into individual vessel trees. Finally, the results of two tasks mutually correct each other to accomplish final A/V separation. Experimental results demonstrate that our method can achieve accuracy values of 94.7%, 96.9%, and 94.5% on three major databases (DRIVE, INSPIRE, WIDE) respectively, which outperforms recent state-of-the-arts.

Purpose: To use structural criteria to reconcile the three-dimensional organization and connectivity of the parafoveal microvasculature., Methods: The parafoveal microvasculature was perfused and labeled in 16 normal human donor eyes for lectin, alpha smooth muscle actin, and filamentous actin. Established structural criteria gathered using confocal microscopy, including vessel diameter, endothelial cell morphology, and presence/density of smooth muscle cells, were used to differentiate arteries, arterioles, capillaries, venules, and veins. Three-dimensional visualization strategies were used to define the connections between retinal arteries and veins within the superficial vascular plexus (SVP), intermediate capillary plexus (ICP), and deep capillary plexus (DCP)., Results: The parafoveal microvasculature has two different inflow patterns and seven different outflow patterns. The SVP and ICP were connected to retinal arteries by arterioles. Inflow into the DCP occurred only via small arterioles (a1; mean diameter, 8.3 µm) that originated from the ICP. Direct connections between the DCP and retinal arteries were not identified. Each capillary plexus formed its own venule that drained independently or in conjunction with venules from other plexuses into a retinal vein at the level of the ganglion cell layer. For the DCP, a1 was significantly smaller than its draining venule (mean diameter, 18.8 µm; P < 0.001)., Conclusions: The SVP and ICP of the parafoveal microvasculature have both in series and in parallel arterial and venous connections. Arterial supply to the DCP originates from the ICP, but with direct drainage to the retinal vein. These findings may help to develop an understanding of the pattern of retinal lesions characterizing a myriad of retinal vascular diseases.

Background and Objective: To correlate between hyperreflective material (HRM) seen on optical coherence tomography (OCT) and visual outcomes in treatment-naïve acute branch retinal vein occlusion (BRVO)., Patients and Methods: Demographic and OCT features of patients with acute BRVO were analyzed retrospectively. OCT parameters noted were macular edema type, HRM presence and location, and outer retinal layer integrity. Eyes were separated into two groups: Group 1 (BRVO without HRM) and Group 2 (BRVO with HRM)., Results: Forty-seven eyes (67%) were included in Group 1 and 23 eyes (33%) were included in Group 2. Mean presenting logMAR visual acuity in Groups 1 and 2 was 0.521 ± 0.428 (mean Snellen equivalent = 20/66) and 0.627 ± 0.427 (mean Snellen equivalent = 20/85), respectively. Poor vision at final visit was noted in the BRVO with HRM group (P = .027). More intravitreal injections were required for macular edema resolution in the HRM group (4.83 vs. 2.67)., Conclusions: Presence of HRM in BRVO is associated with poorer vision. It can be a useful prognostic biomarker. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:320-327.]., (Copyright 2020, SLACK Incorporated.)

Background: Assessment of spontaneous venous pulsation (SVP) is commonly undertaken to help determine whether intracranial pressure (ICP) is elevated. Previous studies using direct ophthalmoscopy or slit-lamp assessments have found that SVP is not observed in 67%-81% of subjects with normal ICP, and that interobserver agreement when grading SVP is poor., Methods: Patients (n = 105) undergoing clinically indicated retinal OCT scans, who were all believed to have normal ICP, had 10-second infrared video recordings performed with the Heidelberg Spectralis OCT system (Heidelberg Engineering GmbH, Heidelberg, Germany). The presence and amplitude of SVP in each video was independently graded by 2 neuro-ophthalmologists., Results: The 2 observers found SVP present in 97% and 98% of right eyes and in one or both eyes in 99% and 100% of subjects. Interobserver agreement was high (Cohen's kappa 0.82 for right eyes). Optic discs with a smaller cup had a significantly lower SVP amplitude (Spearman's rho = 0.22, P = 0.02)., Conclusions: Infrared video is widely available in eye clinics by the use of OCT imaging systems and is substantially more sensitive in detecting SVP than traditional assessments using ophthalmoscopy. SVP is absent in as few as 1% of people with presumed normal ICP.

Precis: After a short-term intraocular pressure (IOP) elevation, the central retinal vein caliber may be widened at lower IOP rise levels, while be compressed at higher IOP rise values., Purpose: The purpose of this study was to investigate changes in the calibers of the central retinal vein trunk (CRVT) and central retinal artery trunk (CRAT) trunk during a short-term elevation of IOP., Methods: A prospective observational study. Acute primary angle-closure suspects underwent a dark room prone provocative test (DRPPT) for 2 hours. Before and at the end of the test, tonometry, swept-source optical coherence tomography, and nonmydriatic fundus photography were performed. The calibers of the CRVT and CRAT were measured on the fundus photos taken at baseline and at the end of the DRPPT., Results: The study included 101 eyes (61 individuals; mean age: 54.8±9.3 y; range: 30 to 70 y) which showed an increase in IOP by 9.6±9.0 mm Hg (range: 2.3 to 46.7 mm Hg). From baseline to the end of the DRPPT, the mean CRVT caliber increased from 101.8±25.9 to 107.7±26.6 μm (P<0.001), while the CRAT caliber did not differ significantly (110.3±24.2 vs. 109.7±21.5 μm; P=0.54) during the test. The CRVT widening was larger in the subgroup with IOP rise of <6 mm Hg than in the subgroup with an IOP rise of 6 to 15 mm Hg, while in the subgroup with an IOP rise of >15 mm Hg the CRVT caliber did not change significantly (P=0.20) during the test., Conclusions: A physiological short-term IOP rise at lower levels of IOP elevation led to a widening of the CRVT, while at higher IOP values, the further IOP-rise may have compressed the retinal vein. Because of higher intraluminal pressure values, the retinal artery diameters were not affected by the IOP-rise.

Background: Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) is a rare hereditary disease presenting with distinct imaging features in middle-aged adults. This article describes the typical imaging features focusing on the longitudinal course of RVCL-S lesions., Methods: In this study six subjects (five male, five related) with RVCL-S were retrospectively included from two university hospitals. The median age of symptom onset was 40 ± 6 years. Magnetic resonance imaging (MRI) covering baseline and a median follow-up period of 33 months was reviewed in a structured way focusing on morphology, contrast enhancement and diffusion restriction of brain lesions., Results: All patients showed patchy, T2 hyperintense white matter lesions (mean number 7.7 ± 1.8) with a periventricular predominance at the frontal lobes (59%). In all subjects, rim-enhancing white matter lesions with temporary diffusion restriction were present for a mean of 5.0 ± 3.9 months. Median duration of blood brain barrier disruption was 20 months., Conclusion: Periventricular patchy white matter lesions in the frontal lobes as well as rim-enhancing lesions with prolonged diffusion restriction and long-lasting contrast enhancement are characteristic imaging findings in RCVL-S and can be helpful in the differential diagnosis.

Retinal vascular tortuosity may occur in a wide range of ocular disorders. When retinal vascular tortuosity involves both arteries and veins, and presents unilaterally and without hemorrhage, a diagnosis of Wyburn Mason syndrome (WMS) should be considered due to the potential morbidity and mortality associated with cerebral involvement. Magnetic resonance imaging (MRI) and MRI angiography (MRA) are important tools for identifying cerebral arteriovenous malformations (AVMs), but these imaging modalities have limited spatial resolution to detect very small vascular lesions. Annular array contact ocular ultrasound is a new imaging modality capable of detecting small intraorbital AVMs. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:239-243.]., (Copyright 2020, SLACK Incorporated.)

Purpose: Dynamic assessment of retinal vascular characteristics can aid in identifying glaucoma-specific biomarkers. More specifically, a loss of spontaneous retinal venous pulsations (SVPs) has been reported in glaucoma, but a lack of readily available tools has limited the ability to explore the full potential of SVP analysis in glaucoma assessment. Advancements in smart technology have paved the way for the development of portable, noninvasive, and inexpensive imaging modalities. By combining off-the-shelf optical elements and smart devices, the current study aims to determine whether SVPs can be detected and quantified using a novel tablet-based ophthalmoscope in glaucoma and glaucoma suspects., Methods: Thirty patients, including 21 with confirmed glaucoma (9 men; average age 75 ± 8 years) and 9 glaucoma suspects (5 men; average age 64 ± 9 years), were studied. All patients had intraocular pressure measurements, Humphrey visual field assessment, optical coherence tomography, and a 10-second videoscopy of the retinal circulation. The retinal vasculature recordings (46° field of view at 30 frames per second) were analyzed to extract SVP amplitudes., Results: SVPs were detected and quantified in 100% of patients with glaucoma and those with suspected glaucoma using the novel device. The average SVP amplitudes in glaucoma and glaucoma suspects were 42.6% ± 10.7% and 34% ± 6.7%, respectively., Conclusions: Our results suggest that a novel tablet-based ophthalmoscope can aid in documenting and objectively quantifying SVPs in all patients., Translational Relevance: Outcomes of this study provide an innovative, portable, noninvasive, and inexpensive solution for objective assessment of SVPs, which may have clinical relevance in glaucoma screening., Competing Interests: Disclosure: S. Shariflou, None; A. Agar, None; K. Rose, None; C. Bowd, None; S.M. Golzan, None, (Copyright 2020 The Authors.)

Purpose: Examine associations between the vasculature at arteriovenous (AV) crossings and the onset of branch retinal vein occlusion (BRVO)., Methods: We included 78 patients with major BRVO, 35 patients with macular BRVO, and 110 controls without BRVO and determined the vessel positions at AV crossings, where the first- or second-order branches of the retinal veins associate, using a viewing angle of 12 × 12 mm2 in optical coherence tomography angiography (OCTA)., Results: We reviewed 1349 and 1276 AV crossings in BRVO patients and control subjects, respectively. The proportions of venous overcrossing were 26.5%, 28.6%, and 26.8% at non-causative crossings in BRVO eyes, non-BRVO fellow eyes, and unaffected control eyes, respectively; however, the rate of venous overcrossings at the causative crossings was 45.1%. In OCTA analyses, we divided the branches into macular- or non-macular veins. The rate of venous overcrossing was 52.5% at causative crossings in major BRVO but was 28.6% in macular BRVO. Odds ratios for whether venous overcrossing was a risk factor for BRVO were 3.09 (95% confidence interval [CI], 1.96-4.88) and 0.94 (95% CI, 0.44-2.00) for non-macular veins and macular veins, respectively. The patients with major BRVO caused by venous overcrossing were younger than patients for whom the cause was arterial overcrossing (P < 0.001). The onset of macular BRVO did not differ between crossing patterns at causative crossings (P = 0.60)., Conclusions: In eyes with BRVO, venous overcrossing was a common angiographic feature at causative crossings and might be a risk factor for major BRVO onset.