Acute macular neuroretinopathy following Valsalva manoeuvre: an insight into the pathophysiology.

Acute macular neuroretinopathy (AMN) affects the outer retina and is most likely induced by non-inflammatory ischaemia of the retinal deep capillary plexus and choriocapillaris. A man in his early 20s developed Valsalva retinopathy following weightlifting at the gym and presented with blurring of vision in the left eye 1 month after the initial retinal haemorrhages had resolved. A diffuse, purplish, donut-shaped, perifoveal lesion was seen on funduscopy and was well defined by an optical coherence tomography angiography (OCTA) en face image in the left eye. Outer retinal changes on optical coherence tomography (OCT) and a dense co-localised scotoma on a visual field (VF) examination confirmed the diagnosis of AMN, and the patient was started on a tapering dose of oral steroids. Improvement was seen in OCT, OCTA and VF during the 6-month follow-up visit. The use of OCTA en face imaging enabled the accurate identification of the lesion in the affected layers of the retina.

Visual Field Dependence Persists in Age-Related Central Visual Field Loss.

Purpose: To examine whether the age-related increase in visual field dependence persists in older adults with central field loss (CFL). Methods: Twenty individuals with CFL were grouped into participants with age-related binocular CFL (CFL, n = 9), age-related monocular CFL/relative scotomata (mCFL, n = 8), and CFL occurring at a young age (yCFL, n = 3). Seventeen controls were age-matched to the older CFL groups (OA) and three to the yCFL group (yOA). Participants judged the tilt direction of a rod presented at various orientations under conditions with and without a visual reference. Visual field dependence was determined as the difference in judgment bias between trials with and without the visual reference. Visual field dependence was examined between groups and relative to visual acuity and contrast sensitivity. Results: All older groups performed similarly without the visual reference. The CFL group showed greater visual field dependence than the OA group (Mann-Whitney U test; U = 39, P = 0.045). However, there was no group difference when considering all three older groups (Kruskal-Wallis ANOVA; H(2, N = 34) = 4.31, P = 0.116). Poorer contrast sensitivity correlated with greater visual field dependence (P = 0.017; ρ = -0.43). Conclusions: Visual field dependence persists in older adults with CFL and seems exacerbated in those with dense binocular scotomata. This could be attributed to the sensitivity of the spared peripheral retina to orientation and motion cues. The relationship with contrast sensitivity further suggests that a decline in visual function is associated with an increase in visual field dependence beyond the effects of normal aging. These observations can guide tailored care and rehabilitation in older adults with CFL.

Visual Field Deficits in Albinism in Comparison to Idiopathic Infantile Nystagmus.

Purpose: This is the first systematic comparison of visual field (VF) deficits in people with albinism (PwA) and idiopathic infantile nystagmus (PwIIN) using static perimetry. We also compare best-corrected visual acuity (BCVA) and optical coherence tomography measures of the fovea, parafovea, and circumpapillary retinal nerve fiber layer in PwA. Methods: VF testing was performed on 62 PwA and 36 PwIIN using a Humphrey Field Analyzer (SITA FAST 24-2). Mean detection thresholds for each eye were calculated, along with quadrants and central measures. Retinal layers were manually segmented in the macular region. Results: Mean detection thresholds were significantly lower than normative values for PwA (-3.10 ± 1.67 dB, P << 0.0001) and PwIIN (-1.70 ± 1.54 dB, P < 0.0001). Mean detection thresholds were significantly lower in PwA compared to PwIIN (P < 0.0001) and significantly worse for left compared to right eyes in PwA (P = 0.0002) but not in PwIIN (P = 0.37). In PwA, the superior nasal VF was significantly worse than other quadrants (P < 0.05). PwIIN appeared to show a mild relative arcuate scotoma. In PwA, central detection thresholds were correlated with foveal changes in the inner and outer retina. VF was strongly correlated to BCVA in both groups. Conclusions: Clear peripheral and central VF deficits exist in PwA and PwIIN, and static VF results need to be interpreted with caution clinically. Since PwA exhibit considerably lower detection thresholds compared to PwIIN, VF defects are unlikely to be due to nystagmus in PwA. In addition to horizontal VF asymmetry, PwA exhibit both vertical and interocular asymmetries, which needs further exploration.

Tuberculum meningioma with recovery of glaucoma-like visual field defects after chiasmal decompression: a case report.

BACKGROUND: To report a case of tuberculum meningioma with recovery of glaucoma-like visual field defects after chiasmal decompression. CASE PRESENTATION: A 39-year-old woman presenting with headache was found to have bilateral arcuate retinal nerve fiber layer (RNFL) thinning on optical coherence tomography (OCT) with a corresponding arcuate scotomas consistent with glaucomatous change. However a suprasellar tumor compressing the anterior chiasm from below was found on magnetic resonance imaging of the brain. After resection of the mass, which was diagnosed as meningothelial meningioma by the pathological examination, the glaucoma-like visual field defects resolved despite the RNFL thinning on the OCT showing no improvement. CONCLUSIONS: Chiasmal compression may mimic glaucoma and produce arcuate scotoma rather than temporal visual field loss. There is a possibility that the development of chiasmal compression somehow converted preperimetric glaucoma into a more advanced form accompanied by visual field defects and that the glaucoma reverted to the preperimetric state after chiasmal decompression.

The characteristics of acute macular neuroretinopathy following COVID-19 infection.

BACKGROUND: In this study, we report a case series of acute macular neuroretinopathy (AMN) associated with COVID-19 infection. METHODS: This retrospective observational study was conducted at Beijing Tongren Hospital. We reviewed patients who were diagnosed with AMN within one month of testing positive for COVID-19 using real-time reverse transcription-polymerase chain reaction (RT-PCR). RESULTS: A total of 11 AMN patients (20 eyes) were included in the study. The mean age was 33.8 ± 12.6 years. The average interval between a positive COVID-19 PCR test and the onset of ocular symptoms was 2.8 ± 2.5 days. The mean follow-up period for the patients was 12.5 ± 3.8 weeks. Imaging characteristics of AMN patients following COVID-19 infection included areas of low reflectivity on near-infrared reflectance (NIR) imaging, hyperreflective lesions at the level of the outer plexiform layer (OPL) and outer nuclear layer (ONL) and disruption of the ellipsoid zone (EZ) on spectral domain optical coherence tomography (SD-OCT) B-scans. Visual field examinations revealed parafoveal scotomas that closely corresponded to the clinical lesions. Optical coherence tomography angiography (OCT-A) demonstrated impaired perfusion in the deep retinal vascular plexus. Fluorescein angiography (FA), indocyanine green angiography (ICGA), and spontaneous fundus autofluorescence showed no significant abnormalities. During follow-up, partial improvement in retinal lesions was observed in NIR imaging and SD-OCT in some patients, but a proportion of patients still exhibited persistent retinal damage and no improvement in visual field scotomas. CONCLUSION: COVID-19-related AMN share similar clinical and imaging features with AMN due to other causes, as evidenced by the persistent presence of visual field scotomas over a longer duration. TRAIL REGISTRATION: https://www.chictr.org.cn/ ; identifier: ChiCTR2100044365.

Retinopathy Associated With Coxsackie B Virus Infection.

This case report describes a diagnosis of unilateral retinopathy secondary to coxsackie B virus in a male patient aged 41 years who presented with a central scotoma and blistering rash of the hands, feet, and mouth for 4 days.

Bilateral Acute Macular Neuroretinopathy (AMN) after COVID-19 and its Clinical Course. / Bilaterale akute makuläre Neuroretinopathie (AMN) nach COVID-19 und Verlauf.

Acute macular neuroretinopathy (AMN) is a rare disease entity. It is mainly observed in young women with a history of influenza-like infection or who have been taking oral contraceptives for several years. Patients typically describe subjective visual deterioration and mono- or bilateral paracentral relative scotomas. In some cases, funduscopic ophthalmic examination may reveal subtle sharply demarcated flat lesions of reddish-brown or orange colour in the macular region. Diagnosis is usually made by near-infrared fundus imaging which shows hyporeflective areas, and SD-OCT imaging which manifests changes in the outer retinal layers. In the following, three patient cases with bilateral AMN are described which occurred in direct temporal relationship to a recent SARS-CoV-2 infection.

Caronia Glaucoma Card Versus Amsler Grid for Monitoring Patients With Advanced Glaucoma.

PRCIS: Caronia Glaucoma Card (CGC) design incorporates a vertical and horizontal line and seven concentric circles corresponding to 1 o arc at normal reading distance. The study showed that the CGC is preferred by study patients compared to Amsler Grid and can detect central scotomas. PURPOSE: Evaluate patient acceptance and efficacy of the Caronia Glaucoma Card (CGC) in monitoring patients with severe glaucoma. METHODS: A retrospective study of consecutive patients with severe glaucoma who agreed to monitor their visual function. Patients were given a two-sided sheet of paper. An Amsler grid was printed on one side, and a CGC was printed on the other side. The patient was given verbal and written instructions on how to perform each test. They were asked to use either one or both tests to monitor their visual function on a weekly basis. Upon return, they were asked which test they preferred and if they appreciated or noticed any change in their scotoma. Patient comments were recorded. RESULTS: Sixty-seven patients were given the test card whose reported past ocular history consisted solely of severe glaucoma. Seventeen patients were eventually excluded. Fourteen patients had 1 eye that fulfilled the inclusion criteria for the study, while the other eye was excluded. Eighty-six eyes of fifty patients satisfied the inclusion criteria and were enrolled in the study. Thirty of the patients were male (60%) and 20 were female (40%). The average age was 71.8 +/- 10.7 years. They all carried a diagnosis of severe glaucoma. When asked the question which test design they preferred to use, 30 patients preferred the CGC (60%), while 8 patients preferred the Amsler grid (16%). Twelve patients found no difference between the 2 test designs (24%). [χ 2 (2, 50)=16.480, ρ=0.000]. Five patients noticed a change in their scotoma while using the card (5.8%, 5 of 86 eyes). All were exclusively using the CGC. CONCLUSION: The CGC is a convenient and inexpensive tool that was able to detect a change in scotoma. It may be helpful to add to the armamentarium to monitor glaucoma and its possible progression in patients with advanced disease.

Detection and characterisation of visual field defects using Octopus perimetry in congenital glaucoma.

PURPOSE: To detect and characterise visual field (VF) defects using static Octopus perimetry in patients with primary congenital glaucoma (PCG) and to determine VF quality and time duration. MATERIAL AND METHODS: Eighty-eight eyes of 70 patients diagnosed with PCG were included. Assessments were performed using an Octopus 900 and each eye was assessed with the tendency-oriented perimetry (G-TOP) algorithm. Quantitative VF data were collected: quality data (false positive and negative response, and time duration) and results of mean deviation (MD) and square root of loss variance (sLV). Qualitative data were collected: the presence of diffuse and localized defects, the affected hemifield and grade of defects using the Aulhorn and Karmeyer classification. Correlations between perimetric results and clinical variables were analysed. RESULTS: Median age was 11 (8-17) years. 65.9% (58/88) of PCG eyes showed VF defects. Diffuse defects were observed in 10/58 eyes (16.94%) (mean MD = 23.92 [SD: 2.52]) dB) and localized defects in 48/58 eyes (82.75%). The most frequent defect was spot-like/stroke-like/incipient paracentral scotoma (n = 15), nasal step (n = 8), adding arcuate defect (n = 2), half ring-shaped (n = 13) and concentric defect with a central island (n = 9). And the most frequent affected visual hemifield was inferior hemifield. Mean test duration was 2 min 12 s (SD: 21.6 s). MD and sLV values were correlated with best corrected visual acuity (BCVA), cup to disc ratio and number of antiglaucoma surgeries (all P < .001). CONCLUSION: A high number of diffuse and localized defects were identified using Octopus perimetry in PCG patients. The most frequent defect was paracentral scotoma and inferior hemifield was the most affected.

MULTIPLE EVANESCENT WHITE DOT SYNDROME FROM A PATIENT'S PERSPECTIVE.

PURPOSE: Multiple evanescent white dot syndrome is a self-limiting inflammatory condition of the outer retina. Only little information is available how patients experience their symptoms. METHOD: We report a case of a 28-year-old male graphic designer with multiple evanescent white dot syndrome, who precisely illustrated the development and course of the characteristic symptoms while looking at the ceiling, at a face and on his phone. RESULT: At onset, the scotoma was sparking/shiny and appeared on the temporal field of view, consistent with an enlarged blind spot. Over the course of the disease, the scotoma decreased in intensity and moved superiorly and nasally until it completely faded. CONCLUSION: Illustrations from a patient's perspective over the course of the disease can be beneficial for physicians and other multiple evanescent white dot syndrome patients for a better understanding and monitoring of their disease.

High false-positive response in perimetry.

Background: In Humphrey visual field analyzer, the false-positive (FP) responses imply that the patient has pressed the response button despite no stimulus being seen at the time of response and FP rates >15% are flagged. The classical "Trigger happy" visual field has increased fixation loss, very high threshold retinal sensitivity with the values in supernormal range, "white scotoma" on grayscale map, high positive mean deviation (MD), glaucoma hemifield test (GHT) gives classification of "abnormally high sensitivity, 'Excessive high false positive' message is displayed," and pattern deviation probability plot has more defects than total deviation probability plot known as "reverse cataract pattern." However, these classical findings are not seen in all the cases of FP as the same thumb rule cannot be applied to all the visual fields with high FP. Purpose: This video emphasizes the significance of careful examination of all the parameters in a visual field printout of high FP to interpret the test results and the caution needed when an FP response is seen in a patient with advanced glaucoma. Synopsis: The video presents some interesting visual fields in normal and glaucoma patients and the effect of high FP responses on MD, the different classification messages displayed for GHT, patterns of total deviation probability plot and pattern deviation probability plot, and how to identify the hidden FP. Highlights: This video highlights the importance of careful examination of all the parameters in a visual field printout to interpret the test results. One should be especially cautious when an FP response is noted in a patient with advanced glaucoma, as the retinal sensitivity values may not be in supernormal range but are significantly affected by the increased FP. Clinician should be able to identify this and repeat the test as high FP reponses can lead to underestimation of visual field loss. Video link: https://youtu.be/T2SGZf16UzA.

OUTER RETINOPATHY AND MICROANGIOPATHY IN ACUTE MYELOGENOUS LEUKEMIA.

PURPOSE: To describe a patient with acute myelogenous leukemia who presented with a recurrent, bilateral, outer retinopathy, before and after consolidative peripheral blood stem cell transplantation complicated by chronic graft-versus-host disease. METHODS: This is a retrospective review of records from a 23-year-old woman with acute myelogenous leukemia who underwent comprehensive ophthalmic evaluations for over a year including chromatic perimetry and multifocal electroretinograms, imaging with spectral domain optical coherence tomography, near-infrared and short-wavelength fundus reflectance and autofluorescence, fluorescein and optical coherence tomography angiography. RESULTS: The patient presented with recurrent, unilateral paracentral scotomas. There was localized loss of inner segment ellipsoid (EZ) and photoreceptor outer segment signals (IZ) in the pericentral retina of both eyes co-localizing with hyperreflective lesions on near-infrared reflectance. She subsequently lost vision (visual acuity = 20/200) in the right eye a year after consolidative peripheral blood stem cell transplantation complicated by steroid-resistant-chronic graft-versus-host disease. There was loss of the EZ and IZ signals corresponding to a dense central cone scotoma and multifocal electroretinograms depression. Near-infrared autofluorescence, fluorescein and optical coherence tomography angiography were within normal limits. Visual acuity (20/20) and retinal sensitivities improved with restoration of the EZ/IZ signals after oral prednisone and intravenous rituximab, but left a residual photoreceptor loss and paracentral scotoma. CONCLUSION: We propose that an immune-mediated microangiopathy may explain the protracted, recurrent course of primary photoreceptor abnormalities in our patient, which was further complicated by manifestations of chronic graft-versus-host disease following consolidative peripheral blood stem cell transplantation. Outer retinal findings previously documented in leukemia may be explained by a similar mechanism.

Altered Eye Movements During Reading With Simulated Central and Peripheral Visual Field Defects.

Purpose: Although foveal vision provides fine spatial information, parafoveal and peripheral vision are also known to be important for efficient reading behaviors. Here we systematically investigate how different types and sizes of visual field defects affect the way visual information is acquired via eye movements during reading. Methods: Using gaze-contingent displays, simulated scotomas were induced in 24 adults with normal or corrected-to-normal vision during a reading task. The study design included peripheral and central scotomas of varying sizes (aperture or scotoma size of 2°, 4°, 6°, 8°, and 10°) and no-scotoma conditions. Eye movements (e.g., forward/backward saccades, fixations, microsaccades) were plotted as a function of either the aperture or scotoma size, and their relationships were characterized by the best fitting model. Results: When the aperture size of the peripheral scotoma decreased below 6° (11 visible letters), there were significant decreases in saccade amplitude and velocity, as well as substantial increases in fixation duration and the number of fixations. Its dependency on the aperture size is best characterized by an exponential decay or growth function in log-linear coordinates. However, saccade amplitude and velocity, fixation duration, and forward/regressive saccades increased more or less linearly with increasing central scotoma size in log-linear coordinates. Conclusions: Our results showed differential impacts of central and peripheral vision loss on reading behaviors while lending further support for the importance of foveal and parafoveal vision in reading. These apparently deviated oculomotor behaviors may in part reflect optimal reading strategies to compensate for the loss of visual information.

The Invisibility of Scotomas I: The Carving Hypothesis.

SIGNIFICANCE: Veridical depictions of scene appearance with scotomas allow better understanding of the impact of field loss and may improve the development and implementation of rehabilitation. Explanation and depiction of the invisibility of scotoma may lead to patients' understanding and thus better compliance with related treatments. PURPOSE: Simulations of perception with scotomas guide training, patient education, and rehabilitation research. Most simulations incorrectly depict scotomas as black patches, although the scotomas and the missing contents are usually invisible to patients. We present a novel approach to capture the reported appearance of scenes with scotomas. METHODS: We applied a content-aware image resizing algorithm to carve out the content elided under the scotomas. With video sequences, we show how and why eye movements fail to increase the visibility of the carved scotomas. RESULTS: Numerous effects, reported by patients, emerge naturally from the scotoma carving. Carving-eliminated scotomas over natural images are barely visible, despite causing substantial distortions. Low resolution and contrast sensitivity at farther eccentricities and saccadic blur reduce the visibility of the distortions. In a walking scenario, static objects moving smoothly to the periphery disappear into and then reemerge out of peripheral scotomas, invisibly. CONCLUSIONS: Scotoma carving provides a viable hypothetical simulation of vision with scotomas due to loss of neurons at the retinal ganglion cell level and higher. As a hypothesis, it generates predictions that lend themselves to future clinical testing. The different effects of scotomas due to loss of photoreceptors are left for follow-up work.

Case Report: An Unusual Presentation of Leber's Hereditary Optic Neuropathy.

SIGNIFICANCE: Leber's hereditary optic neuropathy (LHON) is often associated with onset in the young, adult male demographic. This case report serves as a reminder that it can affect both sexes with onset into middle age. PURPOSE: Leber's hereditary optic neuropathy is a maternally inherited mitochondrial disorder that typically affects men during young adulthood. It presents with a rapid, yet painless loss of vision, with the fellow eye often affected within a few months. The optic neuropathy causes a dense central scotoma with visual acuities reduced to less than 20/400. CASE REPORT: A 60-year-old White woman presented with reports of decreased vision in both eyes for the previous 2 months. She had been followed up for the previous 5 years for glaucoma suspect monitoring, with full fields and normal optical coherence tomography scans. Entering visual acuity was finger counting at 1 m in the right eye and 20/100 in the left eye. Pupil testing revealed a grade 1 relative afferent pupillary defect in the right eye. Dilated fundus examination revealed stable moderate optic nerve cupping and intact neuroretinal rim tissue. Humphrey 24-2 Swedish Interactive Thresholding Algorithm standard visual field testing showed a significant superior altitudinal defect and inferior paracentral defect in the right eye and a partial superior arcuate in the left eye. The result of the MRI with contrast of the head and orbits was normal. A history of alcoholism was elicited, and LHON testing revealed positive 11778 mutation at homoplasmy. CONCLUSIONS: Although still uncommon, presentation of LHON in a middle-aged woman is possible and should be considered a viable differential diagnosis when individuals present with painless vision loss and central/centrocecal scotomas.

Detecting progression of acute zonal occult outer retinopathy (AZOOR) with optical coherence tomography angiography: A case report.

CLINICAL CASE: A 48-year-old woman with persistent superotemporal scotomas and photopsias for 2 months, and depigmented zones in the retina of both eyes with a trizonal pattern on multimodal imaging. Brain magnetic resonance imaging, positron emission tomography, antiretinal antibodies, immunological, infectious and tumor markers tests were negative, thus acute zonal occult outer retinopathy was diagnosed. Patient was treated with adalimumab. Nevertheless, 19 months later symptoms increased, and progression was detected on optic coherence tomography angiography, as well as in Humphrey visual field test and electroretinogram, thus, mycophenolate mofetil was added showing improvement and stabilization of the disease in a 4-year follow-up. DISCUSSION: Optic coherence tomography angiography may be a potential tool to monitor progression and response to treatment in addition to other imaging modalities in acute zonal occult outer retinopathy, and the combination of adalimumab and mycophenolate may be useful in recurrent disease.