Chorioretinopathy presenting as bitemporal hemianopia.

INTRODUCTION: Bitemporal hemianopia is usually caused by chiasmal pathology. Rarely, chorioretinal lesions may develop symmetrically in both eyes and mimic chiasmopathy. METHODS: This case series included three patients who presented to a tertiary neuro-ophthalmology centre with bitemporal hemianopic defects between 2021 and 2023 and were subsequently diagnosed with bilateral chorioretinopathy. All patients received comprehensive examinations from a fellowship-trained neuro-ophthalmologist and uveitis specialist to rule out other causes of visual dysfunction. RESULTS: Three males aged 64, 62, and 72 years were included. All patients showed bitemporal hemianopic defects crossing the vertical midline on automated perimetry and binasal thinning of the macular ganglion cell complex on spectral-domain optical coherence tomography (OCT). Fundus autofluorescence (FAF) showed classical features of acute zonal occult outer retinopathy (AZOOR) in two patients and central serous chorioretinopathy (CSCR) in another. AZOOR diagnosis was preceded by neuroimaging in both cases, whereas the patient with CSCR had longstanding, electroretinography-confirmed lesions and did not require neuroimaging. Fundus appearance and visual field defects remained stable in all patients across 3-6 months of follow-up. CONCLUSIONS: Bilateral chorioretinopathy should be considered in the differential diagnosis of bitemporal hemianopia in specific cases, including when visual field defects cross the vertical midline and when neuroimaging fails to reveal chiasmal pathology. FAF and macular OCT have high diagnostic yield as initial investigations.

Pearls & Oy-sters: Case Report of a Ruptured Suprasellar Dermoid Cyst Presenting With Junctional Scotoma of Traquair.

A 27-year-old woman with a known suprasellar dermoid cyst and stable idiopathic intracranial hypertension (IIH) presented with new monocular vision change and new-onset headaches. Formal visual field testing accurately identified progressive chiasmal compression due to her suprasellar dermoid cyst before radiographic change was appreciable on magnetic resonance imaging. Accurate interpretation of her visual field findings avoided the common pitfall of attributing new visual symptoms to her IIH diagnosis. This case highlights the value of recognizing visual field changes that localize to the chiasm even in patients with history of other ophthalmologic conditions.

[Bilateral acute regional occult outer retinopathy: a case report].

A 63-year-old female patient presented with "flashes of light in both eyes accompanied by decreased visual acuity for one year." Visual field examination revealed partial defects in the central 30° of the visual field in both eyes. Scanning laser ophthalmoscopy showed extensive atrophic lesions across the entire posterior pole of the retina in both eyes. Optical coherence tomography revealed atrophy and thinning of the retina in the macular regions, with significant atrophy of the photoreceptor inner segment and outer segment layer in the areas corresponding to the visual field defects. Fluorescein fundus angiography demonstrated thinning of the retinal arterioles. Early-phase imaging showed large window-like defects in the posterior retina with background fluorescence from choroidal macrovessels. In the late phase, patchy fluorescence staining with a circumferential hyperfluorescent area was observed. Pattern visual evoked potential and pattern electroretinography tests revealed a significant decrease in the P100 amplitude in both eyes. The patient was diagnosed with acute regional occult outer layer retinopathy in both eyes.

Impact and visualization of scotomatic glare in central visual field perception.

Strong monochromatic point light sources such as Light Emitting Diodes (LED) or Lasers have been increasingly used in recent decades. This also raises the risk of misuse resulting in glare phenomena and associated visual impairment. The objective of this prospective and partially blinded study was the visualization and characterization of glare-induced scotomas in visual field by dazzling with monochromatic point light sources in terms of disability and discomfort glare. Automated threshold perimetry under dazzling by LED exposure at three different wavelengths (470, 530 and 625 nm) and four different intensities (25, 50, 75, and 100%) was performed in 31 healthy subjects resulting in 434 visual field examinations. Visual disability was measured by sensitivity loss in the central 30°as compared to unexposed controls and visualized by reconstruction of mean visual fields for each group via backward-calculation. Psychological glare was assessed by subsequent questionnaire and evaluated based on the de Boer rating scale of discomfort. Increasing glare intensities resulted in a significant decrease in mean sensitivity for all wavelengths tested, paralleled by an increase of discomfort glare. The loss of sensitivity was scattered over all quadrants with accentuation of the corresponding mean exposure area. Reconstructed visual fields confirmed visual impairment in all quadrants at an extent of at least 30°. We conclude that even off-axis light exposure may affect central visual field perception. Our results extend previous research on directed light interaction and contribute in explaining its incapacitating impact on human performance.

Efficacy of a wearable night-vision aid in patients with concentric peripheral visual field loss: a randomized, crossover trial.

PURPOSE: To investigate the efficacy of our wearable night-vision aid in patients with concentric peripheral visual field loss. STUDY DESIGN: Prospective, single blind, three-group, and three-period crossover clinical study. METHODS: The study included patients with concentric peripheral visual field loss, a best-corrected visual acuity (decimal visual acuity) of 0.1 or higher in the better eye, and the presence of a central visual field. HOYA MW10 HiKARI® (HOYA Corporation), our original wearable night-vision aid, was used as the test device with three types of camera lenses (standard-, middle-, and wide-angle lenses). Under both bright and dark conditions, the angle of the horizontal visual field was measured using each of the three lens types for each group. The baseline angle was measured when each participant wore the night-vision aid (powered off). RESULTS: The study included 21 participants. Under bright condition, the perceived horizontal visual field was significantly wider than the baseline setup when using the standard-angle lens ("the standard lens"); the middle-angle lens ("the middle lens") was significantly wider than both the baseline setup and the standard lens; and the wide-angle lens ("the wide lens") was significantly wider than the other lenses. Under dark condition, the perceived horizontal visual field was again significantly wider when using the middle lens than the baseline setup and the standard lens, and when using the wide lens, the perceived horizontal visual field was again wider than when using the other lenses. The control in the bright condition was significantly wider (p < 0.001) than when used in the dark condition, while the standard-angle lens in the dark condition was significantly wider (p = 0.05) than when used in the bright condition. In regards to the middle and wide lenses, there was no statistically significant result emerging from either of the illumination conditions. CONCLUSION: Our wearable night-vision aid with a middle-angle or wide-angle lens appears to provide wider visual field images in patients with concentric peripheral visual field loss, regardless of whether the illumination conditions are bright or dark.

Peripapillary Versus Macular Thinning to Detect Progression According to Initial Visual Field Loss Location in Normal-Tension Glaucoma.

PURPOSE: To investigate the predictive capabilities of peripapillary retinal nerve fiber layer (pRNFL) and macular ganglion cell-inner plexiform layer (mGCIPL) thinning to detect visual field (VF) progression in normal-tension glaucoma patients with an initial parafoveal scotoma (IPFS) or nasal step (INS). DESIGN: Retrospective cohort study. METHODS: A total of 185 early-stage glaucoma eyes, followed for 10 years, were retrospectively stratified into IPFS and INS groups. Progressive pRNFL and mGCIPL thinning were assessed using spectral-domain optical coherence tomography and VF progression using both event- or trend-based analysis. Kaplan-Meier survival analysis compared VF survival in each VF phenotype with or without progressive pRNFL and mGCIPL thinning. Cox proportional regression analysis identified VF progression factors. RESULTS: VF progression was detected in 42 IPFS (n = 86) and 47 INS (n = 99) eyes. Among VF progressors, pRNFL thinning was significantly faster in INS group compared to IPFS group (P < .01), while mGCIPL thinning was similar (P = .16). At 5 years, eyes with progressive mGCIPL thinning showed significantly lower VF survival in both VF phenotypes (all P < .05). Progressive pRNFL thinning showed significantly lower VF survival only in INS eyes (P = .015). Cox multivariate regression revealed that mGCIPL thinning predicted subsequent VF progression in IPFS eyes, while mGCIPL and pRNFL thinning had significant associations with VF progression in INS eyes. CONCLUSIONS: mGCIPL outperforms pRNFL at early follow-up in detecting VF progression in IPFS eyes but not INS eyes. Appropriate selection of structural parameters (mGCIPL vs. pRNFL) maximizes early VF progression detection according to initial VF defect location.

Diagnostic and therapeutic complexity of Susac syndrome.

A 40-year-old man who attended the emergency department with a scotoma in right eye. He mentioned hearing difficulties and headache for months and he had sensory and motor deficits in the previous days. In the ophthalmic examination, the right eye had areas of arterial occlusion. MRI revealed hyperintense lesions. The patient was diagnosed with Susac syndrome. He was treated with systemic steroids, however, it was not enough to control the condition. Rituximab and intravenous immunoglobulins were added, which allowed the improvement of neurological symptoms, but the alteration of the visual field and the hearing defect did not recover. Early diagnosis of this pathology is essential, since delaying treatment can cause irreversible consequences. Sometimes it is difficult given the wide variety of symptoms and the course of the disease. Ocular manifestations may raise suspicion when the general symptoms are nonspecific.

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.

Assessing fall risk and equilibrium function in patients with age-related macular degeneration and glaucoma: An observational study.

BACKGROUND: Falls in older adults are a significant public health concern, and age-related macular degeneration (AMD) and glaucoma have been identified as potential visual risk factors. This study was designed to assess equilibrium function, fall risk, and fall-related self-efficacy (an individual's belief in their capacity to act in ways necessary to reach specific goals) in patients with AMD and glaucoma. METHODS: This observational study was performed at the Otorhinolaryngology Department of Shinseikai Toyama Hospital. The cohort comprised 60 participants (AMD; n = 30; median age, 76.0 years; and glaucoma; n = 30; median age, 64.5 years). Visual acuity and visual fields were assessed using the decimal best-corrected visual acuity and Humphrey visual field tests, respectively. The evaluation metrics included pathological eye movement analysis, bedside head impulse test, single-leg upright test, eye-tracking test, optokinetic nystagmus, and posturography. Furthermore, we administered questionnaires for fall risk determinants including the Dizziness Handicap Inventory, Activities-Specific Balance Confidence Scale, Falls Efficacy Scale-International, and Hospital Anxiety and Depression Scale. The collected data were analyzed using descriptive statistics, and Spearman's correlation analysis was employed to examine the interrelations among the equilibrium function, fall risk, and other pertinent variables. RESULTS: Most participants exhibited standard outcomes in equilibrium function evaluations. Visual acuity and field deficits had a minimal impact on subjective dizziness manifestations, degree of disability, and fall-related self-efficacy. Both groups predominantly showed high self-efficacy. No significant correlation was observed between visual acuity or field deficits and body equilibrium function or fall risk. However, greater peripheral visual field impairment was associated with a tendency for sensory reweighting from visual to somatosensory. CONCLUSION: Self-efficacy was higher and fall risk was relatively lower among patients with mild-to-moderate visual impairment, with a tendency for sensory reweighting from visual to somatosensory in those with greater peripheral visual field impairment. Further studies are required to validate these findings.

Reduced spatial attentional distribution in older adults.

Older adults show decline in visual search performance, but the underlying cause remains unclear. It has been suggested that older adults' altered performance may be related to reduced spatial attention to peripheral visual information compared with younger adults. In this study, 18 younger (M = 21.6 years) and 16 older (M = 69.1 years) participants performed pop-out and serial visual search tasks with variously sized gaze-contingent artificial central scotomas (3°, 5°, or 7° diameter). By occluding central vision, we measured how attention to the periphery was contributing to the search performance. We also tested the effect of target eccentricity on search times and eye movements. We hypothesized that, if attention is reduced primarily in the periphery in older adults, we would observe longer search times for more eccentric targets and with central occlusion. During the pop-out search, older adults showed a steeper decline in search performance with increasing eccentricity and central scotoma size compared with younger adults. In contrast, during the serial search, older adults had longer search times than younger adults overall, independent of target eccentricity and scotoma size. Longer search times were attributed to higher cost-per-item slopes, indicating increased difficulty in simultaneously processing complex symbols made up of separable features in aging, possibly stemming from challenges in spatially binding individual features. Altogether, our findings point to fewer attentional resources of simultaneous visual processing to distribute over space or separable features of objects, consistent with decreased dorsal visual stream functioning in aging.

Optic nerve head factors associated with initial central visual field defect in primary open-angle glaucoma.

We investigated optic nerve head factors associated with initial parafoveal scotoma (IPFS) in primary open-angle glaucoma. Eighty (80) patients with an IPFS and 84 patients with an initial nasal step (INS) were compared. Central retinal vascular trunk (CRVT) deviation from the Bruch's membrane opening (BMO) center was measured as a surrogate of lamina cribrosa (LC)/BMO offset, and its obliqueness was defined as the absolute value of angular deviation from the fovea-BMO axis. Proximity of retinal nerve fiber layer defect (RNFLD) was defined as the angular deviation of the inner RNFLD margin from the fovea-BMO axis. Microvasculature dropout (MvD) was defined as a focal sectoral capillary dropout with no visible microvascular network identified in the choroidal layer. Factors associated with IPFS, as compared with INS, were assessed using logistic regression analyses and conditional inference tree analysis. The IPFS group had more oblique CRVT offset (P < 0.001), RNFLD closer to the fovea (P < 0.001), more MvD (P < 0.001), and more LC defects (P < 0.001) compared to the INS group. In logistic regression analyses, obliqueness of CRVT offset (P = 0.002), RNFLD proximity (P < 0.001), and MvD (P = 0.001) were significant factors influencing the presence of IPFS. Conditional inference tree analysis showed that RNFLD closer to the fovea (P < 0.001) in the upper level, more oblique CRVT offset (P = 0.013) and presence of MvD (P = 0.001) in the lower level were associated with the probability of having IPFS. IPFS was associated with closer RNFLD location to the fovea when assessed from the BMO. Oblique LC/BMO offset may not only mask RNFLD proximity to the fovea due to a deviated funduscopic disc appearance, but also potentiate IPFS via focal LC defect and MvD.

Paracentral acute middle maculopathy and cotton wool spots in a patient with ocular migraine: A case report.

In this study, we report paracentral acute middle maculopathy (PAMM) and cotton wool spots (CWS) in a patient with ocular migraine. A 74-year-old man presented with persistent paracentral scotoma in the right eye that began a week prior. His visual acuity was 20/25 in the right eye and 20/40 in the left. Dilated fundoscopy revealed CWS in the right eye. Spectral-domain optical coherence tomography (SD-OCT) showed hyper-reflective bands in the inner nuclear layer corresponding to parafoveal lesions seen on near-infrared imaging in the right eye consistent with the diagnosis of PAMM. Further laboratory studies were unremarkable, and a transthoracic echocardiogram and a carotid ultrasound were unrevealing. The patient was started on brimonidine three times daily in both eyes. The patient reported subjective improvement in the paracentral scotoma and the absence of ocular migraine symptoms at two-month follow-up. We conclude from this case that PAMM and CWS can occur simultaneously in ocular migraine, and we suggest that retinal vascular changes associated with ocular migraine may contribute to ischemia underlying both entities. Additionally, we suggest a potential therapy in brimonidine due to its proposed beneficial effects on retinal vasculature and neuroprotection.

Acute retinal pigment epitheliitis during treatment of hyperprolactinaemia.

BACKGROUND: Acute retinal pigment epitheliitis (ARPE) is a rare, idiopathic and self-limiting disease. The article aims to present ARPE in a patient using D2 dopamine receptor agonists for the treatment of hyperprolactinemia. CASE PRESENTATION: A 28-year-old female during hyperprolactinaemia treatment suffered from a dyschromatopsia and a central visual field defect in the left eye. She noticed a deterioration of vision and discontinued the cabergoline administration. The woman had not been diagnosed with other chronic conditions and exhibited no symptoms of infection. Upon admission, the patient was subjected to a test for COVID-19, which was negative. The ophthalmological examination revealed a decrease in visual acuity to distance in the left eye, which amounted to 18/20 on the Snellen chart. A central scotoma was noted on the Amsler chart and a loss of pigment epithelium was visible on the fundus of the left eye. Fluorescein angiography showed a discrete window defect in the left one, with no signs of leakage. Optical coherence tomography (OCT) scans of the maculae revealed a characteristic change in the photoreceptor layer and retinal pigment epithelium (RPE) in the fovea in the left eye. The electrophysiological tests revealed decreased function of cells in macular region. A magnetic resonance imaging (MRI) of the head and orbits demonstrated an asymmetric pituitary gland without chiasm compression and discrete signal enhancement from the left optic nerve. The patient underwent observation during hospitalisation. She reported improved colour vision and a decreased scotoma in the centre of her visual field. In regular outpatient follow-ups, successive improvements in visual acuity, as well as a decreased RPE damage and outer photoreceptor layer loss during an OCT test were observed. CONCLUSIONS: A case of ARPE is reported in a patient taking medications for hyperprolactinemia. The role of dopamine receptor antagonists in the photoreceptor function and causation of ARPE needs further evaluation.

Current understanding of acute zonal occult outer retinopathy (AZOOR).

Acute Zonal Occult Outer Retinopathy (AZOOR) is characterized by sudden visual impairment, often presenting with scotoma and photopsia in young to middle-aged adults, showing a female predominance. This condition, distinct from genetic disorders like retinitis pigmentosa, lacks a hereditary basis and exhibits unique fundus changes and imaging features indicative of outer retinal dysfunction. Recent advancements have broadened our understanding, identifying variants like Acute Annular Outer Retinopathy (AAOR) and Multizonal outer retinopathy and retinal pigment epitheliopathy (MORR), each with specific clinical presentations and imaging characteristics. The diagnosis of AZOOR and its variants primarily relies on excluding other conditions through comprehensive evaluation, including imaging and serological testing. Treatment approaches, including the use of corticosteroids and immunosuppressives, remain debated, with some evidence suggesting benefits in the early stages. The prognosis of AZOOR varies, with most patients experiencing stabilization, although complications like choroidal neovascularization may occur, requiring targeted therapy. This manuscript elucidates the complexity of AZOOR, emphasizing the necessity of high clinical suspicion and the role of advanced imaging in diagnosis and management.

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.

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.

Measuring spatial visual loss in rats by retinotopic mapping of the superior colliculus using a novel multi-electrode array technique.

BACKGROUND: The retinotopic map property of the superior colliculus (SC) is a reliable indicator of visual functional changes in rodents. Electrophysiological mapping of the SC using a single electrode has been employed for measuring visual function in rat and mouse disease models. Single electrode mapping is highly laborious requiring long-term exposure to the SC surface and prolonged anesthetic conditions that can adversely affect the mapping data. NEW METHOD: To avoid the above-mentioned issues, we fabricated a fifty-six (56) electrode multi-electrode array (MEA) for rapid and reliable visual functional mapping of the SC. Since SC is a dome-shaped structure, the array was made of electrodes with dissimilar tip lengths to enable simultaneous and uniform penetration of the SC. RESULTS: SC mapping using the new MEA was conducted in retinal degenerate (RD) Royal College of Surgeons (RCS) rats and rats with focal retinal damage induced by green diode laser. For SC mapping, the MEA was advanced into the SC surface and the visual activities were recorded during full-filed light stimulation of the eye. Based on the morphological examination, the MEA electrodes covered most of the exposed SC area and penetrated the SC surface at a relatively uniform depth. MEA mapping in RCS rats (n=9) demonstrated progressive development of a scotoma in the SC that corresponded to the degree of photoreceptor loss. MEA mapping in the laser damaged rats demonstrated the presence of a scotoma in the SC area that corresponded to the location of retinal laser injury. COMPARISON WITH EXISTING METHODS AND CONCLUSIONS: The use of MEA for SC mapping is advantageous over single electrode recording by enabling faster recordings and reducing anesthesia time. This study establishes the feasibility of the MEA technique for rapid and efficient SC mapping, particularly advantageous for evaluating therapeutic effects in retinal degenerate rat disease models.

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.