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.

[Nonarteritic anterior ischemic optic neuropathy (nAION)]. / Nichtarteriitische anteriore ischämische Optikusneuropathie (nAION).

Nonarteritic anterior ischemic optic neuropathy (nAION) is the second most common degenerative disease of the optic nerve. The pathogenesis remains elusive. A transient ischemia in the short posterior ciliary arteries not triggered by thromboembolic events is suspected. The typical history of a sudden onset of scotoma without associated pain in conjunction with (sectorial) optic disc swelling, an afferent pupillary defect and a visual field defect are of decisive diagnostic importance. The most urgent diagnostic measure is the exclusion of arteritic AION. There are no proven treatment approaches. Frequently used but without clear study results, is the treatment with steroids and secondary prophylaxis with acetylsalicylic acid (ASA). Recurrence in the ipsilateral or contralateral eye is possible.

TRAUMA-RELATED ACUTE MACULAR NEURORETINOPATHY. A CASE REPORT.

AIMS: To introduce a case report and review the literature on trauma-related acute macular neuroretinopathy as an unusual etiology of acute macular neuroretinopathy. MATERIAL AND METHODS: A 24-year-old man presented with unilateral paracentral scotoma following non-ocular trauma in a car accident. The relative afferent pupillary defect was negative and the best corrected visual acuities of both eyes were 10/10 (by the Snellen chart scale). RESULTS: Retinoscopy revealed a reduced foveal reflex, along with a small pre-retinal hemorrhage over the mid-pathway of the supranasal arteriole. OCT images showed an obvious ellipsoid zone (EZ) layer disruption in the macula of the left eye. The infrared fundus photograph of the same eye revealed a distinct hyporeflective area involving the macula. On fundus angiography, no macular vascular lesion was detected. The scotoma persisted after 3 months follow-up. CONCLUSION: Non-ocular trauma including head or chest trauma without direct ocular injury accounts for most cases of trauma-related acute macular neuroretinopathy. It is important to distinguish this entity, given that there are also unremarkable findings in the retinal examination of these patients. Indeed, proper clinical suspicion leads to further suitable investigations and impedes other extraordinary images, which are the basic rules in the management of traumatic patients suffering multiple injuries and incurring medical expenses.

Coincident Acute Macular Neuroretinopathy and Paracentral Acute Middle Maculopathy in COVID-19.

An ophthalmology consultation was requested for a 29-year-old woman complaining of visual field defects. The patient had presented to the emergency department with cough and high fever one day earlier. Chest computed tomography demonstrated pneumonia and two severe acute respiratory syndrome coronavirus 2 polymerase chain reaction tests were positive. The patient had undergone renal transplantation 11 years ago due to glomerulonephritis. Best-corrected visual acuity (BCVA) was 20/40 in the right eye and 20/30 in the left eye. Fluorescein angiography showed macular hypoperfusion, and optical coherence tomography (OCT) showed hyperreflectivity in the inner nuclear, outer plexiform, and outer nuclear layers, as well as discontinuity of the ellipsoid zone. Perimetry confirmed bilateral central scotoma. Levels of D-dimer and fibrinogen were 0.86 g/mL and 435.6 g/mL, respectively. The patient was diagnosed as having concurrent acute macular neuroretinopathy and paracentral acute middle maculopathy and was given low-molecular-weight heparin treatment for one month. Her BCVA improved to 20/20 in both eyes, and regression was observed in the retinal findings, hyperreflectivity and ellipsoid zone disruption on OCT, and scotoma in perimetry. Inflammation, thrombosis, and glial involvement may play a role in the pathogenesis of retinal microvascular impairment in COVID-19.

Permanent damage of the inner retinal layers in a patient with migraine: a different case of paracentral acute middle maculopathy.

We report the case of a 20-year-old patient who presented a paracentral scotoma in her left eye that had not disappeared after a migraine attack two days before. Ocular examination showed two pale paracentral areas suggesting an acute ischemia insult. Several causes of retinal ischemia were ruled out and the patient was diagnosed with secondary retinal vasospasm. One year later, the patient suffers an atrophy of the inner layers of the affected retina and has a paracentral chronic scotoma in her left eye. Retinal vasospasms can result in irreversible lesions and chronic symptoms due to ischemia and cell destruction. This unusual case reports a permanent damage due to retinal vasospasm secondary to migraine attack. The finding of typical lesions and the history of migraine could be compatible with the diagnosis of paracentral acute middle maculopathy (PAMM).

Longitudinal Impact of Vision Impairment on Concern About Falling in People With Age-Related Macular Degeneration.

PURPOSE: To explore the longitudinal impact of central vision loss on concern about falling (CF), over a 12-month period, in people with age-related macular degeneration (AMD). METHODS: Participants included 60 community-dwelling older people (age, 79.7 ± 6.4 years) with central vision impairment due to AMD. Binocular high-contrast visual acuity, contrast sensitivity, and visual fields were assessed at baseline and at 12 months. CF was assessed at both time points using the Falls Efficacy Scale-International (FES-I). Sensorimotor function (sit to stand, knee extension, postural sway, and walking speed) and neuropsychological function (reaction time, symptoms of anxiety and depression) were also assessed at both time points using validated instruments. Falls data were collected using monthly diaries during the 12 months. RESULTS: CF increased by a small but significant amount over the 12-month follow-up (2.1 units; P = 0.01), with increasing prevalence of high levels of CF (FES-I score ≥ 23), from 48% at baseline to 65% at 12 months. Linear mixed models showed that reduced contrast sensitivity was significantly associated with increased concern about falling (P = 0.004), whereas declines in both visual acuity and contrast sensitivity during the follow-up period were associated with increases in CF over the 12-month follow-up (P = 0.041 and P = 0.054, respectively), independent of age, gender, falls history, or number of comorbidities. CONCLUSIONS: Higher levels of CF are common in older people with AMD, and levels increase over time; this increase is associated with declines in both visual acuity and contrast sensitivity. These findings highlight the need for regular assessment of both visual acuity and contrast sensitivity to identify those at greatest risk of developing higher CF. TRANSLATIONAL RELEVANCE: Routine assessment of visual acuity and contrast sensitivity in older people with AMD will assist in identifying those at risk of developing high CF.

Oclusión arterial asociada con un asa vascular prepapilar. Cambios anatómicos en el seguimiento a largo plazo / Arterial occlusion associated with a prepapillary vascular loop. Anatomical changes on the long term follow up

Paciente de 13 años de edad que presenta un escotoma del campo visual superior de 48 horas de evolución. Mejor agudeza visual corregida de 20/20 en ambos ojos. La exploración del fondo de ojo derecho reveló un asa vascular prepapilar torsionada asociada a un área de blanqueamiento retiniano y atenuación vascular tanto en el sector nasal como temporal, la cual no afectaba fóvea. Durante el seguimiento el asa vascular desaparece y es reemplazada por tejido glial sobre el disco óptico. La mejor agudeza visual corregida permaneció en 20/20. Este caso muestra el comportamiento del asa vascular posterior a la oclusión de la misma. La ausencia de flujo sanguíneo produce un colapso de las paredes arteriales, que con el tiempo llevan a la formación de tejido glial

We report the case of a 13-year-old patient who complains of an acute superior visual field scotoma in the last 48hours. Best corrected visual acuity (BCVA) was 20/20 in both eyes. The right eye fundus examination revealed torsion of a prepapillary loop in the inferior branch of the retinal artery, associated with a arterial vascular attenuation and whitenning of the inferior retina that involved both nasal and temporal branches but spared the foveal region. During the follow-up the vascular loop dissapeared and only glial tissue was seen in front of the optic nerve head. BCVA remained 20/20 in both eyes. This report shows the evolution of the vascular loop after an occlusion. The absence of blood flow produces a collapse of the arterial walls, in time the vascular loop is replaced by glial tissue

Visual Function at the Atrophic Border in Choroideremia Assessed with Adaptive Optics Microperimetry.

PURPOSE: Recent advances in retinal imaging allow visualization of structural abnormalities in retinal disease at the cellular level. This study used adaptive optics (AO) microperimetry to assess visual sensitivity with high spatial precision and to examine how function varies across 2 phenotypic features observed in choroideremia: atrophic lesion borders and outer retinal tubulations (ORTs). DESIGN: Cross-sectional study. PARTICIPANTS: Twelve choroideremia patients. METHODS: A custom AO scanning light ophthalmoscope (AOSLO) equipped with both confocal and nonconfocal split-detection imaging methods was used to image the photoreceptor inner and outer segment mosaics. For AO microperimetry, circular 550-nm stimuli were presented through the AOSLO system; stimuli were either 9.6 or 38.3 arcmin2 (approximately 60 or 15 times smaller than a Goldman III stimulus). Test locations were identified in structural images and stimuli were targeted to these locations using real-time retinal tracking combined with measurements of transverse chromatic aberration. Psychophysical detection thresholds were measured at the atrophic border in 12 patients. Additionally, visual sensitivity was probed along ORTs in 4 patients. MAIN OUTCOME MEASURE: Visual sensitivity thresholds measured with AO microperimetry at retinal locations corresponding to structural phenotypes observed on AOSLO retinal images. RESULTS: In choroideremia, sharp borders between intact central islands of the photoreceptor mosaic and complete atrophy of the outer retina and retinal pigment epithelium were observed in both split-detection and confocal structural images. Adaptive optics microperimetry at locations spanning these borders showed a commensurately sharp decrease in function, with readily measurable visual sensitivity on one side and dense scotoma on the other. These functional transitions often occurred over a distance smaller than the diameter of the Goldman III stimulus. Thresholds measured along ORTs showed dense scotoma over the tubule in all 4 participants, despite the visibility of remnant cone inner segments on the AO images. CONCLUSIONS: Choroideremia patients exhibited sharp functional transitions that collocated with structural transitions from intact to severely degenerated retina. We found no evidence of visual sensitivity over ORTs. Measuring cone function with high resolution offered insight into disease mechanisms and may enable precise assessment of whether experimental therapies, such as gene therapy, provide a functional benefit.

Arterial occlusion associated with a prepapillary vascular loop. Anatomical changes on the long term follow up. / Oclusión arterial asociada con un asa vascular prepapilar. Cambios anatómicos en el seguimiento a largo plazo.

We report the case of a 13-year-old patient who complains of an acute superior visual field scotoma in the last 48hours. Best corrected visual acuity (BCVA) was 20/20 in both eyes. The right eye fundus examination revealed torsion of a prepapillary loop in the inferior branch of the retinal artery, associated with a arterial vascular attenuation and whitenning of the inferior retina that involved both nasal and temporal branches but spared the foveal region. During the follow-up the vascular loop dissapeared and only glial tissue was seen in front of the optic nerve head. BCVA remained 20/20 in both eyes. This report shows the evolution of the vascular loop after an occlusion. The absence of blood flow produces a collapse of the arterial walls, in time the vascular loop is replaced by glial tissue.

Comparison of pattern electroretinograms of glaucoma patients with parafoveal scotoma versus peripheral nasal step.

Retinal ganglion cells are distributed disproportionately with retinal eccentricity. Pattern electroretinogram (PERG) stimuli resulted in reduced responses with more eccentric stimuli. Therefore, we investigated whether PERG amplitude is associated with the location of visual field (VF) defect in primary open-angle glaucoma. Data from Twenty-nine glaucoma patients with a parafoveal scotoma (PFS) within the central 10° of fixation, 23 glaucoma patients with a peripheral nasal step (PNS), and 27 normal control subjects were analyzed in this study. Electroretinograms (ERGs) were obtained using a commercial ERG stimulator (Neuro-ERG). The thickness of the ganglion cell-inner plexiform layer (GCIPL) was measured using spectral-domain optical coherence tomography. A lower N95 amplitude was observed in both PFS and PNS compared to the normal control (Both P < 0.001). The N95 amplitude of the PFS group was significantly lower than that of the PNS group (P = 0.034). Average GCIPL thickness correlated positively with N95 amplitude (r = 0.368, P = 0.002), but did not correlate significantly with global mean sensitivity (r = 0.228, P = 0.073) or mean deviation on 24-2 standard automated perimetry (r = 0.173, P = 0.176). In conclusion, parafoveal VF defects were associated with the lower PERG amplitude. Therefore, it is necessary to take into account the location of VF defects in evaluating PERGs of glaucoma patients.

Visual rehabilitation using microperimetric acoustic biofeedback training in individuals with central scotoma.

BACKGROUND: Patients with central scotoma have poor fixation stability and poor visual acuity. Acoustic biofeedback training can be an effective way to train such patients to eccentrically fixate. This study analyses the mean retinal sensitivity, saccadic velocity, and fixation stability after acoustic biofeedback training and shows correlation with age and scotoma size. METHODS: Patients with irreversible central scotoma in both the eyes secondary to macular diseases were selected. After undergoing comprehensive low vision assessment, 19 patients who were willing were recruited for the acoustic biofeedback training to the better eye in 10 sessions, using the MP-1 Microperimeter. Mean retinal sensitivity, saccadic velocity, fixation stability before and after the acoustic biofeedback were recorded. RESULTS: There were 17 men and two women. Ages ranged from 19-94 years (mean 54.63 ± 24.66). The scotoma size ranged from four to 20 degrees. Ten patients had age-related macular degeneration, four had Stargardt disease, three had traumatic macular scar, two had scarred myopic choroidal neovascular membrane, and one had myopic macular degeneration. The vision improved from 1.06 ± 0.36 to 0.86 ± 0.33 logMAR (p < 0.0001). The mean retinal sensitivity improved from 2.1 ± 2.9 dB to 2.7 ± 3.1 dB (p = 0.01), with negative correlation with age (p = 0.01) and scotoma size (p = 0.02). Fixation stability improved with reduction in the bivariate contour ellipse area (p = 0.01). It showed negative correlation with age (p = 0.02) and scotoma size (p = 0.10). The saccadic velocity reduced from 0.34°/second to 0.26°/second but was not significant (p > 0.99). The majority (58 per cent) had their preferred retinal locus superior to the fovea. There was good agreement between bivariate contour ellipse area and MP-1 Microperimeter inbuilt fixation parameters. The effect was maintained at six months with slight reduction in fixation stability. CONCLUSION: Acoustic biofeedback can improve fixation behaviour, visual acuity and retinal sensitivity in patients with central scotoma. The results are better with younger age and smaller scotoma size.

Recurrent focal choroidal excavation following multiple evanescent white dot syndrome (MEWDS) associated with acute idiopathic blind spot enlargement.

PURPOSE: To present a recurrent case of conforming focal choroidal excavation (FCE) following multiple evanescent white dot syndrome (MEWDS) in a 25-year-old woman. METHODS: Following spontaneous MEWDS sings resolution our patient noted a recurrent decrease in vision. Repeated OCT revealed elevation and mild disruption of RPE layer at fovea without previous angiographic MEWDS signs. At this time, short-term systemic steroid therapy was started and visual acuity became normal. RESULTS: Following quiescence of the new-onset phase, the conforming type of FCE located in inferior macula appeared in OCT. In the following next 2 years recurrence of presumptive focal subfoveal choriocapillaritis occurred for three times presenting with blurred vision. During every acute attack, above-mentioned FCE disappeared and returned back again after resolution of presumptive focal choriocapillaritis. CONCLUSIONS: This is the first and unique case of recurrent type of FCE following MEWDS. It seems to disappear during active phase of presumptive focal choriocapillaritis and then returns after the eye has become quiescent.

Usefulness of frequency doubling technology perimetry 24-2 in glaucoma with parafoveal scotoma.

The standard automated perimetry (SAP) 24-2 test cannot adequately test the paracentral region because test points are located sparsely in macular area where is crowded with retinal ganglion cells (RGCs), even though paracentral scotoma is clinically related to a risk of losing visual function. More sensitive visual field (VF) tests are needed to assess paracentral VF defects precisely. We investigated the structure-function relationship on the SAP 10-2 test and the frequency doubling technology (FDT) 24-2 test as well as the SAP 24-2 test in glaucoma with parafoveal scotoma (PFS). Glaucoma patients with PFS (134 patients) were included in this cross-sectional study. Sub-analysis was performed with isolated PFS (51 patients). Global and sectoral mean sensitivities (MS) were evaluated using SAP 24-2, 10-2, and FDT 24-2 program. MS was analyzed as dB or unlogged 1/lambert (SAP) or 1/Michelson contrast (FDT). Ganglion cell-inner plexiform layer (GCIPL) thickness was measured using spectral domain optical coherence tomography. Topographic relationships between the structure and the function were analyzed. In the total PFS group, good structure-function correlations were found in all zones with SAP 24-2, 10-2, and FDT 24-2 test. For glaucoma with isolated PFS, average GCIPL thickness was significantly correlated with central cluster MS (dB) using the SAP 10-2 test (r = 0.279, P = .047) and the FDT 24-2 test (r = 0.289, P = .039), but not the SAP 24-2 test (r = 0.264, P = .061). Topographically, the FDT 24-2 test showed significant correlations in all sectors between sectoral MS and corresponding GCIPL thickness. With regard to the SAP 10-2 test, there was significant topographical structure-function correlations for the superotemporal, inferotemporal, and inferonasal sectors. For SAP 24-2, only inferonasal GCIPL thickness was correlated with the corresponding VF sensitivity. Topographical structure-function on the macula was better with the SAP 10-2 test (superotemporal sector) and the FDT 24-2 test (superotemporal sector) than with the SAP 24-2 test in glaucoma with isolated PFS. In conclusion, FDT 24-2 and SAP 10-2 tests performed more favorably than the SAP 24-2 test in the structure-function relationship of glaucoma patients with isolated paracentral scotoma. FDT 24-2 tests can be another good option for detecting and monitoring RGC loss on the macular area while not missing VF defects outside the central 10°.

Peripapillary and Macular Vessel Density in Patients with Glaucoma and Single-Hemifield Visual Field Defect.

PURPOSE: To compare hemifield differences in the vessel density of the peripapillary and macula in open-angle glaucoma eyes with visual field (VF) defect confined to one hemifield using optical coherence tomography angiography (OCT-A). DESIGN: Cross-sectional study. PARTICIPANTS: A total of 58 eyes of 58 patients with glaucoma with VF loss confined to a single hemifield and 28 healthy eyes. METHODS: Retinal vasculature information was summarized as circumpapillary vessel density (cpVD) and perifoveal vessel density (pfVD). Circumpapillary retinal nerve fiber layer (cpRNFL) and macular ganglion cell complex (mGCC) thickness were calculated using spectral domain optical coherence tomography (SD OCT). Paired and unpaired t tests were used to evaluate differences between the perimetrically affected and intact hemiretinae and healthy hemiretinae. Linear regression analyses were performed to evaluate the associations between VF measures with vascular and structural measurements. MAIN OUTCOME MEASURES: Total and hemispheric cpVD, pfVD, cpRNFL, mGCC, and mean sensitivity (MS). RESULTS: Mean cpVD and pfVD in the intact hemiretinae of glaucoma eyes (59.0% and 51.1%, respectively) were higher than in the affected hemiretinae (54.7% and 48.3%, respectively; P < 0.001) but lower than in healthy eyes (62.4% and 53.8%, respectively; P < 0.001). Similar results were noted with cpRNFL and mGCC thickness measurements (P < 0.05 for both). The strongest associations between MS in the affected hemifields were found for cpVD (r = 0.707), followed by pfVD (r = 0.615), cpRNFL (r = 0.496), and mGCC (r = 0.482) in the corresponding hemiretinae (P < 0.001 for all). Moreover, the correlations in the intact hemifields between MS with cpVD and pfVD were higher (r = 0.450 and 0.403) than the correlations between MS and cpRNFL and mGCC thickness measurements (r = 0.340 and 0.290; P values <0.05 for all). CONCLUSIONS: Reduced peripapillary and macular vessel density was detectable in the perimetrically intact hemiretinae of glaucoma eyes with a single-hemifield defect. Vessel density attenuation in both affected and intact hemiretinae was associated with the extent of VF damage in the corresponding hemifields. Optical coherence tomography angiography potentially shows promise for identifying glaucomatous damage before focal VF defects are detectable.

[Migraine with visual aura]. / « Migraine ophtalmique ¼ ou migraine avec aura visuelle.

Migraine with visual aura is marked by recurrent episodes of transient visual disturbance, often followed by headaches. Its pathophysiology has not been fully understood, but visual auras might be related to a self-propagating wave of cortical depolarization called "cortical spreading depression", triggering a trigemino-vascular "storm" ultimately leading to headaches. The most specific visual symptom is the "fortification spectrum" consisting of glimmering jagged lines spreading from the center to the periphery, and leaving a transient scotoma in its wake. Other visual symptoms are numerous, ranging from elementary positive or negative visual phenomena to complex and elaborate hallucinations. The diagnosis can be made according to the International Classification of Headache Disorders revised in 2013. The main goal of the treatment is to relieve the patient's pain quickly and to decrease the frequency of the episodes.

Neuropatía óptica tras la cirugía de descompresión orbitaria / Optic neuropathy after orbital decompression surgery

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OCT Angiography Identification of Choroidal Neovascularization Secondary to Acute Zonal Occult Outer Retinopathy.

A 74-year-old female with acute zonal occult outer retinopathy presented with a new lesion suspicious for choroidal neovascularization (CNV) in her right eye. Optical coherence tomography angiography (OCTA) confirmed the presence of CNV. OCTA is a new imaging technique that may help guide diagnosis and management of choroidal neovascular membranes in uveitic diseases.

[Progressive scotoma with perception of photopsia]. / Progredientes Skotom mit Photopsiewahrnehmung.

Acute annular outer retinopathy (AAOR) is a very rare variant of acute zonal occult outer retinopathy (AZOOR). Both variants are characterized by the perception of a progressive visual field defect and can be accompanied by photopsia. In contrast to AZOOR, a progressive whitish and annular demarcation of the involved retina is observed in AAOR using ophthalmoscopy. Optical coherence tomography (OCT) reveals morphological changes predominantly in the outer retinal layers. Although there is no proof of an effective therapy, in the case presented here further progression could be stopped by a combination of antiviral and immunosuppressive therapy.