Paracentral Scotoma Research Articles

Sparkle in the eye: A rare case of Bietti\'s crystalline dystrophy

: Bietti’s Crystalline Dystrophy is an uncommon autosomal recessive tapetoretinal dystrophy resulting from a mutation in the CYP4V2 gene. It is defined by the existence of sub-epithelial corneal deposits at the limbus and small sized, crystalline looking deposits in the retina, especially at the posterior pole. This is bolstered by retinal pigment epithelial atrophy and the choriocapillaris.: In this report, we present the case of a 55-year-old male exhibiting reduced visual acuity, nyctalopia, impaired color vision, and yellow-white crystal deposition in the posterior pole of both eyes. Additionally, areas of retinal pigment epithelium atrophy and pigment clumping were observed. Spectral-domain optical coherence tomography (SD-OCT) of the macula revealed hyperreflective dots in the outer retinal layers, retinal pigment epithelium, and choroid, along with outer retinal tubulations. Electroretinography (ERG) results were subnormal, and visual field assessment indicated paracentral scotomas in both eyes. The patient has been prescribed spectacles and is under regular follow-up.: This case is highlighted due to its rarity in the lack of genetic association and underscores the significance of employing SD-OCT and electrophysiological studies for early disease diagnosis. Additionally, it emphasizes the role of genetic counselling in preventing the inheritance of this condition.

Persistent visual field deficit after half-fluence photodynamic therapy for chronic central serous chorioretinopathy.

To report a rare case of a persistent paracentral scotoma after half-fluence photodynamic therapy for chronic central serous chorioretinopathy. A 65-year-old man with a history of chronic central serous chorioretinopathy presented with a temporal paracentral visual distortion one week after undergoing repeat off-label treatment with half-fluence verteporfin photodynamic therapy for recurrent intraretinal fluid exudation in the right eye. Fundus photography, fundus autofluorescence, optical coherence tomography, fluorescein angiography, indocyanine green angiography, and automatic visual field testing were obtained. Humphrey visual field 10-2 demonstrated a focal temporal paracentral area of reduced sensitivity, which remained persistent on repeat testing up to 9 months. OCT macula and fluorescein angiography demonstrated subtle corresponding changes in the nasal macula, while indocyanine green angiography demonstrated no visible alteration in choroidal perfusion. Symptomatic scotoma in the location of laser treatment may be a rare complication of verteporfin photodynamic therapy even at reduced fluence, in the setting of repeat treatment in the same location, and may be identified and monitored with automated 10-2 visual field testing.

Macular Outer Retinal Atrophy Following Vitrectomy and Hypotony: Two Cases.

To characterize two cases of focal outer retinal atrophy and hypotony after vitrectomy. Retrospective chart review of two patients' records between 2019 and 2023. Patient 1 underwent vitrectomy, epiretinal membrane peel, and cataract extraction for visually significant macular pucker. She developed hypotony without a wound leak and was noted to have a focal parafoveal area of ellipsoid zone disruption by 1 week post-operatively, which evolved into outer retinal and chorioretinal atrophy within 6 weeks after surgery. This area of atrophy remained stable in size, but the patient later reported a paracentral scotoma. Patient 2 had multiple previous surgeries for retinal detachment with proliferative vitreoretinopathy. Seven years later, the IOL dislocated and was exchanged with scleral fixation of a new IOL. On post-operative day 1, he had hypotony with macular folds secondary to a leaking sclerotomy wound. The sclerotomies were sutured on post-operative day 3, and his intraocular pressure normalized. However, he developed a central, focal area of chorioretinal atrophy within 1 week of the initial surgery. The size of this area of atrophy remained stable over years but resulted in reduced central vision. Hypotony following vitrectomy may rarely predispose patients to the development of focal chorioretinal atrophy.

Acute Macular Neuroretinopathy Associated with COVID-19 Pandemic: A Real-world Observation Study

To evaluate the clinical and retinal imaging features of Chinese patients with acute macular neuroretinopathy (AMN) associated with COVID-19. A prospective observational study. Retinal imaging, including color fundus photography, near-infrared imaging (NIR), swept-source optical coherence tomography (SS-OCT), optical coherence tomography angiography (OCTA), and Humphrey perimetry, were conducted for each case. All cases were included within the first three months following the pandemic outbreak. A total of 12 male patients (36.36 %) and 21 female patients (63.64 %) were prospectively recruited, and 29 cases (87.88 %) were bilaterally affected. The median interval between the onset of fever and the appearance of ocular symptoms was two days (range, 0.5-5.0 days). Apart from the outer retinal changes typical of AMN, changes in the inner retinal layers were observed, including intraretinal hemorrhage (8.06 %), cotton wool spots (9.68 %), and paracentral acute middle maculopathy (PAMM) (8.06 %). Smaller retinal inner nuclear layer hyperreflective speckles (RIHS) (41.94 %) were identified as a distinguishing feature from typical PAMM. Voids of vessel signals were found in the superficial (11.54 %), intermediate (82.69 %), and deep capillary plexus (98.08 %), and in the choriocapillaris (19.23 %) on OCTA. Humphrey perimetry illustrated central, paracentral, and peripheral scotomas. The occult lesions associated with AMN, PAMM, and some of the RIHS illustrated by OCT were visualized topographically and further confirmed by OCTA as perfusion defects. An increase in AMN cases correlated with the SARS-CoV-2 outbreak. Additional features, including widespread inner retinal perfusion deficits, were observed and may serve as potential biomarkers for systemic microcirculation dysregulation in COVID-19.

Open and Endoscopic Endonasal Optic Nerve Decompression for Craniofacial Fibrous Dysplasia in an Adolescent: 2-Dimensional Operative Video.

Craniofacial fibrous dysplasia (CFFD) is a benign, bony disease that may affect the skull base.1,2 Most cases are asymptomatic and observed; however, advanced disease can present with cranial neuropathy or craniofacial deformity requiring intervention.3-5 A 16-year-old adolescent girl with known CFFD involving the sphenoid and frontal bones with severe bilateral optic canal narrowing developed progressive right eye visual decline and frontal cosmetic deformity. Visual acuity worsened oculus dextrus (OD) to 20/30 with a new superior nasal scotoma and 20% loss in the retinal nerve fiber layer and remained oculus sinister (OS) 20/20. The patient was recommended a staged subfrontal craniotomy for right optic decompression and simultaneous correction cosmetic deformity followed by endonasal right optic decompression. On postoperative day one, visual acuity OD improved to 20/20; however, she developed OS visual decline to 20/800. Curiously, there were no episodes of intraoperative hypotension or additional iatrogenic compression. Use of methylprednisolone led to improvement OS 20/400. Given persistent visual decline, urgent second stage endonasal bilateral optic nerve decompression, rather than unilateral, was performed. Postoperatively, vision improved to OS 20/200. At one month, her vision improved to OD 20/15 and OS returned to 20/20 with a paracentral scotoma and 29% decline in left retinal nerve fiber layer with further improvement anticipated. This video describes a multidisciplinary, multistaged approach in treatment of optic nerve compression due to CFFD in addition to the management of unanticipated contralateral visual decline. The patient consented to the procedure and publication of her image. No Institutional Review Board/ethics committee approval was necessary for this case report.

Solar retinopathy: A literature review.

Solar retinopathy (SR) refers to retinal injury that results from unprotected excessive exposure to light. It has been associated with direct sungazing, sunbathing, laser pointers, and welding arc exposure. Symptoms are typically bilateral and are characterized by asymmetric decreased vision, central or paracentral scotoma, photophobia, metamorphopsia, and headache. In most cases, recovery occurs spontaneously with no specific treatment within weeks to 6 months after exposure. However, few cases have been reported in the literature using steroids in acute SR because of their anti-inflammatory effects. The aim of this review is to present an update about this entity, describing the pathogenesis, risk factors, and diagnostic methods, with focus on management and outcomes of SR.

Homonymous Central Scotomatous Visual Field Defect in a Patient with Lateral Geniculate Nucleus Infarction

Incongruous visual field defects, such as sectoranopia, can result from lateral geniculate nucleus infarction. We present a rare case of right lateral geniculate nucleus infarction that manifests as a left inferior homonymous paracentral scotoma. According to the retinotopic organization of the visual tract, central vision from the macula is delivered to the occipital pole via the posterior portion of the lateral geniculate nucleus. Consequently, not only can occipital pole infarction lead to central visual loss, but partial lateral geniculate nucleus infarction can also produce similar manifestations of central visual loss.

Visual field evaluation following brain injury in school-aged children

Introduction. Brain injury may affect both afferent and efferent visual pathways. In children it is quite difficult to determine visual disturbances since they are often non cooperative. Visual field examination is an objective evaluation method that can outline visual pathway alteration in the acute period of head trauma. Materials and methods. Forty-eight patients with persisting visual symptoms after mild traumatic brain injury were examined. A control group of the same size has been evaluated. Results. Patients in the research group showed an obvious alteration of the fixation capacity of more than 20% in 91.7%95.8%, while in the control group the fixation capacity was up to 20% in 68.7%-70.8%. The ability to fix false positive points was up to 20% in 43.8%-45.8% patients in the research group and 70.8%-83.3% in the control group. The rate to fix false negative points was within the range of up to 20% for the research group in 93.7%-95.8% and the control group 91.7%-97.9%. The index of localized defects was up to 3dB in 62.5%-70.8% in the research group and predominantly 91.6%-95.8% for the control group. The average elevation index was within the range of < -3dB, 3dB> in 12.5%-20.8% research group and respectively 54.1%-56.2% control group. The graphic interpretation of changes in the visual field revealed a prevalence of the incidence of diffuse retinal depression with relative paracentral scotomas in 64.6%-68.7%. Conclusions. Based on the results, we can conclude that perimetric examination in the case of brain injured pediatric patients fulfils the requirements of credibility. Perimetric examination could be a landmark in the initial phase of settling post brain injured visual disturbances.

Hemorrhagic unilateral retinopathy: a report of multimodal imaging in three cases.

To report three cases of hemorrhagic unilateral retinopathy, diagnosed by multimodal imaging. Case report of 3 patients, 2 women and one man, aged 51, 74, and 52, respectively. Symptoms were acute floaters, blurred vision, or central scotoma, unilateral in all cases. The best-corrected visual acuity was 20/20 in the affected eye in 2 patients with a paracentral scotoma, and 20/160 in the third patient. Funduscopic examination revealed multiple unilateral posterior hemorrhages located in the Henle fiber layer in the macula and beneath the internal limiting membrane around the optic disc on spectral-domain optical coherence tomography (SD-OCT). Fluorescein angiography and indocyanine green angiography (ICGA) did not show any vascular abnormalities. SD-OCT angiography (SD-OCT-A) did not show any capillary drop-out or choroidal abnormalities. In all patients, the visual symptoms completely disappeared within a few weeks, with spontaneous regression of the hemorrhages. Hemorrhagic unilateral retinopathy is a rarely reported and poorly understood disorder. ICGA and SD-OCT-A did not allow better understanding the condition. No etiology has been associated with this entity so far. The spontaneous resolution of the present cases confirmed the favorable visual prognosis of the condition.

The visual field-testing maze and vision maze: Feasible techniques to evaluate visual field loss in animals

Visual field loss due to glaucoma is a severe and concerning problem, leading to limited visual range and poor quality vision. The progression of this loss begins with a para-central arcuate scotoma which eventually advances to a ring scotoma and constricted visual fields in later stages. Currently, no animal model is available for screening this pattern of vision loss. However, we have successfully developed two mazes to evaluate visual field loss - the visual field-testing maze (VFTZ) for peripheral vision loss and the vision maze (VM) for central vision loss. Our studies involved inducing glaucoma in Wistar and Sprague Dawley rats using lipopolysaccharide (LPS) and testing them in VFTZ and VM. We used Latanoprost and dorzolamide eye drops as standard drug candidates during the study. We evaluated the animals for intraocular pressure, retinal vasculature imaging, and anxiety using tonometry, ophthalmoscopy, and light and dark model techniques. Furthermore, we quantified the antioxidant parameters of the retina using UV spectroscopy. Our findings showed that animals with peripheral visual field loss in VFTZ took significantly more time to reach the goal and spent more time within the maze compared to normal or drug-treated animals (P<0.001). Additionally, animals with compromised central visual field in VM spent more time in a particular arm and changed arms less frequently (P<0.001) compared to normal or drug-treated animals. Moreover, we observed that glaucomatous rats exhibited elevated anxiety levels and impaired performance in the mazes, emphasizing the impact of vision loss on anxiety. Finally, the antioxidant and ATPase alterations in the retinal layers verified the glaucomatous changes in the experimental animals. Based on our remarkable findings, we strongly recommend the use of VFTZ and VM to evaluate visual field loss in animals.

Interpretation of the Visual Field in Neuro-ophthalmic Disorders.

In this review, we will describe current methods for visual field testing in neuro-ophthalmic clinical practice and research, develop terminology that accurately describes patterns of field deficits, and discuss recent advances such as augmented or virtual reality-based perimetry and the use of artificial intelligence in visual field interpretation. New testing strategies that reduce testing times, improve patient comfort, and increase sensitivity for detecting small central or paracentral scotomas have been developed for static automated perimetry. Various forms of machine learning-based tools such as archetypal analysis are being tested to quantitatively depict and monitor visual field abnormalities in optic neuropathies. Studies show that the combined use of optical coherence tomography and standard automated perimetry to determine the structure-function relationship improves clinical care in neuro-ophthalmic disorders. Visual field assessment must be performed in all patients with neuro-ophthalmic disorders affecting the afferent visual pathway. Quantitative visual field analysis using standard automated perimetry is critical in initial diagnosis, monitoring disease progression, and guidance of therapeutic plans. Visual field defects can adversely impact activities of daily living such as reading, navigation, and driving and thus impact quality of life. Visual field testing can direct appropriate occupational low vision rehabilitation in affected individuals.

Detección y caracterización de defectos del campo visual mediante perimetría Octopus en glaucoma congénito primario

Detección y caracterización de defectos del campo visual mediante perimetría Octopus en glaucoma congénito primario

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

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. 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. 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 2min 12s (SD: 21.6s). MD and sLV values were correlated with best corrected visual acuity (BCVA), cup to disc ratio and number of antiglaucoma surgeries (all P<.001). 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.

Paracentral Acute Middle Maculopathy in Behçet's Disease.

To report a novel presentation of bilateral paracentral acute middle maculopathy (PAMM) and peripheral vascular occlusions in Behçet's disease (BD). A retrospective case report with multimodal imaging studies of a patient with BD. A 58-year-old Chinese man presented with a paracentral scotoma, fever, arthralgias and skin rash. HLA typing revealed HLA-B51 positivity. Ophthalmic examination showed peripheral retinal hemorrhages and fluorescence angiography (FA) demonstrated vascular occlusions in the peripheral retina bilaterally. Optical coherence tomography (OCT) showed classic acute PAMM lesions in both eyes. PAMM and peripheral vascular occlusion are infrequent and unconventional presentations of BD. To the best of our knowledge, this is the first report in the ophthalmic literature of PAMM and peripheral vascular occlusion in BD.

Decreased perifoveal ganglion cell complex thickness - a first sign for macular damage in patients using hydroxychloroquine.

Aim: To examine ganglion cell complex (GCC) thickness detected by optical coherence tomography (OCT) in patients using hydroxychloroquine (HCQ), without any structural and functional macular changes to evaluate the initial symptoms of macular toxicity for early diagnosis before clinical evaluation. Methods: Eighty eyes of forty patients (Group 1) and forty eyes of twenty healthy volunteer persons (Group 2) were included in the study. Detailed ophthalmologic and mydriatic fundus examination were applied to all patients and volunteers (controls). Spectral domain OCT, visual field (VF) and color vision test were performed. Measurements of macula thickness, GCC thickness (involving nerve fiber layer, ganglion cell layer and inner plexiform layer) and peripapillary retinal nerve fiber layer (RNFL) were performed with OCT. Patients with retinal pigment epithelial changes, VF paracentral scotoma and defected color vision were excluded from the planned study. Results: Perifoveal GCC layer thickness in all quadrants was significantly thinner in group 1 compared to group 2 (p=0.017, p=0.001, p=0.019, p=0.001). The mean global inferior hemifield and nasal quadrant RNFL thickness were lower than in the control groups (p=0,012, p=0,009, p=0,005, respectively). Conclusion: Changes in the thickness of nerve fiber layer and ganglion cell layer detected by optical coherence tomography can be thought to be used as a diagnostic aid for the early diagnosis of hydroxychloroquine-toxic maculopathy Abbreviations: GCC = Ganglion cell complex, OCT = Optical coherence tomography, HCQ = Hydroxychloroquine, BCVA = Best-corrected visual acuity, IOP = Intraocular pressure, VF = Visual field, RNFL = Retinal nerve fiber layer, SD OCT = Spectral-domain optical coherence tomography, mfERG = Multifocal electroretinogram, FAF = Fundus autofluorescence, IS/ OS = Inner segment-outer segment junction, SITA = Swedish Interactive Threshold Algorithm, RA = Rheumatoid arthritis, SLE = Systemic lupus erythematosus, SS = Sjogren syndrome.

Factors Associated with Vascular Changes at the Level of Retinal Ganglion Cell Axon versus Soma/Dendrite in Glaucoma Patients.

Superficial and deep macular vessel density (VD) is decreased in eyes with glaucoma. Superficial VD comprises both the retinal nerve fiber layer (RNFL) and ganglion cell/inner plexiform layer (GC/IPL), and various terms have been used previously to describe the layers of macular VD. In our study, we readjusted the macular segmentation. We obtained RNFL and GC/IPL VDs separately to evaluate VD changes of axon versus soma/dendrite of the retinal ganglion cells (RGCs) in detail. We included 66 eyes of normal tension glaucoma patients with inferior localized RNFL defects solely impacting the inferior hemiretina. Macular VD was measured as RNFL VD and GC/IPL VD. VD ratio was calculated by dividing the VD from the affected hemiretina by the VD from the unaffected hemiretina. RNFL VD ratio was related to RNFL and GC/IPL thicknesses (p = 0.005, p = 0.001), whereas GC/IPL VD ratio was not (p = 0.596, p = 0.783). A lower GC/IPL VD ratio was associated with lower RNFL VD (p = 0.017) and systemic hypertension (p = 0.03) in multivariate analysis. Patients with a reduced GC/IPL VD ratio were more prone to poor visual field defects (p = 0.022) and paracentral scotoma (p = 0.046) and more likely to be on treatment for systemic hypertension (p = 0.024). Therefore, glaucoma patients on systemic hypertension treatment and reduced GC/IPL VD require cautious management.

Paracentral acute middle maculopathy presenting as a sign of impending central retinal artery occlusion: a case report

BackgroundTo report a case of paracentral acute middle maculopathy (PAMM) that progressed to central retinal artery occlusion (CRAO) on spectral domain-optical coherence tomography (SD-OCT).Case PresentationA 63-year-old male presented with a paracentral scotoma that began several days ago. His past medical history consisted of third-degree atrioventricular heart block requiring a pacemaker. Giant cell arteritis was unlikely given the patient’s labs, demographics and review of systems. SD-OCT revealed a characteristic hyperreflective band in the inner nuclear layer consistent with PAMM in his left eye. Fluorescein angiography was obtained and was unremarkable. Five days later, the patient developed no light perception in the left eye. SD-OCT showed a diffuse inner retinal hyperreflectivity consistent with CRAO.ConclusionPAMM can be a harbinger event for complete CRAO. Complete stroke evaluation should be performed to prevent a cerebrovascular event or progression to complete blindness in the involved eye.

Bilateral Acute Macular Neuroretinopathy (AMN) after COVID-19 and its Clinical Course.

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.

Optic Disc Morphology and Paracentral Scotoma in Patients with Open-Angle Glaucoma and Myopia.

This study's aim was to investigate the association between optic disc morphology and the occurrence of paracentral scotoma in eyes with open-angle glaucoma (OAG) and myopia. Two-hundred and eleven myopic eyes with OAG were classified into three groups according to the location of visual field (VF) defect (99 paracentral scotoma, 65 peripheral scotoma, and 47 no VF defect). Optic disc morphology was assessed based on the tilt ratio and eccentricity of the central retinal vessel trunk (CRVT). Clinical characteristics of the three groups were compared, and factors affecting the occurrence of paracentral scotoma were determined. Eyes with paracentral scotoma had a higher tilt ratio than the other groups (ps ≤ 0.04). Multiple linear regression showed that a nasal location of CRVT (p < 0.001), longer axial length (p = 0.001), and lower VF mean deviation (p = 0.021) were significantly associated with higher tilt ratio. In logistic regression analysis, tilt ratio was the only factor that was significantly associated with the occurrence of paracentral scotoma (odds ratio = 7.12, p = 0.032). In conclusion, the optic disc tilt ratio increased with nasal shift of CRVT, longer axial length, and lower VF mean deviation. Higher optic disc tilt was significantly associated with the occurrence of paracentral scotoma in eyes with OAG and myopia.

Trauma-Related Acute Macular Neuroretinopathy. A Case Report

To introduce a case report and review the literature on trauma-related acute macular neuroretinopathy as an unusual etiology of acute macular neuroretinopathy. 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). 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. 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.