Progressive Visual Impairment Research Articles

Corneal collagen cross-linking in patients with keratoconus from the Dresden protocol to customized solutions: theoretical basis.

Keratoconus is an ectatic condition characterized by gradual corneal thinning, corneal protrusion, progressive irregular astigmatism, corneal fibrosis, and visual impairment. The therapeutic options regarding improvement of visual function include glasses or soft contact lenses correction for initial stages, gas-permeable rigid contact lenses, scleral lenses, implantation of intrastromal corneal ring or corneal transplants for most advanced stages. In keratoconus cases showing disease progression corneal collagen crosslinking (CXL) has been proven to be an effective, minimally invasive and safe procedure. CXL consists of a photochemical reaction of corneal collagen by riboflavin stimulation with ultraviolet A radiation, resulting in stromal crosslinks formation. The aim of this review is to carry out an examination of CXL methods based on theoretical basis and mathematical models, from the original Dresden protocol to the most recent developments in the technique, reporting the changes proposed in the last 15y and examining the advantages and disadvantages of the various treatment protocols. Finally, the limits of non-standardized methods and the perspectives offered by a customization of the treatment are highlighted.

Movement Disorders in Patients with Subacute Sclerosing Panencephalitis: A Systematic Review.

Subacute sclerosing panencephalitis (SSPE) is a complication of measles, occurring after a latency of 4-10 years. It continues to occur in developing countries although resurgence is being reported from developed countries. Characteristic features include progressive neuropsychiatric issues, myoclonus, seizures, movement disorders and visual impairment. Electroencephalography (EEG) typically shows periodic generalized discharges, and elevated CSF anti-measles antibodies are diagnostic. Movement disorders are being increasingly recognized as part of the clinical spectrum, and range from hyperkinetic (chorea, dystonia, tremor, tics) to hypokinetic (parkinsonism) disorders and ataxia. This article aims to comprehensively review the spectrum of movement disorders associated with SSPE. A literature search was conducted in PubMed and EMBASE databases in December 2023 and articles were identified for review. Movement disorders reported in SSPE included hyperkinetic (chorea, dystonia, tremor and tics), hypokinetic (parkinsonism), ataxia and extraocular movement disorders. Myoclonus, a core clinical feature, was the most frequent "abnormal movement." Movement disorders were observed in all clinical stages, and could also be a presenting feature, even sans myoclonus. Hyperkinetic movement disorders were more common than hypokinetic movement disorders. An evolution of movement disorders was observed, with ataxia, chorea and dystonia occurring earlier, and parkinsonism later in the disease. Neuroradiological correlates of movement disorders remained unclear. A wide spectrum of movement disorders was observed throughout the clinical stages of SSPE. Most data were derived from case reports and small case series. Multicentric longitudinal studies are required to better delineate the spectrum and evolution of movement disorders in SSPE.

Visual dysfunction and neurodegeneration caused by severe inflammatory optic neuropathy after coil embolization of a paraclinoid aneurysm: illustrative case.

Visual impairment due to delayed optic neuropathy is a rare complication of the endovascular treatment of paraclinoid aneurysms. An inflammatory response induced in the treated aneurysm wall is a known mechanism underlying this pathophysiology. The authors describe a case with severe and progressive optic neuropathy leading to neuronal degeneration and severe visual dysfunction. A 42-year-old female with a history of surgical clipping for a paraclinoid aneurysm presented with a recurrence. Although the patient was unaware of any visual dysfunction, a preoperative ophthalmological examination revealed mild inferior quadrantanopia in the left eye. The coil embolization procedure was uneventful; however, the following day, the patient experienced progressive visual impairment, which worsened despite the initiation of steroid therapy. Ophthalmological examination revealed a severe decrease in visual acuity and further deterioration of the visual field. Magnetic resonance imaging showed remarkable swelling and edema of the left optic nerve adjacent to the treated aneurysm. Despite continued steroid therapy, the patient's visual function did not recover well due to subsequent optic nerve degeneration. Optic neuropathy after endovascular procedures can lead to severe visual dysfunction. Careful management is essential, particularly when treating a symptomatic paraclinoid aneurysm, even if symptoms are only apparent on detailed examination.

Exploring clinical variability in gelsolin amyloidosis: Brazilian family case study with confocal microscopy.

Genetic mutations or inflammatory, degenerative, or neoplastic conditions can trigger amyloidosis. Hereditary gelsolin amyloidosis is a genetic disorder primarily marked by amyloid fibrils composed of misfolded gelsolin fragments. We present three sisters with AGel amyloidosis, illustrating its clinical diversity. Patient 1, a 51-year-old, had bilateral ptosis, ocular discomfort, and dry eye syndrome due to cranial nerve involvement. Patient 2, a 53-year-old, experienced progressive bilateral visual impairment. Patient 3, a 50-year-old, exhibited right eye ectropion. Genetic analysis, with the identical mutation, heterozygous c.640G > A (p.Asp214Asn) mutation, confirmed AGel amyloidosis diagnoses, with common findings including lattice corneal amyloidosis, reduced corneal sensitivity, and recurrent corneal erosions. Neurological manifestations included ataxia and peripheral neuropathy, with skin abnormalities observed in patient 1. Ocular involvement severity and distribution varied among patients. Common ocular and neurological manifestations validated AGel amyloidosis diagnoses, reinforcing its hereditary basis. Neurological symptoms highlighted the disorder's impact on various organ systems, while skin abnormalities contributed to ocular discomfort. Variable ocular involvement emphasized the disorder's heterogeneity. These patients emphasize hereditary gelsolin amyloidosis's clinical diversity and suggest potential environmental influences on disease expression. Genetic confirmation and confocal microscopy findings reaffirm the genetic basis while raising questions about assessing systemic disease severity, necessitating further investigation in larger cohorts. Ophthalmologists' specialized care is crucial for managing ocular symptoms, given the absence of a universal cure.

Diagnosing glaucoma in primary eye care and the role of Artificial Intelligence applications for reducing the prevalence of undetected glaucoma in Australia.

Glaucoma is the commonest cause of irreversible blindness worldwide, with over 70% of people affected remaining undiagnosed. Early detection is crucial for halting progressive visual impairment in glaucoma patients, as there is no cure available. This narrative review aims to: identify reasons for the significant under-diagnosis of glaucoma globally, particularly in Australia, elucidate the role of primary healthcare in glaucoma diagnosis using Australian healthcare as an example, and discuss how recent advances in artificial intelligence (AI) can be implemented to improve diagnostic outcomes. Glaucoma is a prevalent disease in ageing populations and can have improved visual outcomes through appropriate treatment, making it essential for general medical practice. In countries such as Australia, New Zealand, Canada, USA, and the UK, optometrists serve as the gatekeepers for primary eye care, and glaucoma detection often falls on their shoulders. However, there is significant variation in the capacity for glaucoma diagnosis among eye professionals. Automation with Artificial Intelligence (AI) analysis of optic nerve photos can help optometrists identify high-risk changes and mitigate the challenges of image interpretation rapidly and consistently. Despite its potential, there are significant barriers and challenges to address before AI can be deployed in primary healthcare settings, including external validation, high quality real-world implementation, protection of privacy and cybersecurity, and medico-legal implications. Overall, the incorporation of AI technology in primary healthcare has the potential to reduce the global prevalence of undiagnosed glaucoma cases by improving diagnostic accuracy and efficiency.

Co-occurring Usher syndrome Type 1 and Renal Failure.

To describe a patient with a rare co-occurrence of Usher syndrome type 1C (USH1C) and renal disease, suspected to be secondary to Alport syndrome. Case report and literature review of cases with Usher syndrome and renal failure. Clinical examination, color fundus photography, visual field tests, electroretinography and whole exome sequencing were used to diagnose and document the patient's clinical presentation. An 18-year-old female with known history of congenital hearing loss and chronic renal failure, presents with progressive night and peripheral visual impairment suspicious for an inherited retinal disease. Visual field testing, fundus exam and electroretinography findings supported the diagnosis of Usher syndrome. Whole exome sequencing (WES) identified a novel homozygous frameshift variant (c.238del) in USH1C. WES also identified a homozygous COL4A3 variant of unknown significance, which may be responsible for concomitant Alport syndrome. By presenting this rare case of co-occurring Usher syndrome Type 1 and renal failure, we highlight the importance of conducting further investigations that could reveal an additional underlying etiology when these entities are present.

Hydrogel-Based Therapy for Age-Related Macular Degeneration: Current Innovations, Impediments, and Future Perspectives.

Age-related macular degeneration (AMD) is an ocular disease that leads to progressive photoreceptor death and visual impairment. Currently, the most common therapeutic strategy is to deliver anti-vascular endothelial growth factor (anti-VEGF) agents into the eyes of patients with wet AMD. However, this treatment method requires repeated injections, which potentially results in surgical complications and unwanted side effects for patients. An effective therapeutic approach for dry AMD also remains elusive. Therefore, there is a surge of enthusiasm for the developing the biodegradable drug delivery systems with sustained release capability and develop a promising therapeutic strategy. Notably, the strides made in hydrogels which possess intricate three-dimensional polymer networks have profoundly facilitated the treatments of AMD. Researchers have established diverse hydrogel-based delivery systems with marvelous biocompatibility and efficacy. Advantageously, these hydrogel-based transplantation therapies provide promising opportunities for vision restoration. Herein, we provide an overview of the properties and potential of hydrogels for ocular delivery. We introduce recent advances in the utilization of hydrogels for the delivery of anti-VEGF and in cell implantation. Further refinements of these findings would lay the basis for developing more rational and curative therapies for AMD.

Feasibility and efficacy of endoscopic transorbital optic canal decompression for meningiomas causing compressive optic neuropathy.

The endoscopic transorbital approach (ETOA) and transorbital anterior clinoidectomy have been suggested as novel procedures through which to reach the superolateral compartments of the orbit, allowing optic canal decompression. However, there is limited literature describing the technical details and surgical outcomes of these procedures. In this study, the authors aimed to analyze the feasibility and efficacy of endoscopic transorbital decompression of the optic canal through anterior clinoidectomy for compressive optic neuropathic lesions. Between 2016 and 2022, the authors performed ETOA for compressive optic neuropathic lesions in 14 patients. All these patients underwent transorbital anterior clinoidectomy through the surgically defined "intraorbital clinoidal triangle," which is composed of the roof of the superior orbital fissure, the medial margin of the optic canal, the medial border of the superior orbital fissure, and the optic strut. Demographic data, tumor characteristics, pre- and postoperative imaging, pre- and postoperative visual examinations, and surgical outcomes were retrospectively reviewed. The mean age at the time of ETOA was 53.3 years (range 41-64 years), and the mean follow-up was 16.8 months (range 6.7-51.4 months). The inclusion criterion in this study was having a meningioma (14 patients). In the preoperative visual function examination, 7 patients with a meningioma showed progressive visual impairment. After endoscopic transorbital optic canal decompression, visual function improved in 5 patients, remained unchanged in 8 patients, and worsened in 1 patient. No new-onset neurological deficit was associated with ETOA and anterior clinoidectomy in any patients. Endoscopic transorbital decompression of the optic canal with extradural anterior clinoidectomy is a safe and feasible technique that avoids significant injury to the clinoidal internal carotid artery and surrounding neurovascular structures.

Alström Syndrome: A Review Focusing on Its Diverse Clinical Manifestations and Their Etiology as a Ciliopathy.

Alström syndrome is a form of inherited obesity caused by a single gene abnormality and is inherited as an autosomal recessive trait. It is characterised by a variety of clinical manifestations, including progressive visual and hearing impairment, type 2 diabetes mellitus, dilated cardiomyopathy, and hepatic and renal dysfunction, in addition to obesity. Recent insights underline the pivotal involvement of the disease-associated gene (ALMS1) in cilia formation and function, leading to the classification of its clinical manifestations as a ciliopathy. This review delineates the diverse clinical indicators defining the syndrome and elucidates its pathological underpinnings.

Disappearing diplopia - An unusual presentation of Tolosa-Hunt syndrome: A case report

Tolosa-Hunt syndrome (THS) is a rare benign treatable cause of painful ophthalmoplegia with frequent VI, III, IV cranial nerve (CN) involvement. Optic and trigeminal nerve involvement is rare but described. The disease is characterized by severe preceding or concomitant headache and remarkable response to steroids. Although labelled benign residual neurology is not uncommon in THS.We present a case of THS in a middle-aged Sri Lankan male who presented initially with vertical diplopia and normal contrast enhanced CT scan, treated as for idiopathic IV nerve palsy. A month henceforth he developed severe episodic headache with autonomic symptoms, right sided ptosis and progressive visual impairment with improved diplopia, normal fundoscopy, inflammatory and infective markers and autoimmune profile. MRI brain showed enhancing lesion of cavernous sinus extending to orbital apex consistent with THS. He showed dramatic improvement with steroids with complete resolution of neurology at the end of 4 weeks.THS should be considered in a patient presenting with disappearing painful diplopia. Headache may mimic primary headache syndromes and may not precede ophthalmoplegia. Imaging may be initially normal. MRI is mandatory for diagnosis as histology is not always feasible. Steroid responsiveness can be diagnostic if more sinister differentials are ruled out.

Progression of retinitis pigmentosa on static perimetry, optical coherence tomography, and fundus autofluorescence

In retinitis pigmentosa (RP), photoreceptor degeneration leads to progressive visual field loss and visual impairment. Several therapeutic trials are ongoing aiming to establish effective treatments. Although functional evaluations are commonly used in clinical trials, residual ellipsoid zone (EZ) measurement on optical coherence tomography has been shown to be more sensitive to detect disease progression. Establishment of sensitive outcome measurement is essential to develop new therapeutic strategies. In the current study, we evaluated the progression rates of the disease in 76 eyes of 76 patients with RP, using the residual EZ length, ring-shaped macular hyperautofluorescent (AF), and visual field. Decrease rates measured by the residual EZ area and by the hyper-AF ring area were strongly positively correlated (P < 0.0001, r = 0.71). The reduction rates of the residual EZ length and hyper-AF ring radius were constant regardless of their baseline measurements. Faster annual reduction rates of the hyper-AF ring area or radius were significantly correlated with faster visual field progression (P = 0.03, r = 0.25 and P = 0.004, r = 0.33, respectively). These findings support the usage of morphological measurements such as EZ or hyper-AF ring measurements as outcome measurement for future clinical trials for RP.

Generation of a gene-corrected isogenic iPSC cell line from an X-linked retinoschisis patient with a hemizygous mutation c.304C > T (p.R102W) in RS1 gene

X-linked retinoschisis (XLRS) is one of the most common retinal genetic diseases with progressive visual impairment in childhood affecting males. It is manifested with macular and/or peripheral schisis in neural retinas with no effective treatment. Previously, we successfully generated a human-induced pluripotent stem cell (iPSC) line from an XLRS patient carrying the hemizygous RS1 c. 304C > T (p.R102W) mutation. Here, we corrected the c.304C > T mutation in the RS1 gene using CRISPR/Cas9 technology to generate an isogenic control. This cell line is valuable for the study of XLRS.

Craniofacial Fibrous Dysplasia in Fronto-Orbital Region: A Single-Center Retrospective Study of 38 Cases

This study presents the clinical characteristics, imaging manifestations, and surgical experience in 38 patients diagnosed with craniofacial fibrous dysplasia (CFD) in fronto-orbital region (foFD). We retrospectively analyzed the clinical data from 38 patients who had surgery for foFD. The surgical procedure typically involved extensive tumor removal, followed by immediate reconstruction of the frontal bone and orbit using synthetic materials. Additionally, 9 patients underwent simultaneous microscopic decompression of the optic canal. Common clinical manifestations included progressive fronto-orbital bone deformity (35), proptosis (28), orbital dystopia (21), and visual impairment (9). The disease primarily affecting the frontal bone (38), the sphenoid bone (28) and the ethmoid bone (24). The optic canal was involved in 9 patients with functional impairment. CT scans in all 38 cases revealed satisfactory repair material positioning and complete resolution of frontal deformities. Among the 9 patients who underwent optic canal decompression, 7 experienced partial recovery of visual acuity after surgery. In the surgical treatment of foFD, it is crucial to achieve maximal bone resection and repair skull defects, while decompressing the optic canal can provide significant benefits for patients with decreased visual function preoperatively. The use of preformed artificial materials offers advantages in aesthetic restoration after lesion excision.

Application of Electrophysiology in Non-Macular Inherited Retinal Dystrophies.

Inherited retinal dystrophies encompass a diverse group of disorders affecting the structure and function of the retina, leading to progressive visual impairment and, in severe cases, blindness. Electrophysiology testing has emerged as a valuable tool in assessing and diagnosing those conditions, offering insights into the function of different parts of the visual pathway from retina to visual cortex and aiding in disease classification. This review provides an overview of the application of electrophysiology testing in the non-macular inherited retinal dystrophies focusing on both common and rare variants, including retinitis pigmentosa, progressive cone and cone-rod dystrophy, bradyopsia, Bietti crystalline dystrophy, late-onset retinal degeneration, and fundus albipunctatus. The different applications and limitations of electrophysiology techniques, including multifocal electroretinogram (mfERG), full-field ERG (ffERG), electrooculogram (EOG), pattern electroretinogram (PERG), and visual evoked potential (VEP), in the diagnosis and management of these distinctive phenotypes are discussed. The potential for electrophysiology testing to allow for further understanding of these diseases and the possibility of using these tests for early detection, prognosis prediction, and therapeutic monitoring in the future is reviewed.

The Effect of Visual Impairment and Its Severity on Vision-Related and Health-Related Quality of Life in Jordan: A Comparative Cross-Sectional Study.

To assess the effect of visual impairment (VI), its severity, and ocular diseases on vision-related and health-related quality of life (QoL) in Jordan. A comparative, cross-sectional, hospital-based study was conducted among a group of 278 patients with VI aged ≥ 18 years, and age and sex-matched control group of 278 individuals with no VI. An interviewer administered the National Eye Institute Visual Function Questionnaire (NEI VFQ-25) and the Medical Outcomes Study 12-Item Short Form Health Survey (SF-12) to all participants. All the mean VFQ-25 subscales scores, physical component scale (PCS) and the mental component scale (MCS) of the SF-12 were significantly lower in patients with VI compared to controls with no VI. The VFQ-25 subscales (except general health and ocular pain), PCS, and MCS scores significantly decreased with more severity of VI. In the adjusted multivariate analysis, lower level of education (p=0.013), male sex (p=0.016), and the presence of cerebrovascular disease (p=0.019) were significantly associated with lower VFQ-25 composite scores in visually impaired patients compared to controls. Ocular disease duration of >5 years and progressive VI were significantly associated with lower VFQ-25 composite scores (p= 0.026 and p<0.001) respectively, in patients with VI. Glaucoma had a significantly larger reduction in mean scores of all the VFQ-25 subscales, and the PCS of the SF-12 compared to all other ocular diseases. Both VI and increasing severity of impairment were associated with reduced vision-related and health-related quality of life in adult Jordanians. Glaucoma patients and less educated people were particularly affected. Routine assessment of QoL in visually impaired patients and improving referral protocols to vision rehabilitation services is recommended to improve the QoL in those patients.

Gene therapy for inherited retinal diseases: exploiting new tools in genome editing and nanotechnology

Inherited retinal diseases (IRDs) encompass a diverse group of genetic disorders that lead to progressive visual impairment and blindness. Over the years, considerable strides have been made in understanding the underlying molecular mechanisms of IRDs, laying the foundation for novel therapeutic interventions. Gene therapy has emerged as a compelling approach for treating IRDs, with notable advancements achieved through targeted gene augmentation. However, several setbacks and limitations persist, hindering the widespread clinical success of gene therapy for IRDs. One promising avenue of research is the development of new genome editing tools. Cutting-edge technologies such as CRISPR-Cas9 nucleases, base editing and prime editing provide unprecedented precision and efficiency in targeted gene manipulation, offering the potential to overcome existing challenges in gene therapy for IRDs. Furthermore, traditional gene therapy encounters a significant challenge due to immune responses to viral vectors, which remain crucial obstacles in achieving long-lasting therapeutic effects. Nanotechnology has emerged as a valuable ally in the quest to optimize gene therapy outcomes for ocular diseases. Nanoparticles engineered with nanoscale precision offer improved gene delivery to specific retinal cells, allowing for enhanced targeting and reduced immunogenicity. In this review, we discuss recent advancements in gene therapy for IRDs and explore the setbacks that have been encountered in clinical trials. We highlight the technological advances in genome editing for the treatment of IRDs and how integrating nanotechnology into gene delivery strategies could enhance the safety and efficacy of gene therapy, ultimately offering hope for patients with IRDs and potentially paving the way for similar advancements in other ocular disorders.

Living with an iconic aid

There are plenty of objects that are conspicuous in the way they signal to the surroundings that the user has some kind of physical impairment. Most obvious are perhaps wheelchairs, motorized wheelchairs, hearing aids and crutches. These objects can be described as aids in the sense that the purpose is either to mitigate the effects of reduced physical abilities or to compensate for the loss of a sensory faculty. The focus of this article concerns an object that both increases mobility and replaces such a faltering capacity. It might even be the most iconic of all aids: the mobility cane, also known as the white cane, long cane or white stick. Based on the work at a Swedish Low Vision and Resource Centre, this article discusses the use of the white cane among people with severe and progressive visual impairment. The fact is that this aid, which for an outsider would easily seem to be a rather natural choice, can arouse completely different feelings in somebody who has a severe visual impairment. It is a well-known circumstance amongst low visions teachers and therapists that persons with acquired or progressive sight impairment are often not quite happy about using the aid. Therefore, the aim of the article is to utilize notions of everyday experiences to gain further insights into why the white cane for many of its potential users are associated with strong feelings of both personal ambiguity and social stress. In order to highlight these emotional, and still cultural and political, tensions, the discussion is grounded in critical disability studies and informed by ableism, stigma, and passing as theoretical concepts.

Stress resilience-enhancing drugs preserve tissue structure and function in degenerating retina via phosphodiesterase inhibition

Chronic, progressive retinal diseases, such as age-related macular degeneration (AMD), diabetic retinopathy, and retinitis pigmentosa, arise from genetic and environmental perturbations of cellular and tissue homeostasis. These disruptions accumulate with repeated exposures to stress over time, leading to progressive visual impairment and, in many cases, legal blindness. Despite decades of research, therapeutic options for the millions of patients suffering from these disorders remain severely limited, especially for treating earlier stages of pathogenesis when the opportunity to preserve the retinal structure and visual function is greatest. To address this urgent, unmet medical need, we employed a systems pharmacology platform for therapeutic development. Through integrative single-cell transcriptomics, proteomics, and phosphoproteomics, we identified universal molecular mechanisms across distinct models of age-related and inherited retinal degenerations, characterized by impaired physiological resilience to stress. Here, we report that selective, targeted pharmacological inhibition of cyclic nucleotide phosphodiesterases (PDEs), which serve as critical regulatory nodes that modulate intracellular second messenger signaling pathways, stabilized the transcriptome, proteome, and phosphoproteome through downstream activation of protective mechanisms coupled with synergistic inhibition of degenerative processes. This therapeutic intervention enhanced resilience to acute and chronic forms of stress in the degenerating retina, thus preserving tissue structure and function across various models of age-related and inherited retinal disease. Taken together, these findings exemplify a systems pharmacology approach to drug discovery and development, revealing a new class of therapeutics with potential clinical utility in the treatment or prevention of the most common causes of blindness.

Endoscopic endonasal optic nerve decompression: treatment of fibrous dysplasia in a pediatric population.

Patients with fibrous dysplasia (FD) of the anterior skull base can experience progressive visual loss and impairment. The authors reviewed their experience with endonasal decompression of the optic nerve (ON) in this patient population. Endoscopic ON decompression (EOND) is a feasible surgical approach for children with FD and visual deficit due to structural ON compression. Electronic medical records of children between 1 and 17 years of age with unilateral FD of the anterior skull base and concomitant ON compression, who required EOND between 2017 and 2022 (n = 4), were reviewed for demographic data, both pre- and postoperative imaging, and evaluations by an otolaryngologist, neurosurgeon, and ophthalmologist in a multidisciplinary fashion. EOND was found to be a safe and effective surgery for children with FD. Visual acuity was stable in 80% of the eyes postoperatively. Visual fields improved in 40% of the eyes and remained stable in the rest. EOND is beneficial for progressive optic neuropathy that is unresponsive to steroid therapy and can prevent permanent disability if performed prior to irreversible damage to the nerve. EOND can decompress the edematous ON with proper exposure of the optic canal and orbital apex, without any major complications.

Combined Cilioretinal Artery and Branch Retinal Vein Occlusion Revealing a Carotid Artery Disease

We report the case of a healthy 55-year-old patient, who consulted at the ophthalmology emergency for a rapidly progressive unilateral visual impairment of the left right eye without neurological signs. Fundus examination of the right eye found retinal whitening following the territory of the cilioretinal artery corresponding to cilioretinal artery occlusion; dilated and tortuous retinal veins, and scattered retinal hemorrhages on the lower quadrants as well a puddle-like hemorrhages at the interpapillomacular area (Figure 1: Panel A). Fluoresceine angiography targeted a delayed perfusion of the inferior temporal branch vein (arrows) (Figure1: panel B). Macular OCT showed moderate macular thickening in the nasal macula and hyper reflectivity of the inner layers of the retina (Figure 2).