JR5558 mice are a reliable model to investigate subretinal fibrosis.

Subretinal fibrosis is a major untreatable cause of poor outcomes in neovascular age-related macular degeneration. Mouse models of subretinal fibrosis all possess a degree of invasiveness and tissue damage not typical of fibrosis progression. This project characterises JR5558 mice as a model to study subretinal fibrosis.

Retinal Dystrophies Associated With Peripherin-2: Genetic Spectrum and Novel Clinical Observations in 241 Patients.

Purpose: To describe the clinical, electrophysiological and genetic spectrum of inherited retinal diseases associated with variants in the PRPH2 gene.

Methods: A total of 241 patients from 168 families across 15 sites in 9 countries with pathogenic or likely pathogenic variants in PRPH2 were included. Records were reviewed for age at symptom onset, visual acuity, full-field ERG, fundus colour photography, fundus autofluorescence (FAF), and SD-OCT.

Immunomodulatory Treatment Versus Systemic Steroids in Inflammatory Choroidal Neovascularization Secondary to Idiopathic Multifocal Choroiditis.

Purpose: To evaluate the influence of immunomodulatory therapy (IMT) on visual and treatment outcomes of inflammatory choroidal neovascularization (iCNV) in patients affected by multifocal choroiditis (MFC), and to compare them to patients treated with steroids as needed.

Design: Multicenter retrospective matched cohort study.

Methods: Patients affected by MFC with iCNV were divided into a IMT group and a "steroids as needed" group and matched according to the time between diagnosis and beginning of systemic treatment.

A natural history study of autosomal dominant GUCY2D-associated cone-rod dystrophy.

Purpose: To describe the natural history of autosomal dominant (AD) GUCY2D-associated cone-rod dystrophies (CRDs), and evaluate associated structural and functional biomarkers.

Methods: Retrospective analysis was conducted on 16 patients with AD GUCY2D-CRDs across two sites. Assessments included central macular thickness (CMT) and length of disruption to the ellipsoid zone (EZ) via optical coherence tomography (OCT), electroretinography (ERG) parameters, best corrected visual acuity (BCVA), and fundus autofluorescence (FAF).

-associated autosomal dominant retinitis pigmentosa: natural history of novel variant Lys314Gln and a comprehensive literature search.

Background: Inosine monophosphate dehydrogenase (IMPDH) is a key regulatory enzyme in the de novo synthesis of the purine base guanine. Mutations in the inosine monophosphate dehydrogenase 1 gene (IMPDH1) are causative for RP10 autosomal dominant retinitis pigmentosa (adRP). This study reports a novel variant in a family with IMPDH1-associated retinopathy.

Management of macular oedema due to retinal vein occlusion: An evidence-based systematic review and meta-analysis.

Background: Central retinal vein occlusion and branch retinal vein occlusion are common causes of visual loss due to associated macular oedema. The aim of this review was to assess the effectiveness of interventions improving vision and treating macular oedema in central retinal vein occlusion and branch retinal vein occlusion.

Methods: Medical search engines and clinical trial registries were systematically searched.

Early recognition of CLN3 disease facilitated by visual electrophysiology and multimodal imaging.

Background: Neuronal ceroid lipofuscinosis is a group of neurodegenerative disorders with varying visual dysfunction. CLN3 is a subtype which commonly presents with visual decline. Visual symptomatology can be indistinct making early diagnosis difficult.

OUTCOMES OF SWITCHING FROM PROACTIVE TO REACTIVE TREATMENT AFTER DEVELOPING ADVANCED CENTRAL NEOVASCULAR AGE-RELATED MACULAR DEGENERATION.

Purpose: We assessed outcomes of eyes with neovascular age-related macular degeneration (nAMD) that switched from proactive (treat-and-extend) to reactive (pro re nata) treatment regimen after developing macular atrophy (MA) or submacular fibrosis (SMFi).

Methods: Data were collected from a retrospective analysis of a prospectively designed, multinational registry of "real-world" nAMD treatment outcomes. Eyes without MA or SMFi when starting treatment with a vascular endothelial growth factor inhibitor regimen that subsequently developed MA or SMFi were included.

Acute posterior multifocal placoid pigment epitheliopathy: clinical presentation and risk of stroke and transient ischaemic attack.

Background/aims: Acute posterior multifocal placoid pigment epitheliopathy is a rare but important disease that can be associated with life-threatening complications due to cerebral vasculitis. The primary objective was to determine the incidence of neurological complications and risk factors for stroke and transient ischaemic attack (TIA) associated with acute posterior multifocal placoid pigment epitheliopathy. Secondary objectives included the clinical presentation, visual outcomes and recurrence rates.

Acute zonal occult outer retinopathy-like presentation secondary to scleral buckle.

Purpose: To describe a case of acute zonal occult outer retinopathy-like (AZOOR-like) presentation following scleral buckle surgery for rhegmatogenous retinal detachment.

Observations: A 48-year-old man underwent successful scleral buckle with cryotherapy for repair of a left eye inferior macula-on rhegmatogenous retinal detachment. Five years later he presented with a six-month history of left peripheral field restriction.

Electrophysiological Assessment in Birdshot Chorioretinopathy: Flicker Electroretinograms Recorded With a Handheld Device.

Purpose: The flicker electroretinogram (ERG) is a sensitive indicator of retinal dysfunction in birdshot chorioretinopathy (BCR). We explored recordings from a handheld device in BCR, comparing these with conventional recordings in the same patients and with handheld ERGs from healthy individuals.

Methods: Non-mydriatic flicker ERGs, using the handheld RETeval system (LKC Technologies), were recorded with skin electrodes at two centers.

Electronegative electroretinogram in the modern multimodal imaging era.

Background: The electronegative electroretinogram (ERG) reflecting inner retinal dysfunction can assist as a diagnostic tool to determine the anatomical location in eye disease. The aim of this study is to determine the frequency and aetiology of electronegative ERG in a tertiary ophthalmology centre and to develop a clinical algorithm to assist patient management.

Methods: Retrospective review of ERGs performed at the Save Sight Institute from January 2011 to December 2020.

Five-year outcomes of eyes initially enrolled in the 2-year BEVORDEX trial of bevacizumab or dexamethasone implants for diabetic macular oedema.

Background: The BEVORDEX trial compared outcomes of eyes with diabetic macular oedema (DMO) randomised to receive either intravitreal dexamethasone (DEX-) implant or bevacizumab over 2 years. We assessed long-term efficacy and safety outcomes 5 years from enrolment.

Methods: Patients received standard clinical care after they finished the study.

retinopathy: biomarkers assessing vision loss.

Purpose: Mer tyrosine kinase-retinitis pigmentosa (RP) causes a primary defect in the retinal pigment epithelium, which subsequently affects rod and cone photoreceptors. The study aims to identify the most appropriate -RP biomarkers to measure disease progression for deciding the optimum therapeutic trial intervention time.

Materials And Methods: Patients' data from baseline (BL) and last follow-up (LFU) were reviewed.

The electroretinogram in the genomics era: outer retinal disorders.

The inherited retinal diseases (IRDs) have traditionally been described phenotypically with the description evolving to incorporate more sophisticated structural and functional assessments. In the last 25 years there has been considerable advances in the understanding of underlying genetic aetiologies. The role of the ophthalmologist is now to work in a multi-disciplinary team to identify the disease-causing genotype, which might be amenable to gene-directed intervention.

Neovascular age-related macular degeneration at treatment intervals of 14 weeks or greater.

Background: We assessed the proportion of eyes with neovascular age-related macular degeneration (nAMD) in routine clinical practice that reach ≥14 week treatment intervals and their outcomes.

Method: We analysed data from the Fight Retinal Blindness! (FRB!) Project database, a prospectively designed registry of 'real-world' outcomes. Treatment-naive eyes starting vascular endothelial growth factor (VEGF) inhibitors for nAMD from 1st January 2006 were included.

Assessing Residual Cone Function in Retinitis Pigmentosa Patients.

Purpose: The purpose of this study was to investigate cone function deterioration in patients with retinitis pigmentosa (RP) using full field electroretinogram (ffERG), pattern electroretinogram (pERG), and optical coherence tomography (OCT) and their correlation with visual acuity (VA).

Methods: Clinical records (2008-2018) of patients with RP undergoing repeat electrophysiology were reviewed. Results of ffERG (30 Hz flicker and fused flicker amplitude [FFAmp]), pERG [p50 and n95], and macular OCT (ellipsoid zone [EZ] and outer segment thickness) were collected.

Natural history and clinical biomarkers of progression in X-linked retinitis pigmentosa: a systematic review.

X-linked retinitis pigmentosa (XLRP) accounts for a significant proportion of certifiable blindness in working-age adults. The objectives of this study were to: (1) synthesize the best available evidence regarding the natural history of disease progression and (2) identify the best current clinical biomarkers for monitoring disease progression, which will be important in planned gene therapy trials for this condition. Patient population: XLRP affected males.

OPTICAL COHERENCE TOMOGRAPHY FEATURES OF CHOROIDAL NEOVASCULARIZATION AND THEIR CORRELATION WITH AGE, GENDER, AND UNDERLYING DISEASE.

Purpose: To investigate the influence of age, gender, and underlying disease on the optical coherence tomography (OCT) features of choroidal neovascularization (CNV) secondary to inflammation, myopia (mCNV), and age-related macular degeneration (AMD-CNV).

Methods: Demographic and clinical data of eyes with treatment-naive inflammatory CNV, mCNV, and Type 2 AMD-CNV were collected. Optical coherence tomography images were reviewed to determine the presence of pitchfork sign, pigment epithelial detachment, subretinal fluid (SRF), intraretinal cysts, subretinal hyperreflective material, atrophy, and outer retinal disruption graded 1 to 4.

Imaging the Choroid: From Indocyanine Green Angiography to Optical Coherence Tomography Angiography.

The choroid is the vascular structure nourishing the retinal pigment epithelium and the outer retina and it plays a key role in the homeostasis of the eye both under physiological and pathological conditions. In the last 20 years we have moved from "guessing" what was happening beyond the retinal pigment epithelium to actually visualize structural and functional changes of the choroid in vivo noninvasively. In this review we describe the state of the art of choroidal imaging, focusing on the multiple techniques available in the clinical and research setting including indocyanine green angiography, labeled-cells angiographies, optical coherence tomography (OCT), enhanced depth imaging, swept source OCT, and OCT angiography.