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Wei Jia Liu,1 Laura J Taylor,2,3 Robert E MacLaren,2,3 Jasleen K Jolly4 1School of Medicine and Biomedical Sciences, University of Oxford, Oxford, UK; 2Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK; 3Oxford Eye Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, UK; 4Vision and Eye Research Institute, Anglia Ruskin University, Cambridge, UKCorrespondence: Jasleen K Jolly, Vision and Eye Research Institute, Anglia Ruskin University, Cambridge, UK, Tel/Fax +44 122 369 5034, Email jasleen.jolly@aru.ac.ukPurpose: Clinical research brings the potential of improved diagnostics, sight-saving treatments, and more accessible services to those suffering with severe sight impairment (SSI). This report investigates whether registered ophthalmology clinical studies address the leading causes of SSI in the general and working populations of the United Kingdom (UK).Methods: The latest statistics on the leading causes of SSI in the UK general and working populations were identified by searching PubMed, Cochrane Library, and TRIP databases. Clinical study registries were searched to identify registered clinical studies (on or prior to 1st December 2022) on the leading causes of SSI. The relationship between the number of clinical studies on leading causes of SSI and the percentage of SSI certifications they account for was analyzed.Results: In the UK general population, the number of registered clinical studies on the leading causes of SSI is statistically significantly correlated (Spearman’s rho = 0.86, p < 0.01) with the percentage of SSI certifications they account for. However, there is no correlation between the two in the UK working population (aged 16– 64) (Spearman’s rho = 0.15, p = 0.70). Eye conditions accounting for the most SSI certifications in individuals of working age have significantly less clinical research activity than those that cause the most SSI certifications in the general population. Out of the leading causes of SSI certifications studied, disorders of the visual cortex and congenital anomalies of the eye have the least clinical research activity.Conclusion: Clinical research into the leading causes of SSI in the general population is essential. However, it is important to consider eye conditions that cause the most severe visual impairment in individuals of working age due to the significant health and socioeconomic implications of sight loss in this population.Keywords: ophthalmology, blindness, clinical study, healthcare

Colm D Andrews,1,2 Aislin A Sheldon,3 Holly Bridge,3 Susan M Downes,2,4 Robert E MacLaren,2,4 Jasleen K Jolly2– 5 1Nuffield Department of Population Health, University of Oxford, Oxford, UK; 2Oxford Eye Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, UK; 3Oxford Centre for Functional MRI of the Brain (FMRIB), Wellcome Centre for Integrative Neuroimaging, University of Oxford, Oxford, UK; 4Nuffield Laboratory of Ophthalmology, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK; 5Vision and Eye Research Institute, Anglia Ruskin University, Cambridge, UKCorrespondence: Jasleen K Jolly, Vision and Eye Research Institute, School of Medicine, Anglia Ruskin University, Young Street, Cambridge, CB1 2LZ, UK, Email jasleen.jolly@aru.ac.ukPurpose: We aimed to assess the performance of the modified-Esterman test (mET) as a rapid suprathreshold binocular quantification tool for the assessment of peripheral visual fields. The mET consists of an even spread of test points across the visual field.Materials and Methods: The mET was implemented on the Octopus 0900 perimeter using the Open Perimetry Interface (OPI) and consisted of 160 points. Patients with choroideremia, a rod-cone dystrophy, Stargardt disease, a cone-rod dystrophy, and healthy volunteers underwent both the mET and the standard Esterman tests twice. Disease severity (mild/moderate/severe) was graded on both tests independently. Voronoi tessellation was utilised to compare the tests.Results: The Voronoi visualisation was able to demonstrate that the mET was able to provide more information about the disease state at all stages of diseases. This was confirmed by the agreement statistic, which showed that the mET detected 27% more points of visual field loss compared to the Esterman test, being most useful in patients with rod-cone dystrophies.Conclusion: The mET provides a speedy quantitative measure of the peripheral visual field loss, which can be used in clinical trials to monitor longitudinal assessment of peripheral visual function. The mET provides a more even coverage across the visual field compared to the Esterman test points, making it more suitable for this purpose. This is a key part of safety monitoring in retinal clinical trials. The mET can easily be implemented on commercially available perimeters that allow Open Perimetry.Keywords: visual field, functional testing, screening, Stargardt disease, choroideremia

Laura J Wood,1,2,* Jasleen K Jolly,1,2,* Markus Groppe,3 Larry Benjamin,3 James F Kirwan,4 Nishal Patel,5 Mostafa A Elgohary,6 Robert E MacLaren1,2 1Nuffield Laboratory of Ophthalmology, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK; 2Oxford Eye Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, UK; 3Stoke Mandeville Hospital, Buckingham Healthcare NHS Trust, Walton, UK; 4Queen Alexandra Hospital, Portsmouth Hospitals NHS Trust, Portsmouth, UK; 5Kent and Canterbury Hospital, East Kent Hospitals University NHS Foundation Trust, Canterbury, UK; 6Kingston Hospital, Kingston Hospital NHS Foundation Trust, Kingston Upon Thames, UK*These authors contributed equally to this workCorrespondence: Laura J WoodNuffield Laboratory of Ophthalmology, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UKEmail enquires@ndcn.ox.ac.ukBackground/Aim: An increasing number of pre-presbyopic patients are undergoing uniocular cataract extraction. We aim to compare the binocular status of subjects with uniocular cataracts, implanted either with a multifocal or a monofocal intraocular lens (IOL).Materials and Methods: Subjects were recruited from outpatient ophthalmology clinics and randomized to an IOL type. Corrected and uncorrected LogMAR distance visual acuity (VA) and near and intermediate VA using the Radner reading test were completed. The binocular tests included the Worth Four Dot Test, fixation disparity, TNO stereoacuity and foveal suppression assessment. In addition to the near activity vision questionnaire. The trial was closed early because the chosen multifocal lens had been superseded by newer models. We report two subjects, one receiving the multifocal IOL and a monofocal IOL control with the most comparable baseline characteristics.Results: Both subjects experienced uncomplicated cataract surgery, showing clinically significant improved corrected distance VA, 0.06 LogMAR and − 0.16 LogMAR in the monofocal and multifocal IOL, respectively. The multifocal subject had 30 seconds of arc stereoacuity indicating normal binocular vision. Only gross binocular single vision with no stereopsis was found in the monofocal IOL subject. The latter subject also had reduced near vision quality-of-life questionnaire results.Conclusion: This two-patient case series demonstrates greater binocular near ability, with the multifocal IOL, in the pre-presbyopic patient undergoing uniocular cataract surgery. The case series highlights the need, and methodology for investigating further the functional and quality-of-life benefits of implanting multifocal IOLs in pre-presbyopic patients, those in their twenties and thirties, undergoing uniocular cataract surgery.Keywords: binocular vision, cataracts, pseudophakia, multifocal intraocular lens, accommodation

Myrte B Breukink,1,* Alexander JM Dingemans,1,* Anneke I den Hollander,1,2 Jan EE Keunen,1 Robert E MacLaren,3,4 Sascha Fauser,5 Giuseppe Querques,6 Carel B Hoyng,1 Susan M Downes,3,4 Camiel JF Boon1,7 1Department of Ophthalmology, 2Department of Human Genetics, Radboud University Medical Center, Nijmegen, the Netherlands; 3Oxford Eye Hospital, Oxford University Hospitals NHS Trust, 4Nuffield Laboratory of Ophthalmology and NIHR Biomedical Research Centre, Department of Clinical Neurosciences, University of Oxford, Oxford, UK; 5Department of Ophthalmology, University Hospital of Cologne, Cologne, Germany; 6Department of Ophthalmology, University Paris Est Creteil, Centre Hospitalier Intercommunal de Creteil, Creteil, France; 7Department of Ophthalmology, Leiden University Medical Center, Leiden, the Netherlands *These authors contributed equally to this work Purpose: To describe the clinical findings and long-term outcome of patients with chronic central serous chorioretinopathy (cCSC).Materials and methods: This was a retrospective case series in 52 eyes of 36 patients with a follow-up period of at least 1 year. Extensive ophthalmic examination and a validated questionnaire concerning vision-related quality of life (National Eye Institute Visual Function Questionnaire [NEI-VFQ]-39) were analyzed.Results: Mean visual acuity showed a significant decline over time of 0.16 logarithm of minimum angle of resolution ([logMAR] range: -0.22 to 1.3; P=0.009) after a mean follow-up period of 10.6 years. Also, patients reported lower vision-related quality of life based on the NEI-VFQ-39 for almost all categories compared to healthy controls. Macular atrophy was diagnosed more often on optical coherence tomography compared to other diagnostic entities. Retinal pigment epithelium detachments in the macula were documented on optical coherence tomography in 56% of the patients. A significant thinning of foveal thickness was measured over time compared to unaffected fellow eyes (P=0.002). On long-term follow-up, 13 eyes (37%) showed an increase in number of hot spots on fluorescein angiography.Conclusion: This study indicates that cCSC is a progressive disease in many patients, causing a progressive decline in visual acuity, accompanied by lower reported vision-related quality of life. In deciding whether or not to treat, the progressive nature of cCSC should be taken into account in this relatively young and often still professionally active patient group. Keywords: chronic central serous chorioretinopathy, micropulse laser, NEI-VFQ-39, PDT, vision-related quality of life

Martin S Zinkernagel,1,2 Robert E MacLaren3,41Department of Ophthalmology, 2Department of Clinical Research, Inselspital, Bern University Hospital, and University of Bern, Bern, Switzerland; 3Nuffield Laboratory of Ophthalmology, University of Oxford and Oxford Eye Hospital, Oxford University NHS Trust NIHR Biomedical Research Centre, Oxford, UK; 4Moorfields Eye Hospital NIHR Biomedical Research Centre, London, UKAbstract: Choroideremia is a complex and rare disease that is frequently misdiagnosed due to its similar appearance to classic retinitis pigmentosa. Recent advances in genetic testing have identified specific genetic mutations in many retinal dystrophies, and the identification of the mutation of the CHM gene on the X chromosome 25 years ago has paved the way for gene replacement therapy with the first human trials now underway. This article reviews the epidemiological and pathological features of choroideremia and new prospects in imaging to monitor disease progression, as well as potential treatment approaches for choroideremia.Keywords: choroideremia, gene replacement therapy, retinal imaging, clinical trials

Purpose Autosomal dominant optic atrophy (ADOA) due to mutations in OPA1 is a slowly progressive optic neuropathy. OPA1 is ubiquitously expressed and plays a key role in mitochondrial fusion. Heterozygous Opa1 mutant mice (B6;C3‐Opa1Q285STOP), with previously reported visual defects and optic nerve changes, were assessed for retinal sequelae. Methods ERGs and VEPs were recorded in 12 month old B6;C3‐Opa1Q285STOP mice (n=4) and age/sex matched wildtype littermate controls. Full scotopic and photopic intensity series were recorded in response to brief (4 ms) single flash stimuli delivered in a ganzfeld dome. End‐organ evidence for apoptosis was explored on TUNEL stained retinal sections. Results Quantification of the major ERG components (a‐wave and b‐wave) confirmed no detectable difference in the amplitude or implicit time of scotopic ERGs across the full intensity range tested. This was also true for the VEP components. Quantification of the photopic ERG responses revealed a significant reduction in the PhNR amplitude in Opa1+/‐ animals relative to wildtypes at the brightest intensity tested. The photopic VEP was also abnormal in mutant mice. There was no increased apoptosis in retinal sections from mutant animals. Conclusion Opa1+/‐ mice display a functional deficit on electrophysiology that is indicative of specific ganglion cell dysfunction. Despite a 50% reduction in Opa1 protein there does not appear to be a significant increase in apoptosis in the retina. This suggests that the protein truncating mutation in this model may lead to RGC dysfunction without triggering RGC death. [ABSTRACT FROM AUTHOR]