Defining reference values of arterioles in healthy individuals for studies with adaptive optics imaging.

Purpose: To investigate age-dependent wall to lumen ratio (WLR) reference values for healthy individuals in adaptive optics imaging (AO). WLR serves as an objective, dimensionless parameter for the evaluation of structural changes in vessels caused by conditions like arterial hypertension, diabetes or vascular stenosis.

Methods: 50 right eyes of healthy individuals were examined by adaptive optics imaging.

A morphometric analysis of the retinal arterioles with adaptive optics imaging in RPE65-associated retinal dystrophy after treatment with voretigene neparvovec.

Purpose: To investigate the changes in retinal arterial architecture after treatment with voretigene neparvovec in patients with retinal dystrophy caused by bi-allelic mutations in the RPE65 gene.

Methods: Sixteen eyes treated with voretigene neparvovec at the University Eye Clinic in Tuebingen, Germany, underwent adaptive optics ophthalmoscopy (AO) imaging at baseline and 2 weeks, 1, 3, 6 and 12 months after treatment. Follow-up was performed in six eyes of four patients.

Rod and Cone Function Measured Objectively by Chromatic Pupil Campimetry Show a Different Preservation Between Distinct Genotypes in Retinitis Pigmentosa.

Purpose: Verifying whether specific genotypes causing retinitis pigmentosa (RP) show differences in the preservation of rod and cone function measured by chromatic pupil campimetry (CPC).

Methods: Sixty-three RP eyes (37 male, 14-58 years) were measured using CPC with specific photopic and scotopic protocols, and the relative maximal constriction amplitudes and latencies to constriction onset were analyzed per genotype (RP due to variants in EYS, n = 14; PDE6A, n = 10; RPE65, n = 15; USH2A, n = 10; and RPGR, n = 14). Correlation analyses between the pupillary responses were performed with age, full-field stimulus threshold (FST), and optical coherence tomography (OCT) for cones and rods, respectively, to the genotype.

Central Visual Function and Genotype-Phenotype Correlations in PDE6A-Associated Retinitis Pigmentosa.

Purpose: Autosomal recessive retinitis pigmentosa (arRP) can be caused by mutations in the phosphodiesterase 6A (PDE6A) gene. Here, we describe the natural course of disease progression with respect to central retinal function (i.e.

Clinical Phenotype of -Associated Retinitis Pigmentosa.

In this retrospective, longitudinal, observational cohort study, we investigated the phenotypic and genotypic features of retinitis pigmentosa associated with variants in the gene. Patients underwent clinical examination and genetic testing at a single tertiary referral center, including best-corrected visual acuity (BCVA), kinetic visual field (VF), full-field electroretinography, full-field stimulus threshold, spectral domain optical coherence tomography, and fundus autofluorescence imaging. The genetic testing comprised candidate gene sequencing, inherited retinal disease gene panel sequencing, whole-genome sequencing, and testing for familial variants by Sanger sequencing.

Identification of Postoperative Foveal Displacement after Macular Surgery for Idiopathic Epiretinal Membrane.

: To analyze foveal displacement after macular surgery for idiopathic epiretinal membrane (iERM). : Twenty-eight patients who underwent macular surgery for symptomatic iERM in one eye by one physician were included in this retrospective study. Spectral domain optical coherence tomography (SD-OCT) volume scans were acquired with a Spectralis OCT device (Heidelberg Spectralis).

Clinical Phenotype and Course of PDE6A-Associated Retinitis Pigmentosa Disease, Characterized in Preparation for a Gene Supplementation Trial.

Importance: Treatment trials require sound knowledge on the natural course of disease.

Objective: To assess clinical features, genetic findings, and genotype-phenotype correlations in patients with retinitis pigmentosa (RP) associated with biallelic sequence variations in the PDE6A gene in preparation for a gene supplementation trial.

Design, Setting, And Participants: This prospective, longitudinal, observational cohort study was conducted from January 2001 to December 2019 in a single center (Centre for Ophthalmology of the University of Tübingen, Germany) with patients recruited multinationally from 12 collaborating European tertiary referral centers.

Chromatic Pupil Campimetry Reveals Functional Defects in Exudative Age-Related Macular Degeneration with Differences Related to Disease Activity.

Purpose: The purpose of this study was to use chromatic pupil campimetry (CPC) for an objective evaluation of local retinal function in exudative age-related macular degeneration (AMD) and to assess disease activity.

Methods: Gaze-controlled CPC was performed in 19 subjects with optical coherence tomography-confirmed exudative AMD (75 ± 4 years; 11 women) and the results compared with those of an age-matched control group (n = 11; 72 ± 6 years; 8 women). Local retinal function was evaluated by measuring pupil responses to 3° red stimuli (60 cd/m, 1 second) at 41 positions covering 30° of the central visual field on a dim blue background (test duration 6 minutes).

Clinical Protocols for the Evaluation of Rod Function.

This work presents a quick clinical protocol for dark-adapted chromatic (DAC) perimetry as well as a novel clinical tool, scotopic chromatic pupil campimetry (CPC). The goal of the study was to explore the applicability of these methods in a clinical setting, their test-retest repeatability, and the congruence of the results. Local rod sensitivity was assessed at 36 locations within 30° eccentricity of the visual field in 15 healthy subjects (mean age 43 ± 16 years; 7 females and 8 males) with DAC perimetry (red and cyan stimuli) and CPC 2 times in repeated measurements.

The perception threshold of the panda illusion, a particular form of 2D pulse-width-modulated halftone, correlates with visual acuity.

To call attention to the danger of extinction of the panda bear, the Lithuanian artist Ilja Klemencov created the artwork "They can disappear". The illustration is composed of black-and-white zigzagged lines, which form the famous panda logo of the World Wild Fund For Nature (WWF) when seen from a distance. If one is too close to the artwork, it is difficult to spot the bear, however, if one steps back or takes off one's glasses the panda suddenly appears.

Safety and Vision Outcomes of Subretinal Gene Therapy Targeting Cone Photoreceptors in Achromatopsia: A Nonrandomized Controlled Trial.

Importance: Achromatopsia linked to variations in the CNGA3 gene is associated with day blindness, poor visual acuity, photophobia, and involuntary eye movements owing to lack of cone photoreceptor function. No treatment is currently available.

Objective: To assess safety and vision outcomes of supplemental gene therapy with adeno-associated virus (AAV) encoding CNGA3 (AAV8.

Rebound Phenomenon after Intravitreal Injection of Triamcinolone Acetonide for Macular Edema.

Introduction: The aim of this article was to report on a rebound phenomenon after intravitreal triamcinolone acetonide (IVTA) injection for macular edema secondary to diabetic retinopathy (DR) and central or branch retinal vein occlusion (CRVO/BRVO).

Methods: The data were analyzed retrospectively. Complete ophthalmic examinations, including spectral domain optical coherence tomography, were performed before and 2 months after IVTA injection.

Chromatic Full-Field Stimulus Threshold and Pupillography as Functional Markers for Late-Stage, Early-Onset Retinitis Pigmentosa Caused by Mutations.

Purpose: Mutations in the gene cause early-onset retinal degeneration (EORD). Clinical disease progression markers, such as visual fields or electrophysiology, are not reliably measurable in most patients to follow the retinal function in patients with -mutations.

Methods: Ten patients (five females, five males; age 22-56 years) with EORD caused by mutations were examined in a cross-sectional manner using best corrected visual acuity (BCVA), perimetry, full-field and multifocal electroretinography, full-field stimulus threshold (FST), and pupillography to red and blue light.

Objective Measurement of Local Rod and Cone Function Using Gaze-Controlled Chromatic Pupil Campimetry in Healthy Subjects.

Purpose: We introduce a new approach for functional mapping of rod and cone activity by measuring pupillary responses to local stimulation via gaze-controlled chromatic pupil campimetry (CPC).

Methods: Pupillary constriction amplitude and latency to constriction onset to local photopic and scotopic light stimuli at different locations within the 30° central visual field were analyzed in 14 healthy subjects (4 males, 34 ± 11 years, mean ± standard deviation [SD]). All subjects were measured twice for evaluating the test-retest variability and reproducibility of the method.

Efficacy and Safety of Retinal Gene Therapy Using Adeno-Associated Virus Vector for Patients With Choroideremia: A Randomized Clinical Trial.

Importance: Choroideremia (CHM) is a rare, degenerative, genetic retinal disorder resulting from mutation of the CHM gene, leading to an absence of functional ras-associated binding escort protein 1 (REP1). There is currently no approved treatment for CHM.

Objective: To assess the safety and efficacy of retinal gene therapy with an adeno-associated virus vector (AAV2) designed to deliver a functional version of the CHM gene (AAV2-REP1) for treatment of patients with choroideremia.

[Development and validation of novel clinical endpoints in intermediate age-related macular degeneration in MACUSTAR].

Background: Currently, no validated clinical endpoints for treatment studies exist for intermediate age-related macular degeneration (iAMD).

Objective: The European MACUSTAR study aims to develop and clinically validate adequate clinical endpoints for future treatment studies in iAMD and to identify early determinants of disease progression to late stage AMD.

Material And Methods: The MACUSTAR study protocol was developed by an international consortium of researchers from academia, the pharmaceutical industry and medical device companies.

Changes in microchip position after implantation of a subretinal vision prosthesis in humans.

Purpose: Retinal prosthetic devices have been developed to partially restore very low vision in legally blind patients with end-stage hereditary retinal dystrophies. Subretinal implants, unlike epiretinal implants, are not fixated by a tack. The aim of this study was to assess and analyse possible changes over time in the subretinal position of the RETINA IMPLANT Alpha IMS and Alpha AMS (ClinicalTrials.

Phenotypic spectrum of autosomal recessive retinitis pigmentosa without posterior column ataxia caused by mutations in the FLVCR1 gene.

Purpose: Posterior column ataxia and retinitis pigmentosa (PCARP) is a rare form of syndromic RP associated with mutations in the FLVCR1 gene. Recent evidence has suggested a spectrum in the phenotype depending on the genotype.

Methods: Six individuals with retinitis pigmentosa (RP) carrying mutations in the FLVCR1 gene underwent detailed ophthalmological examinations at the Center for Ophthalmology and two of these also an extensive neurological examination at the Department of Neurology in Tuebingen, Germany.

CHANGES IN RETINAL SENSITIVITY AFTER GENE THERAPY IN CHOROIDEREMIA.

Purpose: Choroideremia (CHM) is a rare inherited retinal degeneration resulting from mutation of the CHM gene, which results in absence of functional Rab escort protein 1 (REP1). We evaluated retinal gene therapy with an adeno-associated virus vector that used to deliver a functional version of the CHM gene (AAV2-REP1).

Methods: THOR (NCT02671539) is a Phase 2, open-label, single-center, randomized study.

Intravitreal Ranibizumab Therapy for Diabetic Macular Edema in Routine Practice: Two-Year Real-Life Data from a Non-interventional, Multicenter Study in Germany.

Introduction: The prospective, non-interventional OCEAN study examined the use of intravitreal ranibizumab injections for the treatment of diabetic macular oedema (DME) in a real-world setting in Germany.

Methods: Adults with DME receiving ≥ 1 ranibizumab (0.5 mg) injections were recruited by 250 ophthalmologists.