The Progression of Stargardt Disease as Determined by Spectral-Domain Optical Coherence Tomography over a 24-Month Period (ProgStar Report No. 18).

Introduction: The aim of this study was to evaluate the progression of atrophy as determined by spectral-domain optical coherence tomography (SD-OCT) in patients with molecularly confirmed ABCA4-associated Stargardt disease type 1 (STGD1) over a 24-month period in a multicenter prospective cohort study.

Methods: SD-OCT images from 428 eyes of 236 patients were analyzed. Change of mean thickness (MT) and intact area were estimated after semiautomated segmentation for the following individual layers in the central subfield (CS), inner ring (IR), and outer ring (OR) of the ETDRS grid: retinal pigment epithelium (RPE), outer segments (OSs), inner segments (IS), outer nuclear layer (ONL) inner retina (IR), and total retina.

An Open-Label Phase II Study Assessing the Safety of Bilateral, Sequential Administration of Retinal Gene Therapy in Participants with Choroideremia: The GEMINI Study.

Choroideremia, an incurable, progressive retinal degeneration primarily affecting young men, leads to sight loss. GEMINI was a multicenter, open-label, prospective, two-period, interventional Phase II study assessing the safety of bilateral sequential administration of timrepigene emparvovec, a gene therapy, in adult males with genetically confirmed choroideremia (NCT03507686, ClinicalTrials.gov).

Role of inflammation in a rat model of radiation retinopathy.

Radiation retinopathy (RR) is a major side effect of ocular tumor treatment by plaque brachytherapy or proton beam therapy. RR manifests as delayed and progressive microvasculopathy, ischemia and macular edema, ultimately leading to vision loss, neovascular glaucoma, and, in extreme cases, secondary enucleation. Intravitreal anti-VEGF agents, steroids and laser photocoagulation have limited effects on RR.

Ptosis secondary to orbital metastasis undetected by magnetic resonance imaging: A case report.

We report a patient with isolated ptosis secondary to orbital metastasis but no evidence of a neoplastic process on magnetic resonance imaging (MRI). A 69-year-old male was referred to our hospital with ptosis of the right upper eyelid and secondary frontalis muscle overaction on the left side for six months. The palpebral fissure was 3mm on the right and 16mm on the left, and levator function was 6mm and 19mm respectively.

Coding and non-coding variants in the ciliopathy gene cause early-onset non-syndromic retinal degeneration.

Inherited retinal degenerations are blinding genetic disorders characterized by high genetic and phenotypic heterogeneity. The implementation of next-generation sequencing in routine diagnostics, together with advanced clinical phenotyping including multimodal retinal imaging, have contributed to the increase of reports describing novel genotype-phenotype associations and phenotypic expansions. In this study, we describe sixteen families with early-onset non-syndromic retinal degenerations in which affected probands carried rare bi-allelic variants in , a ciliary gene previously associated with syndromic recessive Jeune syndrome.

FAST® questionnaire for identifying glaucoma patients at risk for ocular surface disease.

Objectives: To evaluate the validity and reliability of the new Fast Assessment of the Ocular Surface Trouble (FAST®) questionnaire for identifying glaucoma or ocular hypertension (OHT) patients at risk of ocular surface disease (OSD).

Methods: A multicenter, international, cross-sectional, epidemiological survey evaluated the most accurate interview items and ocular signs on the initial 14-item version of FAST® to develop a shorter version for routine, quick clinical use. Rasch analysis and least absolute shrinkage and selection operator (LASSO) method was used to reduce the number of items on the questionnaire.

Splenic monocytes drive pathogenic subretinal inflammation in age-related macular degeneration.

Age-related macular degeneration (AMD) is invariably associated with the chronic accumulation of activated mononuclear phagocytes in the subretinal space. The mononuclear phagocytes are composed of microglial cells but also of monocyte-derived cells, which promote photoreceptor degeneration and choroidal neovascularization. Infiltrating blood monocytes can originate directly from bone marrow, but also from a splenic reservoir, where bone marrow monocytes develop into angiotensin II receptor (ATR1) splenic monocytes.

Functional Vision in Patients With Biallelic USH2A Variants.

Purpose: To describe functional vision (FV) and investigate the relationship between FV, visual acuity (VA), and hill of vision (V) at baseline in patients with biallelic USH2A variants.

Design: Multicenter, international, cross-sectional study.

Methods: In individuals with biallelic disease-causing variants in USH2A, clinical diagnosis of Usher syndrome type 2 (USH2) or autosomal recessive nonsyndromic retinitis pigmentosa (ARRP) was based on history of hearing loss and audiology examinations.

Comparison of optical aberrations in keratoconus with scleral versus rigid gas permeable lenses.

Purpose: to assess optical aberrations under scleral (SL) versus rigid gas permeable (RGP) lenses in patients with keratoconus.

Methods: A prospective study including 25 eyes of 14 patients. The best-corrected visual acuity (BCVA) with corrective glasses, RGP and SL, stage of keratoconus (Amsler-Krumeich classification), minimum pachymetry, maximum keratometry, and corneal higher-order aberrations (i.

Corneal neuroepithelial compartmentalized microfluidic chip model for evaluation of toxicity-induced dry eye.

Part of the lacrimal functional unit, the cornea protects the ocular surface from numerous environmental aggressions and xenobiotics. Toxicological evaluation of compounds remains a challenge due to complex interactions between corneal nerve endings and epithelial cells. To this day, models do not integrate the physiological specificity of corneal nerve endings and are insufficient for the detection of low toxic effects essential to anticipate Toxicity-Induced Dry Eye (TIDE).

Use of the Three-dimensional Viewing System and Microscope Tilting to Extend the Peripheral Retinal View.

Purpose: To describe and evaluate the effectiveness of the microscope and lens tilting technique associated with the three-dimensional viewing system for improving the peripheral retinal view in noncontact lens vitreoretinal surgeries.

Methods: Prospective, single-center, single-surgeon, consecutive case series of 25 patients undergoing vitrectomy for macular surgeries with three-dimensional visualization system. At the end of each surgery, the microscope and the noncontact lens were rotated by 20° in a direction opposite to the rotation of the eye to extend the peripheral visual field.

[Biomechanics of the lamina cribrosa: A determining factor in glaucomatous neuropathy. A review of the literature].

Glaucoma is a chronic optic neuropathy characterized by progressive sclero-laminar remodeling. The main factor at the origin of these deformations is the intraocular pressure (IOP), the effect of which varies according to the biomechanical properties of the individual lamina cribrosa (LC). In this environment, the LC represents a malleable zone of weakness within a rigid corneoscleral shell.

A new method for in vivo assessment of corneal transparency using spectral-domain OCT.

Corneal transparency is essential to provide a clear view into and out of the eye, yet clinical means to assess such transparency are extremely limited and usually involve a subjective grading of visible opacities by means of slit-lamp biomicroscopy. Here, we describe an automated algorithm allowing extraction of quantitative corneal transparency parameters with standard clinical spectral-domain optical coherence tomography (SD-OCT). Our algorithm employs a novel pre-processing procedure to standardize SD-OCT image analysis and to numerically correct common instrumental artifacts before extracting mean intensity stromal-depth (z) profiles over a 6-mm-wide corneal area.

Evaluation of the efficacy and safety of pars plana vitrectomy with irido-zonulo-hyaloidotomy for malignant glaucoma.

Purpose: To assess the efficacy and safety of pars plana vitrectomy with irido-zonulo-hyaloidotomy (IZH) for fluid misdirection syndrome (FMS) in pseudophakic eyes.

Methods: This was a retrospective case series study of patients treated with pars plana vitrectomy with IZH for FMS between February 2017 and March 2020. Complete success was defined as central anterior chamber (AC) deepening with an intraocular pressure (IOP) of 21mmHg or less (on 2 consecutive visits at least 1 week apart) without topical or systemic glaucoma medications.

Freeze-dried amniotic membrane graft with a spongy layer in bilateral peripheral ulcerative keratitis: a case report.

Background: Peripheral ulcerative keratitis (PUK) is a group of inflammatory corneal ulcers with stromal thinning and peripheral localization. Amniotic membranes (AM) are used for their anti-inflammatory and healing properties. A freeze-drying process now allows maintaining the AM viable for a long time at room temperature without altering its physical, biological, and morphologic characteristics.

Management of Neovascular Age-Related Macular Degeneration Treatment in France from 2008-2018: The Nationwide LANDSCAPE Study.

Introduction: The aim of this study was to describe the management of neovascular age-related macular degeneration (nAMD) in French patients between 2008 and 2018.

Methods: This was a retrospective longitudinal cohort study using exhaustive nationwide health records from the French National Health Information database. Enrollment criteria were adults aged ≥ 50 years, nAMD diagnosis, or reimbursement for nAMD treatments (anti-vascular epithelial growth factor [VEGF] injection or dynamic phototherapy with verteporfin).