Publications by authors named "Etienne Schonbach"
Purpose: Ultra-widefield (UWF) imaging is commonly used in ophthalmology in tandem with scleral depressed examinations (SDE) to evaluate peripheral retinal disease. Because of the increased reliance on this technology in tele-ophthalmology, it is critical to evaluate its efficacy for detecting the peripheral retina when performed in isolation. Therefore, we sought to evaluate UWF imaging sensitivity in detecting retinal horseshoe tears (HSTs).
View Article and Find Full Text PDFPurpose: To estimate the progression rate of atrophic lesions in Stargardt disease derived from fundus autofluorescence (FAF).
Design: International, multicenter, prospective cohort study.
Methods: A total of 259 participants aged ≥6 years with disease-causing variants in the ABCA4 gene were enrolled from 9 centers and followed over a 24-month period.
Purpose: To investigate closure rates and functional outcomes of surgery for refractory and recurrent macular holes (MHs) in a real-world setting.
Methods: Retrospective review of secondary MH surgeries.
Results: A total of 72 eyes from 72 patients were included.
Purpose: Stargardt disease type 1 (STGD1) is the most common macular dystrophy. The assessment of fixation describes an important dimension of visual function, but data on its progression over time are limited. We present longitudinal changes and investigate its usefulness for clinical trials.
View Article and Find Full Text PDFPurpose: To report the yearly rate of change in macular function in patients with Stargardt disease type 1 (STGD1) over 24 months and to establish a new volumetric visual function index for use in clinical trials investigating the efficacy on retinal sensitivity.
Methods: Design: International, multicenter, prospective cohort study with 5 study visits every 6 months over 24 months.
Participants: A total of 233 individuals with genetically confirmed STGD1 (≥1 disease-causing ABCA4 variant).
Purpose: To report a case of von Hippel-Lindau syndrome in a monocular patient with progressive vision loss due to a juxtapapillary capillary retinal angioma and an epiretinal membrane (ERM) formation.
Patient: We describe a 37-year-old white male patient with von Hippel-Lindau syndrome who presented for retinal evaluation. The right eye was blind with no light perception vision.
Importance: Functional end points for clinical trials investigating the efficacy of emerging treatments for Stargardt disease type 1 (STGD1) are needed.
Objective: To assess the yearly rate of change of macular function in patients with STGD1 using microperimetry.
Design, Setting, And Participants: This multicenter prospective cohort study was conducted in an international selection of tertiary referral centers from October 21, 2013, to February 15, 2017.
Purpose: Sensitive, reproducible visual function biomarkers are necessary to evaluate the efficacy of emerging treatments for Stargardt disease type 1 in clinical trials. We previously demonstrated that fixation stability may serve as a secondary outcome parameter for visual function loss. However, the test duration and protocol have an unknown effect on the assessment of fixation stability.
View Article and Find Full Text PDFPurpose: Mean sensitivity (MS) derived from a standard test grid using microperimetry is a sensitive outcome measure in clinical trials investigating new treatments for degenerative retinal diseases. This study hypothesizes that the functional decline is faster at the edge of the dense scotoma (eMS) than by using the overall MS.
Design: Multicenter, international, prospective cohort study: ProgStar Study.
Background/purpose: To report a case of unilateral choroidal detachment and serous retinal detachment in a patient with a history of untreated sarcoidosis.
Methods: Case report. The patient is a 67-year-old African American man with a history of nontreated sarcoidosis and prostate cancer.
The Progression of Atrophy Secondary to Stargardt Disease (ProgStar) studies were designed to measure the progression of Stargardt disease through the use of fundus autofluorescence imaging, optical coherence tomography, and microperimetry. The overarching objectives of the studies were to document the natural course of Stargardt disease and identify the most appropriate clinical outcome measures for clinical trials assessing the efficacy and safety of upcoming treatments for Stargardt disease. A workshop organized by the Foundation Fighting Blindness Clinical Research Institute was held on June 11, 2018, in Baltimore, MD, USA.
View Article and Find Full Text PDFImportance: It is important to establish reliable outcome measures to detect progression in retinitis pigmentosa (RP).
Background: To evaluate progression of RP using multimodal imaging, including spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF) and microperimetry (MP).
Design: Retrospective longitudinal study at a tertiary teaching hospital.
Purpose: To assess the sensitivity and specificity of microperimetry as a screening test to detecting hydroxychloroquine retinopathy.
Methods: Retrospective cohort study. Patients with history of hydroxychloroquine use for more than 5 years and with concomitant microperimetry and multifocal electroretinogram testing were retrospectively reviewed.
Background/aims: To describe the design and baseline characteristics of patients enrolled in the multicenter, prospective natural history study of Stargardt disease type 4.
Methods: Fifteen eligible patients aged 6 years and older at baseline, harboring disease-causing variants in the PROM1 gene, and with specified ocular lesions were enrolled. They were examined at baseline using a standard protocol, with 6 monthly follow-up visits for a 2-year period including best-corrected ETDRS visual acuity, spectral-domain optical coherence tomography, fundus autofluorescence (FAF), mesopic and scotopic microperimetry (MP).
Purpose: To describe the study design and characteristics at first visit of participants in the longitudinal Scotopic Microperimetric Assessment of Rod Function in Stargardt Disease (SMART) study.
Methods: Scotopic microperimetry (sMP) was performed in one designated study eye in a subset of participants with molecularly proven ABCA4-associated Stargardt disease (STGD1) enrolled in a multicenter natural history study (ProgStar). Study visits were every 6 months over a period ranging from 6 to 24 months, and also included fundus autofluorescence (FAF).
Importance: Limited data from prospective studies are available to understand the natural history of ABCA4-related Stargardt disease (STGD1). Such data are important for determining appropriate outcome measures for future STGD1 trials.
Objective: To estimate the rate of loss of best-corrected visual acuity (BCVA) during 2 years and to estimate the associations of BCVA loss with foveal phenotype and genotype in patients with STGD1.
Purpose: To investigate the natural history of Stargardt disease (STGD1) using fixation location and fixation stability.
Design: Multicenter, international, prospective cohort study.
Methods: Fixation testing was performed using the Nidek MP-1 microperimeter as part of the prospective, multicenter, natural history study on the Progression of Stargardt disease (ProgStar).
Purpose: To compare different metrics and acquisition modes of fixation stability as a new visual function biomarker in a large cohort of patients with ABCA4-related Stargardt disease from the multicenter prospective ProgStar study.
Methods: Fixation was tested during a separate fixation exam and also dynamically during a sensitivity exam, using fundus-tracking microperimetry (Nidek MP-1). Fixation data were analyzed using the bivariate contour ellipse area (BCEA), the 2/4 degree method, and the Fujii classification.
Purpose: We present the multifocal electroretinogram (mfERG) with a 7-hexagon array as an objective test of macular function that can be recorded in 14 s. We provide normal values and investigate its reproducibility and validity.
Methods: Healthy participants underwent mfERG testing according to International Society for Clinical Electrophysiology of Vision (ISCEV) standards using the Espion Profile/D310 multifocal ERG system (Diagnosys, LLC, Lowell, MA, USA).
Importance: New outcome measures for treatment trials for Stargardt disease type 1 (STGD1) and other macular diseases are needed. Microperimetry allows mapping of light sensitivity of the macula and provides topographic information on visual function beyond visual acuity.
Objective: To measure and analyze retinal light sensitivity of the macula in STGD1 using fundus-controlled perimetry (microperimetry).
Purpose: To investigate the impact of areas of decreased fundus autofluorescence (AF) on visual acuity (VA) in molecularly confirmed Stargardt disease (STGD1) with recent symptom onset, and investigate the association between these structural and functional measures over time.
Design: Prospective, international, multicenter observational study of Stargardt disease.
Participants: Sixty-four patients (124 eyes) aged ≥6 years at first study visit, with onset of symptoms ≤2 years before the first visit.
Purpose: To determine fixation location and fixation stability in Stargardt disease (STGD1) and their association with best-corrected visual acuity (BCVA).
Design: Cross-sectional analysis within the multicenter, prospective ProgStar study.
Participants: A total of 238 patients and 440 eyes with ABCA4-related STGD1.
Background We have recently shown that the presence of headache in ischemic stroke is associated with lesions of the insular cortex. The aim of this post-hoc subgroup analysis was to investigate the association of specific headache features with stroke location in patients with acute ischemic stroke. Methods In this observational study, patients (mean age: 61.
View Article and Find Full Text PDFBackground Context: Determining pain intensity is largely dependent on the patient's report.
Purpose: The objective of this study was to test the hypothesis that patients initially reporting a pain score of 10 out of 10 on the visual analog scale (VAS) would experience symptom improvement to a degree similar to patients reporting milder pain.
Study Design: This study is a retrospective chart review.