Purpose: To investigate changes in retinal layer thickness and intact retinal area in patients with molecularly confirmed PROM1-associated retinal degeneration (RD) over a 24-month period, utilizing spectral-domain optical coherence tomography (SD-OCT). Retinal layer thickness and intact area are crucial markers of retinal health, particularly in conditions like Stargardt disease, and SD-OCT offers precise imaging for detecting subtle alterations in these parameters. Design: international, multicenter, prospective case series Methods: Thirteen eyes from 13 patients affected by PROM1-associated RD were enrolled at five sites, with SD-OCT images captured at baseline and after 24 months. Mean thickness (MT) loss and intact area were estimated through semi-automated segmentation for various individual retinal layers within the central subfield (CS), inner ring, and outer ring of the early treatment diabetic retinopathy grid (ETDRS): retinal pigment epithelium (RPE), outer segments (OS), inner segments (IS), outer nuclear layer (ONL), inner retina (IR), and total retina (TR). Changes in mean thickness and intact area of individual retinal layers were analyzed as yearly rate differences and progression patterns. Results: Six male and seven female patients, with a mean age of 38.2±14.2 years at baseline and symptom onset at 30.3±16.3 years (available for twelve patients) were enrolled. Mean best-corrected visual acuity at baseline was 0.51±0.53 LogMAR. Statistically significant thickness losses were observed in the RPE and TR in the CS and inner ring, and in the ONL and IS in the outer ring (all p
Evaluation of spectral-domain optical coherence tomography for analysis of progression of atrophy secondary to Stargardt disease typ IV
Großpötzl, M. (Autor/-in). 2024
Studienabschlussarbeit: Dissertation