Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
Grosspoetzl, M.
Evaluation of spectral-domain optical coherence tomography for analysis of progression of atrophy secondary to Stargardt disease typ IV
Doktoratsstudium der Medizinischen Wissenschaft; Humanmedizin; [ Dissertation ] Medizinische Universität Graz; 2024. pp. 83
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- Autor*innen der Med Uni Graz:
- Betreuer*innen:
- Seidel Gerald
- Strauß Rupert
- Wedrich Andreas
- Altmetrics:
- Abstract:
- 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<0.05). Additionally, a significant decrease in intact area of the RPE and IS was noted in the inner ring, and of the ONL in the outer ring (all p<0.05). Trends of decline were observed in MT and intact area of the other layers over the 24-month observational period. Conclusions: Significant thickness losses in outer retinal layers were detected by SD-OCT over 24 months in patients with PROM1-associated RD. Effective treatments to halt or reverse maculopathy in Stargardt's disease are currently lacking. Loss of thickness and/or intact area in these layers may serve as potential endpoints for clinical trials aiming to slow down the disease progression of PROM1-associated RD.