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Ex Vivo OCT-Based Multimodal Imaging of Human Donor Eyes for Research into Age-Related Macular Degeneration
Published on: May 26, 2023
Multimodal fundus imaging in Best vitelliform macular dystrophy
Daniela C Ferrara1, Rogério A Costa, Stephen Tsang
1Department of Ophthalmology, Otolaryngology and Head and Neck Surgery, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brazil.
Summary
Best vitelliform macular dystrophy (BVMD) is characterized by thickening of the photoreceptors' outer segments, not subretinal fluid, in early stages. These changes in reflective layers and outer nuclear layer thinning correlate with vision loss in BVMD patients.
Area of Science:
- Ophthalmology
- Medical Imaging
- Genetics
Background:
- Best vitelliform macular dystrophy (BVMD) is a rare, autosomal dominant retinal disorder with significant variability in its clinical presentation.
- Existing research on BVMD mechanisms presents conflicting interpretations regarding the primary location and extent of retinal alterations.
- This study focuses on characterizing macular morphology in BVMD using advanced imaging techniques.
Purpose of the Study:
- To describe the detailed macular morphological features in patients diagnosed with BVMD.
- To compare the observed macular features in BVMD patients with those of age-matched healthy individuals.
- To utilize simultaneous multimodal fundus imaging for enhanced diagnostic insights into BVMD.
Main Methods:
- A comparative study involving seven BVMD patients (14 eyes) and seven age-matched healthy controls (14 eyes).
- Comprehensive ophthalmological examinations were performed on all participants.
- Standardized multimodal fundus imaging, including Fourier-domain optical coherence tomography (Fd-OCT), near-infrared reflectance, and fundus autofluorescence (FAF), was employed.
Main Results:
- Early-stage BVMD (subclinical) showed Fd-OCT-detected thickening of the middle highly reflective layer (HRL) between photoreceptor inner/outer segments and RPE/Bruch's membrane.
- Vitelliform stage BVMD exhibited homogeneous hyper-reflective material on Fd-OCT and increased FAF, with thinning of the outer nuclear layer (ONL) and no subretinal fluid.
- Advanced stages (pseudohypopyon, vitelliruptive, atrophic) revealed diverse HRL and ONL changes correlating with visual acuity, with HRL thickening extending beyond visible lesions.
Conclusions:
- Multimodal imaging identifies photoreceptor outer segment thickening as the earliest BVMD feature, preceding subretinal fluid.
- Progressive changes in photoreceptor reflective layers (middle- and inner-HRLs) and ONL thinning are key indicators of disease progression in BVMD.
- These imaging-derived changes directly correlate with the degree of visual acuity impairment across different BVMD stages.
