Multimodal imaging in subclinical best vitelliform macular dystrophy

Area of Science:

• Ophthalmology
• Retinal Imaging
• Macular Dystrophy

Background:

• Best Vitelliform Macular Dystrophy (BVMD) is a genetic condition affecting central vision.
• Early detection of subclinical BVMD is crucial for understanding disease progression.
• Multimodal imaging offers insights into structural and functional changes in retinal diseases.

Purpose of the Study:

• To analyze multimodal imaging alterations in the subclinical form of Best Vitelliform Macular Dystrophy (BVMD).
• To quantitatively assess changes in retinal structure and vasculature in early-stage BVMD.

Main Methods:

• Observational, cross-sectional case series including 7 subclinical BVMD patients and 12 controls.
• Multimodal imaging: fundus autofluorescence (blue and NIR-AF), structural OCT, and OCT angiography (OCTA).
• Quantitative analysis of vessel density, tortuosity, dispersion, rarefaction, foveal avascular zone (FAZ) area, outer retinal band reflectivity, and choriocapillaris porosity (CCP).

Main Results:

• Subclinical BVMD eyes showed significant reflectivity attenuation of outer retinal bands (p<0.001).
• A restricted foveal avascular zone (FAZ) area in the deep capillary plexus (p<0.001) and increased choriocapillaris porosity (CCP) (p<0.001) were observed.
• Vessel density, tortuosity, dispersion, and rarefaction remained comparable to controls.

Conclusions:

• Subclinical BVMD is characterized by significant alterations in outer retinal bands and the choriocapillaris.
• Quantitative multimodal imaging indicates functional impairment of outer retinal structures in subclinical BVMD.
• These impairments may lead to altered melanin and growth factor production in early BVMD stages.