Fundus Autofluorescence Patterns in Best Vitelliform Macular Dystrophy

Fundus autofluorescence (FAF) is a non-invasive retinal imaging modality used in clinical practice to provide a density map of lipofuscin, the predominant ocular fluorophore, in the retinal pigment epithelium. Multiple commercially available imaging systems, including the fundus camera, the confocal scanning laser ophthalmoscope, and the ultra-widefield imaging device, are available to the clinician. Each offers unique advantages for evaluating various retinal diseases. The clinical applications of FAF continue to expand. It is now an essential tool for evaluating age related macular degeneration, macular dystrophies, retinitis pigmentosa, white dot syndromes, retinal drug toxicities, and various other retinal disorders. FAF may detect abnormalities beyond those detected on funduscopic exam, fluorescein angiography, or optical coherence tomography, and can be used to elucidate disease pathogenesis, form genotype-phenotype correlations, diagnose and monitor disease, and evaluate novel therapies. Given its ease of use, non-invasive nature, and value in characterizing retinal disease, FAF enjoys increasing clinical relevance. This review summarizes common ocular fluorophores, imaging modalities, and FAF findings for a wide spectrum of retinal disorders.

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“…Shed photoreceptor debris and lipofuscin accumulate in the subretinal space, resulting in bilateral yolk-like lesions in the macula [124–126]. …”

Section: Macular Dystrophiesmentioning

confidence: 99%

“…The patchy subtype occurred most frequently, followed by the hyper-autofluorescent, then hypo-autofluorescent patterns. These findings did not correlate with the stages of progression or best corrected visual acuity [124]. …”

Section: Macular Dystrophiesmentioning

confidence: 99%

Clinical applications of fundus autofluorescence in retinal disease

2016

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“…subclinical to atrophic stages, and variable functional impairment has been investigated. [2][3][4][5][6][7] Histopathologic findings from donor eyes are limited and refer to an advanced stage of the disease, demonstrating an abnormal accumulation of lipofuscin granules within the RPE cells and photoreceptor degeneration over areas of an intact RPE. [18][19][20][21][22] It is conceivable that the impaired metabolic exchanges related to VMD have a direct impact on the choroidal vasculature of the macula, but unfortunately no histopathologic analysis of the choroid status in eyes affected by the disease is available at present.…”

Section: Discussionmentioning

confidence: 99%

Choroidal Thickness in Best Vitelliform Macular Dystrophy

Parodi

Sacconi

Iacono

et al. 2016

Retina

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“…It tends to decrease in the RPE with age, whereas lipofuscin accumulation gradually increases. Thus, the amount of melanin within RPE cells may have an indirect effect on lipofuscin accumulation and/or the photo-oxidation of lipofuscin-related compounds 30 31…”

Section: Discussionmentioning

confidence: 99%