Fundus Fluorescein Angiography in Fundus Flavimaculatus and Stargardts Disease

Abstract

Three siblings who had fundus flavimaculatus and two patients who had Stargardts disease were studied by means of fundus fluorescein angiography. The angiograms revealed in all cases an abolished visibility of the chorioidal circulation. New flecks are usually non-fluorescent. Later on, hyperfluorescent areas are seen at identical places both in the preretinal and retinal phases, strongly indicating a window effect of the retinal layer. The missing chorioidal flush is probably due to a blocking effect of the emitting and exciting light. Some of the retinal flecks may fade away, leaving corresponding areas of hyperfluorescence that usually persist. In some cases, however, a previous fluorescent area may become non-fluorescent. The similar angiographic picture may indicate that fundus flavimaculatus and Stargardts disease are different expressions of the same disease.

Clinical spectrum, genetic complexity and therapeutic approaches for retinal disease caused by ABCA4 mutations

“…Most ABCA4 -associated retinopathy patients exhibit a spatially homogenous and localized increase in RPE lipofuscin because of ABCA4 dysfunction ( Cideciyan et al, 2004 ). Augmented RPE lipofuscin confers a Vermillion hue to the fundus under white light imaging and can obstruct fluorescence emanating from the underlying choroid in during fluorescein angiograms (FA) giving rise to the distinct “dark” or “silent” choroid in up to 62% of patients ( Anmarkrud, 1979 ; Ernest and Krill, 1966 ; Fish et al, 1981 ). Despite the historical utility of FA, its role in the evaluation of STGD1 has been increasingly limited as it is no longer performed on a routine basis in favor of newer, less invasive imaging modalities.…”

Macular Dystrophies

“…107,108 In these angiograms, the retinal vessels stand out in sharp contrast to the dark background (Fig. With angiography, the A2E blocks the exciting blue light from reaching the dye in the choroidal circulation, resulting in a finding that is variously known as a dark, silent, or masked choroid.…”

Distrofia macular Stargardt-like dominante

“…bisretinoide acumulado adjacente à coroide bloqueia a excitação luminosa do corante situado na circulação coroidea, achado denominado silêncio coroideo -deixando assim os vasos retinianos em evidência banhados em um fundo hipofluorescente 3,4 . Observase também atrofia do EPR, maculopatia em "olho de boi", "flecks" hiperfluorescentes e preservação do EPR peridiscal ("peripapillary sparing") 5 .…”