Discrete arcs of increased fundus autofluorescence in retinal dystrophies and functional correlate on microperimetry

Abstract

Purpose To describe the occurrence of discrete arcs of increased fundus autofluorescence (FAF) associated with various retinal dystrophies and to assess their functional significance by fundus-controlled microperimetry. Methods Seven patients, three with pigmented paravenous retinochoroidal atrophy (PPRCA), one with sector retinitis pigmentosa (RP), one with typical RP, and two with macular dystrophy were assessed by retinal imaging including FAF imaging. Serial images were obtained within a review period of 6 and 10 years in a patient with PPRCA and macular dystrophy, respectively. Funduscontrolled microperimetry was performed in eight eyes of five patients to determine light increment sensitivity. Results A discrete arched line of increased FAF was observed without obvious correlate on fundus biomicroscopy. The orientation of this line differed from ring shape in RP and macular dystrophy, a semi-circle structure in sector RP to crescent shape with tiplike extensions towards branching retinal veins in PPRCA. Longitudinal investigation revealed slight migration of the arc in PPRCA and peripheral extension of the ring diameter in macular dystrophy. Microperimetry revealed that the arc of increased FAF sharply delineated areas of severely impaired retinal sensitivity. Conclusions The findings indicate that arcs of increased FAF in PPRCA and other retinal dystrophies demarcate areas of impaired retinal function and may migrate over time. FAF imaging may therefore reveal the exact extent of neurosensory dysfunction that may exceed the dimensions anticipated by conventional examinations.

Fundus autofluorescence and retinal structure as determined by spectral domain optical coherence tomography, and retinal function in retinitis pigmentosa

“…This is assessed by multifocal ERG [2,8,10,12] and with the visual field determined by several methods, such as kinetic perimetry [9], Humphrey static perimetry [10,13], and microperimetry [6]. The current study confirmed these results by showing that the MD of the Humphrey static perimetry correlated well with the area of and within the hyperautofluorescent ring.…”

“…That is, absent autofluorescence that represents RPE atrophy and autofluorescence that surrounds the residual area in a dot-like or patch-like pattern can be clearly detected. Additionally, an abnormal hyperautofluorescent ring in the parafoveal area has attracted much attention [1][2][3][4][5][6][7][8][9][10][11][12][13]. Recent investigations have suggested that the hyperautofluorescent ring may be an excellent indicator of retinal dysfunction as evaluated by focal electroretinography (ERG), pattern ERG [3,8,12,13] and microperimetry [6,12].…”

Two-photon microscopy reveals early rod photoreceptor cell damage in light-exposed mutant mice

“…Finally, we provide evidence that phagocytosis-mediated transfer of rod-derived toxic compounds to the retinal pigmented epithelium is required to elicit damage to that cell layer. direct toxicity to the retina (1,(26)(27)(28). Identification of the primary cause of retinal degeneration, whether it is atRAL or its condensation products, as well as determination of which cell types are initially affected comprise two particularly intriguing questions that need answers to guide the development of optimal therapeutic interventions.…”

Wide-Field Fundus Autofluorescence for Retinitis Pigmentosa and Cone/Cone-Rod Dystrophy

“…The most studied feature is a ring of increased FAF around the fovea. The hyperautofluorescent ring demarcates the preserved and damaged areas of the retina (Robson et al 2004(Robson et al , 2006Murakami et al 2008;Fleckenstein et al 2009;Lima et al 2009Lima et al , 2012. Retinal sensitivity decreases and outer retinal structures depicted on OCT become disrupted outside the ring.…”