Fundus autofluorescence in stargardt macular dystrophy–fundus flavimaculatus

Lois, Noemi; Halfyard, Anthony S; Bird, Alan C.; Holder, Graham E.; Fitzke, F.W.

doi:10.1016/j.ajo.2004.02.056

Cited by 186 publications

(161 citation statements)

References 17 publications

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“…32 In various degenerative retinal disorders, increased FAF correlated with lipofuscin accumulation, whereas reduced or absent FAF indicated blockage (eg, retinal vessels), loss of RPE cells, or absence of RPE phagocytosis. 26,27,[33][34][35][36][37][38][39][40] The patterns of FAF distribution in RP patients observed in this study are similar to those reported previously. Ophthalmoscopically preserved areas of RPE at the posterior pole correspond to detectable FAF.…”

Section: Discussionsupporting

confidence: 80%

Lipofuscin- and melanin-related fundus autofluorescence visualize different retinal pigment epithelial alterations in patients with retinitis pigmentosa

Kellner

Weber

et al. 2008

Eye

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Aims To compare melanin-related nearinfrared fundus autofluorescence (FAF; NIA, excitation 787 nm, emission 4800 nm) with lipofuscin-related FAF (excitation 488 nm, emission 4500 nm) in retinitis pigmentosa (RP). Methods Thirty-three consecutive RP patients with different modes of inheritance were diagnosed clinically, with full-field ERG, and if possible with molecular genetic methods. FAF and NIA imaging were performed with a confocal scanning laser ophthalmoscope (Heidelberg Retina Angiograph 2). Results Rings of increased FAF were present within an area of preserved retinal pigment epithelium (RPE) at the posterior pole (31/33). Rings of increased NIA were located in the same region as rings of increased FAF. In contrast to FAF, NIA showed a precipitous decline of NIA peripheral to the ring. In larger areas of preserved NIA (11/31), pericentral and foveal NIA were of similar intensity with an area of lower NIA in between. In smaller areas of preserved NIA (20/31), NIA was homogeneous from the perifovea to the fovea. In one patient without a ring of increased FAF, NIA distribution was normal. In the remaining patient with severely advanced RP, no residual RPE as well as no FAF and NIA were detectable. Conclusion Characteristic features for FAF and NIA alterations in a heterogeneous group of RP patients indicate a common pathway of RPE degeneration. Patterns of NIA and FAF indicate different pathophysiologic processes involving melanin and lipofuscin. Combined NIA and FAF imaging will provide further insight into the pathogenesis of RP and noninvasive monitoring of future therapeutic interventions.

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Section: Discussionsupporting

confidence: 80%

Lipofuscin- and melanin-related fundus autofluorescence visualize different retinal pigment epithelial alterations in patients with retinitis pigmentosa

Kellner

Weber

et al. 2008

Eye

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“…The accumulation of autofluorescent pigment is seen in multiple disorders that affect the RPE and choroid. 4,5 FAF derives from RPE and Bruch membrane, since the signal appears to be derived from the anterior to the choriocapillaris and posterior to the neuroretina. 3 In dry age-related macular degeneration (ARMD) increased FAF was found to correlate with the areas of hyperpigmentation, soft drusen, hard drusen, or normal fundus appearance.…”

Section: Introductionmentioning

confidence: 99%

Changes in fundus autofluorescence of choroidal melanomas following treatment

Amselem

Pulido

Günduz

et al. 2007

Eye

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Purpose We have previously shown that fundus autofluorescence (FAF) associated with pigmented choroidal lesions can be attributed to mainly lipofuscin (orange pigment) but also to hyperpigmentation, drusen, or fibrous metaplasia. The purpose of this study is to describe the effects of treatment on FAF in choroidal melanomas after plaque radiotherapy alone or in combination with transpupillary thermotherapy (TTT). Methods Retrospective chart review of eight consecutive patients with choroidal melanoma treated with plaque radiotherapy alone or in combination with TTT who underwent FAF photography before and after treatment. The correlation between FAF patterns and foci of orange pigment, hyperpigmentation, drusen, or fibrous metaplasia was evaluated. Results The median follow-up time was 4 (range 2-9) months. Foci of orange pigment and hyperpigmentation became larger and more numerous after treatment. Fibrous metaplasia was also increased. A complete correlation between increased FAF and orange pigment was found in all eight tumours (100%) before and after treatment. No correlation between hyperpigmentation and increased FAF was found before treatment but a partial correlation was found in all eyes after treatment. Before treatment, correlation between fibrous metaplasia was present in three eyes and increased FAF was partial in two eyes with no correlation in one case. After treatment, this correlation was partial in all presenting eyes (7). Conclusions Following treatment, choroidal melanomas may show increased FAF, mainly due to an increase in the amount of lipofuscin (orange pigment) and hyperpigmentation.

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“…Stargardt disease patients commonly show a "dark choroid" during fluorescein angiography due to this light-absorbing lipofuscin material in the RPE (111). Stargardt disease patients also show fundus autofluorescence by scanning laser ophthalmoscopy, again owing to these lipofuscin pigments (112). Stargardt disease is caused by mutations in the ABCR (ABCA4) gene (6).…”

Section: Stargardt Macular Degenerationmentioning

confidence: 99%

Diseases Caused by Defects in the Visual Cycle: Retinoids as Potential Therapeutic Agents

Travis

Golczak

Moise

et al. 2007

Annu. Rev. Pharmacol. Toxicol.

369

452

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Absorption of a photon by an opsin pigment causes isomerization of the chromophore from 11-cisretinaldehyde to all-trans-retinaldehyde. Regeneration of visual chromophore following light exposure is dependent on an enzyme pathway called the retinoid or visual cycle. Our understanding of this pathway has been greatly facilitated by the identification of disease-causing mutations in the genes coding for visual cycle enzymes. Defects in nearly every step of this pathway are responsible for human-inherited retinal dystrophies. These retinal dystrophies can be divided into two etiologic groups. One involves the impaired synthesis of visual chromophore. The second involves accumulation of cytotoxic products derived from all-trans-retinaldehyde. Gene therapy has been successfully used in animal models of these diseases to rescue the function of enzymes involved in chromophore regeneration, restoring vision. Dystrophies resulting from impaired chromophore synthesis can also be treated by supplementation with a chromophore analog. Dystrophies resulting from the accumulation of toxic pigments can be treated pharmacologically by inhibiting the visual cycle, or limiting the supply of vitamin A to the eyes. Recent progress in both areas provides hope that multiple inherited retinal diseases will soon be treated by pharmaceutical intervention.

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Fundus autofluorescence in stargardt macular dystrophy–fundus flavimaculatus

Cited by 186 publications

References 17 publications

Lipofuscin- and melanin-related fundus autofluorescence visualize different retinal pigment epithelial alterations in patients with retinitis pigmentosa

Lipofuscin- and melanin-related fundus autofluorescence visualize different retinal pigment epithelial alterations in patients with retinitis pigmentosa

Changes in fundus autofluorescence of choroidal melanomas following treatment

Diseases Caused by Defects in the Visual Cycle: Retinoids as Potential Therapeutic Agents

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