Fundus Autofluorescence and Clinical Applications

Pole, Cameron; Ameri, Hossein

doi:10.18502/jovr.v16i3.9439

Cited by 18 publications

(18 citation statements)

References 212 publications

(248 reference statements)

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“…Autofluorescence from other tissues, as well as the absorption of excitation and emitted light, especially by the crystalline lens, may confound the detection of FAF. Therefore, appropriate imaging technology is required to amplify the FAF signal and to record images of adequate quality and contrast [ 16 , 34 ]. Different devices need to be considered, which differ in their applied excitation and emission wavelengths, scanning mode, and image acquisition.…”

Section: Fundus Autofluorescence Imagingmentioning

confidence: 99%

Fundus Autofluorescence Imaging in Patients with Choroidal Melanoma

Bindewald-Wittich

Holz

Ach

et al. 2022

Cancers

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Choroidal melanocytic lesions require reliable and precise clinical examination and diagnosis to differentiate benign choroidal nevi from choroidal melanoma, as the latter may become life-threatening through metastatic disease. To come to an accurate diagnosis, as well as for monitoring, and to assess the efficacy of therapy, various imaging modalities may be used, one of which is non-invasive fundus autofluorescence (FAF) imaging using novel high-resolution digital imaging technology. FAF imaging is based on the visualization of intrinsic fluorophores in the ocular fundus. Lipofuscin and melanolipofuscin within the postmitotic retinal pigment epithelium (RPE) cells represent the major fluorophores that contribute to the FAF signal. In addition, the presence or loss of absorbing molecular constituents may have an impact on the FAF signal. A choroidal melanoma can cause secondary retinal and RPE alterations that affect the FAF signal (e.g., occurrence of orange pigment). Therefore, FAF imaging supports multimodal imaging and gives additional information over and above conventional imaging modalities regarding retinal metabolism and RPE health status. This article summarises the features of FAF imaging and the role of FAF imaging in the context of choroidal melanoma, both before and following therapeutic intervention.

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Section: Fundus Autofluorescence Imagingmentioning

confidence: 99%

Fundus Autofluorescence Imaging in Patients with Choroidal Melanoma

Bindewald-Wittich

Holz

Ach

et al. 2022

Cancers

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“…CNV used to be reported as a rare complication of BCD. Actually, the incidence of CNV may be higher than reported in the early stage of BCD, different from other non-syndromic RP [30] . Previously reported cases showed the lesions of CNV in BCD were usually observed in the foveal or parafoveal retina, except in one case of peripapillary CNV [34] .…”

Section: Discussionmentioning

confidence: 65%

“…Hyper-fluorescence spots scatter around the hypo-fluorescence regions in the posterior pole. Though autofluorescence in early stage is usually normal, hyper-fluorescence can sometimes be observed [30] .…”

Section: Discussionmentioning

confidence: 99%

A novel mutation of CYP4V2 gene associated with Bietti crystalline dystrophy complicated by choroidal neovascularization

Han¹,

Tang²,

Huang³

et al. 2022

Int J Ophthalmol

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AIM: To investigate the clinical characteristics and genetic features of a Bietti crystalline dystrophy (BCD) proband in a Chinese family. METHODS: A Chinese female diagnosed with BCD complicated by bilateral choroidal neovascularization (CNV) and her parents underwent complete ophthalmic examinations, including fundus autofluorescence (AF), fundus photography (FP), fundus fluorescein angiography (FFA), visual field testing, full-field electroretinography (ERG), optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA). The sequencing of the CYP4V2 gene was performed to the whole family. RESULTS: Bilateral tiny glittering crystal-like deposits and differing extent of atrophy of the retinal pigment epithelium (RPE) were found in the posterior pole of her fundus. The diffuse hypo-fluorescence shown on AF images and window defects shown on FFA both indicated the atrophy of the RPE and choriocapillaris. OCT showed the thinning of the RPE and choriocapillaris layer, ellipsoid zone (EZ) band defect and CNV in both eyes. OCTA images proofed bilateral type 2 CNV. The visual field test showed central and paracentral scotoma. ERG showed a slightly decreased b-wave in scotopic ERG. Gene sequencing identified three mutations of the CYP4V2 gene, c.802_807del, c.810delT, and c.1388G>A. The mutation c.1388G>A was a novel substitution mutation. CONCLUSION: The novel mutation c.1388G>A may be a possible cause that could induce the clinical phenotype of BCD.

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“…Normal SW‐AF shows homogeneous autofluorescence of RPE with a gradual decline in intensity toward the foveola due to high lutein pigment in the foveal region. Foci of hyper‐ and hypo‐autofluorescence, major signs of morbid or atrophic RPE, respectively (Sparrow et al , 2012 ; Pole & Ameri, 2021 ), can be seen in SW‐AF images of the patients with DFO retinopathy. Case I showed stippled hyper‐autofluorescence mainly in the macular region without obvious hypo‐autofluorescence, indicating an RPE injury at the early stage of DFO retinopathy (Fig 1A ), whereas Cases II, III and IV displayed multiple areas of RPE loss as indicated by diffuse hypo‐autofluorescence (Fig 1B–D ).…”

Section: Resultsmentioning

confidence: 99%

HIF2α activation and mitochondrial deficit due to iron chelation cause retinal atrophy

Kong

Liu

et al. 2023

EMBO Mol Med

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Iron accumulation causes cell death and disrupts tissue functions, which necessitates chelation therapy to reduce iron overload. However, clinical utilization of deferoxamine (DFO), an iron chelator, has been documented to give rise to systemic adverse effects, including ocular toxicity. This study provided the pathogenic and molecular basis for DFO‐related retinopathy and identified retinal pigment epithelium (RPE) as the target tissue in DFO‐related retinopathy. Our modeling demonstrated the susceptibility of RPE to DFO compared with the neuroretina. Intriguingly, we established upregulation of hypoxia inducible factor (HIF) 2α and mitochondrial deficit as the most prominent pathogenesis underlying the RPE atrophy. Moreover, suppressing hyperactivity of HIF2α and preserving mitochondrial dysfunction by α‐ketoglutarate (AKG) protects the RPE against lesions both in vitro and in vivo. This supported our observation that AKG supplementation alleviates visual impairment in a patient undergoing DFO‐chelation therapy. Overall, our study established a significant role of iron deficiency in initiating DFO‐related RPE atrophy. Inhibiting HIF2α and rescuing mitochondrial function by AKG protect RPE cells and can potentially ameliorate patients' visual function.

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Fundus Autofluorescence and Clinical Applications

Cited by 18 publications

References 212 publications

Fundus Autofluorescence Imaging in Patients with Choroidal Melanoma

Fundus Autofluorescence Imaging in Patients with Choroidal Melanoma

A novel mutation of CYP4V2 gene associated with Bietti crystalline dystrophy complicated by choroidal neovascularization

HIF2α activation and mitochondrial deficit due to iron chelation cause retinal atrophy

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