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

Oishi, Akio; Oishi, Maho; Ogino, Ken; Morooka, Shigenori; Yoshimura, Nagahisa

doi:10.1007/978-3-319-17121-0_41

Cited by 19 publications

(10 citation statements)

References 24 publications

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“…22–24 In patients with RP, the area of abnormal hypoautofluorescence correlated inversely with the visual field areas, suggesting that the extent of retinal degeneration in the periphery seen on UWF-FAF impacts peripheral vision. 23 Likewise, the abnormal autofluorescence centered in the macula in patients with cone and cone-rod dystrophy was also correlated with the I4e visual field area in those patients. 22 …”

Section: Discussionmentioning

confidence: 86%

Peripheral Visual Fields in ABCA4 Stargardt Disease and Correlation With Disease Extent on Ultra-widefield Fundus Autofluorescence

Abalem

Otte

Andrews

et al. 2017

American Journal of Ophthalmology

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Purpose To evaluate the disease extent on ultra-widefield fundus autofluorescence (UWF-FAF) in patients with ABCA4-Stargardt disease (STGD) and correlate this data with functional outcome measures. Design Retrospective cross sectional study Setting Kellogg Eye Center, University of Michigan Study population Sixty-five patients with clinical diagnosis and proven pathogenic variants in the ABCA4 gene. Observational procedures The UWF-FAF images were obtained using Optos 200-degrees and classified into three types. Functional testing included: kinetic widefield perimetry, full-field electroretinogram (ffERG) and visual acuity (VA). All results were evaluated with respect to UWF-FAF classification. Main outcome measures Classification of UWF-FAF; area comprising the I4e, III4e, and IV4e isopters; ffERG patterns; and VA. Results For UWF-FAF, 27 subjects (41.5%) were classified as type I; 17 (26.2 %) as type II; and 21 (32.4%) as type III. The area of each isopter correlated inversely with the extent of the disease and all isopters were able to detect differences among UWF-FAF types (IV4e, p = 0.0013; III4e, p = 0.0003; I4e, p < 0.0001= 3.93e−8). ffERG patterns and VA were also different among the three UWF-FAF types (p < 0.001= 6.61e-6 and p < 0.001= 7.3e−5, respectively). Conclusion Patients with widespread disease presented with more constriction of peripheral visual fields, had more dysfunction on ffERG and worse VA compared to patients with disease confined to the macula. UWF-FAF images may provide information for estimating peripheral and central visual function in STGD.

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Section: Discussionmentioning

confidence: 86%

Peripheral Visual Fields in ABCA4 Stargardt Disease and Correlation With Disease Extent on Ultra-widefield Fundus Autofluorescence

Abalem

Otte

Andrews

et al. 2017

American Journal of Ophthalmology

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“…Fundus autofluorescence (FAF) and optical coherence tomography (OCT) imaging have greatly improved the characterisation of IRDs. Using wide-field FAF, Oishi et al 36 demonstrated an association between abnormal AF and the severity of functional impairment in COD/CORD, also correlating the extent of reduced autofluorescence with symptom duration 37. These findings are substantiated by their correlation with ERG abnormalities 38.…”

Section: Genetic and Clinical Characteristics Of Cod And Cordmentioning

confidence: 96%

Progressive cone and cone-rod dystrophies: clinical features, molecular genetics and prospects for therapy

et al. 2019

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Progressive cone and cone-rod dystrophies are a clinically and genetically heterogeneous group of inherited retinal diseases characterised by cone photoreceptor degeneration, which may be followed by subsequent rod photoreceptor loss. These disorders typically present with progressive loss of central vision, colour vision disturbance and photophobia. Considerable progress has been made in elucidating the molecular genetics and genotype–phenotype correlations associated with these dystrophies, with mutations in at least 30 genes implicated in this group of disorders. We discuss the genetics, and clinical, psychophysical, electrophysiological and retinal imaging characteristics of cone and cone-rod dystrophies, focusing particularly on four of the most common disease-associated genes: GUCA1A, PRPH2, ABCA4 and RPGR. Additionally, we briefly review the current management of these disorders and the prospects for novel therapies.

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“…The FAF represents lipofuscin from predominantly the retinal pigment epithelium (Mercado & Louprasong, 2015). Therefore, FAF is useful for RP diagnosis (Oishi et al, 2016; Robson et al, 2003). In RP eyes, annular enhancement findings are observed in the macular region, whereas granular and mottled attenuation regions are observed in the peripheral region (Oishi et al, 2013).…”

Section: Introductionmentioning

confidence: 99%

Accuracy of a deep convolutional neural network in detection of retinitis pigmentosa on ultrawide-field images

Masumoto¹,

Tabuchi²,

Nakakura³

et al. 2019

PeerJ

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Evaluating the discrimination ability of a deep convolution neural network for ultrawide-field pseudocolor imaging and ultrawide-field autofluorescence of retinitis pigmentosa. In total, the 373 ultrawide-field pseudocolor and ultrawide-field autofluorescence images (150, retinitis pigmentosa; 223, normal) obtained from the patients who visited the Department of Ophthalmology, Tsukazaki Hospital were used. Training with a convolutional neural network on these learning data objects was conducted. We examined the K-fold cross validation (K = 5). The mean area under the curve of the ultrawide-field pseudocolor group was 0.998 (95% confidence interval (CI) [0.9953–1.0]) and that of the ultrawide-field autofluorescence group was 1.0 (95% CI [0.9994–1.0]). The sensitivity and specificity of the ultrawide-field pseudocolor group were 99.3% (95% CI [96.3%–100.0%]) and 99.1% (95% CI [96.1%–99.7%]), and those of the ultrawide-field autofluorescence group were 100% (95% CI [97.6%–100%]) and 99.5% (95% CI [96.8%–99.9%]), respectively. Heatmaps were in accordance with the clinician’s observations. Using the proposed deep neural network model, retinitis pigmentosa can be distinguished from healthy eyes with high sensitivity and specificity on ultrawide-field pseudocolor and ultrawide-field autofluorescence images.

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

Cited by 19 publications

References 24 publications

Peripheral Visual Fields in ABCA4 Stargardt Disease and Correlation With Disease Extent on Ultra-widefield Fundus Autofluorescence

Peripheral Visual Fields in ABCA4 Stargardt Disease and Correlation With Disease Extent on Ultra-widefield Fundus Autofluorescence

Progressive cone and cone-rod dystrophies: clinical features, molecular genetics and prospects for therapy

Accuracy of a deep convolutional neural network in detection of retinitis pigmentosa on ultrawide-field images

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