Comparison of Fundus Autofluorescence with Photopic and Scotopic Fine Matrix Mapping in Patients with Retinitis Pigmentosa: 4- to 8-Year Follow-up

2012 ◽  
Vol 53 (10) ◽  
pp. 6187 ◽  
Author(s):  
Anthony G. Robson ◽  
Eva Lenassi ◽  
Zubin Saihan ◽  
Vy A. Luong ◽  
Fred W. Fitzke ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Fundus Autofluorescence ◽  
Matrix Mapping

Comparison of Fundus Autofluorescence with Photopic and Scotopic Fine-Matrix Mapping in Patients with Retinitis Pigmentosa and Normal Visual Acuity

2004 ◽  
Vol 45 (11) ◽  
pp. 4119 ◽  
Author(s):  
Anthony G. Robson ◽  
Catherine A. Egan ◽  
Vy A. Luong ◽  
Alan C. Bird ◽  
Graham E. Holder ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Retinitis Pigmentosa ◽  
Fundus Autofluorescence ◽  
Normal Visual Acuity ◽  
Matrix Mapping

Bilateral noncalcified astrocytic hamartomas in retinitis pigmentosa: Multimodal imaging evaluation over 8 years of follow-up

2018 ◽  
Vol 29 (5) ◽  
pp. NP18-NP21
Author(s):  
Claudio Iovino ◽  
Giamberto Casini ◽  
Enrico Peiretti
Keyword(s):  
Retinitis Pigmentosa ◽  
Multimodal Imaging ◽  
Fundus Autofluorescence ◽  
Optic Disk ◽  
Imaging Evaluation ◽  
Retinal Degenerations ◽  
Indocyanine Angiography ◽  
The Right ◽  
First Time

Purpose: To report the evolution of optic disk noncalcified astrocytic hamartomas in a patient with retinitis pigmentosa throughout 8 years of follow-up. Methods: Case report. Results: A 42-year-old white man with a diagnosis of retinitis pigmentosa was referred to our medical retinal center for the first time in 2010, for the development of a new optic nerve head lesion in the right eye. Fundus examination, fundus autofluorescence, fluorescein and green indocyanine angiography, spectral-domain optical coherence tomography were performed and confirmed the presence of a papillary noncalcified astrocytic hamartoma in the right eye. Four years later, multimodal imaging evaluation revealed in the left eye the presence of a first optic disk benign tumor, and in 2018, a second in the nasal retina, while in the right eye the sole papillary lesion increased slightly in size. Conclusions: Noncalcified astrocytic hamartomas can occur in patients with retinitis pigmentosa and can increase in size and number in time. A complete multimodal imaging evaluation is necessary to identify and classify any kind of new lesion that, as known, are associated with these group of hereditary retinal degenerations.

scholarly journals Clinical Phenotype of PDE6B-Associated Retinitis Pigmentosa

2021 ◽  
Vol 22 (5) ◽  
pp. 2374
Author(s):  
Laura Kuehlewein ◽  
Ditta Zobor ◽  
Katarina Stingl ◽  
Melanie Kempf ◽  
Fadi Nasser ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Genetic Testing ◽  
Retinitis Pigmentosa ◽  
Fundus Autofluorescence ◽  
Retinal Disease ◽  
New Drugs ◽  
Autofluorescence Imaging ◽  
Full Field ◽  
Tertiary Referral Center ◽  
The Right

In this retrospective, longitudinal, observational cohort study, we investigated the phenotypic and genotypic features of retinitis pigmentosa associated with variants in the PDE6B gene. Patients underwent clinical examination and genetic testing at a single tertiary referral center, including best-corrected visual acuity (BCVA), kinetic visual field (VF), full-field electroretinography, full-field stimulus threshold, spectral domain optical coherence tomography, and fundus autofluorescence imaging. The genetic testing comprised candidate gene sequencing, inherited retinal disease gene panel sequencing, whole-genome sequencing, and testing for familial variants by Sanger sequencing. Twenty-four patients with mutations in PDE6B from 21 families were included in the study (mean age at the first visit: 32.1 ± 13.5 years). The majority of variants were putative splicing defects (8/23) and missense (7/23) mutations. Seventy-nine percent (38/48) of eyes had no visual acuity impairment at the first visit. Visual acuity impairment was mild in 4% (2/48), moderate in 13% (6/48), and severe in 4% (2/48). BCVA was symmetrical in the right and left eyes. The kinetic VF measurements were highly symmetrical in the right and left eyes, as was the horizontal ellipsoid zone (EZ) width. Regarding the genetic findings, 43% of the PDE6B variants found in our patients were novel. Thus, this study contributed substantially to the PDE6B mutation spectrum. The visual acuity impairment was mild in 83% of eyes, providing a window of opportunity for investigational new drugs. The EZ width was reduced in all patients and was highly symmetric between the eyes, making it a promising outcome measure. We expect these findings to have implications on the design of future PDE6B-related retinitis pigmentosa (RP) clinical trials.

Characterisation of macular neovascularisation in geographic atrophy

2021 ◽  
pp. bjophthalmol-2021-318820
Author(s):  
Riccardo Sacconi ◽  
Maria Brambati ◽  
Alexandra Miere ◽  
Eliana Costanzo ◽  
Vittorio Capuano ◽  
...  
Keyword(s):  
Fundus Autofluorescence ◽  
Geographic Atrophy ◽  
Age Related Macular Degeneration ◽  
First Year ◽  
Age Related ◽  
Treatment Naïve ◽  
Type 3 ◽  
Endothelial Growth Factor

AimTo characterise macular neovascularisation (MNV) developing in eyes affected by geographic atrophy (GA).MethodsIn this multicentric longitudinal study involving three retina referral centres, patients previously affected by GA who developed an active MNV were included. Patients were investigated using structural optical coherence tomography (OCT), fundus autofluorescence, OCT-angiography and dye angiographies. Patients were treated with ProReNata antivascular endothelial growth factor (VEGF) injections and were revaluated after treatment.ResultsAmong 512 patients previously diagnosed with GA, 40 eyes of 40 patients (mean age 80.8±7.9 years, mean GA area 8.73±7.39 mm2) presented with treatment-naïve exudative MNV (accounting for an estimated prevalence of 7.81%; 5.49 to 10.13, 95% CIs) and thus were included in the analysis. 67.5% of MNVs were classified as type 2 MNV, 25% as type 1, 2.5% as type 3 and 5% as mixed phenotype. In 92.5% of cases, active MNV in GA showed subretinal hyperreflective material with or without evidence of subretinal/intraretinal hyporeflective exudation. During a mean follow-up of 28±25 months, patients were treated with 6.6±6.3 anti-VEGF injections, with 2.9±1.4 injections in the first year of treatment. No patient developed GA enlargement in the area of MNV.ConclusionsMNVs in GA showed different features and therapeutic response in comparison to previously reported features of MNV in age-related macular degeneration (AMD) without GA. For these reasons, the combined phenotype (ie, GA with neovascular AMD) should be considered as a distinct entity in the research and clinical setting.

scholarly journals Beyond Sector Retinitis Pigmentosa: Expanding the Phenotype and Natural History of the Rhodopsin Gene Codon 106 Mutation (Gly-to-Arg) in Autosomal Dominant Retinitis Pigmentosa

Genes ◽  
2021 ◽  
Vol 12 (12) ◽  
pp. 1853
Author(s):  
Brian G. Ballios ◽  
Emily M. Place ◽  
Luis Martinez-Velazquez ◽  
Eric A. Pierce ◽  
Jason I. Comander ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Autosomal Dominant ◽  
Peripheral Vision ◽  
Visual Fields ◽  
Macular Disease ◽  
History Of ◽  
Intrafamilial Variability ◽  
Long Term Prognosis

Sector and pericentral are two rare, regional forms of retinitis pigmentosa (RP). While usually defined as stable or only very slowly progressing, the available literature to support this claim is limited. Additionally, few studies have analyzed the spectrum of disease within a particular genotype. We identified all cases (9 patients) with an autosomal dominant Rhodopsin variant previously associated with sector RP (RHO c.316G > A, p.Gly106Arg) at our institution. Clinical histories were reviewed, and testing included visual fields, multimodal imaging, and electroretinography. Patients demonstrated a broad phenotypic spectrum that spanned regional phenotypes from sector-like to pericentral RP, as well as generalized disease. We also present evidence of significant intrafamilial variability in regional phenotypes. Finally, we present the longest-reported follow-up for a patient with RHO-associated sector-like RP, showing progression from sectoral to pericentral disease over three decades. In the absence of comorbid macular disease, the long-term prognosis for central visual acuity is good. However, we found that significant progression of RHO p.Gly106Arg disease can occur over protracted periods, with impact on peripheral vision. Longitudinal widefield imaging and periodic ERG reassessment are likely to aid in monitoring disease progression.

scholarly journals Multimodal Imaging of Central Retinal Disease Progression in a 2-Year Mean Follow-up of Retinitis Pigmentosa

2015 ◽  
Vol 160 (4) ◽  
pp. 786-798.e4 ◽  
Author(s):  
Tharikarn Sujirakul ◽  
Michael K. Lin ◽  
Jimmy Duong ◽  
Ying Wei ◽  
Sara Lopez-Pintado ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Disease Progression ◽  
Multimodal Imaging ◽  
Retinal Disease

Wide-Field Fundus Autofluorescence Imaging of Retinitis Pigmentosa

Ophthalmology ◽  
2013 ◽  
Vol 120 (9) ◽  
pp. 1827-1834 ◽  
Author(s):  
Akio Oishi ◽  
Ken Ogino ◽  
Yukiko Makiyama ◽  
Satoko Nakagawa ◽  
Masafumi Kurimoto ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Fundus Autofluorescence ◽  
Wide Field ◽  
Autofluorescence Imaging

Choriocapillaris flow impairment could predict the enlargement of geographic atrophy lesion

2020 ◽  
Vol 105 (1) ◽  
pp. 97-102 ◽  
Author(s):  
Riccardo Sacconi ◽  
Eleonora Corbelli ◽  
Enrico Borrelli ◽  
Luigi Capone ◽  
Adriano Carnevali ◽  
...  
Keyword(s):  
Fundus Autofluorescence ◽  
Control Area ◽  
Geographic Atrophy ◽  
Age Related Macular Degeneration ◽  
Age Related ◽  
Slow Progression ◽  
Minimum Angle ◽  
Perfusion Density ◽  
Prospective Longitudinal

AimTo analyse the choriocapillaris (CC) flow status in the area that subsequently showed geographic atrophy (GA) expansion secondary to age-related macular degeneration (AMD) during 1-year follow-up, matching optical coherence tomography angiography (OCT-A) and fundus autofluorescence (FAF).MethodsIn this prospective longitudinal observational study, 30 eyes of 20 consecutive patients with GA secondary to AMD (mean age 75.5±7.4 years) were included. All patients underwent OCT-A and FAF at baseline and 1-year follow-up. Main outcome measures included analysis of perfusion density (PD) in the ‘area surrounding GA margin’ (between the GA border and 500 µm distance) in comparison with the ‘control area’ (area outside the 500 µm line), and of the ‘expansion area’ (area that subsequently developed GA expansion during 1-year follow-up).ResultsDuring the 1-year follow-up, visual acuity significantly decreased from 0.34±0.38 Logarithm of the Minimum Angle of Resolution (LogMAR) to 0.39±0.40 LogMAR (p<0.001), and mean GA area increased from 6.82±5.47 mm2 to 8.76±6.28 mm2 (p<0.001). CC PD of the area surrounding the GA margin revealed a significant flow impairment compared with control area (PD 0.679±0.076 and 0.734±0.057, respectively (p<0.001)). Furthermore, the PD of the expansion area showed a greater CC flow impairment in comparison to the remaining area surrounding GA margin (p<0.001).ConclusionsWe reported a greater CC impairment in the area that subsequently developed GA expansion, suggesting that the CC flow impairment could predict the enlargement of GA lesion. The CC impairment could be considered as a new a risk factor for GA progression and a biomarker to be measured to determine efficacy of new interventions aiming to slow progression of GA.

Sign in / Sign up

Export Citation Format

Share Document