The Role of Fundus Autofluorescence Imaging in the Study of the Course of Posterior Uveitis Disorders

Background.To evaluate the correlation of fundus autofluorescence (FAF) with indocyanine green angiography (ICGA) in patients with various posterior uveitis disorders.Methods. Interventional case series including 23 eyes of 15 patients with diagnosis of a specific type of retinochoroiditis, such as acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous-like choroiditis, multifocal choroiditis (MFC), Harada disease, and syphilitic retinochoroiditis. Also, some cases with undefined retinochoroiditis were included. FAF and ICGA were performed and correlated at baseline and during follow-up after treatment.Results. In ICGA, early hypofluorescence was found to be the hallmark of acute choroidal inflammation, resolving in later stages and remaining in the late phase in areas with retinal pigment epithelium (RPE) damage. Poorly defined hyperautofluorescent areas correlated with acute choroidal lesions. Hypoautofluorescent delineation suggested the initiation of RPE healing processes, correlating well with the late phase of ICGA and delineating the RPE damage. Early hyperautofluorescence with late hypofluorescence in ICGA indicated the presence of primary RPE involvement.Conclusion. FAF contributes to the interpretation of RPE disease and may be a useful tool for the follow-up of progressive inflammatory disorders. Comparative evaluation of FAF and ICGA allows a characterization of the sequence of inflammatory events and the level of tissue affected.

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Role of Autofluorescence in Inflammatory/Infective Diseases of the Retina and Choroid

Journal of Ophthalmology ◽

10.1155/2014/418193 ◽

2014 ◽

Vol 2014 ◽

pp. 1-9 ◽

Cited By ~ 7

Author(s):

Ahmed Samy ◽

Sue Lightman ◽

Filis Ismetova ◽

Lazha Talat ◽

Oren Tomkins-Netzer

Keyword(s):

Case Reports ◽

Pigment Epithelium ◽

Fundus Autofluorescence ◽

Retinal Pigment ◽

Visual Outcome ◽

Posterior Uveitis ◽

Fluorescent Properties ◽

Promising Tool ◽

Noninvasive Imaging Technique

Fundus autofluorescence (FAF) has recently emerged as a novel noninvasive imaging technique that uses the fluorescent properties of innate fluorophores accumulated in the retinal pigment epithelium (RPE) to assess the health and viability of the RPE/photoreceptor complex. Recent case reports suggest FAF as a promising tool for monitoring eyes with posterior uveitis helping to predict final visual outcome. In this paper we review the published literature on FAF in these disorders, specifically patterns in infectious and noninfectious uveitis, and illustrate some of these with short case histories.

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Near-infrared fundus autofluorescence imaging in a case of photic maculopathy

International Journal of Diagnostic Imaging ◽

10.5430/ijdi.v1n1p28 ◽

2014 ◽

Vol 1 (1) ◽

pp. 28

Author(s):

Alberto Neri ◽

Alessandra Protti ◽

Monica Camparini ◽

Claudio Macaluso

Keyword(s):

Near Infrared ◽

Imaging Modality ◽

Pigment Epithelium ◽

Fundus Autofluorescence ◽

Retinal Pigment ◽

Parallel Evolution ◽

Conventional Imaging ◽

Autofluorescence Imaging ◽

Rpe Cells

Herein we describe the characteristics of scanning laser ophthalmoscope imaging (SLO), and particularly of near-infrared fundus autofluorescence modality (IRAF), in photic maculopathy. IRAF visualizes selectively the melanin granules contained in the cells of the Retinal Pigment Epithelium (RPE), which are normally localized in the apical portion of RPE cells, a favorite target of the photic damage to the retina. In the present work we report a three-month follow-up of a case of photic maculopathy, showing that the retinal alterations found by IRAF precisely matched the retinal anomalies found by macular optical coherence tomography, and had a parallel evolution. We think that IRAF could represent a useful imaging modality, together with more conventional imaging, in the management of photic maculopathy.

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Fundus autofluorescence of retinal angiomatous proliferation

PLoS ONE ◽

10.1371/journal.pone.0243458 ◽

2020 ◽

Vol 15 (12) ◽

pp. e0243458

Author(s):

Masaaki Saito ◽

Kanako Itagaki ◽

Tetsuju Sekiryu

Keyword(s):

Near Infrared ◽

Hot Spot ◽

Pigment Epithelium ◽

Fundus Autofluorescence ◽

Retinal Pigment ◽

Late Phase ◽

Retinal Angiomatous Proliferation ◽

Subretinal Space ◽

Stage 1

Purpose The present study aimed to evaluate the characteristics of fundus autofluorescence in Japanese patients with retinal angiomatous proliferation (RAP). Methods We retrospectively reviewed 100 eyes from 76 patients (male, n = 45; female, n = 31; age range, 50–94 years; mean ± standard deviation, 81.4 ± 6.4 years) with treatment-naïve RAP, which was diagnosed based on the identification of retinal–retinal anastomosis on early-phase fluorescein angiography or indocyanine green angiography (ICGA) and the identification of a hot spot on late-phase ICGA. RAP was classified into the following three stages: stage 1, proliferation of intraretinal capillaries originating from the deep retinal complex (intraretinal neovascularization); stage 2, growth of the retinal vessels into the subretinal space (subretinal neovascularization); and stage 3, clinically or angiographically observed choroidal neovascularization. In all cases, short-wavelength and near-infrared autofluorescence (SW-AF, NIR-AF) was evaluated using a confocal scanning laser ophthalmoscope. Results The conditions of the 100 eyes were as follows: stage 1 RAP, n = 6 (6%); stage 2 RAP without retinal pigment epithelial detachment (PED), n = 40 (40%); stage 2 RAP with PED, n = 44 (44%); and stage 3 RAP, 10 (10%). On NIR-AF imaging, the number of abnormalities that were observed to correspond to the RAP lesions on ICGA (87 eyes, 87%) was significantly greater in comparison to SW-AF imaging (27 eyes, 27%). The mean follow-up period in all 76 patients was 39.2 months. In the 76 patients with unilateral disease, 21 (21%) eyes developed RAP in the fellow eye during the follow-up period. Among 18 eyes that were examined by both SW-AF and NIR-AF imaging before the onset of RAP lesions, NIR-AF imaging showed hypoautofluorescence in 15 (83%) eyes before the onset of RAP lesions. Conclusions SW-AF and NIR-AF abnormalities may be related to the dysfunction of the photoreceptor/retinal pigment epithelium complex. Hypoautofluorescence on NIR-AF imaging may accurately indicate the presence or onset of RAP lesions.

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Multimodal imaging of chorioretinal folds induced by orbital vascular malformation in two cases

European Journal of Ophthalmology ◽

10.1177/1120672120957582 ◽

2020 ◽

pp. 112067212095758

Author(s):

Ahmet Kaan Gündüz ◽

Carol L Shields ◽

Şükran Bekdemir ◽

Jerry A Shields

Keyword(s):

Functional Status ◽

Multimodal Imaging ◽

Pigment Epithelium ◽

Fundus Autofluorescence ◽

Retinal Pigment ◽

Late Phase ◽

Vascular Tumor ◽

Fundus Photography ◽

Swept Source ◽

Chorioretinal Folds

Purpose: To investigate the alterations in the retinal pigment epithelium (RPE) in the crest and trough portions of chorioretinal folds (CRFs) induced by an orbital vascular tumor. Methods: Review of multimodal imaging in two eyes of two patients with globe compression and CRFs from an orbital vascular tumor. Results: Fundus photography demonstrated obliquely extending CRFs with alternating hyperpigmented and hypopigmented linear alterations in both eyes. Fundus autofluorescence (AF) imaging showed obliquely oriented hypoAF lines, incompletely alternating with hyperAF lines. In Case 1, the hyperAF lines had interspersed hypoAF segments and Case 2 had peripapillary mottling of AF. Fluorescein angiography (FA) showed alternating hyper and hypofluorescent lines in the late phase in Case 1. Optical coherence tomography (OCT) documented relative thinning of RPE at the folded crests in Case 1 and preservation of RPE in Case 2. Swept-source OCT angiography (SS-OCTA) demonstrated oblique hyporeflective lines in the outer retina and choriocapillaris layers in Case 2. These findings suggest that the crest of a CRF represents thinned or rarified RPE with hypoAF, transmission hyperfluorescence (FA), partially attenuated RPE layer (OCT), and isoreflectivity (SS-OCTA) while the trough represents compressed RPE with irregular hyperAF, transmission hypofluorescence (FA), thickened RPE layer (OCT), and hyporeflectivity (SS-OCTA). Conclusion: The anatomic and functional status of the RPE in CRFs based on multimodal imaging reveals normal to attenuated RPE with hypofunctionality at the fold crest and compacted, thickened RPE at the trough with segmental functional impairment on AF imaging. Anatomic information regarding CRFs is evident on OCT, FA, and SS-OCTA while the functional status is depicted on AF.

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Long-Term Effect of Half-Fluence Photodynamic Therapy on Fundus Autofluorescence in Acute Central Serous Chorioretinopathy

Journal of Ophthalmology ◽

10.1155/2020/8491712 ◽

2020 ◽

Vol 2020 ◽

pp. 1-7

Author(s):

Martin Stattin ◽

Stefan Hagen ◽

Daniel Ahmed ◽

Eva Smretschnig ◽

Florian Frommlet ◽

...

Keyword(s):

Photodynamic Therapy ◽

Central Serous Chorioretinopathy ◽

Pigment Epithelium ◽

Fundus Autofluorescence ◽

Retinal Pigment ◽

Long Term Follow Up ◽

Acute Central Serous Chorioretinopathy ◽

Mean Differences

Purpose. To evaluate normalized short-wavelength fundus autofluorescence (SW-FAF) imaging changes over time as a predictive parameter for the retinal pigment epithelium (RPE) function in eyes compromised by acute central serous chorioretinopathy (CSCR) after indocyanine green angiography-guided verteporfin (Visudyne®, Novartis Pharma, Basel, Switzerland) photodynamic therapy (PDT) with a half-fluence rate (25 J/cm2). Methods. Quantitative data of SW-FAF grey values (SW-FAF GV) from a 350 μm (SW-350) and 1200 μm (SW-1200) diameter circle centered on the fovea and normalized with the level of SW-FAF GV in a 30° image of 20 eyes in 11 patients initially treated for unilateral acute symptomatic CSCR were collected and retrospectively analyzed after 7 years. A 2-sided t-test was calculated to explore the differences of SW-350 and SW-1200 between one month and the long-term follow-up. Results. Mean differences (95% CI) in SW-FAF GV between 1 month and 7 years after half-fluence PDT were 0.07 ± 0.11 for SW-350 ([95% CI: −0.002; 0.14], p=0.06) and 0.11 ± 0.15 for SW-1200 ([95% CI: 0.01; 0.21], p=0.03). Mean differences in SW-FAF GV of the contralateral untreated eye were 0.06 ± 0.14 for SW-350 ([95% CI: −0.04; 0.17], p=0.22) and 0.05 ± 0.13 for SW-1200 ([95% CI: −0.04; 0.15], p=0.22). Conclusion. After 7 years, normalized SW-FAF GV were significantly lower in eyes with resolved acute CSCR treated with reduced-fluence PDT compared to the follow-up after 1 month without correlation to explicit pattern changes or structural damages. Half-fluence PDT remains a safe and considerable treatment option in acute CSCR.

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Expanding the retinal phenotype of RP1: from retinitis pigmentosa to a novel and singular macular dystrophy

British Journal of Ophthalmology ◽

10.1136/bjophthalmol-2018-313672 ◽

2019 ◽

Vol 104 (2) ◽

pp. 173-181 ◽

Cited By ~ 2

Author(s):

Marina Riera ◽

Víctor Abad-Morales ◽

Rafael Navarro ◽

Sheila Ruiz-Nogales ◽

Pilar Méndez-Vendrell ◽

...

Keyword(s):

Visual Acuity ◽

Retinitis Pigmentosa ◽

Pigment Epithelium ◽

Fundus Autofluorescence ◽

Retinal Pigment ◽

Retinal Dystrophy ◽

Macular Dystrophy ◽

Nucleotide Polymorphisms ◽

Autofluorescence Imaging ◽

New Genotype

PurposeThis study aimed to identify the underlying genetic cause(s) of inherited retinal dystrophy (IRD) in 12 families of Kuwaiti origin affected by macular dystrophy and four Spanish patients affected by retinitis pigmentosa (RP).MethodsClinical diagnoses were based on standard ophthalmic evaluations (best-corrected visual acuity, retinography, fundus autofluorescence imaging, optical coherence tomography, electroretinography and visual field tests). Panel-based whole exome sequencing was used to simultaneously analyse 224 IRD genes in one affected member of each family. The putative causative variants were confirmed by Sanger sequencing and cosegregation analyses. Haplotype analysis was performed using single nucleotide polymorphisms.ResultsA homozygous missense mutation c.606C>A (p.Asp202Glu) in RP1 was found to be the molecular cause of IRD in all 12 families from Kuwait. These patients exhibited comparable symptoms, including progressive decline in visual acuity since adolescence. Fundus autofluorescence images revealed bilateral macular retinal pigment epithelium disturbances, with neither perimacular flecks nor peripheral alterations. A shared haplotype spanning at least 1.1 Mb was identified in all families, suggesting a founder effect. Furthermore, RP1 variants involving nonsense and/or frameshifting mutations (three of them novel) were identified in three Spanish autosomal-recessive RP families and one dominant RP pedigree.ConclusionThis study describes, for the first time, a macular dystrophy phenotype caused by an RP1 mutation; establishing a new genotype-phenotype correlation in this gene, expanding its mutation spectrum and further highlighting the clinical heterogeneity associated with IRD.

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Congenital Simple Hamartoma of Retinal Pigment Epithelium: Clinical and Imaging Findings

Case Reports in Ophthalmological Medicine ◽

10.1155/2012/654502 ◽

2012 ◽

Vol 2012 ◽

pp. 1-3 ◽

Cited By ~ 3

Author(s):

Mehmet Yasin Teke ◽

Pinar Ç. Özdal ◽

Figen Batioglu ◽

Ufuk Elgin ◽

Faruk Öztürk

Keyword(s):

Retinal Pigment Epithelium ◽

Pigment Epithelium ◽

Benign Lesion ◽

Fundus Autofluorescence ◽

Retinal Pigment ◽

Autofluorescence Imaging ◽

Imaging Findings ◽

Characteristic Finding ◽

Tumor Margins ◽

Surface Optical

Congenital simple hamartoma of retinal pigment epithelium (CSHRPE) is a rare, asymptomatic, and incidentally detected benign lesion. However, it is very important to do the differential diagnosis from other pigmented retinal lesions. Its clinical presentation and imaging findings are very helpful in doing this differentiation. This paper presents clinical and imaging findings of a 56-year-old woman with incidentally detected CSHRPE. The lesion was small, heavily pigmented, well circumscribed, and slightly elevated. Optical coherence tomography (OCT) scanning was diagnostic and showed an elevated retina at the site of the lesion, increased optical reflectivity on its inner surface, optical shadowing of deeper structures, and clearly cut tumor margins. Ocular ultrasonography, fluorescein angiography, and fundus autofluorescence imaging which is firstly described in this report did not show any characteristic finding.

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CRB1-associated retinal dystrophies in a Belgian cohort: genetic characteristics and long-term clinical follow-up

British Journal of Ophthalmology ◽

10.1136/bjophthalmol-2020-316781 ◽

2021 ◽

pp. bjophthalmol-2020-316781

Author(s):

Mays Talib ◽

Caroline Van Cauwenbergh ◽

Julie De Zaeytijd ◽

David Van Wynsberghe ◽

Elfride De Baere ◽

...

Keyword(s):

Visual Function ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Posterior Uveitis ◽

Cone Dystrophy ◽

Macular Dystrophy ◽

Genetic Characteristics ◽

Retinal Dystrophies ◽

Retinal Structure

AimTo investigate the natural history in a Belgian cohort of CRB1-associated retinal dystrophies.MethodsAn in-depth retrospective study focusing on visual function and retinal structure.ResultsForty patients from 35 families were included (ages: 2.5–80.1 years). In patients with a follow-up of >1 year (63%), the mean follow-up time was 12.0 years (range: 2.3–29.2 years). Based on the patient history, symptoms and/or electroretinography, 22 patients (55%) were diagnosed with retinitis pigmentosa (RP), 15 (38%) with Leber congenital amaurosis (LCA) and 3 (8%) with macular dystrophy (MD), the latter being associated with the p.(Ile167_Gly169del) mutation (in compound heterozygosity). MD later developed into a rod-cone dystrophy in one patient. Blindness at initial presentation was seen in the first decade of life in LCA, and in the fifth decade of life in RP. Eventually, 28 patients (70%) reached visual acuity-based blindness (<0.05). Visual field-based blindness (<10°) was documented in 17/25 patients (68%). Five patients (13%) developed Coats-like exudative vasculopathy. Intermediate/posterior uveitis was found in three patients (8%). Cystoid maculopathy was common in RP (9/21; 43%) and MD (3/3; 100%). Macular involvement, varying from retinal pigment epithelium alterations to complete outer retinal atrophy, was observed in all patients.ConclusionBi-allelic CRB1 mutations result in a range of progressive retinal disorders, most of which are generalised, with characteristically early macular involvement. Visual function and retinal structure analysis indicates a window for potential intervention with gene therapy before the fourth decade of life in RP and the first decade in LCA.

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Syphilitic Outer Retinopathy: A Case Report and Review of the Literature

Journal of VitreoRetinal Diseases ◽

10.1177/24741264211018300 ◽

2021 ◽

pp. 247412642110183

Author(s):

Claudia Amaral ◽

Leilani Joy ◽

Hiram Jimenez ◽

Yousef J. Cruz-Inñigo ◽

Jan P. Ulloa-Padilla ◽

...

Keyword(s):

Visual Acuity ◽

Case Report ◽

Vision Loss ◽

Pigment Epithelium ◽

Retinal Pigment ◽

Oral Prednisone ◽

Posterior Uveitis ◽

Central Scotoma ◽

Demarcation Line

Purpose: This work presents a case of syphilitic outer retinopathy with findings similar to those of acute zonal occult outer retinopathy (AZOOR). We also discuss the clinical characteristics, treatment, and prognosis of this entity. Methods: A case report and systematic literature review are presented. Results: A 56-year-old woman presented with acute vision loss, localized photopsia, a central scotoma, and retinal findings that were all consistent with AZOOR. A further workup led to a diagnosis of syphilis. Oral prednisone and intravenous penicillin resulted in the resolution of the posterior uveitis and the restoration of visual acuity. However, the central scotoma remained at the 3-year follow-up visit. Conclusions: Syphilitic outer retinopathy is a distinct entity characterized by the disruption of the ellipsoid zone visible on optical coherence tomography and a corresponding increase in fundus hyperautofluorescence in the affected areas. Although some patients may present with a demarcation line, as is seen with AZOOR, the fundus is oftentimes unremarkable or may show only subtle retinal pigment epithelium changes. Uveitis resolution and visual acuity restoration may be expected following treatment; however, visual field disturbances may persist.

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Hyperautofluorescence in Outer Retinal Layers Thinning

BioMed Research International ◽

10.1155/2014/741538 ◽

2014 ◽

Vol 2014 ◽

pp. 1-6 ◽

Cited By ~ 2

Author(s):

Marina Bertolotto ◽

Luigi Borgia ◽

Michele Iester

Keyword(s):

Retinal Thickness ◽

Pigment Epithelium ◽

Fundus Autofluorescence ◽

Retinal Pigment ◽

Age Related Macular Degeneration ◽

Retinal Layer ◽

Autofluorescence Imaging ◽

Cross Sectional ◽

Age Related ◽

Window Effect

Purpose. To evaluate if paracentral hyperautofluorescence (HAF) retinal regions, which can be occasionally found and analyzed by optical coherence tomography (OCT), were related to retinal layer changes and to detect which layer was involved.Methods. This is a cross-sectional and retrospective study. 648 OCT files were revised. OCTs that showed a paracentral HAF area by using the fundus autofluorescence imaging in Heidelberg Spectralis (Heidelberg Engineering, Germany) were selected. Then retinal layer morphology was analyzed observing OCT scans and a retinal thickness was measured.Results. 31 patients were selected: 20 patients had chronic serous epitheliopathy (CSE), 8 patients had resolved central serous chorioretinopathy (CSC), and 3 patients wet age related macular degeneration (ARMD). The HAF zones corresponded to areas of thickness reduction of the external hyporeflective band. In all these areas the retinal pigment epithelium was not atrophic and the neuroepithelium was more or less dystrophic. In particular the retinal thickness was 264 um, 232 um, and 243 um in wet ARMD, CSE, and CSC, respectively; the reduction was significant (P<0.01) compared to the same area of the other eye.Discussion. The presence of HAF imaging might be mostly due to a “window effect” rather than an accumulation of lipofuscin.

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