Hypofluorescent Lesions Research Articles

Housing RCS rats under specific pathogen-free conditions mildly ameliorates retinal degeneration and alters intestine microbiota

Retinitis pigmentosa (RP) is a genetic blinding disease with over 80 causative genes. Disease progression varies between patients with similar genetic backgrounds. We assessed the association between environment, gut microbiota, and retinal degeneration in the RP rat model Royal College of Surgeons (RCS). The rats were born and raised for two generations under specific pathogen-free (SPF, n = 69) or non-SPF conditions (n = 48). At the age of four weeks, SPF rats had significantly shorter dark-adapted a-wave and dark and light-adapted b-wave implicit times by electroretinogram (p = 0.014, p = 9.5*10−6, p = 0.009, respectively). The SPF rats had significantly less photoreceptor apoptosis at ages four, eight, and twelve weeks (all p < 0.022), significantly thicker debris zone at age 14 weeks, and smaller hypofluorescent lesions in SPF rats at ages 10–16 weeks, especially in the inferior retina. The non-SPF rats had significantly higher microbiota alpha diversity (p = 0.037) and failed to present the age-related maturation of Proteobacteria, Spirochaetes, Actinobacteria, and Bacteroidetes seen in SPF conditions. Specific microbial amplicon sequence variants were reduced in rats with more severe retinal degeneration. Our data suggest an environmental effect on retinal deterioration in RCS rats. These findings may lead to the development of novel microbiome-related interventions for retinal degeneration.

High prevalence of exon-13 variants in USH2A-related retinal dystrophies in Taiwanese population

BackgroundBiallelic pathogenic variants in USH2A lead to Usher syndrome or non-syndromic retinitis pigmentosa, and shown to have geographical and ethnical distribution in previous studies. This study provided a deeper understanding of the detailed clinical features using multimodal imaging, genetic spectrum, and genotype–phenotype correlations of USH2A-related retinal dystrophies in Taiwan.ResultsIn our cohort, the mean age at first visit was 47.66 ± 13.54 years, and the mean age at symptom onset, which was referred to the onset of nyctalopia and/or visual field constriction, was 31.21 ± 15.24 years. Among the variants identified, 23 (50%) were missense, 10 (22%) were splicing variants, 8 (17%) were nonsense, and 5 (11%) were frameshift mutations. The most predominant variant was c.2802T>G, which accounted for 21% of patients, and was located in exon 13. Patients with truncated alleles had significantly earlier symptom onset and seemly poorer disease progression regarding visual acuity, ellipsoid zone line length, and hypofluorescent lesions in the macula than those who had the complete gene. However, the clinical presentation revealed similar progression between patients with and without the c.2802T>G variant. During long-term follow-up, the patients had different ellipsoid zone line progression rates and were almost evenly distributed in the fast, moderate, and slow progression subgroups. Although a younger onset age and a smaller baseline intact macular area was observed in the fast progression subgroup, the results showed no significant difference.ConclusionsThis is the first cohort study to provide detailed genetic and longitudinal clinical analyses of patients with USH2A-related retinal dystrophies in Taiwan. The mutated allele frequency in exon 13 was high in Taiwan due to the predominant c.2802T>G variant. Moreover, truncated variants greatly impacted disease progression and determined the length of therapeutic windows. These findings provide insight into the characteristics of candidates for future gene therapies.

Acute posterior multifocal placoid pigment epitheliopathy following inactivated SARS-CoV-2 vaccination: A case report

A 25-year-old woman presented with redness and pain in both eyes after the first dose of the inactivated vaccine Corona Vac (Sinovac Biotech Ltd., Beijing, China). She had non granulomatous anterior segment inflammation and multiple creamy placoid lesions within the posterior pole and midperipheral retina bilaterally. Fluorescein angiography (FA) revealed early hypofluorescent and late hyperfluorescent lesions, while indocyanine green angiography (ICGA) demonstrated hypofluorescent lesions throughout all phases. A diagnosis of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) was made, and the patient was started on a course of oral prednisolone that was tapered and the inflammation was completely resolved.

Value and Significance of Hypofluorescent Lesions Seen on Late-Phase Indocyanine Green Angiography

The hypofluorescence of fundus lesions observed during the late phase of indocyanine green angiography (ICGA) in various diseases has often been overlooked or misinterpreted. This article explores the significance of fundus lesions that are initially isofluorescent during the early phase of ICGA but become hypofluorescent later in the examination. Pathologies such as multiple evanescent white spot syndrome, acute posterior placoid syphilitic chorioretinitis, chronic central serous chorioretinopathy, choroidal hemangioma, and some fundus with drusen, present this phenomenon of late hypofluorescence. The interpretation of ICGA images and the role of indocyanine green (ICG) uptake by the retinal pigment epithelium (RPE) in late fundus fluorescence is debated. Experimental evidence suggests that ICG accumulates progressively in the RPE after intravenous injection of the dye or after direct contact invitro, making it a potential marker of RPE activity. Although the exact mechanisms of ICG diffusion through the choroid and its binding to the RPE require further investigation, the late hypofluorescence observed in certain ICGA diseases provides information on different modalities of RPE dysfunction. The author has no proprietary or commercial interest in any materials discussed in this article.

Unilateral Multiple Evanescent White Dot Syndrome-Like Reaction Following the CyberKnife Stereotactic Radiotherapy for Choroidal Malignant Melanoma.

A 58-year-old otherwise healthy man received a diagnosis of choroidal malignant melanoma (CMM) in June 2021 and underwent a single session of (21 Gy) CyberKnife stereotactic radiotherapy (SRT). Eleven months later, we noticed 3+ anterior chamber cells with occasional vitreous cells in the left eye. Though the tumor looked regressed, there were mild optic disc leakage, early hypofluorescent and late hyperfluorescent punctate lesions scattered 360 degrees, and late staining of the mass on fluorescein angiogram. The findings were compatible with a unilateral multiple evanescent white dot syndrome (MEWDS)-like reaction that was most likely related to CyberKnife SRT-induced tumor necrosis, and a dexamethasone implant was administered intravitreally into the left eye together with topical steroids. A second intravitreal injection of dexamethasone was given three months later due to remittance of the angiographic features. As there are only a few reports on CyberKnife SRT for the treatment of CMM, we wanted to share our interesting observation of a post-treatment MEWDS-like reaction likely related to tumor necrosis syndrome with the ophthalmic community.

PREDICTIVE ROLE OF SWEPT-SOURCE OCT-ANGIOGRAPHY IN RELAPSING VOGT-KOYANAGI-HARADA DISEASE.

to describe the Optical Coherence Tomography Angiography (OCTA) findings as a predictive role in chronic-relapsing stage of Vogt-Koyanagi-Harada disease (VKHD) and its comparison with other imaging modalities such as fluorescein angiography (FA), indocyanine green angiography (ICGA), and Spectral-Domain OCT (SD-OCT). A 37-year-old-lady from Blangadesh was diagnosed with VKHD. She was evaluated eight months before for a routine examination at which time she was in clinical remission. Full ophthalmic evaluation with multimodal imaging and OCTA was performed. Ophthalmic evaluation was unremarkable. SD-OCT disclosed increased choroidal thickness OU while SS-OCTA imaging showed choroidal flow voids well matching hypofluorescent round lesions found by ICGA. A week later the disease reactivated. CTA may provide novel insights into inflammatory activity of the choroid in and potentially have a predictive role in relapsing VKHD.

Birdshot chorioretinopathy in a South Asian patient

To describe the clinical features of a South Asian patient with HLA-A29-positive birdshot chorioretinopathy (BSCR). A 54-year-old South Asian woman presented with blurred vision and floaters. The ophthalmic examination showed bilateral vitritis and retinal vasculitis. Fluorescein angiography revealed a bilateral disc and retinovascular leakage along the arcades. On indocyanine green angiography, multiple hypo-fluorescent lesions were found. Work-up revealed positive HLA-A29. BSCR is primarily seen in patients of European descent, but it can occur in other ancestries. We present an HLA-A29-positive BSCR South Asian patient to highlight that this disease can be seen in patients of South Asian descent.

Clinical features and multimodal imaging findings in primary vitreoretinal lymphoma

AbstractPurpose: To describe clinical features and multimodal imaging findings in primary vitreoretinal lymphoma (PVRL).Methods: Medical records and imaging findings of 4 patients (4 eyes) diagnosed with PVRL were retrospectively reviewed. Detailed ophthalmic examination, fundus photography, fundus autofluorescence (FAF), fluorescein angiography (FA), optical coherence tomography (OCT) and OCT angiography (OCTA) were analysed in all patients. All patients underwent vitreous biopsy for diagnosis confirmation. Brain magnetic resonance imaging (MRI) was performed in all cases.Results: There were 2 women and 2 male patients with a mean age of 68.75 years. Visual blurring was the presenting symptom in all patients (100%). Clinical findings at presentation included vitritis in 4 eyes (100%), vitreous clumps in 2 eyes (50%), subretinal deposits in 3 eyes (75%) and exudative retinal detachment in one eye (25%). FAF showed granular hyperautofluorescence and hypoautofluorescence lesions in 3 eyes (75%) and blockage by mass lesion was seen in 1 eye. The most common pattern on FA was hypofluorescent lesions with a “leopard spot” appearance (75%). OCT revealed hyperreflective sub retinal lesions between retinal pigment epithelium and Bruch's membrane with an undulating shape (seasick sign) in 3 eyes (75%) and epiretinal membrane in one eye. OCTA showed areas of decreased signal on choriocapillaris slab in 2 eyes (50%). MRI of the globes and brain showed no abnormality in all cases. Vitreous cytology revealed atypical mononuclear cells consisting with the diagnosis of PVRL in all cases. The patients were referred to oncology unit.Conclusions: The diagnosis of PVRL is challenging. Multimodal imaging provides novel insights into features of PVRL.

LONGITUDINAL FOLLOW-UP OF CHOROIDAL GRANULOMAS WITH INDOCYANINE GREEN ANGIOGRAPHY AND OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY: A Lesion-Based Analysis.

To investigate choroidal granulomas visualized by indocyanine green angiography (ICGA) and optical coherence tomography angiography (OCTA) in response to treatment. Ten eyes of eight patients with tubercular, sarcoid, or Vogt-Koyanagi-Harada-associated choroidal granulomas were evaluated in this multicentric study. All patients underwent ICGA and OCTA at baseline, 1, and 3 months after treatment onset. Granulomas were identified as hypofluorescent lesions on intermediate ICGA phases. Late ICGA behavior and OCTA visualization were assessed. On baseline intermediate ICGA, 222 choroidal granulomas were detected. Overall, 174/222 granulomas were detected on baseline OCTA images. At 1 month, 28% of lesions were healed and 48 late ICGA hyperfluorescent lesions were identified. At 3 months, 63% of baseline lesions were healed, with 33 persistent late hyperfluorescent lesions. Optical coherence tomography angiography sensitivity was reduced at 1 and 3 months compared with baseline. Some flow-voids detected on OCTA at 1 and 3 months did not correspond to any visible lesion on ICGA. Different healing behaviors of choroidal granulomas were identified combining ICGA and OCTA analysis. Late ICGA hyper-fluorescent lesions may be the consequence of a possible fibrotic shift. Structural changes in the choroid may persist after active granulomas resolution resulting in persistent flow voids on OCTA.

Classification of Non-Infectious and/or Immune Mediated Choroiditis: A Brief Overview of the Essentials.

The choroid was poorly accessible to imaging investigation until the last decade of the last century. With the availability of more precise imaging methods such as indocyanine green angiography (ICGA) and, later, optical coherence tomography (OCT), enhanced depth OCT (EDI-OCT), and OCT angiography (OCTA), appraisal of choroidal inflammation has substantially gained in accuracy. This allowed to precisely determine which structures were touched in the different non-infectious choroiditis entities and made it possible to classify this group of diseases, ICGA signs, mainly hypofluorescent lesions, were identified and described. Previous publications have divided angiographic findings into two main sets of signs: (1) irregular “geographic” hypofluorescent areas corresponding to choriocapillaris non-perfusion and (2) round more regular, hypofluorescent dark dots more evenly distributed in the fundus corresponding to more deep choroidal stromal foci. These distinct findings allowed to subdivide and classify choroiditis into choriocapillaritis and stromal choroiditis. Additional signs were identified from EDI-OCT and OCTA examination supporting the classification of choroiditis into choriocapillaritis and stromal choroiditis. Results: Diseases involving principally the choriocapillaris included Multiple Evanescent White Dot Syndrome (MEWDS), Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE), Idiopathic Multifocal Choroiditis (MFC), and Serpiginous Choroiditis (SC) as well as mixed forms. Diseases primarily involving the choroidal stroma included HLA-A29 Birdshot Retinochoroiditis (BRC), Vogt-Koyanagi-Harada disease (VKH), Sympathetic Ophthalmia (SO), and Sarcoidosis chorioretinitis (SARC). Thanks to new imaging investigations of the choroid, it is now possible to classify and understand the diverse clinicopathological mechanisms in the group of non-infectious choroiditis entities.

Evaluation of fluocinolone acetonide 0.19 mg intravitreal implant in the management of birdshot retinochoroiditis

PurposeTo report treatment outcomes and efficacy of the fluocinolone acetonide 0.19 mg intravitreal implant (Iluvien) in controlling retinal and choroidal inflammation in 11 patients with birdshot retinochoroiditis.MethodsA single-centre, retrospective, interventional...

Indocyanine green angiographic findings in presumed intraocular tuberculosis.

To study features of Indocyanine green angiography (ICGA) in patients with presumed intraocular tuberculosis. Retrospective study of 48 consecutive patients (77 eyes) who underwent ICGA. The following signs were analysed: choroidal perfusion inhomogeneity, early hyperfluorescent stromal vessels, round or oval hypofluorescent dark dots (HDDs), hypofluorescent geographic lesions (HGLs), fuzzy or lost pattern of large stromal choroidal vessels, disc hyperfluorescence and diffuse late choroidal hyperfluorescence. Among 44 eyes of 29 patients with no clinical evidence of choroidal involvement, only 7 eyes of 6 patients had no ICGA evidence of choroidal involvement. On the other hand, ICGA findings suggesting choroidal involvement were noted in 37 (84.1%) eyes of 23 patients in the form of HDDs in all 37 (100%) eyes, HGLs in 7 (18.9%) eyes, disc hyperfluorescence in 20 (45.5%) eyes, fuzzy stromal vessels in 17 (38.6%) eyes, early hyperfluorescent stromal vessels in 13 (29.5%) eyes, late pinpoint hyperfluorescence in 11 (25%) eyes and late diffuse choroidal hyperfluorescence in 7 (15.9%) eyes. Among 33 eyes of 19 patients with clinically evident choroidal involvement, the following findings were identified; HDDs in 12 (36.4%) eyes, HGLs in 10 (30.3%) eyes, both HDDs and HGLs in 9 (27.3%) eyes, disc hyperfluorescence in 11 (33.3%) eyes, early hyperfluorescent stromal vessels in 7 (21.2%) eyes, fuzzy stromal vessels in 6 (18.2%) eyes and late diffuse choroidal hyperfluorescence was present in 2 (6.1%) eyes. ICGA is necessary in identifying and diagnosing subclinical tuberculous choroidal involvement. The most prevalent ICGA finding was persistent HDDs.

Multimodal imaging in sclerochoroidal calcification: a case report and literature review

BackgroundSclerochoroidal calcification (SCC), a rare condition found in elderly people, is idiopathic or occasionally secondary to disorders affecting calcium metabolism. Findings of multimodal imaging including choroidal circulation are, however, largely unknown. We present a patient of SCC with systemic background, who underwent multimodal imaging evaluations.Case presentationA 70-year-old Japanese man was referred to our clinic because of bilateral fundus lesions. He had a history of chronic kidney disease (CKD) and secondary hyperparathyroidism. Fundus photography showed a cluster of choroidal folds in the superotemporal extra-macular region OS. Swept-source optical coherence tomography demonstrated ellipsoid zone disruption OD, retinal pigment epithelium undulation OS, dilated Haller layer veins OU, and central choroidal thickening OU and thinning of the overlying choroid due to scleral elevation OS. Fluorescein angiography detected macular hyperfluorescence OD. Indocyanine green angiography demonstrated choroidal vascular hyperpermeability together with numerous scattered hypofluorescent lesions OU. Fundus autofluorescence showed multiple hypoautofluorescent spots surrounded by hyperautofluorescent areas OD. Laser speckle flowgraphy exhibited choroidal blood flow reduction represented by a cold color pattern OU. B-mode echography displayed hyperechoic solid lesions with acoustic shadowing and orbital computed tomography revealed high density areas in the sclera, both of which were consistent with calcification. The patient was diagnosed with SCC, and these imaging findings remained unchanged 7 months after the diagnosis.ConclusionsWe reported a case of SCC with the background of CKD. Our detailed multimodal observations indicated choroidal hypoperfusion possibly caused by mechanical compression due to calcium deposition in the sclera.

CHOROIDAL GRANULOMAS VISUALIZED BY SWEPT-SOURCE OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

To assess the visualization of choroidal granulomas with swept-source optical coherence tomography angiography (OCTA). Consecutive patients with granulomatous choroiditis due to tuberculosis, sarcoidosis, or Vogt-Koyanagi-Harada disease underwent baseline OCTA images using a 12 × 12-mm field of view, and the choroidal slabs were analyzed by two independent examiners who counted the oval areas of flow void. Simultaneously, indocyanine green angiography (ICGA) and enhanced-depth imaging OCT were performed to mark visible choroidal changes corresponding to granulomatous lesions. The lesion areas on OCTA and ICGA were assessed using the in-built caliper tool. Three hundred and one round-shaped areas of flow void on OCTA, 209 hypofluorescent round lesions on ICGA, and 42 hyporeflective choroidal lesions on enhanced-depth imaging OCT were identified in 23 eyes from 14 patients. Of the 209 ICGA granulomas, 197 (94.3%) had a corresponding round area of flow void on OCTA that was interpreted as a granuloma. One hundred and four additional round flow voids were identified on OCTA that did not correspond to any hypofluorescent lesion on ICGA. The mean area of the 197 granulomas detected with both imaging modalities was significantly larger on ICGA (mean 0.33 mm2) than that on OCTA (mean 0.28 mm2). Optical coherence tomography angiography seems to be an optimal imaging method for the visualization of choroidal granulomas.

Pseudo-inflammatory manifestations of choroidal lymphoma resembling Vogt-Koyanagi-Harada disease: case report based on multimodal imaging

BackgroundHematologic malignancies occasionally cause serous retinal detachment (SRD); however, its pathogenesis remains unclear. Here we present the imaging characteristics of metastatic choroidal lymphoma masquerading as Vogt-Koyanagi-Harada (VKH) disease.Case presentationA 45-year-old Japanese woman was referred to our clinic because of bilateral SRD with blurred vision. Fluorescein angiography revealed multiple pinpoint leakage followed by pooling OU. Enhanced depth imaging optical coherence tomography showed marked choroidal thickening OU. Laser speckle flowgraphy detected choroidal circulation impairment OU. Although these results totally agreed with the inflammatory manifestations of acute VKH disease, indocyanine green angiography demonstrated various sizes of sharply marginated hypofluorescent lesions that seemed atypical for the finding of VKH disease, i.e., vaguely marginated hypofluorescent small dots. Cerebrospinal fluid pleocytosis was not detected. Blood tests revealed leukocytosis together with elevation of lactate dehydrogenase and soluble interleukin-2 receptor levels. Corticosteroid pulse therapy did not improve any ocular findings. Bone marrow biopsy was then performed, leading to a definite diagnosis of diffuse large B-cell lymphoma. After starting systemic chemotherapy, both SRD and choroidal thickening resolved rapidly with visual recovery. However, choroidal hypoperfusion persisted, which contrasted distinctly with the inflammatory pattern of VKH disease, i.e., the restoration of choroidal blood flow in parallel with normalization of choroidal thickness.ConclusionsOur detailed multimodal observations highlighted the differential imaging features of choroidal lymphoma despite close resemblance to VKH disease especially at the initial stage. Impaired circulation in the thickened choroid marked the pseudo-inflammatory pathogenesis of SRD due to choroidal involvement with neoplastic, but not inflammatory cells.

The diagnostic value of multicolor scanning laser imaging combined with swept-source optical coherence tomography for lacquer cracks and myopia stretch lines of pathological myopia

Objective To analyze the diagnostic value of multicolor scanning laser imaging (confocal scanning laser ophthalmoscopy, cSLO) combined with swept-source optical coherence tomography (SS-OCT) for lacquer cracks (LC) and myopia stretch lines (MSL) of pathological myopia. Methods A observational study. A total of 83 eyes of 58 patients with pathological myopia were recruited from May 2017 to January 2018 in Department of Ophthalmology of The First People’s Hospital Affiliated to Shanghai Jiao Tong University. Among 58 patients, 20 were males (30 eyes) and 38 were females (53 eyes). The mean age was 50.65±12.02 (range from 24 to 70) years old; the average BCVA was 0.37±0.32; the average diopter was −11.38±4.96 D; and the average axial length was 28.91±2.15 mm. All participants underwent FFA and ICGA examination to obtain FFA, ICGA, infrared light reflection (IR) and autofluorescence (AF) images. SS-OCT was applied for scanning macular and optic disc at 9 mm × 9 mm range. cSLO was performed with macular as the center. All images were inspected carefully by three independent observers and the consistency test was detect. LC were diagnosed as hyperreflective line in FFA and hypofluorescent linear lesions in late ICGA. MSL were defined as both hypofluorescent linear lesions in FFA and late ICGA. The accuracy of each inspection item in the diagnosis of LC was detected. The optimal technique was applied with SS-OCT to further explore the detection rate of LC. Results The intra-observer reproducibility was good to excellent for all measurements (Kappa=0.938, P<0.01). The positive detection rate of LC and MSL was highest in the standard images of cSLO (77.1%), followed by SS-OCT red free (73.1%), fundus photography (72.3%), IR (72.3%) and AF (49.4%). The cSLO was optimal in the test consistency (Kappa=0.520, P<0.01) and accuracy (the area under the receiver operating characteristic was 0.750). SS-OCT and cSLO were jointly applied to diagnosis of LC and MSL in high myopia. The positive detection rate of LC, MSL and LC+MSL were 91.7%, 91.2% and 93.3% respectively. The characteristics of LC in SS-OCT were irregularities and discontinuous of the RPE-Bruch membrane line, discontinuous inner ellipsoid zone, thinner choroid, an increased light penetrance into deeper tissues, and RPE fracture in severe cases. MSL was mainly manifested as RPE clumps, visible large choroidal vessels protruding and pushing the overlying RPE toward the vitreous. Conclusions The diagnosis rate of LC in pathological myopia by cSLO is 77.1%. The standard images of cSLO combined with SS-OCT can diagnose LC, MSL and LC+MSL at rates of 91.7%, 91.2% and 93.3% respectively. Key words: Myopia, degenerative/diagnosis; Microscopy, confocal; Tomography, optical coherence

Multimodal Imaging of Multiple Evanescent White Dot Syndrome: A New Interpretation

ABSTRACT Purpose To investigate the pathogenesis of Multiple Evanescent White Dot Syndrome (MEWDS) using multimodal imaging (MMI). Methods Retrospective case series of 7 patients with acute MEWDS. Each patient underwent: near-infrared reflectance (IR), blue and near-infrared autofluorescence (FAF and NIRAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), enhanced depth imaging optical coherence tomography (EDI-OCT) on Spectralis, and optical coherence tomography angiography on OCTA Spectralis, XR Avanti or Plex Elite 9000. Results OCTA and FA findings of early hyperfluorescence depict an unaffected choriocapillaris. On ICGA early to late hypofluorescent lesions corresponded to the hyporeflectivity on IR, consistent with altered reflectivity of the RPE. The SDI-OCT showed ellipsoid zone disruption as confirmed by FAF hyperautofluorescence. Some lesions showed a hypertransmission sign underneath the RPE, possibly due to changes in RPE intracellular melanin as suggested by NIRAF hypoautofluorescence. Conclusions The MMI findings of MEWDS are secondary to RPE reflectivity changes, suggesting its pivotal role.

TOXIC EFFECTS OF HYDROXYCHLOROQUINE ON THE CHOROID: Evidence From Multimodal Imaging.

To investigate the choroidal changes that occur in hydroxychloroquine (HCQ) retinopathy using multimodal imaging including fluorescein angiography, indocyanine green angiography, and optical coherence tomography (OCT) angiography and to correlate these changes with retinal findings obtained using OCT and fundus autofluorescence. In 20 patients (n = 40 eyes) with systemic lupus erythematosus or rheumatoid arthritis diagnosed to have HCQ retinopathy, imaging modalities including swept-source OCT, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, and OCT angiography were used to evaluate retinal and choroidal changes associated with retinopathy. The assessments included specific findings such as presence of hyperfluorescent or hypofluorescent lesions on angiography and signal void zones on OCT angiography, their frequencies, and the correlations among the retinal and choroidal findings. These findings were also correlated with the severity of retinopathy. Retinopathy progression was defined using fundus autofluorescence and OCT and correlated with the retinal/choroidal findings. Fluorescein angiography demonstrated a hyperfluorescent area, which reflects a defective retinal pigment epithelium, with multiple tiny dark spots within the area. Indocyanine green angiography showed a hypofluorescent area with dark spots, which was matched to the hypoautofluorescent area on fundus autofluorescence. Although there were no specific morphologic abnormalities in the choroid layers using en face choroidal imaging, OCT angiography demonstrated signal void areas on the choriocapillaris in the areas of the retinal pigment epithelium defect. Even after cessation of HCQ, there was progression of retinopathy in eyes with choroidal involvement, particularly on the area of choroidal findings. Multimodal imaging demonstrates choriocapillaris degeneration in eyes with HCQ retinopathy, particularly those with severe retinopathy. The choroidal change was associated with outer retinal toxicity of HCQ.

Blue Autofluorescence Fundus Imaging for Monitoring Retinal Degeneration in Royal College of Surgeons Rats.

PurposeDevelopment of a method for noninvasive longitudinal follow-up of retinal degeneration in the whole retina for Royal College of Surgeons (RCS) rats, a commonly used model of retinitis pigmentosa associated with mutations in the MER-proto-oncogene tyrosine kinase (MERTK) gene.MethodsPigmented RCS rats at postnatal (p) days p28 to p84 were subjected to a biweekly spectral-domain optical coherence tomography (SD-OCT), blue laser fundus autofluorescence (BL-FAF) imaging, and multicolor fundus imaging. Wild-type (WT; Long Evans) rats were tested as control.ResultsHyperautofluorescence developed throughout the fundus at p42, concomitant with a significant increase in SD-OCT thickness and reflectivity of the debris zone (DZ) layer as well as thinning of the photoreceptor outer nuclear layer (ONL). From p56 to p84, discrete hypofluorescent lesions surrounded by hyperfluorescent flecks were demonstrated around the optic disc that gradually spread throughout the retina. The hypofluorescent lesions were associated with loss of ONL and gradual thinning of the DZ layer. No hypofluorescent BL-FAF lesions were observed in WT rats.ConclusionsThis study suggests that BL-FAF imaging may present a new method for noninvasive longitudinal follow-up of retinal degeneration in nearly the whole retina in RCS rats.Translational RelevanceA clinical test was developed that may be implemented in translational studies in the RCS rat model of MERTK-associated retinitis pigmentosa.

Comparative analysis of autofluorescence and OCT angiography in Stargardt disease

AimsTo characterise the vasculature of the retina in patients with Stargardt disease (STGD) using optical coherence tomography angiography (OCTA) and to compare these functional findings with fundus autofluorescence (FAF) imaging.MethodsThis...