Fundus Autofluorescence Imaging Research Articles

Deep phenotypic characterization of the retinal dystrophy in patients with RNU4ATAC-associated Roifman syndrome.

To characterize the retinal phenotype in RNU4ATAC-associated Roifman syndrome. Ten patients (including 8 males) with molecularly confirmed Roifman syndrome underwent detailed ophthalmologic evaluation including fundus imaging, fundus autofluorescence (FAF) imaging, spectral-domain optical coherence tomography (SD-OCT), and electroretinography (ERG). Six patients had follow-up eye exams. All patients also underwent comprehensive examination for features of extra-retinal Roifman syndrome. All patients had biallelic RNU4ATAC variants. Nyctalopia was common (7/10). Visual acuity at presentation ranged from 20/20 to 20/200 (Age Range: 5-41 years). Retinal exam revealed features of generalized retinopathy with mid-peripheral pigment epithelial changes. A para or peri-foveal ring of hyper-autofluorescence was the commonest FAF abnormality noted (6/8). The SD-OCT demonstrated relative preservation of the foveal ellipsoid zone in six cases; associated features included cystoid changes (5/10) and posterior staphyloma (3/10). The ERG was abnormal in all patients; nine showed generalized rod-cone dystrophy, whilst one patient with sectoral retinal involvement only had isolated rod dystrophy (20 years old). On follow-up examination (Mean duration: 8.16 years), progressive loss of visual acuity (2/6), mid-peripheral retinal atrophy (3/6) or shortening of ellipsoid zone width (1/6) were observed. This study has characterized the retinal phenotype in RNU4ATAC-associated Roifman syndrome. Retinal involvement is universal, early-onset, and overall, the retinal and FAF features are consistent with rod-cone degeneration that is slowly progressive over time. The sub-foveal retinal ultrastructure is relatively preserved in majority of patients. Phenotypic variability independent of age exists, and more study of allelic- and sex-based determinants of disease severity are necessary.

Retinal Displacement after Scleral Buckle versus Combined Buckle and Vitrectomy for Rhegmatogenous Retinal Detachment: ALIGN Scleral Buckle versus Pars Plana Vitrectomy with Scleral Buckle

To assess the risk of retinal displacement after scleral buckle (SB) versus pars plana vitrectomy with SB (PPV-SB). Multicenter prospective nonrandomized clinical trial. The study took place at VitreoRetinal Surgery in Minneapolis, Minnesota, Sankara Nethralaya in Chennai, India, and St. Michael's Hospital in Toronto, Canada from July 2019 to February 2022. Patients who underwent successful SB or PPV-SB for fovea-involving rhegmatogenous retinal detachment with gradable postoperative fundus autofluorescence (FAF) imaging were included in the final analysis. Two masked graders assessed FAF images 3 months postoperatively. Metamorphopsia and aniseikonia were assessed with M-CHARTs and the New Aniseikonia Test, respectively. The primary outcome was the proportion of patients with retinal displacement detected with retinal vessel printings on FAF in SB versus PPV-SB. Ninety-one eyes were included in this study, of which 46.2% (42 of 91) had SB and 53.8% (49 of 91) underwent PPV-SB. Three months postoperatively, 16.7% (7 of 42) in the SB group and 38.8% (19 of 49) in the PPV-SB group had evidence of retinal displacement (difference= 22.1%; odds ratio= 3.2; 95% confidence interval [CI], 1.2-8.6; P= 0.02) on FAF. The statistical significance of this association increased after adjustment for extent of retinal detachment, baseline logarithm of the minimum angle of resolution, lens status, and sex in a multivariate regression analysis (P= 0.01). Retinal displacement was detected in 22.5% (6 of 27) of patients in the SB group with external subretinal fluid drainage and 6.7% (1 of 15) of patients without external drainage (difference= 15.8%; odds ratio= 4.0; 95% CI, 0.4-36.9; P= 0.19). Mean vertical metamorphopsia, horizontal metamorphopsia (MH), and aniseikonia were similar between patients in the SB and PPV-SB groups. There was a trend to worse MH in patients with retinal displacement versus those without retinal displacement (P= 0.067). Scleral buckle is associated with less retinal displacement compared with PPV-SB, indicating that traditional PPV techniques cause retinal displacement. There is a trend toward increased risk of retinal displacement in SB eyes that underwent external drainage compared with SB eyes without drainage, which is consistent with our understanding that the iatrogenic movement of subretinal fluid, such as that which occurs intraoperatively during external drainage with SB, may induce retinal stretch and displacement if the retina is then fixed in the stretched position. There was a trend to worse MH at 3 months in patients with retinal displacement. The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Tumor-Associated Retinal Pigmentation in Choroidal Melanoma

To report a previously unrecognized choroidal melanoma clinical feature termedtumor-associated retinal pigmentation(TARP) and determine any correlation with tumor biology. Imaging and histologic analysis of a retrospective cohort of patients. Patients with choroidal melanoma identified as having TARP on funduscopy at the Liverpool Ocular Oncology Centre (LOOC), United Kingdom, from January 2020 through January2023. Clinical and imaging characteristics of patients diagnosed with choroidal melanoma and exhibiting TARP on fundoscopy were documented. Details of these choroidal melanomas were collated and correlated with histopathology and molecular genetic reports. The chromosome 3 status of each tumor was assessed. In enucleated samples, immunostaining was undertaken to determine the nature of the TARP using specific markers (CD68 and MelanA). Features of TARP on widefieldfundus color imaging, fundus autofluorescence (FAF), and OCT were described. Tumor chromosome 3 status and the immunoprofile of the TARP also were collated. Tumor-associated retinal pigmentationhad a prevalence rate of 7.47 per 100 cases of choroidal melanoma at the LOOC. Twenty-three eyes with TARP were analyzed, with a mean age of 71.4 years (range, 51-88 years). The median largest basal diameter was 16.10 mm (range, 9.17-21.32 mm), and the mean tumor thickness was 8.04 mm (range, 1.40-13.80 mm).Tumor-associated retinal pigmentationwas observed on widefield color fundus imaging, with hypofluorescence on FAF images and represented hyperreflective foci located in intraretinal and subretinal spaces on OCT scans. Seventeen patients (73.9%) underwent enucleation, and 6 patients (26.1%) underwent globe-sparing treatment. Molecular genetic analysis of 20 choroidal melanomas (after enucleation or radiotherapy biopsy) revealed monosomy 3 in 18 tumors (90%). Immunostaining of the TARP in enucleated eyes showed CD68+ melanophages in all 17 patients appearing as scattered cells and aggregates; MelanA findings were negative. Tumor-associated retinal pigmentationrepresents tumor-associated macrophages, not melanocytes, within intraretinal and subretinal spaces of larger choroidal melanomas. Radiation treatments need not involve this area in the treatment plan, minimizing radiation-related complications. This novel clinical sign seems to be linked to tumors of high metastatic-risk clinical and genetic characteristics, with a preponderance having monosomy 3 anomalies. The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Retinal displacement following repair of rhegmatogenous retinal detachment.

Retinal displacement following the repair of rhegmatogenous retinal detachment (RRD) has been reported in recent studies. It was described as vertical movement of the retinal relative to its original location, as evidenced by retinal vessel printing on fundus autofluorescence imaging. This review reports the current literature on retinal displacement. We conducted an English literature search using Medline, PubMed, Embase, and Web of Science. We have reviewed 22 articles describing the diagnosis, frequency, and possible risk factors for retinal displacement. The reported rate of retinal displacement ranged from 6.4% to 62.8%, and the possible risk factors included the detachment-to-repair time, location and extent of RRD, macula-off RD, presence of subretinal fluid, use of perfluorocarbon, use of tamponade, postoperative facedown positioning, type of RRD repair, and presence of proliferative vitreoretinopathy, especially high grade. This review increases awareness of retinal displacement and its associated visual effects.

Prospective Study Comparing Quantitative Self-Monitoring Metamorphopsia Measurement Tools in Myopic Choroidal Neovascularization (mCNV).

To assess the ability of two self-monitoring digital devices to detect metamorphopsia in myopic choroidal neovascularization (mCNV) and compare their usability. This was a 12-month prospective observational study at a tertiary care eye hospital, Switzerland. Twenty-three Caucasian patients with mCNV were recruited, 21 eyes were analyzed. Primary and secondary outcome measures: Primary outcome measures were the metamorphopsia index scores as assessed by the two self-monitoring digital devices (Alleye App and AMD - A-Metamorphopsia-Detector software) at baseline, at 6 and 12 months and individual optional visits in between. Secondary outcome measures included best corrected visual acuity and morphological parameters (including disease activity) as evaluated by spectral-domain optical coherence tomography and fundus autofluorescence imaging. Location of mCNV was graded using the Early Treatment of Diabetic Retinopathy Study grid overlay. A usability questionnaire was administered at 12 months. Bland-Altman plots evaluated the limits of agreement of both devices. Linear regression analysis assessed the correlation between the difference and the average of the two scores. A total of 202 tests were performed. Disease activity of mCNV was observed at least once in 14 eyes. Both scores concordantly detected metamorphopsia exhibiting a displaced scale of measurement yielding a coefficient of determination of 0.99. Concordance rate for pathological scores was 73.3%. Both scores were not significantly different in active and inactive mCNV. Overall, the usability scores were higher for the Alleye App than the AMD - A-Metamorphopsia-Detector software (4.61±0.56 vs 3.31±1.20; p<0.001). In subjects aged >75 years, scores were slightly lower (4.08±0.86 vs 2.97±1.16; p= 0.032). Whilst both self-monitoring devices concordantly identified metamorphopsia, they might act as an adjunct to hospital visits, but due to slight reactivations in mCNV and presence of metamorphopsia also in inactive disease the ability of detecting early mCNV activity might be limited.

Minimal Gas Vitrectomy with Scleral Buckle to Minimize Retinal Displacement in Rhegmatogenous Retinal Detachment with Inferior Breaks.

To present a novel technique, minimal gas vitrectomy with scleral buckle (MGV-SB), as a means of potentially minimizing retinal displacement compared to standard vitrectomy or vitrectomy/scleral buckle for select cases. A patient with a right macula off retinal detachment and retinal breaks at 7 o'clock and 10 o'clock underwent a 23-gauge pars plana vitrectomy, localization and cryopexy of the breaks along with an inferior temporal segmental scleral buckle. No air-fluid exchange was performed. Suturing of sclerotomies, anterior chamber paracentesis of 0.3 ml and intravitreal injection of 0.6 mL pure sulfur hexafluoride were then performed. The patient was advised to perform the steamroller maneuver with initial face down positioning for 6 hours. The patient achieved retinal reattachment and post-operative wide-field fundus autofluorescence imaging demonstrated a high integrity retinal attachment (HIRA) with no retinal displacement. The MGV-SB technique has the potential to minimize retinal displacement in cases with inferior break(s) by using a small volume expansile gas tamponade and localized scleral buckle compared to standard vitrectomy or vitrectomy/scleral buckle with a full gas fill.

Insights Into PROM1-Macular Disease Using Multimodal Imaging.

To describe the features of genetically confirmed PROM1-macular dystrophy in multimodal images. Thirty-six (36) eyes of 18 patients (5-66 years; mean age, 42.4 years) were prospectively studied by clinical examination and multimodal imaging. Short-wavelength autofluorescence (SW-AF) and quantitative fundus autofluorescence (qAF) images were acquired with a scanning laser ophthalmoscope (HRA+OCT, Heidelberg Engineering) modified by insertion of an internal autofluorescent reference. Further clinical testing included near-infrared autofluorescence (NIR-AF; HRA2, Heidelberg Engineering) with semiquantitative analysis, spectral domain-optical coherence tomography (HRA+OCT) and full-field electroretinography. All patients were genetically confirmed by exome sequencing. All 18 patients presented with varying degrees of maculopathy. One family with individuals affected across two generations exhibited granular fleck-like deposits across the posterior pole. Areas of granular deposition in SW-AF and NIR-AF corresponded to intermittent loss of the ellipsoid zone, whereas discrete regions of hypoautofluorescence corresponded with a loss of outer retinal layers in spectral-domain optical coherence tomography scans. For 18 of the 20 eyes, qAF levels within the macula were within the 95% confidence intervals of healthy age-matched individuals; nor was the mean NIR-AF signal increased relative to healthy eyes. Although PROM1-macular dystrophy (Stargardt disease 4) can exhibit phenotypic overlap with recessive Stargardt disease, significantly increased SW-AF levels were not detected. As such, elevated bisretinoid lipofuscin may not be a feature of the pathophysiology of PROM1 disease. The qAF approach could serve as a method of early differential diagnosis and may help to identify appropriate disease targets as therapeutics become available to treat inherited retinal disease.

Deep phenotyping of PROM1-associated retinal degeneration

Background/aimsThe purpose of this study was to investigate retinal structure in detail of subjects with autosomal-dominant (AD) and autosomal-recessive (AR) PROM1-associated retinal degeneration (PROM1-RD), study design: institutional, cross-sectional study.MethodsFour eyes...

The Chrysanthemum Phenotype of Idiopathic Multifocal Choroiditis.

To describe the clinical characteristics and multimodal imaging (MMI) features of a distinctive subtype of active idiopathic multifocal choroiditis (iMFC) lesions with grey-yellow chorioretinal lesions surrounded by smaller satellite dots, a presentation referred to as "chrysanthemum lesions". Retrospective, observational, multi-center case series of eyes with active iMFC and chrysanthemum lesions. Multimodal imaging features were reviewed and presented. Twenty-five eyes from 20 patients (12 women and 8 men), with a mean age of 35.8±17.0 years (range, 7 - 78 years) were included. Chrysanthemum lesions were equally located in the macula (48.0%) or the mid/far-periphery (52.0%). The number of lesions per eye varied from 1 (16.0%) to more than 20 (56.0%). On optical coherence tomography (OCT), chrysanthemum lesions showed typical features of iMFC, including subretinal hyperreflective material splitting the retinal pigment epithelium/Bruch's membrane (RPE/BrM). Chrysanthemum lesions were hypoautofluorescent on fundus autofluorescence imaging, hyperfluorescent on fluorescein angiography, hypofluorescent on indocyanine green angiography, and associated with choriocapillaris flow signal deficit on OCT-angiography. Active iMFC may present with findings resembling chrysanthemum lesions. The distinctive lesion morphology on ophthalmoscopic examination, the high number of lesions, and the high prevalence of exclusive mid- and far-peripheral involvement may represent a distinctive phenotype of iMFC.

Retinal displacement following successful pars plana vitrectomy for rhegmatogenous retinal detachment

Background The appearance of hyperfluorescent lines next to the retinal blood vessels after successful retinal detachment (RD) surgery was explained by the occurrence of retinal displacement. This can attribute to the metamorphopsia experienced by some patients after their surgeries. Patients and methods A prospective interventional study was performed that included 30 eyes of 30 patients with primary rhegmatogenous RD, who had undergone successful RD surgery via pars plana vitrectomy and silicone oil tamponade. The entire patients had preoperative and postoperative fundus autofluorescence and optical coherence tomography. They were followed up for 3 months after surgery with testing of the best-corrected visual acuity and Amsler grid testing. Results The mean age of the included group was 53.7±9.2 years, and 17 (56.7%) patients were males. The most frequent tear site was upper temporal (43.3%), followed by multiple peripheral (23.33%), upper nasal (13.3%), lower temporal (13.3%), and the least was lower nasal (6.7%). Retinal displacement was detected by hyperfluorescent lines adjacent to the retinal blood vessels in the fundus autofluorescence imaging in 16 eyes (53.3% with 95% confidence interval: 36.7–70.0%). Patients with retinal displacement were significantly younger than patients without (P=0.019). The presence of proliferative vitreoretinopathy was significantly related to the occurrence of displacement (P=0.028). Metamorphopsia was significantly more frequent among patients with displacement (P&lt;0.001). Conclusion Retinal displacement had a high possibility to occur after successful repair of primary rhegmatogenous RD. Young patients and patients with proliferative vitreoretinopathy are at a higher risk for displacement.

Morphology of Serous Retinal Detachment in Morning Glory Optic Disc Anomaly in a Patient before and after Treatment with Systemic Carboanhydrase Inhibitors

Morning glory optic disc anomaly (MGODA) is a rare congenital defect of the optic nerve head. The optic nerve is enlarged, and its conical excavation is filled with glial tissue. It may be associated with cerebral malformations and ocular complications, whereas serous retinal detachment occurs in 38% of affected patients. Surgical treatment of detachment showed poor visual outcome in the past and conservative treatment options are scarce. A woman with MGODA presented in our clinic with sudden vision loss due to serous retinal detachment. She denied any previous ophthalmological problems and her past medical history was unremarkable. Vision testing showed normal visual acuity in her left eye and finger counting in her right eye. Slit lamp examination was unremarkable. Fundus examination of the right eye showed retinal detachment without holes or traction membranes and an enlarged optic disc with raised peripapillary tissue and glial tissue in the center of the optic disc. Due to the pathognomonic otpic disc finding, we diagnosed MGODA complicated by a serous retinal detachment. We treated the patient with systemic carboanhydrase inhibitors and documented the initial clinical findings as well as the course of disease under treatment by optical coherent tomography (OCT), fundus autofluorescence imaging (FAF), and visual field testing. During follow-up, we detected noticeable subretinal fluid regression and improvement in visual acuity. The application of oral carboanhydrase inhibitors appears to be a valid therapeutic option in patients with MGODA-associated serous macular detachment. OCT and FAF imaging are useful modalities for documentation of subretinal fluid regression and structural changes in the peripapillary region.

Topographic patterns of retinal lesions in multiple evanescent white dot syndrome.

To demonstrate different topographic distributions of multiple-evanescent white dot syndrome (MEWDS) and secondaryMEWDS disease and to describe possible associations. Clinical evaluation and multimodal retinal imaging in 27 subjects with MEWDS (29 discrete episodes of MEWDS). Ophthalmic assessment included best-corrected visual acuity testing and multimodal retinal imaging with OCT, blue-light autofluorescence, fluorescein and indocyanine green angiography, fundus photography, and widefield pseudocolor and autofluorescence fundus imaging. The topographic distribution of MEWDS lesions was centered on or around the optic disc (n = 17, 59%), centered on the macula (n = 7, 24%), sectoral (n = 2, 7%), or was indeterminate (n = 3, 10%). The MEWDS episodes either occurred in the absence ('primary MEWDS'; n = 14, 48%) or presence of concurrent chorioretinal pathology ('secondary MEWDS'; n = 15, 52%). In patients with the latter, MEWDS lesions were often centered around a coexisting chorioretinal lesion. The majority of patients in both groups experienced resolution of their symptoms and retinal changes on multimodal imaging by 3months. Distinct distributions of MEWDS lesions were identified. MEWDS may occur in tandem with other chorioretinal pathology, which may impact the topography of MEWDS lesions.

Pentosan polysulfate maculopathy: a brief primer for general practitioners, ophthalmologists, optometrists and urologists.

Pentosan polysulfate maculopathy: a brief primer for general practitioners, ophthalmologists, optometrists and urologists.

Detection of CSR from Blue Wave Fundus Autofluorescence Images using Deep Neural Network Based on Transfer Learning

Fluid clot below the retinal surface is the root cause of Central Serous Retinopathy (CSR), often referred to as Central Serous Chorioretinopathy (CSC). Delicate tissues that absorb sunlight and enable the brain to recognize images make up the retina. This important organ is vulnerable to damage, which could result in blindness and vision loss for the affected person. Therefore, complete visual loss may be reversed and, in some circumstances, may return to normal with early diagnosis discovery. Therefore, timely and precise CSR detection prevents serious damage to the macula and serves as a foundation for the detection of other retinal disorders. Although CSR has been detected using Blue Wave Fundus Autofluorescence (BWFA) images, developing an accurate and efficient computational system is still difficult. This paper focuses on the use of trained Convolutional Neural Networks (CNN) to implement a framework for accurate and automatic CSR recognition from BWFA images. Transfer Learning has been used in conjunction with pre-trained network architectures (VGG19) for classification. Statistical parameter evaluation has been used to investigate the effectiveness of DCNN. For VGG19, the statistic parameters evaluation revealed a classification accuracy of 97.30%, a precision of 99.56%, an F1 score of 97.25%, and a recall of 95.04% when using a BWFA image dataset collected from a local eye hospital in Cochin, Kerala, India. Identification of CSR from BWFA images is not done before. This paper illustrates how the proposed framework might be applied in clinical situations to assist physicians and clinicians in the identification of retinal diseases.

Characteristics of subretinal particles detected after pars plana vitrectomy for rhegmatogenous retinal detachment

BackgroundTo determine the incidence and characteristics of the multiple subretinal particles (SRPs) present after pars plana vitrectomy (PPV) for rhegmatogenous retinal detachment (RRD).MethodsThe medical records of 224 eyes of 224 patients that underwent PPV for RRD were reviewed. The presence of SRPs in the subretinal fluid blebs and the presence of subretinal deposits were determined by optical coherence tomography (OCT) and fundus autofluorescence (FAF). The characteristics of the FAF and infrared reflectance (IR) images of a scanning laser ophthalmoscope in eyes with SRPs (SRPs group) were compared to that of eyes without SRPs (control group).ResultsSRPs were observed in 27 eyes (12%), and they were completely resolved in 20 eyes (74%) after 6 months. The incidence of macula-off RRD (85%) and preoperative precipitates (41%) were significantly higher in the SRPs group than that in the control group (64%, P = 0.046; 12%, P = 0.002). The axial length was significantly shorter in the SRPs group than that in the control group (25.04 ± 1.54 mm, 26.00 ± 1.78 mm, P = 0.012). The preoperative and postoperative best-corrected visual acuity were not significantly different between the two groups (P = 0.702, P = 0.337). The subretinal fluid bleb determined by OCT were hyperfluorescent in the FAF images in 24 eyes (89%), and the subretinal deposits were hypofluorescent with solid appearance by OCT other than fluid in 3 eyes (11%). The hypofluorescent subretinal deposits in the FAF images were bright in the IR images in 2 eyes.ConclusionsThe SRPs consist of lipofuscin-related hyperfluorescent subretinal fluid and the subretinal deposits containing bright IR melanin particles of proliferating retinal pigment epithelial cells.

Randomized Phase IIb Study of Brimonidine Drug Delivery System Generation 2 for Geographic Atrophy in Age-Related Macular Degeneration

To evaluate the safety and efficacy of repeat injections of Brimonidine Drug Delivery System (Brimo DDS) Generation 2 (Gen 2) containing 400-μg brimonidine in patients with geographic atrophy (GA) secondary to age-related macular degeneration (AMD). A phase IIb, randomized, multicenter, double-masked, sham-controlled, 30-month study (BEACON). Patients diagnosed with GA secondary to AMD and multifocal lesions with total area of > 1.25 mm2 and ≤ 18 mm2 in the study eye. Enrolled patients were randomized to treatment with intravitreal injections of 400-μg Brimo DDS (n= 154) or sham procedure (n= 156) in the study eye every 3 months from day 1 to month21. The primary efficacy endpoint was GA lesion area change from baseline in the study eye, assessed with fundus autofluorescence imaging, at month 24. The study was terminated early, at the time of the planned interim analysis, because of a slow GA progression rate (∼ 1.6 mm2/year) in the enrolled population. Least squares mean (standard error) GA area change from baseline at month 24 (primary endpoint) was 3.24 (0.13) mm2 with Brimo DDS (n= 84) versus 3.48 (0.13) mm2 with sham (n= 91), a reduction of 0.25 mm2 (7%) with Brimo DDS compared with sham (P= 0.150). At month 30, GA area change from baseline was 4.09 (0.15) mm2 with Brimo DDS (n= 49) versus 4.52 (0.15) mm2 with sham (n= 46), a reduction of 0.43 mm2 (10%) with Brimo DDS compared with sham (P= 0.033). Exploratory analysis showed numerically smaller loss over time in retinal sensitivity assessed with scotopic microperimetry with Brimo DDS than with sham (P= 0.053 at month 24). Treatment-related adverse events were usually related to the injection procedure. No implant accumulation was observed. Multiple intravitreal administrations of Brimo DDS (Gen 2) were well tolerated. The primary efficacy endpoint at 24 months was not met, but there was a numeric trend for reduction in GA progression at 24 months compared with sham treatment. The study was terminated early because of the lower-than-expected GA progression rate in the sham/control group. Proprietary or commercial disclosures may be found after the references.

Discriminating Healthy Optic Discs and Visible Optic Disc Drusen on Fundus Autofluorescence and Color Fundus Photography Using Deep Learning—A Pilot Study

The aim of this study was to use deep learning based on a deep convolutional neural network (DCNN) for automated image classification of healthy optic discs (OD) and visible optic disc drusen (ODD) on fundus autofluorescence (FAF) and color fundus photography (CFP). In this study, a total of 400 FAF and CFP images of patients with ODD and healthy controls were used. A pre-trained multi-layer Deep Convolutional Neural Network (DCNN) was trained and validated independently on FAF and CFP images. Training and validation accuracy and cross-entropy were recorded. Both generated DCNN classifiers were tested with 40 FAF and CFP images (20 ODD and 20 controls). After the repetition of 1000 training cycles, the training accuracy was 100%, the validation accuracy was 92% (CFP) and 96% (FAF), respectively. The cross-entropy was 0.04 (CFP) and 0.15 (FAF). The sensitivity, specificity, and accuracy of the DCNN for classification of FAF images was 100%. For the DCNN used to identify ODD on color fundus photographs, sensitivity was 85%, specificity 100%, and accuracy 92.5%. Differentiation between healthy controls and ODD on CFP and FAF images was possible with high specificity and sensitivity using a deep learning approach.

CORRELATION BETWEEN INFLAMMATORY FOCI REACTIVATION AND ATROPHY GROWTH IN EYES WITH IDIOPATHIC MULTIFOCAL CHOROIDITIS.

To correlate the number of inflammatory reactivations in atrophic foci of multifocal choroiditis (MFC) with their growth rate over a 4-year span. Comparative case series. Optical coherence tomography scans of patients affected by MFC were reviewed to identify reactivations within or at the margin of atrophic MFC foci. The area of selected lesions was semiautomatically delineated on fundus autofluorescence images and recorded at yearly intervals for a total follow-up of 4 years. The main outcome was the difference in annual square-root transformed area growth rate between lesions that reactivated and lesions that did not. Sixty-six foci of 30 eyes of 24 patients were included. All MFC foci enlarged over time, but the annual growth rate was more than double in lesions that reactivated compared with those that did not (mean [SD], 0.051 [0.035] vs. 0.021 [0.015] mm/year, P < 0.001), despite starting from comparable baseline areas. For each additional inflammatory reactivation, the annual growth rate increased by more than 20% (+0.009 mm/year, 95% CI [0.006, 0.012], P < 0.001). Increasing number of reactivations of atrophic foci led to proportional increments in their growth rate, highlighting the need for a tight control of inflammatory relapses in patients affected by MFC.

Estimation of Visual Function Using Deep Learning From Ultra-Widefield Fundus Images of Eyes With Retinitis Pigmentosa

There is no widespread effective treatment to halt the progression of retinitis pigmentosa. Consequently, adequate assessment and estimation of residual visual function are important clinically. To examine whether deep learning can accurately estimate the visual function of patients with retinitis pigmentosa by using ultra-widefield fundus images obtained on concurrent visits. Data for this multicenter, retrospective, cross-sectional study were collected between January 1, 2012, and December 31, 2018. This study included 695 consecutive patients with retinitis pigmentosa who were examined at 5 institutions. Each of the 3 types of input images-ultra-widefield pseudocolor images, ultra-widefield fundus autofluorescence images, and both ultra-widefield pseudocolor and fundus autofluorescence images-was paired with 1 of the 31 types of ensemble models constructed from 5 deep learning models (Visual Geometry Group-16, Residual Network-50, InceptionV3, DenseNet121, and EfficientNetB0). We used 848, 212, and 214 images for the training, validation, and testing data, respectively. All data from 1 institution were used for the independent testing data. Data analysis was performed from June 7, 2021, to December 5, 2022. The mean deviation on the Humphrey field analyzer, central retinal sensitivity, and best-corrected visual acuity were estimated. The image type-ensemble model combination that yielded the smallest mean absolute error was defined as the model with the best estimation accuracy. After removal of the bias of including both eyes with the generalized linear mixed model, correlations between the actual values of the testing data and the estimated values by the best accuracy model were examined by calculating standardized regression coefficients and P values. The study included 1274 eyes of 695 patients. A total of 385 patients were female (55.4%), and the mean (SD) age was 53.9 (17.2) years. Among the 3 types of images, the model using ultra-widefield fundus autofluorescence images alone provided the best estimation accuracy for mean deviation, central sensitivity, and visual acuity. Standardized regression coefficients were 0.684 (95% CI, 0.567-0.802) for the mean deviation estimation, 0.697 (95% CI, 0.590-0.804) for the central sensitivity estimation, and 0.309 (95% CI, 0.187-0.430) for the visual acuity estimation (all P < .001). Results of this study suggest that the visual function estimation in patients with retinitis pigmentosa from ultra-widefield fundus autofluorescence images using deep learning might help assess disease progression objectively. Findings also suggest that deep learning models might monitor the progression of retinitis pigmentosa efficiently during follow-up.

Great imitator — ocular syphilis

We examined a 44-year-old woman without any known systemic disease with a decreased vision of a month duration in her left eye. She had undergone left uneventful cataract surgery with intraocular lens implantation elsewhere 14 years ago. On examination, her Snellen visual acuity was 1.0 (with the correction of –0.50–0.25 × 120) in the right eye and 0.4 (with the correction of +0.75–2.25 × 115) in the left. There was left hypochromic heterochromia. While the right anterior segment was unremarkable, there were small to medium-sized keratic precipitates, 3+ anterior chamber cells, a posterior chamber intraocular lens with an intact posterior capsule, and a few vitreous cells in the left eye. Fundus autofluorescence imaging revealed peripapillary hyperautofluorescence in the left eye, and fluorescein angiography revealed a marked optic nerve head and perivascular leakage at the posterior pole. The findings were not compatible with Fuchs uveitis, so the full infectious panel was worked out. Serologic investigations yielded the presence of syphilis, and the patient was treated successfully with systemic antibiotics. Our case demonstrates the mimicking nature of ocular syphilis and the importance of high clinical suspicion when reaching the correct diagnosis.