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OBJECTIVE: To refine retinal PRPH2-associated retinal degeneration (PARD) phenotypes using multimodal imaging. DESIGN: Retrospective review of clinical records and multimodal imaging. SUBJECTS: Patients who visited the inherited retinal degeneration (IRD) clinic at two tertiary referral eye centers with molecularly confirmed IRD due to PRPH2 variants. METHODS: Retinal imaging was reviewed using ultra-widefield (UWF) pseudocolor, UWF fundus autofluorescence (FAF), and spectral-domain optical coherence tomography (SD-OCT). Phenotypes were identified in the macular or peripheral region. A combined phenotype was considered if any phenotypes were present in both macular and peripheral regions. Mixed phenotypes in the macula or peripheral retina were considered if there were two distinct phenotypes identified in the same eye. The presence or absence of atrophy in the macular or peripheral area was also noted. MAIN OUTCOME MEASURE: Grading of multimodal imaging by phenotype and atrophy. RESULTS: A total of 144 eyes of 72 patients were included in this study. The majority of the eyes had combined macular and peripheral phenotypes (89/14, 61.8%), while 44 (30.6%) eyes had isolated macular findings, and 11 (7.6%) had isolated peripheral findings. Twenty-five eyes were classified with mixed macular phenotypes while fundus flavimaculatus dystrophy type was the most common combined macular and peripheral phenotype (54/144, 37.5%): n = 10 with macular dystrophy and macular flavimaculatus dystrophy, and n = 15 with butterfly pattern dystrophy and macular flavimaculatus dystrophy. Nearly half of the eyes (71/144, 49.3%) were identified to have concomitant outer retinal atrophy. Fundus flavimaculatus type dystrophy was also associated with the highest proportion of concomitant atrophy (57/71, 80.3%). CONCLUSION: PARD demonstrates a wide array of phenotypes using multimodal imaging. We report that combinations of classically described phenotypes were often seen. Additionally, macular and peripheral atrophy were often associated with PARD phenotypes. Refinement of PARD phenotypes using newer multimodal imaging techniques will likely assist diagnosis and future clinical trials.
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In this retrospective case series on neovascular age-related macular degeneration (nAMD), we aimed to improve Choroidal Neovascularization (CNV) visualization in Optical Coherence Tomography Angiography (OCTA) scans by addressing segmentation errors. Out of 198 eyes, 73 OCTA scans required manual segmentation correction. We compared uncorrected scans to those with minimal (2 corrections), moderate (10 corrections), and detailed (50 corrections) efforts targeting falsely segmented Bruch's Membrane (BM). Results showed that 55% of corrected OCTAs exhibited improved quality after manual correction. Notably, minimal correction (2 scans) already led to significant improvements, with additional corrections (10 or 50) not further enhancing expert grading. Reduced background noise and improved CNV identification were observed, with the most substantial improvement after two corrections compared to baseline uncorrected images. In conclusion, our approach of correcting segmentation errors effectively enhances image quality in OCTA scans of nAMD. This study demonstrates the efficacy of the method, with 55% of resegmented OCTA images exhibiting enhanced quality, leading to a notable increase in the proportion of high-quality images from 63 to 83%.
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Neovascularización Coroidal , Degeneración Macular , Tomografía de Coherencia Óptica , Humanos , Neovascularización Coroidal/diagnóstico por imagen , Neovascularización Coroidal/patología , Tomografía de Coherencia Óptica/métodos , Femenino , Masculino , Estudios Retrospectivos , Anciano , Degeneración Macular/diagnóstico por imagen , Degeneración Macular/patología , Degeneración Macular/complicaciones , Anciano de 80 o más Años , Procesamiento de Imagen Asistido por Computador/métodos , Persona de Mediana Edad , Angiografía con Fluoresceína/métodosRESUMEN
Purpose: Use of the electronic health record (EHR) has motivated the need for data standardization. A gap in knowledge exists regarding variations in existing terminologies for defining diabetic retinopathy (DR) cohorts. This study aimed to review the literature and analyze variations regarding codified definitions of DR. Design: Literature review and quantitative analysis. Subjects: Published manuscripts. Methods: Four graders reviewed PubMed and Google Scholar for peer-reviewed studies. Studies were included if they used codified definitions of DR (e.g., billing codes). Data elements such as author names, publication year, purpose, data set type, and DR definitions were manually extracted. Each study was reviewed by ≥ 2 authors to validate inclusion eligibility. Quantitative analyses of the codified definitions were then performed to characterize the variation between DR cohort definitions. Main Outcome Measures: Number of studies included and numeric counts of billing codes used to define codified cohorts. Results: In total, 43 studies met the inclusion criteria. Half of the included studies used datasets based on structured EHR data (i.e., data registries, institutional EHR review), and half used claims data. All but 1 of the studies used billing codes such as the International Classification of Diseases 9th or 10th edition (ICD-9 or ICD-10), either alone or in addition to another terminology for defining disease. Of the 27 included studies that used ICD-9 and the 20 studies that used ICD-10 codes, the most common codes used pertained to the full spectrum of DR severity. Diabetic retinopathy complications (e.g., vitreous hemorrhage) were also used to define some DR cohorts. Conclusions: Substantial variations exist among codified definitions for DR cohorts within retrospective studies. Variable definitions may limit generalizability and reproducibility of retrospective studies. More work is needed to standardize disease cohorts. Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
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PURPOSE: The authors hypothesize that optical coherence tomography angiography (OCTA)-visualized vascular morphology may be a predictor of choroidal neovascularization status in age-related macular degeneration (AMD). The authors thus evaluated the use of artificial intelligence (AI) to predict different stages of AMD disease based on OCTA en face 2D projections scans. METHODS: Retrospective cross-sectional study based on collected 2D OCTA data from 310 high-resolution scans. Based on OCT B-scan fluid and clinical status, OCTA was classified as normal, dry AMD, wet AMD active, and wet AMD in remission with no signs of activity. Two human experts graded the same test set, and a consensus grading between two experts was used for the prediction of four categories. RESULTS: The AI can achieve 80.36% accuracy on a four-category grading task with 2D OCTA projections. The sensitivity of prediction by AI was 0.7857 (active), 0.7142 (remission), 0.9286 (dry AMD), and 0.9286 (normal) and the specificity was 0.9524, 0.9524, 0.9286, and 0.9524, respectively. The sensitivity of prediction by human experts was 0.4286 active choroidal neovascularization, 0.2143 remission, 0.8571 dry AMD, and 0.8571 normal with specificity of 0.7619, 0.9286, 0.7857, and 0.9762, respectively. The overall AI classification prediction was significantly better than the human (odds ratio = 1.95, P = 0.0021). CONCLUSION: These data show that choroidal neovascularization morphology can be used to predict disease activity by AI; longitudinal studies are needed to better understand the evolution of choroidal neovascularization and features that predict reactivation. Future studies will be able to evaluate the additional predicative value of OCTA on top of other imaging characteristics (i.e., fluid location on OCT B scans) to help predict response to treatment.
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Neovascularización Coroidal , Atrofia Geográfica , Degeneración Macular Húmeda , Humanos , Inteligencia Artificial , Tomografía de Coherencia Óptica/métodos , Estudios Retrospectivos , Estudios Transversales , Angiografía con Fluoresceína/métodos , Neovascularización Coroidal/diagnóstico , Neovascularización Coroidal/tratamiento farmacológico , Degeneración Macular Húmeda/diagnóstico , Degeneración Macular Húmeda/tratamiento farmacológicoRESUMEN
PURPOSE: Mutations in the SCAPER gene have previously been reported to be a rare cause of syndromic and non-syndromic autosomal recessive retinitis pigmentosa (RP). We report a case of syndromic RP caused by a frameshift heterozygous mutation in SCAPER. Our case has a relatively mild ocular phenotype with the presence of cone involvement noted on full field electroretinogram (ffERG) without impacting central or color vision. MATERIALS AND METHODS: A 17-year-old male presented with progressive nyctalopia in both eyes. He underwent ophthalmic examination and multimodal imaging. A complete retinal degeneration panel consisting of 322 genes was used to screen for molecular causes of retinal dystrophy in this patient along with family segregation analysis. RESULTS: Fundus examination of the proband revealed mild RP phenotype with waxy pallor of optic discs, attenuated retinal arterioles, and single bone spicule like pigmentary change in the mid-periphery bilaterally. Multimodal imaging and ffERG demonstrated a picture of RP with cone dysfunction without impacting central or color vision bilaterally. Examined family members were found to be normal. The proband was found to be heterozygous for two novel frameshift pathogenic variants in SCAPER c.3781del, p. (Val1261Serfs*26), c.868_869del, p. (Glu290Serfs*7) both leading to predicted premature termination. The family members tested were found to be heterozygous for SCAPER c.868_869del, p. (Glu290Serfs*7) pathogenic variant confirming their carrier status. CONCLUSION: We report a case of a syndromic RP of previously unreported ocular phenotype associated with SCAPER pathogenic variant, which will add to the phenotypic spectrum of retinopathy and systemic features associated with pathogenic variants in SCAPER.
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Distrofias Retinianas , Retinitis Pigmentosa , Masculino , Humanos , Adolescente , Retinitis Pigmentosa/diagnóstico , Retinitis Pigmentosa/genética , Retinitis Pigmentosa/patología , Mutación del Sistema de Lectura , Mutación , Fenotipo , Células Fotorreceptoras Retinianas Conos/patología , Linaje , Proteínas Portadoras/genéticaRESUMEN
PURPOSE: This study aimed to compare a new Artificial Intelligence (AI) method to conventional mathematical warping in accurately overlaying peripheral retinal vessels from two different imaging devices: confocal scanning laser ophthalmoscope (cSLO) wide-field images and SLO ultra-wide field images. METHODS: Images were captured using the Heidelberg Spectralis 55-degree field-of-view and Optos ultra-wide field. The conventional mathematical warping was performed using Random Sample Consensus-Sample and Consensus sets (RANSAC-SC). This was compared to an AI alignment algorithm based on a one-way forward registration procedure consisting of full Convolutional Neural Networks (CNNs) with Outlier Rejection (OR CNN), as well as an iterative 3D camera pose optimization process (OR CNN + Distortion Correction [DC]). Images were provided in a checkerboard pattern, and peripheral vessels were graded in four quadrants based on alignment to the adjacent box. RESULTS: A total of 660 boxes were analysed from 55 eyes. Dice scores were compared between the three methods (RANSAC-SC/OR CNN/OR CNN + DC): 0.3341/0.4665/4784 for fold 1-2 and 0.3315/0.4494/4596 for fold 2-1 in composite images. The images composed using the OR CNN + DC have a median rating of 4 (out of 5) versus 2 using RANSAC-SC. The odds of getting a higher grading level are 4.8 times higher using our OR CNN + DC than RANSAC-SC (p < 0.0001). CONCLUSION: Peripheral retinal vessel alignment performed better using our AI algorithm than RANSAC-SC. This may help improve co-localizing retinal anatomy and pathology with our algorithm.
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Inteligencia Artificial , Retina , Humanos , Retina/diagnóstico por imagen , Retina/patología , Vasos Retinianos/diagnóstico por imagen , Vasos Retinianos/patología , Algoritmos , Redes Neurales de la ComputaciónRESUMEN
Ultra-widefield retinal imaging is increasingly used in ophthalmology and optometry practices to image patients identifying peripheral abnormalities. However, the clinical relevance of these peripheral retinal abnormalities is unclear. This cross-sectional study aims to firstly validate a new grading system, secondly, assess the prevalence of peripheral retinal abnormalities in retinal patients, and finally understand how peripheral findings may associate with retinal disease. Ultra-widefield pseudocolor fundus images were taken from the eyes of clinic patients. Demographic data and clinical diagnosis for each patient was noted. The grading system was validated using masked retinal specialists. Logistic regression identified associations between retinal disease and peripheral retinal findings. Using the grading system, inter-observer agreement was 76.1% with Cohen's Kappa coefficient 0.542 (p < 0.0001) and the test-retest agreement was 95.1% with Kappa 0.677(p < 0.0001). 971 images were included, with 625 eyes (64.4%) having peripheral abnormalities. Peripheral drusen was the most common abnormality (n = 221, 22.76%) and correlated with age-related macular degeneration (p < 0.001). Novel correlations were also identified between diabetic retinopathy and retinal pigmentation as well as pigmentary degeneration. This study provides a validated system for identifying peripheral abnormalities and adds to literature highlighting peripheral retinal associations with retinal disease which would benefit from further study.
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Retina , Drusas Retinianas , Humanos , Estudios Transversales , Prevalencia , Retina/diagnóstico por imagen , Fondo de Ojo , Angiografía con Fluoresceína/métodosRESUMEN
Objective: To analyze recent publications in Ophthalmology, the journal of the American Academy of Ophthalmology. Design: Retrospective review of published articles. Participants: No human participants were involved in the study. Methods: Articles published in Ophthalmology from January 2018 to December 2022 were reviewed and analyzed. Main Outcome Measures: Research and review articles were included and analyzed per the following: total number of published articles based on related subspecialty area, level of evidence using the modified Oxford level of evidence, number of citations, number of listed authors, gender of the corresponding author, country of affiliation of the corresponding and contributing author(s), and involvement of consortium(s), group(s), or committee(s). Results: A total of 965 articles were included. The mean (standard deviation) number of authors per article was 8.6 (5.7) and the majority of corresponding authors were male (665, 70.7%). The greatest number of published articles were related to retina (296, 30.7%) followed by glaucoma (172, 17.8%). The greatest number of Preferred Practice Pattern guidelines were also related to retina (7/24, 29.1%), followed by cornea/dry eye syndrome/external disease (6/24, 25%). Retina (77) had the most level 1 evidence, glaucoma (30) for level 2 evidence, and retina for levels 3 (69) and 4 (65). There were 223 articles contributed by consortia/groups/committees, with most from retina (73, 32.7%) followed by glaucoma (40, 17.9%). The mean number of citations per subspecialty article was highest in retina (45.8/article), followed by uveitis (31.7/article). The United States had the greatest number of affiliated corresponding authors (544, 56.4%), followed by the United Kingdom (68, 7.0%). There were 357 (37.0%) articles with coauthors affiliated outside the corresponding author's country of affiliation, although with a downward trend over the most recent 5-year period. There has been an increasing trend in the number of authors and consortia/group/committee involvement in publications. Conclusions: Although team science and collaborations have increased recently, ongoing efforts to diversify individuals, groups, and subspecialties may be needed. Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
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This study aimed to understand the profile of hydroxychloroquine-treated patients, referral patterns, and dosing and to assess the adherence of eye care providers to the latest 2016 screening guidelines provided by the American Academy of Ophthalmology. Patients were identified using electronic health records (EHR) taking hydroxychloroquine and were seen by optometrists, retinal specialists, and non-retinal ophthalmologists. Review of EHR data includes demographic characteristics, indications, and dosing profile of hydroxychloroquine use, eye care provider managing the patient, and imaging modalities performed. A total of 166 patients were included in the study. The most common indications for screening were systemic lupus erythematosus and discoid lupus (52.4%) followed by rheumatoid arthritis (18.7%) and Sjögren's syndrome (9.6%). Ninety-two (55.4%) patients were on a higher-than-recommended dose of > 5 mg/kg/day. Patients who weighed less (mean 63.9 kg) were taking a higher-than-recommended dose (vs. 81.5 kg, p < 0.001). Although retinal specialists adhered best to the use of all three recommended imaging modalities, visual field testing was done appropriately for only 8.3% of Asian and 71.1% of non-Asian patients. In conclusion, there is substantial variability in screening by ophthalmic providers and prescribing practices compared with the current recommendations. In particular, there is a marked deficiency in correct visual field testing in Asian patients. These findings are important to highlight potential interventions to improve screening for hydroxychloroquine toxicity.
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Artritis Reumatoide , Lupus Eritematoso Sistémico , Humanos , Hidroxicloroquina/efectos adversos , Artritis Reumatoide/diagnóstico , Artritis Reumatoide/tratamiento farmacológico , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Academias e Institutos , RetinaRESUMEN
Purpose: To report the structural and functional changes in a 67-year-old male with pentosan polysulfate sodium (PPS) maculopathy with a progressive resolution of bilateral vitelliform lesions after PPS cessation. Observations: The patient was initially seen after taking daily PPS for over 26 years. Three months after discontinuing PPS, the bilateral vitelliform lesions identified on spectral-domain optical coherence tomography (SD-OCT) at initial consultation had completely resolved. Bilateral resolution of vitelliform lesions was associated with a decline in best-corrected visual acuity, and ellipsoid zone disruption on SD-OCT. Conclusions and importance: Several PPS maculopathy phenotypes have been previously described including vitelliform lesions. Our case highlights that discontinuing PPS may lead to rapid resolution of vitelliform lesions in PPS maculopathy and may be associated with a rapid reduction in vision.
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Macular edema (ME), the accumulation of intraretinal fluid in the macula, is a common sight affecting sequelae of retinitis pigmentosa (RP). However, it is unclear why some patients develop ME, and others do not. This study aims to identify associations between clinical-genetic factors in RP with ME. Patients with clinically confirmed RP cases were identified from the inherited retinal disease database at a large tertiary referral academic center. Demographic and genetic testing findings were noted. Additionally, optical coherence tomography volume scans were graded using a validated grading system. One hundred and six patients (73.1%) were found to have ME in at least one eye (OD = 88, mean = 37.9%, OS = 98, mean = 31.7%). Structurally, the presence of epiretinal membrane (ERM) (p < 0.007) and vitreo-macular traction (VMT) (p < 0.003) were significantly associated with ME. Additionally, X-linked (p < 0.032) and autosomal dominant inheritance (p < 0.039) demonstrated a significant association with ME, with RP1 (p < 0.045) and EYS (p < 0.017) pathogenic variants also significantly associated with ME. This study, in a large cohort of RP patients, confirms previous retinal structural associations for ME in RP and identifies potential new genetic associations.
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Mácula Lútea , Edema Macular , Enfermedades de la Retina , Retinitis Pigmentosa , Humanos , Edema Macular/genética , Retinitis Pigmentosa/complicaciones , Retinitis Pigmentosa/genética , Retina/diagnóstico por imagen , Proteínas del OjoRESUMEN
PURPOSE: Ultra-widefield (UWF) imaging is commonly used in ophthalmology in tandem with scleral depressed examinations (SDE) to evaluate peripheral retinal disease. Because of the increased reliance on this technology in tele-ophthalmology, it is critical to evaluate its efficacy for detecting the peripheral retina when performed in isolation. Therefore, we sought to evaluate UWF imaging sensitivity in detecting retinal horseshoe tears (HSTs). STUDY DESIGN: Retrospective clinical validity and reliability study. METHODS: A single-institutional retrospective analysis was performed on patients at the Shiley Eye Institute, University of California, San Diego. Patients with HSTs seen on SDE who underwent treatment with laser were included in the study. A total of 140 patients with HSTs in the right and/or left eyes met the inclusion criteria. Those with concomitant ruptured globes, retinal detachments, and vitreous hemorrhages were excluded. A total of 123 patients with 135 HSTs were included in the final analysis. The primary outcome was the number of HSTs detected by UWF imaging. A secondary outcome was HST location. Sensitivity was measured with respect to HST location, and statistical significance was calculated by Fisher exact testing. RESULTS: A total of 69 (51.1%) HSTs were visualized on UWF images and 66 (48.9%) were not visualized. The sensitivity of UWF imaging in capturing HSTs was 7 of 41 (17.1%), 8 of 25 (32.0%), 7 of 14 (50.0%), and 47 of 55 (85.5%) for the superior, inferior, nasal, and temporal quadrants, respectively. Sensitivities between HST visibility and location were statistically significant (P < .001). CONCLUSIONS: Nearly half of HSTs were missed by UWF imaging. This study demonstrates that UWF imaging alone is not sufficiently sensitive to exclude the presence of HSTs.
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Purpose: Widespread electronic health record adoption has generated a large volume of data and emphasized the need for standardized terminology to describe clinical concepts. Here, we undertook a systematic concept coverage analysis to determine the representation of clinical concepts in ophthalmic infection and ophthalmic trauma among standardized medical terminologies, including the Systematized Nomenclature of Medicine Clinical Terms (SNOMED-CT), the International Classification of Diseases (ICD) version 10 with clinical modifications (ICD-10-CM), and ICD version 11 (ICD-11). Design: Extraction of concepts related to ophthalmic infection and ophthalmic trauma and structured search in terminology browsers. Data Sources: The American Academy of Ophthalmology Basic and Clinical Science Course (BCSC), SNOMED-CT, and ICD-10-CM terminologies from the Observational Health Data Sciences and Informatics Athena browser, and the ICD-11 terminology browser. Methods: Concepts pertaining to ophthalmic infection and ophthalmic trauma were extracted from the 2022 BCSC free text and index terms. We searched terminology browsers to identify corresponding codes and classified the extent of semantic alignment as equal, wide, narrow, or unmatched in each terminology. The overlap of equal concepts in each terminology was represented in a Venn diagram. Main Outcome Measures: Proportions of clinical concepts with corresponding codes at various levels of semantic alignment. Results: A total of 443 concepts were identified: 304 concepts related to ophthalmic infection and 139 concepts related to ophthalmic trauma. The SNOMED-CT had the highest proportion of equal coverage, with 82.0% (249 of 304) among concepts related to ophthalmic infection and 82.0% (115 of 139) among concepts related to ophthalmic trauma. Across all concepts, 28% (124 of 443) were classified as equal in ICD-10-CM and 52.8% (234 of 443) were classified as equal in ICD-11. Conclusions: The SNOMED-CT had significantly better semantic alignment than ICD-10-CM and ICD-11 for ophthalmic infections and ophthalmic trauma. This demonstrates opportunity for continuing advancement of representation of ophthalmic concepts in standardized medical terminologies.
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INTRODUCTION: The aim of this study was to investigate retinal layer thickness and vessel density differences between patients with reticular pseudodrusen (RPD) and intermediate dry age-related macular degeneration (iAMD). METHODS: Participants included in the study were patients diagnosed by retinal specialists with RPD, iAMD, and both RPD and iAMD at our academic referral center, seen from May 2021 until February 2022. The central 3 mm retinal thickness was measured using spectral-domain optical coherence tomography (Heidelberg Spectralis HRA+OCT System; Heidelberg Engineering, Heidelberg, Germany). Individual retinal thickness measurements were obtained from the innermost layer (nerve fiber layer) until the outermost layer (retinal pigment epithelium [RPE]). Each thickness measurement was subdivided into nine Early Treatment Diabetic Retinopathy Study (ETDRS) sectors. For the vessel density, OCT angiography from the Heidelberg Spectralis System was measured using proprietary third-party software (AngioTool; National Institutes of Health, National Cancer Institute, Bethesda, MD). Clinical and demographic characteristics were compared across the three groups (iAMD, RPD, iAMD and RPD) and analyzed with necessary adjustments. Linear mixed-effects models with necessary corrections were employed to compare continuous eye-level measurements between our three groups as well as in pairwise fashion using the R statistical programming software (R version 4.2.1). RESULTS: A total of 25 eyes of 17 patients with RPD, 20 eyes of 15 patients with iAMD, and 14 eyes of 9 patients with both iAMD and RPD were analyzed. Retinal thickness analysis identified that the superior inner (p = 0.028) and superior outer (p = 0.027) maculas of eyes with both iAMD and RPD were significantly thinner than those with iAMD alone. In eyes with RPD, the superior inner and superior outer RPE (p = 0.011 and p = 0.05, respectively), outer plexiform layer (p = 0.003 and p = 0.013, respectively), and inner nuclear layer (p = 0.034 and p = 0, respectively) were noted to be thinner compared to eyes with iAMD alone. In addition, the macular deep capillary plexus vessel density was significantly reduced in eyes with RPD compared to eyes with iAMD (p = 0.017). CONCLUSION: Patients with RPD had inner retinal structural as well as vascular changes compared to iAMD patients. Inner retinal vascular attenuation should be investigated further to see if there is a causal association with retinal thinning.
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Atrofia Geográfica , Degeneración Macular , Drusas Retinianas , Humanos , Coroides , Drusas Retinianas/diagnóstico , Retina , Degeneración Macular/diagnóstico , Tomografía de Coherencia Óptica/métodosRESUMEN
This study aimed to identify retinal vessel whitening outside the standard Early Treatment Diabetic Retinopathy Study (ETDRS) fields and to correlate the findings with vision and severity of diabetic retinopathy. Patients with diabetes mellitus who were seen in the retinal clinic to assess diabetic retinopathy status were included. Retinal vessel whitening was identified using ultra-widefield imaging. Four hundred and forty-five eyes of 260 patients were included. Thirty-five eyes in 24 patients (7.9%) were noted to have peripheral retinal vessel whitening. Thirty-one eyes with peripheral retinal vessel whitening did not have vessel whitening within the standard 7 ETDRS fields (p < 0.001). The proportion of whitening increased as DR severity increased, from 4.0% for patients with no DR (OR 0.249) to 33.3% for those with severe NPDR and PDR (OR 6.430 and 7.232, respectively). In addition, patients with peripheral retinal vessel whitening had worse visual acuity (logMAR = 0.34) compared to those without (logMAR = 0.15) (p < 0.001). In conclusion, we found an association between peripheral retinal vessel whitening in diabetic patients which correlated with diabetic retinopathy severity. Additionally, we found an association between vessel whitening and reduced vision, suggesting that vessel whitening identified using ultra-widefield imaging may be a prognostic indicator of vision in diabetic retinopathy.
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Diabetes Mellitus , Retinopatía Diabética , Humanos , Retina/diagnóstico por imagen , Vasos Retinianos/diagnóstico por imagen , Diagnóstico por Imagen/métodos , Arterias , Angiografía con Fluoresceína/métodosRESUMEN
PURPOSE: To refine the retinal phenotypes of suspected pentosan polysulfate sodium toxicity using ultra-widefield imaging. METHODS: Patients with complete dosing profiles who visited the ophthalmology department and with ultra-widefield and optical coherence tomography imaging records were identified using electronic health records at a large academic center. Retinal toxicity was initially identified using previously published imaging criteria, while grading was categorized using both previously reported and new classification systems. RESULTS: One hundred and four patients were included in this study. Twenty-six (25%) were identified as having toxicity from PPS. The mean duration of exposure and cumulative dose between the retinopathy group (162.7 months, 1,803.2 g) were longer and higher compared with the nonretinopathy group (69.7 months, 972.6 g) (both P < 0.001). There was variability of extramacular phenotype in the retinopathy group, with four eyes having only peripapillary involvement and six eyes having far peripheral extension. CONCLUSION: Retinal toxicity in the setting of prolonged exposure and increased cumulative dosing from PPS therapy produces phenotypic variability. Providers should be aware of the extramacular component of toxicity when screening patients. Understanding the different retinal phenotypes may prevent continued exposure and reduce the risk of vision-threatening foveal-involving disease.
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Poliéster Pentosan Sulfúrico , Enfermedades de la Retina , Humanos , Poliéster Pentosan Sulfúrico/efectos adversos , Angiografía con Fluoresceína/métodos , Retina , Enfermedades de la Retina/inducido químicamente , Enfermedades de la Retina/diagnóstico , Tomografía de Coherencia Óptica/métodos , FenotipoRESUMEN
This cross-sectional study aimed to investigate the hypothesis that permanent capillary damage may underlie the long-term COVID-19 sequela by quantifying the retinal vessel integrity. Participants were divided into three subgroups; Normal controls who had not been affected by COVID-19, mild COVID-19 cases who received out-patient care, and severe COVID-19 cases requiring intensive care unit (ICU) admission and respiratory support. Patients with systemic conditions that may affect the retinal vasculature before the diagnosis of COVID-19 infection were excluded. Participants underwent comprehensive ophthalmologic examination and retinal imaging obtained from Spectral-Domain Optical Coherence Tomography (SD-OCT), and vessel density using OCT Angiography. Sixty-one eyes from 31 individuals were studied. Retinal volume was significantly decreased in the outer 3 mm of the macula in the severe COVID-19 group (p = 0.02). Total retinal vessel density was significantly lower in the severe COVID-19 group compared to the normal and mild COVID-19 groups (p = 0.004 and 0.0057, respectively). The intermediate and deep capillary plexuses in the severe COVID-19 group were significantly lower compared to other groups (p < 0.05). Retinal tissue and microvascular loss may be a biomarker of COVID-19 severity. Further monitoring of the retina in COVID-19-recovered patients may help further understand the COVID-19 sequela.
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COVID-19 , Humanos , Angiografía con Fluoresceína/métodos , Estudios Transversales , Retina/diagnóstico por imagen , Vasos Retinianos/diagnóstico por imagen , Microvasos/diagnóstico por imagen , Tomografía de Coherencia Óptica/métodosRESUMEN
PURPOSE: To assess genetic associations for pentosan polysufate sodium maculopathy. METHODS: Genetic testing for inherited retinal dystrophy genes using exome testing and for 14 age-related macular degeneration-associated single nucleotide polymorphisms (SNPs) using panel testing were performed. In addition, full-field electroretinograms (ffERG) were obtained to identify any cone-rod dystrophy. RESULTS: Eleven of 15 patients were women, with a mean age of 69 (range 46-85). Inherited retinal dystrophy exome testing in five patients revealed six pathogenic variants, but failed to confirm inherited retinal dystrophy in any patient genetically. FfERG performed in 12 patients demonstrated only nonspecific a- and b-wave abnormalities in 11 cases and was normal in one case. For age-related macular degeneration single nucleotide polymorphisms, CFH rs3766405 ( P = 0.003) and CETP ( P = 0.027) were found to be statistically significantly associated with pentosan polysulfate maculopathy phenotype compared with the control population. CONCLUSION: Pentosan polysulfate maculopathy is not associated with Mendelian inherited retinal dystrophy genes. However, several age-related macular degeneration risk alleles were identified to be associated with maculopathy compared with their frequency in the normal population. This suggests a role for genes in disease pathology, particularly the alternative complement pathway. These findings would benefit from further investigation to understand the risk of developing maculopathy in taking pentosan polysulfate.
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Distrofias de Conos y Bastones , Cistitis Intersticial , Degeneración Macular , Distrofias Retinianas , Femenino , Masculino , Humanos , Poliéster Pentosan Sulfúrico/efectos adversos , Degeneración Macular/inducido químicamente , Degeneración Macular/diagnóstico , Degeneración Macular/genéticaRESUMEN
The Ultra-Wide-Field (UWF) retina images have attracted wide attentions in recent years in the study of retina. However, accurate registration between the UWF images and the other types of retina images could be challenging due to the distortion in the peripheral areas of an UWF image, which a 2D warping can not handle. In this paper, we propose a novel 3D distortion correction method which sets up a 3D projection model and optimizes a dense 3D retina mesh to correct the distortion in the UWF image. The corrected UWF image can then be accurately aligned to the target image using 2D alignment methods. The experimental results show that our proposed method outperforms the state-of-the-art method by 30%.