Simultaneous Intravitreal Steroid and Anti-VEGF Therapy for Monotherapy-Resistant Chronic Wet Age-Related Macular Degeneration.

BACKGROUND AND OBJECTIVE: The purpose of this study was to analyze the safety and efficacy of combined intravitreal anti-vascular endothelial growth factor (anti-VEGF) and steroid therapeutic agents for resistant choroidal neovascularization (CNV). A retrospective observational clinical study was performed assessing anatomic and visual changes in a consecutive cohort of patients with refractory chronic wet age-related macular degeneration resistant to high-dose aflibercept therapy. PATIENTS AND METHODS: Twelve eyes of 12 patients with unresponsive CNV despite aggressive monthly anti-VEGF (4-mg aflibercept [mean: 43.75 ± SD23.08]) were included. Combination consisted of simultaneous administration of anti-VEGF and corticosteroids. Study measures evaluated visual acuity, central retinal thickness (CRT), and intraocular pressure. RESULTS: Paired tests revealed significant CRT reduction from the baseline at the 1-month (388.58 ± 89.31 versus 334.00 ± 92.88, P = 0.0117), 2-month (388.58 ± 89.31 versus 312.08 ± 75.61, P = 0.0185), and 3-month (388.53 ± 89.31 versus 304.56 ± 53.28, P = 0.046) visit. The Kaplan-Meier curve showed a median time of remission (no retinal fluid) of 70 days (95% CI 53, 147 days). CONCLUSION: Combination treatment demonstrated clear anatomic improvement in eyes with anti-VEGF-resistant CNV. [Ophthalmic Surg Lasers Imaging Retina 2024;55:XX-XX.].

Ethical considerations for large language models in ophthalmology.

PURPOSE OF REVIEW: This review aims to summarize and discuss the ethical considerations regarding large language model (LLM) use in the field of ophthalmology. RECENT FINDINGS: This review of 47 articles on LLM applications in ophthalmology highlights their diverse potential uses, including education, research, clinical decision support, and surgical assistance (as an aid in operative notes). We also review ethical considerations such as the inability of LLMs to interpret data accurately, the risk of promoting controversial or harmful recommendations, and breaches of data privacy. These concerns imply the need for cautious integration of artificial intelligence in healthcare, emphasizing human oversight, transparency, and accountability to mitigate risks and uphold ethical standards. SUMMARY: The integration of LLMs in ophthalmology offers potential advantages such as aiding in clinical decision support and facilitating medical education through their ability to process queries and analyze ophthalmic imaging and clinical cases. However, their utilization also raises ethical concerns regarding data privacy, potential misinformation, and biases inherent in the datasets used. Awareness of these concerns should be addressed in order to optimize its utility in the healthcare setting. More importantly, promoting responsible and careful use by consumers should be practiced.

Using Multimodal Imaging to Refine the Phenotype of PRPH2-associated Retinal Degeneration.

OBJECTIVE: To refine retinal peripherin-2 (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 2 tertiary referral eye centers with molecularly confirmed IRD due to PRPH2 variants. METHODS: Retinal imaging was reviewed using ultrawidefield (UWF) pseudocolor, UWF fundus autofluorescence, and spectral-domain 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 2 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 (89/144, 61.8%), whereas 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, whereas 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 (MFD), and n = 15 with butterfly pattern dystrophy and MFD. 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%). CONCLUSIONS: Peripherin-2-associated retinal degeneration 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. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Differences in macular thickness associated with peripheral retinal vessel whitening in diabetic patients.

This study aimed to determine the difference in macular thickness among patients with diabetes mellitus (DM) with and without peripheral retinal vessel whitening (PRVW). PRVW was defined by retinal vessel whitening outside the standard seven ETDRS fields. Subjects were divided into DM with PRVW, DM without PRVW, and normal age-matched controls. Optical coherence tomography scans were divided into total, inner, and outer retinal layer thicknesses and were compared in the macula's central, inner, and outer rings. Forty-seven eyes were included: DM with PRVW = 15, DM without PRVW = 16, and Controls = 16. Overall, the mean retinal thickness in patients with DM with PRVW was lower than in patients with DM without PRVW and controls. In the inner macula, DM patients with PRVW showed a significantly lower mean inner superior, nasal, inferior, and temporal macula compared to DM patients without PRVW (p = 0.014, 0.008, 0.005, < 0.001, respectively). DM patients with PRVW also showed a significantly lower mean outer superior, nasal, inferior, and temporal macula than controls (p = 0.005, 0.005, 0.016, 0.025, respectively). This study demonstrates that PRVW in DM patients may be associated with global structural changes to the macular region, promoting a decrease in inner and outer retinal thickness. Further studies should investigate the functional correlation with PRVW in DM patients in order to better understand its potential implications in diabetic patients.

Evaluation of Retinal Arterial Occlusion and its Visual and Systemic Prognosis after Hyperbaric Oxygen Therapy.

PURPOSE: To evaluate the systemic and ocular outcomes of patients with branch retinal artery occlusion (BRAO) and central retinal artery occlusion (CRAO) after hyperbaric oxygen therapy (HBOT). METHODS: This is a single-institution study of 75 subjects diagnosed with BRAO (28, 37.3%) and CRAO (47, 62.7%) who visited the emergency department or stroke clinic. Twenty-seven (36%) subjects received HBOT on initial presentation (BRAO-14.3%, CRAO-48.9%). The primary outcome was the best corrective visual acuity (BCVA) change in non-HBOT and HBOT subjects. Secondary outcomes included subsequent development of an acute cerebrovascular accident (CVA)/stroke or neovascular glaucoma (NVG). RESULTS: Overall BCVA did not change from the initial presentation to the final timepoint (logMAR 1.5) in either the conservative management or HBOT cohorts for either BRAO subjects (non-HBOT-logMAR 0.4 vs. 0.6, p=0.658; HBOT-logMAR 0.1 vs. 0.4, p=0.207) or CRAO subjects (non-HBOT-logMAR 2.1 vs. 2.2, p=0.755; HBOT-logMAR 2.1 vs. 2.0, p=0.631). Seven (9.3%) subjects developed CVA (BRAO: non-HBOT-4.2% and HBOT-25.0%, p=0.207; CRAO: non-HBOT-16.7% and HBOT-4.3%, p=0.348) and five subjects (6.7%) developed NVG (BRAO: non-HBOT-4.2% and HBOT-0%, p=1.00; CRAO: non-HBOT-16.7% and HBOT-0%, p=0.109). CONCLUSIONS: Our findings suggest that HBOT does not significantly improve BCVA or mitigate the subsequent development of stroke and NVG in patients with RAOs.

Retinal structural changes after cessation of pentosan polysulfate sodium.

PURPOSE: To quantify baseline and longitudinal structural changes post-cessation in pentosan polysulfate sodium (PPS) retinopathy patients. METHODS: This is a retrospective cohort study. Retinal thickness and volume of choroidal and hyperreflective retinal pigment epithelium (RPE) excrescences were manually segmented from optical coherence tomography (OCT) volume scans. Baseline measurements were compared against age-matched controls. Longitudinal measurements were performed on patients with follow-up data. RESULTS: Twenty-four eyes of 13 patients were included. At baseline, the mean total retinal thickness was lower in the PPS retinopathy cohort than in age and sex-matched controls (269.1 µm vs. 290.2 µm, p = 0.006).The median (range) of follow-up was 18.6 (4.1 to 34.7) months, with the mean last follow-up of 35.2 months after cessation. During the follow-up period, the thickness of the retina decreased significantly by 11.3 µm (CI: 16.8, 5.8) (p<0.001), with an annual mean decrease of 6.70 µm. However, the mean hyperreflective RPE excrescence volume did not change significantly (p = 0.140) over the follow-up period. CONCLUSIONS: Following PPS discontinuation, although RPE excrescence volumes do not change significantly in volume, there continues to be a progressive long-term thinning of the retina which continues at a rate greater than that associated with normal aging. Consequently, long-term follow-up is suggested to monitor patients with PPS maculopathy.

Developing an image-based grading scale for peripheral drusen to investigate associations of peripheral drusen type with age-related macular degeneration.

Age-related macular degeneration (AMD) is a leading cause of blindness. It is associated with peripheral drusen which has not been categorized. We investigated peripheral drusen to validate an image grading system and to understand possible associations between peripheral drusen and AMD. We collated clinical data, ultra-widefield (UWF) pseudocolor fundus images and Spectral-Domain Optical Coherence Tomography (SD-OCT) scans from consecutive retinal patients. SD-OCT scans were used to determine AMD stage. A masked retinal specialist recorded the types of peripheral drusen observed in UWF images. Eyes whose UWF images did not pass quality screening and those without AMD and peripheral drusen were excluded from the study. Statistical tests were utilized to determine the validity of our grading system and associations of peripheral drusen with AMD. A total of 481 eyes (283 subjects) were included in the study (mean age 73.1 ± 1.2years, 64.3% female). Interobserver and test-retest statistical analyses to evaluate the UWF image grading system resulted in Cohen's Kappa 0.649 (p < 0.001) and 0.922 (p < 0.001) respectively. A total of 284 (59.0%), 28 (5.8%), 15 (3.1%), 22 (4.6%), 4 (0.8%), 39 (8.1%), and 32 (6.7%) eyes had hard, soft, reticular, cuticular, atrophic, mixed drusen, and mixed drusen and atrophy respectively in at least one peripheral retinal quadrant. Hard peripheral drusen was significantly associated with the presence of AMD (p = 0.010). Peripheral drusen types were variably seen in retinal patients with and without AMD. We validated a peripheral drusen grading system and provided an image library to assist in the identification of peripheral drusen. Our study found an association between peripheral hard drusen and an AMD diagnosis but did not find a link between peripheral drusen and severity of AMD.

Frequency and determinants of phytotherapy use in patients with type 2 diabetes in the Dschang Health District, Cameroon: a cross-sectional study.

Introduction: phytotherapy is widely used in Africa for the management of many diseases. Data on the use of phytotherapy in people with type 2 diabetes are scarce. We aimed to determine the frequency and factors associated with the consumption/use of phytotherapy products among patients with type 2 diabetes in the Dschang Health District. Methods: we conducted a cross-sectional study from January to May 2022, including community-dwelling or hospitalized patients with type 2 diabetes who had lived in the Dschang Health District for at least one year. Informed consent was obtained from all patients. Data were collected using a pre-designed questionnaire. Variables collected included socio-demographic characteristics, diabetes knowledge and practices, and perceptions of care. Results: we included 403 (249 women) patients with type 2 diabetes with a mean (SD) age of 63 (± 14.86) years). Among them, 240 (59.55%) used phytotherapy, either in combination with conventional treatment (168 (41.69%) participants) or not (72 (17.86%) participants), to treat diabetes. The most common reasons for using phytotherapy were easy accessibility and belief in its efficacy. Most patients used both treatments because they thought the combination was more effective. In univariable analysis, we observed a statistically significant association between level of education (p=0.003), socioeconomic level (p<0.001), place of residence (p=0.003), duration of diabetes (p=0.007), and use of phytotherapy. In multivariable analysis, only age between 51 and 60 years (OR: 0.50, 95% CI 0.298 - 0.8521; p=0.01) was associated with the use of phytotherapy. Conclusion: people living with T2D in the Dschang Health District frequently use phytotherapy as an antidiabetic remedy, especially those aged between 51 and 60 years, those with low education level, low socioeconomic level and medium duration of diabetes. There is a need to evaluate its effectiveness in treating diabetes and its adverse effects.

Effect of manual OCTA segmentation correction to improve image quality and visibility of choroidal neovascularization in AMD.

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%.

Robust and heritable knockdown of gene expression using a self-cleaving ribozyme in Drosophila.

The current toolkit for genetic manipulation in the model animal Drosophila melanogaster is extensive and versatile but not without its limitations. Here, we report a powerful and heritable method to knockdown gene expression in D. melanogaster using the self-cleaving N79 hammerhead ribozyme, a modification of a naturally occurring ribozyme found in the parasite Schistosoma mansoni. A 111-bp ribozyme cassette, consisting of the N79 ribozyme surrounded by insulating spacer sequences, was inserted into 4 independent long noncoding RNA genes as well as the male-specific splice variant of doublesex using scarless CRISPR/Cas9-mediated genome editing. Ribozyme-induced RNA cleavage resulted in robust destruction of 3' fragments typically exceeding 90%. Single molecule RNA fluorescence in situ hybridization results suggest that cleavage and destruction can even occur for nascent transcribing RNAs. Knockdown was highly specific to the targeted RNA, with no adverse effects observed in neighboring genes or the other splice variants. To control for potential effects produced by the simple insertion of 111 nucleotides into genes, we tested multiple catalytically inactive ribozyme variants and found that a variant with scrambled N79 sequence best recapitulated natural RNA levels. Thus, self-cleaving ribozymes offer a novel approach for powerful gene knockdown in Drosophila, with potential applications for the study of noncoding RNAs, nuclear-localized RNAs, and specific splice variants of protein-coding genes.

Variations in Electronic Health Record-Based Definitions of Diabetic Retinopathy Cohorts: A Literature Review and Quantitative Analysis.

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.

ARTIFICIAL INTELLIGENCE FOR OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY-BASED DISEASE ACTIVITY PREDICTION IN AGE-RELATED MACULAR DEGENERATION.

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.

Syndromic retinitis pigmentosa caused by biallelic SCAPER frameshift variant.

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.

Ultra-wide field and new wide field composite retinal image registration with AI-enabled pipeline and 3D distortion correction algorithm.

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.

Prevalence of peripheral retinal findings in retinal patients using ultra-widefield pseudocolor fundus imaging.

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.

Evaluation of Publications from the American Academy of Ophthalmology: A 5-Year Analysis of Ophthalmology Literature.

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.

Trends and practices following the 2016 hydroxychloroquine screening guidelines.

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.

An unusual case of rapid resolution of bilateral vitelliform deposits after discontinuation of pentosan polysulfate sodium.

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.

Investigating the associations of macular edema in retinitis pigmentosa.

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.