September 2023

A healthy 32-year-old woman presented with the acute onset of left sided eye pain, upper eyelid fullness, and binocular diplopia during light weightlifting. Examination elevated intraocular pressure, proptosis, upper eyelid ptosis, and motility deficits. CT demonstrated a well-circumscribed, homogeneous-appearing extraconal mass in the superior left orbit. The patient underwent an urgent orbitotomy with the excision of a hemorrhagic mass. Histopathology showed a glomus tumor with atypical features and hemorrhagic infarction, best classified as having uncertain malignant potential. A B-Raf proto-oncogene V600E mutation was detected with immunohistochemistry, which suggests a more aggressive tumor behavior yet presents an opportunity for targeted primary or adjunctive therapy. This is the first reported case of a B-Raf proto-oncogene-mutant atypical glomus tumor arising in the orbit.

The pervasiveness of mobile devices and other associated technologies has affected all aspects of our daily lives. People with visual impairments are no exception, as they increasingly tend to rely on mobile apps for assistance with various visual tasks in daily life. Compared to dedicated visual aids, mobile apps offer advantages such as affordability, versatility, portability, and ubiquity. We have surveyed hundreds of mobile apps of potential interest to people with vision impairments, either released as special assistive apps claiming to help in tasks such as text or object recognition (n = 68), digital accessibility (n = 84), navigation (n = 44), and remote sighted service (n = 4), among others, or marketed as general camera magnification apps that can be used for visual assistance (n = 77). While assistive apps as a whole received positive feedback from visually impaired users, as reported in various studies, evaluations of the usability of every app were typically limited to user reviews, which are often not scientifically informative. Rigorous evaluation studies on the effect of vision assistance apps on daily task performance and quality of life are relatively rare. Moreover, evaluation criteria are difficult to establish, given the heterogeneity of the visual tasks and visual needs of the users. In addition to surveying literature on vision assistance apps, this review discusses the feasibility and necessity of conducting scientific research to understand visual needs and methods to evaluate real-world benefits.

Astrocytes are a major category of glial support cell in the central nervous system and play a variety of essential roles in both health and disease. As our understanding of the diverse functions of these cells improves, the extent of heterogeneity between astrocyte populations has emerged as a key area of research. Retinal astrocytes, which form the direct cellular environment of retinal ganglion cells somas and axons, undergo a reactive response in both human glaucoma and animal models of the disease, yet their contributions to its pathology and progression remain relatively unknown. This gap in knowledge is largely a function of inadequate isolation techniques, driven in part by the sparseness of these cells and their similarities with the more abundant retinal Müller cells. Here, we present a novel method of isolating retinal astrocytes and enriching their RNA, tested in both normal and ocular hypertensive mice, a common model of experimental glaucoma. Our approach combines a novel enzyme assisted microdissection of retinal astrocytes with selective ribosome immunoprecipitation using the Ribotag method. Our microdissection method is rapid and preserves astrocyte morphology, resulting in a brief post-mortem interval and minimizing loss of RNA from distal regions of these cells. Both microdissection and Ribotag immunoprecipitation require a minimum of specialized equipment or reagents, and by using them in conjunction we are able to achieve > 100-fold enrichment of astrocyte RNA.

BACKGROUND/AIMS: Trachomatous trichiasis (TT) is a severe consequence of chronic inflammation/conjunctival scarring resulting from trachoma, the leading infectious cause of blindness worldwide. Our prospective cohort study evaluated the effectiveness of refresher training (RT) for experienced surgeons (1-22 years) on the outcomes of upper lid (UL) TT surgery in rural Ethiopia. METHODS: Patients undergoing UL TT surgery in at least one eye by a participating surgeon were included. Patients were split into two cohorts: patients enrolled prior to (C1) and after (C2) RT. RT consisted of a 1-week programme with practice on a HEAD START mannequin and supportive supervision in live surgery by expert trainers. Data were collected at preoperative enrolment, and at 6-month and 12-month follow-up visits. The primary outcome was development of postoperative TT (PTT). A series of multivariate generalised estimating equations were fit to model PTT involving potential covariates of interest. RESULTS: A total of 261 eyes contributed by 173 patients were studied between 2017 and 2019. By 1-year postoperatively, 37/128 eyes (28.9%) in C1 and 22/133 eyes (16.5%) in C2 had developed PTT (p=0.03). Other than surgeon RT participation, no factors studied were associated with differences in PTT. CONCLUSION: Our results indicate a significant reduction in the risk of PTT after experienced surgeons' participation in RT as compared with eyes receiving surgery before RT. This observation suggests a significant potential benefit of the RT with HEAD START mannequin practice and supportive supervision during surgery, and suggests RT may be a valuable strategy to improve surgical outcomes.

PURPOSE: To assess the rates of postoperative steroid response following dropless cataract surgery using a subconjunctival depot of triamcinolone versus conventional cataract surgery using topical prednisolone. PATIENTS AND METHODS: We reviewed consecutive cataract surgery cases performed by a single surgeon to determine the likelihood of steroid response, defined as intraocular pressure (IOP) 50% above baseline or IOP > 24 mmHg postoperatively, excluding the first 72 hours. Logistic regression models were performed including baseline characteristics as exposures in the model and steroid response as the outcome. Main outcome measures were the proportion of eyes developing steroid response, risk factors for developing steroid response, and duration of steroid response. RESULTS: Of the 150 dropless and 218 conventional cases, 26 eyes developed steroid response (15 dropless and 11 conventional cases [10% vs 5%, P=0.096]). Risk factors for steroid response included dropless surgery (OR=2.43, 95% CI=1.03-6.02], P=0.046) and prior diagnosis of glaucoma (OR=7.18, 95% CI=2.66-19.22], P<0.001). Baseline IOP, age, sex, race, and axial length did not increase risk for steroid response. Of the eyes with steroid response, more dropless cases had an IOP elevation ≥30 days (9/15 eyes vs 1/11 eyes; P=0.008), including one patient with refractory IOP elevation in the dropless group who required urgent bilateral trabeculectomy for IOP control. CONCLUSION: Dropless cataract surgery increases the risk of prolonged steroid response postoperatively. Patients with glaucoma have an increased risk of steroid response and may not be good candidates for dropless cataract surgery with subconjunctival triamcinolone.

Objective: Visual acuity (VA) testing is crucial for early intervention in cases of visual impairment, especially in rural health care. This study aimed to determine the potential of a web-based VA test (PocDoc) in addressing the unique health care needs of rural areas through the comparison in its effectiveness against the conventional VA test in identifying visual impairment among an Indian rural population. Methods: Prospective comparative study conducted in December 2022 at a tertiary referral eye care center in central India. We evaluated all patients with the PocDoc VA tests using three device types, and the conventional VA test. Bland-Altman plot (BAP) compared PocDoc and conventional VA tests. Fisher's exact tests evaluated associations between categorical parameters. Kruskal-Wallis tests followed by post hoc Dunn's tests identified association between categorical parameters and numerical parameters. Results: We evaluated 428 patients (792 measurements of VA) with mean age 36.7 (±23.3) years. PocDoc resulted in slightly worse VA scores (mean logMAR: 0.345) than conventional (mean logMAR: 0.315). Correlation coefficient between the conventional and PocDoc logMAR VA values was rho = 0.845 and rho2 = 0.7133 (p = 6.617 × 10-215; adjusted p = 2.205 × 10-214). Most data points fell within the interchangeable range of ±0.32 on BAP. Difference between the two methods increased with higher logMAR values, indicating poorer agreement for worse VA scores. Conclusions: Identifying and addressing the unique health care needs of rural populations is critical, including access to appropriate and effective VA testing methods. Validating and improving VA testing methods can ensure early intervention and improve the quality of life for individuals with visual impairment.

PURPOSE: To test the hypothesis that a simple model having properties consistent with activation and deactivation in the rod approximates the whole time course of the photoresponse. METHODS: Routinely, an exponential of the form f = α·(1 - exp(-(τ·(t - teff)s-1))), with amplitude α, rate constant τ (often scaled by intensity), irreducible delay teff, and time exponent s-1, is fit to the early period of the flash electroretinogram. Notably, s (an integer) represents the three integrating stages in the rod amplification cascade (rhodopsin isomerization, transducin activation, and cGMP hydrolysis). The time course of the photoresponse to a 0.17 cd·s·m-2 conditioning flash (CF) was determined in 21 healthy eyes by presenting the CF plus a bright probe flash (PF) in tandem, separated by interstimulus intervals (ISIs) of 0.01 to 1.4 seconds, and calculating the proportion of the PF a-wave suppressed by the CF at each ISI. To test if similar kinetics describe deactivation, difference of exponential (DoE) functions with common α and teff parameters, respective rate constants for the initiation (I) and quenching (Q) phases of the response, and specified values of s (sI, sQ), were compared to the photoresponse time course. RESULTS: As hypothesized, the optimal values of sI and sQ were 3 and 2, respectively. Mean ± SD α was 0.80 ± 0.066, I was 7700 ± 2400 m2·cd-1·s-3, and Q was 1.4 ± 0.47 s-1. Overall, r2 was 0.93. CONCLUSIONS: A method, including a DoE model with just three free parameters (α, I, Q), that robustly captures the magnitude and time-constants of the complete rod response, was produced. Only two steps integrate to quench the rod photoresponse.

BACKGROUND: A cross-sectional study was conducted to assess the comparative effectiveness of virtual visits for preoperative evaluation and surgical decision-making in three pediatric surgical subspecialties. METHODS: Patients who underwent surgical procedures in the departments of Urology, Ophthalmology, and Plastic and Oral Surgery at a tertiary care pediatric hospital over a one-year period during the COVID-19 pandemic were included. Patients were assigned to one of three clinical pathways based on their preoperative visit(s): only in-person visit(s) (IP), a combination of in-person and virtual visit(s) (IP/VV), and only virtual visit(s) (VV). Demographics, procedure information, and patient experience survey results were collected. We then assessed variations in procedure types and patient experience scores in these three patient groups. RESULTS: There were 431 patients who completed the modified patient experience survey. The most common procedures were circumcision (17%), excision of lesion (16%), and strabismus repair (11%). Survey results were positive, with 90% of participants rating that they would recommend the service to others. No significant differences were found among groups in their demographics, overall care rating, and duration between preoperative clinic visit and procedure. Post-hoc power analysis indicated 87% power to detect a 10% difference in survey ratings between IP and VV cases, confirming non-inferiority in patient satisfaction for virtual preoperative visits. CONCLUSION: This study demonstrated the non-inferiority of preoperative virtual visits in three pediatric surgical subspecialties as measured by patient experience scores. Additional studies with more granular scope are necessary to further elucidate telemedicine's safety and efficacy for select diagnoses. LEVEL OF EVIDENCE: III.

PURPOSE: To assess the validity of visual field (VF) results from the Iowa Head-Mounted Display (HMD) Open-Source Perimeter and to test the hypothesis that VF defects and test-retest repeatability are similar between the HMD and Octopus 900 perimeters. METHODS: We tested 20 healthy and nine glaucoma patients on the HMD and Octopus 900 perimeters using the Open Perimetry Interface platform with size V stimuli, a custom grid spanning the central 26° of the VF, and a ZEST thresholding algorithm. Historical data from the Humphrey Field Analyzer (HFA) were also analyzed. Repeatability was analyzed with the repeatability coefficient (RC), and VF defect detection was determined through side-by-side comparisons. RESULTS: The pointwise RCs were 2.6 dB and 3.4 dB for the HMD and Octopus 900 perimeters in ocular healthy subjects, respectively. Likewise, the RCs were 4.2 dB and 3.5 dB, respectively, in glaucomatous patients. Limits of agreement between the HMD and Octopus 900 perimeters were ±4.6 dB (mean difference, 0.4 dB) for healthy patients and ±8.9 dB (mean difference, 0.1 dB) for glaucomatous patients. Retrospective analysis showed that pointwise RCs on the HFA2 perimeter were between 3.4 and 3.7 dB for healthy patients and between 3.9 and 4.7 dB for glaucoma patients. VF defects were similar between the HMD and Octopus 900 for glaucoma subjects. CONCLUSIONS: The Iowa Virtual Reality HMD Open-Source Perimeter is as repeatable as the Octopus 900 perimeter and is a more portable and less expensive alternative than traditional perimeters. TRANSLATIONAL RELEVANCE: This study demonstrates the validity of the visual field results from the Iowa HMD Open-Source Perimeter which may help expand perimetry access.

PURPOSE: Determine sensitivity, specificity, and cut-off of macular ganglion cell layer (GCL) volume consistent with optic atrophy in children with syndromic craniosynostosis (CS). Investigate whether obstructive sleep apnea (OSA), Chiari malformation, history of elevated intracranial pressure (ICP), CS diagnosis, age, or sex independently alter GCL volume with CS. DESIGN: Retrospective cross-sectional study. SUBJECTS: Patients with syndromic CS evaluated at Boston Children's Hospital (2010 - 2022) with reliable macular optical coherence tomography (OCT) scans. METHODS: Latest ophthalmic examination that included OCT macula scans was identified. Age at examination, sex, ethnicity, best-corrected logMAR visual acuity, cycloplegic refraction, and funduscopic optic nerve appearance were recorded in addition to history of primary or recurrent elevated ICP, Chiari malformation and OSA. Spectral domain-OCT software quantified segmentation of macula retinal layers, and was manually checked. MAIN OUTCOME MEASURES: Primary outcome was determining sensitivity, specificity and optimal cutoff of GCL volume consistent with optic atrophy. Secondary outcome was determining possible independent association of previously elevated ICP, OSA, Chiari, CS diagnosis, logMAR acuity, age, or sex with altered GCL volume. RESULTS: Median age at examination was 11.9 years (interquartile range (IQR), 8.5-14.8 years). Fifty-eight of 61 patients had reliable macula scans, 74% were female, and diagnoses were Apert (n=14); Crouzon (n=17); Muenke (n=6); Pfeiffer (n= 6); and Saethre-Chotzen (n=15). Optimal cutoff identifying optic atrophy was GCL volume <1.02mm3 with a sensitivity of 83% and specificity of 77%. Univariate analysis demonstrated significantly lower macular GCL volume with optic atrophy on fundus exam (P<0.001), Apert syndrome (P<0.001), history of elevated ICP (P=0.015), Chiari malformation (P=0.001), OSA (P<0.001), in males (P=0.027) and with worse logMAR acuity (-0.36, P<0.001). Multivariable median regression analysis confirmed that only OSA (P=0.005), optic atrophy on fundus exam (P=0.003), and worse logMAR acuity (P=0.042) independently associated with lower GCL volume. CONCLUSIONS: Macular GCL volume <1.02mm3 predicted optic atrophy in patients with CS with sensitive of 83% and specificity of 77%. OSA, a treatable often concomitant disorder, was independently associated with lower GCL volume. Surveillance for optic neuropathy by GCL volume proved effective in a population where cognitive skills can limit acquisition of other key ophthalmic measures.

PURPOSE: We describe the unusual clinical presentation of a 33-year-old woman subsequently identified as a carrier of RP2-associated X-linked retinitis pigmentosa. METHODS: Case report. RESULTS: A 33-year-old woman without a known family history of retinal disease presented with unilateral reduced visual acuity and central scotoma in the left eye. Examination showed underlying macular atrophy in the left eye and a bilateral tapetal-like reflex. Full-field electroretinogram was abnormal in the left eye but normal in the right eye. Notable findings on wide-field imaging included bilateral peripheral vascular leakage on fluorescein angiography and a bilaterally symmetric radial pattern of hyperfluorescence on fundus autofluorescence. Genetic testing demonstrated a pathogenic variant in the gene RP2 confirming that she was a carrier of X-linked retinitis pigmentosa. CONCLUSION: We describe clinical features of the carrier state of RP2-XLRP and expand potential findings to include peripheral vascular leakage. This case highlights the importance of awareness of the carrier state, particularly if a family history cannot be provided.

OBJECTIVES: To describe trends in vision screening based on insurance claims for young children in the United States. METHODS: This cross-sectional study used administrative claims data from the 2010-2019 IBM MarketScan Commercial Claims and Encounters Database. We included children aged 1 to <5 years at the beginning of each calendar year. The primary outcome was a vision screening claim within 12 months for chart-based or instrument-based screening. Linear regression was used to evaluate trends over time in vision screening claims and practitioner payment. RESULTS: This study included a median of 810 048 (interquartile range, 631 523 - 1 029 481) children between 2010 and 2019 (mean [standard deviation] age, 2.5 [1.1] years; 48.7% female). The percentage of children with vision screening claims increased from 16.7% in 2010 to 44.3% in 2019 (difference, 27.5%; 95% confidence interval, 27.4% to 27.7%). Instrument-based screening claims, which were identified in <0.2% of children in 2010, increased to 23.4% of children 1 to <3 years old and 14.4% of children 3 to <5 years old by 2019. From 2013 to 2018, the average of the median practitioner payment for instrument-based screening was $23.70, decreasing $2.10 per year during this time (95% confidence interval, $0.85 to $3.34; P = .009). CONCLUSIONS: Vision screening claims among young children nearly tripled over the last decade, and this change was driven by increased instrument-based screening for children aged <3 years. Further investigation is needed to determine whether the decreasing trends in practitioner payment for screening devices will reduce the adoption of vision screening technology in clinical practice.

PURPOSE: As a potentially sight-threatening disease with ocular, systemic, and treatment-related complications, uveitis diminishes quality of life (QOL) and affects psychosocial well-being. This review summarizes the existing tools for evaluating psychosocial well-being and/or QOL in patients with uveitis, explores the biological and non-biological factors affecting psychosocial well-being and/or QOL, and proposes future directions for incorporating these tools into clinical practice. METHODS: A systematic search of the MEDLINE, Embase, and Cochrane databases from inception to June 8, 2022 was conducted, screening for articles focused on psychosocial well-being and/or QOL in patients with uveitis. Both quantitative and qualitative analyses were performed. RESULTS: In uveitis research, the most frequently studied patient-reported outcome measures were vision-related QOL (e.g. Visual Function Questionnaire [VFQ-25]) and health-related QOL (e.g. Short Form Survey [SF-36]), followed by mental health indicators including depression and anxiety. Instruments have also been developed specific to the pediatric population (e.g. Effects of Youngsters' Eyesight on Quality of Life [EYE-Q]). Generally, studies report worse psychosocial outcomes and QOL in patients with uveitis compared to the general population. Contributory factors include both clinical (e.g. visual impairment, ocular comorbidities) and patient-related (e.g. older age, female sex) factors. CONCLUSION: Given the heterogeneity of instruments used, it is worth considering standardization across large uveitis studies and trials. Beyond research, given the biopsychosocial effects on patients with uveitis, there are benefits to incorporating QOL and psychosocial assessments into clinical practice. Simplification of questionnaires into abridged forms, focusing on the most clinically relevant aspects of patient care, may be considered.