Erdheim-Chester Disease With Eyelid and Orbital Involvement: A Review of Treatment Modalities at One Institution From 2014 to 2022.

PURPOSE: To review all cases of Erdheim-Chester disease (ECD) with orbital involvement treated at Bascom Palmer Eye Institute in Miami, Florida from 2014 to 2022 and compare presentations, treatment modalities, and outcomes. METHODS: A retrospective chart review of all patients diagnosed with ECD who presented to Bascom Palmer Eye Institute from 2014 to 2022 was performed. Data collected included demographics, pretreatment history and ophthalmic examination, pathology report, treatment, subsequent examination, and relevant laboratory results. Histopathology, treatments, and outcomes were reviewed and compared between patients. RESULTS: Four cases were included. Primary treatments included vemurafenib (n = 2), cobimetinib (n = 1), and prednisone (n = 1). All patients demonstrated improvement of ophthalmic symptoms. Vemurafenib was the only medical treatment that was tolerated well and resulted in significant improvement in proptosis despite some reported dry eye; all other medications were discontinued due to intolerable side effects. CONCLUSIONS: BRAF inhibitors such as vemurafenib have been used as novel therapy in the treatment of ECD. Vemurafenib demonstrated its utility in reducing proptosis in ECD patients at one ophthalmic institution. Vemurafenib may be a favorable treatment option for BRAF-positive ECD patients presenting with orbital disease.

Vision Screenings and Ophthalmology Referrals Among a Sample of Pediatricians in Florida.

Introduction In Florida, mandated school vision screenings begin around the age of five. However, a joint statement in the ophthalmology community recommends that primary care providers, including pediatricians, screen for eye and vision symptoms and signs starting at birth. This suggests that pediatricians may be the first to catch signs of early vision loss and refer pediatric patients to an ophthalmologist. This study aims to understand the current vision screening practices of a sample of pediatricians in Florida and how comfort levels with vision screenings may impact ophthalmology referrals. Methods A survey with 36 questions was created by the authors of the study and sent to pediatricians through digital newsletters to the Florida Chapter of the American Academy of Pediatrics and pediatric departments at Florida universities. Descriptive statistics were gathered regarding the demographics of pediatricians surveyed, vision screening attitudes and practices, vision concerns and ophthalmology referrals from pediatric clinics, and the most common reasons for referral to an ophthalmologist. The Jonckheere-Terpstra nonparametric trend test was used to examine whether decreased comfort performing vision screening on a child was associated with lower rates of urgent referrals to an optometrist or ophthalmologist. Results Forty-six responses were collected. Seventy-eight percent of pediatricians reported performing early childhood vision screening (n=36). There was considerable variability in vision screening practices among the pediatricians studied, with only 66% beginning screenings from zero to two years of age (n=24). Fifty percent of respondents reported receiving no previous training on performing vision screening, and less than half of respondents reported feeling "somewhat comfortable" or "extremely comfortable" with performing the exam (n=22, 48%). The trend between decreased comfort performing pediatric vision screening and lower rates of urgent eye care referrals was approaching statistical significance (p=0.0705). The majority of urgent referrals were provided by respondents who were somewhat or extremely comfortable with screening (n=13, 65%). Conclusion From this sample of pediatricians in Florida, most respondents reported performing early childhood vision screening, but there was notable variability in the way screenings were performed among pediatricians. Moreover, many had never received training on performing the exam or did not feel comfortable performing them. Decreased comfort with vision screenings was almost significantly associated with decreased urgent referrals to an ophthalmologist. Future studies should examine whether increased training on vision screenings may help improve standardization of screening practices among pediatrics and comfort with vision screenings.

Compliance with web content accessibility guidelines in ophthalmology social media posts.

This is a cross-sectional exploratory analysis of publicly available Internet data to examine compliance to web content accessibility guidelines (WCAG) on patient education social media posts in ophthalmology. WCAG ensures web content accessibility for those with disabilities (including visual impairment). A total of 100 social media posts were sampled from ten ophthalmology patient education social media pages and ten non-ophthalmology (cardiopulmonary) pages as the comparison group. Three independent graders evaluated the selected posts based on the WCAG 2 checklist by WebAIM, a non-profit affiliated with Utah State University, after its adaptation for social media posts. Validated accessibility standard labels: "0" for not meeting any standards, "1" or "A" for meeting bare minimum accessibility requirements, "2" or "AA" for meeting legal accessibility requirements, or "3" or "AAA" for exceeding accessibility requirements. There was not enough evidence to detect a difference in WCAG scores between ophthalmology and non-ophthalmology posts (p = 0.80). Forty-nine percent of scores for ophthalmology social media posts showed no compliance with any WCAG. The most common reasons that ophthalmology posts failed to meet criteria were due to color and contrast issues (39%). Most ophthalmology social media posts had low WCAG scores, indicating poor compliance to WCAG. Because social media is highly visual, reduced compliance to WCAG may create barriers for low vision individuals to successfully access patient education social media content.

Esterman Visual Field Testing Using a Virtual Reality Headset in Glaucoma.

Purpose: To test the use of a virtual reality visual field headset (VRVF) for implementation of the Esterman visual field (EVF) test as compared with standard automated perimetry (SAP) among people with glaucoma. Design: Experimental design. Subjects: Patients with mild to severe glaucoma ranging from 10 to 90 years who presented for follow-up at a glaucoma clinic in Miami, Florida were eligible. Methods: Participants performed the EVF test on both SAP and VRVF. Five glaucoma-trained ophthalmologists were then asked to rate all anonymized SAP and RVF tests as a "pass" or "failure" based on Florida state law. Main Outcome Measures: Point-by-point concordance between original VRVF EVF test results and SAP EVF test results was calculated using the Kappa statistic. Concordance between SAP and VRVF was secondarily assessed with a conditional logistic regression based on the pass-failure determinations by the glaucoma-trained ophthalmologists. Interrater agreement on test pass-failure determinations was also calculated. Finally, test results on SAP versus VRVF were compared based on Esterman efficiency score (EES), the number of correct points divided by the number of total points, and duration of testing. Results: Twenty-two subjects were included in the study with ages ranging from 14 to 78 years old. Concordance between VRVF and SAP test using point-by-point analysis was poor (κ = 0.332, [95% confidence intervals {CI}: 0.157, 0.506]) and somewhat increased using pass-failure determinations from ophthalmologists (κ = 0.657, [95% CI: 0.549, 0.751]). Ophthalmologists were more likely to agree amongst themselves on pass-failure determinations for VRVF tests (κ = 0.890, [95% CI: 0.726, 0.964]) than for SAP (κ = 0.590, [95% CI: 0.372, 0.818]); however, VRVF demonstrated significantly lower EES than SAP (median EES difference: 4.5 points, P = 0.021). Conclusions: This pilot study is the first to assess the implementation of the EVF test using a virtual reality headset. Based on the weak overall agreement between VRVF and SAP, the current VRVF EVF test is not an acceptable determinant of driver's licensing. However, ophthalmologists were more likely to agree amongst themselves on VRVF test reports than on SAP reports. With further testing and improvement, virtual reality may eventually become a portable and convenient method for administering the EVF test. Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.