Pseudoxanthoma elasticum-associated angioid streaks near a scleral buckle.

Purpose: We report a patient with pseudoxanthoma elasticum (PXE) with angioid streaks near a scleral buckle site. Observations: A 46-year-old male with PXE presented for evaluation of blurry vision and was found to have classic PXE findings in both eyes and angioid streaks adjacent to the site of a scleral buckle in his left eye. He underwent multimodal imaging, genetic testing, and intravitreal aflibercept in the right eye. Conclusions and importance: Bruch's membrane is known to be fragile in PXE, and patients are often counseled about the heightened risk of playing contact sports. This report raises the question of whether tension from a scleral buckle in the setting of a calcified and brittle BM may increase the likelihood of angioid streaks near the buckle site. In the setting of retinal detachment, it may be worthwhile to carefully weigh the pros and cons of vitrectomy versus buckle for PXE patients.

Review of the Disparities in Access to Pediatric Eye Care Among Low Socioeconomic Status and Underrepresented Racial Minority Groups Exacerbated by the Economic Downturn in Pediatric Ophthalmology.

PURPOSE: To explore the association between race and socioeconomic status on health disparities in pediatric eye care, characterize the status of diversity within the pediatric ophthalmology workforce, and propose potential solutions to improve access to vision care, particularly for children from vulnerable populations. METHODS: The major studies examining the socioeconomic and racial disparities in visual outcomes and the impact of diversity in the ophthalmology workforce were reviewed. RESULTS: Currently, the pediatric ophthalmology workforce has limited racial diversity, and increasing the number of underrepresented in medicine providers has potential to improve access to pediatric eye care, particularly for underserved populations. Mobile eye clinics, school-based vision programs, and health literacy initiatives may engage communities to bring eye care directly to children and enhance follow-up. CONCLUSIONS: Families with lower socioeconomic status may represent a vulnerable population facing significant access to pediatric eye care barriers, particularly for asymptomatic pediatric eye conditions. Emphasis on strategies that directly engage communities and bring free vision care to families should be given to improve disparities in visual health outcomes. Ophthalmologists and pediatric ophthalmologists should be aware of the current racial underrepresentation within the pediatric ophthalmology workforce, and the importance of workforce diversity in accelerating physician-patient concordance and access to eye care. [J Pediatr Ophthalmol Strabismus. 20XX;X(X):XXX-XXX.].

Creating consistency in the diagnosis of convergence insufficiency: screening methods.

PURPOSE: To determine the sensitivity of various clinical tests in the diagnosis of convergence insufficiency. METHODS: A total of 254 patients were recruited with complaints consistent with convergence problems but no prior history of strabismus surgery, eye exercises, prism use, recent concussion, or other ocular or neurological diseases. Each patient completed the convergence insufficiency symptom survey (CISS), and the following data were collected: ocular alignment at distance and near, convergence and divergence fusional amplitudes at distance and near, near-point of convergence (NPC) using an accommodative target and red lens, and assessment of quality of convergence movement (QoCM) and quality of fusional movements (QoFM). The sensitivity of each clinical test was calculated. RESULTS: Measurement of NPC using red lens and subjective assessment of the QoCM and QoFM were the most sensitive diagnostic tools for near symptoms consistent with convergence insufficiency: 93.3%, 98.4%, and 94.5% respectively. CISS score, convergence fusional amplitude at near, and exophoria at near had lower sensitivities: 62.9%, 46.0%, and 72.0%, respectively. Although the majority of our patients had a heterophoria or heterotropia at distance (96.8%) and/or near (98.8%), most presented with only small phorias. Furthermore, of those who had a deviation at near, only 22% had the near exophoria exceeding the distance exophoria by 10Δ. CONCLUSIONS: In our study cohort, NPC with red lens and subjective assessment of QoCM and QoFM proved to be the most sensitive screening tools for near symptoms consistent with convergence insufficiency.

What We Learned About the Economic and Workforce Issues in Pediatric Ophthalmology: Access to Eye Care and Possible Solutions.

PURPOSE: To provide a blueprint reviewing the major economic and workforce issues in pediatric ophthalmology and its effect on access to pediatric eye care, as well as potential solutions. METHODS: This was a systematic review of the landmark studies examining the economic and workforce issues in pediatric ophthalmology was performed. RESULTS: The number of residents pursuing pediatric ophthalmology fellowship has steadily declined since the early 2000s. Increasing overhead costs, low Medicaid reimbursements, practice pattern changes, and workforce shortages have all contributed to a significant crisis in access to pediatric eye care. CONCLUSIONS: Solutions to improve access to pediatric eye care involve pursuing more economical strategies within the confines of the pediatric ophthalmology practice, early exposure to the field during medical education, and enhancing residents' interest through faculty mentorship. Low reimbursements are a major driver contributing to extended appointment wait times for pediatric ophthalmology visits at major academic eye centers. Legislative changes at both the federal and state levels are needed to make appropriate increases in reimbursements, starting with states in the lowest quartile of Medicaid reimbursements. [J Pediatr Ophthalmol Strabismus. 2023;60(5):323-329.].

Visual acuity improvement in the amblyopic eye of a 50-year-old man following strabismus surgery.

It is generally believed that the visual acuity of an amblyopic eye cannot improve significantly in adulthood. However, there have been reports of this occurring following visual loss in the fellow eye. Best-corrected visual acuity improvement in amblyopic eyes of children with strabismic amblyopia following strabismus surgery has also been reported. We present the case of an adult whose visual acuity in his amblyopic eye improved 4 Snellen lines over 28 months following strabismus surgery.

A Comprehensive Report of Intrinsically Disordered Regions in Inherited Retinal Diseases.

BACKGROUND/PURPOSE: A comprehensive review of the degree of disorder in all genes in the Retinal Information Network (RetNet) Database is implicated in inherited retinal diseases (IRDs). Their association with a missense variation was evaluated. METHODS: IRD genes from RetNet were included in this study. Publicly available data on the genome aggregation database (gnomAD) were used to analyze the number of total and pathogenic missense variants. Metapredict, an accurate and high-performance predictor that reproduces consensus disorder scores, was used to calculate disorder. MAIN OUTCOME MEASURES: The main outcome measures were percent disorder, percent pathogenicity, number of total missense variants, and percent total missense variation. RESULTS: We included 287 RetNet genes with relevant data available from gnomAD. Mean percent disorder was 26.3% ± 26.0%, mean percent pathogenicity was 5.2% ± 11.0%, mean number of total missense variants was 424.4 ± 450.0, and mean percent total missense was 50.0% ± 13.4%. The percent disorder followed a bimodal distribution with the highest number of occurrences in the 0 to 10th disorder decile. The five outlier proteins in the first disorder decile with a higher-than-expected number of total missense variation were identified (HMCN1, ADGRV, USH2A, DYNC2H1, LAMA1, and SLC38A8). When excluded, % total missense was significantly associated with percent disorder (R = 0.238 and p = 0.0240). CONCLUSIONS: This novel study examining all genes implicated in IRDs found that the majority genes had a disorder in the 0 to 10th decile and were relatively intolerant to missense variation. This may have future utility when interpreting variants of undetermined significance and missense variants.

Unanswered Questions Pertaining to the Economic and Workforce Issues in Pediatric Ophthalmology.

PURPOSE: To create a survey that examines remaining unanswered questions related to the economic and workforce issues in pediatric ophthalmology. METHODS: A 12-question survey was distributed to U.S.-based pediatric ophthalmologists on the American Association for Pediatric Ophthalmology and Strabismus (AAPOS) discussion board and various other social media forums. Statistical analysis was performed on the basis of practice setting groups. RESULTS: Two hundred eighty-seven pediatric ophthalmologists completed the survey. Of all respondents, 150 (52.3%) believed pediatric ophthalmology is a prestigious subspecialty, 137 (47.7%) thought that a hybrid fellowship combining pediatric ophthalmology and adult cataract surgery would increase the number of practicing pediatric ophthalmologists, 189 (65.9%) felt that early exposure during medical school would increase the number of residents who ultimately apply to pediatric ophthalmology fellowship, and 81 (28.2%) believed that the lobbyists hired by the AAPOS will be successful in preventing further Centers for Medicare and Medicaid Services reimbursement cuts. Medicaid patients comprised more than 25% of the total practice for 156 (54.4%) respondents. New patient, follow-up, and surgery wait times were greater than 2 months in 50.9%, 46.7%, and 38.7% of respondents, respectively. CONCLUSIONS: As the economic crisis in pediatric ophthalmology continues to worsen, questions related to increasing interest in the field through early exposure, hybrid fellowship, prestige, appointment wait times, and belief in the success of AAPOS lobbyists remain. Exceptional measures are required to revitalize the field and improve access to care. [J Pediatr Ophthalmol Strabismus. 2023;60(3):163-169.].

The Medicaid Dilemma and Its Profound Effect on the Economic and Workforce Issues in Pediatric Ophthalmology and Ultimately on Access to Pediatric Eye Care.

PURPOSE: To identify new patient, follow-up, and surgery appointment wait times for children seeking care at academic eye centers/children's hospitals (AEC/CHs) in the setting of very low Medicaid reimbursements and workforce issues in pediatric ophthalmology. METHODS: Each state in the United States with AEC/CHs was contacted by phone or email. Using a standardized script, the authors inquired about approximate wait times for non-emergency new patient, follow-up, and surgery appointment times, as well as percentage of patients insured by Medicaid seen at the AEC/CH. Medicaid reimbursements for new patient visits, follow-up visits, sensorimotor examinations, and one-muscle strabismus surgery were examined for each state. RESULTS: The mean percentage of children enrolled in Medicaid across the United States was 38.0%. Appointment and Medicaid inquiry calls were conducted across 41 states. Mean wait times across the United States for new patient, follow-up, and surgery appointments were 3.9, 3.4, and 2.7 months, respectively. Mean percentage of patients insured by Medicaid at AEC/CHs was 57.1%. High Medicaid-insured states offered longer wait times for new patient appointments than low Medicaid-insured states (4.4 versus 3.5 months; P = .20). The lowest Medicaid reimbursements for new patient visit, follow-up visit, sensorimotor examination, and one-muscle strabismus surgery occurred in Illinois, California, Ohio, and Michigan, respectively. CONCLUSIONS: Across the United States, states with higher percentages of patients insured by Medicaid exhibited longer new patient appointment wait times, suggesting that challenges related to Medicaid reimbursements directly contribute to disparities in access to pediatric eye care. [J Pediatr Ophthalmol Strabismus. 2023;60(3):170-177.].

Semi-Automated Analysis of Foveal Maturity in Premature and Full-Term Infants Using Handheld Swept-Source Optical Coherence Tomography.

Purpose: To develop a semi-automated method of measuring foveal maturity using investigational handheld swept source-optical coherence tomography (SS-OCT). Methods: In this prospective, observational study, full-term newborns and preterm infants undergoing routine retinopathy of prematurity screening were imaged. Semi-automated analysis measured foveal angle and chorioretinal thicknesses at the central fovea and average two-sided parafovea by three-grader consensus, correlating with OCT features and demographics. Results: One hundred ninety-four imaging sessions from 70 infants were included (47.8% girls, 37.6 ± 3.4 weeks postmenstrual age, 26 preterm infants with birth weight 1057 ± 325.0, gestational age 29.0 ± 3.0 weeks). Foveal angle (96.1 ± 22.0 degrees) steepened with increasing birth weight (P = 0.003), decreasing inner retinal layer thickness, and increasing gestational age, postmenstrual age, and foveal and parafoveal choroidal thickness (all P < 0.001). Inner retinal fovea/parafovea ratio (0.4 ± 0.2) correlated with increasing inner foveal layers, decreasing postmenstrual age, gestational age, and birth weight (all P < 0.001). Outer retinal F/P ratio (0.7 ± 0.2) correlated with ellipsoid zone presence (P < 0.001), increased gestational age (P = 0.002), and birth weight (P = 0.003). Foveal (447.8 ± 120.6 microns) and parafoveal (420.9 ± 109.2) choroidal thicknesses correlated with foveal ellipsoid zone presence (P = 0.007 and P = 0.01, respectively), postmenstrual age, birth weight, gestational age, and decreasing inner retinal layers (all P < 0.001). Conclusions: Foveal development is dynamic and partially observed through semi-automated analysis of handheld SS-OCT imaging. Translational Relevance: Semi-automated analysis of SS-OCT images can identify measures of foveal maturity.

In the Setting of Heightened Economic and Workforce Issues, What Are Third-Year (PGY-4) Ophthalmology Residents' Perspectives of Pediatric Ophthalmology?

PURPOSE: To investigate the factors influencing residents' choice to pursue a pediatric ophthalmology fellowship. METHODS: A nine-question survey was distributed to third-year (PGY-4) ophthalmology residents of United States-based Accreditation Council for Graduate Medical Education residency training programs in ophthalmology. RESULTS: One hundred two of 502 residents completed the survey. Fifty percent of all respondents had no debt and 14.7% had more than $300,000 in debt. Forty-four (43.1%) rated the quality of pediatric ophthalmology teaching in their residency program as "excellent," 37 (36.3%) did not enjoy performing clinical ophthalmologic examinations on children, 83 (81.4%) had a clinical role model in pediatric ophthalmology, 29 (28.4%) believed pediatric ophthalmology is a prestigious subspecialty, 47 (46.1%) reported that economic factors influenced their post-residency decisions, 60 (58.8%) believed a hybrid fellowship combining adult cataract surgery and pediatric ophthalmology would be a popular fellowship, and 58 (56.9%) had a first-year (PGY-2) residency rotation of at least 1 month in pediatric ophthalmology. Enjoying the ophthalmologic examination of children most strongly predicted whether a resident would pursue a pediatric ophthalmology fellowship (Phi = 0.482). CONCLUSIONS: The desire to work with children overwhelmingly superseded other factors influencing a resident's decision to pursue a pediatric ophthalmology fellowship. Improving economic issues may be one of several factors that increase the attractiveness of the pediatric ophthalmology subspecialty. There is a dilemma in encouraging more residents to pursue pediatric ophthalmology in the setting of fewer technological advances consistent with lower perceived prestige. [J Pediatr Ophthalmol Strabismus. 2023;60(2):95-100.].

Initial Investigations of Intrinsically Disordered Regions in Inherited Retinal Diseases.

Intrinsically disordered regions (IDRs) are protein regions that are unable to fold into stable tertiary structures, enabling their involvement in key signaling and regulatory functions via dynamic interactions with diverse binding partners. An understanding of IDRs and their association with biological function may help elucidate the pathogenesis of inherited retinal diseases (IRDs). The main focus of this work was to investigate the degree of disorder in 14 proteins implicated in IRDs and their relationship with the number of pathogenic missense variants. Metapredict, an accurate, high-performance predictor that reproduces consensus disorder scores, was used to probe the degree of disorder as a function of the amino acid sequence. Publicly available data on gnomAD and ClinVar was used to analyze the number of pathogenic missense variants. We show that proteins with an over-representation of missense variation exhibit a high degree of disorder, and proteins with a high amount of disorder tolerate a higher degree of missense variation. These proteins also exhibit a lower amount of pathogenic missense variants with respect to total missense variants. These data suggest that protein function may be related to the overall level of disorder and could be used to refine variant interpretation in IRDs.

Is There a Path for an Economic Turnaround in Pediatric Ophthalmology?

PURPOSE: To create a survey that assesses potential solutions for an economic turnaround in pediatric ophthalmology and the frequency of their use. METHODS: A 12-question survey was distributed to United States-based pediatric ophthalmologists on the American Association for Pediatric Ophthalmology and Strabismus discussion board and various social media fora. Demographic, economic, and workforce pattern data were collected and analyzed. RESULTS: Three hundred one pediatric ophthalmologists completed the survey. Thirty-one (10.3%) respondents used optical coherence tomography in children with amblyopia, 81 (26.9%) were a partner in an ambulatory surgery center, 133 (44.2%) had a retinopathy of prematurity financial contract arrangement with a neonatal intensive care unit to examine premature children, and 95 (31.6%) had a financial contract arrangement with an optical shop in their office. Twenty-four (8.0%) respondents were currently in the process of being or had been bought out by a private equity investor, 52 (17.3%) owned a Spot Vision Screener (Welch Allyn) for refraction, 158 (52.5%) had hired a pediatric ophthalmology extender (pediatric optometrist, technician, or orthoptist), 87 (29.5%) had recently increased their marketing profile, and 90 (29.9%) owned the property that their practice occupied. CONCLUSIONS: Potential for a revitalization and economic turnaround in pediatric ophthalmology depends on several income-generating suggestions that simultaneously improve workforce and manpower issues, thus increasing access to pediatric eye care. [J Pediatr Ophthalmol Strabismus. 2023;60(1):25-32.].

The Economic Downturn of Pediatric Ophthalmology and Its Impact on Access to Eye Care.

PURPOSE: To discuss the economic downturn of pediatric ophthalmology in the setting of an alarming decline in the number of residents pursuing pediatric ophthalmology and its impact on limiting access to eye care. METHODS: Survey studies and articles examining the recent economic impact of reimbursement cuts and practice pattern changes in pediatric ophthalmology were reviewed. Population data from the 2020 U.S. Census Bureau and the American Association for Pediatric Ophthalmology and Strabismus (AAPOS) were analyzed to illustrate provider-to-population relationships by state. RESULTS: Survey studies demonstrated a deteriorating economic situation marked by progression in reimbursement cuts, reductions in pediatric ophthalmologists accepting Medicaid patients, and dwindling numbers of residents pursuing pediatric ophthalmology fellowships. Provider-to-population relationships revealed that pediatric ophthalmologists are not evenly distributed to meet population demand, and that many states are suffering from a shortage of pediatric ophthalmologists. Furthermore, many states with high percentages of Medicaid coverage simultaneously have lower AAPOS members/million person ratios. CONCLUSIONS: The simple economic principles of supply and demand reveal a crisis in access to pediatric eye and adult strabismus care. The economic downtown and change in practice patterns may produce rippling effects outside pediatric ophthalmology in fields such as pediatrics and comprehensive ophthalmology. An interdisciplinary effort among health care providers and governmental officials is needed to revive the field of pediatric ophthalmology and improve access to eye care. [J Pediatr Ophthalmol Strabismus. 2023;60(1):18-24.].

The Economic Factors Impacting the Viability of Pediatric Ophthalmology.

PURPOSE: To create a survey that assesses the economic factors impacting the viability of pediatric ophthalmology between January 2021 and July 2022. METHODS: A 12-question survey was distributed to United States-based pediatric ophthalmologists on the American Association for Pediatric Ophthalmology and Strabismus discussion board and various social media fora. Demographic, economic, and workforce pattern data were collected and analyzed. RESULTS: A total of 243 pediatric ophthalmologists completed the survey. One hundred seven (44.0%) respondents reported a surgical revenue decrease between 10% and 25%, 117 (48.1%) a clinical revenue decrease of less than 10%, 111 (45.6%) an overall income decrease of less than 10%, and 127 (52.2%) an overhead cost increase between 10% and 25%. Seventy-two (29.6%) respondents reported subsidizing income with pursuits outside of pediatric ophthalmology, 27 (11.1%) stopped operating due to reimbursement cuts, 75 (30.8%) limited the number of Medicaid or other public funded patients, 16 (6.5%) retired in the past 3 years, and 92 (37.8%) would not recommend a resident pursue a pediatric ophthalmology fellowship. CONCLUSIONS: There is a potential upheaval in the field of pediatric ophthalmology marked by increasing levels of disillusionment among current providers, a progressive decline in the number of trainees pursuing fellowship programs, and workforce issues suggestive of diversification of practice patterns outside of pediatric ophthalmology. The current economic crisis, in conjunction with these complex workforce issues, is and will continue to create a shortage of practicing pediatric ophthalmologists, seriously limiting access to pediatric and adult strabismus eye care. [J Pediatr Ophthalmol Strabismus. 2022;59(6):362-368.].

Healthcare workers, epidemic biological risks - recommendations based on the experience with COVID-19 and Ebolavirus.

Infectious disease outbreaks frequently cause illness and death among Healthcare Workers (HCWs). We compare strategies from recent, past and ongoing outbreak measures used to protect HCWs, including those facing additional challenges such as racial disparities, violence and stigmatization. Outbreaks and pandemics superimposed on countries with preexisting crises have also affected emergency response to these viral outbreaks. Strategies to protect HCWs include adherence to recommended infection prevention and control measures; new technology such as rapid point-of-care tests and remote monitoring; adopting national public health preparedness plans to ensure the supply and allocation of PPE, staff, and testing supplies; occupational health and mental health support services. Lessons learned from recent pandemics should be used by Infection Prevention and Control and Occupational Health staff to refine preparedness plans to protect HCWs better.

Challenges Associated With Ellipsoid Zone Intensity Measurements Using Optical Coherence Tomography.

Translational Relevance: Qualitative evaluation of the ellipsoid zone band on optical coherence tomography is a valuable clinical tool for assessing photoreceptor structure, though more quantitative metrics are emerging. Awareness of the challenges involved in interpreting quantitative metrics is important for their clinical translation.

Infection Rates and Risk Factors for Infection Among Health Workers During Ebola and Marburg Virus Outbreaks: A Systematic Review.

Background: Infection in health workers (HWs) has characterized outbreaks of Ebola virus disease (EVD) and Marburg virus disease (MVD). We conducted a systematic review to investigate infection and mortality rates and common exposure risks in HWs in EVD and MVD outbreaks. Methods: We searched the EMBASE and PubMed databases to identify articles posted before 27 December 2017, with no language restrictions. Data on the number, frequency, and mortality of HW infection and exposure risks were extracted. Results: Ninety-four articles related to 22 outbreaks were included. HW infections composed 2%-100% of cases in EVD and 5%-50% of cases in MVD outbreaks. Among exposed HWs, 0.6%-92% developed EVD, and 1%-10% developed MVD. HW infection rates were consistent through outbreaks. The most common exposure risk situations were inadequate personal protective equipment and exposure to patients with unrecognized EVD/MVD. Similar risks were reported in past EVD/MVD outbreaks and in the recent outbreak in West Africa. Conclusions: Many outbreaks reported high proportions of infected HWs. Similar HW infection rates and exposure risk factors in both past and recent EVD and MVD outbreaks emphasize the need to improve the implementation of appropriate infection control measures consistently across all healthcare settings.

Closing the loop on impulsivity via nucleus accumbens delta-band activity in mice and man.

Reward hypersensitization is a common feature of neuropsychiatric disorders, manifesting as impulsivity for anticipated incentives. Temporally specific changes in activity within the nucleus accumbens (NAc), which occur during anticipatory periods preceding consummatory behavior, represent a critical opportunity for intervention. However, no available therapy is capable of automatically sensing and therapeutically responding to this vulnerable moment in time when anticipation-related neural signals may be present. To identify translatable biomarkers for an off-the-shelf responsive neurostimulation system, we record local field potentials from the NAc of mice and a human anticipating conventional rewards. We find increased power in 1- to 4-Hz oscillations predominate during reward anticipation, which can effectively trigger neurostimulation that reduces consummatory behavior in mice sensitized to highly palatable food. Similar oscillations are present in human NAc during reward anticipation, highlighting the translational potential of our findings in the development of a treatment for a major unmet need.

Immune Evasion Strategies of Glioblastoma.

Glioblastoma (GBM) is the most devastating brain tumor, with associated poor prognosis. Despite advances in surgery and chemoradiation, the survival of afflicted patients has not improved significantly in the past three decades. Immunotherapy has been heralded as a promising approach in treatment of various cancers; however, the immune privileged environment of the brain usually curbs the optimal expected response in central nervous system malignancies. In addition, GBM cells create an immunosuppressive microenvironment and employ various methods to escape immune surveillance. The purpose of this review is to highlight the strategies by which GBM cells evade the host immune system. Further understanding of these strategies and the biology of this tumor will pave the way for developing novel immunotherapeutic approaches for treatment of GBM.