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.].

Hypomorphic variants in inherited retinal and ocular diseases: A review of the literature with clinical cases.

Hypomorphic variants decrease, but do not eliminate, gene function via a reduction in the amount of mRNA or protein product produced by a gene or by production of a gene product with reduced function. Many hypomorphic variants have been implicated in inherited retinal diseases (IRDs) and other genetic ocular conditions; however, there is heterogeneity in the use of the term "hypomorphic" in the scientific literature. We searched for all hypomorphic variants reported to cause IRDs and ocular disorders. We also discuss the presence of hypomorphic variants in the patient population of our ocular genetics department over the past decade. We propose that standardized criteria should be adopted for use of the term "hypomorphic" to describe gene variants to improve genetic counseling and patient care outcomes.

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.

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.].

The big warp: Registration of disparate retinal imaging modalities and an example overlay of ultrawide-field photos and en-face OCTA images.

PURPOSE: To develop an algorithm and scripts to combine disparate multimodal imaging modalities and show its use by overlaying en-face optical coherence tomography angiography (OCTA) images and Optos ultra-widefield (UWF) retinal images using the Fiji (ImageJ) plugin BigWarp. METHODS: Optos UWF images and Heidelberg en-face OCTA images were collected from various patients as part of their routine care. En-face OCTA images were generated and ten (10) images at varying retinal depths were exported. The Fiji plugin BigWarp was used to transform the Optos UWF image onto the en-face OCTA image using matching reference points in the retinal vasculature surrounding the macula. The images were then overlayed and stacked to create a series of ten combined Optos UWF and en-face OCTA images of increasing retinal depths. The first algorithm was modified to include two scripts that automatically aligned all the en-face OCTA images. RESULTS: The Optos UWF image could easily be transformed to the en-face OCTA images using BigWarp with common vessel branch point landmarks in the vasculature. The resulting warped Optos image was then successfully superimposed onto the ten Optos UWF images. The scripts more easily allowed for automatic overlay of the images. CONCLUSIONS: Optos UWF images can be successfully superimposed onto en-face OCTA images using freely available software that has been applied to ocular use. This synthesis of multimodal imaging may increase their potential diagnostic value. Script A is publicly available at https://doi.org/10.6084/m9.figshare.16879591.v1 and Script B is available at https://doi.org/10.6084/m9.figshare.17330048.

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.].

Is There Gender Bias in Perceptions of Strabismus Among Adults?

PURPOSE: To investigate the effect of gender, type, and size of horizontal deviation on the perceived severity or need for intervention for strabismus among adults in the general population. METHODS: Eight photographs of individuals aged 25 to 35 years were digitally manipulated to create 30 prism diopters (PD) of exotropia, 30 PD of esotropia, 50 PD of exotropia, and 50 PD of esotropia in both men and women. A survey asked respondents to rank these photographs by their severity or need for intervention from 1 (greatest need) to 8 (least need) to assess for unconscious bias for gender, strabismus type, or deviation size. RESULTS: A total of 203 adult respondents completed the survey. Participants ranked men with both large esotropia and large exotropia (median rank = 1.5, interquartile range [IQR] = 1.5 to 1.5) as needing correction more urgently than similar women (median rank = 3.5, IQR = 3.5 to 4) (P < .0001). The median rank was 4 (IQR = 3.75 to 4.25) for all men combined and 5 (IQR = 4.75 to 5.25) for all women (P < .0001). Photographs with exotropia (median rank = 4, IQR = 3.75 to 4.5) were perceived as more severe than photographs with esotropia (median rank = 5, IQR = 4.5 to 5.25) (P < .0001). Respondents' gender, age, or history of strabismus did not significantly impact their rankings. CONCLUSIONS: Male photographs were perceived as having a greater need for strabismus intervention than female photographs despite similar type and size of deviations. Exotropia was perceived as more severe than esotropia for smaller deviations only. More studies on the perceptions of strabismus are needed to understand whether unconscious bias affects patient care. [J Pediatr Ophthalmol Strabismus. 2023;60(6):396-401.].

Understanding the Use of the Newcastle, PEDIG, and LACTOSE Control Scores Among Pediatric Ophthalmologists for Intermittent Exotropia.

PURPOSE: To determine the clinical use patterns of control scores for intermittent exotropia. The Newcastle Control Score, Pediatric Eye Disease Investigator Group (PEDIG) score, and Look And Cover, then Ten seconds of Observation Scale for Exotropia (LACTOSE) Control Score were developed to quantify control of exodeviations. METHODS: A short survey was posted on the American Association for Pediatric Ophthalmology and Strabismus forum in August and September 2021. Respondents were asked about their assessment of control in intermittent exotropia, including knowledge and use of the various control scales. RESULTS: One hundred fourteen pediatric ophthalmologists responded; 54.4% (n = 62) reported not using any specific control score for intermittent exotropia, although 61.4% (n = 70) were familiar with the PEDIG score, 37.7% (n = 43) with the Newcastle Control Score, and 7.9% (n = 9) with the LACTOSE Control Score. The PEDIG score was the most widely used (26.3%, n = 30), but 36.7% (n = 11) of respondents reported that the scale is too time-intensive, limiting its use. To improve the use of the control scores, participants recommended promoting wider understanding of the scales (45.6%, n = 52). CONCLUSIONS: Most responding pediatric ophthalmologists do not use a specific control score in managing intermittent exotropia. The PEDIG score is the most frequently used but is reported as time-intensive. Although the LACTOSE Control Score was designed to provide a quicker alternative, it is not widely known. Promoting wider awareness and understanding of intermittent exotropia control scores may be helpful to allow for more objective quantification of control in intermittent exotropia. [J Pediatr Ophthalmol Strabismus. 2023;60(2):131-138.].

Indications and Outcomes for Extraocular Muscle Disinsertion in Strabismus Surgery.

PURPOSE: To describe a case series of extraocular muscle (EOM) disinsertions, discuss the indications for muscle disinsertion, and examine deviation outcomes. METHODS: All consecutive patients who underwent EOM disinsertion by one surgeon from 2013 to 2021 were included in the study. Age, sex, best corrected visual acuity, diagnosis, preoperative deviation, postoperative deviation, and follow-up interval were recorded. RESULTS: Twelve patients (13 eyes) underwent EOM disinsertion. Ten were men and two were women. Eight eyes had an abducens nerve (cranial nerve 6) palsy, four had thyroid eye disease, and one had an oculomotor nerve palsy (cranial nerve 3). Preoperatively, the average age was 57.7 years, best corrected visual acuity was 0.24 logarithm of the minimum angle of resolution (logMAR) in the right eye and 0.35 logMAR in the left eye, and deviation was 56.4 prism diopters (PD). The average postoperative residual deviation was 5.3 PD with an average follow-up time of 290 days. Four patients underwent reoperation, one of whom underwent a third surgery. CONCLUSIONS: EOM disinsertion is an effective treatment in certain specific strabismus conditions. Patients who may benefit are those with a severe cranial nerve 6 palsy who cannot abduct the eye even to the midline and those with thyroid eye disease whose EOMs are so tight intraoperatively that even a small hook cannot be easily passed under the operative muscle adequately to perform a large recession technique. [J Pediatr Ophthalmol Strabismus. 2023;60(4):253-256.].

Signal Peptide Variants in Inherited Retinal Diseases: A Multi-Institutional Case Series.

Signal peptide (SP) mutations are an infrequent cause of inherited retinal diseases (IRDs). We report the genes currently associated with an IRD that possess an SP sequence and assess the prevalence of these variants in a multi-institutional retrospective review of clinical genetic testing records. The online databases, RetNet and UniProt, were used to determine which IRD genes possess a SP. A multicenter retrospective review was performed to retrieve cases of patients with a confirmed diagnosis of an IRD and a concurrent SP variant. In silico evaluations were performed with MutPred, MutationTaster, and the signal peptide prediction tool, SignalP 6.0. SignalP 6.0 was further used to determine the locations of the three SP regions in each gene: the N-terminal region, hydrophobic core, and C-terminal region. Fifty-six (56) genes currently associated with an IRD possess a SP sequence. Based on the records review, a total of 505 variants were present in the 56 SP-possessing genes. Six (1.18%) of these variants were within the SP sequence and likely associated with the patients' disease based on in silico predictions and clinical correlation. These six SP variants were in the CRB1 (early-onset retinal dystrophy), NDP (familial exudative vitreoretinopathy) (FEVR), FZD4 (FEVR), EYS (retinitis pigmentosa), and RS1 (X-linked juvenile retinoschisis) genes. It is important to be aware of SP mutations as an exceedingly rare cause of IRDs. Future studies will help refine our understanding of their role in each disease process and assess therapeutic approaches.

Economic Impact of the COVID-19 Pandemic Post-Mitigation on Pediatric Ophthalmologists.

PURPOSE: To create a survey to assess the economic impact that the coronavirus disease 2019 (COVID-19) pandemic post-mitigation (post-shutdown) has had on pediatric ophthalmologists. METHODS: A 14-question survey was disseminated to United States-based pediatric ophthalmologists on the American Association for Pediatric Ophthalmology and Strabismus (AAPOS) discussion board and on social media. Demographic data and data on the economic effect of the COVID-19 pandemic post-mitigation were collected. RESULTS: A total of 129 pediatric ophthalmologists completed the survey. Eighty-four (65.1%) respondents reported a clinical revenue decrease of greater than 10%, 83 (64.3%) a surgical decrease of greater than 10%, and 66 (51.2%) an income decrease of greater than 10%. Fifteen (11.6%) respondents reported limiting the number of Medicaid patients. This was more prevalent among those in private practice (P = .027). Twenty-seven (20.9%) pediatric ophthalmologists responded that they planned to retire earlier than anticipated because of the pandemic and 2 (1.6%) responded that they have retired since the start of the pandemic. Six (4.7%) respondents reported that they have sold their practice since the start of the COVID-19 pandemic. CONCLUSIONS: Pediatric ophthalmologists continue to face economic challenges introduced by the COVID-19 pandemic. Reduced revenue may represent a new normal for the near future in pediatric ophthalmology. These unprecedented economic upheavals come at a time when the field of pediatric ophthalmology already faces difficulties with low reimbursement and attracting new trainees. This multitude of issues may escalate into a crisis in providing appropriate pediatric ophthalmic care. [J Pediatr Ophthalmol Strabismus. 2022;59(5):291-295.].

Resolution of traumatic mydriasis and accommodative dysfunction eight years after sweetgum ball ocular injury.

Purpose: To present a case of traumatic mydriasis (MD) and accommodative dysfunction (AD) secondary to a sweetgum ball ocular injury that resolved 8 years after the inciting trauma. Observations: A 6-year-old female presented with left eye ocular trauma after being hit with a sweetgum ball. Sweetgum balls are the small, spiky fallen fruits of the American Sweetgum tree (Liquidambar styraciflua). Due to their size and shape, children often use them as projectiles during play. On presentation, the patient had a partial thickness corneal laceration, traumatic mydriasis (TM), and accommodative dysfunction (AD). Her corneal laceration was repaired. Her TM and AD persisted. She was treated with bifocal spectacles and patching. At her 7-year follow-up visit, her TM and AD showed minimal signs of improvement. Eight years post-injury, her TM and AD had both improved significantly. Conclusion and Importance: Sweetgum balls, when used as projectiles, pose a risk of serious ocular injury. Pupillary and accommodative function in TM may improve much later than previously appreciated. Young age may contribute to parasympathetic neuroregeneration. Patching may have prevented amblyopia in this case, allowing her left eye to achieve its full visual potential once her pupillary and accommodative function returned.