An unexpected turn: a case of torsional anomalous retinal correspondence after myectomy of previously anteriorized inferior oblique muscles in a child.

We report a case of torsional diplopia caused by presumed torsional anomalous retinal correspondence after myectomy of previously asymmetrically anteriorized inferior oblique muscles for inferior oblique overaction. Given this patient's experience, it may be prudent to operate with caution on previously anteriorized inferior oblique muscles, especially when anteriorization is performed at a very young age.

The Nictavi Tarsus Patch: A New Device for Achieving Temporary Eyelid Closure in Lagophthalmos.

Purpose: To evaluate the effectiveness, tolerability, and safety of the Nictavi Tarsus Patch™ (NTP) in inducing temporary eyelid closure for the management of lagophthalmos in the pediatric and young adult population. Methods: We prospectively enrolled 20 patients <21 years of age who had previously been managed for lagophthalmos to trial the NTP in clinic. Inter-palpebral fissure distance (IPFD) was compared before and after the placement of the NTP in the eyes-closed position using paired t-tests. Subjects then underwent a 3-night home trial with the NTP, and parent and subject perceptions of effectiveness, comfort, and complications with the patch were analyzed using Likert scale survey questions. Results: Twenty subjects ages 2-20 years with paralytic (65%) and non-paralytic (35%) lagophthalmos were enrolled. The NTP improved lagophthalmos from a mean pre-placement IPFD of 3.3 mm to post-placement IPFD of 0.4 mm (p < 0.01). Overall, 80% of subjects achieved successful eyelid closure defined as ≤1 mm of post-placement IPFD. When stratified by subtype, 100% of subjects with paralytic lagophthalmos achieved successful eyelid closure compared to 71% of subjects with non-paralytic lagophthalmos. On a scale of 1 (worst) to 5 (best), parents rated the NTP at 4.3±0.7 for comfort while wearing, 4.3±1.0 for comfort in removing, 4.6±0.7 for ease of use, and 4.3±0.9 for effectiveness. Ninety-three percent of parents reported preferring NTP to other eyelid closure methods previously tried and indicated that they would use it again. Conclusion: The NTP is an effective, tolerable, and safe method of eyelid closure for children and young adults.

Evaluation of Streamed Hardware-to-Software Telemedicine Strabismus Consultations Utilizing Video Glasses.

Background: While video glasses have been shown to be an effective tool for real-time pediatric strabismus telemedicine consultations, the high cost of the hardware-to-hardware conferencing system and bandwidth limitations may present barriers to accessibility and widespread adoption. This study evaluates the use of video glasses with a more affordable hardware-to-software video conferencing system for real-time strabismus consultations across multiple graders. Methods: A pediatric ophthalmologist (Grader 1) wearing video glasses simultaneously performed and recorded strabismus examinations in primary gaze, with and without correction, both at distance and near. Recorded parameters included strabismus category, angle measurements, and ocular motility. Three years later, four pediatric ophthalmologists (Graders 1-4) reviewed and graded streamed video feed transmitted at 1 megabit per second (Mbps) from a hard-wired codec to software. Agreement between streamed and gold standard in-person findings was determined by weighted kappa (κ) for categorical variables, intraclass correlation coefficient (ICC) for continuous variables, and percent agreement. Results: Eighteen patients aged 4-11 years (median, 7 years) were included. Agreement in strabismus category between in-person and streamed examinations was perfect for both horizontal and vertical deviations (κ=1.0). Almost perfect agreement was found for degree manifest (tropia vs intermittent tropia vs phoria) across graders (κ=0.91, range 0.86-0.97). Agreement for angle measurements was excellent across graders (ICC = 0.97, range 0.97-0.98). Extraocular motility agreement was 90% for all graders combined, with Grader 1 having 100% agreement between her in-person and streamed examinations. Conclusion: Feed obtained from video glasses streamed through a hardware-to-software video conferencing system at 1 Mbps is a reliable tool for pediatric strabismus telemedicine evaluations.

Refractive Error Findings in Students Who Failed School-based Vision Screening.

PURPOSE: To report refractive error findings in Baltimore City schoolchildren who failed school-based vision screenings. METHODS: In this cross-sectional analysis, students pre-kindergarten through 8th grade who failed screenings during school years 2016-2019 received an eye examination, including non-cycloplegic autorefraction and visual acuity (VA) measurements. Refractive error was identified when there was at least: -0.50 diopter (D) spherical equivalent (SE) myopia, +0.50D SE hyperopia, 1.00D astigmatism, or 1.00D anisometropia in either eye. Generalized estimating equation models were used to identify factors associated with clinically significant refractive error, defined as decreased VA and more severe refractive error. RESULTS: Of 7520 students who failed screening, 6627 (88%) were analyzed. Clinically significant refractive error and any refractive error were found in 2352 (35.5%) and 5952 (89.8%) students, respectively. Mild myopia (45%, -0.50 D to <-3.00 D SE) and low astigmatism (47%, 1.00 D to <3.00 D cylinder) were the most prevalent types of refractive error. Proportions of students with myopia increased with higher grade levels (Ptrend<0.001). Myopia and astigmatism were more common in black and Latinx. Risk factors for clinically significant refractive error included higher grades (odds ratios [OR] ranged from 1.30 to 2.19 compared with 1st grade, P < .05) and Latinx ethnicity (OR = 1.31, 95%CI: 1.08-1.59). CONCLUSION: A Baltimore school-based vision program identified a substantial number of students with refractive error in a high-poverty urban community. Over 1/3 students who failed vision screening had clinically significant refractive error, with black and Latinx students at higher risk of having myopia and astigmatism.

Endophthalmitis after pediatric cataract surgery in the United States: report using an insurance claims database.

PURPOSE: To describe the characteristics and incidence of children developing endophthalmitis within the first 90 days following pediatric cataract surgery. SETTING: Deidentified commercial and Medicare Advantage health claims across the United States. DESIGN: Population-based retrospective cohort study. METHODS: In a retrospective review of approximately 58 million charts in Optum's deidentified Clinformatics Data Mart Database, patients aged <13 years who underwent cataract surgery in one or both eyes with or without primary intraocular lens (IOL) implantation between 2003 and 2017 were identified. Excluded were patients with traumatic cataract, <90 days of continuous insurance coverage, a prior diagnosis of endophthalmitis, and a diagnosis of endophthalmitis occurring after 90 days of cataract surgery. The main outcome measure was the incidence of endophthalmitis occurring within the first 90 days of cataract surgery and the odds ratio for developing endophthalmitis according to demographic and intraoperative factors. RESULTS: Cataract surgery was performed on 789 eyes (52.6% male), with a median age of 4 (interquartile range 1-8) years. The rate of IOL implantation at the time of cataract surgery was 66.8%. Endophthalmitis was diagnosed in 4 of 789 eyes (0.51%). The median time to diagnosis of endophthalmitis was 6.5 days (range: 5-44 days). There was no significant association between endophthalmitis and age, sex, or primary IOL implantation. CONCLUSIONS: In this large insurance claims database, the incidence of endophthalmitis following pediatric cataract surgery reported was more than the rate previously reported by any study with patients of a similar age.

Punctal agenesis and delayed-onset dacryocystocele in CHARGE syndrome.

Punctal agenesis and other nasolacrimal abnormalities have been infrequently reported in CHARGE syndrome-a constellation of findings affecting the eyes, heart, choana, and ears-which generally presents at birth. We present a rare case of punctal agenesis with delayed-onset dacryocystocele/lacrimal sac mucocele in a teenager with CHARGE syndrome.

The Association of Glaucomatous Visual Field Loss and Balance.

PURPOSE: To relate balance measures to visual field (VF) damage from glaucoma. METHODS: The OPAL kinematic system measured balance, as root mean square (RMS) sway, on 236 patients with suspect/diagnosed glaucoma. Balance was measured with feet shoulder width apart while standing on a firm/foam surface with eyes opened/closed (Instrumental Clinical Test of Sensory Integration and Balance [ICTSIB] conditions), and eyes open on a firm surface under feet together, semi-tandem, or tandem positions (standing balance conditions). Integrated VF (IVF) sensitivities were calculated by merging right and left eye 24-2 VF data. RESULTS: Mean age was 71 years (range, 57-93) and mean IVF sensitivity was 27.1 dB (normal = 31 dB). Lower IVF sensitivity was associated with greater RMS sway during eyes-open foam-surface testing (ß = 0.23 z-score units/5 dB IVF sensitivity decrement, P = 0.001), but not during other ICTSIB conditions. Lower IVF sensitivity also was associated with greater RMS sway during feet together standing balance testing (0.10 z-score units/5 dB IVF sensitivity decrement, P = 0.049), but not during other standing balance conditions. Visual dependence of balance was lower in patients with worse IVF sensitivity (ß = -21%/5 dB IVF sensitivity decrement, P < 0.001). Neither superior nor inferior IVF sensitivity consistently predicted balance measures better than measures of overall VF sensitivity. CONCLUSIONS: Balance was worse in glaucoma patients with greater VF damage under foam surface testing (designed to inhibit proprioceptive contributions to balance) as well as feet-together firm-surface conditions when somatosensory inputs were available. TRANSLATIONAL RELEVANCE: Good balance is essential to avoid unnecessary falls and patients with VF loss from glaucoma may be at higher risk of falls because of poor balance.

Incorporating students into clinic may be associated with both improved clinical productivity and educational value.

BACKGROUND: In this study, we aimed to evaluate ambulatory clinic responsibilities that neurology clerkship students perceive as having the highest educational value and to evaluate the association between a student's presence and level of responsibility and a preceptor's clinical and financial productivity during a clinic session. METHODS: Physician preceptors (n = 43) and medical students (n = 67) in the Johns Hopkins Neurology clerkship from 2014 to 2015 were included. Students rated their experience and responsibilities in 291 neurology clinic sessions. Productivity metrics (e.g., relative value units [RVU]/clinic) were collected for each preceptor in the presence and absence of students. RESULTS: A student's rating of a clinic as an effective learning experience increased with each additional patient the student interviewed (odds ratio [OR] 1.89, p < 0.001), presented (OR 1.86, p < 0.001), or documented (OR 2.00, p < 0.001). The mean RVU/session for preceptors also increased based on the number of patients interviewed (ß = 2.64, p = 0.026), presented (ß = 2.42, p = 0.047), and documented (ß = 2.70, p = 0.036) by students. On average, preceptor RVU/session increased by 42% (mean 5.6 ± 1.2, p < 0.0001) when a student was present in clinic compared to sessions without students. In addition, preceptor invoices increased by 35% (mean 2.7 ± 0.6, p < 0.0001) and charges by 39% (mean $929 ± $210, p < 0.0001) when a student was present in clinic. CONCLUSIONS: This observational study suggests a mutual benefit to preceptor clinical productivity and student-perceived educational value when students have active responsibilities in neurology clinics. Despite concerns that students slow down preceptors in clinic, these results suggest that preceptors may have an overall boost in productivity, potentially by performing billable work while students independently see patients.

Physical activity restriction in age-related eye disease: a cross-sectional study exploring fear of falling as a potential mediator.

BACKGROUND: Fear of falling (FoF) is predictive of decreased physical activity. This study sought to determine if FoF mediates the relationship between decreased vision and physical activity restriction in individuals with glaucoma and age-related macular degeneration (AMD). METHODS: Accelerometers were used to measure physical activity over 1 week in 59 control, 83 glaucoma, and 58 AMD subjects. Subjects completed the University of Illinois at Chicago Fear of Falling Questionnaire, and the extent of FoF was estimated using Rasch analysis. In negative binomial models adjusting for demographic, health, and social factors, FoF was investigated as a potential mediator between the severity of visual field (VF) loss (in glaucoma patients) or the severity of contrast sensitivity (CS) loss (in AMD patients) and decreased engagement in physical activity, defined as minutes spent in moderate-to-vigorous physical activity (MVPA) per day. RESULTS: In multivariate negative binomial regression models, 5-decibels worse VF mean deviation was associated with 26 % less engagement in MVPA [rate ratio (RR) = 0.74, p < 0.01] amongst glaucoma subjects. When FoF was added to the model, the RR increased from 0.74 to 0.78, and VF loss severity remained associated with less MVPA at a statistically significant level (p < 0.01). Likewise, 0.1 log units worse CS was associated with 11 % less daily MVPA (RR = 0.89, p < 0.01) amongst AMD subjects. When FoF was added to the model, the RR increased from 0.89 to 1.02, and CS loss was no longer associated with MVPA at a statistically significant level (p = 0.53). CONCLUSIONS: FoF may mediate the relationship between vision loss and physical activity restriction amongst patients with AMD. Future work should determine optimal strategies for reducing FoF in individuals with vision loss in order to prevent the deleterious effects of physical activity restriction.

Evaluation of real-world mobility in age-related macular degeneration.

BACKGROUND: Previous research has suggested an association between poor vision and decreased mobility, including restricted levels of physical activity and travel away from home. We sought to determine the impact of age-related macular degeneration (AMD) on these measures of mobility. METHODS: Fifty-seven AMD patients with bilateral, or severe unilateral, visual impairment were compared to 59 controls with normal vision. All study subjects were between the ages of 60 and 80. Subjects wore accelerometers and cellular network-based tracking devices over 7 days of normal activity. Number of steps taken, time spent in moderate-to-vigorous physical activity (MVPA), number of excursions from home, and time spent away from home were the primary outcome measures. RESULTS: In multivariate negative binomial regression models adjusted for age, gender, race, comorbidities, and education, AMD participants took fewer steps than controls (18% fewer steps per day, p = 0.01) and spent significantly less time in MVPA (35% fewer minutes, p < 0.001). In multivariate logistic regression models adjusting for age, sex, race, cognition, comorbidities, and grip strength, AMD subjects showed an increased likelihood of not leaving their home on a given day (odds ratio = 1.36, p = 0.04), but did not show a significant difference in the magnitude of time spent away from home (9% fewer minutes, p = 0.11). CONCLUSION: AMD patients with poorer vision engage in significantly less physical activity and take fewer excursions away from the home. Further studies identifying the factors mediating the relationship between vision loss and mobility are needed to better understand how to improve mobility among AMD patients.

Reading ability and reading engagement in older adults with glaucoma.

PURPOSE: We evaluated the impact of glaucoma-related vision loss on reading ability and reading engagement in 10 reading activities. METHODS: A total of 63 glaucoma patients and 59 glaucoma suspect controls self-rated their level of reading difficulty for 10 reading items, and responses were analyzed using Rasch analysis to determine reading ability. Reading engagement was assessed by asking subjects to report the number of days per week they engaged in each reading activity. Reading restriction was determined as a decrement in engagement. RESULTS: Glaucoma subjects more often described greater reading difficulty than controls for all tasks except puzzles (P < 0.05). The most difficult reading tasks involved puzzles, books, and finances, while the least difficult reading tasks involved notes, bills, and mail. In multivariable weighted least squares regression models of Rasch-estimated person measures of reading ability, less reading ability was found for glaucoma patients compared to controls (ß = -1.60 logits, P < 0.001). Among glaucoma patients, less reading ability was associated with more severe visual field (VF) loss (ß = -0.68 logits per 5-dB decrement in better-eye VF mean deviation [MD], P < 0.001) and contrast sensitivity (ß = -0.76 logits per 0.1-unit lower log CS, P < 0.001). Each 5-dB decrement in the better-eye VF MD was associated with book reading on 18% fewer days (P = 0.003) and newspaper reading on 10% fewer days (P = 0.008). No statistically significant reading restriction was observed for other reading activities (P > 0.05). CONCLUSIONS: Glaucoma patients have less reading ability and engage less in a variety of different reading activities, particularly those requiring sustained reading. Future work should evaluate the mechanisms underlying reading disability in glaucoma to determine how patients can maintain reading ability and engagement.

Nutritional amblyopia combined with night blindness.

We report the case of an 18-year-old male who developed both nutritional amblyopia and night blindness. After nearly a lifetime of consuming a bizarre diet limited to French fries, pretzels, crackers, and carbonated sodas, he had a relatively sudden onset of night blindness and bilateral visual loss. The night blindness resolved after taking daily oral vitamin A supplements. Visual acuity gradually improved from light perception, both eyes, to 20/20 right eye and 20/25 left eye after multivitamin supplementation and vitamin B(12) injections. The patient had bilateral optic atrophy and bilateral ring scotomas around a small area of fixation. The patient was unable to modify his diet despite professional advice and counseling.

The role of TLR4 in photoreceptor {alpha}a crystallin upregulation during early experimental autoimmune uveitis.

Purpose. Previous studies indicate that the upregulation of alphaA crystallin prevents photoreceptor mitochondrial oxidative stress-mediated apoptosis in experimental autoimmune uveitis (EAU). In this study, the role of TLR4 was investigated in the upregulation of alphaA crystallin in the retinas of animals with EAU. Methods. TLR4(-/-), iNOS(-/-), TNF-alpha(-/-), MyD88(-/-), wild-type (WT) control (C57BL/6), and nude mice (B6.Cg-Foxn1(nu)) were immunized with IRBP mixed with complete Freund's adjuvant; eyes were enucleated on day 7 after immunization. Real-time polymerase chain reaction was first used to detect upregulated inflammatory cytokines and alphaA crystallin in retinas with EAU; confirmed with Western blot analysis, and the site of upregulation was localized by immunohistochemistry. Oxidative stress was localized using 8-OHdG, and TUNEL staining was used to detect apoptosis. Results. In early EAU, increased expression of TNF-alpha, iNOS, and alphaA crystallin genes were detected in the retinas of WT mice, whereas such upregulation was absent in TLR4-deficient mice (P < 0.001). alphaA Crystallin was not elevated in MyD88(-/-), TNF-alpha(-/-), and iNOS(-/-) mice with EAU. Immunostaining revealed TNF-alpha, iNOS, and alphaA crystallin localization in the photoreceptor inner segments and outer plexiform layer in the WT controls with EAU; but such staining was absent in TLR4-deficient mice with EAU. 8-OHdG staining showed oxidative stress in the photoreceptors in WT mice with EAU and there was no apoptosis. Conclusions. TLR4 plays an important role in the upregulation of alphaA crystallin through the interaction of MyD88 and the subsequent generation of TNF-alpha and iNOS in the EAU retina. Such crystallin upregulation may prevent oxidative stress-mediated apoptosis of photoreceptors in uveitis.

Oxidative photoreceptor cell damage in autoimmune uveitis.

Uveitis comprises an extensive array of intraocular inflammatory diseases and often results in irreversible visual loss. Experimental autoimmune uveitis (EAU) is an animal model used to study human uveitis. Both innate and adaptive immune responses are known to mediate retinal damage in EAU. The innate immune response occurs first with activation of toll-like receptors which upregulate inflammatory cytokines, leading to oxidative stress; subsequently, the adaptive immune response results in inflammatory cytokine upregulation and mitochondrial oxidative stress. In early EAU, mitochondrial DNA is damaged before inflammatory cellular infiltration and alters mitochondrial protein levels and the functions of mitochondria in AU. Our recent study confirms the importance of TLR4 in the generation of inflammatory cytokines, initiation of oxidative DNA damage, and induction of mitochondrial oxidative stress. Like EAU, sympathetic ophthalmia also results in photoreceptor mitochondrial oxidative damage. Agents that prevent mitochondrial oxidative stress and photoreceptor apoptosis may help prevent retinal damage and preserve vision in uveitis.