Association of Speckle-Based Blood Flow Measurements and Fluorescein Angiography in Infants with Retinopathy of Prematurity.

Purpose: To determine the correlation between blood flow metrics measured by intravenous fluorescein angiography (IVFA) and the blood flow velocity index (BFVi) obtained by laser speckle contrast imaging (LSCI) in infants with retinopathy of prematurity (ROP). Design: Prospective comparative pilot study. Subjects: Seven eyes from 7 subjects with ROP. Methods: Unilateral LSCI and IVFA data were obtained from each subject in the neonatal intensive care unit. Five LSCI-based metrics and 5 IVFA-based metrics were extracted from images to quantify blood flow patterns in the same region of interest. Correlation between LSCI-based and IVFA-based blood flow metrics was compared between 2 subgroups of ROP severity: moderate ROP (defined as stage ≤ 2 without Plus disease) and severe ROP (defined as stage ≥3 or Plus disease). Main Outcome Measures: Pearson and Kendall rank correlation coefficients between IVFA and LSCI metrics; Student t test P values comparing LSCI metrics between "severe" and "moderate" ROP groups. Results: Pearson correlations between IVFA and LSCI included arterial-venous transit time (AVTT) and peak BFVi (pBFVi; r = -0.917; P = 0.004), AVTT and dip BFVi (dBFVi; r = -0.920; P = 0.003), AVTT and mean BFVi (r = -0.927- P = 0.003), and AVTT and volumetric rise index (r = -0.779; P = 0.039). Kendall rank correlation between AVTT and dBFVi was r = -0.619 (P = 0.051). pBFVi was higher in severe ROP than in moderate ROP (8.4 ± 0.6 and 4.4 ± 1.8, respectively; P = 0.0045 using the 2-sample t test with pooled variance and P = 0.0952 using the Wilcoxon rank-sum test). Conclusions: Correlation was found between blood flow metrics obtained by IVFA and noninvasive LSCI techniques. We demonstrate the feasibility of obtaining quantitative metrics using LSCI in infants with ROP in this pilot study; however, further investigation is needed to evaluate its potential use in clinical assessment of ROP severity. Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Eye Disease Prevalence and VF-14 Validation Among Patients Experiencing Homelessness and Presenting for Ophthalmic Examination in Baltimore, Maryland.

Purpose: The coronavirus disease 2019 (COVID-19) pandemic is projected to drive 1.5 million Americans toward homelessness, adding to the 3.5 million currently affected. Homelessness poses both socioeconomic and public health challenges because housing status is a social determinant of health. Given ophthalmic health's importance in daily functioning, we characterized ophthalmic disease and vision-related quality of life (VRQOL) among a population experiencing homelessness in Baltimore, Maryland. Methods: Questionnaires, including a Visual Function Index-14 (VF-14) for measuring VRQOL, were administered among patients seeking eye examinations at Health Care for the Homeless (HCH) from October 2018 to March 2020. Results: One hundred sixty-two participants were enrolled in this study. The average age was 53 years. Participants' most common vision concerns were blurry vision (70%) and desire for glasses (52%). Best corrected visual acuity (BCVA) measurements revealed significant vision loss (18%, P < 0.001). Physicians mostly diagnosed refractive error (77%), cataracts (36%), glaucoma/glaucoma suspect (25%), and dry eye (24%). Nearly half were referred to additional ophthalmic care (46%). VRQOL trends reflected functional vision categories (P = 0.042 and P = 0.021). The 1:1 VRQOL and BCVA comparison showed correlation (rho = -0.3, P < 0.001). Cronbach's alpha demonstrated VF-14 reliability (alpha = 0.92). Conclusions: We find high ophthalmic disease prevalence within a population experiencing homelessness. Comparison to studies worldwide reveals healthcare disparities despite healthcare system differences, suggesting a need for more targeted solutions. VF-14 is valid and reliable in assessing those experiencing homelessness. Intragroup VRQOL comparisons may reveal subgroup needs. It is imperative that future studies continue monitoring those experiencing homelessness. Translational Relevance: Validation of VF-14 will allow future studies to utilize this patient-oriented metric within populations experiencing homelessness.

Multimodal prenatal and postnatal imaging of microphthalmia with cyst.

Congenital ocular anomalies may be detected on prenatal imaging using fetal ultrasound and magnetic resonance imaging (MRI), although standard prenatal ultrasound for fetal physical development does not currently include ocular and orbital evaluation. We present the case of a male infant born at 39 weeks' gestation with microphthalmia with cyst that was characterized using serial multimodal imaging, including fetal ultrasound and MRI, B-scan ultrasonography, ultrasound biomicroscopy, and postnatal MRI. Multiple prenatal and postnatal imaging modalities yielded comparable evaluations of the ocular and orbital pathology, validating the prenatal assessments.

Surgical Management of Iris Bombe in Muscle-Eye-Brain Disease.

A 2-year-old girl with severe muscular dystrophy presented with unilateral eye pain and corneal clouding. She was found to have absent red reflex, hypotonia, cerebral hypoplasia, and iris bombe on ultrasound biomicroscopy, a feature not previously reported in this syndrome. She responded favorably to surgical management. Iris bombe can be a cause of glaucoma in muscle-eye-brain disease. This highlights the importance of incorporating ultrasound biomicroscopy into the diagnostic algorithm of muscle-eye-brain disease and other types of congenital syndromic glaucoma. [J Pediatr Ophthalmol Strabismus. 2023;60(4):e35-e37.].

Quality assessment of polymer materials for human model eye development.

PURPOSE: We developed model eyes using six polymer materials to determine which materials were most appropriate in simulating real human sclera and extraocular muscle (EOM). METHODS: Five three-dimensional (3-D) printed polymers (FlexFill, PolyFlex, PCTPE, Soft PLA, and NinjaFlex) and one silicone material were systematically tested by board-certified ophthalmologists and senior ophthalmology residents. Material testing included scleral passes with 6-0 Vicryl sutures through each eye model. Participants completed a survey designed to collect demographic data, subjective assessment of each material's accuracy in simulating real human sclera and EOM, and a ranking for each polymer material to identify which would be most suitable for an ophthalmic surgery training tool. The Wilcoxon signed-rank test was conducted to determine if there was a statistically significant difference in the distribution of ranks between the polymer materials. RESULTS: The distribution of ranks for silicone material's "sclera" and "EOM" components were statistically significantly higher than that of all other polymer materials (all p < 0.05). Silicone material received the highest rank for both "sclera" and "EOM" components. Survey results indicated that the silicone material effectively simulated real human tissue. CONCLUSION: Silicone model eyes performed better than 3-D printed polymers as an educational tool for incorporation into a microsurgical training curriculum. Silicone models provide a low-cost teaching tool that allows for independent practice of microsurgical techniques without requiring a wet-laboratory facility.

Congenital ankyloblepharon in a newborn with an IRF6 mutation.

We present the case of a boy born at 41 weeks' gestational age who was found to have multiple anatomic anomalies, including abnormalities of the oral cavity, eyelids, and digits. He had ankyloblepharon that was localized to the lateral portion of the palpebral fissure bilaterally. Genetic testing confirmed a mutation in the interferon regulatory factor 6 (IRF6) gene, a known etiology for a spectrum of rare disorders that includes eyelid abnormalities. We present a novel surgical technique for bedside ankyloblepharon repair and describe the relevant clinical features of this case.

Retinal hemorrhages in abusive head trauma with atraumatic neuroimaging.

We report 3 cases of suspected abusive head trauma with retinal hemorrhages on fundus examination and neuroimaging findings not necessarily suggestive of shaking injury. Previous studies have suggested that retinal hemorrhages are rare in patients without neuroimaging abnormalities. These cases demonstrate some common features (rib fractures, developmental delay, and history of abuse) that may increase suspicion for abusive head trauma. Our findings suggest a potential role for ophthalmic consultation in scenarios with high clinical suspicion for abusive head trauma without definitive neuroimaging evidence of head injury. The nonspecific neuroimaging features of these 3 cases highlight the importance of interpreting cases with global clinical context.

Baltimore pediatric ocular trauma study: Health disparities and outcomes in pediatric and adolescent open globe trauma.

Purpose Children represent approximately one-third of patients with serious ocular injuries. Our study evaluates associations between race and socioeconomic status in presentation and outcomes of pediatric and adolescent traumatic open globe injuries. Methods We conducted a retrospective chart review of traumatic open globe injuries in pediatric and adolescent patients presenting to Johns Hopkins Hospital and University of Maryland Medical Center between 2006 and 2020. Variables assessed included age, gender, parent-identified race, median household income, mechanism of injury, initial and final visual acuity (VA), and length of follow-up. Results Eighty patients ranging from 4 months to 17.7 years (mean 9.3 years) presented with traumatic open globe injury. Identifications were 28 White (35%), 38 Black (48%), and 5 Hispanic (6%). Initial presenting and final VA, pediatric ocular trauma score (POTS), and length of follow-up did not differ significantly among race, gender, or income. Black patients had higher rates of blunt trauma (odds ratio (OR) 3.81; 95% confidence interval (CI) 0.95-15.24, p = 0.07), uveal prolapse (OR 3.58; 95% CI 1.03-12.43; p = 0.049), and enucleation (OR 10.55; 95% CI 1.26-88.31). Hispanic patients presented at a younger age of 2.8 years mean age vs. 9.9 years (p = 0.004) for others. Conclusion Visual outcomes following traumatic open globe injury were independent of race, gender, or income. However, blunt trauma, uveal prolapse, and enucleation rates were higher in Black patients, and ocular trauma occurred at a younger age in Hispanic patients.

A novel method for strabismus surgery education using an asynchronous video module.

Conjunctival closure is an important step in strabismus surgery and a fundamental surgical skill that requires dexterity and understanding of general surgical principles. Traditionally, ophthalmology residents have improved their surgical technique in supervised wet labs. Social distancing guidelines during the COVID-19 pandemic may have limited the ability for direct supervised surgical teaching. We developed a safe, reusable, low-cost teaching module that allows residents to train independently to develop skills necessary for conjunctival closure. This module uses stepwise teaching and video instruction to improve resident confidence and preparedness in conjunctival suturing.

A Novel Interactive Virtual Medical Student Clinical Rotation for Ophthalmology.

Objective This article describes a novel clinical rotation that uses technology to create a remote ophthalmology learning experience with the goal of improving virtual exposure to medical and surgical ophthalmic training for medical students. Methods Our unique curriculum incorporates mobile-mounted tablets which allow students to virtually participate in inpatient consults, clinic, and ophthalmic surgery. An adaptable mounting device attached to the slit lamp allows students to observe examinations in real time, enhancing recognition of ocular pathologies. Students participate in a robust curriculum that includes independent learning modules, video lectures, interactive modules, podcasts, and surgical video rounds. Students engage with residents and faculty in interactive-guided lectures and case-based discussions that focus on the American Academy of Ophthalmology white paper teaching objectives. Students are mailed surgical instruments and participate in surgical modules and faculty-led virtual wet laboratories. Results Our unique virtual curriculum combines didactic learning, interactive content, and novel technology applications such as mobile tablets, slit lamp-mounted devices, and faculty-led virtual wet laboratories. Conclusion Virtual technologies can be utilized to enhance ophthalmology medical student education in a safe and effective way during the coronavirus disease 2019 pandemic, and to improve educational access in the future.

A Novel Method for Teaching Laser Peripheral Iridotomy and Selective Laser Trabeculoplasty Using Video Instruction and Model Eyes.

Objective The aim of the study is to describe an inexpensive and easily-constructed model eye for the purpose of teaching laser peripheral iridotomy (LPI) and selective laser trabeculoplasty (SLT) to ophthalmology residents. Methods Easily constructed, inexpensive model eyes were utilized to teach residents SLT and LPI utilizing a remote self-study module. A teaching microscope attachment allowed for video-based instruction and feedback. Results This model eye, used in conjunction with video modules is an effective low-cost teaching tool for laser surgery among ophthalmology residents. Attending ophthalmologists rated the use of these model eyes using surveys and found them to be appropriate teaching tools that could lead to improved knowledge and translate to better patient care. Conclusion Our novel method for teaching glaucoma laser surgery allows residents to learn the principles and theory behind common laser procedures while having the opportunity to practice repetitive procedures on low-cost model eyes.

Corneal Structural Changes in Congenital Glaucoma.

OBJECTIVE: To identify corneal structure differences on quantitative high-frequency ultrasound biomicroscopy (UBM) among subjects with congenital glaucoma compared with controls. METHODS: This prospective case-control study evaluated 180 UBM images from 44 eyes of 30 subjects (18 control and 12 glaucoma, mean age 5.2±8.0 years, range 0.2-25.8 years) enrolled in the Pediatric Anterior Segment Imaging and Innovation Study (PASIIS). ImageJ was used to quantify a comprehensive set of corneal structures according to 21 quantitative parameters. Statistical analysis compared corneal measurements in glaucoma subtypes and age-matched controls with significance testing and mixed effects models. RESULTS: Significant differences between congenital glaucoma cases and controls were identified in 16 of 21 measured parameters including angle-to-angle, central and peripheral corneal thicknesses, scleral integrated pixel density, anterior corneal radius of curvature, and posterior corneal radius of curvature. Eight parameters differed significantly between primary congenital glaucoma and glaucoma following congenital cataract surgery. CONCLUSION: Multiple measurable corneal structural differences exist between congenital glaucoma and control eyes, and between primary and secondary congenital glaucoma, including but not limited to corneal width and thickness. The structural differences can be quantified from UBM image analysis. Further studies are needed to determine whether corneal features associated with glaucoma can be used to diagnose or monitor progression of congenital glaucoma.

Spontaneous absorption of congenital cataract in trisomy 21.

We report a case of spontaneous cataract absorption over a period of 19 weeks in a 5-week-old boy with trisomy 21. Ultrasound biomicroscopy images at 5 and 24 weeks of age showed that 71% of the initial lens thickness was absorbed during this period. At surgery, the lens thickness was 0.92 mm in the right eye and 0.91 mm in the left eye. Uncomplicated cataract extraction was performed. Trypan blue staining revealed an area of possible lens leakage through the anterior lens capsule in the left eye, suggesting a mechanism for cataract absorption.

Semiautomated Assessment of Anterior Segment Structures in Pediatric Glaucoma Using Quantitative Ultrasound Biomicroscopy.

The study was a prospective observational study comparing semiautomated to manual quantitative ultrasound biomicroscopy image analysis among 82 images from 41 eyes of 32 subjects (21 controls and 11 glaucoma) enrolled in the Pediatric Anterior Segment Imaging Innovation Study. Intraclass correlation coefficients and correlation coefficients were >0.8 for all parameters, and comparison of respective analysis speed was 7 times faster for the semiautomated method compared with manual image quantification.

Evaluating the Efficacy of Microsurgical Training Methods in Ophthalmology Education: A Systematic Review and Meta-analysis.

Objective The objective of our paper is to review all of the relevant literature in ophthalmology microsurgical education and identify which teaching methodologies were most effective. Methods A systematic review and meta-analysis of the literature was conducted. Electronic databases, including Ovid MEDLINE, Cochrane CENTRAL, and EMBASE, were searched with preset terms. The search was through December 11, 2019. Eligibility criteria included studies with sufficient data for analyzing associations between surgical teaching techniques and success rates in surgical skills and the organization of the intervention as a microsurgical skills course, curriculum, or program. The articles were independently reviewed by two authors. Each included study was evaluated for quality using the Grades of Recommendation, Assessment, Development, and Evaluation (GRADE) approach and risk of bias using the Cochrane Collaboration's tool for assessing bias. Data Extraction and Synthesis Data extraction was performed by two reviewers and disagreements were checked by a third reviewer. A random-effects analysis was used to pool the outcomes of studies. Main Outcomes and Measures Outcomes included time for completion of surgical task, level of preparedness, competency score, and number of surgeries with complications. Results A total of 439 studies were reviewed and 13 studies ( n = 8,790 surgical cases; n = 115 trainees) were included in the meta-analysis. Excluded articles studied cataract simulation training as the primary intervention or were not related to ophthalmology. All pooled results demonstrated a positive association with surgical outcomes; however, video-based education (standardized mean difference [SMD] = 2.49 [95% confidence interval (CI): 0.36-4.63]; four effects [four studies]; n = 69; I 2 = 90%) and stepwise teaching method (odds ratio [OR = 3.84 [95% CI: 2.66-5.55]; six effects [six studies]; n = 6,968; I 2 = 39%) interventions were the most favorable. Conclusion and Relevance The following five interventions evaluated in this paper were found to be effective methods of improving performance outcomes in ophthalmic microsurgery: (1) didactic lectures, (2) video-based education, (3) surgical wet-laboratory, (4) stepwise method, and (5) direct supervision and feedback. Our meta-analysis concludes that video-based education and stepwise teaching interventions are the most effective methods for a microsurgical ophthalmology training curriculum. Combining the strengths of the interventions analyzed in this study should be considered when implementing and adjusting ophthalmic surgical skills curriculums.

Novel Homozygous Missense Variant in GJA3 Connexin Domain Causing Congenital Nuclear and Cortical Cataracts.

Congenital cataracts (CC) are responsible for approximately one-tenth of childhood blindness cases globally. Here, we report an African American family with a recessively inherited form of CC. The proband demonstrated decreased visual acuity and bilateral cataracts, with nuclear and cortical cataracts in the right and left eye, respectively. Exome sequencing revealed a novel homozygous variant (c.563A > G; p.(Asn188Ser)) in GJA3, which was predicted to be pathogenic by structural analysis. Dominantly inherited variants in GJA3 are known to cause numerous types of cataracts in various populations. Our study represents the second case of recessive GJA3 allele, and the first report in African Americans. These results validate GJA3 as a bona fide gene for recessively inherited CC in humans.

A systematic review of ultrasound biomicroscopy use in pediatric ophthalmology.

Ultrasound biomicroscopy (UBM) is the only available option for noninvasive, high-resolution imaging of the intricate iridociliary complex, and for anterior segment imaging with corneal haze or opacity. While these unique features render UBM essential for specific types of trauma, congenital anomalies, and anterior segment tumors, UBM imaging has found clinical utility in a broad spectrum of diseases for structural assessments not limited to the anterior intraocular anatomy, but also for eyelid and orbit anatomy. This imaging tool has a very specific niche in the pediatric population where anterior segment disease can be accompanied by corneal opacity or clouding, and anomalies posterior to the iris may be present. Pediatric patients present additional diagnostic challenges. They are often unable to offer detailed histories or fully cooperate with examination, thus amplifying the need for high-resolution imaging. This purpose of this systematic review is to identify and synthesize the body of literature involving use of UBM to describe, evaluate, diagnose, or optimize treatment of pediatric ocular disease. The collated peer-reviewed research details the utility of this imaging modality, clarifies the structures and diseases most relevant for this tool, and describes quantitative and qualitative features of UBM imaging among pediatric subjects. This summary will include information about the specific applications available to enhance clinical care for pediatric eye disease.

Deep Learning Model for Accurate Automatic Determination of Phakic Status in Pediatric and Adult Ultrasound Biomicroscopy Images.

Purpose: Ultrasound biomicroscopy (UBM) is a noninvasive method for assessing anterior segment anatomy. Previous studies were prone to intergrader variability, lacked assessment of the lens-iris diaphragm, and excluded pediatric subjects. Lens status classification is an objective task applicable in pediatric and adult populations. We developed and validated a neural network to classify lens status from UBM images. Methods: Two hundred eighty-five UBM images were collected in the Pediatric Anterior Segment Imaging Innovation Study (PASIIS) from 80 eyes of 51 pediatric and adult subjects (median age = 4.6 years, range = 3 weeks to 90 years) with lens status phakic, aphakic, or pseudophakic (n = 33, 7, and 21 subjects, respectively). Following transfer learning, a pretrained Densenet-121 model was fine-tuned on these images. Metrics were calculated for testing dataset results aggregated from fivefold cross-validation. For each fold, 20% of total subjects were partitioned for testing and the remaining subjects were used for training and validation (80:20 split). Results: Our neural network trained across 60 epochs achieved recall 96.15%, precision 96.14%, F1-score 96.14%, false positive rate 3.74%, and area under the curve (AUC) 0.992. Feature saliency heatmaps consistently involved the lens. Algorithm performance was compared using 2 image sets, 1 from subjects of all ages, and the second from only subjects under age 10 years, with similar performance under both circumstances. Conclusions: A neural network trained on a relatively small UBM image set classified lens status with satisfactory recall and precision. Adult and pediatric image sets offered roughly equivalent performance. Future studies will explore automated UBM image classification for complex anterior segment pathology. Translational Relevance: Deep learning models can evaluate lens status from UBM images in adult and pediatric subjects using a limited image set.