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BACKGROUND: Postnatal exposure to red blood cell transfusion (RBCT) in premature infants is an important risk factor for Retinopathy of Prematurity (ROP) progression. We hypothesized that higher number and earlier timing of RBCT are associated with worse ROP severity and result in laser treatment at an earlier postmenstrual age (PMA) in very low birth weight (≤1500 g, VLBW) infants. METHODS: A retrospective medical record review of 631 VLBW infants over a 5-year period was performed. Demographic features and potential clinical risk factors including number of RBCT, ROP severity, and progression to laser treatment were collected to evaluate predictors of severe ROP. ANCOVA, pairwise post-hoc analyses, and multivariate regression were used to determine associations between frequency and timing of RBCT and ROP severity. RESULTS: Of the 456 eligible infants, 61 developed severe ROP (13%). There was significant correlation between number of RBCTs and ROP severity, adjusted for gestational age and birthweight (Adjusted R2 = 0.53; p

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.

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.

Objectives To determine the repeatability of corneal measurements from anterior segment optical coherence tomography (AS-OCT) images using ImageJ software in healthy eyes compared with eyes with keratoconus. Methods Anterior segment OCT images of 25 eyes from 14 healthy subjects and 25 eyes from 15 subjects with keratoconus between the ages of 20 and 80 years were evaluated. Two trained observers used ImageJ to measure the central corneal cross-sectional area and anterior and posterior corneal arc lengths. MedCalc statistical software was used to generate the intraclass correlation coefficient (ICC) and Bland-Altman plots (BAPs) for observer measurements. Results Observer measurements of the central corneal cross-sectional area and anterior and posterior corneal arc lengths yielded an ICC >0.7. The ICC comparing the 3 parameters ranged from 0.75 to 0.84 for the control and 0.96 to 0.98 for the keratoconus group. No systematic proportional bias was detected by the BAPs. There were minimal differences between the 2 observer's measurements, with a mean of the difference of 0.3 mm2, 0 mm, and 0 mm, for the 3 measurements, respectively. Conclusions This study suggests that ImageJ software is a repeatable and reliable tool in the analysis of corneal parameters from AS-OCT images among patients with keratoconus and may be applicable to AS-OCT imaging protocol development, an area of active keratoconus research.

Most of the ciliary body and ciliary processes of the eye cannot be directly visualized in vivo because of the posterior location of the pars plicata to the posterior chamber and iris. However, ciliary anatomy can be effectively imaged using ultrasound biomicroscopy (UBM) by placing the probe close to the limbus, perpendicular to this structure. Previous studies measuring ciliary body parameters in meridian UBM images found that these parameters were measured with poor reliability and repeatability. This study evaluates the intra-observer reliability and inter-observer agreement of a standardized protocol for measuring six ciliary parameters in transverse or quadrant UBM images that capture an entire row of ciliary processes. All six ciliary parameters have high intra-observer reliability, with ciliary body thickness, ciliary process length and ciliary process density measurements being the most consistent for each observer. The coefficient of variation for each observer ranged from 1.4%-15%. Inter-observer agreement was also high for all six parameters, with an intra-class correlation coefficient >0.8. Utilizing transverse UBM images of the pars plicata allows for consistent quantitative analysis in control subjects.

Purpose: To assess the repeatability and reliability of semi-automated EyeMark Python program measurements compared to manual ImageJ image processing of anterior segment-optical coherence tomography (AS-OCT) structures in healthy and keratoconus eyes. Methods: Heidelberg AS-OCT was used to image 25 eyes from 14 healthy subjects and 25 eyes from 15 subjects with keratoconus between the ages of 20 and 80 years, collected prospectively, in this observational case-control study. Visual axis scan containing vertical fixation light beam was selected from the 15-line AS-OCT scan raster. Central corneal thickness (CCT), anterior corneal radius of curvature (ACRC), posterior corneal radius of curvature (PCRC), and truncated anterior vault (TAV) were measured using ImageJ software and the EyeMark Python program. MedCalc and R were used to calculate the intraclass correlation coefficient (ICC) and generate Bland-Altman plots (BAP). Results: When comparing the measurements of CCT, ACRC, PCRC, and TAV between manual ImageJ analysis and the EyeMark Python program, ICC values were consistently greater than 0.9, indicating excellent agreement. BAPs comparing the ImageJ and Python measurements of anterior segment structures show no systematic proportional bias and the average differences were near zero and within 95% of the limits of agreement. Conclusions: Semi-automated tools may provide the necessary efficiency for point-of-care quantitative corneal analysis of raw AS-OCT images. The semi-automated EyeMark Python program offers a repeatable and reliable tool compared to manual ImageJ analysis for measuring anterior segment structures from AS-OCT images among individuals with keratoconus.

The study was a prospective observational study comparing semi-automated 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. Interclass correlation coefficients and correlation coefficients were greater than 0.8 for all parameters, and comparison of respective analysis speed was 7 times faster for the semi-automated method compared to manual image quantification.

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.摘要: 对于复杂的虹膜睫状体结构以及合并角膜混浊或不透明的眼前节进行非侵入性、高分辨率成像, 超声生物显微镜(UBM)是唯一选择。虽然这些特征使UBM对于特定类型的创伤、先天性畸形和眼前节肿瘤的诊断是必要的, 但人们已经在广泛的疾病谱中, 发现了UBM成像在结构评估中的临床用途。其不仅可用于眼内前节解剖, 也可用于眼睑和眼眶解剖的评估。在儿童群体中, 无论伴有角膜混浊或是云翳的眼前节疾病, 该成像工具都有非常特殊的地位。对于儿科患者的诊断, 医生会面临额外的挑战。儿童通常无法提供详细病史也无法全力配合相关检查, 因此对高分辨率成像的需求更大。本综述的目的是针对涉及应用UBM对儿童眼病进行描述、评估、诊断或优化治疗的文献进行筛选与整合。所筛选出的研究详细说明了这种成像方式的用途, 阐明了最适用于该工具的结构和疾病, 并描述了儿童受试者UBM成像的定量和定性特征。同时, 本文得出的结论将包括可用于加强儿童眼病临床护理的具体应用。.

Purpose: To quantitatively describe the structural corneal changes from infancy to early adulthood using ultrasound biomicroscopy. Methods: In this prospective study, 168 ultrasound biomicroscopy images were obtained from 24 healthy eyes of 24 patients who consented and enrolled in the Pediatric Anterior Segment Imaging Innovation Study. Their ages ranged from birth to 26 years. An established ultrasound biomicroscopy imaging protocol including seven views of one eye per patient were obtained and measured using ImageJ software (National Institutes of Health). Twelve corneal structural parameters were measured. Means were compared between younger and older groups. Results: Among the 12 measured structures, 5 demonstrated statistically significant differences ( P < .05) between patients younger than 1 year and patients older than 1 year. The mean values for corneal cross-sectional width and length, central corneal thickness, and radii of curvature (anterior and posterior) were significantly different in patients younger than 1 year. Curvature and limbus-to-limbus dimensions changed more dramatically than thickness and tissue density. When comparing the youngest to oldest subgroups, anterior curvature flattened (6.14 to 7.55 radius), posterior curvature flattened (5.53 to 6.72 radius), angle-to-angle distance increased (8.93 to 11.40 mm), and endothelial cross-sectional distance increased (10.63 to 13.61 mm). Conclusions: Pediatric corneal structures change with age. The most significant changes occur in the first months of life, with additional changes later in childhood. This study further demonstrates the importance of age in pediatric corneal imaging analysis. [ J Pediatr Ophthalmol Strabismus. 2020;57(4):238–245.]

We describe the emergency management of a man who experienced acute vision loss diagnosed as direct traumatic optic neuropathy (TON) in his right eye (no light perception) after falling from a height. TON is caused by a high-impact mechanism of injury. Clinical findings include acute vision loss, which is typically immediate, afferent pupillary defect, decreased color vision, and visual field defects. Treatment is controversial because of the lack of strong evidence supporting intervention over observation. In this case report, our treatment strategy comprised immediate hyperbaric oxygen (HBO2) and daily high doses of a steroid. On the second day, minocycline was added to the treatment regimen for its neuroprotective effects. The patient was discharged after receiving six HBO2 treatments and six days of intravenous solumedrol transitioned to oral prednisone. After the third HBO2 treatment, his vision improved to 20/100; after the fourth treatment, it was 20/40 and plateaued. At the time of discharge, it was 20/40. At two-month follow-up, his corrected visual acuity was 20/60+2 in the affected eye. Immediate HBO2 for ischemic and mechanical injury to the optic nerve following trauma is a therapeutic option.

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.

An extremely low-birthweight female newborn, weighing 545 g, is born at 23 weeks of gestation via cesarean section. Following a pregnancy complicated by preterm labor with prolonged rupture of membranes and positive maternal group B Streptococcus status, the newborn requires positive pressure ventilation, chest compressions, intubation, and surfactant administration in the delivery room. After initial stabilization, she is admitted to the NICU. She receives continuous positive pressure ventilation on day 37 after birth and is ultimately weaned to room air. She initially receives enteral feeds with breast milk and gradually advances to full enteral feeds by day 40 after birth. Within a week of tolerating full enteral feeds, she makes a transition to 24 kcal/oz of formula because of insufficient maternal breast milk supply. Her eyes are regularly screened for retinopathy of prematurity (ROP) from 31 weeks’ postmenstrual age (PMA). Before each ROP screening, she receives cyclopentolate/phenylephrine and proparacaine ophthalmic eye drops. Her initial eye examination reveals bilateral zone 1 stage 1 ROP. After the diagnosis of bilateral zone 2 stage 3 ROP with plus disease is made at PMA of 36 weeks, she is successfully treated with laser ablation therapy to the peripheral avascular retina of both eyes after a brief period of having nothing orally for 4 hours. Her enteral feeds are resumed later on the same day after laser surgery. She is subsequently started on daily ophthalmic 0.5% atropine drops and tobramycin/dexamethasone ophthalmic drops 4 times a day. On postoperative day 1, she is noted to have tachycardia with pulses ranging from 190 to 200 beats/min 1 hour after ophthalmic atropine is applied. She continues to have intermittent tachycardia, but enteral feeds are continued as she continues to have normal physical examination …

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.

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.

Niacin, a treatment for dyslipidemia, is known to induce vasodilation as a secondary effect. Previous instances of patients with chronic central retinal vein occlusion (CRVO) and cystoid macular edema (CME) have been observed to spontaneously improve when placed on systemic niacin for hypercholesterolemia. The purpose of this study was to evaluate the effects of niacin on CRVO and associated ocular complications. A prospective, single-center, non-randomized, interventional case series of niacin for CRVO was conducted. Best-correct visual acuity (BCVA), central macular thickness (CMT), and ocular complications were analyzed in 50 patients over 1 year. Eight patients were controls. The mean initial logMAR BCVA was 0.915, and improved with niacin to 0.745 (P = 0.12), 0.665 (P = 0.02) and 0.658 (P = 0.03) after 3, 6, and 12 months of follow-up, respectively. At baseline, mean CMT was 678.9 μm, and improved to 478.1 μm (P = 0.001), 388.6 μm (P

An 88-year-old woman with a history of complicated cataract extraction by phacoemulsification with sulcus intraocular lens (IOL) placement developed pseudophakic pupillary block after reverse implantation of the IOL. In the postoperative period, she had persistently elevated intraocular pressures (IOP) and was diagnosed with chronic angle-closure glaucoma that was treated medically. She presented 5 years later with acute pupillary block. The diagnosis of reverse IOL implantation was confirmed by ultrasound biomicroscopy. An IOL repositioning, anterior synechialysis, and Baerveldt tube shunt placement led to resolution of the pupillary block and a well-controlled IOP.

The antiepileptic drug vigabatrin is known to cause permanent loss of vision. Both visual field testing and electroretinogram are used to detect retinal damage. Adult data on optical coherence tomography (OCT) shows that retinal nerve fiber layer (RNFL) thinning may be an early indicator of vigabatrin-induced retinal toxicity. The purpose of this study was to investigate whether OCT can detect early vigabatrin-induced retinal toxicity in children.Pediatric patients (≤18 years of age) requiring vigabatrin for seizure control who were followed at our institution were invited to participate. Patients were examined according to manufacturer guidelines, with most examinations taking place under general anesthesia. RNFL thickness was measured by OCT (Stratus Model 3000, Zeiss) and compared to total cumulative dose of vigabatrin. In most cases, indirect ophthalmoscopy, fundus photography, and electroretinography were also performed.OCT and complete dosing data was available for 19 patients. Patients with tuberous sclerosis (TS, n = 12) received higher cumulative doses (mean, 1463 g) than non-TS patients (mean, 351 g, P = 0.044). RNFL thinning was detected in the nasal (P 0.01), superior (P 0.01), and inferior (P 0.05) quadrants in patients with TS, particularly once cumulative dose exceeded 1500 g.In our study population of patients with TS, higher cumulative doses of vigabatrin were associated with RNFL thinning in the nasal, superior, and inferior quadrants. These findings were pronounced once cumulative dose exceeded 1500 g. This pattern of RNFL thinning is similar to what has been shown in adult patients taking vigabatrin.

In this novel study, we demonstrate a standardized imaging and measurement protocol of anterior segment (AS) structures with reliability analysis using ultrasound biomicroscopy (UBM) and ImageJ software.Ten pediatric and young adult patients undergoing examination under anesthesia for AS pathology were imaged using UBM. Four trained observers analyzed 20 images using ImageJ. Forty-five structural parameters were measured. Those that relied on the trabecular-iris angle (TIA) as a reference landmark were labeled TIA-dependent (TD) and all others were labeled non-TIA dependent (NTD). Intra-observer repeatability (IOR) and inter-observer agreement (IOA) of measurements were determined using coefficient of variation (CV) and intra-class correlation (ICC) followed by assessment of Bland-Altman plots (BAP) for each pair of observers, respectively.For NTD parameters, non-ciliary body (CB) related measurements showed CV range 0.60-16.22% and ICC range 0.84-0.89, whereas CB-related parameters showed CV range 2.86-23.40% and ICC range 0.29-0.92. For TD parameters, parameters 2 degrees removed from reference showed CV range 0.02-5.40% and ICC range 0.89-1.00, whereas parameters 1 degree removed showed CV range 0.63-27.44% and ICC range 0.22-1.00. No systematic proportional bias was detected by BAPs.Preplaced landmarks yielded good IOR and IOA in quantitative assessment of AS structures that were NTD and non-CB-related or less removed from the reference. CB-related NTD measurements varied greatly in IOR and IOA, indicating protocol modifications or CB qualitative assessments needed to improve accuracy. Variability in TD measurements increased the further removed from the reference, which supports implementation of a reliable reference landmark to minimize variation.

A 3-month-old boy with primary congenital glaucoma developed a transient corneal endothelial opacity after needle bleb revision with adjunctive 5-fluorouracil. This case presents a rare toxicity that, although transient, is concerning due to the potential of amblyopia. The authors review prior cases of corneal opacity associated with 5-fluorouracil. [ J Pediatr Ophthalmol Strabismus . 2016;53:e54–e57.]

Pediatric uveitis is relatively uncommon, accounting for only 5-10% of all patients with uveitis. However, owing to high prevalence of complications and devastating outcomes, its lifetime burden can be significant.Immunomodulatory therapy has been associated with better outcomes in noninfectious pediatric uveitis. However, effective treatments are limited by medication-related complications, including multiorgan toxicities and systemic side effects.We review the current therapies available to treat pediatric uveitis, discuss novel and future therapies, and provide clinical recommendations utilizing these new agents. The consideration for treatment regimens in noninfectious pediatric uveitis is multifactorial. Understanding past, present, and future technology will aid in treatment of a complex and refractory disease.

We report the case of a 7-year-old girl who presented with bilateral anterior uveitis, acute interstitial nephritis, and asymptomatic pulmonary granulomas and provide novel clinical evidence of an association between tubulointerstitial nephritis and uveitis syndrome and sarcoidosis.

Frosted branch angiitis is a rare vascular reaction believed to be a nonspecific immune response to an infective, neoplastic, or idiopathic insult. The clinical presentation is characteristic and typically affects children and younger adults, and the prognosis is good. We report a case of frosted branch angiitis during immune recovery in a 2-year-old boy with Langerhans cell histiocytosis on systemic immunosuppressive therapy.