Bilateral Retinal and Lateral Geniculate Nucleus Infarction in an Adolescent With Influenza.

We describe a 13-year-old female with influenza complicated by bilateral vision loss due to retinal and lateral geniculate nucleus (LGN) infarctions. She continues to have near-total vision loss in her left eye 3.5 years later. This is the second reported case of bilateral retinal and LGN infarctions in the setting of influenza. The mechanism of infarction remains to be determined, but it is important to recognize this entity and counsel patients appropriately as visual recovery may be poor.

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.

Update on the Treatment of Retinoblastoma.

Retinoblastoma (Rb) is the most common pediatric ocular malignancy and accounts for 2% of all childhood cancers. Rb is initiated by a mutation of the RB1 tumor suppressor gene and occurs in 2 forms: 1) unilateral and unifocal, characterized by a single tumor in 1 eye, and 2) bilateral or unilateral, multifocal Rb with multiple tumor foci in 1 or both eyes. Rb is a disease of young children and if left untreated can result in visual morbidity as well as systemic mortality. Fortunately, because of the greater availability of genetic testing and earlier diagnosis, novel targeted therapies, and multimodal treatment approaches, disease-free survival rates and visual prognoses have improved dramatically. Current efforts to expand the accessibility of the newest Rb treatments aim to improve Rb outcomes worldwide. In this article, we will review the clinical presentation, diagnosis, and management of Rb, with a focus on the newest treatment approaches.

Repeatability and Reliability of Quantified Ultrasound Biomicroscopy Image Analysis of the Ciliary Body at the Pars Plicata.

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.

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.

Pediatric Corneal Structural Development During Childhood Characterized by Ultrasound Biomicroscopy.

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.].

Back to the Future: Vitreous Pupillary Block and Glaucoma in a Child After Lensectomy.

The authors present a case of poorly controlled glaucoma following lensectomy. An examination under anesthesia revealed iris bombe of the anterior vitreous face. This case helps emphasize the importance of a generous anterior vitrectomy at the time of lensectomy in the pediatric population to avoid secondary glaucoma. [J Pediatr Ophthalmol Strabismus. 2020;57:e48-e50.].

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.

Visual outcomes in children with neurofibromatosis type 1 and orbitotemporal plexiform neurofibromas.

PURPOSE: To describe the visual outcomes and volumetric magnetic resonance imaging (3D MRI) in children with neurofibromatosis type 1 (NF1) and orbitotemporal plexiform neurofibromas. DESIGN: Multicenter retrospective case series. METHODS: Two institutions with dedicated NF1 clinical research programs queried their established clinical databases for children with orbitotemporal plexiform neurofibromas. Visual acuity, refractive error, ambylopia, and treatment history were abstracted. Extent of orbitotemporal plexiform neurofibroma involvement was assessed clinically and with 3D MRI analysis. Children with optic pathway gliomas or ocular causes of decreased visual acuity (ie, cataracts, glaucoma) other than strabismus or anisometropia were excluded. RESULTS: Twenty-one children met inclusion criteria (median age 8 years, range 0.33-23 years). Orbitotemporal plexiform neurofibroma location was classified as isolated eyelid (n = 6), eyelid and orbit (n = 7), orbit and temporal region (n = 7), or diffuse orbit (n = 1). Three subjects had bilateral orbital involvement. Amblyopia secondary to the orbitotemporal plexiform neurofibroma was present in 13 subjects (62%) and was caused by strabismus (n = 2, 10%), occlusion from ptosis (n = 9, 43%), or anisometropia (n = 9, 43%), or a combination of factors (n = 6, 29%). MRI-derived volumes were measured in 19 subjects (median 41.8 mL, range 2.7-754 mL). All subjects with amblyopia had orbitotemporal plexiform neurofibroma volumes greater than 10 mL. CONCLUSION: In our series, amblyopia occurs in more than half of NF1 children with orbitotemporal plexiform neurofibromas, most commonly because of ptosis and anisometropia. The 3D MRI analysis allowed for sensitive measurement of orbitotemporal plexiform neurofibroma size, and larger volumes were associated with development of amblyopia.

Recent developments in the field of superior oblique palsies.

PURPOSE OF REVIEW: The superior oblique muscle is a complex structure that evidences considerable variation in its anatomy as well as its rotational effects upon the eye. Our understanding and treatment of patients with superior oblique dysfunction are complicated by our incomplete, though developing, understanding of the sensory and motor adaptations to these dysfunctions. We review articles published over the previous year, which further our understanding of these issues. RECENT FINDINGS: A thought-provoking three-part series, using monkeys this past year, may provide some insights into time-dependent changes after superior oblique palsy as well as stimulate some interesting conjecture about what, if any, role proprioception may have in strabismus patients. There are some studies, which further our understanding of the torsional effects in superior oblique palsy, the fusional capabilities of normal and palsied patients, and the changes that our surgeries create in the torsional state of the eye. Another case of an ocular torticollis associated with plagiocephaly is presented this year. This past year also saw continuing attempts to determine the best methods for surgically correcting a superior oblique palsy. SUMMARY: Superior oblique muscle dysfunction continues to stimulate research into its complex and varied clinical manifestations.

A review of pediatric uveitis: part II. Autoimmune diseases and treatment modalities.

Uveitis is a manifestation of complex processes that can represent an infectious process or a dysfunction of the immune system that may have grave effects on the eye. Although infectious causes, once properly identified, may be successfully treated by addressing the inciting organism with recognized interventions, the immune-modulated chronic forms of uveitis often provide more complex challenges in management. Recent strides in understanding the inflammatory pathway and better bioengineering capabilities have resulted in some new modalities of treatment.

A review of pediatric uveitis: Part I. Infectious causes and the masquerade syndromes.

Uveitis is a manifestation of complex processes that can represent an infectious or a purely immune system modulated condition and may have grave effects on the eye. Much of the morbidity in these conditions is the result of the immune response to these stimuli. These infectious diseases may be successfully treated by addressing the inciting organism with recognized interventions. Treatment of the immune response to the organism often must be pursued simultaneously to minimize long-term complications caused by structural changes within the eye. Assisting the individual's immune response to eliminate the organism while minimizing the immune response's damaging effects remains a unique challenge drawing on both the science and the art of medicine. Several non-infectious conditions that are not autoimmune diseases may commonly masquerade as uveitis, leading to delays in appropriate treatment.