Pediatric Ophthalmology

Pediatric Ophthalmology Publications

Lu JE, Yoon MK. The Role of Steroids for Pediatric Orbital Cellulitis - Review of the Controversy. Semin Ophthalmol 2023;:1-4.Abstract
Orbital cellulitis in the pediatric population is treated primarily with antibiotic therapy. This leaves the inflammatory component unchecked. Corticosteroid therapy has been used to accelerate recovery and decrease the long-term morbidity in other infectious conditions. Its use has also been proposed for pediatric orbital cellulitis. The aim of this manuscript is to conduct a literature review to summarize existing evidence and understand ongoing controversies. Overall, prior investigations on corticosteroid therapy for pediatric orbital cellulitis are limited by their study design and sample sizes. One of the most discussed potential benefits is that adjuvant steroid therapy for pediatric orbital cellulitis is associated with shorter hospitalization without major infectious complications. However, decreased hospitalization length is an imperfect metric, especially without standardized criteria for hospital discharge. Future studies are warranted to better guide the use of adjuvant steroid therapy and to optimize its potential in the management of pediatric orbital cellulitis.
Yavuz Saricay L, Gonzalez Monroy JE, Fulton AB. Can Nerve Growth Factor (NGF) Be a Treatment Option for Pediatric Eye Diseases?. Semin Ophthalmol 2023;:1-6.Abstract
A critical review of mechanisms of action and pharmacokinetics of nerve growth factor (NGF), including topical administration, and the studies showing the NGF treatment for anterior and posterior segment diseases in adult and pediatric population are summarized in our paper. Nerve growth factor is commonly used for many different ocular conditions in the adult population to promote nerve regeneration or cellular rescue. Clinical trials for recombinant human NGF have also treated several challenging ocular conditions, such as neurotrophic keratopathy, glaucoma, and retinitis pigmentosa with cystoid macular edema. The safety and efficacy of NGF have been demonstrated in pediatric patients as well. This leads us to consider new applications of NGF for the treatment of pediatric eye diseases.
Harris CK, Stagner AM. The Eyes Have It: How Critical are Ophthalmic Findings to the Diagnosis of Pediatric Abusive Head Trauma?. Semin Ophthalmol 2023;38(1):3-8.Abstract
Pediatric abusive head trauma (AHT), still colloquially known as shaken baby syndrome, is a leading cause of morbidity and mortality among infants. Controversy has grown surrounding this diagnosis, and the specificity of the clinical findings-subdural hemorrhage, cerebral edema, and retinal hemorrhages-has been challenged. A literature search of peer reviewed publications on PubMed pertaining to the history, clinical, and pathologic features of AHT was conducted using the terms "shaken baby syndrome," "non-accidental trauma," "abusive head trauma," "inflicted traumatic brain injury," "shaken impact syndrome," and "whiplash shaken infant syndrome." Focus was placed on articles discussing ophthalmic findings in AHT. Retinal hemorrhages-particularly those that are too numerous to count, occurring in all layers of the retina (preretinal, intraretinal, subretinal), covering the peripheral pole and extending to the ora serrata, and accompanied by retinoschisis and other ocular/periocular hemorrhages-are highly suggestive of AHT, particularly in the absence of otherwise explained massive accidental trauma. Although the diagnosis has grown in controversy in recent years, AHT has well-documented clinical and pathologic findings across a large number of studies.
Alnahdi MA, Alkharashi M. Ocular manifestations of COVID-19 in the pediatric age group. Eur J Ophthalmol 2023;33(1):21-28.Abstract
The coronavirus disease 2019 (COVID-19) is now known to be associated with several ocular manifestations. The literature thoroughly discussed those that affect adults, with a lesser focus in the pediatric age group. We aim to outline the various pediatric ocular manifestations described in the literature. The manifestations may be divided into isolated events attributed to COVID-19 or occurring in the new multisystem inflammatory syndrome in children (MIS-C), a novel entity associated by COVID-19 infection. Ocular manifestations have virtually affected all ages. They manifested in neonates, infants, children, and adolescents. Episcleritis, conjunctivitis, optic neuritis, cranial nerve palsies, retinal vein occlusion, retinal vasculitis, retinal changes, orbital myositis, orbital cellulitis were reported in the literature with this emerging viral illness. Conjunctivitis was the most common ocular manifestation in MIS-C in nearly half of the patients. Other ocular manifestations in MIS-C were anterior uveitis, corneal epitheliopathy, optic neuritis, idiopathic intracranial hypertension, and retinitis. The clinical outcome was favorable, and children regain their visual ability with minimal or no deficits in most of the cases. Further follow-up may be warranted to better understand the long-term effects and visual prognosis.
Oke I, Hwang B, Heo H, Nguyen A, Lambert SR. Risk Factors for Retinal Detachment Repair After Pediatric Cataract Surgery in the United States. Ophthalmol Sci 2022;2(4):100203.Abstract
PURPOSE: To determine the cumulative incidence of retinal detachment (RD) repair following pediatric cataract surgery and identify the associated risk factors. DESIGN: US population-based insurance claims retrospective cohort study. PARTICIPANTS: Patients ≤ 18 years old who underwent cataract surgery in 2 large databases: Optum Clinformatics (2003-2021) and IBM MarketScan (2007-2016). METHODS: Individuals with ≥ 6 months of prior enrollment were included, and those with a history of RD, RD repair, traumatic cataract, spherophakia, or ectopia lentis were excluded. The primary outcome was time between initial cataract surgery and RD repair. The risk factors investigated included age, sex, persistent fetal vasculature (PFV), prematurity, intraocular lens (IOL) placement, and pars plana lensectomy approach. MAIN OUTCOME MEASURES: Kaplan-Meier estimated cumulative incidence of RD repair 5 years after cataract surgery and hazard ratios (HRs) with 95% confidence intervals (CIs) from multivariable Cox proportional hazards regression models. RESULTS: Retinal detachment repair was performed on 47 of 3289 children included in this study. The cumulative incidence of RD repair within 5 years of cataract surgery was 2.0% (95% CI, 1.3%-2.6%). Children requiring RD repair were more likely to have a history of prematurity or PFV and less likely to have an IOL placed (all P < 0.001). Factors associated with RD repair in the multivariable analysis included a history of prematurity (HR, 6.89; 95% CI, 3.26-14.56; P < 0.001), PFV diagnosis (HR, 8.20; 95% CI, 4.11-16.37; P < 0.001), and IOL placement (HR, 0.44; 95% CI, 0.21-0.91; P = 0.03). Age at surgery, sex, and pars plana lensectomy approach were not significantly associated with RD repair after adjusting for all other covariates. CONCLUSIONS: Approximately 2% of patients will undergo RD repair within 5 years of pediatric cataract surgery. Children with a history of PFV and prematurity undergoing cataract surgery without IOL placement are at the greatest risk.
Franco JJ, Liu KX, Ioakeim-Ioannidou M, Davila JR, Chen Y-L, Kim IK, Gragoudas ES, Mukai S, MacDonald SM. Low-dose proton radiotherapy for pediatric choroidal hemangioma: A case series. Pediatr Blood Cancer 2022;69(12):e29925.Abstract
Management of pediatric choroidal hemangioma complicated by large exudative retinal detachment can be challenging, with few options available. Limited data have been published on outcomes following proton radiotherapy (PRT) for management of these patients. In this retrospective case series, nine patients were treated with a low-dose PRT regimen of 20 Gy(relative biological effectiveness [RBE]) in 10 fractions, and two were treated with 15 Gy(RBE) in four fractions. Visual acuity improved in seven patients (64%) and remained stable in the remaining four (36%). In patients with imaging follow-up (10 patients), subretinal fluid resolved in nine patients (90%) and tumor thickness decreased or remained stable in 10 (100%). Complications were observed in eight of 11 patients (73%). One patient developed grade 2 cataract; otherwise, no grade ≥2 complications were observed.
Michalak SM, Chinn RN, Shoshany TN, Bishop K, Staffa SJ, Hunter DG. Subthreshold Amblyopia: Characterization of a new cohort. Am J Ophthalmol 2022;Abstract
PURPOSE: Published studies of amblyopia include only patients with visual acuity (VA) worse than 20/40 in one or both eyes. This study evaluates patients diagnosed and treated as amblyopic despite not meeting traditional visual acuity criteria for amblyopia. DESIGN: Retrospective clinical cohort study. METHODS:  SETTING: INSTITUTIONAL PRACTICE. PATIENT POPULATION: All patients diagnosed with amblyopia at Boston Children's Hospital between 2010-2014. INCLUSION CRITERIA: VA better than 20/40 but not correctable to 20/20 in one or both eyes; age 2-12 years. OBSERVATIONS: Demographics, VA, baseline characteristics. OUTCOME MEASURES: Resolution, defined as VA 20/20 in both eyes; stereopsis at last follow-up. RESULTS:  Of 2311 patients reviewed, 464 (20.1%) had subthreshold amblyopia. A majority (61.7%) had an amblyogenic factor, most commonly anisometropia (32.8%). Patients were followed for a median of 3.1 years; nearly all (97.5%) were treated. Of 318 patients who returned for follow-up, 47.8% achieved resolution, including 55.7% of treatment-naïve patients and 62.5% (5 of 8 patients) offered observation alone. Median stereopsis improved by 0.4 log units in those who achieved resolution, with no change in those with persistent amblyopia. In the multivariate analysis, a longer length of follow-up was significantly associated with resolution of subthreshold amblyopia (OR 1.38; 95% confidence interval 1.22 to 1.57, p<.001). DISCUSSION: Patients with subthreshold amblyopia represent a sizeable cohort in real-world amblyopia practice. When offered treatment, half achieved 20/20 vision in both eyes with improved stereopsis as well. Further studies are needed to assess whether observation alone would result in similar outcomes.
Oke I, VanderVeen DK, McClatchey TS, Lambert SR, McClatchey SK, McClatchey SK. The contribution of intraocular lens calculation accuracy to the refractive error predicted at 10 years in the Infant Aphakia Treatment Study. J AAPOS 2022;26(6):294.e1-294.e5.Abstract
PURPOSE: To determine the relative contribution of intraocular lens (IOL) calculation accuracy and ocular growth variability to the long-term refractive error predicted following pediatric cataract surgery. METHODS: Pseudophakic eyes of children enrolled in the Infant Aphakia Treatment Study (IATS) were included in this study. Initial absolute prediction error (APE) and 10-year APE were calculated using the initial biometry, IOL parameters, postoperative refractions, and mean rate of refractive growth. The cohort was divided into children with a low-initial APE (≤1.0 D) and a high-initial APE ( >1.0 D). The 10-year APE was compared between the two groups using the Mann-Whitney U test. Linear regression was used to estimate the variability in prediction error explained by the initial IOL calculation accuracy. RESULTS: Forty-two children with IOL placement in infancy were included. Seventeen eyes had a low initial APE, and 25 eyes had a high initial APE. There was no significant difference in APE 10 years following surgery between individuals with a low initial APE (median, 2.67 D; IQR, 1.61-4.12 D) and a high initial APE (median, 3.45 D; IQR, 1.64-5.10 D) (P = 0.7). Initial prediction error could explain 12% of the variability in the prediction error 10 years following surgery. CONCLUSIONS: IOL calculation accuracy contributed minimally to the refractive error predicted 10 years after cataract surgery in the setting of high variability in the rate of refractive growth.
Adomfeh J, Chinn RN, Michalak SM, Shoshany TN, Bishop K, Hunter DG, Jastrzembski BG, Oke I. Association of Neighborhood Child Opportunity Index with presenting visual acuity in amblyopic children. J AAPOS 2022;Abstract
PURPOSE: To demonstrate the use of a novel measure of neighborhood quality, the Child Opportunity Index (COI), for investigating health disparities in pediatric ophthalmology. METHODS: This study included children 2-12 years of age from a registry of patients diagnosed with amblyopia at an urban pediatric hospital between 2010 and 2014. Children previously treated for amblyopia were excluded. Patient demographics, residential addresses, and logMAR visual acuities were collected. The association between visual acuity at presentation and COI was examined using linear mixed-effects models adjusting for individual-level factors, including age, sex, race, ethnicity, and insurance type. RESULTS: This study included 1,050 amblyopic children, of whom 317 (37%) were non-White and 149 (19%) were Hispanic; 461 (44%) had public insurance. Regarding residence, 129 (12%) lived in areas of very low opportunity (COI <20); 489 (47%) in areas of very high opportunity (COI ≥80). Children residing in the lowest opportunity neighborhoods correctly identified approximately two fewer letters at presentation with their better-seeing eye compared with children from the highest opportunity neighborhoods after adjusting for individual-level factors (-0.0090 logMAR per 20 unit increase in COI; 95% CI, -0.0172 to -0.0008; P = 0.031). No difference was appreciated in the worse-seeing eye. CONCLUSIONS: Amblyopic children residing in communities with low neighborhood opportunity had slightly worse visual acuity in the better-seeing eye at presentation. Although statistically significant in the better-seeing eye, the two-letter difference attributable to neighborhood environment may not be clinically significant, and the impact of this disparity on treatment outcomes deserves further investigation.
Young AK, VanderVeen DK. Controversies in Pediatric Angle Surgery and Secondary Surgical Treatment. Semin Ophthalmol 2022;:1-7.Abstract
Pediatric glaucoma is a constellation of challenging ophthalmic conditions that, left untreated, can result in irreversible vision loss. The mainstay of treatment for primary congenital glaucoma and select secondary glaucoma subtypes is angle surgery, either trabeculotomy or goniotomy. More recently, MIGS devices have been utilized to enhance the efficacy of these procedures. Despite the high success rates of these primary surgical options, refractory cases are challenging to manage. There is no consensus on the next step of treatment following primary angle surgery. Glaucoma drainage devices and trabeculectomies have been the traditional options, with laser treatment reserved for more severe cases. The benefits and disadvantages of each of these options are discussed.
Yavuz Saricay L, Baldwin G, Leake K, Johnston A, Shah AS, Patel NA, Gonzalez E. Cytomegalovirus retinitis and immune recovery uveitis in a pediatric patient with leukemia. J AAPOS 2022;Abstract
Immune recovery uveitis (IRU) is an ocular form of immune reconstitution inflammatory syndrome, which is rare in the pediatric population. We report a case of IRU in an 11-year-old girl with a history of cytomegalovirus (CMV) retinitis in the setting of acute leukemia, who developed uveitis, vitritis, retinitis, and vasculitis during immune reconstitution. She was found to have negative CMV antigenemia, and the disease occurred during concurrent systemic antiviral therapy. Anterior chamber tap confirmed the absence of the CMV in the eye, and recurrent blood samples continued to reveal absent CMV viral particles systemically while her lymphocyte count was steadily increasing. The patient responded to oral steroids, leading to resolution of active retinitis. Tapering the steroids caused a mild reactivation of the ocular immune response.
Hennein L, Jastrzembski B, Shah AS. Use of Telemedicine in Pediatric Ophthalmology in the Underserved Population. Semin Ophthalmol 2022;:1-8.Abstract
Access to pediatric eye care is critical in diagnosing and treating eye disease promptly to prevent visual impairment. The demand for pediatric ophthalmology is high, even in developed countries, and significant socioeconomic disparities exist in access to care. The purpose of this article is to summarize the current literature on the use of telemedicine in pediatric ophthalmology in the underserved population and to identify areas of opportunity. A detailed literature review was performed in PubMed and Google Scholar on October 1, 2021. All articles in English that described the use of telemedicine in pediatric ophthalmology, with particular attention to the underserved pediatric population, were included. There is a paucity of literature on the visual outcomes from pediatric teleophthalmology alone, and even less in underserved populations specifically. Literature supports its use in subacute to chronic eye disease, return and postoperative visits, and screening for retinopathy in prematurity in particular. Collaboration between pediatric optometrists and pediatric ophthalmologists for both asynchronous and synchronous care delivery models has shown promise in several studies. It is essential to operate within the limits of pediatric teleophthalmology and utilize this valuable service for its strengths. Telemedicine may expand access to pediatric ophthalmologists in underserved populations and may reduce the burden of eye disease.
Delavogia E, Ntentakis DP, Cortinas JA, Fernandez-Gonzalez A, Alex Mitsialis S, Kourembanas S. Mesenchymal Stromal/Stem Cell Extracellular Vesicles and Perinatal Injury: One Formula for Many Diseases. Stem Cells 2022;40(11):991-1007.Abstract
Over the past decades, substantial advances in neonatal medical care have increased the survival of extremely premature infants. However, there continues to be significant morbidity associated with preterm birth with common complications including bronchopulmonary dysplasia (BPD), necrotizing enterocolitis (NEC), neuronal injury such as intraventricular hemorrhage (IVH) or hypoxic ischemic encephalopathy (HIE), as well as retinopathy of prematurity (ROP). Common developmental immune and inflammatory pathways underlie the pathophysiology of such complications providing the opportunity for multisystem therapeutic approaches. To date, no single therapy has proven to be effective enough to prevent or treat the sequelae of prematurity. In the past decade mesenchymal stem/stromal cell (MSC)-based therapeutic approaches have shown promising results in numerous experimental models of neonatal diseases. It is now accepted that the therapeutic potential of MSCs is comprised of their secretome, and several studies have recognized the small extracellular vesicles (sEVs) as the paracrine vector. Herein, we review the current literature on the MSC-EVs as potential therapeutic agents in neonatal diseases and comment on the progress and challenges of their translation to the clinical setting.
Galli J, Loi E, Strobio C, Micheletti S, Martelli P, Merabet LB, Pasini N, Semeraro F, Fazzi E, Fazzi E. Neurovisual profile in children affected by Angelman syndrome. Brain Dev 2022;Abstract
BACKGROUND: Angelman syndrome (AS) is a rare neurogenetic disorder caused by altered expression of the maternal copy of the UBE3A gene. Together with motor, cognitive, and speech impairment, ophthalmological findings including strabismus, and ocular fundus hypopigmentation characterize the clinical phenotype. The aim of this study was to detail the neurovisual profile of children affected by AS and to explore any possible genotype-phenotype correlations. METHODS: Thirty-seven children (23 females, mean age 102.8 ± 54.4 months, age range 22 to 251 months) with molecular confirmed diagnosis of AS were enrolled in the study. All underwent a comprehensive video-recorded neurovisual evaluation including the assessment of ophthalmological aspects, oculomotor functions, and basic visual abilities. RESULTS: All children had visual impairments mainly characterized by refractive errors, ocular fundus changes, strabismus, discontinuous/jerky smooth pursuit and altered saccadic movements, and/or reduced visual acuity. Comparing the neurovisual profiles between the deletion and non-deletion genetic subgroups, we found a significant statistical correlation between genotype and ocular fundus hypopigmentation (p = 0.03), discontinuous smooth pursuit (p < 0.05), and contrast sensitivity abnormalities (p < 0.01) being more frequent in the deletion subgroup. CONCLUSIONS: Subjects affected by AS present a wide spectrum of neurovisual impairments that lead to a clinical profile consistent with cerebral visual impairment (CVI). Moreover, subjects with a chromosome deletion show a more severe visual phenotype with respect to ocular fundus changes, smooth pursuit movements, and contrast sensitivity. Early detection of these impaired visual functions may help promote the introduction of neurovisual habilitative programs which can improve children's visual, neuromotor, and cognitive outcomes.

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