Pediatric Ophthalmology

Kran BS, Lawrence L, Mayer LD, Heidary G. Cerebral/Cortical Visual Impairment: A Need to Reassess Current Definitions of Visual Impairment and Blindness. Semin Pediatr Neurol 2019;31:25-29.Abstract
Cerebral/cortical visual impairment (CVI) is characterized by higher order visual dysfunction caused by injury to the retrogeniculate visual pathways and brain structures which subserve visual processing. CVI has become the leading cause of significant vision loss in children in developed countries, but continues to be an under-recognized cause of visual disability with respect to services aimed at maximizing visual development. Current criteria which are used to define visual disability rely on measures of visual acuity and visual field. Many children who require specialized vision services do not qualify, because these standard definitions of vision impairment do not account for CVI. In order to appropriately identify patients with CVI and offer the resources which may positively impact functional use of vision, the definition of visual impairment and blindness needs to be modified. This commentary calls for a change in the definition of visual impairment and blindness to acknowledge those persons with brain-based vision impairment.
Mazel EC, Bailin ES, Tietjen MW, Palmer PA. A Questionnaire Assessing What Teachers of the Visually Impaired Know About Cortical/Cerebral Vision Impairment. Semin Pediatr Neurol 2019;31:41-47.Abstract
Cortical/cerebral visual impairment (CVI) is now the main cause of visual impairment in developed countries, yet it remains poorly understood. Four hundred and ninteen teachers of the visually impaired (TVIs) from across the United States responded to a questionnaire targeted at evaluating the preparedness of TVIs to serve their students with CVI. The TVIs were asked about their background knowledge, their abilities to assess a student with CVI, and their abilities to apply what they know to best help their students. The primary finding was that there is a perceived unmet need for TVIs to receive formal training in CVI during their certification. The results of this survey provide a foundation for future research on CVI knowledge and education among TVIs.
Akula JD, Ambrosio L, Howard FI, Hansen RM, Fulton AB. Extracting the ON and OFF contributions to the full-field photopic flash electroretinogram using summed growth curves. Exp Eye Res 2019;189:107827.Abstract
Under cone-mediated (photopic) conditions, an "instantaneous" flash of light, including both stimulus onset and offset, will simultaneously activate both "ON" and "OFF" bipolar cells, which either depolarize (ON) or hyperpolarize (OFF) in response and, respectively, produce positive-going and negative-going deflections in the electroretinogram (ERG). The stimulus-response (SR) relationship of the photopic ON response demonstrates logistic growth, like that manifested in the rod-mediated (scotopic) b-wave, which is driven by a single class of depolarizing bipolar cell. However, the photopic b-wave SR function is importantly shaped by OFF responses, leading to a "photopic hill." Furthermore, both on and off stimuli elicit activity in both ON and OFF bipolar cells. This has made it difficult to produce meaningful parameters for ready interpretation of the photopic b-wave SR relationship. Therefore, we evaluated whether the sum of sigmoidal SR functions, as descriptors of the depolarizing and hyperpolarizing components of the photopic flash ERG, could be used to elucidate and quantitate the mechanisms that produce the photopic hill. We used a novel fitting routine to optimize a sum of simple sigmoidal curves to SR data in five groups of subjects: Healthy adult, 10-week-old infant, congenital stationary night blindness (CSNB), X-linked juvenile retinoschisis (XJR), and preterm-born, both without and with a history of retinopathy of prematurity (ROP). Differences in ON and OFF amplitude, sensitivity, and implicit time among the groups were then compared using parameters extracted from these fits. We found that our modeling procedure enabled plausible derivations of ON and OFF pathway contributions to the ERG, and that the parameters produced appeared to have physiological relevance. In adult subjects, the ON and OFF amplitudes were similar in magnitude with respectively longer and shorter implicit times. Infant, CSNB, and XJR subjects showed significant ON pathway deficits. History of preterm-birth, without or with a diagnosis of ROP, did not much affect cone responses.
Bennett CR, Bex PJ, Bauer CM, Merabet LB. The Assessment of Visual Function and Functional Vision. Semin Pediatr Neurol 2019;31:30-40.Abstract
The complete assessment of vision-related abilities should consider visual function (the performance of components of the visual system) and functional vision (visual task-related ability). Assessment methods are highly dependent upon individual characteristics (eg, the presence and type of visual impairment). Typical visual function tests assess factors such as visual acuity, contrast sensitivity, color, depth, and motion perception. These properties each represent an aspect of visual function and may impact an individual's level of functional vision. The goal of any functional vision assessment should be to measure the visual task-related ability under real-world scenarios. Recent technological advancements such as virtual reality can provide new opportunities to improve traditional vision assessments by providing novel objective and ecologically valid measurements of performance, and allowing for the investigation of their neural basis. In this review, visual function and functional vision evaluation approaches are discussed in the context of traditional and novel acquisition methods.
Simmons NL, Robb RM, Tybor DJ, Gilbert AL. Older age and larger cyst size in children with spontaneous rupture of periorbital dermoid cysts. J AAPOS 2019;Abstract
We analyzed clinical and histopathologic data of 97 pediatric patients who underwent excision of dermoid cysts. On review, 16.5% of the sample population demonstrated localized chronic inflammatory changes, including the presence of giant cells and epithelial disruption. These features were considered indicative of prior cyst rupture. Age at time of initial presentation was significantly older and cyst size was significantly larger in patients with histopathologic signs of previous rupture. Longer time to presentation and time to excision were associated with increased odds of spontaneous rupture.
Lundgren P, Hellgren G, Pivodic A, Sävman K, Smith LEH, Hellström A. Erythropoietin serum levels, versus anaemia as risk factors for severe retinopathy of prematurity. Pediatr Res 2019;86(2):276-282.Abstract
BACKGROUND: Preterm infants with anaemia are treated with recombinant human erythropoietin (rhEPO). It is debated whether rhEPO treatment is a risk factor for retinopathy of prematurity (ROP). We evaluated longitudinal EPO and haemoglobin levels, blood transfusions and neonatal morbidities as risk factors for severe ROP. METHOD: This prospective study included 78 Swedish infants, born <28 weeks gestational age (GA), screened for ROP. We tested serum EPO levels on postnatal days 1, 7, 14 and 28 and at postmenstrual ages 32, 36 and 40 weeks. Haemoglobin levels and blood transfusions were recorded during postnatal weeks 1-4. Anaemia was defined as haemoglobin ≤110 g/L. RESULTS: During postnatal week 1, infants with severe ROP requiring treatment (28%) more frequently developed anaemia (42.9% versus 8.0%, P = 0.003) and had higher mean EPO levels (postnatal day 7: 14.2 versus 10.8 mIU/mL, P = 0.003) compared to infants with no or less severe ROP not requiring treatment. In multivariable analyses, GA and anaemia during week 1 remained significant risk factors, but elevated EPO level postnatal day 7 was no longer significant. CONCLUSIONS: Among infants born <28 weeks GA, anaemia during week 1 was a significant risk factor for severe ROP requiring treatment but not elevated EPO levels.
Bulka CM, Dammann O, Santos HP, VanderVeen DK, Smeester L, Fichorova R, O'Shea MT, Fry RC. Placental CpG Methylation of Inflammation, Angiogenic, and Neurotrophic Genes and Retinopathy of Prematurity. Invest Ophthalmol Vis Sci 2019;60(8):2888-2894.Abstract
Purpose: Extremely preterm infants are at increased risk for retinopathy of prematurity (ROP). We previously identified several inflammatory proteins that were expressed early in life and are associated with an increased risk of ROP and several angiogenic and neurotrophic growth factors in the neonatal systemic circulation that are associated with a lower risk of ROP. In this paper, we report the results of a set of analyses designed to test the hypothesis that placental CpG methylation levels of 12 inflammation-, angiogenic-, and neurotrophic-associated genes predict the occurrence of prethreshold ROP in extremely preterm newborns. Methods: We used placental CpG methylation data from 395 newborns from the Extremely Low Gestational Age Newborns study. Results: Multivariable regression models revealed that placental DNA methylation of 16 CpG sites representing 8 genes were associated with prethreshold ROP. Specifically, CpG methylation in the serum amyloid A SAA1 and SAA2, brain-derived neurotrophic factor (BDNF), myeloperoxidase (MPO), C-reactive protein (CRP), angiopoietin 1 (ANGPT1), and tumor necrosis factor receptor superfamily member 1B (TNFRSF1B) genes was associated with a lower risk of prethreshold ROP. Conversely, CpG methylation at three probes within tumor necrosis factor receptor superfamily member 1A (TNFRSF1A) and in two alternative probes within the BDNF and ANGPT1 genes was associated with an increased risk of ROP. Conclusions: CpG methylation may be a useful marker for improving ROP prediction, opening the opportunity for early intervention to lessen disease severity.
Drack AV, Utz VM, Wang K, Alcorn DM, Brooks BP, Costakos DM, Couser NL, Heon E, Levin AV, Lloyd CI, Morse CL, Schmitt MA, Whitman MC, Traboulsi EI. Survey of practice patterns for the management of ophthalmic genetic disorders among AAPOS members: report by the AAPOS Genetic Eye Disease Task Force. J AAPOS 2019;Abstract
To better understand AAPOS member pediatric ophthalmologists' knowledge and needs regarding genetic eye disorders, the AAPOS Genetic Eye Disease Task Force developed a 16-question survey that was circulated to national and international AAPOS members. Responses to questions on practice patterns, baseline knowledge, and educational interests regarding patients with suspected ophthalmic genetic disorders were collected. A majority of respondents (93%) evaluate patients with suspected genetic disorders. Knowledge gaps were present in heritability of certain conditions, genetic testing strategies, and referral to clinical trials. Most respondents expressed interest in further education in these areas. A model for care is proposed as a first step in the education process.
Nguyen JQN, Resnick CM, Chang Y-H, Hansen RM, Fulton AB, Moskowitz A, Calabrese CE, Dagi LR. Impact of obstructive sleep apnea on optic nerve function in patients with craniosynostosis and recurrent intracranial hypertension. Am J Ophthalmol 2019;Abstract
PURPOSE: Assessment of combined impact of intracranial pressure (ICH) and obstructive sleep apnea (OSA) on optic nerve function in children with craniosynostosis (CS). DESIGN: Retrospective cross-sectional study METHODS: Patients treated at Boston Children's Hospital for CS who had an ophthalmic examination that included pattern reversal (pr)VEP (2013-2014) and history of ICH based on direct measurement, papilledema, or classic features on neuroimaging and during cranial vault expansion were included. History of OSA was determined by polysomnography and associated conditions, including apnea and (adeno)tonsillectomy. Subjects were divided into four groups: (1) resolved ICH absent history of OSA; (2) resolved ICH with history of OSA; (3) recurrent ICH absent history of OSA; and (4) recurrent ICH with history of OSA. Predictor variables included latency of P100 component of prVEP, best-corrected visual acuity, optic nerve appearance, visual fields and global RNFL. Primary outcome was association of prolonged P100 latency with resolved versus recurrent ICH and OSA. RESULTS: Twenty-eight children met inclusion criteria (mean age 11.6 ± 6.9 years): group 1 (N = 3); group 2 (N = 6); group 3 (N = 8); group 4 (N = 11). P100 latencies were not prolonged in groups 1 and 2. Three of 8 in group 3 and 9 of 11 in group 4 had prolonged P100 latency. Group 4 was significantly worse than group 3 (P=0.005). CONCLUSIONS: History of OSA, in addition to recurrent ICH, is associated with greatest risk of optic neuropathy with CS. Ophthalmologists should encourage early management of OSA as well as ICH to optimize ophthalmic outcomes.
Gise R, Gaier ED, Heidary G. Diagnosis and Imaging of Optic Nerve Head Drusen. Semin Ophthalmol 2019;:1-8.Abstract
The presence of optic nerve swelling in pediatric patients is a frequent cause for referral to pediatric ophthalmologists and neuro-ophthalmologists because this finding can be the harbinger of serious neurologic disease including brain tumor, demyelinating disease, infiltrative disease of the optic nerve, or idiopathic intracranial hypertension. Optic nerve head drusen (ONHD) are common and can be particularly difficult to distinguish from true optic nerve swelling in pediatric patients because the ONHD are typically buried beneath the substance of the optic nerve. Correct identification of ONHD is relevant because of the visual morbidity associated with this condition and because of the need to distinguish pseudopapilledema secondary to ONHD from true optic nerve swelling. A variety of imaging modalities may be employed to evaluate for the presence of ONHD, including ultrasound, optical coherence tomography (OCT), enhanced depth imaging-OCT, fluorescein angiography, fundus autofluorescence, and optical coherence tomography angiography. To date, there is no consensus as to which of these techniques is most accurate and which should be part of a standardized evaluation for children suspected of ONHD. This review examines the recent literature analyzing these diagnostic tools and summarizes data regarding best practices for identifying ONHD.
Lambert SR, Aakalu VK, Hutchinson AK, Pineles SL, Galvin JA, Heidary G, Binenbaum G, VanderVeen DK. Intraocular Lens Implantation during Early Childhood: A Report by the American Academy of Ophthalmology. Ophthalmology 2019;126(10):1454-1461.Abstract
PURPOSE: To compare the visual outcomes and adverse events associated with optical correction using an intraocular lens (IOL), contact lenses, or spectacles after cataract surgery in children 2 years of age or younger. METHODS: Literature searches were conducted in PubMed, the Cochrane Library, and the databases of clinical trials in February 2019, without date or language restrictions. The search resulted in 194 potentially relevant citations, and 34 were selected for full-text review. Fourteen studies were determined to be relevant to the assessment criteria and were selected for inclusion in this assessment. The panel methodologist then assigned a level of evidence rating to these studies. RESULTS: Intraocular lenses were associated with visual outcomes similar to outcomes for contact lenses or spectacles for children who had both bilateral and unilateral cataracts. Intraocular lenses were also associated with an increased risk of visual axis opacities. All treatments were associated with a similar incidence of glaucoma. Although ocular growth was similar for all treatments, infants younger than 6 months who underwent IOL implantation had large myopic shifts that often resulted in high myopia or severe anisometropia later in childhood. Corneal endothelial cell counts were lower in eyes that underwent IOL implantation. The incidence of strabismus was similar with all treatments. CONCLUSIONS: Intraocular lens implantation is not recommended for children 6 months of age or younger because there is a higher incidence of visual axis opacities with this treatment compared with aphakia. The best available evidence suggests that IOL implantation can be done safely with acceptable side effects in children older than 6 months of age. However, the unpredictability of ocular growth means that these children will often have large refractive errors later in childhood that may necessitate an IOL exchange or wearing spectacles or contact lenses with a large refractive correction. In addition, the training and experience of the surgeon as well as ocular and systemic comorbidities should be taken into consideration when deciding whether IOL implantation would be appropriate.
Cheung CS, VanderVeen DK. Intraocular Lens Techniques in Pediatric Eyes with Insufficient Capsular Support: Complications and Outcomes. Semin Ophthalmol 2019;:1-10.Abstract
Intraocular lens (IOL) implantation in pediatric eyes with insufficient capsular support is challenging and there are multiple IOL options. These include placement of an IOL within the capsular bag with a capsular tension ring, a scleral-fixated posterior-chamber IOL (PCIOL) with or without capsular tension segment or ring, an intra-scleral fixated IOL, an iris-sutured PCIOL, or an anterior chamber iris-fixated IOL. We reviewed 48 articles and 1 published abstract describing the surgical techniques, complications and visual outcomes of different IOL options in the management of aphakic pediatric eyes with insufficient capsular support. The present review found that the visual acuity outcomes of various IOLs are comparable. Furthermore, each .
Raghuram A, Cotter S, Gowrisankaran S, Kanji J, Howell DR, Meehan WP, Shah AS. Post-Concussion: Receded Near Point of Convergence is Not Diagnostic of Convergence Insufficiency. Am J Ophthalmol 2019;Abstract
PURPOSE: To determine the frequency of receded near point of convergence (NPC) in patients with chronic concussion-related symptoms, and among those with receded NPC to enumerate the frequency of convergence insufficiency and other oculomotor disorders. STUDY: Design: Retrospective cross-sectional study METHODS: Clinic charts were retrospectively reviewed for the prior 3.5 years to identify all patients <21 years old who were >28 days post-concussion, had chronic concussion-related symptoms, had normal visual acuity, and received a comprehensive sensorimotor examination. The frequency of receded NPC and oculomotor diagnoses were determined. RESULTS: Of the 83 eligible patients, 74 (89%) had receded NPC. Of these, 70 (95%) had oculomotor disorders; 30 (41%) had disorders of accommodation only, 21 (28%) had convergence insufficiency and accommodation deficits, and 6 (8%) had convergence insufficiency only. Six (8%) had a convergence deficit other than convergence insufficiency (all with concurrent accommodative disorders), 4 (5%) had both a non-specific vergence dysfunction and accommodation deficits, 2 (3%) had convergence excess only, and 1 (1%) had both convergence excess and accommodative deficits. CONCLUSION: A receded NPC was present in the majority of young patients with chronic post-concussion symptoms. Associated with numerous underlying oculomotor dysfunctions, the clinical finding of a receded NPC is not synonymous with the diagnosis of convergence insufficiency. Because treatment options for the various oculomotor dysfunctions differ, it is prudent that these patients undergo a thorough examination of their vergence and accommodative systems so that an accurate diagnosis can be made and appropriate treatment prescribed.

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