Comparison of OCT imaging in children with foveal hypoplasia born full term versus preterm.

PURPOSE: It is unknown if foveal hypoplasia in full-term born children differs in structure and function from that observed in children born preterm. We compared macular structure and visual function in children with history of prematurity and full-term children diagnosed with foveal hypoplasia. METHODS: We compared three groups of subjects (3-18 years old): (1) full-term hypoplasia (FH, n = 56, gestational age ≥ 36 weeks); (2) preterm hypoplasia (n = 57, gestational age ≤ 31 weeks, birth weight ≤ 1500 g); (3) control (n = 54), full-term normal. Using spectral-domain optical coherence tomography volume-scan images, macular structure within 3 mm of Early-Treatment-Diabetic-Retinopathy-Study circle was segmented. Total, inner, and outer foveal thickness of right eyes were compared. Foveal hypoplasia was graded according to the Leicester Grading System. RESULTS: The mean total foveal thickness in micrometers was 263 ± 19 for the control, 299 ± 30 for the full-term hypoplasia, and 294 ± 28 for the preterm hypoplasia groups (F = 33, p < 0.001). Foveal inner retinal layer thickness differed among groups (p < 0.001), but not in the outer layers (p = 0.10). The full-term hypoplasia group had significantly thicker foveal inner layers (p < 0.05) and greater frequency of higher-grade hypoplasia than the preterm hypoplasia group. LogMAR visual acuity was worse in the full-term hypoplasia group (0.35 ± 0.36) than in the preterm hypoplasia group (0.19 ± 0.27, p < 0.001). CONCLUSION: Fovea was thicker in both hypoplasia groups. The full-term hypoplasia group is associated with more severe structure changes and poorer visual function than the preterm hypoplasia group.

Effect of gestational age at birth, sex, and race on foveal structure in children.

PURPOSE: Premature birth, race, and sex are contributing risk factors for retinopathy of prematurity (ROP) and have long-term impact on children's retinal structure. Few studies investigate impact of race and sex on macular structure in children born preterm. This study compared foveal structure in preterm and full-term children. METHODS: Children aged 4-18 years were enrolled into three groups: (1) ROP-risk group (n = 81), born at < 32 weeks gestational age with and without history of ROP; (2) preterm group (n = 46), born at 32-36 weeks gestational age; and (3) control group (n = 68) with full-term birth. Using spectral-domain optical coherence tomography volume-scan images, foveal structure within 1-mm and 3-mm early treatment diabetic retinopathy study circular grid was measured and segmented. Total inner and outer retina thickness of the right eye was compared among the three groups. RESULTS: The mean total foveal thickness (in microns) was 287 ± 26 for the ROP-risk group, 276 ± 19 for the preterm group, and 263 ± 20 for the control group (F = 26, p < 0.001). Foveal thickness of the ROP-risk group was significantly higher than that of the preterm group and the control group (all p < 0.05). Foveal thickness was thinner in black children than in white children and thinner in females than in males (all p < 0.001). A similar disparity in race and sex was found in the thickness of the inner and outer layers. CONCLUSIONS: The fovea was significantly thicker in the ROP-risk group than the control group. Foveal thickness decreases with increased gestational age. Race and sex are significant factors in foveal structure in children.

Pediatric optic neuritis.

PURPOSE OF REVIEW: Pediatric optic neuritis may be challenging to diagnose and treat. Significant clinical investigation of optic neuritis occurring in adults guides current clinical practices. Differences in presentation and prognosis exist for pediatric patients with optic neuritis when compared with adults including the risk of developing multiple sclerosis. The aim of this review is to provide an update on latest advances in the diagnosis, treatment and current research concerning pediatric optic neuritis. RECENT FINDINGS: Limited case series and retrospective reviews constitute much of the data we know about patients with pediatric optic neuritis. Pediatric optic neuritis is included in the spectrum of neuroinflammatory diseases. Testing modalities (ocular coherence tomography and visual evoked potentials) and serologic testing (antibodies against aquaporin-4 and myelin oligodendrocyte glycoprotein) are being investigated for diagnostic and prognostic value. The low incidence of pediatric optic neuritis results in small sample sizes may contribute to conflicting results of different studies. SUMMARY: Recent advances in diagnostic and serologic testing in pediatric neuritis may offer better diagnosis, treatment and prediction of prognosis. Validation requires well designed prospective research.

Cortical visual impairment in children: identification, evaluation and diagnosis.

PURPOSE OF REVIEW: Cortical visual impairment (CVI) is a major cause of visual loss in children worldwide. The definition of this condition is constantly evolving with respect to definition, identifying those at risk and technology for diagnosis. These advances can be used for early diagnosis, design of accommodations and services, as well as future therapies and prevention strategies. RECENT FINDINGS: Clinical questionnaires are being developed and tested for reliability in an attempt to identify those at risk for CVI. The definition is constantly being modified and now includes deficits in vision-guided motor planning and higher level executive functions. Neuroimaging techniques, such as MRI, functional MRI, and diffusion tensor imaging; electrophysiologic testing, such as sweep visual-evoked potentials; and perceptual testing, allow for further refinements in correlating structural defects and deficits in function. SUMMARY: Recent developments will allow identification of those children at risk for CVI and earlier interventions for specific deficits. A child's performance is built on previously mastered skills, making timely prediction of deficits and intervention essential.

Visits to the Pediatric Emergency Department for Eye Conditions Before and During the COVID-19 Pandemic.

INTRODUCTION: The use of the emergency department (ED) has been increasing, and many visits occur for non-urgent conditions. A similar trend was found among adult visits to the ED for ocular conditions. In this study we analyzed the impact of sociodemographic factors, presentation timing, and the COVID-19 pandemic on pediatric ED (PED) encounters for ophthalmologic conditions. It is important to identify the multifold factors associated with overutilization of the ED for non-urgent conditions. Caring for these patients in an outpatient clinical setting is safe and effective and could decrease ED crowding; it would also prevent delays in the care of other patients with more urgent medical problems and lower healthcare costs. METHODS: We retrospectively reviewed electronic health records of PED ocular-related encounters at two children's hospitals before (January 2014-May 2018) and during the COVID-19 pandemic (March 2020-February 2021). Encounters were categorized based on the International Classification of Diseases codes into "emergent," "urgent," and non-urgent" groups. We analyzed associations between sociodemographic factors and degrees of visit urgency. We also compared visit frequencies, degrees of urgency, and diagnoses between pre-pandemic and pandemic data. RESULTS: Pre-pandemic ocular-related PED encounters averaged 1,738 per year. There were highly significant sociodemographic associations with degrees of urgency in PED utilization. During the 12-month pandemic timeframe, encounter frequency contracted to 183. Emergent visits decreased from 21% to 11%, while the proportions of urgent and non-urgent encounters were mostly unchanged. The most common pre-pandemic urgent diagnosis was corneal abrasion (50%), while visual disturbance was most common during the pandemic (92%). During both time periods, eye trauma was the most frequent emergent encounter and conjunctivitis was the most common non-urgent encounter. CONCLUSION: Sociodemographic factors may be associated with different types of PED utilization for ocular conditions. Unnecessary visits constitute major inefficiency from a healthcare-systems standpoint. The marked decrease in PED utilization and differing proportions of ocular conditions encountered during the pandemic may reflect a decrease in incidence of many of those conditions by social distancing; these changes may also reflect altered parental decisions about seeking care.

Update on retinopathy of prematurity: treatment options and outcomes.

PURPOSE OF REVIEW: To evaluate and review the recent scientific literature on retinopathy of prematurity (ROP). Recent studies have shown advancement in treatment options in ROP as well as improved functional and structural outcomes. This review compiles some of these recent findings. RECENT FINDINGS: New guidelines on ROP screening and treatment criteria have recently been developed and are now the standard of care for practitioners taking care of children having ROP. Recent advances in antiangiogenic therapies offer possible primary or adjunct treatment to the well established and effective laser treatment options. Follow-up of treated ROP patients informs us of the short-term and long-term complications requiring lifetime ophthalmology care. SUMMARY: This review offers an update on the screening and treatment guidelines, new treatment options, and short-term and long-term complications in ROP.

Using OCT Fixation Shift to Assess Eccentric Fixation in Children With Residual Amblyopia.

Purpose: Eccentric fixation in amblyopia is often estimated grossly without precision. Although the usefulness of optical coherence tomography (OCT) fixation shift in the quantification of eccentric fixation in a small cohort of amblyopic children was recently reported, there is a lack of understanding of characteristics of OCT fixation shift. In a retrospective cohort study, we evaluated eccentric fixation with OCT in a large cohort of children with residual amblyopia. Methods: Children, age 4 to 17 years, with residual amblyopia (amblyopic, n = 56) and without amblyopia (control, n = 75) were enrolled. Amblyopia was associated with anisometropia alone (anisometropia subtype, n = 28) and strabismus without or with anisometropia (strabismic subtype, n = 28). Spectral domain OCT was used to estimate fixation. The OCT fixation shift, defined as the distance between the fovea and the fixation point, was measured and adjusted with calculated axial length and converted into visual degrees. Fixation shift in amblyopic eyes, fellow nonamblyopic eyes, and right eyes of the control group were compared. Fixation shift between the anisometropic and strabismic amblyopia subtypes was also compared. Its correlation with visual acuity was estimated. Results: The mean fixation shift was significantly different: 0.17° ± 0.29° for control right eyes, 0.94° ± 1.24° for amblyopic eyes, and 0.34° ± 0.57° for fellow eyes (χ2 = 23.3; P < 0.001). There was no significant difference between fellow eyes and control eyes (P = 0.11). Fixation shift in amblyopic eyes was significantly correlated with visual acuity (R = 0.44; P < 0.001), and it was significantly smaller in the anisometropic subtype than in the strabismic subtypes (0.34° ± 0.46° vs. 1.54° ± 1.48°, W = 338, P < 0.001). Conclusions: OCT fixation shift can be used both in detection and quantification of eccentric fixation in children with residual amblyopia, especially in those with strabismus. Translational Relevance: OCT fixation shift offers a convenient clinical approach in quantitative evaluation of eccentric fixation in children with strabismic amblyopia.

Funduscopic examination and SD-OCT in detecting sickle cell retinopathy among pediatric patients.

PURPOSE: To compare the results of fundus examination and spectral domain optic coherence tomography (SD-OCT) in detecting retinal changes in pediatric patients with sickle cell disease at a single center. METHODS: In this prospective study, conducted over a period of 19 months, consecutive African American patients with sickle cell disease underwent complete ophthalmologic examination, and SD-OCT images of the maculas of both eyes were obtained. RESULTS: A total of 69 (37 males) patients aged 5-20 years (mean 12.89 ± 4.09; range, 2-20) with sickle cell disease (SC, 26; SS, 36; Sß+, 5; Sß0 thalassemia, 2) were examined. Patients' visual acuity range was 20/20 to 20/40. On funduscopic examination, 11 of 69 showed signs of retinopathy, whereas 47 of 68 showed inner retina thinning in the watershed zone temporal to the fovea on SD-OCT. On average, SD-OCT diagnosed disease 1.78 years earlier than fundus examination. Of patients <10 years of age, 1 was diagnosed with retinopathy by funduscopy, whereas retinal changes were evident on SD-OCT in 12 of 22. Fundus examination showed no significant difference in retinal findings between SS/Sß0 and SC genotypes. On SD-OCT, SS/Sß0 showed worse disease process than SC in frequency of diagnosis (82% vs 56%), bilateral involvement (87% vs 43%), and foveal involvement (18% vs 0). CONCLUSIONS: The peripheral retina could be visualized on fundus examination but not easily imaged on SD-OCT, which, however, had a higher detection rate and offered earlier diagnosis. In our patient cohort SD-OCT showed that the severity of retinal change was associated with more severe sickle cell disease genotypes (SS and Sß0).

Risk factors for amblyopia in congenital anterior lens opacities.

PURPOSE: We sought to identify the features of congenital anterior lens opacities (CALOs), which are associated with increased risk for amblyopia. METHODS: We undertook a retrospective study of 59 patients with CALO from 3 clinical practices. The following variables were examined: type (polar, subcapsular, or pyramidal), location (central, paracentral, or peripheral), diameter, presence of adherent iris tissue, anisometropia (spherical equivalent), unilateral versus bilateral, and presence or absence of amblyopia. Pearson chi-square and independent sample t-tests were conducted to evaluate if any of the aforementioned variables were independently associated with amblyopia. Relative risk was then calculated for significantly related variables (P < 0.05). RESULTS: Amblyopia was present in 17 of 59 patients (28.8%). Patients with amblyopia had a mean anisometropia of 1.23 diopters whereas nonambyopic patients had a mean anisometropia of 0.25 (P = 0.023). The relative risk for amblyopia with anisometropia of one diopter or greater was 6.5 (95% confidence interval = 3.79-7.45). The mean cataract size in the amblyopic eyes was 1.22 mm (range, 0.7-2.0 mm). Mean cataract size in the nonamblyopic eyes was 0.95 mm (range, 0.5-2.0 mm). This difference in mean cataract size was significant with independent sample t-tests (P = 0.02); however, this difference did not remain significant with logistic regression. Cataract size was not a significant relative risk factor for amblyopia. CONCLUSIONS: Our overall incidence of amblyopia was 28.8%. The only variable associated with increased relative risk for amblyopia in this group of patients was anisometropia. Patients with CALO who have anisometropia of 1 diopter or greater are 6.5 times more likely to develop amblyopia.

Visual outcome and success of amblyopia treatment in unilateral small posterior lens opacities and lenticonus initially treated nonsurgically.

PURPOSE: We sought to assess the success of amblyopia treatment in patients with small posterior lens opacities as well as the factors associated with a good visual outcome. METHODS: This was a retrospective study of patients with posterior lens opacities that initially were thought to be too small in size to warrant cataract surgery. The following variables were examined: cataract type, location, diameter, persistent hyaloid vessel, anisometropia, strabismus, and age of detection. Success of treatment of amblyopia was defined as improvement by at least 0.3 logMAR units. Good visual outcome was defined as 20/40 or better. Amblyopia was treated by glasses, patching, and/or atropine. Patients who failed with conservative treatment or had an increase in cataract size underwent surgery. RESULTS: Forty-eight (91%) of 53 eyes were amblyopic. Thirty amblyopic eyes had pre- and post-treatment Snellen acuities. Twenty (67%) had their visual acuity (VA) improved by 0.3 logMAR units or greater. None of the measured variables were associated with successful amblyopia treatment. Twenty-five (49%) of 51 patients had a final VA of 20/40 or better. The only variable associated with good visual outcome was cataract type: 18 of 25 (72%) posterior subcapsular cataract and 6 of 23 (32%) posterior lenticonus eyes achieved VA of 20/40 or better (P = 0.008). Six patients who went on to have cataract surgery experienced a larger improvement in BCVA (4.50 logMar units +/- 2.52 lines) compared with patients treated without cataract surgery (2.36 logMar units +/- 3.11 lines). DISCUSSION: Amblyopia treatment was successful in most cases. A small group of patients who underwent cataract surgery experienced a greater VA improvement; however, it was not statistically significant. Further studies are needed to determine which patients would benefit from cataract surgery.

Ophthalmologic disorders in children with syndromic and nonsyndromic hearing loss.

OBJECTIVE: To determine the rate of ophthalmologic anomalies among patients with syndromic and nonsyndromic, congenital sensorineural hearing loss (SNHL) to assess the need for comprehensive ophthalmologic evaluation in these children. DESIGN: Retrospective medical chart review of children with SNHL who underwent comprehensive evaluation by pediatric ophthalmologists and geneticists. SETTING: Tertiary care pediatric hospital. PATIENTS: Seventy-seven patients with SNHL. MAIN OUTCOME MEASURES: Degree of hearing loss (HL) and presence of ophthalmologic and genetic disorders. RESULTS: The overall rate of ophthalmologic disorders was 32% (25 of 77 patients). When children with multisystem genetic disorders known to be related to visual loss were excluded, the rate fell to 23% (12 of 53 vs 13 of 24; P = .006). There was no statistically significant difference in the degree of HL between patients with and without eye disorders (mean [SD], 46.5 [29.9] vs 49.1 [32.3] dB HL; P = .75). Patients with eye disorders were significantly more likely to have a multisystem genetic disorder (13 of 25 [52%] vs 11 of 52 [21%]; P = .006). No patients with ocular abnormalities had isolated otologic disorders, but 9 of 52 (17%) of those patients without ocular abnormalities did. CONCLUSIONS: Comprehensive ophthalmologic examination revealed a rate of ophthalmologic disorders in children with SNHL in the lower end of the previously reported rates of 31% to 61%. Children with nonsyndromic SNHL have an approximately 2- to 3-fold increase in ocular abnormalities compared with the general pediatric population. Ophthalmologic and genetic consultations are warranted in patients with congenital SNHL.

Strabismus in craniosynostosis.

PURPOSE OF REVIEW: To present peer-reviewed articles related to strabismus in craniosynostosis. RECENT FINDINGS: Improved neurosurgical treatment has resulted in amblyopia replacing optic atrophy as the main cause of visual impairment in Apert's and Crouzon syndromes. Patients with nonsyndromic craniosynostosis have an increased incidence of significant refractive error and horizontal strabismus similar to syndromic craniosynostosis. Imaging of the orbit and extraocular muscles continues to be recommended as helpful for presurgical planning. Surgical techniques continue to be explored in order to improve outcomes for craniosynostosis patients. SUMMARY: Attention to diagnosis and treatment of amblyopia and strabismus is necessary to preserve vision in craniosynostosis patients. Orbital imaging may aid in planning for these patients whose strabismus surgery remains a challenge.