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Background: Handheld optical coherence tomography (HH-OCT) can image awake, young children but lacks integrated segmentation/analysis software. OCT imaging of eyes with optic neuropathies demonstrates ganglion cell layer (GCL) and ganglion cell complex (GCC) thinning, with a normal or thickened inner nuclear layer (INL). We compared pediatric normative data with GCL/INL and GCC/INL ratios from HH-OCT macular scans of awake young children with clinically diagnosed optic neuropathies., Methods: Macular HH-OCT from awake children with optic neuropathies was prospectively obtained using Bioptigen (Leica Microsystems, Wetzlar, Germany). The GCL, GCC, and INL were manually measured by two readers using ImageJ from single-line macular scans at the thickest points nasal and temporal to the fovea, respectively, and the GCL/INL and GCC/INL ratios were calculated and compared with normative data., Results: HH-OCT images from 17 right eyes of 17 children (mean age, 4.3 ± 2.9 years) with optic neuropathies were analyzed. Mean nasal (17 eyes) and temporal (16 eyes) GCL/INL ratios with optic neuropathies were 0.44 ± 0.38 (95% CI, 0.26-0.62) and 0.26 ± 0.22 (95% CI, 0.15-0.36), respectively. Corresponding normative GCL/INL ratios are 1.26 ± 0.20 (95% CI, 1.19-1.34) and 1.23 ± 0.27 (95% CI, 1.13-1.33), respectively (P < 0.0001). Severe thinning precluded GCL measurements in 2 eyes nasally and 5 eyes temporally, resulting in GCL measurements of zero. Mean nasal (17 eyes) and temporal (16 eyes) GCC/INL ratios were 1.93 ± 0.70 (95% CI,1.60-2.27) and 1.67 ± 0.44 (95% CI,1.46-1.87). Corresponding normative ratios are 2.85 ± 0.38 (95% CI, 2.71-2.99) and 2.87 ± 0.42 (95% CI, 2.70-3.03), respectively (P < 0.0001)., Conclusions: GCL/INL and GCC/INL ratios calculated from single-line macular HH-OCT scans in awake young children with optic neuropathies differ significantly from normative values and may thus have utility in helping to establish a diagnosis of optic neuropathy., (Copyright © 2024 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Background: Infantile nystagmus syndrome can be associated with an afferent problem (anterior or posterior segment) or constitute an isolated idiopathic disorder. With a normal ophthalmic examination, current guidelines recommend electroretinography (ERG) rather than magnetic resonance (MRI) for preliminary workup. Given the limited use of optical coherence tomography (OCT) in preverbal children, the purpose of this study was to evaluate the role of handheld OCT (HH-OCT) in the initial diagnostic evaluation of infantile nystagmus., Methods: In this cross-sectional case series, the medical records of all children with infantile nystagmus and HH-OCT imaging at the Duke Eye Center from August 2016 to July 2021 were retrospectively reviewed. Children with anterior segment disorders or obvious retina/optic nerve structural pathology, bilateral ophthalmoplegia, or Down syndrome were excluded. Two masked pediatric ophthalmologists graded HH-OCT images for optic nerve head and macular abnormalities. A neuro-ophthalmologist reviewed clinical findings of each patient's presenting visit and recommended appropriate testing (MRI vs ERG), initially without, and again with HH-OCT image review., Results: A total of 39 cases were included, with mean presenting age of 1.3 years. Final diagnoses included retinal or foveal abnormalities (7), optic nerve pathology (13), idiopathic (10), or unknown (9). HH-OCT findings included optic nerve hypoplasia (1), optic nerve elevation (3), persistence of the inner layers at the fovea (9), thin ganglion cell layer (8), ellipsoid zone abnormality (3), and thin choroid (1). HH-OCT findings altered initial clinical-only management in 16 cases (41%), including avoiding MRI (5) and ERG (10) testing., Conclusions: Our results suggest that HH-OCT has the potential to augment and streamline the evaluation of infantile nystagmus., (Copyright © 2024 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Background/aims: The optic nerve development during the critical postnatal weeks of preterm infants is unclear. We aimed to investigate the change of retinal nerve fibre layer (RNFL) in preterm infants., Methods: We used an investigational handheld optical coherence tomography (OCT) system to serially image awake preterm infants between 30 and 60 weeks postmenstrual age (PMA) at the bedside. We assessed RNFL thickness in the papillomacular bundle and nasal macular ganglion cell layer+inner plexiform layer (GCL+IPL) thickness. We applied a segmented mixed model to analyse the change in the thickness of RNFL and GCL+IPL as a function of PMA., Results: From 631 OCT imaging sessions of 101 infants (201 eyes), RNFL thickness followed a biphasic model between 30 and 60 weeks, with an estimated transition at 37.8 weeks PMA (95% CI: 37.0 to 38.6). RNFL thickness increased at 1.8 μm/week (95% CI: 1.6 to 2.1) before 37.8 weeks and decreased at -0.3 μm/week (95% CI: -0.5 to -0.2) afterwards. GCL+IPL thickness followed a similar biphasic model, in which the thickness increased at 2.9 μm/week (95% CI: 2.5 to 3.2) before 39.5 weeks PMA (95% CI: 38.8 to 40.1) and then decreased at -0.8 μm/week (95% CI: -0.9 to -0.6)., Conclusion: We demonstrate the feasibility of monitoring RNFL and GCL+IPL thickness from OCT during the postnatal weeks of preterm infants. Thicknesses follow a biphasic model with a transition age at 37.8 and 39.5 weeks PMA, respectively. These findings may shed light on optic nerve development in preterm infants and assist future study designs., Competing Interests: Competing interests: SFF: Qlaris—consultant; CT: Alcon Laboratories—royalties; EMMES—consultant; Theia Imaging—owner equity and consultant., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Handheld optical coherence tomography (HH-OCT) lacks integrated segmentation/analysis software. Optic neuropathies cause ganglion cell layer (GCL) thinning, with normal to thickened inner nuclear layer (INL), suggesting the potential value of estimating the GCL/INL ratio on single-line foveal HH-OCT scans. This study determines this ratio in macular scans from healthy eyes of young children using HH-OCT and overhead-mounted OCT., Methods: Macular OCT scans were obtained using either HH-OCT or overhead-mounted Spectralis FLEX-OCT in children ages 0-5 years undergoing clinically indicated anesthesia/sedation. Exclusion criteria included gestational age <37 weeks, neurologic disease, amblyopia, ocular disease, or large refractive error (spherical equivalent beyond -3.00 D to +8.00 D). For HH-OCT, the GCL and INL were manually measured in pixels from single-line macular scans at the thickest point nasal and temporal to the fovea. For FLEX-OCT images, measurements were aided by automated software., Results: HH-OCT was obtained on 38 eyes (38 children, mean age 2.4 ± 1.8 years) and FLEX-OCT on 56 eyes (56 children, mean age 2.3 ± 1.5 years). Mean nasal GCL/INL was 1.24 ± 0.18 (min/max = 0.92/1.75) for HH-OCT and 1.29 ± 0.18 (min/max = 0.96/1.66) for FLEX-OCT (P = 0.11). Mean temporal GCL/INL was 1.22 ± 0.24 (min/max = 0.66/1.70) for HH-OCT and 1.19 ± 0.16 (min/max = 0.86/1.47) for FLEX-OCT (P = 0.47)., Conclusions: Mean normative GCL/INL ratios were approximately 1.2 at the thickest macular areas both nasal and temporal to the fovea, with either HH-OCT or FLEX-OCT in young children's eyes. These values may prove useful when HH-OCT is used to assess optic neuropathies, in which the GCL/INL ratio is expected to be decreased., (Copyright © 2023 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Purpose: Deformations of the retina such as staphylomas in myopia or scleral flattening in high intracranial pressure can be challenging to quantify with en face imaging. We describe an optical coherence tomography-based method for the generation of quantitative posterior eye topography maps in normal and pathologic eyes., Methods: Using "whole eye" optical coherence tomography, we corrected for subjects' optical distortions to generate spatially accurate posterior eye optical coherence tomography volumes and created local curvature (KM, mm-1) topography maps for each consented subject. We imaged nine subjects, three normal, two with myopic degeneration, and four with papilledema including one that was imaged longitudinally., Results: Normal subjects mean temporal KM was 0.0923 mm-1, nasal KM was 0.0927 mm-1, and KM local variability was 0.0162 mm-1. In myopic degeneration, subjects KM local variability was higher at 0.0836 mm-1. In papilledema subjects nasal KM was flatter compared with temporal KM (0.0709 vs. 0.0885 mm-1). Mean intrasession KM repeatability for all subjects was 0.0036 mm-1., Conclusion: We have developed an optical coherence tomography based method for quantitative posterior eye topography that offers the ability to analyze local curvature with micron scale resolution and offers the potential to help clinicians and researchers characterize subtle, local retinal deformations earlier in patients and follow their development over time.

Purpose: To detect retinal features and abnormalities on optical coherence tomography (OCT) without pupil dilation and relate these to brain injury in infants with a clinical diagnosis of hypoxic ischemic encephalopathy (HIE)., Methods: Under an institutional review board-approved protocol, we imaged eight infants without pharmacologic mydriasis, using handheld, non-contact spectral-domain (Leica Microsystems, IL) or investigational swept-source OCT at the bedside in an intensive care nursery, after birth (depending on primary clinical care team permission based on health status) and weekly until discharge. The newborn infant with HIE is neurologically unstable; therefore, pharmacologic mydriasis and stimulation with visible light for retinal examination are usually avoided. We analyzed images for retinal pathologies, central foveal thickness, and retinal nerve fiber layer (RNFL) thickness at the papillomacular bundle and compared them to historical controls and published normative data, HIE clinical assessment, and abnormalities on brain magnetic resonance imaging (MRI)., Results: On OCT, three of eight infants had bilateral multiple small macular and perimacular cystoid spaces; two of these three infants also had pronounced retinal ganglion cell layer thinning and severe brain injury on MRI and the third had bilateral paracentral acute middle maculopathy and mild brain injury on MRI. Other findings in HIE infant eyes included abnormally thin fovea and thin RNFL and markers of retinal immaturity such as the absence of sub-foveal photoreceptor development and sub-foveal fluid., Conclusions: Bedside handheld OCT imaging within the first 2 weeks of life revealed retinal injury in infants with HIE-related brain injury. Future studies may determine the relationship between acute/subacute retinal abnormalities and brain injury severity and neurodevelopmental outcomes in HIE.

PRéCIS:: Overhead mounted spectral-domain optical coherence tomography (OCT) enables high-quality imaging of the optic nerve and macula in childhood glaucoma, and is particularly useful when standard tabletop OCT has failed or is not possible., Purpose: Tabletop OCT, integral to adult glaucoma management, can be limited in childhood glaucoma patients because of young age, poor cooperation, and/or technical challenges. To address these imaging difficulties, we determined the feasibility and quality of an overhead mounted unit in childhood glaucoma. Secondary aims included evaluation of peripapillary retinal nerve fiber layer (pRNFL), parafoveal total retinal thickness, and parafoveal ganglion cell complex (GCC) thickness., Materials and Methods: Children and adults with a diagnosis of childhood glaucoma were imaged with an overhead mounted spectral-domain OCT as part of a prospective cross-sectional study. Participants had poor quality or unobtainable tabletop OCT and were scheduled for an examination under anesthesia and/or surgery as part of standard care., Results: A total of 88 affected eyes in 60 of 65 (92.3%) enrolled patients (mean age, 5.9±5.9 y; range, 0.2 to 24.5) were successfully imaged. The mean image quality for analyzed scans was 22.9±6.0 dB (n=236 images). Mean values for pRNFL (80.5±31.0 µm; n=86), parafoveal total retinal thickness (301.10±39.9 µm; n=79), and parafoveal GCC thickness (96.0±21.6 µm; n=74) were calculated., Conclusions: Overhead mounted OCT allowed high-quality image acquisition and analysis in childhood glaucoma patients unable to be imaged with the tabletop counterpart, presenting an opportunity for improved clinical management and study of childhood glaucoma-related pathophysiology. pRNFL, parafoveal total retinal thickness, and parafoveal GCC thickness were decreased for affected eyes of children under 6 years of age compared with age-matched controls from a companion normative study.

Purpose: Compared with fluorescein angiography (FA), the gold standard for diagnosing choroidal neovascularization (CNV) activity, optical coherence tomography angiography (OCTA) is non-invasive without risks associated with fluorescein dye use, and may be especially advantageous in the diagnosis and monitoring of children with CNV., Methods: Eight eyes from eight patients aged 12 months to 18 years were imaged with the investigational Spectralis OCTA (version 6.9, Heidelberg Engineering, Heidelberg, Germany) and the RTVue XR Avanti (Optovue Inc., Fremont, CA, USA). Two patients were imaged during examination under anesthesia while six patients were imaged in the clinic. Demographic information, ocular characteristics, treatment history, and imaging studies (color photos, fluorescein angiography, OCT) were collected and reviewed., Results: Three eyes had active CNV while five had quiescent CNV at the time of imaging. CNV was idiopathic or secondary to trauma, retinal vascular dysgenesis versus retinopathy of prematurity, pigmentary retinopathy, Best vitelliform macular dystrophy, panuveitis, morning glory disc anomaly, and optic disc drusen. OCTA of two active CNV demonstrated presence of a main trunk with multiple fine capillaries, vessel loops, and anastomoses. OCTA was repeated after treatment for two CNV and demonstrated a decrease in size with loss of fine capillaries, vessel loops, and anastomoses. For the third active CNV, OCTA verified flow in the CNV complex despite the uncertainty of FA hyperfluorescence in the setting of grossly abnormal retinal vasculature. The five quiescent CNV all lacked fine capillaries, vessel loops, and anastomoses on OCTA., Conclusion: OCTA demonstrates morphological differences between active and quiescent pediatric CNV.

Purpose: Measurements of the ganglion cell complex (GCC), comprising the retinal nerve fiber (RNFL), ganglion cell, and inner plexiform layers, can be correlated with vision loss caused by optic nerve disease. Handheld optical coherence tomography (HH-OCT) can be used with sedation in children who are not amenable to traditional imaging. We report GCC and RNFL measurements in normal children using HH-OCT., Design: Prospective observational study of normal children ≤5 years of age., Methods: Healthy, full-term children ≤5 years of age undergoing sedation or anesthesia were enrolled. Exclusion criteria included prematurity and pre-existing neurologic, genetic, metabolic, or intraocular pathology. Demographic data, axial length (Master-Vu Sonomed Escalon, Lake Success, New York, USA), and HH-OCT macular and optic nerve volume scans at 0° (Bioptigen, Inc., Morrisville, North Carolina, USA) were obtained. Retinal segmentation was completed with DOCTRAP software, creating average volume thickness maps., Results: Sixty-seven children (67 eyes, 31 males ranging in age from 3.4-70.9 months) were enrolled. Average axial length was 21.2 ± 1.0 mm with mean spherical equivalent +1.49 ± 1.34 diopters (range -2.25 to 4.25). Average GCC volume for the total retina was 0.28 ± 0.04 mm 3 . Forty-seven of these eyes had RNFL analysis. Average RNFL thickness of the papillomacular bundle was 38.2 ± 9.5 μm. There was no correlation between GCC volume, RNFL thickness, patient age, or axial length., Conclusion: Average GCC volume and RNFL thickness was stable from 6 months to 5 years of age. This study provides normative data for GCC and RNFL obtained by HH-OCT in healthy eyes of young children, to serve in evaluating those with optic neuropathies., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: To determine whether Spectralis (Heidelberg, Germany) spectral domain optical coherence tomography (SD-OCT) measurements are reproducible over time in children with physiologic cupping and stable glaucoma., Methods: Subjects were identified from a subset of participants in an earlier retrospective study conducted by our group and included children (<18 years of age) with physiologic cupping and stable primary congenital glaucoma (PCG) having had at least 2 SD-OCTs over a period of more than 1 between April 2010 and September 2015. Thicknesses of average peripapillary retinal nerve fiber layer (pRNFL) and six individual sectors and volumes of three segmented retinal layers and total retina were measured. Spectralis review software was used for segmentation. Intraclass correlation coefficients (ICC) and coefficient of variation (COV) were calculated., Results: A total of 35 eyes of 35 children were included: 15 eyes had physiologic cupping; 20 eyes, PCG. Mean ages at initial SD-OCT were 11.2 ± 3.3 years and 9.7 ± 3.3, respectively; mean intervals between first and last imaging were 2.2 ± 1.1 and 3.0 ± 1.4 years, respectively. ICCs across three visits for both groups for average and sectoral pRNFL thicknesses were 0.887-0.997 and for segmented retinal volumes were 0.806-0.993. ICCs for total retinal volume for physiologic cupping and PCG were 0.993 and 0.954, respectively. COVs for average pRNFL thickness were 0.9% and 1.7%, respectively. For all other measurements, COVs ranged from 0.3% to 5.4%., Conclusions: Reproducibility of longitudinal SD-OCT measurements for average pRNFL thickness in children with stable glaucoma over about 2 years is comparable to short-term reproducibility (COV) in normal children (1.16%) and normal and glaucoma adults (1.62%-3.4%)., (Copyright © 2019 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Purpose: To explore vitreoretinal pathologies and their longitudinal changes visible on handheld optical coherence tomography (OCT) of young children with familial exudative vitreoretinopathy., Methods: The authors retrospectively analyzed handheld OCT images for vitreoretinal interface and retinal abnormalities and optic nerve head (ONH) elevation., Results: From 26 eyes of 16 children (mean age 32 months) with familial exudative vitreoretinopathy, 10 had ONH dragging on photographs, and in these, handheld OCT revealed temporal and anterior retinal displacement, prominent vitreopapillary adhesion or traction, and retinal nerve fiber layer thickening at ONH margins with adjacent retinal elevation. Despite a nearly normal photographic appearance, handheld OCT revealed ONH elevation with vitreopapillary traction (6/16 eyes), ONH edema (1/16 eye), and retinal vascular protrusion (5/16 eyes). Handheld OCT-visualized vitreous abnormalities (18/26 eyes) were more prevalent at higher stages of disease. Handheld OCT-visualized elevation of ONH and the retina worsened over time in nine eyes and improved in 5/6 eyes after vitrectomy., Conclusion: Handheld OCT can detect early ONH, retinal, and vitreous changes in eyes with familial exudative vitreoretinopathy. Contraction of strongly adherent vitreous in young patients with familial exudative vitreoretinopathy appears to cause characteristic ONH dragging and tractional complications without partial posterior vitreous detachment. Vitreopapillary dragging may be visible only on OCT and may progress in the absence of obvious retinal change on conventional examination.

An investigational, portable spectral-domain optical coherence tomography (SD-OCT) unit revealed small hyperreflective opacities in both eyes of a patient with bilateral retinoblastoma. There was no evidence of vitreous seeding on ophthalmoscopy of either eye. Although the opacities may initially raise concern for potential vitreous seeding, this report suggests alternate potential etiologies for such opacities, which were also seen on imaging of a cohort of normal eyes in pediatric patients. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:50-52.]., (Copyright 2019, SLACK Incorporated.)

Limited cooperation and attention span often lead to poorly reliable assessments of visual acuity and visual fields in children, making diagnosis and monitoring of pediatric optic neuropathies challenging. As a noninvasive imaging modality, optical coherence tomography (OCT) could offer particular utility in this patient population. OCT provides high-resolution characterization of the optic nerve head, peripapillary retinal nerve fiber layer, and cellular layers of the macula, all of which can be used to assess the severity of optic nerve disease qualitatively and quantitatively. Application of OCT to pediatric patients has been limited by technical factors and lack of pediatric normative databases, but with recent technological improvements and rapidly expanding research efforts OCT is poised to revolutionize the management of optic neuropathies in children. We review current and emerging applications of OCT to important pediatric optic neuropathies such as glaucoma, papilledema, optic neuritis, optic pathway gliomas, and congenital optic disc anomalies., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Nonglaucomatous cupping is commonly encountered in neuro-ophthalmic practice. However, the progression of clinical and imaging findings over time has not been well described. We present serial fundus photographs and spectral domain optical coherence tomography from a pediatric patient with neuromyelitis optic spectrum disorder, which demonstrated progression of both cupping and optic atrophy in the setting of normal intraocular pressure.

Background: Recent Spectralis (Heidelberg, Germany) spectral domain optical coherence tomography (SD-OCT) research software can automatically quantify the thickness of each individual retinal layer. The macular ganglion cell layer (GCL) and ganglion cell complex may be more sensitive for detecting glaucoma than the peripapillary retinal nerve fiber layer (pRNFL). The aim of this study was to characterize and compare the volume of each macular layer in the eyes of children with glaucoma versus those of normal controls., Methods: The medical records of children with primary glaucoma and physiologic cupping who had undergone Spectralis SD-OCT imaging of the macula and pRNFL were reviewed retrospectively. Controls were recruited from a separate prospective study. Children with refractive error of <±5 or retinal or neurologic abnormalities were excluded. The average volume of each of the 8 retinal layers in the macula (central 6 mm) and pRNFL were compared among diagnostic groups., Results: A total of 80 eyes of 80 children were included: 37 glaucoma eyes (25 with primary congenital and 12 with juvenile open-angle glaucoma) and 43 nonglaucoma eyes (28 with physiologic cupping). Eyes with glaucoma had significantly thinner mean macular nerve fiber layers, ganglion cell layers, inner plexiform layers, and pRNFLs than nonglaucomatous eyes: 0.82 ± 0.24 μm versus 1.00 ± 0.12 μm; 0.93 ± 0.22 μm versus 1.13 ± 0.10 μm; 0.80 ± 0.14 μm versus 0.91 ± 0.07 μm; 81.6 ± 26.5 μm versus 102.7 ± 10.0 μm, respectively (P < 0.00556 for all). Eyes without cupping and those with physiologic cupping were equivalent for all variables tested., Conclusions: Children with glaucoma have thinning of the three innermost retinal macular layers., (Copyright © 2016 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Purpose: To assess retinal nerve fiber layer (RNFL) thickness at term-equivalent age in very preterm (<32 weeks gestational age) vs term-born infant cohorts, and compare very preterm infant RNFL thickness with brain anatomy and neurodevelopment., Design: Cohort study., Methods: RNFL was semi-automatically segmented (1 eye per infant) in 57 very preterm and 50 term infants with adequate images from bedside portable, handheld spectral-domain optical coherence tomography imaging at 37-42 weeks postmenstrual age. Mean RNFL thickness was calculated for the papillomacular bundle (-15 degrees to +15 degrees) and temporal quadrant (-45 degrees to +45 degrees) relative to the fovea-optic nerve axis. Brain magnetic resonance imaging (MRI) scans clinically obtained in 26 very preterm infants were scored for global structural abnormalities by an expert masked to data except for age. Cognitive, language, and motor skills were assessed in 33 of the very preterm infants at 18-24 months corrected age., Results: RNFL was thinner for very preterm vs term infants at the papillomacular bundle ([mean ± standard deviation] 61 ± 17 vs 72 ± 13 μm, P < .001) and temporal quadrant (72 ± 21 vs 82 ± 16 μm, P = .005). In very preterm infants, thinner papillomacular bundle RNFL correlated with higher global brain MRI lesion burden index (R(2) = 0.35, P = .001) and lower cognitive (R(2) = 0.18, P = .01) and motor (R(2) = 0.17, P = .02) scores. Relationships were similar for temporal quadrant., Conclusions: Thinner RNFL in very preterm infants relative to term-born infants may relate to brain structure and neurodevelopment., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: To apply automated spectral domain optical coherence tomography (SD-OCT) segmentation to eyes with resolving papilledema., Methods: Ninety-four patients with idiopathic intracranial hypertension seen at the Duke Eye Center neuro-ophthalmology clinic between November 2010 and October 2011 were reviewed. Excluded were eyes with papilledema with Frisén grade >2, other optic neuropathies or retinopathies, and those that did not have SD-OCT imaging. The remaining 43 patients were split into 2 groups: non-atrophic papilledema and atrophic papilledema. Automated SD-OCT segmentation was performed on patients with non-atrophic papilledema and age-matched controls for each of the 9 regions of the Early Treatment Diabetic Retinopathy Study map. Bonferroni correction was used for multiple comparisons. All SD-OCT scans were reviewed for retinal structural abnormalities., Results: Total macular thickness was significantly thinner within the fovea and inner macular ring in non-atrophic papilledema vs control eyes (266 vs 276 μm, P = 0.04; 333 vs 344 μm P < 0.01, n = 26 non-atrophic papilledema, 30 controls). SD-OCT demonstrated thinning within the fovea, inner macular ring, and outer macular ring of the outer plexiform layer plus nuclear layer in non-atrophic papilledema vs control (124 vs 131 μm, P < 0.01; 112 vs 118 μm, P = 0.03; 95 vs 100 μm, P = 0.03). Retinal structural changes were seen in 21/33 eyes with atrophic papilledema vs none of the eyes with non-atrophic papilledema or controls., Conclusions: SD-OCT shows qualitative and quantitative changes in the macula of eyes with resolved papilledema.

Purpose: To determine the reproducibility of spectral-domain optical coherence tomography (OCT) measurements of peripapillary retinal nerve fiber layer (RNFL) and macular retinal thickness of the central 20-degree area of the posterior pole in adult and pediatric glaucoma and normal subjects., Study Design: Prospective., Methods: Normal, glaucoma suspect and glaucoma eyes of adult and pediatric subjects were recruited. Spectralis OCT scans were obtained 3 times on the same day (intravisit) and 4 weeks later (intervisit). Six individual sectors, average RNFL, and macular retinal thickness using an 8×8 mm grid centered on the foveal pit were measured for all the eyes. The central 16 square areas centered on the foveal pit were called the central macular area and the surrounding 48 square areas were called the perimacular area. Intraclass correlation coefficient, coefficient of variation (COV), and test-retest variability were calculated., Results: Included were 51 adult eyes and 25 pediatric eyes. The intravisit and intervisit intraclass correlation coefficient for the central macular, perimacula, and average RNFL was very good (>0.8) for all adult and pediatric normal and glaucomatous eyes. The mean COVs of normal and glaucomatous eyes were higher for average RNFL (1.5 ± 1.5% adults, 1.0 ± 0.9% pediatric) compared to the central macula (0.56 ± 0.54% adults, 0.68 ± 0.64% pediatric) and perimacular thickness (0.64 ± 0.51% adults, 0.57 ± 0.42% pediatric) (1-way analysis of variance, P<0.05). No difference was noted in the intravisit and intervisit COVs between the adult and pediatric eyes of central macula, perimacula, and average RNFL thickness (unpaired t test, P>0.05). No intervisit reproducibility analysis was available for pediatric glaucoma eyes., Conclusion: The reproducibility of Spectralis spectral-domain-OCT were very good for both RNFL and macular thickness measurements in glaucoma/glaucoma suspect eyes of adult and pediatric subjects.

Purpose: To identify optic nerve head (ONH) cupping reversal and associated optical coherence tomography (OCT) and Humphrey visual field changes in pediatric glaucoma., Design: Retrospective observational case series., Methods: Sequential surgical cases of juvenile open-angle glaucoma (OAG) or primary congenital glaucoma (PCG) with sustained postoperative intraocular pressure (IOP) reduction. Group 1 had preoperative and postoperative ONH photographs and OCT; Group 2 had preoperative clinical ONH assessment and postoperative imaging. Cupping evaluation was confirmed by masked glaucoma and neuro-ophthalmology specialists., Results: Of 80 cases, 9 eyes (9 children) met criteria for Group 1; 24 eyes (19 children) met criteria for Group 2. Group 1: Five of 9 eyes (56%) demonstrated cupping reversal, with preoperative vs postoperative mean IOP 34.2 ± 6.6 mm Hg vs 10.6 ± 4.1 mm Hg (P < .00001) and mean average retinal nerve fiber layer (RNFL) 71.0 ± 30 μm vs 62.8 ± 24 μm (P = .4), respectively. RNFL was stable in 4 of 5 eyes (all juvenile OAG), but thinned (Δ = -41 μm) in 1 eye with PCG. Humphrey visual fields (reliable in 2 of 3 eyes) showed no significant change. Group 2: Fourteen of 24 PCG eyes (58%) demonstrated cupping reversal, with preoperative vs postoperative mean IOP 36.1 ± 8.9 mm Hg vs 13.3 ± 2.1 mm Hg (P < .00001). Two eyes had thin RNFL postoperatively despite healthy-appearing ONH. Postoperative RNFL showed statistically significant linear correlation with preoperative (but not postoperative) cup-to-disc ratio. Limitations include small numbers, few reliable Humphrey visual fields, and absent preoperative imaging (Group 2)., Conclusion: Some eyes with IOP reduction and ONH cupping reversal show continued RNFL thinning postoperatively. The preoperative ONH cup-to-disc ratio predicted the postoperative RNFL better than the postoperative "reversed and smaller" cup-to-disc ratio. Cupping reversal in pediatric glaucoma may not predict improved ONH health and deserves further study., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Purpose: To evaluate effects of prematurity on early optic nerve (ON) development and the usefulness of ON parameters as indicators of central nervous system (CNS) development and pathology., Design: Prospective, cross-sectional, longitudinal study., Participants: Forty-four preterm infants undergoing retinopathy of prematurity (ROP) screening and 52 term infants., Methods: We analyzed ON from portable handheld spectral-domain optical coherence tomography (SD-OCT) images (Bioptigen, Inc, Research Triangle Park, NC) of 44 preterm and 52 term infants. The highest-quality ON scan from either eye was selected for quantitative analysis. Longitudinal analysis was performed at 31-36 weeks and 37-42 weeks postmenstrual age (PMA). Preterm ON parameters also were assessed for correlation with indicators of cognitive, language, and motor development and CNS pathology., Main Outcome Measures: Vertical cup diameter (vCD), vertical disc diameter (vDD), vertical cup-to-disc ratio (vCDR), cup depth, and indicators of neurocognitive development and CNS pathology., Results: At 37-42 weeks PMA, preterm infants had larger vCD and vCDR than term infants (908 vs. 700 μm [P<0.001] and 0.68 vs. 0.53 μm [P<0.001], respectively), whereas cup depth and vDD were not significantly different. Longitudinal changes (n = 26 preterm eyes; mean interval, 4.7 weeks) in vDD and in vCDR were an increase of 74 μm (P = 0.008) and decrease of 0.05 (P = 0.015), respectively. In preterm infants (n = 44), periventricular leukomalacia was associated with larger vCD (1084 vs. 828 μm; P = 0.005) and vCDR (0.85 vs. 0.63; P<0.001), posthemorrhagic hydrocephalus was associated with shallower cup (331 vs. 456 μm; P = 0.030), and clinical magnetic resonance imaging was associated with larger vCDR (0.73 vs. 0.64; P = 0.023). In 23 preterm infants with Bayley Scales of Infant Development scores, larger vCDR was associated with lower cognitive scores (P = 0.049)., Conclusions: This is the first analysis of ON parameters in premature infants using SD-OCT. It demonstrated that by age of term birth, vCD and vCDR are larger in preterm infants who were screened for ROP than in term infants. In this prospective pilot study, ON parameters in these preterm infants associate weakly with CNS pathology and future cognitive development. Future prospective studies with larger numbers are necessary before further conclusions can be made., (Copyright © 2014 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To characterize optic nerve head (ONH) morphology and parameters, including vertical disk diameter, vertical cup diameter, and vertical cup/disk ratio in healthy, full-term newborns using a handheld spectral domain optical coherence tomography (SD-OCT) device., Methods: In this prospective observational case series, healthy white, black, and Hispanic full-term newborns delivered at the Duke Birthing Center between August 2010 and May 2011 underwent dilated fundus examination and SD-OCT imaging of the optic nerve in each eye. OCT parameters were calculated and compared for each group of infants., Results: A total of 58 consecutive newborns of white (n = 22), black (n = 15) and Hispanic (n = 21) ethnicity were included. Mean vertical disk diameter in white, black, and Hispanic newborns was 1.29 ± 0.15 mm (standard deviation), 1.38 ± 0.14 mm, and 1.38 ± 0.14 mm, respectively (white versus Hispanic, P = 0.02; white versus black, P = 0.07). Mean vertical cup diameter in white, black, and Hispanic newborns was 0.44 ± 0.15 mm, 0.56 ± 0.23 mm, and 0.46 ± 0.30 mm, respectively (white versus black, P = 0.03). Mean vertical cup/disk ratio was 0.34 ± 0.10 for white, 0.40 ± 0.17 for black, and 0.33 ± 0.20 for Hispanic newborns (P = 0.07 for white versus black)., Conclusions: Handheld SD-OCT is an effective means of imaging the ONH in newborns. Racial differences in cup/disk ratio are present at birth. These data may serve as the beginning of a normative dataset for characterizing development of the ONH as well as for comparison to the neonatal ONH in disease states., (Copyright © 2013 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Purpose: To address the misrepresentation of the eye in retinal optical coherence tomography (OCT) images and to examine the effect of this misrepresentation on retinal thickness measurements., Design: Prospective case series., Methods: Five subjects with recent orbital magnetic resonance imaging (MRI) scans and normal eye examinations were consented from the clinics of the Duke Eye Center. Each subject had both eyes imaged using a retinal spectral-domain OCT system and ocular biometry measured. Two types of individualized optical models of the subject eyes-numerical and analytical-were used to determine the spatial paths of the OCT A-scans. These paths were used to reorient the A-scans in the associated retinal OCT images and generate corrected images. Using curvature as a general measure of shape, the radii of curvature of the retinal pigment epithelium in the original and corrected OCT images were compared to the ocular radii of curvature in the MRI images. Differences between the retinal thickness maps derived from the original and corrected OCT images were then determined., Results: The retinal curvatures were substantially flatter in the original OCT than in the MRI images (mean paired difference: 52.8 ± 41.8 mm, P < .001). Correcting the OCT images decreased the paired differences between OCT and MRI (numerical: 1.6 ± 2.3 mm, P = .091; analytical: 1.9 ± 4.3 mm, P = .278). Retinal thickness measurements between the corrected and uncorrected images differed, with a root mean square difference of 5.61 μm over the entire 6-mm extent of the image; this difference was greater peripherally (6.02 μm) than centrally (2.54 μm)., Conclusions: Optically based algorithms can be used to correct the shape of the retina as represented in OCT; this correction makes OCT more consistent with other clinical imaging techniques. Resultant retinal thickness maps were minimally affected by the change in shape. Ocular shape correction should be considered in future development of posterior segment OCT-based morphologic measurements., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background/aims: Spectral-domain (SD)- Optical Coherence Tomography (OCT) can track eye movements, has faster acquisition time and higher resolution than time-domain(TD)-OCT. The aim of the study was to assess the utility of SD-OCT in paediatric glaucoma and determine its agreement with TD-OCT., Methods: Children who had SD-OCT(Spectralis, Heidelberg-Engineering,Germany) were retrospectively and prospectively identified from Duke paediatric glaucoma clinic. The peripapillary retinal nerve fibre layer (RNFL) and macular thickness and volume (MV) were compared amongst four groups: normal eyes, eyes with physiologic cupping (C:D >0.5 and <0.8, IOP <21), mild glaucomatous eyes (C:D <0.5, intra-ocular pressure (IOP) >21) and severe glaucoma (C:D>0.5, IOP>21). SD-OCT values were compared to TD-OCT(OCT-3, Carl-Zeiss-Meditec, Dublin, CA) values in a subset of subjects who had same day scans using both instruments. Children with neurologic disorders, refractive error >±5D, pseudophakia and prematurity were excluded., Results: Included were 83 eyes of 83 children, mean age 11.9 ± 4.2 years. SD-OCT measurements of average RNFL thickness and MV differed among normals(n=24), physiologically cupped (n=31), mild (n=15) versus severe glaucoma (n=13): (RNFL:104 ± 9, 99 ± 6, 98 ± 9 vs 62 ± 18 µm, respectively, p<0.05; MV: 8.7 ± 0.3, 8.6 ± 0.3, 8.8 ± 0.4 vs 8.0 ± 0.6 mm³;, respectively, p<0.05). Same-day SD-OCT and TD-OCT measures in 53 eyes correlated linearly (RNFL r(2)=0.88; MVr(2)=0.58). SD-OCT measured lower RNFL and higher macular thickness than TD-OCT. Among eyes with severe glaucoma, 4 of 13 (30%) had unreliable TD-OCT but reliable SD-OCT., Conclusions: SD-OCT was easier to obtain than TD-OCT in children. SD-OCT and TD-OCT measurements correlated, but values were not interchangeable.

Purpose: To evaluate the longitudinal reproducibility of optical coherence tomography (OCT) measurements in normal and glaucomatous eyes of children., Methods: In this 2-setting prospective study, OCT-3 was used to obtain fast retinal nerve fiber layer (RNFL) and macular thickness scans. In the first study setting, the normal eyes of healthy children were scanned on presentation, at 2 weeks, and 3 years, with axial length measured at the first and last examinations. In the second setting, OCT scans of patients in the pediatric glaucoma clinic were performed over 4 years as clinically indicated. Eyes were classified as "normal" (normal eyes and those with physiologic cupping but normal intraocular pressure [IOP]); "mild glaucoma" (increased IOP and a normal optic nerve appearance); or "advanced glaucoma" (severe cupping or progressive glaucoma). Intraclass correlation coefficients were used to evaluate the reproducibility of measurements on the same day and over time., Results: In the first setting, 8 normal eyes were included. Axial length increased 0.11 ± 0.04 mm/year over an average of 3.3 years (P = 0.03); there was no statistically significant change in RNFL thickness (P = 0.30). In our second setting, 27 normal eyes and 37 eyes with glaucoma were included. Intraclass correlation coefficients across the 3 visits for total macular volume were 0.80-0.91 and for average RNFL were 0.73-0.95., Conclusions: Global OCT measurements in children were reproducible over years and were not affected by normal increase in axial length. OCT shows promise as an objective tool for longitudinal assessment of children., (Copyright © 2012 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Children with large optic nerve head cups often pose diagnostic difficulty due to concern over possible glaucoma. This study's purpose was to evaluate optical coherence tomography (OCT) measurement of the peripapillary retinal nerve fiber layer (RNFL) and macular thickness in healthy eyes of black and white children, comparing values for eyes with large cup-to-disc ratios against those with small cup-to-disc ratios (normal controls). Using Stratus OCT (OCT 3) (Carl Zeiss Meditec, Dublin, CA) a fast macular thickness and fast RNFL 3.4 protocol were performed on normal eyes of normal children. Included were children (aged 5-17 years) with normal ophthalmic examinations, drawn from data generated during an ongoing study of OCT in children. Excluded were eyes with corrected vision below 20/20, spherical equivalent >5 diopters, intraocular pressure >21 mm Hg, abnormal optic nerve rim, prematurity, family history of glaucoma. Clinical examination identified children with large cup-disc-ratios (0.5-0.8), and each was matched for age and race to a "normal control" with a small cup-to-disc ratio (<0.5). OCT-3 analyses were compared between eyes with optic nerve heads showing large cup-to-disc ratios (≥0.5) vs. those showing small cup-to-disc ratios (<0.5) for different sections of the macula and the peripapillary RNFL thickness. Analyses included OCT values and optic cup grading for 162 eyes of 162 children. White children with large cup-to-disc ratios had thinner mean RNFL thickness and macular volume compared to those with small cup-to-disc ratios (average RNFL = 100 ± 9 vs. 110 ± 8 μm, respectively, p = 0.0001, macular volume = 6.84 ± 0.41 vs. 7.03 ± 0.33 mm(3), respectively, p = 0.0186). On the other hand, black children with large cup-to-disc ratios had an equivalent mean RNFL thickness and macular volume compared to those with small cup-to-disc ratios average RNFL = 107 ± 10 vs. 107 ± 8 for both groups, macular volume = 6.81 ± 0.34 vs. 6.85 ± 0.28 respectively. Positive equivalence testing assumed a clinically significant difference of 10 μm for RNFL, and 0.2 mm(3) for macular volume. We report that white children with large cup-to-disc ratios have evidence of reduced/thinner RNFL and macular volume versus white children with smaller cup-to-disc ratios, as assessed by OCT. By contrast, this distinction was not found for black children in the present study., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Purpose: Neurofibromatosis type 1 (NF1) is the most common neurocutaneous disorder, with an approximate incidence of 1 in 3,500. Optic pathway gliomas (OPGs) develop in 15% of individuals with NF1, commonly in childhood. OPGs are difficult to detect via a clinical inspection in children, often requiring magnetic resonance imaging (MRI). Given the significant visual risks associated with OPGs in NF1, there is a need for improved noninvasive techniques to diagnose OPGs in children; therefore, we studied optical coherence tomography (OCT) as a potential tool to assess optic nerve and retinal nerve fiber layer (RNFL) abnormalities. This prospective study was designed to evaluate OCT detection of RNFL loss from optic atrophy attributable to OPGs in a cohort of pediatric patients with NF1., Methods: With the use of Stratus OCT, directed testing with the Fast Macular Thickness and Fast RNFL Thickness protocol scans were performed on 9 subjects with NF1 and known OPGs, 6 subjects with NF1 without OPGs, and 15 controls., Results: NF1 subjects with OPGs had thinner RNFLs and macula when compared with age-matched controls and to NF1 subjects without OPGs. After applying the equivalence equation, the average RNFL thickness and macular volume in NF1 subjects without OPGs was equivalent to controls., Conclusions: Our study suggests that OCT can be used to detect RNFL thinning secondary to OPGs in NF1 subjects. This objective tool shows promise as a useful adjunct to routine clinical ophthalmologic evaluation in children with NF1., (Copyright © 2010 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Objective: To collect a normative database of macular thickness, retinal nerve fiber layer (RNFL) thicknesses, and optic nerve topography in the healthy eyes of children aged 3 to 17 years using optical coherence tomography (OCT) measurements., Methods: Scans were obtained for 286 healthy children (black, 114; white, 154; other, 18). Each child had a dilated eye examination, an axial length measurement using the IOL Master (Carl Zeiss Meditec, Dublin, California), and OCT measurements using the fast macular map, fast RNFL thickness, and fast optic disc protocols of the Stratus OCT (OCT-3; Carl Zeiss Meditec)., Results: Black children had smaller macular volume and foveal thickness, larger RNFL thickness, and larger cup-disc area ratios compared with white children. Macular volume and average outer macular thickness correlated negatively with axial length in white children. Foveal thickness correlated positively with age in black children only. Average RNFL correlated negatively with axial length in white children only (P < .05 for all). Normative data for all variables were recorded and compared with reported adult values., Conclusions: Stratus OCT-3 measurements of macular and RNFL thickness and optic nerve topography vary with race, axial length, and age in healthy children. Normative pediatric OCT data should facilitate the use of OCT in assessing childhood glaucoma and other diseases.

Purpose: To evaluate optical coherence tomography (OCT) measurement of peripapillary retinal nerve fiber layer (RNFL) and macular thickness in children with pseudotumor cerebri., Methods: Prospective observational series of children with pseudotumor cerebri compared against controls matched for age and gender. We included 11 pediatric subjects with clinical pseudotumor cerebri and 37 normal controls. Subjects underwent a complete eye examination including stereo disk photographs and OCT. Peripapillary RNFL and macular thickness were evaluated using the Stratus OCT 3000. The peripapillary RNFL was evaluated with two protocols: Fast RNFL Map protocol with values divided in eight sections, made up of four quadrants, each with an inner and outer ring, and Fast RNFL Thickness (3.4) protocol, using values presented for four quadrants. A Fast Macular Thickness Map was also obtained, and results reported similarly included eight sections, made up of four quadrants, each with an inner and outer ring., Results: The Fast RNFL Map protocol showed an increased RNFL thickness (pseudotumor cerebri vs controls) in the temporal and superior quadrants. The Fast RNFL Thickness (3.4) protocol showed a thicker average RNFL for pseudotumor cerebri eyes compared with controls (125.7 vs 106.5 microm, p < 0.0001). The Fast Macular Thickness Map showed an increased RNFL thickness in the nasal quadrant for the inner ring. The macular volumes for pseudotumor cerebri versus control eyes were 7.21 versus 6.97 mm(3), respectively (p = 0.005)., Conclusions: OCT shows increased RNFL and macular thickness in pseudotumor cerebri and may be a useful clinical tool in these children.