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PURPOSE: Prior studies on compressive optic neuropathy (CON) have come from large tertiary centers, which may contain referral bias toward more severe or atypical disease. To our knowledge, there are no studies to determine the population-based etiologies and clinical outcomes of compressive optic neuropathy (CON). This study aims to bridge that gap using the Rochester Epidemiology Project database. DESIGN: Retrospective, population-based cohort METHODS: Medical records of all residents living in Olmsted County, Minnesota from January 1, 2000 through December 31, 2018 were screened for CON. Demographic and clinical information were collected before and after treatment. RESULTS: 23 patients had a confirmed diagnosis of CON during our study period, which provided an overall incidence of 1.14 per 100,000 per year. Average age at onset of CON was 51 years (SD 24), and 39% were male. The most common etiologies were pituitary adenoma and meningioma. There was significant improvement in visual fields (p < 0.003) but not in visual acuity (p = 0.08) after patients underwent treatment for CON. There was also a significant relationship between the time until treatment and the degree of visual field improvement at follow-up (Pearson correlation rho = −0.58, p < 0.047). CONCLUSION: To our knowledge, this study provides the first population-based incidence of CON. The finding that earlier treatment leads to better visual outcomes stresses the importance of having CON on the differential diagnosis of patients with optic neuropathy.

PURPOSE This study is the first to evaluate the prevalence of retinal thinning and the correlation with papilledema and visual acuity (VA) in a large population with craniosynostosis. DESIGN Prospective clinical cohort study. METHODS All and complex patients syndromic and complex with craniosynostosis who visited the only national referral center between 2018 and 2020 were included. Retinal layers were segmented using optical coherence tomography. Patients were seen by an ophthalmologist for VA assessment and fundoscopy. Multivariate regression models were developed to evaluate correlations between retinal thickness, papilledema and VA. RESULTS We included 127 patients. Retinal thinning was most prevalent in the peripapillary retinal nerve fiber layer (≤38%). A longer duration of papilledema in early childhood correlated with an increased peripapillary retinal nerve fiber layer and total retinal thickness optic nerve head later in life (+0.3 ± 0.2, P = .04 and +1.0 ± 1.0, P = .003); however, its thickness was not correlated with the VA (P = .20 and P = .53). Macular retinal thinning was associated with a worse VA (P = .01); however, it was not correlated with the duration of papilledema (P = .95). CONCLUSIONS Following a preventative treatment strategy for syndromic and complex craniosynostosis, the prevalence of retinal ONH thinning is low. Although the prevalence of peripapillary retinal nerve fiber layer thinning is considerable, its thickness is not correlated with VA. In contrast, macular thinning is correlated with worse VA scores and should, therefore, be evaluated during follow-up. Future studies should evaluate the (1) causative mechanism for macular thinning, (2) correlation between the time to surgery and macular thinning, and (3) results of reactive treatment strategies and compare those results to the current study.

Purpose To evaluate the relationship between the occurrence of optic disc hemorrhages (DH) and glaucoma progression as determined by multiple glaucoma testing modalities. Design A prospective cohort study. Methods A longitudinal study of 124 open angle glaucoma patients who had yearly disc photography (DP), visual fields (VFs), spectral-domain optical coherence tomography (SD-OCT) retinal nerve fiber layer (RNFL) thickness scans, and optic nerve volume scans (Spectralis, Heidelberg Engineering, Heidelberg, Germany), all performed on the same day over a 5-year period. The minimum distance band (MDB) thickness, a 3D neuroretinal rim parameter, was calculated from optic nerve volume scans. Patients were classified as glaucoma progressors or non-glaucoma progressors using event-based analysis. Results Out of 124 open angle glaucoma patients, 19 (15.3%) had one or more DHs on yearly DPs. Presence of a DH was associated with localized 3D neuroretinal rim thickness progression (superior MDB progression; OR 3.96, P = 0.04), but not with global or inferior MDB progression (P = 0.14 and 0.81 respectively), DP progression (P = 0.08), VF progression (P = 0.45), or RNFL global, inferior or superior progression (P = 0.17, 0.26 and 0.76 respectively). In the majority of patients with MDB progression (14/17, or 82%), the progression was noted before or concurrently with the first instance of DH. Conclusions Glaucoma progression detected by high-density 3D SD-OCT neuroretinal rim measurements preceded DH occurrence in the majority of patients. These findings support the hypothesis that DHs are indicators of ongoing glaucoma progression rather than discrete events that cause subsequent progression.

To investigate the clinical characteristics of optic nerve head (ONH) prelaminar schisis in eyes with advanced glaucoma.Cross-sectional study.One hundred sixteen eyes with advanced glaucoma (30-2 mean deviation-12 dB) were included. ONH prelaminar schisis was identified using the spectral-domain optical coherence tomography independently by 2 evaluators and only eyes that reached consensus for the presence of ONH prelaminar schisis were included. Bruch membrane opening-minimum rim width (BMO-MRW), thickness and depth of lamina cribrosa (LC), peripapillary retinal nerve fiber layer (RNFL) thickness, macular thickness (total, RNFL, ganglion cell layer, inner plexiform layer), and peripapillary and subfoveal choroidal thickness were additionally obtained. Clinical characteristics were compared between the 2 groups based on the presence of ONH prelaminar schisis.ONH prelaminar schisis was identified in 48 of 116 eyes. Multivariate logistic regression analysis revealed that short axial length, thin and deep LC, and thick macula were associated with the presence of ONH prelaminar schisis. When the structure-function relationships were determined, macular structural parameters tended to have a better relationship with functional parameters than the BMO-MRW and peripapillary RNFL thickness parameters in eyes with ONH prelaminar schisis.The ONH prelaminar schisis was associated with thin and deep LC, short axial length, and generally thick macula. In patients with this features, the macular measurements, rather than peripapillary or ONH measurements, better predict the functional status of the eye. Our findings may have significant clinical implications for management of advanced glaucoma eyes with and without ONH prelaminar schisis.

The purpose of this study was to investigate the association between the vertical position of the central retinal vessel (CRV) within the optic nerve head (ONH) and the site of visual field defects (VFDs) in glaucoma.Cross-sectional study.The vertical position of the CRV was identified in 134 glaucoma eyes and 61 normal eyes at the point at which CRV exited the lamina cribrosa (LC) onto the ONH surface, by using spectral-domain optical coherence tomography (exit position). The position was also identified at the entry point into the LC from the retrolaminar ONH region (entry position), which was little influenced by glaucomatous LC deformation, therefore close to the original position before the glaucoma development. Positions were compared among glaucoma eyes with different sites of VFDs, and between glaucoma and normal eyes.In glaucoma eyes, the entry position of the CRV was in the superior ONH region in 63.0% of eyes with superior VFDs and in the inferior ONH region in 97.8% of eyes with inferior VFDs (P.0001). The exit position exhibited a similar percentage. The vertical CRV positions were not significantly different between glaucoma and normal eyes, both at the entry and exit positions.Eyes with CRVs in the superior ONH region were significantly more likely to form VFDs in the superior hemifields and vice versa. The vertical position of the CRV was little altered by the development of glaucoma. The original position of the CRV before the development of glaucoma may influence regional susceptibility to glaucomatous stress and may be useful in predicting initial sites of VFDs.

Purpose To evaluate clinically the efficacy of 360-degree minimally invasive endoscopic surgery in patients with orbital intraconal tumors. Design Retrospective interventional case series. Methods A series of consecutive patients with orbital intraconal tumors were treated with minimally invasive surgery using an endonasal endoscopic approach or an anterior endoscopic orbitotomy over a 5-year period between January 2015 and January 2020 at the National Institute of Oncology and Radiobiology of Cuba. Results In 22 patients, the mean age was 52.3 ± 11 years (range 18-71 years) with a female to male ratio of 1.8:1. Visual loss was present in 3 patients and proptosis was present in 19 patients. The most common diagnosis was cavernous hemangioma (72.7%) followed by orbital sheet meningioma (9.1%). The lower-inner (type II) location was the most usual, followed by the lower-outer (type IV). A gross total resection was achieved in 95.5% of patients. In only 1 case with an orbital pseudotumor was the resection subtotal because of the fibrous consistency, irregularity, and adherences to the medial rectus muscle. All patients with visual loss improved to normal visual function after surgery at follow-up and orbital proptosis recovered in all patients with these symptoms. Both immediate and long-term morphologic and aesthetic results were good in all patients. Conclusion The minimally invasive 360-degree surgical approach with a full endoscopic visualization can be safe and efficient in patients with select orbital intraconal tumors. Optic nerve location constitutes the main aspect in the surgical approach selection. A large case series is necessary to standardize this surgical philosophy.

Purpose To identify characteristics of patients with early open-angle glaucoma exhibiting greater macular perfusion density (PD) loss compared with macular ganglion cell layer (GCL) thickness loss. Design Cross-sectional study. Methods Optical coherence tomography (OCT) imaging of the optic nerve head and macula was conducted in patients and healthy control subjects. Minimum rim width, retinal nerve fiber layer and GCL thickness, and PD from OCT angiography were derived. Only high-quality images were included. For direct comparison, raw PD and GCL thickness values in patients were converted to relative age-corrected loss values based on data from controls. Demographic and ocular variables related to greater PD loss compared with GCL thickness loss were identified with multivariate logistic regression. Results Data from 89 patients (median mean deviation with the 24-2 and 10-2 tests, Humphrey Field Analyzer: −1.96 dB and −1.49 dB, respectively) and 54 controls were analyzed. Sixty-three (71%) patients had relatively more GCL thickness loss, whereas 26 (29%) had relatively more PD loss. More PD loss was associated with lower OCT and OCT-angiography signal strength (odds ratio [95% confidence interval], 0.64 [0.40, 0.96] and 0.60 [0.38, 0.86], per dB, respectively), thicker retinal nerve fiber layer thickness (1.08 [1.01, 1.16] per μm), and female sex (6.57 [1.25, 48.79]). Conclusion Less than one-third of patients with early glaucoma had more loss of perfusion compared with conventional structural loss in the macula. Even within a range of high-quality images, lower signal strength may be at least partially responsible for apparent perfusion loss.

To evaluate the International Classification of Disease, Tenth Revision (ICD-10) codes used for glaucoma severity classification, which are based on the location of visual field (VF) defects; given the known poor sensitivity of the 24-2 visual field test to early disease and macular damage, we hypothesized that the ICD-10 codes would not accurately reflect the extent of glaucomatous damage.Retrospective validity and reliability analysis.We evaluated 80 eyes with glaucomatous optic neuropathy (GON). Masked reviewers assigned an ICD-10 severity grade based on 24-2 VF. Two additional masked examiners determined the presence of optical coherence tomography (OCT) structural damage in each hemifield and/or central 5 degrees to define an OCT-based equivalent ICD-10 classification.A total of 80 eyes with GON were classified as mild, moderate and advanced in 15, 23, and 42 cases, respectively, based on the 24-2 VF, and in 6, 7, and 67 cases, respectively, based on OCT. The OCT classifications were more severe in 29 of 80 cases (36%). In 33 cases (41.3%), macular damage detected by OCT was missed by the 24-2. In 4 of 80 cases (5%), the VF overestimated the severity, likely due to variability of the 24-2 test.The ICD-10 system relies solely on damage seen on the 24-2 and as provides a 24-2 functional score rather than a "glaucoma" severity score. OCT revealed wide variation of damage across grades, with a significant proportion of the eyes showing macular structural damage missed with the 24-2 VF. Adding OCT information to the ICD-10 system would help it to more accurately reflect the extent of glaucomatous damage.

Purpose To identify optical coherence tomography angiography (OCTA) derived vessel metrics of the macula and optic nerve head (ONH) that predict diabetic retinopathy (DR) disease progression. Design Secondary analysis of clinical trial data Methods This was a sub-analysis of prospectively collected data from 73 subjects that participated in the TIME-2b Study (Aerpio Pharmaceuticals), a multi-center clinical trial for patients with moderate to severe DR treated with AKB-9778 and followed over a 12-month period. Eligible subjects were tested every 3 months with color fundus photography, spectral domain OCT and slit-lamp biomicroscopy. OCTA of the macula and ONH was obtained for a subset of patients enrolled at participating sites. En-face, full-depth retinal projections centered at the macula were analyzed for multiple metrics including foveal avascular zone (FAZ) area and perimeter, non-perfusion area, vessel density (VD) and presence of intraretinal microvascular abnormalities (IRMA). VD of the radial peripapillary capillaries was evaluated in four quadrants surrounding the optic disc for ONH images. Progression was defined as a ≥ 2-step increase in DR severity scale score or development of diabetic macular edema. Results Over a follow-up period of 12 months, 15 of 73 (20.5%) subjects progressed. At pre-treatment baseline, larger FAZ area, presence of IRMA, and reduced peripapillary VD in the superior temporal and inferior temporal regions were significantly associated with increased odds of progression. Conclusions FAZ area and temporal peripapillary VD are predictors of DR progression. OCTA metrics may improve progression risk assessment in DR when compared to established risk factors alone.

Purpose A challenging clinical scenario is distinguishing between normal tension glaucoma (NTG) and non-glaucomatous optic neuropathies (NGON). The key to the assessment remains identifying the presence of optic nerve head cupping. Recent optical coherence tomography (OCT) measurements now allow objective assessment of cupping by minimum rim width at Bruch's membrane opening (MRW-BMO). This study assessed the hypothesis that the MRW-BMO measurement quantifies cupping and therefore can differentiate between NTG and NGON. Design Diagnostic evaluation with area under the curve. Methods Setting: multicenter tertiary hospitals and outpatient clinics. Patient population: 81 eyes of 81 patients were enrolled, 27 with NTG and 54 with NGON, including ischemic optic neuropathy, previous optic neuritis, and compressive and inherited optic neuropathies. All NGON patients with intraocular pressure >21 mm Hg, narrow drainage angles, or a family history of glaucoma were excluded. Observational procedure: optic disc OCT images were obtained of both the retinal nerve fiber layer thickness and the MRW-BMO. Main outcome measurements: the utility of the MRW-BMO in differentiating GON from NGON was assessed using the area under the curve (AUC) estimated from a logistic regression model. Results The 5-fold cross-validated AUC for glaucoma versus nonglaucoma from logistic regression models using MRW-BMO values from all sectors was 0.95 (95% confidence interval: 0.86-1.00). Conclusions The measurement of MRW-BMO effectively differentiates between NTG and NGON with a high level of sensitivity and specificity. Incorporating this measurement into routine glaucoma assessment may provide a robust method of assisting clinicians to improve diagnosis and therefore treatment of optic nerve diseases.

Purpose To measure the magnitude and direction of anterior scleral canal opening (ASCO) offset relative to the Bruch membrane opening (BMO) (ASCO/BMO offset) to characterize neural canal obliqueness and minimum cross-sectional area (NCMCA) in 69 highly myopic and 138 healthy, age-matched, control eyes. Design Cross-sectional study. Methods Using optical coherence tomography (OCT) scans of the optic nerve head (ONH), BMO and ASCO were manually segmented and their centroids and size and shape were calculated. ASCO/BMO offset magnitude and direction were measured after projecting the ASCO/BMO centroid vector onto the BMO plane. Neural canal axis obliqueness was defined as the angle between the ASCO/BMO centroid vector and the vector perpendicular to the BMO plane. NCMCA was defined by projecting BMO and ASCO points onto a plane perpendicular to the neural canal axis and measuring their overlapping area. Results ASCO/BMO offset magnitude was greater (highly myopic eyes 264.3 ± 131.1 μm; healthy control subjects 89.0 ± 55.8 μm, P Conclusions Our data suggest that increased temporal displacement of BMO relative to the ASCO, increased BMO and ASCO area, decreased NCMCA, and increased neural canal obliqueness are characteristic components of ONH morphology in highly myopic eyes.

Purpose To investigate the structural characteristics of the posterior sclera around the optic nerve head (ONH) in patients with normal-tension glaucoma (NTG) and myopia with central visual field (VF) defect. Design Cross-sectional study. Methods Ninety-seven eyes of 97 NTG patients with myopia were included in this study. Swedish interactive thresholding algorithm (SITA) 24-2 and 10-2 were both performed to compare the central VF with peripheral VF. Optic disc torsion, tilt, and peripapillary atrophy area were calculated in 2-dimensional disc photographs. The most posterior point—that is, the deepest point of the eye (DPE)—was identified in 3-dimensional en face optical coherence tomography results and related measurements were calculated using built-in software. Results Forty-two eyes had worse SITA 10-2 mean deviation (MD) than SITA 24-2 MD and they were assigned to the central dominant VF defect group. The central dominant VF defect group had larger disc torsion and ONH tilt angle and shallower disc-DPE depth than the peripheral dominant VF defect group. According to logistic regression analysis, large ONH tilt angle and shallow disc-DPE depth were significant factors associated with central visual function impairment. Conclusions Subjects with worse central VF than peripheral showed greater peripapillary scleral deformation, such as in torsion and tilt. Their posterior scleral deformation appeared to be closer to the ONH. Scleral deformation around the ONH may be associated with papillomacular bundle and central VF defects.

Purpose To evaluate and compare the morphologic characteristics of buried optic disc drusen (ODD) and optic disc edema (ODE) by using en face optical coherence tomography (OCT) and OCT angiography (OCTA). Design Retrospective, cross-sectional study. Methods We reviewed the medical records of 61 patients (92 eyes) with buried ODD, 45 patients (62 eyes) with ODE, and 42 normal-appearing fellow eyes examined at 1 referral center between November 1, 2017 and April 30, 2019. Characteristic en face OCT and OCTA findings of buried ODD compared to those of ODE and normal optic discs were investigated. Results On en face OCT, all buried ODD were visualized as hyperreflective kidney-shaped masses well demarcated from the optic nerve axons, whereas ODE was visualized as ill-defined boundaries confluent with the retinal nerve fibers. On OCTA, 25.0% of the eyes with buried ODD showed a C-shaped vessel density decrease in the nasal radial peripapillary capillary layer, while 40.3% of the eyes with ODE had nonspecific focal vessel density decrease around the optic nerve head. Larger ODD were significantly associated with a vessel density decrease on OCTA (P = .009). The disc diameter positively correlated with the ODD area (r = 0.245; P = .018) and negatively correlated with the ODD height (r = −0.237; P = .023). Conclusions En face OCT showed the characteristic features of buried ODD compared to those of ODE or normal optic discs. The demarcation of buried ODD from the optic nerve axons on en face OCT and the poor vascular perfusion of buried ODD on OCTA suggest that buried ODD are materials deposited around the optic disc rather than the herniated optic nerve axon fibers.

Purpose This study evaluated the ability of topographically correspondent (TC) minimum rim width (MRW) and peripapillary retinal nerve fiber layer thickness (pRNFLT) criteria to detect optical coherence tomography (OCT) structural abnormality in glaucoma (GL) and glaucoma suspect (GLS) eyes. Design Retrospective cross-sectional study. Methods A total of 196 GL eyes, 150 GLS eyes, and 303 heathy eyes underwent pRNFL and 24 radial optic nerve head OCT imaging and manual correction of the internal limiting membrane, Bruch's membrane opening (BMO), and outer pRNFL segmentations. MRW and pRNFLT were quantified in 6 Garway-Heath or 12 30-degree (clock-hour) sectors. OCT abnormality for each parameter was defined to be less than the 5th percentile of the healthy eye distribution. OCT abnormality for individual eyes was defined using global, sectoral, and combined parameter criteria that achieved ≥95% specificity in the healthy eyes. TC combination criteria required the sectoral location of MRW and pRNFLT abnormality to be topographically aligned and included comMR (a previously reported TC combination consisting of MRW and pRNFLT parameter: [MRW + pRNFLT × (average MRW healthy eyes/average pRNFLT healthy eyes) MRW]. Results TC sectoral criteria (1 Garway-Heath MRW + corresponding Garway-Heath RNFLT), (one 30-degree MRW + any 1 corresponding or adjacent 30-degree pRNFLT), 30-degree and Garway-Heath comMR-TI and global comMR were the best performing criteria, demonstrating (96%-99% specificity), 86%-91% sensitivity for GL, 80%-84% sensitivity for early GL (MD ≥ −4.0 dB) and 93%-96% sensitivity for moderate-to-advanced GL (MD Conclusions Clinically intuitive TC MRW and pRNFLT combination criteria identified the sectoral location of OCT abnormality in GL eyes with high diagnostic precision.

Purpose Periventricular leukomalacia (PVL) is a structural loss of white matter pathways that carry visual information from the lateral geniculate bodies to the visual cortex. It is observed radiologically in patients with a history of prematurity and is associated with visual field (VF) defects and optic disc cupping. Advances in perinatal care have improved survival for premature babies, so many now present as adolescents and adults to comprehensive eye doctors who are unaware of the relationship of cupping, field defects, and prematurity and who may diagnose manifest or suspected normal tension glaucoma. We describe 2 such patients to raise awareness of this entity. Design Case series. Methods Review of clinical information of 2 patients identified during clinical practice. Charts were reviewed for gestational age, optic nerve appearance, intraocular pressure (IOP), and sequelae of prematurity. Magnetic resonance imaging (MRI), optical coherence tomography (OCT), VF, and optic disc photographs were reviewed. Results Two young patients with a history of prematurity presented with enlarged cup-to-disc ratio and normal IOP. OCT thinning was most prominent superiorly, with VF defects more notable inferior and homonymous. No progression on VF or OCT was noted in the index case over almost 4 years. Conclusions Periventricular leukomalacia should be added to the differential diagnosis of normal tension glaucoma (NTG) when there is a history of prematurity. Careful examination of the optic nerve will assist in differentiating from NTG. Specifically, horizontal cupping with minimal or no nasal displacement of vessels, and superior optic nerve thinning with inferior VF defects, suggest PVL.

PURPOSE: We studied effects of age and horizontal duction on deformation of the optic nerve (ON) head and peripapillary retina as reflected by displacement of vascular landmarks to explore the influence of adduction tethering. DESIGN: Cross-sectional study. METHODS: Setting: University. Study Population: Single eyes of 20 healthy young adults (average age 23.9±3.9 SD) years were compared to 20 older subjects (average age 61.4±9.3) years. Observational Procedure: The disc and peripapillary retina were imaged by scanning laser ophthalmoscopy in central gaze, and 35° abd- and adduction. Main Outcome Measure: Deformations of the disc and adjacent PPR were measured by comparing positions of epipapillary and epiretinal blood vessels. RESULTS: Vessels within the ONH of younger subjects shifted temporally during adduction and nasally during abduction. Displacement of the nasal hemi-disc in adduction was greater at 38.5±1.7μm (SEM) than the temporal half at 4.1±2.1μm (P

To evaluate the effect of optic nerve sheath fenestration (ONSF) on the recovery of visual function in pediatric patients with optic disc swelling owing to increased intracranial pressure.Retrospective case series.Medical chart review of all pediatric patients who underwent ONSF between 2009 and 2020 at the Children's Hospital of Philadelphia. Visual function was assessed at pre and postoperative visits. The main outcome measures were visual acuity, color vision, extraocular motility, visual field mean deviation, retinal nerve fiber layer thickness measured by optical coherence tomography.Fourteen pediatric patients (10 females; mean ± SD age of 14 ± 2.6 years; range, 8.5-17.5 years) were included. Five patients underwent bilateral surgeries. Ten patients were diagnosed with idiopathic intracranial hypertension. Of the 10 idiopathic intracranial hypertension patients, 3 had a previous history of weight gain and 2 of systemic steroid treatment; these can be referred to as pseudotumor cerebri. The mean ± SD follow-up length was 16.4 ± 12.3 months. VA improved from 20/138 to 20/68 in the operated eye (P = .0003) and from 20/78 to 20/32 in the nonoperated eye (P = .02). Color vision improved in the operated eye (P = .04), extraocular motility improved in the operated and nonoperated eye (P = .002 and P = .04 respectively). Visual field mean deviation improved in the operated (-23.4 dB to -11.5 dB, P.0001) and nonoperated eye (-19.8 dB to -6.8 dB, P = .02). Retinal nerve fiber layer thickness improved in the operated eye (349.1 to 66.2 µm; P.0001). The postoperative improvement was observed as early as the postoperative day 1.ONSF produces a rapid and persistent vision improvement in both the operated eye and the nonoperated eye. In children and young adults with papilledema and elevated intracranial pressure causing vision loss that is severe at presentation or refractory to standard medical management, ONSF should be considered.

Purpose To compare intravitreal therapy with the natural course of radiation optic neuropathy after primary proton beam therapy for choroidal melanoma with respect to long-term visual acuity and development of optic atrophy. Design Retrospective comparative case series. Methods Inclusion criteria: patients treated with primary proton beam therapy for choroidal melanoma with a minimum follow-up of 24 months after the occurrence of radiation optic neuropathy and optic disc imaging during follow-up. Exclusion criteria: pathologic condition of the optic disc before irradiation and intravitreal therapy to treat cystoid macular edema not originating from the optic disc. Results Of 93 patients, 48 were observed only after radiation optic neuropathy, and 45 were treated with intravitreal therapy (triamcinolone, bevacizumab, and/or dexamethasone). Median follow-up was 55 months (29-187 months); median interval between onset of radiation optic neuropathy and the last patient visit was 34 months (24-125 months). Of 48 observed patients, 41 (85.4%) developed an optic atrophy after a median of 14 months (3-86 months) after radiation optic neuropathy; and of 45 intravitreally treated patients, 34 (75.5%) presented with an optic atrophy after a median of 12.5 months (1-55 months) following optic neuropathy, indicating no statistically significant differences between the groups. Comparing the change in visual acuity from occurrence of optic neuropathy to final visual acuity, no statistically significant differences were found between either group (P = 0.579). Conclusions Patients treated with intravitreal therapy for radiation optic neuropathy showed no statistically significant differences related to visual acuity or optic atrophy development from patients who underwent only observation.

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 mm3. 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.

PURPOSE:To evaluate the association between optical coherence tomography angiography (OCTA) macular and circumpapillary vessel density and visual field mean deviation (MD) in advanced primary open angle glaucoma. DESIGN:Cross-sectional study. METHODS:Macula (superficial layer) and optic nerve head (ONH) with capillary density (CD) and without vessel density (VD) automated removal of large vessels OCTA of 34 eyes (34 patients, MD < -10 dB) were investigated as macula whole image VD (wiVD), parafoveal VD (pfVD), ONH wiVD, wiCD, circumpapillary VD, and cpCD. Spectral domain OCT circumpapillary retinal nerve fiber layer, macular ganglion cell complex, and ganglion cell inner plexiform layer were also analyzed. RESULTS:Macular and ONH VD decreased significantly with worsening MD. Each 1-dB decrease in MD was associated with a reduction of 0.43% and 0.46% for macular wiVD and pfVD with R2 of 0.28 and 0.27, respectively (all P < .01). The association between MD and VD was strongest for measures of ONH with large vessels removed, wiCD, and cpCD, followed by wiVD and circumpapillary VD with R2 of 0.26, 0.22, 0.17, 0.14, and a VD reduction of 0.43%, 0.51%, 0.33%, and 0.40%, respectively (all P < .02). There was a reduction of 1.19μm in Avanti parafoveal ganglion cell complex, 1.13μm in Spectralis ganglion cell inner plexiform layer, and 1.01μm in Spectralis circumpapillary retinal nerve fiber layer, with R2 of 0.19 (P= .006), 0.23 (P= .002), and 0.24 (P= .002), respectively. CONCLUSIONS:ONH and macula OCTA VD and thickness are associated with the severity of visual field damage in advanced primary open angle glaucoma.

To determine the rate of retinal nerve fiber layer (RNFL) thinning in patients with sickle cell hemoglobinopathies.This was a prospective cohort study.Sixty-seven patients averaging 35.8 ± 11.5 years of age at enrollment with electrophoretically confirmed sickle cell hemoglobinopathies followed by the University of Illinois at Chicago retina clinic for ≥1 year were included. Exclusion criteria included a history of diabetes, uncontrolled hypertension, glaucoma, ocular opacities, other retinopathies, and previous retinal procedures. The optic nerve head RNFL thicknesses were measured with spectral-domain optical coherence tomography (Heidelberg Engineering, Inc) at enrollment and subsequent follow-ups. Linear mixed models were used to estimate rates of thinning.A total of 122 eyes were followed for 3.8 ± 2.0 years (range 1-8 years). Mean global peripapillary RNFL thickness was 100.9 ± 13.0 μm at baseline. Global peripapillary RNFL thickness decreased at a rate of 0.98 μm per year (95% confidence interval [CI] 0.77-1.19 μm/year). A history of stroke was associated with a faster rate of global RNFL thinning (1.72 ± 0.20 vs 0.79 ± 0.12 μm/year, P.001), whereas a history of hypertension was associated with a slower rate of thinning (0.33 ± 0.27 vs 1.14 ± 0.12 μm/year, P = .002).Peripapillary RNFL thinning in patients with sickle cell hemoglobinopathies occurred faster in patients with a history of stroke and slower in patients with controlled hypertension. Future studies will compare these rates to those of healthy age- and race-matched individuals.

PURPOSE: To assess glaucoma specialists’ detection of optic nerve head (ONH) rim tissue that is thin by optical coherence tomography (OCT) criteria. DESIGN: Reliability analysis METHODS: 5 clinicians marked the disc margin (DM) and rim margin (RM) on stereo-photos of 151 glaucoma or glaucoma suspect eyes obtained within 3 months of OCT imaging. The photo and OCT infrared image for each eye were colocalized and regionalized into twelve sectors relative to the axis between Bruch’s membrane opening (BMO) centroid and the fovea. For each clinician, the distance from BMO centroid to their DM (DM radius) and RM (RM radius) was used to generate sectoral rim width (RW) (DM radius – RM radius) and cup-to-disc ratio (CDR) (RM radius / DM radius) estimates. OCT minimum rim width (MRW) was determined by sector. Among all eyes, for each OCT MRW suspicious sector (< 5% of OCT normative data base), we determined each clinician’s detection (clinician CDR ≥ 0.7). RESULTS: Clinicians most commonly failed to detect OCT suspicious rim tissue in the nasal sectors. Among 502 sectors with suspicious OCT MRW all 5 clinicians rated CDR ≥ 0.7 in only 29.5% and all 5 clinicians rated CDR < 0.7 in 21%. OCT suspicious rim thickness was most common (32% of eyes) in the nasal and inferior sectors. MRW vs clinician RW discordance was greatest nasally, while BMO vs clinician DM discordance was greatest temporally. CONCLUSIONS: Clinicians most commonly failed to detect OCT suspicious rim thickness nasally where suspicious rim tissues were also most common.

Purpose To evaluate the association of optic disc deformation with open-angle glaucoma (OAG) progression risk. Design Meta-analysis and meta-regression analysis. Methods Reports on the association of optic disc tilt and/or torsion with OAG progression published to June 2020 were identified in the PubMed, EMBASE, and Cochrane databases. Tilt ratio was measured as the longest-to-shortest diameter ratio of the optic disc. The angle (in degrees) between the vertical meridian and the disc's long axis was defined as the torsional angle. We used random-effects models to combine the pooled effects. Random-effects meta-regression was used to quantify the study characteristics’ potential moderating influences. The protocol was registered in the PROSPERO International Database (CRD42020201151). Results Eleven longitudinal studies (1100 participants) were included. For 10 of them evaluated for tilt ratio and glaucoma progression, the pooled hazard ratio (HR) was 0.988 (95% confidence interval [CI] 0.921-1.059; I2 = 59%) per 0.1-unit increase. In the meta-regression–based subgroup analysis, risk tended to decrease as the patients’ mean age increased (P = .08). For 7 of the studies (605 patients) reporting data on torsion and glaucoma progression, the pooled HR was 0.936 (95% CI 0.860-1.018; I2 = 32%) per 10-degree increase. The meta-regression analyses showed that the glaucoma progression assessment methods had a modulating influence (P = .01). In studies assessing just functional glaucomatous progression, the pooled HR was 0.890 (95% CI 0.831-0.952; I2 = 0%). Conclusion Evidence for the effects of optic nerve deformation on glaucoma progression is still lacking. Future research using stratified analysis according to age and tailored diagnostic criteria will allow for more rigorous analyses for this topic.

To identify prognostic factors for axial length (AL) elongation and incidence of posterior staphyloma (PS) in adult Japanese patients with high myopia.Retrospective, observational cohort study.Six-year follow-up data for 345 patients (620 eyes with AL ≥ 26.5 mm and spherical equivalent [SE] ≤- 8.00 diopters) admitted to the Tokyo Medical and Dental University Hospital from 2007 to 2017 were analyzed retrospectively. Main outcome measures were change in AL from baseline, factors associated with AL, categorization of eyes with high myopia, factors associated with incidence of PS, and impact of PS on myopic maculopathy and visual function.The mean annual increase in AL was 0.03 mm. Presence of optic nerve disc conus (P = .025), steeper corneal curvature, lower SE, and decreased choroidal thickness (CT) (all P.001) were associated with increased AL in univariate and multivariate analyses. Younger age (P = .003) and no use of intraocular pressure-lowering medications (P = .046) were associated with increased AL. Eyes with high myopia were categorized using factor analysis as associated with glaucoma, severe pathologic myopia, and mild-to-moderate pathologic myopia. Older age, increased AL, glaucoma, and choroidal thinning (all P ≤ .001) were identified as significant risk factors for the incidence of PS in univariate and/or multivariate analyses. Incidence of PS was a precursor for myopic maculopathy and visual field defects.Optic nerve disc conus, steeper corneal curvature, lower SE, decreased CT, and no use of intraocular pressure-lowering medications were prognostic factors for increased AL. Older age, increased AL, glaucoma, and decreased CT were prognostic factors for PS.

Purpose To study the effects of age, sex, intraocular pressure (IOP), corneal thickness, axial length (AXL), disc area, and the signal strength of the scan on optical coherence tomography (OCT) parameters of normal subjects in the L V Prasad Eye Institute-Glaucoma Epidemiological and Molecular Genetic Study (LVPEI-GLEAMS), a population-based study. Design Cross-sectional study. Methods A total of 1,100 eyes (1,100 subjects) of normal adults aged between 40 and 80 years from LVPEI-GLEAMS underwent macular and optic nerve head imaging with spectral-domain OCT (SDOCT). Effect of age, sex, IOP, central corneal thickness (CCT) and AXL, disc area, and signal strength of the OCT scan on retinal nerve fiber layer (RNFL) thickness, rim area, and ganglion cell–inner plexiform layer (GC-IPL) thickness measurements were evaluated using univariate and multivariate regression models. Results Mean rim area, RNFL, and GC-IPL thickness were 1.31 mm2 (standard deviation [SD] = 0.22), 93.7 μm (SD = 9.3) and 79.6 μm (SD = 8.7), respectively. Age had a negative association with RNFL thickness (coefficient: −0.18, P Conclusion Age, sex, AXL, disc area, and signal strength of the scan were significantly associated with OCT measurements. These factors may need to be considered while interpreting the OCT parameters in pathologic conditions such as glaucoma.

To determine whether parapapillary choroidal microvasculature (PPCMv) density as measured by optical coherence tomography angiography differs between pseudoexfoliation syndrome (PXS) and pseudoexfoliation glaucoma (PXG).Cross-sectional study.One hundred ninety-two eyes of 120 subjects from 2 academic referral institutions were enrolled. Automated PPCMv density was calculated using custom Matlab software in inner and outer annuli around the optic nerve region in addition to peripapillary superficial vasculature. Linear modeling was used to compare vessel densities among groups.Data from 64 eyes with PXS, 84 eyes with PXG, and 44 eyes healthy control subjects were analyzed. The differences of visual field mean deviation and peripapillary retinal nerve fiber layer thickness among study groups were statistically significant with lower values in PXG eyes compared with the PXS and control groups. Peripapillary superficial retinal vessel densities were significantly reduced in patients with PXG compared with patients with PXS and normal control subjects (all P.001) without a difference between PXS and control eyes. Customized outer annular PPCMv density in the PXG group with a value of 11.1% (SD 5.1%) was lower than that in PXS with a value of 13.2% (SD 5.3%; P = .001). Similarly, PXS values were lower than those of control eyes with a value of 18.6% (SD 5.1%; P.001).A progressive decrease in outer PPCMv from the control group to those with PXS without glaucoma to those with PXS and glaucoma (PXG) showed deep peripapillary vasculopathy in pseudoexfoliation syndrome. Choroidal vessel density may be affected early in the course of pseudoexfoliation before glaucoma develops.

To determine baseline clinical features associated with conversion to glaucoma in elderly patients with large optic-disc cupping.Retrospective cohort study.Seventy-two eyes of 72 untreated elderly (≥65-year-old) patients with large vertical cup-to-disc ratio (CDR ≥0.7) and without any other glaucomatous findings were included. They had undergone a full ophthalmologic examination twice per year for at least 5 years. The optic nerve head (ONH), peripapillary retinal nerve fiber layer (RNFL), and macular ganglion cell-inner plexiform layer (GCIPL) were imaged with Cirrus high-definition optical coherence tomography (OCT). Presence of temporal raphe sign on the OCT's GCIPL thickness map was assessed as one of the morphologic factors. Conversion to normal-tension glaucoma (NTG) was defined as structural or functional deterioration on either red-free RNFL photography or standard automated perimetry, respectively. The utility of the baseline factors associated with conversion to NTG were identified.During the 5.5-year follow-up, 19 eyes (26.4%) converted to NTG. There were no significant differences in demographics, systemic factors, intraocular pressure factors, or OCT parameters between the nonconverters and converters. Interestingly, the temporal raphe sign was observed in the converters (18/19, 94.7%) much more frequently than in the nonconverters (3/53, 5.7%, P.001) at baseline. A Cox proportional hazards model indicated the significant influences of temporal raphe sign positivity (hazard ratio 6.823, 95% confidence interval 2.574, 18.088, P.001) on conversion to NTG.In elderly subjects with large CDR, temporal raphe sign positivity on the baseline macular GCIPL thickness map was associated with faster conversion to NTG.

Purpose This study reports on neurofibromatosis type 1 (NF1)-associated optic pathway gliomas (OPGs) and a follow-up period of at least 10 years in a cohort of children. OPGs are a common manifestation of NF1 and can cause significant visual morbidity. Long-term follow-up in children with NF1-associated OPGs has not been reported previously. Design Retrospective observational case series. Methods This study included children with a documented follow-up of at least 10 years. Three final outcomes were evaluated: visual acuity (VA) per eye (i.e., in the more severely affected eye), VA per patient (i.e., VA when both eyes were open), and the presence of optic nerve head pallor. Results A total of 45 children were included, followed for a mean of 14 years (range, 10-21 years). At the end of follow-up, abnormal VA (considered moderate to severe impairment) in the more severely affected eye was present in 36% of the patients and in both eyes in 11%. Optic nerve head pallor of 1 or both nerves was present in 62%. In multivariate analysis, only initial VA and optic nerve head appearance at presentation were found to predict the final outcomes. All patients, except for 1, were asymptomatic at presentation and had normal VA and nerves that appeared normal, preserved their good vision in both eyes. Only 1 patient, who had normal VA and normal appearing nerves at presentation, had moderate to severe VA loss at long term follow-up. Conclusions In this study, children with NF1-associated OPG whose examination signs and symptoms were normal had a normal initial examination and excellent long-term visual and anatomical outcomes. VA and the appearance of the optic nerve head at presentation predict long-term outcome.

To evaluate ultrasound biomicroscopy (UBM) characteristics of trabeculodysgenesis and explore its correlation with the outcomes of microcatheter-assisted trabeculotomy (MAT) in eyes with primary congenital glaucoma (PCG).A prospective, interventional case series.Patients with newly diagnosed PCG were consecutively recruited, and subsequently MAT was tried as their first glaucoma surgery. All participants underwent UBM prior to surgery. The trabeculodysgenesis was classified into 3 types according to the severity of the anterior insertion of iris and ciliary processes (type 1, severe trabeculodysgenesis; type 2, moderate trabeculodysgenesis; type 3, mild trabeculodysgenesis). Surgical success was defined as a postoperative intraocular pressure of ≤ 21 mm Hg with at least a 30% reduction from preoperative intraocular pressure without additional medical or surgical therapy, and with decreased corneal edema, stabilized corneal diameter, and no additional optic nerve damage for at least 6 months after surgery.MAT was tried on 49 eyes with PCG (33 patients) as the first glaucoma surgery. The ratios of the type of trabeculodysgenesis (type 1: type 2: type 3) were 1:1:1.7 among eyes. In multivariate analysis, age (at the time of operation) (P.001) and sex (P = .002) were factors associated with the type of trabeculodysgenesis. At 24-month follow-up, type 1 achieved a 57.1% surgical success rate, type 2 achieved 70.5%, and type 3 achieved 95.5% (P = .022). Severe trabeculodysgenesis (P = .014), as well as poorer corneal transparency (P = .037), was associated with worse surgical outcome.UBM grading of trabeculodysgenesis in PCG is helpful for MAT prognosis. Combined with preoperative corneal opacity score, it may be used to predict the outcome of MAT surgery.

Purpose To use spectral-domain optical coherence tomography (SD-OCT) to assess whether epipapillary vitreous traction from evolving posterior vitreous detachment (PVD) is mechanistically involved in the pathogenesis of nonarteritic anterior ischemic optic neuropathy (NAION). Design Retrospective cohort study. Methods Setting : Single academic center. Patient or Study Population : Eighty eyes in 74 subjects presenting within 2 weeks of symptom onset of NAION. Intervention or Observation Procedures : SD-OCT imaging of the optic nerve head, macula, and peripapillary retinal nerve fiber layer (pRNFL) were reviewed for PVD and vitreopapillary traction (VPT). Main Outcome Measures : Prevalence and incidence of PVD and VPT, logMAR best-corrected visual acuity (BCVA), mean deviation (MD) on automated perimetry, and pRNFL thickness at presentation and final follow-up. Results The co-prevalence of PVD at the time of acute presentation for NAION was 30% (n = 24/80). This was similar to the prevalence of PVD in contralateral uninvolved eyes (34.5%, n = 19/55). In 8 NAION eyes, PVD had been documented prior to the onset of NAION. No eyes had SD-OCT evidence of VPT on presentation or final follow-up. Only 5 eyes developed a PVD following the development of NAION, and this typically occurred months after resolution of optic disc edema. When comparing eyes with PVD and without PVD, there was no significant difference in BCVA, MD, and pRNFL thickness at baseline, final follow-up, or the change from baseline to final follow-up (all P > .10). Conclusions VPT was not mechanistically related to the development of classic NAION. Preexisting PVD did not preclude the future development of NAION. The co-prevalence of PVD with acute NAION had no impact on final visual outcome.

To evaluate the rate of peripapillary choroidal thinning in glaucoma patients and healthy controls using spectral domain optical coherence tomography.Cohort study.Participants from the multicenter African Descent and Glaucoma Evaluation Study and Diagnostic Innovations in Glaucoma Study were included. The San Diego Automated Segmentation Algorithm was used to automatically segment and measure peripapillary choroidal thickness (PCT) from circle scans centered on the optic nerve head. The rate of PCT thinning was calculated using mixed effects models.Two hundred ninety-seven eyes with a median follow-up of 2.6 years were included. At baseline, the global mean PCT was significantly thinner in glaucoma patients than healthy control subjects (141.7 ± 66.3 μm vs 155.7 ± 64.8 μm, respectively; P.001). However, when age was included in the model, this difference was no longer significant (P = .38). Both healthy controls and glaucoma patients had a significant decrease in mean (95% confidence interval) PCT change over time (-2.18 [-2.97 to -1.40 μm/year] and -1.88 [-3.08 to -0.67 μm/year], respectively) and mean PCT percent change over time (-3.32% [-4.36 to -2.27 μm/year] and -2.85% [-4.64 to -0.99 μm/year], respectively). No significant difference was found between healthy control subjects and glaucoma patients in the mean rate of PCT change (P = .28) or PCT percentage change over time (P = .23).The rate of peripapillary choroidal thinning was not significantly different between healthy and glaucoma eyes during this relatively short follow-up period. Longer follow-up is needed to determine whether monitoring the rate of PCT change has a role in glaucoma management.

Purpose To measure the area of the optic nerve subarachnoid space (ONSASA) in patients with normal tension glaucoma (NTG), primary open-angle glaucoma (POAG), and controls and examine its association with relevant ocular and systemic parameters. Design Cross-sectional study. Methods The study included 40 patients with NTG, 42 with POAG, and 45 healthy controls. B-scan ultrasound was performed binocularly, using a 12.5-MHz linear array probe. The measurement of the optic nerve subarachnoid space (ONSAS) and calculation of the ONSASA using ImageJ 1.51e analysis software was done by 2 experienced observers in a masked manner. Results The ONSASA between 3 and 7 mm behind the globe in NTG (5.15 ± 0.81 mm 2 ) was significantly smaller than that in the POAG (6.24 ± 1.62 mm 2 , P = .0008) or control (6.40 ± 2.20 mm 2 ; P = .0007) groups. ONSASA in the POAG and control groups were not significantly different ( P = .13). ONSASA was significantly associated with mean IOP ( P = .0004) and highest IOP ( P = .0007). The optic nerve sheath diameter in NTG compared to POAG was significantly different at 3 mm (4.46 ± 0.43 mm vs 4.79 ± 0.40 mm, P = .0007), 5 mm (4.40 ± 0.39 mm vs 4.65 ± 0.47 mm, P = .003), and 7 mm (4.36 ± 0.35 mm vs 4.61 ± 0.30 mm, P = .004) behind the globe. Conclusions The ONSASA is smaller in NTG as compared to normal control. This is compatible with a lower cerebrospinal fluid pressure in the optic nerve in NTG, implying that trans–lamina cribrosa pressure difference might be abnormally higher in the NTG group than in normal controls.