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Background: The continued increase in idiopathic intracranial hypertension (IIH) prevalence has many implications for societal health care. Its potential vision-threatening consequences make ophthalmologists key players in its diagnosis and management. Newer technology such as optical coherence tomography angiography (OCT-A) enables evaluation of the branching complexity of the peripapillary capillary plexus, a region where accurate imaging via fluorescein angiography was previously limited., Methods: A cross-sectional, observational study of 23 (46 eyes) consecutive patients with IIH. Peripapillary total vasculature was recorded using commercial OCT-A en face vessel density mapping. In addition, OCT-A blood flow slab was compared with papilledema grading. OCT-A images were analyzed using a customized image analysis protocol using ImageJ software (v1.51w) and Photoshop software (Adobe Systems, CA). SPSS software version 25 was used for statistical analysis (SPSS Inc, IBM, Chicago, IL)., Results: Skeletonized vessel density peripapillary capillary plexus was significantly associated with Frisen papilledema grades, OCT retinal nerve fiber layer (RNFL), and macular ganglion cell layer (GCL) thickness with a P < 0.001, P = 0.022, and P = 0.006, respectively. Every point increase in grade was correlated with a decrease of 9.1 pixels/mm2 in vessel density (R = 0.512, β = -0.115 ± 0.029; P < 0.001). Increased papilledema was correlated with an increased retinal blood flow percentage (R = 0.300, β = 2.114 ± 1.013; P < 0.05) and decreased choroidal blood flow (CBF) percentage (R = 0.300, β = 2.114 ± 1.013; P < 0.05). Every point increase in grade was correlated with a decrease in CBF by 47.4%, as calculated using a linear best-fit line inclusive for all of the data points., Conclusions: OCT-A allows for effective visualization and quantification of the peripapillary retinal vasculature. Our results demonstrate a correlation between skeletonized peripapillary density and papilledema grading, OCT RNFL thickness, and GCL thickness. In addition, we show a significant negative correlation between CBF and papilledema grading. These changes provide key findings regarding the pathophysiology of optic neuropathy in papilledema and highlight the potential of OCT-A as a diagnostic tool for papilledema and a clinical marker for detecting early optic nerve damage., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by North American Neuro-Ophthalmology Society.)

Purpose: The aim of this study was to compare the ultrasonographic optic nerve sheath diameter (ONSD) in different grades of papilledema and in controls and to evaluate ONSD in atrophic papilledema/optic atrophy when raised ICP was suspected., Methods: Prospective cross-sectional case-control study. Following an ocular examination, papilledema was graded clinically using modified Frisén's grading. An ultrasonographic cross section of the retrobulbar optic nerve was obtained with a posterior transverse scan. Independent t-test and analysis of variance were the statistical tools used in the study., Results: The study included 55 cases and 55 age- and gender-matched controls; mean (± standard deviation) age was 37.17 (±11.25) years and male: female ratio was 49:61. There was a statistically significant difference in the mean ultrasonographic ONSD between cases [4.89 (±0.65) mm] and controls [3.12 (±0.22) mm] (P < 0.001). There was a significant difference in the mean ONSD across Frisén's grades of papilledema (P < 0.001). The mean ONSD in atrophic papilledema was 6.2 (±0.75) mm., Conclusion: In the presence of symptoms, ultrasonographic ONSD >4 mm is diagnostic of papilledema. Ultrasonographic ONSD correlates well with the severity of papilledema and can be used to follow-up patients with chronically elevated ICP. It is useful in detecting raised ICP in the presence of optic atrophy and to distinguish true papilledema from pseudopapilledema., Competing Interests: There are no conflicts of interest

Purpose: To identify the most accurate diagnostic imaging modality for classifying pediatric eyes as papilledema (PE) or pseudopapilledema (PPE)., Design: Prospective observational study., Subjects: Nineteen children between the ages of 5 and 18 years were recruited. Five children (10 eyes) with PE, 11 children (19 eyes) with PPE owing to suspected buried optic disc drusen (ODD), and 3 children (6 eyes) with PPE owing to superficial ODD were included., Methods: All subjects underwent imaging with B-scan ultrasonography, fundus photography, autofluorescence, fluorescein angiography (FA), optical coherence tomography (OCT) of the retinal nerve fiber layer (RNFL), and volumetric OCT scans through the optic nerve head with standard spectral-domain (SD OCT) and enhanced depth imaging (EDI OCT) settings. Images were read by 3 masked neuro-ophthalmologists, and the final image interpretation was based on 2 of 3 reads. Image interpretations were compared with clinical diagnosis to calculate accuracy and misinterpretation rates of each imaging modality., Main Outcome Measures: Accuracy of each imaging technique for classifying eyes as PE or PPE, and misinterpretation rates of each imaging modality for PE and PPE., Results: Fluorescein angiography had the highest accuracy (97%, 34 of 35 eyes, 95% confidence interval 92%-100%) for classifying an eye as PE or PPE. FA of eyes with PE showed leakage of the optic nerve, whereas eyes with suspected buried ODD demonstrated no hyperfluorescence, and eyes with superficial ODD showed nodular staining. Other modalities had substantial likelihood (30%-70%) of misinterpretation of PE as PPE., Conclusions: The best imaging technique for correctly classifying pediatric eyes as PPE or PE is FA. Other imaging modalities, if used in isolation, are more likely to lead to misinterpretation of PE as PPE, which could potentially result in failure to identify a life-threatening disorder causing elevated intracranial pressure and papilledema., (Copyright © 2017 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To describe the methods used by the Photographic Reading Center (PRC) of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) and to report baseline assessments of papilledema severity in participants., Methods: Stereoscopic digital images centered on the optic disc and the macula were collected using certified personnel and photographic equipment. Certification of the camera system included standardization and calibration using a model eye. Lay readers assessed disc photos of all eyes using the Frisén grade and performed quantitative measurements of papilledema. Frisén grades by PRC were compared with site investigator clinical grades. Spearman rank correlations were used to quantify associations among disc features and selected clinical variables., Results: Frisén grades according to the PRC and site investigator's grades, matched exactly in 48% of the study eyes and 42% of the fellow eyes and within one grade in 94% of the study eyes and 92% of the fellow eyes. Frisén grade was strongly correlated (r > 0.65, P < 0.0001) with quantitative measures of disc area. Cerebrospinal fluid pressure was weakly associated with Frisén grade and disc area determinations (r ≤ 0.31). Neither Frisén grade nor any fundus feature was associated with perimetric mean deviation., Conclusions: In a prospective clinical trial, lay readers agreed reasonably well with physicians in assessing Frisén grade. Standardization of camera systems enhanced consistency of photographic quality across study sites. Images were affected more by sensors with poor dynamic range than by poor resolution. Frisén grade is highly correlated with quantitative assessment of disc area. (ClinicalTrials.gov number, NCT01003639.).

Background and Purpose: Changes at the optic nerve head on DWI in the presence of papilledema have not been systematically studied. The purpose of this study was to evaluate if hyperintensity of ON heads on DWI is associated with papilledema., Materials and Methods: In this retrospective study, 19 patients (4 men, 15 women; median age, 32 years) with papilledema and 20 control participants (7 men, 13 women; median age, 48 years) who had undergone prior MR imaging of the brain were identified. Two neuroradiologists blinded to the diagnosis independently reviewed the DWI for the presence of hyperintense signal at the ON head of each eye. If present, they graded the signal as mild or prominent. Groups with and without papilledema were compared for the prevalence of ON head hyperintensity by using the Fisher exact test, with analyses performed both for groups of patients and for individual eyes. Presence of ON head hyperintensity was also studied as a function of Frisen papilledema grade on fundoscopy, when available., Results: Hyperintensity of the ON heads on DWI was significantly associated with papilledema (P = .001). For the 2 readers, hyperintensity at both ON heads was 26.3% and 42.1% sensitive and 100% specific in the detection of papilledema. The presence of unilateral ON head hyperintensity was not specific for papilledema and was invariably graded as mild when seen in the control group. Patients with higher papilledema grades had a higher prevalence of hyperintensity at the ON heads., Conclusions: Hyperintensity of the ON heads on DWI can serve as a useful imaging marker for papilledema, especially if bilateral. Its absence, however, does not exclude papilledema.

The appearance of the optic disc is a key measure of disease status in idiopathic intracranial hypertension (IIH). The Frisén classification describes stages of optic disc swelling (grades 0-5). It is the only classification of papilloedema, and is used internationally in clinical and research practice. Despite this, there has been very limited evaluation of the scale. We assessed the inter-rater reproducibility and ability to discriminate optic disc changes over time using the Frisén classification compared with a system of ranking papilloedema severity in patients with IIH. Paired disc photographs (before and after treatment) were obtained from 47 patients with IIH (25 acute and 22 chronic). Six neuro-ophthalmologists blinded to patient identity, clinical information and chronology of the photographs reviewed the discs and allocated a Frisén grade and ranked the paired discs in order of papilloedema severity (disc ranking). A total of 188 optic disc photographs were reviewed. All six reviewers agreed in only three comparisons (1.6%) when using the Frisén classification, compared with 42 comparisons (45.2%) when using disc ranking. The probability of agreement between any two reviewers was 36.1% for Frisén grade and 70.0% for disc ranking. Disc ranking had significantly greater sensitivity for finding differences in degree of disc oedema, identifying a difference in 75.3% of paired photographs compared to 53.2% detected using the Frisén classification (p < 0.001). This study demonstrated the limited reproducibility and discriminative ability of the Frisén classification in identifying changes in serial optic disc photographs in IIH. Simple optic disc ranking appears to be a more sensitive and reliable tool to monitor changes in optic disc appearance. The use of disc ranking in clinical practice and research studies is recommended to monitor alterations in optic disc appearance until alternative schemes, specific to IIH, have been developed.

Purpose: To develop an automated system that analyzes digital fundus images for staging and monitoring of optic disc edema (i.e., papilledema), due to raised intracranial pressure., Methods: A total of 294 retrospective, digital photographs of the right and left eyes of 39 subjects with papilledema acquired over the span of 2 years were used. Software tools were developed to analyze three features of papilledema from digital fundus photographs: (1) sharpness of the optic disc border, (2) discontinuity along major vessels overlying the optic nerve, and (3) texture properties of the peripapillary retinal nerve fiber layer (RNFL). A classifier used these features to assign a grade of papilledema according to a standard protocol used by an expert neuro-ophthalmologist (RK)., Results: The algorithm showed substantial agreement (κ = 0.71, P < 0.001) with the neuro-ophthalmologist when grading papilledema per patient. Vessel features showed statistical significance (P < 0.05) in differentiating grades 0, 1, and 2 from grades 3 and 4, whereas disc obscuration differentiated grades 0 or 1 from the rest (P < 0.05)., Conclusions: These results show that this algorithm can be used to automatically grade papilledema. The algorithm provides objective and quantitative assessment of the stage of papilledema with accuracy that is comparable to grading by a neuro-ophthalmologist. One application is in rapid assessment of digital optic nerve photographs acquired in clinical, intensive care, and emergency response settings by nonophthalmologists to evaluate for the presence and severity of papilledema, due to intracranial hypertension.

Bilateral optic disc swelling requires following a number of steps from discovery to causal diagnosis. First, it is necessary to differentiate between true optic disc swelling and disc elevation without true swelling. Then fundus examination, visual acuity and visual field, fluorescein angiography, and optical coherence tomography are performed in order to differentiate papilledema secondary to increased intracranial pressure from optic disc swelling secondary to optic neuropathy. Even if the most frequent etiology is idiopathic intracranial hypertension, the clinician must check for the absence of any signs or symptoms related to hypertension secondary to a cerebral tumor or to cerebral venous thrombosis. Fortunately, modern imaging techniques have facilitated the differential diagnoses of optic disc swelling, and the combination of magnetic resonance imaging (MRI) and magnetic resonance venography appears to be necessary each time the diagnosis of idiopathic hypertension is suggested., (Copyright (c) 2010 Elsevier Masson SAS. All rights reserved.)

Objectives: To compare and contrast 2 methods of quantitating papilledema, namely, optical coherence tomography (OCT) and Modified Frisén Scale (MFS)., Methods: Digital optic disc photographs and OCT fast retinal nerve fiber layer (RNFL) thickness, fast RNFL map, total retinal thickness, and fast disc images were obtained in 36 patients with papilledema. Digital optic disc photographs were randomized and graded by 4 masked expert reviewers using the MFS. We performed Spearman rank correlations of OCT RNFL thickness, OCT total retinal thickness, and MFS grade from photographs., Results: OCT RNFL thickness and MFS grade from photographs correlated well (R = 0.85). OCT total retinal thickness and MFS grade from photographs had a similar correlation of 0.87. Comparing OCT RNFL thickness with OCT total retinal thickness, a slope of 1.64 suggests a greater degree of papilledema thickness change when using the latter., Conclusions: For lower-grade abnormalities, OCT compares favorably with clinical staging of optic nerve photographs. With higher grades, OCT RNFL thickness processing algorithms often fail, with OCT total retinal thickness performing more favorably.

Objective: To investigate the significance of optic nerve head swelling (ONHS) in relation to the pattern of vascular nonperfusion, visual acuity (VA), and demographic profile in retinal venous occlusions (RVOs) occurring within the optic nerve., Methods: Cases of RVO occurring within the optic nerve were divided on the basis of the presence (105 cases) or absence (163 cases) of ONHS. This division was performed by examining the color stereo fundus photographs in conditions masked from other clinical parameters. Duration of symptoms before assessment, age, and sex distributions were compared. The vein involved was identified, and the occlusion was confirmed to have occurred within the optic nerve by observing that the vein pierced the lamina cribrosa as a dilated vein. Fluorescein angiographs were examined, and the extent of vascular nonperfusion in the macula and peripheral retina was quantified from grade 1 to grade 4. The extent of break in the perifoveal capillary arcade was graded as 0, less than or equal to 90 degrees, and greater than 90 degrees. Best-corrected VA was assessed using the Snellen chart., Results: The 2 groups were comparable in terms of the duration of the symptoms before examination. The mean age was significantly younger in the group with ONHS (58.3 vs 65.1 years, P<. 001). Age distribution by sex demonstrated a higher proportion of men younger than 50 years in the ONHS group (19.1% vs 8.6%, P =.01). The group without ONHS involved the papillary vein more frequently (31.3% vs 17.1%, P =.01). The respective proportions of grade 1, 2, 3, and 4 vascular nonperfusion in the macula were 90.5%, 9.5%, 0%, and 0% in the ONHS group, and 62.6%, 14.7%, 13.5%, and 9.2% in the group without ONHS (P<.001). The corresponding proportions for the peripheral retina were 90.4%, 8.7%, 0%, and 1.0% in the ONHS group, and 62.7%, 13.0%, 18.0%, and 6.2% in the group without ONHS (P<.001). In 64.6% of cases with ONHS and 42.9% of cases without, the perifoveal arcade was intact. A break greater than 90 degrees in the perifoveal arcade was present in 12.5% of cases with and 23.6% of cases without ONHS (P =.004). The median VA was significantly better in the ONHS group (6/24 vs 6/48, P =.005)., Conclusions: The RVOs occurring within the optic nerve can be subdivided into 2 distinct groups on the basis of ONHS. The presence of ONHS is associated with younger age, less severe vascular nonperfusion, and better VA. This is consistent with a retrocribrosal site of occlusion, which has access to the pial plexus that can provide collateral channels for retinal venous drainage.

Purpose: Visual loss is the main morbidity of idiopathic intracranial hypertension (IIH). The relationship between papilledema grade and visual loss is unclear. The goal of this study was to determine whether there is a relationship between papilledema grade and visual loss., Methods: Fundus photographs of 478 patients with IIH were reviewed, and their degree of papilledema was graded using Frisén's scheme. We identified 46 patients (10%) with IIH and highly asymmetric papilledema, as defined by an interocular difference of two or more grades. Nine of these patients with active asymmetry agreed to return for a series of visual tests. They underwent three visual field tests-Humphrey visual field analyzer 24-2, motion perimetry, and ring perimetry. The perimetry outcome measures were mean deviation, foveal threshold, and means for eccentric zones (3 degrees, 9 degrees, 15 degrees, and 21 degrees). The patients participated also in visual acuity, Farnsworth-Munsell 100-hue, Pelli-Robson contrast sensitivity, and foveal flicker fusion testing. Their relative afferent pupillary defect was graded using neutral density filters., Results: The intereye comparisons showed vision to be worse in the eye with the high-grade papilledema for all outcome measures. The magnitude of the loss with the perimetry tests increased with eccentricity. The measures of central visual function, although in the normal range, were relatively depressed in the eye with high-grade papilledema., Conclusions: Visual loss in patients with asymmetric papilledema caused by IIH was most pronounced in the eye with the higher grade of papilledema. Foveal visual functions, although they remained in the normal range, were also decreased in patients with high-grade papilledema. In patients with high-grade papilledema, visual loss appeared to affect the entire visual field, and the peripheral field showed the most deficit. Our findings showed that high-grade papilledema was associated with visual dysfunction in patients with IIH.

Progress over the past few years in knowledge concerning disorders of the optic nerve and disc leads to changes in their classification. A new terminology is proposed. It uses a minimum of terms, their very general sense being understandable by any nonophthalmologist physician. It is based upon clinical symptoms and signs, thus being immediately applicable in clinical practice.