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Purpose: Giant cell arteritis (GCA) involving ophthalmic circulation often manifests as anterior ischemic optic neuropathy (AAION), presenting with severe vision loss and pallid optic disc edema. Non-arteritic anterior ischemic optic neuropathy (NAION) classically presents with segmental optic disc edema and corresponding altitudinal visual field defect (VFD) with small cup-to-disc ratio in the fellow eye. Differentiating these two entities is critical as GCA requires immediate treatment to prevent vision loss in the fellow eye. This study investigated how often GCA mimics NAION at presentation., Methods: Retrospective chart review of patients with temporal artery biopsy (TAB) positive GCA with ocular manifestations seen at a tertiary neuro-ophthalmology practice between 2015 and 2020. Patients presenting with segmental non-pallid optic disc swelling and corresponding altitudinal VFD mimicking NAION were identified., Results: The clinical presentation of 7.1% (3/42) of patients with TAB-positive GCA mimicked NAION. Two of three patients had cup-to-disc ratio of <0.3 in the fellow eye. Two patients were women, mean age was 67.3 ± 6.5 years, and mean presenting visual acuity was 0.45 ± 0.48 LogMAR. Two patients had a normal temporal artery ultrasound. Two of three patients had at least one systemic symptom of GCA at presentation and all had elevation of one or both inflammatory markers., Conclusions: There should be high index of suspicion for GCA, even in patients highly suspected to have NAION. Inflammatory markers must be checked in every patient with presumed NAION and TAB performed if one or both are elevated to avoid missing GCA., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 Elsevier B.V. All rights reserved.)

To investigate differences in clinical features based on magnetic resonance imaging (MRI) in idiopathic isolated optic neuritis patients. We retrospectively analyzed 68 eyes of 59 patients diagnosed with optic neuritis and showed inflammatory findings indicative of optic neuritis on MRI. We investigated clinical features, such as the presence of accompanying pain, visual acuity, and optic disc swelling. Optic disc swelling was classified as normal, mild, or severe. The MRI results were divided into intraorbital, intracanalicular, and whole optic nerve according to the lesion, and these were compared and analyzed with clinical features. The study included 29 men and 30 women, with a mean age of 42.6 ± 16.6 years. Among 59 patients, 48 (81.4%) complained of pain. Optic disc swelling was not observed in 48.5% of patients (33 eyes). Inflammatory changes were the most common in the intraorbital region (33 eyes), intracanalicular region (20 eyes), and the entire optic nerve (15 eyes). There was no statistical difference in the pain pattern according to the location of the lesion (p = .677), but when inflammation was present in the entire optic nerve, optic disc swelling was severe (p = .023). The initial and final visual acuity did not significantly correlate with the MRI pattern, presence of pain, or optic disc swelling (p = .156, p = .714, and p = .436). The MRI contrast enhancement pattern was associated with optic disc swelling but was not associated with pain or initial visual acuity. It should be noted that it is insufficient to judge the clinical features of optic neuritis based on MRI findings., (© 2023. The Author(s).)

Purpose: To report a case of unilateral disc swelling as the only presentation of enterovirus meningitis in a 43-year-old male who presented with reduction in visual quality., Methods: Vision was 20/20 unaided in both eyes and the only ocular finding was left optic disc edema accompanied by left relative afferent pupillary defect. Visual field examination revealed a generalized reduction in sensitivity, a paracentral scotoma and a superior and inferior arcuate defect. Apart from a marginally elevated white blood cell count, the evaluation for common diseases associated with unilateral disc swelling was negative. A comprehensive neurology and rheumatology assessment,the orbital, brain and cervical spine Magnetic Resonance Imaging (MRI), the chest and abdomen Computed Tomography (CT) and chest radiograph had no findings. However, the cerebrosipinal fluid (CSF) examination indicated the presence of enterovirus RNA., Results: A diagnosis of enterovirus meningitis was established.Patient's general health was not affected and symptoms gradually settled, with no specific anti-enteroviral treatment., Conclusion: Only the CSF analysis provided interesting evidence of the site of the visual dysfunction.

Headache is a common complaint in children who present at the pediatric emergency department (PED). Serious conditions such as intracranial tumors and idiopathic intracranial hypertension (IIH) should be rapidly ruled out. Ophthalmoscopy for the presence of papilledema has long been considered critical to the assessment of headaches in children; however, the yield of this procedure is poorly validated. This retrospective study implemented a computerized search of the medical records of a single tertiary center to identify all children aged 2-18 years who presented at the PED complaining of headache between 2007 and 2017. The clinical, demographic, radiographic, and laboratory data were analyzed. Of the 948 children aged 2-18 years who presented at the PED complaining of headache, 536 had an ophthalmoscopy examination carried out by an ophthalmologist. Forty-one had papilledema, of whom 7 had an intracranial tumor, 15 had IIH, and 9 had optic nerve head drusen. Of the 495 children without papilledema, 3 had intracranial tumor, and 11 had IIH. The sensitivity and specificity of papilledema for the diagnosis of intracranial tumor were 70% and 93.5%, respectively, with an NPV and PPV of 99.4% and 17.1%, respectively. The sensitivity and specificity of papilledema for the diagnosis of intracranial pathology in general were 61.1% and 96.2%, respectively, with an NPV and PPV of 97.2% and 53.7%, respectively.  Conclusion: Assessment by ophthalmoscopy for papilledema in children presenting to the PED with headache had high sensitivity and high specificity, thus reinforcing the importance of ophthalmoscopy as a screening tool in these children. What is Known: • Headache is a common complaint in children. Serious intracranial pathologies need to be rapidly excluded. • Ophthalmoscopy for the presence of papilledema is commonly used as a screening tool for intracranial pathology, but this procedure is poorly validated. What is New: • Ophthalmoscopy for the assessment of papilledema in children who present with headache to the pediatric emergency department is shown to exhibit sensitivity and specificity for the diagnosis of intracranial pathology., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Peripapillary hyperreflective ovoid mass-like structures (PHOMS) are a laterally bulging herniation of distended axons into the peripapillary region above the level of Bruch's membrane opening. Increased use of enhanced depth imaging-optical coherence tomography (EDI-OCT) in our evaluation of the optic nerve head (ONH) and greater recognition of the vast range of optic nerve pathologies with which PHOMS is associated provides convincing evidence that PHOMS is not just buried optic disc drusen (ODD) as previously described. The frequent coexistence of PHOMS with ODD, papilloedema, anterior ischaemic optic neuropathy, tilted optic disc syndrome, inflammatory demyelinating disorders and other diseases associated with axoplasmic stasis provides insight into its underlying pathophysiology. The present review will discuss the role of key imaging modalities in the differential diagnosis of PHOMS, explore the current literature on the relationship between PHOMS and common neuro-ophthalmic conditions, and highlight the gaps in our knowledge, with respect to disease classification and prognosis, to pave the way for future directions of research., (© 2022 The Authors. Clinical & Experimental Ophthalmology published by John Wiley & Sons Australia, Ltd on behalf of Royal Australian and New Zealand College of Ophthalmologists.)

Background: Our objective was to assess optic nerve sheath diameter (a marker of elevated intracranial pressure) and optic disc elevation (a marker of papilledema) in pseudotumor cerebri syndrome using transorbital sonography., Methods: The study was a prospective case-control study. We included patients with new-onset pseudotumor cerebri syndrome and matched healthy controls. All had fundoscopy, lumbar puncture with opening pressure and transorbital sonography. Sonography was assessed by a blinded observer., Results: We evaluated 45 patients and included 23 cases. We recruited 35 controls. Optic nerve sheath diameter was larger in pseudotumor cerebri syndrome compared to controls (6.3 ± 0.9 mm versus 5.0 ± 0.5 mm, p < 0.001) and so was optic disc elevation (0.9 ± 0.4 mm versus 0.4 ± 0.1 mm, p < 0.001). The optimal cut-off point for optic nerve sheath diameter was 6 mm with a sensitivity of 74% for prediction of pseudotumor cerebri syndrome and 68% for prediction of elevated opening pressure. Specificity was 94%. The optimal cut-off point for optic disc elevation was 0.6 mm. Sensitivity was 100% and specificity 83% for prediction of pseudotumor cerebri syndrome., Conclusion: Optic disc elevation and optic nerve sheath diameter are increased in new-onset pseudotumor cerebri syndrome. Optic disc elevation achieved high specificity and excellent sensitivity for diagnosis of pseudotumor cerebri syndrome. Transorbital sonography (TOS) is a potential, non-invasive screening tool for pseudotumor cerebri syndrome in headache clinics.

Purpose: The optic nerve (ON) is an extension of the central nervous system via the optic canal to the orbital cavity. It is accompanied by meninges whose arachnoid layer is in continuity with that of the chiasmatic cistern. This arachnoid layer is extended along the ON, delimiting a subarachnoid space (SAS) around the ON. Not all forms of chronic intracranial hypertension (ICH) present papilledema. The latter is sometimes asymmetric, unilateral, or absent. The radiological signs of optic nerve sheath (ONS) dilation, in magnetic resonance imaging, are inconsistent or difficult to interpret. The objective of this study was to analyze the anatomy, the constitution, and the variability of the SAS around the ON in its intraorbital segment to improve the understanding of the pathophysiologic mechanism of asymmetric or unilateral or absent papilledema in certain ICH., Methods: The study was carried out on nine cadaveric specimens. In four embalmed specimens, macroscopic analysis of the SAS of the ONS were performed, with description about density of the arachnoid trabecular meshwork in three distinct areas (bulbar segment, mid-orbital segment and the precanal segment). In three other embalmed specimens, after staining of SAS by methylene blue (MB), we performed macroscopic analysis of MB progression in the SAS of the ONS. Then, in two non-embalmed specimens, light and electron microscopy (EM) analysis were also done., Results: On the macroscopic level, after staining of SAS, we found in all cases that MB progressed on 16 mm average throughout the SAS of the ONS without reaching the papilla. In four embalmed specimens, in the SAS of the ONS, the density of the arachnoid trabecular meshwork showed inter-individual variability (100%) and intra-individual variability with bilateral variability (50%) and/or variability within the same ONS (88%). On the microscopic level, the arachnoid trabeculae of the ONS are composed of dense connective tissue. The EM perfectly depicted its composition which is mainly of collagen fibers of parallel orientation., Conclusion: The variability of the SAS around the ONS probably impacts the symmetrical or asymmetrical nature of papilledema in ICH., (© 2022. The Author(s), under exclusive licence to Springer-Verlag France SAS, part of Springer Nature.)

Introduction: There is a scarcity of information available on clinical and laboratory features of adult-onset idiopathic autoimmune uveitis. Therefore, we conducted a single centre descriptive cross-sectional study. Patients and Methods . A chart review of all patients with idiopathic autoimmune uveitis with onset after 18 years of age who were referred to the rheumatology department between January 2017 and December 2018 was performed. Their clinical features, demographic features, and HLA-B genotypes were documented and described., Results: Out of 210 patients referred to rheumatology, 66 were found to have uveitis, and 16 of these had an adult-onset idiopathic autoimmune uveitis. Apart from a slight female preponderance (62.5%), our patients were characterized by a high proportion of panuveitis (4 out of 16, i.e., 25%). There was an increased frequency of occurrence of synechiae (5 out of 16, i.e., 31.3%), retinal vasculitis (4 out of 16, i.e., 25%), optic disc edema (3 out of 16, i.e., 18.8%), and cystoid macular edema (seen in 2 patients, i.e., 12.5%). These features correlated with the anatomical subtypes. Retinal vasculitis and optic disc edema present in three fourth of all panuveitis cases were the most prominent features. The odds of finding HLA-B∗35 in retinal vasculitis were 33 times higher than odds of finding it in idiopathic autoimmune uveitis patients not having retinal vasculitis (OR 33; 95% CI 1.6-698)., Conclusion: Idiopathic autoimmune uveitis in our patients is characterized by a high frequency of panuveitis and retinal vasculitis, and complications with a probable association between HLA-B∗35 and retinal vasculitis., Competing Interests: The authors declare that there are no conflicts of interest., (Copyright © 2022 Jyothi Visalakshy et al.)

Papilledema (choked disk) is a sign of intracranial hypertension (ICH) - condition that presents danger not only for patient's vision, but also for their life. Despite the fact that ICH is usually a neurosurgical pathology, sometimes an ophthalmologist is the first doctor such patients visit, most often in a primary healthcare clinic. At the same time, as practice shows, not all ophthalmologists are well aware about in this pathology; difficulties occur in differential diagnosis of papilledema against similar changes of the optic nerve head seen during ophthalmoscopic examination. This article reviews scientific literature on ICH, including benign ICH, diagnosis and differential diagnosis of papilledema. The authors also share their decades-long experience of working in a neurosurgical facility.

Spaceflight associated neuro-ocular syndrome (SANS) is hypothesized to develop as a consequence of the chronic headward fluid shift that occurs in sustained weightlessness. We exposed healthy subjects (n = 24) to strict 6° head-down tilt bed rest (HDTBR), an analog of weightlessness that generates a sustained headward fluid shift, and we monitored for ocular changes similar to findings that develop in SANS. Two-thirds of the subjects received a daily 30-min exposure to artificial gravity (AG, 1 g at center of mass, ~0.3 g at eye level) during HDTBR by either continuous (cAG, n = 8) or intermittent (iAG, n = 8) short-arm centrifugation to investigate whether this intervention would attenuate headward fluid shift-induced ocular changes. Optical coherence tomography images were acquired to quantify changes in peripapillary total retinal thickness (TRT), retinal nerve fiber layer thickness, and choroidal thickness, and to detect chorioretinal folds. Intraocular pressure (IOP), optical biometry, and standard automated perimetry data were collected. TRT increased by 35.9 µm (95% CI, 19.9-51.9 µm, p < 0.0001), 36.5 µm (95% CI, 4.7-68.2 µm, p = 0.01), and 27.6 µm (95% CI, 8.8-46.3 µm, p = 0.0005) at HDTBR day 58 in the control, cAG, and iAG groups, respectively. Chorioretinal folds developed in six subjects across the groups, despite small increases in IOP. Visual function outcomes did not change. These findings validate strict HDTBR without elevated ambient CO 2 as a model for investigating SANS and suggest that a fluid shift reversal of longer duration and/or greater magnitude at the eye may be required to prevent or mitigate SANS., (© 2021 The Authors. Physiological Reports published by Wiley Periodicals LLC on behalf of The Physiological Society and the American Physiological Society. This article has been contributed to by US Government employees and their work is in the public domain in the USA.)

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.

Anti-vascular endothelial growth factor (VEGF) therapies are now the first-line treatment for many ocular diseases, but some patients are non-responders to these therapies. The purpose of this study was to determine whether the level of adiponectin increased the pathogenesis of retinal edema and neovascularization in the retina of progressive ocular vascular diseases. We examined the role played by adiponectin in two types of cells and animal models which are retinal vein occlusion (RVO) and oxygen-induced retinopathy (OIR) mice. Our results showed that an injection of anti-adiponectin antibody ameliorated the retinal edema and ischemia through the depression of the expression level of VEGF-related factors and tight junction-related proteins in the retina of RVO mice. The intravitreal injection of anti-adiponectin antibody also decreased the degree of retinal neovascularization in an OIR mice. In addition, exposure of human retinal microvascular endothelial cells and human brain microvascular pericytes in culture to adiponectin increased both the vascular permeability and neovascularization through the increase of inflammatory factor and the dropout of the pericytes. These findings indicate that adiponectin plays a critical role in retinal edema and neovascularization, and adiponectin is a potential therapeutic target for the treatment of diabetic macular edema, proliferative diabetic retinopathy, and RVO., (© 2021 Federation of American Societies for Experimental Biology.)

Purpose: Confocal laser scanning microscopy (CLSM) is a non-invasive technique for cellular in vivo imaging of the human cornea. CLSM screening was evaluated for early detection of corneal nerve morphology changes and neuropathogenic events in different stage multiple myeloma (MM) patients. As MM patients show disease as well as therapy-related neuropathological symptoms, CLSM potentially provides a tool for non-invasive early detection of neuropathogenic events. CLSM findings were compared with the severity of peripheral neuropathic (PNP) symptoms., Methods: The study enrolled 25 MM patients in which bilateral ophthalmologic examination was performed including unilateral CLSM. Further peripheral nerve function was clinically evaluated using the conventional neuropathy symptom and neuropathy deficit scores (NDSs)., Results: In 18/25 MM patients, CLSM detected atypical morphological appearance of bulb-like enlarged nerve endings in the corneal sub-basal nerve plexus. These neuromas were only found in patients showing moderate to severe PNP, in patients with mild or lacking PNP neuromas were absent., Conclusions: CLSM provides a novel non-invasive diagnostic tool for identification of neuromas in cancer patients affected by therapy or disease-related neuropathologies, perspectival allowing early neuronal degenerative process detection and monitoring., (© 2020 The Author(s).)

Aim: To evaluate the impact of risk factors for intracranial hypertension (ICH) on cerebral cortex thickness in syndromic craniosynostosis., Method: ICH risk factors including papilloedema, hydrocephalus, obstructive sleep apnea (OSA), cerebellar tonsillar position, occipitofrontal circumference (OFC) curve deflection, age, and sex were collected from the records of patients with syndromic craniosynostosis (Apert, Crouzon, Pfeiffer, Muenke, Saethre-Chotzen syndromes) and imaging. Magnetic resonance images were analysed and exported for statistical analysis. A linear mixed model was developed to determine correlations with cerebral cortex thickness changes., Results: In total, 171 scans from 107 patients (83 males, 88 females [including repeated scans], mean age 8y 10mo, range 1y 1mo-34y, SD 5y 9mo) were evaluated. Mean cortical thickness in this cohort was 2.78mm (SD 0.17). Previous findings of papilloedema (p=0.036) and of hydrocephalus (p=0.007) were independently associated with cortical thinning. Cortical thickness did not vary significantly by sex (p=0.534), syndrome (p=0.896), OSA (p=0.464), OFC (p=0.375), or tonsillar position (p=0.682)., Interpretation: Detection of papilloedema or hydrocephalus in syndromic craniosynostosis is associated with significant changes in cortical thickness, supporting the need for preventative rather than reactive treatment strategies., What This Paper Adds: Papilloedema is associated with thinning of the cerebral cortex in syndromic craniosynostosis, independently of hydrocephalus., (© 2020 Mac Keith Press.)

Syphilis produces myriad nonspecific signs and symptoms. For example, optic disk swelling might be seen in patients with syphilis as a result of cranial hypertension (papilloedema), inflammatory optic neuritis with papillitis, or optic perineuritis. We report a case involving differential diagnosis of syphilitic bilateral papillitis mimicking papilloedema.

Aim: To determine whether changes in the serum levels of vascular endothelial growth factor (VEGF) after thalidomide therapy will affect the peripapillary retinal thickness (pRT) associated with optic disc oedema (ODE) in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome., Methods: This was a retrospective, observational case series of 23 right eyes of 23 treatment-naïve patients with POEMS syndrome and ODE whose intracranial pressure was within the normal range. The pRT was determined by spectral-domain optical coherence tomography, and the serum level of VEGF was determined by ELISA at baseline and 6 months after the thalidomide therapy. We determined whether a change in the pRT from baseline was significantly correlated with the serum level of VEGF from that at 6 months after the thalidomide treatment., Results: Six months after treatment, the mean serum level of VEGF was significantly reduced from 7153±4214 pg/mL to 1067±769 pg/mL (p<0.001), and the pRT was significantly decreased from 471.2±203 µm to 318.1±53.9 µm (p<0.001). The change in the pRT from baseline was significantly and linearly correlated with the change in the serum level of VEGF from that at 6 months after treatment (r=0.67, p=0.00039)., Conclusions: The close relationship between the pRT and the serum level of VEGF may offer clues on the pathogenesis of POEMS syndrome and potentially add a new candidate cause for the pathogenesis of ODE., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

In kidney biopsies reviews, scleroderma renal crisis (SRC) is characterized by vascular endothelial injuries, C4d deposits on peritubular vessels, and acute and chronic injuries coexisting on the same biopsy. The clinical signs of thrombotic microangiopathy (TMA) are described in systemic sclerosis (SSc), nevertheless, it has not been related to acute injuries described on kidney biopsies. We report a case of SRC in a patient with scleroderma-dermatomyositis overlap syndrome, which also showed clinical and histopathological data of TMA. On fundus examination, a severe acute hypertensive retinopathy was found. The kidney biopsy showed severe endothelial damage with widening of mucoid cells at the level of the intima, focal concentric proliferation on most small arterioles, and C3, C4d, and IgM deposits along the capillary walls. The genetic study of complement only showed the presence of membrane cofactor protein (MCP) risk haplotypes, without other genetic complement disorders. We understand that in a patient with TMA and SSc, the kidney damage would be fundamentally endothelial and of an acute type; moreover, we would observe clear evidence of complement activation. Once further studies correlate clinical-analytical data with anatomopathological studies, it is likely that we will be forced to redefine the SRC concept, focusing on the relationship between acute endothelial damage and complement activation.

Aim: To establish the correlation between clinical grading of papilloedema and diffusion abnormalities of optic nerve head (ONH) on diffusion-weighted imaging (DWI)., Materials and Methods: Brain magnetic resonance imaging (MRI), including readout segmented echo planar imaging-based DWI, was performed in 32 patients with papilloedema and the same number of age- and sex-matched controls. Clinical grading of papilloedema was done according to the modified Frisén scale. Two neuroradiologists independently evaluated the MRI for ONH hyperintensity and apparent diffusion coefficient (ADC) value of ONH. The comparison between papilloedema clinical grade and qualitative grade of ONH hyperintensity and its presence between cases and control groups were done using the Chi-square test and Fisher's exact test, respectively. The comparison between mean ADC value of ONH among different grades and between cases and controls were done using analysis of variance (ANOVA)-F-test and Student's t-test, respectively. Receiver operating characteristic (ROC) analysis was done to calculate a cut-off ADC value between the case and control groups., Results: Significant correlation between ONH hyperintensity and mean ADC value of ONH with clinical grades of papilloedema and between cases and control groups were found. ONH hyperintensity was found to be a highly sensitive (87.5% for both) and specific (specificity 97.1% and 98.6% for two observers) sign of papilloedema. A mean cut-off ONH ADC value was found to have high sensitivity (96.83%) and specificity (95.31%) to distinguish between the cases and controls., Conclusions: Diffusion parameters of ONH have significant correlation with clinical grading of papilloedema and can serve as a surrogate marker for intracranial pressure., (Copyright © 2019 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

Purpose: To evaluate the prevalence, clinical characteristics, and types of optic nerve involvement in patients with ocular toxoplasmosis., Methods: For this retrospective cross-sectional study, we examined all patients with active ocular toxoplasmosis referred to our Uveitis Section during the last 12 years, and we included patients with optic nerve involvement in the study. The primary outcome was the prevalence of optic nerve involvement, and secondary outcomes included the types of optic nerve involvement and the final best-corrected visual acuity after treatment., Results: The prevalence of optic nerve involvement was 14.4%, with the leading cause being the activation of a juxtapapillary lesion (70.5%). We found papillitis in two eyes and neuroretinitis in two eyes (11.7% for each). We only detected one optic nerve involvement secondary to a distant active lesion (5.8%). Sixteen patients (94.1%) had unilateral ocular toxoplasmosis. The overall final best-corrected visual acuity after treatment was 10/10 (LogMAR = 0.0) excluding the three patients with a juxtapapillary scar involving the macula., Conclusions: Optic nerve involvement was common in patients with ocular toxoplasmosis. The main type of optic nerve involvement was caused by activation of an old juxtapapillary lesion. Treatment was quickly effective, but the best-corrected visual acuity was dependent on the presence of a scar in the papillomacular bundle.

Vogt-Koynagi-Harada (VKH) disease is a clinical syndrome with classical ocular and extraocular findings that is not uncommonly described in Asian, Middle-Eastern and South American populations. We describe a case of VKH in an elderly Polish-Australian distinguished by prominent bilateral disc swelling rather than uveitis and marked leptomeningeal enhancement on MRI which led to extensive investigation including brain biopsy. Both disc oedema and MRI abnormalities improved dramatically with systemic steroid therapy. VKH disease is an important differential to consider in older patients with an uveo-meningeal picture and atypical eye findings where other causes have been excluded., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose : This study was undertaken to analyze the characteristics of optic neuritis in Korean patients seropositive for Toxocara . Methods : We retrospectively reviewed data from patients diagnosed with optic neuritis and followed up for at least one month between 2012 and 2016. Patients were grouped according to Toxocara serological testing outcomes (positive or negative) and clinical characteristics were compared. Results : The seropositive and seronegative groups comprised 13 and 12 patients, respectively. The seropositive patients were older (56.8 years versus 34.5 years), reported ocular pain less frequently (30.8% versus 91.7%), and showed more frequent asymmetric optic disc swelling (72.7% versus 22.2%). During follow-up, visual acuity of all seronegative patients improved to 20/40 or better, compared with 38.5% of the seropositive group. Conclusion : Atypical features such as painless, older age, or asymmetric disc swelling in optic neuritis may be related to seropositivity for Toxocara , suggesting the possibility of undiagnosed Toxocara optic neuropathy.

Hypoxia-induced retinal edema primarily induced by vascular lesion is seen in various conditions such as diabetic retinopathy (DR) and retinal vein occlusion (RVO). The edematous changes in these conditions occur mainly in intermediate and deep layers of retina as a result of disruption of the inner blood-retinal barrier (iBRB). However, the effect of direct and acute hypoxia on iBRB remains to be elucidated. To investigate direct and acute hypoxia-induced changes in retina, especially in astrocytes/Müller cells that are involved in the maintenance of retinal structure and function, we developed an adult mouse model of hypoxia-induced retinal edema by 24-h exposure in a 6% oxygen environment. Immunohistochemical staining of glial fibrillary acidic protein (GFAP) was enhanced mainly in the superficial layer of the hypoxic retina, corresponding to edematous change. Electron microscopic observation of the hypoxic retina showed vacuole formation in astrocyte/Müller cell foot processes around capillaries in the superficial layer, while no abnormal findings in the perivascular areas were found in intermediate and deep layers. Increase in vascular leakage quantified by Evans blue dye and tight junction breakdown detected by electron-dense tracer were observed in the hypoxia group. In the hypoxic retina, microglia was activated and relative gene expressions of pro-inflammatory cytokines were significantly upregulated. Dexamethasone suppressed these hypoxia-induced pathological reactions. Thus, unlike DR and RVO that induce iBRB breakdown in deeper retinal layers, atmospheric hypoxia induced iBRB disruption with subsequent edematous change mainly in the superficial layer of the retina, and that dexamethasone prevented these pathological changes. In this mouse model, direct and acute hypoxia induces retinal edema in the superficial layer of the retina with morphological changes of astrocytes/Müller cells, and is potentially useful for ophthalmic research in the field related to retinal hypoxia and its treatment., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Purpose: To assess the usefulness of spectral-domain optical coherence tomography (SD-OCT) peripapillary retinal nerve fiber layer (RNFL) thickness measurement in discriminating early phase optic disc edema (ODE) from pseudoedema (PODE)., Methods: Hospital-based, multicenter, cross-sectional study involving external patients referred for recent identification of "presumed ODE". Patients underwent SD-OCT optic nerve head (ONH) RNFL thickness measurement at their first evaluation. In 155 of these, the causative etiology was subsequently ascertained and the respective eyes (one per patient) were assigned to the ODE (95 eyes) or PODE (60 eyes) group. Admission SD-OCT data were retrieved and used for the analysis. ROC curve analysis was used to calculate specificity, sensitivity and predictive value (PV) of the RNFL values., Results: The PODE group was significantly younger than the ODE group (p = 0.007). The average and any single-quadrant RNFL thickness was significantly higher in the ODE group compared with the PODE and control groups. The average and the inferior quadrant thicknesses tested the most powerful parameters to differentiate ODE from PODE. A cutoff value of ≥ 110 μm for the average area, or of ≥ 150 μm for the inferior quadrant was associated with maximal sensitivity and specificity with positive PV greater than 80%., Conclusions: The SD-OCT evaluation of the peripapillary RNFL achieved good specificity, sensitivity and positive PV in discriminating between ODE and PODE. Despite the correct differential diagnosis between these categories still relies on a careful medical history taking and other ancillary testing, we proved the usefulness of SD-OCT RNFL measurement in supporting the diagnostic process., Competing Interests: The authors have declared that no competing interests exist.

An 81-year-old man with bilateral progressively blurry vision and optic disc swelling was referred for evaluation. Examination and ancillary testing confirmed a diagnosis of bilateral vitreopapillary traction accompanied by unilateral tractional retinoschisis in the right eye. Pars plana vitrectomy was performed to release the traction in both the eyes. Visual acuity improved in the right eye and stabilized in the left eye. Retinoschisis in the right eye resolved. The visual field improved in both the eyes although the left eye demonstrated a persistent hemifield defect likely attributable to a prior optic neuropathy. Distinguishing vitreopapillary traction optic neuropathy from nonarteritic anterior ischemic optic neuropathy is discussed., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: To report the neuro-ophthalmological findings in the TAFRO syndrome in a South American patient., Methods: This is a case report of a patient with TAFRO syndrome., Results: We present the case of a 66-year-old woman with TAFRO syndrome and multicentric Castleman disease, who developed ophthalmic manifestations, as a rare complication, including optic disk edema and serous retinal detachment, which improved with conventional therapy., Conclusions: The optic disk edema could be present as a neuro-ophthalmological finding in TAFRO syndrome. To the best of our knowledge, this is the first report on the ophthalmic manifestations in the TAFRO syndrome.

Purpose: To determine whether the diameter of Bruch's membrane opening (BMO) can distinguish mild papilledema from pseudopapilledema using optical coherence tomography (OCT)., Methods: The medical records of pediatric patients with pseudopapilledma due to optic nerve head (ONH) drusen, patients with papilledema, and normal control subjects were retrospectively reviewed. All eyes underwent OCT imaging of the BMO and retinal nerve fiber layer (RNFL). Transverse horizontal diameter of the BMO and papillary height were measured. Mean BMO, papillary height, and RNFL were compared and receiver operating characteristic (ROC) curves were used to calculate the area under the curve (AUC) and determine BMO and RNFL cut-offs for papilledema and pseudopapilledema., Results: A total of 90 eyes of 90 subjects were included: 58 with pseudopapilledema, 19 with papilledema, and 13 controls. In eyes with papilledema, mean BMO, papillary height, and RNFL decreased as papilledema resolved (1893.8 vs 1582.2 [P = 0.0003], 193.0 vs 108.9 [P < 0.0001], 893.3 vs 695.5 [P = 0.0007], resp.). Eyes with mild papilledema had greater mean BMO and RNFL than those with pseudopapilledema and controls (1893.8 vs 1541.9 vs 1628.8 [P < 0.0001, P = 0.0265] and 193.0 vs 108.7 vs 104.1 [P < 0.0001, P < 0.0001], resp.). Papillary height in mild papilledema was similar to pseudopapilledema but greater than controls (893.3 vs 863.2 vs 593.5 [P = 0.47 and P = 0.0001], resp.). ROC showed good diagnostic discrimination for BMO (AUC = 0.81; 95% CI, 0.70-0.92) and RNFL (AUC = 0.96; 95% CI, 0.93-1.0) in distinguishing mild papilledema from pseudopapilledema., Conclusions: The horizontal transverse diameter of BMO is enlarged in eyes with mild papilledema and narrows as papilledema resolves. BMO and RNFL can be used together to help distinguish mild papilledema from pseudopapilledema in children., (Copyright © 2017 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Background and Objectives: Hypertensive Retinopathy (HR) is a retinal disease which happened due to consistent high blood pressure (hypertension). In this paper, an automated system is presented that detects the HR at various stages using arteriovenous ratio and papilledema signs through fundus retinal images., Methods: The proposed system consists of two modules i.e. vascular analysis for calculation of arteriovenous ratio and optic nerve head (ONH) region analysis for papilledema.  First module uses a set of hybrid features in Artery or Vein (A/V) classification using support vector machine (SVM) along with its radial basis function (RBF) kernel for arteriovenous ratio. In second module, proposed system performs analysis of ONH region for possible signs of papilledema. This stage utilizes different features along with SVM and RBF for classification of papilledema., Results: The first module of proposed method shows average accuracies of 95.10%, 95.64% and 98.09%for images of INSPIRE-AVR, VICAVR, and local dataset respectively. The second module of proposed method achieves average accuracies of 95.93% and 97.50% on STARE and local dataset respectively., Conclusions: The system finally utilizes results from both modules to grade HR with good results. The presented system is a novel step towards automated detection and grading of HR disease and can be used as clinical decision support system., (Copyright © 2017 Elsevier B.V. All rights reserved.)

A significant proportion of the astronauts who spend extended periods in microgravity develop ophthalmic abnormalities. Understanding this syndrome, called visual impairment and intracranial pressure (VIIP), has become a high priority for National Aeronautics and Space Administration, especially in view of future long-duration missions (e.g., Mars missions). Moreover, to ensure selection of astronaut candidates who will be able to complete long-duration missions with low risk of the VIIP syndrome, it is imperative to identify biomarkers for VIIP risk prediction. Here, we hypothesize that the optic nerve sheath response to alterations in intracranial pressure may be a potential predictive biomarker for optic disc edema in astronauts. If confirmed, this biomarker could be used for preflight identification of astronauts at risk for developing VIIP-associated optic disc edema.

Differentiating optic disc edema (ODE) from pseudo optic disc edema (PODE) continues to pose a diagnostic dilemma. Current report highlights the role of multicolor imaging (MC) in differentiating ODE from PODE. Composite multicolor images of the disc in ODE show greenish hyperreflectance that extends beyond the optic disc margins with irregular blurry margins and obscured disc vasculature whereas PODE shows a greenish hyperreflectance with clear and distinct margins and well delineated disc vasculature. MC imaging adds to the present armamentarium of imaging modalities obviating needless neurological evaluation mandatory in a case of true disc edema.

Purpose: Optic neuritis is highly correlated with multiple sclerosis and is a major cause of acute visual loss and long-term neuronal degeneration. Primary cerebral hypoperfusion has been reported in brain demyelinating diseases. This study investigated whether peripapillary perfusion is changed in patients with acute optic neuritis (AON)., Methods: This three-year cohort study was conducted from September 1 2012, to August 31, 2015. Two hundred and forty-one patients with non-glaucomatous acute optic neuropathy were screened, and 42 non-highly myopic patients who had suffered their first episode of unilaterally idiopathic AON were studied. All cases received spectral-domain optical coherence tomography (OCT) examination, general survey, and standard corticosteroid therapy. OCT images were analyzed using a customized MATLAB program for measuring peripapillary choroidal thickness (PCT). Multivariate regression models were constructed to identify factors that are significantly related to peripapillary perfusion., Results: Decreased PCT was found in eyes experiencing AON combined with disc swelling (the ratio of lesion eye PCT/fellow eye PCT was 0.87 ± 0.08; range, from 0.75 to 1.00). In comparison to the healthy fellow eyes, approximately every 26% increase in the thickness of the retinal nerve fiber layer due to axonal swelling was associated with a 10% decreased thickness of PCT. Thinner PCT is also correlated with poorer trough vision, which may lead to poorer final vision. These findings were obvious in patients with optic papillitis but not in patients with retrobulbar neuritis., Conclusions: Peripapillary vascular hypoperfusion was found in patients experiencing AON combined with disc swelling. These findings are unlike those for other ocular inflammatory diseases but are consistent with cerebral hypoperfusion, which is found in brain demyelinating diseases; thus, these findings may represent a new neurovascular model in this field.

Purpose of Review: Mild papilledema may be difficult to distinguish by clinical observation from pseudopapilledema. An accurate diagnosis is critical to avoid invasive workup and unwarranted treatment. In this review, we focus on the development and subsequent role of optical coherence tomography (OCT) in detecting and differentiating optic nerve head drusen (ONHD) from papilledema and other causes of acquired swelling of the optic disc., Recent Findings: Newer OCT technologies which permit deeper penetration to improve detection of ONHD were also reviewed. Enhanced depth imaging (EDI) spectral-domain OCT and swept-source (SS) OCT are currently recognized as the most reliable and sensitive tools to diagnose ONHD. OCT devices currently available provide a means to quantify drusen dimensions, to evaluate the integrity of neighboring structures and to monitor axonal and neuronal damage, yielding additional information to better understand the relationship between the morphological features of drusen, and their effects on the structure and function of the optic nerve.

Kallidinogenase has been used to treat retinal vein occlusion (RVO) in patients, although there are no evidences on the effects of kallidinogenase on the retinal edema and the non-perfused areas in eyes with a RVO. We have established a murine RVO model with retinal edema and non-perfused areas. The purpose of this study was to evaluate the effects of kallidinogenase on the retinal edema and size of the non-perfused areas in the mouse RVO model. We evaluated the thickness of the retinal layers and size of the non-perfused areas, and the blood flow by laser speckle flowgraphy in RVO model. The effects of an intravenous injection of kallidinogenase on the retinal edema and size of the non-perfused areas were determined. In addition, the expressions of phosphorylated protein kinase B (Akt) and endothelial nitric oxide synthase (eNOS) were measured by Western blotting. Our results showed that kallidinogenase reduced the degree of retinal edema and size of the non-perfused areas by an increase in the blood flow in RVO model. Kallidinogenase also increased the levels of phosphorylated Akt and eNOS. These findings indicate that kallidinogenase acted through Akt/eNOS-dependent phosphorylation. Thus, kallidinogenase should be considered as a possible therapeutic agent for RVO patients., (Copyright © 2017 The Authors. Production and hosting by Elsevier B.V. All rights reserved.)

Correct differential diagnosis in cases of blurred optic disc margins is a challenging task for ophthalmologists. The reliable differentiation of pseudopapilloedema and true papilloedema has significant implications for proper patient management. Conditions that give rise to pseudopapilloedema include small crowded discs, tilted discs and optic nerve head drusen. Conditions that cause bilateral true swelling of the optic nerve head with initially good visual acuity include those that are secondary to raised intracranial pressure (optic disc edema, ODE). The majority of cases, however, present with unilateral optic nerve head swelling and normal intracranial pressure. They have systemic signs or symptoms which either precede ocular manifestation or have ophthalmoscopic signs other than elevation of the optic disc pointing to its diagnosis. Ancillary testing has been utilized to aid in identification of true ODE or swelling, including ultrasonography, fluorescein angiography, cranial and orbital MRI with venography, and lumbar puncture. Optical coherence tomography is also evolving as a modality for differentiation of buried optic disc drusen from ODE. This presentation will discuss each modality, with examples, advantages, and disadvantages for each., Competing Interests: Interessenkonflikt: Die Autoren geben an, dass kein Interessenkonflikt besteht., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: The time of onset of optic disc swelling in non-arteritic anterior ischaemic optic neuropathy (NAION) is not known, and it is commonly assumed to arise simultaneously with vision loss. Our goal is to report the presence and persistence of optic disc swelling without initial vision loss and its subsequent evolution to typical, symptomatic NAION., Methods: Clinical case series of patients with optic disc swelling and normal visual acuity and visual fields at initial presentation who progressed to have vision loss typical of NAION. All subjects underwent automated perimetry, disc photography and optic coherence tomography and/or fluorescein angiography to evaluate optic nerve function and perfusion., Results: Four patients were found to have sectoral or diffuse optic disc swelling without visual acuity or visual field loss; the fellow eye of all four had either current or prior NAION or a 'disc at risk' configuration. Over several weeks of clinical surveillance, each patient experienced sudden onset of visual field and/or visual acuity loss typical for NAION., Conclusions: Current treatment options for NAION once vision loss occurs are limited and may not alter the natural history of the disorder. Subjects with NAION may have disc swelling for 2-10 weeks prior to the occurrence of visual loss, and with the development of new therapeutic agents, treatment at the time of observed disc swelling could prevent vision loss from NAION., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Objective: To compare two methods for diagnosing mild papilloedema (PO) using peripapillary total retinal (PTR) and retinal nerve fibre layer (RNFL) thickness measurement by spectral domain optical coherence tomography (OCT) in patients suffering from posterior uveitis., Methods: 17 eyes in 17 patients with PO caused by posterior uveitis, 15 eyes in 15 patients with uveitis but with no PO based on slit lamp analysis were studied. High-quality OCT fundus images were analysed and graded by three masked observers using the Modified Frisén Scale. Eyes with PO were divided into two subgroups: mild (n=15) and moderate-severe PO (n=2). Two measurement methods were evaluated and compared: RNFL and PTR thickness measurements centred on the optic disc. Thickness values were calculated overall and for each quadrant and compared between groups. The main outcome measures were RNFL and PTR thickness, and thickness variation between control and affected patients for both protocols., Results: Average RNFL and PTR thickness in the moderate-severe PO, mild PO and control groups were 274.5±54.45 µm, 134±31.69 µm, 97.4±14.43 µm and 722.25±29.34 µm, 437.53±84.47 µm, 327.8±25.92 µm, respectively. Mild PO differed from the control groups according to both the RNLF (p=0.0006) and the PTR (p=0.0002) measurements. The average thickness variation between control and mild PO was significantly different between RNFL and PTR measurements: 36.6 µm vs 109.73 µm (p<0.0001), respectively., Conclusions: PTR thickness measurement increases the sensitivity of detection of mild PO and could be useful for diagnosing and monitoring papillitis. A new protocol should be developed to measure PTR in the same 3.5 mm disc as the RNFL measurement., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Objective: To study the influence of optic disc edema (ODE) on the dimensions of the disc and the thickness of the retinal nerve fiber layer (RNFL) by optical coherence tomography (OCT) in Lomé., Patients and Methods: This was a retrospective study which took place in a general ophthalmology office in the city. Twenty-six eyes were examined for ODE (cases) between January 2013 and May 2015, and 26 healthy eyes (control group) were considered in this study. The control group was recruited taking age into account with a variation not exceeding 5 years compared to the ODE cases. Topcon 3D-OCT 2000 software was used to analyze the disc and to measure the thickness of the peripapillary RNFL. The results were compared with software integrated into the OCT device, to the normal range of databases (normative basis) of RNFL thickness. The variables studied were age, gender, etiology of the edema, disc dimensions (optic disc surface area, vertical cup to disc ratio [CDR]), and the thickness of the peripapillary RNFL. Chi 2  tests were used for comparison of proportions with a significance level less than or equal to 0.05., Results: Twenty-six eyes of 16 patients with ODE (mean age 25.88±14.35years) and 26 eyes of 16 control group patients (mean age 25.73±15.90 years) were recruited. The ODE was caused by inflammatory optic neuropathies in 10 cases, malignant hypertension in 8 cases and intracranial hypertension in 8 cases. The surface area of the disc was 3.77±1.82mm 2  for the ODE group vs. 2.74±0.57mm 2  for the control group (P=0.009). The vertical CDR was 0.11±0.15 for the ODE group vs. 0.50±0.15 for the control group (P=0). The mean RNFL thickness was 117.12±18.30μm for the ODE group vs. 95.77±2.52μm for the control group (P=0.006). This study reveals an increase in RNFL in all quadrants (superior, P=0.027; inferior, P=0.007; nasal, P=0.026; temporal, P=0.032). Of the 26 ODE cases, we noted an increase beyond the normative base; superiorly (in 9 cases), inferiorly (in 12 cases), nasally (in 10 cases) and temporally (in 11 cases)., Conclusion: The study of papillary changes with OCT in ODE has allowed us to note an increase in the surface area of the disc, a reduction of the vertical CDR, and an increase in the thickness of the average RNF in all quadrants., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

The mechanism underlying the effects of anti-vascular endothelial growth factor (VEGF) antibody in retinal vein occlusion (RVO) treatment is poorly understood, partly due to the lack of RVO animal models that mimic clinical pathology. The aims of this study were to establish a suitable RVO model, clarify the pathogenic mechanisms, and evaluate the effects of anti-VEGF antibody in the model. Mouse retinal veins were occluded by laser photocoagulation after rose bengal injection. Reduction of the b/a wave amplitude ratio, retinal nonperfusion, cystoid edema, and hard exudates were observed after occlusion, and expression of RVO-related genes was altered. Administration of anti-VEGF antibody immediately, or 7 days, after occlusion resulted in reduction and increase of the nonperfused area, respectively. We conclude that the present model will be useful for clarification of the pathogenic mechanisms, and that the timing of anti-VEGF antibody administration is important for the successful amelioration of retinal nonperfusion.

Two critical functions for the control of intracranial fluids dynamics are carried on the venous side of the perfusion circuit: the first is the avoidance of cortical veins collapse during the physiological increases of cerebrospinal fluid (CSF) pressure in which they are immersed. The second, is the generation of an abrupt venous pressure drop at the confluence of the cortical veins with the dural sinuses that is required to allow a CSF outflow rate balanced with its production. There is evidence that both of these effects are ensured by a Starling resistor mechanism (a fluid dynamic construct that governs the flow in collapsible tubes exposed to variable external pressure) acting at the confluence of cortical veins in the dural sinus. This implies that, in normal circumstances of perfusion balance, a certain degree of venous collapse physiologically occurs at the distal end of the cortical vein. This is passively modulated by the transmural pressure of the venous wall (i.e. the difference between internal blood pressure and external CSF pressure). The mechanism provides that the blood pressure of the cortical vein upstream the collapsed segment is dynamically maintained a few mmHg higher than the CSF pressure, so as to prevent their collapse during the large physiological fluctuations of the intracranial pressure. Moreover, the partial collapse of the vein confluence also generates a sharp pressure drop of the blood entering into the sinus. The CSF is drained in dural sinus through arachnoid villi proportionally to its pressure gradient with the sinus blood. The venous pressure drop between cortical veins and dural sinus is therefore needed to ensure that the CSF can leave the cranio-spinal space with the same speed with which it is produced, without having to reach a too high pressure, which would compress the cortical veins. Notably, the mechanism requires that the walls of the dural sinuses are rigid enough to avoid the collapse under the external cerebrospinal fluid pressure, and predicts that in the presence of excessively flexible dural sinuses, the system admits a second point of balance between cerebral fluid pressure and dural sinus pressure, at higher values. The second balance state is due to the triggering of a self-limiting venous collapse feedback loop between the CSF pressure, that compresses the sinus, and the subsequent increase of the dural sinus pressure, that further raises the intracranial pressure. The loop may stabilize only when the maximum stretching allowed by the venous wall is reached. Then, a new relatively stable and self-sustaining balance state is achieved, at the price of a higher CSF and dural sinus pressure values. We propose that this model is crucially involved in Idiopatic Intracranial Hypertension pathogenesis with and without papilledema, a condition that could be described as a pathological new balance state, relatively stable, between intracranial and dural venous pressure, at higher absolute values.

Idiopathic intracranial hypertension (IIH) is a disorder of unknown origin, which is characterized by elevated intracranial pressure (ICP) without underlying etiological evidence of neurological disease. The purpose of the current study was to evaluate epidemiological features, clinical presentation, diagnostic findings and treatment of sixteen children (7 males and 9 females) with IIH. Medical records of the patients were obtained from the University Paediatric Hospital of Catania, Italy. Clinical features, investigations and treatment approaches were retrieved. The mean age of the sixteen children at onset of symptoms was 9 years (range: 4 to 16 years). Most of the patients were classified as pre-pubertal. Mean BMI was 28.9 kg/m2. In 93.75% of patients headache was the presenting clinical symptom; and in the same percentage papilledema was detected as the accompanied sign during diagnostic flow-chart. The mean lumbar puncture opening pressure (LPOP) was 350 mm H2O. Fifty percent of the cases had normal brain imaging, while 12.5% showed enlarged optic nerve diameter and one patient had an intraocular protrusion of the optic nerve on MRI. Two patients (12.5%) had venous sinus stenosis, and one case showed an abnormal spinal MRI. With regard to therapeutic approaches, 93.75% of the cases were successfully treated with Acetazolamide. None of the patients required surgical procedures, and all neuroimaging findings disappeared after receiving treatment. In the present study we investigated the association of IIH with venous sinus stenosis. We also found ocular ultrasound to be a useful non-invasive alternative method for determining papilledema in paediatric IIH, specifically in an emergency.

The purpose of this study was to investigate papilledema (PA) as a diagnostic criterion for the presence of antemortem or agonal cerebral edema despite normal postmortem brain swelling on postmortem magnetic resonance imaging (PMMR) in comparison with conventional autopsy.One hundred subjects with head PMMR and autopsy were included in this study. The sensitivities, spec icities, positive predictive values (PPVs), negative predictive values (NPVs), and accuracies were calculated in terms of the PA, PMMR, and cerebral edema on autopsy. Spearman r tests were used to analyze the linear correlations of PA and the radiological and autoptic determination of cerebral edema.In autopsy, the sensitivity regarding the presence of PA and cerebral edema was 66.2% (PPV, 70.5%), and specificity was 48.6% (NPV, 28.3%), with an overall accuracy of 60%. On PMMR, the sensitivity was 86.6% (PPV, 95%). The specificity was 90.9% (NPV, 34%), with an overall accuracy of 88%. The Spearman correlation revealed a statistically significant result (P < 0.001), which indicated a strong linear correlation of the presence of PA and cerebral edema with the autopsy results and the PMMR results.The presence of PA may aid in the diagnoses of cerebral edema despite normal postmortem brain swelling based on PMMR.

Optic disc oedema describes blurred optic disc margins. It describes an unspecific clinical sign with various possible underlying causal mechanisms. The correct diagnosis and the detection of the underlying disease is however of special clinical importance. This article summarizes the most important differential diagnoses and gives advice to find the correct diagnosis.

Arnold Chiari malformation type 1 is defined as a herniation of the cerebellar tonsils into the foramen magnum of more than 5 mm. Symptoms are most commonly dominated by occipital headache, torticollis and sometimes swallowing disorders. Ophthalmologically abnormal convergences, oculomotor palsy and diplopia are the main clinical signs. We report the case of a 9 year old child, who presented with visual loss evolving since 6 months. Ophthalmologic examination showed visual acuity of 4/10 in both eyes, retained ocular motility and rotational nystagmus. The examination of the anterior segment of the eye showed megalocornea with no evidence of goniodysgenesis, iridodonesis associated with atrophy of the dilator muscle and microcoria with lazy photomotor reflex. Normal intraocular pressure was 14 mmHg. Ocular fundus examination, despite difficulties in performing it, objectified bilateral papilledema (stage II). General physical examination showed torticollis, scoliosis and a tetra-pyramidal syndrome. MRI showed Chiari malformation type I associated with hydrocephalus and syringomyelia. Neurosurgical intervention based on internal CSF drainage with occipitocervical osteo-dural decompression was proposed. The evolution was favorable with regression of clinical signs. Ophthalmologically, there was a regression of papilledema but visual acuity remained stationary. The occurrence of papilledema associated with Chiari malformation type 1 is rare, it has been only reported in 2% of symptomatic patients. Its pathophysiology is still poorly understood. The originality of our study consists in the association of cerebellar malformations with ocular malformations including megalocornea and microcoria which make ophthalmologic examination more difficult to perform., Competing Interests: Conflits d’intérêts Les auteurs ne déclarent aucun conflit d'intérêt.