The yield of ophthalmoscopy as a screening tool for intracranial pathology in pediatric headache.

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.

Peripapillary hyperreflective ovoid mass-like structures: Multimodal imaging-A review.

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.

Excess adiponectin in eyes with progressive ocular vascular diseases.

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.

Transorbital sonography: A non-invasive bedside screening tool for detection of pseudotumor cerebri syndrome.

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.

Describing the Clinical and Laboratory Features and HLA-B Pattern of Adult-Onset Idiopathic Autoimmune Uveitis at a Tertiary Hospital in South India: A Cross-Sectional Study.

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.

Subarachnoid space of the optic nerve sheath and intracranial hypertension: a macroscopic, light and electron microscopic study.

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.

[Papilledema as a sign of intracranial hypertension]. / Zastoinyi disk zritel'nogo nerva kak priznak vnutricherepnoi gipertenzii.

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.

QUANTITATIVE TOPOGRAPHIC CURVATURE MAPS OF THE POSTERIOR EYE UTILIZING OPTICAL COHERENCE TOMOGRAPHY.

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.

Diagnosis of Syphilitic Bilateral Papillitis Mimicking Papilloedema.

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.

Intracranial hypertension and cortical thickness in syndromic craniosynostosis.

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.

Hipertensión intracraneal y grosor cortical en craneosinostosis sindrómica OBJETIVO: Evaluar el impacto de los factores de riesgo de hipertensión intracraneal (HIC) en el grosor de la corteza cerebral en la craneosinostosis sindrómica. MÉTODO: La neuroimagen, y, los factores de riesgo para HIC que incluyeron papiledema, hidrocefalia, apnea obstructiva del sueño (SAOS), posición de la tonsila cerebelosa, edad de desviación de la curva de circunferencia occipitofrontal (CFO) y el sexo, se recogieron de los registros de pacientes con craneosinostosis sindrómica (Apert, Crouzon, Pfeiffer, Muenke, Saethre-Chotzensis). Las imágenes de resonancia magnética fueron analizadas y exportadas para análisis estadístico. Se desarrolló un modelo mixto lineal para determinar las correlaciones con los cambios en el espesor de la corteza cerebral. RESULTADOS: En total se evaluaron 171 exploraciones de 107 pacientes (83 varones, 88 mujeres [incluyendo escaneos repetidos], edad media 8 años 10 meses, rango 1 año 1 mes - 34 años, DE 5 años 9 meses). El espesor medio cortical en esta cohorte fue de 2,78 mm (DS 0,17). Los hallazgos anteriores de papiledema (p=0,036) y de hidrocefalia (p=0,007) se asociaron de forma independiente con el adelgazamiento cortical. El grosor cortical no varió significativamente por sexo (p=0,534), síndrome (p=0,896), SAOS (p=0,464), CFO (p=0,375), o posición tonsilar (p=0,682). INTERPRETACIÓN: La detección de papiledema o hidrocefalia en la craneosinostosis sindrómica, se asocia con cambios significativos en el grosor cortical. Esto apoya la necesidad de estrategias de tratamiento preventivo en lugar de tratamientos reactivos.

Hipertensão intracrianiana e espessura cortical na craniossinostose sindrômica OBJETIVO: Avaliar o impacto de fatores de risco para hipertensão intracraniana (HIC) na espessura cortical em craniossinostose sindrômica. MÉTODO: Fatores de risco para HIC incluindo papiloedema, hidrocefalia, apnéia obstrutiva do sono (AOS), posição das tonsilas do cerebelo, idade de deflexão da curva da circunferência occipitofrontal (COF), e sexo foram coletados dos registros de pacientes com craniossinostose sindrômica (síndromes de Apert, Crouzon, Pfeiffer, Muenke, Saethre-Chotzen) e imagens. As imagens de ressonância magnética foram analisadas e exportadas para análise estatística. Um modelo linear misto foi desenvolvido para determinar correlações com mudanças na espessura do córtex cerebral. RESULTADOS: No total, 171 imagens de 107 pacientes (83 do sexo masculino, 88 do sexo feminino [incluindo varreduras repetidas], média de idade 8a 10m, variação 1a 1m-34a, DP 5a 9m) foram avaliados. A espessura cortical média nesta coorte foi 2,78mm (DP 0,17). Achados prévios de papiloedema (p=0,036) e de hidrocefalia (p=0,007) foram independentemente associados com a redução cortical. A espessura cortical não variou significativamente com o sexo (p=0,534), síndrome (p=0,896), AOS (p=0,464), COF (p=0,375), ou posição tonsilar (p=0,682). INTERPRETAÇÃO: A detecção do papiloedema ou hidrocefalia na craniossinostose sindrômica se associa com mudanças significativas na espessura cortical, sustentando a necessidade de estratégias de tratamento preventivas e não reativas.

Protective effects of dexamethasone on hypoxia-induced retinal edema in a mouse model.

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.

Papilloedema: diffusion-weighted imaging of optic nerve head.

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.

Neuro-ophthalmological findings in TAFRO syndrome in a patient from South America, a variant of multicentric Castleman's disease.

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.

Effects of kallidinogenase on retinal edema and size of non-perfused areas in mice with retinal vein occlusion.

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.

Optical Coherence Tomography to Differentiate Papilledema from Pseudopapilledema.

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.

Idiopathic intracranial hypertension in a paediatric population: a retrospective observational study on epidemiology, symptoms and treatment.

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.

Die randunscharfe Papille: eine diagnostische Herausforderung.

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.

Papilledema as a Diagnostic Sign of Cerebral Edema on Postmortem Magnetic Resonance Imaging.

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.

Clinical and multimodal imaging characteristics of acute Vogt-Koyanagi-Harada disease unassociated with clinically evident exudative retinal detachment.

The purpose of this study was to describe the clinical and multimodal imaging findings in acute Vogt-Koyanagi-Harada (VKH) disease without clinically evident exudative retinal detachment (ERD). We retrospectively reviewed the charts of 18 patients (36 eyes), diagnosed with acute VKH disease without clinically evident ERD. All patients underwent complete ophthalmic examination, fundus photography, optical coherence tomography (OCT), B-scan ultrasonography, fluorescein angiography (FA), and indocyanine green angiography (ICGA). Of 18 patients, twelve (66.7 %) were female and 6 (33.3 %) were male. Mean age was 39 years (range, 23-60). Ten patients had been referred with an erroneous diagnosis of primary optic nerve disorder (8; 44.4 %) or isolated anterior uveitis (2; 11.1 %). Anterior chamber or vitreous inflammatory reaction was noted in 22 eyes (61.1 %), each. Fundus findings included optic disc swelling in 30 eyes (83.3 %), retinal striae in 20 eyes (55.5 %), and yellowish deep lesions in 3 eyes (8.3 %). OCT showed a shallow, localized subclinical ERD in 18 eyes (50 %), and retinal pigment epithelial folds in 23 eyes (63.9 %). B-scan ultrasonography showed diffuse, low- to medium-reflective choroidal thickening in all eyes. FA disclosed delayed choroidal perfusion in at least one eye of all patients (100 %), mild pinpoint leakage in 21 eyes (58.3 %), optic disc hyperfluorescence in 35 eyes (97.2 %) and choroidal folds in 13 eyes (36.1 %). ICGA findings included delayed choroidal perfusion in 24 eyes (66.7 %), decrease in the number of large choroidal vessels in 36 eyes (100 %), fuzzy choroidal vessels in 35 eyes (97.2 %), and hypofluorescent dark dots in 28 eyes (77.8 %). The association of bilateral optic disc edema with retinal striae and intraocular inflammatory reaction highly suggests acute VKH disease. A multimodal imaging approach including fundus photography, OCT, B-scan ultrasonography, FA, and ICGA provides important clues for the definite diagnosis and help differentiate VKH disease from primary optic nerve disorders.