Optic Disc Elevation Research Articles

Chapter 6 - The role of optical coherence tomography in the diagnosis of afferent visual pathway problems: A neuroophthalmic perspective

Optical coherence tomography (OCT) is a noninvasive ocular imaging technique that has become a standard tool in neuroophthalmic practice. Specifically, OCT captures retinal manifestations of neuroaxonal injury caused by lesions along anterior and posterior regions of the afferent visual pathway, in patients presenting with vision loss. More recently, the advent of OCT angiography (OCTA) has enabled evaluation of the choroidal and retinal microvasculature, thus informing our understanding regarding vascular mechanisms associated with optic nerve and retinal injuries. Much of our longitudinal experience with OCT in the field of neuroophthalmology has been acquired from the study of optic neuritis (ON) caused by inflammatory disorders of the central nervous system (CNS). Over the past two decades, OCT has emerged as a surrogate endpoint for CNS neuroaxonal injury in multiple sclerosis (MS) research trials. On a more pragmatic level, OCT is used in the clinical arena to diagnose ON associated with: MS, neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein (MOG) antibody associated disease (MOGAD). Subsequent advancements in swept-source (SS) and enhanced depth imaging (EDI) have established OCT as the new "gold standard" in the diagnosis of optic disc drusen. Recent studies have highlighted pathognomonic OCT features that distinguish cases of true papilledema from pseudopapilledema, in patients presenting with undifferentiated optic disc elevation. Preoperative OCT measures of neuroaxonal integrity have shown prognostic value in predicting postoperative visual outcomes for patients with compressive anterior visual pathway lesions. Finally, OCT is indispensable in differentiating optic neuropathies from retinal diseases in patients with visual loss and a nondiagnostic fundus examination. An in-depth discussion regarding the technical aspects of OCT is beyond the scope of this chapter. Instead, we wish to highlight the value OCT brings to the diagnosis and management of common neuroophthalmic conditions, with emphasis on optic neuropathies and retinal disorders.

Utility of Point-of-Care Ultrasound in the Diagnosis of Idiopathic Intracranial Hypertension in the Emergency Department.

Emergency physicians are frequently required to identify and triage patients with increased intracranial pressure (ICP). Idiopathic intracranial hypertension (IIH) is a possible cause that must be considered. Its prognosis depends on prompt recognition and treatment, and progression of the disease can lead to permanent vision loss and considerable morbidity. Point-of-care ultrasound can rapidly identify elevated ICP. Measurements of the optic nerve sheath diameter (ONSD) and optic disc elevation (ODE) can act as surrogates for ICP. We describe five cases in which ultrasound was used to identify increased ICP and aid clinical decision-making. In several of the cases, ultrasound was used to confirm a suspicion for IIH and initiate therapy while awaiting the results of a more time-consuming and technically challenging test, such as lumbar puncture or optical coherence tomography. One of the patients was pregnant, and sonographic evidence of elevated ICP helped avoid exposing the patient to unnecessary radiation. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Ultrasound is a quick and versatile tool for screening patients with neurologic symptoms, and when integrated into the proper clinical context, can reduce the use of more invasive tests. It can be particularly useful in patients with pathology that may not show abnormalities on computed tomography scan or in whom lumbar puncture is technically difficult, making patients at risk for IIH well-suited to examination by ultrasound. We use a cutoff of 5mm for ONSD and 0.6mm for ODE, though there are no universally agreed on cutoff values.

A Prospective Study of Optic Nerve Ultrasound for the Detection of Elevated Intracranial Pressure in Severe Traumatic Brain Injury.

Intracranial pressure monitoring plays a critical role in the management of severe traumatic brain injury. Our objective was to evaluate the accuracy of optic nerve sheath diameter as a noninvasive screening test for the detection of elevated intracranial pressure and prediction of intracranial pressure treatment intensity. Prospective, blinded study of diagnostic accuracy. Neurotrauma ICU. Consecutive patients with severe traumatic brain injury. Optic nerve ultrasound was performed daily and optic nerve ultrasound measured at the point-of-care as well as remotely by an expert blinded to all patient details. Optic disc elevation was also measured. The index test was the highest remote-expert optic nerve ultrasound for the admission. The reference standard was the concurrent invasive intracranial pressure, with test-positivity set at intracranial pressure greater than 22 mm Hg. A priori the minimally acceptable sensitivity threshold was 90% with corresponding specificity 60%. We also evaluated the ability of optic nerve ultrasound to predict a therapeutic intensity level greater than 10. One hundred twenty patients were enrolled. The intraclass correlation coefficient between point of care and expert optic nerve sheath diameter after enrollment of 50 subjects was poor at 0.16 (-0.08 to 0.41) but improved to 0.87 (0.81-0.92) for the remaining subjects after remedial training. The area under the curve of the receiver operating characteristic curve of the highest expert-measured optic nerve sheath diameter to detect intracranial pressure greater than 22 mm Hg was 0.81 (0.73-0.87); area under the curve for prediction of therapeutic intensity level greater than 10 was 0.51 (0.42-0.60). Optic nerve sheath diameter greater than 0.72 demonstrated sensitivity 82% (48-98%) and specificity 79% (70-86%) for intracranial pressure greater than 22 mm Hg. The area under the curve of highest measured optic disc elevation to detect intracranial pressure greater than 22 mm Hg was 0.84 (0.76-0.90). Optic disc elevation greater than 0.04 cm attained sensitivity 90% (56-100%) and specificity 71% (61-79%). While optic nerve sheath diameter demonstrated a modest, statistically significant correlation with intracranial pressure, a predetermined level of diagnostic accuracy to justify routine clinical use as a screening test was not achieved. Measurement of optic disc elevation appears promising for the detection of elevated intracranial pressure, however, verification from larger studies is necessary.

Utility of Spectral-Domain Optical Coherence Tomography in Differentiating Papilledema From Pseudopapilledema: A Prospective Longitudinal Study.

Prospective and longitudinal studies assessing the utility of spectral-domain optical coherence tomography (SD-OCT) to differentiate papilledema from pseudopapilledema are lacking. We studied the sensitivity and specificity of baseline and longitudinal changes in SD-OCT parameters with 3D segmentation software to distinguish between papilledema and pseudopapilledema in a cohort of patients referred for evaluation of undiagnosed optic disc elevation. Fifty-two adult patients with optic disc elevation were enrolled in a prospective longitudinal study. A diagnosis of papilledema was made when there was a change in the appearance of the optic disc elevation on fundus photographs as noted by an independent observer at or before 6 months. The degree of optic disc elevation was graded using the Frisen scale and patients with mild optic disc elevation (Frisen grades 1 and 2) were separately analyzed. SD-OCT parameters including peripapillary retinal nerve fiber layer (pRNFL), total retinal thickness (TRT), paracentral ganglion cell layer-inner plexiform layer (GCL-IPL) thickness, and optic nerve head volume (ONHV) at baseline and within 6 months of follow-up were measured. Twenty-seven (52%) patients were diagnosed with papilledema and 25 (48%) with pseudopapilledema. Among patients with mild optic disc elevation (Frisen grades 1 and 2), baseline pRNFL (110.1 µm vs 151.3 µm) and change in pRNFL (ΔpRNFL) (7.3 µm vs 52.3 µm) were greater among those with papilledema. Baseline and absolute changes in TRT and ONHV were also significantly higher among patients with papilledema. The mean GCL-IPL thickness was similar at baseline, but there was a small reduction in GCL-IPL thickness among patients with papilledema. Receiver operator curves (ROCs) were generated; ΔpRNFL (0.93), ΔTRT (0.94), and ΔONHV (0.95) had the highest area under the curve (AUC). The mean baseline and absolute changes in SD-OCT measurements (pRFNL, TRT, and ONHV) were significantly greater among patients with papilledema, and remained significantly greater when patients with mild optic disc elevation were separately analyzed. ROCs demonstrated that ΔpRNFL, ΔTRT, and ΔONHV have the highest AUC and are best able to differentiate between papilledema and pseudopapilledema.

Role of Ocular Ultrasonography to Distinguish Papilledema From Pseudopapilledema.

We prospectively evaluated the sensitivity and specificity of ocular ultrasonography (OUS) to distinguish papilledema from pseudopapilledema. Forty-nine study participants, with optic disc elevation, underwent neuro-ophthalmic evaluation, OUS, fundus photography, and optical coherence tomography (OCT) of the optic nerve head at the initial and follow-up visits (≤6 months apart). Participants were classified as having papilledema if there was a change in optic nerve appearance on fundus photographs, as determined by a masked observer, between initial and follow-up visits ≤6 months apart. OUS was considered positive when the optic nerve sheath width was >3.3 mm and the 30° test was positive. Ocular ultrasonographic findings were correlated in patients who had papilledema vs patients who had pseudopapilledema. In a subanalysis, OUS findings were also correlated with change in peripapillary retinal nerve fiber layer thickness on OCT of the optic nerve head between initial and follow-up visits. OUS was 68% (17/25) sensitive for papilledema and 54% (13/24) specific for pseudopapilledema. When using OCT parameters to define papilledema, the sensitivity of OUS to diagnose papilledema decreased to 62%. Positive OUS correlated with elevated opening pressure on lumbar puncture and with signs of increased intracranial pressure on MRI. OUS alone was less sensitive in diagnosing papilledema than previously thought. Therefore, OUS may not be helpful in distinguishing between papilledema and pseudopapilledema.

Microvascular Changes Associated with Optic Disc Drusen: Case Report

Optic disc drusen (ODD) is an important clinical entity that is sometimes misdiagnosed as papilledema because of elevated and blurred disc margins. A 17-year-old male who presented with headaches underwent detailed ophthalmological examination as well as colored fundus photography, B-scan ultrasonography (USG), fundus autofluorescence (FAF), optical coherence tomography (OCT), optical coherence tomography angiography (OCTA), and visual field testing. His visual acuity was 10/10 in both eyes. Fundus examination revealed bilateral blurred and elevated optic disc margins. Diagnosis of bilateral ODD was confirmed with B-scan USG. FAF imaging revealed hyperautofluorescent areas on both optic discs. Optic nerve head OCT scans showed elevated irregular disc borders and thinning of the retinal nerve fiber layer in both eyes. On visual field testing, loss of the nasal visual field was detected in the left eye. OCTA imaging showed focal capillary dropout, especially in the nasal peripapillary area, in both eyes and reduced peripapillary and macular vessel density. In this case report, we evaluated the clinical findings and the structural features of bilateral ODD with multimodal imaging modalities including OCTA.

Accuracy of machine learning for differentiation between optic neuropathies and pseudopapilledema

BackgroundThis study is to evaluate the accuracy of machine learning for differentiation between optic neuropathies, pseudopapilledema (PPE) and normals.MethodsTwo hundred and ninety-five images of optic neuropathies, 295 images of PPE, and 779 control images were used. Pseudopapilledema was defined as follows: cases with elevated optic nerve head and blurred disc margin, with normal visual acuity (> 0.8 Snellen visual acuity), visual field, color vision, and pupillary reflex. The optic neuropathy group included cases of ischemic optic neuropathy (177), optic neuritis (48), diabetic optic neuropathy (17), papilledema (22), and retinal disorders (31). We compared four machine learning classifiers (our model, GoogleNet Inception v3, 19-layer Very Deep Convolution Network from Visual Geometry group (VGG), and 50-layer Deep Residual Learning (ResNet)). Accuracy and area under receiver operating characteristic curve (AUROC) were analyzed.ResultsThe accuracy of machine learning classifiers ranged from 95.89 to 98.63% (our model: 95.89%, Inception V3: 96.45%, ResNet: 98.63%, and VGG: 96.80%). A high AUROC score was noted in both ResNet and VGG (0.999).ConclusionsMachine learning techniques can be combined with fundus photography as an effective approach to distinguish between PPE and elevated optic disc associated with optic neuropathies.

Bedside Transorbital Ultrasound in the Clinical Evaluation of Pediatric Optic Neuritis in the Emergency Department.

Headache and monocular visual disturbance are worrisome pediatric presenting complaints in the emergency department. Appropriate and timely initial evaluation is critical. Most would opt for urgent computer tomography in such cases. Pediatric optic neuritis is a rare condition and is better evaluated by magnetic resonance imaging. With the increase in the use and scope of bedside ultrasound, there might be a potential role for transorbital ultrasound to be part of the emergency department evaluation of pediatric optic neuritis. This is the first pediatric case report on the use of bedside transorbital ultrasound in the emergency department evaluation of a 15-year-old girl with optic neuritis who presented with unilateral headache and left visual disturbance. Transorbital ultrasound of her left eye revealed an irregularly enlarged optic nerve sheath with increased optic nerve sheath diameter (5.1mm) and an elevated optic disc height (0.5mm). Ultrasound examination of her right eye was contrastingly normal, showing an optic nerve sheath diameter of 3.8mm and that the optic disc was not elevated. The ultrasound findings correlated well with her magnetic resonance imaging of her orbits. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: The clinical findings and monocular ultrasound abnormalities facilitated the emergency department decision-making process and choice of neuroimaging. This highlights the use of transorbital ultrasound as a clinical adjunct and potential role in the emergency department clinical evaluation of a pediatric patient with optic neuritis. The finding of an irregularly enlarged optic nerve might be of potential clinical value but further studies are required.

The Role of Optical Coherence Tomography in Differentiating Optic Disc Drusen from Optic Disc Edema.

An elevated optic nerve head can be an ominous sign, sometimes signifying an underlying basis for raised intracranial pressure. Alternatively, patients may harbor a different mechanism for this optic nerve head appearance, including optic disc drusen (ODD), which does not confer any life-threatening implications. It is important to refine the approach to distinguishing ODD from papilledema because this is a relatively common, and important, conundrum encountered in clinical practice. Optical coherence tomography (OCT) is a noninvasive, readily accessible, and cost-effective ocular imaging technique that can improve the diagnostic accuracy for detecting ODD; or, instead, increase the index of suspicion for papilledema, thus prompting additional investigations needed to identify (or exclude) potential causes of raised intracranial pressure. In this review, we will discuss the value of OCT in the diagnostic approach to cases of mild optic disc elevation, with acknowledgment of the potential pearls and pitfalls of this imaging technology. In particular, we will emphasize the helpful role OCT can play in differentiating cases of ODD from mild papilledema in the context of idiopathic intracranial hypertension (IIH).

B-Mode Transorbital Ultrasonography for the Diagnosis of Idiopathic Intracranial Hypertension: A Systematic Review and Meta-Analysis.

In patients with idiopathic intracranial hypertension (IIH), transorbital sonography (TOS) may reveal an enlargement of the optic nerve sheath diameter (ONSD) and the presence of optic disc elevation (ODE), as a sign of an increase in intracranial pressure (ICP). We systematically reviewed the TOS findings inadults with IIH. MEDLINE, EMBASE, Cochrane Library and CENTRAL (1966 - May 2017) were searched to identify studies reporting data on patients with IIH assessed by B mode-TOS. Data were extracted and included in a meta-analysis, and the quality of the included studies was evaluated. 5 studies with 96 patients were included. The values of ODE were 0.8 - 1.2 mm and ONSD was 6.2 - 6.76 mm in IIH patients vs. 4.3 - 5.7 mm in controls. In IIH patients the ONSD was significantly higher compared to controls (overall weighted mean difference of 1.3 mm (95 % CI: 0.6 - 1.9 mm)). The meta-analysis of proportion of papilledema based on results of three studies revealed a pooled estimator of 87 % (95 % CI: 76 - 94 %). IIH patients have higher ONSD values and higher frequency of ODE compared to controls. The indirect, noninvasive ICP assessment using TOS may be useful in supporting the clinical diagnosis ofIIH in adults by detecting increased ONSD values and the presence of ODE.

Elevated Optic Disc Height and Increased Optic Nerve Sheath Diameter on Bedside Ultrasound in a Pediatric Patient With Orbital Cellulitis: More Than Meets the Eye

BackgroundOrbital cellulitis is an uncommon ophthalmological emergency in children, but rapid emergency department (ED) diagnosis is essential. Case ReportA 13-year-old boy presented to our pediatric ED with left orbital cellulitis secondary to pansinusitis. Emergency bedside ocular ultrasonography was used to evaluate and expedite his management. Besides inflammatory changes observed on ultrasound of his affected orbit, the patient had an elevated optic disc height and increased nerve sheath diameter, which were not commonly reported in published literature on orbital cellulitis. Emergent computed tomography of the orbits and head showed orbital cellulitis without complications of orbital abscess or cavernous sinus thrombosis. Despite initiating early appropriate antibiotics, there was rapid progression of his disease and he developed intraconal abscess and cavernous sinus thrombosis the following day. After emergency surgical drainage of his pansinusitis, antibiotics, and anticoagulation, he was discharged well after a 2-week hospitalization. Why Should an Emergency Physician Be Aware of This?There are important advantages of using bedside ultrasonography for diagnosis of pediatric orbital cellulitis in the ED setting. Further research should be done to evaluate the clinical significance of an enlarged optic nerve sheath diameter and raised optic disc height in pediatric orbital cellulitis.

Identification of Optic Nerve Swelling Using Point-of-Care Ocular Ultrasound in Children.

The aim of this study was to determine the feasibility and accuracy of point-of-care (POC) ocular ultrasound (US) when performed by a pediatric emergency medicine (PEM) physician to detect optic nerve abnormalities concerning for swelling, as compared with the fundus examination performed by an ophthalmologist. This was a single-center, prospective cohort pilot study of children aged 12 months to 18 years who required optic disc evaluation by an ophthalmologist. Eligible subjects were enrolled from the emergency department, inpatient wards, and neuro-ophthalmology outpatient clinic of an urban, tertiary care children's hospital. Point-of-care ocular US, specifically assessing optic nerve sheath diameter and optic disc elevation, was performed. Findings on US were compared with findings identified by an ophthalmologist on dilated fundus examination. Seventy-six subjects were enrolled; 20 (26%) of 76 had findings concerning for optic nerve swelling diagnosed by an ophthalmologist on fundus examination. Using a sonographic definition for optic nerve swelling of optic nerve sheath diameter greater than 4.5 mm or the presence of optic disc elevation, the sensitivity and specificity were 90% and 55%, respectively. The success rate of POC ocular US was 100%, and the mean time to completion was 8 minutes. For emergency department subjects in whom direct fundus examination was attempted, the PEM physician could visualize the optic disc and assess for swelling in only 40% (14/35) of examinations. The results of our study suggest that POC ocular US performed by PEM physicians was feasible and determined to be sensitive but nonspecific in the detection of optic nerve swelling. Additional larger studies may determine generalizability to other nonophthalmologist physicians performing POC ocular US.

Ocular ultrasound for monitoring pseudotumor cerebri syndrome.

The aim of this study was to evaluate the feasibility of ocular ultrasound in the follow-up of pseudotumor cerebri syndrome and to correlate ultrasound with clinical parameters. In a cohort study of 22 consecutive adult patients, ocular ultrasound was performed measuring optic nerve sheath diameter, optic nerve diameter, and optic disc elevation compared with symptoms referred by the patients. The patients showed increased optic nerve sheath diameter [median, 6.51mm (interquartile range 6.13-7.10)], optic nerve diameter [3.02mm (2.86-3.27)], and optic disc elevation [0.90mm (0.64-1.36)] at the time of admission and had ophthalmologically confirmed the presence of papilledema in all 22 patients. After 6months all parameters decreased significantly for optic nerve sheath diameter [6.08mm (5.59-6.73), P=0.002], optic nerve diameter [2.87mm (2.70-3.15), P=0.007], and optic disc elevation [0.48mm (0.30-0.70), P<0.001]. In addition, a discrete negative correlation between optic nerve sheath diameter and headache change after 6months was observed with ρ=-0.477 and P=0.02. No correlation was found between optic disc elevation and headache. In conclusion, longitudinal follow-up with ocular ultrasound combined with clinical information may provide support for treatment of this condition.

Optical coherence tomography in the differential diagnosis of true edema versus pseudoedema of the optic disc

PurposeTo assess the predictive value of Spectral Domain OCT (SD‐OCT) to discriminate between pathological true optic disc edema (ODE) and mimickers responsible for pseudoedema (PODE).MethodsWe carried out an observational cross‐sectional study of subjects consecutively referred from 2011 to 2017 to the Neuro‐Ophthalmology service of 4 different hospitals for presumed acute ODE. Among a total of 155 eyes, 95 were identified as ODE and 60 as PODE. Patients underwent the optic nerve OCT evaluation using Zeiss Cirrus Optic Disc Cube 200x200 analysis. Average and four principal quadrants RNFL values were compared between ODE and PODE groups and then with age‐matched controls. ROC curve and AUC were calculated to determine optimal cut‐off values. Mann‐Whitney and chi‐square tests were used to compare groups. Statistical correlation was tested by Spearman's rho test. All tests were two sided with p&lt;.05 being considered statistically significant. Statistical analysis was performed with IBM SPSS Statistics 24.ResultsAverage and RNFL thickness of each optic nerve quadrants resulted significantly higher in ODE compared to control and PODE; among quadrants, the highest correlation was reported for the inferior one. Combining the appropriate cut‐off value for average and inferior quadrant, we found a negative predictive value of 91% for ODE when compared to PODE. No statistically significant differences were seen between control and PODE.ConclusionsOur data show the usefulness of SD‐OCT in the management of acute optic disc elevation of uncertain causes. In particular, the results herein reported provide high negative predictive value in differentiating benign mimickers from true edema of the optic disc, thus avoiding misdiagnosis and invasive procedures not necessary for such patients.

Decision Support System for Detection of Papilledema through Fundus Retinal Images.

A condition in which the optic nerve inside the eye is swelled due to increased intracranial pressure is known as papilledema. The abnormalities due to papilledema such as opacification of Retinal Nerve Fiber Layer (RNFL), dilated optic disc capillaries, blurred disc margins, absence of venous pulsations, elevation of optic disc, obscuration of optic disc vessels, dilation of optic disc veins, optic disc splinter hemorrhages, cotton wool spots and hard exudates may result in complete vision loss. The ophthalmologists detect papilledema by means of an ophthalmoscope, Computed Tomography (CT), Magnetic Resonance Imaging (MRI) and ultrasound. Rapid development of computer aided diagnostic systems has revolutionized the world. There is a need to develop such type of system that automatically detects the papilledema. In this paper, an automated system is presented that detects and grades the papilledema through analysis of fundus retinal images. The proposed system extracts 23 features from which six textural features are extracted from Gray-Level Co-occurrence Matrix (GLCM), eight features from optic disc margin obscuration, three color based features and seven vascular features are extracted. A feature vector consisting of these features is used for classification of normal and papilledema images using Support Vector Machine (SVM) with Radial Basis Function (RBF) kernel. The variations in retinal blood vessels, color properties, texture deviation of optic disc and its peripapillary region, and fluctuation of obscured disc margin are effectively identified and used by the proposed system for the detection and grading of papilledema. A dataset of 160 fundus retinal images is used which is taken from publicly available STARE database and local dataset collected from Armed Forces Institute of Ophthalmology (AFIO) Pakistan. The proposed system shows an average accuracy of 92.86% for classification of papilledema and normal images. It also shows an average accuracy of 97.85% for classification of already classified papilledema images into mild and severe papilledema. The proposed system is a novel step towards automated detection and grading of papilledema. The results showed that the technique is reliable and can be used as clinical decision support system.

New classification of acute papilledema in children with severe malaria

Raised intracranial pressure is a feature of cerebral malaria in children living in Africa. We investigated specific clinical optic disc features of papilledema to establish their prognostic significance in this encephalopathy. We developed a classification of acute papilledema and tested it against disease outcome. Kenyan children admitted with severe falciparum malaria (cerebral or impaired consciousness) underwent dilated fundal examination using direct and indirect ophthalmoscopy. Clinical features of the optic disc were systematically recorded and compared to the child's outcome. Poor outcome defined as death or neurological impairment on discharge was used to construct and test a clinical classification of papilledema. Forty-five children were examined (26 cerebral malaria, 17 severe malaria with an impaired conscious level or prostration) of whom seven had a poor outcome (three died, four had residual neurological impairment). Loss of the optic disc cup and marked optic disc elevation were significantly correlated with a poor outcome (P < 0.05). Increasing severity in the proposed classification of acute papilledema was positively correlated with a poor outcome (P < 0.05, chi-square test for trend). Loss of the optic disc cup and marked elevation of the optic disc head appear to be correlated with poor outcome in children with severe malaria whereas the presence of dilated veins suggests a good outcome. The proposed classification of acute papilledema is useful as a prognostic indicator and may be applicable to other encephalopathies with raised intracranial pressure.

Quantification of Peripapillary Total Retinal Volume in Pseudopapilledema and Mild Papilledema Using Spectral-Domain Optical Coherence Tomography

To distinguish differences in retinal nerve fiber layer (RNFL) thickness and peripapillary total retinal volume between eyes with papilledema, pseudopapilledema, and normal findings. Cohort study. Forty-two eyes with mild papilledema, 37 eyes with congenitally elevated optic disc (pseudopapilledema), and 34 normal eyes met the inclusion criteria at 1 academic institution (in Iran) and underwent neuro-ophthalmic examination. Spectral-domain optical coherence tomography scans surrounding the optic disc were performed in each eye of patients and subjects. Main outcome measures were mean RNFL thickness and peripapillary total retinal volume measurements (inner and outer ring volumes) that were compared between groups, using the generalized estimating equation approach. Area under receiver operating characteristic curves were also calculated. A statistically significant difference was found in mean RNFL thickness between both groups of patients with papilledema and pseudopapilledema and normal subjects. Average inner peripapillary total retinal volume in the papilledema, pseudopapilledema, and control groups were 1.95 ± 0.24 mm(3), 1.81 ± 0.23 mm(3), and 1.06 ± 0.10 mm(3), respectively. Average outer peripapillary total retinal volume in the papilledema and pseudopapilledema groups were 2.68 ± 0.49 mm(3) and 2.03 ± 0.24 mm(3), respectively (P < .001). However, the outer ring peripapillary total retinal volume was not different between pseudopapilledema and normal (1.90 ± 0.11 mm(3)) eyes (P = .17). Area under the curve to discriminate pseudopapilledema vs papilledema eyes for average RNFL thickness and inner and outer peripapillary total retinal volumes was 0.82, 0.68, and 0.88, respectively. Outer peripapillary total retinal ring volumes might be useful in differentiating papilledema from pseudopapilledema.

Multiple Myeloma Recurrence with Optic Nerve Infiltration Diagnosed by Vitrectomy, Immunohistochemistry, and in Situ Hybridization

We present a case of multiple myeloma recurrence diagnosed by optic nerve infiltration. Interventional case report with clinical, surgical, immunohistochemical, and fluorescence in situ hybridization correlation. A 51-year-old woman with a history of bilateral invasive ductal breast carcinoma and multiple myeloma, both in remission on maintenance bortezomib, was referred for sudden, painless loss of vision OS. Examination demonstrated anterior vitritis with severe optic disc elevation, with yellow-white thickening, peripapillary hemorrhages, and a retinal detachment inferiorly. Diagnostic vitrectomy showed CD138-positive and BRST2-negative cells. Fluorescence in situ hybridization was positive for del(13q) and p53 deletion and negative for CCND1/IGH. This is the first report of optic nerve infiltration of multiple myeloma as evidence of recurrence while on maintenance chemotherapy. We demonstrate that diagnostic vitrectomy and immunohistochemistry of vitreous fluid is feasible for the diagnosis of recurrent multiple myeloma.

Teaching Neuro Images : Chiasmal enlargement and enhancement in Leber hereditary optic neuropathy

A 19-year-old man was admitted for subacute severe painless bilateral loss of vision. The patient was known to be a healthy carrier of the mitochondrial G11778A mutation, with a family history of Leber hereditary optic neuropathy. Funduscopy revealed bilateral optic disc elevation and hyperemia (figure). Goldmann visual fields demonstrated large central scotomas in both eyes. CSF disclosed normal opening pressure and cytochemical examination. Unexpectedly, brain MRI revealed an asymmetrical chiasmal enlargement and enhancement (figure). Similar cases have rarely been reported.1,2 These findings would suggest disruption of the blood–brain barrier at the early stage of the disease,2 likely due to an inflammatory-like mechanism.

Optic Nerve Sonography to Monitor Treatment Efficacy in Idiopathic Intracranial Hypertension: A Case Report

To report values of optic nerve sheath diameter (ONSD) and optic disc elevation (ODE) obtained with optic nerve sonography (US) in the diagnosis and monitoring of treatment efficacy in an adult with idiopathic intracranial hypertension (IIH). Serial measurements of the ONSD and ODE using B mode US were performed in a 45-years-old woman with IIH before and during after treatment with acetazolamide and diet. At first evaluation US showed a significantly enlarged ONSD (.68 cm right; .66 cm left side) and the presence of increased ODE (.1 cm right; .15 cm left side). Post-punctural assessments showed a bilateral decrease of ONSD (.58 cm right; .58 cm left side), without changes in ODE values. After 12 months of treatment with acetazolamide and diet ODE completely normalized (0 cm on both sides). ODE values correlated directly with ONSD, and both ODE and ONSD values correlated directly with BMI. Correlations were statistically significant. ONSD changes occurred rapidly after the lumbar puncture, whereas the papilloedema required longer to reduce. US of ONSD and ODE was useful to support the diagnosis of IIH and to monitor the efficacy of diet and pharmacological treatment. Further studies are required to evaluate whether this promising technique may be considered a reliable and accurate method to longitudinally evaluate patients with increased ICP secondary to IIH.