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Background: Papilledema is the optic disc swelling caused by increased intracranial pressure (ICP) that can damage the optic nerve and cause subsequent vision loss. Pseudopapilledema refers to optic disc elevation without peripapillary fluid that can arise from several optic disc disorders, with optic disc drusen (ODD) being the most frequent cause. Occasionally, pseudopapilledema patients are mistakenly diagnosed as papilledema, leading to the possibility of unneeded procedures. We aim to thoroughly examine the most current evidence on papilledema and pseudopapilledema causes and several methods for distinguishing between both conditions., Methods: An extensive literature search was conducted on electronic databases including PubMed and google scholar using keywords that were relevant to the assessed pathologies. Data were collected and then summarized in comprehensive form., Results: Various techniques are employed to distinguish between papilledema and pseudopapilledema. These techniques include Fundus fluorescein angiography, optical coherence tomography, ultrasonography, and magnetic resonance imaging. Lumbar puncture and other invasive procedures may be needed if results are suspicious., Conclusion: Papilledema is a sight-threatening condition that may lead to visual affection. Many disc conditions may mimic papilledema. Accordingly, differentiation between papilledema and pseudopailledema is crucial and can be conducted through many modalities., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

A 44-year-old woman presented with decreased vision in both eyes. The retina in both eyes had drusen distributed along vascular arcades, central macula and in peripapillary region. Macula had pigmented scarring and exudation. Fundus autofluorescence showed drusen. Optical coherence tomography showed drusen, subretinal and intraretinal fluid. Fundus fluorescein and indocyanine green angiography showed drusen, retinal pigment epithelial atrophy and vascular network. Younger age at presentation, bilateral symmetry, typical distribution of drusen along the arcades in a radiating pattern, peripapillary involvement, scarring and atrophy at macula were suggestive of doyne honeycomb retinal dystrophy. The reduced vision was due to macular atrophy and an active choroidal neovascular membrane. The patient was treated with antivascular endothelial growth factor injections for choroidal neovascular membrane. Our case highlights the importance of pattern recognition and multimodal imaging for diagnosing the type of macular dystrophy as doyne honeycomb retinal dystrophy, while simultaneously managing choroidal neovascular membrane., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: Optic disc drusen (ODD), present in 2% of the general population, have occasionally been reported in patients with nonarteritic anterior ischemic optic neuropathy (NA-AION). The purpose of this study was to examine the prevalence of ODD in young patients with NA-AION., Design: Retrospective, cross-sectional multicenter study., Methods: All patients with NA-AION 50 years old or younger, seen in neuro-ophthalmology clinics of the international ODDS (Optic Disc Drusen Studies) Consortium between April 1, 2017, and March 31, 2019, were identified. Patients were included if ODD were diagnosed by any method, or if ODD were excluded by enhanced-depth imaging optical coherence tomography (EDI-OCT) using ODDS Consortium guidelines. NA-AION eyes with ODD were termed "ODD-AION"; those without were termed "NODD-AION"., Results: A total of 65 patients (127 eyes) with NA-AION were included (mean 41 years old). Of the 74 eyes with NA-AION, 51% had ODD-AION, whereas 43% of fellow eyes without NA-AION had ODD (P = .36). No significant differences were found between ODD-AION and NODD-AION eyes in terms of Snellen best-corrected VA or perimetric mean deviation. According to EDI-OCT results, 28% of eyes with NODD-AION had peripapillary hyperreflective ovoid mass-like structures (PHOMS); 7% had hyperreflective lines, whereas 54% with ODD-AION had PHOMS; and 66% had hyperreflective lines (P = .006 and P < .001, respectively)., Conclusions: Most of these young NA-AION patients had ODD. This indicates that ODD may be an independent risk factor for the development of NA-AION, at least in younger patients. This study suggests ODD-AION be recognized as a novel diagnosis., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Sudden visual loss and optic disc edema caused by optic neuritis (ON) is usually followed by significant visual recovery. However, little or no recovery occurs when the loss is caused by atypical ON, especially in patients with neuromyelitis optica (NMO). Optic disc drusen (ODD) is a cause of pseudo optic disc edema and may be a predisposing factor for non-arteritic anterior ischemic optic neuropathy (NAION), thereby mimicking atypical ON. In such cases, if globular concretions are seen protruding from the disc substance, ODD may be suspected. The purpose of this paper is to describe two patients with acute visual loss followed by optic disc atrophy initially labeled as atypical ON. Though not suspected on clinical examination, optical coherence tomography (OCT) revealed deeply buried ODD as a predisposing factor for NAION., Case Presentations: Case 1: A 48-year-old woman had bilateral sequential visual loss associated with optic disc edema. Despite treatment, vision did not improve and severe disc pallor ensued. Atypical ON was suspected. Eventually, she was started on immunosuppressant therapy based on a tentative diagnosis of NMO-spectrum disorder. On examination 5 years later, only severe optic disc pallor was observed, but OCT radial B-scans showed ovoid hyporeflective areas in the retrolaminar region of both eyes, compatible with ODD; this led to a diagnosis of NAION and deeply buried ODD. Case 2. A 35-year-old woman with suspicion of ON in the left eye and a history of previous atypical ON in the right eye was referred for neuro-ophthalmic examination which revealed diffuse optic disc pallor and a dense arcuate visual field defect in the right eye. OCT B-scans passing through the disc showed large ovoid areas of reduced reflectivity in the retrolaminar region of the optic disc in the right eye. These findings helped confirm the diagnosis of NAION in one eye, with deeply buried ODD as predisposing factor., Conclusions: Deeply buried ODD may be associated with NAION causing irreversible visual loss and optic disc pallor, a condition easily mistaken for atypical ON. Awareness of such occurrence is important to avoid unnecessary testing and minimize the risk of mismanagement.

Optic disc drusen may be a cause of visual field defects and visual loss. The mechanism by which this occurs is unclear. We report a patient who developed decreased vision in the right eye and was found to have a heavy burden of superficial optic disc drusen. Optical coherence tomography (OCT) confirmed focal retinal nerve fiber layer thinning that corresponded with the distribution of drusen. OCT angiography, with superficial laminar segmentation, showed focal capillary attenuation overlying the most prominent drusen. These findings demonstrate alterations in the superficial retinal capillary network associated with optic disc drusen.

Purpose: To compare optical coherence tomography angiography (OCT-A) with traditional multimodal imaging in patients with Malattia Leventinese., Design: Retrospective case series., Methods: Eight eyes of 4 consecutive patients with Malattia Leventinese were retrospectively studied. All patients underwent a complete ophthalmologic examination including color fundus photography, fluorescein and indocyanine green angiographies, spectral-domain optical coherence tomography, and optical coherence tomography angiography., Results: The choriocapillaris segmentation of OCT-A revealed the presence of a hyperflow signal corresponding to active choroidal neovascularization in 3 eyes and inactive choroidal neovascularization in 1 eye. Traditional multimodal imaging did not show each vascular layer and any leakage in these cases., Conclusion: OCT-A, unlike traditional multimodal imaging, helps diagnose choroidal neovascularization in patients with Malattia Leventinese suffering from metamorphopsia and reduced visual acuity., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: To investigate choriocapillaris (CC) alteration in patients with nascent geographic atrophy (nGA) and/or drusen-associated geographic atrophy (DAGA) using swept-source optical coherence tomography angiography (OCTA)., Methods: A 1,050-nm wavelength, 400 kHz A-scan rate swept-source optical coherence tomography prototype was used to perform volumetric swept-source optical coherence tomography angiography over 6 mm × 6 mm fields of view in patients with nGA and/or DAGA. The resulting optical coherence tomography (OCT) and OCTA data were analyzed using a combination of en face and cross-sectional techniques. Variable interscan time analysis (VISTA) was used to differentiate CC flow impairment from complete CC atrophy., Results: A total of 7 eyes from 6 patients (mean age: 73.8 ± 5.7 years) were scanned. Seven areas of nGA and three areas of DAGA were identified. Analysis of cross-sectional OCT and OCTA images identified focal alterations of the CC underlying all seven areas of nGA and all three areas of DAGA. En face OCTA analysis of the CC revealed diffuse CC alterations in all eyes. Variable interscan time analysis processing suggested that the observed CC flow alterations predominantly corresponded to flow impairment rather than complete CC atrophy., Conclusion: The OCTA imaging of the CC revealed focal CC flow impairment associated with areas of nGA and DAGA, as well as diffuse CC flow impairment throughout the imaged field. En face OCT analysis should prove useful for understanding the pathogenesis of nGA and DAGA and for identifying the formation of nGA and DAGA as endpoints in therapeutic trials., Competing Interests: There are no conflicting relationships for any other author.

In a case of Malattia Leventinese, optical coherence tomography angiography led to the diagnosis of type 1 neovascularization, despite absence of evidence on conventional dye-based angiography. The authors hypothesize that, at least in some cases, accumulation of subretinal fluid in Malattia Leventinese could be due to a subretinal pigment epithelium (RPE) neovascular component rather than creation of hydrophobic barrier at the RPE and Bruch's membrane. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:602-604.]., (Copyright 2016, SLACK Incorporated.)

Cases Report: Two patients presented with headache and bilateral papillary edema. Patient 1 was found to have a papilledema (P) with intracranial pressure of 32cmH2O. Patient 2 was found to have a migraine with a pseudopapilledema (PP) (optic nerve head drusen). SD-OCT was used to image the optic disc, subretinal hyporeflective space (SHS), and alpha-angle (Aα)., Discussion: Optic disc SD-OCT may be useful for differentiating disc morphology in P and PP. The area of the SHS and the Aα were higher in the P patient than in the patient with PP., (Copyright © 2014 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.)

Optic nerve head drusen (ONHD) are incidental ophthalmologic finding in the optic nerve. Patients with ONHD are often asymptomatic, but sometimes present with transient visual obscuration's (TVO), the reported incidence of which is 8.6%. Optic nerve head drusen are of two types: Superficial; visible and deep. The deep-buried drusen mimic papilledema. Because of the varied presentation deep-buried drusen pose a diagnostic challenge to the ophthalmologists. In young patients, they are mistaken for papilledema as it is clinically difficult to detect a buried drusen in the optic nerve head, but are seen on the surface with aging as the retinal nerve fiber layer thins out. They are observed as pale yellow lesions more often located towards the poles. Clinical examination aided with diagnostic tests like computed tomography (CT) orbits and ultrasound B scan can help establish the diagnosis. Herein, we report a rare case of optic nerve head drusen in a young lady, who presented with loss of vision and clinical evaluation and investigations suggested ONHD with anterior ischemic optic neuropathy.

Purpose: To describe the clinical features of hemorrhagic complications secondary to optic nerve head drusen (ONHD) using spectral domain optical coherence tomography (SD-OCT)., Methods: Sixty-three consecutive patients with SD-OCT-documented ONHD who presented at Seoul National University Bundang Hospital from December 2009 to July 2012 were included. Full ophthalmologic examinations, including fundus photographs, SD-OCT, fundus angiography, and visual field tests were analyzed in a total of 101 ONHD-positive eyes from 63 patients., Results: Hemorrhagic ONHD complications were found in 7 eyes (7%) from a total of 101 eyes with ONHD. All of them had buried ONHD (visualized with SD-OCT) and myopia (mean spherical equivalent = -4.00 ± 2.35 diopters). Patients with ONHD hemorrhagic complications had smaller disk diameters than patients without hemorrhagic complications (1,308 ± 166 vs. 1,555 ± 217 μm, P = 0.004). Peripapillary hemorrhages were classified into the following 3 types based on SD-OCT findings: subretinal (6 eyes, 86%), retinal (5 eyes, 71%), and vitreous hemorrhage (4 eyes, 57%). Six patients (86%) complained of the recent onset of visual symptoms, but visual acuities at presentation were 20/20 in all patients. In the three patients who were followed up, most hemorrhages were absorbed without complications., Conclusion: Peripapillary hemorrhage can often occur in patients with buried ONHD and small disk diameters. As SD-OCT can be used to visualize ONHD beneath hemorrhage, it is helpful with the differential diagnosis and follow-up evaluation.

Background: Optic nerve head drusen (ONHD) are calcific deposits buried or at the surface of the optic disc. Although ONHD may be associated with progressive visual field defects, the mechanism of drusen-related field loss is poorly understood. Methods for detecting and imaging disc drusen include B-scan ultrasonography, fundus autofluorescence, and optical coherence tomography (OCT). These modalities are useful for drusen detection but are limited by low resolution or poor penetration of deep structures. This review was designed to assess the potential role of new OCT technologies in imaging ONHD., Evidence Acquisition: Critical appraisal of published literature and comparison of new imaging devices to established technology., Results: The new imaging modalities of enhanced depth imaging optical coherence tomography (EDI-OCT) and swept source optical coherence tomography (SS-OCT) are able to provide unprecedented in vivo detail of ONHD. Using these devices it is now possible to quantify optic disc drusen dimensions and assess integrity of neighboring retinal structures, including the retinal nerve fiber layer., Conclusions: EDI-OCT and SS-OCT have the potential to allow better detection of longitudinal changes in drusen and neural retina and improve our understanding of drusen-related visual field loss.

Background: To assess the efficacy of quantitative analysis of the optic nerve head and peripapillary retinal nerve fiber layer (RNFL) with the spectral-domain optical coherence tomography (SD-OCT) in differentiating optic disc edema (ODE) from optic nerve head drusen (ONHD)., Methods: Prospective clinical study. Twenty-five eyes of 25 ODE patients (group 1), 25 eyes of 25 ONHD patients (group 2), and 25 eyes of 25 healthy subjects were included. The thickness of the peripapillary RNFL, the thickness of the subretinal hyporeflective space (SHYPS), the area of the SHYPS, the horizontal length of the optic nerve head, and the angle between the temporal RNFL and the optic nerve head (α-angle) were evaluated with SD-OCT., Results: The mean RNFL thickness was significantly greater in group 1 when compared with group 2 and control group (P < 0.001). The receiver operating characteristic curve areas for temporal and nasal RNFL thicknesses in differentiating group 1 and group 2 were 0.819 and 0.851, respectively (for temporal RNFL thickness >101.5 μm: sensitivity 92%, specificity 65%; for nasal RNFL thickness >74.5 μm: sensitivity 92%, specificity 47%). The mean SHYPS thickness, SHYPS area, and degree of the α-angle were greater in group 1 when compared with group 2 (P < 0.05). For the SHYPS thickness >464 μm: 85% sensitivity and 60% specificity; for the SHYPS area >811 μm: 85% sensitivity and 89% specificity; and for the α-angle >141°: 77% sensitivity and 95% specificity were obtained., Conclusion: The quantitative analysis of the optic nerve head and peripapillary RNFL with SD-OCT can provide useful data in differentiating ODE from ONHD.

Purpose: Buried disc drusen are an important differential diagnosis for papilledema. Spectral domain optical coherence tomography (SD-OCT) affords clinicians with new non-invasive opportunities to probe below the surface of the optic nerve. Clinicians may use the knowledge of this appearance to rule out buried disc drusen in patients with irregular optic nerve borders or a bulging, hyperemic appearance., Methods: SD-OCTs were obtained in a patient with one surfacing disc druse, identifying the nature of the appearance of this disc druse and others in this and the contralateral eye when imaged with this technology. B-scan ultrasonography was used to confirm the presence of disc drusen. Additional scans in multiple patients with confirmed buried drusen were obtained for comparison., Results: Drusen appear as rounded hyporeflectant areas on SD-OCT, similar in appearance to blood vessels. They share the appearance of cysts but show a fine hyperreflective border anteriorly. These same discrete hyporeflective areas were found at various depths within optic nerve heads with confirmed buried disc drusen., Conclusions: The hyporeflective appearance may not be anticipated by clinicians, as B-scans show calcified drusen as hyperreflective on echo. It is hypothesized that the hyporeflectant appearance of drusen is due to a constancy in refractive index through the druse, as OCT detects changes in optical reflectivity. Thus, drusen are likely dense and homogenous. SD-OCT may be more useful in those patients with buried disc drusen which are not calcified as B-scan often contributes little in such cases.

Purpose: To evaluate the effect of optic nerve head drusen on retinal nerve fiber layer (RNFL) thickness in children, with the use of optical coherence tomography (OCT)., Material and Methods: 21 patients (mean age 13.9 years) with optic nerve head drusen were studied prospectively. For statistic comparison a group of 15 control subjects (mean age 14.1 years) participated. Ophthalmologic examination, automated visual field testing and optical coherence tomography were performed in each patient. For statistical analysis we used Shapiro-Wilk and U Mann-Whitney tests., Results: In the OCT measurements the nasal RNFL was significantly thinner as compared to the reference group. Neither statistically significant thinning of global RNFL between groups nor relation between subjects age and RNFL thickness was found., Conclusions: Optic nerve head drusen can lead to visual function defects, therefore early and correct diagnosis is mandatory. Optical coherence tomography is a usefull diagnostic tool used to determine the cause of disc elevation, which is especially important in childhood, when drusen can cause disc to appear papilledema-like. Overall OCT appears to be a non-invasive, sensitive and early-indicating method of RNFL thinning.

Objective: To demonstrate a new algorithm that can determine the shape, location, and volume of optic nerve head drusen (ONHD), which were imaged with spectral domain optical coherence tomography (SDOCT)., Methods: One exenteration patient and 4 glaucoma patients with bilateral ONHD were recruited from the Massachusetts Eye and Ear Infirmary and from a private practice office. Images were obtained using an experimental SDOCT system developed at the Wellman Center for Photomedicine, Massachusetts General Hospital. With axial resolutions of about 6 mum, SDOCT can obtain 2-dimensional images in 1/29 of a second, compared with commercially available time domain OCT instruments with 10 mum resolution images in 1.28 seconds. The volumes of ONHD were calculated with a new algorithm and were then correlated with visual field mean deviation., Results: SDOCT can display 2-dimensional images comparable with histology and 3-dimensional videos of ONHD. ONHD are signal-poor regions with high-signaled borders. Larger ONHD volumes are directly correlated with larger mean deviation absolute values on Humphrey visual field testing., Conclusions: SDOCT is a potentially better technique for ONHD imaging and may improve the diagnosis and management of patients with both OHND and glaucoma.

Case Reports: Three cases of pigmentary epithelial tears are described in this study. One of them was related to previous extrafoveal photocoagulation, whereas the other two were idiopathic. Fluorescein angiography (FA) and optical coherence tomography (OCT) were performed to confirm the clinical diagnosis., Discussion: Few studies using OCT to assess pigmentary retinal epithelial (RPE) tears have been published in the world literature. The main findings of our study included hyper-reflectant line breaks, choroidal hyper-reflectance due to the lack of RPE and RPE double layering where it was folded. Images were frequently associated with RPE tears and neurosensorial retinal detachment.

Aim: To describe the appearance of the non-exudative forms of age related macular degeneration (AMD) as imaged by ultrahigh resolution optical coherence tomography (UHR-OCT)., Methods: A UHR-OCT ophthalmic imaging system, which utilises a femtosecond laser light source capable of approximately 3 mum axial resolution, was employed to obtain retinal cross sectional images of patients with non-exudative AMD. Observational studies of the resulting retinal images were performed., Results: 52 eyes of 42 patients with the clinical diagnosis of non-exudative AMD were imaged using the UHR-OCT system. 47 of the 52 (90%) eyes had the clinical diagnosis of drusen and/or retinal pigment epithelial (RPE) changes. In these patients, three patterns of drusen were apparent on UHR-OCT: (1) distinct RPE excrescences, (2) a saw toothed pattern of the RPE, and (3) nodular drusen. On UHR-OCT, three eyes (6%) with a clinical diagnosis of non-exudative AMD had evidence of fluid under the retina or RPE. Two of these three patients had findings suspicious for subclinical choroidal neovascularisation on UHR-OCT., Conclusion: With the increased resolution of UHR-OCT compared to standard OCT, the involvement of the outer retinal layers are more clearly defined. UHR-OCT may allow for the detection of early exudative changes not visible clinically or by angiography.