Are Peripapillary Hyperreflective Ovoid Mass-like Structures with an Elevated Optic Disc Still a Diagnosis Dilemma?

PURPOSE: To identify clinical characteristics that would help make or rule out the diagnosis of idiopathic intracranial hypertension (IIH) in patients referred for papilledema (PE) with peripapillary hyperreflective ovoid mass-like structures (PHOMS). DESIGN: A retrospective cohort study. PARTICIPANTS: All patients referred for PE excluding PE with Frisén grade ≥ 3, optic neuritis, ischemic optic neuropathy, and compressive optic neuropathy. Patients were divided into 2 groups: group 1 = isolated PHOMS; group 2 = PHOMS associated with IIH. METHODS: We analyzed the location of PHOMS based on OCT-enhanced depth imaging (EDI) and calculated their volume. MAIN OUTCOME MEASURES: Peripapillary retinal nerve fiber layer (pRNFL), ganglion cell complex, and volume of PHOMS. RESULTS: A total of 154 patients (308 eyes) were included. Patients' mean age was 29 years, with a female predominance (78%). Peripapillary hyperreflective ovoid mass-like structures were associated with these etiologies: IIH (38.3%), isolated (35.7%), posterior uveitis (11%), optic disc drusen (ODD) (10%), and tilted optic disc (5%). Magnetic resonance imaging (MRI) was performed in 83.1% of cases. More than half of the MRI scans were interpreted as consistent with IIH. However, only 39.7% of these patients had confirmed IIH with 44.5% sensitivity and 55.5% specificity. Peripapillary hyperreflective ovoid mass-like structures were overrepresented in the nasal region (95.5%).The location of PHOMS in the superior or inferior quadrant was significantly associated with IIH or ODD, whereas their presence in the temporal or nasal sector was strongly associated with isolated lesions. The mean and median volume of PHOMS were 1.66 µm3 and 1.50 µm3, respectively. There was a significant difference in PHOMS volume, with a higher volume in patients with IIH (P = 0.0037). Follow-up of these patients at 3 and 6 months demonstrated no significant changes in visual function, as per visual field mean deviation, visual acuity measurements, and ganglion cell layer. Mean pRNFL showed a decrease of -4.225 µm at 3 months and -6.489 µm at 6 months when compared with the initial measurement independent of the etiology. CONCLUSIONS: Isolated PHOMS should be considered as a distinct entity. In asymptomatic patients, PHOMS should be carefully studied. Nasal or temporal location, small volume, and stable aspect over the course of weeks or months are suggestive of this entity. This strategy would considerably reduce the impact on patients' anxiety and morbidity. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Validation of retinal oximetry vessel selection using fluorescein angiography in patients with optic disc drusen.

Retinal oximetry could provide insights into the pathophysiology of optic nerve disease, including optic disc drusen (ODD). Vessel selection for oximetry analysis is based on morphological characteristics of arterioles and venules and supported by an overlay of estimated blood oxygen saturations. The purpose of this cross-sectional study was to determine the validity of this vessel selection procedure by comparing it with vessel selection supported by video fluorescein angiography (FA). The study included 36 eyes of 36 patients with ODD who underwent retinal oximetry (Oxymap retinal oximeter T1) followed by FA (Heidelberg Spectralis). Two trained graders selected vessel segments in a pre-defined measurement area around the optic disc. One of these graders additionally performed the vessel segment selection with the support of FA images. When performed by the same grader, FA-supported and non-FA-supported vessel selection did not lead to significant differences in total vessel segment length, estimated oxygen saturations or vessel diameters (all p > 0.05). Inter-grader differences were found for arterial and venous segment lengths and arterial saturation (p < 0.05). A similar tendency was found for the arteriovenous saturation difference (p = 0.10). In conclusion, identifying vessel segments for retinal oximetry analysis based on vessel morphology and supported by a color-coded saturation overlay appears to be a valid method without the need for invasive angiography. A numerically small inter-grader variation may influence oximetry results. Further studies of retinal oximetry in ODD are warranted.

Morphological and Immunohistochemical Differentiation of Neuronal and Glial Cells of the Vascular and Avascular Regions of the Donkey's Paurangiotic Retina.

INTRODUCTION: Ocular diseases pose a significant health concern for donkeys. However, studies examining the microanatomy and cell populations of the donkey retina are scarce. The current study aimed to describe the vascular pattern of the donkey retina and document its cellular components. METHODS: The donkey retina specimens were obtained from different retinal regions and prepared for semithin sectioning and immunohistochemistry. RESULTS: The donkey has a paurangiotic retina in which retinal vessels are confined to a narrow area around the optic disc. Glial cells coexist with the blood vessels being very numerous in the vascular region and become scanty in the avascular ones. S-100-positive astrocytes could be observed in these avascular areas. Ganglion cells are organized in a single layer with the least population existing in the peripheral retina. Acidic fibroblast growth factor (AFGF) is immunoreactive in amacrine and ganglion cells. A subpopulation of amacrine cells reacted strongly to tyrosine hydroxylase (TH), and others reacted positively to S-100 protein. Ganglion cell nuclei exhibited a strong immunoreactivity to S-100 protein as well. Furthermore, glial fibrillary acidic protein (GFAP) is used to identify Müller cells that extend their processes across the retina from the inner to the outer limiting membrane. CONCLUSIONS: In conclusion, our findings provide novel insights into the normal retinal organization. The donkey retina shows the characteristic expression of immunohistochemical markers for the major cell types. In addition, the distribution of glial cells is comparable between the vascular and avascular regions.

(What's the story) morning glory? MRI findings in morning glory disc anomaly.

PURPOSE: Morning glory disc anomaly (MGDA) is a rare congenital ophthalmologic disorder. Historically it has been diagnosed fundoscopically, with little in the literature regarding its imaging findings. The purpose of this study is to further characterize the orbital and associated intracranial magnetic resonance imaging (MRI) findings of MGDA in our tertiary pediatric center. METHODS: A retrospective review was performed of fundoscopically-diagnosed cases of MGDA, that had been referred for MRI. All MRI studies were scrutinized for orbital and other intracranial abnormalities known to occur in association with MGDA. RESULTS: 18 of 19 cases of MGDA showed three characteristic MRI findings: funnel-shaped morphology of the posterior optic disc, abnormal soft tissue associated with the retrobulbar optic nerve, and effacement of adjacent subarachnoid spaces. The ipsilateral (intraorbital) optic nerve was larger in one patient and smaller in six. The ipsilateral optic chiasm was larger in two patients and smaller in one. CONCLUSION: This study represents a comprehensive radiological-led investigation into MGDA. It describes the most frequently-encountered MRI findings in MGDA and emphasizes the importance of MRI in this cohort, i.e., in distinguishing MGDA from other posterior globe abnormalities, in assessing the visual pathway, and in screening for associated intracranial abnormalities - skull base/cerebral, vascular, and facial. It hypothesizes neurocristopathy as an underlying cause of MGDA and its associations. Caliber abnormalities of the ipsilateral optic nerve and chiasm are a frequent finding in MGDA. Optic pathway enlargement should not be labeled "glioma". (239/250).

Ocular manifestations of congenital anomalies of the kidney and urinary tract (CAKUT).

Congenital anomalies of the kidney and urinary tract (CAKUT) are among the most common birth defects worldwide and a major cause of kidney failure in children. Extra-renal manifestations are also common. This study reviewed diseases associated with the Genomics England CAKUT-associated gene panel for ocular anomalies. In addition, each gene was examined for expression in the human retina and an ocular phenotype in mouse models using the Human Protein Atlas and Mouse Genome Informatics databases, respectively. Thirty-four (54%) of the 63 CAKUT-associated genes (55 'green' and 8 'amber') had a reported ocular phenotype. Five of the 6 most common CAKUT-associated genes (PAX2, EYA1, SALL1, GATA3, PBX1) that represent 30% of all diagnoses had ocular features. The ocular abnormalities found with most CAKUT-associated genes and with five of the six commonest were coloboma, microphthalmia, optic disc anomalies, refraction errors (astigmatism, myopia, and hypermetropia), and cataract. Seven of the CAKUT-associated genes studied (11%) had no reported ocular features but were expressed in the human retina or had an ocular phenotype in a mouse model, which suggested further possibly-unrecognised abnormalities. About one third of CAKUT-associated genes (18, 29%) had no ocular associations and were not expressed in the retina, and the corresponding mouse models had no ocular phenotype. Ocular abnormalities in individuals with CAKUT suggest a genetic basis for the disease and sometimes indicate the affected gene. Individuals with CAKUT often have ocular abnormalities and may require an ophthalmic review, monitoring, and treatment to preserve vision.

Relationship between the structure and microcirculation of the optic disc region and myopic traction maculopathy in highly myopic eyes.

PURPOSE: To explore the characteristics and influencing factors structural and microcirculatory of optic disc and peripapillary tissue in eyes with myopia traction maculopathy (MTM). METHODS: There were 100 eyes from 77 patients in this study. We used 1:1 matching axial length in myopic eyes. Patients were divided into two groups according to the presence or absence of MTM. Fundus structure parameters were obtained by swept source optical coherence tomography (SS-OCT), and the optic disc microcirculation parameters were obtained by OCT angiography (OCTA). RESULTS: MTM group were older (P = 0.001) and had poorer Best-corrected Visual Acuity (BCVA) (P = 0.011), the optic disc-fovea distance (DFD) was longer (P < 0.019), optic disc tilt was greater (P < 0.001), area of peripapillary atrophy (PPA) was larger (P < 0.001), and PPA/optical disc area (ONH) was higher (P < 0.001). The peripapillary scleral thickness (PST) was lower in the MTM group (P < 0.001). The mean peripapillary choroidal thickness (PCT) (P < 0.001) and PCT in the 10 orientations were significantly lower in the MTM group than in the NMTM group (all P < 0.01). Vascular density in the nasosuperior (NS) region of the optic disc was significantly lower in the MTM group (P = 0.037). The generalized estimating equation suggested that PPA area (P = 0.028), mean PCT (P = 0.008), superior PCT (P = 0.027), inferonasal PCT (P = 0.040), temporoinferior PCT (P = 0.013), and PST (P = 0.046) correlated with MTM. Age, axial length, optic disc tilt, PPA area, mean PCT, and optic disc central zone (0-2 mm) vascular density (all P < 0.05) were significantly correlated with PST. CONCLUSIONS: The enlarged PPA area and thinner PCT and PST in eyes with MTM are more significant. Lower PST in high myopia was related to abnormalities of PCT and microcirculation. TRIAL REGISTRATION: Clinical Trial Registration number: ChiCTR2100046590.

Peripapillary RNFL cross-sectional area and its association with other parameters in a Chinese population.

BACKGROUND: Quantitative analysis of retinal nerve fibers is important for the diagnosis and treatment of optic nerve diseases. Peripapillary retinal nerve fiber layer (RNFL) cross-sectional area may give a more accurate quantitative assessment of retinal nerve fibers than RNFL thickness but there have been no previous reports of the peripapillary RNFL cross-sectional area or other parameters. The purpose of the current study was to determine peripapillary RNFL cross-sectional area and its association with other factors in an adult Chinese population. METHODS: RNFL cross-sectional area was measured during peripapillary circular optical coherence tomography (OCT) scan with a diameter of 12° centered on the optic disc. Correlation between RNFL cross-sectional area and other parameters was evaluated by linear regression analysis in a cross-sectional study of an adult Chinese population. RESULTS: A total of 2404 eyes from 2404 subjects were examined. Multivariate linear regression analysis showed that larger RNFL cross-sectional area correlated with younger age (p < 0.001), female gender (p = 0.001), no history of diabetes (p = 0.012) and larger optic disc area (p < 0.001). CONCLUSIONS: Peripapillary RNFL cross-sectional area is correlated positively with optic disc area, suggesting that eyes with larger optic discs have thicker RNFL. Further studies are needed to confirm whether this correlation is due to differences in the numbers of retinal nerve fibers or other factors.

Microcephaly with or without chorioretinopathy, lymphedema, or mental retardation (MCLMR)- the new lacunae: a case report.

BACKGROUND: Microcephaly with or without chorioretinopathy, lymphedema, or mental retardation is a rare autosomal dominant disease caused by mutations in KIF11 which disrupt EG5 protein function, impacting the development and maintenance of retinal and lymphatic structures due to its expression in the retinal photoreceptor cilia. The primary ocular finding in MCLMR is chorioretinopathy. Additional features can include microphthalmia, angle-closure glaucoma, persistent hyperplastic primary vitreous, cataract, pseudo-coloboma, persistent hyaloid artery, and myopic or hypermetropic astigmatism. The appearance of the chorioretinal lesions as white to pinkish, round, non-elevated atrophic areas devoid of blood vessels resembles the lacunae in Aicardy syndrome. Due to the lack of systematic description of the lesions and significant phenotypical variability, there is an impending need for a detailed report of each case. CASE PRESENTATION: A child with microcephaly detected in the third trimester of gestation began her following in the ophthalmology department due to a non-visually significant cataract. Shortly after, she developed nystagmus and large-angle alternating esotropia with cross-fixation. Her fundus initially showed a pallid optic disc and pigmentary changes, developing thereafter retinal lacunae and a retinal fold. Her differential diagnosis accompanied the dynamic changes in her fundus, which included congenital infections, Leber´s Congenital Amaurosis and Aicardy syndrome. At 19 months old, genetic testing identified a heterozygous mutation (c.1159 C > T, p.Arg387*) in the KIF11 gene. The patient underwent bilateral medial rectus muscle recession surgery at 2 years old for persistent esotropia, with significant improvement. Refraction revealed a hyperopic astigmatism in both eyes (+ 0.25 -2.50 × 180 OD and + 0.75 -2.00 × 170 OS). She continues to require right eye patching for 2 hours daily. CONCLUSIONS: This case report expands the phenotypic spectrum of MCLMR by demonstrating a unique combination of retinal features which sheds new light on differential diagnosis from Aicardy syndrome. Our findings emphasize the significant phenotypic variability associated with MCLMR, particularly regarding ocular involvement. This underscores the importance of detailed clinical evaluation and comprehensive reporting of cases to improve our understanding of the disease spectrum and genotype-phenotype correlations.

Self-supervised pre-training for joint optic disc and cup segmentation via attention-aware network.

Image segmentation is a fundamental task in deep learning, which is able to analyse the essence of the images for further development. However, for the supervised learning segmentation method, collecting pixel-level labels is very time-consuming and labour-intensive. In the medical image processing area for optic disc and cup segmentation, we consider there are two challenging problems that remain unsolved. One is how to design an efficient network to capture the global field of the medical image and execute fast in real applications. The other is how to train the deep segmentation network using a few training data due to some medical privacy issues. In this paper, to conquer such issues, we first design a novel attention-aware segmentation model equipped with the multi-scale attention module in the pyramid structure-like encoder-decoder network, which can efficiently learn the global semantics and the long-range dependencies of the input images. Furthermore, we also inject the prior knowledge that the optic cup lies inside the optic disc by a novel loss function. Then, we propose a self-supervised contrastive learning method for optic disc and cup segmentation. The unsupervised feature representation is learned by matching an encoded query to a dictionary of encoded keys using a contrastive technique. Finetuning the pre-trained model using the proposed loss function can help achieve good performance for the task. To validate the effectiveness of the proposed method, extensive systemic evaluations on different public challenging optic disc and cup benchmarks, including DRISHTI-GS and REFUGE datasets demonstrate the superiority of the proposed method, which can achieve new state-of-the-art performance approaching 0.9801 and 0.9087 F1 score respectively while gaining 0.9657 D C disc and 0.8976 D C cup . The code will be made publicly available.

Retinal nerve fiber layer thickness and radial peripapillary capillaries density in myopic adults with optical coherence tomography angiography.

PURPOSE: To evaluate retinal nerve fiber layer thickness (RNFLT) and radial peripapillary capillaries (RPC) density in adults with different degrees of myopia using optical coherence tomography angiography (OCTA) and explore their relationship with ocular factors, such as axial length (AL) and disc area. METHODS: A total of 188 subjects were included in this cross-sectional study. The eyes were divided into four groups according to AL. OCTA was used for the assessment of RNFLT, RPC density, and other optic disc measurements, such as disc area. One-way analysis of variance was performed to compare differences between four groups, and P value < 0.01 was considered significant. RESULTS: The RNFLT was significantly thinner in high myopia (HM) group at inferior nasal (IN) quadrant (P = 0.004) than low myopia (LM) group, but thicker at temporal inferior (TI) quadrant (P = 0.006). The RPC density of nasal superior (NS) quadrant, nasal inferior (NI) quadrant, and inferior nasal (IN) quadrant significantly decreased as AL increasing. By simple linear regression analysis, the inside disc RPC (iRPC) density tended to be correlated significantly with AL (0.3997%/mm, P < 0.0001). Peripapillary RPC (pRPC) density was in significant correlation with AL (-0.2791%/mm, P = 0.0045), and peripapillary RNFLT (pRNFLT) was in significant correlation with disc area (0.2774%/mm2, P = 0.0001). CONCLUSION: RNFLT and RPC density were closely associated with AL and disc area. They might be new indexes in assessing and detecting myopia development via OCTA.

Corneal stromal lenticule transplantation for the treatment of congenital optic disc pit maculopathy : a case report and review.

BACKGROUND: Congenital optic disc pit (ODP) is a relatively uncommon congenital anomaly of the optic disc, which seriously affects the patient's vision when combined with optic disc pit maculopathy(ODP-M). Currently, the treatment of ODP-M remains a clinical challenge and a focus of research. CASE PRESENTATION: A boy had a pit in the inferotemporal segment of the optic disc with ODP-M. Optical Coherence Tomography(OCT) showed ODP and serous retinal detachment. He was treated with pars plana vitrectomy(PPV), followed by Corneal Stromal Lenticule (CSL) sealing and C3F8 tamponade. In the end, significant anatomical improvement was achieved, and the Best Corrected Visual Acuity(BCVA) was improved. CONCLUSIONS: The CSL transplantation may be a viable therapeutic option for improving ODP-M with stable anatomical and functional result. However, more cases and longer follow-up are needed to confirm the safety and effectiveness of the technology.

Intrapapillary hemorrhage with adjacent peripapillary subretinal hemorrhage of both eyes after COVID-19 infection: a case report.

BACKGROUND: Intrapapillary hemorrhage with adjacent peripapillary subretinal hemorrhage is commonly observed in myopia with tilted optic disc. It presents with typical features on the fundus and follows a self-limiting course. However, due to its complex etiology, clinicians sometimes lack sufficient understanding of it which can easily lead to misdiagnosis or overtreatment. In this case report, we describe a rare case of intrapapillary hemorrhage with adjacent peripapillary subretinal hemorrhage in both eyes. CASE PRESENTATION: An 18-year-old female who has no past medical history experienced sudden black shadow blocking of her right eye in the right eye for the past 2 days after a 5-day history of COVID-19 infection. The best corrected visual acuity is 0.5 in the right eye and 0.6 in the left eye. Optical coherence tomography (OCT) showed tilted optic discs in both eyes, bulged nasal optic discs, and the presence of strong reflective material under the parafoveal retina of the optic discs. Fundus fluorescein angiography (FFA) showed subretinal fluorescence occlusion above and nasolateral to the optic disc in the right eye, with hypofluorescence below the optic disc; the subretinal below the optic disc was obscured by vitreous hemorrhage; hypofluorescence was seen in the optic disc region of the left eye.COVID-19 antigen was positive. The patient was in the early stage of the third COVID-19 infection when the disease occurred. We speculate that it may be related to it. After 5 months of conservative treatment, the patient's hemorrhage disappeared in both eyes and her best corrected visual acuity returned to normal. CONCLUSIONS: Intrapapillary hemorrhage with adjacent peripapillary subretinal hemorrhage usually occurs in myopia with tilted optic disc. In most patients, the cause of the bleeding is unknown, but it can gradually resolve under clinical observation or conservative treatment.

Papilloedema: a highly specific predictor of raised intracranial pressure in a complex neurosurgical paediatric cohort.

PURPOSE: Papilloedema is recognised as an indicator of raised intracranial pressure, although there is a paucity of literature describing the utility of fundoscopy in screening for raised ICP in children with craniofacial synostosis, particularly young children. We sought to investigate the association of optic disc morphology with ICP in children, and to define the sensitivity and specificity of papilloedema as a clinical indicator of raised ICP and determine if age, or underlying conditions impact the findings. METHOD: Retrospective analysis of all patients undergoing ICP monitoring at a designated paediatric neurosurgical and craniofacial unit in the United Kingdom between October 2009 and October 2018. The fundoscopy findings and ICP monitoring data were analysed for 31 children with craniosynostosis and 29 children without craniosynostosis. RESULTS: All children who had papilloedema had raised ICP confirmed with monitoring. Across the 60-patient cohort, confirmed papilloedema on fundoscopy had Positive Predictive Value (PPV) of 1.00, Negative Predictive Value (NPV) of 0.64 with sensitivity 48% and specificity 100% for the presence of raised ICP (p = < 0.0001). In the craniosynostosis group, PPV was 1.00, NPV was 0.39, sensitivity 48% and specificity 100% (p = < 0.03). There is no correlation between severity of optic disc swelling using Frisen grading and elevation of ICP. Age did not affect the presence of papilloedema in those with raised ICP. CONCLUSION: The presence of papilloedema is a strong indicator of raised ICP in a child, regardless of underlying aetiology. Detailed fundoscopy can prevent the need for further investigations including imaging-related radiation and invasive CSF pressure monitoring.

Transorbital point-of-care ultrasound versus fundoscopic papilledema to support treatment indication for potentially elevated intracranial pressure in children.

PURPOSE: To compare transorbital point-of-care ultrasound techniques -optic nerve sheath diameter (US-ONSD) and optic disc elevation (US-ODE)- with fundoscopic papilledema to detect potentially raised intracranial pressure (ICP) with treatment indication in children. METHODS: In a prospective study, 72 symptomatic children were included, 50 with later proven disease associated with raised ICP (e.g. pseudotumour cerebri, brain tumour, hydrocephalus) and 22 with pathology excluded. Bilateral US-ONSD and US-ODE were quantified by US using a 12-MHz-linear-array transducer. This was compared to fundoscopic optic disc findings (existence of papilledema) and, in 28 cases, invasively measured ICP values. RESULTS: The sensitivity and specificity of a cut-off value of US-ONSD (5.73 mm) to detect treatment indication for diseases associated with increased ICP was 92% and 86.4%, respectively, compared to US-ODE (0.43 mm) with sensitivity: 72%, specificity: 77.3%. Fundoscopic papilledema had a sensitivity of 46% and a specificity of 100% in this context. Repeatability and observer-reliability of US-ODE examination was eminent (Cronbach's α = 0.978-0.989). Papilledema was detected fundoscopically only when US-ODE was > 0.67 mm; a US-ODE > 0.43 mm had a positive predictive value of 90% for potentially increased ICP. CONCLUSION: In our cohort, transorbital point-of-care US-ONSD and US-ODE detected potentially elevated ICP requiring treatment in children more reliably than fundoscopy. US-ONSD and US-ODE indicated the decrease in ICP after treatment earlier and more reliably than fundoscopy. The established cut-off values for US-ONSD and US-ODE and a newly developed US-based grading of ODE can be used as an ideal first-line screening tool to detect or exclude conditions with potentially elevated ICP in children.

Can optic disc vessel density help in cases of residual disc elevation after shunt surgery in cases of idiopathic intracranial hypertension?

AIM: To detect if we can use the reduction in the optic disc vessel density as an indicator to the reduction in intracranial tension in patients with residual optic disc elevation after shunt surgery as fundus examination in those cases is not conclusive. PATIENTS AND METHOD: 21 patients with papilledema due to idiopathic intracranial hypertension underwent shunt surgery. Full neurological and ophthalmological assessments were done. The optic disc vessel density was measured before and 3 months after surgery. Patients were then divided according to the resolution of papilledema into 2 groups: 1) Residual disc elevation group. 2) Completely resolved disc edema group. CSF pressure was measured via lumber puncture preoperative for all patients and 3 months post-operative only for patients with residual disc edema. A comparison between both groups was done. RESULTS: There was a highly statistically significant difference between the two groups as regard the papilledema grade (the residual disc elevation group had a higher grade of papilledema) with P-value=0.000. As regard the difference in the preoperative optic disc vessel density between the two groups, there were statistically significant differences (optic disc vessel density was more in the residual disc elevation group). As regard the postoperative optic disc vessel density, there were non-significant differences between the two groups in whole image, inside disc and peripapillary vessel density (either in macro or microvasculature). CONCLUSION: The optic disc vessel density decreased with normal postoperative CSF opening pressure in cases with residual disc elevation postoperatively. Thus, in cases of residual optic disc swelling after shunt surgery, we can detect the reduction of intracranial pressure by the reduction in the optic disc vessel density which is a safe non-invasive technique. That may help in cases of residual disc elevation.

Epidemiology of optic disc edema in 2021/2022: Results from a cohort of 197 patients.

OBJECTIVE: The aim of our study was to determine the etiologies of optic disc edema between 2021 and 2022. MATERIALS AND METHODS: This was a multicentric study at the Timone and Nord university hospitals in Marseille. Patients were retrospectively followed in ophthalmology departments, with inclusion between January 2021 and December 2022. All patients presenting with newly diagnosed uni- or bilateral optic disc edema, both adults and children, were included. Their ophthalmological evaluation included a fundus examination and optical coherence tomography if feasible. RESULTS: In total, 197 patients were included. Intracranial hypertension (IH) was the most frequent etiology (37.06%). The primary causes of IH were idiopathic (27/73), intracranial tumors (21/73), and cerebral venous thrombosis (12/73). The second etiology of optic disc edema was retinal vein occlusion in 19.9% of cases (39/197). Edema reactive to uveitis was found in 13.2% of cases (26/197). Finally, inflammatory (17/197) and ischemic (30/197) optic neuropathies were identified. CONCLUSION: This study updates the most frequent etiologies of optic disc edema in 2021 and 2022 to facilitate diagnostic hypotheses for de novo optic disc edema. It highlights the importance of a comprehensive and personalized evaluation in diagnosing optic disc edema, taking into account recent advances in imaging techniques and biomarkers.

[Pulmonary and testicular sarcoidosis revealed by optic disc edema]. / Cas clinique. Sarcoïdose pulmonaire et testiculaire révélée par un oedème de la tête du nerf optique.

Sarcoidosis is a chronic inflammatory granulomatous disease of unknown aetiology. Although the lungs and thoracic lymph nodes are the most frequently affected structures, every tissue can be affected. Ophthalmological involvement occurs in 25 % of cases. We report a clinical case in which the diagnosis of sarcoidosis was revealed by isolated unilateral optic disc edema.

La sarcoïdose est une pathologie inflammatoire granulomateuse chronique dont l'étiologie reste inconnue. Bien que les poumons et les ganglions thoraciques soient les structures les plus fréquemment atteintes, tous les tissus peuvent être affectés. L'atteinte ophtalmologique est présente dans 25 % des cas. Nous rapportons un cas clinique dont le diagnostic de sarcoïdose fut révélé par un œdème papillaire unilatéral isolé.

Diagnostic dilemma of papilledema and pseudopapilledema.

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.

True or pseudo optic disc edema: clinically-based approach to the differential diagnosis.

PURPOSE: To present a clinically based approach to the differentiation of optic disc edema (DE) cases, commonly seen in neuro-ophthalmology. METHODS: Consecutive patients who were considered to have unilateral or bilateral DE during examinations in the outpatient clinic and were referred to the neuro-ophthalmology department were included in this prospective study. The examination findings and differential diagnosis based on clinical signs and symptoms, and neuro-ophthalmological approach were evaluated in cases of DE. RESULTS: Of the 119 cases with DE, 69 (58%) were women and 50 (42%) were men, where 89 (75%) had true optic DE (ODE) and 30 (25%) had pseudo optic DE  (PODE). Non-arteritic anterior ischemic optic neuropathy (n = 40), increased intracranial pressure (n = 32), and anterior optic neuritis (n = 17) were determined as the causes of true ODE, whereas small and crowded optic disc (n = 12), tilted optic disc (n = 8), myelinated nerve fibers (n = 5) and optic disc drusen (n = 5) as the causes of PODE. Patients with optic neuritis were the youngest (28.41 years) group of ODE cases while those with non-arteritic anterior ischemic optic neuropathy were the oldest (59.98 years). The first symptoms were sudden and painless loss of vision and/or visual field in cases with non-arteritic anterior ischemic optic neuropathy, pain increasing with eye movements and loss of vision and/or visual field in cases with optic neuritis, headache, and from time to time blurred vision in cases with increased intracranial pressure. Patients having vision loss due to amblyopia constituted (30%) of PODE cases while 70% were determined incidentally and they had the best visual acuity. The accuracy of the preliminary diagnosis based on neuro-ophthalmologic examination findings was 79% in all cases. CONCLUSION: Detailed history taking and neuro-ophthalmological examination are essential in the differential diagnosis of ODE and PODE.

Investigation of optic nerve function in Behçet's patients with optic disc hyperfluorescence in fundus fluorescein angiography.

PURPOSE: The aim was to investigate the changes in optic nerve function that may help in the diagnosis of subclinical optic nerve involvement in patients with Behçet's disease (BD) and isolated optic disc (OD) hyperfluorescence in fluorescein angiography (FA). MATERIALS AND METHODS: Three groups were formed; BD patients with isolated OD hyperfluorescence in FA, BD patients without ocular involvement (normal FA) and control group. A total of 88 eyes of 45 patients were included. The groups were compared in terms of OCT-RNFL, contrast sensitivity and VEP latency. RESULTS: When the OCT-RNFL values were compared, there was a significant difference between the control group and Behçet's patients with normal FA. Contrast sensitivity values differed significantly among the groups, and the lowest mean contrast sensitivity was observed in the group with OD hyperfluorescence (p < 0.05). CONCLUSION: As far as we know, this is the first publication that investigates optic nerve function in BD patients with isolated OD hyperfluorescence in FA. Assessment with FA of asymptomatic BD patients with visual complaints and low contrast sensitivity may be helpful at early detection of inflammatory optic neuropathy by close follow-up in patients with OD hyperfluorescence.