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Background: The Onyx™ Liquid Embolic System is a non-adhesive liquid embolic agent, which has been proved by the US FDA for embolization of lesions in the peripheral and neurovasculature since 2005. We reported a case of ischemic optic neuropathy after using Onyx-18 to embolize the anterior ethmoid arteries that feeding dural arteriovenous fistulas (DAVF)., Case Presentation: A 57-year-old Asian male presented with anterior cranial fossa DAVF underwent embolotherapy by delivering Onyx-18 through a microcatheter into the anterior ethmoid arteries under angiography guidance. The interventional procedure was successful and no clear evidence was found pointing to untargeted occlusive embolus. But after the surgery the patient experienced delayed painless vision loss in the right eye (RE). The fundoscopy showed unilateral papilledema with pale optic disc in RE, accompanied by significant edema and thickening in the retinal nerve fiber layer (RNFL) of macula. The fundus fluorescence angiography showed that most of the optic disc in RE had postponed or absent fluorescence filling. Visual evoked potential (VEP) confirmed that the amplitude of the P100 component was decreased in RE without significant prolongation of the latency. The patient was diagnosed with anterior ischemic optic neuropathy, but immediate pulse steroid therapy failed to rescue his vision., Conclusion: Preoperative evaluation of the patient's hemodynamic status and fundus examination are essential for assessing the risk of ischemic ocular complications, and the non-adhesive liquid embolic agent Onyx-18 should be used cautiously during endovascular embolization of intracranial artery., (© 2024. The Author(s).)

Purpose: The evaluation and management of Nonarteritic Anterior Ischemic Optic Neuropathy (NAION) lacks standardized guidelines. This study aimed to investigate the real-world practices of neuro-ophthalmologists in the evaluation and management of typical NAION cases., Methods: A national survey, conducted between 2019 and 2021, involved all practicing neuro-ophthalmologists. A structured questionnaire assessed their approach to risk factor evaluation and treatment of NAION, with 19 questions about risk factors and six questions concerning treatment and prevention of fellow-eye involvement., Results: Thirty-six out of 37 neuro-ophthalmologists participated. Most physicians referred patients for evaluation of the following risk factors: obstructive sleep apnea (83.3%), diabetes mellitus (83.3%), hypertension (77.7%), dyslipidemia (72.2%), and optic disc drusen (38.8%). However, there was considerable variation in the choice of diagnostic tests recommended. Furthermore, nearly 47% recommended an embolism workup. Regarding treatment, the majority (91%) did not recommend routine treatment for NAION, although in 16.7%, high-dose corticosteroids were occasionally prescribed. Secondary prevention with aspirin (80.6%), smoking cessation advice (86.1%), and advising against erectile dysfunction medications for men (80.6%) were common recommendations., Conclusion: While the risk factors associated with NAION are well-reported, there is a lack of uniformity on which tests should be ordered to evaluate these risk factors. Most neuro-ophthalmologists concur that routine treatment for NAION is not warranted, but not unanimously. Future studies to develop a consensus guideline for post-NAION work-up and management recommendations may assist in the detection and management of preventable risk factors., (© 2024. The Author(s).)

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., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Purpose: The objective of this study was to analyze the nycthemeral variations in blood pressure (BP) in individuals who presented with non-arteritic anterior ischemic optic neuropathy (NAION)., Methods: BP was recorded for 24 h (ambulatory blood pressure monitoring, ABPM) in 65 patients with acute NAION. Three definitions of nighttime periods were used: definition 1, 1 a.m.-6 a.m.; definition 2, 10 p.m.-7 a.m.; and definition 3, 10 p.m.-8 a.m. For each of these definitions, patients were classified according to the value of nocturnal reduction in BP into dippers (10-20%), mild dippers (0-10%), reverse dippers (< 0%), and extreme dippers (> 20%)., Results: The proportions of dippers, mild dippers, reverse dippers, and extreme dippers varied significantly depending on the definition chosen. We found the highest number of patients with extreme dipping (23%) when using the strictest definition of nighttime period (definition 1, 1 a.m.-6 a.m.), as compared with 6.2% and 1.5% for the other definitions, respectively. Overall, 13 of 33 patients without known systemic hypertension (39%) were diagnosed with hypertension after ABPM. No risk factor for NAION was associated with the extreme-dipping profile. Finally, the prevalence of systemic hypertension was high (69%)., Conclusion: In our population of patients who had an episode of NAION, the proportion of extreme dippers was higher than that usually found in the literature. However, extreme dipping is not a frequent feature of patients with NAION as compared to patients with systemic hypertension. ABPM is recommended for all patients with NAION and unknown history of systemic hypertension., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: This study aims to determine the population-based incidence and characterize the features of nonarteritic anterior ischemic optic neuropathy (NAION) using the Rochester Epidemiology Project (REP)., Methods: All patients diagnosed with an optic neuropathy from January 1, 1990, to December 31, 2016, were retrospectively reviewed to identify incident cases of NAION using the REP database, which is a record-linkage system of medical records for all patient-physician encounters among Olmsted County, Minnesota residents. The overall incidence of NAION was estimated using the age-specific and sex-specific population figures for Olmsted County census data for 1990 through 2016. Visual outcomes and risk factors were evaluated. The systemic risk factors were compared with age-matched controls., Results: One hundred four patients were diagnosed with NAION during the 26-year study period. The overall age and sex adjusted incidence was 3.89 per 100,000 individuals (95% confidence interval [CI]: 3.14-4.65). The incidence was 7.73 (CI: 6.24-9.22) in patients aged 40 years or older and 10.19 (CI: 8.15-12.23) in patients aged 50 years or older. Median age at diagnosis was 65 years (range, 40-90 years), and 59 (56.7%) were male. The median logMAR visual acuity at presentation was 0.35 (Snellen equivalent of 20/40) with 14 patients (13.5%) having vision of counting fingers or worse. Among the 91 patients with final visual acuity outcome data available, the median visual acuity was 0.40 (Snellen equivalent of 20/50) with 12 patients (13.2%) having vision of counting fingers or worse. Twenty-four patients (26.4%) were noted to have final acuity at least 3 Snellen lines worse than at presentation, whereas 17 patients (18.7%) were noted to improve by at least 3 lines. The median mean deviation on automated visual field testing was -10.2 dB at presentation and -11.1 dB at follow-up. Twenty-two patients (21.2%) suffered NAION in the fellow eye; the median interval between involvement of the first and second eyes was 1.39 years. Systemic diseases present in the NAION cohort included hypertension (79.8%), diabetes mellitus (39.4%), obstructive sleep apnea (23.1%), and hyperlipidemia (74.0%), which were all statistically higher than age-matched controls., Conclusions: NAION is a relatively common optic neuropathy in elderly patients with vascular risk factors. Our data indicate that the incidence of NAION has remained relatively stable in the population of Olmsted County over the past 4 decades., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 by North American Neuro-Ophthalmology Society.)

Background: Administrative claims have been used to study the incidence and outcomes of nonarteritic ischemic optic neuropathy (NAION), but the validity of International Classification of Diseases (ICD)-10 codes for identifying NAION has not been examined., Methods: We identified patients at 3 academic centers who received ≥1 ICD-10 code for NAION in 2018. We abstracted the final diagnosis from clinical documentation and recorded the number of visits with an NAION diagnosis code. We calculated positive predictive value (PPV) for the overall sample and stratified by subspecialty and the number of diagnosis codes. For patients with ophthalmology or neuro-ophthalmology visit data, we recorded presenting symptoms, examination findings, and laboratory data and calculated PPV relative to case definitions of NAION that incorporated sudden onset of symptoms, optic disc edema, afferent pupillary defect, and other characteristics., Results: Among 161 patients, PPV for ≥1 ICD-10 code was 74.5% (95% CI: 67.2%-80.7%). PPV was similar when restricted to patients who had visited an ophthalmologist (75.8%, 95% CI: 68.4%-82.0%) but increased to 86.8% when restricted to those who had visited neuro-ophthalmologists (95% CI: 79.2%-91.9%). Of 113 patients with >1 ICD-10 code and complete examination data, 37 (32.7%) had documented sudden onset, optic disc swelling, and an afferent pupillary defect (95% CI: 24.7%-42.0%). Of the 76 patients who did not meet these criteria, 54 (71.0%) still received a final clinical diagnosis of NAION; for most (41/54, 75.9%), this discrepancy was due to lack of documented optic disc edema., Conclusions: The validity of ICD-10 codes for NAION in administrative claims data is high, particularly when combined with provider specialty., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 by North American Neuro-Ophthalmology Society.)

Purpose: To report a case of progressive ischemic retinopathy and optic neuropathy in a patient with heavy chain deposition disease (HCDD), a rare form of monoclonal immunoglobulin deposition disease (MIDD)., Observations: Our case describes a 74-year-old woman diagnosed with IgG1 lambda HCDD. After treatment with daratumumab and intravenous IVIG therapy, the patient developed worsening ischemic retinopathy and optic neuropathy, neovascular glaucoma, and bilateral sequential vitreous hemorrhages, necessitating surgical intervention. We present multimodal imaging from the onset of ischemic retinopathy to end-stage maculopathy illustrated by optical coherence tomography (OCT) angiography. Despite discontinuing treatment with daratumumab and providing maximal ocular interventions to control the complications of neovascular disease, the patient's condition progressed, resulting in profound vision loss., Conclusions and Importance: Our case illustrates the potential for HCDD to cause end-organ disease, including ischemic retinopathy and optic neuropathy, possibly worsened by the patient's underlying cardiovascular risk factor status and medications. Daratumumab, a humanized IgG1 kappa monoclonal antibody that binds to CD38 used to treat specific blood cancers, has been reported to cause disturbances in retinal blood flow, including retinal artery and vein occlusions. It remains to be determined whether careful patient selection or dose adjustments and timing of HCDD treatments could protect vision by reducing the risk of these rare yet severe ocular complications., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Competing Interests: The authors report no conflicts of interest.

A nearly 3-year-old boy on nightly dialysis presented emergently with sudden loss of vision. On examination, his visual acuity was light perception in the right eye and no light perception in the left eye. There was bilateral optic disk edema, diffuse pallor of posterior poles, and a cherry red spot in the left fundus. The patient was subsequently found to be hemodynamically unstable and admitted to the pediatric intensive care unit with presumed septic shock. Optical coherence tomography revealed paracentral acute middle maculopathy lesions in the right eye and diffusely thick retina in the left eye. Magnetic resonance imaging and magnetic resonance angiography of the brain and vessels did not reveal any acute findings. The patient's presentation was most consistent with bilateral nonarteritic ischemic optic neuropathy and unilateral central retinal artery occlusion. On repeat evaluation 9 months later, vision was largely unchanged., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Objective: To review treatment modalities that have been studied in acute non arteritic anterior ischemic optic neuropathy (NAION)., Methods: We performed a comprehensive literature search of English language publications in the last 5 years, with human species and NAION. Articles were reviewed to identify those that described original research on treatment of acute NAION. Study type, setting, duration, interventions, and results were extracted and articles were reviewed for biases and limitations., Results: We identified 22 kinds of treatment varying by compound and modality. These include topical, intravitreal, and systemic drugs as well as surgical approaches. Evidence for efficacy ranges from expert opinion to randomized control trials., Conclusions: Although several treatments are utilized in practice, none of these have high quality evidence of efficacy to improve visual outcomes.  Continued collaborative research is necessary to complete high quality studies in order identify effective therapies for this rare and blinding disease., (© 2024. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

A 71-year-old woman developed sudden, painful, decreased vision in the left eye accompanied by progressive instability. Initial examination revealed left optic disc edema, and macular optical coherence tomography confirmed the presence of intraretinal and subretinal fluid, as well as hyperreflective material under the retinal pigment epithelium. Subsequent investigations, including brain magnetic resonance imaging and a comprehensive serological analysis, ruled out infectious and autoimmune causes, further complicating the diagnostic picture. The patient's vision in both eyes continued to deteriorate, prompting empirical corticosteroid treatment. While the vision improved, the case took an unexpected turn with worsening neurological symptoms. Ultimately a brain biopsy was consistent with diffuse large B-cell lymphoma., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: To report a case of non-arteritic anterior ischemic optic neuropathy (NAION) in an elderly patient with ischemia of the left splenium of the corpus callosum, providing details of the diagnostic work-up and subsequent follow-up., Methods Section: Case report., Results: A pseudophakic 80 years-old woman referred complaining sudden visual impairment in the left eye (LE) in concomitance with episode of hypertensive crisis. Fundus examination showed diffuse swelling of optic disc associated with flame peripapillary hemorrhages in LE and small crowded disc in right eye (RE). A superior altitudinal defect with arcuate defect including the blind spot were detected at the visual field in the LE. The patient was diagnosed with NAION. Five days later the patient complained a further vision loss and a pathological area within the left splenium of corpus callosum, consistent ischemia, was depicted at magnetic resonance imaging of brain. Corpus callosum infarction was completely asymptomatic and neurological evaluation was normal. At 45 days follow-up fundus examination showed white ischemic nerve while visual field was irreversibly constricted with tubular defect in LE., Conclusion: In case of NAION linked with corpus callosum ischemia multimodal imaging and systemic work-up play a pivotal role for an early diagnosis., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: We describe the baseline ophthalmic and cardiovascular risk factors across countries, race, and sex for the Quark207 treatment trial for acute nonarteritic anterior ischemic optic neuropathy (NAION)., Design: Prospective, randomized controlled clinical trial., Participants: Adults 50 to 80 years of age with acute NAION recruited from 80 sites across 8 countries., Main Outcome Measures: Ophthalmic features of NAION and cardiovascular risk factors., Methods: We evaluated demographics and clinical and ophthalmologic data, including best-corrected visual acuity (BCVA) and average visual field total deviation (TD), in affected eyes and cup-to-disc ratio in fellow eyes at enrollment. We report the prevalence (mean and standard devition, and median and interquartile range [IQR]) of ophthalmic features and cardiovascular risk factors, stratified by country, race, and sex. We corrected for multiple comparisons using Dunn's test with Bonferroni correction for continuous variables and chi-square testing with Holm-Bonferroni correction for categorical variables., Results: The study enrolled 500 men and 229 women with a median age of 60 and 61 years (P = 0.027), respectively. Participants were predominantly White (n = 570) and Asian (n = 149). The study eye BCVA was 71 characters (IQR, 53-84 characters; approximately 0.4 logarithm of the minimum angle of resolution), and the TD was -16.5 dB (IQR, -22.2 to -12.6 dB) for stimulus III and -15.7 dB (IQR, -20.8 to -10.9 dB) for stimulus V. The vertical and horizontal cup-to-disc ratio was 0.1 (IQR, 0.1-0.3) for unaffected fellow eyes. The prevalence of cardiovascular risk factors varied among countries. The most notable differences were in the baseline comorbidities and ophthalmologic features, which differed between Asian and White races. Men and women differed with respect to a few clinically meaningful features., Conclusions: The cardiovascular risk factors in the NAION cohort varied among the 7 countries, race, and sex, but were not typically more prevalent than in the general population. Ophthalmic features, typical of NAION, generally were consistent across countries, race, and sex, except for worse BCVA and TD in China. Men have a frequency of NAION twice that of women. Having a small cup-to-disc ratio in the fellow eye was the most prevalent risk factor across all demographics. This study suggests that factors, not yet identified, may contribute to the development of NAION., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2024 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Background: Ischemic optic neuropathy (ION) is exceedingly rare in children on dialysis, resulting from poor perfusion of the optic nerve, and presents as sudden acute painless vision loss., Case-Diagnosis/treatment: We report the case of a 3-year-old male with stage 5 chronic kidney disease (CKD 5) due to focal segmental glomerulosclerosis (FSGS) status post-bilateral nephrectomy on chronic hemodialysis who had acute loss of vision several hours after a hemodialysis session. Earlier that day, he had a drop in blood pressure intra-dialysis to 89/67 mmHg, with at home blood pressures ranging 90/60 to 150/100 mmHg. The patient was treated with tight blood pressure control to maintain blood flow and prevent blood pressure lability, received high-dose corticosteroids with a corticosteroid taper, and placed on high-dose erythropoietin for neuroprotective effect. He regained partial vision beginning approximately 1 month after presentation., Conclusions: The exact cause of our patient's simultaneous bilateral anterior and posterior ION, confirmed via MRI and fundoscopic examination, is unclear; however, is likely secondary to a combination of fluctuating blood pressure, anemia, anephric status, and hemodialysis. This highlights the need for close blood pressure monitoring, management of anemia, and more diligent ophthalmologic screening in pediatric patients on chronic hemodialysis., (© 2024. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Background: There are few reports of histopathology of any form of optic neuropathy. This article provides histopathologic findings of an adult-onset, nonprogressive optic neuropathy that was diagnosed clinically as nonacute, nonarteritic anterior ischemic optic neuropathy (NAION) but which was found by a pathological study to be associated with diffuse calcium oxalosis that was confined in the involved orbit., Methods: This is a case report that includes results of a neuro-ophthalmologic examination and histopathology of a complete autopsy, including en bloc removal of both orbits and the brain. The unaffected orbit/optic nerve served as a control. The affected orbit was serially sectioned into 2,550 increments each separated by 10 μm; the uninvolved orbit was sectioned into 150 equally spaced sections. The main outcome measures were derived from the autopsy, especially from the thin-section histopathologic study of both orbits that focused on blood vessels and the site of neural damage within the optic nerve., Results: The neuro-ophthalmologic examination revealed a unilateral optic neuropathy with pallor of the left optic nerve head that had been documented just before death. The general autopsy showed acute bacterial endocarditis and a recent cerebral hematoma that caused death. Histopathology revealed sectoral loss of optic nerve axons in the left eye. Numerous arterial walls in the left orbit, including short posterior ciliary arteries and the central retinal artery, contained hundreds of crystals with anisotropic, colorful birefringence consistent with calcium oxalosis. Crystals were not found in the right, control orbit or elsewhere in the body., Conclusions: The patient developed an optic neuropathy late in life that was diagnosed by an experienced neuro-ophthalmologist as being most consistent with nonacute, nonarteritic anterior ischemic optic neuropathy. The autopsy identified sectoral loss of optic nerve fibers consistent with that diagnosis. However, the unexpected discovery of calcium oxalate crystals in blood vessels of the involved orbit, which curiously were not present elsewhere in the body, raises a question of their etiological role in this particular optic neuropathy. Whether the crystals were causal, epiphenomenal, or purely incidental to the optic neuropathy cannot be answered by our study., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by North American Neuro-Ophthalmology Society.)

Background and Objectives: Myelin oligodendrocyte glycoprotein antibody-associated disease optic neuritis (MOGAD-ON) and nonarteritic anterior ischemic optic neuropathy (NAION) can cause acute optic neuropathy in older adults but have different managements. We aimed to determine differentiating factors between MOGAD-ON and NAION and the frequency of serum MOG-IgG false positivity among patients with NAION., Methods: In this international, multicenter, case-control study at tertiary neuro-ophthalmology centers, patients with MOGAD presenting with unilateral optic neuritis as their first attack at age 45 years or older and age-matched and sex-matched patients with NAION were included. Comorbidities, clinical presentations, acute optic disc findings, optical coherence tomography (OCT) findings, and outcomes were compared between MOGAD-ON and NAION. Multivariate analysis was performed to find statistically significant predictors of MOGAD-ON. A separate review of consecutive NAION patients seen at Mayo Clinic, Rochester, from 2018 to 2022, was conducted to estimate the frequency of false-positive MOG-IgG in this population., Results: Sixty-four patients with unilateral MOGAD-ON were compared with 64 patients with NAION. Among patients with MOGAD-ON, the median age at onset was 56 (interquartile range [IQR] 50-61) years, 70% were female, and 78% were White. Multivariate analysis showed that eye pain was strongly associated with MOGAD-ON (OR 32.905; 95% CI 2.299-473.181), while crowded optic disc (OR 0.033; 95% CI 0.002-0.492) and altitudinal visual field defect (OR 0.028; 95% CI 0.002-0.521) were strongly associated with NAION. On OCT, peripapillary retinal nerve fiber layer (pRNFL) thickness in unilateral MOGAD-ON was lower than in NAION (median 114 vs 201 μm, p < 0.001; median pRNFL thickening 25 vs 102 μm, p < 0.001). MOGAD-ON had more severe vision loss at nadir (median logMAR 1.0 vs 0.3, p < 0.001), but better recovery (median logMAR 0.1 vs 0.3, p = 0.002). In the cohort of consecutive NAION patients, 66/212 (31%) patients with NAION were tested for MOG-IgG and 8% (95% CI 1%-14%) of those had false-positive serum MOG-IgG at low titers., Discussion: Acute unilateral optic neuropathy with optic disc edema in older adults can be caused by either MOGAD-ON or NAION. Detailed history, the degree of pRNFL swelling on OCT, and visual outcomes can help differentiate the entities and prevent indiscriminate serum MOG-IgG testing in all patients with acute optic neuropathy.

Perioperative vision loss following non-ophthalmic surgical procedures represents a rare but potentially serious complication. Although its occurrence in urology is infrequent, the consequences for patients and legal implications are significant. We present the case of a 53-year-old woman with no notable medical history, treated for renal lithiasis. Following the ureteroscopy, the patient experienced a sudden reduction in visual acuity. The diagnosis made was that of posterior ischemic optic neuropathy. This case illustrates a rare complication associated with ureteroscopy, highlighting the importance of increased awareness and rigorous postoperative monitoring, especially in patients with risk factors such as anemia or hypertension. Fortunately, the patient's rapid and complete recovery is encouraging, suggesting that early identification and appropriate management can lead to a favorable prognosis., Competing Interests: The authors declare no competing interests., (Copyright: Wael Gazzah et al.)

Aim: To evaluate the role of papillary vitreous detachment in the pathogenesis of non-arteritic anterior ischaemic optic neuropathy (NAION) by comparing the features of vitreopapillary interface between NAION patients and normal individuals., Methods: This study included 22 acute NAION patients (25 eyes), 21 non-acute NAION patients (23 eyes) and 23 normal individuals (34 eyes). All study participants underwent swept-source optical coherence tomography to assess the vitreopapillary interface, peripapillary wrinkles and peripapillary superficial vessel protrusion. The statistical correlations between peripapillary superficial vessel protrusion measurements and NAION were analysed. Two NAION patients underwent standard pars plana vitrectomy., Results: Incomplete papillary vitreous detachment was noted in all acute NAION patients. The prevalence of peripapillary wrinkles was 68% (17/25), 30% (7/23) and 0% (0/34), and the prevalence of peripapillary superficial vessel protrusion was 44% (11/25), 91% (21/23) and 0% (0/34) in the acute, non-acute NAION and control groups, respectively. The prevalence of peripapillary superficial vessel protrusion was 88.9% in the eyes without retinal nerve fibre layer thinning. Furthermore, the number of peripapillary superficial vessel protrusions in the superior quadrant was significantly higher than that in the other quadrants in eyes with NAION, consistent with the more damaged visual field defect regions. Peripapillary wrinkles and visual field defects in two patients with NAION were significantly attenuated within 1 week and 1 month after the release of vitreous connections, respectively., Conclusion: Peripapillary wrinkles and superficial vessel protrusion may be signs of papillary vitreous detachment-related traction in NAION. Papillary vitreous detachment may play an important role in NAION pathogenesis., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: The author reports no conflicts of interest.

Competing Interests: The authors report no conflicts of interest.

Competing Interests: The authors report no conflicts of interest.

Competing Interests: The authors report no conflicts of interest.

Competing Interests: The authors report no conflicts of interest.

Competing Interests: A. Gupta, T. Tejpal, R. A. Sharma report no conflicts of interest; I. I. K. Ahmed—Consultant—Aequus, Aerie Pharmaceuticals, Akorn, Alcon, Allergan, Aquea Health, Inc, ArcScan, Bausch Health, Beaver Visitec, Beyeonics, Carl Zeiss Meditec, Centricity Vision, Inc, CorNeat Vision, Costum Surgical, ELT Sight, ElutiMed, Equinox, Genentech, Glaukos, Gore, Iantrek, InjectSense, Iridex, iStar, Ivantis, Johnson & Johnson Vision, LayerBio, Leica Microsystems, Long Bridge Medical, Inc, MicroOptx, MST Surgical, New World Medical, Ocular Instruments, Ocular Therapeutix, Oculo, Omega Ophthalmics, PolyActiva, Radiance Therapeutics, Inc, Ripple Therapeutics, Sanoculis, Santen, Shifamed, LLC, Sight Sciences, Smartlens, Inc, Stroma, Thea Pharma, ViaLase, Vizzario; Financial support—Aerie Pharmaceuticals, Alcon, Allergan, Glaukos, Ivantis, Johnson & Johnson Vision, New World Medical, Santen; Lecturer—Alcon, Allergan, Carl Zeiss Meditec, Johnson & Johnson Vision, MST Surgical, Mundipharma.

Competing Interests: The authors report no conflicts of interest.

Competing Interests: The authors report no conflicts of interest.

Background: To evaluate the classification performance of machine learning based on the 4 vessel density features of peripapillary optical coherence tomography angiography (OCT-A) for classifying healthy, nonarteritic anterior ischemic optic neuropathy (NAION), and optic neuritis (ON) eyes., Methods: Forty-five eyes of 45 NAION patients, 32 eyes of 32 ON patients, and 76 eyes of 76 healthy individuals with optic nerve head OCT-A were included. Four vessel density features of OCT-A images were developed using a threshold-based segmentation method and were integrated in 3 models of machine learning classifiers. Classification performances of support vector machine (SVM), random forest, and Gaussian Naive Bayes (GNB) models were evaluated with the area under the receiver-operating-characteristic curve (AUC) and accuracy., Results: We divided 121 images into a 70% training set and 30% test set. For ON-NAION classification, best results were achieved with 50% threshold, in which 3 classifiers (SVM, RF, and GNB) discriminated ON from NAION with an AUC of 1 and accuracy of 1. For ON-Normal classification, with 100% threshold, SVM and RF classifiers were able to discriminate normal from ON with AUCs of 1 and accuracies of 1. For NAION-normal classification, with 50% threshold, the SVM and RF classified the NAION from normal with AUC and accuracy of 1., Conclusions: ML based on the combined peripapillary vessel density features of total vessels and capillaries in the whole image and ring image could provide excellent performance for NAION and ON distinction., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by North American Neuro-Ophthalmology Society.)

Competing Interests: The authors report no conflicts of interest.

A 33-year-old woman developed hypertensive emergency (268/168 mmHg) with renal failure and hypertensive retinopathy. Four hours after the initiation of antihypertensive therapy with the continuous infusion of nicardipine, her blood pressure (BP) decreased to 168/84 mmHg; however, the patient developed blindness. She was diagnosed with posterior ischemic optic neuropathy (PION). Her BP was maintained at approximately 175/90 mmHg until her vision improved. Olmesartan was initiated on day 13, and her BP decreased to approximately 135/95 mmHg without the re-exacerbation of vision loss. Although the prognosis of PION is poor, its early diagnosis and gradual antihypertensive therapy may help preserve the patient's vision.

This study aimed to investigate the association between nonarteritic anterior ischemic optic neuropathy (NAION) and Parkinson's disease (PD) using a retrospective, nationwide, population-based cohort in South Korea. This study utilized data from the Korean National Health Insurance database, including 43,960 NAION patients and 219,800 age- and sex-matched controls. Cox proportional hazards regression models were used to assess the risk of developing PD in the NAION group compared to the control group after adjusting for various confounding factors. Subgroup analyses were conducted based on sex, age, and comorbidities. The incidence rate of PD was higher in the NAION group (1.326 per 1000 person-years) than in the control group (0.859 per 1000 person-years). After adjusting for confounding factors, the risk of developing PD was significantly higher in the NAION group (adjusted hazard ratio [aHR] 1.516, 95% confidence interval [CI] 1.300-1.769). Subgroup analyses did not reveal a significant difference in the risk of PD development based on sex, age, or comorbidities. This retrospective, nationwide, population-based cohort study revealed a significant association between NAION and an increased risk of developing PD in a South Korean population. The incidence rate of PD was observed to be higher in individuals diagnosed with NAION than in age- and sex-matched controls even after adjusting for potential confounding variables, with the risk being approximately 51.6% higher in the NAION group. Further research is necessary to elucidate the underlying pathophysiological mechanisms linking NAION to PD and to determine whether similar associations exist in other ethnic and geographical populations., (© 2024. The Author(s).)

Purpose: To offer a comprehensive review of the available data regarding non-arteritic anterior ischaemic optic neuropathy and its phenocopies, focusing on the current evidence to support the different existing aetiopathogenic hypotheses for the development of these conditions., Conclusions and Importance: Due to the limited array of responses of the neural tissue and other retinal structures, different aetiopathogenic mechanisms may result in a similar clinical picture. Moreover, when the insult occurs within a confined space, such as the optic nerve or the optic nerve head, in which different tissues (neural, glial, vascular) are highly interconnected and packed together, determining the primary noxa can be challenging and may lead to misdiagnosis. Anterior ischaemic optic neuropathy is a condition most clinicians will face during their everyday work, and it is important to correctly differentiate among resembling pathologies affecting the optic nerve to avoid unnecessary diagnostic procedures. Combining a good clinical history and multimodal imaging can assist diagnosis in most cases. The key remains to combine demographic data (e.g. age), with ophthalmic data (e.g. refractive error), systemic data (e.g. comorbidities and medication), imaging data (e.g. retinal OCT) with topographic signs (e.g. focal neurology)., Methodology: Papers relevant for this work were obtained from the MEDLINE and Embase databases by using the PubMed search engine. One author (MPMG) performed the search and selected only publications with relevant information about the aetiology, pathogenic mechanisms, risk factors as well as clinical characteristics of phenocopies (such as vitreopapillary traction, intrapapillary haemorrhage with adjacent peripapillary subretinal haemorrhage or diabetic papillopathy) of non-arteritic anterior ischaemic optic neuropathy (NAION). The terms "non-arteritic ischaemic optic neuropathy/NAION", "vitreopapillary traction", "vitreopapillary traction AND non-arteritic ischaemic optic neuropathy/NAION", "posterior vitreous detachment AND non-arteritic ischaemic optic neuropathy/NAION", "central retinal vein occlusion AND non-arteritic ischaemic optic neuropathy/NAION", "disc oedema/disc oedema", "diabetes mellitus AND non-arteritic ischaemic optic neuropathy/NAION" and "diabetic papillopathy" were searched on PubMed. From each of these searches, publications were selected based on their title, obtaining a total of 115 papers. All papers not written in English were then excluded, and those whose abstracts were not deemed relevant for our review, according to the aforementioned criteria. Subsequent scrutiny of the main text of the remaining publications led us (MPMG, AP, ZS) to include references which had not been selected during our first search, as their titles did not contain the previously mentioned MeSH terms, due to their significantly relevant contents for our work. A total of 62 publications were finally consulted for our review. The literature review was last updated on 24-Aug-2022., (© 2023. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Purpose: To determine the characteristics of peripapillary hyperreflective ovoid mass-like structures (PHOMS) in patients with nonarteritic anterior ischemic optic neuropathy (NAION) and in normal adults., Methods: A total of 406 included eyes were divided into four groups: acute NAION group, chronic NAION group, unaffected group, and normal eyes group. PHOMS were detected on optical coherence tomography slices from optical coherence tomography angiography scans centered on the optic nerve head (ONH). The differences in age, sex, and ONH parameters were investigated between eyes with PHOMS and eyes without PHOMS among groups., Results: The prevalence of PHOMS in acute eyes (43.48%) and fellow eyes (28.20%) was significantly higher than that in normal eyes (11.76%) (acute vs. normal, P < 0.001; fellow vs. normal, P = 0.014). In the acute group, the PHOMS score of size was negatively correlated with age in acute eyes (r = -0.486, P = 0.03). The size of PHOMS was negatively correlated with age and cup/disc ratio and positively correlated with retinal nerve fiber layer thickness in the nasal and inferior sectors in the normal groups. No differences in age, sex, ONH parameters, or visual field defects were found between eyes with PHOMS and eyes without PHOMS., Conclusions: The prevalence of PHOMS increased significantly in acute nonoptic disc drusen (NODD)-NAION eyes and fellow eyes. PHOMS could also be found among normal adults. PHOMS may be a nonspecific sign secondary to ONH edema and axoplasmic stasis., Translational Relevance: The high prevalence of PHOMS in acute NODD-NAION eyes may indicate axoplasmic stasis secondary to tissue edema.

Introduction: Anterior ischemic optic neuropathy (AION) can mimic glaucoma and consequently cause difficulties in differential diagnosis. The purpose of this paper was to summarize differences in diagnostic tests that can help perform a correct diagnosis., Methods: The search strategy was performed according to the PRISMA 2009 guidelines, and four databases were used: MEDLINE, Embase, Web of Science, and Cochrane. Totally, 772 references were eligible; 39 were included after screening with respect to inclusion criteria that included English language and published in the 20 years before search date., Results: Ninety percent (n = 35) of included studies used optical coherence tomography (OCT). Glaucomatous eyes had a significantly greater cup area, volume and depth, cup-to-disk ratio, a lower rim volume and area, and a thinner Bruch's membrane opening-minimum rim width. Retinal nerve fiber layer (RNFL) thinning in glaucomatous eyes occurred primarily at the superotemporal, inferotemporal, and inferonasal sectors, while AION eyes demonstrated mostly superonasal thinning. Glaucoma eyes showed greater macular ganglion cell layer thickness, except at the inferotemporal sector. OCT angiography measurements demonstrated a significant decrease in superficial and deep macular vessel density (VD) in glaucoma compared to AION with similar degree of visual field damage; the parapapillary choroidal VD was spared in AION eyes compared to glaucomatous eyes., Conclusion: By use of OCT imaging, optic nerve head parameters seem most informative to distinguish between glaucoma and AION. Although both diseases affect the RNFL thickness, it seems to do so in different sectors. Differences in structure and vascularity of the macula can also help in making the differential diagnosis., (© 2024 The Author(s). Published by S. Karger AG, Basel.)

Objective: To evaluate the accuracy of peripapillary optical coherence tomography angiography (OCTA) segmentation in eyes with acute nonarteritic anterior ischemic optic neuropathy (NAION) and healthy eyes., Method: In this retrospective study, en face OCTA images of the optic disc of healthy eyes and eyes with unilateral acute NAION were obtained. The disc boundary and radial peripapillary capillary (RPC) segmentation were generated automatically by the instrument software and then corrected by 2 expert investigators. The frequency of segmentation errors and its impact on vessel density and nerve fibre layer (NFL) thickness measurements were evaluated., Results: Thirty-eight eyes of 38 subjects (18 in the healthy group and 20 in the acute NAION group) were studied. A misidentified disc border was noted in 5 healthy eyes (27.7%) and 19 eyes with NAION (95.0%; p < 0.001). Segmentation error at the RPC level was found in 6 healthy eyes (33.33%) and 19 eyes with NAION (95.0%; p < 0.001). The nerve fibre layer thickness and RPC density did not change statistically significantly after error corrections in both groups., Conclusions: Misidentification of disc border and segmentation error of the RPC layer are common in OCTA images of the optic disc. Accuracy of OCTA imaging in disc boundary detection and RPC network segmentation is reduced in edematous optic discs following acute NAION., (Copyright © 2022 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Competing Interests: The authors report no conflicts of interest.