Papillary optical coherence tomography angiography in optic neuropathies.

AIM: to describe and analyze peripapillary microvascular changes in eyes with optic neuropathies using optical coherence tomography angiography (OCTA). METHODS: In this cross-sectional observational study, 63 eyes of 48 patients with different causes of optic neuropathy and 36 healthy control eyes were evaluated by optical coherence tomography (OCT) and OCTA (Topcon DRI OCT; Triton) between January 2018 and December 2018 at the Fattouma Bourguiba ophthalmology department in Monastir. RESULTS: Vascular changes were more frequent in acute non arteritic anterior ischemic optic neuropathy (NAION) than in optic neuritis. Eyes with optic atrophy had capillary rarefaction corresponding to the area of thinning of the peripapillary optic fiber layer (RNFL). The peripapillary vascular density (PVD) was lower (p=0.036) in case of optic atrophy compared to normal eyes. For non arteritic ischemic optic neuropathy and for chronic retrobulbar optic neuritis (NORB), a positive correlation between visual acuity and PVD was observed (p<0.05). An inversely proportional correlation between the PVD and the duration of evolution was noted. In the multivariate study, high blood pressure was a systemic factor correlated with peripapillary microvascular damages. CONCLUSION: OCTA showed a reduction in PVD and greater vascular changes in acute non-arteritic ischemic optic neuropathy than in edematous optic neuritis. At the atrophic stage, the PVD was reduced. Hypertension was an independent factor correlated with peripapillary microvascular damages.

Posterior short ciliary arteries ischemia following embolization of anterior ethmoid artery to treat dural arteriovenous fistulas: a case report.

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.

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.

Sudden transient blindness following ureteroscopy: an uncommon complication of a common procedure (a case report).

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.

Bilateral nonarteritic ischemic optic neuropathy and retinal ischemia in a pediatric dialysis patient.

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.

Not everything is ischemic optic neuropathy.

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.

Bilateral sequential ischemic retinopathy and optic neuropathy in IgG1 heavy chain deposition disease.

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.

Evaluation and management of nonarteritic anterior ischemic optic neuropathy: a national survey.

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.

Review of evidence for treatments of acute non arteritic anterior ischemic optic neuropathy.

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.

Validity of Administrative Coding for Nonarteritic Ischemic Optic Neuropathy.

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.

A rare case of simultaneous occurrence of non-arteritic anterior ischemic optic neuropathy and corpus callosum ischemia.

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.

Study of nycthemeral variations in blood pressure in patients with non-arteritic anterior ischemic optic neuropathy.

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.

Acute Optic Neuropathy in Older Adults: Differentiating Between MOGAD Optic Neuritis and Nonarteritic Anterior Ischemic Optic Neuropathy.

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.

Reexamining the Incidence of Nonarteritic Anterior Ischemic Optic Neuropathy: A Rochester Epidemiology Project Study.

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.

Nonarteritic anterior ischemic optic neuropathy and incidence of Parkinson's disease based on a nationwide population based study.

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.