Search

Immune checkpoint inhibitor therapy is frequently associated with immune-related adverse events, which occasionally manifest with visual symptoms. Here, we describe a case of unilateral and sudden-onset painless vision loss in an 82-year-old man with metastatic non-small cell lung cancer receiving immunotherapy with the anti-programmed death-ligand 1 agent atezolizumab. Examination demonstrated a right-sided relative afferent pupillary defect, diffusely swollen optic disc, and delayed choroidal and retinal arterial filling on fundus fluorescein angiography, consistent with an arteritic anterior ischemic optic neuropathy. Histology of an ipsilateral temporal artery biopsy revealed a transmural eosinophilic infiltrate without granulomas, while serology revealed the presence of antineutrophil cytoplasmic antibodies. Peripheral eosinophilia was also noted, which preceded treatment by several months. This report highlights the importance of clinician awareness of immune checkpoint inhibitors and their systemic and ophthalmic complications, which rarely appear to extend to eosinophilic temporal arteritis.

The optical coherence tomography angiography (OCTA) is a noninvasive imaging technology which aims at imaging blood vessels in retina by studying decorrelation signals between multiple sequential OCT B-scans captured in the same cross section. Obtaining various vascular plexuses including deep and superficial choriocapillaris, is possible, which helps in understanding the ischemic processes that affect different retina layers. OCTA is a safe imaging modality that does not use dye. OCTA is also fast as it can capture high-resolution images in just seconds. Additionally, it is used in the assessment of structure and blood flow. OCTA provides anatomic details in addition to the vascular flow data. These details are important in understanding the tissue perfusion, specifically, in the absence of apparent morphological change. Using these anatomical details along with perfusion data, OCTA could be used in predicting several ophthalmic diseases. In this paper, we review the OCTA techniques and their ability to detect and diagnose several retinal vascular and optical nerve diseases, such as diabetic retinopathy (DR), anterior ischemic optic neuropathy (AION), age-related macular degeneration (AMD), glaucoma, retinal artery occlusion and retinal vein occlusion. Then, we discuss the main features and disadvantages of using OCTA as a retinal imaging method.

In the setting of a pale optic disc, distinguishing a previous episode of optic neuritis (ON) from that of nonarteritic anterior ischemic optic neuropathy (NAION) may be difficult on clinical examination. Differences in peripapillary vascular network structures, if present, might be of diagnostic utility.Thirty-five eyes with demyelinating ON, 33 eyes with NAION, and 81 eyes of normal subjects were imaged with optical coherence tomography angiography (OCT-A) to assess peripapillary vascular density (VD). In addition, OCT was used to measure peripapillary retinal nerve fiber layer (RNFL) thickness. Areas under the receiver operating characteristic curves were used to differentiate ON vs NAION.NAION eyes had significantly thinner RNFL thickness than ON eyes. Age-adjusted analysis showed that the peripapillary VD values were significantly reduced in NAION (48.3 ± 7.4%) and ON eyes (54.7 ± 6.1%) compared with healthy controls (62.1 ± 4.6%); pairwise comparisons showed statistically significant differences among all 3 groups. After adjustment for severity of optic nerve injury according to mean RNFL thickness, all VD parameters were not significantly different between ON and NAION eyes. The area under the receiver operating characteristic curves for differentiating NAION from ON eyes was similar for VD (0.75) and RNFL thickness (0.74).Peripapillary VD measurement performs as well as RNFL thickness for distinguishing previous episodes ON and NAION. VD decline might be secondary to RNFL damage and, therefore, VD data have a limited role differentiating these 2 disorders.

Background No proven treatment exists for nonarteritic anterior ischemic optic neuropathy (NAION), either in the acute or late phase. Objective To assess safety and changes in visual function and structure after RPh201/placebo treatment in participants with previous NAION. Design and setting Phase 2a, single-site, prospective, randomized, placebo-controlled, double-masked trial (registration NCT02045212). Main outcomes measures Early Treatment Diabetic Retinopathy Study best-corrected visual acuity (BCVA), visual fields, retinal nerve fiber layer, and visual evoked potential at weeks 13, 26, and after a 13-week wash-out ("off-drug") period; and safety. Study population Twenty-two participants aged 18 years or older with previous NAION. Intervention(s) RPh201 (20 mg) or placebo (cottonseed oil vehicle) administered subcutaneously twice weekly at the study site. Results Thirteen men and 9 women were randomized, of which 20 completed all visits. The mean (±SD) age was 61.0 ± 7.6 years. In a post hoc analysis, after 26 weeks of treatment, BCVA improved by ≥15 letters in 4/11 (36.4%) eyes with RPh201, compared to 1/8 (12.5%) eyes with placebo (P = 0.24). Overall, 7/11 (63.6%) of participants on RPh201 showed some improvement in BCVA, compared with 3/8 (37.5%) on placebo (P = 0.26). Improvement in BCVA from a calculated baseline was 14.8 ± 15.8 letters for RPh201 and 6.6 ± 15.3 for placebo (P = 0.27). Of the 154 adverse effects (AEs), 52 were considered related to the study procedures/treatment. Across the study and 1,017 injections, the most frequently reported AE was injection site pain (23 events in 5 participants). There were no clinically significant changes in vital signs or laboratory values. Conclusions This Phase 2a was designed to assess safety, feasibility, and explore potential efficacy signals in treating previous NAION with RPh201. No safety concerns were raised. The results support a larger trial in patients with previous NAION.

To evaluate the optic disc microvasculature in optic nerve head drusen (ONHD) vasculature in comparison to acute nonarteritic anterior ischemic optic neuropathy (NAION) and normal eyes using optical coherence tomography angiography (OCT-A).Ten eyes with ONHD, 10 eyes with acute NAION, and 10 healthy eyes were included in this prospective, comparative, observational case series. OCT-A imaging was performed on the optic discs. Qualitative grading was performed for dilation and tortuosity of the peripapillary vessels by 3 graders. Quantitative comparison was performed for peripapillary and inside disc vessel densities in nerve head (NH) and retinal peripapillary capillary (RPC) slabs.The intergrader agreement for dilation and tortuosity of the peripapillary vessels was poor (0.313 and 0.182 for vascular dilation in nerve head and radial peripapillary capillary enface images, respectively, and 0.478 and 0.490 for vascular tortuosity in nerve head and radial peripapillary capillary enface images, respectively). In NH en face images, the vessel density measurements were statistically significantly different between the 3 groups (all p0.05). In RPC en face images, the vessel density measurements were statistically significantly different between the 3 groups (all p0.05) except for nasal peripapillary sector (0.08).Despite poor intergrader agreement in qualitative analysis, quantitative OCT-A evaluation may differentiate optic disc edema due to NAION from pseudodisc edema due to ONHD.

Case-control analysis and systematic literature review.To illustrate the prognosis and perioperative risk factors associated with this condition.Ischemic optic neuropathy (ION) is the most common pathological diagnosis underlying postoperative vision loss. It comes in two primary forms-anterior (AION)-affecting the optic disc or posterior (PION) affecting the optic nerve proximal to the disc. Spine surgery remains one of the largest sources of acute perioperative visual loss.We performed a 1:4 case-control analysis (by age and year of surgery) for patients with ION and those who didn't develop ION following spine surgery at our institution. A systematic literature search of Medline, Embase, Scopus from inception to September 2017 as also performed.We identified 12 cases from our institution. Comparison to 48 matched controls revealed fusion, higher number of operative levels, blood loss, and change in hemoglobin, hematocrit to be significantly associated with ION. Majority were diagnosed with PION (83%, 10/12) and had bilateral presentation (75%, 9/12). Only 30% patients (3/10) demonstrated improvement in visual acuity while the rest remained either unchanged (40%, 4/10) or worsened (20%, 2/10) at last follow-up. Literature review identified 182 cases from 42 studies. Posterior ischemic optic neuropathy (PION) was found in 58.7% (114/194) of cases, anterior ischemic optic neuropathy (AION) in 17% (33/19) and unspecified ION in 24% (47/194). PION was associated with higher odds of severe visual deficit at immediate presentation (odds ratio [OR]: 6.45, confidence interval [CI]: 1.04-54.3, P = 0.04) and last follow-up.PION is the most common cause of vision loss following spine surgery and causes more severe visual deficits compared with AION. Prone spine surgery especially multi-level fusions with longer operative time, higher blood loss, and intraoperative hypotension are most associated with the development of this devastating complication.3.

Obstructive sleep apnea (OSA) is a disorder characterized by breathing cessation caused by obstruction of the upper airway during sleep. It is associated with multiorgan comorbidities such as obesity, hypertension, heart failure, arrhythmias, diabetes mellitus, and stroke. Patients with OSA have an increased prevalence of ophthalmic disorders such as cataract, glaucoma, central serous retinopathy (detachment of retina, macular hole), eyelid laxity, keratoconus, and nonarteritic anterior ischemic optic neuropathy; and some might require surgery. Given that OSA is associated with a high incidence of perioperative complications and more than 80% of surgical patients with OSA are unrecognized, all surgical patients should be screened for OSA (eg, STOP-Bang questionnaire) with comorbidities identified. Patients suspected or diagnosed with OSA scheduled for ophthalmic surgery should have their comorbid conditions optimized. This article includes a review of the literature and highlights best perioperative anesthesia practices in the management of ophthalmic surgical patients with OSA.

The Giant Cell Arteritis is considered one of the most prevalent autoimmune vasculitis syndromes, which affects arteries of medium and large caliber, being the most feared complication, anterior ischemic optic neuropathy that can evolve to an irreversible loss of vision. The symptoms are caused by local ischemia due to endovascular damage accompanied by systemic damage mediated by cytokines. In recent years, doppler ultrasound, Magnetic Resonance Imaging (MRI) and PET scans have become important in the search for tools that can confirm or exclude the diagnosis of GCA. However, the Temporal Artery Biopsy (BAT) continues to be the gold standard test for the diagnosis of this disease.

Background: Incipient nonarteritic anterior ischemic optic neuropathy is a heavily underdiagnosed ophthalmic condition due to its nature of being asymptomatic. Only a handful of cases have been reported and, therefore, limited information is available in respect to it. To date, incipient nonarteritic anterior ischemic optic neuropathy has been viewed as threatened nonarteritic anterior ischemic optic neuropathy, which does not cause any symptoms or structural change to the affected eye. Case presentation: A 68-year-old female was diagnosed with right incipient nonarteritic anterior ischemic optic neuropathy and left classic nonarteritic anterior ischemic optic neuropathy. Despite her right eye remaining asymptomatic, the optical coherence tomography scan of her right macula at her 3-month follow-up appointment showed thinning of retinal nerve fibre layer and ganglion cell complex. Conclusions: To our knowledge, this is the first ever case report describing a thinning of ganglion cell complex in a patient with incipient nonarteritic anterior ischemic optic neuropathy. This suggests that contrary to current belief, incipient nonarteritic anterior ischemic optic neuropathy can result in subclinical structural changes to the affected eye.

Background Nonarteritic anterior ischemic optic neuropathy (NAION) is the most common acute optic neuropathy in individuals older than 50 years. Demographic, ocular, and systemic risk factors for NAION have been identified, and we sought to determine which, if any, of these factors also increase risk of NAION in the fellow eye. Methods We performed a retrospective chart review of patients with "ischemic optic neuropathy" (based on International Classification of Disease [ICD] codes) seen at a single eye center between 2007 and 2017. Patients who met diagnostic criteria for unilateral NAION without fellow eye optic neuropathy at diagnosis were included. Demographic information, ocular comorbidities, and systemic diagnoses were recorded, in addition to whether the fellow eye developed NAION during the follow-up period. Univariate and multivariate Cox proportional hazard regression were used to calculate hazard ratios (HRs) for fellow eye involvement. Results Three hundred eighteen patients were identified by ICD codes, and 119 were included in the study. Twenty-nine (24%) patients developed NAION in the fellow eye over the mean follow-up period of 3.6 years (range: 1 month-11 years). Significant risk factors for fellow eye NAION included the presence of bilateral optic disc drusen (ODD, HR 2.78, 95% confidence interval [CI] 1.12-6.90, P = 0.02) and noncompliance with continuous positive airway pressure (CPAP) in patients with moderate-to-severe obstructive sleep apnea (HR 4.50, 95% CI 1.79-11.3, P = 0.0015). Conclusions Bilateral ODD and noncompliance with CPAP when indicated are associated with increased risk of NAION in the fellow eye. Patients with these risk factors should be counseled on the potentially devastating visual consequences of bilateral NAION, and compliance with CPAP should be stressed when appropriate.

A 71-year-old woman was admitted with fever, headache, and weight loss associated with elevated inflammatory markers. She developed acute bilateral ophthalmoplegia and asymmetrical ptosis, rapidly followed by anterior ischemic optic neuropathy. Although the first temporal artery biopsy was negative, contralateral temporal artery biopsy revealed features consistent with giant cell arteritis. Even while under steroid therapy, she died a few days later from myocardial infarction. Acute bilateral complete ophthalmoplegia is a rare presentation of a limited number of possible diseases. Among these, giant cell arteritis should be suspected in the appropriate clinical scenario.

Purpose This study aimed to evaluate the effects of retinal artery occlusions caused by cosmetic facial filler injections on the retrobulbar blood flow parameters. Methods This was a retrospective, noncomparative case series. Ten consecutive patients with fundus artery occlusions caused by facial filler injections were evaluated using color Doppler flow imaging (CDFI). The peak systolic velocity and end diastolic velocity of the ophthalmic artery, central retinal artery, and posterior ciliary arteries were determined. The clinical features, including the filler material, injection site, best-corrected visual acuity, fundus fluorescein angiography, and associated ocular and systemic manifestations were also collected. Results Injected materials included autologous fat (7 cases) and hyaluronic acid (3 cases). In 6 of the patients with ophthalmic artery occlusions, the CDFI showed no or drastic declines in the retrobulbar blood flow in the ophthalmic artery, central retinal artery, and posterior ciliary arteries. In 3 of the patients with central retinal artery occlusions, the CDFI showed no or a grossly decreased retrobulbar blood flow in the central retinal artery. In one patient with anterior ischemic optic neuropathy, the CDFI showed decreased end diastolic velocities and increased pulsatility and resistance indices in the central retinal artery, posterior ciliary arteries, and ophthalmic artery. During the follow-up period, the retrobulbar blood flow recovered to some degree. Overall, ophthalmic artery occlusion patients receiving autologous fat may have neurologic complications. Conclusions The results suggest that the retrobulbar ocular blood flows measured with CDFI were distinctly different in the different types of ophthalmic artery occlusion. Color Doppler flow imaging could provide a practicable and convenient method for the diagnosis and follow up of retinal artery occlusions caused by cosmetic facial filler injections.

Meningiomas represent about 20% of intracranial tumors. Involvement of the medial sphenoid wing includes anterior clinoid, cavernous sinus and superior orbital fissure meningiomas. Due to the proximity of these tumors to the optic nerve, typically progressive unilateral vision loss, over several months to years, is the classic clinical presentation. We report three cases of acute monocular vision loss, two transient and one permanent, ipsilateral to a sphenoid meningioma. Ophthalmological involvement with sphenoid meningiomas is most often chronic, due to interruption of axoplasmic flow and demyelination of the optic nerve by local compression. However, vascular involvement with ischemia of the optic nerve or transient low blood flow secondary to compression of the carotid branches vascularizing these structures is another possible mechanism. In our series, two patients had amaurosis fugax, and one patient had sudden, persistent visual loss in relation to acute anterior ischemic optic neuropathy on the side of the meningioma. The mean age of patients with acute visual manifestations was 62 years. These ischemic and non-compressive visual symptoms, ipsilateral to sphenoid meningiomas, are difficult to interpret. Whether these temporary visual disturbances of vascular origin should be considered an early sign of future severe or permanent visual impairment when no optic nerve compression is observed is not certain. The place of these acute visual disturbances in the therapeutic decision, particularly surgical, remains to be defined. Larger multicentric prospective studies are needed to better understand the role of local circulatory factors attributable to meningioma in the occurrence of these acute visual signs.

Reproducible skills are essential for successful induction of a rat model of anterior ischemic optic neuropathy (rAION). We established an in vivo validation index by measuring the natural course of optic nerve head (ONH) width and retinal nerve fiber layer (RNFL) thickness in the rAION model using optical coherence tomography (OCT). The rAION model was induced by photodynamic operations. We measured the ONH width, RNFL, Inner Plexiform layer (IPL) and Ganglion cell complex (GCC) thickness in the acute stage (3 days), subacute stage (day-7 to day-14) and later stage (day-14 to day-28) post-infarct by OCT. Retinal layers were measured by hematoxylin and eosin stain (HE) to confirm the OCT findings. The RGCs survival rate was determined by retrograde Fluoro-gold labeling, and the visual function was assessed with flash visual-evoked potentials (FVEPs) 4 weeks post-infarct. We observed significant thinning in GCC, IPL, and RNFL at day-14 and day-28 but only RNFL showed significant thinning between day-14 and day-28. The ONH showed significant swelling in the acute stage which correlated at a greater extent with RNFL than GCC and IPL. Further RNFL correlated at a greater extent at with GCC than IPL. HE-stained retina cross sections also showed IPL and RNFL thinning, which further confirmed our OCT findings. The RGC density and P1-N2 amplitude were significantly reduced in rAION. Our data suggest that Swelling, reduction of swelling, and atrophy of RNFL in acute, sub-acute, and later stage, respectively and ONH swelling in the acute stage are essential events for confirming the successful induction of rAION.

PURPOSE The purpose of this study was to evaluate ocular hemodynamics in patients with a disease believed to be related to a chronic vascular damage [ie, normal tension glaucoma (NTG)] in comparison with an entity with an acute ischemic impact on the optic nerve [ie, acute nonarteritic anterior ischemic optic neuropathy (NAION)]. MATERIALS AND METHODS Blood-flow velocities [peak systolic velocity (PSV), enddiastolic velocity (EDV)] of the ophthalmic artery (OA), central retinal artery (CRA), and nasal and temporal posterior ciliary arteries were measured using color Doppler imaging. Resistive index (RI) of all vessels was calculated (PSV-EDV/PSV). A total of 41 patients suffering from acute NAION (onset of symptoms

Purpose To determine whether nonarteritic ischemic optic neuropathy (NAION) raises the risk of subsequent stroke in the general population. Design Population-based, retrospective cohort study. Methods Setting : Nationwide, population-based, retrospective cohort study. Patients : Of 1 025 340 beneficiaries in the National Health Insurance Service–National Sample Cohort database (2002-2013), we included 400 952 eligible individuals in the analysis. Observations : To determine the effect of incident NAION on the occurrence of subsequent stroke, we used time-varying covariate Cox regression models. Model 1 included only incident NAION as a time-varying covariate. Model 2 included Model 1 and defined demographics. Model 3 included Model 2, comorbidity, co-medication, and Charlson index score. Main Outcome Measures : Effect (hazard ratio [HR]) of NAION on stroke development. Results Of 400 952 eligible individuals, 1125 patients developed NAION and 16 998 patients suffered from stroke. NAION was not associated with an increased risk of subsequent stroke in Model 1, with HR of 1.31 (95% confidence interval [CI], 0.89-1.92). This was consistent, after adjusting for demographics and/or confounding factors, in Model 2 (HR = 1.19, 95% CI, 0.81-1.75) and Model 3 (HR = 1.10, 95% CI, 0.75-1.62). Conclusions Our results suggest that NAION per se is not associated with a subsequent risk of stroke in the general population.

Introduction. Giant cell (temporal) arteritis refers to a group of chronic and acute systemic vasculitis mainly affecting the extracranial and intracranial arteries of large and medium caliber. Loss of vision due to anterior ischemic optic neuropathy (AION) or occlusion of the central retinal artery is one of the most severe and most common complications of giant cell arteritis. This case report describes a patient with giant cell arteritis, the outcome of it was a total vision loss in the right eye. The patient repeatedly visited the ophthalmologists in the outpatient clinics with complaints of intermittent episodes of vision loss.Purpose. To present methods of diagnosis and treatment, through which the doctor at the initial reception will be able to suspect the disease and start treatment timely.Conclusions. The anamnestic criteria for the GCA diagnosis in the practice of ophthalmologist are: female, age over 50 years, headaches with paresthesia, intermittent lameness of the mandible, short-term episodes of vision loss. The necessary laboratory methods of research include: clinical blood test with determination of erythrocyte sedimentation rate, determination of C-reactive protein level.

Purpose This study aimed to characterize the injuries involving periorbital region in our severely burned patients. Method A 2 years retrospective study was conducted with a total of 210 severe burns admissions. Periorbital burn injuries (all produced in association with facial injuries) were encountered in 126 patients, representing the study group that was further analyzed for multiple parameters: demographics, mechanism of injury, TBSA (total body surface area), burn depth, inhalation injury, need for intubation and mechanical ventilation. The presence and severity of ocular injuries were also evaluated. Results Analyzing our study group (n=126), we observed the presence of multiple negative prognosis factors: elderly patients, extensive burns, deep burns affecting functional areas, unfavorable mechanism (electric, chemical or explosions), inhalation injuries, need for intubation and mechanical ventilation, leading to severe morbidity and high mortality level. Ocular injuries were encountered in 37 patients (30 primary and 7 secondary lesions). The predominance of primary ocular lesions is explained trough high severity burns encountered in our patients with high mortality and lack of long-term clinical observations. Conclusion The clinical outcome for periorbital burn injuries depends on patient characteristics, etiology, burn extension and depth, associated lesions, infectious risk and the quality of the treatment applied. Presence of ocular injuries in various severity degrees impose an adequate evaluation and specialized treatment, being associated with important morbidity. In severely burned patients, it is mandatory to apply preventive measures to avoid ocular complications. If exposure keratopathy is detected, prompt ophthalmologic treatment is essential to avoid functional impairment including loss of vision. Abbreviations: TBSA = total body surface area, MSOF = multisystem organ failure, OCS = orbital compartment syndrome, AION = anterior ischemic optic neuropathy.

While often idiopathic, anterior ischemic optic neuropathy occasionally may occur from an identifiable cause.Observational case report.A 19-year-old woman with unremarkable medical and ophthalmic histories developed visual loss from nonarteritic anterior ischemic optic neuropathy in her right eye after otherwise uneventful dental extraction of the inferior third molars.Anterior ischemic optic neuropathy may rarely occur after dental extraction. Potential pathophysiologic mechanisms of this rare occurrence are discussed.

Background: Invasive aspergillosis is often fatal. Here, we report a patient with invasive aspergillosis primarily involving the optic nerve diagnosed on autopsy. Case Presentation: A 77-year-old female with underlying diabetes mellitus, hyperlipidemia, and hypertension presented with disc swelling of the left eye. Although mini-pulse steroid therapy improved visual acuity (VA) of the left eye, it abruptly decreased to no light perception within a month, followed by a decrease in VA of the right eye to 0.5. At referral, VA was 0.3 in the right eye, and there was no light perception in the left eye. Results: Fundus examination revealed optic disc swelling of both eyes. Goldmann perimetry showed irregular visual field defects, whereas magnetic resonance imaging (MRI), general, and cerebrospinal fluid (CSF) examinations revealed no distinct abnormalities. We suspected anterior ischemic optic neuropathy and invasive optic neuropathy. As with the left eye, steroid pulse therapy temporarily improved VA of the right eye and then decreased to 0.2. Additional anticoagulant therapy did not improve VA. Concurrent to therapy, the patient became febrile with depressed consciousness. Repeat MRI identified suspected midbrain infarction, and CSF examination indicated cerebral meningitis. In spite of administering transfusions and antibiotics, she died on hospital day 40. Autopsy revealed large amounts of Aspergillus hyphae mainly localized in the dura mater of the optic nerve and destruction of the cerebral artery wall, suggesting an etiology of subarachnoid hemorrhage. Conclusions: When examining refractory and persistent disc swelling, we should rule out fungal infections of the optic nerve.

A 53-year-old male developed acute diminution of vision in his right eye while on FOLFOX chemotherapy for stage C colon cancer. Examination revealed bilateral optic nerve head swelling with flame shaped hemorrhages over the right optic disc and anomalous left retinal vasculature. Computed tomography scan of the brain and orbit revealed no cerebral pathology, however bilateral optic disc drusen (ODD) was suspected. B scan ultrasonography confirmed the presence of bilateral ODD. Fluorescein angiography showed early hypofluorescence of the right optic disc with bilateral late disc staining and a diagnosis of right anterior ischemic optic neuropathy (AION) with bilateral ODD was made. A literature review was performed and possible mechanisms for the development of AION in this case were discussed.

INTRODUCTION: Traumatic optic neuropathy is a devastating potential complication of closed head injury. The hallmark of an optic neuropathy, traumatic loss of visual function, which can manifest by subnormal visual acuity, visual field loss, or colour vision dysfunction. The presence of an afferent pupillary defect strongly suggests a prechiasmal location for the injury and is necessary to validate the diagnosis of traumatic optic neuropathy. Vision loss associated with traumatic optic neuropathy can be partial or complete and temporary or permanent. Approximately 1.5% to 5% of patients with closed head injuries have damage to visual pathways (4-6 / 100,000 general population / yr). These injuries can be divided into anterior and posterior lesions. Anterior lesions shows ophthalmoscopic abnormalities (eg. Central retinal artery occlusion) and usually associated with a variety of easily recognized injuries to the globe. Anterior lesions may include optic nerve avulsion, traumatic anterior ischemic optic neuropathy, anterior optic nerve sheath hematoma. Posterior lesions are free of ophthalmoscopic findings, but disc edema and disc pallor do occur. Posterior traumatic optic neuropathy is characterised by visual loss that occur in the presence of an afferent pupillary defect but without evidence of injury to the eye or optic nerve. AIM AND OBJECTIVES: To analyse the clinical profile, response to mega dose steroid therapy and visual function outcome in patients with traumatic optic neuropathy presenting to the neuroophthalmology clinic at Regional Institute of Ophthalmology and Government Ophthalmic Hospital, Chennai. MATERIALS AND METHODS: A prospective observational case study on the pattern of traumatic optic neuropathy and analysis of clinical profile, response to treatment and visual function outcome was conducted in the department of neuroophthalmology at RIO-GOH, Chennai. The study was conducted from June 2008 to November 2010. All eligible traumatic optic neuropathy patients according to inclusion criteria, presented to the neuroophthalmology clinic at RIO-GOH during June 2008 to may 2010 were included in the study. DISCUSSION Hippocrates might have been the first to identify the phenomenon of acute and delayed vision loss after injuries placed to and slightly above the brow. While there is little controversy on the macroscopic mechanism of trauma theory, multiple hypothesis have been proposed at microscopic level of damage to the optic nerve including contusion necrosis, nerve fibre tears and nerve infarction secondary to closed space edema, haemorrhage, thrombosis, vasospasm, impingement by bone spicules and shearing of dural vessels in the optic canal. In our study it is evident that traumatic optic neuropathy is devastating cause of permanent visual loss. Concussive force to head especially forehead transmits shock wave to optic canal. Visual loss is usually instantaneous. SUMMARY: Traumatic optic neuropathy is a rare but significant cause of post traumatic visual loss. The responsible blunt trauma to the frontal region may be minor or severe and accompanied by multiple adjacent fractures. Careful documentation of visual acuity, pupillary function, and red desaturation is essential to guide management. CT imaging should be performed to document such structural abnormalities as optic nerve avulsion, optic nerve sheath hematoma, orbital hematoma, or optic canal fracture with fragments. Based on the data from the International Optic Nerve Trauma Study, observation without intervention is a viable option. Patients and their families should be made aware of the information regarding mega dose corticosteroid therapy and participate in an informed decision. In particular, if visual acuity begins to deteriorate, then corticosteroid therapy should be considered. If a structural abnormality is present that may be contributing to optic nerve dysfunction (hematoma or fragment) or if the patient’s visual acuity deteriorates on corticosteroids, optic canal decompression should be offered. Management of this disorder remains very controversial; involvement of other appropriate subspecialists and careful discussions with the patient and family are essential to maximize visual outcome. FUTURE AND CONTROVERSIES: A better understanding of the cellular and biochemical mechanisms involving normal and traumatized axons, glia, and myelin sheaths may eventually leads to better intervention for traumatic optic neuropathy. Although corticosteroids are a type of neuroprotective agents, the recent expansion in basic scientific research regarding other neuroprotective agents may lead to a redirection in future therapy for this condition. CONCLUSION: In our study the clinical profile of 100 eligible patients who presented with loss of vision following trauma during June 2008 – May 2010 were analyzed. RESULTS: Based on exclusion and inclusion criteria 100 cases were taken up for the study.

Purpose Increased endoplasmic reticulum (ER) stress is one of the earliest subcellular changes in neuro-ophthalmic diseases. In this study, we investigated the expression of key molecules in the ER stress pathways following nonarteritic anterior ischemic optic neuropathy (AION), the most common acute optic neuropathy in adults over 50, and assessed the impact of chemical chaperon 4-phenylbutyric acid (4-PBA) in vivo. Methods We induced AION using photochemical thrombosis in adult mice and performed histologic analyses of key molecules in the ER stress pathway in the retina and optic nerve. We also assessed the effects of daily intraperitoneal injections of 4-PBA after AION. Results In the retina at baseline, there was low proapoptotic transcriptional regulator C/EBP homologous protein (CHOP) and high prosurvival chaperon glucose-regulated protein 78 (GRP78) expression in retinal ganglion cells (RGCs). One day after AION, there was significantly increased CHOP and reduced GRP78 expressions in the ganglion cell layer. In the optic nerve at baseline, there was little CHOP and high GRP78 expression. One day after AION, there was significantly increased CHOP and no change in GRP78 expression. Treatment immediately after AION using daily intraperitoneal injection of chemical chaperone 4-PBA for 19 days significantly rescued Brn3A+ RGCs and Olig2+ optic nerve oligodendrocytes. Conclusions We showed for the first time that acute AION resulted in increased ER stress and differential expression of ER stress markers CHOP and GRP78 in the retina and optic nerve. Rescue of RGCs and oligodendrocytes with 4-PBA provides support for ER stress reduction as possible treatment for AION.

Anemia appears frequently in patients with alcoholic liver disease (ALD) but has never been linked to bilateral nonarteritic anterior ischemic optic neuropathy (NAION). A 65-year-old woman with a medical history of alcoholic cirrhosis was admitted for bilateral NAION. On admission, she was found to have a low arterial pressure and severe normocytic anemia (48 g/L). The anemia was related to chronic bleeding due to antral gastritis along with other factors associated with ALD. The applied treatment consisted of urgent transfusion followed by high doses of proton-pump inhibitors, iron and vitamin supplementation, and support in lifestyle measures. Her hemoglobin levels remained stable after 2 years but the patient still suffered from visual loss. This case highlights the link between anemia and bilateral NAION in ALD patients. The optic nerve head is prone to infarction in this context due to the vascularization characteristics of ALD. Hemoglobin levels should be monitored in ALD patients to avoid the severe complication of NAION.

The management of nonarteritic anterior ischemic optic neuropathy centers around prevention of second eye involvement, without a uniformly accepted therapy for the involved eye. Several researchers have assessed the benefit of steroids with conflicting results. This experimental study was designed to evaluate the efficacy of a single intravitreal triamcinolone acetonide injection (IVTA) in preserving retinal ganglion cells (RGCs) in a rodent model of anterior ischemic optic neuropathy (rAION).The rAION was induced in female Wistar rats. Animals were randomized into 3 groups: 1) untreated, 2) treated with 56 μg IVTA, and 3) intravitreal saline (placebo). Procedures were performed in the left eye, with the right eye serving as control. After 30 days, animals were sacrificed and eyes were assessed histologically for RGC number.The average number of RGC was significantly lower in rAION subgroups when compared with the control group (P0.001). No significant difference was seen between rAION eyes treated with IVTA, placebo, and untreated eyes (P0.05%).In this rodent model for AION, no therapeutic benefit of intravitreal steroid injection was identified.

Purpose To use spectral-domain optical coherence tomography (SD-OCT) to assess whether epipapillary vitreous traction from evolving posterior vitreous detachment (PVD) is mechanistically involved in the pathogenesis of nonarteritic anterior ischemic optic neuropathy (NAION). Design Retrospective cohort study. Methods Setting : Single academic center. Patient or Study Population : Eighty eyes in 74 subjects presenting within 2 weeks of symptom onset of NAION. Intervention or Observation Procedures : SD-OCT imaging of the optic nerve head, macula, and peripapillary retinal nerve fiber layer (pRNFL) were reviewed for PVD and vitreopapillary traction (VPT). Main Outcome Measures : Prevalence and incidence of PVD and VPT, logMAR best-corrected visual acuity (BCVA), mean deviation (MD) on automated perimetry, and pRNFL thickness at presentation and final follow-up. Results The co-prevalence of PVD at the time of acute presentation for NAION was 30% (n = 24/80). This was similar to the prevalence of PVD in contralateral uninvolved eyes (34.5%, n = 19/55). In 8 NAION eyes, PVD had been documented prior to the onset of NAION. No eyes had SD-OCT evidence of VPT on presentation or final follow-up. Only 5 eyes developed a PVD following the development of NAION, and this typically occurred months after resolution of optic disc edema. When comparing eyes with PVD and without PVD, there was no significant difference in BCVA, MD, and pRNFL thickness at baseline, final follow-up, or the change from baseline to final follow-up (all P > .10). Conclusions VPT was not mechanistically related to the development of classic NAION. Preexisting PVD did not preclude the future development of NAION. The co-prevalence of PVD with acute NAION had no impact on final visual outcome.

We present a case of Non-Arteritic Anterior Ischemic Optic Neuropathy (NA-AION) with uncertain etiology but a good recovery with a total gain of central visual acuity.

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

Brain-derived neurotrophic factor (BDNF) and activation of its high affinity receptor tropomyosin kinase (Trk) B promote retinal ganglion cells (RGCs) survival following injury. In this study, we tested the effects of LM22A-4, a small molecule TrkB receptor-specific partial agonist, on RGC survival in vitro and in experimental nonarteritic anterior ischemic optic neuropathy (AION), the most common acute optic neuropathy in those older than 50 years.We assessed drug effects on immunopanned, cultured RGCs and calculated RGC survival and assessed TrkB receptor activation by mitogen-activated protein (MAP) kinase translocation. To assess effects in vivo, we induced murine AION and treated the animals with one intravitreal injection and three-week systemic treatment. We measured drug effects using serial spectral-domain optical coherence tomography (OCT) and quantified retinal Brn3AIn vitro, LM22A-4 significantly increased the survival of cultured RGCs at day 2 (95% CI control: 8.4-13.6; LM22A-4: 23.7-30.3; BDNF: 24.3-29.9; P ≤ 0.0001), similar to the effect of the endogenous TrkB receptor ligand BDNF. There was also significant nuclear and cytoplasmic translocation of MAP kinase (95% CI control: 0.9-6.8; LM22A-4: 38.8-84.4; BDNF: 64.0-93.0; P = 0.0002), a known downstream event of TrkB receptor activation. Following AION, LM22A-4 treatment led to significant preservation of the ganglion cell complex (95% CI: AION-PBS: 66.8-70.7%; AION-LM22A-4: 70.0-73.1; P = 0.03) and total retinal thickness (95% CI: AION-PBS: 185-196%; AION-LM22A-4: 195-203; P = 0.002) as measured by OCT compared with non-treated eyes. There was also significant rescue of the Brn3ATrkB receptor partial agonist LM22A-4 promoted survival of cultured RGCs in vitro by TrkB receptor activation, and treatment in vivo led to increased survival of RGCs after optic nerve ischemia, providing support that LM22A-4 may be effective therapy to treat ischemic optic neuropathy.AION: anterior ischemic optic neuropathy, BDNF: Brain-derived neurotrophic factor, GCC: ganglion cell complex, MAP: mitogen-activated protein, OCT: spectral-domain optical coherence tomography, OD: right eye, ON: optic nerve, ONH: optic nerve head, OS: left eye, RGC: retinal ganglion cell; Trk: tropomyosin kinase.

Purpose To examine the role of oral steroid therapy in the treatment of nondiabetic cases of acute nonarteritic anterior ischemic optic neuropathy (NAAION). Design Randomized double-blind clinical trial. Participants Thirty-eight patients with acute nondiabetic NAAION divided into 2 arms of 19 patients each. One arm constituted the cases and the other constituted the controls. Methods Cases received oral steroid therapy and were designated the steroid group, whereas controls received placebo and were designated the nonsteroid group. Best-corrected visual acuity (BCVA), visual evoked response (VER), and OCT were performed at baseline, 1 month, 3 months, and 6 months after recruitment into the trial. Main Outcome Measures Best-corrected visual acuity, VER, and retinal nerve fiber layer changes on OCT. Results Both groups showed significant improvement in BCVA, VER latency, and resolution of disc edema on OCT parameters over 6 months. Final outcome showed no statistically significant difference with regard to visual acuity, although VER was better in the steroid group (P = 0.011). Best-corrected visual acuity, VER amplitude, and VER latency (P = 0.02, P = 0.02, and P = 0.04, respectively) showed a greater percentage improvement in the steroid group, which also saw a faster resolution of disc edema on OCT (1-month follow-up). Conclusions Oral steroids in acute NAAION did not improve the visual acuity significantly at 6 months. However, they improved resolution of disc edema significantly and enabled a greater improvement in VER parameters. This subtle benefit of oral steroids in NAAION is clinically unimportant and does not provide support for its use.

Purpose: It has been reported that anterior ischemic optic neuropathy (AION) is an infrequent complication of Vogt-Koyanagi-Harada (VKH) disease; however, the physiological changes have not been understood. We quantitatively examined sequential changes in the morphology and circulation hemodynamics using an optical coherence tomography (OCT) C-scan and laser speckle flowgraphy (LSFG) in a patient with VKH disease accompanied by AION.Case presentation: A 65-year-old female complained of blurred vision in both of her eyes. She presented with optic disc swelling and remarkable choroidal thickening detected by OCT bilaterally. Indocyanine green angiography in the middle phase showed multiple hypofluorescent dark dots scattering around the fundus. With the use of Goldmann perimetry, bilateral visual field defects were detected; these were similar to those of inferior altitudinal hemianopsia. Pleocytosis was detected. The patient was diagnosed with VKH disease, suspected to be accompanied by AION in both eyes. She received methylprednisolone pulse therapy followed by oral prednisolone. With these treatments, optic disc swelling disappeared; however, optic disc atrophy with visual field defects remained in both eyes. An OCT C-scan showed the ganglion cell complex (GCC) and circumpapillary retinal nerve fiber layer (cpRNFL) thickness getting thinner below the normal range, and LSFG showed a decrease in optic nerve head tissue microcirculation during follow-up. These results supported the occurrence of AION in this patient with VKH disease.Conclusion: The analyses of GCC and cpRNFL thicknesses with an OCT C-scan and optic nerve head microcirculation with LSFG would be useful for supporting the occurrence of AION in cases of VKH disease.

Ophthalmologic nvolvement in SARS-CoV-infected patients is variegated. One of the ophthalmologic pathologies is optic neuritis. Optic neuritis in SARS-CoV-infected patients may precede the classical pulmonary manifestations of COVID-19 and can be unilateral or bilateral. Optic neuritis has been repeatedly reported in COVID-19 patients and may occur with or without affection of other cranial nerves. Since cerebro-spinal fluid parameters can be abnormal in COVID-19 associated optic neuritis, these patients require a spinal tap. Before diagnosing SARS-CoV-2 associated optic neuritis various differentials need to be excluded. Since SARS-CoV-2 causes endothelial damage complicated by thrombus formation and thromboembolism, ophthalmologic vascular complication due to an infection with SARS-CoV-2 such as anterior ischemic optic neuropathy (AION), central retinal artery occlusion (CRAO), and retinal vein occlusion need to be excluded. CRAO may result from arterial hypertension, myocarditis, heart failure, Takotsubo syndrome, atrial fibrillation, or atrial flutter, frequent cardiac complications of COVID-19. Since CRAO can be accompanied by ischemic stroke, patients with SARS-CoV-2 associated optic neuritis need to undergo a cerebral MRI.

This cross-sectional study compared the peripapillary vessel density (VD), peripapillary retinal nerve fiber layer (RNFL) thickness, and optic nerve head (ONH) parameters between eyes with atrophic non-arteritic anterior ischemic optic neuropathy (NAION) and eyes with advanced primary open-angle glaucoma (POAG) matched for visual field mean deviation. Peripapillary VDs and RNFL thicknesses in the peripapillary region, and 4 sectors (superior, inferior, nasal, and temporal), and scanning laser ophthalmoscopy parameters of the ONH were evaluated with optical coherence tomography angiography (OCTA) among 21 atrophic NAION cases, 26 advanced POAG cases, and 30 age- and sex-matched healthy controls. The POAG eyes had lower peripapillary VDs in all areas compared with the NAION eyes, which was most marked in the inferior and nasal sectors (p=0.005 for both). RNFL loss was similar between the 2 groups in all areas, except for a preserved thickness in the inferior sector in NAION eyes (p=0.01). Peripapillary VD demonstrated stronger correlations with global RNFL thickness in the peripapillary region in the NAION eyes compared with that of the POAG eyes (r=0.91 p

BACKGROUND: With the development and widespread adoption of spectral-domain optical coherence tomography (OCT), peripapillary hyper-reflective ovoid mass-like structures (PHOMS) have become a frequent OCT finding in neuro-ophthalmic practice. Although originally assumed to represent a form of buried optic disc drusen (ODD), PHOMS differ from ODD in many important ways. The histopathological underpinnings of PHOMS are now becoming more clearly understood. EVIDENCE ACQUISITION: Review of literature. RESULTS: PHOMS can be broadly classified as disk edema-associated PHOMS, ODD-associated PHOMS, or anomalous disk-associated PHOMS. PHOMS are seen in many conditions, including papilledema, nonarteritic anterior ischemic optic neuropathy, central retinal vein occlusion, acute demyelinating optic neuritis, ODD, and tilted disks (myopic obliquely inserted disks) and in many cases resolve along with the underlying condition. The histopathological study of these diverse entities reveals the common feature of a bulge of optic nerve fibers herniating centrifugally over Bruch membrane opening into the peripapillary space, correlating exactly with the location, shape, and space-occupying nature of PHOMS on OCT. Because of the radial symmetry of these herniating optic nerve fibers, PHOMS are best thought of as a complete or partial torus (i.e., donut) in 3 dimensions. CONCLUSIONS: PHOMS are a common but nonspecific OCT marker of axoplasmic stasis in the optic nerve head. They are not themselves ODD or ODD precursors, although they can be seen in association with ODD and a wide spectrum of other conditions. They do not exclude papilledema and often accompany it. The circumferential extent and characteristic 3D toroidal nature of a PHOMS are best appreciated by scrolling through consecutive OCT images.

Purpose: To analyze the data and retinal microvasculature for nonarteritic anterior ischemic optic neuropathy (NAİON) patients referred to have carotid Doppler ultrasound from 2016 to 2020. Methods: In this case-control observational study, 30 NAİON patients evaulated with carotid doppler sonography. 22 NAION patients (at least 3 months after onset of symptoms) and 9 normal subjects underwent a complete ophthalmic examination including optical coherence tomography (OCT), optical coherence tomography angiography (OCT-A). NAİON eyes and fellow eyes were further divided into the groups according to presence of carotid stenosis (CS). NAION patients with CS were termed "CS-NAION"; those without were termed "NCS-NAION". Measurements of radial peripapillary capillary vessel density (RPC VD) and ganglion cell complex (GCC), disk retinal nerve fiber layer (RNFL) thickness were compared among groups. Results: Fourteen of 30 NAİON patients referred for carotid Doppler were positive for CS with all of such referrals having less than 50% stenosis. RNLF, GCC and RPC VDs were reduced in NAION eyes, when compared to controls and the fellow eyes. RPC VD was significantly lower in the temporal-superior (P=0.037) and the superior-temporal (P=0.012) sectors of the NCS-NAION patients than in the CS-NAION patients. No significant differences were found between CS-fellow eyes and NCS-fellow eyes in terms of RPC VDs, RNLF or GCC. Conclusion: Results of the study highlight the effect of carotid artery stenosis on ocular perfusion pressure in the pathogenesis of NAİON. More extensive studies are necessary.

Background: Fluoxetine enhances the levels of brain-derived neurotrophic factor (BDNF); considering its known improving effects on neurogenesis and plasticity, it seems to improve the Anterior Ischemic Optic Neuropathy (AION). This study aimed to evaluate the effect of Fluoxetine on clinical prognosis of patients with AION.Methods: In this double-blind placebo-controlled randomized clinical trial, subjects with AION who were referred to Rasool Akram Hospital were divided into two study groups; the fluoxetine group that received 20 mg Fluoxetine daily(n=50) and the control group (n=50) that received placebo for a period of six months. Patients underwent clinical and paraclinical evaluations before and after the trial. This study was a registered trial with IRCT code IRCT20181109041596N1.Results: One hundred patients were enrolled from August 2019 to December 2020 and assessed in this study. Subjects in Fluoxetine group showed significant improvement in visual acuity in comparison to the placebo group with less score in LogMAR scale (P: 0.008 and 0.002, respectively), improvement in MD parameters of perimetry (P: 0.003 and 0.002, respectively), and decrease in VEP latencies (P (in 1st minute): st minute): 0.038 and 0.011, respectively). There were no differences in color vision, Rnfl in all dimensions, PSD parameter of perimetry or VEP amplitudes following the trial of Fluoxetine therapy (Ps> 0.05).Conclusion: Fluoxetine showed promising therapeutic value for patients with AION besides its safety as an additive treatment option to corticosteroids.

Mesenchymal stem cell (MSC) therapy has been investigated intensively for many years. However, there is a potential risk related to MSC applications in various cell niches. Methods: The safety of intravitreal MSC application and the efficacy of MSC-derived conditioned medium (MDCM) were evaluated in the normal eye and the diseased eye, respectively. For safety evaluation, the fundus morphology, visual function, retinal function, and histological changes of the retina were examined. For efficacy evaluation, the MDCM was intravitreally administrated in a rodent model of anterior ischemic optic neuropathy (rAION). The visual function, retinal ganglion cell (RGC) density, and neuroinflammation were evaluated at day 28 post-optic nerve (ON) infarct. Results: The fundus imaging showed that MSC transplantation induced retinal distortion and venous congestion. The visual function, retinal function, and RGC density were significantly decreased in MSC-treated eyes. MSC transplantation induced astrogliosis, microgliosis, and macrophage infiltration in the retina due to an increase in the HLA-DR-positive MSC proportion in vitreous. Treatment with the MDCM preserved the visual function and RGC density in rAION via inhibition of macrophage infiltration and RGC apoptosis. Conclusions: The vitreous induced the HLA-DR expression in the MSCs to cause retinal inflammation and retina injury. However, the MDCM provided the neuroprotective effects in rAION.

Purpose To characterize the diagnostic yield of the spot sign in the diagnostic workup of acute arterial occlusions of the eye in elderly patients. Methods Clinical characteristics of consecutive patients aged ≥ 50 years with acute central retinal artery occlusion (CRAO), branch retinal artery occlusion (BRAO) or anterior ischemic optic neuropathy (AION) were recorded. Videos of transocular sonography were assessed for the presence of the spot sign by two blinded readers. Group comparisons were made between CRAO-patients with and without the spot sign. Two experienced cardiovascular physicians allocated CRAO-cases to a presumed aetiology, without and with knowledge on the presence/absence of the spot sign. Results One-hundred-twenty-three patients were included, 46 of whom suffered from CRAO. A spot sign was seen in 32 of 46 of patients with CRAO and in 7 of 23 patients with BRAO. Interobserver agreement was excellent (Cohen`s kappa 0.98). CRAO-patients with the spot sign significantly more frequently had a medical history of cardiovascular disease (62.8 vs. 21.4%, p = 0.03) and left heart valve pathologies (51.9 vs. 10%, p = 0.03). The spot sign was not found in any of the three patients with CRAO secondary to cranial giant cell arteritis. The assumed CRAO aetiology differed in 37% of cases between two cardiovascular physicians, regardless whether transocular sonography findings were known or not. Conclusion The spot sign is a simple sonographic finding with excellent interobserver agreement, which proofs the embolic nature of CRAO, but does not allow exact attribution of the underlying aetiology.

Purpose of Review Giant cell arteritis (GCA), a medium and large vessel vasculitis occurring in the aged, remains a formidable disease, capable of taking both vision and life, through a multitude of vascular complications. Our understanding of the spectrum of its manifestations has grown over the years, to include limb claudication, aortitis, and cardiac disease, in addition to the more classic visual complications resulting from of ischemia to branches of the external and internal carotid arteries. While a clinical presentation of headache, jaw claudication, scalp tenderness, fever and other systemic symptoms and serum markers are together highly suggestive of the disease, diagnosis can be challenging in those cases in which classic symptoms are lacking. The purpose of this review is to update the reader on advances in the diagnosis and treatment of giant cell arteritis and to review our evolving understanding of the immunological mechanism underlying the disease, which have helped guide our search for novel therapies. Recent Findings There is increasing evidence supporting the use of Doppler ultrasound, dedicated post-contrast T1-weighted spin echo MRI of the scalp arteries and PET scan, which can together improve our diagnostic accuracy in cases in which temporal artery biopsy is either inconclusive or not feasible. Advances in our understanding of the immunological cascades underlying the disease have helped guide our search for steroid-sparing treatments for the GCA, the most important of which has been the IL-6 receptor antibody inhibitor tocilizumab, which has been shown to reduce cumulative steroid dose in a large multicenter, placebo-controlled prospective study. Other biologic agents, such as abatacept and ustekinumab have shown promise in smaller studies. Summary GCA is no longer a disease whose diagnosis is based exclusively on temporal artery biopsy and whose complications are prevented solely with the use of corticosteroids. Modern vascular imaging techniques and targeted immunologic therapies are heralding a new era for the disease, in which practitioners will hopefully be able to diagnosis it with greater accuracy and treat it with less ischemic complications and iatrogenic side effects.

International audience; Purpose: The purpose of this study was to describe capillary changes in patients with nonarteritic anterior ischemic optic neuropathy (NAION) using optical coherence tomography-angiography (OCT-A) and correlate the results with best corrected visual acuity (BCVA), visual field, OCT retinal nerve fiber layer (RNFL), and combined thickness of ganglion cell and inner plexiform layers (GCIPL) thicknesses.Methods: We enrolled 22 eyes with acute NAION and 30 normal control (NC) subjects in this study. Whole en face image vessel density (WiVD) was measured in the radial peripapillary capillary plexus (RPC), superficial capillary plexus (SCP), and deep vascular complex (DVC) using OCT-A. The examination was repeated at 1 (M1), 3 (M3), 6 (M6), and 9 (M9) months after presentation for NAION.Results: The initial RPC WiVD was significantly reduced in the acute NAION group compared to the NC group (P < 0.0001). Over the course of NAION follow-up, RPC WiVD was significantly reduced at M1 (P < 0.001 compared to M0) and M3 (P < 0.0001 compared to M1). However, there was no significant further decrease at M6 and M9. The initial SCP WiVD was significantly reduced in the NAION group compared to the NC group (P < 0.0001 for both). Over the course of NAION follow-up, a significant decrease was observed for SCP WiVD at M1 (P < 0.001 compared to M0), but no significant change was seen at M3, M6, or M9. DVC was normal in the NAION group. Correlations were found between GCIPL and SCP WiVD in the NAION acute phase (R = 0.604, P = 0.003) and in the M9 atrophic stage (R = 0.551, P = 0.009). At M9, RPC WiVD was correlated with BCVA (R = -0.562, P = 0.007), mean deviation (R = 0.518, P = 0.01), and RNFL (R = 0.655, P = 0.001).Conclusions: Over the course of NAION, OCT-A provided detailed visualization of retinal capillary plexus involvement.

Optic disc cupping is usually considered a hallmark of glaucoma, but it is not necessarily caused due to glaucoma. There are conditions which may manifest as ‘non-glaucomatous optic disc cupping’. It may not always be possible to distinguish glaucomatous from non-glaucomatous optic disc cupping based on the optic disc appearance alone. This chapter addresses some conditions that cause non-glaucomatous cupping and may mimic glaucoma and how a proper clinical examination always provides valuable clues to the diagnosis.

Non-arteritic anterior ischemic optic neuropathy (NAION) is the most common acute unilateral optic nerve-related cause of vision loss in people over age 50. Patients usually present with sudden onset of painless vision loss in one eye. Fundoscopy demonstrates optic disc swelling in the affected eye acutely. Risk factors for NAION include diabetes, hypertension, obstructive sleep apnea, and an anatomically crowded disc. There is no treatment available that has proven efficacious to treat NAION. Therefore, management consists of evaluation for and treatment of underlying risk factors, which could place the patient at risk of NAION in the fellow eye, as well as other systemic complications, in the future.