Anterior and posterior ischemic optic neuropathy in a child with focal segmental glomerulosclerosis on hemodialysis.

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.

Frequency of visual involvement in a 10-year interdisciplinary cohort of patients with giant cell arteritis.

BACKGROUND: We present the largest study of the frequency and nature of visual complications in a cohort of 350 patients consecutively diagnosed with giant cell arteritis (GCA). METHODS: All individuals were assessed using structured forms and diagnosed using imaging or biopsy. A binary logistic regression model was used to analyse data for predicting visual loss. RESULTS: Visual symptoms occurred in 101 (28.9%) patients, with visual loss in one or both eyes in 48 (13.7%) patients. Four patients had binocular visual loss. Anterior ischaemic optic neuropathy (N=31), retinal artery obstruction (N=8) and occipital stroke (N=2) were the main causes of visual loss. Of the 47 individuals who had repeat visual acuity testing at 7 days, three individuals had improvement to 6/9 or better. After introducing the fast-track pathway, the frequency of visual loss decreased from 18.7% to 11.5%. Age at diagnosis (odds ratio (OR) 1.12) and headache (OR 0.22) were significant determinants of visual loss in a multivariate model. Jaw claudication trended to significance (OR 1.96, p=0.054). CONCLUSIONS: We recorded a visual loss frequency of 13.7% in the largest cohort of patients with GCA examined from a single centre. Although improvement in vision was rare, a dedicated fast-track pathway reduced visual loss. Headache could result in earlier diagnosis and protect against visual loss.

Ischemic optic neuropathy as first presentation in patient with m.3243 A > G MELAS classic mutation.

BACKGROUND: Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a systemic disorder in which multi-organ dysfunction may occur from mitochondrial metabolism failure. Maternally inherited mutations in the MT-TL1 gene are the most frequent causes for this disorder. Clinical manifestations may include stroke-like episodes, epilepsy, dementia, headache and myopathy. Among these, acute visual failure, usually in association with cortical blindness, can occur because of stroke-like episodes affecting the occipital cortex or the visual pathways. Vision loss due to optic neuropathy is otherwise considered a typical manifestation of other mitochondrial diseases such as Leber hereditary optic neuropathy (LHON). CASE PRESENTATION: Here we describe a 55-year-old woman, sister of a previously described patient with MELAS harbouring the m.3243A > G (p.0, MT-TL1) mutation, with otherwise unremarkable medical history, that presented with subacute, painful visual impairment of one eye, accompanied by proximal muscular pain and headache. Over the next weeks, she developed severe and progressive vision loss limited to one eye. Ocular examination confirmed unilateral swelling of the optic nerve head; fluorescein angiography showed segmental perfusion delay in the optic disc and papillary leakage. Neuroimaging, blood and CSF examination and temporal artery biopsy ruled out neuroinflammatory disorders and giant cell arteritis (GCA). Mitochondrial sequencing analysis confirmed the m.3243A > G transition, and excluded the three most common LHON mutations, as well as the m.3376G > A LHON/MELAS overlap syndrome mutation. Based on the constellation of clinical symptoms and signs presented in our patient, including the muscular involvement, and the results of the investigations, the diagnosis of optic neuropathy as a stroke-like event affecting the optic disc was performed. L-arginine and ubidecarenone therapies were started with the aim to improve stroke-like episode symptoms and prevention. The visual defect remained stable with no further progression or outbreak of new symptoms. CONCLUSIONS: Atypical clinical presentations must be always considered in mitochondrial disorders, even in well-described phenotypes and when mutational load in peripheral tissue is low. Mitotic segregation of mitochondrial DNA (mtDNA) does not allow to know the exact degree of heteroplasmy existent within different tissue, such as retina and optic nerve. Important therapeutic implications arise from a correct diagnosis of atypical presentation of mitochondrial disorders.

Systemic lupus erythematosus with acute ischemic optic neuropathy complicated with neuromyelitis optica: a case report.

BACKGROUND: Neuromyelitis optica is a relapsing-remitting disease characterized by a recurrent attack of optic neuritis and transverse myelitis; sometimes associated with acute brainstem syndrome. Systemic lupus erythematosus is an autoimmune multisystem disorder in which ocular involvement such as acute ischemic optic neuropathy is a rare manifestation. However, neuromyelitis optica can be associated with systemic lupus erythematosus. CASE PRESENTATION: A 24-year-old Bangladeshi woman was admitted to the hospital with complaints of sudden, progressive, painless vision loss in both eyes, and progressive weakness in both lower limbs for 48 hours. She also gave a history of arthralgia, a photosensitive skin rash, intermittent fever, oral ulcerations, and alopecia for the last 2 months. On examination, the fundus was suggestive of bilateral acute ischemic neuropathy, and examinations of the lower limb revealed spastic paraparesis with sensory abnormality. Laboratory investigations revealed the presence of positive anti-aquaporin 4 antibody, strongly positive antinuclear antibody, and anti-ds DNA with the longitudinally extensive lesion on magnetic resonance imaging of the spinal cord. She was treated with methylprednisolone, hydroxychloroquine, and mycophenolate, and was discharged with improvement of her paraparesis. However, her vision did not improve substantially. CONCLUSION: The importance of this report is to shed some light on the occurrence of two devastating complications that is, bilateral acute ischemic optic neuropathy in systemic lupus erythematosus complicated by neuromyelitis optica, as well as evidence of rare presentations for systemic lupus erythematosus and treatment modalities of ischemic optic neuropathy with systemic lupus erythematosus.

A Taiwan Nationwide Population-Based Study of Socio-Demographic Factors and Comorbid Conditions Associated with Non-Arteritic Anterior Ischaemic Optic Neuropathy.

AIMS: The aim of the study was to investigate the socio-demographic factors and systemic conditions associated with non-arteritic anterior ischaemic optic neuropathy (NAION). METHODS: This was a nationwide population-based retrospective case-controlled study that recruited 9,261 NAION patients selected from the Taiwan National Health Insurance Research Database. The control group consisted of 9,261 age-, sex-, and index date-matched non-NAION patients recruited from the Taiwan Longitudinal Health Insurance Database, 2000. NAION was designated in the database by the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) as "code 377.41: ischaemic optic neuropathy without ICD-9-CM code 446.5: giant cell arteritis." Associated socio-demographic factors and systemic medical conditions were analysed using the McNemar's test, and continuous variables were analysed using the paired t test. The odds ratio (OR) and adjusted OR of developing NAION were compared using univariate logistic regression and multivariable logistic regression analyses, respectively. RESULTS: Patients with systemic conditions such as diabetes mellitus, hypertension, hyperlipidaemia, chronic kidney disease, and hypotension were more likely to develop NAION than controls (adjusted OR = 1.81, 95% confidence interval [CI] = 1.67-1.97, p < 0.0001; adjusted OR = 1.46, 95% CI = 1.36-1.57, p < 0.0001; adjusted OR = 1.44, 95% CI = 1.33-1.57, p < 0.0001; adjusted OR = 3.26, 95% CI = 2.65-4.01, p < 0.0001; adjusted OR = 2.32, 95% CI = 1.31-4.10, p = 0.0039, respectively). CONCLUSIONS: NAION is strongly associated with diabetes mellitus, hypertension, hyperlipidaemia, chronic kidney disease, and hypotension.

Influence of obstructive sleep apnea syndrome on the contralateral optic nerve in patients with unilateral nonarteritic anterior ischemic optic neuropathy.

STUDY OBJECTIVES: The aim was to quantitatively evaluate the influence of obstructive sleep apnea syndrome (OSAS) on the morphology and function of the contralateral optic nerve in patients with unilateral nonarteritic anterior ischemic optic neuropathy (NAION). METHODS: Fifty patients with unilateral NAION were divided into non-OSAS (n = 16), mild OSAS (n = 15), and moderate-severe OSAS (n = 19) groups based on their apnea-hypopnea index (AHI) scores. Systemic and ocular characteristics were compared between these groups. Spearman correlation and multiple linear regression analyses were used to determine the independent factors that most influenced the thickness of the peripapillary retinal nerve fiber layer (pRNFL). RESULTS: Body mass index and hypertension occurrence were higher in the moderate-severe OSAS group than in the non-OSAS group. Temporal pRNFL was thinner in the moderate-severe group than in the mild and non-OSAS groups, whereas no difference was found between the mild and non-OSAS groups. Spearman correlation showed that the AHI (r = -.469, P = .001) and the percentage of total sleep time with oxygen saturation < 90% (T90%; r = -.477, P = .001) correlated with temporal pRNFL thickness. Multiple linear regression showed that the AHI was negatively associated with temporal pRNFL thickness (ß = -0.573, P = .003). CONCLUSIONS: OSAS may cause subclinical temporal pRNFL thinning in the contralateral optic nerve among patients with unilateral NAION without any significant change in visual function. Advanced optic nerve observation and intervention may be warranted in patients with moderate-severe OSAS. CITATION: Li X, Zhang Y, Guo T, et al. Influence of obstructive sleep apnea syndrome on the contralateral optic nerve in patients with unilateral nonarteritic anterior ischemic optic neuropathy. J Clin Sleep Med. 2023;19(2):347-353.

Unilateral Optic Nerve Diffusion Restriction After Sinus Surgery Secondary to Central Retinal Artery Occlusion: Case Report and Literature Review.

INTRODUCTION: Permanent perioperative vision loss is caused by ischemic optic neuropathy (ION) or central retinal artery occlusion (CRAO). Whereas diffusion restriction of the optic nerve (ON) on brain magnetic resonance imaging has been previously reported in perioperative posterior ION (PION), there are no reports of ON diffusion restriction in patients diagnosed with acute perioperative CRAO. We present a case of perioperative CRAO to highlight this neuroimaging finding for neuroradiologists and neurologists. CASE REPORT: A 71-year-old male without vascular risk factors underwent maxillary bilateral antrostomy and septoplasty for chronic sinusitis. Twenty to thirty minutes upon awakening, he complained of painless left eye vision loss. Ophthalmoscopic examination showed retinal whitening, segmented arterioles, and hyperemic disc. Brain MR-diffusion weighted imaging/apparent diffusion coefficient revealed ON diffusion restriction in the proximal segment. Despite attempted reperfusion, left eye remained with no light perception at 6 months. Patients undergoing nonocular surgeries who develop perioperative vision loss related to PION may exhibit ON diffusion restriction but usually have normal ophthalmoscopic findings. CRAO shows retinal whitening, edema, segmentation of arterioles, and cherry red spot on ophthalmoscopy. A recent study reported that ON diffusion restriction in nonperioperative CRAO cases has a sensitivity and specificity of 55% and 70% to 100%. Here, PION was initially considered based on imaging. However, given the neuro-ophthalmic findings, a proximal embolus in the central retinal artery, obstructing its entrance into the proximal ON was deemed more likely. CONCLUSION: We highlight that proximal ON diffusion restriction on brain magnetic resonance imaging can be diagnostic of proximal thromboembolic CRAO. Future studies should evaluate the diagnostic utility and accuracy of MR-diffusion weighted imaging/apparent diffusion coefficient in perioperative visual loss.

Angioinvasive aspergillosis mimicking giant cell arteritis in an 81-year-old man with jaw pain and vision loss.

The present case report focuses on an immunocompromised 81-year-old patient initially diagnosed with Waldenström's disease. The patient experienced a gradual vision loss and jaw pain with high erythrocyte sedimentation rate. We first suspected giant cell arteritis, despite inconclusive assessment, including a negative temporal artery biopsy. We rapidly started a corticosteroid pulse therapy followed by high-dose corticosteroid therapy that was followed even after discharge from the hospital. The patient was readmitted 20 days later with severe left retro-orbital pain and progressive left vision loss. Clinical examination revealed complete left eyelid ptosis and unilateral blindness with fixed mydriasis and no eye movement. MRI showed signs of ischaemic optic neuropathy with lysis of the left ethmoid sinus wall; thus, indicating ischaemic optic neuropathy related to lymphoplasmacytic infiltration of Waldenström's disease (Bing-Neel syndrome). Oncological treatment of ibrutinib, a tyrosine kinase inhibitor, was then administered. Despite a favourable prognosis, no improvement was seen. An infectious aetiology was finally confirmed. The left sphenoid sinus biopsy highlighted an angioinvasive aspergillosis with rhino-orbital infiltration observed as ischaemic optic neuropathy. Oncologic treatment was discontinued and antifungal therapy with voriconazole was introduced, leading to a favourable radiological development and analgesic control, without ophtalmological improvement.

Rare Ischemic Complications of Giant Cell Arteritis: Case Series and Literature Review.

BACKGROUND Some ischemic complications due to giant cell arteritis (GCA) are rare and underdiagnosed. We describe the clinical features and outcomes of patients with GCA presenting with rare ischemic complications. CASE REPORT Our single-center retrospective database of patients with GCA was reviewed from 1994 to 2020. We describe 3 cases of rare ischemic complications secondary to GCA. We review the literature regarding ischemic complications due to GCA and their outcomes. All 3 cases met the American College of Rheumatology criteria for GCA. All patients experienced rare ischemic complications due to GCA. In case 1, the patient presented with a sixth cranial nerve palsy. In case 2, the patient presented with tongue and scalp necrosis, and with permanent visual loss due to anterior ischemic optic neuropathy. In case 3, the patient presented with scalp necrosis. In all 3 cases, the patients received glucocorticoids either intravenously and/or orally, which led to improvement. They all improved within the course of their followup visits. A literature review was performed to identify similar cases and outcomes. CONCLUSIONS Ischemic complications due to GCA can be part of the initial presentation of the vasculitis, making confirmation of the diagnosis more difficult. Physicians should be aware of these rare complications since rapid diagnosis and initiation of glucocorticoids may alter the course of the disease.

Optic Nerve Head Anatomy and Vascular Risk Factors in Patients With Optic Disc Drusen Associated Anterior Ischemic Optic Neuropathy.

PURPOSE: Optic disc drusen (ODD) is an anatomical risk factor for nonarteritic anterior ischemic optic neuropathy (NA-AION). This study aimed to investigate the anatomical and vascular risk factors of patients with ODD-AION (ODD-associated NA-AION) and compare them with similar data from patients with nODD-AION (NA-AION without ODD). DESIGN: Case-control study. METHODS: Thirty-four ODD-AION and 34 nODD-AION patients who had all been systematically optical coherence tomography scanned using a standardized ODD scanning protocol were retrospectively analyzed and compared regarding demographics, vascular risk factors, clinical characteristics, and specific optic nerve head anatomical characteristics. RESULTS: In patients with ODD-AION, the ODD were predominantly deeply located (82%) but with no significant difference in size (52% large, 48% small). When compared with nODD-AION patients, ODD-AION patients were significantly younger at the time of diagnosis (P = .012) and had fewer vascular risk factors (P = .015). The ODD-AION patients had significantly more peripapillary hyperreflective ovoid mass-like structures (PHOMS) (P < .001) and prelaminar hyperreflective lines (P < .001) as well as smaller Bruch's membrane opening diameters (P = .017) compared with nODD-AION patients. No significant differences were found between ODD-AION and nODD-AION patients regarding visual acuity, refraction, lamina cribrosa position, ganglion cell layer volume, or retinal nerve fiber layer thickness. CONCLUSION: In ODD-AION, location of the ODD within the optic nerve head is important, while the size of the ODD is not. The ODD-AION and nODD-AION patients presented with distinctly different vascular risk factors and anatomical characteristics, establishing ODD and potentially also PHOMS as independent risk factors for developing NA-AION.

Non-arteritic anterior ischaemic optic neuropathy (NA-AION) and COVID-19 vaccination.

A woman in her 50s presented with diminution of vision in her left eye (OS) 4 days after COVISHIELDTM vaccination. She had been diagnosed with non-arteritic anterior ischaemic optic neuropathy (NA-AION) of right eye (OD) 8 months earlier. The present episode revealed a best-corrected visual acuity (BCVA) of 20/50 in OD and 20/20 in OS with grade 1 relative afferent pupillary defect. Fundus evaluation showed pale disc in OD and temporal disc oedema in OS. Humphrey's visual field analysis showed incomplete inferior altitudinal defect in OD and a centro-caecal scotoma in OS. Systemic investigations were normal. OS was diagnosed with NA-AION. She was started on oral aspirin 75 mg. At 1-month follow-up, disc oedema of OS had resolved with BCVA maintaining at 20/20. The patient was lost to follow-up later. The relationship between the vaccine and the ocular event is temporal with no causal association.

Non-arteritic Anterior Ischemic Optic Neuropathy with Macular Star following COVID-19 Vaccination.

PURPOSE: To report a case of non-arteritic anterior ischemic optic neuropathy (NAAION) with macular star after receiving the second dose of SARS-CoV-2 vaccination. METHOD: Case report. OBSERVATION: A 51-year-old male presented with acute visual disturbances one day after the second dose of BNT162b2 mRNA SARS-CoV-2 vaccination. At presentation, best corrected visual acuity (BCVA) was 20/25 right eye (OD) and counting fingers at 3 feet left eye (OS). Anterior segment examination was normal in both eyes. Dilated fundoscopy was unremarkable OD, however, it disclosed optic nerve swelling and subretinal fluid OS. Patient was treated with a gradual tapering dose of oral prednisone over 1 month. At the five-week follow-up visit, optic disc swelling and subretinal fluid resolved with minimal improvement in BCVA to 20/400 OS. CONCLUSION: It is unclear whether COVID-19 vaccination was the triggering agent to the NAAION or just a coincidence, yet ophthalmologists should be aware of such a possible association.

A Potential Role of Acute Choroidal Expansion in Nonarteritic Anterior Ischemic Optic Neuropathy.

Purpose: Nonarteritic anterior ischemic optic neuropathy (NAION) has been associated with a thickened choroid at the optic nerve head (ONH). Here, we use computational modeling to better understand how choroidal expansion and choroidal geometry influence tissue deformation within the ONH relative to intraocular pressure (IOP) and intracranial pressure (ICP) effects. Methods: Using a model of the posterior eye that included the sclera, peripapillary sclera, annular ring, pia mater, dura mater, neural tissues, Bruch's membrane, choroid, and lamina cribrosa, we examined how varying material properties of ocular tissues influenced ONH deformations under physiological and supra-physiological, or "pathological," conditions. We considered choroidal expansion (c. 35 µL of expansion), elevated IOP (30 mm Hg), and elevated ICP (20 mm Hg), and calculated peak strains in the ONH relative to a baseline condition representing an individual in the upright position. Results: Supra-physiological choroidal expansion had the largest impact on strains in the prelaminar neural tissue. In addition, compared to a tapered choroid, a "blunt" choroid insertion at the ONH resulted in higher strains. Elevated IOP and ICP caused the highest strains within the lamina cribrosa and retrolaminar neural tissue, respectively. Conclusions: Acute choroidal expansion caused large deformations of the ONH and these deformations were impacted by choroid geometry. These results are consistent with the concept that compartment syndrome due to the choroid geometry and/or expansion at the ONH contributes to NAION. Prolonged deformations due to supra-physiological loading may induce a mechanobiological response or ischemia, highlighting the potential impact of choroidal expansion on biomechanical strains in the ONH.

Dialysis-Associated Nonarteritic Anterior Ischemic Optic Neuropathy: A Case Series and Review.

BACKGROUND: Dialysis-associated nonarteritic ischemic optic neuropathy (DA-NAION) occurs secondary to intradialytic hypotension often with catastrophic consequences and is one of the rare situations where NAION can recur in the same eye. We describe 3 cases of DA-NAION associated with hypotension, review the current literature on DA-NAION, and provide recommendations for decreasing the risk of intradialytic hypotension. METHODS: In addition to describing 3 cases of DA-NAION, PubMed was searched for all reports of DA-NAION in adults with documented episodes of hypotension preceding the onset of NAION. A total of 50 eyes of 31 patients were included. Age, visual acuity at presentation, rate of bilateral involvement at presentation, sequential involvement of the fellow eye, and recurrence of NAION in the same eye were analyzed. RESULTS: We found that most cases of DA-NAION occur in relatively young patients (47.7 ± 14.7 years) with a high rate of bilateral involvement at presentation (23%) and bilateral sequential involvement (39%). Vision loss is severe with 64% of patients presenting with 20/200 acuity or worse in the involved eye and 19% of patients with final visual acuity of 20/200 or worse in both eyes. 3 patients (9.7%) had recurrence of NAION in the previously affected eye. CONCLUSIONS: Neuro-ophthalmologists have an important role in identifying patients who have suffered DA-NAION and communicating their findings to nephrologists to minimize the chance of involvement of the fellow eye and recurrence in the same eye. Intradialytic blood pressure must be closely monitored, and fluid balance, dialysate composition, and dialysis protocol must be optimized to prevent occurrence of intradialytic hypotension, which is the culprit for DA-NAION.

Paracentral Acute Middle Maculopathy Associated With Anterior Ischemic Optic Neuropathy and Cilioretinal Artery Occlusion in Giant Cell Arteritis.

ABSTRACT: Paracentral acute middle maculopathy (PAMM) is a relatively new optical coherence tomography finding, defined by hyperreflectivity in the inner nuclear layer. In this article, we present a case of a 73-year-old woman who presented with transient vision loss followed by the sudden onset of complete vision loss to counting fingers at 1 foot for one day in the left eye. Dilated examination showed a right cotton wool spot, left pallid optic disc edema, and retinal edema in the distribution of the cilioretinal artery. OCT demonstrated hyperreflective band at the level of the inner nuclear layer, compatible with PAMM. Clinical and laboratory findings were consistent with GCA, for which she was prescribed high-dose oral prednisone, with confirmation of GCA on a subsequent temporal artery biopsy. PAMM may be seen in the context of GCA, and OCT of the macula serves as an important adjunct to define the retinal manifestations of this condition.

Sectoral Sparing Associated With a Cilioretinal Artery in Arteritic Anterior Ischemic Optic Neuropathy.

ABSTRACT: Giant cell arteritis (GCA) is a life-threatening vasculitis occurring in older adults that can cause blindness by ischemia of the choroid, retina, and optic nerve. We report a case of a patient who presented with "occult" GCA with severe anterior ischemic optic neuropathy affecting both optic nerves, delayed choroidal filling, and a concomitant cilioretinal artery occlusion in the left eye. The retinal territory supplied by the affected cilioretinal artery was hypoperfused, yet this retinal territory at least partially corresponded to the only preserved visual field in that eye. The sector of the optic disc corresponding to the emergence of the cilioretinal artery was the only sector spared by pallid edema. This pattern of sectoral sparing associated with a cilioretinal artery has been observed in other patients with GCA and in animal models of posterior ciliary artery occlusion. This case serves as a clear example of an incompletely understood phenomenon in posterior pole circulation in vascular occlusive disease that deserves further study.