This is the first Canadian study to examine practice patterns and perceptions of central retinal artery occlusion (CRAO) management among neurologists. An online survey was distributed nationwide to neurologists practicing in hyperacute stroke in Canada. Of 64 respondents, over half reported never having provided thrombolysis, although there was willingness to provide it if patients met the criteria. Ophthalmology support was deemed necessary in most cases of suspected CRAO to facilitate thrombolysis; however, virtual consultation would always be accepted by 41% of providers. These survey findings help to understand multidisciplinary practice patterns to develop educational strategies and inform future frameworks to guide care for patients with CRAO.

We report a case of unilateral presumed nonarteritic anterior ischemic optic neuropathy (NAION) associated with acute posterior multifocal placoid pigment epitheliopathy (APMPPE).

Giant cell arteritis (GCA) is a systemic granulomatous vasculitis of medium and large-sized arteries, which represents an ophthalmic emergency because of the risk of sudden, severe and often irreversible loss of vision. Its most important ocular manifestation is arteritic anterior ischemic optic neuropathy; less frequent manifestations include central retinal artery occlusion, cilioretinal artery occlusion, posterior ischemic optic neuropathy, choroidal ischemia and ocular motor nerve palsies. Giant cell arteritis should be actively considered in every patient over 50 years of age presenting with new-onset ischemic optic neuropathy, amaurosis fugax, diplopia or another cranial ischemic manifestation. The diagnosis is based on a combination of the clinical findings, inflammatory laboratory markers, ultrasonography of the temporal and axillary arteries, temporal artery biopsy when appropriate, and additional imaging modalities. However, in patients with high clinical suspicion, treatment must not be deferred until definitive diagnostic confirmation. Systemic glucocorticoids remain the cornerstone of acute therapy; in patients with ocular ischemic involvement, intravenous methylprednisolone pulses followed by high-dose oral glucocorticoids are usually recommended. New glucocorticoid-sparing treatment options, particularly interleukin-6 receptor blockade with tocilizumab and JAK1 inhibition with upadacitinib, expand the possibilities for long-term disease control and reduction of cumulative steroid exposure. Early identification of the pathology by the ophthalmologist and close interdisciplinary collaboration with rheumatologists, internists, neurologists, infectious disease and imaging specialists are essential to prevent bilateral visual loss and systemic ischemic complications.

Purpose: To investigate whether glucagon-like peptide-1 receptor agonists are protective against retinal vascular occlusions. Methods: Using a large aggregated, de-identified electronic health records data platform in the United States, patients with type 2 diabetes ages ≥18 years prescribed glucagon-like peptide-1 receptor agonists or non-glucagon-like peptide-1 receptor agonists (controls) and having ophthalmology follow-up of at least 1, 3, or 5 years were included. The glucagon-like peptide-1 receptor agonist and control cohorts were propensity score matched (1:1) on demographics and risk factors, and outcomes were compared at 1, 3, and 5 years. Hazard ratios (HRs) with 95% CIs were reported, with significance defined as P < .05. Results: After matching, each cohort comprised 53 413 patients after 1 year, 25 738 patients after 3 years, and 14 695 patients after 5 years. Significantly reduced risk of all vascular occlusions was observed in the glucagon-like peptide-1 receptor agonist cohort at 1 year (HR, 0.66, 95% CI, 0.54-0.80), 3 years (HR, 0.71, 95% CI, 0.59-0.86), and 5 years (HR, 0.79, 95% CI, 0.64-0.97). Retinal vein occlusion risk was significantly reduced in the glucagon-like peptide-1 receptor agonist cohort up to 3 years (HR, 0.75, 95% CI, 0.60-0.94), but not after 5 years (HR, 0.83, 95% CI, 0.64-1.08). While there was no reduction in retinal artery occlusion risk at 1 year (HR, 0.69, 95% CI, 0.48-1.00), there were significant reductions at later time points. Conclusions: Results suggest that glucagon-like peptide-1 receptor agonists may reduce the risk of retinal vascular occlusions. Additional prospective trials and analysis of long-term outcomes are needed to further investigate this association.

Branch retinal artery occlusion (BRAO) is an extremely rare vascular event in the pediatric population. Unlike elderly patients, in whom atherosclerosis is the primary cause, BRAO in children and adolescents is often associated with systemic conditions, including hypercoagulation and genetic predisposition to thrombosis. Ophthalmoscopy and OCT revealed local ischemic retinal edema along the inferior temporal branch of the central retinal artery. Systemic examination and molecular genetic testing revealed combined genetic thrombophilia: heterozygous mutation of the MTHFR gene (A1298C) and homozygous PAI-1 polymorphism (4G/4G). There was also an increase in the level of D-dimer (0.65 µg/ml). The patient received comprehensive conservative therapy aimed at improving microcirculation in the retinal vessels, dilating capillaries, lowering intraocular pressure, stabilizing the rheological properties of blood, and enhancing neuroprotection. Despite the fact that the best-corrected visual acuity (BCVA) was restored to 1.0, there was a persistent absolute scotoma in the upper nasal quadrant, corresponding to the area of secondary atrophy of the inner retinal layers in the affected zone recorded on follow-up OCT. This case highlights the clinical importance of a multidisciplinary approach in the treatment of retinal vascular occlusions in children. BRAO in a child should be considered as a potential marker of serious systemic disease, in particular hereditary thrombophilia, which requires early intervention to prevent further complications.

Primary myelofibrosis (PMF) is a rare myeloproliferative neoplasm that typically affects older adults but may present aggressively in younger patients. Thromboembolic complications are well recognized, though arterial occlusions are uncommon in this age group. Ocular ischemia associated with systemic inflammatory conditions such as Sweet's syndrome is exceedingly rare. A 22-year-old female with PMF, diagnosed 18 months earlier and managed initially with ruxolitinib, presented with sudden, complete loss of vision in her left eye. She also had a history of Sweet's syndrome managed with oral corticosteroids and had received multiple blood transfusions. Ophthalmic evaluation revealed no light perception in the left eye, a pale retina with attenuated vessels, and a cherry-red spot. Optical coherence tomography (OCT) showed hyperreflectivity of the inner retinal layers, and OCT angiography demonstrated markedly reduced perfusion in all vascular plexuses. Carotid Doppler, echocardiography, and ECG excluded embolic sources. Despite ocular massage, anterior chamber paracentesis, acetazolamide, and anticoagulation with apixaban, vision did not recover. One week later, she developed anterior uveitis, keratic precipitates, and elevated intraocular pressure. Fundus showed Amalric's sign of choroidal infarction and Elschnig spots, confirmed on fluorescein angiography. Inflammation responded to topical and systemic corticosteroids, with normalization of intraocular pressure. This is the first documented case of combined central retinal artery occlusion and posterior ciliary artery occlusion with anterior uveitis in a young PMF patient with Sweet's syndrome. The case underscores the multifactorial contribution of hypercoagulability, systemic inflammation, and transfusion-related factors in ocular ischemia.