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Retinal artery occlusion (RAO) is an ophthalmic emergency that can lead to poor visual outcomes and is associated with an increased risk of stroke and cardiovascular events. Wide-field swept-source OCT-A (WF SS-OCTA) can provide quick and non-invasive angiographic information with a wide field of view. Here, we looked for associations between OCT-A vascular imaging metrics and vision in RAO patients.PurposeRetinal artery occlusion (RAO) is an ophthalmic emergency that can lead to poor visual outcomes and is associated with an increased risk of stroke and cardiovascular events. Wide-field swept-source OCT-A (WF SS-OCTA) can provide quick and non-invasive angiographic information with a wide field of view. Here, we looked for associations between OCT-A vascular imaging metrics and vision in RAO patients.Patients with diagnoses of central (CRAO) or branched retinal artery occlusion (BRAO) were included. 6mm × 6mm Angio and 15mm × 15mm AngioPlex Montage OCT-A images were obtained for both eyes in each patient using Zeiss Plex Elite 9000 WF SS-OCTA device. Each 6mm × 6mm image was divided into nine Early Treatment Diabetic Retinopathy Study (ETDRS) subfields. Non-perfusion area (NPA) was manually measured using 15mm × 15mm images. A linear regression model was utilized to identify correlation between imaging metrics and vision. P-values less than 0.05 were considered as statistically significant.MethodsPatients with diagnoses of central (CRAO) or branched retinal artery occlusion (BRAO) were included. 6mm × 6mm Angio and 15mm × 15mm AngioPlex Montage OCT-A images were obtained for both eyes in each patient using Zeiss Plex Elite 9000 WF SS-OCTA device. Each 6mm × 6mm image was divided into nine Early Treatment Diabetic Retinopathy Study (ETDRS) subfields. Non-perfusion area (NPA) was manually measured using 15mm × 15mm images. A linear regression model was utilized to identify correlation between imaging metrics and vision. P-values less than 0.05 were considered as statistically significant.Twenty-five subjects were included. For RAO eyes, there was a statistically significant inverse correlation between retinal thickness as well as superficial capillary plexus (SCP) vessel density (VD) and vision. An inverse correlation was found between deep capillary plexus (DCP) VD and vision without statistical significance. There was a positive correlation between choroidal thickness as well as choroidal volume and vision without statistical significance. No significant correlation was found between the metrics and vision in contralateral eyes. For NPA and vision, no significant correlation was identified.ResultsTwenty-five subjects were included. For RAO eyes, there was a statistically significant inverse correlation between retinal thickness as well as superficial capillary plexus (SCP) vessel density (VD) and vision. An inverse correlation was found between deep capillary plexus (DCP) VD and vision without statistical significance. There was a positive correlation between choroidal thickness as well as choroidal volume and vision without statistical significance. No significant correlation was found between the metrics and vision in contralateral eyes. For NPA and vision, no significant correlation was identified.This is the first study to investigate the utility of WF SS-OCTA in RAO and to demonstrate correlations between retinal vascular imaging metrics and visual outcomes. The results of this study provide a basis to understand the structural changes involved in vision in RAO and may guide management of RAO and prevention of cerebral stroke and cardiovascular accidents.ConclusionThis is the first study to investigate the utility of WF SS-OCTA in RAO and to demonstrate correlations between retinal vascular imaging metrics and visual outcomes. The results of this study provide a basis to understand the structural changes involved in vision in RAO and may guide management of RAO and prevention of cerebral stroke and cardiovascular accidents.

Background Susac syndrome (SuS) is a rare disorder thought to be caused by autoimmune-mediated occlusions of microvessels in the brain, retina and inner ear leading to central nervous system (CNS) dysfunction, visual disturbances due to branch retinal artery occlusions (BRAO), and hearing deficits. Recently, a role for anti-endothelial cell antibodies (AECA) in SuS has been proposed. Objectives To report the clinical and paraclinical findings in the largest single series of patients so far and to investigate the frequency, titers, and clinical relevance of AECA in SuS. Patients and methods A total of 107 serum samples from 20 patients with definite SuS, 5 with abortive forms of SuS (all with BRAO), and 70 controls were tested for AECA by immunohistochemistry employing primate brain tissue sections. Results IgG-AECA >1:100 were detected in 25% (5/20) of patients with definite SuS and in 4.3% (3/70) of the controls. Median titers were significantly higher in SuS (1:3200, range 1:100 to 1:17500) than in controls (1:100, range 1:10 to 1:320); IgG-AECA titers >1:320 were exclusively present in patients with SuS; three controls had very low titers (1:10). Follow-up samples (n = 4) from a seropositive SuS patient obtained over a period of 29 months remained positive at high titers. In all seropositive cases, AECA belonged to the complement-activating IgG1 subclass. All but one of the IgG-AECA-positive samples were positive also for IgA-AECA and 45% for IgM-AECA. SuS took a severe and relapsing course in most patients and was associated with bilateral visual and hearing impairment, a broad panel of neurological and neuropsychological symptoms, and brain atrophy in the majority of cases. Seropositive and seronegative patients did not differ with regard to any of the clinical or paraclinical parameters analyzed. Conclusions SuS took a severe and protracted course in the present cohort, resulting in significant impairment. Our finding of high-titer IgG1 and IgM AECA in some patients suggest that humoral autoimmunity targeting the microvasculature may play a role in the pathogenesis of SuS, at least in a subset of patients. Further studies are warranted to define the exact target structures of AECA in SuS.

Aims To review clinical experience, efficacy, and safety of transluminal Nd:YAG laser embolectomy/embolysis (TYE) for retinal artery occlusion. Methods: Electronic databases were searched for all published clinical studies and case-reports reporting on TYE in central (CRAO) or branch (BRAO) retinal artery occlusion. Individual patient data was evaluated in a weighted pooled analysis. Results Sixty-one cases were reported, 47 with BRAO and 14 with CRAO. Visual acuity (VA) at onset averaged 20/252 (1.1 LogMAR) and improved following the procedure to 20/47 (0.37 LogMAR) at first follow-up (avg. 6 days, P  < 0.001) and to 20/30 (0.18 LogMAR) at last reported follow-up (avg. 1.1 years, P  = 0.02). Patients with worse VA (<20/200) improved further (12 vs. three lines, P  < 0.001). VA was not improved when using higher pulse energies (≥ 2.4 mJ) which were associated with more vitrectomies. In a weighted analysis vitreous/sub-retinal hemorrhage was estimated to occur in 54% of cases and required vitrectomy in 18% of cases. Conclusions TYE was followed by significant visual improvement in the vast majority of cases, including CRAO, and was frequently associated with vitreous hemorrhage. Patients with poor visual acuity appear to benefit further and higher pulse energies may be detrimental. Lack of randomization and intrinsic biases prevent any definite conclusions regarding the benefits and further research is warranted.

Background Branch retinal artery occlusion (BRAO) is an acute, vision-threatening condition that often signals underlying systemic vascular disease and the need for urgent vascular risk assessment and mitigation. Although prompt evaluation is critical for accurate diagnosis and early identification of modifiable vascular risk factors, patterns of clinical presentation remain poorly characterized. Methods This retrospective study included BRAO patients who presented to Kaiser Permanente Northern California (KPNC) within 30 days of symptom onset from 2014 to 2023. Demographic data, symptom timing, healthcare contact, and ophthalmologic evaluation were collected. The type of contact was categorized as eye care provider, call center, emergency department/urgent care, or other. Delays in presentation were defined as time from symptoms to initial contact and were analyzed across subgroups. Results From 2014 to 2023, 760 patients were diagnosed with acute BRAO. Mean age of the study population was 70.2 ± 12.6 years, and 330 (43.4%) were female. Initial contact most commonly occurred with eye care providers (370; 48.7%), followed by call centers (251; 33.0%), emergency/urgent care (73; 9.6%), and other providers (66; 8.7%). Only 219 (28.8%) presented within 1 day of symptom onset, while 97 (12.8%) presented after 8 days. The majority (541; 71.2%) were not evaluated by an eye care provider until > 24 h after symptom onset. Among the 153 (20.1%) with known time of symptom onset, median delay to any healthcare contact was 4.0 h. 80 (52.3%) patients presented within 4.5 h, but just 5 (6.3%) were seen by an eye care provider within that window. Conclusions In a large, multi-center, community population, delays in BRAO care were common. Although no Level 1 evidence currently exists to support BRAO treatment, success of future therapies will likely require early administration. Prompt evaluation is also critical to enable risk assessment and mitigation. Most patients in this cohort presented in a delayed fashion, underscoring the need for public education and workflow improvements to support earlier recognition and care. Précis/Summary statement Patients with branch retinal artery occlusion often experience delays in care. Most are not evaluated promptly by eye care providers, which may preclude timely diagnosis, stroke prevention, and potential future therapies.

The combination of branch retinal vein occlusion (BRVO) and branch retinal artery occlusion (BRAO) is extremely rare. At present, the pathogenic mechanism underlying the occurrence of combined BRVO and BRAO remains unclear. Prompt treatment of macular edema (ME) secondary to combined BRVO and BRAO with anti-vascular endothelial growth factor (anti-VEGF) therapy and laser photocoagulation can achieve a satisfactory prognosis. This report presents a rare case of ME secondary to combined BRVO and BRAO treated with anti-VEGF agents and laser photocoagulation. A 59-year-old man presented with a 3-month history of blurred vision in the left eye. The patient had a 5-year history of diabetes and hypertension. On examination, the best-corrected visual acuity (BCVA) was 20/20 in the right eye and 20/125 in the left eye, and the intraocular pressure in both eyes was within the normal range. Slit lamp examination revealed unremarkable anterior segments in both eyes, except for lens opacity. According to the fundus examination findings, a diagnosis of ME secondary to combined BRVO and BRAO in the left eye was established. The central macular thickness (CMT) in the left eye was 302 μm. The patient subsequently underwent angiography-guided sectoral laser photocoagulation and received intravitreal injections of anti-vascular endothelial growth factor (anti-VEGF) agents in the left eye. After 3 months of treatment, the BCVA in the left eye improved to 20/40, the CMT decreased to 182 μm, and the intraretinal fluid was resolved. Thereafter, the patient did not return to the clinic for further ophthalmic examinations. Combined BRVO and BRAO is an uncommon and extremely rare retinopathy. Intravitreal injection of anti-VEGF drugs and laser photocoagulation are effective treatments for ME secondary to combined BRVO and BRAO. In addition, systemic evaluation and close monitoring of cardiovascular risk factors should not be overlooked.

Purpose: To systematically review the evidence regarding the characteristics of Optical Coherence Tomography Angiography (OCTA) in acute retinal arterial occlusion (RAO), with a particular focus on vascular alterations across the superficial and deep capillary plexuses, choroid, and peripapillary regions. Methods: A comprehensive literature search was performed across PubMed, Web of Science, Scopus, EMBASE, Google Scholar, and the Cochrane Database up to April 2025. The search terms included “Optical coherence tomography angiography,” “OCTA,” “Retinal arterial occlusion,” “Central retinal artery occlusion,” and “Branch retinal artery occlusion.” Studies were included if they evaluated the role of OCTA in diagnosing or assessing acute RAO. Case reports, conference abstracts, and non-English articles were excluded. Two reviewers independently conducted the study selection and data extraction. The methodological quality of the included studies was assessed using the Risk of Bias in Non-randomized Studies of Interventions (ROBINS-I) tool. Results: The initial search yielded 457 articles, from which 10 studies were ultimately included in the final analysis after a rigorous screening process excluding duplicates, non-English publications, and ineligible articles based on title, abstract, or full-text review. The included studies consistently demonstrated that OCTA is a valuable, noninvasive modality for evaluating microvascular changes in RAO. Key OCTA findings in acute RAO include significant perfusion deficits and reduced vessel density in both the superficial capillary plexus (SCP) and deep capillary plexus (DCP). Several studies noted more pronounced involvement of the SCP compared to the DCP. OCTA parameters, such as vessel density in the macular region, have been found to correlate with visual acuity, suggesting a prognostic value. While findings regarding the foveal avascular zone (FAZ) were mixed, the peripapillary area frequently showed reduced vessel density. Conclusion: Acute RAO is an ocular emergency that causes microvascular ischemic changes detectable by OCTA. This review establishes OCTA as a significant noninvasive tool for diagnosing, monitoring, and prognosticating RAO. It effectively visualizes perfusion deficits that correlate with clinical outcomes. However, limitations such as susceptibility to motion artifacts, segmentation errors, and the lack of standardized normative data must be considered. Future standardization of OCTA protocols and analysis is essential to enhance its clinical application in managing RAO.

Background/purposeSusac syndrome is a rare microangiopathy of suspected autoimmune origin affecting arteries of the retina, the cochlea and the brain. The aim of the study was to give a review of the disease entity and determine the proportion of cases and their characteristics in a uveitis referral centre.Patients and methodsCharts of patients with the diagnosis of Susac syndrome seen in the Uveitis Clinic of the Centre for Ophthalmic Specialised Care (COS), Lausanne, Switzerland were reviewed retrospectively to determine the frequency of such cases in a uveitis referral centre. Clinical symptoms and signs, functional data, imaging signs and evolution were analysed in the 3 COS cases and one case shared with the Uveitis Clinic of the Department of Ophthalmology, University of Innsbruck, Austria. Characteristic signs were searched possibly allowing a prompt diagnosis.ResultsDuring the period from 1994 to 2019 (24 years, 2045 patients), 3 charts with the diagnosis of Susac syndrome were found (0.15%). The whole collective, including the additional case, comprised three women aged 28, 32 and 63 at presentation and one man, aged 42. None of the 3 cases that were referred were diagnosed beforehand. The characteristic item found in all 4 cases was the abrupt arterial stop or segmental interruption of arteries and increased staining of arterial wall on angiography more clearly shown on indocyanine green angiography that can potentially be proposed as a crucial diagnostic element. All 4 cases responded to dual steroidal and non-steroidal immunosuppression. Under treatment, all four patients did not show any further evolution.ConclusionSusac syndrome is a multilocation arteritis of the head that can involve the eye, ear and brain often first diagnosed by the ophthalmologist. The diagnosis is rapidly reached in uveitis referral centres but seems to be missed otherwise, A helpful angiographic sign to be searched is an abrupt or segmental arterial stop and increased staining of the arterial wall more clearly seen on indocyanine green angiography. Patients often present first to the ophthalmologist who should be acting as a whistleblower to avoid severe involvement of the brain.

Susac's syndrome is a rare microangiopathy affecting small vessels of the retina, inner ear and brain. It is characterised by a triad of symptoms: encephalopathy, visual defects, and sensorineural hearing loss. The disease is probably caused by an autoimmune process. Diagnosis is based on the typical symptoms, brain MRI, and, most importantly, fluorescein angiography. It is important to distinguish between Susac's syndrome and multiple sclerosis or migraine with aura, because misdiagnosis leads to the wrong treatment. To date, no detailed guidelines for the treatment of Susac's syndrome have been developed. Immunosuppression seems to be effective. It must be remembered that early and aggressive treatment is crucial, and that delays in diagnosis, and as a result in treatment implementation, worsen the prognosis.