Central retinal artery occlusion in a young child affected by COVID-19: a first case report.

BACKGROUND: Central retinal artery occlusion (CRAO) is an ophthalmic emergency, and its etiology is generally ascribed to vessel occlusion by a thrombus or embolus, eventually due to a hypercoagulable state. CRAO occurrence is described even in the pediatric population, but its incidence is very rare. SARS-CoV-2 infection has a multitude of presentations, and almost any organ may be involved including the ocular district. Cases of CRAO in patients affected by COVID-19 are reported in the literature in the adult population, but not in the pediatric one. CASE PRESENTATION: We describe the case of a six-year-old otherwise healthy girl, who presented a sudden and complete bilateral vision loss after a one-day fever. All the clinical, ophthalmological, laboratory and instrumental investigations led to the diagnosis of a right CRAO and the suspicion of a contralateral posterior optic nerve affection. These manifestations could not be ascribed to any etiological condition apart from the documented ongoing mild SARS-CoV-2 infection. Treatment with anticoagulants and steroids was tried but the visual outcome was poor during the one-month hospitalization and at the last follow-up. CONCLUSIONS: To the best of our knowledge, this is the first report of CRAO in the course of SARS-CoV-2 infection in the pediatric age. In our review of the literature, we found few cases of CRAO in adults with COVID-19; we highlighted differences in anamnestic, clinical, and interventional aspects and therefore we tried to summarize the state of the art on this topic to facilitate further studies. Even if rare, the prognosis of CRAO is poor and the thrombolytic treatment could be effective only if rapidly administered, so the disease suspicion should be high in a patient with sudden vision loss, also in pediatric age.

Acute unilateral vision loss from central retinal artery occlusion associated with an atrial septal aneurysm and a patent foramen ovale: a case report.

BACKGROUND: Acute unilateral vision loss is an ophthalmological emergency. It can have multiple aetiologies, and physicians must be able to quickly determine the cause of the condition, as immediate intervention may prevent permanent vision loss. Acute unilateral vision loss has not previously been associated with a patent foramen ovale (PFO). OBJECTIVE: We describe a patient who presented with painless sudden loss of vision in his left eye. He was diagnosed to have central retinal artery occlusion, likely from a paradoxical embolus associated with an atrial septal aneurysm containing a PFO. The patient underwent successful percutaneous closure of the PFO without complication. This report details the evaluation and differential diagnosis of acute unilateral vision loss. Additionally, the controversy of whether a PFO should be closed prophylactically for stroke prevention is discussed. CONCLUSION: Acute unilateral vision loss from central retinal artery occlusion may be caused by a paradoxical embolus originating from an atrial septal aneurysm with a PFO. This case should be considered in the controversy of whether prophylactic closure of a PFO could be beneficial for primary prevention of stroke.

Sudden loss of vision in one eye is an emergency, and rapid evaluation and treatment are essential. We report a case of acute vision loss in one eye that was associated with a communication between the right and left receiving chambers of the heart, with blood clot at the communication. It was assumed that clot passing from the venous to arterial side of the heart, was carried in the blood stream, and subsequently blocked the main artery to the affected eye. The communication was closed with a device, without the need for surgery. This potential cause of vision loss has not previously been reported. It is important that physicians search for this type of communication in the heart when there is abrupt vision loss in one eye. The merits of closing such a communication is discussed.

Central Retinal Artery Occlusion: Visual Outcomes from a Large Northern California Cohort.

PURPOSE: To assess visual acuity (VA) outcomes in a large cohort of patients diagnosed with nonarteritic central retinal artery occlusion (CRAO), and to ascertain whether time from symptom onset to presentation, presenting VA, or conservative treatment delivery (anterior chamber paracentesis, ocular massage, intraocular pressure lowering drugs, hyperventilation, or some combination of those) impacted ultimate VA outcomes. DESIGN: Retrospective cohort study. SUBJECTS: The study included 794 patients who presented with CRAO between 2011 and 2020. Within this cohort, 484 individuals presented within 30 days of symptom onset and had comprehensive documentation regarding the details of their presentation, management, and follow-up ≥ 90 days postdiagnosis. METHODS: Retrospective chart review was conducted for all patients with a diagnosis of CRAO initially identified via International Classification of Diseases coding, followed by confirmation of diagnosis by 2 retina specialists. Cases of arteritic CRAO were excluded. MAIN OUTCOME MEASURES: Visual acuity recovery, defined as improvement from ≤ 20/200 or worse at presentation to ≥ 20/100 ≥ 90 days after diagnosis. RESULTS: Of the 794 identified patients, 712 (89.7%) presented with VA of ≤ 20/200. Similarly, 447 (92.4%) of the 484-patient subset that presented within 30 days and had comprehensive documentation presented with VA ≤ 20/200. Of the 441 of those patients with documented follow-up, 380 (86.2%) remained at that level. Of the 244 patients who presented within 4.5 hours of symptom onset, 227 (93%) presented ≤ 20/200 and 201 (92.6%) of the 217 of those with follow-up data did not improve beyond that threshold. There was no significant difference (P < 0.05) in final VA between patients presenting before versus after 4.5 hours from time of vision loss. There was also no significant difference (P < 0.05) in VA outcomes between patients who did or did not receive conservative treatment. CONCLUSIONS: This large retrospective study further highlights the poor visual prognosis for patients with CRAO. Earlier time to presentation did not seem to impact final VA outcome, nor did conservative treatment efforts. Efficacious evidence-based treatment options are needed for this patient population. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

A Triggering Event of Central Retinal Artery Occlusion With Concurrent Ischemic Stroke.

We report a case of central retinal artery occlusion with concurrent ischemic stroke in a young patient. A 34-year-old Malay gentleman, an ex-smoker with underlying dyslipidemia, however, not on medication or follow-up, presented with acute, generalized, and painless right eye blurring of vision for one day. He also complained of on-and-off headaches for the past three months prior to the presentation. Visual acuity assessment demonstrated hand movement in the right eye, whereas in the left eye, it was 6/6, along with a right eye relative afferent pupillary defect. His right eye showed reduced optic nerve function and unremarkable anterior segment, with fundus examination revealing the presence of a cherry red spot, pale macula, boxcarring pattern over superior arcuate, and vascularized retina over inferior optic disc with blurred optic disc margin. The left eye examination was unremarkable. All cranial nerves were intact, except for the optic nerve. He was admitted to the ward. While in the ward, he developed a sudden onset of left-sided upper and lower limb weakness and numbness and was diagnosed with acute ischemic stroke. Blood investigations showed raised low-density lipoprotein cholesterol of 3.51 mmol/L, anti-nuclear antibody (ANA) positive, with electrocardiogram (ECG) sinus rhythm, and no atrial fibrillation. The echocardiogram was normal, and computed tomography angiography of the brain showed non-opacification at the origin and proximal part of the right ophthalmic artery, suspicious of thrombosis with distal reconstitution, with no evidence of thrombosis in the rest of neck and intracranial arteries. The patient was started on aspirin 150 mg once a day and atorvastatin 20 mg at night; subsequently, his vision improved slightly.

An Unusual Ocular Manifestation of Disseminated Tuberculosis: Combined Retinal Vascular Occlusion Inducing Acute Loss of Vision.

Disseminated tuberculosis (TB) is a life-threatening disease caused by the hematogenous spread of Mycobacterium tuberculosis. Acute loss of vision as a symptom of disseminated TB is uncommon, as per the literature. Uveitis is the most common ocular manifestation of TB, and tubercular retinal arterial or venous occlusion, with or without ocular signs, has been rarely described before. We discuss the case of a 34-year-old truck driver who presented with fever, cough, and sudden painless loss of vision in the right eye. Examination revealed optic neuropathy, as well as central retinal artery and venous occlusion. Investigations showed bilateral miliary shadows on chest X-ray and multiple ring-enhancing brain lesions on MRI brain, consistent with disseminated TB. Anti-tubercular therapy led to clinical improvement. We report this case to highlight the rarity of this condition.

Central Retinal Artery Occlusion: Time to Presentation and Diagnosis.

OBJECTIVE: To evaluate the presentation patterns of patients diagnosed with central retinal artery occlusion (CRAO) from 2011 to 2020. DESIGN: Retrospective cohort study SUBJECTS: The present study was conducted in 484 patients presenting within 30 days of symptom onset with accurate documentation of time of symptom onset, time of presentation to the health care system, and time of presentation to an ophthalmologist. METHODS: An independent chart review of patients with CRAO was conducted. MAIN OUTCOME MEASURES: Demographic information including age, sex, and race were collected. Presentation patterns such as time of first symptoms, time of first contact with the health care system, and time of evaluation by an ophthalmologist were analyzed. Additionally, information regarding the medical venue or specialty of initial patient contact was collected. RESULTS: A total of 247 (51%) patients contacted the health care system within 4.5 hours of system onset, whereas 86 (17.8%) patients waited over 24 hours. Only 81 (32.8%) of the 247 patients who presented within 4.5 hours saw an ophthalmologist within that time frame, whereas 172 (35.5%) of the entire cohort of 484 did not present to an ophthalmologist within 24 hours of vision loss. There was significant variability with regards to medical specialty of initial patient contact, with 292 (60.3%) patients first presenting to an emergency department and 133 (27.5%) patients first presenting to an ophthalmologist. Black and Hispanic patients presented later than patients of White, Asian, or other racial backgrounds (40.4 ± 10.2 hours versus 23.0 ± 3.4 hours, P = 0.05). CONCLUSIONS: Although no level 1 evidence-based treatment is currently available for CRAO, thrombolytic therapy may be promising. Even though over half of patients with CRAO within our institution connected with the health care system within a potential window for thrombolytic therapy, most did not receive a definitive ophthalmic diagnosis within that time frame. Public health educational campaigns and infrastructure optimization must speed up presentation times, decrease the time to ophthalmic diagnosis, and target vulnerable populations to offer and research timely administration of thrombolytic therapy. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any materials discussed in this article.

The diagnosis and treatment of central retinal artery occlusion with severe cardio-cerebrovascular disease: a case report.

Background: Central retinal artery occlusion (CRAO) is an acute eye disease that seriously damages vision. Patients with CRAO often have a combination of various cardio-cerebrovascular diseases (CCVDs), and CRAO patients often ignore their cardio-cerebrovascular disorders because of their ocular symptoms. In addition, there are few reports about CRAO patients with CCVDs received effective interventions implemented. We report the diagnosis and treatment of a Chinese CRAO patient with CCVD who received timely multidisciplinary interventional therapy to provide ideas for clinical ophthalmologists in the diagnosis and treatment of similar diseases. Case Description: A 76-year-old male patient, who had previously been diagnosed with hypertension, was admitted to hospital due to a sudden decrease in vision in his right eye for >2 days with a severe headache. After fundus photography, he was diagnosed with CRAO in the right eye. His cerebral angiography revealed multiple stenoses at arteries of his neck and brain included the right ophthalmic artery. Neurosurgery was attempted to perform a thrombolysis of the right ophthalmic artery while performing the angiography, but failed to find the opening of the right ophthalmic artery. However, through electrocardiogram (ECG) monitoring during the operation, we found that the patient had frequent ventricular premature beats, so the Department of Cardiology performed coronary arteriography for him which revealed severe stenosis of the left anterior descending (LAD) artery. The cardiologists performed a percutaneous coronary intervention (PCI) at the same time as the coronary angiography. Some 2 months later, the patient was admitted to the Neurosurgery Department to implant stent at the left vertebral artery. After stent implantation, his headache symptom improved significantly and his right eye vision improved. Conclusions: Through timely cerebral angiography and ophthalmic examinations, the patient was diagnosed with CRAO combined with CCVD, and after received multidisciplinary interventional therapy, the patient's right eye vision and headache symptom improved and more severe cardio-cerebrovascular adverse events were avoided. In treating CRAO patients, in addition to aggressive eye treatment, the systemic cardio-cerebrovascular situation of each patient should also be assessed, a timely diagnosis made, and effective interventions implemented to reduce morbidity- and mortality-related cardio-cerebrovascular events.

Intravenous Thrombolysis for Central Retinal Artery Occlusion: A Look at the Literature for the Emergency Medicine Physician.

Central retinal artery occlusion (CRAO) is a subtype of ischemic stroke and true ocular emergency presenting with acute, painless, monocular vision loss. Typical findings include poor visual acuity (VA), impaired color vision, relative afferent pupillary defect, and on fundoscopic evaluation, retinal edema, cherry red spot, and occasionally visualization of retinal artery emboli. While there are no proven treatments for CRAO, options include orbital massage, hyperbaric oxygen therapy, and intra-arterial or intravenous thrombolysis (IVT). This study reviews the current literature on the efficacy of IVT for patients affected by acute, symptomatic CRAO and provides an up-to-date, evidence-based background for emergency physicians (EPs) who evaluate and manage these patients.

COVID-19-Related Rhino-Orbital-Cerebral Mucormycosis With Angioinvasion: A Lethal Sequelae.

A middle-aged man with multiple comorbidities including uncontrolled diabetes mellitus presented with shortness of breath and lethargy for six days. He was treated for COVID-19 pneumonia, requiring high cumulative steroid therapy. After 15 days of treatment, he developed right orbital apex syndrome with central retinal artery occlusion secondary to invasive mucormycosis. The infection progressed rapidly despite aggressive medical treatment, systemic anti-fungal therapy along with local transcutaneous retrobulbar amphotericin B injection. We report our battle in fighting this vicious disease. Judicious use of immunomodulators in COVID-19 treatment and close monitoring is crucial, especially in high-risk patients.

Neovascular Glaucoma Complicating Central Retinal Artery Occlusion Despite Cilioretinal Artery Sparing.

This case report aims to describe a case of unilateral central retinal artery occlusion (CRAO) with cilioretinal artery sparing, which was complicated by neovascular glaucoma (NVG). A 75-year-old Indian woman with underlying normal tension glaucoma presented with the sudden onset of painless generalized blurring of the right eye's vision for a week. Her right eye vision was hand motion with the presence of a right relative afferent pupillary defect. Fundus examination revealed retinal whitening over the macula sparing the papillomacular bundle with generalized retinal arteriolar attenuation, which was suggestive of right CRAO with cilioretinal artery sparing. Systemic examination revealed high blood pressure (175/75 mmHg) without ocular bruit or audible murmur on auscultation. Optical coherence tomography of the macula showed inner retinal thickening over the temporal macula. Ultrasound carotid Doppler and computed tomography angiography of the carotid showed more than 75% stenosis over the right distal internal carotid artery. Unfortunately, she developed rubeosis iridis over her right eye two weeks after her presentation, which required pan-retinal photocoagulation. She subsequently progressed to NVG, requiring maximum anti-glaucoma medications to stabilize intraocular pressure. In conclusion, CRAO is a sight-threatening medical emergency. Thorough investigations are required to determine the underlying cause so that early intervention can be done to reduce the risk of a similar attack in the fellow eye and the risk of a cerebrovascular event or cardiac ischemia, which could be life-threatening. The presence of a cilioretinal artery does not prevent ocular neovascularization in CRAO. Hence, patients should also be closely monitored after the initial diagnosis to prevent devastating complications such as NVG.

Central Retinal Artery Occlusion: Current Practice, Awareness and Prehospital Delays in Switzerland.

Background and Purpose: Central retinal artery occlusion (CRAO) often leads to permanent monocular blindness. Hence, early recognition and rapid re-perfusion is of paramount importance. This study aims to describe prehospital pathways in CRAO compared to stroke and study the knowledge about CRAO. Methods: (1) Description of baseline characteristics, prehospital pathways/delays, and acute treatment (thrombolysis/thrombectomy vs. standard of care) of patients with CRAO and ischemic stroke registered in the Swiss Stroke Registry. (2) Online survey about CRAO knowledge amongst population, general practitioners (GPs) and ophthalmologists in Eastern Switzerland. Results: Three hundred and ninety seven CRAO and 32,816 ischemic stroke cases were registered from 2014 until 2019 in 20 Stroke Centers/Units in Switzerland. In CRAO, 25.6% arrived at the hospital within 4 h of symptom onset and had a lower rate of emergency referrals. Hence, the symptom-to-door time was significantly longer in CRAO compared to stroke (852 min. vs. 300 min). The thrombolysis/thrombectomy rate was 13.2% in CRAO and 30.9% in stroke. 28.6% of the surveyed population recognized CRAO-symptoms, 55.4% of which would present directly to the emergency department in contrast to 90.0% with stroke symptoms. Almost 100% of the ophthalmologist and general practitioners recognized CRAO as a medical emergency and 1/3 of them considered IV thrombolysis a potentially beneficial therapy. Conclusions: CRAO awareness of the general population and physician awareness about the treatment options as well as the non-standardized prehospital organization, seems to be the main reason for the prehospital delays and impedes treating CRAO patients. Educational efforts should be undertaken to improve awareness about CRAO.

Inner Retinal Layer Hyperreflectivity Is an Early Biomarker for Acute Central Retinal Artery Occlusion.

Purpose: To investigate inner retinal hyperreflectivity on optical coherence tomography (OCT) as a potential biomarker indicating acute central retinal artery occlusion (CRAO). Methods: A total of 56 patients at two university hospitals with acute CRAO (symptom onset ≤48 h) were included in this retrospective study. The optical intensity of the inner retinal layers was determined in both eyes and the relationship between symptom onset and inner retinal layer optical intensity in OCT scans compared to the unaffected fellow eye was analyzed. Several differential diagnoses [central retinal vein occlusion, anterior ischemic optic neuropathy, diabetic macular edema, and subretinal fibrosis/disciform scar (Junius-Kuhnt)] served as controls to validate optical intensity-based diagnosis of CRAO. Results: CRAO strongly correlated with an increased inner retinal layer hyperreflectivity in this cohort with acute CRAO with a time since symptom onset ranging from 1.1 to 48.0 h. Receiver operating characteristic (ROC) analysis showed an area under the curve of 0.99 to confirm CRAO with a true positive rate of 0.93 and a false positive rate of 0.02. No correlation between optical intensity and time since symptom onset was noticeable. None of the differential diagnoses did show an elevated optical intensity of the inner retinal layers as it was detectable in CRAO. Conclusion: OCT-based determination of inner retinal layer hyperreflectivity is a very promising biomarker for a prompt diagnosis of CRAO in an emergency setting. This may be of major interest to speed up the administration of a possible thrombolytic treatment.

Changes in macrophage-like cells characterized by en face optical coherence tomography after retinal stroke.

Purpose: The retina could serve as a window of neuroinflammation, but the in vivo changes in macrophage-like cell (MLC), such as microglia, in acute ischemic retinal stroke remain unclear. Thus, the current study aimed to investigate the in vivo changes in MLC characterized by en face optical coherence tomography (OCT) after acute ischemic retinal stroke. Methods: Twenty patients with unilateral acute nonarteritic reperfused central retinal artery occlusion (CRAO) were participated in this study, and their contralateral eyes served as control group. A 3 µm en face OCT slab on the inner limiting membrane of the optic nerve head (ONH) region or macular region was used to visualize and binarize the MLCs. The MLCs were binarized and quantified using a semiautomated method. OCT angiography was used to evaluate the reperfusion status and obtain the structural data of the inner retina in the ONH and macula. The thickness of the ganglion cell complex in the macular region was measured. The optical intensity and optical intensity ratio of the inner retina were calculated to evaluate the ischemia severity. Results: In the ONH region, decreased vessel densities of radial peripapillary capillaries accompanied by increased thickness of the retinal nerve fiber layer were found in the CRAO eyes in comparison to the unaffected eyes (p=0.001, p=0.009, respectively). In the macular region, significantly lower vessel densities in both the superficial and deep capillary plexus and increased thickness of the ganglion cell complex were also found in the CRAO eyes (all p ≤ 0.001). The ONH and macular MLC quantities and densities in CRAO eyes were significantly higher than those in the unaffected eyes (both p<0.001). Larger and plumper MLCs were observed in the CRAO eyes compared with their unaffected eyes. ONH and macular MLC densities were positively associated with the disease duration in the acute phase and the optical intensity ratio of inner retina. Conclusions: The increased density and morphological changes of MLCs may indicate the aggregation and activation of MLCs following acute reperfused CRAO. The aggregation of MLCs may be more pronounced in CRAO eyes with longer disease duration and more severe ischemia. MLCs characterized by en face OCT may serve as an in vivo visual tool to investigate neuroinflammation in the ischemic-reperfusion process of stroke.

Abnormal Retinal Vasculature in a Young Child: A Diagnostic Dilemma.

Aim: To report the case of a young child who presented with sudden visual loss and characteristic abnormal retinal vasculature.Design: Case ReportMethods: A 13-year-old Indian boy presented with sudden visual loss in the left eye (20/1200) for 1 month, associated with recent-onset of seizures. Ocular examination showed a relative afferent pupillary defect, neovascularization of iris, absence of retinal vasculature beyond second branching, and arterio-venous shunts at the junction of vascular and avascular retina. Fundus fluorescein angiography showed delayed arm-to-retina time, leading edge of fluorescein dye in arteries and peripheral capillary non-perfusion areas.Peripheral pulses were absent and carotid doppler showed bilateral carotid artery occlusion (left one being worse affected), suggestive of Takayasu's arteritis (TA). Despite being started on immunotherapy, he lost complete vision in the left eye.Conclusion: TA may present initially to ophthalmologists, who need to maintain a high index of suspicion for early diagnosis and management of this life-threatening disease.

Acute retinal arterial ischemia.

Acute retinal arterial ischemia, which includes transient monocular vision loss (TMVL), branch retinal artery occlusion (BRAO), central retinal artery occlusion (CRAO) and ophthalmic artery occlusion (OAO), is most commonly the consequence of an embolic phenomenon from the ipsilateral carotid artery, heart or aortic arch, leading to partial or complete occlusion of the central retinal artery (CRA) or its branches. Acute retinal arterial ischemia is the ocular equivalent of acute cerebral ischemia and is an ophthalmic and medical emergency. Patients with acute retinal arterial ischemia are at a high risk of having further vascular events, such as subsequent strokes and myocardial infarctions (MIs). Therefore, prompt diagnosis and urgent referral to appropriate specialists and centers is necessary for further work-up (such as brain magnetic resonance imaging with diffusion weighted imaging, vascular imaging, and cardiac monitoring and imaging) and potential treatment of an urgent etiology (e.g., carotid dissection or critical carotid artery stenosis). Since there are no proven, effective treatments to improve visual outcome following permanent retinal arterial ischemia (central or branch retinal artery occlusion), treatment must focus on secondary prevention measures to decrease the likelihood of subsequent ischemic events.

Correlation Between Ischemic Retinal Accidents and Radial Peripapillary Capillaries in the Optic Nerve Using Optical Coherence Tomographic Angiography: Observations in 6 Patients.

BACKGROUND: Perfusion of the optic nerve has been widely studied using fluorescein angiography (FAG), which is currently regarded as the criterion standard. However, FAG has adverse effects associated with intravenous contrast administration and is limited in its capacity to characterize and stratify the different vascular layers of the optic nerve and retina. The use of new imaging techniques, such as optical coherence tomographic angiography (Angio-OCT), is therefore important. AIM: A qualitative description is made of the vascular layers of the optic nerve and of how vascular events affect radial peripapillary capillaries (RPC). Two patients with central retinal artery occlusion (CRAO), 1 with arteritic anterior ischemic optic neuropathy (AAION), and 3 healthy subjects were studied. RESULTS: The Angio-OCT imaging afforded better visualization of the depth of the RPC and rest of the vascular layers of the retina compared with FAG. Optic nerve surface perfusion was affected in AAION and proved normal in CRAO. CONCLUSIONS: Our results indicate that perfusion of the papilla and RPC mainly arises from the papillary plexus that depends on the posterior ciliary artery.