Aetiology, Diagnosis and Treatment of Arterial Occlusions of the Retina-A Narrative Review.

Arterial occlusions of the retina are potentially sight-threatening diseases which often result in profound visual loss. The aim of this narrative review is to provide an overview of the aetiology, discuss major risk factors, describe the management and systemic assessments and evaluate existing therapies. For this review, an extensive literature search in PubMed was performed. Emboli from the heart or the carotid arteries can cause ophthalmic artery occlusion (OAO), central retinal artery occlusion (CRAO) and branch retinal artery occlusion (BRAO). Most patients with arterial occlusions have vascular risk factors such as arterial hypertension, hyperhomocysteinaemia, carotid stenosis and atrial fibrillation, which also increase the risk of cerebral stroke and myocardial infarction. Therapies such as ocular massage, thrombolysis and anterior chamber paracentesis have been suggested but are still equivocal. However, it is evident that retinal artery occlusion should be immediately treated and accompanied by interdisciplinary collaboration, since early diagnosis and the proper treatment of possible risk factors are important to reduce the risk of further damage, recurrences, other vascular diseases and mortality.

Unusual case of retinal arterial branch occlusion: possible variant of Sneddon syndrome.

Sneddon's syndrome (SS) manifests through multiple strokes and livedo reticularis. Livedoid vasculopathy (VL) is characterized by a long history of foot and leg ulceration and histopathology indicating a thrombotic process. Arterial retinal branch occlusion is described in a 52-year-old male with VL. He did not present noticeable laboratory abnormalities, such as antiphospholipid antibodies, or a history of strokes. Retinal artery occlusion accompanied by VL could be a variant of Sneddon's syndrome. Optical coherence tomography angiography revealed a reduction in the macula's vascular layers in the asymptomatic eye, indicating localized microvascular changes as an evolving marker in the pathogenesis of SS.

Exploring Superselective Intraarterial Thrombolysis for Autologous Fat Injection-Induced Vision Loss.

BACKGROUND: Intravascular injection represents the most severe complication in fat transplantation procedures. Currently, the prognosis for patients who suffer from blindness due to fat transplantation-induced ocular vascular occlusion is far from optimistic. OBJECTIVES: The aim of this study was to explore and evaluate the efficacy and safety of arterial thrombolysis in the treatment of ocular vascular occlusion caused by fat transplantation. METHODS: We analyzed the data of 12 patients who underwent intraarterial thrombolysis and conservative treatments for facial autologous fat grafting-associated ocular vascular occlusion. Among the cases, there were 6 instances of ophthalmic artery embolism and 6 cases of central retinal artery occlusion. All patients suffered with sudden blindness, sometimes accompanied by eye pain, ptosis, strabismus, skin necrosis at the injection site, or cerebral microinfarction. They received symptomatic conservative treatments and intraarterial thrombolysis, encompassing mechanical vessel recanalization, vessel dilation, and dissolution of thrombus constituents. RESULTS: Following intraarterial thrombolysis, a noteworthy improvement in the blood flow of both the main trunk and peripheral branches of the ophthalmic artery was observed in the majority of patients when contrasted with their pretreatment status. One patient experienced a headache intraoperatively, while no significant discomfort was reported by the remaining patients. After conservative treatments and intraarterial thrombolysis, all patients experienced improvement in ocular symptoms, skin necrosis, and cerebral infarction. Three patients demonstrated improvement in visual acuity. These patients had surpassed the recommended time window for treatment, yet the occlusion of the ophthalmic artery was not complete. CONCLUSIONS: Intraarterial thrombolysis combined with conservative treatments achieves early perfusion and is expected to promote visual recovery. Hospitals that possess the necessary treatment capabilities are encouraged to establish this therapeutic pathway.

Occlusive Retinal Vasculitis in a Pediatric Patient With Kikuchi-Fujimoto Disease and Sickle Cell Trait.

A 13-year-old Black male patient with a history of Kikuchi-Fujimoto disease (KFD) and sickle cell trait presented with acute painless vision loss and no light perception vision (NLP) in his left eye. The examination was indicative of occlusive retinal vasculitis with near total central retinal artery occlusion (CRAO). He was started on oral steroids with dramatic reperfusion and improvement of the retinal hemorrhages. However, his vision remained at NLP. Oral steroids were tapered, and rituximab infusion was initiated. While ocular involvement is uncommon in KFD, vision-limiting complications, such as occlusive retinal vasculitis, ophthalmic artery occlusion, and CRAO can occur. Early systemic immunosuppression is key in achieving rapid remission. [Ophthalmic Surg Lasers Imaging Retina 2024;55:235-239.].

Retinal arterial occlusion and patent foramen ovale: A case study-based review.

PURPOSE: To identify all reported cases of retinal artery occlusion (RAO) associated with patent foramen ovale (PFO) in the literature and present a similar case of CRAO from our clinic. METHODS: PubMed database was searched for studies reporting RAO in individuals with PFO. Relevant data were tabulated and reviewed. We estimated each case's Risk of Paradoxical Embolism (RoPE) score. RESULTS: 23 cases of CRAO (n=10; including ours), BRAO (n=10), and CILRAO (n=3) were reviewed. Most cases were under 50 years of age (78.3%). The reported predisposing factors were: hypertension (26.1%), migraine (17.3%), smoking (13.0%), recent immobilization (13.0%), strenuous exertion (8.7%), pregnancy (8.7%), and diabetes (4.3%). A high RoPE score (≥7; suggestive of paradoxical embolism via PFO) was estimated for 71.4% of patients. In most cases, the neurological and cardiovascular examinations, laboratory studies, and imaging were unremarkable, except for the PFO±atrial septal aneurysm (present in 21.7%). In only 28.6% of cases, transthoracic echocardiography (TTE) (± saline contrast) could visualize the PFO; transesophageal echocardiography (TEE) was necessary to detect the PFO in 71.4%. Approximately one-half of the patients underwent percutaneous closure of the PFO; no complications or subsequent acute ischemic events ensued. The visual prognosis was poorer for CRAO than for BRAO or CILRAO. CONCLUSION: Timely diagnosis, acute management, and ensuring urgent initiation of stroke workup in cases with RAO or transient monocular vision loss are crucial. Clues to a possible paradoxical embolism as the cause include the absence of known cardiovascular risk factors, young age, migraine, recent immobility, vigorous exercise, and pregnancy.

Retinal Artery and Vein Occlusion Risks after Coronavirus Disease 2019 or Coronavirus Disease 2019 Vaccination.

PURPOSE: To evaluate the incidence of new retinal artery occlusion (RAO) and retinal vein occlusion (RVO) after the diagnosis of coronavirus disease 2019 (COVID-19) or vaccination against COVID-19 and compare the incidences with the population with neither. DESIGN: Nationwide population-based cohort study. PARTICIPANTS: From a nationwide population-based cohort, 8 418 590 patients were categorized into control (group 1), COVID-19 infection (group 2), and COVID-19 vaccination (group 3) groups. METHODS: The cumulative incidence of RAO and RVO was calculated in groups 1, 2, and 3 using the Kaplan-Meier method. We calculated hazard ratios (HRs) and 95% confidence intervals (CIs) based on the Poisson distribution for RAO and RVO according to each group and subgroup using Cox proportional hazards models, with group 1 as the reference. We conducted univariable and multivariable analyses for the risk factors of RAO and RVO according to each subgroup. MAIN OUTCOME MEASURES: Cumulative incidence and risks of incidence of RAO and RVO from the index date to day 60. RESULTS: In multivariable analysis, no significant increase in RAO and RVO risks after COVID-19 or COVID-19 vaccination were observed in either men or women. These results were observed consistently across various conditions in sensitivity analyses. In subgroup analysis, individuals who were vaccinated before infection showed no significant increase in RAO or RVO risks in both sexes compared with the control group. In the subgroup analysis of vaccinated patients, the HRs of RAO and RVO for different vaccine types did not show an increase compared with the control group; however, an exception was observed in women who received mRNA-1273 vaccines, who showed a higher RAO HR (4.65; 95% CI, 1.27-17.03; P = 0.021). CONCLUSIONS: Within 60 days of COVID-19 diagnosis or vaccination, RAO and RVO occurred rarely. We observed no increase in the HR of RVO and RAO relative to COVID-19 or COVID-19 vaccination except for a possible increase in the RAO HR in women who received mRNA-1273, for which the raw incidence was extremely low. Further investigation is required to validate this result. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Retinal artery occlusion after ophthalmic surgery under regional anaesthesia: A narrative review.

Two recent cases of central retinal artery occlusion under otherwise uncomplicated sub-Tenon's block that resulted in significant visual loss after cataract surgery prompted us to undertake a literature review of such cases. We identified 97 cases of retinal artery occlusion after ophthalmic surgery under regional anaesthesia that had no immediate signs of block-related complications. These occurred after various intraocular (87%) and extraocular (13%) operations, across a wide range of ages (19-89 years) on patients with (59%) or without (39%) known risk factors. The anaesthetic techniques included 40 retrobulbar blocks, 36 peribulbar blocks, 19 sub-Tenon's blocks, one topical anaesthetic and one unspecified local anaesthetic. Different strengths of lidocaine, bupivacaine, mepivacaine and ropivacaine, either alone or in various combinations, were used. The details of the anaesthetic techniques were often incomplete in the reports, which made comparison and analysis difficult. Only nine cases had their cause (optic nerve sheath injury) identified, while the mechanism of injury was unclear in the remaining patients. Various mechanisms were postulated; however, the cause was likely to be multifactorial due to patient, surgical and anaesthetic risk factors, especially in those with compromised retinal circulation. As there were no definite risk factors identified, no specific recommendations could be made to avoid this devastating outcome. We have provided rationales for some general considerations, which may reduce this risk, and propose anaesthetic options for ophthalmic surgery on the fellow eye if required, based both on our literature review and our personal experience.

Central retinal artery occlusion after spinal surgery: Case report and literature review.

AIM: To report a rare case of unilateral central retinal artery occlusion (CRAO) following spinal surgery. METHODS: Observational case report. RESULTS: A 15-year-old female patient underwent scoliosis surgery under general anesthesia in a prone position, her head being supported by a horseshoe headrest for approximately four hours, with stable vitals and without significant blood loss during surgery. Upon waking up from general anesthesia, the patient immediately reported severe visual loss in her right eye (RE), associated to marked periocular ecchymosis and chemosis. Visual acuity was limited to light perception. Fundus examination showed normal optic disc appearance with diffuse retinal pallor and a macular cherry red spot. Optical coherence tomography (OCT) showed increased reflectivity in the inner retina, consistent with ischemic maculopathy in the RE. Brain and neck magnetic resonance imaging angiograms were unremarkable. Further investigations ruled out collagen vascular disease, Behcet disease, syphilis, sickle cell disease and hypercoagulable states. CONCLUSION: Central retinal artery occlusion is rarely observed following spinal surgery. The cause was presumed to be compression of the orbit by a horseshoe headrest in a prone position due to an accidental shift in position during surgery. This catastrophic complication, albeit rare, is usually irreversible and thus must be prevented. Proper positioning and vigilance by both the surgeon and the anesthesiologist during surgery are fundamental to ensure that the orbits are not under pressure.

Branch retinal artery occlusion with congenital common carotid artery and internal carotid artery occlusion: A case report.

PURPOSE: To report a case of branch retinal artery occlusion (BRAO) of the left eye combined with left congenital common carotid artery occlusion (CCAO) and internal carotid artery occlusion (ICAO). METHODS: Case report. RESULTS: A 36-year-old man presented with sudden vision loss of only the left eye without any signs or symptoms of brain diseases. Fluorescein fundus angiography (FFA) showed left BRAO, and computed tomography angiography (CTA) of the head and neck showed entire left CCAO and ICAO. The patient's left vertebral artery was anastomosed with the left occipital artery via the muscular branch, supplying blood retrogradely to the left external carotid artery. The right internal carotid artery compensated for blood supply to the left anterior cerebral artery and middle cerebral artery via anterior communication, and the left posterior communication artery compensated for blood supply to the left middle cerebral artery. CONCLUSIONS: To our knowledge, this study was the first to report a case of BRAO combined with congenital CCAO and ICAO with vision loss as the first symptom and proposed the importance of head and neck examination in retinal artery occlusion at the first visit to a doctor.

Spontaneous Late Visual Recovery in Pediatric Central Retinal Artery Occlusion: A Case Report and Literature Review.

The authors report a rare case of pediatric central retinal artery occlusion (CRAO) and spontaneous late recovery, with associated literature review. A retrospective review of patient medical records and literature search of four relevant databases was conducted. A 7-year-old girl with sudden, painless loss of vision in her right eye was referred to the authors' center where she was found to have a CRAO with hand motion visual acuity. One month later, the patient developed multiple retinal hemorrhages throughout the fundus. Despite undergoing extensive investigations, a cause for her condition was not determined. Seventeen months after the original event, the patient regained a visual acuity of 20/40. A CRAO in the pediatric population is relatively rare and requires extensive investigation into the potential cause. Compared to adult CRAO, presentation in the pediatric age group should warrant a high index of suspicion and extensive work-up to treat any underlying systemic conditions and possibly prevent involvement of the other eye. [J Pediatr Ophthalmol Strabismus. 2023;60(6):e70-e74.].

Central retinal artery occlusion following pediatric blunt ocular trauma.

BACKGROUND: To analyze the clinical profile, presentation, possible pathophysiology, and outcomes of central retinal artery occlusion (CRAO) following blunt trauma in pediatric subjects. METHODOLOGY: The medical charts of subjects aged 18 years or less with a diagnosis of CRAO following blunt ocular trauma were analyzed retrospectively for demography, details of the trauma, ocular findings, additional imaging reports if any, and final outcome. A Medline search was done (key words like central retinal artery occlusion, blunt trauma, children, pediatric subjects, and adolescents) to gather information available in the literature on the subject. RESULTS: A total of 11 patients (11 eyes), mean age of 14.3 ± 3.4 years, and 100% male preponderance, with an average time duration from trauma to presentation to the hospital of 8.1 days were included. Visual acuity ranged from no light perception (four eyes) to finger count at a 1 m distance. Intraocular pressure was raised in three patients, of which two were suffering from sickle cell disease. In two eyes, the CRAO coexisted with optic nerve avulsion and the cilioretinal artery was spared. Disk pallor was seen in six eyes as early as 12 days from the trauma. None of the cases revealed any bony fracture in the CT scan. CONCLUSION: CRAO was observed to be an important primary or contributory cause of visual loss in children following blunt trauma, reflex vasospasm being the most common etiology. Early onset disk pallor could suggest an underlying vascular compromise of both retinal and optic disk circulation in addition to direct disk damage.

Cardioembolic stroke in the setting of diffuse caseous calcification of the anterolateral papillary muscle.

A woman in her 70s was admitted for acute, painless vision loss in the left eye. Examination showed cherry red spot in the macula and plaque in the nasal vessels, consistent with central retinal artery occlusion. MRI orbits revealed multifocal subclinical acute infarcts in the right middle cerebral artery (MCA) territory and bilateral cerebella. Transthoracic echocardiogram showed calcification of the anterolateral papillary muscle. Further characterisation with cardiac MRI elucidated caseous 'toothpaste-like' calcification of the muscle complex. Stroke workup was otherwise unremarkable. The patient underwent hyperbaric treatment with mild improvement. Anticoagulation and surgical intervention were deferred due to known risks and unknown benefit for calcific emboli. The patient was continued on her home dual anti-platelet therapy (DAPT) and discharged with a loop monitor. Caseous calcification of the papillary muscle (CCPM) may be a risk factor for cardioembolic stroke. Further discussions on medical and surgical guidelines for CCPM would be beneficial for stroke prevention.

Case report: acute isolated cilioretinal artery occlusion secondary to percutaneous coronary intervention.

INTRODUCTION: This case report aims to describe in detail the acute isolated cilioretinal artery occlusion (CLRAO) secondary to complicated therapeutic percutaneous coronary intervention (PCI). CASE DESCRIPTION: A 68-year-old Chinese man with coronary artery disease (CAD) complained of sudden, sharp chest pain. Coronary angiography revealed severe stenoses of the coronary arteries. The patient was then treated with PCI. One hour after the procedure, the patient presented with a sudden reduction in vision in the right eye. The patient was diagnosed with acute isolated CLRAO and treated with Salvia miltiorrhiza injections. CONCLUSIONS: This is the report to provide a detailed description of acute isolated CLRAO secondary to therapeutic PCI treated with Salvia miltiorrhiza. The visual prognosis of the untreated patients is poor. Suitable management and prevention are essential for interventional cardiologists to prevent these complications.

CENTRAL RETINAL ARTERY OCCLUSION AS PRESENTATION OF BARTONELLA ENDOCARDITIS.

BACKGROUND/PURPOSE: To describe a rare ocular presentation of a systemic illness and the important lifesaving diagnosis made by a complete eye examination. METHODS: The patient was evaluated with a comprehensive ophthalmic examination and followed closely in the outpatient setting with optical coherence tomography, fluorescein angiography, and color fundus photos. RESULTS: A 66-year-old man presented with acute vision loss of the left eye. A complete eye examination revealed that he had a central retinal artery occlusion. Systemic workup revealed that he had a mitral valve vegetation and blood cultures grew Bartonella henselae. His kidney biopsy showed membranoproliferative glomerulonephritis, which is often seen with septic emboli. Furthermore, the patient lacked any ocular inflammatory signs. This constellation of findings was diagnostic for a thromboembolic etiology causing his central retinal artery occlusion. At follow-up, the optical coherence tomography demonstrated inner retinal hyperreflectivity and the fluorescein angiogram showed segmented flow and no neovascularization. On follow-up, the patient had a stable examination with light perception vision and completed antibiotic therapy for bartonella endocarditis. CONCLUSION: The detection of a fatal systemic illness was made promptly with a thorough ocular examination. We highlight the importance of a multidisciplinary approach in making a lifesaving diagnosis.

Central retinal artery occlusion without cherry-red spots.

BACKGROUND: Cherry-red spots are a very important sign for the clinical diagnosis of central retinal artery occlusion (CRAO). We retrospectively summarized the clinical manifestations of CRAO and analysed the causes and characteristics of CRAO without cherry-red spots. In this study, we explored a diagnostic method for CRAO without cherry red spots. METHODS: Seventy patients (70 eyes) with CRAO were examined retrospectively. Corrected distance visual acuity, fundus photos, FA and OCT images were collected at the first outpatient visit. The causes of CRAO without cherry-red spots were analysed through fundus photos. The incidence of increased hyperreflectivity of the inner retina, central macular thickness (CMT) and arteriovenous transit time in patients with and without cherry-red spots were compared. RESULTS: Fundus examination showed posterior retinal whitening in 57 cases (81.43%) and cherry-red spots in 39 cases (55.71%). Thirty-one patients presented at the first outpatient visit without cherry-red spots. The reasons for the absence of cherry-red spots included leopard fundus (32.26%), retinal vein occlusion (25.81%), no obvious inner retinal coagulative necrosis (19.35%), ciliary retinal artery sparing (12.90%), high macular oedema (9.68%) and cherry-red spot enlargement (3.23%). OCT revealed increased hyperreflectivity of the inner retina in 67 CRAO patients (95.71%). All 3 patients without increased hyperreflectivity of the inner retina did not present with cherry-red spots at the first visit. The median CMT in patients without cherry-red spots was 166.00 µm, while the median MCT in patients with cherry-red spots was 180.00 µm; there was no significant difference between these two groups (P = 0.467). FA showed delayed arteriovenous transit time > 23 s in 20 patients (28.57%), > 15 s in 43 patients (61.43%) and no delay in 27 patients (30.77%). The median arteriovenous transit time in patients without cherry-red spots was 19.00 s, while it was 18.00 s in patients with cherry-red spots; there was no significant difference between these two groups (P = 0.727). CONCLUSIONS: There are multiple factors that could cause the absence of cherry-red spots in CRAO. The use of OCT to observe increased hyperreflectivity of the inner retina is the most effective imaging method for the early diagnosis of CRAO without cherry-red spots.