Is Central Retinal Artery Occlusion (CRAO) associated with Reversible Cerebral Vasoconstriction Syndrome (RCVS)?

From the Guidelines

Central Retinal Artery Occlusion (CRAO) is not typically associated with Reversible Cerebral Vasoconstriction Syndrome (RCVS). These are distinct clinical entities with different underlying pathophysiologies. CRAO is an ocular emergency caused by obstruction of blood flow through the central retinal artery, leading to sudden, painless vision loss. It is commonly associated with embolic phenomena, thrombosis, or vasculitis, and risk factors include cardiovascular disease, carotid artery stenosis, and hypercoagulable states [ 1 ]. RCVS, on the other hand, is characterized by reversible segmental constriction of cerebral arteries, typically presenting with thunderclap headaches and sometimes neurological deficits. RCVS is often triggered by vasoactive substances, postpartum state, or certain medications. While both conditions involve vascular pathology, they affect different vascular territories and have different mechanisms.

Key Considerations

• CRAO is a form of acute ischemic stroke that causes severe visual loss and is a harbinger of further cerebrovascular and cardiovascular events [ 1 ].
• The management of CRAO should focus on identifying cardiovascular risk factors and potential sources of emboli rather than RCVS [ 1 ].
• Evaluation and treatment of CRAO should be a collaborative effort among a neurologist, an ophthalmologist, and an internist.
• Further studies are necessary to evaluate long-term quality of life after CRAO and to develop novel biomarkers of retinal tissue viability [ 1 ].

Clinical Implications

• If a patient presents with CRAO, urgent medical attention is necessary to prevent permanent tissue damage.
• The use of intravenous tPA may be a reasonable treatment for patients with CRAO after a discussion of the benefits and risks with the patient or surrogate [ 1 ].
• Historical strategies, including anterior chamber paracentesis, ocular massage, and hemodilution, are not beneficial with respect to visual outcome [ 1 ].

From the Research

Association between CRAO and RCVS

• There is no direct evidence in the provided studies to suggest a association between Central Retinal Artery Occlusion (CRAO) and Reversible Cerebral Vasoconstriction Syndrome (RCVS) [ 2, 3, 4, 5, 6 ].
• The studies primarily focus on the treatment and management of CRAO, with no mention of RCVS [ 2, 3, 4, 5, 6 ].
• CRAO is often associated with cardio-cerebrovascular diseases, and patients with CRAO are at risk of subsequent ischemic events, including cerebral stroke [ 2, 4 ].
• However, there is no research paper among the provided evidence to assist in answering the question about the association between CRAO and RCVS [ 2, 3, 4, 5, 6 ].

CRAO Treatment and Management

• The treatment of CRAO is controversial, and there is no consensus on a standardized treatment regimen [ 2, 3, 5 ].
• Various treatments have been suggested, including thrombolytic therapy, ocular massage, anterior chamber paracentesis, and medication-induced reduction of intraocular pressure [ 2, 3, 5 ].
• The management of CRAO patients should focus on reducing the risk of subsequent ischemic events, including cerebral stroke [ 2 ].
• Multidisciplinary interventional therapy may be effective in improving vision and reducing cardio-cerebrovascular risk in patients with CRAO [ 4 ].

Visual Field Defects in CRAO

• Visual field defects are common in patients with CRAO, and can be characterized into five types: peripheral constriction only, paracentral scotoma, central and cecocentral scotoma, temporal island, and no visual field [ 6 ].
• The severity of visual field defects is associated with the severity of retinal ischemia and baseline visual acuity [ 6 ].
• Improvements in visual field defects can occur in some patients with CRAO, and are associated with mild CRAO stages, good baseline visual acuity, and mild retinal morphologic changes [ 6 ].