![]() ![]() Macular infarction due to various causes such as retinal artery and vein occlusions, carotid artery occlusions, malignant hypertension, diabetes, radiation retinopathy, posterior uveitis, systemic inflammatory vasculopathies, and trauma.(Note that an ophthalmic artery occlusion may not manifest a cherry red spot due to compromise of both the choroidal and retinal circulations.) Compromise of the choroidal and retinal circulation due to intraorbital hemorrhage or mass.Acute occlusion of the retinal circulation following cataract surgery (phacoemulsification) causing outer retinal whitening.Other inflammatory disorders are less common causes of arteritic CRAO and include Susac syndrome, systemic lupus erythematosus, polyarteritis nodosa, and granulomatosis with polyangiitis. Arteritic CRAO occurs in the setting of inflammatory conditions like giant cell arteritis (GCA) and are an estimated 5% of CRAO cases. Non-thromboembolic vascular causes of CRAO include nocturnal arterial hypotension, retinal migraine, and transient vasospasm. Associations with thromboembolic causes of CRAO include myxoma or vegetations of the cardiac valves and hypercoagulable states (hyperhomocysteinemia, factor V Leiden, protein C and S deficiencies, anti-thrombin III deficiencies, anti-phospholipid antibodies or prothrombin gene mutations, sickle cell disease). Thrombus or thromboembolism may be composed of cholesterol, fibrin-platelet, or calcium they usually originate from the carotid plaque and less commonly from the heart or the aorta. CRAO is classified as arteritic and non-arteritic, which will further guide the management discussed below.ĬRAO is most commonly (95% of cases) the non-arteritic CRAO, which is caused by atherothrombosis, thromboembolism, or vasospasm which occludes the central retinal artery. ![]() Cherry red spot has been found to be present on the initial examination in 90% of permanent CRAO cases and prognosticates poorer visual recovery. Central Retinal Artery Occlusion (CRAO) - This disease entity characteristically presents as a sudden onset of painless, unilateral vision loss in an elderly patient.The causes of cherry red spot in the macula can be categorized by etiology: Knapp initially believed that the cherry red spot was a benign fundoscopic finding, but later realized its grave implications. Bernard Sachs acknowledged that his patient had also been examined by ophthalmologist Herman Joseph Knapp, who practiced in both New York and Berlin, and described the striking retinal findings at an ophthalmology meeting in Heidelberg and used the term “cherry red color” to describe the fovea. The patient was confirmed upon neuropathologic examination to have lipid storage in the brain. The term “cherry red spot” was first introduced in 1887 by American neurologist Bernard Sachs, who described the fundus appearance of a child with "amaurotic familial idiocy" in his publication on “arrested development with special reference to its cortical pathology”. It should be noted that as a condition resolves or evolves over the course of time, the appearance of a cherry red spot diminishes and may not be present therefore, absence of a cherry red spot does not necessarily indicate absence of disease. Cherry red spot is an important fundoscopic sign, and when found with key clinical features and a good history, often guides one to the diagnosis of the disease. As ganglion cells are absent at the foveola, this area retains relative transparency and contrasts with the surrounding retina. In the case of lipid storage diseases, the lipids are stored in the ganglion cell layer of the retina, giving the retina a white appearance. The appearance of a cherry red spot results when retinal edema, most commonly due to central retinal artery occlusion or traumatic retinal ischemia, causes the perimacular tissue of the retina to appear translucent, while the fovea maintains its normal color. The fovea receives its blood supply from the choroid, which is supplied by the long and short posterior ciliary arteries. The fovea retains relative transparency as it is the thinnest part of the retina and devoid of ganglion cells. The term refers to the appearance of a red-tinted region at the center of the macula surrounded by retinal opacification, usually present due to the thickening and loss of transparency of the posterior pole of the retina. Ĭherry red spot at the macula is a clinically significant sign observed on fundus examination in a variety of pathological conditions, including retinal infarction, retinal ischemia, and several lysosomal storage disorders. Cherry red spot in patient with central retinal artery occlusion. ![]()
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