A cherry-red spot is a finding in the macula of the eye in a variety of lipid storage disorders and in central retinal artery occlusion.General Practice Notebook It describes the appearance of a small circular choroid shape as seen through the fovea centralis. Medical Dictionary Its appearance is due to a relative transparency of the macula; storage disorders cause the accumulation of storage material within the cell layers of the retina, however, the macula, which is relatively devoid of cellular layers, does not build up this material, and thus allows the eye to see through the macula to the red choroid below.Suvarna JC, Hajela SA. Cherry-red spot. J Postgrad Med 2008;54:54-7.
The sign was first described by Warren Tay, founding member of the British Ophthalmological Society, in 1881, with reference to a patient with Tay–Sachs disease.
The cherry red spot is seen in central retinal artery occlusion, appearing several hours after the blockage of the retinal artery occurs.Rakel, Robert E., Textbook of Family Medicine, 7th ed, Chapter 53 The cherry red spot is seen because the macula receives its blood supply from the choroid, supplied by the long and short posterior ciliary arteries, while the surrounding retina is pale due to retinal artery infarction.""USMLE First AID 2010 page 417 It is also seen in several other conditions, classically Tay–Sachs disease, but also in Niemann–Pick disease, Sandhoff disease, and mucolipidosis.
Differential diagnosis
Metabolic storage diseases:Hereditary Ocular Disease, University of ArizonaFenichel's Clinical Pediatric Neurology: A Signs and Symptoms Approach, 2013;127 ff Tay–Sachs disease Farber disease GM1 and GM2 gangliosidoses Metachromatic leukodystrophy Niemann–Pick disease Sandhoff disease Sialidosis Congenital developmental diseases (e.g., Leber's congenital amaurosis) Hereditary/familial: Pantothenate kinase-associated neurodegeneration Vascular (e.g., central retinal artery occlusion) Drugs: Quinine toxicity Dapsone toxicity Poisoning: Carbon monoxide Methanol Blunt ocular trauma
See also Lipid storage disease
References