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Images in Clinical Practice

Indian Pediatrics 2002; 39:399  

Cherry Red Spot

An 8-year-old child presented with short stature, coarse facial characters, developmental delay and seizure disorder. Ophthalmoscopic examination of the the eye revealed a ‘cherry red spot’ in the retina (Fig. 1).

Throughout most of the retina the ganglion cells form a single cell layer except in the macula where they are present in 8-10 layers. Because of this special histological feature of the macular region, certain disease conditions affecting the retina produce a distinctive cherry red spot. It is seen with the aid of an ophthalmoscope as a bright to dull red area at the center of the macula, surrounded by a greyish white halo. The halo results from swelling and loss of transparency of the multilayered ganglion cell ring, due to edema or abnormal lipid accumulation or both. The central red area is normal choroidal vasculature, accentuated by the dull halo.

The typical cherry red spot occurs in several of the neuronal lipid storage disorders – most characteristically in Tay Sach’s disease (virtually all cases) and in the Sandhoff variant. Other causes include GM2 gangliosidosis, metachromatic leukodystrophy, Niemann Pick disease, Farber’s disease, Goldberg’s Syndrome, Gaucher’s disease, Hurler’s Syndrome (mucopolysaccharidosis 1H), b-galactosidase deficiency (mucopoly-saccharidosis VII), Hallervorden Spatz Syndrome, Batten-Mayou-Vogt-Spielmeyer Syndrome and Spranger’s disease.

Besides the metabolic neurodegenerative diseases, it may also develop as a result of retinal ischemia secondary to central retinal artery occlusion or orbital contusion. It should be appreciated that cherry red spot is not a disease condition in itself but an ophthalmoscopic evidence of a pathology that may involve the peripheral layers of the retina and multiple systems of the human body.

Fig. 1. Retinal photograph showing the cherry red spot at the center of the macula surrounded by a dull ‘halo’.

Tarun Gera,
Kirti Singh*,

Department of Pediatrics,

Maulana Azad Medical College,
New Delhi 110002 and
*Guru Nanak Eye Center, New Delhi 110002, India




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