Presumed glial retinal hamartomas in Usher's syndrome.
The presence of glial hamartomas of the retina and the optic disc in retinitis pigmentosa is discussed. This association is described in a 26 year old white male with Usher's syndrome who had at the posterior pole of each eye a mass which closely resembled the mulberry-like lesions of tuberose sclerosis. Several fine blood vessels were seen on the surface of these masses. The significance of the association of these presumed hamartomas with retinitis pigmentosa is not clear. They might be the result of a focal reactive hyperplasia of the glial cells of the retina.
D000328:Adult; D003638:Deafness / Q000150:complications*; D005134:Eye Neoplasms / Q000209:etiology* / Q000473:pathology; D006222:Hamartoma / Q000209:etiology / Q000473:pathology; D006801:Humans; D008297:Male; D009898:Optic Disk / Q000473:pathology*; D009900:Optic Nerve / Q000473:pathology*; D012160:Retina / Q000473:pathology; D012174:Retinitis Pigmentosa / Q000150:complications*