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Congenital Rubella: Retinopathy

Rubella

"Salt-and-pepper" retinopathy
This fine speckling of the retina is the commonest ocular manifestation of congenital rubella.

It is called "salt-and-pepper" because there are tiny flecks of dark pigment mixed with fine areas of whitish depigmentation. Usually the pigment alteration is diffuse, but it may be most prominent either around the macula or in the retinal periphery.

These signs reflect diffuse damage to the retinal pigment epithelium (RPE). The good news is that the RPE is never damaged enough to interfere with vision or to cause major abnormalities in the electroretinogram (ERG), the standard objective test used to evaluate outer retinal function. The preservation of vision and a relatively or completely normal ERG allows you to differentiate rubella retinopathy from the hereditary retinal degenerations called retinitis pigmentosa.

Other causes of "salt-and-pepper" retinopathy are congenital syphilis, and toxicities of systemically administered thioridazine, choroquine, and deferoxamine.

Other ophthalmic complications include microphthalmia (small eye), cataract, glaucoma, corneal opacification, and uveitis. Vision is usually quite poor from these problems.

Congenital rubella is a multisystem disorder resulting from exposure of the fetus to maternal rubella during the first trimester of pregnancy. Ophthalmic complications are present in over 70% of patients.

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