(pathology) A degenerative condition of the vitreous and retinal parts of the eye, and may eventually lead to progressive vision loss.
(genetics) A disease of the connective tissue in the eye that can be inherited in an autosomal dominant pattern.
Wagner syndrome is a very rare hereditary vitreo-retinal disease. It is one of the connective tissue disorders affecting the collagen.
The main feature is an ‘empty’ vitreous that lacks the normal structure. Missing or weak vasculature in the peripheral retina makes the retina prone to tear or getting detached. Diminished visual acuity at night is also a problem as is the early onset of cataract (from the age of 30) and choroidal atrophy. A lot of people are moderate (> -2) to highly (> -6) myopic. Wagner syndrome is ocular only.
The genetic cause for Wagner syndrome has been found in 2005. The gene responsible for this disorder is the Versican gene (5q13.4). About 20 families with Wagner syndrome have been found in Switzerland, the Netherlands, France, the UK, the USA and Japan.
For more information see: www.wagnersyndrome.eu
Synonym: Wagner’s hyaloid retinal degeneration, Wagner’s vitreoretinal herdodegeneration, Wagners syndrome.
See also: autosome, dominant.