Wagner's disease

Wagner's disease is a familial disease of the eye that can cause reduced visual acuity. It was originally described in 1938, when Hans Wagner reported 13 members of a family from the Canton of Zurich with a peculiar lesion of the vitreous and retina. It was long confused with Stickler syndrome but lacks that condition's systemic features and high rate of retinal detachment, and the confusion ended once the responsible gene was identified in 2005 as the versican gene, VCAN, at 5q14.3. Inheritance is autosomal dominant, and the disease affects fewer than 1,000 people in the United States. The most common sign is a worsening of vision at a young age without obvious cause, sometimes as early as age two. Reported features include cataract, atrophy of the choroid and retina, glaucoma, myopia, an optically empty vitreous cavity, peripheral retinal detachment and visual field defects. Diagnosis rests on fundus examination showing an empty vitreous with vitreoretinal degeneration and no systemic associations. Most people need laser repair of the retina, and about 60 percent need further surgery to remove cataracts. This entry records what is known and is not medical advice.