Wagner syndrome

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Disease Overview

Wagner syndrome is a hereditary eye disorder that leads to progressive vision loss. It is characterized by changes to the thick, clear gel that fills the eyeball (the vitrous), in which it becomes thin and watery and appears empty.[12954] The first signs and symptoms usually appear in childhood, but onset may be as early as age 2. Signs and symptoms may include: thinning of the light-sensitive tissue that lines the back of the eye (retinal detachment), abnormalities of the blood vessels within the retina (known as the choroid), and degeneration of the retina and choroid. Wagner syndrome is caused by mutations in the VCAN gene and is inherited in an autosomal dominant manner.[12954][1832] Treatment varies depending on the signs and symptoms in each individual and may include the use of glasses or contact lenses and vitreoretinal surgery.[1832][1833]


Synonyms

  • Wagner syndrome type 1
  • Wagner vitreoretinal degeneration
  • Hyaloideoretinal degeneration of Wagner
  • WGN1
  • Erosive vitreoretinopathy
  • ERVR
  • Wagner disease (formerly)

For more information, visit GARD.

National Organization for Rare Disorders