Wagner’s syndrome

 

Wagner’s syndrome is a disease of the connective tissue in the eye that causes blindness. This disease is an an autosomal dominant genetically inherited disease.

The disease is characteriszed by degenerative changes in the:

  • retina of the eye
  • vitrious humor of the eye

Retina:

  • lines posterior 2/3rds of eyeball

  • contains rods and cones

  • Rods: detect presence of light and are more numerous towards the retinal periphery

    • The retina is the film of the eye.
    • converts light rays into electrical signals and sends them to the brain through the optic nerve.
    • The sides of the retina are responsible for our peripheral vision.

Vitreous Humor
The vitreous humor is a jelly like liquid that fills most of the eye (from the lens back). As we age it changes from a gel to a liquid and gradually shrinks separating from the retina. This is when people start seeing floaters, dark specs in their vision. This is a normal sign of aging, but in a few cases the retina can become detached as the vitreous separates.
    • The center area, called the macula, is used for our fine central vision and color vision.

Maumenee, I H, Mets, M B and Stoll, H U.  1982. The Wagner syndrome versus hereditary arthroophthalmopathy. Transaction of the American Opthamological Society. 80: 349–365.

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