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Wagner syndrome

Other Names for this Disease
  • Erosive vitreoretinopathy
  • ERVR
  • Hyaloideoretinal degeneration of Wagner
  • Wagner disease (formerly)
  • Wagner syndrome type 1
More Names
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Wagner syndrome is a hereditary eye disease characterized by a central vitreous cavity which appears empty on slit-lamp examination.[1][2][3] The condition was named after the Swiss ophthalmologist Hans Wagner, who described a family with this condition in 1938. Since then less than 50 families have been described.[1] The first signs usually emerge during early adolescence, but onset may be as early as age 2.[2] Wagner syndrome is caused by mutations in the VCAN gene.[1][2] It is inherited in an autosomal dominant manner.[2]

Last updated: 12/7/2009


  1. Van Aerde K, Kloeckener-Gruissem B. Wagner Syndrome. Wagner Syndrome Website. 2009; Accessed 12/7/2009.
  2. Kloeckener-Gruissem B, Amstutz C. VCAN-Related Vitreoretinopathy. GeneReviews. 2009; Accessed 12/7/2009.
  3. Edery P. Wagner disease. Orphanet. 2004; Accessed 12/7/2009.
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In Depth Information

  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Wagner syndrome. Click on the link to view a sample search on this topic.