Vitreoretinal degeneration as a sign of generalized connective tissue diseases

Am J Ophthalmol. 1979 Sep;88(3 Pt 1):432-49. doi: 10.1016/0002-9394(79)90645-7.


Dominant vitreoretinal degeneration was first described by Wagner as a benign ocular condition. This term has since become widely associated with a familial retinal degenerative process accompanied by retinal detachments, even though nobody in the family described by Wagner suffered from a retinal detachment. Vitreoretinal degeneration, often progressing to detachments, occurs also in several distinct bone dysplasias. I have provided a classification of syndromes with vitreoretinal degeneration and its signficance as a sign of connective tissue diseases (probably involving type II collagen). In this sense, it is similar to ectopia lentis as a sign of various connective tissue diseases.

MeSH terms

  • Abnormalities, Multiple / complications
  • Adolescent
  • Adult
  • Bone Diseases, Developmental / complications
  • Bone Diseases, Developmental / genetics*
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Male
  • Marfan Syndrome / complications
  • Mucopolysaccharidosis IV / complications
  • Myopia / complications
  • Myopia / genetics*
  • Retinal Degeneration / complications
  • Retinal Degeneration / genetics*
  • Syndrome
  • Vitreous Body*