Fuchs' dystrophy

• Fuchs' corneal dystrophy
  • a dominantly inherited disorder that affects corneal endothelial cells (1)
  • slowly progressive, bilateral corneal dystrophy, which is typically first observed clinically in patients older than 50 years of age
    • however can be seen in some patients in their late 20s
  • characterised by a progressive loss of endothelial cells with secretion of an abnormally thickened basement membrane, leading to guttata formation
  • there is a decline in visual acuity as a result of deterioration of endothelial function and increasing corneal thickness increases

Reference:

1. P. Rosenblum, W.J. Stark and I.H. Maumenee et al., Hereditary Fuchs' dystrophy, Am J Ophthalmol 90 (1980), pp. 455–46