Fuchs' endothelial corneal dystrophy is a hereditary disease that results in corneal blindness. It is a common reason for corneal transplantation.
Features:
(1) hereditary disorder of corneal endothelium in Descemet membrane
(2) onset of symptoms is in middle age as blurry morning vision
(3) guttae (drops) appear in the central Descemet membrane and increase in number over time
(4) coalescence of guttae is associated with corneal edema
(5) generalized involvement results in corneal blindness
Genetics: complex and heterogeneous with multiple causal genes (ZEB1, SLC4A11, LOXHD1, other).
Some patients may have hearing loss.
It may be exacerbated by cataract surgery and by smoking.
