Fuchs'

Fuchs' Dystrophy
  • Occasionally it is inherited as an autosomal dominant trait.
  • Affects women more frequently and more severely than men.

Clinical Features

  • Symptoms:
    • Foreign body sensation, pain, tearing, photophobia
    • Significant blurring of vision when these blisters rupture
  • Signs:
    • Asymmetrical bilateral endothelial dystrophy that often presents in the fifth or sixth decade, but can be seen clinically in younger patients
    • Multiple central corneal guttata (excresences of Descemet's membrane) associated with pigment dusting on the endothelium
    • With specular reflection, they appear as black holes in the endothelial mosaic
  • Advanced stage:
    • Decreased endothelial cell density
    • Compromised endothelial cell pump function results in corneal edema
    • Bullous keratopahy usually developed in the decompensated corneal
    • As the corneal edema progresses and becomes chronic, diffuse subepithelial scarring may form, separating the epithelium from stroma
    • Corneal sensation is usually diminished

Treatment

  • Hypertonic agents such as sodium chloride 5% drops or ointment to treat early epithelial edema.
  • Topical or oral intraocular pressure lowering medications.
  • Lubricant eye drops or soft bandage contact lens may relieve discomfort by covering the exposed corneal nerve endings.
  • Penetrating keratoplasty has a high success rate when performed in advanced case where visual rehabilitation is desired.
Slitlamp view shows moderate stromal edema and metal-beaten appearance of the Descemet's membrane.