Fuchs' dystrophy

Etiology: idiopathic Epidemiology: 1) slightly more common in women than in men 2) rarely affects vision until 50s & 60s Pathology: 1) endothelial cells gradually deteriorate without any apparent reason 2) as more endothelial cells are lost over the years, the endothelium becomes less efficient at pumping water out of the stroma 3) this causes the corneal edema & distortion of vision 4) corneal edema in later phases, resulting in pain & severe visual impairment. 5) epithelial swelling damages vision by changing the cornea's normal curvature, & causing a sight-impairing haze to appear in the tissue. 6) epithelial swelling will also produce tiny blisters on the corneal surface 7) when these blisters burst, they are extremely painful. Clinical manifestations: 1) slowly progressing disease 2) usually affects both eyes 3) initially, patients awaken with blurred vision that will gradually clear during the day - this occurs because the cornea is normally thicker in the morning - it retains fluids during sleep that evaporate in the tear film while we are awake 4) as the disease worsens, this swelling will remain constant & reduce vision throughout the day Management: 1) reduce the swelling with drops, ointments, or soft contact lenses 2) use a hair dryer, held at arm's length or directed across the face, to dry out the epithelial blisters 2-3 times a day 3) corneal transplant: short-term success is good

References

Fuchs' dystrophy http://www.nei.nih.gov/health/cornealdisease/index.asp#e