- 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.
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