- A slowly progressive, uncommon, dominantly inherited condition.
- Clinical features:
- Usually bilateral but often asymmetric.
- Variable clinical features include isolated or coalescent posterior
corneal vesicular (the most distinctive characteristic), multi-layered
Descemet's membrane thickening and band-like configuration with
sharp scalloped margin.
- The peripheral abnormal multi-layered endothelial cells may
cause localized iridocorneal adhesion.
- Symptom: most cases are asymptomatic and do not require treatment.
- If the condition progresses to corneal decompensation, definitive
treatment with penetrating keratoplasty is usually required.
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