- Autosomal dominantly inherited with very high penetrance and moderately
variable expressivity.
- Clinical features:
- Symptoms: glare, foreign body sensation, decreased vision or
recurrent painful epithelial erosions.
- Signs:
- Bilateral, often asymmetric, grey-white well-circumscribed
central anterior stromal opacity similar to those seen in
granular dystrophy
- Deep lattice-like stromal deposits
- Older patients have anterior stromal haze between deposits
- Family trace study revealed that patients with this dystrophy
were from Avellino, Italy
- Management:
- Superficial debridement, lamellar keratoplasty, and excimer
laser phototherapeutic keratectomy.
- Penetrating keratoplasty is indicated for deeper involvement.
- Recurrence may occur after keratoplasty.
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