Avellino

• 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.