Granular

• Autosomal dominantly inherited.

Clinical Features

• Symptoms:
  • Glare, foreign body sensation, decreased vision or recurrent painful epithelial erosions
  • In young adults, vision is usually unaffected
  • By the age 40, visual acuity will decrease insidiously as the lesions increase in size, multiply, coalesce, and distribute to a deeper stroma
• Signs:
  • Bilateral
  • Relatively symmetric, discrete, well demarcated crumb-like, white-grey granules in the axial anterior central stroma sparing the 1-3 mm of periphery cornea
  • Stroma in between lesions remains clear
  • Usually becomes apparent with recurrent corneal erosions during the first and second decade of life

Management

• Superficial debridement, lamellar keratoplasty, and excimer laser phototherapeutic keratectomy usually provide good outcomes.
• Penetrating keratoplasty is rarely required but indicated in advanced cases with extensive stromal involvement and significant decrease in visual acuity.
• Recurrent granular dystrophy may occur after keratoplasty.