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Dystrophies
Granular dystrophy demonstrating discrete, well-defined round and oval shapes lesions simulating bread crumbs with clear stroma in between lesions.

  • 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.

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