Granular

Granular dystrophy and recurrent dystrophy post penetrating keratoplasty. 

  • 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.