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Reis-Bucklers' Dystrophy

  • Autosomal dominantly inherited.
  • Clinical features:
    • Symptoms:
      • Decreased vision
      • Spontaneous corneal erosions usually present during early childhood
      • Recurrent photophobia and irritation.
    • Signs:
      • Fine reticular superficial corneal opacities
      • By the second and third decades, central corneal opacities develop in a honeycomb, fishnet, or ring-like pattern
      • Mainly affect the central and mid peripheral cornea, sparing the peripheral cornea
      • Diffuse superficial stromal haze evolves with increased central corneal thickness, irregular astigmatism, and decreased corneal sensation.
      • Prominent corneal nerves are often present
  • Management:
    • Superficial keratectomy to treat superficial corneal scarring.
    • Lamellar or penetrating keratoplasty may be performed in more severe cases.
    • Recurrence of the dystrophy in the graft is not uncommon.
    • Excimer laser phototherapeutic keratectomy

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