Reis- Bucklers'

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