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- Autosomal dominantly inherited.
- Decreased vision
- Spontaneous corneal erosions usually present during early childhood
- Recurrent photophobia and irritation.
- 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
- 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