- 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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