- 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.
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