Reis-Buckler's dystrophy
 

Reis-Buckler's dystrophy with ferritin lines An advanced case of Reis-Buckler's

Of all the epithelial dystrophies you may see in the examination, this is probably the most common. There are multiple subepithelial ring-shaped opacities (so called honeycomb apperance) in the centre of the cornea  (but this may extend towards the periphery in severe cases). The corneal surface is irregular with ferritin deposition. Look at the opposite eye for: similar changes corneal graft which may be penetrating or lamellar .

Question:

1. What are the features of Reis-Buckler's dystrophy?

Answer
Autosomal dominant
Appear in the first few years of life and mainly affects the Bowman's membrane
Symptoms begin in the first or second decade with painful, recurrent epithelial erosion. Vision is affected by both anterior scarring with surface irregularity and anterior stromal oedema (from recurrent corneal erosion)
Histologically, there is disruption and absence of Bowman's layer, with replacement by fibrocellular tissue that corresponds clinically to areas of subepithelial opacification.
Keratoplasty is the treatment of choice (either penetrating or lamellar), recurrence in the graft is common.

 

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