REIS-BUCKLER’S’ CORNEAL DYSTROPHY

Reis-Bucklers’ corneal dystrophy is bilateral and is autosomal dominant with strong penetrance.
It presents in early childhood with the complaint of irritations and light sensitivity and then visual loss starts to occur as the cornea opacifies in the bowman’s layer.  The corneal surface becomes very distorted and there is a honeycomb appearance. 
Hard lenses, especially scleral lenses, are beneficial for vision, even in moderate to advanced stages.  In severe cases corneal transplants can be performed, however recurrence of the dystrophy in the grafts is common.  Therapeutic Laser Keratectomy may also be performed. 
This photo is in a 32 year old male whose mother also had the condition. 

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