Scott Burk, MD/PhD
Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA

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Figure 1-3: These are anterior segment photographs of 3 different individuals with the same condition.

1. What is your diagnosis?
   
2. What are the symptoms?
   
3. What is the usual presentation for this condition?
   
4. What is the pathophysiology of this condition?
   
5. What is the inheritance pattern of this condition?
   
6. What treatment would you recommend?
   
7. Dose this condition recur?
   

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1. What is your diagnosis?
   Answer: Reis-Bucklers' superficial corneal dystrophy, also known as the superficial variant of granular dystrophy.

    

2. What are the symptoms?
   Answer: Frequent recurrent corneal epithelial erosion leading to marked visual loss.

    

3. What is the usual presentation for this condition?
   Answer: The disease is diagnosed in the first years of life, symptoms become prominent in the first decade, leading to progressing to scarring and visual loss in the second, third, and fourth decades.

    

4. What is the pathophysiology of this condition?
   Answer: Disruption and loss of Bowman's membrane with replacement by fiberous scar tissue resulting in superficial corneal opacification.

    

5. What is the inheritance pattern of this condition?
   Answer: Autosomal dominant.

    

6. What treatment would you recommend?
   Answer: In the early stages of Reis-Bucklers' dystrophy treatment focuses on factors which control recurrent erosive episodes. These modalities include copious lubrication, patching, or bandage contact lenses. When visual acuity is limited by superficial scarring, excimer phototherapeutic keratectomy is the initial treatment of choice.

    

7. Does this condition recur?
   Answer: Yes, Reis-Bucklers' dystrophy recurs after excimer phototherapeutic keratectomy or corneal transplant.