Corneal Dystrophy, Reis-Bücklers

Background and History: 

The cornea is the normally transparent front part (windshield) of the eye.  A number of gene mutations can cause cloudiness of the cornea which leads to blurry vision and irritation.  This type is named for two German ophthalmologists, Heinrich Maria Wilhelm Reis and Max Bucklers who first described it in separate papers in the early part of the 20th century.

Clinical Correlations: 

Irregularly confluent opacities often appear in the first decade of life and even then can cause some clouding of vision.  The surface layer of the eye often breaks down causing severe pain, tearing, light sensitivity, and discomfort.  These episodes may become less frequent after the end of the second decade.  However, the corneal cloudiness continues to worsen and may require surgical treatment.


This is an autosomal dominant disorder caused by a gene mutation.  It can be passed in a vertical pattern directly from parent to child with a probability of 50%.

Diagnosis and Prognosis: 

Reis-B√ºcklers corneal dystrophy can only be diagnosed by an ophthalmologist.  The surface irregularities causing discomfort may respond to conservative treatment using drops or bandages.  However, surgical treatment of the cornea may be required in severe cases but the breakdown of tissue can recur.

Additional Information
Autosomal dominant