Corneal Dystrophy, Thiel-Behnke

Background and History: 

The cornea is the normally clear ‘windshield’ of the eye.  A number of gene mutations lead to diseases of this tissue, often called dystrophies (degeneration), and various deposits of abnormal material can accumulate.  This type is distinctive for the honeycomb pattern of opacities in the cornea.

Clinical Correlations: 

 Symptoms in the first and second decades of life are primarily due to defects (like scratches) on the surface of the cornea which are painful and slow to heal.  Light sensitivity and excessive tearing are often associated with these erosions.  The disease is progressive as the cornea becomes increasingly cloudy which, of course, interferes with vision.  No other parts of the body seem to be affected.


This is an autosomal dominant disorder in which the family pattern of disease is a vertical one as parents pass the mutant gene to half their offspring on average.

Diagnosis and Prognosis: 

It may be possible to see a cloudy cornea by simple inspection but only your ophthalmologist can specifically diagnose this type of dystrophy.  Various treatments can temporarily alleviate symptoms and improve vision but the corneal clouding often recurs.

Additional Information
Autosomal dominant