Corneal Dystrophy, Band-Shaped

Background and History: 

The cornea is the clear tissue in the front of the eye and serves as the windshield.  It is an active, multilayered tissue subject to many metabolic errors leading to progressive degeneration (dystrophy) and opacification (clouding) that result in loss of vision.  These errors are usually inherited but other causes such as trauma and chronic eye inflammation may also be responsible.

The condition described here occurs when the cloudiness is located in a horizontal band across the cornea.

Clinical Correlations: 

The band across the cornea was noted at birth in one child but usually it is not evident until later in the first decade of life.  Since it is usually located in front of the pupil, vision is decreased.  The eyes appear red and irritated.  It may create a sensation of a foreign body with scratchiness and excessive tearing.

There are no known general health problems associated with this condition.


Only three families with familial, isolated band keratopathy have been reported.  These were described in the mid-twentieth century and it is possible that they had underlying ocular and corneal disease.  In one family 3 of 9 children, the product of a first-cousin mating, were affected consistent with autosomal recessive inheritance.  In two of these the keratopathy was first noted during puberty while it was present at birth in the third child.  Although no causative gene mutation has been found, the family pattern suggests that such families can expect a risk of 25% for each subsequent child to have the same corneal disease.

In another family the band keratopathy was seen in a brother and sister at 11 and 16 years old.

In the third family a father and son were affected which is a vertical pattern of transmission consistent with autosomal dominant inheritance.  In this case, an affected parent could expect that half of their children will also have this corneal dystrophy.

Diagnosis and Prognosis: 

The diagnosis is made by an ophthalmologist (medical eye doctor).  No treatment has been reported but a corneal transplant might restore vision and reduce irritation.  Longevity is not impacted.

Additional Information
Autosomal dominant
Autosomal recessive