Corneal Dystrophy, Band-Shaped

Clinical Characteristics
Ocular Features: 

Symptoms of ocular irritation with tearing, conjunctival injection and decreased vision can be present at birth but more often is evident later in the first decade of life.  The band is located in the cornea in the palpebral fissure area in a horizontal pattern.  Apparently no other lesions are present in the eye.    

Systemic Features: 

None reported.

Genetics

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.

 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.

Treatment
Treatment Options: 

Topically applied EDTA solutions are sometimes effective in removing lesions consisting of calcium deposits but this has not been reported to be effective in the hereditary form of band keratopathy. 

References
Article Title: 

References

Glees, M. Ueber familiaeres Auftreten der primaeren, bandfoermigen Hornhautdegeneration. Klin. Monatsbl. Augenheilkd. 116: 185-187, 1950.

Streiff, E. B., Zwahlen, P. Une famille avec degenerescence en bandelette de la cornee. Ophthalmologica III: 129-134, 1946

Fuchs, A. Ueber primaere guertelfoermige Hornhauttruebung. Klin. Monatsbl. Augenheilkd. 103: 300-309, 1939.