Corneal Dystrophy, Posterior Amorphous

Background and History: 

This form of corneal disease has only been described in a few families.   It is one of a few corneal dystrophies that have other eye abnormalities.

Clinical Correlations: 

This seems to be a congenital disorder, that is, developmental abnormalities are present from birth.  The iris may be partially attached to the cornea and the pupil may be abnormal.  The most apparent feature though is a variable degree of clouding of the cornea.  This is usually not significant enough to cause a decrease in vision although the amount of clouding varies widely.


No genetic mutation has yet been identified.  However, all reported families have had an inheritance pattern consistent with autosomal dominant transmission.  That is, the disease seems to pass directly from parent to child with a probability of 50% for each child.

Diagnosis and Prognosis: 

Only an eye doctor can diagnose this disorder.  Most patients do well throughout their lives since this seems to be a nonprogresssive disorder but a few have required corneal transplantation to achieve good vision.

Additional Information
Autosomal dominant