Congenital Herditary Stromal Dystrophy of the Cornea

ABSTRACT

In 1978, a nonprogressive corneal dystrophy was seperated from other causes of congenital opacification on the basis of unique clinincal findings and characteristic electronmicroscopic findings. This disorder, termed congenital hereditary stromal dystrophy, appears to the result of disordered stromal fibrogenesis. Recently, the autors have experienced 4 members of a family with typical electronmicroscopic findings of congenital heaeditary stromal dystrophy of the cornea. In this report we describe the characteristic findings of congenital hereditary stromal dystrophy.