Corneal Dystrophy, Band-Shaped

Clinical Features

Streiff and Zwahlen (1946) observed the disorder in 3 of 9 children of a first-cousin mating. The opacity began at puberty in 2 but was already present at birth in the third. The opacity forms a well-delimited band across the cornea at the level of the pupil and occupying the region of the palpebral fissure. It is denser centrally and consists of many small grayish elements like tapioca grains. Corneal diagrams and the pedigree were reproduced by Waardenburg et al. (1961).

Waardenburg et al. (1961) noted that this rare hereditary form of band-shaped corneal dystrophy was reported in brother and sister, aged 11 and 16 years, by Fuchs (1939) and in father and son by Glees (1950).