ABSTRACT
The Scheie phenotype has been of particular interest to ophthalmologists, because patients live long enough for the severe corneal clouding to affect vision. It is most dense on the periphery. The patient may first be aware early in the second decade, but it is diagnosable by slit lamp very early. There may also be pigmentary degeneration of the retina. Some patients develop glaucoma. Visual impairment may progress to blindness.
