Fuchs' corneal dystrophy. A clinicopathologic study of the variation in corneal edema.

Corneal buttons from six patients with Fuchs' dystrophy had varying degrees of clinical edema measured in most cases by preoperative optical or ultrasonic pachymetry. These were sectioned in the operating room so that histologic correlations could be made. Histologically, marked thickening of Descemet's membrane and abnormal corneal endothelium corresponded to areas of severe clinical edema and were usually located in the central and paracentral regions. Descemet's membrane displayed multiple prominent guttata of varying size and shape, either facing the anterior chamber, or buried within multilaminar Descemet's membrane. In some corneas, aggregates of 10 nm fibrils were seen at the edges of guttata, corresponding to areas that stained for oxytalan fibrils. The endothelium was attenuated underlying the guttata. Clinical edema was not present unless accompanied by marked thickening of Descemet's membrane with multiple guttata and attenuation of corneal endothelium. The peripheral cornea was relatively clear clinically and showed minimal histologic changes.