Fuchs’ endothelial corneal dystrophy (FECD) is a bilateral, asymmetric, slowly progressive disorder specific to the corneal endothelium, resulting in decreased visual function and in some cases pain, secondary microbial infection, and corneal neovascularization (Figure 5.1A). The disease was first described in 1910 by Ernst Fuchs, an Austrian ophthalmologist. FECD is an age-related disorder that affects 4% of the population over 40 years of age and its typical symptomatic onset is in the fifth or sixth decade of life^[1]; however, an early form of the disease does exist.^[2,3] Women are predominantly affected and familial clustering is commonly seen with this disease, which suggests an autosomal-dominant inheritance with incomplete penetrance.^[4–6]

The two different forms of FECD are mainly distinguished by the time of onset of disease. The more typical form presents in the …