Fuchs endothelial corneal dystrophy and corneal endothelial diseases: East meets West

Abstract

Fuchs endothelial corneal dystrophy (FECD) is amongst one of the most common indications for endothelial keratoplasty worldwide. Despite being originally described among Caucasians, it is now known to be prevalent among a large number of populations, including Asians. While the FECD phenotype is classically described as that of central guttate and pigment deposits associated with corneal endothelial dysfunction, there are subtle yet important differences in how FECD and its phenocopies may present in Caucasians vs Asians. Such differences are paralled by genotypic variations and disease management preferences which appear to be geographically and ethnically delineated. This article provides a succinct review of such differences, with a focus on diagnostic and management issues which may be encountered by ophthalmologists practicing in the different geographic regions, when evaluating a patient with FECD.

Fig. 1

Retroilluminated anterior segment image of Fuchs endothelial corneal dystrophy. Left—Caucasian patient; Right—Asian patient

Fig. 2

Anterior segment photographs. a Fuchs endothelial corneal dystrophy (FECD) in an Asian eye. b FECD with stromal scarring (scar indicated by *). c FECD complicated by bullous keratopathy (epithelial bullae indicated by ^). d Slit-beam demonstrating concommitant shallow anterior chamber and a brunescent cataract in the same eye. e Krukenberg’s spindle in an Asian eye with pigment dispersion syndrome (white arrowhead). f CMV endotheliitis with endothelial pigment deposits and a ‘coin-lesion’/nummular keratic precipitate (black arrowhead). g ICE syndrome in a Caucasian patient with corectopia (white arrowhead) and peripheral anterior synechiae at the 5 o’clock position (black arrowhead). h ICE syndrome in an Asian patient of the essential iris atrophy subtype

Fig. 3

Anterior segment photographs of a phakic Asian eye with the concommitant diagnoses of a Fuchs endothelial corneal dystrophy (white arrowhead indicating endothelial pigments and guttata) and b primary angle closure suspect with shallow anterior chamber, for which c laser peripheral iridotomy (black arrowhead) has been performed

Fig. 4

Current and experimental therapeutic options for the management of Fuchs endothelial corneal dystrophy