Fuch’s Endothelial Dystrophy

Abstract

Fuchs endothelial corneal dystrophy (FED) is a bilateral, slowly progressive, often asymmetric corneal disease characterized by deterioration of endothelial cells and development of guttata, which are excrescences of Descemet’s membrane. FED slowly progresses to a great extent of endothelial cell loss, subsequent loss of corneal deturgescence, and bilateral corneal edema of the stroma or epithelial layers, leading to ocular pain, glare, halos, and decreased visual acuity. FED is the most common form of corneal dystrophy that affects the endothelium. It is also the most common dystrophy that requires keratoplasty for management worldwide. In general, the distinguishing feature between “cornea guttata” and FED is the presence of corneal edema in FED. Of note, there is a form of non-guttate form of Fuchs dystrophy in which patients develop corneal edema due to degenerating endothelial cells that do not contain excrescences of Descemet’s membrane. Since non-guttate forms of FED are rare and uncommonly reported in the literature, the majority of the discussion here will focus on FED with the presence of guttata.FED was first documented in 1910 when Viennese ophthalmologist Ernst Fuchs reported 13 elderly patients with bilateral central clouding. Later, Kraupa described the continuum of corneal changes in FED, and Vogt coined the term “guttata” (Latin: gutta = droplet) in 1921. Since that time, there have been several research studies conducted on guttata and their association with FED. These studies have contributed to the understanding of the pathophysiology of the disease, although more research is necessary to determine its exact mechanism and progression. Additionally, the management of FED has dramatically evolved over the past 20 years and has significantly improved the quality of life of patients. This activity reviews the etiology, epidemiology, pathophysiology, diagnosis, evaluation, management, and prognosis of FED.