Fuchs Endothelial Corneal Dystrophy: Clinical, Genetic, Pathophysiologic, and Therapeutic Aspects

Annu Rev Vis Sci. 2019 Sep 15:5:151-175. doi: 10.1146/annurev-vision-091718-014852.

Abstract

Fuchs endothelial corneal dystrophy (FECD) is a bilateral corneal endothelial disorder and the most common cause of corneal transplantation worldwide. Professor Ernst Fuchs described the first 13 cases of FECD more than 100 years ago. Since then, we have seen far-reaching progress in its diagnosis and treatment. In the field of diagnostics, new technologies enable the development of more accurate classification systems and the more detailed breakdown of the genetic basis of FECD. Laboratory studies help in deciphering the molecular pathomechanisms. The development of minimally invasive surgical techniques leads to a continuous improvement of the postoperative result. This review highlights and discusses clinical, genetic, pathophysiologic, and therapeutic aspects of this common and important corneal disorder.

Keywords: FECD; Fuchs endothelial corneal dystrophy; corneal dystrophy; corneal endothelium; keratoplasty.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Corneal Transplantation
  • Descemet Stripping Endothelial Keratoplasty
  • Endothelium, Corneal / physiopathology
  • Fuchs' Endothelial Dystrophy* / genetics
  • Fuchs' Endothelial Dystrophy* / physiopathology
  • Fuchs' Endothelial Dystrophy* / surgery
  • Humans
  • Visual Acuity / physiology