Molecular and Histopathological Changes Associated with Keratoconus

Abstract

Keratoconus (KC) is a corneal thinning disorder that leads to loss of visual acuity through ectasia, opacity, and irregular astigmatism. It is one of the leading indicators for corneal transplantation in the Western countries. KC usually starts at puberty and progresses until the third or fourth decade; however its progression differs among patients. In the keratoconic cornea, all layers except the endothelium have been shown to have histopathological structural changes. Despite numerous studies in the last several decades, the mechanisms of KC development and progression remain unclear. Both genetic and environmental factors may contribute to the pathogenesis of KC. Many previous articles have reviewed the genetic aspects of KC, but in this review we summarize the histopathological features of different layers of cornea and discuss the differentially expressed proteins in the KC-affected cornea. This summary will help emphasize the major molecular defects in KC and identify additional research areas related to KC, potentially opening up possibilities for novel methods of KC prevention and therapeutic intervention.

Figure 1

Cone shaped phenotype of the cornea in a keratoconus patient.

Figure 2

Sign of Vogt's striae showing fine vertical lines in deep stroma and Descemet's membrane of a keratoconus patient.

Figure 3

Münson's sign in a keratoconus patient which appears as bulging of the lower lid during downgaze.

Figure 4

A potential physiological model for the pathogenesis of keratoconus.