Characterized by progressive loss of corneal endothelial cells, thickening of Descement’s membrane and deposition of extracellular matrix in the form of guttae.
- M/C endothelial dystrophy
Pathophysiology
FECD is characterized by the morphological changes of the hexagonal mosaic, accelerated loss of endothelial cells, and a concomitant increase in the extracellular matrix deposition at the level of DM. As a result, the endothelial layer is eventually no longer able to support corneal deturgescence (a state in which corneal stroma is maintained relatively dehydrated), leading to corneal edema and decreased in visual acuity
Clinical features
4 distinct clinical stages:
- Stage 1: Asymptomatic (however, slit-lamp examination exhibits central nonconfluent guttata and thickened Descemet’s membrane)
- Stage 2: Guttata coalesce; corneal endothelial polymegathism (variation in cell size), pleomorphism (variation in cell shape), and cell loss present; symptoms of blurry vision and glare, especially when awakening
- Stage 3: Dysfunction of the endothelial pump; formation of bullae in epithelial and subepithelial layers; development of corneal edema
- Stage 4: Corneal edema results in haziness, scarring, and decreased vision
Management
Corneal transplantation
Only modality used to restore vision.