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Ocular System

Fuchs endothelial corneal dystrophy (FECD)

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

Pathogenesis of FECD. Genetic and environmental factors lead to corneal endothelial cell loss, resulting in corneal edema and blurring of vision. Some characteristic histological findings are endothelial cell morphological changes, thickened Descemet’s membrane, and guttae formation. | Elhalis, H., Azizi, B., & Jurkunas, U. V. (2010). Fuchs endothelial corneal dystrophy. The ocular surface, 8(4), 173–184. https://doi.org/10.1016/s1542-0124(12)70232-x

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.

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