Corneal dystrophy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Human corneal dystrophies is a group of disorders, characterised by a noninflammatory, inherited, bilateral opacity of the transparent front part of the human eye called the cornea. There is a number of rare forms varying in expression and degree of vision loss.^[1]

The International Committee for Classification of Corneal Dystrophies (IC3D) was established in 2005 and created a new classification system employing the latest data of molecular biology an genetics.^[2]

• Fuchs’ dystrophy
• Macular corneal dystrophy
• Reis-Bucklers corneal dystrophy (CDB1)
• Thiel-Behnke dystrophy (CDB2)
• Meesmann juvenile epithelial corneal dystrophy (MECD, Stocker-Holt dystrophy, OMIM 122100)
• Gelatinous drop-like corneal dystrophy
• Subepithelial mucinous corneal dystrophy
• Lisch epithelial dystrophy
• Granular corneal dystrophy type I
• Granular corneal dystrophy type II (also Avellino corneal dystrophy)
• Lattice corneal dystrophy type I
• Lattice corneal dystrophy type II
• Fleck dystrophy
• Schnyder corneal dystrophy
• Posterior amorphous corneal dystrophy
• Congenital stromal dystrophy (CSCD)
• Posterior polymorphous dystrophy type 1
• Posterior polymorphous dystrophy type 2
• Posterior polymorphous dystrophy type 3
• Congenital endothelial dystrophy type 1 (CHED1)
• Congenital endothelial dystrophy type 2 (CHED2)
• X-linked endothelial corneal dystrophy
• Epithelial basement membrane dystrophy (OMIM 121820), called a corneal dystrophy but in reality this condition is not inherited in the majority of cases, representing a non-specific reaction to a variety of corneal insults.^[1]

• Keratoconus
• Keratoglobus

• The IC3D Classification of the Corneal Dystrophies (PDF) – The Cornea Society website.

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