Degenerative or hereditary corneal diseases are sometimes difficult to discriminate. Corneal dystrophies affect approximately 0.09% of the population. They are identified by the IC3D classification based on their phenotype, genotype and evidence gathered for their diagnosis. In practice, the ophthalmologist manages functional symptoms such as recurrent erosions, visual loss and amblyopia, photophobia, foreign body sensation, and sometimes pain and aesthetic concerns. Medical treatments consist of drops to promote healing, ointments, hyperosmotic agents and bandage contact lenses. Less invasive surgical treatments are used as second line therapy (phototherapeutic keratectomy, lamellar keratectomy). More invasive procedures may eventually be utilized (lamellar or penetrating keratoplasty). Anterior lamellar or endothelial keratoplasty are now preferred to penetrating keratoplasty, although the latter still remains the only possible option in some cases. Some rare dystrophies require coordinated and comprehensive medical care.Le texte complet de cet article est disponible en PDF.
Keywords : Corneal dystrophy, Hereditary corneal condition, Keratectomy, Keratoplasty, Genetic, Epithelial dystrophy, Stromal dystrophy, Granular dystrophy, Endothelial dystrophy, Classification
|☆|| See this article unabridged, illustrated and detailed, with electronic enhancements in EMC-Ophtalmologie: Bourges JL. Les dystrophies de cornée. EMC – Ophtalmologie 2017 [Article 21-200-D-30].