Congenital Endothelial Dystrophy

Congenital Hereditary Endothelial Dystrophy (CHED) is a rare bilateral corneal condition characterized by cloudy cornea that may be present from birth or may be infantile in onset. As a consequence of the endothelial dystrophy, the cornea becomes edematous, which leads to a degradation in the new born vision.

 

Treatment

CHED children have varying visual acuities depending on the degree of corneal clouding.

Depending on the severity of the disease, a surgery might be indicated in order to replace the diseased endothelium. New techniques of selective keratoplasty (Descemet Membrane Endothelium Keratoplasty – DMEK)) are promising. In all cases, patients should be carefully followed and amblyopia should be treated.

Reference: Danielle Trief, MD, David C. Ritterband, MD, John Seedor, MD, and Emily Waisbren, MD. Congenital Hereditary Endothelial Dystrophy. Review of Ophthalmology - May 2016

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