This disease makes a type of cornea cells (called endothelial cells) stop working. Congenital Hereditary Endothelial Dystrophy (CHED), one of the Congenital Corneal Opacities (CCO), is a bilateral corneal condition characterized by cloudy cornea that may be present from birth or may be infantile in onset. There are different kinds of corneal dystrophy based on which layer of the cornea is affected and the reasons for deterioration, including: Endothelial dystrophy: This affects the innermost layer of the cornea. For endothelial dystrophies, such as Fuchs’ dystrophy, a partial cornea transplant (or endothelial keratoplasty) is used. Also called Fuchs' corneal dystrophy and Fuchs' endothelial dystrophy, the disease usually affects both eyes and causes a gradual decline in vision due to corneal … Fuchs’ dystrophy is caused by the destruction of endothelium cells in the cornea. The Observational Sub-study will enroll approximately 25 to 50 subjects. Congenital hereditary endothelial corneal dystrophy is characterized by a diffuse ground-glass appearance of both corneas and markedly thickened (2–3 times thicker than normal) corneas from birth or infancy. Though only a single cell layer thick, this layer is vital in maintaining a clear cornea for functional vision. Up to 71 eligible subjects with moderate to severe corneal endothelial dystrophy (defined as Endothelial Cell Density < 2000 mm^2) in at least one eye will be enrolled and randomized in the main study. Fuchs’ Corneal Dystrophy Causes and Risk Factors The innermost layer of your cornea, called the endothelium, removes fluids from the cornea to keep it clear. The precise cause of this cellular destruction isn’t known. Symptoms of Fuchs’ dystrophy include: [1] As a consequence of the endothelial dystrophy, the cornea becomes edematous, which leads to a degradation in the patient’s vision. When these cells stop working, the cornea swells and gets thicker. The endothelium keeps the cornea clear for good vision by pumping out excess fluid that could cause corneal swelling. The general term corneal dystrophy refers to a group of corneal diseases. A corneal dystrophy is a rare genetic eye condition in which one or more parts of the clear outer layer of the eye (the cornea) lose their normal clarity as a result of a buildup of cloudy material. Transplanting the inner layer of the cornea. Endothelial Dystrophy – The cornea is normally the transparent “windshield” of the eye, serving to protect the internal structures of the eye while allowing light to enter for vision. Epithelial dystrophy: This affects the outermost layer of the cornea. Posterior Polymorphous Corneal Dystrophy (PPMD, PPCD), also known as Schlichting dystrophy, is an autosomal dominant disorder of the corneal endothelium and Descemet’s membrane, producing a wide variability in clinical presentation. The procedure is usually done with local anesthesia in an outpatient setting. Most people with Fuchs’ dystrophy start to have symptoms around age 50 to 60. These cornea changes can cause vision problems. In more severe cases, a corneal transplant (called keratoplasty) may be necessary. Transplanting the cornea. Known as Descemet membrane endothelial keratoplasty (DMEK), this procedure involves replacing the back layer of the cornea with healthy endothelial cells from a donor. The endothelium is a single layer of cells that lines the inside of the cornea. 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