Fuchs' Corneal Dystrophy is a slowly progressing disease that occurs when endothelial cells lining the inside of the cornea begin to die off. The disease generally affects people over 50, although early signs of the disease may be evident in the thirties or forties. There is no known cause of Fuchs' dystrophy, but often the disorder is inherited. Fuchs' dystrophy tends to affect women more than men and generally involves both eyes. The disease is one of the leading causes of corneal transplant surgery.
The cornea is the clear, dome-shaped outermost layer of the eye responsible for refracting light. It consists of five primary layers: the epithelium, Bowman's layer, the stroma, Descemet's membrane and the endothelium. Normally, the endothelium, or the delicate innermost layer, acts like a pump, moving fluid out of the stroma. In Fuchs' dystrophy, cells in the endothelium gradually deteriorate, and the pumping action becomes less efficient. When this happens, the cornea begins to swell and cloud, and vision is distorted. As the disease progresses, the endothelium may begin taking on water, causing further vision loss and pain.
Generally, doctors treat early stages of Fuchs' dystrophy with drops, ointments and/or contact lenses to reduce corneal swelling. However, as the disease progresses, vision impairment may begin to affect the patient's quality of life. In this case, corneal transplant surgery offers the best hope for restoring vision.