The cornea functions as the clear ‘windshield’ of the eye. Diseases of the cornea such as this one often lead to visual difficulties when it becomes clouded due to disease. There are numerous dystrophies (degenerative) disorders of the cornea and most are inherited. Various layers of the cornea may be involved. A number of corneal dystrophies are caused by malfunction of the most posterior layer called the endothelium. The original report was by a German ophthalmologist, E. Fuchs, in 1910.
As a result of the malfunction of the endothelium, fluid in the eye, known as the aqueous humor, diffuses into the cornea causing swelling and blurry vision. Two forms of this disorder are recognized, one with an early onset from the first through the 4th decade of life, and a later form in which clinical symptoms may not be significant until the 7th or 8th decade of life. However, your ophthalmologist can often detect signs of the disease at least 2 to 3 decades earlier. The swelling of the cornea is progressive and in advanced cases may lead to painful, recurrent erosions of the anterior layers and, of course, significant visual handicaps. No other part of the body is affected.
This disorder is usually inherited directly from one generation to the next (autosomal dominant), i.e., parent to child. Curiously, women seem to be more often affected and have more severe disease than males.
Only your eye doctor can make this diagnosis. It may be evident long before symptoms are noted but the clinical disease is relentless and in advanced cases, may require a corneal transplant to restore vision and comfort. The post-surgical prognosis is excellent, however.