Fuchs' corneal dystrophy is an inherited disease of the cornea. It affects both eyes, causes corneal swelling and loss of vision, and may need to be treated with corneal transplant surgery. Fortunately, patients have a great chance of maintaining their vision in the long term.
The cornea is the clear outer layer of the eye. It consists of several layers. The innermost layer is called the endothelium. There is a natural tendency for fluid within the eye to enter the cornea and cause it to swell. The endothelium is responsible for pumping out this fluid from the cornea and maintaining a relative state of dehydration. It is in this state that the cornea is transparent and light may be transmitted and focused for clear vision. If the fluid cannot be pumped out, then the cornea swells and loses its transparency. As a result, light cannot be focused and vision is blurred.
Patients with advanced Fuchs dystrophy have a poor endothelium that is no longer able to pump the fluid out of the cornea, resulting in corneal edema and loss of vision. As the swelling develops, a patients’ vision may fluctuate. It may begin with cloudy vision early in the morning that seems to clear later in the day. As the swelling progresses, the vision remains cloudy longer during the day and eventually remains cloudy throughout the day. In the early stages of the swelling, medications may be helpful; however, the underlying problem of progressive endothelial cell loss cannot be halted.
Because this is an inherited condition, there is often a family history of Fuchs' Dystrophy or corneal transplant surgery. The inheritance pattern is autosomal dominant, which means that ½ of the offspring of an affected individual (both male and female) will receive the gene for Fuchs' Dystrophy and are at risk for developing this corneal deterioration.
Once the vision is cloudy, corneal transplant surgery is recommended. The new donor cornea comes with its own healthy and normal endothelium. Thus, once the donor cornea is healed, the endothelial function is restored and the cornea becomes transparent. Clear vision is recovered. There is a 95% success rate for corneal transplant surgery for this condition.
A surgery called Descemet’s Stripping Automated Endothelial Keratoplasty (DSAEK) has been developed and allows for a quicker recovery and better outcome than patients recieving a traditional cornea transplant. This surgery is commonly performed by our surgeons at Manatee Eye Clinic.
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