Fuchs' Dystrophy

Fuchs’ endothelial dystrophy is a non-inflammatory, sporadic or autosomal dominant, dystrophy involving the endothelial layer of the cornea. With Fuchs’ dystrophy the cornea begins to swell causing glare, halo, and reduced visual acuity. The damage to the cornea in Fuchs’ endothelial dystrophy can be so severe as to cause corneal blindness.

Fuchs’ dystrophy is often inherited in an autosomal dominant manner. This means if you have an inherited form of Fuchs’ endothelial dystrophy there is a 50% chance you will pass it on to your children. Spontaneous mutation in the gene for Fuchs’ dystrophy also can cause new Fuchs’ dystrophy in a person with no family history. Currently there are studies underway to try and determine exactly which gene is responsible for the dystrophy, but as of yet we still do not know the exact gene.

Symptoms

Some of the earliest symptoms of Fuchs’ endothelial dystrophy include reduced contrast sensitivity and mild reduction of visual acuity. Patients will often notice glare around a point source of light or have difficulty with nighttime driving. As the dystrophy is often slow in onset, patients may not even notice that their vision is reduced. Once fluid begins to collect in the stroma, patients will start to notice fluctuation in vision, typically worse in the early AM and improving toward the end of the day. Vision may be worse on humid or rainy days, and better on dry days. Eventually the diurnal fluctuation reduces and vision becomes reduced all day long. Slowly the vision continues to worsen and eventually intermittent pain can be felt if bullae form and rupture leaving corneal epithelial defects.

 

Solution

As Fuchs’ dystrophy progresses its medical treatment may fail, at that point surgical management becomes necessary. For many years the only option for patients with visually significant Fuchs’ dystrophy was a full thickness corneal transplant or penetrating keratoplasty (PKP). A corneal transplant involves replacement of the full thickness of the cornea in order to replace the endothelial cells. The cornea is held in place with multiple sutures and some sutures may stay in place for several moths to years. Though the surgery is very successful, recovery can be relatively slow sometimes taking a year or more for full visual recovery. The advantage of a full thickness corneal transplant is that it can restore vision even in the most advanced stages of Fuchs’ dystrophy and other necessary intraocular surgery can be performed at the same time as the transplant.

 

For more information on Fuch's Dystrophy, See our Patient Education Brochure, Here.