What is Fuch’s Dystrophy?

Fuch’s Corneal Dystrophy is an inherited, slowly progressive disease of the cornea (the clear “windshield” of the eye). Most people with Fuch’s Dystrophy won’t experience symptoms until they reach their 50’s or 60’s.

Our cornea is made up of 5 distinct layers, with the innermost layer, the endothelium, being responsible for controlling the proper hydration of the cornea.  The endothelium is made up of thousands of pump cells that draw fluid out of the cornea to keep our cornea clear.  Patients with Fuch’s Dystrophy slowly lose pump cells and this results in corneal swelling or edema.  Increased corneal swelling leads to a cornea that becomes less clear and more cloudy or less transparent. A swollen and cloudy cornea leads to the symptoms patients with advancing Fuch’s Dystrophy experience.

Symptoms

Diagnosis

Your eye doctor can examine the cornea and look for signs of Fuch’s Dystrophy.  Specifically, your doctor will look for the presence of corneal guttata which are microscopic excrescences that form within the corneal endothelium.  The amount or degree of guttata usually correlates with the amount of endothelial pump cell loss or dysfunction.  Your doctor can further quantify the degree of Fuch’s Dystrophy present by measuring the corneal thickness (corneal pachymetry) and by performing an endothelial cell count (count the number of healthy/normal endothelial cells).  After baseline measurements, these tests can be used to monitor the progression of Fuch’s Dystrophy on a yearly basis at your eye examination.

Treatment

How do you treat Fuch’s Dystrophy?

The majority of “treatment” needed for Fuch’s Dystrophy is yearly monitoring of the corneal status by measuring the cornea for increased swelling and loss of endothelial pump cells.  There currently is no therapy to stop the progression of endothelial pump cell loss.  Fortunately, Fuch’s Dystrophy usually progresses very slowly over the years.  Your doctor may recommend an over the counter eye drop or ointment to reduce the symptoms associated with corneal swelling. When symptoms become bothersome, a corneal transplant (Endothelial Keratoplasty) of the endothelial cells can be performed to restore your vision.

When do you treat Fuch’s Dystrophy?

The two most common reasons to proceed with surgery (Endothelial Keratoplasty) are vision changes that do not improve with conservative treatment and/or eye pain due to the formation of corneal “blisters” on the surface of the cornea.

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