Fuch’s Corneal Dystrophy

Cornea, Fuch's Cornea Dystrophy
Eye cross section pointing out the cornea

Fuch’s corneal dystrophy is an inherited, slowly progressive disease of the cornea (the clear “windshield” of the eye).

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 thousand of pump cells that draw fluid through the cornea and maintain a perfect balance of hydration that keeps our cornea clear.  Patients with Fuch’s Dystrophy slowly lose pump cells which results in corneal swelling.  Increased corneal swelling leads to a cornea that becomes less clear and more cloudy or less transparent.

Symptoms of Fuch’s Dystrophy

  • consistent hazy or cloudy vision
  • blurred vision upon wakening that clears hours later
  • increased halo’s around lights
  • with advanced disease:  episodic pain due to blister formation on the corneal surface

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 excressences 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).  Upon 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 and measuring the cornea for increased swelling and loss of endothelial pump cells.  There is no current therapy to stop or halt the progression of endothelial pump cell loss.  Fortunately, Fuch’s Dystrophy usually progresses VERY slowly over YEARS.  When necessary a corneal transplant (Endothelial Keratoplasty) of the endothelial cells can be performed.

When do you treat Fuch’s Dystrophy?  The two most common reasons to proceed with surgery (Endothelial Keratoplasty) are constant blurred vision and/or consistent eye pain due to the formation of corneal “blisters” on the surface of the cornea.

Endothelial Keratoplasty (also referred to as DSEK, DSAEK, DMEK)

Endothelial Keratoplasty involves removing the sickened endothelial cells of a patient with Fuch’s Dystrophy and replacing those cells with healthy endothelial cells from a person who has recently passed away.

Watch the video below to learn more about Fuch’s Cornea Dystrophy:

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