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Fuchs (Corneal) Dystrophy

Fuchs Dystrophy

Fuchs’(pronounced fÅ«ks not fyooks) endothelial dystrophy is a non-inflammatory, autosomal dominant, dystrophy involving the endothelial (innermost) 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.

Eye examination shows cloudiness and swelling in the center of the cornea and roughness on the inner layer of the cornea. It appears like orange peel rather than the glossy apple skin. This makes it seem like looking through frosted glass.

Treatment

General treatment is symptomatic. At this time there is no treatment to reverse or halt the progression of Fuchs dystrophy.

Medical therapy

Medical treatment of Fuchs’ dystrophy begins once patients notice fluctuation in vision. Typically, it is worse upon awakening and improves towards the end of the day as the eyes dry from air exposure between blinks. Here are the medical treatment options:

  • Hypertonic eye drop to pull fluid from the cornea is the early treatment option. Usually it is in the form of hypertonic saline drops and/or ointments (Muro 128). Use of the hypertonic saline may accelerate the time to visual recovery.
  • Air drying the cornea is helpful in moderate cases. Any activity that helps to evaporate fluid off the cornea will help shorten the time to visual recovery, this may include pointing car vents toward the face or blowing air by the eyes using a hair dryer at arms length.
  • Bandage contact lenses can also be quite helpful in management of painful ruptured bullae (blisters) in more severe disease.

Surgery

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 months to years. Over the past several years there has been a trend to try and treat endothelial dystrophies by transplanting only the posterior, or endothelial, portion (posterior lamella) of the cornea.  Posterior lamellar surgery has been refined over the past several years and now is becoming the standard of care in treatment of early to moderate Fuchs’ endothelial dystrophy.  The most popular surgical procedure is currently named Descemet’s Stripping Endothelial Keratoplasty (DSEK) or Descemet’s Stripping Automated Endothelial Keratoplasty (DSAEK).  Please refer to Corneal Transplant and DSEK sections on this website for more details.