Fuchs' dystrophy, also known as Fuchs' endothelial dystrophy, is a slowly progressing rare corneal disease that usually affects both eyes and is slightly more common in women than in men. Although doctors can often see early signs of Fuchs' dystrophy in people in their 30s and 40s, the disease rarely affects vision until people reach their 50s and 60s.
The cause of Fuchs' dystrophy is unknown, although it is an apparently inherited disorder. The inheritance is autosomal dominant with genetic and environmental modifiers such as increased prevalence in the elderly and in females.
This disease causes the endothelial cells that line the back surface of the cornea to deteriorate and die. Normally these cells prevent excess fluid from accumulating in the cornea. Without them the fluid builds up, which can cause swelling, cloudy vision, pain and loss of corneal transparency. This disease causes a variety of vision problems and can lead to blindness.
Endothelial cell loss may be aggravated or accelerated by intraocular trauma or surgery. A common scenario involves excessive corneal swelling or edema following cataract surgery or other types of ocular surgery. Hence, patients with a history of Fuchs' dystrophy may be at a greater risk of corneal edema after ocular surgery as they have fewer functioning endothelial cells.
Foggy or blurred vision upon awakening that may gradually clear as the day progresses
Visual impairment, distorted vision and changes in vision
Eye pain from tiny blisters on the surface of the cornea, caused by swelling in the cornea due to the water building up inside.
Sensitivity to light
Seeing halos around lights
Difficulty seeing at night
Blindness
The cornea is cloudy or hazy in appearance
If the disease isn't very far advanced you can get temporary relief under the care of an eye professional. At that point, treatment usually consists of topical hypertonic saline and the use of a hair dryer to dehydrate the precorneal tear film. In using a hair dryer, the patient is instructed to hold a hair dryer at an arm's length or directed across the face, to dry out the epithelial blisters. This can be done two or three times a day. Therapeutic soft contact lenses can be worn to improve vision and help reduce discomfort.
However, Fuchs' dystrophy isn't going to go away by itself. It's possible it may never progress very far, but it's also possible that it may become advanced rather quickly. Sometimes Fuchs' dystrophy will progress to the point where painful blisters burst on the surface of the cornea, scarring the tissue.
The only real cure for Fuchs' dystrophy is either a conventional corneal transplant, called penetrating keratoplasty (PKP), or Descemet’s stripping with endothelial keratoplasty (DSEK), an advanced technique for corneal transplantation. DSEK avoids the surgical complications of PKP such as wound dehiscence and infections and high postoperative astigmatism. The eye surgeons at Harvard Eye Associates are experts in this area of treatment.
The time to get a cornea transplant with Fuchs' dystrophy is as soon the symptoms begin to affect your daily life. Since the healing process takes quite awhile you want to to be able to depend on having good vision in the non-transplant eye during the healing phase of the transplant.
As always, Harvard Eye is first and foremost concerned about what is best for each individual patient. Call us today for your personalized consultation!
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