Fuchs’ Dystrophy and its Progression

Fuchs’ dystrophy (fooks DIS-truh-fee) causes the innermost layer of cells in the cornea, the endothelium, to degenerate. The endothelium regulates the amount of fluid in the cornea, pumping out excess fluid that could cause swelling. Fuchs’ dystrophy causes endothelial cells to die, leading to edema in the cornea. Fuchs’ dystrophy is generally bilateral, and the resultant swelling causes gradual vision loss. The swelling in the endothelium can also cause the development of bullae, resulting in bullous keratopathy.

Genetic Causes of Fuchs’ Dystrophy
The cause of Fuchs’ Dystrophy is not always known, although it appears to sometimes have a genetic component. If your mother or father has Fuchs’ dystrophy, you have a 50% chance of getting it. We know that Fuchs’ dystrophy begins causing vision problems after age 50, and especially in women.

Symptoms of Fuchs’ Dystrophy

  • Glare and Sensitivity to Light
  • Pain
  • Blurred or clouded vision
  • Halos around lights
  • Reduced night vision
  • Poor morning vision
  • Foreign body sensation

Diagnosis of Fuchs’ Dystrophy

While visual symptoms don’t develop until later in life, a doctor can often diagnose Fuchs’ dystrophy before then. The doctor will use a slit lamp, a mechanism that shines a high-intensity sheet of light into the eye, to examine the cornea. The slit lamp allows for high magnification. If Fuchs’ dystrophy is present, the doctor may see a reduced number of endothelial cells and small lesions (corneal guttata). The doctor might also measure the thickness of the cornea, looking for potential swelling.

Treatment of Fuchs’ Dystrophy

Early on, special eye drops can be used to reduce excess water from the cornea. This can help to improve your vision.

Symptoms can be managed in other ways; for example, photophobia can be reduced with special eyeglasses (photochromic lenses). Anti-reflective coating on your glasses can also help prevent annoying glare.

Your doctor will want to manage any other condition that can worsen Fuchs’ dystrophy. For example, if you have a condition that can cause high intraocular pressure, the doctor may recommend glaucoma eye drops.
Later in the progression of Fuchs’ dystrophy, the bullae described above can rupture. If the eye is damaged with corneal abrasions and the eyesight is significantly harmed, the doctor may recommend a corneal transplant. Amniotic membrane can be used to heal after such a transplant. Because of the difficulty in finding corneal donors, and the risks of rejection, less intrusive procedures are also being developed, such as penetrating keratoplasty, and FS-DSEK, a procedure called Descemet’s stripping. Research suggests that by selectively replacing the diseased endothelium and Descemet’s membrane, vision might be restored with fewer complications than with PK, at least for a time.

Fuchs’ dystrophy develops over 10-20 years.

Precautions for Fuchs’ dystrophy

Talk to your doctor before having any eye surgery such as LASIK or cataract surgery, as these can worsen Fuchs’ dystrophy.


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