Corneal Fuchs Dystrophy

[Sally58]

I was recently diagnosed with Corneal Fuchs Dystrophy and I'm a 43-yr old woman.  How fast does Fuchs Dystrophy usually progress?  Can it start out progressing at a slow pace and later progress faster?  or vice versa?  How often should I have followup visits?  Every year?  What tests should I have at every followup visit?  Which are the most important tests to have to determine the progression of my disease?  What symptoms should I look out for that require an immediate visit to my dr's office?

Thank you for your answers.

Sally

[John C Hagan III, MD, FACS]

We have discussed Fuch's Dystrophy in the past at length. Please use the search feature on this page to research previous postings. This is one of the most informative:

There is no exact definition of how many guttatta it takes to diagnosis Fuchs' Endothelial Corneal Dystrophy. Some guttatta are part of aging and do not ever become a problem. Two tests that are often helpful are corneal endothelial cell counts (the number decreases as the disease develops and progresses) and corneal pachymetry (corneal thickness) the thickness increasing as the disease develops and progresses. Family history is useful as many cases of Fuchs' are hereditary (affecting females more than males). Guttata are 'bumps' that develop on the back of the cornea when there are not enough endothelial cells to cover it. These cells are very important as they pump fluid out of the cornea and into the anterior chamber of the eye. The cornea is hydrophilic (water loving) like a sponge. When it swells and gets thicker it starts to become cloudy and blur the vision. When this happens and the impairment is severed than a corneal transplant is indicated. Recently a new operation for decompensated Fuchs' dystrophy called DSEAK (Descemet's membrane epithelial automated keratoplasty) has dramatically speeded the healing, safety and results over full thickness penetrating keratoplasty. Corneal endothelial cells do not multiply or replace themselves when damaged or cell death occurs.

Cataract surgery will not cause Fuchs' dystrophy but cataract surgery (or for that matter any surgery inside the eye) will stress the cornea and in severe Fuchs' could be the final straw that causes the cornea to compensate.

Your doctor is absolutely correct. In my patients with progressive Fuchs's dystrophy I recommend cataract surgery SOONER rather than later because the advanced, "hard" cataract will put much more stress and strain on the cornea endothelium than a moderately firm or softer cataract. Fuchs' dystrophy can decompensate on its own without any surgery taking place or any cataract in the eye.

It would be helpful to know if your mother's problem with her eye's was Fuchs' dystrophy. It is unusual for Fuchs' to cause an eye to be removed, so there's a good chance she had something else.

I would go ahead and have the cataract surgery when you vision starts to be a moderate problem for you and the cataract is the cause. By using the endothelial cell count and corneal thickness, your ophthalmologist can give you a reasonable risk of the cornea being able to stand your cataract surgery.

Good luck, new cataract surgical techniques are much, much more gentle on the eye than techniques used in the past.

JCH MD