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Fuchs dystrophy, corneal endothelial dystrophy - A State of Sight #19

Fuchs dystrophy, an inherited problem of the cornea, can cause blurred vision due to swelling of the cornea. Isaac Porter, MD, from Lowry Porter Ophthalmology, explains Fuchs dystrophy, including the changes that patients may notice and the treatment options available.



Welcome to A State of Sight, I’m Isaac Porter from Porter Ophthalmology and this is your update in ophthalmology and eye care. In this episode, I would like to discuss Fuchs dystrophy since we recently had a patient with Fuchs dystrophy and there’s a lot of information to share about it.
 
Fuchs dystrophy, or corneal endothelial dystrophy, is a genetic problem that  we see frequently in patients over 50 years old. To understand Fuchs dystrophy, let’s take a look at the cornea (the clear shield that covers the front of the eye) which is where this problem is.
 
The back of the cornea has a set of pumps that pump water out of the cornea and into the eye to keep the cornea thin and clear. In Fuchs dystrophy, these pumps don’t work like they should and the dysfunction can lead to swelling of the cornea and a bumpy or irregular layer on the inside of the cornea.
 
This usually starts in the center and can spread towards the sides of the cornea to involve the entire area.
 
This is a genetic condition, which means it’s passed down from parents to their children, and it’s passed in a dominant manner. If one of the parents has this problem there is a 50% chance that they will pass it on to their children.
 
When people have Fuchs dystrophy, they may notice that their vision initially begins to blur or become cloudy. Later, they may notice trouble with glare from lights at night, or that their vision is not as sharp as it used to be.
 
As the disease worsens, the cornea may swell and become even more cloudy, causing decreased vision, particularly after waking in the morning.
 
Often initially, vision may be clearer later in the day after the eyes have been open and exposed to the air because the cornea then has a chance to dry out some. Overnight, the swelling can return when patients’ eyes are closed during sleep since there is not as much evaporation from the cornea.
 
In the later stages, this can lead to painful swelling and breakdown of the corneal surface that can be very uncomfortable.
 
We can diagnose Fuchs dystrophy during an eye exam. Another test that gives useful information is pachymetry. With this, we measure the thickness of the cornea with a special ultrasound, which will give us an idea of the severity of the disease and help follow its progression over time.
 
Treatments for Fuchs dystrophy begin with drops that are similar to a strong salt solution which can help decrease the swelling. When this no longer works, patients may need to consider a corneal transplant in order to clear the cornea, improve vision, and also to help reduce pain (if present).
 
There are many successful treatments available for Fuchs dystrophy, including inner layer corneal transplantation with DSEK (Descemet’s Stripping Endothelial Keratoplasty) and DMEK (Descemet’s Membrane Endothelial Keratoplasty).

If you have any questions about Fuchs dystrophy please post below. We hope to see you again soon next time on A State of Sight.