What is Fuchs’ Dystrophy?
Fuchs’ dystrophy is a disease in which the corneas become cloudy, reducing vision and eventually even leading to blind, painful eyes.
The cornea is the outermost part of the eye that covers the iris and the pupil. The cornea helps focus light as it enters the eye, which helps us see. The innermost layer of the cornea – the endothelium – contains pump cells that constantly remove excess water, keeping the cornea clear to allow light to pass through.
However, these pump cells can diminish or even fail, causing a build up of fluid in the cornea, which causes the cornea to thicken and lose transparency. Fuchs’ dystrophy is a condition in which the pump cells wear out prematurely, and bumps called guttae are deposited on the back of the cornea.
Fuchs’ dystrophy is a progressive disease, meaning it continues to worsen if left untreated.
- Causes of Fuchs’ Dystrophy
- Symptoms of Fuchs’ Dystrophy
- Diagnosis of Fuchs’ Dystrophy
- Treatment of Fuchs’ Dystrophy
Fuchs’ dystrophy is usually inheredited. Individuals who have a parent with the disease have a 50 percent chance of having the disease themselves. However, because Fuchs’ dystrophy was often misdiagnosed as cataracts many may not know they have a family history.
Some individuals may develop Fuchs’ dystrophy without a family history due to a genetic mutation.
Fuchs’ dystrophy usually first begins causing symptoms after age 40, and is slightly more common in women than men.
Cataracts and Fuchs’ dystrophy may have overlapping symptoms.
However, there are two main differences between the symptoms of Fuchs’ dystrophy and other eye diseases:
- Fluctuations in vision – With Fuchs’ dystrophy, vision may be poor in the early morning, but grow progressively better throughout the day. Additionally, vision may fluctuate depending on the humidity. You may experience better vision in drier areas, like on an airplane or in the desert.
- Eye pain – Fuchs’ dystrophy may cause pain in the eye in its advanced stages, especially if blisters have formed on the front of the cornea.
- Hazy, fuzzy or cloudy vision
- Glare around sources of light, especially at night
- Less color contrast
- Reduced visual acuity
- Difficulty driving at night
The two main methods for diagnosing Fuchs’ dystrophy are viewing the eye with a slit-lamp and visualization of the endothelial cells with specular microscopy. Both of these tests can give a specific diagnosis of Fuchs’ dystrophy and its stage and can be performed in our office.
In the past, the only treatment for Fuchs’ dystrophy was a complete sutured corneal transplant. However today, symptomatic Fuchs’ dystrophy is usually best treated with DMEK surgery.
Fuchs’ Dystophy Q&A
If you or a loved one has been diagnosed with Fuchs’ dystrophy, Parker Cornea is here to help and answer any questions you may have about the disease. Here are a few of the questions we routinely answer in our office:
What is Fuchs’ dystrophy, and how can I tell if I have it?
Fuchs’ dystrophy is an inherited condition that causes a premature aging of the pump cells on the back of your cornea (endothelial cells). As the cells die off, deposits called guttae are left on the back your cornea. If you have guttae in your central cornea, you have Fuchs’ dystrophy.
Can I see my guttae?
Guttae are too small to be seen with the naked eye, but are easily visible in photos taken with an endothelial microscope.
I have been told that I have Fuchs’ dystrophy. Will I have to have a corneal transplant?
Hopefully not. Many people never have enough guttae to have symptoms from their Fuchs’.
How can I tell if my cornea is swelling?
Often, corneal swelling begins in an intermittent fashion. Your vision tends to be more blurred in humid environments or when you open your eyes after sleeping. Hazy vision persists as corneal swelling progresses.
Why should I consider Parker Cornea for my Fuchs' dystrophy surgery?
Dr. Parker was among the first ophthalmologists to offer modern surgery for Fuchs’ dystrophy in the U.S. He has performed more DMEK and DSAEK surgeries than any other physician in the region. This recent scientific review of Fuchs’ transplant surgery was co-authored with the Dutch originator of both DSEK and DMEK surgery. Dr. Parker has extensive experience teaching and performing all transplant procedures and can offer you the very best treatment for your unique situation.
When should I consider having a corneal transplant for Fuchs’ dystrophy?
Corneal transplantation is done to improve your vision when it has been reduced by Fuchs’. Modern transplants, such as DMEK, are most effective when they are done before Fuchs’ causes significant swelling of your cornea.
Besides having a transplant, is there anything I can do to make my corneal swelling go away?
Not really. Hypertonic saline drops and ointment and a reduction of the humidity in your environment can provide small temporary benefits.
What is the difference between a transplant and an implant?
Inert objects, such as acrylic lenses left in the eye following cataract surgery, are termed “implants.” “Transplants” are composed of living cells that continue to live after the transplant surgery. Your immune system can attack a transplant, and even kill the living cells that are transplanted. Such an attack by your immune system is called a rejection. Rejections are very rare after DMEK.
What different types of corneal transplants are available for the treatment of Fuchs’?
DSAEK, DSEK and DMEK are all options for the treatment of Fuchs’ dystrophy. Sutured full thickness corneal transplantation is obsolete in the treatment of Fuchs’.
What is DSAEK?
In the U.S., DSAEK (Descemet’s Stripping Automated Endothelial Keratoplasty) is the most commonly performed surgery for Fuchs’. In DSAEK, a thin piece of living donor cornea (usually prepared by an eye bank using a microkeratome) is placed on the back surface of your cornea and supported by an air bubble. Within a few days, the air will diffuse out of your eye and leave your cornea clearer due to the pumping action of the transplanted endothelial cells. After DSAEK your cornea is a bit thicker than normal, and there is often some reduction in your best possible vision compared with the best vision you had before Fuchs’ developed.
What is DSEK?
DSEK (Descemet’s Stripping Endothelial Keratoplasty) is the same surgery as DSAEK, but no microkeratome is used in the preparation of the donor cornea. DSAEK and DSEK provide similar visual results, and surgeon preference determines which is selected.
What is DMEK?
DMEK is a procedure similar to DSAEK, but rather than placing a piece of cornea on the back surface of your cornea, no extra tissue is placed in your eye. Your defective pump cells and attached basement membrane are removed and replaced with healthy pump cells and attached basement membrane. There is no increase in the thickness of your cornea. In fact, it is often very difficult, or impossible, to tell that an eye that has had DMEK surgery ever had Fuchs’ at all.
Is there any difference in the vision I would expect after DSAEK compared with DMEK surgery?
Yes. Seventy-five percent of the time after DSAEK, your best vision will be limited to 20/30 or worse. Seventy-five percent of the time after DMEK, your best vision is expected to be 20/25 or better.
Other than better vision, is there any other reason for me to prefer DMEK to DSAEK?
Yes. DMEK has been shown to provide the most rapid return of vision. What’s more, the risk of rejection is 15 times lower with DMEK compared to DSAEK (even though the risk of rejection with DSAEK is low, especially compared with the transplants of old).
Are there any reasons to prefer DSAEK to DMEK surgery?
Rarely. Unusual conditions that are sometimes associated with corneal edema can occasionally be difficult to treat with DMEK.
How soon can I go back to work after having DMEK or DSAEK surgery?
Particularly vigorous activity should be avoided for two weeks, although most people return to full duty at work after 5-7 days.
How long do I have to use eye drops after corneal transplantation for Fuchs’?
Eye drops are usually used every 1-2 days for the rest of your life after a transplant. The drops are used to reduce the risk of rejection.
How often do I need eye check-ups after transplant surgery for Fuchs’?
After the eye drops have been reduced to every 1-2 days, check-up frequency is generally every 6-12 months.
Can I have my check-ups with my local eye doctor, or do I have to continue returning to Birmingham?
While we will always be happy to see you in Birmingham, and have available after-hours emergency ophthalmic care, it is usually fine to follow up with your local eye doctor once your eye and medication regimen are stable.
Examples of Fuchs’ Dystrophy
Seen here with high power electron microscopy, the back surface of a cornea with Fuchs’ has many bumps called guttae. Image © 2009 by the American Academy of Ophthalmology.
This cornea is swollen and hazy due to advanced Fuchs’ dystrophy. Image © 2009 by the American Academy of Ophthalmology.
A slit of light which passes from left to right in the photo reveals a thin normal cornea with a smooth front and back surface. Image © 2009 by the American Academy of Ophthalmology.
The slit of light passing from left to right in this photo reveals the irregular posterior corneal surface characteristic of Fuchs’ dystrophy. Image © 2009 by the American Academy of Ophthalmology.