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Fuchs dystrophy

Fuchs' dystrophy; Fuchs' endothelial dystrophy; Fuchs' corneal dystrophy

Definition

Fuchs (pronounced "fooks") dystrophy is an eye disease in which cells lining the inner surface of the cornea slowly start to die off. The disease most often affects both eyes.

Causes

Fuchs dystrophy can be inherited, which means it can be passed down from parents to children. If either of your parents has the disease, you have a 50% chance of developing the condition.

However, the condition may also occur in people without a known family history of the disease.

Fuchs dystrophy is more common in women than in men. Vision problems do not appear before age 50 years in most cases. However, a health care provider may be able to see signs of the disease in affected people by their 30s or 40s.

Fuchs dystrophy affects the thin layer of cells that lines the back part of the cornea. These cells help pump excess fluid out of the cornea. As more and more cells are lost, fluid begins to build up in the cornea, causing swelling and a cloudy cornea.

At first, fluid may build up only during sleep, when the eye is closed. As the disease gets worse, small blisters may form. The blisters get bigger and may eventually break. This causes eye pain. Fuchs dystrophy can also cause the shape of the cornea to change, leading to more vision problems.

Symptoms

Symptoms may include:

Eye pain
Eye sensitivity to light and glare
Foggy or blurred vision, at first only in the mornings
Seeing colored halos around lights
Worsening vision throughout the day

Exams and Tests

A provider can diagnose Fuchs dystrophy during a slit-lamp exam.

Other tests that may be done include:

Pachymetry -- measures the thickness of the cornea
Specular microscope examination -- allows the provider to look at the thin layer of cells that line the back part of the cornea
Visual acuity test

Treatment

Eye drops or ointments that draw fluid out of the cornea are used to relieve symptoms of Fuchs dystrophy.

If painful sores develop on the cornea, soft contact lenses or surgery to create flaps over the sores may help reduce pain.

The only cure for Fuchs dystrophy is a corneal transplant.

Until recently, the most common type of corneal transplant was penetrating keratoplasty. During this procedure, a small round piece of the cornea is removed, leaving an opening in the front of the eye. A matching piece of cornea from a human donor is then sewn into the opening in the front of the eye.

A newer technique called endothelial keratoplasty (DSEK, DSAEK, or DMEK) has become the preferred option for people with Fuchs dystrophy. In this procedure, only the inner layers of the cornea are replaced, instead of all the layers. This leads to a faster recovery and fewer complications. Stitches are most often not needed.

Outlook (Prognosis)

Fuchs dystrophy gets worse over time. Without a corneal transplant, a person with severe Fuchs dystrophy may become blind or have severe pain and very reduced vision.

Mild cases of Fuchs dystrophy often worsen after cataract surgery. A cataract surgeon will evaluate this risk and may modify the technique or the timing of your cataract surgery.

When to Contact a Medical Professional

Contact your provider if you have:

Eye pain
Eye sensitivity to light
The feeling that something is in your eye when there is nothing there
Vision problems such as seeing halos or cloudy vision
Worsening vision

Prevention

There is no known prevention. Avoiding cataract surgery or taking special precautions during cataract surgery may delay the need for a corneal transplant.

References

Castellucci M, Novara C, Casuccio A, et al. Bilateral ultrathin descemet's stripping automated endothelial keratoplasty vs. bilateral penetrating keratoplasty in Fuchs' dystrophy: corneal higher-order aberrations, contrast sensitivity and quality of life. Medicina (Kaunas). 2021;57(2):133. PMID: 33546152 pubmed.ncbi.nlm.nih.gov/33546152/.

Folberg R. The eye. In: Kumar V, Abbas AK, Aster JC, eds. Robbins & Cotran Pathologic Basis of Disease. 10th ed. Philadelphia, PA: Elsevier; 2021:chap 29.

Patel SV. Towards clinical trials in Fuchs endothelial corneal dystrophy: classification and outcome measures - the Bowman Club Lecture 2019. BMJ Open Ophthalmology. 2019;4(1):e000321. PMID: 31414054 pubmed.ncbi.nlm.nih.gov/31414054/.

Price MO, Price FW Jr. Randomized, double-masked, pilot study of netarsudil 0.02% ophthalmic solution for treatment of corneal edema in Fuchs dystrophy. Am J Ophthalmol. 2021;227:100-105. PMID: 33737034 pubmed.ncbi.nlm.nih.gov/33737034/.

Rosado-Adames N, Afshari NA. Diseases of the corneal endothelium. In: Yanoff M, Duker JS, eds. Ophthalmology. 5th ed. Philadelphia, PA: Elsevier; 2019:chap 4.21.

Salmon JF. Cornea. In: Salmon JF, ed. Kanski's Clinical Ophthalmology. 9th ed. Philadelphia, PA: Elsevier; 2020:chap 7.

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Review Date: 8/22/2022

Reviewed By: Franklin W. Lusby, MD, Ophthalmologist, Lusby Vision Institute, La Jolla, CA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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