Navigate Up

Full Library - A-Z Index


Print This Page

Fuchs' dystrophy

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 usually affects both eyes.

Alternative Names

Fuchs' endothelial dystrophy, Fuchs' corneal dystrophy

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 persons without a known family history of the disease.

Fuchs' dystrophy is more common in women than in men. Vision problems usually do not appear before age 50, although doctors may be able to see signs of the disease in affected persons by their 30s or 40s.

Fuchs' dystrophy affects the thin layer of cells that line 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, causing eye pain . Fuchs' dystrophy can also cause the shape of the cornea to change, leading to more vision problems.

Symptoms

Exams and Tests

A doctor can diagnose Fuchs' dystrophy during a slit-lamp examination .

Other tests that may be done include:

  • Pachymetry -- measures the thickness of the cornea
  • Specular microscope examination -- allows the doctor 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 patients 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 usually not needed.

Outlook (Prognosis)

Fuchs' dystrophy gets worse over time. Without a corneal transplant, a patient 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

Call your health care 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

Cockerham GC, Kenyon KR. The corneal dystrophies. In: Tasman W, Jaeger EA, eds. Duane’s Ophthalmology. 2013 ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2013:chap 16.

Vanmeter WS, Lee WB, Katz DG. Corneal edema. In: Tasman W, Jaeger EA, eds. Duane’s Ophthalmology. 2013 ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2013:chap 16A.

Shamie N, Phillips P, Terry MA. Descemet stripping automated endothelial keratoplasty. In: Tasman W, Jaeger EA, eds. Duane's Ophthalmology. 2013 ed. Philadelphia, Pa: Lippincott, Williams & Wilkins; 2013:chap 29.

Updated: 9/2/2014

Franklin W. Lusby, MD, Ophthalmologist, Lusby Vision Institute, La Jolla, California. Also reviewed by David Zieve, MD, MHA, Isla Oglvie, PhD, and the A.D.A.M. Editorial team.


©  UPMC | Affiliated with the University of Pittsburgh Schools of the Health Sciences
Supplemental content provided by A.D.A.M. Health Solutions. All rights reserved.

For help in finding a doctor or health service that suits your needs, call the UPMC Referral Service at 412-647-UPMC (8762) or 1-800-533-UPMC (8762). Select option 1.

UPMC is an equal opportunity employer. UPMC policy prohibits discrimination or harassment on the basis of race, color, religion, ancestry, national origin, age, sex, genetics, sexual orientation, marital status, familial status, disability, veteran status, or any other legally protected group status. Further, UPMC will continue to support and promote equal employment opportunity, human dignity, and racial, ethnic, and cultural diversity. This policy applies to admissions, employment, and access to and treatment in UPMC programs and activities. This commitment is made by UPMC in accordance with federal, state, and/or local laws and regulations.

Medical information made available on UPMC.com is not intended to be used as a substitute for professional medical advice, diagnosis, or treatment. You should not rely entirely on this information for your health care needs. Ask your own doctor or health care provider any specific medical questions that you have. Further, UPMC.com is not a tool to be used in the case of an emergency. If an emergency arises, you should seek appropriate emergency medical services.

For UPMC Mercy Patients: As a Catholic hospital, UPMC Mercy abides by the Ethical and Religious Directives for Catholic Health Care Services, as determined by the United States Conference of Catholic Bishops. As such, UPMC Mercy neither endorses nor provides medical practices and/or procedures that contradict the moral teachings of the Roman Catholic Church.

© UPMC
Pittsburgh, PA, USA UPMC.com