Fuchs' Dystrophy: Causes, Symptoms, and Treatments

Published on March 20th, 2024

Updated on December 10th, 2024

Fuchs' dystrophy is a type of corneal dystrophy and is a genetic disease that affects the cornea, which is the clear dome located at the front of the eye. Fuchs' dystrophy causes the endothelium cells, which are responsible for the cornea’s fluid levels, to break down and cause small bumps medically known as guttae to form on the back of the cornea. This can also lead to a build-up of fluid and then swelling in the cornea. Symptoms of Fuchs’ eye disease can include sensitivity to light, a rough or gritty sensation in the eye, eye pain, eye problems exacerbated in humid areas and blurry vision. According to John Hopkins, while Fuchs' dystrophy occurs at birth, symptoms may not occur until middle age. Fuchs' dystrophy cannot be cured, therefore, yearly eye tests are recommended especially for patients who are over 40 years or older.

What is Fuchs' Dystrophy?

Fuchs' dystrophy is an inherited disease of the eye that affects the cornea as it causes the endothelium cells to deteriorate, which can lead to a build-up of fluid and then swelling in the cornea, known as corneal edema. The endothelium cells are what control fluid levels in the cornea. Risk factors for Fuchs' dystrophy include having a history of Fuchs' dystrophy in the family and older age, according to the Centre For Eye Research Australia. Symptoms of Fuchs' dystrophy include light sensitivity, exacerbated eye problems in humid areas, blurry vision and eye pain.

The figure below shows the cloudy appearance of the eye as a result of Fuchs’ dystrophy and the appearance of a thickened stroma due to corneal swelling.

A Fuchs’ dystrophy-affected eye exhibiting symptoms of cloudiness next to an illustrated image of a thickened stroma.

What Causes Fuchs' Dystrophy?

The main causes of Fuchs' dystrophy are having a history of Fuchs' dystrophy in the family, increased age and gender. Increased age is a common cause as Fuchs' dystrophy typically presents symptoms after the age of 50 years. According to the American Academy of Ophthalmology, Fuchs' dystrophy was found to occur in women more than men.

Is Fuch Dystrophy Hereditary?

Yes, Fuchs' dystrophy is hereditary. Fuchs' dystrophy can be inherited through an autosomal dominant nature, according to the National Institutes of Health, which means that a single copy of the mutated gene from just one parent is sufficient to cause the genetic disease to develop.

Diagram shows the genetic pathway, autosomal dominant, which is how Fuchs’ Dystrophy can be inherited.

Is Fuchs' Dystrophy an Autoimmune Disease?

No, Fuchs' dystrophy is not an autoimmune disease. An autoimmune disease refers to conditions that cause the immune system to attack its own body and can include common forms such as rheumatoid arthritis, psoriasis or thyroid diseases. Fuchs' dystrophy is a corneal condition passed down through parents.

What are the Symptoms of Fuchs' Dystrophy?

The symptoms of Fuchs' dystrophy are sensitivity to light, a rough or gritty sensation in the eye, eye pain, eye problems exacerbated in humid areas and blurry vision. The symptoms of Fuchs' dystrophy are listed below.

Sensitivity to Light: Sensitivity to light refers to the discomfort or pain experienced when the eyes are exposed to light. Light sensitivity can develop as a symptom of Fuchs' dystrophy due to swelling in the cornea, which can leave the eyes tender and more vulnerable to stimuli.
Rough or Gritty Sensation: A rough or gritty sensation in the eye can occur as a consequence of the tiny bumps that form on the back of the cornea due to Fuchs’ dystrophy.
Eye Pain: Eye pain can arise during Fuchs' dystrophy as the swelling that occurs can cause increased pressure in the cornea. Corneal swelling can leave the eye more sensitive and tender as the cornea is the most innervated structure in the eye and, therefore, more susceptible to pain.
Eye Problems Exacerbated in Humid Areas: The exacerbation of eye problems in humid areas may be due to the quicker evaporation of tears in the eye causing dry eye. This can leave the eye in discomfort as the eye requires a certain amount of moisture for optimal health and comfort.
Blurry Vision: Blurry vision can occur in relation to Fuchs' dystrophy from the build-up of fluid in the cornea. Blurry vision can be described as vision that lacks sharpness and detail.

Graphic of a healthy eye displaying no symptoms next to an eye affected by Fuchs’ Dystrophy showing signs of cloudiness.

How to Diagnose Fuchs' Dystrophy?

To diagnose Fuchs' dystrophy, a standard eye test completed by an optometrist or ophthalmologist is required. A slit lamp exam in particular is typically performed as part of standard eye tests, which can identify corneal swelling and guttae, which is a clear indication of Fuchs’ dystrophy, according to the Lions Eye Institute. Eye tests are important in maintaining eye health as they are the surest way to detect a wide range of eye problems such as Fuchs' dystrophy. To specifically examine the corneal endothelium, it requires a diagnostic test known as specular microscopy, which is usually performed before surgery by an ophthalmologist.

What are the Fuchs' Dystrophy Treatments?

There are no treatments for Fuchs' dystrophy, however, there may be treatments to help reduce occurring symptoms such as eye drops or eye ointments. According to the Centre for Eye Research Australia, eye drops or ointments may aid in alleviating corneal swelling. For severe cases of Fuchs' dystrophy where vision loss has occurred, the patient may require a corneal transplant. A corneal transplant is a surgery performed in the eyes and involves replacing all or parts of the affected corneal cells with a healthy donated cornea.

How to Prevent Fuchs' Eye Disease?

It is not possible to prevent Fuchs' eye disease, therefore, it is important and strongly recommended to get regular eye tests with an optometrist, especially after the age of 50. The recommended frequency of eye tests is generally once every 2 years unless advised otherwise by your optometrist. According to the Cleveland Clinic, while Fuchs' eye disease cannot be prevented, it may be beneficial to your overall health by managing sugar levels if you have diabetes and quitting smoking.

What is the Difference Between Fuchs' Dystrophy and Corneal Dystrophy?

There is no difference between Fuchs' dystrophy and corneal dystrophy. Corneal dystrophy is the term used for genetic and progressive eye diseases that impair the cornea. Other than Fuchs' dystrophy, types of corneal dystrophy also include keratoconus and lattice corneal dystrophy.

What is the Difference between Fuchs' Dystrophy and Cone-rod Dystrophy?

The difference between Fuchs’ dystrophy and cone-rod dystrophy is which region of the eye they affect and how they can impair vision. Fuchs’ dystrophy impacts the cornea, causing the endothelium cells in the cornea to deteriorate over time leading to increased fluid and swelling, which causes cloudy and/or blurry vision. Fuchs’ dystrophy normally presents symptoms during middle age, whereas cone-rod dystrophy can develop in patients from childhood to early adulthood. Cone-rod dystrophy impairs the light-sensitive cells, known as the rods and cones in the retina, impairing visual acuity and the ability to distinguish between certain colors. Fuchs’ dystrophy and cone-rod dystrophy are both inherited types of eye diseases and can eventually lead to partial loss of vision.

Can I Use Glasses if I Have Fuchs' Dystrophy?

Yes, you may be able to use glasses if you have Fuchs’ dystrophy and are experiencing sensitivity to light but it will also depend on what is advised by your optometrist. According to the American Academy of Ophthalmology, a specific strength of glasses may be prescribed to Fuchs’ dystrophy patients who undergo corneal transplant or penetrating keratoplasty to help achieve optimal vision. According to the National Library of Medicine, therapeutic contact lenses may be used in combination with 5% sodium chloride for a week to help alleviate symptoms (Erdinest et al., 2022).

Does Having Fuchs' Dystrophy Make You Blind?

No, while Fuchs’ dystrophy can lead to a partial loss of vision over time, it may not result in complete blindness unless it is a severe case, according to the NW Cornea Institute. Fuchs’ dystrophy impairs vision as it causes cloudy or blurry vision as a result of the swelling in the cornea.

Can I Drive a Vehicle if I Have Fuchs' Dystrophy?

Yes and no, you may still be able to drive with Fuchs' dystrophy depending on your visual condition. However, the optometrist or ophthalmologist who is treating you will be able to inform you whether you are fit to drive with visual impairment. According to Optometry Australia, the minimum visual condition required in Australia is 6/12 with one or both eyes. Nevertheless, it is important to consult with an eye care professional as circumstances may differ according to your condition.