What are the other Names for this Condition? (Also known as/Synonyms)

• Fuchs' Corneal Dystrophy
• Fuchs' Endothelial Dystrophy (FED)
• Fuchs’ Endothelial Corneal Dystrophy (FECD)

What is Fuchs' Dystrophy? (Definition/Background Information)

• Fuchs' Dystrophy is a rare degenerative disorder that affects the cornea of both the eyes, causing death of the endothelial cells (cells that line the inner corneal surface)
• There is fluid accumulation within the cornea, producing blurred vision, inflammation, and pain. This may result in opacity of the transparent cornea and loss of vision
• Fuchs' Dystrophy is more commonly observed in parts of US, Europe, and Asia; also, women are more prone to this condition. The onset of this condition begins in the early 30s-40s
• The disorder is also slowly progressive, which means that it becomes worse with time. A corneal transplant provides the best solution for Fuchs' Dystrophy, which can bring about a cure

Who gets Fuchs' Dystrophy? (Age and Sex Distribution)

• Adult men and women are susceptible to Fuchs' Dystrophy; however, the incident rate is higher among women
• Though indications of the condition may commence between the ages 30-40, vision loss begin to occur after the age of 50 years
• It is more common in some parts of US and in certain European and Asian countries, than the rest of the world

What are the Risk Factors for Fuchs' Dystrophy? (Predisposing Factors)

The risk factors for Fuchs’ Dystrophy may include:

• Genetic factors play a major role in Fuchs' Dystrophy - individuals with a family history of the disorder are at a high risk (particularly, if the person is a parent or a sibling)
• An advancing age - individuals over the age of 50 years, may have a higher risk

The risk factors are yet to be fully identified, since this disorder has not yet been completely understood.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Fuchs' Dystrophy? (Etiology)

• The endothelium cells help drain excess water from the eyes, thereby preventing them from flooding the cornea; the cornea acts like a ‘window’ to the eye
• Fuchs’ Dystrophy causes a progressive destruction of these cells, which leads to swollen corneas in both eyes, thereby distorting the vision
• As the condition worsens, both nighttime and daytime inflammation remains constant. Thus, the cornea becomes opaque and the vision is slowly reduced
• It is reported that in some cases, Fuchs’ Dystrophy is inherited as an autosomal dominant feature

Autosomal dominant: Autosomal dominant conditions are traits or disorders that are present when only one copy of the mutation is inherited on a non-sex chromosome. In these types of conditions, the individual has one normal copy and one mutant copy of the gene. The abnormal gene dominates, masking the effects of the correctly function gene. If an individual has an autosomal dominant condition, the chance of passing the abnormal gene on to their offspring is 50%. Children who do not inherit the abnormal gene will not develop the condition or pass it on to their offspring.

What are the Signs and Symptoms of Fuchs' Dystrophy?

The signs and symptoms of Fuchs’ Dystrophy may include:

• In the early stages of the disease, blurred vision occurs in the mornings, which gets better during the day
• Gradually deteriorating vision; blurred or foggy vision, which remains throughout the day, as the disorder progresses
• Light and glare sensitivity, appearance of ‘halo’ around lights
• Pain and inflammation of the eye; continuous discomfort
• Additionally, blister formation may occur, which can break and cause pain and discomfort

How is Fuchs' Dystrophy Diagnosed?

A few tests and exams conducted to diagnose Fuchs’ Dystrophy include:

• The ophthalmologist (or physician) performs a complete eye examination. He/she may also evaluate the individual’s family medical history
• The ophthalmologist may use certain special studies to aid in the diagnosis. These include:
  • Specular microscopy
  • Biometric exam of cornea
  • Corneal pachymetry
  • Confocal biomicroscopy

Other diagnostic tools may include:

• Reading (letters or numbers) from a chart, to test visual acuity
• Glare test that is performed by directing a bright light into the eye
• Slit lamp exam: Using an optical microscope to study the eye condition
• Testing the cornea for pressure, thickness, and cell count

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Fuchs' Dystrophy?

Complications due to Fuchs’ Dystrophy include:

• Complete blindness, especially if the condition is left untreated
• Severe pain and inflammation
• Complications that might occur from other eye disorders or eye-related conditions; a cataract surgery is known to sometimes worsen the problem

How is Fuchs' Dystrophy Treated?

The treatment measures for Fuchs’ Dystrophy include:

• Symptomatic treatment by using eye drops (hypertonic saline eye drops) or ointments, to drain-out fluid from the cornea and temporarily restore vision
• Pain may be relieved by the use of special soft contact lenses, or through a minimally-invasive procedure of forming surgical flaps
• Corneal transplant, where the entire cornea is replaced using a donor cornea. This procedure provides a permanent relief
• Management of Fuchs’ Dystrophy is also accomplished by only a partial replacement of the cornea, through a procedure called deep lamellar keratoplasty (DLK). It is a novel technique that is faster and less complicated

How can Fuchs' Dystrophy be Prevented?

Fuchs’ Dystrophy may not be prevented, but awareness of the condition (especially if you are genetically at risk) and taking certain basic steps to prevent it from deteriorating completely is beneficial.

• Be prompt in seeking medical attention, if any signs and symptoms indicating vision problems, or any other eye-related discomfort is observed
• For those individuals in the high risk group - regular eye checkups and periodic screening for any eye defect is a good practice. This can alert one to any early-onset signs

The progression of Fuchs’ Dystrophy may be accidentally accelerated by a complicated cataract surgery, especially when the cataract becomes dense. A cataract surgery under such circumstances warrants special care and precaution.

What is the Prognosis of Fuchs' Dystrophy? (Outcomes/ Resolutions)

• Delayed treatment or lack of treatment of Fuchs’ Dystrophy may cause the disorder to progressively worsen with time; permanent vision loss is a possibility, in such circumstances
• Resolution of the disorder can be completely achieved through a corneal transplant surgery
• Individuals, who have early-onset of the signs and symptoms, tend to have an aggressive type of the disorder

Additional and Relevant Useful Information for Fuchs' Dystrophy:

• In the US, most corneal transplant surgeries (keratoplasty) are performed due to Fuchs’ Dystrophy that severely affect the cornea

The following article link will help you understand corneal transplant surgery.

http://www.dovemed.com/common-procedures/procedures-surgical/cornea-transplant/