Is there a problem with your cornea? Let's learn about corneal dystrophy!

Is there a problem with your cornea? Let's learn about corneal dystrophy!

Do your eyes feel blurry? Do you sometimes have difficulty looking at light? Or do you constantly feel like something is wrong with your eyes? These can sometimes be symptoms of a condition related to the cornea of ​​the eye. Today we are going to talk about one such condition, "Corneal Dystrophy". Don't worry, we will talk about this simply, in a way that you can understand.

What is Corneal Dystrophy?

Simply put, corneal dystrophy is a general term for a group of conditions that affect the cornea , the transparent part of your eye. There are more than 20 different types of diseases under this category. These types of dystrophy can cause changes or damage to the tissues of the cornea. It's like having small spots or streaks on a clear glass that you can't see clearly when you look through it. If this damage becomes severe, you can have changes in your vision and even vision loss.

The important thing is that these corneal dystrophies are genetic conditions. That is, they are caused by changes in our DNA . Some changes you can inherit from your parents. Others can occur unexpectedly, spontaneously. Some types of the disease occur for reasons that experts still do not fully understand.

Are there types of corneal dystrophy?

Yes, they are classified according to how they affect the different layers of the cornea. Mainly as follows:

  • Those that affect the outermost layers of the cornea (epithelial and subepithelial).
  • Those that affect the outer and middle layers of the cornea (Epithelial-stromal).
  • Those that affect the middle layer of the cornea (Stromal).
  • Those that affect the innermost layer of the cornea (endothelial).

Now let's look at each of these types in a little more detail.

Epithelial and subepithelial corneal dystrophies

These are diseases that affect the top layers of the cornea. Some examples are:

  • Epithelial basement membrane dystrophy
  • Epithelial recurrent erosion dystrophies (there are also three subtypes)
  • Gelatinous drop-like dystrophy
  • Lisch epithelial dystrophy
  • Meesmann dystrophy
  • Subepithelial mucinous dystrophy

Most of these dystrophies appear in childhood . However, epithelial basement membrane dystrophy usually (though not always) appears in adulthood. The other is gelatinous drop-like corneal dystrophy . This can occur at any age before the age of 20.

Epithelial-stromal dystrophies

These affect both the outer layer of the cornea and the middle layer underneath it.

  • Granular corneal dystrophy (types 1 and 2)
  • Lattice corneal dystrophy (this also has subtypes and different varieties)
  • Reis-Bückler's dystrophy
  • Thiel-Behnke dystrophy

All of these conditions are more likely to occur in childhood . Lattice corneal dystrophy type 1 can occur as late as 20 years of age.

Stromal dystrophies

These mainly affect the middle, thick layer of the cornea.

  • Macular dystrophy
  • Schneider crystalline dystrophy
  • Congenital hereditary stromal dystrophy
  • Fleck dystrophy
  • Posterior amorphous dystrophy
  • Pre-Descemet dystrophy
  • Central cloudy dystrophy of François

Stromal dystrophies usually affect children . However, two of these can develop as you get older. Schneider corneal dystrophy can develop as late as age 30, and pre-Descemet corneal dystrophy usually develops after age 30.

Endothelial corneal dystrophies

These are diseases that affect the very delicate layer inside the cornea.

  • Fuchs endothelial dystrophy
  • Posterior polymorphous dystrophy
  • Congenital hereditary endothelial dystrophy
  • X-linked endothelial dystrophy

Of all the corneal dystrophies, Fuchs endothelial corneal dystrophy is the most common , accounting for 39% of all corneal transplants performed in the United States.

What are the symptoms of corneal dystrophy?

The symptoms of corneal dystrophy vary depending on the specific type you have. Some people may not have any symptoms at all . When symptoms do appear, they usually affect both eyes and gradually worsen over time.

Imagine that your cornea is like a perfectly shaped, clear, flawless piece of glass. But corneal dystrophy can change that. In many of these conditions, new tissue grows on the cornea, or it builds up in places where it shouldn't. Then, instead of looking through clear glass, it's like looking through old, flawed, blown glass. It's hard to see through that, isn't it?

Similarly, new tissue formation or accumulation makes it difficult to see through the affected layers of the cornea. This is why blurred or foggy vision is common among people with corneal dystrophy.

The most common symptoms are these:

  • Visual acuity loss: This occurs when the surface of the cornea becomes distorted or does not heal properly. This means that your vision is less clear even if you wear glasses or contact lenses.
  • Corneal haze: This can make your vision appear cloudy. This is often a residual effect of damage or changes to the cornea.
  • Foreign body sensation: It feels like something is stuck in the eye , but nothing can be found when you look inside.
  • Eye pain or eye irritation: These symptoms may be more noticeable when you wake up after being closed for a long time, for example when you wake up in the morning.
  • Light sensitivity (Photophobia): This means that bright light becomes unbearable and may cause discomfort or pain.
  • Watery eyes (Epiphora): If your eyes feel dry, this may be because your tear system is trying to produce more tears to compensate.
  • Nystagmus: Uncontrolled rapid eye movements from side to side or in a circular motion.

What causes corneal dystrophy?

Each of these different forms of corneal dystrophy is linked to genetic factors . These can be "de novo" changes (meaning that they occur randomly in your body) or they can be inherited from one or both of your parents.

Some inherited variations require only one copy of the gene from one parent. Other variations require two copies of the mutated gene, one from each parent. Also, there are still some types of corneal dystrophy, and further research is needed to find out exactly what specific genetic variations cause them.

Who is most at risk for this condition?

Most corneal dystrophies do not affect people differently by race or sex. The only exception is Fuchs endothelial corneal dystrophy , which has several specific risk factors:

  • Race: White people are more likely to develop it.
  • Age: People over 30 years of age are more likely to develop it.
  • Gender: Women are more likely to develop it.

What are the possible complications of corneal dystrophy?

These conditions, which fall under the umbrella of corneal dystrophy, affect the way light passes through your cornea. As a result, the clarity of your cornea decreases, distorting the light that passes through it. This means that corneal dystrophy can cause severe vision loss , but it does not lead to total blindness .

However, severe vision loss can be a serious problem. It can affect your ability to drive, read, and perform many everyday tasks.

How is corneal dystrophy diagnosed?

An eye specialist, either an ophthalmologist or an optometrist, can diagnose corneal dystrophy. They use a combination of tools and techniques to do so. The most important part is an eye exam , especially a slit lamp exam . A slit lamp exam involves looking inside your eye with a special microscope. This allows doctors to see any changes or damage that may be associated with corneal dystrophy.

Other tests your eye specialist may use include:

  • Corneal topography: This creates a "map" of the surface of your cornea. It shows if there are any areas of the corneal surface that are higher or lower than the surrounding areas.
  • Pachymetry: This measures the thickness of your cornea. This can help identify problems in the deeper layers of the cornea (such as endothelial dystrophy).
  • Genetic testing: This usually involves taking a sample of your saliva or blood and looking for specific DNA changes. This can help confirm the specific type of corneal dystrophy you have.
  • Optical Coherence Tomography (OCT): Although this is usually used to look at your retina, this test can also provide valuable scans of your cornea. This is especially useful when planning certain corneal treatments.
  • Corneal biopsy: An eye specialist may take a small tissue sample from your cornea and send it to a lab for testing. A pathologist will analyze the sample to find out if you have corneal dystrophy and, if so, what type. This test is not very common for corneal dystrophy, but it can help with the diagnosis.

Additional tests may be done depending on your symptoms and other factors. Your eye specialist can tell you more about other tests they may recommend.

How is corneal dystrophy treated?

There are many ways to treat corneal dystrophy. Treatment depends on the layer of the cornea affected and the severity of the condition.

Because treatment options can vary, your eye doctor is the best source of information about your treatment options. They can explain the details of the treatment options, possible complications, side effects, and other details to you.

Here are some of the main treatment methods:

Topical treatments

These focus on treating the symptoms and the effects on the surface of your cornea. Examples:

  • Medicated eyedrops or ointments: These can treat symptoms such as pain, a feeling of something in the eye, and dryness.
  • Antibiotics: These can help prevent infection if your cornea is damaged and is susceptible to germs.
  • Special contact lenses: Just like regular contact lenses, these are worn on the surface of the cornea. The main difference is that they help your cornea heal and prevent further damage.

Phototherapeutic keratectomy (PTK)

Phototherapeutic keratectomy (PTK) involves removing parts of the cornea using a very finely tuned laser beam. Your eye specialist can precisely target and remove the damaged areas.

Cornea transplant

In cases where changes or damage to your cornea are severe enough to affect your vision, a cornea transplant may be an option. These surgeries involve replacing your corneal tissue with new tissue from a deceased donor. These can be full or partial transplants.

What can you expect if you have corneal dystrophy?

If you have corneal dystrophy, what you can expect can be a little different. Most people are diagnosed with one of these conditions, usually before the age of 20.

Corneal dystrophy is usually a progressive condition. This means that its effects gradually increase over time - usually over years, sometimes even decades. Some people have no symptoms at first, so they may not even know they have one of these conditions for years. Some people never have any symptoms or problems at all.

Because there are so many different things you can experience, it's a good idea to ask your eye doctor about what to expect. They can tell you what might be most relevant to you.

How long does corneal dystrophy last?

Corneal dystrophies are lifelong conditions. You are born with them. Corneal transplants can "cure" these conditions, but that is not a guarantee. Many people who have a corneal transplant will eventually develop the disease in the transplanted cornea. If that happens, further treatment – ​​even a second corneal transplant – may be needed.

What is the outlook for this situation?

Corneal dystrophy often causes some degree of vision loss , but it is very rare for that vision loss to progress to complete blindness (a state where you cannot even perceive light).

If you have a cornea transplant, you will face the same risks as other transplant recipients. You will need to take immunosuppressant drugs for the rest of your life to prevent your immune system from attacking the transplanted tissue. Also, even with immunosuppressant treatments, rejection can occur.

Because there are so many variables that can affect your outlook, the best person to ask about the outlook for your condition is your eye doctor. They can provide information specific to your specific situation.

Can corneal dystrophy be prevented?

Corneal dystrophies are genetic conditions, meaning there is no way to prevent them or reduce the risk of developing them.

If I have corneal dystrophy, how do I take care of myself?

If you have corneal dystrophy, you should see your eye doctor regularly, as recommended. They can monitor your condition, look for changes in your eye tissue, and any symptoms you're experiencing. They can also adjust and update your treatment plan to keep it effective for as long as possible.

If your eye specialist prescribes medications for your eyes, you should use them exactly as prescribed . Medications can help limit the symptoms of these conditions or slow down tissue changes or damage.

Especially if you have had any type of corneal transplant, it is very important to take your medications exactly as prescribed. Doing so will help ensure that your body does not reject the transplanted tissue. This can help prevent serious complications and even permanent vision loss.

When should I see my ophthalmologist?

You should see your eye doctor for regular follow-up visits on a regular schedule. Also, if you notice any changes in your symptoms or vision, or if any treatment is causing side effects or seems to be less effective, you should talk to them about it. They will try to help you find solutions to the problems you are facing.

What questions should I ask my ophthalmologist?

You can ask your ophthalmologist these questions:

  • What type of corneal dystrophy do I have?
  • What treatment(s) do you recommend?
  • Are there any symptoms that require emergency medical attention?
  • What is the long-term outlook for my condition?
  • Is there anything I can do to improve my outlook?

Finally, what to remember

Corneal dystrophies are eye diseases that often become apparent when you're young . If you're feeling overwhelmed trying to understand these diseases, you're not alone. There are more than twenty different types of corneal dystrophies. The effects of these diseases can also vary greatly.

But you don't have to try to understand or manage corneal dystrophy on your own. Your eye doctor can help you understand your specific condition, its effects, and how to treat it. And, with so many treatment options, you have a better chance of managing your condition and minimizing its impact on your life. Don't be afraid, stay strong, and follow your doctor's advice.


` Corneal Dystrophy, cornea of ​​the eye, genetic eye diseases, blurred vision, eye treatment, corneal transplant, eye health

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