Cornea Guttata and Fuchs’ Dystrophy

Written by Dr Osama Bahsas Zaky
Updated on October 27, 2021

What is Fuchs’ Dystrophy?

Fuchs’ Dystrophy Signs and Symptoms.

What is Cornea Guttata?

What is Bullous Keratopathy?

Fuchs’ Dystrophy Causes and Diagnosis.

Fuchs’ Dystrophy Treatment Options.

Corneal Transplant Surgery.

Is Corneal Transplant Really Effective?

Fuchs’ Dystrophy and Cataracts.

Fuchs’ Dystrophy Surgery Cost.

Cornea guttata is not the same as Fuchs’ dystrophy. The latter is a progressive eye disease, and cornea guttata is the initial sign or structural alteration of Fuchs’ dystrophy.

The problem with this disease is that you may not know you have Fuchs’ dystrophy until it causes noticeable symptoms.

So, getting a regular eye exam can help diagnose Fuchs’ dystrophy before it progresses further.

It’s best to catch the disease early to treat vision problems and control any eye discomfort.

What is Fuchs’ Dystrophy?

In Fuchs’ dystrophy, fluid tends to build up in the clear outer layer on the front of your eye (cornea), causing it to swell, thicken, and get cloudy.

Normally, the cells covering the inside of the cornea (endothelial cells) help maintain a normal balance of fluid within the cornea and prevent any swelling (edema).

Indeed, the endothelial cells are in charge of pumping out excess fluid and keeping the cornea clear enough for you to ‘see properly.’

But, with Fuchs’ dystrophy, the corneal endothelial cells gradually die or do not work properly.

Fuchs’ Dystrophy Signs and Symptoms

As the cornea is one of the more important refractive elements of your eye, Fuchs’ dystrophy can lead to blurred vision, halos, or glare.

You may also experience eye discomfort in bright light and reduced contrast sensitivity. Your eyes can feel gritty and painful too.

Fuchs’ dystrophy typically affects both eyes and can cause your vision to gradually worsen over the years. Although for many patients, the condition progresses slowly and doesn’t develop any visual acuity problems.

Blurred vision in the morning is the first sign of Fuchs’ dystrophy (stage 1). At the early onset, you might also have difficulty seeing in low light.

The excess fluid builds up overnight as you sleep. So, you get a fluctuation in vision, with worse symptoms in the morning after awakening and gradually improving during the day.

This visual improvement happens because when your eyes are open, the excess fluid in the cornea evaporates from the surface. The blurriness usually gets worse when the humidity level is high, though.

As the disease progresses then comes stage 2. So, impaired vision can take longer to improve (even up to the entire day) or doesn’t improve at all.

Although a patient is born with the disease, it is not detectable or symptomatic until the 30s and 40s. Some patients don’t develop symptoms until they reach their 50s or 60s.

What is Cornea Guttata?

During the disease’s progression, the layer of cells (endothelium) responsible for maintaining proper fluid levels in the cornea will deteriorate and cause tiny bumps (guttae) to form on the back of the cornea.

Hence, cornea guttata is the initial alteration of Fuchs’ dystrophy.

These tiny drop-shaped irregular lumps in the corneal endothelium can only be seen using a special microscope.

Cornea guttata contributes to the ongoing cell death within the cornea, leading to worsening vision problems. They can cause glare and halos.

What Is Bullous Keratopathy?

If your cornea is swollen due to Fuchs’ dystrophy, you may also develop blisters on its surface. These are called epithelial ‘bullae.’ If this occurs, it is known as bullous keratopathy.

Bullous keratopathy can also appear after corneal endothelial trauma. This can occur with intraocular surgery (cataract removal) or after a malpositioned intraocular lens implant placement.

If blisters develop and burst, they can cause corneal scarring, which will reduce vision in the affected eye.

Blisters can alter the shape of the cornea, making it uneven and affecting the way light is focused through the eye. Hence, with bullous keratopathy, you get blurred vision.

If these blisters burst, you may experience sharp pain, discomfort, and foreign body sensation of an object trapped in the eye.

You can also present with light sensitivity, glare, halos around lights, and watery eyes.

If you have bullous keratopathy, your eye doctor will prescribe medication to help with pain or any eye discomfort.

You may also need to wear soft therapeutic contact lenses. These act as a bandage that relieves pain by protecting exposed nerve endings on the surface of your cornea. They also give the burst blisters time to heal.

Also, bacteria can invade a ruptured bulla, leading to a corneal ulcer. So antibiotics are important too in bullous keratopathy.

Fuchs’ Dystrophy Causes and Diagnosis

In many cases, the cause is unknown.

The dystrophy can be inherited, but the genetics of the disease are complex. Family members can present it to varying degrees or not at all.

If one of your parents has it, there may be a 50 % chance of developing the condition yourself. This is called autosomal dominant inheritance.

Also, women are more likely to have Fuchs’ dystrophy. Other risk factors include diabetes and smoking.

Signs and symptoms can be difficult to detect at first. So, the condition is often diagnosed during a routine eye exam.

As a general rule, you should always go to an eye doctor if you experience vision changes or any discomfort in your eyes.

Your eye doctor will perform a slit lamp examination to do a detailed evaluation of your cornea surface.

The doctor may apply eye drops containing a yellow-green dye called fluorescein—the fluorescein works by temporarily staining damaged areas of the cornea that are not otherwise visible.

Your doctor may notice small bumps on your cornea, which are a telltale sign of Fuchs’ (cornea guttata).

Also, a test called pachymetry allows measuring the corneal thickness, indicating corneal swelling from the disease.

Confocal/specular microscopy allows photographs of the corneal endothelium. It can effectively measure the number, shape, and density of your endothelial cells.

What are Fuchs’ Dystrophy Treatment Options?

There’s no cure for Fuchs’ dystrophy.

However, the early stages of the disease (cornea guttata) can be treated with non-surgical solutions such as 5% sodium chloride eye drops or salty eye ointments. They are used to draw the excess fluid from the cornea.

You could use a hairdryer, held at arm’s length, to gently blow warm air on your face (two or three times a day). This helps dry the surface of your cornea.

If you experience light sensitivity, you can find light-activated sunglasses or photochromic lenses helpful. These lenses turn darker in brighter sunlight.

Polarised lenses or anti-reflective coating may also help minimize glare reflected from flat surfaces such as water.

If Fuchs’ dystrophy isn’t affecting your vision or is only causing mild symptoms, you won’t need any treatment. But, your eyes should be routinely examined to monitor the progression of the condition.

However, the best option is cornea transplant surgery when the disorder is pretty advanced or you develop corneal scarring.

Corneal Transplant Surgery

Endothelial keratoplasty is a special type of corneal transplantation.

The portions of the cornea affected — the corneal endothelium and Descemet’s membrane — are replaced with healthy corneal tissue from a donor.

Types of endothelial keratoplasty commonly performed include Descemet stripping automated endothelial keratoplasty (DSAEK) and Descemet’s membrane endothelial keratoplasty (DMEK).

These procedures can replace the innermost layers of the cornea.

In DSAEK, you receive a new corneal endothelium, Descemet’s membrane, and some of the stroma from a donor cornea.

Conversely, with DMEK, you only get the endothelium and the Descemet’s membrane.

The new layers of corneal tissue are kept in place with a temporary air bubble, which acts as a bandage, holding the tissue.

DSAEK and DMEK have short recovery times (a few weeks or months), and the risk of rejecting the new corneal graft after surgery is also low.

This is because endothelial keratoplasty is considered ‘keyhole’ surgery, meaning that it does not require any stitches or very few stitches at best.

Less commonly, a few patients may present with a scarred stroma. In that case, a full-thickness transplant or ‘penetrating keratoplasty’ (PK) is necessary.

With penetrating keratoplasty, your whole cornea is replaced by a donor’s cornea. This type of transplant uses stitches and generally has a recovery period of about 18 months.

Is Corneal Transplant Really Effective?

Yes. However, sometimes a corneal transplant may need to be repeated if you present transplant rejection.

While Fuchs’ dystrophy or cornea guttata does not re-occur in the new corneal graft, the transplant may fail for other reasons. This includes rejection, infection, trauma, and natural loss of cells.

Corneal graft rejection happens when your immune system recognizes the transplanted tissue as a foreign body.

It is possible to treat corneal transplant rejection with anti-inflammatory steroid drops to reduce swelling, though.

Although rejection is a risk, nearly 75% of all corneal transplants last at least five years, and more than half last up to 10 years.

Fuchs’ Dystrophy and Cataracts

Patients with Fuchs’ dystrophy or cornea guttata can also develop cataracts.

Fuchs’ dystrophy and cataracts are more common as you get older, so they can often happen together. However, they do not make each other worse.

In cases of mild Fuchs’ severity with cataracts, cataract surgery alone may be an option.

Following cataract surgery, a corneal transplant may be necessary only if Fuchs’ dystrophy worsens after surgery.

Even though cataract surgery is usually very successful, any surgery to the eye can further damage the already-delicate endothelial cells or reduce their number.

This may result in corneal decompensation, aka corneal swelling, and may need to be followed up by, or combined with, an endothelial keratoplasty transplant.

So, a careful assessment of the cornea is mandatory before cataract surgery is performed.

In cases of advanced Fuchs’ dystrophy, both cataract surgery and a corneal transplant can be done simultaneously. By using this approach, patients can benefit from a shorter recovery time.

How Much does Fuchs’ Dystrophy Surgery Cost?

Fuchs’ Dystrophy surgery cost depends on the technique performed.

If you want to receive a quote, please, fill in the contact form. You can send us your recent medical reports, so we can offer you the solution that best suits your needs.

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