Cornea Diseases & External Eye Disease

Corneal diseases are a group of rare, genetic eye disorders.

General Overview

What are cornea & external eye diseases?

Corneal diseases are a group of rare, genetic eye disorders. With corneal diseases, abnormal material builds up in the cornea (the clear, front window of the eye). Most corneal diseases affect both eyes. They progress slowly and run in families.

The cornea has five layers:

  • Epithelium: the outermost, protective layer of the cornea.
  • Bowman’s membrane: this second protective layer is strong.
  • Stroma: the thickest layer of the cornea. It is made up of water, collagen fibers and other connective tissue. This strengthens the cornea and makes it flexible and clear.
  • Descemet’s membrane: a thin, strong inner layer that is also protective.
  • Endothelium: the innermost layer made up of cells that pump excess water out of the cornea.

What causes cornea & external eye diseases?

Corneal diseases are caused by the build-up of material in one or more layers of the cornea. The material may cause the cornea to lose its transparency. This can cause loss of vision or blurred vision.

There are more than 20 different types of corneal diseases. They are generally grouped into three categories:

  • Anterior or superficial corneal diseases affect the outermost layers of the cornea (the epithelium and Bowman’s membrane).
  • Stromal corneal diseases affect the stroma, which is the middle and thickest layer of the cornea.
  • Posterior corneal diseases affect the innermost parts of the cornea (the endothelium and the Descemet’s membrane). The most common posterior corneal disease is Fuchs’ dystrophy.

What are the symptoms?

The symptoms of corneal disease depend upon the type of corneal dystrophy. Some people experience no symptoms. In others, the build-up of material in the cornea causes it to become opaque (not clear). This leads to blurred vision or vision loss.

How are corneal & external eye diseases diagnosed?

If your ophthalmologist thinks you have a corneal dystrophy, they will examine your eye. They will also ask about your family history of eye disease.

Your ophthalmologist will use a slit lamp microscope to shine a thin, bright sheet of light into your eye. This helps the doctor examine the front part of your eye thoroughly.

If someone has no symptoms, a routine eye examination may show that they have corneal diseases. In some cases, genetic testing can identify corneal diseases.

What are the treatments?

Treatment for corneal diseases depends on:

  • the type of disease, and
  • the severity of symptoms

If you do not have any symptoms, your ophthalmologist may monitor your eyes closely to see if the disorder is progressing. In other cases, eye drops, ointments or laser treatment may be appropriate.

Cornea Abrasions

What is a corneal abrasion?

A corneal abrasion is a scratch or scrape on the surface of your cornea.

What causes a corneal abrasion?

Fingernails, makeup brushes, and tree branches are common culprits of corneal abrasions. Some other causes of corneal abrasion are rubbing your eye and having very dry eyes.

What are the symptoms?

  • The feeling that something is stuck in your eye
  • Red, painful, watery eyes
  • Blurry or hazy vision
  • Being extra sensitive to light

Why do corneal abrasions and corneal erosions hurt so much?

The cornea has many nerve cells. Cells called pain receptors transmit pain to tell us about possible damage to the eye’s surface. In fact, there are hundreds of times more pain receptors in our cornea than there are in our skin.

How are corneal abrasions diagnosed?

Your ophthalmologist will put dye called fluorescein on your eye’s surface. Then they will look at your cornea with an instrument called a slit lamp. The dye will highlight a cut or scratch on the cornea.

What are the treatments?

Your ophthalmologist will treat your eye based on what they find in the exam. Following are some options.

  • You might wear a patch over your injured eye. This is to keep you from blinking and making the corneal abrasion worse.
  • You may use moisturizing eye drops or ointment. This adds a soothing layer over the cornea.
  • Your ophthalmologist may prescribe antibiotic eye drops or ointment to prevent an eye infection.
  • You may be given special eye drops to dilate (widen) your pupil. This can help relieve pain.
  • You may be given a special contact lens to reduce pain and speed healing.

If your corneal abrasion is small, it probably will heal in 1–2 days. A larger corneal abrasion may take about a week to heal.

Cornea Ulcers

What is a corneal ulcer?

A cornea ulcer is an inflammation of the cornea — the clear, dome-shaped tissue on the front of your eye that covers the pupil and iris. Cornea ulcers may or may not be associated with an infection. Noninfectious cornea ulcers can be caused by a relatively minor injury, such as from wearing your contact lenses too long or getting a foreign body in the eye. Infectious cornea ulcers can be caused by bacteria, viruses, fungi, and parasites.

What causes a cornea ulcer?

Causes of cornea ulcers include:

  • Injury - If any object scratches or injures the surface of your cornea, noninfectious cornea ulcers may result. In addition, an injury may allow microorganisms to gain access to the damaged cornea, causing infectious cornea ulcers.
  • Bacteria, fungi or parasites - These organisms may live on the surface of a contact lens or contact lens carrying case. The cornea may become contaminated when the lens is in your eye, resulting in infectious cornea ulcers. Poor contact lens hygiene or contact lens overwear can cause both noninfectious and infectious cornea ulcers.
  • Viruses - The herpes viruses — herpes simplex and herpes zoster — may cause cornea ulcers.
  • Bacteria - Staphylococcus, streptococcus, and pseudomonas are common bacteria involved in cornea ulcers.
  • Contaminated water - Bacteria, fungi, and parasites in water — particularly in oceans, rivers, lakes and hot tubs — can enter your eyes when you’re swimming and result in cornea ulcers. However, even if you’re exposed to these organisms, a healthy cornea is unlikely to become infected unless there has been some previous breakdown of the corneal surface — for example, from wearing a contact lens too long.

What are the symptoms?

Symptoms of cornea ulcers include:

  • Eye redness
  • Eye pain
  • Excess tears or other discharge from your eye
  • Difficulty opening your eyelid because of pain or irritation
  • Blurred vision
  • Decreased vision
  • Sensitivity to light, called photophobia
  • A feeling that something is in your eye

When to see a doctor

If you notice any of the symptoms of cornea ulcers or infection, make an appointment to see an eye specialist right away. Delays in diagnosis and treatment of cornea ulcers can lead to serious complications, including blindness.

How are cornea ulcers diagnosed?

Diagnosing cornea ulcers typically involve the following:

  • Eye exam - Although it may be uncomfortable to open your eyes for the exam, it’s important to have your eye care provider examine your eyes.
  • Slit-lamp exam - Your eye care provider will examine your eyes with a special instrument called a slit lamp. It provides a bright source of light and magnification to detect the character and extent of cornea ulcers, as well as the effect it may have on other structures of the eye.
  • Laboratory analysis - Your eye care provider may take a sample of tears or some cells from your cornea for laboratory analysis to determine the cause of cornea ulcers and to help develop a treatment plan for you.

What are the treatments?

Noninfectious cornea ulcers

Treatment of noninfectious cornea ulcers vary depending on the severity. For example, with mild discomfort from a corneal scratch, artificial tear drops may be the only treatment. However, if a cornea ulcer is causing significant tearing and pain, topical eye medications may be necessary.

Infectious cornea ulcers

Treatment of infectious cornea ulcers vary depending on the cause of the infection.

Bacterial cornea ulcers

Antibiotic eye drops are the primary treatment for bacterial cornea ulcers. Depending on the severity of the infection, drop frequency can range from around four times a day to every 30 minutes, even during the night. Sometimes oral antibiotics are used as a supplement.

Fungal cornea ulcers

Cornea ulcers caused by fungi typically require antifungal eye drops and oral antifungal medication.

Viral cornea ulcers

If a virus is causing the infection, antiviral eye drops and oral antiviral medications may be effective. Other viruses need only supportive care such as artificial tear drops.

Acanthamoeba cornea ulcers

Cornea ulcers caused by the parasite acanthamoeba can be difficult to treat. Antiparasitic eye drops are used, but some acanthamoeba infections are resistant to medication and can require treatment for several months. Severe cases of acanthamoeba cornea ulcers may require a cornea transplant.

If a cornea ulcer doesn’t respond to medication, or if it causes permanent damage to the cornea that significantly impairs your vision, your eye care provider may recommend a cornea transplant.

What is dry eye?

Our eyes need tears to stay healthy and comfortable. If your eyes do not produce enough tears, it is called dry eye. Dry eye is also when your eyes do not make the right type of tears or tear film.

What causes dry eye?

People tend to make fewer tears as they get older due to hormonal changes. Both men and women can get dry eye. However, it is more common in women—especially those who have gone through menopause.

Here are some other causes of dry eye:

  • Certain diseases, such as rheumatoid arthritis, Sjögren’s syndrome, thyroid disease, and lupus
  • Blepharitis (when eyelids are swollen or red)
  • Entropion (when eyelids turn in); ectropion (eyelids turn outward)
  • Being in smoke, wind, or a very dry climate
  • Looking at a computer screen for a long time, reading, and other activities that reduce blinking
  • Using contact lenses for a long time
  • Having refractive eye surgery, such as LASIK
  • Taking certain medicines, such as:
    • Diuretics (water pills) for high blood pressure
    • Beta-blockers, for heart problems or high blood pressure
    • Allergy and cold medicines (antihistamines)
    • Sleeping pills
    • Anxiety and antidepressant medicines
    • Heartburn medicines

Tell your ophthalmologist about all the prescription and non-prescription medicines you take.

What are the symptoms?

Here are some of the symptoms of dry eye:

  • You feel like your eyes are stinging and burning.
  • Blurred vision, especially when reading.
  • There is a scratchy or gritty feeling like something is in your eye.
  • There are strings of mucus in or around your eyes.
  • Your eyes are red or irritated. This is especially true when you are in the wind or near cigarette smoke.
  • It is painful to wear contact lenses.
  • You have lots of tears in your eyes.

Having a lot of tears in your eyes with dry eye might sound odd. But your eyes make more tears when they are irritated by dry eye.

How is dry eye diagnosed?

Your ophthalmologist will begin with an eye exam. They will look at your eyelids and the surface of the eye. They will also check how you blink.

There are many different tests that help diagnose dry eyes. Your ophthalmologist may do a test that measures the quality or the thickness of your tears. They may also measure how quickly you produce tears.

What are the treatments?

Treating dry eye by adding tears

Your ophthalmologist might tell you to use artificial tears. These are eye drops that are like your own tears. You can use artificial tears as often as you need to. You can buy artificial tears without a prescription. There are many brands. Try a few until you find a brand that works best for you.

If you use artificial tears more than six times a day or are allergic to preservatives, you should use preservative-free tears. This is because the tears with preservatives may start to irritate your eyes.

Stop dry eye by saving tears

Your ophthalmologist may suggest blocking your tear ducts. This makes your natural tears stay in your eyes longer. Tiny silicone or gel plugs (called punctal plugs) may be inserted in your tear ducts. These plugs can be removed later as needed. Your ophthalmologist could also recommend surgery that permanently closes your tear ducts.

Stop dry eye by increasing your tears

Your ophthalmologist might have you use a prescription eye drop medication. This helps your eyes make more of their own tears.

Treating dry eye culprits

If your eyes are irritated, your ophthalmologist can treat those problems. They may recommend:

  • Prescription eye drops or ointments
  • Warm compresses on the eyes
  • Massaging your eyelids
  • Certain eyelid cleaners

Fuchs’ Dystrophy

What is fuchs’ dystrophy?

Fuchs’ Dystrophy is a disease of the cornea. It is when cells in the corneal endothelium gradually die off. These cells normally pump fluid from the cornea to keep it clear. When they die, fluid builds up and the cornea gets swollen and puffy. Vision becomes cloudy or hazy.

Fuchs’ Dystrophy has two stages. In the early stage, vision is usually hazy in the morning. In the disease’s second stage, vision remains blurry all day.

People in their 30s and 40s may have Fuchs’ Dystrophy but not know it. Vision problems might not appear until age 50 or later. Women are more likely than men to have Fuchs’ Dystrophy. Family history of Fuchs’ Dystrophy also increases your risk of getting it.

What causes fuchs’ dystrophy?

Normally, the cells lining the inside of the cornea (endothelial cells) help maintain a healthy balance of fluid within the cornea and prevent the cornea from swelling. But with Fuchs’ Dystrophy, the endothelial cells gradually die or do not work well, resulting in fluid buildup (edema) within the cornea. This causes corneal thickening and blurred vision.

Fuchs’ Dystrophy is usually inherited. The genetic basis of the disease is complex — family members can be affected to varying degrees or not at all.

What are the symptoms?

In the early stage (stage 1) of Fuchs’ Dystrophy, you may notice few, if any, symptoms. Your vision may be hazy or blurry when you first wake up but improve throughout the day. This is because your eyes normally stay moist when they are closed during sleep. But when you are awake, the fluid dries normally.

In the later stage (stage 2), your blurry or hazy vision will not get better as the day goes on. Too much fluid builds up during sleep and not enough dries up during the day. Also, tiny blisters may form in the cornea. The blisters get bigger and eventually break open, causing eye pain. Here are other symptoms:

  • Sandy or gritty feeling in your eyes
  • Being extra sensitive to bright light
  • Eye problems get worse in humid areas
  • Very blurry or hazy vision from scarring at the center of the cornea

How is fuchs’ dystrophy diagnosed?

Your ophthalmologist will look closely at your cornea and measure its thickness. This is called pachymetry. They will also check for tiny blisters on the front surface of the cornea and drop-like bumps of the back surface of the cornea (guttae). Using a special photograph of your cornea, your ophthalmologist may count your endothelial cells.

What are the treatments?

There is no cure for Fuchs’ Dystrophy. However, you can control vision problems from corneal swelling. Your treatment depends on how Fuchs’ Dystrophy affects your eye’s cells.

Here are treatments for early Fuchs’ Dystrophy:

  • Use an eye drop medicine or ointment to reduce swelling of the cornea’s cells.
  • Use a hair dryer, held at arm’s length, to blow warm air on your face. This helps dry the surface of your cornea.

For very poor vision or scarred corneas, you may need a corneal transplant. This surgery could be one of three types:

  • Endothelial keratoplasty (EK): Healthy endothelial cells are transplanted into your cornea.
  • Full corneal transplant: The center of your cornea is replaced with a healthy donor cornea.
  • Descemet Stripping Only (DSO): Removal of a very thin, unhealthy membrane inside the eye. The procedure does not require a corneal graft but relies on healthier endothelial cells from the cornea periphery to repopulate and “rejuvenate” the central cornea after removal of the dysfunctional central endothelium.

Your ophthalmologist will discuss what treatments are best for your condition.

Keratoconus

What causes keratoconus?

Doctors do not know for sure why people have keratoconus. In some cases, it appears to be genetic (passed down in families). About 1 out of 10 people with keratoconus have a parent who has it too. Keratoconus is also associated with:

  • Eye allergies
  • Excessive eye rubbing, and
  • Connective tissue disorders like Marfan syndrome and Ehlers-Danlos syndrome

What are the symptoms?

Keratoconus often starts when people are in their late teens to early 20s. The vision symptoms slowly get worse over a period of about 10 to 20 years.

Keratoconus often affects both eyes, and can lead to very different vision between the two eyes. Symptoms can differ in each eye, and they can change over time.

In the early stage, keratoconus symptoms can include:

  • mild blurring of vision
  • slightly distorted vision, where straight lines look bent or wavy
  • increased sensitivity to light and glare
  • increased nearsightedness or astigmatism (when your eye cannot focus as well as it should). As a result, you may need new eyeglass prescriptions often.

In later stages, keratoconus symptoms often include:

  • more blurry and distorted vision
  • not being able to wear contact lenses. They may no longer fit properly and they are uncomfortable.

Keratoconus usually takes years to go from early- to late-stage. For some people, though, keratoconus can get worse quickly. The cornea can swell suddenly and start to scar. When the cornea has scar tissue, it loses its smoothness and becomes less clear. As a result, vision grows even more distorted and blurry.

How is keratoconus diagnosed?

A routine eye exam may cause a suspicion of keratoconus, but it is best diagnosed by special scans of your cornea. Your optometrist or ophthalmologist will review these scans for information about thickness and curvature to diagnose keratoconus and monitor for progression.

What are the treatments?

Keratoconus treatment depends on your symptoms. When your symptoms are mild, your vision can be corrected with eyeglasses. Later you may need to wear special hard contact lenses to help keep vision in proper focus.

Here are other ways that your ophthalmologist might treat keratoconus:

  • Corneal cross-linking (CXL). Your ophthalmologist uses a special UV light and eye drops to strengthen the cornea. Doing this helps to flatten or stiffen your cornea, keeping it from bulging further.
  • Corneal transplant. When symptoms are severe, your ophthalmologist may suggest a corneal transplant. Your ophthalmologist replaces all or part of your diseased cornea with healthy donor cornea tissue.
  • Scleral Contact Lens. These are large-diameter lenses that provide clear and comfortable vision for keratoconus patients because the lenses do not actually touch the cornea.
  • Gas Permeable Contact Lens. Rigid Gas Permeable lenses (also known as RGPs or simply GPs) are one of the most popular contact lenses for Keratoconus. These stable contact lenses essentially act as a second surface for the eye, compensating for the irregularities of the cornea and functioning as the eye’s new reflective surface.

Do not rub your eyes!

With keratoconus, try to avoid rubbing your eyes. This can damage thin corneal tissue and make your symptoms worse.

If you have itchy eyes that cause you to rub, speak to your ophthalmologist about medicines to control your allergies.

Pterygium

What is Pterygium?

Pterygium is a growth of fleshy tissue (has blood vessels) that may start as a pinguecula. It can remain small or grow large enough to cover part of the cornea. When this happens, it can affect your vision.

What causes pterygium?

Pterygium is believed to be caused by a combination of exposure to ultraviolet (UV) light from the sun, wind, and dust.

What are the symptoms?

Symptoms of pterygium can range from mild to severe. They include:

  • Redness and swelling of the conjunctiva, mostly while the pterygium grows
  • A yellow spot or bump on the white of your eye
  • Dry, itchy, burning eyes or feeling like sand or grit is stuck in your eye
  • Blurry vision

How is pterygium diagnosed?

The diagnosis is made by slit-lamp examination of the wing-shaped limbal growth at the characteristic location within the palpebral fissure. The diagnosis is most often clear clinically, but histopathologic confirmation is performed routinely, as there can be associated dysplasia of the overlying tissue.

What are the treatments?

In many cases pterygium does not need to be treated. However, if your eyes are uncomfortable or your vision is affected, you may need treatment.

Your ophthalmologist can treat the discomfort, redness, or swelling from a pterygium with lubricating or steroid eye drops.

If a pterygium grows large enough to cause problems, your ophthalmologist will probably recommend surgery to remove it. Once the pterygium is removed, your surgeon may transplant a thin piece of normal tissue onto the affected area. This technique helps reduce the chance that your pterygium will grow back.

The best way to keep pterygium from coming back is to avoid sunlight, dryness, and dust.