Corneal Disease

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The most prevalent diseases/conditions we see in practice are related to Fuch’s Endothelial Dystrophy, post trauma injuries, post surgical damage, and Keratoconus.  We screen for these problems at every eye health examination – see Endothelial Testing for more information.

Endothelial Cell Disease / Loss

The Endothelial Cells are single layer of specialized, flattened cells that line the posterior surface of the cornea and face the anterior chamber of the eye. The corneal endothelium governs fluid and solute transport across the posterior surface of the cornea and actively maintains the cornea in the slightly dehydrated state that is required for optical transparency.

Fuch’s Endothelial Dystrophy (excerpted from the National Eye Institute)

Fuch’s Endothelial Dystrophy is a slowly progressing disease that usually affects both eyes and is slightly more common in women than in men. Although doctors can often see early signs of Fuchs’ dystrophy in people in their 30s and 40s, the disease rarely affects vision until people reach their 50s and 60s.

Fuchs’ dystrophy occurs when endothelial cells gradually deteriorate without any apparent reason. As more endothelial cells are lost over the years, the endothelium becomes less efficient at pumping water out of the stroma. This causes the cornea to swell and distort vision. Eventually, the epithelium also takes on water, resulting in pain and severe visual impairment.

Epithelial swelling damages vision by changing the cornea’s normal curvature, and causing a sight-impairing haze to appear in the tissue. Epithelial swelling will also produce tiny blisters on the corneal surface. When these blisters burst, they are extremely painful.

At first, a person with Fuchs’ dystrophy will awaken with blurred vision that will gradually clear during the day. This occurs because the cornea is normally thicker in the morning; it retains fluids during sleep that evaporate in the tear film while we are awake. As the disease worsens, this swelling will remain constant and reduce vision throughout the day.

When treating the disease, doctors will try first to reduce the swelling with drops, ointments, or soft contact lenses. They also may instruct a person to use a hair dryer, held at arm’s length or directed across the face, to dry out the epithelial blisters. This can be done two or three times a day.

When the disease interferes with daily activities, a person may need to consider having a corneal transplant to restore sight. The short-term success rate of corneal transplantation is quite good for people with Fuchs’ dystrophy. However, some studies suggest that the long-term survival of the new cornea can be a problem.

Corneal Dystrophies  (excerpted from the National Eye Institute)

A corneal dystrophy is a condition in which one or more parts of the cornea lose their normal clarity due to a buildup of cloudy material. There are over 20 corneal dystrophies that affect all parts of the cornea. These diseases share many traits:

  • They are usually inherited.
  • They affect the right and left eyes equally.
  • They are not caused by outside factors, such as injury or diet.
  • Most progress gradually.
  • Most usually begin in one of the five corneal layers and may later spread to nearby layers.
  • Most do not affect other parts of the body, nor are they related to diseases affecting other parts of the eye or body.
  • Most can occur in otherwise totally healthy people, male or female.

Corneal dystrophies affect vision in widely differing ways. Some cause severe visual impairment, while a few cause no vision problems and are discovered during a routine eye examination. Other dystrophies may cause repeated episodes of pain without leading to permanent loss of vision.

Some of the most common corneal dystrophies include Fuchs’ dystrophy, keratoconus, lattice dystrophy, and map-dot-fingerprint dystrophy. 

Post Surgical Damage

We see a significant number of patients who have had less than ideal outcomes from refractive surgeries RK, LASIK, LASEK, etc).  Post surgical contact lens fitting can often restore functional vision.  See What is OrthoK? for more information.

Keratoconus (excerpted from the National Eye Institute)

This disorder–a progressive thinning of the cornea–is the most common corneal dystrophy in the U.S., affecting one in every 2000 Americans. It is more prevalent in teenagers and adults in their 20s. Keratoconus arises when the middle of the cornea thins and gradually bulges outward, forming a rounded cone shape. This abnormal curvature changes the cornea’s refractive power, producing moderate to severe distortion (astigmatism) and blurriness (nearsightedness) of vision. Keratoconus may also cause swelling and a sight-impairing scarring of the tissue.

Studies indicate that keratoconus stems from one of several possible causes:

  • An inherited corneal abnormality. About seven percent of those with the condition have a family history of keratoconus.
  • An eye injury, i.e., excessive eye rubbing or wearing hard contact lenses for many years.
  • Certain eye diseases, such as retinitis pigmentosa, retinopathy of prematurity, and vernal keratoconjunctivitis.
  • Systemic diseases, such as Leber’s congenital amaurosis, Ehlers-Danlos syndrome, Down syndrome, and osteogenesis imperfecta.

Keratoconus usually affects both eyes. At first, people can correct their vision with eyeglasses. But as the astigmatism worsens, they must rely on specially fitted contact lenses to reduce the distortion and provide better vision. Although finding a comfortable contact lens can be an extremely frustrating and difficult process, it is crucial because a poorly fitting lens could further damage the cornea and make wearing a contact lens intolerable.

In most cases, the cornea will stabilize after a few years without ever causing severe vision problems. But in about 10 to 20 percent of people with keratoconus, the cornea will eventually become too scarred or will not tolerate a contact lens. If either of these problems occur, a corneal transplant may be needed. This operation is successful in more than 90 percent of those with advanced keratoconus. Several studies have also reported that 80 percent or more of these patients have 20/40 vision or better after the operation.