Corneal dystrophy

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at August 3, 2016
StartDiseasesCorneal dystrophy

Corneal dystrophy is a group of rare and degenerative eye disorders. They are known for causing foreign material to accumulate inside of the cornea and cause chronic vision loss. There are more than twenty different types of corneal dystrophy and the effects of these disorders largely depend upon the type of dystrophy.

Contents

Definition & Facts

In order for a disorder to be labeled under the category of corneal dystrophy, it must be hereditary, degenerative, and cause material to accumulate in the cornea. There is currently an international system to categorize these different types of dystrophies. Some of the most common disorders include:

While there are many different corneal dystrophies, they all have several features in common. Corneal dystrophies affect both eyes, worsen with time and do not affect other parts of the body. These disorders pass on due to dominant genes and recessive genes.

Symptoms & Complaints

The symptoms of corneal dystrophy vary wildly based upon the type of disorder that one inherits. While some dystrophies show few to little symptoms, others can worsen to severe and debilitating symptoms. When corneal dystrophy gets to its later stages, the buildup of material in the cornea often causes blurred vision and even blindness.

This corneal degeneration can be easily seen by looking at the eyes of someone suffering from corneal dystrophy. Often, their eyes will turn white and glassy to reflect their loss of vision. Patients with corneal dystrophy will also often experience corneal erosion, which results in the outermost layer of the cornea being unable to connect to the next layer of the cornea.

Corneal erosion is typically accompanied by mild to severe pain in the eye, light sensitivity, and eye irritation. For those suffering from corneal dystrophy, symptoms can begin at any point in their lives. Many genetic carriers go their entire lives without showing symptoms. However, once the first symptom appears, it is only a matter of time until the condition of the eye deteriorates.

Causes

All corneal dystrophies are genetic disorders, but the ways that they are passed on can vary. Certain disorders are passed on from dominant traits, which means that only one parent needs to be a carrier to pass the disorder onto the child. The disorders that spread as recessive traits are less common because they require both parents to be carriers in order to pass on the trait.

In many cases of corneal dystrophy, the only solid information about the cause is an issue in the genes. More is known about the effects of the disease than the causes. The degeneration can be caused by different factors, but all of them are centered around buildup in the cornea and other issues that prevent the cornea from functioning properly. This material then makes it difficult to impossible for the individual to see.

Diagnosis & Tests

When an optometrist or ophthalmologist suspects corneal dystrophy in a patient, the first thing that they do is ask about the patient's family history of eye disorders. In all likelihood, someone with a corneal disorder will have a history of it in their family.

Beyond that, the practitioner will then conduct a slit-lamp examination, using a special kind of lamp to look at the eye. This lamp creates a thin line of light that allows them to see directly into the front portion of the eye. From there, they can closely examine any corneal irregularities in the eye and determine if the cause is corneal dystrophy.

If a patient is suffering from corneal dystrophy, it is usually obvious to see the foreign material that has built up in their corneas. It is also possible to find evidence of corneal dystrophy through genetic testing as genetic mutations cause these disorders. Many people have also had their corneal dystrophies discovered during routine eye examinations in which their eye care practitioner noticed the early signs of degenerative disease.

Treatment & Therapy

Treatment of corneal dystrophy largely depends upon the type of disorder that the patient is suffering from. If there are no symptoms but the disorder has been confirmed through genetic testing, then the practitioner will closely monitor the patient for future symptoms.

If symptoms have been displayed, there are several different treatment varieties depending upon the condition. Corneal erosion is treated through repeated rounds of antibiotics, moisturizing eye drops, and soft bandage lenses that are designed to protect the cornea. Many cases require continued laser surgery and even corneal scraping to remove foreign material.

In the most severe cases of corneal dystrophy, the patient may require a cornea transplant. The damaged cornea is surgically removed and the new, healthy cornea is put in place.

In cases where a disorder affects the deepest part of the cornea, a partial transplant may be used instead. While they are largely used only as a last resort, corneal transplants have proven quite successful with patients that have partially lost their vision due to corneal degeneration.

Prevention & Prophylaxis

There are currently no known ways to prevent corneal dystrophy in someone with a genetic predisposition to the disorders. The most recommended method to keep optimal eye health is to regularly schedule eye appointments to monitor symptoms.

This allows the patient to receive treatments before loss of vision has a chance to occur. While there is no way to prevent these disorders, early and thorough treatments can do much to slow their progression.