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A corneal dystrophy is a bilateral (both eyes) abnormality occasionally present at birth but more frequently appearing later in life. Any of the five layers of the cornea can be involved. Of the many known dystrophies, the one most commonly encountered in clinical practice is Fuchs’ Corneal Dystrophy.

Fuchs’ dystrophy is an inherited eye disease that causes the cells in the endothelium, the last layer of the cornea, to deteriorate. This leads to distorted vision and corneal swelling (corneal edema). The exact cause of Fuchs’ dystrophy is unknown, but is believed to be a combination of hereditary, hormonal and inflammatory factors. This condition affects both eyes and is more common in women than men.

 

The cornea is made up of five different layers, each of which has a specific function that controls vision. The endothelium is the back layer of the cornea, which removes excess fluid from the cornea. Excess fluid can cause the cornea to swell and vision to become distorted. Occasionally the swelling can become so severe that bullae (blister-like lesions) will develop that can result in pain. Once endothelial cells are lost, they do not grow back, so this condition will continue to progress with time.

Symptoms

Although this condition is inherited, symptoms do not usually appear until the patient is over the age of 50. Patients with Fuchs’ dystrophy may experience blurred vision as the first sign of this condition. Blurred vision occurs in the morning as a result of fluid accumulating in the cornea while you sleep. As this fluid evaporates throughout the day, vision may improve. Progressive swelling and thickening of the cornea may also lead to painful erosions and difficulty recovering from cataract surgery. Other symptoms of Fuchs’ dystrophy may include:

  • Sensitivity to bright lights

  • Halos around lights

  • Poor night vision

  • Sharp pains in the eyes

  • Sandy, gritty sensation in the eyes

  • Fluctuating vision

Treatment

While there is no cure for Fuchs’ dystrophy, there are several treatment options available to help relieve the symptoms of this condition and to prevent permanent damage. In its early stages, Fuchs’ dystrophy can often be treated with a salt solution to remove fluid from the eye and reduce swelling. In more advanced stages, Fuchs’ dystrophy may begin to interfere with daily activities and prevent patients from functioning normally. In this stage, patients may require a corneal transplant to replace the cornea with full, thick layers that allows them to see clearly. The DSAEK procedure is an innovative, new, partial corneal transplant procedure performed on an out-patient basis.

DSAEK

An innovative procedure known as DSAEK is getting high marks from our doctors at the Cornea and Laser Vision Institute. Until recently, cornea transplant patients in the St. Louis area had very few treatment choices if they ever wanted to see clearly again. The most likely option was to undergo full thickness corneal transplant – a complicated procedure that surgically replaces the entire damaged cornea with a donor cornea.

 

Short for Descemet’s Stripping Automated Endothelial Keratoplasty, DSAEK (pronounced de-sak), is not nearly as invasive, and requires considerably less recovery time. Rather than removing your entire cornea, our cornea specialists make a small incision in the side of the eye and replace only the damaged portion at the back inner dome of the cornea with donor cells. DSAEK uses the innovative instrumentation designed for LASIK. The donor cornea is prepared using the LASIK instrumentation to create only a thin layer of tissue to transplant. This thin layer contains a new healthy layer of pump cells. The surgeon then delicately pushes the new cells in place with a bubble of air. It’s like replacing the wallpaper instead of replacing the wall. During the first twenty-four hours, it is important to stay on your back as much as possible to keep the air bubble positioned correctly, anchoring the new tissue in place. The transplanted cells begin pumping fluid out of the cornea, thus creating a suction that causes them to adhere to the back of the host cornea.

We’re excited about DSAEK at our practice. Rather than facing a potential one-year recovery period (a huge drawback for full thickness corneal transplant patients), the healing time is mere months for DSAEK patients. Another benefit: the procedure involves little or no stitches. The incision required for the surgery is very small. DSAEK is performed as an outpatient procedure under local anesthesia. The patient can go home after surgery without even having to have an eye patch. Finally, there is also less risk of rejection because the majority of the cornea remains in place.

The main disadvantage of the DSAEK procedure is that the new layer of cells is manipulated more directly than in a full thickness corneal transplantation, potentially producing more damage and possibly resulting in graft failure. There is a 10% chance that the new tissue will not adhere properly requiring repositioning and a new bubble, or in less than 1% of cases, re-operation. The cornea is also left somewhat thicker than the original due to the addition of tissue to your current cornea. This may make it more difficult to follow glaucoma. Antibiotic eye drops will be used for a week or so to help prevent infection, and a mild steroid eye drop will need to be used for long term to help prevent rejection.

 

1 day after surgery with air bubble holding new cornea in place

The results of corneal transplantation are excellent, but as you can see, it takes a long time to heal. That is why it is such a wonderful thing to be able to replace full thickness corneal transplantation with the new partial thickness DSAEK procedure. This is now an option for many who would otherwise have been limited to a full thickness transplant. Although DSAEK is one of the most sophisticated and precise microsurgical procedures performed by an eye surgeon, as far as the patient is concerned, a big procedure has now been replaced by a much simpler, outpatient procedure that allows you to return to normal activities and restores your vision sooner.

 

Fuchs Dystrophy

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