Fuchs' endothelial dystrophy

What is Fuchs' dystrophy?

Fuchs endothelial dystrophy (or simply Fuchs dystrophy) is a hereditary disease of the cornea, the transparent front part of the eye.

It affects the endothelium - the innermost layer of corneal cells - which plays a critical role in maintaining corneal clarity. When the number of these cells decreases and they degenerate, fluid accumulates within the cornea, resulting in corneal edema, visual blurring, and other associated symptoms.
Although the disease may be present earlier in life, it most commonly manifests after the age of 50.

Those at higher risk include women (who are affected more frequently than men), individuals with a family history of the disease, and patients with a history of other ocular conditions or previous ocular surgeries.
The most common symptoms include blurred vision (particularly in the morning), photophobia, a foreign body sensation, and vision that improves during the day but progressively deteriorates over time. In later stages, patients may experience ocular pain and persistent visual impairment, often described as a constant haze or fog.

The good news is that effective treatment options are available, and the approach depends on the stage of the disease.
In the early stages, symptoms can be alleviated with artificial tears, hypertonic saline eye drops, or ointments that help draw excess fluid out of the cornea.
In advanced stages, when corneal clouding is pronounced, surgical management in the form of corneal transplantation remains the definitive treatment.

Today, modern techniques such as DSAEK (Descemet’s Stripping Automated Endothelial Keratoplasty) and DMEK (Descemet’s Membrane Endothelial Keratoplasty) are preferred, in which only the innermost corneal layer is transplanted - a procedure also known as posterior lamellar keratoplasty. These approaches enable faster recovery and carry a lower risk of complications compared to traditional full-thickness transplantation.
However, in cases where chronic stromal changes have occurred and lamellar keratoplasty is unlikely to yield visual improvement, penetrating keratoplasty (full-thickness corneal transplantation) may be required, as the remaining corneal tissue is too opacified to support visual recovery.