Fuchs' endothelial dystrophy

What is Fuchs' dystrophy?

Fuchs endothelial dystrophy (often referred to simply as Fuchs dystrophy) is a hereditary condition affecting the cornea, the transparent front part of the eye.

It specifically targets the endothelium - the innermost layer of corneal cells - which plays a crucial role in maintaining corneal clarity. As these cells degenerate and decrease in number, fluid content accumulates in the cornea, leading to visual blurring and other symptoms.

Although the disease may be present earlier in life, it most commonly manifests after the age of 50. Risk factors include being female, as women are more frequently affected than men, having a family history of the disease, and having a history of other eye conditions or prior eye surgeries.

The most common symptoms include blurred vision (especially in the morning), light sensitivity, a sensation of having something in the eye, and vision that improves throughout the day but gradually worsens over time. In later stages, patients may experience pain and persistent vision loss, often described as a constant haze or fog.

The good news is that Fuchs dystrophy is treatable, and the treatment approach depends on the stage of the disease. In the early stages, symptoms can often be managed with artificial tear eye drops, hypertonic saline eye drops, or ointments that help draw excess fluid out of the cornea. In more advanced cases, when corneal clouding becomes significant, surgical intervention may be necessary in the form of corneal transplantation. Today, modern techniques such as DSAEK (Descemet's stripping automated endothelial keratoplasty) and DMEK (Descemet's membrane endothelial keratoplasty) are commonly used. These procedures involve transplants of the inner layer of the cornea and are known also as posterior lamellar keratoplasty. They offer faster recovery times and fewer complications compared to traditional methods. In cases where chronic stromal changes have developed and lamellar transplantation is unlikely to improve vision, a full-thickness corneal transplant may be required, as the remaining corneal tissue is too clouded to support visual recovery.