Fuchs’ Dystrophy

Fuchs' dystrophy, also called endothelial dystrophy, is a disease that affects the cornea. Healthy endothelial cells are required to keep the cornea clear, but with Fuchs', the endothelial cells on the back layer of the cornea are not normal. Most patients with Fuchs dystrophy have a very mild form that never affects their vision. When it does affect vision, it usually occurs in middle age or later. In later stages, it can cause pain.

What are the symptoms?

In mild Fuchs' dystrophy, the vision may be slightly decreased. As the Fuchs' dystrophy progresses and the corneal swelling worsens, the vision slowly declines. It is often worse in the morning than later in the day. Late in the course, pain or severely decreased vision can occur. 

What are the causes?

Poorly functioning corneal endothelial cells.

What are the risk factors?

Often runs in families 6. Complications: corneal scarring, painful corneal bullae (blisters).

Tests and Diagnosis

Slit lamp examination is essential. Corneal thickness testing (pachymetry). Imaging of the corneal endothelial cells with specular microscopy may be helpful.

Treatment

Treatment options include 5% saline drops and/or ointment to decrease swelling. Blowing cool or warm (not hot) air from a blow dryer upon awaking can often improve vision earlier in the day. Later, corneal transplantation, either a partial thickness endothelial transplant (e.g. Descemet’s stripping endothelial keratoplasty [DSEK] or Descemet’s membrane endothelial keratoplasty [DMEK]) or a full thickness corneal transplant.

Prevention

Medical treatment may prevent or delay the onset or corneal haze. Endothelial keratoplasty is more successful in eyes with minimal to no corneal haze. In eyes with significant corneal scarring, a full thickness cornel may be required to achieve good vision.

Slit lamp imageFuchs' Dystrophy Slit Lamp

Patient with Fuchs'Fuchs' Dystrophy

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