Fuchs Dystrophy

Facts About Fuchs Dystrophy

 

Fuchs Dystrophy is a progressive degenerative disease resulting in the loss of corneal endothelial cells.

Progression of the disease is usually very slow. The endothelial cells are responsible for pumping excess fluid out of the cornea, thus maintaining the fluid balance necessary for a transparent cornea and clear vision.

The early stages are marked by the presence of guttae (collagen deposits on Descemet’s membrane thought to be secreted by abnormal endothelial cells) and mild blurred vision.

In the mid stages of Fuchs Dystrophy, the endothelial cells, which are now less numerous and distorted in appearance, are unable to actively pump out enough fluid to maintain the necessary balance, and corneal edema/ vision impairment results.

In the late and end stages, the corneal epithelium is also affected, resulting in swelling, bullae (blisters), subepithelial scarring, and severe blurred vision.

Fuchs Dystrophy is a genetically inherited disease, which is more prevalent in females and in the elderly population.

Fuchs Dystrophy is commonly diagnosed by specular microscopy and slit lamp examination.

Treatment for Fuchs Dystrophy ranges from application of topical hypertonic saline drops to surgical intervention, depending on the progression.

Treatment Options for Fuchs Dystrophy

  • 5% sodium chloride drops and ointment are hypertonic saline drops and ointment that help reduce corneal edema by osmotically drawing fluid out from the cornea.
  • Soft contact lenses can keep the eye comfortable when bullae form (bullous keratopathy).
  • Drying your eyes using a hair dryer to “dry your eyes” with warm air a few times a day can help dry out blisters and reduce excess fluid thru evaporation.
  • Keratoplasty – DSEK (partial corneal transplant) and PKP (full corneal transplant. which is not commonly used to treat Fuchs’ corneal dystrophy anymore) may be necessary in the late or end stages of Fuchs’.
  • DSEK – (Descemet’s Stripping Endothelial Keratoplasty), or a partial corneal transplant, involves replacing only part of the stromal layer along with Descemet’s membrane and inner endothelial layer. This process leaves most of the patient’s own healthy cornea intact, allows for faster healing, and minimizes the chance of graft failure.

New treatments include DMEK (similar to DSEK), and DALK (for deep corneal scars), which are the next evolution of endothelial transplantation.

  • DMEK – (Descemet’s Membrane Endothelial Keratoplasty), involves replacing only Descemet’s membrane and the inner endothelial layer. The absence of stromal transplantation allows for better post-op visual acuity when compared to DSEK. The drawback to DMEK is the fact that it is a more challenging surgery.  The transplanted layer is very thin and hard to handle, thus damaging the transplant during the procedure is a very real risk.
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