Fuch's Endothelial Dystrophy is the gradual deterioration and loss of endothelial cells for no apparent reason. Unfortunately, these cells cannot be replaced by the body so when they die they die. As the number of cells decrease over time, the cornea is less capable of removing water form the stroma, causing it to swell and distort vision. Haze and small blisters on the corneal surface may also appear.

As a slowly progressing disease, signs of Fuch's Endothelial Dystrophy begin to appear in people 30 to 40 years old. However, the disease doesn't normally affect vision until about 20 years later. It is more common in women than men.

 

What Causes Fuch's Dystrophy?

Fuchs’ endothelial dystrophy (FED) is a degeneration of corneal endothelial cells.  This is associated with accumulation of focal growths called guttae (drops) and thickening of Descemets Membrane.  Th combination and increasing severity of FED leads swelling of the cornea, called cornal oedema and loss of vision. The visual loss is like looking through a broken windscreeen of a car.  The endothelial cells are the major “pump” cells of the cornea. In FED, Descemet’s membrane is thickened with accumulation of abnormal wide-spaced 'debris' and numerous guttae. Corneal endothelial cells in end-stage FED are reduced in number and appear attenuated, causing progressive stromal edema. Progressive endothelial cell loss causes relative influx of fluid into the cornea, leading to swelling (corneal stromal edema), which results in distorted vision. Thecorneal surface becomes oedematous (full of water), resulting in more severe visual impairment. Focal areas of blisters on the corneal surface ("bullae") may be particularly painful.  Vision is often more distorted in the early stages of disease but recovers during the day as when the eyelids are open, evapouration is possible and the cornea thins  In later stages the vision remains blurred as too may cells have died.

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FED is an inherited disorder.  From a genetics point of view, this is termed 'autosomal dominant'.  There are also factors such as increased age and female sex that are termed envirnomental factors that are associated with FED.  Further loss of endothelial cells maybe associated with surgery, such as cataract surgery, or trauma.  Commonly, excessive swelling of the cornea follow cataract surgery, resulting in cornea oedema.

Diagnosis is made by clinical examination and monitoring may include measuring corneal thickness, visual acuity and specular microscopy.

 

Treatment includes:

  • Hypertonic saline eyedrops
  • Ointments and lubricants
  • Soft contact lenses
  • Drying of blisters with a hair dryer two or three times per day
  • Corneal transplant in the fom of DSEK

If you have been affected by any of the symptoms mentioned above, please don't hesitate to give us a call.

We have a team of experts on stand-by who are ready to take your call at our Manchester (0161 907 2685) Clinic or our Blackpool (01253 308 031) Clinic.