Endothelial corneal dystrophy (Fuchs’ dystrophy)

Endothelial corneal dystrophy (Fuchs’ dystrophy)is a hereditary disease that affects the innermost layer of the cornea – the endothelium. The endothelium works in the cornea as a pump, constantly pumping out fluid from the thickness of the cornea, which gets there under the influence of normal intraocular pressure. Excess fluid in the cornea reduces its transparency to the point where the cornea becomes like frosted glass. Patients gradually lose endothelial cells as the dystrophy progresses. The fact is that endothelial cells do not divide, and their number is constantly decreasing. The remaining cells occupy the vacated area with a large spreading. For the time being, the condition is compensated by the increased work of the remaining cells. But over time, the pumping system becomes less efficient, causing corneal swelling, clouding, and eventually reduced vision.

In the early stages of the disease, patients report glare and increased light intolerance. Gradually, vision may deteriorate in the morning, somewhat recovering in the evening. This is due to the fact that during nighttime sleep there is no evaporation of moisture from the plane of the cornea, and moisture accumulates in the cornea. When the eyelids are open during the day, this mechanism for removing fluid from the cornea is also activated, and the balance shifts closer to normal. It is clear that as new endothelial cells die, vision is constantly reduced.

Fuchs endothelial dystrophy affects both eyes and is somewhat more common among women. It usually manifests itself in 30-40 years and gradually progresses. If the vision deteriorates so much that the patient loses the ability to take care of himself, there are indications for corneal transplantation.

Signs (symptoms)

  • blurry vision, which is often worse after sleep;
  • unstable visual acuity;
  • blinding (glare) when looking at a light source;
  • intolerance to bright light;
  • feeling of sand in the eyes.

Diagnosis

Endothelial dystrophy is detected by examining the eye behind a slit lamp. In addition, ultrasonic pachymetry is used to assess the thickness of the cornea and, therefore, the severity of its edema. A clear image of the endothelium and the ability to determine the density of cells per unit area, their average size is given by endothelial microscopy.

Treatment

Medicines cannot cure this disease. Solutions with high osmolarity, i.e. the ability to attract water, help dehydrate the cornea and temporarily improve vision. Corneal transplantation, with a successful outcome, can give a good result. But due to the relatively high risk of complications associated with the operation, it is usually prescribed for visual acuity less than 0.1.

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