Corneal Dystrophy

What is corneal dystrophy?

Corneal dystrophies are a group of hereditary diseases of the cornea. It is a non-inflammatory disease that usually affects both eyes. In most cases, it manifests itself by a reduction in the transparency of the cornea and a deterioration in vision.

Its peak age is between 10 and 50 years of age. Corneal dystrophies are divided into different forms, each of which affects different areas of the cornea. They are caused by mutations in various genes.

Causes

Corneal dystrophies are hereditary diseases. They are caused by various mutations in different gene sequences. In recent years, intensive medical research has made it possible to localize the gene sequences, which has led to a better understanding and better diagnostic and therapeutic options.

Molecular tests can be used to diagnose the specific form of corneal dystrophy in the patient and to treat it accordingly. Often these diseases are passed on from generation to generation in a family. Corneal dystrophies are non-inflammatory diseases, i.e. they do not arise as a consequence of a previous inflammation or other corneal disease. Nor can they occur as a result of accidents or injuries. Corneal dystrophies are often discovered as a chance finding in an ophthalmological examination of physically healthy people.

What forms are there?

There are many different forms of corneal dystrophy, each affecting different areas of the cornea. They are classified according to their gene mutation and named after their typical appearance. They can be divided into 3 groups according to their localization.

One group mainly affects the superficial layers, such as the epithelium, another the middle stroma layer and the third group the posterior layers of the cornea, such as the endothelium. The most common form is Fuchs endothelial dystrophy. It affects the endothelial cells on the inner side of the cornea and leads to an increasing degradation of the cornea.

It mainly affects women over the age of 50. It leads to deterioration of vision and can be treated with eye drops or, in the worst case, with a corneal transplant. In map-dot fingerprint dystrophy, the basement membrane of the epithelium is affected. This form occurs at a young age and leads to symptoms such as visual deterioration, eye pain and blurred vision. Other forms are: granular corneal dystrophy, lattice corneal dystrophy, honeycomb corneal dystrophy, macular corneal dystrophy, epithelial dystrophy.