The cornea is the clear window on the front of the eye that focuses light and allows a person to see. The innermost layer of the cornea is called the endothelium. The endothelium is made up of a layer of endothelial cells that pump fluid out of the cornea, thereby keeping it clear. Fuchs’ endothelial dystrophy is a condition which affects the endothelium.
In the earliest stages, there is deposition of abnormal material on the endothelium (called ‘guttae’). This can cause reduced vision and glare as it interferes with the clarity of the cornea. This leads to loss of endothelial cell function and swelling of the cornea. This swelling causes reduced vision. In the most advanced cases, the cornea becomes so swollen that blisters (bullae) can form on the surface (epithelial layer) of the cornea leading to irritation and pain [1,2]. It is named after the ophthalmologist who first described the condition, Ernst Fuchs.
The following sections will briefly outline why Fuch’s endothelial dystrophy develops, its signs and symptoms, and some insights into its management and treatment.
Fuchs’ endothelial dystrophy is a very common condition, affecting between 3.7 to 11% of the population. It is more common in females than males . It is one of the most common reasons for corneal transplantation worldwide.
Fuchs’ endothelial dystrophy is often inherited. Different genetic abnormalities have been implicated with Fuchs’ endothelial dystrophy, but an exact gene has not been identified. Other factors that may influence the onset of the disease are environmental factors such as smoking, ultraviolet light exposure and diabetes.
Most people with Fuchs’ endothelial dystrophy develop symptoms when they reach their 40s or 50s. However, there is a group of people who develop symptoms in their 20s or earlier.
Late stages (when bullae, or blisters, develop)
Fuchs’ endothelial dystrophy is diagnosed based on a comprehensive eye examination by an eye health professional. A specialised microscope called a slit lamp is used to examine the cornea in detail. The signs that are seen include:
If you experience any of these signs and symptoms, schedule an appointment with an eye health professional to get your eyes checked. It is also important to note that the development of eye conditions may even start before symptoms appear, which makes going for regular and timely eye checks that much more essential.
Fuchs’ endothelial dystrophy gradually worsens with age but often only very slowly.
Fuchs’ endothelial dystrophy is diagnosed by an eye health professional based on routine questions and a comprehensive eye exam. Using a slit lamp, the eye health professional can look for the changes typical of Fuchs’ endothelial dystrophy (e.g. guttae with, or without corneal swelling).
To help detect early stages of corneal swelling, different machines can be used to measure the thickness of the cornea. This can be useful to monitor the condition over time but is not essential.
A different type of microscope (specular microscope) with high magnification can also be used to take an image of the endothelial cell layer. This allows direct imaging of the guttae, and of the number, size and shape of the endothelial cells (Figure 4).
There are non-surgical and surgical treatment options that can be offered.
Non-surgical treatment will only improve the symptoms in the short term, but do not reverse the underlying problem. Using a hairdryer to dry the cornea in the morning can be helpful to improve vision. This needs to be repeated every morning since the cornea will become swollen again overnight. Another option is the use of special eye drops containing a high concentration of salt, which also helps to dry the cornea.
There are ongoing studies with eye drops that may improve endothelial cell function. These eye drops, however, are not yet approved for Fuchs’ endothelial dystrophy and are still under investigation .
The main treatment for Fuchs’ endothelial dystrophy is corneal transplantation surgery to replace the abnormal endothelial layer of the cornea with a thin layer from a donor cornea. While there are risks of surgery, such as rejection, failure of the transplant over time and raised eye (intraocular) pressure, the success rate is high.
Newer surgical treatments without the need for a corneal transplant are currently being investigated in clinical studies. Other treatments under early investigation include gene therapy, which may reduce the need for surgery.
There are no preventative measures you can take to stop the condition from worsening. Regular eye check ups are recommended to monitor your vision and eye health. If your necessary, you will be offered treatment by the eye health professional.
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