The disease usually affects the central cornea of the eye. It usually causes the cornea to deform into a cone shape (like a cone) and become thinner.
Keratoconus usually affects both eyes. However, it only very rarely occurs simultaneously and to a similar extent in both eyes. If the disease is detected in time, it can be counteracted with early treatment. The aim is then to prevent the corneal deformation and thinning from progressing too drastically.
Keratoconus is a cone-shaped deformation of the cornea
Those affected usually suffer from short-sightedness, which becomes increasingly severe over time as the deformation becomes more severe. However, the form of short-sightedness can usually not be corrected completely and only with a visual aid (such as glasses). The reason for this is the cone-shaped protrusion of the cornea.
Keratoconus occurs relatively rarely. In Germany alone, "only" around 0.5 percent of the population is affected. However, the percentage varies depending on the region and the different examination methods.
In addition, twice as many men are affected by the disease as women. Keratoconus usually occurs between the ages of 20 and 30. In rare cases, however, it can also occur in childhood or later, for example between the ages of 40 and 50.
Despite numerous studies, it is still not fully known what causes or promotes keratoconus. The deformation often occurs in connection with hereditary diseases such as Down's syndrome.
It also occurs more frequently in people who suffer from
suffer from dry eyes.
The structure of the human eye © bilderzwerg | AdobeStock
According to various assumptions, a metabolic disorder is the trigger for those affected. However, a hereditary disease could also be considered.
In addition, a deviation in the chemical composition of the cornea has been observed in keratoconus in various long-term studies. However, individual observations have shown that the natural curvature of the sclera in one eye often deviates slightly from the norm.
An infection or a weakening of the collagen present in the cornea could also promote keratoconus. However, these assumptions have not yet been substantiated by further studies.
In rare cases, the cornea can tear in the eye during the course of the disease. Fluid from the anterior chamber of the eye can then enter the cornea. The result is acute keratoconus, which can be painful and often needs to be treated directly (e.g. by surgery). Otherwise, vision deteriorates quickly and drastically (foggy vision).
Acute keratoconus is the only case of the disease in which the eye can cause pain and noticeable discomfort. The pure deformation and thinning of the eye is only manifested by the loss of vision.
In the early stages of the disease, the patient's visual impairment only worsens slowly. He sees objects twice or perceives shadows on objects. Star-shaped rays appear to emanate from light sources and streaks from letters.
With sufficient lighting, semi-circular or circular green-brown to yellow-brown lines appear, which doctors call
- Keratoconus lines,
- Fleischer's rings or
- Kayser-Fleischer corneal ring
called.
Other symptoms are
- impaired night and twilight vision
- permanently reddened eyes
- increased sensitivity to light
- severely tense, overtired facial muscles
- Contact lenses often slip or fall out
Left: the logo with full vision, right: stage 4 keratoconus
Keratoconus is usually diagnosed during a routine examination by an ophthalmologist or as soon as myopia becomes noticeable. Therefore, the first signs of keratoconus are frequent spectacle corrections with drastic changes in the axis. Visual acuity also changes considerably.
Added to this is the increasing curvature of the cornea, which initially often only affects the eye. As keratoconus is a relatively rare disease, the symptoms are usually not recognized at the beginning. Many ophthalmologists are only aware of keratoconus in theory. In case of uncertainty, patients who fear they may be suffering from keratoconus should consult another ophthalmologist.
Keratoconus can be diagnosed using various diagnostic procedures. The best known are
- a close examination of the cornea with a skiascope (the typical "fish mouth effect" is present in keratoconus),
- a slit lamp (to determine the thickness and number of corneal layers),
- a keratograph (to examine the surface structure of the cornea) and
- an optical coherence tomography (OCT).
The latter produces a cross-sectional image of the anterior segment of the eye. Among other things, this includes the thickness of the cornea and the detailed course of the surface. This allows the typical deformation and thinning to be recognized.
Today, doctors can treat keratoconus in two different ways. The aim is to eliminate the visual defect caused by the keratoconus.
Visual aids to compensate for defective vision
In the early stages of keratoconus, the symptoms are still mild. Then the deformation and thinning of the cornea can still be compensated for with glasses. In advanced keratoconus, however, the changes to the cornea are usually too drastic.
In this case, however, the defective vision must or can usually be compensated for with dimensionally stable contact lenses or special lenses( calledkeratoconus lenses ).
Around 80 percent of patients suffering from keratoconus are able to cope permanently with hard contact lenses. In some cases, however, these need to be combined with an additional visual aid (such as glasses). Only then can the defective vision in the eye be largely corrected.
The protrusion of the cornea (keratoconus) is clearly visible here © Zarina Lukash | AdobeStock
Cross-linking of the cornea
On the other hand, timely treatment should prevent further progression of the disease. In the best case, the further deformation and thinning of the cornea is largely stopped.
This is possible, for example, with a cross-linking treatment (also known as cross-linking or collagen cross-linking). Cross-linking prevents the progression of keratoconus, but is only recommended by experts in stages I and II. Cross-linking the cornea stiffens the soft cornea in the corneal tissue.
To date, however, cross-linking is the only treatment method that can halt the progression of keratoconus for several years.
Various studies assume that keratoconus can be stopped permanently if the cornea is cross-linked in good time. However, there is currently a lack of conclusive long-term study data.
Circular keratotomy
Circular keratotomy (CKT) is another procedure used to try and stop keratoconus to a large extent. A 7 mm wide incision is made around the optical axis of the cornea. The incision is then closed with a double suture.
In this way, the cornea is tightened and the cone is evened out. However, the cornea must be at least 400 µm thick and stable enough in the area of the incision. In addition, CKT is also only suitable for stage I and II keratoconus.
The actual effect of CKT has not yet been proven.
Corneal transplantation
If visual aids cannot sufficiently correct keratoconus, the cornea should generally be replaced by a transplant(keratoplasty). The transplant is performed in an eye clinic specializing in transplants. H
n rare cases, the affected person's cornea is stabilized using various methods, but more often it is replaced by a donor cornea.
Often the cornea is only punched out. Only a minimal edge is created, the cut-out piece of cornea is replaced by the healthy donor tissue and sutured with a fine double suture.
The costs for this are usually covered by health insurance. The prerequisite for this is that vision is impaired to such an extent that it has a serious impact on the patient's quality of life.
Whether treated or untreated, keratoconus can have an enormous impact on everyday life. The visual acuity in one eye often fluctuates immensely. This can only be counteracted to a limited extent and with various visual aids.
In any case, keratoconus has an impact on the concentration processes of the person affected due to the constantly fluctuating visual values. This can result in impaired vision, thinking and concentration as well as headaches and migraine-like pain attacks.
Another problem is the wearing comfort of the lenses, which are not necessarily comfortable in the eye and can lead to
- increased lacrimation,
- irritation and
- redness of the eyes
irritation and redness of the eyes. In many cases, keratoconus can also have a significant impact on driving. However, the treating ophthalmologist must clarify whether this applies in each individual case.
Poor prognosis
A further burden for those affected by this disease is the lack of prognosis regarding the course of the disease. Neither the exact causes for the development of keratoconus nor the influence of external factors are precisely known.
It is therefore difficult to say
- how quickly and how drastically keratoconus will progress,
- which treatment is the right one and
- how the affected person should behave.
It is often not possible to answer seemingly simple questions such as whether sport is permitted and whether rubbing the eye is harmful.
In some patients, for example, a corneal transplant is necessary after just a few weeks or months. Other patients, however, manage for decades or for the rest of their lives with glasses or suitable keratoconus lenses.
The only prognosis that applies to most sufferers is that keratoconus usually comes to an actual - and not treatment-induced - standstill between the ages of 40 and 50.
Lack of financial help
Contact lenses and frequently changing spectacles, on the other hand, are an everyday and financial burden. Both types of visual aids need to be replaced and adjusted to the new vision more frequently than average. Often, several pairs of contact lenses or glasses are necessary to compensate for constant fluctuations in visual acuity.
The costs for the necessary visual aids, especially for suitable contact lenses, are quite high. They are only partially or not at all covered by statutory health insurance. Most supplementary insurance policies, on the other hand, no longer cover keratoconus if it was diagnosed before the policy commenced.
Those affected are therefore often completely on their own with the immense costs of treatment.
According to all current studies and findings, it is currently not possible to prevent this disease.
However, it is generally considered sensible to avoid anything that could strain the eye and cornea. A very common and typical example is eye rubbing. This is often related to frequent work at a computer screen and the associated dryness of the eyes. Doctors therefore advise those affected in particular not to work at a PC.
People should also avoid spending time in dusty and smoky rooms and in rooms with air conditioning.
Anyone affected by keratoconus should definitely drink plenty of fluids and spend a lot of time in the fresh air. On the other hand, some experts are skeptical about wearing contact lenses all the time. Many studies claim - but so far unproven - that this could also put a strain on the cornea in the long term. However, opinions are still divided here.