What is keratoconus?

Keratoconus is a disease of the cornea which is characterized by a weakened tissue. As a result of this weaking the cornea is getting thinner and deforms to an irregular shape which causes loss of vision.

Normal Cornea

optics of the normal cornea.
Farbliche Darstellung der Topographie einer normalen Hornhaut
topography of the normal cornea.

In a healthy eye the cornea has a more or less single curvature over the entire cornea. Objects can therefore be focused in one single focal point to create a sharp image. The pictures on the left show this relation. The cornea is illustrated by the regularly curved line. All light rays converge to a focal point. The second picture shows the related topography of a normal cornea. The homogeneous curvature over the entire corneal surface  is colour coded and represented by more or less a single color over the entire corneal surface.

In a normal eye of zero dioptres the focal point is located on the retina (similar to a film inside a camera) and the eye sees precisely without further optical aids. In “normal” eyes suffering from myopia (shortsightedness) and hyperopia (farsightedness) the eye is either "too long" or "too short". In these cases the focal point is still present but is located either in front or behind the retina causing a blurred image without optical aids. Consequently, glasses are necessary to move the focal point on the retina in order to allow for a sharp imaging of the object. This is not the case in keratoconus where the irregularily shaped cornea does not allow to create a single focal point.

Keratoconus cornea

Verlust der scharfen Abbildung und der Sehschärfe bei Keratokonus
keratoconic cornea
Topographie des Keratokonus farblich dargestellt
topography in keratoconus

The situation is totally different in eyes suffering from keratoconus. The cornea is irregularly shaped to an extent that the incomming light-rays cannot be focused anymore to one single focal point. Consequently, a sharp imaging of objects is not possible in this eye anymore. As there is no proper focal point, even glasses fail to create a sharp image at the retina. At a certain stage of the disease the vision is always blurred, with or without glasses.

The illustration on the left shows how the keratoconic cornea with the irregular curvature looks like. The irregular bulging causes a loss of the focal point. A sharp imaging is not possible anymore. The right picture shows a typical topography of keratoconus. In this illustration the irregular and inhomogeneous curvature is colour coded as well. The steeper areas range from red to purple, whereas the flatter ones are green.

Ultrastructure and Biomechanics

Regelmäßige Anordnung der Kollagen Fibrillen in der nornalen Hornhaut
Collagen arrangement in a normal cornea
Verlust der regelmäßigen Anordnung des Kollagens in der Hornhaut bei Keratokonus
collagen arrangement in keratoconus

The irregular corneal structure explained above is created by the following process: On an ultrastructural level the system of collagen fibrils which comprises the static elements of the cornea is destroyed (Daxer A and Fratzl P. Collagen fibril orientation in the human corneal stroma and its implications in keratoconus. Invest Ophthalmol Vis Sci 1997; 38:121-129). The left image shows the orthogonally regular arrangement of the collagen fibrils inside a normal cornea. In keratoconus collagen fibrils are degraded and dissolved and the regular arrangement of the lamellar structure which is the basis of the intact corneal biomechanics is destroyed (illustration on the right). Consequently, a thinning of the cornea takes places. As a result the elastic tensions within the cornea increase which, in addition, might add to the progression of the degenerative process.

Occurrence, Frequency, Symptoms and Diagnosis

Keratoconus is a mainly genetically caused disease. In Europe and North America, the disease seems to appear sporadic and approximately one out of 1000-2000 inhabitants suffers from keratoconus. However, there are also so-called endemic regions where the disease is diagnosed more frequently. One such endemic region could, for example, be a mountain valley which was more or less isolated from the rest of the region for generations. The same applies to islands and other isolated areas of settlement where, based on a decrease of the gene pool, the probability of genetically caused diseases increases accordingly. Furthermore, there are cultural factors which require consideration. In some regions, such as the Middle East where, traditionally, weddings among relatives are more common than in Europe and North America, keratoconus is diagnosed more frequently. Thus, there are regions in the Middle East where up to 10% of the local population suffer from keratoconus. In addition to a genetic origin other factors like eye-rubbing are accused to be causative.

In Europe, keratoconus mostly occurs around 20 years of age (between 16 and 25 years of age). However, also more aggressive variants of the disease which affect children under the age of 15 even exist.

Keratoconus is a progressive disease. This means that the disease is characterized by a continuous decrease of visual acuity. The probability of progression is depending on age. Usually, the following rule applies: the older the patient, the less likely a progression of the disease. However, there are exceptions as well. There are, for example, patients aged 40 who, even after crosslinking, still suffer from a progression of the disease. A regular medical examination performed by an ophthalmologist is, thus, absolutely necessary.

Given its genetic origin, the disease always affects both eyes. However, it is possible that, clinically, it only shows on one side. The eye which does not show the effects of keratoconus, is referred to as “forme fruste”

Initially, patients often observe a rather frequent change of the power of their glasses and double contours. Usually, keratoconus is diagnosed by the atypical topographic pattern.