Carl Gustav Carus
   

Department of Ophthalmology
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Keratoconus

What is keratoconus?

Keratoconus is a congenital disease of the cornea (autosomal dominant or auosomal recessive) and it belongs to the large group of corneal dystrophies (hereditary determined pathological changes of the cornea). The disease is characterised by a thinning and conuslike protrusion of the cornea due to alterations in the corneal structure. At first, the protrusion occurs in the inferior parts but later it affects also the central part of the cornea.
Keratoconus mostly happens to be bilateral but often the progression is asymmetrical. First symptoms generally start during puberty or early adolescence. In most cases patients realise a decrease in vision, or an increasing myopia with a significant progression of astigmatism can be found. The incidence of keratoconus in the general population is about 1 in 2000.


(Fig. left: conuslike protrusion of the cornea; right: Munsons's sing: a conuslike shape of the inferior lid that can be seen when the patient looks down.)

How does your doctor diagnose / recognise a keratoconus?

When examined under / with mangnification (so called slit lamp examination) the disease shows specific and typical changes. There is an increase in iron precipitations coming from the tear film (Fleischer's ring). Further advanced cases show a visible thinning of the inferior third of the cornea and so called Voigt's striae. Due to an increased content of water there can be opacifications of the corneal stroma which are associated with a significant drop in visual acuity.
In far progressed / advanced cases one can even see the conuslike protrusion of the cornea with the naked eye. (see above)


(Figure: stromal opacification of the cornea, Voigt's striae)


(Figure: circular iron precipitations, so called Kayser-Fleischer's ring)

Other equipment such as corneal topography (visualisation of the corneal surface and measurement of curvature and refractive power of different parts of the cornea) can be used to diagnose early cases and, during follow up, can help to measure / estimate a possible progression.

 
(Figure left: corneal topographyinstrument; right: Placido circles of the topography machine)

What is the prognosis of this disease?

It has to be said that the prognosis can vary a lot. At all times the progression of the disease can stop spontaneously. And if there is a progression the speed of a possible worsening is different in each patient. Everything is possible from quick progression already in early adolescence to a slow development which up to a high age doesn't lead to any significant symptoms.

 
(Figure: Reflection picture during corneal topography (so called Placido rings). Left: beginning keratoconus; right: keratoconus)

 
(Figure: corneal topography Left: beginning keratoconus; right: keratoconus)

What are the treatment options / possible treatments?

There is no cure in the classic sense since the disease is some kind of "stuck in our gens". However, as mentioned above, a spontaneous stop of progression is possible at any time. In progressing / advancing cases rigid contact lenses can improve visual acuity, yet they can not stop the process.
Even a few years ago, the only therapeutic option for advanced cases with significant reduction of visual acuity was the transfer of corneal tissue (penetrating keratoplasty) to achieve a better vision.
Recently a new procedure has been developed at our hospital which is supposed to stop the progression of keratoconus. This procedure is called Cornea Cross Linking and is performed since 1999 as part of a clinical trial / study (approved by the ethic committee). It usually is performed as day surgery.

What is Cross Linking?

The aim of the treatment is to stabilise the cornea. The main part of the corneal tissue (stroma) is built from single collagen fibres which are linked /connected. With this specific treatment of the corneal stroma (removing of the superior protective layer, application of the photosensitizer Riboflavin (vitamin B2) and 30 min radiation of the corneal tissue with UVA light) further links between the collagen fibers can be induced, so called cross links. Therefore the single fibres form a "denser network" which leads to an increase in the overall stability of the cornea.
After in the meantime more than 3 ½ years, our results show that none of the treated patients showed any more progression of keratoconus within that time.
Due to the newness of the procedure there are no long term (10 or 15 year) results yet. At this moment in time, however, there are no clues for possible late complications or unwanted side effects. Therefore we are optimistic that - as long as a keratoconus is diagnosed early enough - a stabilisation of clinical findings and visual acuity can be achieved without a corneal transplant.

Collagen Cross Linking as treatment option

Keratoconus is a mostly bilateral degeneration which leads to a conuslike protrusion of the cornea. In the early 19th century it was named by Mr. Ammon. The disease is characterised by a progressive thinning of the cornea, Voigt's striae (vertical lines) and ruptures of Descemet- as well as Bowman membrane. Roughly 21% of all patients will eventually need a cornea transplant. The cause of the disease is still unknown - according to M. Kohlhaas (Dresden). Enzyme variations in the epithelium with an increased expression of lysosomal and proteolytic enzyms and changes in the stromal matrix (imbalance between keratan- and dermatansulfat) are discussed.
The incidence of keratoconus is about 1/2000. The ectasia generally starts during puberty. A genetic predisposition is very likely. The amount of family run diseases varies in literature between 5% and 20%. The hereditary pattern can be autosomal dominant as well as autosomal recessive. Various ocular and non-ocular disorders can be combined with keratoconus, i.e. atopic dermatitis and keratitis, keratoconjunctivitis vernalis (up to 35%), Down Syndrome (with an incidence of 5-15%) and Ehlers-Danlos-Syndrome.
Hints for a keratoconus: frequent changes of refraction in patients history (increase in myopia and astigmatism), distorted light reflex during sciascopy and the slit lamp findings of an inferiorly located apex of the cone and a beginning irregular astigmatism.
Treatment options include glasses, rotation symmetrical or not rotation symmetrical contact lenses, epikeratophaky (no used anymore), lamellar and penetrating keratoplasty, so called intacs (intracorneal ring segments) and collagen cross linking with Riboflavin and UVA light. The treatment of keratoconus with collagen cross linking is based on a significant stiffening of the corneal stroma due to photochemical cross linking of the collagen fibres. This procedure is performed under topical anaesthesia. After an epithelium abrasio, Riboflavin (vitamin B) is applied to the corneal surface and it is exposed to 365nm UVA light for 30min. During the follow up of the 48 patients (60 eyes) so far treated with collagen cross linking none of the patients showed any more progression (follow up time 1-38 month). In 81.7% of the patients a regression of the maximal K-values of -2.87 +/-2.56 dpt (0.18 to 9.97 dpt) could be seen. Post surgical visual acuity rose by 1.4 +/- 2.04 lines of Snellen. No unwanted side effects such as opacification of the lens or loss of endothelial calls could be found. Only during the first 2 to 3 months after the cross linking a slight superficial corneal haze occurred. Generally this minor haze disappears without any treatment, but, if wanted, a supportive therapy with soothing ointment or with a local steroid (i.e. Efflumidex or Vexol) can be used.
Collagen cross linking which only stiffens the anterior 200-250µm of the cornea modifies to biomechanical characteristics of the cornea. Deeper layers are not damaged. This procedure stopps the progression of the keratectasia. Therefore the number of penetrating keratoplasties could be significantly reduced in the future. The treatment is relatively easy and low in costs.

Conclusion: Keratoconus patients have to be checked / examined every 6-12 month. If the findings are stable a treatment with glasses or contact lenses is sufficient. If there is a progression an early cross linking treatment is recommended / indicated.

Talks about the treatment of keratoconus (Prof. Kohlhaas):

Dear homepage visitor,

If you are looking for more information about keratoconus feel free to have a look at our slide show. There we demonstrate again the characteristics of keratoconus as well as explain the various diagnostics and treatment options.

Information for english speaking visitors about the treatment of keratoconus and keratectasia after Lasik with Riboflavin/UVA-light
(Collagen cross-linking, keratoconus, keratectasia, Lasik, Riboflavin)

Links

Publications / Literature:

  1. Spörl, E.; Huhle, M.; Kasper, M.; Seiler, T.:
    Erhöhung der Festigkeit der Hornhaut durch Vernetzung.
    Erschienen 1997 in: Der Ophthalmologe 94 - Seiten: 902-906

  2. Spoerl, E.; Huhle, M.; Seiler, T.:
    Induction of cross-links in corneal tissue.
    Erschienen 1998 in: Exp. Eye Res. 66 - Seiten: 97-103

  3. Spoerl, E.; Seiler, T.:
    Techniques for stiffening the cornea.
    Erschienen 2000 in: J. Refract. Surg. 15 - Seiten: 711-713

  4. Spörl, E.; Schreiber, J.; Hellmund, K.; Seiler, T.; Knuschke, P.:
    Untersuchungen zur Verfestigung der Hornhaut am Kaninchen.
    Erschienen 2000 in: Der Ophthalmologe 97 - Seiten: 203-206

  5. Seiler, T.; Huhle, S.; Spörl, E.; Kunath, H.:
    Manifest diabetes and keratoconus - a retrospective case-control study.
    Erschienen 2000 in: Graefe's Arch. Clin. Exp. Ophthalmol. 238 - Seiten: 822-825

  6. Schnitzler, E.; Spörl, E.; Seiler, T.:
    Bestrahlung der Hornhaut mit UV-Licht und Riboflavingabe als neuer Behandlungsversuch bei einschmelzenden Hornhautprozessen.
    Erschienen 2000 in: Klin. Monatsbl. Augenheilkd. 217 - Seiten: 190-193

  7. Wollensak, G.; Spörl, E.; Seiler, T.:
    Riboflavin/ultraviolet-A-induced collagen crosslinking for the treatment of keratoconus.
    Erschienen 2003 in: Am. J. Ophthalmol 135 - Seiten: 620-627

  8. Wollensak, G.; Spoerl, E.; Seiler, T.:
    Stress-strain measurements of human and porcine cornea after riboflavin/ultravuilet-A-induced crosslinking.
    Erschienen 2003 in: J. Cataract Refract. Surg. 29 - Seiten: 1780-1785

  9. Wollensak, G.; Spoerl, E.; Wilsch, M.; Seiler, T.:
    Endothelial cell damage after riboflavin-ultraviolet-A-treatment in the rabbit.
    Erschienen 2003 in: J. Cataract Refract. Surg. 29 - Seiten: 1786-1790

  10. Wollensak, G.; Spoerl, E.; Wilsch, M.; Seiler, T.:
    Keratocyte apoptosis after corneal collagen-crosslinking using riboflavin-UVA treatment.
    Erschienen 2004 in: Cornea 23 - Seiten: 43-49

  11. Wollensak, G.; Spoerl, E.; Reber, F.; Seiler, T.:
    Keratocyte cytotoxicity of riboflavin/UVA-treatment in vitro.
    Erschienen 2004 in: Eye 18 - Seiten: 718-722

  12. Wollensak, G.; Wilsch, M.; Spoerl, E.; Seiler, T.:
    Collagen fiber diameter in the rabbit cornea after collagen-crosslinking.
    Erschienen 2004 in: Cornea 23 - Seiten: 503-507

  13. Spoerl, E.; Wollensak, G.; Seiler, T:
    Increased resistance of crosslinked cornea against enzymatic digestion.
    Erschienen 2004 in: Curr. Eye Res. 29 - Seiten: 35-40

  14. Sandner, D.; Spörl, E.; Kohlhaas, M.; Unger, G.; Pillunat, L. E.:
    Collagen crosslinking by combined riboflavin/ultraviolet-A (UVA) treatment can stop the progression of keratoconus
    Erschienen 2004 in: ARVO 2004

  15. Spörl, E.; Wollensak, G.; Dittert, D.D.; Seiler, T.:
    Thermomechanical behaviour of collagen-crosslinked porcine cornea.
    Erschienen 2004 in: Ophthalmologica 218 - Seiten: 136-140

  16. Kohlhaas, M.; Schilde, T.; Spoerl, E.; Sandner, D.; Pillunat, L. E.:
    Tiefenabhängigkeit der Verfestigungswirkung von Riboflavin/UVA an der Hornhaut.
    Erschienen in: Band des 18. Kongresses der Deutschsprachigen Gesellschaft für Intraokularlinsen-Implantation und refraktive Chirurgie, Biermann-Verlag, Köln - Seiten: 543-546

  17. Kohlhaas, M.; Sandner, D.; Spoerl, E.; Pillunat, L. E.:
    Behandlung der Keratektasie nach Lasik durch Kollagen-crosslinking.
    Erschienen in: Band des 18. Kongresses der Deutschsprachigen Gesellschaft für Intraokularlinsen-Implantation und refraktive Chirurgie, Biermann-Verlag, Köln - Seiten: 525-529

  18. Kohlhaas, M.:
    Keratokonus: Kollagenvernetzung als Therapieoption.
    Erschienen 2004 in: Z. prakt. Augenheilkd. 25 - Seiten: 449-453