Information
Editors
Corneal Degeneration
Essentials
- Corneal degenerations (dystrophies) are progressive disorders of both eyes.
- They are divided into superficial, stromal and posterior types.
- They may cause few symptoms or various degrees of impaired vision.
- Exacerbations may involve acute eye pain.
- For conical degeneration of the cornea (keratoconus), see separate article Keratoconus (Conical Degeneration of the Cornea).
Prevalence
- Superficial degeneration is in most cases detected before the age of 20 years. It is not associated with other eye disorders or with systemic diseases.
- Posterior dystrophy is quite common. The patients are often older than 40.
Symptoms and findings
- Depending on the form of disease, the following may be seen:
- map- or fingerprint-like or, sometimes, blister-like lesions of the superficial cornea
- reduced visual acuity, increased astigmatism
- painful corneal erosions
- recurrent epithelial erosions, particularly in the morning when opening the eyes
- accumulations of some material in various corneal layers.
- In some forms of the disease, visual acuity can remain good for a long time and the eye can be asymptomatic.
Workup
- Visual acuity
- Corneal tomography and biomicroscopy
- Gene tests are recommended for the differential diagnosis of certain types of degeneration.
- In stromal degeneration, they are recommended for testing the TGFBI gene defects.
- If the Meretoja syndrome (Finnish-type familial amyloidosis http://www.orpha.net/en/disease/detail/85448) is suspected, the gelsolin gene c.640G>A gene defect should be tested for.
Treatment
- An ophthalmologist will perform a more detailed diagnosis and classification of corneal degeneration.
- Treatment varies depending on the type of disease.
- In superficial degeneration, phototherapeutic keratectomy (PTK, laser treatment of the superficial corneal tissue) may be considered.
- In stromal degeneration, moisturising drops are useful.
- Partial thickness or penetrating corneal transplantation is considered in advanced disease.
References
- Seppänen M, Kaarniranta K, Setälä N, Uusitalo H (eds.). [Handbook of Ophthalmology]. 3rd revised edition. Duodecim Publishing Company 2022. Available in Finnish.