Corneal dystrophies
Corneal dystrophies are a group of rare eye diseases that involve a build-up of abnormal material in the cornea (the clear, outer layer of the eye).
These deposits can affect your vision in a variety of ways. They can cause blurring of vision, sight loss, pain, light sensitivity and grittiness in the eye.
What causes corneal dystrophies?
The cornea has five layers:
- epithelium � the outer, smooth layer with thousands of tiny nerve endings
- Bowman’s layer � the second thin, transparent, protective collagen layer
- stroma � made up of mainly water and some collagen, this strong, clear and elastic layer accounts for 90% of the cornea’s thickness
- Descemet’s membrane � a thin, strong layer of collagen
- endothelium � the innermost layer that pumps fluid out of the stroma
Corneal dystrophies are caused by a build-up of material in any one or more of these layers. As a result, there are different types of corneal dystrophies, depending on which layer is affected. Broadly speaking, these are grouped into:
- epithelial and subepithelial corneal dystrophies � epithelial basement membrane dystrophy and Meesman’s corneal dystrophy
- Bowman’s layer corneal dystrophies � Reis–Bucklers corneal dystrophy and Thiel–Behnke corneal dystrophy
- stromal corneal dystrophies � granular dystrophy, macular dystrophy and lattice corneal dystrophy
- Descemet’s membrane and endothelial corneal dystrophies � Fuchs� endothelial corneal dystrophy
Corneal dystrophies normally affect both eyes and are progressive disorders, meaning they get worse over time.
Main types of corneal dystrophies
Below are the main types of corneal dystrophies. However, there are other, rarer types of corneal dystrophy that are not listed here.
Epithelial and subepithelial corneal dystrophies
This is the most common epithelial dystrophy and is also known as map-dot-fingerprint dystrophy or Cogan’s dystrophy. It presents with map-like or pebble-like erosions on the corneal surface and causes pain, grittiness and blurred vision, which usually begins around the age of 30.
It is treatable with antibiotic drops, ointments and a soft bandage contact lens.
This is a very rare condition that often does not present any symptoms. However, a feeling of grit in the eyes can be severe enough to cause painful erosions.
It can be diagnosed in a baby under the age of one, though symptoms often do not start until the early 20s, or even middle age.
Bowman’s layer corneal dystrophies
The symptoms of this condition usually begin in childhood, as it is typically inherited from a parent.
Similarly to epithelial basement membrane dystrophy, people with this condition experience pain caused by map-like erosions. Additionally, the surface of the cornea becomes irregular. Other symptoms include a sensitivity to light and a gritty feeling.
If damage to the cornea worsens, a corneal transplant may be recommended.
The symptoms of this condition may present similarly to those of Reis-Buckler's corneal dystrophy, beginning in childhood or during the teen years. However, they tend to be milder and only impair vision later in life, with erosions beginning during teenage or early adult years.
Stromal corneal dystrophies
Corneal erosions are likely to be seen by the age of ten years, and signs of dot-like deposits will appear in the stroma of the cornea before the age of 20. However, these do not start to affect vision until the person with the condition is in their 50s.
This type of corneal dystrophy can be treated by a corneal transplant, but the deposits may recur within five years.
This type presents with cloudy areas and grainy, ground glass-like deposits appearing in both corneas, typically in the teenage years or early 20s.
As a result, a corneal transplant is often needed before the age of 30. The deposits can recur, but this takes around 20 years on average, much longer than in granular dystrophy. Typical symptoms include a loss of visual acuity and a sensitivity to light.
This is another corneal disease that occurs in relatively young people, though variants of the condition can occur in people in their 40s with similar symptoms, including sight loss, a gritty feeling and pain. Lattice corneal dystrophy presents with branching deposits that form a lattice structure.
A corneal graft may be offered if vision loss is significant, but the donor cornea may become affected within 5�10 years. Other treatments aim to reduce the pain.
Descemet’s membrane and endothelial corneal dystrophies
Of the corneal dystrophies that affect the deeper layers of the cornea, this is the most common. This condition tends to occur more often in women than men, but its genetics are complex.
In someone who is affected, the endothelial cells deteriorate, causing the cornea to become swollen and lose its transparency. Fuchs� endothelial corneal dystrophy presents causes reduced visual acuity, blurriness, pain and sensitivity to light.