Fuchs’ endothelial dystrophy is a progressive disease resulting in clouding of the cornea and eventual reduction in vision. It affects the corneal endothelial cells which are a specialised single layer of cells on the back surface of the cornea that pump water out of it. When healthy, this keeps the cornea thin and clear.
Endothelial cells do not have the ability to regenerate and hence if they are damaged the cornea gradually begins to swell with water, making it hazy and leading to loss of vision.
A healthy cornea is clear and bright, however, in advanced Fuchs’ dystrophy, the cornea can become hazy and dull due to swelling which affects your quality of vision. Typical symptoms include:
Whilst early signs of Fuchs’ dystrophy can be detected by an eye surgeon even in patients in their thirties, it is relatively uncommon for patients to become symptomatic and require surgery until they are in their fifties.
Some reduction in endothelial cells is normal and happens because of ageing. A baby is born with roughly 4000 endothelial cells per square millimetre, whilst an 80-year-old without Fuchs’ dystrophy has an endothelial cell count usually between 1500-2500 per square millimetre. The cornea remains clear even up to around 800 cells per square millimetre. However, in a patient with Fuchs’ there is an accelerated rate of cell loss, such that a 50 years old may have less than 500 cells per square millimetre.
In addition, endothelial cells in Fuchs’ patients are abnormal and can accumulate abnormal proteins resulting in small bumps on the back of the cornea called guttata.
Fuchs’ dystrophy occurs as a result of abnormal genes that affect the function of endothelial cells, not all of these are well understood yet. Whilst Fuchs’ can be expressed by different generations of the same family, it can also commonly occur sporadically where there is no previous family history. Inheritance of the Fuchs’ gene is also variable and the fact that you have Fuchs’ doesn’t always mean that your offspring will express Fuchs’ endothelial dystrophy.
During the early stage of Fuchs dystrophy when swelling of the cornea is still mild, your doctor may prescribe you sodium chloride (salt) drops or ointment. By increasing the salt content of the tears water is drawn out of the cornea by osmosis.
Salt drops do not cure Fuchs’ Dystrophy and simply delay the need for surgery in early cases.
The surgical treatment is endothelial transplantation which involves the transplantation of healthy endothelial cells to replace the absent or diseased cells and may be combined with cataract surgery. The gold standard of corneal transplantation for Fuchs Dystrophy is an operation called endothelial keratoplasty (DSAEK or DMEK surgery).
It is no longer considered modern practice to perform full thickness corneal transplantation (PK) for Fuchs’ dystrophy.
Routine cataract surgery in patients with Fuchs’ dystrophy can accelerate corneal failure unless particular care and an alteration to the standard technique of surgery is made to minimise corneal endothelial cell damage (soft shell technique). Ideally your surgery should be undertaken by a corneal surgeon that is familiar with the management of patients with Fuchs’ dystrophy. Some patients may elect to have femtosecond laser cataract surgery which has the advantage of reducing energy levels used during cataract removal and results in less intra-operative endothelial cell damage. In moderate to advanced cases of Fuchs’ dystrophy your surgeon may decide to perform cataract surgery and endothelial corneal transplantation at the same time.
Endothelial cell numbers can be assessed in the clinic pre-operatively by specular microscopy and will help guide the decision whether to perform cataract surgery alone or combined with DMEK/DSAEK.
Together our surgeons and their teams carry out over 5% of all corneal transplant surgery in England making Ophthalmic Consultants of London the most highly specialised surgical corneal unit in Central London. They have cared for well over one thousand corneal transplant patients.
DSAEK and DMEK surgery is a highly specialised and technically demanding surgical technique. Even in Europe, only 1 out of 4 corneal surgeons are able to perform this technique. In the recent past, patients with Fuchs’ Dystrophy would need to have a full thickness corneal transplant (penetrating keratoplasty) leading to a long recovery period.
Our surgeons are experts in DSAEK and DMEK surgery and are Consultant Eye Surgeons at teaching hospitals in London and the surrounding area. They have completed fellowships at Moorfields Eye Hospital and international centres of excellence. Our surgeons are also actively engaged in research that has led to significant improvement in the DSAEK surgical technique and they were awarded the ‘Best Paper’ award by the American Academy of Ophthalmology and the ‘Founders Gold Medal’ for the presentation and publication of this work.
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