Fuchs' Dystrophy

Fuchs dystrophy is an inherited eye condition that causes your vision to become cloudy. The cornea is the clear layer that covers the iris and is made up of layers of cells. The endothelium layer, which is the inner most layer, acts like a pump, it removes excess fluid out of the cornea, thus ensuring the cornea stays clear. The endothelium’s main job is to control the amount of fluid and nutrients that moves in & out of the cornea. As we age we lose endothelial cells, with Fuchs dystrophy this process is sped up. Many endothelium cells are lost and can no longer ‘pump out’ excess fluid. A build up of fluid accumulates in the cornea, this is known as oedema. The oedema will cause clouding of the vision.  Fuchs dystrophy eyes can also develop guttata; collagen deposits which occur due to the stressed endothelium cells. The guttata causes the light entering your eyes to scatter, this causes glare. Fuchs dystrophy is slow developing eye condition and it may take a long time before the eyes start to experience any visual problems.

• Misty or blurry vision especially on waking

• Blurry vision gets better throughout the day (early stages)

• Glare in bright conditions

• Halos around lights

• Poorer contrast sensitivity (more difficulty in seeing detail, when text and background are similar colours)

• Later stages; blurry vision all day due to significant loss of endothelium cells

• Later stages: gritty eyes

• Later stages: pain in the eyes

• Later stages: sharp pain from bursting blisters (bullous keratopathy) on the front of the cornea caused by the oedema buildup

Fuchs dystrophy is inherited and it is a dominant genetic condition. If you suffer from Fuchs dystrophy it is very likely that 50% of your children will also get Fuchs dystrophy.

Treatment is only required when a patient’s eyes start to experience problems. Many patients that have Fuchs dystrophy do not experience any visual problems with their eyes and they do not require treatment.

• If you are experiencing blurring of the vision on waking only, saline formulated eye drops, gels or ointments can be used.  The saline-based topical treatment helps draw excess fluid out of the cornea, which helps clear the vision by reducing the clouding caused by the oedema.

• Good quality polarised sunglasses can help against glare in bright conditions

• Gritty eyes can be lubricated with an eye gel to aid comfort

• If a patient gets bullous keratopathy (blisters on the cornea), soft contact lenses can be used. The soft contact lenses act as a bandage and protect the corneal nerve endings from exposure. Oral painkillers can also be used to help relieve pain.

Corneal transplants are advised for patients that are experiencing significant visual problems and discomfort to their eyes caused by Fuchs dystrophy. The corneal transplant you may require is dependent on which layer the Fuchs dystrophy has affected.

A corneal transplant replaces your endothelial layer (innermost layer of the cornea) with a donor endothelial layer; this is called endothelial keratoplasty (EK).

• Descemet’s stripping (automated) endothelial keratoplasty transplant (DSEK or DSAEK). With this corneal transplant, the donor’s, endothelium, descemet’s membrane and some of the stoma are used to replace yours. The descemet’s and stroma are other layers in the cornea.

• Descemet's membrane endothelial keratoplasty (DMEK) is a newer type of procedure where the donor’s endothelium and descemet’s membrane are only used. This gives the patient a better recovery rate and rejection of the donor tissue is lower.

Penetrating keratoplasty (PK) corneal transplant is used if the patient has stromal (central layer of the cornea, above the endothelium & descemet’s membrane) scarring. This is where the whole of the patient’s cornea is replaced with a donor cornea.