What do you need to know?
- Fuchs' dystrophy is a chronic disease of the cornea leading to swelling of the cornea and deterioration of vision, most commonly occurring in middle-aged and older women.
- Początkowo może przebiegać bezobjawowo, ale z czasem powoduje zamglone widzenie, wrażliwość na światło i ból oczu, zwłaszcza rano.
- Treatment includes hypertonic and moisturising drops and, in advanced stages, a corneal transplant - either layered or full-thickness.
- Współistniejące choroby oczu, jak jaskra i zaćma, wymagają zintegrowanego leczenia, czasem łączącego zabiegi chirurgiczne w jednym etapie.
Dystrofia Fuchsa to przewlekła choroba oczu, która dotyka śródbłonka rogówki, czyli wewnętrznej warstwy odpowiedzialnej za utrzymanie jej przejrzystości. Choroba ta powoduje postępujący obrzęk rogówki, co prowadzi do pogorszenia widzenia i utraty ostrości widzenia. Dystrofia rogówki śródbłonka Fuchsa jest często uwarunkowana genetycznie, choć jej rozwój może być przyspieszony przez czynniki środowiskowe i wiek.
The disorder mainly affects middle-aged and elderly people, and women are more likely than men to be affected. In the initial stages, the disease is asymptomatic, but as it progresses it can significantly affect the patient's quality of life.
What is fuchs dystrophy?
Fuchs' dystrophy, also known as Fuchs' endothelial corneal dystrophy, is an eye disease that leads to progressive damage to the corneal endothelium - the layer of cells responsible for draining fluid from the corneal surface. The loss of these cells causes corneal swelling and loss of corneal translucency, resulting in impaired vision.
The disease is genetic and Fuchs dystrophy is more common in women than in men. Diagnosis of Fuchs dystrophy includes a detailed medical history, clinical examination and measurement of corneal thickness to assess the degree of swelling and progression of the disease.
Causes of Fuchs' dystrophy
Dystrofia Fuchsa jest chorobą uwarunkowaną genetycznie, co oznacza, że jej rozwój często wiąże się z dziedziczeniem mutacji w genach odpowiedzialnych za prawidłowe funkcjonowanie śródbłonka rogówki. W wielu przypadkach choroba jest przekazywana w sposób dominujący, co oznacza, że ryzyko jej wystąpienia wzrasta, jeśli w rodzinie były już przypadki corneal dystrophy Fuchsa.1
The cause of the disease is an abnormal structure of the cornea and, more specifically, of the corneal endothelium, which gradually loses its ability to drain fluid from the corneal epithelium. This leads to fluid accumulation and the formation of corneal oedema. Factors such as age, gender (the disease is more common in women) and co-morbidities can further accelerate the development of dystrophy.
In some cases, the intensity of symptoms is exacerbated by intraocular pressure abnormalities. The treatment of Fuchs' dystrophy includes both pharmacological agents to stabilise the corneal epithelium and advanced surgical procedures such as corneal transplantation.2
Stages of Fuchs' dystrophy
Fuchs dystrophy develops at different rates and its progression can be divided into two main stages: early and advanced. In each of these, the symptoms of Fuchs dystrophy vary in severity and impact on the quality of vision.
Early stage
In the early stages of the disease, patients may not notice significant vision problems. During this time, Fuchs' corneal endothelial changes do not yet cause swelling and the corneal epithelium remains relatively stable. However, there may be slight visual acuity disturbances in the morning, which improve during the day.
Advanced stage
W miarę postępu choroby zaczyna dochodzić do obrzęku rogówki, co prowadzi do poważniejszych objawów, takich jak hazy vision, nasilona wrażliwość na światło oraz ból oczu. W tej fazie mogą wystąpić pęcherze nabłonkowe na powierzchni rogówki, które bywają bardzo bolesne i dodatkowo pogarszają ostrość widzenia.
Treatment at this stage often requires the use of special agents such as hypertonic drops based on the saline, which reduce swelling. In some cases, soft contact lenses are required to relieve discomfort caused by epithelial blisters.
Advanced Fuchs' dystrophy may require surgical treatment, including corneal transplantation, which may involve the entire thickness of the cornea or its stratified removal, depending on the severity of symptoms.
Treatment of Fuchs' dystrophy
Treatment of Fuchs dystrophy depends on the stage of the disease and the severity of the symptoms. In the early stages, the focus is on relieving the symptoms of Fuchs dystrophy, such as swelling and hazy vision. In more advanced cases, surgical treatment may be necessary, which significantly improves the patient's comfort.
Conservative treatment
In the first stages of Fuchs' dystrophy, the following are used:
- Hypertonic agents, such as saline drops or ointments that reduce corneal swelling.
- Moisturising drops, helping to improve visual comfort and moisturise the cornea.
- Avoiding exposure to dry air and using humidifiers.
These methods can temporarily stabilise the patient's condition, relieving swelling and improving the quality of vision.
Surgical treatment
In more advanced stages of the disease, when the loss of Fuchs' corneal translucency becomes significant, a corneal transplant may be necessary. There are two main techniques:
- Layered graft (DMEK or DSAEK): Removal of the damaged corneal endothelial layer and replacement with healthy donor tissue.
- Full thickness (PK) graft: Recommended in rare cases where lesions involve all layers of the cornea.
W przypadku współistnienia cataracts i dystrofii Fuchsa, operacja usunięcia zaćmy może być połączona z przeszczepem warstwowym rogówki, aby zminimalizować liczbę interwencji chirurgicznych.
Glaucoma and Fuchs' dystrophy
Although glaucoma and Fuchs' dystrophy are two separate eye diseases, they can occur simultaneously, complicating treatment and diagnosis. Glaucoma causes increased intraocular pressure, which can put additional strain on the already weakened Fuchs cornea. In such cases, the symptoms of Fuchs' dystrophy, such as corneal swelling and hazy vision, may be exacerbated.
Treatment requires an approach that addresses both diseases. Intraocular pressure-lowering drops are used to help control glaucoma and reduce the risk of further corneal damage. In advanced cases, surgery may be required, combining treatment of glaucoma and Fuchs' dystrophy, such as corneal transplant surgery combined with glaucoma surgery.
Cataracts and Fuchs' dystrophy
Cataracts and Fuchs dystrophy often co-occur, especially in older patients. Cataracts cause clouding of the lens of the eye, which further exacerbates the vision already reduced by the corneal oedema characteristic of Fuchs' dystrophy. In such cases, treatment of both conditions requires an integrated approach.
During cataract surgery, if the patient also suffers from Fuchs' dystrophy, a corneal layer transplant (DMEK or DSAEK) can be performed at the same time. Such a procedure can effectively restore corneal clarity and improve vision. The decision for combined treatment depends on the severity of both diseases and the individual needs of the patient.
Ważne pytania i odpowiedzi
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No, not in all cases is a corneal transplant necessary. In the early stages of the disease, symptoms can be effectively relieved with hypertonic drops, ointments or other conservative methods. A transplant is only considered in advanced cases where corneal swelling significantly impairs vision.
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Fuchs' dystrophy usually manifests itself in middle age or older, but early stages can be present as early as youth. The disease develops slowly, so that the initial symptoms may not be noticeable for many years.
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Prevention includes regular visits to the ophthalmologist, the use of lubricating drops and avoiding aggravating factors such as dry air or prolonged exposure to light. It is also worth avoiding trauma to the eye, which can accelerate the progression of the disease.
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In advanced stages of dystrophy, when swelling and epithelial blistering are present, contact lens wear can be difficult. In some cases, however, soft therapeutic lenses are used to reduce discomfort.
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In cases of coexisting glaucoma and Fuchs' dystrophy, intraocular pressure-lowering drops and combined surgical procedures such as glaucoma surgery and corneal transplantation are used. The decision depends on the severity of both diseases.
