Our vision depends on the health and clarity of the cornea, the transparent layer at the front of the eye. When this delicate tissue becomes swollen or cloudy, even simple tasks like reading or driving can become difficult. One common cause of such corneal changes is Fuchs’ endothelial dystrophy, a progressive condition that affects the inner layer of the cornea.
At Vision Clinic Sydney, we frequently diagnose and treat patients with Fuchs’ endothelial dystrophy using advanced imaging and surgical techniques. Understanding the symptoms, causes and treatment options can help preserve clear vision.
Understanding Fuchs’ Endothelial Dystrophy
Fuchs’ dystrophy is a progressive, hereditary eye disease that affects the cornea, the clear, dome-shaped surface at the front of the eye that helps focus light.
The condition primarily targets the endothelial cells, a thin layer of specialised cells lining the inner surface of the cornea. These cells play a vital role in keeping the cornea clear by draining out excess fluid and maintaining its proper thickness.
When endothelial cells begin to deteriorate, the cornea can no longer regulate the cornea effectively. Fluid gradually builds up within the corneal tissue, causing swelling and a loss of transparency.
Over time, this leads to blurred, hazy, or cloudy vision, which is often worse in the morning due to fluid accumulation during sleep. As the day progresses, the eye naturally dries out and vision may temporarily improve, though this relief diminishes as the disease advances.
What Causes Fuchs’ Endothelial Dystrophy?
While the exact cause of Fuchs’ dystrophy is not completely understood, genetics plays the strongest role. Many patients inherit the condition from one or both parents, and studies show a link to mutations in the TCF4 gene, which regulates cellular activity in the cornea.
The disease is also more common in women and usually develops after the age of 50, though early-onset forms may appear decades earlier.
Other contributing factors include:
- Oxidative stress, which damages corneal cells over time
- Age-related endothelial cell loss, which accelerates the disease
- Previous eye surgery or trauma can reduce the cornea’s ability to maintain fluid balance
Although it shares some degenerative features with autoimmune disorders, Fuchs’ dystrophy is not an autoimmune disease. It does not involve the immune system attacking healthy cells; rather, it stems from a gradual decline in endothelial cell function.
Common Symptoms of Fuchs’ Dystrophy
In the early stages, many patients are unaware of the condition, as vision changes may be mild or temporary. The most common early symptom is blurry vision upon waking, which clears as the day goes on. This occurs because moisture accumulates in the cornea overnight while the eyes are closed.
As Fuchs’ dystrophy progresses, swelling and light scatter increase, leading to persistent haziness and heightened light sensitivity (photophobia). Patients often report seeing halos or glare around lights, especially when driving at night.
With more advanced disease, the cornea may develop tiny blisters on its surface (epithelial bullae). These can rupture, causing pain, irritation, or the feeling of a foreign object in the eye.
Colours may appear faded and overall visual contrast declines. In severe stages, vision may become so cloudy that it feels like looking through frosted or fogged glass and daily tasks such as reading or recognising faces can become difficult.
At Vision Clinic Sydney, we encourage anyone experiencing these symptoms to book an eye examination.. Early detection can prevent permanent damage and delay the need for surgery.
The Stages of Fuchs’ Endothelial Dystrophy
The disease progresses slowly through distinct stages:
- Early Stage: The corneal endothelium starts forming tiny bumps called guttae. Vision may blur upon waking but improve later in the day.
- Moderate Stage: Corneal swelling increases, leading to fluctuating vision and light sensitivity. Contact lenses may become uncomfortable.
- Advanced Stage: The swelling becomes constant and scarring may develop. Vision remains blurry all day, and activities such as reading or driving become challenging.
- Severe Stage: Persistent pain, corneal blisters and scarring appear. At this point, surgical intervention becomes essential to restore vision.
How Serious Is Fuchs’ Dystrophy?
Fuchs’ dystrophy can range from mild discomfort to severe vision impairment. While early stages can often be managed with non-invasive treatments, advanced cases require surgery to restore corneal function. If left untreated, it may cause permanent corneal damage and significant loss of vision.
What Does Vision Look Like with Fuchs’ Dystrophy?
People with Fuchs’ dystrophy often describe their sight as looking through frosted glass. Vision tends to be foggy in the morning and clears as the day progresses, only to worsen again later. As the disease advances, the haze becomes constant, with glare, ghosting and reduced colour contrast. Night vision may also deteriorate, making driving in low-light conditions difficult.
Diagnosis at Vision Clinic Sydney
Diagnosing Fuchs’ dystrophy involves a series of advanced eye tests. At Vision Clinic Sydney, we use:
- Specular microscopy to count and evaluate endothelial cells.
- Corneal pachymetry to measure corneal thickness and detect swelling.
- Anterior segment OCT (Optical Coherence Tomography) to visualise changes in the cornea’s structure.
These tests allow for accurate detection, even in early stages, ensuring that treatment begins before permanent vision loss occurs.