What is Reis-Bucklers Corneal Dystrophy? Allon Barsam Explains

Consultant Allon Barsam was recently featured on television speaking about a rare eye condition, Reis-Bucklers Corneal Dystrophy.

Appearing on the popular TV series, Medical Mysteries, Mr Barsam was shown performing life-changing surgery to treat the condition, which had been causing painful, worrying and unexplained problems for a man whose eyes seemed to be ‘disintegrating’.

What is it?

Corneal dystrophy is an umbrella term for a number of different dystrophies that affect the cornea – the strong, clear outer layer of the eye that protects it against foreign bodies and helps the eye to focus light.

What they have in common is corneal erosion caused by an accumulation of material that can lead to damage and scarring. The conditions are congenital, meaning that they are inherited from one or both parents and often run in families. Many, including Reis-Bucklers, are progressive, getting worse as time goes by.

Reis-Bucklers corneal dystrophy affects the outer layers of the cornea, so it is classed as an anterior corneal dystrophy. This particular dystrophy affects the Bowman’s layer, which is situated behind the epithelium.

What are the symptoms?

The symptoms of the condition are bilateral, meaning that they affect both eyes equally. Because the cornea is important both for protecting the eye from foreign bodies and for focusing light, erosion of this part of the eye can be doubly damaging. Commonly appearing for the first time in early childhood, symptoms include:

• Painful episodes – which can be agonising and debilitating
• Light sensitivity
• A feeling that there is something in the eye
• A progressive worsening of vision

People suffering from Reis-Bucklers corneal dystrophy often have significantly poorer vision by the age of 20. And a weakened cornea can mean an increased likelihood of infections which can cause blindness.

How is it treated?

Once the condition has been diagnosed (something that does not always happen due to its rarity), the symptoms can be treated. Traditionally, the main option for treatment has been a corneal transplant – a high risk procedure that many people choose not to undergo.

However, cutting edge technology means that the condition can now be treated through sight saving laser eye surgery. A laser is used to remove the clouding and scarring that has been caused by the corneal dystrophy, leading to improved vision and a reduction in pain.

Rare eye conditions

The Medical Mysteries series highlights the plight of patients affected by especially rare diseases like Reis-Bucklers. With symptoms that can baffle specialists and leave patients in limbo, undiagnosed conditions can be physically and psychologically difficult to live with. Patients may believe that their condition will never be explained or treated.

In the case of Reis-Bucklers corneal dystrophy and its pioneering laser eye treatment, it is hoped that greater awareness will lead to many more positive results for patients like Andy, who was featured in the programme. As the technology develops, laser eye surgery will become more and more widespread in the treatment of previously untreatable eye conditions.