Kayser-Fleischer Rings

What are Kayser-Fleischer rings?

Kayser-Fleischer rings are dark rings that appear as circles around the irises of your eyes. The iris is the colored part of your eye. The rings are made of copper deposits that have settled in your eye. The rings can have a green, gold or brown color to them. The rings don’t interfere with your vision.

The rings appear at the limbus, which is the place where your cornea meets your sclera. The color starts as an arc over the top of your eye, but then continues to spread until it forms a ring.

It’s more difficult to see Kayser-Fleischer rings on darker eyes, like brown eyes, than it is to see them on lighter-color eyes.

What is the difference between Kayser-Fleischer rings and arcus senilis?

Arcus senilis develops as a white-grey ring from deposits of lipids. Kayser-Fleischer rings are dark and are caused by copper deposits in your eye.

An ophthalmologist, or eye care specialist, will need to use a slit-lamp examination to see the Kayser-Fleischer rings, but not to see arcus senilis.

In some cases, your eye doctor will have to use gonioscopy to know for sure if you have Kayser-Fleischer rings. This test combines the slit-lamp exam (the special microscope used to examine your eyes) and a special contact lens. The doctor puts the gonioscopy lens on your numbed eye and shines a beam of light onto the lens.

Gonioscopy is commonly used in testing for and treating glaucoma.

What are Kayser-Fleischer-like rings?

Kayser-Fleischer-like rings aren’t actual Kayser-Fleischer rings but may seem similar. Conditions that cause high bilirubin levels are related to forming Kayser-Fleischer-like rings.

What are Fleischer rings?

Fleischer rings are another type of corneal ring. Iron deposits in your cornea cause Fleischer rings. This type of ring is related to keratoconus, a condition that causes your eye to bulge out in a cone shape instead of the usual round shape of your cornea.

What are the most common causes of Kayser-Fleischer rings?

The most common cause of Kayser-Fleischer rings is Wilson’s disease. Wilson’s disease causes copper to build up in your body. It’s a genetic disorder and can cause liver disease. The copper deposits can cause Kayser-Fleischer rings and, more rarely, sunflower cataracts.

Wilson’s disease can also cause nervous system and mental health symptoms after copper starts to accumulate in your central nervous system, including your brain. These symptoms include movement problems, speech issues, anxiety, depression and other personality or behavior changes.

About 95% of the people who have neurological symptoms will have Kayser-Fleischer rings. This figure is about 50% for people who have only liver issues and Wilson’s disease.

There are other conditions associated with Kayser-Fleischer rings, including:

• Primary biliary cholangitis: This chronic condition harms your body’s liver function. The old name of this condition is primary biliary cirrhosis.
• Neonatal cholestasis: This liver condition refers to any type of impaired bile flow into the liver of a newborn child. Bile flow impairment can also happen in adults.

How are Kayser-Fleischer rings treated?

Your healthcare provider will treat the disease that causes Kayser-Fleischer rings, rather than the rings themselves.

Treating Wilson’s disease

Treatments for Wilson’s disease include:

• Copper chelation therapy:Your provider will prescribe medicines that remove copper from your body (chelating agents). These medications include penicillamine (Cupramine®, Depen®) and trientine (Syprine®).
• Zinc supplements:Zinc prevents your body from absorbing copper in your intestines.

Treating primary biliary cholangitis

There’s no cure for primary biliary cholangitis (PBC). However, your provider will help you manage symptoms and slow down the progression of the disease. Medications include:

• Ursodiol (Actigall®) to help remove bile from your liver.
• Obeticholic acid (Ocaliva®) to reduce the amount of bile produced and increase the flow of bile out of your liver. Ocaliva and ursodeoxycholic acid are often combined.
• Dyphenhydramine (Benadryl®), hydroxyzine (Vistaril®) or cholestyramine (Locholest® or Questran®) to treat severe itchiness.

Treating neonatal cholestasis

There isn’t one way to treat neonatal cholestasis. Your healthcare provider may suggest certain types of formulas or recommend products to treat itching. For babies who’ve developed severe liver disease, their provider may suggest liver transplant surgery.

How can I prevent Kayser-Fleischer rings?

You can’t prevent Kayser-Fleischer rings from occurring because you can’t prevent the conditions that cause them. However, if someone in your immediate family (your parent, sibling or child) has Wilson’s disease, you may want to speak to your provider about testing. Early diagnosis can lead to early treatment.

Are Kayser-Fleischer rings permanent?

No, Kayser-Fleischer rings aren’t permanent. They often disappear when the underlying cause is treated.

When should you call your healthcare provider about Kayser-Fleischer rings?

It’s unlikely that you’ll need to talk to your healthcare provider about Kayser-Fleischer rings, but you may need to speak about managing Wilson’s disease or other liver disorders related to the rings.

If you have any symptoms that worry you, whether it’s with your eyes, your vision or any other changes, be sure to mention this to your healthcare provider.

A note from Cleveland Clinic

Your eye care provider will probably be the one to notice if you have Kayser-Fleischer rings. They may be looking specifically for these rings if you have Wilson’s disease or another liver condition. These rings won’t affect your vision at all. They’re likely to disappear when your underlying condition is treated.