Duane Syndrome

What is Duane syndrome?

Duane syndrome is a type of strabismus, an eye misalignment that’s congenital (something you’re born with). If you have Duane syndrome, you’ll have difficulties moving your eyes from side to side. You may have trouble with one or both eyes looking inward toward your nose or outward toward your ear.

You may also have trouble looking upward or downward. Along with your eyes crossing, or being out of alignment with each other, your eyelids might move when you try to move your eyes. This is because the nerves that control the muscles in your eyes don’t work correctly.

About 80% to 90% of Duane syndrome cases are unilateral, which means they affect only one eye. The left eye is more commonly affected.

Are there other names for Duane syndrome?

Yes, there are several names for Duane syndrome. They include:

• Duane retraction syndrome.
• Eye retraction syndrome.
• Retraction syndrome or congenital retraction syndrome.
• Duane radial ray syndrome.
• Stilling-Turk-Duane syndrome.

Are there different types of Duane syndrome?

Yes, there are different types of Duane syndrome: type 1, type 2 and type 3.

Type 1 Duane syndrome

Duane syndrome type 1 is the most common form. It accounts for about 78% of all cases.

If you have type 1, you’ll have trouble moving your eye outward toward your ear. Your eye will be able to cross inward toward your nose, but your eye opening typically becomes smaller and the eyeball retracts. Your eye may look crossed in toward your nose (esotropia).

Type 2 Duane syndrome

Duane syndrome type 2 is the least common form. It accounts for about 7% of people with the condition.

If you have type 2, you have a limited ability to move your eyes inward toward your nose. You will be able to turn your eye outward toward your ear but your eye opening typically becomes smaller and the eyeball retracts. Your eye may look turned out toward your ear (exotropia).

Type 3 Duane syndrome

Duane syndrome type 3 accounts for about 15% of total cases.

If you have type 3, your ability to move your eyes in either direction is limited. Also, your eye opening may become smaller and your eyeball retracts with certain gazes.

How common is Duane syndrome?

It’s estimated that 4% of the population of the U.S., or some 13 million people, have strabismus. It’s further estimated that about 1% to 5% of the total number of people with strabismus have Duane syndrome.

Duane syndrome is slightly more common in people assigned female at birth (AFAB) than people assigned male at birth (AMAB).

What are the signs and symptoms?

Signs and symptoms of Duane syndrome, some of which may be seen in infants, may include:

• An unusual position of the head. People with Duane syndrome sometimes turn their heads to the side to straighten their eyes.
• Eyes that don’t point in the same direction, either at some times or all times (strabismus).
• One eye — the one that’s affected — having lower vision (amblyopia, or lazy eye).
• One eye seems smaller than the other because the eyelids narrow.
• Eyes that move either up or down when you’re looking a certain way. This is called an up-shoot or down-shoot.

Do people with Duane syndrome have other conditions?

Duane syndrome doesn’t always happen with other conditions, but it sometimes does. These conditions may include:

• Hearing disorders.
• Goldenhar syndrome.
• Problems with your spine or vertebrae.

There may also be other eye-related conditions that happen along with Duane syndrome, including:

• Cataract, which refers to the clouding of the lens of your eye.
• Microphthalmos, which means that your eye is unusually small.
• Nystagmus, a condition in which your eyes move uncontrollably.
• Crocodile tears syndrome, where someone with Bell’s palsy sheds tears while eating or drinking.

What causes Duane syndrome?

Duane syndrome is genetic, but not typically inherited. The movement issues are due to problems with your cranial nerves, or the nerves that control the way your eyes move.

Duane syndrome may happen more frequently in people who’ve been exposed to thalidomide in utero.

Only about 10% of people with Duane syndrome have other family members with the condition. In cases that run in the family, Duane syndrome is usually bilateral (it affects both eyes).

If you inherit Duane syndrome, it happens because a variant of CHN1, MAFB, or SALL4 is passed down. The pattern for inheritance in Duane syndrome, called “autosomal dominant,” means that it occurs equally in people AMAB and people AFAB, and it can be inherited if just one parent has Duane syndrome.

Is Duane syndrome contagious?

No, Duane syndrome isn’t contagious. You can’t pass it on to someone like you can with an infection.

How is Duane syndrome diagnosed?

Your healthcare provider will take a complete medical history and will do a thorough eye exam. They’ll measure how much misalignment is present and test how far your eyes, or your child’s eyes, can move from side to side.

Your provider may also want to order tests for other conditions that might be linked to Duane syndrome.

There’s another condition called sixth cranial nerve palsy that’s very similar to Duane syndrome but is much less common than Duane syndrome.

How is Duane syndrome treated?

Treatment will depend on age, severity and other illnesses you might have.

Some people with Duane syndrome won’t need treatment. They’ll just need to keep a regular appointment schedule with their eye care providers.

People who also have amblyopia may benefit from patching their good eye so the weaker one becomes stronger.

If you have a severe case of Duane syndrome, you may need surgery on your eye muscles. The surgery can’t fix the nerve problem but it can change the position of eye muscles to relieve an abnormal head posture, straighten eyes when looking straight ahead, and improve up-shoots/down-shoots.

How can I prevent Duane syndrome?

At this time, there’s no way to prevent Duane syndrome from happening. Thalidomide, the medication that can cause Duane syndrome (in addition to many other congenital conditions), is no longer available.

What can I expect if I have Duane syndrome?

Some people with Duane syndrome can manage the condition by moving their heads slightly to improve their vision. In some cases, you may need surgery.

If you have another condition along with Duane syndrome, your provider will treat that condition also.

You may need glasses to improve vision and/or alignment.

What questions should I ask my doctor?

You may want to ask your healthcare provider questions like these:

• Should I see a genetic counselor?
• Are there any aids that may help with vision?

A note from Cleveland Clinic

A provider will probably diagnose Duane syndrome within the first 10 years of your child’s life if your child has the condition. Contact your healthcare provider if you have any questions about your sight or your child’s sight. Your healthcare provider can work with you to determine the best treatment for Duane syndrome and can help to treat amblyopia if it’s present.