Duane syndrome, also known as Duane's syndrome, Duane retraction syndrome, or Stilling-Turk syndrome, is a rare eye disorder some people are born with. The muscles and nerves around your eye don't work well together, and that keeps it from moving as it should.
It happens when the nerves that control the eye muscles don't grow normally during pregnancy or are missing. As a result, some muscles stretch when they should tighten or stay loose when they need to squeeze.
Usually, Duane syndrome doesn't cause blindness or lead to other health issues. In very rare cases, it has been linked to problems with bones, eyes, ears, kidneys, and the nervous system.
Most of the time, only one eye is affected — usually the left one. But 20% of people have trouble with both eyes. Women are slightly more likely to have Duane syndrome than men.
What Are the Types of Duane Syndrome?
There are three types of Duane syndrome:
- Type 1: People with this form have limited or no ability to move their affected eye out toward their ear. This is the most common type of Duane syndrome.
- Type 2: The affected eye has limited or no ability to move inward toward the nose.
- Type 3: The eye has limited or no ability to move outward or inward.
What Are the Symptoms of Duane Syndrome?
Signs of Duane syndrome can include:
- Eyes that look in different directions (also known as strabismus). It might happen all the time or just sometimes.
- Eyelid narrowing. One eye may look smaller than the other.
- Reduced vision in the affected eye. About 1 out of every 10 people with Duane syndrome have "lazy" eye, a condition called amblyopia.
- Upshoot or downshoot. The affected eye sometimes looks up or down.
- Abnormal head position. People with Duane syndrome may tilt or turn their heads to try to keep their eyes straight.
Some people with Duane syndrome also have double vision and headaches. You might also have neck pain as a result of tilting your head.
What Causes Duane Syndrome?
Duane syndrome is a genetic condition. It can be passed down in families, but this is rare. About 90% of the people with Duane syndrome are the only ones in their family to have it.
Experts believe something happens to cause Duane syndrome between the third and eighth week of pregnancy, when the cranial nerves (which control eye movement) are formed.
When Duane syndrome is inherited, it usually occurs when a variant of the CHN1, MAFB, or SALL4 geneis passed down from one parent or both parents.
How Is Duane Syndrome Diagnosed?
Because Duane syndrome has clear symptoms, most people are diagnosed before age 10. The exam usually includes a close look at your eye, along with a vision test and measurements of how far your eye can move. The doctor may also want to do a hearing test and examine your spine, the roof of your mouth, and your hands.
Some genes are linked to some cases of Duane syndrome. A genetic test can look for changes, or mutations, in the gene that might be passed on to other family members. This is done using a blood test.
Your doctor may also run tests for conditions that may be linked to Duane syndrome.
How Is Duane Syndrome Treated?
There's no cure for Duane syndrome. But your doctor can do things to help align your eyes when you're looking straight ahead and to protect your vision. Prescription glasses or contact lenses can also help.
Your doctor might suggest wearing a patch over the eye you see best with to help avoid lazy eye, or amblyopia. This forces your weaker eye to get stronger. Your doctor may also prescribe special lenses for glasses that shift your vision so you can hold your head in a more natural way.
It's important to have regular eye exams — young children may need to see their doctor every three to six months so a lazy eye can be treated right away.
In severe cases, your doctor may recommend surgery to help other muscles in your eye make up for the ones that are affected. This won't give you normal eye motion, but it usually reduces the head turning.
What Are the Complications of Duane Syndrome?
From time to time, Duane syndrome is seen with other eye concerns, such as:
- Cataract
- Lazy eye or blurred or weak vision (amblyopia)
- Problems with eye movement
- Issues with moving the eye back and forth (nystagmus)
- Optic nerve issues
- Unusually small eye (microphthalmos)
In rare cases, people with Duane syndrome also have hearing problems or trouble with their spine. Children with Duane syndrome might also have weakness in facial muscles that causes trouble with sucking motions (known as Moebius syndrome).
Takeaways
Duane syndrome is a rare eye disorder that some people are born with. The muscles and nerves around your eye don't work well together, and that keeps your eye from moving as it should. There's no cure for Duane syndrome. But your doctor can recommend certain treatments to help your eyes line up when you're looking straight ahead and to protect your vision.
Duane Syndrome FAQs
Who is most likely to get Duane syndrome?
Most cases of Duane syndrome happen sporadically or without a family history. They're caused by a genetic mutation. About 10% of cases are inherited.
Duane syndrome affects more girls than boys and can be seen in those who have other developmental concerns.
Is Duane syndrome associated with autism?
There isn't a direct link between Duane syndrome and autism, but some studies show that early developmental concerns, such as cranial nerve palsies that happen during pregnancy, might be associated with autism more often than in the normal population.
Is Duane syndrome curable?
There isn't a cure for Duane syndrome. Some people with Duane syndrome don't need treatment. For others, an eye patch might help strengthen the unaffected eye. Surgery is also an option. Although it won't cure Duane syndrome, it can help adjust the position of the eye muscles and improve eye movement.
What is the name of the surgery for Duane syndrome?
The surgery to help treat Duane syndrome is called a strabismus surgery or eye muscle surgery. It can help improve eye movement.
