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Understanding Duane Syndrome: Causes, Symptoms, and Treatment Options

Benjamin Seth Martinez | MD

Medically reviewed by Benjamin Seth Martinez | MD, Statpearls - Director of Clinical Content on July 23rd, 2023.

Key Takeaways

  • The condition results from abnormal development of the sixth cranial nerve during weeks 3-8 of pregnancy, causing the lateral rectus muscle to be innervated by the third cranial nerve instead.
  • Type I (most common, 78% of cases) prevents outward eye movement, Type II blocks inward movement, and Type III affects both directions with droopy eyelids.
  • Patients develop compensatory head turns—turning left when the right eye is affected or right when the left eye is affected—to maintain single vision and avoid double vision.
  • Eye muscle surgery can improve head positioning in 80-90% of cases but rarely restores normal eye movement; the goal is functional improvement rather than cure.
  • The condition affects females 60% more often than males and occurs unilaterally in 90% of cases, with the left eye affected slightly more frequently than the right.

Duane syndrome (DS) is a rare eye disorder that some people are born with. It occurs when the muscles and nerves around the eye don't work together properly, which limits the eye's ability to move as it should. Duane syndrome is also known as Duane's syndrome, Duane retraction syndrome, or Stilling-Turk syndrome.

Causes of Duane Syndrome

Duane syndrome is caused by abnormal development of the nerves that control the eye muscles during pregnancy. In some cases, these nerves may be missing entirely. As a result, certain eye muscles may stretch when they should tighten or remain loose when they should contract. While the exact cause of Duane syndrome is unknown, experts believe that a combination of genetic and environmental factors may play a role.

Understanding Duane Syndrome: Causes, Symptoms, and Treatment Options

Symptoms of Duane Syndrome

The most common symptoms of Duane syndrome include:

  • Strabismus (misaligned eyes)

  • Narrowing of one eyelid

  • Reduced vision in the affected eye (amblyopia or "lazy eye")

  • Upward or downward shooting of the affected eye

  • Abnormal head position (tilting or turning to keep eyes straight)

Some people with Duane syndrome may also experience double vision, headaches, and neck pain due to their abnormal head position.

Types of Duane Syndrome

There are three types of Duane syndrome:

  1. Type 1: The affected eye cannot move outward toward the ear (most common)

  2. Type 2: The affected eye cannot move inward toward the nose

  3. Type 3: The eye cannot move outward or inward

Diagnosing Duane Syndrome

Duane syndrome is usually diagnosed before the age of 10 due to its distinct symptoms. An eye doctor will perform a comprehensive eye exam, which may include a vision test, measurements of eye movement, and an examination of the eye itself. In some cases, additional tests such as hearing exams, spine evaluations, or genetic testing may be recommended.

Treatment Options for Duane Syndrome

While there is no cure for Duane syndrome, several treatment options can help manage the condition and protect vision:

  • Prescription glasses or contact lenses to improve vision

  • Eye patching to treat amblyopia (lazy eye)

  • Special lenses that shift vision to promote a more natural head position

  • Regular eye exams to monitor the condition and address any changes

  • In severe cases, eye muscle surgery to help compensate for affected muscles

It is essential for people with Duane syndrome to work closely with their eye doctor to develop an individualized treatment plan that addresses their specific needs. With proper management and regular check-ups, most people with Duane syndrome can maintain good vision and minimize the impact of the condition on their daily lives.

To learn more about Duane syndrome, visit the following reputable sources:

The Bottom Line

Early diagnosis and individualized treatment focusing on preserving binocular vision and reducing abnormal head posturing can significantly improve quality of life, though normal eye movement is rarely achieved. Surgery is reserved for cases with severe head turns or significant eye misalignment that affects daily function. If you notice restricted eye movement or persistent head tilting in yourself or your child, Doctronic can help you understand your symptoms and next steps.

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