Duane Retraction Syndrome

Duane retraction syndrome is a congenital condition affecting eye movement. It occurs because of abnormal wiring during fetal development—the nerve that should control the muscle turning the eye outward doesn't develop properly, and another nerve partially takes over. The result is limited outward eye movement and eye retraction when looking inward.

Understanding Duane Syndrome

During normal development, the sixth cranial nerve controls the muscle that turns the eye outward (lateral rectus). In Duane syndrome, this nerve doesn't develop properly. Instead, a branch from the third nerve (which normally controls other eye muscles) connects to the lateral rectus. This abnormal wiring causes the characteristic features of the syndrome.

Types

Type I (Most Common—70-80%)

• Limited abduction (can't turn eye outward)
• Normal or near-normal adduction (turning inward)
• Eye retracts when looking inward

Type II (Less Common—~7%)

• Limited adduction (can't turn eye inward)
• Normal or near-normal abduction

Type III (Less Common—~15%)

• Limited movement in both directions
• Eye retracts when looking inward or straight

Symptoms

Eye Movement

• Cannot turn affected eye fully outward (Type I)
• Eye pulls back into socket when turning inward
• Eyelid opening narrows when looking inward
• May have abnormal up or down movements

Vision and Alignment

• Often no double vision—brain adapted since birth
• Eyes may appear aligned when looking straight ahead
• Misalignment visible when looking to the side

Compensatory Head Position

• Head turn toward affected side
• Maintains eye alignment and avoids diplopia
• May be the main concern for treatment

Associated Conditions

Some patients have additional findings:

• Hearing abnormalities
• Skeletal abnormalities
• Other eye or systemic conditions

Diagnosis

Clinical Examination

• Characteristic pattern of eye movement limitation
• Eye retraction observed
• Eyelid narrowing during adduction
• Usually apparent in infancy or childhood

Imaging

• MRI brain can show absent sixth nerve
• Usually not necessary for diagnosis if clinical features typical

Rule Out Other Causes

• Sixth nerve palsy (acquired)—different history
• Other forms of strabismus
• Thyroid eye disease

Treatment

Observation

Many patients need no treatment:

• Aligned in primary position
• No significant head turn
• No diplopia
• Good binocular vision

Indications for Surgery

• Significant abnormal head position
• Significant misalignment in primary gaze
• Diplopia (uncommon)
• Cosmetic concerns

Surgical Options

• Strabismus surgery to improve alignment
• May involve muscles in both eyes
• Goals: reduce head turn, improve cosmesis, maintain alignment

Glasses/Prisms

• Rarely needed
• May help if any residual diplopia

Living with Duane Syndrome

Practical Considerations

• Most people adapt well
• Head turn becomes natural
• Vision usually excellent
• Most activities unaffected

Sports and Activities

• Usually no limitations
• May need slight head turn for activities requiring side gaze
• Sports participation generally fine

Driving

• Usually no issues
• Head turn compensates for limited eye movement
• Check with doctor if concerned

Prognosis

Excellent

• Condition is stable—doesn't worsen with age
• Not progressive
• Vision typically normal
• Most people have minimal impact on daily life

Post-Surgery

• Surgery can improve head position and alignment
• Cannot restore normal eye movement
• Generally good outcomes

Frequently Asked Questions

Can Duane syndrome be cured?

There's no cure—the abnormal nerve wiring can't be changed. However, most people with Duane syndrome function normally with minimal impact. Surgery can improve alignment and head position but doesn't restore normal eye movement.

Will my child need surgery?

Not necessarily. Many children with Duane syndrome don't need surgery if their eyes are aligned when looking straight ahead and they don't have a significant head turn. Surgery is considered when there's a noticeable head position, misalignment, or cosmetic concern.

Is Duane syndrome inherited?

Most cases are sporadic (not inherited), though some families show autosomal dominant inheritance. If you have Duane syndrome, your children may have a slightly increased risk, but most cases occur without family history.

Why doesn't my child have double vision?

Because Duane syndrome is present from birth, the brain develops with this visual input and adapts. Children don't experience double vision because their brains have always processed the eyes' slightly different positions as normal.

Will this get worse with age?

No. Duane syndrome is stable and doesn't progress. The eye movement pattern remains the same throughout life.

References