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Duane Syndrome and Vision

Estimates of the incidence of duane Syndrome vary from 1 per 1,000 people to 1 per 10,000. This would translate to between 33,900-339,000 Americans.

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Duane syndrome, also referred to as Duane's syndrome, Stilling–Turk–Duane syndrome, and Duane retraction syndrome, is a type of strabismus estimated to account for between 1%-5% of strabismus cases, a condition characterized by eye misalignment. Unlike most forms of strabismus that typically allow for side-to-side eye movement, Duane syndrome results from a disruption in the coordination of eye muscles, restricting eye movement in specific directions.

Can Duane Syndrome Lead to Blindness?

Duane Syndrome itself is generally not a condition that leads to blindness. It primarily affects the range of eye movement and may cause eye misalignment. This form of strabismus often leads to impaired vision, which is usually treatable through surgery and vision therapy. It usually doesn't directly impact the health of the retina, optic nerve, or other components essential for vision.

However, any eye misalignment can potentially lead to a condition called amblyopia, also known as "lazy eye," if not managed correctly. Amblyopia may result in vision loss in the affected eye, especially when it develops in young children and is not treated promptly. While amblyopia usually doesn't result in complete blindness, it can cause significant vision impairment. Amblyopia caused by Duane syndrome is treatable with vision therapy and/or surgery.

Who Is at Risk for Duane Syndrome?

Gender Differences

Duane Syndrome is more commonly diagnosed in females compared to males. The reasons for this gender disparity are not entirely understood but could involve a combination of genetic and hormonal factors.

Family History

Although most cases of duane Syndrome are sporadic, meaning they occur randomly, there's a higher risk for individuals who have a family history of the condition or other congenital eye movement disorders. A genetic predisposition could potentially make one more susceptible to developing duane Syndrome.

Congenital Factors

Duane Syndrome is generally a congenital condition, meaning it is present from birth. Babies born with other congenital abnormalities or syndromes, such as Wildervanck syndrome or Goldenhar syndrome, may have a higher likelihood of also having duane Syndrome.

Although the evidence is limited, some research suggests that environmental factors during pregnancy, such as exposure to certain medications, toxins, or infections, might increase the risk of developing duane Syndrome. However, these are not confirmed risk factors, and further studies are needed for a conclusive understanding.

Age of Diagnosis

Duane Syndrome is most commonly diagnosed in childhood, often shortly after birth or in the early developmental years. However, being a congenital condition, it can be present but undiagnosed until later in life, depending on the severity and impact on daily activities.

In one study the mean age of presentation of symptoms was 13.5 for type one, and early 20's for type 2 and 3. (Kekunnaya R, Gupta A, Sachdeva V, et al. duane retraction syndrome: series of 441 cases. J Pediatr Ophthalmol Strabismus. 2012;49(3):164–169.)

Three Different Types of Duane Syndrome

Three Different Types of Duane Syndrome

  1. There are three distinct types of duane syndrome, with Type I being the most prevalent. Type I primarily affects the eye's ability to move outward from the nose, and while duane syndrome often impacts a single eye, it can occasionally involve both eyes. Interestingly, it tends to affect the left eye more frequently than the right, though the precise reasons remain unknown. This is the most common type, accounting for about 70-80% of all duane Syndrome cases.
  2. Type 2 is less common, making up approximately 5-10% of cases. Here, the eye has trouble moving inward (adduction), while the outward movement (abduction) is usually normal or only slightly limited. This type is less common and usually less problematic in terms of daily activities compared to Type 1.
  3. This type accounts for around 10-20% of cases. Patients with Type 3 duane Syndrome have restricted movement both inward (adduction) and outward (abduction). This is the most severe form and can significantly affect a person's quality of life, often requiring more intensive treatment.
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Symptoms of Duane Syndrome

Common observable symptoms of duane syndrome include:

  • Headaches and neck discomfort caused by abnormal posture or tilting the head to see clearly
  • Difficulty seeing objects on the side of the affected eye
  • Limited horizontal eye movement: Difficulty moving one or both eyes outward (toward the ear) due to restricted abduction.
  • Abnormal eye movement: Inward movement (adduction) may be more pronounced and compensate for the limited outward movement.
  • Head turn or tilt: Individuals often adopt a head turn or tilt to align their eyes and reduce double vision caused by the limited eye movement.
  • Strabismus (crossed eyes): One eye may appear misaligned, particularly when the affected eye is turned inward to compensate for limited abduction.
  • Esotropia: The inward deviation of the affected eye when attempting to move it outward.
  • Exotropia: Occasional outward deviation of the affected eye when attempting to move it inward.
  • Double vision (diplopia): Due to misalignment of the eyes, individuals with Duane syndrome may experience double vision.
  • Head movement: Some individuals may use head movements to track objects in the peripheral vision since eye movement is limited.
  • Squinting or closing one eye: Individuals with Duane syndrome may squint or close one eye to improve their focus and reduce double vision.
  • Eyelid abnormalities: Some cases of Duane syndrome may be associated with eyelid abnormalities, such as ptosis (drooping eyelid) or narrowing of the palpebral fissure (the opening between the eyelids).
  • Abnormal eye retraction: During eye movement, the affected eye may retract slightly into the eye socket, giving a "pulling" sensation.
  • Upward or downward eye movement limitations: In some cases, individuals with Duane syndrome may also have limited vertical eye movement, either upward or downward.
  • Anomalous head posture: The head posture used to compensate for limited eye movement may vary among individuals, leading to unique head positions.

Other Vision Conditions Associated with Duane Syndrome

Amblyopia: A condition where the brain ignores the signal from one eye, often causing the weaker eye to drift, which is why it is commonly called Lazy Eye. The estimated incidence of amblyopia (lazy eye) among people with duane Syndrome is 14%

Myopia: Also called nearsightedness, this is a refractive error where seeing at a distance is impaired. The estimated incidence of myopia (nearsightedness) among people with duane Syndrome is 15%

Hyperopia: Also called farsighted, is a difficulty seeing things up close due to a refractive error of the eye. The estimated incidence of hyperopia (farsightedness) among people with duane Syndrome is 71%.

Anisometropia: The estimated incidence of anisometropia(different prescriptions in each eye) among people with duane Syndrome is varies greatly between studies between 3%-40%

(P.A. DeRespinis, A.R. Caputo, R.S. Wagner, S. Guo, duane’s retraction syndrome, Survey of Ophthalmology, 38 (3) (1993), pp. 257-288)

Treatment of Duane Syndrome

Duane syndrome is a type of strabismus where there is a limitation in eye movement, often causing the eyes to appear misaligned. Traditional treatments like eyeglasses may not adequately address the complexities of this condition, making vision therapy combined with surgery when appropriate the ideal treatment.

As there is no means to repair or replace the affected sixth cranial nerve causing duane syndrome, a complete cure remains elusive. However, there can be significant improvement to posture and functional vision with the use of surgery, prism lenses, and vision therapy, with many patients combining these approaches to achieve best results.

Surgery for Duane Syndrome

Surgery is one of the most common treatments for duane syndrome. The goal of surgery is to improve the eye's alignment and increase the range of motion. This can reduce or eliminate the need for frequent head turns to see clearly. However, surgery might not fully correct the condition or restore normal eye movement. Surgical options may include:

Recession: Loosening the affected muscle to allow more freedom of movement.

Resection: Tightening the opposite muscle to balance the eye's movement.

Risks include scarring, over-correction, or under-correction. Furthermore surgery alone in many cases does not acheive best vision as the patients missaligned eye has not been reintroduced into the binocular vision system (both eyes working together in a coordinated fashion).  It's essential to discuss your specific case with your eye doctor to see if surgery is suitable for you.

Vision Therapy or Neuro Optometric Rehabilitation

Vision therapy and neuro optometric rehabilitation offer a targeted approach for treating duane syndrome, which is often used alongside surgical interventions.

The goal of vision therapy and neuro optometry is to develop better binocular vision, where both eyes work in harmony. A variety of tailored exercises aim to enhance eye coordination, muscle control, and visual processing skills. It works to repair the connection between the eyes and the brain, which is often compromised in cases of duane syndrome. For those with duane syndrome, vision therapy can be particularly effective in mitigating symptoms, improving visual function and improving the results of surgery alone.

Prism lenses can also play a role in neuro optometry and vision therapy treatment programs for duane syndrome. These special lenses are designed to shift the image seen by each eye, making it easier for the brain to merge these images into one. While they won't cure Duane Syndrome, they can significantly reduce symptoms like eye strain and double vision.

Prism lenses are often employed as a less invasive treatment choice, either on their own or in conjunction with vision therapy or neuro optometric rehabilitation to enhance the efficacy of therapy. By helping the eyes work together more effectively, they offer another avenue for symptom relief and improved quality of life.

Combining Vision Therapy and Surgery

For many patients, a combination of vision therapy/neuro optometry and surgery provides the best results. Even after the eyes are straightened through surgery, the visual system has not been taught how to properly use both eyes together, which is called binocular vision. By training the visual system properly patients will oftentimes have a dramatic improvement in vision when compared to surgery alone.  Vision therapy can help prepare the eye for surgery and improve outcomes or vision therapy can take place after surgery to improve success. 

It's essential to consult a functional optometrist or neuro optometrist for a comprehensive diagnosis and tailored treatment plan. Depending on the severity of your condition and your lifestyle, a combination of these treatments may offer the best chance for improved vision and comfort.

Is Duane Syndrome Congenital?

Yes, duane Syndrome is often congenital, meaning it is present from birth. Most individuals diagnosed with this condition start showing symptoms early in life. However, it's important to note that the symptoms might not be fully noticeable or problematic until later stages of childhood or even adulthood in some cases. Early diagnosis and intervention can improve long-term outcomes, so if you notice any signs or symptoms, it's best to consult an eye care specialist as soon as possible.

Is Duane Syndrome Hereditary?

Duane Syndrome can be hereditary, but most cases are sporadic, meaning they occur without a clear family history. In some instances, duane syndrome has been found to run in families, suggesting a genetic component. When it is hereditary, it often appears as part of a syndrome that affects multiple systems of the body, not just the eyes.

If you have a family history of duane syndrome or similar eye movement disorders, it's essential to inform your eye care specialist for a more accurate diagnosis and risk assessment. However, the majority of cases do not have a known hereditary link.

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