Can Ehlers-Danlos cause thoracic outlet syndrome?

Thoracic outlet syndrome is common in Ehlers Danlos Syndrome

Thoracic Outlet Syndrome (TOS) is a condition that occurs when the nerves or blood vessels between your collarbone and first rib are compressed, leading to symptoms in your upper body. It's not uncommon for people with Ehlers-Danlos syndrome (EDS), a group of inherited connective tissue disorders, to experience Thoracic Outlet Syndrome. In fact, EDS-related hypermobility and tissue fragility can increase the risk of developing Thoracic Outlet Syndrome.

What is Thoracic Outlet Syndrome?

Thoracic Outlet Syndrome happens when the nerves or blood vessels that travel from your neck to your arm are compressed. This space, called the thoracic outlet, can become narrowed due to repetitive arm motions or anatomical variations, such as an extra rib. For people with Ehlers-Danlos syndrome, shoulder hypermobility and instability make them more prone to this compression.

How Ehlers-Danlos Syndrome Increases the Risk of Thoracic Outlet Syndrome

Ehlers-Danlos syndrome affects connective tissues, which provide structure and support to skin, joints, and blood vessels. One of the hallmark features of Ehlers-Danlos syndrome is joint hypermobility, which can affect the stability of the shoulder girdle. This instability can cause the muscles around the thoracic outlet to tighten and compress the nerves or blood vessels, leading to Thoracic Outlet Syndrome​​.

Symptoms of Thoracic Outlet Syndrome in People with Ehlers-Danlos Syndrome

Thoracic Outlet Syndrome can cause a variety of symptoms, depending on whether nerves or blood vessels are affected. These may include:

• Numbness or tingling in the arms or fingers
• Weakness in the arms
• Pain in the shoulders, neck, or arms
• Reduced blood flow, leading to pale or bluish skin and cold fingers

For people with Ehlers-Danlos syndrome, these symptoms can overlap with the more generalized symptoms of the condition, like:

• Joint hypermobility, especially in the shoulders
• Skin that stretches more than usual
• Fragile tissues, which can make healing more difficult​​​.

Causes and Risk Factors

While anyone can develop Thoracic Outlet Syndrome, the risk is higher in those who are tall and thin or engage in activities with repetitive arm movements, such as swimming or playing a musical instrument. In people with Ehlers-Danlos syndrome, hypermobile joints and poor muscle tone further contribute to the compression of nerves and blood vessels in the thoracic outlet.

Common causes of Thoracic Outlet Syndrome in Ehlers-Danlos syndrome include:

• Shoulder girdle hypermobility, which causes poor posture and misalignment of the muscles
• Trigger points, areas of muscle tightness that can contribute to compression of the thoracic outlet​.

Diagnosis and Treatment

Diagnosing Thoracic Outlet Syndrome involves a combination of physical exams, imaging tests, and nerve conduction studies. In patients with Ehlers-Danlos syndrome, it's especially important for healthcare providers to consider hypermobility and other connective tissue issues when making a diagnosis​.

Treatment Options:

1. Physical Therapy: Strengthening exercises can help improve posture and stabilize the shoulder girdle. Proprioceptive training (awareness of body position) and scapular stabilization exercises are particularly useful in people with Ehlers-Danlos syndrome​​.
2. Managing Trigger Points: Trigger points, or muscle knots, are common in people with Ehlers-Danlos syndrome and can exacerbate Thoracic Outlet Syndrome symptoms. Trigger point therapy, such as massage or dry needling, may reduce muscle tension and improve blood flow​​.
3. Proprioception and Stability Training: Strengthening the muscles around the shoulder and improving body awareness can relieve symptoms by preventing hypermobility from causing further compression​.
4. Medications and Injections: Steroid injections may help reduce inflammation, while pain management techniques like nonsteroidal anti-inflammatory drugs (NSAIDs) can relieve discomfort​​.
5. Surgery: In severe cases, surgery may be required to remove any anatomical barriers causing the compression, such as a cervical rib or to release the tight muscles​.

Improving Quality of Life

Living with both Ehlers-Danlos syndrome and Thoracic Outlet Syndrome can be challenging, but with proper management, many people can lead fulfilling lives. Regular physical therapy, posture correction, and muscle-strengthening exercises can help reduce symptoms over time. In severe cases, a multidisciplinary approach involving orthopedic specialists, physical therapists, and pain management experts can help manage the complexity of these conditions​​.

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FAQs

Can Ehlers-Danlos syndrome cause Thoracic Outlet Syndrome?‍

Yes, Ehlers-Danlos syndrome can increase the risk of Thoracic Outlet Syndrome due to joint hypermobility and instability, particularly in the shoulder girdle, which may compress the nerves or blood vessels in the thoracic outlet​​.

What are the uncommon symptoms of Ehlers-Danlos syndrome?‍

Besides joint hypermobility and skin extensibility, Ehlers-Danlos syndrome can cause gastrointestinal issues, dysautonomia (autonomic nervous system dysfunction), and cardiovascular problems​.

Who is prone to Thoracic Outlet Syndrome?‍

People who are tall and thin, or who engage in activities involving repetitive arm motions, are at higher risk. In Ehlers-Danlos syndrome patients, hypermobility adds to the risk​.

What are the red flags of Thoracic Outlet Syndrome?‍

Severe, unrelenting pain in the neck, shoulder, or arm, muscle wasting, and signs of poor blood circulation (e.g., bluish skin or cold fingers) are red flags that require medical attention​.

What can be mistaken for Thoracic Outlet Syndrome?‍

Conditions like carpal tunnel syndrome, cervical disc disorders, and rotator cuff injuries can mimic Thoracic Outlet Syndrome symptoms​.

What is the gold standard test for Thoracic Outlet Syndrome?‍

There isn’t a single gold standard test, but a combination of imaging (e.g., MRI, CT), nerve conduction studies, and physical exams are used​.

What positions make Thoracic Outlet Syndrome worse?‍

Activities that involve lifting your arms above your head or repetitive shoulder movements can aggravate Thoracic Outlet Syndrome​.

What is the Roos test for Thoracic Outlet Syndrome?‍

The Roos test (or elevated arm stress test) involves raising your arms and opening and closing your hands to test for Thoracic Outlet Syndrome symptoms​.

What kind of doctor diagnoses Thoracic Outlet Syndrome?‍

A vascular surgeon, neurologist, or orthopedic specialist typically diagnoses Thoracic Outlet Syndrome​​.

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References

• Bloom, Lara, et al. "The International Consortium on the Ehlers-Danlos Syndromes." American Journal of Medical Genetics Part C: Seminars in Medical Genetics, vol. 175, no. 5, 2017, pp. 5-7. Wiley Online Library.
• Brady, Angela F., et al. "The Ehlers–Danlos Syndromes, Rare Types." American Journal of Medical Genetics Part C: Seminars in Medical Genetics, vol. 175, no. 70, 2017, pp. 70-115. Wiley Online Library.
• Byers, Peter H., et al. "Diagnosis, Natural History, and Management in Vascular Ehlers–Danlos Syndrome." American Journal of Medical Genetics Part C: Seminars in Medical Genetics, vol. 175, no. 40, 2017, pp. 40-47. Wiley Online Library.
• Chopra, Pradeep, et al. "Pain Management in the Ehlers-Danlos Syndromes." American Journal of Medical Genetics Part C: Seminars in Medical Genetics, vol. 175, no. 212, 2017, pp. 212-219. Wiley Online Library.
• Ericson, William B., Jr., and Roger Wolman. "Orthopaedic Management of the Ehlers–Danlos Syndromes." American Journal of Medical Genetics Part C: Seminars in Medical Genetics, vol. 175, no. 1, 2017, pp. 188-194. Wiley Online Library, https://doi.org/10.1002/ajmg.c.31551.
• Hakim, Alan, et al. "Cardiovascular Autonomic Dysfunction in Ehlers–Danlos Syndrome—Hypermobile Type." American Journal of Medical Genetics Part C: Seminars in Medical Genetics, vol. 175, no. 168, 2017, pp. 168-174. Wiley Online Library.

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