Understanding Ehlers-Danlos Syndrome and Its Connection to Scoliosis

Estimated reading time: 7 minutes When I started teaching Pilates mat classes back in 2008, one of the very first clients who joined my classes had scoliosis. I learnt from day one how to work with scoliosis and what was best for this condition (she is still my client today 16 years later, so hopefully I did OK!).

Over the years I have seen many clients with scoliosis and of course, now scoliosis and hypermobility. I have worked with older EDS patients with scoliosis but also several young adults who have undergone surgical interventions. This is called rapid progression scoliosis which can happen in adolescence when rapid growth occurs. The connective tissue laxity struggles to support the spinal structure leading to a change in spinal curvatures. A member of my family underwent this corrective surgery after conventional physiotherapy, exercise, and bracing had been explored.

But what exactly is scoliosis and how might it impact EDS?

What is scoliosis?

According to The International Scientific Society on Scoliosis Orthopaedic and Rehabilitation Treatment, scoliosis is a general term that comprises a range of conditions “consisting in changes in the shape and position of the spine, thorax, and trunk (1).” Scoliosis can be structural or functional (1). Functional scoliosis is not characterized by changes in the skeletal system, and curves are not fixed. This can be due to things like differences in limb length, or muscle asymmetry (1).

“We can have a functional scoliosis – we see shift, we see asymmetries. Functional can become structural if we never do anything about it. We hope it never gets to that point . . . but we can undo things, it’s work. When there’s a structural component to that, when you look at the x-ray you can see the shape of the vertebrae actually change.” -Ann Jacott, DPT

Scoliosis is the lateral curvature of the spine in the shape of a C or an S. The curve is greater than 10 degrees when looking front to back. Rather than just a curve to the side, there is also a 3-dimensional structural change, including rotation (2). The location of the curvature and the severity can vary. It can happen at different regions of the spine and on either side of the spine (3).

The 3D nature of structural scoliosis differentiates it from other spinal curvatures or functional scoliosis that can arise as compensations for things like limb length discrepancy. When those causes are addressed the curvature resolves (2).

There are different types of structural Scoliosis

• Idiopathic Scoliosis – the most common type of scoliosis
  • The cause is unknown and is a diagnosis of exclusion, this is the most common from of scoliosis and is reported to be the cause in up to 80% of children (2). This is usually picked up between the ages of 10-18.
    • This can be further differentiated as infantile idiopathic scoliosis (children ages 0-3), juvenile idiopathic scoliosis ( ages 4 – 10), adolescent idiopathic scoliosis (Ages 11-18), and adult idiopathic scoliosis (ages above 18).
• Congenital Scoliosis
  • This form of scoliosis is present at birth, though may not be found until later due to the effects of growth. This is classified as failure of formation of the vertebra body or failure of the vertebra to segment (2).
• Neuromuscular Scoliosis
  • This is the result of neurological or muscular disorders. Neuropathic scoliosis can be caused by conditions like cerebral palsy, trauma, and poliomyelitis. Myopathic includes conditions like muscular dystrophies. This is attributed to a lack of muscular support in the spine allowing gravity to take over (2).

One review paper classified a 4th category termed Miscellaneous. This category included connective tissue disorders, mentioning Marfan Syndrome and EDS – though they didn’t specify the type (2). Others classify this category as syndromal scoliosis (4). In EDS, the spinal deformity has been attributed to hypotonia of muscles and laxity of the ligaments that can lead to mild or severe curvature (5).

Symptoms and Diagnosis of Scoliosis in Ehlers-Danlos Syndrome

Symptoms of scoliosis range from mild to severe based on the degree of curvature (3, 6). Symptoms include things like

• Changes in posture
• Head tilted to the side
• Hips are uneven
• Tops of shoulders are uneven
• One shoulder blade is more prominent than the other
• One side of the rib cage is higher when bending forward
• Back pain with more severe curvature
• Difficulty breathing in children with severe curvature

Diagnostic criteria

When assessing for scoliosis, physicians or physical therapists will perform a medical history, neurological evaluation, and physical exam. The physical exam will likely allow the provider to view from different angles of the body assessing things like the curvature of the spine, shoulder blade asymmetry, and waistline asymmetry. They may perform an Adam’s test which is a screening tool. With the provider viewing from the rear, the patient bends forward until the spine is horizontal, in scoliosis one side of the back will appear higher than the other.

If indicated, x-rays may be performed standing on the spine and neck. These can be measured to assess the degree of curvature or Cobb angle (2,6).

A diagnosis of scoliosis is confirmed with the Cobb angle measures greater than 10 degrees and axial rotation is observed (rotation of the vertebrae) (1). It is important to note that when left untreated, scoliosis can progress. This progression of idiopathic scoliosis is especially common in girls during the growth spurt of puberty (1).

Prevalence of Scoliosis in Ehlers-Danlos Syndrome

There is a sub-type of EDS called kyphoscoliotic (kEDS). This is a rarer subtype that presents as hypermobility and progressive spinal curvature. It often presents with low muscle tone and early-onset kyphoscoliosis. It affects less than 1 in 1 million people. The Ehlers-Danlos Society has more information on kEDS.

In addition to kEDS, spinal deformities are thought to be common in many types of EDS including spondylodysplastic EDS (sEDS), arthroclasia EDS (aEDS), Myopathic EDS (mEDS), Brittle Cornea Syndrome (BCS), Musculocontractural EDS (mcEDS) and hypermobile EDS (hEDS) (5).

29% of patients with hEDS had scoliosis

A small study was recently published to evaluate prevalence and severity of scoliosis in adults meeting the 2017 diagnostic criteria for hEDS. 28 patients with a mean age of 30.1 (+/-10 years) underwent complete spine x-rays. Scoliosis was found in 29% of patients. The majority of these were classified as mild (87.5%) and 12.5% had moderate scoliosis. Another 32.1 % had a “simple scoliotic inflection”. The authors state that scoliotic inflection is not to be confused with scoliosis but could be a result of things like inequality in lower limb length or pelvic tilt (7).

There was a high prevalence of hypermobility in children with idiopathic scoliosis

Another study took it from a different angle by assessing children with idiopathic scoliosis for generalized joint hypermobility. This study included 70 children aged 9-18 while the control group included 58 children without scoliosis also ranging from 9-18 years. The children were assessed for generalized joint hypermobility. 51.4% of the children with scoliosis were diagnosed with joint hypermobility compared to 19% of controls (8).

Management and Treatment Options for Scoliosis in Ehlers-Danlos Syndrome

• Pain management strategies: Both EDS and scoliosis can cause pain, so a comprehensive pain management plan is often needed. This may include medication and physical therapy.
• Physical therapy can be really helpful for this condition: It can be adapted and customized to the individual. Focus on strengthening the muscles around the spine and joints to improve stability and hopefully reduce pain.
• Additional medical treatment strategies: As mentioned above, more severe cases may need additional strategies like bracing, and casting. Some people may need surgical intervention when all other options have been explored (2). Surgical intervention is often recommended for Cobb angles greater than 50 degrees (1).
• Exercise: appropriate movement practice can help strengthen the muscles and lengthen the tighter muscles. My very first client I mentioned above loves to stretch in one direction and finds it easy, but we always encourage stretching laterally on both sides as both sides do need it.
• Posture Awareness: Working on posture can be really useful – we are not aiming to force or change the shape of the spine, but we want to keep things as functional as possible.
• Breathwork: this can be particularly helpful and is useful for pain and stress relief. In some cases, scoliosis can compress the chest cavity, reducing lung capacity and making breathing harder. When combined with EDS and the respiratory issues that can occur with EDS, this can increase the risk of respiratory problems.

Challenges in Managing Scoliosis with Ehlers-Danlos Syndrome

Working with a physical therapist who has additional training in scoliosis can be very helpful. The Schroth Method is one specialized form of physical therapy designed specifically for scoliosis that is tailored to the persons individual curve pattern. It also uses breathing and postural techniques to help correct spinal imbalances. Ann Jacott, DPT is Schroth trained therapist who came to speak to our members of The Zebra Club about scoliosis management. If you want to dive deeper and learn more about scoliosis treatment and management you can find the presentation by Ann in the Resources Center of the app in the presentations folder.

We also have a dedicated class in the app in the Stability Classes section called Moving with Scoliosis, all about elongation and balance.

Spinal stretching can be helpful for providing spinal elongation. Here is a dynamic stretching class.

FAQ

What are the strange symptoms of EDS?

EDS can manifest with a wide range of symptoms due to its impact on the connective tissues in the whole body. It may sometimes seem like issues are totally unrelated to each other, but remember if you can’t connect the issues, think connective tissues. These wide ranging symptoms can make diagnosis more challenging. Symptoms can range from dental issues, delicate fragile skin, abnormal scarring and wound healing, gastrointestinal issues, autonomic dysfunctions, neurological symptoms, sleep disorders, cardiovascular issues, eye problems, stretch marks, and allergies. This list could go on, but these could all be considered a strange collection of symptoms. Always check with your medical doctor if you have any concerns about new or unusual symptoms.

What can be mistaken for scoliosis?

There are some conditions that may get mistaken for scoliosis so it is always important to have a thorough assessment with a medical doctor.  Scoliosis refers to a sideways curve, but there are other spinal conditions that may appear similar but do need different treatments. For example, postural scoliosis – non structural changes caused by poor posture and muscle imbalances (I have had this in my upper back. Also Kyphosis – an exaggerated forward curvature of the spine and Lordosis – an excessive inward curve of the lower back.

Can someone with EDS go to a chiropractor?

This would really be down to the individual and the expertise of the chiropractor treating the EDS patient. As with any medical practitioner, it is important that the person has specialist knowledge about EDS and HSD and is able to adapt treatments to suit EDS. High velocity adjustments, especially on the neck, are not recommended for EDS patients.

Literature Review by Catherine Nation, MSc, PhD

1. Negrini, et al. (2018) 2016 SOSORT guidelines: orthopaedic and rehabilitation treatment of idiopathic scoliosis during growth. Scoliosis and Spinal Disorders
2. El-Hawary & Chukwunyerenwa (2014). Update on Evaluation and Treatment of Scoliosis. Pediatric Clinics of North America.
3. National Institute of Arthritis and Musculoskeletal and Skin Diseases. Scoliosis in Children and Teens: Diagnosis, Treatment, and Steps to Take. https://www.niams.nih.gov/health-topics/scoliosis/diagnosis-treatment-and-steps-to-take
4. Nnadi & Fairbank (2009). Scoliosis: A Review. Symposium: Surgery and Orthopeadics.
5. Uehara, et al. (2023). Spinal Deformity in Ehlers–Danlos Syndrome: Focus on Musculocontractural Type. Genes.
6. Scoliosis Research Society: Diagnosing Scoliosis. https://www.srs.org/Patients/Diagnosis-And-Treatment/Diagnosing-Scoliosis
7. Gillas, et al. (2021) Prevalence of Scoliosis in Hypermobile Ehlers-Danlos Syndrome. European Medical Journal.
8. Czaprowski, et al. (2011). Joint hypermobility in children with idiopathic scoliosis: SOSORT award 2011 winner. Scoliosis.

Scoliosis Image: Blausen.com staff (2014). “Medical gallery of Blausen Medical 2014”. WikiJournal of Medicine 1 (2). DOI:10.15347/wjm/2014.010. ISSN 2002-4436.

Jeannie Di Bon

Jeannie Di Bon is a movement therapist, educator and author specializing in hypermobility, Ehlers-Danlos Syndrome and chronic pain. She is the founder of The Zebra Club app and the creator of the Integral Movement Method.