Paediatric Joint Hypermobility: A Physiotherapist’s Perspective

Paediatric Joint Hypermobility: A Physiotherapist’s Perspective

By Katie Cleary (Roth)
APA Paediatric Physiotherapist and Director – Ocean Kids Health

In my many years as a Paediatric Physiotherapist and across our Physiotherapy Team at Ocean Kids Health, we’ve assessed and treated thousands of children with Paediatric Joint Hypermobility.
It’s a very common condition that is often overlooked and underdiagnosed can be the culprit underlying challenges that some children face.

The diagnosis of Joint Hypermobility is often delayed due to the diverse and variable presentation in children and adolescents. Very often when we assess a child with symptomatic hypermobility, they have had many consultations with health professionals and specialists for a range of seemingly unrelated health concerns. We then find the issue is with the connective tissues!

Joint Hypermobility often gets a bad wrap. It’s not always a bad thing. If fact, it can be a very good thing! Many of our best athletes, swimmers, gymnasts, dancers and performers are hypermobile.

Here, I’ll share my insights into Paediatric Joint Hypermobility and discuss our latest diagnostic criteria which was introduced by the International Paediatric Consortium of the Ehlers-Danlos Society, which enhances our ability to diagnose and monitor this condition, creating better outcomes for our children.

What is hypermobility?

Hyper = More
Mobility = Movement
Hypermobility is excessive laxity or mobility in our joints which causes increased flexibility!
Other words used to describe hypermobile people include : bendy, flexible, loose, double-jointed, floppy, clumsy or having cool party tricks!

When we are born, we are more flexible as our joints have not developed fully until our musculoskeletal system matures. As we age, we become less flexible due to changes in joint integrity and as our muscles and ligaments develop and strengthen in antigravity positions. Our connective tissues that wrap and form around our joints to keep them in place are made from collagen. The type of collagen you are born with plays a role in determining whether you are hypermobile or not e.g. how much elasticity your collagen has.

What causes Joint Hypermobility?

Joint hypermobility varies widely between individuals. It can be acquired e.g. increased flexibility from increased activity, stretching or training, inherited or as feature of a hereditary connective tissue disorder (HCTD) affecting the type of collagen you have. e.g. Osteogenesis Imperfecta, Marfans, Ehlers Danlos Syndrome OR as a result of bony morphology (the shape of the ends of your bones).

How common is hypermobility in kids?

Babies and children are naturally quite flexible. This is due to the immaturity of their musculoskeletal system e.g. their bones and joints have not fully formed yet and their muscles are not strengthened against gravity). While most kids become less flexible as they grow and enter puberty, around 20% of kids maintain hypermobility into adulthood.
In Australian children, the prevalence of generalised joint hypermobility (Beighton score ⩾ 6) is 26.1% in girls and 11.5% in boys. Children from Asian & ethnic backgrounds are generally more hypermobile than Caucasian children.

When hypermobility doesn’t result in pain or other symptoms, it’s termed ‘asymptomatic joint hypermobility’ and there’s usually no need for treatment. ‘Symptomatic hypermobility’ occurs when there are symptoms associated with hypermobility that start to develop.

Signs and symptoms associated with hypermobility in childhood can include:

Pain: Children with joint hypermobility may experience recurrent joint pain, which can affect one or multiple joints. The pain may be episodic and not necessarily linked to an injury.

Persistent Fatigue: Fatigue or tiredness is a common symptom in childhood and adolescents. This fatigue may be associated with the demands placed on hypermobile joints during daily activities.
Sometimes children with hypermobility may appear less active and be labelled as ‘always tired’ or ‘lazy’. Their muscles are having to work much harder to keep them upright and stable – the job their joints aren’t doing a great job of. No wonder they get tired!

Muscle Weakness: Weakness in the muscles supporting hypermobile joints can contribute to symptoms. Building strong muscles is crucial for our children with hypermobility to manage their symptoms long term. We know that joint instability and muscle weakness can be a recipe for injuries and chronic pain.

Joint Instability: Joints can be more prone to instability, leading to sensations of joint “giving way” or feeling unsteady. This can make children apprehensive of movement and may affect participation in activities. Unstable joints and microtears in soft tissues can me a major source of chronic pain.

Delayed Motor Skills: In some cases, some infants with generalised joint hypermobility may have delays in achieving motor skills milestones, such as crawling, walking, or running. They will often present with hypotonia and increased flexibility.

Joint Subluxations and Dislocations: Whilst not universal, some children with joint hypermobility may experience joint dislocations (complete separation of joint surfaces) or subluxations (partial dislocation). These children need to be closely monitored and provided with supports due to the risk of further subluxations and dislocations.

Soft Tissue Injuries: Joints can be more susceptible to soft tissue injuries, such as muscle strains or ligament sprains.

Skin Differences: Some children with hypermobility associated with a connective tissue disorder may exhibit skin signs including stretch marks, abnormal scarring, stretchy skin, soft doughy skin or very fragile skin with poor wound healing.

Coordination Problems: Some children with hypermobility may appear to be clumsy, poorly coordinated and have regular unexplained bruises, particularly on their legs. This is due to changes in proprioception (our ability to know where our body is in space). There is an increase in prevalence of Developmental Coordination Disorder (DCD) in children with joint hypermobility compared to the rest of the population.

Recurrent Injuries: Children with hypermobility may be more prone to recurrent injuries during physical activities, especially those that involve impact or stress on joints.

Gastrointestinal Symptoms: In some cases, gastrointestinal symptoms, such as functional gastrointestinal disorders, may be associated with hypermobility.

Dysautonomia and ANS symptoms: Dysautonomia in childhood refers to a disruption in the autonomic nervous system, responsible for regulating involuntary bodily functions. Conditions like Postural Orthostatic Tachycardia Syndrome (POTS), Neurocardiogenic Syncope, Pure Autonomic Failure (PAF), Familial Dysautonomia (FD), and Multiple System Atrophy (MSA) can affect children. Symptoms range from increased heart rate upon standing to impaired sensory perception.

Anxiety and Depression: Children with symptomatic joint hypermobility are more prone to anxiety around movement and participation which can impact their mood and wellbeing.

Sensory Processing Issues: Children with generalised joint hypermobility may have sensory processing challenges including reduced proprioception and vestibular processing issues which might look like fear of movement or seeking excessive movement along with sensitives to sensory input (light, sound, touch).

Neurodiversity: There is emerging literature showing the correlation between a small population of children with ASD and ADHD that also have joint hypermobility. There is much more work to be done in the research space to demonstrate the clear link between these co-existing conditions.

What does hypermobility look like?

Castori, et al (2013)

As Paediatric Physiotherapists, how do we diagnose Joint Hypermobility?

Children UNDER the age of 5

• Babies, toddlers and young children have more flexibility and laxity in their joints in early life. Some of these children are very flexible- more so than their peers. They may be delayed in their motor milestones and have difficulty with postural control, chewing and swallowing, fine motor skills and participation in physical activities. These children should be closely monitored and further assessed at age 5 and above using the criteria below.

• Five years old is the minimum age at which generalized joint hypermobility should be assessed using the Beighton scale. Infants and toddlers do not have sufficient bone maturity to accurately diagnose generalized joint hypermobility. As physiotherapists, we can, however assess a little one’s joints for more than expected laxity / flexibility and provide strategies to build their strength and develop their motor skills.

• Infants and toddlers with hypermobility may also have hypotonia (low muscle tone) and receive this diagnosis as a baby or toddler following a clinical examination. In my experience, hypermobility and hypotonia commonly co-exist in young children. There is not a standardised test for hypotonia in infants, it’s based on a clinical examination by an experienced health professional.

Children aged 5 and OVER

Source: Ehlers Danlos Society  https://www.ehlers-danlos.com/wp-content/uploads/2022/11/banner_beighton_score_infographic_landscape-1.jpg

We use the above Beighton Index. It’s quick, easy and reliable. The most widely used tool used internationally to assess Joint hypermobility.

Want to assess your child at home using the Beighton Criteria? Check out our video https://www.youtube.com/watch?v=En0NcXi16Fw

What are the types of hypermobility in children?

Children may have Joint Hypermobility in one, a few, or many of their joints. Joint Hypermobility is classified by which joints are affected.

  • Generalized joint hypermobility: Joint hypermobility present in many different joints throughout the body – Beighton score of ≥ 6 in children.
  • Peripheral joint hypermobility: Joint Hypermobility limited to the hands and feet.
  • Localized Joint Hypermobility: Joint Hypermobility in a single joint or group of joints in the same area.
  • HSD – Hypermobility Spectrum Disorder: The hypermobility spectrum disorders (HSD) occur when a child has symptomatic joint hypermobility that cannot be explained by other conditions. A child with HSD may have joint instability as their only concern or may have other medical issues as well such as pain, fatigue, injuries, joint problems, autonomic nervous system issues and many more.

There are also many Hereditable connective tissue disorders (HCTD’s) of which Joint Hypermobility is a feature. Some of these include:

  • Ehlers Danlos Syndromes
  • Osteogenesis Imperfecta
  • Marfans Syndrome
  • Loeys-Dietz Syndrome

New assessment tool released in 2023 for Health Professionals.

Diagnostic Framework for Pediatric Joint Hypermobility designed to assess children aged 5-18 or biological maturation.
The Paediatric Working Group of the International Consortium on Ehlers-Danlos Syndromes (EDS) and Hypermobility Spectrum Disorders (HSD) met over 2020–2022 to review the challenges of diagnosing children and adolescents using the 2017 diagnostic criteria for hEDS which was developed for adults and not accurate for children.

In May 2023, a new diagnostic framework that can be used to assess children and adolescents from age five years old through to biological maturity. Biological maturity is marked by the completion of puberty and bone growth or when a person reaches eighteen years old, whichever happens first. This is the first assessment tool of its kind to assess joint hypermobility and associated symptoms specifically in children which is an exciting step to allow us, as health professionals to diagnose and provide appropriate supports for children.

Source: Ehlers Danlos Society https://www.ehlers-danlos.com/

Access the new assessment tool here:

2023 Diagnostic Framework for Pediatric Joint Hypermobility


* This tool is to be used by experienced health professionals. Diagnosis is to be confirmed with child’s medical professional (GP, Paediatrician, Rheumatologist).

Assessment by Physiotherapists

As Paediatric Physiotherapists at Ocean Kids Health, our role in assessing and managing Symptomatic Paediatric Joint Hypermobility has evolved with these new diagnostic criteria.
Here’s how we approach it:

1. We listen to your concerns and discuss how we can best help your child
2. Beighton Score Assessment: Using the age and gender-specific Beighton Score to quantify joint hypermobility.
3. Utilising the 2023 Diagnostic Framework for Paediatric Joint Hypermobility to provide a provisional diagnosis.
4. Symptom Evaluation: Assessing musculoskeletal and other symptoms and their impact your child’s daily life.
5. Physical Assessment: We complete a thorough physical examination including evaluating your child’s posture, strength and motor skills.
6. We then liaise with your child’s medical professional e.g., Paediatrician to confirm their diagnosis and to rule out other conditions which may be causing their symptoms.

Then we help!

Depending on your child’s needs and goals, we target our programs and strategies to improve your child’s wellbeing and participation with a big emphasis on Fun and Function!

Strengthening Exercises: We designing fun and personalized exercise programs to enhance muscle strength and stability around hypermobile joints.

Proprioception Training: We teach your child to improve their joint position awareness, aiding coordination and reducing the risk of falls or injuries.

Joint Protection Techniques: We providing strategies on how to protect hypermobile joints during physical activities.

Pain Management: We utilizing techniques like manual therapy, heat, or ice to alleviate pain and discomfort and provide taping and bracing supports.

Education and Lifestyle Modification: We empowering children and their families with knowledge about joint hypermobility and lifestyle adjustments alongside our amazing Exercise Physiologists.

Assistive Devices: We may recommend orthotics, garments or braces when necessary to provide additional joint support.

We love to liaise with our colleagues to support your child e.g., Paediatrician, GP, Exercise Physiologists, Podiatrist, OT, Speech Pathologist, Psychologist, Dietician

Physiotherapy Top Tips for Managing Hypermobility in Childhood
  1. Seek an accurate assessment and diagnosis from a Paediatric Physiotherapist, Paediatrician, Rheumatologist or General Practitioner – find a practitioner in your area here
    https://www.ehlers-danlos.com/healthcare-professionals-directory/
  2. Remember, it’s not always a bad thing. Hypermobility can be very beneficial for certain sports and activities. Most children will NOT be symptomatic. It is the small group of kids who develop symptoms that need our support.
  3. Get educated – Head to https://www.ehlers-danlos.com/diagnostic-criteria/new-diagnostic-framework-for-pediatric-joint-hypermobility-v2/ and https://www.hypermobility.org/
  4. Encourage activities to help your child build and maintain a strong body through low impact strength activities: These include climbing, swimming, dancing, bike riding, kids yoga and lots more.
  5. Allow sufficient rest! Avoid having an activity on every night after school and have some downtime on the weekends. Full body rest during the day can help to manage fatigue, particularly in a beanbag!
  6. Manage pain and injuries well straight away to prevent recurrence, chronic pain and fatigue developing.
  7. Connect: There are many people living with hypermobility to connect with.  Find other parents to get support here: https://www.hypermobilityconnect.com/
For Parents:

If your child is flexible and you are concerned about their symptoms, please reach out, we’d love to help you. https://www.oceankidshealth.com.au/contact/

References:

Beighton, P. H., Grahame, R., & Bird, H. (2011). Hypermobility of joints. Springer Science & Business Media.

Castori, Marco. (2013). Joint hypermobility syndrome (a.k.a. Ehlers-Danlos Syndrome, Hypermobility Type): An updated critique. 148. 13-36.

Castori, M., Tinkle, B., Levy H, et al. (2017). A framework for the classification of joint hypermobility and related conditions. Am J Med Genet C Semin Med Genet; 175. 148-157.

Englelbert R, Bjuul-Kristensen, Pacey V, de Wandelle I, Smeek S, Scheper M, Russek L, Simmonds JV (2017). The evidence- based rationale for physical therapy of children, adolescents and adults with Joint Hypermobility Syndrome/ Ehlers Danlos Syndrome – Hypermobility type. American Journal of Medical Genetics. Part C Medical Seminars

Ehlers-Danlos Society. https://www.ehlers-danlos.com

Nicholson, L., Chan, C., Tofts, L., & Pacey. V. (2022). Hypermobility Syndromes in children and adolescents. Assessment, Diagnosis and multidisciplinary management. AJGP. Volume 51, 6.
O’Sullivan, M. Hypermobility Connect. https://www.hypemobilityconnect.com/the-spectrum-of-hypermobility/

Tofts, L.J., Elliott, E.J., Munns, C. et al. (2009). The differential diagnosis of children with joint hypermobility: a review of the literature. Pediatr Rheumatol 7, 1. https://doi.org/10.1186/1546-0096-7-1

Veriki et al (2021). American J of Med Genetics Pt C. Parental perceptions of bladder dysfunction in children with symptomatic joint hypermobility. 187C:586-592

Wesley et al (2020). J Paediatrics Child Health. Impact of heritable disorders of connective tissue on daily life of children: Parent Perspectives. 57: 626-630

Our Ocean Kids Health team are here to help you and your child thrive!