Neurological and medical

Ehlers-Danlos Syndromes in Children: Signs, Diagnosis, and Support

Why the distinction between hEDS and HSD is diagnostic, not a measure of severity.

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Written and reviewed by the DrSensory editorial team, drawing on the 2017 international classification of the Ehlers-Danlos syndromes, the Ehlers-Danlos Society, and physical therapy management guidance. Sources are listed at the foot of the page. · Last updated August 28, 2026

The Ehlers-Danlos syndromes are a group of inherited connective tissue conditions affecting collagen, producing joint hypermobility, skin differences, and tissue fragility. The 2017 international classification recognizes 13 subtypes. Twelve of them are confirmed by genetic testing; hypermobile EDS is the exception, diagnosed on clinical criteria alone because no causative gene has been identified. Hypermobility spectrum disorders (HSD) were introduced in the same classification for symptomatic joint hypermobility that doesn't meet the criteria for hEDS or another connective tissue condition. The 2017 criteria have been widely criticized, and a revised classification is scheduled for publication on December 2, 2026.

What the Ehlers-Danlos syndromes are

Collagen provides structure and strength to skin, joints, blood vessels, and organs. In the Ehlers-Danlos syndromes, a difference in collagen or in the proteins that process it affects those tissues. The 2017 international classification recognizes 13 subtypes, replacing the 1998 Villefranche nosology of six.1 A crucial distinction: all subtypes except the hypermobile type are confirmed by identifying a causative genetic variant. Hypermobile EDS has no identified gene, so it is diagnosed on clinical criteria — the source of most diagnostic difficulty families experience. Hypermobility spectrum disorders (HSD) were introduced in the same classification for symptomatic joint hypermobility that doesn't meet the criteria for hEDS or another heritable connective tissue disorder.

In practice, HSD is often the more common outcome. In specialist cohorts assessed against the 2017 criteria, the majority of hypermobile patients are classified with HSD rather than hEDS.2 One study reapplied the 2017 criteria to 327 patients from 213 families who had been diagnosed under the older Villefranche and Brighton criteria, and concluded that the criteria are too stringent — placing in HSD a large group who would be better captured within the hEDS spectrum. HSD is not "EDS lite" — a prospective cohort comparing the two found the same spectrum of extra-articular problems and similar overall severity, differing only on pain, movement difficulty and bleeding3 — people with HSD can be affected as significantly as those meeting hEDS criteria. The distinction is diagnostic, not a measure of how much someone is affected.

The criteria are changing

The 2017 criteria have been criticized as too stringent in places, too vague in others, and not capturing how hEDS and HSD actually present. The Road to 2026 initiative, led by the International Consortium on the Ehlers-Danlos Syndromes and Hypermobility Spectrum Disorders, has been reviewing the classification, drawing on research including HEDGE (Hypermobile Ehlers-Danlos Genetic Evaluation), the largest genetic study of hEDS to date.5 A revised classification is scheduled for publication on December 2, 2026, in the American Journal of Medical Genetics, with a diagnostic pathway and hEDS/HSD management guidance scheduled to follow in 2027. As with any scheduled publication, these dates may change.

What this means for families now: the 2017 criteria remain current until then; if you've been told you don't meet hEDS criteria, that may change; and it's reasonable to ask your clinician whether waiting for the revised criteria is sensible in your case. Support and management don't depend on which label applies — treatment for symptomatic hypermobility is broadly the same either way.

Signs in children

Joints — hypermobility beyond typical range; joint pain, often worse after activity; subluxations and dislocations; frequent sprains; a "giving way" feeling; clicking and popping. Skin — soft or velvety; stretchiness; easy bruising; slow or unusual wound healing; scars that widen. Movement and coordination — reduced proprioception (less accurate awareness of limb position); clumsiness; poor balance; poor handwriting endurance. Fatigue — very commonly reported and frequently underestimated; holding unstable joints together takes continuous muscular effort. Also frequently reported: gastrointestinal symptoms; dizziness on standing and other features of dysautonomia including POTS; anxiety; sleep difficulty; and headaches.

Note on hypermobility generally. Many children are hypermobile and most have no associated difficulty. Hypermobility alone isn't EDS. What raises the question is hypermobility with symptoms — pain, instability, fatigue, injuries — or with skin or other features.

Hypermobility, autism, ADHD, and sensory processing

There is a documented association between hypermobility conditions and neurodivergence. A retrospective study of 201 children with HSD or hEDS found that 16% had a confirmed ADHD diagnosis with a further 7% under assessment, and 6% had autism — both above general-population rates.4 The association is real; the explanation isn't settled. More useful is the practical overlap: reduced proprioceptive accuracy is common in hypermobility and is also a sensory-processing pattern (vestibular and proprioceptive processing); differences in interoception affect noticing pain, fatigue, and hunger in time (interoception); and the management approach for hypermobility fatigue shares much with autistic burnout — recognizing limits before they're exceeded, and building in recovery.

How are Ehlers-Danlos syndromes diagnosed?

For 12 of the 13 subtypes, diagnosis rests on genetic testing identifying a causative variant, usually alongside clinical assessment. For hypermobile EDS, diagnosis is clinical, using the 2017 criteria — assessment of generalized joint hypermobility using the Beighton score, additional systemic features and family history, and exclusion of other conditions. Diagnosis in children is harder than in adults, because joint range naturally decreases with age and some features emerge over time; some clinicians reassess as a child grows, which shouldn't delay management.

Management — and one thing to get right

Support doesn't wait for a diagnosis; symptomatic hypermobility is managed similarly whether the label is hEDS or HSD. Physical therapy is central, and the approach matters — what helps is strengthening (particularly around unstable joints), proprioceptive and balance work, graded activity, pacing, and postural support.

Be cautious about stretching-focused programs

In a hypermobile child, joints already move beyond typical range, so approaches centered on increasing flexibility can worsen instability. If a physical-therapy program is mostly stretching, raise it — a therapist familiar with hypermobility will focus on stability and control.

Occupational therapy — joint protection, handwriting endurance, seating, adaptive equipment, fatigue management (find an occupational therapist). Pain is best addressed early. Medical monitoring depends on subtype — some require cardiovascular monitoring, and vascular EDS in particular requires specialist care, which is exactly why knowing the subtype matters. Find a physical therapist.

Therapy for Ehlers-Danlos syndromes

What each discipline actually does — assessment, what sessions look like, and what the evidence supports.

Frequently Asked Questions

Are the criteria changing?

Yes. A revised classification is scheduled for publication on December 2, 2026, following a multi-year review that includes the HEDGE genetic study, though scheduled publication dates can change. The 2017 criteria remain current until then.

How is hypermobile EDS diagnosed?

Clinically, using the 2017 criteria — unlike the other 12 subtypes, no causative gene has been identified, so there's no confirmatory genetic test. Diagnosis involves assessing generalized joint hypermobility, additional systemic features, family history, and excluding other conditions.

Is EDS linked to autism and ADHD?

Research has found higher rates of ADHD and autism among children with hypermobility conditions than in the general population.4 The association is documented; the explanation isn't settled.

What's the difference between hEDS and HSD?

Hypermobility spectrum disorder is diagnosed where someone has symptomatic joint hypermobility that doesn't meet the criteria for hEDS or another heritable connective tissue condition. Research has found substantially overlapping symptoms, and HSD is the more common diagnosis under the 2017 criteria.3 It isn't a milder version.

How many types of Ehlers-Danlos syndrome are there?

The 2017 international classification recognizes 13 subtypes. A revised classification is scheduled for publication on December 2, 2026.

Should my hypermobile child do stretching exercises?

Raise it with a physical therapist familiar with hypermobility. In a child whose joints already move beyond typical range, stretching-focused programs can worsen instability. Strengthening, proprioceptive work, and pacing are generally the focus.

Sources

  1. Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J, et al. The 2017 international classification of the Ehlers-Danlos syndromes. American Journal of Medical Genetics Part C. 2017;175(1):8–26. doi:10.1002/ajmg.c.31552 (PMID 28306229)
  2. Ritelli M, Chiarelli N, Cinquina V, Vezzoli M, Venturini M, Colombi M. Looking back and beyond the 2017 diagnostic criteria for hypermobile Ehlers-Danlos syndrome: a retrospective cross-sectional study from an Italian reference center. American Journal of Medical Genetics Part A. 2024;194(2):174–194. doi:10.1002/ajmg.a.63426 (PMID 37774134)
  3. Aubry-Rozier B, Schwitzguebel A, Valerio F, Tanniger J, Paquier C, Berna C, et al. Are patients with hypermobile Ehlers-Danlos syndrome or hypermobility spectrum disorder so different? Rheumatology International. 2021;41(10):1785–1794. doi:10.1007/s00296-021-04968-3 (PMID 34398260)
  4. Kindgren E, Quiñones Perez A, Knez R. Prevalence of ADHD and autism spectrum disorder in children with hypermobility spectrum disorders or hypermobile Ehlers-Danlos syndrome: a retrospective study. Neuropsychiatric Disease and Treatment. 2021;17:379–388. doi:10.2147/NDT.S290494 (PMID 33603376)
  5. The Ehlers-Danlos Society — Road to 2026 and the HEDGE (Hypermobile Ehlers-Danlos Genetic Evaluation) study. Checked August 18, 2026.

Medical disclaimer. This page is for general educational purposes and does not constitute medical advice, diagnosis, or treatment. If your child has lost skills they previously had, contact their doctor promptly.