Assessment of hypermobility

Bug · Oct 27, 2014

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After spending time just reading some of the new studies coming out with unsurprising results, I continue to be surprised though when chatting with colleagues how few podiatrists actually use a formal scale or any tests for hypermobility especially when working with children.

I commonly use the Beighton with every child and anyone score over a 5, I'll do the LLAS. Anyone use anything else or I'm interested to understand why they aren't being used?

Admin2 · Oct 27, 2014

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Tariff Hogan Jinn · Dec 15, 2014

I prefer the Bulbena et al. 1992 (Hospital Del Mar) hypermobility measure for general/global over the Beighton and always perform Jill's LLAS as part of my standard assessment because I always check hips, knees, ankles, stj, mtj, ect. that are the components anyway.

The reason its not utilized is our professions limited scope of reading as the majority do not have access to the relevant journals, Angela's recent papers in JAFAR looking at reliability have helped disseminate somewhat but we lack authoritive accessible discipline specific texts or even clinical pearls like physiopedia and orthobullets which I write for a fair bit.

Any Q's fire away

NewsBot · Dec 9, 2021

Outcome measures for assessing change over time in studies of symptomatic children with hypermobility: a systematic review
Muhammad Maarj, Andrea Coda, Louise Tofts, Cylie Williams, Derek Santos & Verity Pacey
BMC Pediatrics volume 21, Article number: 527 (2021)

Background
Generalised joint hypermobility (GJH) is highly prevalent among children and associated with symptoms in a fifth with the condition. This study aimed to synthesise outcome measures in interventional or prospective longitudinal studies of children with GJH and associated lower limb symptoms.

Methods
Electronic searches of Medline, CINAHL and Embase databases from inception to 16th March 2020 were performed for studies of children with GJH and symptoms between 5 and 18 years reporting repeated outcome measures collected at least 4 weeks apart. Methodological quality of eligible studies were described using the Downs and Black checklist.

Results
Six studies comprising of five interventional, and one prospective observational study (total of 388 children) met the inclusion criteria. Interventional study durations were between 2 and 3 months, with up to 10 months post-intervention follow-up, while the observational study spanned 3 years. Three main constructs of pain, function and quality of life were reported as primary outcome measures using 20 different instruments. All but one measure was validated in paediatric populations, but not specifically for children with GJH and symptoms. One study assessed fatigue, reporting disabling fatigue to be associated with higher pain intensity.

Conclusions
There were no agreed sets of outcome measures used for children with GJH and symptoms. The standardisation of assessment tools across paediatric clinical trials is needed. Four constructs of pain, function, quality of life and fatigue are recommended to be included with agreed upon, validated, objective tools.
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NewsBot · May 11, 2023

Pediatric joint hypermobility: a diagnostic framework and narrative review
Louise Jane Tofts, Jane Simmonds, Sarah B. Schwartz, Roberto M. Richheimer, Constance O’Connor, Ellen Elias, Raoul Engelbert, Katie Cleary, Brad T. Tinkle, Antonie D. Kline, Alan J. Hakim, Marion A. J. van Rossum & Verity Pacey
Orphanet Journal of Rare Diseases volume 18, Article number: 104 (2023)

Background
Hypermobile Ehlers–Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD) are debilitating conditions. Diagnosis is currently clinical in the absence of biomarkers, and criteria developed for adults are difficult to use in children and biologically immature adolescents. Generalized joint hypermobility (GJH) is a prerequisite for hEDS and generalized HSD. Current literature identifies a large proportion of children as hypermobile using a Beighton score ≥ 4 or 5/9, the cut off for GJH in adults. Other phenotypic features from the 2017 hEDS criteria can arise over time. Finally, many comorbidities described in hEDS/HSD are also seen in the general pediatric and adolescent population. Therefore, pediatric specific criteria are needed. The Paediatric Working Group of the International Consortium on EDS and HSD has developed a pediatric diagnostic framework presented here. The work was informed by a review of the published evidence.

Observations
The framework has 4 components, GJH, skin and tissue abnormalities, musculoskeletal complications, and core comorbidities. A Beighton score of ≥ 6/9 best identifies children with GJH at 2 standard deviations above average, based on published general population data. Skin and soft tissue changes include soft skin, stretchy skin, atrophic scars, stretch marks, piezogenic papules, and recurrent hernias. Two symptomatic groups were agreed: musculoskeletal and systemic. Emerging comorbid relationships are discussed. The framework generates 8 subgroups, 4 pediatric GJH, and 4 pediatric generalized hypermobility spectrum disorders. hEDS is reserved for biologically mature adolescents who meet the 2017 criteria, which also covers even rarer types of Ehlers–Danlos syndrome at any age.

Conclusions
This framework allows hypermobile children to be categorized into a group describing their phenotypic and symptomatic presentation. It clarifies the recommendation that comorbidities should be defined using their current internationally accepted frameworks. This provides a foundation for improving clinical care and research quality in this population.
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