SYMPTOMATIC HYPERMOBILITY IN CHILDREN AND YOUNG PEOPLE: A SCOPING REVIEW OF CLINICAL CHARACTERISTICS USING A DEVELOPMENTAL FRAMEWORK

S. Ward¹, E.J. MacDermott², J. Simmonds³, J. Deane⁴, D. Mockler⁵, S. Dockrell¹

¹Trinity College Dublin, Discipline of Physiotherapy, School of Medicine, Dublin, Ireland, ²Children’s Health Ireland, National Centre of Paediatric Rheumatology, Dublin, Ireland, ³University College London, Great Ormond Street Institute of Child Health, London, United Kingdom, ⁴University of Birmingham and Department of Health Sciences, School of Sports, Exercise and Rehabilitation Sciences, Birmingham, United Kingdom, ⁵Trinity College Dublin, Trinity Centre for Health Sciences, Dublin, Ireland

Background: Symptomatic Hypermobility is a term used to describe the symptoms associated with joint hypermobility. Synonymous terms used include hypermobile Ehlers Danlos Syndrome and Hypermobility Spectrum Disorders. A range of musculoskeletal, gastrointestinal, cardiovascular, psychological, and urogenital characteristics are reported to be associated with Symptomatic Hypermobility. However, varied terminology across different clinical settings makes interpretation difficult in children and young people.

Purpose: The objective of this systematic scoping review was to evaluate the literature on children and young people with Symptomatic Hypermobility using a developmental framework. This review aimed to provide clarity for clinicians assessing and managing children and young people with Symptomatic Hypermobility, especially physiotherapists, who often play a key role.

Methods: The protocol was registered prospectively, according to the PRISMA-ScR guidelines. A search was conducted in EMBASE, Medline, CINAHL, Web of Science and grey literature. Full text articles with a confirmed diagnosis of Symptomatic Hypermobility, (including Hypermobility Spectrum Disorder or hypermobile Ehlers Danlos Syndrome) using internationally recognised criteria were included. Title and abstract screening and full text reviewing were conducted by two independent reviewers. Data extraction forms were piloted (two independent reviewers randomly selected n =20, approximately 12%) and were later categorised into clinical domains, and where possible, developmental stage. Children and young people were defined as; children (0-9years), adolescents (10-19years), and young adults (20-24years).

Results: Of the1619 studies screened, 163 were included in the scoping review. Studies were published from 1967-2021, 30% were narrative reviews and 24% were case reports, from a range of journals. There was only one systematic review specific to children, and it included only two studies. Musculoskeletal and cutaneous characteristics were common in all developmental stages. Cardiovascular characteristics including autonomic dysfunction, fatigue, and gastrointestinal issues were reported more frequently by adolescents. Young adult studies were scarce and included mostly case reports. Varied diagnostic criteria and assessment methods were identified. The Beighton Score was frequently used (93%), but rarely standardised (12%) and lacked consensus regarding the cut-off point of hypermobility. Gaps in evidence and specific needs for further research were identified.

Conclusions: This is the first systematic scoping review to identify the emerging phenotype of symptomatic hypermobility from childhood to young adulthood. This review provided evidence of the lack of guidance regarding the utilisation of standardised tools to assess hypermobility in clinical practice and research. The numerous and inconsistent diagnostic criteria and assessment methods were synthesised. Given the changing temporal pattern of symptoms, longitudinal studies and large case control studies are warranted. Such studies should have bespoke criteria and use standardised assessment protocols so that findings from different studies can be compared and data pooled for future analysis.

Implications: The identification of clear clinical characteristics could assist with the early identification of Symptomatic Hypermobility in children and young people and direct appropriate management.In future, this couldbe achieved through enhanced education of paediatric clinicians in primary and community care.

Keywords:
Joint hypermobility
symptoms
clinical characteristics

Topics:
Paediatrics
Musculoskeletal
Rheumatology

Did this work require ethics approval? No

Reason: Ethics approval is not required for this Scoping Review as human participants are not involved.