Authors
- Holtze, Dorte Buch PT, MPH
- Jessen-Winge, Christina OT, MSc
- Hansen, Karsten Bruun PT, PhD
Abstract
Review question/objective: The objective of this review is to identify what effect a systematic intervention focused on functional stability has on the level of pain (measured by pain scales) in patients with Joint Hypermobility Syndrome (JHS) compared to general exercise.
Background: Joint Hypermobility is defined as a condition with an abnormal range of movement (ROM) in one or more of the synovial joints of an individual. The condition can be hereditary, acquired through a trauma or developed voluntarily through training.1-3
Originally Joint Hypermobility was perceived as a solely local articular problem in an otherwise healthy person. However in recent decades the perception is that it is important to differentiate between General Hypermobility (GH) and Joint Hypermobility Syndrome (JHS).4,5 In GH, the condition is solely classified according to the extensive ROM, whereas JHS is a condition where alongside GH both subjective complaints of musculoskeletal problems and other objective findings such as abnormal connective tissue formation are considered.1,3,4 JHS is considered a benign condition and is distinguished from the joint hypermobility which is a feature of more rare and malign medical conditions such as Ehlers-Danlos syndrome (EDS) (type I and II), Marfan syndrome and Osteogenesis Imperfecta. The type III of EDS is considered benign and indistinguishable from JHS.1,4,6
Symptoms of JHS are related to poor coordination, reduced proprioception, muscle weakness and reduced endurance in both adults and children.7-10 These factors lead to poor posture and decreased functional stability, causing a tendency to rest at the end of the joint range. This position of the joint leads to stress and strain in the supporting structures of the joint, producing fatigue and pain.11,12 Symptoms can be significant, including exercise-related/post-exercise related pain, nocturnal leg pain, recurrent foot/ankle, knee and back arthralgia, joint swelling and stiffness, clumsiness as well as pain and fatigue in the hand, wrist or lower arm associated with writing.6,7 Also due to the joints' capability of excessive ROM, people with JHS are susceptible to injury such as dislocation and sprains.4
Historically, a number of scoring systems have been used to identify the presence of GH of which the Beighton nine-point scoring system has been the most widely used.17 A score of >=4 of 9 points is usually taken to be indicative of widespread GH.4 However the scale was designed for epidemiological use,18 and is not advised to be used as a single measure in diagnosing JHS.4,19
In response to recognition of the need for a better diagnostic tool, alongside the understanding of the more multi-factorial problems in the diagnosis of JHS compared to GH, the Brighton criteria was introduced in 1998.4,20 This set of criteria takes account of not only the presence of GH, but also includes both subjective and objective findings. The Brighton criteria for JHS include two major and/or eight minor criteria in different combinations. The two major Brighton criteria are a Beighton score >=4 out of 9 (either currently or historically) and arthralgia in >=four joints for more than three months, while the eight minor criteria are: considering a lower Beighton and arthralgia score, dislocation/subluxation in one or more joints, soft tissue rheumatism, Marfanoid habitus, abnormal skin, eye signs and varicose veins, hernia or uterine/rectal prolapse.1,4,20,21
Most studies have looked at the prevalence of GH in pediatric populations; however because of the variation of diagnostic methods in these studies, the exact number is unknown. At the same time, no studies have distinguished between GH and JHS. The prevalence worldwide in children aged 4-17 years varies from 2-55 % (depending on age), using the Beighton criteria >= 4/9.5, 6 As an example, the prevalence in Danish schoolchildren aged eight using Beighton >=4 is shown to be 29%, Beighton >=5 is 19% and Beighton >=6 is 10%. If using the Brighton criteria the prevalence was 9%.5 A recent study on British schoolchildren (age 13) showed a prevalence of 27.5% in girls and 10.6% in boys using Beighton >= 4/9.13
Several studies suggest that JHS is strongly related to age (where GH decreases with age),14 gender (with a higher prevalence in females than males;13-15 however these findings are not shown in all studies),5 and ethnicity (with a higher prevalence in patients from Afro and Asian origin compared to Caucasians).14,16 These factors need to be taken into account for comparison between studies.
There is still a lot of dogma in the handling of JHS and the evidence in relation to the effect of treatment on the level of pain has been lacking for both children and adults.1,3 An example is taken from the Danish website for patients who are hypermobile in 2011:"Several well-known specialists say that training doesn't help, however many patients experience a relief of pain during exercise".22
The commonly used physiotherapeutic approach is thus to improve general muscle strength and increase endurance through graded exercises. However a recent review indicates that an intervention focused on functional stability including specific motor-skill training to improve posture, proprioception and strength combined with providing full ROM has a beneficial effect in the management of adult patients' symptoms.11 This however was not a systematic review so the ability to interpret the results is limited.
An initial literature search in PubMed, PEDro, CINAHL, Cochrane Library and Joanna Briggs Library has not provided a detailed systematic review of the effect on level of pain in patients with JHS using interventions focused on functional stability compared to general interventions in adults, children or adolescents. A systematic review will provide a valuable tool for therapists to help optimize training to reduce pain for patients with JHS, who are at risk of an un-focused and deficient treatment which is costly for both the patient and society.
Due to ongoing cognitive, psychosocial and physical growth in children and adolescents, they cannot be considered 'small adults' and should not be managed as such.23 However, due to the small amount of literature available concerning the treatment of JHS, this review will consider patients of all ages. Furthermore, although GH decreases with age, other JHS-symptoms persist in adulthood, especially pain, as such treatment is still relevant.3, 24
Article Content
Inclusion criteria
Types of participants
This review will consider studies that include adults, adolescents and children of all ages with JHS diagnosed using the Brighton criteria.
Ehlers-Danlos syndrome (EDS) type III will be included and since it is indistinguishable from JHS,1,4,6 it will have no implications in the interpretation of data.
The diagnostic and treatment procedure can be done in either an outpatient facility or a hospital.
Other diagnoses and syndromes which present joint hypermobility and abnormal connective tissue formation as a part of the clinical picture, but are more malign such as Marfan syndrome (MF) and Osteogenesis Imperfecta (OI) will be excluded. Also EDS type I and II will be excluded due to other and more severe pathological complications beyond hypermobility.19
Types of intervention(s)
This review will consider studies that evaluate interventions that focus on functional stability in patients with JHS compared to general exercise.
Types of outcomes
This review will consider studies that include the following outcome measures:
The level of pain in patients with JHS, measured with pain scales.
Types of studies
This review will consider both experimental and epidemiological study designs including randomized controlled trials, non-randomized controlled trials, quasi-experimental, before and after studies, prospective and retrospective cohort studies, case control studies and analytical cross sectional studies for inclusion.
This review will also consider descriptive epidemiological study designs including case series, individual case reports and descriptive cross sectional studies for inclusion.
Search strategy
The search strategy aims to find both published and unpublished studies. A three-step search strategy will be utilized in this review. An initial limited search of PubMed and CINAHL will be undertaken followed by analysis of the text words contained in the title and abstract, and of the index terms used to describe the articles. A second search using all identified keywords and index terms will then be undertaken across all included databases. Thirdly, the reference lists of all identified reports and articles will be searched for additional studies. Studies published in English, Danish, Swedish, Norwegian and German will be considered for inclusion in this review. Studies published from 1998-2013 will be considered for inclusion in this review; 1998 is chosen because of the introduction of the Brighton criteria for JHS in the clinical setting.
The databases to be searched include:
PubMed
PEDro
CINAHL
Cochrane Library
EMBASE
Joanna Briggs Library
OT Seeker
NHS Evidence
The search for Grey Literature will include:
AHRQ, Evidence Reports, Dissertation Abstracts, Google Scholar, SIGLE, The New York Academy of Medicine Gray Literature Report, Scirus, Mednar, fysio.dk, UCviden.dk, patients organizations, contact with professionals from the area.
Initial keywords to be used will be:
Joint hypermobility, Joint laxity, Hypermobility Syndrome, Joint hypermobility syndrome, JHS,
Management, Treatment, Exercise, Training, Rehabilitation, Physi* therap*, Stability, Proprioception, Posture, Functional stability
Pain, Pain measurement, Brighton Criteria
Assessment of methodological quality
Papers selected for retrieval will be assessed by two independent reviewers for methodological validity prior to inclusion in the review using standardized critical appraisal instruments from the Joanna Briggs Institute Meta-Analysis of Statistics Assessment and Review Instrument (JBI-MAStARI) (Appendix I). Any disagreements that arise between the reviewers will be resolved through discussion, or with a third reviewer.
Data collection
Data will be extracted from papers included in the review using the standardized data extraction tool from JBI-MAStARI (Appendix II). The data extracted will include specific details about the interventions, populations, study methods and outcomes of significance to the review question and specific objectives.
Data synthesis
Quantitative data will, where possible, be pooled in statistical meta-analysis using JBI-MAStARI. All results will be subject to double data entry. Effect sizes expressed as odds ratios (for categorical data) and weighted mean differences (for continuous data) and their 95% confidence intervals will be calculated for analysis. Heterogeneity will be assessed statistically using the standard Chi-square and also explored using subgroup analyses based on the different study designs included in this review. Where statistical pooling is not possible, the findings will be presented in narrative form including tables and figures to aid in data presentation where appropriate.
Conflicts of interest
None known
References
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Appendix I: Appraisal instruments
MAStARI appraisal instrument[Context Link]
Appendix II: Data extraction instruments
MAStARI data extraction instrument[Context Link]
Keywords: functional stability; joint hypermobility syndrome; pain; physical therapy; treatment