Verity Pacey

Generalized Joint Hypermobility and Risk of Lower Limb Joint Injury During Sport A Systematic Review With Meta-Analysis

Background: Generalized joint hypermobility is a highly prevalent condition commonly associated with joint injuries. The current literature has conflicting reports of the risk of joint injury in hypermobile sporting participants compared with their nonhypermobile peers. Systematic reviews have not been conclusive and no meta-analysis has been performed. Purpose: This review was undertaken to determine whether individuals with generalized joint hypermobility have an increased risk of lower limb joint injury when undertaking sporting activities. Study Design: Systematic review with meta-analysis. Methods: Studies were identified through a search without language restrictions of PubMed, CINAHL, Embase, and SportDiscus databases from the earliest date through February 2009 with subsequent handsearching of reference lists. Inclusion criteria for studies were determined before searching and all included studies underwent methodological quality assessment by 2 independent reviewers. Meta-analyses for joint injury of the lower limb, knee, and ankle were performed using a random effects model. The difference in injury proportions between hypermobility categories was tested with the z statistic. Results: Of 4841 identified studies, 18 met all inclusion criteria with methodological quality ranging from 1 of 6 to 5 of 6. A variety of tests of hypermobility and varied cutoff points to define the presence of generalized joint hypermobility were used, so the authors determined a standardized cutoff to indicate generalized joint hypermobility. Using this criterion, a significantly increased risk of knee joint injury for hypermobile and extremely hypermobile participants compared with their nonhypermobile peers was demonstrated (P < .001), whereas no increased risk was found for ankle joint injury. For knee joint injury, a combined odds ratio of 4.69 (95% confidence interval, 1.33-16.52; P = .02) was calculated, indicating a significantly increased risk for hypermobile participants playing contact sports. Conclusion: Sport participants with generalized joint hypermobility have an increased risk of knee joint injury during contact activities but have no altered risk of ankle joint injury.

Generalized Joint Hypermobility and Risk of Lower Limb Joint Injury During Sport

Background: Generalized joint hypermobility is a highly prevalent condition commonly associated with joint injuries. The current literature has conflicting reports of the risk of joint injury in hypermobile sporting participants compared with their nonhypermobile peers. Systematic reviews have not been conclusive and no meta-analysis has been performed. Purpose: This review was undertaken to determine whether individuals with generalized joint hypermobility have an increased risk of lower limb joint injury when undertaking sporting activities. Study Design: Systematic review with meta-analysis. Methods: Studies were identified through a search without language restrictions of PubMed, CINAHL, Embase, and SportDiscus databases from the earliest date through February 2009 with subsequent handsearching of reference lists. Inclusion criteria for studies were determined before searching and all included studies underwent methodological quality assessment by 2 independent reviewers. Meta-analyses for joint injury of the lower limb, knee, and ankle were performed using a random effects model. The difference in injury proportions between hypermobility categories was tested with the z statistic. Results: Of 4841 identified studies, 18 met all inclusion criteria with methodological quality ranging from 1 of 6 to 5 of 6. A variety of tests of hypermobility and varied cutoff points to define the presence of generalized joint hypermobility were used, so the authors determined a standardized cutoff to indicate generalized joint hypermobility. Using this criterion, a significantly increased risk of knee joint injury for hypermobile and extremely hypermobile participants compared with their nonhypermobile peers was demonstrated (P < .001), whereas no increased risk was found for ankle joint injury. For knee joint injury, a combined odds ratio of 4.69 (95% confidence interval, 1.33-16.52; P = .02) was calculated, indicating a significantly increased risk for hypermobile participants playing contact sports. Conclusion: Sport participants with generalized joint hypermobility have an increased risk of knee joint injury during contact activities but have no altered risk of ankle joint injury.

The differential diagnosis of children with joint hypermobility: a review of the literature

BackgroundIn this study we aimed to identify and review publications relating to the diagnosis of joint hypermobility and instability and develop an evidence based approach to the diagnosis of children presenting with joint hypermobility and related symptoms.MethodsWe searched Medline for papers with an emphasis on the diagnosis of joint hypermobility, including Heritable Disorders of Connective Tissue (HDCT).Results3330 papers were identified: 1534 pertained to instability of a particular joint; 1666 related to the diagnosis of Ehlers Danlos syndromes and 330 related to joint hypermobility.There are inconsistencies in the literature on joint hypermobility and how it relates to and overlaps with milder forms of HDCT. There is no reliable method of differentiating between Joint Hypermobility Syndrome, familial articular hypermobility and Ehlers-Danlos syndrome (hypermobile type), suggesting these three disorders may be different manifestations of the same spectrum of disorders. We describe our approach to children presenting with joint hypermobility and the published evidence and expert opinion on which this is based.ConclusionThere is value in identifying both the underlying genetic cause of joint hypermobility in an individual child and those hypermobile children who have symptoms such as pain and fatigue and might benefit from multidisciplinary rehabilitation management.Every effort should be made to diagnose the underlying disorder responsible for joint hypermobility which may only become apparent over time. We recommend that the term Joint Hypermobility Syndrome is used for children with symptomatic joint hypermobility resulting from any underlying HDCT and that these children are best described using both the term Joint Hypermobility Syndrome and their HDCT diagnosis.

Interventions for congenital talipes equinovarus (clubfoot)

BACKGROUNDnCongenital talipes equinovarus (CTEV), which is also known as clubfoot, is a common congenital orthopaedic condition characterised by an excessively turned in foot (equinovarus) and high medial longitudinal arch (cavus). If left untreated it can result in long-term disability, deformity and pain.xa0Interventions can be conservative (such as splinting or stretching) or surgical. The review was first published in 2012 and we reviewed new searches in 2013 (update published 2014).nnnOBJECTIVESnTo evaluate the effectiveness of interventions for CTEV.nnnSEARCH METHODSnOn 29 April 2013, we searched CENTRAL (2013, Issue 3 in The Cochrane Library), MEDLINE (January 1966 to April 2013), EMBASE (January 1980 to April 2013), CINAHL Plus (January 1937 to April 2013), AMED (1985 to April 2013), and the Physiotherapy Evidence Database (PEDro to April 2013). We also searched for ongoing trials in the WHO International Clinical Trials Registry Platform (2006 to July 2013) and ClinicalTrials.gov (to November 2013). We checked the references of included studies. We searched NHSEED, DARE and HTA for information for inclusion in the Discussion.nnnSELECTION CRITERIAnRandomised controlled trials (RCTs) and quasi-RCTs evaluating interventions for CTEV. Participants were people of all ages with CTEV of either one or both feet.nnnDATA COLLECTION AND ANALYSISnTwo authors independently assessed risk of bias in included trials and extracted the data. We contacted authors of included trials for missing information. We collected adverse event information from trials when it was available.nnnMAIN RESULTSnWe identified 14 trials in which there were 607 participants; one of the trials was newly included at this 2014 update. The use of different outcome measures prevented pooling of data for meta-analysis even when interventions and participants were comparable. All trials displayed bias in four or more areas. One trial reported on the primary outcome of function, though raw data were not available to be analysed. We were able to analyse data on foot alignment (Pirani score), a secondary outcome, from three trials. Two of the trials involved participants at initial presentation. One reported that the Ponseti technique significantly improved foot alignment compared to the Kite technique. After 10 weeks of serial casting, the average total Pirani score of the Ponseti group was 1.15 (95% confidence interval (CI) 0.98 to 1.32) lower than that of the Kite group. The second trial found the Ponseti technique to be superior to a traditional technique, with average total Pirani scores of the Ponseti participants 1.50 lower (95% CI 0.72 to 2.28) after serial casting and Achilles tenotomy. A trial in which the type of presentation was not reported found no difference between an accelerated Ponseti or standard Ponseti treatment. At the end of serial casting, the average total Pirani scores in the standard group were 0.31 lower (95% CI -0.40 to 1.02) than the accelerated group. Two trials in initial cases found relapse following Ponseti treatment was more likely to be corrected with further serial casting compared to the Kite groups which more often required major surgery (risk difference 25% and 50%). There is a lack of evidence for different plaster casting products, the addition of botulinum toxin A during the Ponseti technique, different types of major foot surgery, continuous passive motion treatment following major foot surgery, or treatment of relapsed or neglected cases of CTEV. Most trials did not report on adverse events. In trials evaluating serial casting techniques, adverse events included cast slippage (needing replacement), plaster sores (pressure areas) and skin irritation. Adverse events following surgical procedures included infection and the need for skin grafting.nnnAUTHORS CONCLUSIONSnFrom the limited evidence available, the Ponseti technique produced significantly better short-term foot alignment compared to the Kite technique and compared to a traditional technique. The quality of this evidence was low to very low. An accelerated Ponseti technique may be as effective as a standard technique, according to moderate quality evidence. Relapse following the Kite technique more often led to major surgery compared to relapse following the Ponseti technique. We could draw no conclusions from other included trials because of the limited use of validated outcome measures and lack of available raw data. Future randomised controlled trials should address these issues.

Intravenous Pamidronate Treatment in Children with Moderate-to-Severe Osteogenesis Imperfecta Started under Three Years of Age

Objective: Evaluate clinical outcome of early cyclic intravenous pamidronate treatment in children with moderate-to-severe osteogenesis imperfecta (OI), commenced before three years of age. Methods: A retrospective review of 17 patients with moderate-to-severe OI. Development, anthropometry, fracture history, bone mineral density (BMD) and biochemistry were collected at baseline, 12 and 24 months. Results: Four had OI type I, eleven had type III, one OI-FKBP10 type and one OI type V. Mean age at start of pamidronate was 14 ± 11 months. Pamidronate ranged from 6 to 12 mg/kg/year. No adverse reaction apart from fever and vomiting was noted. Long bone fracture decreased from a mean of 10.4/year to 1.2/year after 12 months and 1.4/year after 24 months (p = 0.02). Lumbar spine age- and height-matched BMD Z-scores increased (p < 0.005). Sixteen with vertebral compression fractures at baseline all showed improved vertebral shape (p < 0.001). Concavity index, likewise, improved (p < 0.005). Motor milestones compared to historical data show earlier attainment in rolling over, crawling, pulling to stand and walking independently but not sitting. Conclusion: Cyclic intravenous pamidronate, started under 3 years of age in children with moderate-to-severe OI, was well tolerated and associated with an increase in lumbar spine BMD, reduced fracture frequency, vertebral remodelling and attainment of motor milestones at an earlier age.

Joint hypermobility syndrome subclassification in paediatrics: a factor analytic approach

Objective To determine if exploratory factor analysis can identify subtypes comprising recognisable clinical patterns of the presenting signs and symptoms of children with joint hypermobility syndrome (JHS). Patients Eighty-nine children with JHS aged 6–16u2005years. Methods Twelve tests comprising anthropometric, musculoskeletal and functional assessments were conducted. Signs, symptoms and family history were recorded. Exploratory factor analysis was performed, factor scores generated, and correlations calculated to identify associations. Results Sixty-six percent of the variance in the score set could be accounted for by five JHS subtypes (Eigenvalues >1). Factor 1, ‘joint affected’ JHS, had loadings on multiple joint pain, recurrent joint instability and postural orthostatic hypotension symptoms, and factor scores were associated with worse pain (r=0.48, p<0.01), fatigue (r=−0.54, p<0.01) and reduced health-related quality of life (HRQOL) (r=−0.5, p<0.01). Factor 2, ‘athletic’ JHS, loaded on muscle endurance, balance and motor skill proficiency, and scores were associated with less fatigue (r=0.3, p<0.01) and better HRQOL (r=0.44, p<0.01). Factor 3, ‘systemic’ JHS, loaded on skin involvement, incontinence symptoms, bowel involvement and recurrent joint instability, and was associated with reduced HRQOL (r=−0.24, p=0.03). Factor 4, ‘soft tissue affected’ JHS, loaded on recurrent soft tissue injuries and reduced muscle length, and was associated with greater fatigue (r=−0.43, p<0.01) and reduced HRQOL (r=−0.44, p<0.0001). Factor 5, ‘high BMI’ JHS, had high loadings on body mass index (BMI) for age, muscle endurance and no gastrointestinal involvement, and was associated with higher pain (r=0.33, p<0.01). Conclusions The presenting signs and symptoms of children with JHS can be summarised in five clinically recognisable subtypes.

Functional performance in young Australian children with achondroplasia

Aimu2002 The aim of this study was to determine population‐specific developmental milestones for independence in self‐care, mobility, and social cognitive skills in children with achondroplasia, the most common skeletal dysplasia.

Developmental milestones in infants and young australasian children with achondroplasia

Background: Achondroplasia, the most common form of chondrodysplasia (inherited skeletal dysplasia), is characterized by a significant delay in the development of communication and motor skills, particularly during the first 2 years. Although some information regarding timing of development for children with achondroplasia is available, no study has evaluated simultaneously the pattern of skill development across multiple key developmental areas. Method: This study used a retrospective questionnaire to quantify developmental data on milestone achievement. Twenty families of children with achondroplasia throughout Australia and New Zealand were asked to document age of acquisition for 41 gross motor, fine motor, and communication and feeding milestones. More than one half of the items assessed were milestones identified in the Australian State Government Personal Health Record Books. The results are compared with previously available information regarding development of motor skills by a cohort of American children with achondroplasia. Results: Although the results support previously reported delays in gross motor and communication skill development, fine motor development does not seem to be as delayed as previously suggested. Information on development of self-feeding skills is presented for the first time and occurs later in this group than the typically developing population. We describe 2 distinctive and previously unreported methods of transitioning between static positions commonly used by children with achondroplasia. Conclusion: Delays were reported across gross motor and communication and feeding skills but were not observed during development of fine motor skills. Additional information is also offered regarding a variety of unusual movement strategies demonstrated by young children with achondroplasia.

Quality of life prediction in children with joint hypermobility syndrome

To assess the child‐ and parent‐reported health‐related quality of life (HRQOL) of children with joint hypermobility syndrome (JHS), to compare these with other chronic paediatric conditions and to determine whether symptoms experienced by children with JHS can predict their HRQOL.

Joint hypermobility syndrome: A review for clinicians

The term ‘joint hypermobility’ describes synovial joints that move beyond a normal range of motion. ‘Joint hypermobilty syndrome’ may also be associated with significant symptoms and impaired quality of life. The purpose of this review is to help the generalist to recognise the condition, exclude significant alternative diagnoses and understand the multidisciplinary approach to management.