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Hypermobility disorders in children and adolescents

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It is well recognized that many if not most children and adolescents attending paediatric rheumatology clinics will have a non-inflammatory origin for their complaints or disorder. Mechanical causes are frequently identified, and hypermobility or ligamentous laxity of joints is increasingly recognized as an aetiological factor in the presentation. Such conditions include ‘growing pains’, recurrent lower-limb arthralgia, anterior knee pain syndromes, and back pain. Studies of significant cohorts of such patients have now been published supporting the link of ligamentous laxity to particular symptom complexes. However, much disagreement remains as to the validity of hypermobility as an aetiogical factor. What seems clear is that not all hypermobile individuals will be symptomatic or indeed possibly have any risk for specific musculoskeletal disorders in later life. Screening tools such as the Beighton score are likely to be inadequate in many paediatric populations. Along with increasing recognition of these disorders in childhood and adolescence has been the development of a multidisciplinary management approach, which usually involves predominantly allied health professionals such as podiatrists, physiotherapists and occupational therapists. The challenge remains to interpret symptoms correctly as being related to the hypermobility and to predict why such children become symptomatic. The answer is likely to involve physiological and psychosocial factors. In addition, early identification and modification of risk factors may have major implications for subsequent prevalence of many adult medical disorders such as low back pain, chronic pain syndromes and degenerative osteoarthritis.

Section snippets

Hypermobility in children: true pathology or normal variation?

The definition of hypermobility is inherently an arbitrary one, and methods used to categorize an individual as being hypermobile or not should seek to identify populations with increased risk of important clinical phenomena. Hypermobility as such is not a medical disorder but merely an observed phenomenon, which may be associated with musculoskeletal symptoms, or symptom complexes, and may predispose to or be associated with more serious medical disorders. The degree of ligamentous laxity has

Joint Hypermobility Syndrome (Benign JH or BJHS) versus Joint Hypermobility

In an effort to clarify the relationship between hypermobility and musculoskeletal disorders the revised (Brighton, 1998) criteria for the diagnosis of BJHS were developed.5 In essence this collection of associated history and examination features indicates that ligamentous laxity alone is not sufficient to establish the diagnosis in such symptomatic patients. The collection of major and minor criteria serves to emphasize that many patients will have a history of particular symptoms (such as

Musculoskeletal features associated with JHS

Virtually all parts of the musculoskeletal system may be affected in JHS, but particular problems or symptom complexes seem to occur at different ages. This probably represents a combination of developmental changes or differences in growth patterns at different ages and the degree of physical activity engaged in as children get older.

Dental and oral health disorders

Abnormal dentition is recognized in Ehlers Danlos syndrome and osteogenesis imperfecta. Temporomandibular joint disease has long been associated with either localized or generalized hypermobility disorders and has more recently been elaborated in young adults. It is less common for children to present with temporomandibular joint disease symptoms57, but by late adolescence symptoms of clicking and instability are not uncommon, as are acute painful episodes and locking, and these may predate

Genetics and the aetiology of hypermobility

Table 5 lists the well-described associations of defined genetic disorders with ligamentous laxity.68, 69, 70, 71, 72, 73, 74, 75, 76 Even well-described disorders such as Ehlers Danlos syndrome have remarkable genetic and phenotypic heterogeneity. Whilst some forms have single gene defects that have been identified (in collagen formation in particular), inheritance is far from straightforward. What this teaches us perhaps is that many genes affect the integrity of joint and other tissue

Key aspects of management for hypermobile children

A positive diagnosis is a key issue for embarking on management of hypermobile patients. Rather than being a diagnosis of exclusion, hypermobility can be easily recognized and understood with appropriate history-taking (including family history) and a detailed examination (see Figure 1). Hypermobility is often ‘missed’ because it is not examined for in a situation where the exclusion of inflammatory or destructive rheumatic conditions is the expectation of patient and physician alike. Many

Joint stability and muscle strength

Improving dynamic muscle control to supplement the ligamentous insufficiency should minimize trauma to joints. It is commonly recognized that children respond well to a muscle-strengthening programme, and while they may not improve their muscle bulk, they do improve strength and their neuromuscular coordination, thus making the muscles more effective.78 It is often useful, especially if the child is experiencing significant pain, to start on some static exercises in the hypermobile range before

Conclusions

Joint hypermobility is a phenomenon with many forms of potential clinical presentation in young people. Positive recognition and avoidance of unnecessary investigations and drug therapy are among the most important interventions. Most patients are well managed with simple advice and reassurance. Modification of activities may be required to redress the balance between healthy physical activities and high-impact physical pursuits. If untreated or undiagnosed, hypermobility can at times result in

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