Joint Hypermobility Syndrome:
Which Intervention?This section is compiled by Frank M. Painter, D.C.
Send all comments or additions to: Frankp@chiro.org
FROM: J Clinical Chiropractic Pediatrics 2011 (Dec); 12 (2): 915–918 ~ FULL TEXT
Joyce E. Miller, BSc, DC, DABCO, FCC and Sally L. Mathews, BSc(Hons)
Anglo European College of Chiropractic,
Bournemouth, Dorset, UK
jmiller@aecc.ac.uk
Objective: Adolescent back pain is becoming increasingly prevalent with levels approaching those of adults by late teens. Joint hypermobility syndrome (JHS) is a common cause of musculoskeletal symptoms in childhood, including back pain. These two facts combined suggest that the pediatric patient with JHS is likely to be a common presentation to the chiropractor’s clinic. Failure to identify the condition could result in inappropriate care. This case report presents an appropriate management plan for hypermobile adolescents with back pain.
Clinical Features: A 15-year-old school girl with a history of childhood scoliosis and a year-long history of anterior rib pain presented to our clinic complaining of low back pain radiating to the lower ribs following an awkward movement 9 days earlier
Intervention and Outcome: The patient was treated with gentle mobilization therapy and post-isometric relaxation to the quadratus lumborum, the external obliques, and the upper trapezius alongside daily proprioceptive exercises and early-stage core isometric strengthening exercises within neutral posture. Overall there was a reduction in pain from 8/10 to 4/10 as of treatment number 5 accompanied by a 28 point reduction on the Bournemouth Questionnaire.
Conclusion: This case report showed the possible beneficial effects of a multimodal treatment approach combining chiropractic treatment with proprioceptive exercises in a patient with generalized joint hypermobility syndrome.
Key words: Adolescent, back pain, joint hypermobility syndrome, proprioceptive exercise
From the FULL TEXT Article
Introduction
Joint hypermobility syndrome (JHS) is a hereditary connective tissue disorder characterized by lax joints and the presence of musculoskeletal symptoms. The syndrome has been under-recognized and has only recently started to be taken more seriously. [1, 2] Prevalence in children has been estimated at 10-25%. [1, 2] Females tend to be affected more than males, and those of African or Asian descent appear to be affected more than Caucasians. JHS may underlie common orthopedic problems such as back pain. Indeed, adolescent back pain is becoming increasingly prevalent as levels approach those of adults by late teens. [3] JHS has been cited as one of the most common differential diagnoses for back pain in children [4] and is a common reason for presentation to a rheumatologist in adults. [2] Back pain during adolescence can have profound effects on schooling and social relationships, with potentially serious repercussions should it lead to lengthy absences from school. This is a case of pediatric back pain complicated by JHS.
Certain sequelae of JHS are common. These include acute ligament and soft tissue injury, overuse injuries, possible increases in fractures and a possible predisposition to degenerative joint disease after years of excessive joint motion. [5] Left untreated or undiagnosed, hypermobility may result in a chronic pain cycle and high levels of disability. [4] Further, it has been shown that there is increased pain sensitivity in teenagers who have had early pain experiences. [6]
Table 1
Table 2
Table 3 There is some disagreement as to the definition of JHS and a number of scales have been used to aid diagnosis. Traditionally, the Beighton Score [7] (Table 1) has been used to detect hypermobility. However, its clinical usefulness is sometimes questioned, primarily due to its focus on the upper body, which causes less disability than the lower limb, and the fact that, in the younger population, it has been suggested to over report hypermobility. [4]
A simple five-part questionnaire was devised by Hakim and Grahame [8] in 2003 (Table 2) and has a sensitivity of 85% and a specificity of 90% for detecting hypermobility should patients answer yes to two or more of the questions. A further refinement has been developed in the form of the Brighton Criteria (Table 3) [9] which also account for patient symptoms and are therefore useful in a clinical setting.
Case report
A 15-year-old girl of Asian descent presented with low back pain of 9 days duration with radiation anteriorly to the lower ribs. The problem had begun when awkwardly alighting from her bed. She described the pain as a dull tightness, escalating to shooting sharp pains on certain movements which she rated 8/10 in intensity. She consulted her general practitioner the following day and was prescribed co-codamol analgesia which was mildly relieving. She also noted a year long history of anterior rib pain on the left after walking long distances or carrying her school bag and discomfort in the upper back related to sitting and studying. She had already missed 2 weeks of school due to pain since the start of the year 9 weeks ago, leading her mother to seek alternative care. At age 3 she was diagnosed with a scoliosis, which had self-resolved by the age of 10. She has always considered herself ‘double jointed’ and noted that her brother was too. She was able to answer yes to 3 of the 5 questions in the Hakim and Grahame [7] questionnaire for the detection of hypermobility (Table 2).
Physical examination confirmed the presence of joint laxity and showed bilateral pes planus. The quadratus lumborum and external obliques were tender to palpation bilaterally. There were a number of tender and restricted areas in the thoracic spine, but no neurological signs were present. JHS is a diagnosis of exclusion with rule-outs of Erlos Danlos syndrome, Marfan syndrome and juvenile idiopathic arthritis.
The diagnosis of JHS leads to questions about appropriate treatment options for this child’s back pain. The presence of generalized joint laxity brings into question whether high velocity manual thrust techniques are appropriate. A search of the scientific literature over the past ten years was conducted to investigate the best evidence for appropriate care and its effectiveness in similar cases. The original paper outlining the Beighton Score [6] was included, despite falling outside the primary search time frame, for completeness.
Discussion
Table 4
Table 5 There is no evidence specific to chiropractic care and the pediatric population with JHS. However there is some level 4 evidence (case report and case series) [10, 11] investigating the use of manual therapy alongside functional rehabilitation training in this group. Table 4 shows the results of a large case series of individuals treated with a three week exercise programme where 69% of participants showed improvement at six week review. There was a significant positive relationship between age and outcome. This may be explained by other factors, such as onset of puberty, natural stiffening up over time or greater compliance in older children.
Such minimal evidence as there is suggests that manual therapy alongside functional rehabilitation training — with an emphasis on improved motor control, proprioception and strength-endurance [10, 11] may lead to long term amelioration of low back pain in hypermobile patients. Treatments focus on improving muscle control in the presence of ligamentous insufficiency to help to minimize trauma to joints. It is commonly recognised that children respond well to muscle-strengthening exercises — although muscle bulk may not increase as improvement in strength and neuromuscular co-ordination results in more efficient muscle use. [4]
It is interesting to note that our patient was very active, swimming competitively until a year ago when she decided to focus on her studies. The cessation of activity coincides with the onset of back and rib pain. Modification of activities, alongside rehabilitation exercises are suggested from the literature to be the key to improved outcomes in the presence of JHS. [10, 11] The patient was instructed in daily proprioceptive exercises and early-stage core isometric strengthening exercises within neutral posture. She was treated with gentle myofascial therapy and post-isometric relaxation to the quadratus lumborum, the external obliques and the upper trapezius.
The patient rated her pain as 8 on the Numerical Rating Scale (NRS) which spans 0 to 10, where 0 is no pain and 10 is the most or worst pain. Although the NRS has been studied extensively in adults and has good evidence of acceptability, reliability and validity, there is little data to support the use of the NRS in the pediatric population. However, there is some indication that the scale is useful for children 8 years and older. [12] The patient’s self-reported pain was reduced from 8/10 to 6/10 as of treatment number 5.
The NRS value remained relatively high. It has been shown that pain is often the last thing to improve in the presence of continuing joint laxity and when it does, it only does so slowly. This is an important point to emphasize at the start of treatment. [4] The clinical course of patients with JHS may be one of ups and downs as patients are subject to recurrent soft tissue injury, a further point of note when discussing treatment options with patients. [4]
Conclusion
JHS is a common cause of musculoskeletal symptoms in childhood [4] and should be born in mind for all pediatric patients as ligament laxity may be the rule rather than the exception. The condition should be diagnosed prior to commencement of treatment and the goal of therapy should be stabilization of lax joints rather than manipulation, which may result in destabilization.
This case report illustrates a relatively common presentation of adolescent low back pain. The case emphasizes the importance of early recognition of JHS and its impact on patient management, as it needs to be long-term. A multimodal treatment approach combining myofascial therapy with proprioceptive exercises is important in amelioration of long term pain.
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Pain 2009;doi:10.1016/j.pain.2009.03.002Post-treatment Bournemouth Questionnaire reproduced with kind permission from the AECC,
http://www.aecc.ac.uk/research/bmth_questionnaire/index.asp
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