Chapter 4 – Hypermobility Syndrome in Children

CHAPTER CONTENTS

Pathophysiology 33

Paediatric conditions with hypermobilityas a clinical finding 34

Joint hypermobility syndrome in childrenDiagnosis and assessment 35Prevalence of joint hypermobility 36Clinical presentation 38

Lower limb arthralgia 39Back pain 39Chronic pain syndromes 39Other symptoms 39Balance and proprioception 40Pain 40

35

4

Hypermobility syndromein children

Susan MaillardKevin J. Murray

Joint hypermobility is understood to result fromeither genetic defects or variations in connectivetissue matrix proteins, which results in moreextensible tissues. Some authors describe thisheterogeneous group as the heritable disordersof connective tissue (HDCT) (Grahame 1999).Within this group are disorders such as EhlersDanlos syndrome, Marfan syndrome and osteogenesis imperfecta. All these genetic connectivetissue disorders have hypermobility as a feature,but hypermobility is also relatively commonwithin the general population and may be a resultof more common genetic variations rather thanmutations as such (Grahame 2000).

Aims1. To describe the different clinical conditions

and phenomena that occur in children withjoint hypermobility

2. To describe how JHS presents in children3. To discuss the multidisciplinary management

of JHS

42

4041

4547

4444

44

Management of the joint hypermobilltysyndrome 40

Medical 40Therapy management 40

Subjective assessmentObjective assessmentAims of treatment 42

Treatment methods 42Joint stability and muscle strengthGail re-education 43Hand function 43Functional tasks 44Balance 44General fitnessHydrotherapyPain reliefPacing 45PsychologyConclusion

PATHOPHYSIOLOGY

Joint hypermobility syndrome appears to beinherited as a gender-influenced dominant trait(Beighton et al. 1988, Beighton and Horan 1970,Carter and Wilkinson 1964, Child 1986, Grahame2000, Henney et al. 1992, Horton et al. 1980, Jesseeet al. 1980). It has not been possible to identify asingle gene abnormality, but some variation in

33

34 HYPERMOBILITY SYNDROME

collagen type I/type III ratios have been observed.However, it may be more likely that the condition represents a complex genetic trait wherebymultiple genes influence the phenotype of theindividual.

Type I collagen is the most common collagenin the human body and is abundant in connectivetissues such as tendon, ligament, joint capsule,skin, demineralized bone and nerve receptors.Type III is also present to a lesser extent in thesestructures, and type II is found predominantly inhyaline cartilage. Type III is thin and elastic andfound in relatively greater amounts in extensibleconnective tissue, such as the lungs, skin andvascular system (Child 1986,Handler et al. 1985,Prockop and Kivirikko 1995). In children withhypermobility the ratio of type III collagen totype III + type I is increased (El-Shahaly andEl-Sherif 1991), and this is thought to underliethe increased tissue flexibility of childhood (orloss of it with ageing).

Neurological structures or tissues may alsobe affected, which is indicated by the increasedincidence of acroparaesthesia (abnormal sensations in hands and feet) in children with JHS.There is also evidence that proprioception andjoint spatial awareness are reduced, particularlyat the end of the range of movement of thejoints (Hall et al. 1995, Mallik et al. 1994). Furtherresearch indicates a poor response to localanaesthesia in some individuals, and there isalso a connection between benign congenitalhypotonia and ligamentous laxity in the floppyinfant.

PAEDIATRIC CONDITIONS WITHHYPERMOBILITY AS A CLINICALFINDING

As in many aspects of paediatric medicine, allpossible causes must have been consideredbefore a diagnosis of JHS can be made (Box 4.1).The degree of hypermobility will vary fromglobal to localized. Specific genetic disorders that

Box 4.1 Definable clinical conditions which have beenassociated with joint hypermobility as a clinical finding

Achondroplasia (Raft and Byers 1996)Clumsy childrenCongenital hip dysplasia (Gulan et al. 2000, Hamilton

and Broughton 1998, Wenger and Frick 1999)Congenital hip dislocation (Carter and Wilkinson 1964)Chondromalacia patellae (AI-Rawi and Nessan 1997,

Lewkonia and Ansell 1983)Down syndromeDyspraxiaEhlers-Danlos syndrome (Dolan et al. 1998)Fibromyalgia (Bridges et al. 1992, EI·Shahaly and

EI Sherif 1991, Gedalia et al. 1993b, Goldman 1991,Hudson et al. 1995)

Growing pains = benign paroxymal nocturnal leg painsInfantile hypotoniaJuvenile episodic arthralgias of childhoodLarsen syndrome (Raft and Byers 1996)Marfan syndrome (Lannoo et al. 1996)Morquio syndrome (Raft and Byers 1996)Motor developmental delay (Davidovitch et al. 1994,

Jafte et al. 1988, Russek 1999, Tirosh et al. 1991)Osteogenesis imperfecta (Engelbert et al. 1999)Spondylolysis!spondylolythesisStickler syndromeTrichorhinopharyngeal syndrome

cause hypermobility in childhood include:

1. Ehlers-Danlos syndrome. All forms of thissyndrome cause clinical problems, such asskin fragility, unsightly bruising and scarring,musculoskeletal discomfort due to recurrentsprains, subluxations and dislocations, andan increased susceptibility to osteoarthritis.One form that is potentially fatal is termedEhlers-Danlos vascular type (formerly EDStype IV), which predominantly involvesvascular structures, and death resultsfrom the rupture of arteries, bowel orother large organs (Pepin et al. 2000, Pyeritz2000).

2. Osteogenesis imperfecta (01). There areseveral forms of this condition, all of whichhave a varying degree of hypermobilityassociated with them. However, researchindicates that among children with 01, thosewith generalized joint hypermobility are lesslikely to develop scoliosis and occipitobasilarimpression (Engelbert et al. 1998).

3. Stickler syndrome. Stickler syndrome is anautosomal dominant disorder withcharacteristic ophthalmological and orofacialfeatures, deafness and arthritis. These childrentypically have a flat midface with a depressednasal bridge, short nose, anteverted nares,micrognathia and severe myopia. Retinaldetachment maylead to blindness. There isgeneralized joint hypermobility, which oftendisappears with age, and mild to severeosteoarthritis that typically develops in thethird or fourth decade. Mildspondyloepiphyseal dysplasia (specificprogressive changes in bone and jointformation due to a genetic mutation) is oftenapparent radiologically (Snead and Yates 1999).

4. Madan syndrome. Marfan syndrome is anautosomal dominant genetic disorder causedby a defect in the gene which encodes fora specific protein, Fibrillin 1 (FBN1). Suchindividuals present with a characteristicallytall stature, long limbs (where the armspan:height ratio exceeds 1.03), littlesubcutaneous fat and muscle hypotonia.There may also be complications with thecardiovascular system and the eyes (seeChapter 2). Joint hypermobility can berecognized in as many as 85% of childrenwith Marfan syndrome (Grahame and Pyeritz1995, Lannoo et al. 1996).

There are other recognized clinical features thatare closely associated with hypermobility. Theseinclude:

1. Anterior knee pain. Generalized joint laxity,and particularly hypermobility of the knee, isconsidered a possible contributing factor inthe development of anterior knee pain andchondromalacia patellae (Al-Rawi andNessan 1997). These authors also found thatchondromalacia patellae was associated withwasting of the quadriceps muscle and withflat feet and backache, the latter two alsocommonly associated with generalized jointhypermobility.

HYPERMOBILITY SYNDROME IN CHILDREN 35

2. Motor development delay. Hypermobilitycan be evident as early as infancy. There issaid to be a higher incidence of both grossand fine motor delay in children who arehypermobile, even in the absence of anidentified neurological deficit (Jaffe et al.1988, Tirosh et al. 1991) (see p 38).

3. Fibromyalgia. See under chronic painsyndromes (p 39).

JOINT HYPERMOBILITY SYNDROMEIN CHILDREN

Joint hypermobility syndrome is diagnosed whenhypermobility becomes symptomatic and whenother heritable disorders of connective tissue andother causes of the symptoms have been considered and ruled out.

Diagnosis and assessment

There have been many scoring systems devisedfor measuring or defining hypermobility. Thefirst, proposed by Carter and Wilkinson (1964)was then modified by Beighton et al. (1973). TheBeighton scoring system has proved the mostpopular worldwide. There is also a scale devisedby Rotes that is used in most Spanish-speakingcountries, which adds more items of clinicalinterest (quoted in Bulbena et al. 1992). Table 4.1lists the three different scoring criteria. The different systems differ slightly in their particularways of assessing a joint. Together with theseclinical manoeuvres, other instrumental methodsto assess joint hypermobility have been used(Bulbena et al. 1992) i.e. clinical goniometers(Beighton et al. 1988, Mishra et al. 1996), fixedtorque devices (Beighton et al. 1973) and hyperextensometer (Bird et al. 1979). There is no universally accepted Beighton score required inorder to accept a diagnosis of JHS in the presenceof associated musculoskeletal symptoms. Somehave taken 5/9, others 6/9.


Table 4.1 Comparisons of the different methods ofmeasuring the hypermobility criteria: Carter and Wilkinson,Beighton and Rotes criteria (Bulbena et al. 1992)

Criteria Carter and Beighton HotesWilkinson et al.

Thumb abduction 1-Elbow extension >10 0 1-Finger extension >900 1-

(metacarpophalangeal)Knee extension > 100 1-Excess ROM ankle

dorsiflexion andeversion

Palms flat on floorExternal shoulder

rotationCervical rotationCervical

side flexionHip abductionMetatarsophalangeal

>90 0

Lumbar lateralhypermobility

Total score 5 9 11

*1 point each side.

Bulbena et a1. (1992) compared the scales proposed by Carter and Wilkinson (1964), Beightonet a1. (1973) and Rotes (quoted in Bulbena et a1.1992) and found a high correlation between thedifferent scores, suggesting high concurrent andpredictive validity. From this they proposed aset of criteria which they considered the bestdefinition of hypermobility.

One intrinsic difficulty in measuring ranges ofpassive movement is that the observed rangedepends upon the force applied to the movingpart, which may vary with the enthusiasm of theexaminer and the pain threshold and cooperationof the child (Silverman et a1. 1975). In children itis good practice to assess all joints for hypermobility initially in order to establish the degreeof hypermobility, i.e. global or localized, as thiswill guide the treatment programme.

A set of classification criteria for joint hypermobility syndrome (as opposed to hypermobility), validated so far for adults only, wasdeveloped in Brighton in 1998 (Grahame et a1.2000).

Careful clinical evaluation and, where appropriate, laboratory tests, may be done to rule outother more serious conditions which may havesimilar symptoms, e.g. juvenile idiopathic arthritis(JIA) and other inflammatory polyarthritis conditions (Kirk et a1. 1967), Ehlers-Danlos andMarfan syndrome. Findings of marked hyperelastic skin and lenticular abnormalities are notusually seen in JHS, but mild or moderateincreases in skin stretchiness, hernias, relativelyeasy bruising and poor wound healing may beseen (El-Shahaly and El-Sherif 1991, Mishra et a1.1996), indicating some overlap with these welldefined genetic conditions.

In the assessment of a child with hypermobility it is important to assess the strength ofmuscles surrounding the joints, as well as theendurance of the muscles and the general stamina of the child. These factors are often reducedin JHS, and improving them will be an importantpart of the treatment programme. It is alsoimportant that balance and proprioception areassessed to determine their impact on the child'ssymptoms, and the management programmeshould address any deficiencies (Augustsson andThomee 2000, Finsterbush and Pogrund 1982,Visser et a1. 2000).

Prevalence of joint hypermobility

The reports of the prevalence of hypermobilitymust be viewed cautiously because of the variability in the diagnostic criteria used. Joint hypermobility has been reported in 6.7-43% of children(Table4.2),depending upon age, ethnicity, and thecriteria used to assess hypermobility. The prevalence is higher in girls: 7.1-57%, compared to6.0-35% in boys (Decoster et a1. 1997, Gedalia eta1. 1985, Kerr et a1. 2000, Russek 1999, Vougioukaet a1. 2000).Given the high proportion of subjectsdocumented as 'hypermobile' in some studies, itis clear that the different systems of assessmenthave major limitations in defining a cohort ofhypermobile subjects at significant risk of musculoskeletal problems in different populations.

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Hypermobility is also more prevalent amongAsians than Africans, and more prevalent inAfricans than Caucasians. However, it appearsthat hypermobility decreases with age (AcasusoDiaz and Collantes-Estevez 1998,Birrell et a1. 1994,Cheng et a1. 1991, El-Garf et a1. 1998, El-Shahalyand El-Sherif 1991, Hudson et a1. 1998, Kirk et a1.1967, Russek 1999), and that far fewer adults arehypermobile compared to the number of hypermobile children.

Clinical presentation

Many children have hypermobile joints, but onlya percentage of those will suffer from symptoms,and if they follow a chronic pattern these children are often diagnosed as having JHS(Grahame 1999). The symptoms and their severity will vary from child to child, and can occur atany age (Table 4.3). Children may present to anorthopaedic surgeon, rheumatologist, paediatricrheumatologist, paediatrician, general practitioner, physiotherapist or other manual therapistwith any of a wide range of traumatic or nontraumatic painful complaints (Box 4.2).

These children typically lack the positivelaboratory findings found in other rheumatological conditions and rarely develop any radiological abnormalities. Occasionally transient mildswelling or puffiness and, more rarely, joint effusions may occur, but normally only last for hoursor very occasionally days (Kirk et a1. 1967,Russek1999,Scharf and Nahir 1982).The occurrence andlocation of symptoms are variable, but appear tobe more common in the lower limbs.

In the very young child hypermobility maybe detected with evidence of delayed motordevelopment and a degree of clumsiness(Davidovitch, et al. 1994,Jaffe et a1. 1988, Moreiraand Wilson 1992,Tirosh et a1. 1991).However, themotor delay is usually self-limiting if hypermobility is the only cause. This problem willeither improve spontaneously or with a motordevelopment rehabilitation programme. A number of parents of hypermobile children do report

Box4.2 Neuromuscular and musculoskeletalcomplications or features seen in children with JHS

Acuteor traumaticSprains (Grahame et al. 1981)Strains - recurrent ankle strains (Finsterbush and

Pogrund 1982)- knee, meniscus tears (Bulbena et al. 1992)

Acute or recurrent dislocations/subluxations, l.e.

• Shoulder joint (Finsterbush and Pogrund 1982)• Patella (Biro et al. 1983, Finsterbush and Pogrund

1982, Runow 1983, Stanitski 1995)• Metacarpophalangeal (MCP) joint

Traumatic arthritis/synovitisBruising (Bird et al. 1978, Bulbena et al. 1993)Fractures (Grahame et al. 1981)

Chronic or non-traumaticSoft-tissue rheumatism (EI-Shahaly and EI-Sherif 1991,

Gedalia et al. 1985, Goldman 1991, Grahame et al.1981, Hudson et al. 1995, Kirk et al. 1967)

• Tendonitis• Synovitis• Juvenile episodic arthritis/synovitis• Bursitis

Anterior knee painBack pain - lumbar or thoracic (AI-Rawi and Nessan

1997, EI-Shahaly and EI-Sherif 1991, Grahame 1999,Howell 1984, Larsson et al. 1993, Larsson et at, 1995,Lewkonia and Ansell 1983)

Scoliosis (Bulbena et al. 1992, EI-Shahaly and EI-Sherif1991, Finsterbush and Pogrund 1982)

Chronic widespread musculoskeletal pain syndromesNerve compression disorders (EI-Shahaly and

EI-Sherif 1991)Acroparaesthesia (Francis et al. 1987)

Flat feet and ankle/foot pain (AI-Rawi and Nessan 1997,Bulbena 1992, Finsterbush and Pogrund 1982)

Unspecified arthralgia or effusion of joints (Biro et al.1983, EI-Shahaly and EI-Sherif 1991, Finsterbush andPogrund 1982, Hudson et at, 1995, Kirk et al. 1967,Lewkonia and Ansell 1983)

ongoing clumsiness in their children well intolater childhood.

There is no evidence to suggest that JHS islinked to more serious conditions of the cardiacsystem, bone, skin or eyes (Grahame et al. 2000,Mishra et a1. 1996).However, the evidence linkinghypermobility and pulled elbows is not clear,as some reports suggest there is a link whereasothers have not found a correlation (Hagroo et a1.1995).

There is, however, evidence that links hypermobility to many other symptoms.

Lower limb arthralgiaPain in the lower limbs may present as anteriorknee pain, generalized knee pain and ankle / footarthralgia. Children will often complain of painafter physical activity and towards the end of theday, and this may be due to muscular pain aswell as joint pain. Early morning stiffness, stiffness after rest periods or prolonged stiffness,which are the hallmark of inflammatory rheumatic disorders, are less prominent in JH5-relatedproblems (Arroyo et a1. 1988,Bensahel et a1. 2000,Grahame 2000, Hall et a1. 1995, Kerr et a1. 2000,Vougiouka et a1. 2000).

It has been recognized that hypermobility maybe a contributing factor in the pathogenesis ofchondromalacia patellae, and that this may beassociated with pain in the ankles, hips and back(Al-Rawi and Nessan 1997).

A number of children with a history of 'growing pains' or 'benign paroxysmal nocturnal legpain' have been documented as having generalized hypermobility. Others who are diagnosedwith a hypermobility problem or JHS in later lifeoften recall a history of 'growing pains'. It isconceivable that these pains may be related toperiods of unaccustomed excessive activity(such as after an intensive physical education(PE) lesson) in those predisposed by underlyinghypermobility.

Back painBack pain in children and adolescents requiresthorough investigation and assessment. However,one of the most common differential diagnoses ishypermobility (Grahame 1999, Scharf and Nahir1982). This aspect affects adolescents most often,and may be related to a particular physical activity. Thus, hypermobility may affect the whole ofthe spine or a specific area only, and often resultsin an S-shaped posture with increased curves inthe cervical, thoracic or lumbar regions. The painis often associated with acute muscle spasm, andif not managed appropriately in the early stagesmay result in chronic back pain, continuing poor


posture, and the associated difficulties that thesebring. Poor sitting posture and the weight of thebooks carried at school often exacerbate this.

If the pain is severe, specific lesions seen morefrequently in hypermobile children, need to beconsidered, such as spondylolysis, spondylolisthesis and, in older children, disc prolapse.

Chronic painsyndromesThere has been significant research establishinglinks between hypermobility and fibromyalgia.Fibromyalgia is a chronic pain syndrome associated with multiple muscle tenderness and localized tender points, marked sleep disturbanceand high levels of disability. The results areinconclusive, but they do suggest an associationbetween joint hypermobility and fibromyalgiain that there appears to be a high percentage offibromyalgic children who are also hypermobile(Acasuso-Diaz and Collantes-Estevez 1998,Buskilaet a1. 1995,Gedalia et a1. 1993,2000).

A link has also been reported between hypermobility and the development of reflex sympathetic dystrophy (Francis et a1. 1987, Murray andWoo 2001), and other localized chronic pain syndromes. Many children who present with thesepain syndromes are also hypermobile, and it isvery difficult to distinguish between the symptoms of JHS and chronic pain syndromes, whichmay complicate it.

In addition, it has been noted that adults withJHS are more likely to have anxiety disordersthan a comparison group with other rheumatological conditions (Bulbena et a1. 1993). It oftenbecomes apparent that there is an element of painamplification in children with JHS, and in thesecases it is appropriate to consider a psychologicalapproach to pain management as well as aphysical approach.

OthersymptomsAlthough subluxations, dislocations, sprains,strains and other soft tissue rheumatic conditionsare more common in children with JHS (Hudson


et al. 1998) the resultant damage occurring withthese acute injuries may, paradoxically, actuallybe decreased because of the increased laxity ofjoint structures, and the true recovery time therebyreduced. However, the increased frequency ofoccurrence in hypermobile children may add tothe overall burden.

Many researchers have found a correlationbetween temporomandibular dysfunction (TMD)and hypermobility (Buckingham et al. 1991,Khanand Pedlar 1996, Perrini et al. 1997), though notuniformly (Conti et al. 2000, Khan and Pedlar1996, Winocur et al. 2000). Symptoms of pain,subluxation and clicking in the temporomandibular joint have been reported.

Many of the childhood problems discussedabove may persist well into adult life and requiresimilar treatment methods.

Balance andproprioceptionChildren with hypermobility are reported by theirparents to be clumsy or have generally poorbalance. This is thought to be due in part toreduced proprioception in joints, most marked atthe extremes of the movements. Generally, spatialawareness is reduced in hypermobile joints, whichhas an impact upon the child's balance mechanisms. These factors are also compounded by thefact that the muscles around the joints are not attheir optimum strength or stamina, often becausejoint pain will inhibit muscle action. Therefore, themuscles will become weaker and more unfit at thesame time as the child is growing and developing.The muscles are also not used through their fullrange, as the hypermobile range is often unstableand not used optimally, and so wasting of themuscles occurs. This muscle wasting and weakness is a factor in the poor control of joint movement, particularly in the hypermobile range, andcan lead to instability.

PainChildren's understanding of pain varies depending upon their age, 'developmental stage, their

coping strategies and their experiences. All ofthis will have an impact upon the degree of painreported and the importance and significance ofthe pain experienced.

Consideration needs to be given to other members of the family, not only because they may beanxious on their child's behalf, but also becausethey may be suffering from the syndrome themselves. A parent's pain problems will have animpact upon the child's experience of pain. Thepsychological component of pain should alwaysbe remembered when dealing with JHS (McGrath1995).

MANAGEMENT OF THE JOINTHYPERMOBILITY SYNDROME

Medical

Children usually present to their medical practitioner with a history of joint, limb or back pain andgeneralized fatigue that has a considerable impactupon their day-to-day independent functioning.It is important that when the diagnosis is made,careful clinical assessment has been made to ruleout any other condition, with the use of investigations where appropriate. It is also important toavoid unnecessary further invasive tests, and toensure that the diagnosis and its implications areclearly presented to the family. The family shouldunderstand that the condition is relatively benign(compared to juvenile idiopathic arthritis) andthat self-management is paramount.

Referral to an allied health professional willprompt an assessment followed by a rehabilitationprogramme, with a view to self-management bythe child and the family.

Therapy management

SUbjective assessmentIt is important for the therapist to take a full history of the presenting condition, to confirm thatother underlying conditions have been ruled out

and to establish a clear picture of the nature andseverity of the symptoms, their site, frequency andduration. This will enable the treatment plan to betailor-made to the child's symptoms, and it canalso be used as the basis of an outcome measure ifrecorded accurately. It is useful to use a visual analogue scale for pain if the child is over 7 years old,as it is less subjective, and a 'happy face scale' or'ouchometer' for younger children.

Children with JHS will often present withpainful joints that are most painful in the eveningsand after physical activity. The child occasionallycomplains of stiffness in the mornings, but thisrarely lasts for long.

In JHS there is also the issue of developing adownward spiral of recurring acute and chronicpain symptoms, which needs to be broken toavoid a continuation of symptoms (Fig. 4.1). Thisnotion should be explained to the family, and fromthe assessment the relative proportion and timingof the rest periods and active periods should bedefined. It is also important to ascertain when thepain-free times occur: for example always atweekends, requiring the child to recover duringthe week instead of attending school because ofthe pain. This should be discussed, as it willensure that the 'pacing programme' and thereturn to normal activity are appropriate.

Previous experiences of pain the child has hador personally observed before should be noted,as this may have an impact upon their copingmechanisms. It has been found that children who

Figure 4.1 Vicious cycle of pain


have experience of another member of the familywith chronic pain show that their coping mechanisms are influenced by their observations ofothers (McGrath 1995).An assessment by a clinical psychologist may be necessary to identify factors that compound the underlying cause of painin such children and their families (Malleson andBeauchamp 2001).

Objective assessmentWhen examining a child with JHS it is importantto assess the distribution and the degree ofhypermobility in the joints involved, as this willinfluence the treatment programme. The distribution of hypermobility can be scored using one ofthe scales mentioned previously, but a full assessment of range of movement of every joint is likelyto be useful, especially in children with multiplejoint symptoms. Some children may have generalized hypermobility, involving most of their joints,whereas others may have a pauciarticular formaffecting a small number of peripheral joints.

Once the distribution of joint hypermobilityhas been assessed, the degree of muscle strengtharound the joints needs to be examined. It isimportant for good joint control and stability thatthe muscles have full strength and normalendurance, especially into the hypermobile range.If this is not the case then arthralgia and musclefatigue are likely to occur with prolonged activity.The risk of subluxation of joints, although small, isincreased when muscle strength and control areless than optimal. Muscle strength can be measured using either the Oxford Scale, the expandedll-point scale (0-10, Kendall et al. 1993), or byusing myometry.

Children with JHS are usually generally unfitand have very poor stamina. This also needs tobe addressed in a rehabilitation programme.Stamina is often assessed subjectively, but the'6-minute walk test' (Boardman et al. 2000,Garofano and Barst 1999, Hamilton and Haennel2000, King et al. 2000, Nixon et al. 1996, Pankoffet al. 2000) is a better objective measure. This testrequires the subject to walk as fast and as far as


he or she can in 6 minutes. The distance walkedand the time taken (if he or she is unable tomanage the 6 minutes) are then recorded.

As with any musculoskeletal assessment, it isimportant to observe the posture and gait of thechild and to watch for any abnormalities thatmight be due to the hypermobility. The mostcommon posture and gait disturbances are:

• Hyperlordosis of the lumbar spine;• Increased kyphosis in the thoracic spine;• Weakness around the hips causing a

Trendelenburg sign;• Hyperextension of the knees in the weight-

bearing position;• Pronated / flat feet.

Once the child has been fully assessed, thespecific problems identified can be included inan individualized rehabilitation programme.

Diminished balance and poor proprioceptionare often an issue for children with JHS (Hallet al. 1995,Mallik et al. 1994), and these also needto be assessed at the initial stage. Asking the childto stand on one leg and observing his or herbalance can give a simple measure, which is thenprogressed further by asking him or her to closehis or her eyes at the same time. Removing thevisual input will assess proprioception morespecifically. A more in-depth assessment can beincluded by isolating each joint and assessingspatial awareness without the visual input, i.e.asking the child to identify the direction of movement of a joint while keeping his or her eyes shut.

Aims of treatmentIt is a common misconception that the purpose oftreatment is to reduce the range of mobility of thejoints. The real purpose of the rehabilitation programme, however, is to improve the stability andcontrol of the joint while maintaining the full normal hypermobile range of movement. Thereforethe aims of treatment are:

• To increase the strength of the muscles,especially into the hypermobile range;

• To improve the stamina of the muscles;

• To improve the general fitness of the child;• To re-educate gait to avoid/correct any

abnormalities in the biomechanics;• To return to normal activities and functioning;• To educate the child and family to enable

them to manage the condition with minimalreliance on medication.

These aims work together to ensure that the childhas more stable joints, protected by strong, fitmuscles enabling him or her to manage his orher symptoms independently at home with theminimum of external support, i.e. splints, professional support etc.

TREATMENT METHODS

Joint stability and muscle strength

The inherent stability of a joint is determined bythe efficiency or integrity of the musculoskeletalsystem (muscle, tendon, capsule, ligament andarticular surfaces) and the neural control systems(motor and sensory). As discussed previously,one hypothesis suggests that JHS may be a resultof abnormal collagen fibres resulting in ligamentous insufficiency. The soft-tissue microtraumacaused by this insufficiency is one accepted explanation for the joint and muscle pains (Gedalia andBrewer 1993). It follows, then, that improvingdynamic muscle control in order to compensatefor the ligamentous insufficiency should minimize the trauma (Kerr et al. 2000).

It is commonly recognized that childrenrespond well to a muscle-strengthening programme, and although they may not improvetheir muscle bulk, they do improve strength andneuromuscular coordination, thereby making themuscles more effective (Barton and Bird 1996,Biroet al. 1983,Finsterbush and Pogrund 1982). In general it appears clear that the treatment of choicefor children with JHS is a muscle-strengtheningprogramme working through their full range ofmovement, including the hypermobile range(Kerr et al. 2000,Russek 1999,2000).

When devising an exercise programme, allaspects of muscle function need to be considered,including isometric, isotonic, concentric andeccentric strength, stamina and general function.It is ideal to start with a programme that isolateseach muscle group individually, and includes allaspects of its function. It is also important toensure that all muscle groups around the joint arestrengthened equally, i.e. for the knee both quadriceps and hamstrings, in order to ensure physiclogical balance. It is often useful, especially if thechild is experiencing significant pain, to startwith some static exercises in the hypermobilerange before progressing to dynamic and thenresisted work (Borms 1986). These should alsobe progressed from non-weight-bearing work toweight-bearing work.

Stamina or endurance training is also veryimportant for the muscles, as it is extremely important to have not only strong muscles, but musclesthat will not fatigue easily. Therefore, an exerciseregimen of high repetitions and low weights isused, which is usually achievable and sustainablein a self-management home exercise programme.

Splinting of hypermobile joints is rarely recommended as this is likely to exacerbate ineffective muscle use, causing them to become weakerstill and more prone to injury. In addition it can,in effect, inhibit the rehabilitation programmeand add to the impression that there is a moreserious medical disorder requiring therapy to beprovided, rather than solely from the parents andchild's own home management. However, theuse of limited types of support, or devices suchas pen grips, can be a good adjunct to a handmuscle-strengthening programme. They reducethe force required to sustain the gripping of apen, thereby reducing the pain and fatigueexperienced in the fingers and wrists duringschool work, for example.

Children with JHS often present with verypronated, flat feet as a result of their hypermobility,which can contribute to lower limb symptoms.This problem will often respond very well to theuse of orthotics in the shoes, which take the form


of heel cups or arch supports that support andcontrol the subtalar joint and medial arch, helping to improve foot position. Although it can beargued that this may encourage weaker footmuscles, in practice the benefits of correcting thebiomechanics of the foot have such a positiveeffect on the whole gait pattern that it is a preferred form of treatment (Agnew 1997). In manycases correcting the biomechanics of the feet significantly reduces the abnormal forces throughout the other joints, thereby reducing the pain(such as an anterior knee pain) experienced.

Gait re-education

A combination of hypermobile joints, reducedproprioception, weak muscles and reduced stamina can profoundly affect the gait of a child withJHS. To correct this, the causes of the abnormalities need to be identified and treated separately.However, it is important that the child recognizesthe abnormalities in his or her gait and is taughthow to correct them. Positive feedback, usingvideo recording and mirrors, can aid this process.

Hand function

Finger joint laxity is one of the most common features of hypermobility in the upper limbs, and asa result hand function is often impaired. This isseen particularly in schoolchildren, where prolonged writing tasks become necessary. In somechildren it is important to include an exerciseprogramme specifically for the fingers and wriststo improve pain-free function and to protect thejoints. This can include exercises with putty,micro-theraband or wax, as well as assessing theposture during the performance of tasks andworking on any ineffectiveness. The use of pengrips or fat pens and regular periods of rest mayhelp with sloppy writing, but the provision ofsplints should be avoided. Occupational therapyassessment of writing function is often very usefulto help to correct abnormal positions of fingers,hand and general posture, as well as provide ideas


to correct this, such as the provision of a slopingwriting board. It is also important to consider theergonomics of the chairs and tables and otherschool equipment being used to ensure the optimum posture is achieved.

Functional tasks

Children with JHS often adapt to their hypermobility by altering the mechanics of how theyfunction, leading to increased pain, pain in otherlocations, and an increase in the fatigue suffered.It is therefore important to work on specific functional activities and to develop with the childenergy-saving, biomechanically correct, safe andpain-free ways of performing these activities.Movements involved in these activities can bepractised and included in the rehabilitation programme, i.e. step-ups on a stair, repeated sittingto stand from a chair, etc.

Balance

Given that proprioception and balance are oftenreduced in children with JHS, techniques relatedto these problems should be incorporated intothe programme. This can easily be done byencouraging the child to practise standing on oneleg, without shoes and socks on, while maintaining a good posture. It is important to ensure thechild is made aware of controlling knee hyperextension, if present. This exercise can be progressed by using a balance (wobble) board. Theuse of rhythmic stabilizations is also a usefulmethod to improve postural stability, both globally and specifically. Weight-bearing exercises areessential and should be given for both upperlimbs (prone kneeling etc.) and lower limbs.

General fitness

Children with JHS will often have been verysedentary because of their pain, and are usuallygenerally unfit. It is therefore important toincorporate some aerobic fitness work into therehabilitation programme. Care needs to be

taken early on, when they still have suboptimalmuscle strength, to ensure that the fitness aspectof the programme is of low impact so that jointsymptoms are not increased and the child andfamily do not lose faith in the programme. Theauthors have frequently seen resistance by thechild and family to physiotherapy interventions,as they have often experienced previous attemptsthat have increased their pain. This is usually aresult of starting the programme too vigorously,progressing too fast, and/or poor compliance.

Swimming is a highly desirable method ofexercise as it is usually 'low stress' for the jointsand is a good aerobic activity. It is preferable thata normal swimming pool is used for ongoingmanagement, as hydrotherapy pools are too hotfor distance swimming.

Bicycling is also very good for aerobic workand, similarly, does not overstress the other joints.However, as soon as strength is improving,normal aerobic and sporting activities can beincluded, providing they form part of the pacingprogramme.

Hydrotherapy

Hydrotherapy can be a very beneficial adjunct tothe treatment of JHS. The heat will provide painrelief and help to reduce any muscle spasm, andthe effects of buoyancy will support the joints,allowing strengthening exercises to be performed.This is often very useful at the beginning of thetreatment programme, when pain and stiffnessare often significant. Hydrotherapy provides manyopportunities to work on the strength of specificmuscles and stamina, as well as postural stability.

Pain relief

It is important for the child and family to understand that the pain is due to the hypermobilityand associated musculoskeletal insufficiencies,and not to any other rheumatological condition.The family often needs to be reassured that thepain will ease, but only when the muscles arestrong and fit and are protecting the joints more

fully, and when the child is functioning normallyboth biomechanically and generally (i.e. walkingcorrectly and attending school fully, etc.). It isoften found that the pain is slow to improve, andthis should be emphasized at the start of theprogramme. Children and parents are oftencounselled that rehabilitation will be prolonged.A child who has had 18 months of pain and disability may take a similar length of time beforereturning to full pain-free independent function.It is difficult to quantify the degree of pain that isacceptable within the rehabilitation programme,but we expect the pain to be moderate to mild indegree. It may be expected to recur on-and-offover the years, often becoming worse if the childbecomes unfit. It is important for the child andfamily to realize that the pain of hypermobilitydoes not signify damage or illness, but is an indicator of poor joint control and therefore benignand manageable.

Other methods of pain relief may be of use,such as hot or cold packs on specific joints. Transcutaneous nerve stimulation (TENS) may alsohave a role to play in this aspect, but it must beseen only as a supportive treatment and not asolution, and must not replace the rehabilitationprogramme. Relaxation and distraction techniques can be used to help the child or youngperson manage their pain. This is often useful atnight if they have difficulty sleeping because ofthe pain and discomfort. It can also be used inmany other situations, for example at school during breaks, to ensure that they are able to managethe rest of the day. Massage may also be a veryeffective way of easing any pain, especially if it isdue to muscle spasm.

Formal psychological input may be required,but in the authors' experience this is usuallynecessary in less than 10% of cases. Psychologicalsupport by healthcare providers for hypermobilepatients with pain is uniformly beneficial andprovided for most patients in our hospital. Thisincludes listening carefully to their pain storyand providing pain management strategies forthem to practise.


Pacing

Pacing is an extremely important part of therehabilitation of a child with ]HS.

It is very often the case that the child will reportthat they will play football, go swimming, go outwith their friends and dance at the weekend. Theyare then very sore on Monday and are unable toattend school; however, by the weekend they haverecovered enough to be able to do many activitiesagain, and the cycle continues. This causes a constant 'peaking and troughing' of symptoms andcauses a major disruption to life. Eventually thismay form part of a 'school refusal' pattern.

Pacing activities are taught to stop this behaviour completely and help them build up againslowly. Therefore specific tasks are set for eachday, which are achievable without giving rise to asignificant increase in symptoms. These tasksmust be completed, no more and no less, whetherit is a good or a bad day. For this programme towork effectively everyone involved in the care ofthe child, such as the child, parents, school andtherapist, should be included in planning the pacing. A written agreement may be used to facilitateprogression and to ensure that the programme isdeveloped between the therapist and the family.It is also useful to develop a 'back-up' plan ifproblems arise, to prevent the original problemsfrom developing again or, if they do, to bringthem more rapidly under control. Further guidance on pacing is given in Chapter 10.

Psychology

In some cases where pain and loss of independentfunction are profound, it is important to includethe clinical psychologist in the management team.They will be able to help develop pain management skills for the child, as well as generally helpthe family cope with the condition and to understand its impact on the whole family. Many familiesfind this support invaluable and are able to changemany unhelpful coping strategies into helpfulones, leaving behind the 'chronic pain cycle' thatmay have developed in the preceding phase.


Case study

RJIsa 12-year-old girl whoat theageof 10 presentedwithpainsIn her ankles. Initially the ankles became painfulIntheevenings of thedayshehadbeendoingPEatschool. Shestopped doingPEfora whileand,as the paindid not Improve, she rested at home fora week. When shereturned to school the painsbecame worse andspread toherknees andhips, andshealsodeveloped pain in herfingers. Shefound that the painwasworse in the eveningsafterschool.

Developmentally RJwasslowto crawlandto walk, andwasoftenfailing over. Shebruised easilyandsufferedpainIn her legsat night.

RJ'smother alsosuffered from growing painsas achild; she hadtrained asa dancer untilshewas21,andwasstili verymobile.

The GP, whocouldfind no evidence of anyswelling ofthejoints,suggested paracetamol and recommendedstaying at school.

Overthe next6 months RJwasableto go to schoollessand lessbecause of the pain, but found that shewasableto playwith herfriends andgoout shopping at weekends, although by Monday shewastoo sorefor school.TheGPthenreferred herto a paediatrician, whowasableto exclude anypathology anddiagnosed Joint hypermobility syndrome. Shewasthenreferred to apaediatric physiotherapist.

At the physiotherapy assessment RJwasstili complainIngof painIn herhands, wrists, hips,knees andankles.Thepainwasreasonably constant andprevented her fromgoingto school; however, shetriedvery hardat the weekendand wasableto go out andplaywithherfriends andspend the dayshopping withthem. Theyalsooftenwentbowling or Ice-skating Inthe evenings. Herschool hadbeenverysupportive andhadbeen sending workhome,butRJwasonlyableto writefor 10minutes before herhands became painful.

Onexamination all Joints were hypermobile andlax,andshescored 9/9on the Belghton score. Shealsohadconsiderable muscle weakness, bothproximally anddistally, averaging 3+/5 (Oxford Scale). Herbalance wasreduced andshehadpronated, flat feet.

An outpatient treatment planwasdesigned, startingwitha specific problem list:1. Pain In fingers, wrists, hips, knees andankles2. Global muscle weakness3. Poorbalance4. Abnormal posture5. Poorschool attendance6. Jointhypermobillty7. Reduced general stamina andfitness.Alma of treatment1. Educate RJandher family about JHSandthe

importance of a paced musculoskeletalself-management approach.

2. Teach RJhowto manage herpainto enable her tofunction independently.

3. Increase muscle strength, stamina andfitnessthroughout the body.

4. Improve general balance andstability.5. Imp(Ove posture.6. Develop a graduated return to school.

Ultimate goals1. Toachieve full attendance at school andparticipation

in all activities.2. Togaina full understanding of hypermobility and the

principles of self-management.3. Tofeel Incontrol of herpain.

Methods of treatmentInitially a home exercise programme was developed whichIncluded exercises to Improve balance, stability and thestrength of lower andupper limbmuscles. Theexerciseswere allstarted with 10repetitions and gradually progressedto 30.Once 30repetitions could beachieved correctly theprogramme was progressed further by theaddition ofweights, Initially 1 IbIncreasing to a maximum of3 lb.

Pacing of activities wasdiscussed at length with RJandher family, anda meeting wasarranged at herschool. Atthis meeting a gradual return to full function wasplanned.During week1 Itwasagreed thatshewould attend schoolfor 1 houra day, perform herexercise programme onceaday, participate Inonefunactivity, such as swimming for30minutes, andtake part ingentle activities around the home.At theweekend 1 hourof schoolwork wassubstituted forthe 1 hourat school.

Inweek2 sheIncreased her attendance at school tohalfa dayandcontinued herexercise programme onceaday, a funactivity for 30 minutes, andat the weekend shedid 1 hourof schoolwork and2 hoursof fun activity.

Thiswascontinued untilRJ achieved full attendance atschool andwasableto participate fully in weekendactivities as well.

Physiotherapy appointments were arranged initiallytwiceweekly for thefirst4 weeks, andthen reduced toonceweekly fora further4 weeks andthentwicemonthlyfor2 months. Shewasthengiven a review appointmentevery 3 months, withtheoption of anearlierappointmentif necessary. Afterthreereview appointments RJwasdischarged.

Initially physiotherapy consisted of teaching the homeexercise programme, relaxation techniques, explaining thecondition anddiscussing the treatment plan.During subsequent sessions the home exercise programme wasprogressed, and included exercising on a balanceboard, working on proximal stability andstrength usinga Swissball andweight-bearing exercises, increasingstamina usinga staticbicycle, Including functionalactivities suchas step-ups and intrinsic foot muscleexercises.

Insoles werealsoprovided to correct the position of thefeetandthereby Improve the biomechanics of the lowerlimbs.

OutcomeAfter1 month of treatment RJ wasbackat school full timeand wasableto join in several activities at the weekend;however, shestili had some pain.At 6 months RJwascompletely Independent andwasableto do all activities,butshestili hadsomepainIn her fingers. wristsandankles. At 1 yearRJ wasdischarged fromtreatment andcomplained of onlyvery ~ccaslona1 pain thatshecouldmanage by maintaining ~er exercise programme 3-4timesweekly with30 repeats anda 31b weight.

CONCLUSION

Joint hypermobility syndrome can present inmany young people. If untreated or undiagnosedit can result in the development of a 'chronic paincycle' and a high level of disability. This requires

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Chapter 4 – Hypermobility Syndrome in Children

Documents

murrayjoint hypermobility

ratio of type

joint stability

joint hypermobilltysyndrome

joint capsule

common genetic variations

marfan syndrome

clinical finding