in older adolescence (Hoft un et al., 2011 ). \231 Multiple common symptoms (including joint pain, headaches, abdominal pain) in childh ood are associated with a moderately increased risk of chronic widespread pain in adulthood (Jones et al., 2007 ). \231 Musculoskeletal disequilibrium \200\224these young people are still growing, oft en in their peripubertal growth spurt, and muscu- loskeletal pain can have lasting eff ects on the fi nal positioning (Lightman et al., 1987 ). Proprioceptive signals from the joints ar e reduced and the limb held in a rigid, fi xed position (Sherry et al., 1999 ; Walco et al. , 2010 ). Legs may \200\230give way \200\231. Knees and hips are held fl exed, feet are in verted, and hands are clenched with fl exed wrists. Th ese positions are oft en described a s the most comfort- able. Muscles and tendons quickly tighten and this complicates the pain and disability. Th e adaptive positioning of a young person with leg or abdominal pains pa rticularly aff ects the gait and resting positions and thus alters the loads on the spine a nd pelvis. Clinical features of chronic musculoskeletal pain As many features of musculoskeletal pain conditions are shared by most children, it is appropriate to describe the general presenta- tions at this stage, and then concentrate on specifi c pain-related conditions in their ow n right. General features It is not unusual for musculoskeletal pain to start in a localized area of the body (Buskila and Neumann, 2000 ). Th e pain can intensify and radiate to other areas. It is associated oft en with a reluctance to mobilize, and avoidance of movement. In adult s, avoidance and fear of pain and movement can reduce fi tness and further increase in pain (Elbaz et al., 2009 ), but in children this occurs on the background of a musculoskeletal system that is still develop- ing. Discomfort and pain intensity increase and become consta nt. As discrete pains multiply and continue, the young person may avoid contact with, or us e of, an area of the body aff ected, and this can then lead to muscular spasms, and abnorma lities of posture and gait. As gait alters, the resultant asymmetry can lead to other vulne rable sites (including lower lumbar, anterior knee, and lateral aspect of pelvis) taking an altered load and causing further discom- fort. Over time, aff ected limbs may adopt fl exe d positions, and ten- dons tighten, further exaggerating imbalance and pain. Th ere are a number of infl uences on physical behavior, including psychosocial factors. Wh at can fi rst appear as a structural anomaly or physical constraint may be better explained by fear of pain on movement, or postural compensation due to overprotection of a painful b ody part. Although an important area, there is very lit- tle research of the eff ects of a chronic stressor such as pain during critical periods of physical development, or in the me asurement of movement constrained by pain. Speci \201 c childhood musculoskeletal pain conditions Di \200 use idiopathic pain syndro mes (juvenile \201 bromyalgia/chronic widespread pain) Th is describes widespread pain, o ft en located over muscles and joints, and with signifi cant pain associated disability (Ma lleson et al., 2001 ). Th e term juvenile fi bromyalgia has been used, with diagnostic crit eria similar to those used in adults (Hoft un et al., 2011 ), including a history of widesp read pain for at least 3 months, and the presence of 11 of 18 bilateral tender points (occi put, low cervical, trapezius, supraspinatus, second rib, lateral epicondyle, gluteal, great er trochanter, and knees). It should be noted that these American College of Rheumatology c riteria have recently been revised and now do not include tender points as it is recognized that widespread pain and related symptoms are good markers for adult fi bromyalgia (Wolfe et al., 2010 ). Associated symptoms and signs \231 Hypervigilance and pain sensitivity that is greater than expected from a given physi cal trigger. Children oft en report a height- ened awareness of pain and pain associated cu es (American Pain Society, 2012 ; Malleson and Clinch, 2003 ; O \200\231Sullivan et al., 20 011). It is unclear whether this is caused by fear of pain, or increased sensitivity of pai n signaling. Clinically, this presents as young people describing unbearable pain on minima l skin contact, and heightened fear of being touched, for example, on examination. CHAPTER 24 non-inflammatory musculoskeletal pain 239 Th e onset of pain in DIP/juvenile fi bromyalgia is oft en gradual. Th ere may have been an initial insult or trauma but oft en there is no obvious trigger and only vague recollectio ns of the time of onset. Th e pain is generalized. Th ere may be areas of allodynia (profou nd hypersensitivity to light touch) and subjective hyperalgesia but there is oft en an abse nce of the autonomic changes (including reduced cutaneous perfusion, localized swelling, sh iny stretched appearance) seen in more localized pain conditions (O \200\231Sullivan et al. , 2011 ). Box 24.1 Budapest clinical diagnostic criteria for complex regional pain syndrome 1. Continuing pain, which is disproportionate to any inciting event. 2. Must report at least one symptom in three of the four following categories: a. Sensory: reports of hyperesthesia and/or allodynia. What is striking in the young people with diff use pain is the asso- ciated fatigue, poor s leep pattern, and extremely low mood. It is widely believed, however, that low mood in adol escents are reactive (to the pain-associated disability) rather than a primary depres- sion . Th is is in contrast to adults with fi bromyalgia where primary depression is frequently seen (Buskila et al., 1995 ). Menstrual cycling has been shown to aff ect fi bromyalgia-rel ated symptoms in nearly 50% of adult patients (Pamuk and Cakir, 2005 ), and this may be rel evant in the adolescent female population. b. Vasomotor: reports of temperature asymmetry and/or skin color changes and/or skin color asymmetry. c. Sudomotor/oedema: reports of oedema and/or sweating changes and/or sweating asymmetry d. Motor/trophic: reports of decreased range of motion and/ or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin). 3. Must display at least one sign at time of evaluation in two or more of the following categories: Complex regional pain syndrome Although it is almost 20 years since this condition fi rst r eceived attention in children, the diagnosis and treatment of CRPS contin- ues to be poorly understood. Many children receive investigations and rehabilitation for other presumed con ditions, resulting in a long journey before CRPS is recognized, and oft en a worsening of t he symptoms and associated disability (Berde and Lebel, 2005 ; Connelly and Schanberg, 2006 ). Th e diagnosis of CRPS remains a clinical one, and in adults is based on the Budapest C riteria ( Box 24.1 ) (Harden et al., 2010 ). Although there is frequently a precipitating t rauma, this is not present in around 10% of cases, and pain is usually out of proportion to the inciting event and accompanied by allodynia (Stanton Hicks, 2010 ). Children most oft en describe the pain as burning, with sensations akin to dysaesthesia. CRPS in pediatric pa tients is clinically distinct from the adult condition (Connelly and Schanberg, 2006 ; Stan ton Hicks, 2010 ): a. Sensory: evidence of hyperalgesia (to pinprick) and/or allo- dynia (to light touch and/o r deep somatic pressure and/or joint movement). b. Vasomotor: evidence of temperature asymmetry and/or skin color changes and/or asymmetry. c. Sudomotor/oedema: evidence of oedema and/or sweating changes and/or sweating asymmetry. d. Motor/trophic: evidence of decreased range of motion and/ or motor dysfunction (weakness , tremor, dystonia) and/or trophic changes (hair, nail, skin). 4. Th ere is no other diagnosis that better explains the signs and symptoms. Reproduced from Harden, R. N. et al., Validation of proposed diagnostic criteria (the \200 \230Budapest Criteria \200\231) for complex regional pain syndrome, Pain , Volume 150, Is sue 2, August 2010, pp. 268 \200\223274 with permission of the International Association fo r the Study of Pain (IASP). Th e fi gure may NOT be reproduced for any other purpose wit hout permission. \231 Th e lower limb is more commonly involved than the upper limb. \231 Th ere is a marked female predominance. \231 Th e peak incidence is in early adolescence (median 13 years). by lack of heat detected) of the wasted, fl exed, ulcerated foot that is shown in Figure 24 .3 . Magnetic resonance imaging (MRI) changes early in the condition can show bone oedema t hat may be confused with other musculoskeletal conditions. Later radiology may show osteope nia secondary to disuse (Berde and Lebel, 2005 ). Limbs can become distorted and feet/hands held in seemingly fi xed and oft en fl exed positions. \231 Dystrophic changes and long-term disability are less common than in adults. Signs and symptoms may completely resolve within several months to a few years, but relapse s may also occur (Connelly and Schanberg, 2006 ; Stanton Hicks, 2010 ). Occasionally more t han one limb may be aff ected at presentation (Stanton Hicks, 2010 ) or symptoms may progre ss or migrate to a hand or other leg. Th is may be due in part to an increase in mechanical load through the arms through the use of crutches and subsequent mechanical pain. Autonomi c changes include swelling, reduced cutaneous perfusion, and thermodynamic instability. Th e aff ected limb develops a pur- plish hue with other color changes that can cause concern to the child and family ( Figure 24.1 ). Th e skin takes on a shiny, stretched appearance, with coarse hairs developing in patches (Harden et al., 2010 ). In rare cases, severe troph ic changes develop with ulcera- tion and marked wasting. Figure 24.2 shows the marked chang es on thermography (reduction in cutaneous blood fl ow represented While the pathophysiology of CRPS is poorly understood, a number of aetiologies have been p roposed: a peripheral small fi bre neuropathy, autonomic dysfunction, and exaggerated regio nal infl ammation (Stanton Hicks, 2010 ). However, infl ammatory aspects diff er from those seen in other conditions involving tissue infl ammation. Th e suggestion that CRPS in chil dren is a diff erent clinical entity than that seen in the adult, is probably incorrect, as recent evidence would suggest that the pathophysiology is most likely to involve similar e ndocrine, behavioral, developmental, and environmental factors (Stanton Hicks, 2010 ). Many features, particularly the neurological abnormalities, suggest both peripheral SECTION 4 pain in specific populations and diseases 240 Figure 24.3 Wasted, ulcerated left leg in advanced CRPS. Reproduced from Richard Watts, Phi lip Conaghan, Chris Denton, Helen Foster, John Issacs, and Ulf M ller-Ladner (eds) Oxfor d Textbook of Rheumatology , Fourth Edition, Oxford University Press, Oxford, UK, Copyright 2014, by permission of Oxford University Press. Factors related to LBP in children and adolescents include: \231 LBP is more common among girls and increases with age (Kjaer et al., 2011 ; Watson et al., 2002 ). Figure 24.1 Early CRPS changes, left leg. Reproduced from Richard Watts, Philip Conaghan, C hris Denton, Helen Foster, John Issacs, and Ulf M ller-Ladner (eds) Oxford Textbook of R heumatology , Fourth Edition, Oxford University Press, Oxford, UK, Copyright 2014, by pe rmission of Oxford University Press. \231 Risk factors are poor physical condition, reduced strength in mus- cles that support the back (Diepenmaat et al., 2006 ), intensive sports (Lundin et al., 2006), and reduced h amstring length (Sjolie, 2004 ). \231 A prospective study of adolescents showed a strong correlation between LBP, emotiona l and behavioral problems, as well as other physical problems such as headaches, stomach ac hes, sore throat, and fatigue (Watson et al., 2002 ). Th e vast majority of children with LBP who come to paediat- ric rheumatology clinics impro ve with physiotherapy; however, a minority proceed to develop increasing pain-related disab ility (Ahlqwist et al., 2008 ). Chronic pain among adults may, in part, be rooted in untrea ted painful experiences in childhood and young people who have suff ered from LBP are more likely to suff er from these problems as adults (Kjaer et al., 2011 ). Few treatment studies on children and adolescents with LBP have been published. One study o f children and adolescents shows that individual assessment and follow-up, including an act ive treatment intervention by an experienced physiotherapist, improves self- perceived heal th and function and reduces pain, while increasing mobility and strength (Ahlqwist et al., 2008 ). Another study shows that an exercise program tailored for children and adolescents with recurrent non-specifi c LBP has benefi cial eff ects, including reduced pain intensity (Jones et al., 2007 ). Figure 24.2 Loss of cutaneous capillary blood \202 ow in left foot on thermography. Repro duced from Richard Watts, Philip Conaghan, Chris Denton, Helen Foster, John Issacs, and Ulf M ller-Ladner (eds) Oxford Textbook of Rheumatology , Fourth Edition, Oxford University Press, Oxford, UK, Copyright 2014, by permission of Oxford University Press. and central nervous system (CNS) involvement, and considerable CNS circuitry changes have r ecently been shown with functional MRI in young people with a diagnosis of CRPS (Lebel et a l., 2008 ). Red fl ags for pathology include nocturnal pain, presentation in young children, or neurolo gical symptoms indicating nerve compres- sion/damage. Th ese cases should be fully investig ated to exclude: \231 Malignancies (e.g. sarcoma, neuroblastoma, metastases). Back pain Recent research has shown that the prevalence of LBP has increased among children and adolescents ranging from 16.4% to 36% (Deere et al., submitted; Kjaer et al., 2011 ). Mid-back pain and neck pain are also frequently seen (9.4% and 8.9% respectively of adolesc ent population; Deere et al., submitted) but the literature evaluating these areas is less robust. \231 Vertebral damage (trauma \200\224accidental and non-accidental). \231 Infection (osteomyelitis, abscess) and infl ammation (arthritis, disciitis). \231 Rare conditions such as chronic recurrent osteomyelitis, Langerhans \200\231 histioc ytosis, and collapse secondary to iatrogenic or idiopathic osteoporosis. CHAPTER 24 non-inflammatory musculoskeletal pain 241 Juvenile idiopathic arthritis and pain Despite signifi cant advances in medical treatments for children with JIA, persistent pain is a common complaint (Anthony and Schanberg, 2007 ; see also Chira and Schanberg, Chapter 22, this volume). Pain has been shown to be a primar y determinant of the physical, emotional, and social functioning in these children (Anthony and Schanberg, 2007 ). Th e degree of disabling pain does not always mirror infl ammatory joint activity, and a growing body of research highlights the importance of environmental a nd cog- nitive behavioral infl uences in the pain experience of children, in addition to th e contribution of disease activity (Connelly et al., 2010 ; Vuorimaa et al., 2009 ). to speculation that there may be a vascular component to the bone pain experienced. A recen t small study challenged this theory (Hashkes et al., 2005 ). Others describe growing pains as a form of overuse injury (Friedland et al., 2005 ). Th e jury remains out. Joint hypermobility and pain Joint hypermobility results from ligamentous laxity and may oc cur in individuals with a primary genetic disorder aff ecting connective tissue matrix prot eins (e.g. osteogenesis imperfecta or Marfan \200\231s syn- drome) or other syndromes (e.g. trisomy 21, bony dysplasias, or velo- cardiofacial syndrome). In the majority of cases, hy permobility exists as an isolated fi nding (referred to as \200\230generalized joint laxit y \200\231), but it may also be associated with musculoskeletal symptoms such as pain and \200\230clicking joints \200\231 in the absence of known genetic causes, in which case it i s referred to as \200\230hypermobility syndrome \200\231 (Clinch et al., 2011 ). A method of examining and scoring for hypermobility ( Figure 24.4 ) was developed by Beighton in 197 3 (Beighton et al., 1973 ). Th e Beighton score was devised in South Africa and based on 10 83 Tswana Africans and has subsequently been used interna- tionally to defi ne generalized joint laxity in all populations and all age groups. It has not been validated in children. Idiopathic chronic limb pains Th ere are many presentations of musculoskeletal pain in chil dren that do not meet the criteria for DIP/juvenile fi bromyalgia or CRPS. Th ese include t he poorly understood \200\230growing pains \200\231, described as recurrent, bilateral, no n-articular pain in the lower extremities that occur late in the evening or at night. Th ey have been observed to coexist with vascular pain problems including migraines, leading (1) The Beighton score (4) Beighton \200\231s modi \201cation of the Carter and Wilkinson scoring system, Give yourse lf 1 point for each of the maneuvers you can do, up to a maximum of 9 points. (2) (5) (3) SCORE Left Right 1 1. Can you put your hands \202at on the \202oor with your knees straight? 2. Can you bend your elbow backwards? 3. Can you bend your knee backwards? 4. Can you bend your thumb back on to the front of your forearm? 5. Can you bend your little \201nger up at 90 (right angles) to the back of y our hand? 1 1 1 1 1 1 1 1 Figure 24.4 Calculation of the Beighton score. Reproduced with kind permission from Arthrit is Research < http://www.arthritisresearchuk.org/ >. 9 SECTION 4 pain in specific populations and diseases 242 It is widely believed that children are more fl exible than ado- lescents, but there is ver y little supporting literature. For example, one rigorous population-based study from Swede n (Jansson et al., 2004 ) investigated 1845 children aged 9, 12, or 15 years from 48 geogra phically randomly selected schools and showed that at all ages, girls had a higher degree o f generalized joint laxity as assessed by the modifi ed Beighton criteria. Girls had the hi ghest degree of general joint laxity at the age of 15 years, whereas joint laxity in boys d ecreased with increasing age. Similarly, a study of high school basketball players (Quatman et al., 2008 ) showed that aft er the onset of puberty, girls exhibited greater joint laxi ty than boys. A recent robust UK population-based cohort study showed that the prevalence o f generalized joint laxity in girls and boys (mean age 13.8 years) was 27.5% and 10.6%, res pectively, when the commonly used cut-off of four hypermobile joints from the modifi ed Bei ghton 9-point scoring system was used (total of 1156/6022 children; Clinch et al., 2011 ). Th is is arguably within the normal variance of the adolescent population, and suggests tha t a higher cut-off (such as \211 6/9) or a new, more meaningful measure is needed. mechanisms for dealing with short-lived demands or disruption. However, a young person with signifi cant musculoskeletal chronic pain typically demands sustained physical, emotional, and fi nancial resources (Roth-Isigkeit et al., 2005 ). Young people with muscu- loskeleta l chronic pain report sleep disturbance, disordered mood, appetite disruption, low feelings (depression is oft en masked in this population), social isolation, and unwelcome dependen cy on parents. Th ese are experienced chronically and can serve to main- tain pain and disa bility. Th ey are also diffi cult to manage in isola- tion from other symptoms. Adolescence is by defi nition a time of change and experimentation; adolescents with chronic pain repo rt that they are less socially developed on virtually every metric than their peers (Eccles ton et al., 2008 ). Parents also report signifi cant distress from living with a child in persistent pain. Leve ls of parental stress are clinically signifi cant (Eccleston et al., 2004 ) and parents exp erience severe distress and confl ict in parenting their child. Typically, they report stru ggling with the desire to cure their child \200\231s pain, and comfort their child, recogni zing that the desire to protect may be counterproductive (Jordan et al., 2007 ). Th e extent to which generalized joint laxity is associated with signifi cant clinical sequ elae, including joint pain, is unclear. A proportion of children noted to be hypermobile ca n present with recurrent lower-limb arthralgia, anterior knee pain syndromes, and back pain . Th e prevalence of pain among children with generalized joint laxity has been reported to range from 30% (El-Garf et al., 1998 ) to 55% (Qvindesland and Jonsson, 1999 ). However, r eports linking hypermobility with joint pain in school children suff er from problems with sample size, methods of assessing hypermobility, and methods of assessing pain. By contrast , a cross-sectional study of schoolchildren showed no association between joint laxity and pain (Leone et al., 2009 ). A recent evaluation of over 4000 17-year- old adolescents using a higher cut-off for the Beighton score ( \211 6 joints) showed that there is a relationsh ip at this age between hypermobility and pain in the knees and shoulders. Th ere was no rel ationship between hypermobility and chronic widespread pain/ fi bromyalgia or back pain (To bias et al., 2013 ). A large cohort study has also shown a positive association between gen eralized joint laxity (Beighton score \211 6) and habitual levels of physical activity, bo dy mass index, and maternal education in girls (Deere et al., submitted). Managing chronic musculoskeletal pain in childhood/adolescence Assessment History, examinat ion, and investigations are required to exclude signifi cant underlying disease. Although i t is common and neces- sary to be clinically conservative and investigate multiple poten- t ial causes, if prolonged this can also lead to a diagnostic vacuum and lack of treatment pl anning that allows for fear, disability, and depression to grow. With serious medical cause s excluded one can then safely move on to rehabilitation. In pediatric practice this may be more diffi cult as there can be a tendency to catastrophize about persistent pain by the f amily, and sometimes by the physician. Th e need to fi nd a cause for the pain can be overw helming and, in this way, the child may be subject to a large number of medical inves- tiga tions and interventions that are, at best, not appropriate and cause anxiety. For example, adolescents commonly present to pae- diatric rheumatology clinics with back pain; it is imp ortant to rule out pathology including malignancy and infl ammation but to then move forwar d swift ly with reassurance and rehabilitation. All too oft en children are moved between d iff erent specialities and have further investigations over a long time period; in many ins tances this causes delay in rehabilitation and increased disability (Clinch and Eccleston, 2009 ) What seems clear is that the majority of hypermobile individuals will not have signifi cant pain or have any risk for specifi c muscu- loskeletal disorders (including osteoarthritis) in later life. Screening tools such as the Beighton score are likely to be inadequate in c hil- dren as they are generally more mobile than adults (Deere et al., submitted). Th e cha llenge remains to interpret symptoms correctly as being related to the joint hypermobility and to predict why some children become symptomatic. Th e answer is likely to involve phys- iological and psychosocial factors. History For the physician, the goals of history taking are to exclude serious possible caus es, identify key problems, build a trusting relation- ship with the patient (and family), a nd, if possible, identify a treat- ment plan. Th e main diff erential diagnoses in musculos keletal pain are trauma, infl ammation, infection and malignancy ( Box 24.2 ). Many childre n with persistent musculoskeletal pain not only have a chronic experience of pain and distr ess, but a chronic experience of medical settings, feelings of being doubted, having the va lidity of their pain contested, and oft en a history of failed and pain exac- erbating inte rventions. Th ree key psychological features of this set- ting are worth keeping in mind: Th e impact of chronic musculoskeletal pain on the child and family Chronic musculoskeletal pain, irrespective of its trigger, can be associated with persistent and recurrent distres s, disability, adult attention, and widespread family disruption (Perquin et al., 2000 ). P ain-related anxiety in JIA is a signifi cant problem for many chil- dren and independently aff ects their social development (Gauntlett- Gilbert et al., 2013a ). Most families have r elatively successful CHAPTER 24 non-inflammatory musculoskeletal pain 243 \231 Both child and family are likely to be fearful, hoping for a cure, desperate to be h elpful, but oft en \200\230on their best behavior \200\231, so may not immedi- ately appea r to be distressed, and may have fl at or neutral aff ect. Box 24.2 Key pathology to exclude in musculoskeletal history 1. Infl ammation: \231 Because of their history, any attempt to shift the treatment goal from one of cure t o management or symptom control may be heard to be patient and carer(s) blaming, and so sho uld be done only aft er building trust. a. JIA: pain centered around joint but may be poorly localized (particularly in younger chi ldren). Usually more severe in morning and accompanied by stiff ness. Reports of joint swel ling in peripheral joints. Inflammatory backpain (including sacroiliitis) worse in morning and can wake child up at night. \231 What matters is not necessarily what information has been given to the patient and c arers, but what they believe to be true. Typically both patient and family will have heard a variety of sto- ries about what may be happening to them (Clinch and Eccleston, 2009 ). F or example, a family who have a child with many pain- ful joints and associated disability may have family members or friends who have a history of bone pain related to other pathol- ogy (arthritis, cancer, infection, etc.). Despite reassurance this fear oft en remains. In consistent messages from health profes- sionals and repeat investigations serve to reinforc e the anxiety. b. Discitis: infl ammation of vertebral disks. More common in young children. Severe back p ain, worse on movement and can wake at night. c. Myositis: infl ammation of muscle groups. Muscle pain and fatigue. May follow infection or a presenting feature of rare conditions such as juvenile dermatomyositis (rash will acco mpany this). d. Chronic recurrent osteomyelitis: rare infl ammatory condi- tion aff ecting discrete port ions of bone. Severe bone pain, may wake at night. Bone swelling seen if severe (particular ly on clavicle). Physical examination Time spent on full physical examination at the beginning may pre- vent repetition and unnecessary, distressing investigations at a later date (Anthony and Schanb erg, 2007 ; Clinch and Eccleston, 2009 ). If there is concern regarding the diagnosis, then this is the time to order all investigations and ensure that these are followed up. Undue delay leads to fear and oft en a worsening of pain symptoms and associated disability. On a ssessment a full musculoskeletal examination would show evidence of, and target investigati ons to rule out: e. Osteoid osteoma: benign painful lesions in bones. Pain at any time. May wake at night. Localized. 2. Infection: a. Septic arthritis: usually acute presentation but can per- sist. Hot, swollen, painful jo int. Child may be systemically unwell. \231 Infl ammation (including JIA, myositis, myopathy, connective tis- sue disease, chronic recurrent mulitfocal osteomyelitis). b. Infective osteomyelitis: again usually acute but can develop Brodie \200\231s abscess an d persistent bone pain if not identifi ed early. \231 Infection (including septic arthritis, infective osteomyelitis). \231 Malignancy (including osteosarcoma, Ewing \200\231s sarcoma, leukae- c. Tuberculosis: TB can seed in the spine (Potts disease) or joint and cause persistent pai n/swelling. A history of travel or contact is important. mia, neuroblastoma). \231 Trauma (including non-accidental injury). Pediatric GALS screening (child version of the Gait, Arms, Legs, and Spine adult examinatio n) is a musculoskeletal examina- tion that can be completed within a short time period on c hildren of all ages (Foster et al., 2006 ; < http://www.arthritisresearchuk.org/ health-pro fessionals-and-students/video-resources/pgals >). 3. Trauma: a. Accidental: enquire about any accidents, sporting injury. Th is can be missed. b. Non-accidental injury: it is extremely important that unu- sual musculoskeletal pain pat terns or inconsistent history of events is investigated. Psychometric instruments Musculoskeletal pain intensity in children has long been meas- ure d using simple severity measurement tools, such as the visual analog scale (VAS; Carlsson, 1983 ; see von Baeyer, Chapter 36, this volume). Th ese give a subjective measure of the pa in intensity according to the adolescent and/or pain perceived by their carer. While useful measures, they give no indication of the impact of pain on the young person \200\231s life (see Th am et al., Chapter 41, this volume). Two recently developed multidisciplinary tool s that have been validated in JIA-related chronic pain and the more general pediatric pain population include: c. Osteogenesis imperfecta: these children may have a history of bone pain with no or very little trauma. Th ey suff er multi- ple fractures. 4. Malignancy: a. Hematological malignancy: bone pain (night and day) and eff usions can be the presenting features of leukemia. b. Osteosarcoma, Ewing \200\231s sarcoma: localized persistent pain (day and night). c. Neuroblastoma: oft en in younger children. Localized joint \231 Bath Adolescent Pain Questionnaire (Eccleston et al., 2005 ) for measuring the impac t of pain on adolescents with chronic pain (now used in many tertiary pediatric rheumatolog y settings). pain with or without history of swelling. Note: a full medical history is important to exclude other diseases asso- ciated with joint pain (including infl ammatory bowel disease, cystic fi brosis). \231 Bath Adolescent Pain Questionnaire for Parents (Jordan et al., 2008 ) for measuring the impact of adolescent chronic pain on parents. SECTION 4 pain in specific populations and diseases 244 Physiological measures of pain-related indices Th ere are little data on the role or utilit y of measuring physiologi- cal processes in the context of childhood chronic musculoskeleta l pain. Th e role of thermography and bone scintigraphy in CRPS has been studied (Goldsmith et al., 1989 ; Lightman et al., 1987 ) in the early and late stages of this pain condition , but opinion is divided on their usefulness. Functional MRI has recently been shown to be a useful tool in evaluating CNS changes in childhood CRPS (Lebel et al., 2008 ). Quantitati ve sensory testing (QST) is a valu- able tool for assessing sensory perception (including p ain) in chil- dren (Blankenburg et al., 2010 ; see also Hermann, Chapter 40, this volume). Education about pain may be diffi cult to grasp because there is a dominant cultural view o f pain in joints and muscles as a warn- ing sign that a disease process or abnormality is p resent, and there is oft en a need for the young person and family to identify a cause. Edu cation can play a valuable role as a fear reduction strat- egy, providing information to co unter beliefs that that they have a unique medical complication, or that their symptoms are related to an underlying mysterious disease (Clinch and Eccleston, 2009 ). Children and th eir parents oft en have infl exible or rigid ideas not only about the cause of pain, but al so of pain as an important signal of damage or disease. Back pain is extremely common but r arely related to malignancy or arthritis. It can be helpful to show and reinforce to the fa mily the fact that many childhood pains have no function, or have outlived their usefulness . Rhetorical devices (metaphors, stories, examples, pictures) that counter this rigid think ing can be helpful. Explaining the fascinating case of phantom limb experiences can also be helpful, to introduce fl exibility to the idea that brain signals must signify peripheral damage. Rehabilitation and self-management Th e primary treatment approach to the young person with chronic musculoskeletal pain is one of symptom management and psycho- social rehabilitatio n. Although it may be tempting to focus solely on the amelioration of the suff ering of the child, one of the most important aspects of rehabilitation is \200\230inclusion \200\231. A dedicated team that works consistently with the adolescent and family will facilitate co mmunication, ensure eff ective delivery of therapy, reduce iatro- genic infl uences and ena ble goals to be reached earlier (Christie and Wilson, 2005 ;Walco et al., 2010 ; see also B irnie et al., Chapter 12, this volume). Most approaches to rehabilitation share common fea- tures that include education, symptom control, behavioral science and physical therapy. Ho w these strands are delivered is debated. Certainly early recognition and intervention in a n outpatient or inpatient setting is key and, in most cases, leads to a more favor- able ou tcome (Anthony and Schanberg, 2007 ). Pharmacotherapy Many analgesics and diff erent interventions have been used for chil- dren with chronic musculoskeletal pain, but are not supported by well-controlled therapeutic tri als. It is becoming widely accepted, however, that any analgesic intervention should only b e used along- side multidisciplinary therapy (Eccleston et al., 2003 , 2009 ). Psychological therapies A Cochrane systematic review reported that psychological thera- pie s are eff ective for headache and may improve pain control in children with musculoskeletal pain (Eccleston et al., 2009 ). Small studies using cognitive-behavioral therapy (CBT) in juvenile fi bromyalgia (chronic widespread pain) show signifi cant improve- ment in the chi ld \200\231s confi dence with managing the pain, but disap- pointing eff ect on functional disability and depressive symptoms (Kashikar-Zuck, 2006 ; see also Logan et al., Chapter 50 , this vol- ume). For children who have signifi cant pain-associated disability (school abs ence, limited mobility, lost independence, extremely low mood, disturbed sleep) there are n o randomized controlled trials of multidisciplinary multicomponent CBT; however, there is e vidence that an interdisciplinary program has a positive impact for adolescents disabled wi th chronic pain (Eccleston et al., 2003 ). Th e role of the physician is oft en as an educa tor, to oversee analge- sic withdrawal and to support the overall message that it is safe t o increase activity despite pain and reduce reliance on medical sup- port (Clinch and Eccle ston, 2009 ). Th ere is no current evidence favoring a dedicated outpatient over inpatient service for mu sculoskeletal pain; however anecdo- tally it is reported that treating children away from b usy acute set- tings and keeping them in their local area, if possible, is benefi cial (Cli nch and Eccleston, 2009 ; Eccleston et al., 2003 ). Dedicated pain residential units are able to provide physical and psychological intense tre atment for children with signifi cant pain associated disability. Musculoskeletal pain comp rises approximately 60% of the presenting conditions in these units and over 80% of the chi ldren have related limb/back pain. Outcomes from these units show that pain does not signif i cantly change but function (includ- ing back to school), anxiety and coping is measurably improved (Eccleston et al., 2003 ). Recently the role of parents in pain behav- iour has b een evaluated (McCracken and Gauntlett-Gilbert, 2011 ), and parents are key in rehabilitati on plans. Specifi c parent pro- grammes in residential pain centers have been developed and show that changing parent behavior and responses to their child \200\231s pain, assists th e child moving on to more optimal outcomes (Gauntlett- Gilbert, 2013a ; McCracken and Gaunt lett-Gilbert, 2011 ). Physical therapies In conditions such as CRPS, early intensive physiotherapy (includ- ing d esensitization) with behavioral support can provide dra- matic reversal of the presenting s igns and symptoms for some patients (Littlejohn, 2004 ). Th e aim is accelerated mobilizati on (Lee et al., 2002 ), but this is hindered if the diagnosis is delayed and access to appr opriate physical and psychological rehabilita- tion is limited (Littlejohn, 2004 ). Many ca ses of diff use pain will require a gentle, paced approach. In all cases, the increase of a ctivity should be consistent despite the pain. With musculoskel- etal pains the more active the musculoskeletal system becomes the more likely the muscle spasms and tightening are to reduce. Education Although the evidence for education alone as a treatment for behav- iour change i s lacking, the provision of a rationale for what are oft en counter-intuitive instructions in self-management is essen- tial. Additionally, one should never underestimate that the av erage understanding of musculoskeletal anatomy and physiology is oft en limited. A critical fi rst step in rehabilitation is to off er, reoff er, and reinforce an understanding of ho w one \200\231s body may be working to maintain pain. CHAPTER 24 non-inflammatory musculoskeletal pain 245 Proprioception improves (Lee et al., 2002 ; Low et al., 2007 ) and autonomic changes subsid e. Where possible the young person should work to devise his or her own \200\230fi tness p lan \200\231. Using a local gym rather than a hospital physiotherapy gym allows them to sta rt to return to a more normal environment (Eccleston et al., 2003 ). Working in this consis tent and paced manner can be extremely hard for the young person and their parents. Th e pa in invariably continues at the beginning (if not throughout) and motivation is poor. Parent al anxiety is high (Lee et al., 2002 ) and there is a fear that damage will be done. Psycho logical support during this time is important. Th e young person will need help setting goa ls, learn- ing how to communicate pain to peers and family, maintaining motivation on \200 \230bad days \200\231, managing low mood, dealing with anger and frustration, and overcomin g fears. Oft en they have not been at school for a long period of time and need help in pre paring again for this diffi cult environment. In some cases, there may be other mental heal th needs that can be identifi ed and appropriately treated. See also Holsti et al. (Chapter 57, this volume) and Tupper et al. (Chapter 56, this volume) for further details. \231 Evidence shows that early dedicated therapy can signifi cantly improve the outcome o f childhood chronic musculoskeletal pain. \231 Key areas for further research include evaluating the physiology of childhood muscul oskeletal pain, the role of parents, and eval- uating the effi cacy of pharmacological inte rventions. Case example