The limping child and an introduction to paediatric musculoskeletal clinical skills

Professor Helen Foster

15 July 2020 This slide deck

• Important aspects of musculoskeletal clinical skills in context of limp – History taking and Examination – Apply Clinical Reasoning in clinical cases

www.pmmonline.org Introduction

• Please feel free to use these slides in your teaching and add your own cases to highlight key points • Some links to key web pages are provided on the slides to help navigate around PMM • There are lots of images, videos and cases on PMM to supplement these slides Learning outcomes

• Be aware and practice how to perform pGALS • Know about limp – Spectrum of causes – Approach to assessment and investigations – Key conditions that can present with limp • Limp as a common presentation to emergency departments e-Resources – all free

www.pmmonline.org

• History • Examination pGALS, pREMS Video demos • Red flags • Normal development • Cases Knowing what is normal is important

• Motor milestones

• Leg alignment / normal variants

• Ranges of joint movement

http://www.pmmonline.org/doctor/approach-to-clinical- assessment Hypermobility is common often ‘benign’ (a normal variant) • Younger the child the more flexible • More so in girls and non-Caucasians. Often familial • Changes are symmetrical, usually symptom free • Advantage in sports – dancers, gymnasts • Remember: – Children with rheumatic disease or other pathology can also be hypermobile ! – Don’t forget rare but significant causes of hypermobility • Marfanoid syndromes (family history, body habitus) • Ehlers Danlos (elastic skin, scars) • Osteogenesis imperfecta (family history, low trauma fractures, blue sclera) http://www.pmmonline.org/doctor/approach-to-clinical-assessment/normal-variants/hypermobility Growing pains

• Young children often get non-specific aches and pains in their legs – Often after exercise – Often runs in families and with hypermobility • Pain can be severe and disturb children (and parents !) at nights • A common cause of presentation to primary care as parents are concerned http://www.pmmonline.org/page.aspx?id=808 The “rules” of Growing pains

• Age 4-12 years • Bilateral (symmetrical) • Never present in the morning • Never limps • Milestones normal • History and examination normal – Hypermobility is common http://www.pmmonline.org/page.aspx?id=808 Adapted from EMD Smith Arch Dis Child Educ Pract Ed 2012;97:185-193

http://www.pmmonline.org/doctor/limping-child Arch Dis Child Educ Pract Ed 2012;97:185-193 What is a limp ?

• Abnormal / asymmetrical gait • Usually painful but not always • Various causes – Congenital/developmental or acquired causes – Pathology (infection, trauma, inflammation, trauma, malignancy, metabolic, developmental, etc) – Site (anywhere in lower limb, spine, abdominal, genitalia)

http://www.pmmonline.org/doctor/limping-child Causes of Limp – by pathology

• Trauma – fracture, contusion, sprain • Infection – septic arthritis, osteomyelitis, TB • Neoplasia – bone, leukaemia, lymphoma • Inflammatory – Juvenile Idiopathic Arthritis, transient synovitis/irritable hip, reactive arthritis • Metabolic – rickets, genetic syndromes (usually symmetrical abnormal gait) • Congenital – Developmental Dysplasia Hip (DDH), short leg, club foot • Neuromuscular – Cerebral palsy, myopathies • Developmental – Perthes, Slipped Capital Femoral Epiphysis (SCFE), Tarsal coalition • Other – Testicular torsion, inguinal hernia, appendicitis, urine infection Causes of limp

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EMD Smith Arch Dis Child Educ Pract Ed 2012;97:185-193 ‘Red flags’

Infection, malignancy or non-accidental injury • Unwell child • Fever, malaise, systemic upset, reduced appetite, weight loss, night sweats • Bone or joint pain with fever – ‘red hot joint’ • Refractory or unremitting pain • Persistent night-waking • Incongruence between history and presentation • Child abuse / safeguarding

http://www.pmmonline.org/page.aspx?id=384 Key Conditions to Focus on

• Common: – Irritable hip/transient synovitis • Important: – Perthes Disease – Slipped Capital (Upper) Femoral Epiphysis (SCFE) – Juvenile Idiopathic Arthritis • Serious/life threatening (red flags): – Acute Lymphoblastic Leukaemia – Septic Arthritis Non-weightbearing or ‘pseudoparalysis’

• Always Serious !

• Sepsis • Fracture • Malignancy

• Acute hip - SCFE / transient synovitis/ Perthes The limping child

• Diagnosis rests on history, examination and careful consideration (and interpretation) of investigations

http://www.pmmonline.org/doctor/limping-child

http://www.pmmonline.org/doctor/limping-child/making-a- diagnosis • Key points History – Age – What has been observed - What are the concerns ? – Onset and duration - acute / chronic / precipitating factors ? – Course - intermittent / getting worse ? – Pain / weakness / falls / ‘clumsy’ ? • Lack of reported pain does not mean no pain • Change in mood and behaviour may be surrogates for pain – Nature (of pain) • Site – remember may not localise / consider referred pain • Resolving or getting worse? Night pain ? Response to analgesics or NSAIDS ? – Systemic Upset / red flags ? • Weight loss / Night sweats / Fever / Night pain – Development and Motor Milestones – Social history - concerns about safeguarding – Trauma (careful… all children fall over ! ) Also ask…

• What can the child do / no longer do ? – Home / Schoolwork (handwriting) / Play / Sport – Report from others (teachers?) • Consider motor milestones (e.g walking, stairs, sitting) – Regression – suggests acquired pathology – Delay – suggests developmental / congenital cause Also ask…

• Any suggestion of diurnal variation ? – Morning stiffness / gelling (car rides, after sitting) • Consider inflammation – Joints / muscles / both – Ask about swollen joints – Ask about joints not just in the legs ! Examination • Screening - overall assessment – Vital signs (‘sick child’?) – Abdomen, pelvis, genitalia, back, and extremities • Inspection and palpation • Stance and Gait ..pGALS is a good start ! – Barefoot and minimally clothed – Observe several gait cycles – Running often accentuates subtle abnormalities • Specific – ‘Look, Feel, Move, Function’ and upper limbs too – Occult trauma (soles of feet / shoes!) – Spine – Neurological • Measure and compare leg lengths • Comparing both sides really helpful Investigations • Imaging studies – Plain X Rays -AP pelvis +/- AP and lateral views of both lower extremities • Long bones as well as joints / look out for unsuspected fractures – Ultrasound (Joint / muscle +/- abdo) – CT scan (Abdo / pelvis / bony detail) – MRI (soft-tissue pathology, evaluation of bone tumours) – Isotope bone scan very rarely used if MRI available • occult fractures, osteomyelitis, Perthes disease • Laboratory studies – FBC, differential +/- film, ESR and CRP – Urinalysis +/- culture – Blood cultures / Joint aspiration if suspicion of septic arthritis / TB • Synovial fluid analysis - cell count, glucose, culture and Gram / ZN stain – +/- Muscle enzymes, vitamin D, Bone chemistry – +/- autoantibodies (ANA / Rheumatold factor) – +/- bone marrow – +/- catecholamines (neuroblastoma) http://www.pmmonline.org/doctor/investigation-and-management Autoantibodies

• Antinuclear antibody (ANA) – Not diagnostic! Positive in many transient illnesses – Detected in JIA, SLE • Rheumatoid factor – Not diagnostic ! Often negative in JIA • BUT in a clinical picture of arthritis or suspected multisystem disease they help to establish diagnosis and prognosis – JIA (ANA associates with risk of uveitis, RF associates with worse prognosis) – SLE (dsDNA more helpful)

http://www.pmmonline.org/doctor/investigation- and-management/blood-and-urine-tests/auto-abs Some cases Boy aged 6 years

• Limp 2 days, getting worse • Today can hardly weight at all • Pain in knee • Fever and generally unwell • Examination – red ‘hot’ swollen knee – he winces when you approach his knee and cries when you touch it. No movement is possible. Thoughts

Investigations

Differential diagnosis

WCC high ESR high Xray normal Septic Arthritis and Osteomyelitis • Pathophysiology: – Bacterial infection of the joint +/- bone • Clinical features: – Acute onset of fever with unexplained limp – Reluctance to use a limb or inability to weight bear – Bone or joint pain with a hot swollen joint, bone or joint tenderness, or complete reluctance to move a joint or limb Septic arthritis

• Early Orthopaedic assessment – Washout and culture – Prolonged antibiotics • Microbiology input • Don’t forget TB ! – Often more indolent presentation – Immunosuppressed

EMD Smith Arch Dis Child Educ Pract Ed 2012;97:185-193 A 5 year old girl • Limp about 1 week, pains in legs • Pain at night – crying ++ • Tired in the day (naps), less interested in play • Pale and quiet - “not herself” • Joints normal Thoughts

Investigations

Differential diagnosis A 5 year old girl • Limp about 1 week, pains in legs • Pain at night – crying ++ • Tired in the day (naps), less interested in play • Pale and quiet - “not herself” • Joints normal

• Hb 10.9 • WBC 7.5 (lymph 4, neut 3) • Platelets 100 • ESR 10 Diagnosis – Acute leukaemia

• Worrying indicators – Night waking (every night, difficult to console) – Reduced function (play, daytime activity, energy) – Systemic features (pallor, fatigue) – Abnormal bloods – thrombocytopenia Acute Lymphoblastic Leukaemia • Pathophysiology: – Malignancy of lymphocyte precursor cells, infiltrates marrow & can metastasise to bone – Commonest malignancy in childhood

• Clinical features: – Bone pain common, often at night – Weight loss, lethargy, pallor, bruising, lymphadenopathy A 6 years old boy

• Limping on and off a few days • Pain on walking • Today cannot weight bear at all • Pain left thigh and knee • Otherwise well Examination

• Looks well, apyrexial • Cannot weight bear • Knee normal • Hip reduced flexion / internal rotation Thoughts

Investigations

Differential diagnosis Examination

• Looks well, apyrexial • Cannot weight bear • Knee normal • Hip reduced flexion / internal rotation

FBC, ESR normal Xray hips normal US fluid left hip Irritable Hip

• Pathophysiology: – Synovitis, often preceded by a virus (gastroenteritis or upper respiratory tract infections) • Clinical Presentation: – Well child – Limp with or without pain or referred pain at knee – Reluctance to weight bear, reduced movement of hip – Normal/mildly elevated inflammatory markers

http://www.pmmonline.org/page.aspx?id=396 Transient synovitis or septic arthritis

EMD Smith Arch Dis Child Educ Pract Ed 2012;97:185-193 Perthes Disease

• Pathophysiology: – Avascular necrosis of the femoral head • Clinical features: – Often boys, aged 4-8 – Limp may be insidious – Hip/thigh/knee pain (Referred) – Usually unilateral (Can be bilateral but think of dysplasia / syndromes) – Restricted internal rotation of hip http://www.pmmonline.org/page.aspx?id=394 A 13 year old boy

• Pain in leg > 1 week, worse on walking • Pain in right groin and thigh • Limping at end of the day • No trauma • Otherwise well Examination

• Looks well, apyrexial • Can just weight bear • Right leg held externally rotated • Right hip reduced flexion and rotation • Other joints normal Thoughts

Investigations

Differential diagnosis Slipped Capital/Upper Femoral Epiphysis • Pathophysiology: – Femoral epiphysis slips out of normal position • Clinical features: – Often overweight boys aged 11-16 – Hip/knee pain and limping, may be insidious – Can be bilateral – Shortened and externally rotated leg – Reduced range of movement in the hip • Diagnosed with Xray (‘frog view’) http://www.pmmonline.org/page.aspx?id=395 A 2 year old girl

• Limping intermittently about 2 weeks • “Slow in the mornings” • Falls more than usual

• Otherwise well

• No past history of note • All milestones normal Points from the video

• Importance of observation • Check for non-verbal clues of pain • Check upper limbs too • Importance of comparing both sides • Ankles best observed from behind A 2 year old girl

• Limping intermittently about 2 weeks • “Slow in the mornings” • Falls more than usual • Otherwise well • No past history of note • Milestones normal

• Looks well, afebrile • Limping • Swollen ankles, knees • Restricted wrist Thoughts

Investigations

Differential diagnosis Investigations

• Full Blood Count / film • ESR, CRP • +/-US joints (rather than Xrays) • Autoantibodies (ANA)

FBC, ESR, CRP normal • Eye screening ANA 1/640 Thoughts

Investigations

Differential diagnosis Diagnosis Juvenile Idiopathic Arthritis • Suggestive Indicators – Inflammatory symptoms • Worse in the mornings (ask about stairs, getting dressed, mood) • Pain may be “absent” (ask about behaviour / mood changes) • Gelling after rest (ask about car rides / sitting ) – Reduced function (walking / “clumsy” / prone to falls) • Regression of achieved milestones • Joint involvement in multiple joints (may not be noticed by parent – so you have to look !! – pGALS ) – Swollen joint(s), muscle wasting • Wasting suggests chronicity Bloods (FBC/ESR) can be normal - so beware !! Juvenile Idiopathic Arthritis (JIA) • Common, 1 in 1000 prevalence – 16y, swollen joint/restriction > 6 weeks – Wide differential diagnosis • Several JIA subtypes determined mainly by clinical presentation • Good prognosis for most children with early diagnosis and access to treatment – Joint injections, methotrexate, +/- biologics http://www.pmmonline.org/doctor/child-with-a-swollen-joint/diagnosis-of-jia www.pmmonline.org Differential diagnosis is broad

Avascular necrosis (e.g. Perthes) Reactive (viral, streptococcal, post gastroenteritis or sexually acquired) Trauma (including non-accidental injury) Haematological (e.g. haemoglobinopathies, leukaemia) Rickets (metabolic and endocrine) Idiopathic (reflex sympathetic dystrophy, fibromyalgia) Tumour (benign, malignant) Infection (viral, bacterial, mycobacterial) Systemic (e.g Systemic Lupus Erythematous, Vasculitis, Dermatomyositis, Sarcoidosis, Inflammatory bowel disease, cystic fibrosis) Juvenile Idiopathic Arthritis (JIA) is a diagnosis of exclusion Oligo-articular JIA

• Most common sub-type • Tends to affect the knees • Girls > boys, often pre-school • Often a well child with limp

• Remember eyes – Can be asymptomatic uveitis – Blindness a possibility Vasculitis & multisystem

Juvenile Systemic Lupus Erythematosus • Arthralgia more so than arthritis • Limp not common (consider AVN) • Fatigue, systemic upset, fever • Rashes (photosensitivity), Alopecia • Can be indolent or acute ‘crises’ – Renal failure, stroke / seizures, lung disease • Abnormal investigations – Cytopenias – Raised ESR (Normal CRP) – Abnormal urinalysis – Autoantibodies (ANA, ds DNA) – Low Complement – Clotting disorders

http://www.pmmonline.org/doctor/child-with-a-swollen- joint/multi-system/jsle When to consider a joint assessment ?

• Child with – Limping (acute or sub-acute or chronic or intermittent) – Gait problems – Limb, bone or back pain – Joint pain, swelling, stiffness – Systemic upset (fever) – Suspected multisystem disease – Delay or regression in motor milestones • ‘Clumsy’ without overt neurological disease – Chronic disease with known musculoskeletal features • Inflammatory bowel disease, psoriasis, Down’s syndrome www.pmmonline.org Who to refer to paediatric rheumatology ?

Child with Limp and/or Painful/Restricted/Swollen Joint(s)

YES Review at 3 weeks YES Septic Arthritis If symptoms persist Trauma excluded Excluded Hip pathology Malignancy REFER TO excluded Excluded PAEDIATRIC RHEUMATOLOGY

Limping child protocols do exist Limping child

• Take a history (probe…) & perform physical examination (general assessment and pGALS as a minimum) MOST important • Always assess for red flags • Investigations (consider these & maybe more) – Blood tests - FBC (film +/- BM aspirate), acute phase (ESR/CRP), Infection screen (and TB), Autoantibodies (ANA / RF of limited value), bone chemistry – Xray (long bone above and below suspect joint), US joints (hip) – MRI (consider paed rheum referral before..)) – Synovial biopsy and arthroscopy (rarely needed) • Refer to paed rheum even if not sure of clinical signs www.pmmonline.org Pitfalls to be avoided

• Limp is not a diagnosis – do not discharge if not medically explained – Trauma is common - may not be relevant • Think beyond the hip ! Examine all joints (pGALS) and more – Think about referred pain (Abdomen, testes, spine, hip) • Sepsis is easily masked in the immunosuppressed – Have low threshold to pursue TB as a diagnosis • Radiographs are often normal in early sepsis / JIA / arthritis • Bloods tests (FBC, ESR, CRP) can be normal in JIA – Rheumatoid factor is usually negative in JIA • Normal variants are not painful – And do not cause limp • Growing pains do not cause limp • Dual pathology occurs (e.g. Slipped Capital Femoral Epiphysis in JIA) Summary

• Clinical assessment often gives the diagnosis – The history may be illocalised and non specific especially in the young so joint examination is important – Joint examination is an integral part of general paediatric assessment • pGALS is a useful quick examination – Guides further assessment – quick, simple and high pick up rate – i.e worth doing ! – Consider pGALS in children with limp, fever (even if no overt joint symptoms), clumsiness, abnormal milestones etc • Check out the e-resources (PMM and pGALS) e-Resources – all free www.pmmonline.org

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