COMMON SENARIOS IN PEDIATRIC ORTHOPAEDICS OSCE STATION

history , Clinical Dx and management

1. Hstory of arthritis -SLE
2. history of arthritis SJIA
3.Management of juvenile idiopathic arthritis (Clinical Dx, investigations , Rx)

Examination
1 .musculoskeletal examination
X ray
1. leg x ray perth disease
Communication
1.communication skill about pt with dermatomyositis need steroid
2.Counselling Oligoarthritis
Orthopedics History taking
1.Initial approach to the patient

2.personal data , Refer to the child by name

3. Open question about the chief Complaint (Locomotor proplem)

4.Asks about Current symptoms / complain (Pain ,Stiffness ,Swelling) and determines its:

Pain History points to elicit can easily be remembered using the mnemonic SOCRATES.

S-site/O-onset /C-character /R-radiation /A- associations /T-timing /E-exacerbating &

relieving factors S severity
ask about use of medication for pain relief.

Stiffness (early morning, night)

Swelling : site (Which joint is start involved) ( small, large or back)
Pattern of joint is involved (mono/ polyarthritis) /
Monoarticular only one joint affected
Pauciarticular (or oligoarticular) less than four joints affected
Polyarticular a more than four of joints affected
Axial the spine is predominantly affected

5.Evolution of condition
Acute or chronic?
When did the symptoms start and how have they evolved? Was the onset sudden or
gradual?
Associated events
Was the onset associated with a particular event, e.g. trauma or infection?

Management Hx:
Which treatments has the condition responded to?
drugs given (steroids, NSAID, methotroxate)
Physical therapy and rehabilitation, sports activity

Complications Hx: drugs S/E, deformity, wheel chair, splinting

6. associated general symptoms

fever, night sweats ,Malaise, weight loss , increase weight or fracture.

7. Systemic review:
cardiac (chest pain, exercise intolerance)
respiratory (cough, SOB)
Renal: (H.urea ,odema,oligure)
Eye:redness
skin rash .etc.

8. Impact of lifestyle
Patients needs/ aspirations
Ability to adapt with functional loss
Daily activities: housing ,eating (TMJ involvement), dressing, writing, walking, limping,
school attendance, sport
9.Screening questions:
Do you have any pain or stiffness in your muscles, joints or back?
Can you dress yourself completely without any difficulty?
Can you walk up and down stairs without any difficulty?

10.Social psychological history

Asses social support
Rules out depressive disorder

11. past history

trauma, infection, surgery, medications (steroids) ( laxative excess ), blood transfusion.
12. Immunization history (esp. polio.)

13.Developmental history
delayed milestones esp. motor, sun exposure, vit D supplementation
14. Family History (consanguinity, Similar illness , other diseases.)

15.Ask the relative if he has any question or information.

16.thanks

17.Presentation

Brief introduction (personal data and current state /active main problem other problem
in priorities(
DDx initially then most likely Dx
List the problem by priorities /Social and psychological impact of problem on child and
family
Management plane. Investigation/ Medical and Surgical management/ MDT/ Refer
Advice and reassurance/ education / F.up
Arthritis
Presentation may be acute when there is a combination of pain, swelling, heat, redness
and restricted movement in a joint.

DDx of acute monoarthritis

Septic arthritis: high fever, sever tenderness
Reactive arthritis
Oligo-JIA
Haematological disorders /SCD,Hemophilia: recurrent bleeding and joint affection
Malignancy/ Leukaemia neuroblastoma (infection, blood trans, fever, wt loss)

DDx of Chronic monoarthritis:

Oligoarticular JIA
Juvenile psoriatic arthritis
Juvenile ankylosing spondylitis
synovitis
Sarcoidosis

DDx of polyarthritis
Juvenile idiopathic arthritis JIA (Polyarticular and systemic )
small, peripheral joints, long duration, deformities,
systemic , skin and eye symptoms
Juvenile psoriatic arthritis
Reactive /GIT infection - streptococcal infection
Inflammatory bowel disease / Crohn's disease - ulcerative colitis with erythema nodosum
Rheumatic fever
large joints, migratory, dramatic response to salicylates, (minor and major criteria)
Vasculitis
Henoch-Schnlein purpura with purpura, abdominal pain, red urine
Kawasaki skin rash ,conjunctivitis ,lymphoadenopathy
Connective tissue disorders
SLE : multiple organ affection (skin, CNS, renal, hematological)
Dermatomyositis: muscle pain, wasting, face rash, Gottron papules , Calcification
Infection
Bacterial /Septicaemia - septic arthritis TB
Viral /Rubella - Mumps Adenovirus -coxsackie B -herpes - hepatitis parvovirus
Other /Mycoplasma - Lyme disease rickettsia
Other / Cystic fibrosis
Painfull Joint swelling /Stiffness (arthritis) history taking
1.Initial approach to the patient
2. Introduced your self
3. Open question about the chief Complaint (Joint pain, swelling)
4. HOPI : Onset / Duration :How long the pain been present (sudden, insidious, acute , chronic)
what is the character of the pain:
What makes it worse(e.g. exercise), and what makes it better(e.g. rest or analgesia).
Dose the pain interfere with function? (Rheumatic fever arthritis pain usually very painful)
Is their diurnal variation in the severity of the pain? (Morning stiffness in JIA arthritis)
Is the pain presents at night? (Growing pain typically at night)
Quality of pain (sharp, aching, deep, etc)
site (Which joint is start involved) ( small, large or back)
Pattern of joint is involved
Monoarticular only one joint affected
Pauciarticular (or oligoarticular) less than four joints affected
Polyarticular a more than four of joints affected
Axial the spine is predominantly affected
Dose the painful part looks different?(swelling,stifness,worm ,redness , limping)
Course (Constant /migratory -Rheumatic fever ) progressive, stationary, regressive
5.associated symptoms :fever,fatigue, rash, night sweat , back pain, Weakness, loss /increase wt
6. Ask about recent events of ( acute )
recent history of URTI, influenza, skin infection ,diarrhea , mumps, rubella,varicella ,HAV.
recent history of trauma or drugs (antibiotics) in the previous 7-12 days(serum sickness)
recent vaccination /MMR.
7. Systemic review:
CVS: chest pain, palpitations, CHF symptoms - JIA,SLE
Resp: chest pain, dyspnea, cough, JIA,SLE
GIT: abdominal pain, diarrhea, bloody stool.IBD
Urinary: hematuria, oliguria, urthral discharge , HTN,HSP ,SLE
Eye: redness /uveitis, vision, xerosis, cataract - JIA,SLE
Neurological: seizures, headache, psychosis and depression - SLE
Hematological: bleeding,Lymphadenopathy,recurrent infection, (hemophilia/malignancy)
8. if chronic ,ask about Management and Complications Hx:
ask about use of medication for pain relief.
Which treatments has the condition responded to?
drugs given (steroids, NSAID, methotroxate)
Physical therapy and rehabilitation, sports activity
ask about deformity, wheel chair, splinting , drugs S/E
9. Impact of lifestyle
Patients needs/ ability to adapt with functional loss
Daily activities: housing ,eating (TMJ), dressing, writing, walking , school attendance, sport
10.Social psychological history Asses social support ,and rules out depressive disorder
11. past history
medical Hx :same illness ,admissions, trauma ,blood transfusions , surgical illnesses or allergy
Drugs Hx: precipitate arthritis frusemide , thiazide ,hydralazine, phenytoin, hlorpromazine, INH
Immunization Hx: polio/MMR.
Travel history , Tick exposure : dengue fever/lyme.
12.Family History (IBD, hemophilia, R.arthritis.)
14.Ask the relative if he has any question or information.
15.thanks
Historical approach to pt with arthritis
If chronic , small, peripheral joints, long duration, deformities, fever , skin , eye symptoms
this Juvenile idiopathic arthritis JIA

If chronic , monoarthritis ,small, peripheral joints, long duration, with early morning stifness
this Oligoarticular JIA
If chronic monoarthritis, small finger ,long duration with nail and skin symptoms
this Juvenile psoriatic arthritis

If chronic polyarthritis, with skin rash,intermittent fever ,abdominal distintion

this SJIA

If chronic polyarthritis, with Depression organ affection (skin, CNS, renal, hematological)
this SLE

If chronic polyarthritis, with muscle pain, weakness ,facial rash, papules

this Dermatomyositis:

If chronic polyarthritis, abdominal pain, diarrhea, bloody stool ,skin lession ,wt loss
this Inflammatory bowel disease / Crohn's disease - ulcerative colitis

If chronic painful polyarthritis, post URTI, large joints, migratory, fever, response to ASA
this Rheumatic fever

If acute polyarthritis, post URTI, with purpura, abdominal pain, red urine
this Henoch-Schnlein purpura

If acute polyarthritis, , remittent fever ,skin rash ,conjunctivitis ,lymphoadenopathy

this Kawasaki

If acute polyarthritis, post GIT infection diarrhea , streptococcal infection

this Reactive arthritis

If acute polyarthritis, dysuria,urthral disharge , conjunctivitis

this Reiter's syndrome (remember the triad: arthritis ,urithritis, conjunctivitis).
If acute monoarthritis with recent local trauma or skin infection, fever, redness
this spetc arthritis

If acute monoarthritis with recurrent infection, blood trans, fever, wt loss

this Malignancy/ Leukaemia neuroblastoma

If acute recurrent monoarthritis with recurrent bleeding and joint affection

this Hemophilia:
If acute recurrent monoarthritis with recurrent chest infection ,palor ,BT,+ve family history
this SCD: (salmonella osteomyellitis).
Historical approach to pt with arthritis and skin rash
If chronic monoarthritis, scaly erythematous rash , nail pitting
this Juvenile psoriatic arthritis

If chronic polyarthritis, salmon skin rash, intermittent fever

this SJIA

If chronic polyarthritis, Heliotrope rash MCPJ ,facial rash , papules ,muscle pain, weakness ,
this dermatomyositis

If chronic polyarthritis, butterfly malar rash, depression , red urine

this SLE

If polyarthritis, purpuric rash in extensor legs, abdominal pain, red urine

this Henoch-Schnlein purpura

If polyarthritis ,plemophric skin rash,remittent fever,conjunctivitis ,lymphoadenopathy

this Kawasaki

If chronic polyarthritis,tibial E. nodosum rash ,abdominal pain ,bloody stool , wt loss

this Inflammatory bowel disease - Crohn's disease - ulcerative colitis

If chronic polyarthritis, back erythema marginatum rash , post URTI, large joints, migratory
this Rheumatic fever

If chronic polyarthritis, erythema chronicum migrans rash , large joints, , fever

this Lyme disease

If polyarthritis, slapped face rash , fever

this Parvovirus
The painfull knee swelling DDX:
1-Trauma

2-Septic arthritis

3-Juvenile rheumatoid arthritis

4-Rhaumatic fever

5-FMF

6-HSP

7-Reiter's syndrome (remember the triad: arthritis urithritis, conjunctivitis).

8-Toxic synovitis of hip

9-Hemophilia

10-Sickle cell disease (salmonella osteomyellitis).

11-Tumours (bone tumor, soft tissue tumor, leukemia).

12-Others: TB arthritis, psoriatic arthritis, brucellosis).

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knee joint pain history taking
1.Initial approach to the patient
2. Introduced your self
3. Open question about the chief Complaint (joint pain)

4. Describe
Onset (sudden, insidious, acute).
duration
Course (progressive, stationary, regressive).
Aggravating factors (e.g. exercise) or relieving factors (e.g. rest).
recent hx of trauma/exercise.

5. associated symptoms
associated joint swelling, tenderness ,decreased mobility, limping.
associated back and hip pain
associated constitutional symptoms, fever, fatigue, night sweat ,wt loss,increase weight.

6. Systemic review:
associated skin rash.
associated GI symptoms (abdominal pain, diarrhea).
associated urinary symptoms (hematuria, dysurea).
associated eye symptoms (pain & redness).
cardiac (chest pain, SOB)
respiratory (cough, exercise intolerance)
hematological, (bleeding tendency).

7. past history
previous knee pain or other joint,trauma, surgery, hx of bleeding disorder or B transfusion

8. Immunization history esp. polio/MMR.

9. Developmental history

10. Family History (family hx of joint disorders)

11.Ask the relative if he has any question or information.

12.thanks

12.Presentation
Investigation:
1-CBC, blood culture.

2-ESR, RF, ANA.

3-Joint aspiration.

4-Joint x-ray, bone scan.

5-PT, PTT.

6-ASO, throat swab.

7-Brucella titer.

8-Tuberculin test.
Musculoskeletal examination
Systemic examination is combined with :-
pGALS, paediatric Gait, Arms, Legs, Spine: a MSK locomotor screening examination
pREMS: paediatric regional examination of the MSK system based on the look, feel, move.
Listen carefully to open statement of the Examiner:
if said general MSK or joint examination: Start screening by pGALS
If the general instructions are vague : Start screening by pGALS
If said pt has a history of joint pain, joint stiffness or a limp : Start screening by pGALS
If said examine the legs : Start screening by Gait, then legs of pGALS
If said examine the spesfic joint Start by pREMS
if said examine one joint(inflammatory) start by pREMS then pGALS
If said examine the gait: All aspects of gait examination

1.initial approach & attitude to the patient (WIPER) /Undressed /remove shoe and socks
2.Look to surroundings or attached :Walking Aids, orthosis or splint (take look at shoes)
3,General appearance of child 4Ds (Diseases , Distress , Dysmorphism ,Dimensions)
4.Start screening by pediatric Gait ,arms, legs and spine (pGALS)
Three screen questions: 1. Hurting : 2. Dressing dificulty : 3. Stair difficulty
If yes to any of these questions will alert you to the possibility of problems in MSK
Ask child to standing upright straight,scan from head to toe,from front,side and behind
Look at the front posture ,asymmetry , bruises ,skin rashes & hair loss ( psoriasis).
Look at the front and side of childs face For a small jaw or facial asymmetry
Look at the back for asymmetry of skin creases and shoulder level (scoliosis).
Look at the back for the knees (valgus a bow-leg , varus knock-knee or neutral)
Look for any signs of leg length discrepancy
Ask child to walk - pGALS - Gait:
1st Ask child to walk across the room , run then turn back (limping , pain ,turning difficulty)
look if symmetrical gait,with heel strike, stance, then toeing off ,with reciprocal arm swing
look for evidence of flat feet and excessive pronation (hypermobility), or walk on toes
then Ask child to walk on their heels then on their tiptoes (high Stepping /foot drop).
Look at foot posture, and whether arches are present(normal ,absent ,high arch)
then ask to walk on outside and the inside of their feet (Fog walk)
then ask to walk heel-toe (Tandem) or walk in line
Ask child to sit facing you -pGALS Arms examination; exposed the arms (comare)
Ask about any pain anywhere and as you go through your examination
See evidence of rashes such as psoriasis (check the nails pitting as well)
Comment on muscle bulk ,atrophy ,asymmetry , joint swelling, hand contraction deformity
ask to put his hand out straight infont of you turn your hand and make fist then counting
Turn the hands back over and gently squeeze the joints (tenderness(fingers , hands joints).
Ask to put their hands together(palm/back) with horizontal elbows (wrist , finger, elbow)
Then raise their arms straight above their head (shoulders and elbows) .
Then put their hands behind their head and elbows right back (shoulders and elbows).
Then hide your hand back and scratch your back (shoulders and elbows).
Then exten his neck back (look at the ceiling) , then Flextion , rotation, lateral flextion(neck)
Finally open mouth see asymmetry and malocation which may suggest (TMJ disease).
Ask child to insert three (normal) of their own fingers into their mouth
Ask child to stand- pGALS legs examination: exposed legs from toes to hips
Ask about any pain anywhere and as you go through your examination
Inspect while standing: Compare and comment on
discrepancies ,quadriceps bulk , symmetry, knee swelling or deformity? foot arches
then Lying down (supine) and exposed the legs appropriately
Feel for knee effusion, press on patella
bulge test : for a small effusion
patellar tap test: for medium sized effusion
cross fluctuation: for large effusion

Look joints movement on both sides: (Active/Passive)compare movement ranges

Firstly active bend and straighten your knees (feel for crepitus)
Then passive full flex knee and hip then flex hip to 90o with ext. and int. rotations
Then passive plantar flexion and dorsiflexion of foot and big toe
Then passive inversion and eversion of forefoot

Finally Ask child to stand again pGALS spine examination:

Bend forward and touch your toes look for spine curve from the side ,back (scoliosis)
After Screening by pediatric Gait ,arms, legs and spine (pGALS)
Summarizing finding of your examining and preceding to next examination as follows :
Descripe general observations and appearance
Descripe finding of gait screen examination
Descripe if normal gait or normal variants in gait and stance
- Bow legs (genu varum) is normal at birth to 1824 months
- knock knees (genu valgus) is common at 34 years, usually correcting by 7 years.
- Flat feet with normal arches evident on tiptoeing resolve by 6 years.
- Out-toeing : resolves spontaneously at 12 months
- In-toeing : resolves spontaneously at 12 months
- Toe walking : this is common in 1- to 3-year-old children.
Descripe if abnormal gait and Preceding to.
trendelburge test if Limbing gait
hypermobility test , palate and CVS if tall stature + Hyprmobile gait and joints
CNS examination if Abnormal neurological gait or hemiplegic posture
Descripe finding of Arms screen examination
Descripe if normal Arms
- Normal joints appearance,no spesfic posture ,no deformitis or swelling
- normal skin and muscle bulk (no stigma or visible scar)
- normal symmetrical range of movement of all wrist/elbow joint in all direction
- normal function.(writing , dressing )
Descripe if there Arms joint abnormalities (site , distribution and type of abnormalities )
- Preceding to pREMS: (look, feel, move and function)
Descripe finding of legs screen examination
Descripe if normal leg
- Normal joints appearance,no spesfic posture ,no deformitis , swelling
- normal skin and muscle bulk (no stigma or visible scar)
- normal symmetrical range of movement of all joint in all direction
- normal function (child walking normaly )
Descripe if there legs joint abnormalities (site , distribution and type of abnormalities )
- Preceding to pREMS: (look, feel, move and function)
Descripe finding of spine screen examination
Descripe if normal spine
- Normal joints appearance,no spesfic posture ,no deformitis , swelling or kyphosis
- normal skin and muscle bulk (no tenderness , stigma or visible scar)
- normal range movement of spine in all direction
- normal function.(child can bending and )
Descripe if there spine abnormalities (site , type of abnormalities and direction )
- Preceding to pREMS: (look, feel, move and function)
finding of gait screen examination
child wearing shorts and in bare feet, and the following noted:
Symmetrical or asymmetrical?
Reciprocal arm swing?
Heel strike present or walking flat footed or on toes?

Gait patterns may be normal variants or pathological.

Normal gait rhythm, heel strike, stance, then toeing off with reciprocal arm swing
Normal variants in gait and stance
Bow legs(genu varum) increased distance between knees, accentuated on stand
- Physiological :normal Seen in 1-3 year old age group
- Rickets , Blount's disease , Epiphysial dysplasia , OI

knock knees (genu valgus) increased distance bw feet, the knees touch one another
- intermalleolar distance : It is pathological if it is more than 5 cm in child over 8 years
- Physiological : common at 35 years, more in female ,usually correcting by 7 yrs.
- Rickets ,Ligamentous laxity ,Renal osteodystrophy ,JIA

Out-toeing :bilateral rotation of the hips and resolves spontaneously at 12 months

In-toeing : There are three main causes:
metatarsus varus; forefoot adduction, self correcting 1-2 yrs
medial tibial torsion; at the lower leg, self correcting by 5 yrs
persistent anteversion of femoral neck;self correcting 8 yrs

Flat feet (pes planus) :increased planter contact area and normal arches resolve by 6 years
It is best examined while the child is in standing on tiptoeing.
Marked flat feet can be the presentation of a collagen disorder such as
Ehlers-Danlos syndrome
Hypermobility-marfan syndrom.
cerebral palsy
 Toe walking : this is common in 1- to 3-year-old children. It may become persistent
Bilateral
1. Normal variation ,usually from habit up to 3 years of age
2. Cerebral diplegia
3. Duchen muscular dystrophy
4. Spinal muscular atrophy
5. Autism
Unilateral
1. Unilateral congenital hip dislocation.
2. Spastic hemiplegia.
3. Congenital shortening of tendo achilles.

Pathological gait variants:

High-arched feet- pes clavus: It is a complex deformity composed of :

High arched foot ,Clawing toes ,Inversion of the fore foot ,Varus of the hind foot
It is best examined while the child is in standing on tiptoeing.
Do pressure print method: Ask the child to stand on powdered glass.
It is often a manifestation of neurological disease , Causes:
- Idiopathic 10% , CNS 90%: (SMA,DMD,CP,HMSN ,Frederick's ataxia,Polio)

Limp gait :
A limp, or asymmetrical gait, is most common abnormality,Several types recognized:
Antalgic limp (painfull):
If Painful limping gait (antalgic),pain on weight-bearing is minimized by shortening stance
So the affected hip higher than other side , child will limp or fall away from affected side
May refusal to weight-bear or walk.
Need to exam affected higher side joints for painfull joint swelling

DDx of painfull limbing at Toddler up to 5 years:

Infection: septic arthritis, osteomyelitis, discitis ,trauma: non-accidental injury
DDx of painfull limbing at 510 years:
trauma: overuse, sports injuries ,Transient synovitis/ irritable hip ,Perthes disease
Less common: juvenile idiopathic arthritis, malignant disease
DDx of painfull limbing at 1017 years:
Mechanical trauma: overuse injuries, sport injuries ,Slipped capital femoral epiphysis
Juvenile idiopathic arthritis
Malignant disease: leukaemia, lymphoma, bone tumour
Idiopathic pain syndromes, conversion disorder
Trendelenberg gait:
Normaly when walk the pelvis on side of non bearing weight side, will rises (intact abductor)
if Painless limping ,when walking the pelvis on side of non bearing weight, will drops rather
than rises (affected abductor weakness) child will limp or fall towards the affected side
if bilateral drops of non bearing weight side this waddling gait occure bilateral pathology
now teests of stability for trendelenburg's sign:
Ask child to stand on both legs ,the affected side will be lower than other side
Ask child to stand on one leg , see the glutei creases :
normally the glutei contracts so that the opposite side of the pelvis is tilted up for balance.
The glutei creasrs and ASIS will be at same level .
Ask child to stand on other affected leg (glutei action are deficient),opposite side tilt down
The glutei and ASIS of normal non bearing weight side will be lower.
balance can be maintained only by leaning over the affected.

Trendelenberg gait seen in:

unilateral Occurs in congenital and chronic hip disease
CDH with short legs, slipped femoral epiphysis, necrosis of femoral head(SCD)
Hemiplegic cerebral palsy.
poliomylitis
waddling gait seen in Bilateral pathology and in DMD
Need to exam legs lengh , affected side exam for deformitis , movement range(hip abductor)

Unilateral foot-drop:
weak ankle dorsiflexors as in peroneal neuropathy leads to a high stepping gait.
Ask child to walk on their heels and on their tiptoes

Unilateral equinus gait:

limited ankle dorsiflexion by shortening Achilles tendon or gastrocnemius tightening.
Ask child to walk on their heels and on their tiptoes.
Specific joints examination
The examination should be based on the pREMS: paediatric regional examination of the
MSK system based on look, feel, move approach to each joint ,function for some joint.
According to finding in PGALS screen which detect MSK abnormalities in spesfic joint or if
examiner ask you .
This child has joint pains. Examine his musculoskeletal system.
This child has been complaining of pain in his legs. Examine him.
This child has difficulty climbing stairs. Examine him.
These parents have noticed that their child has a limp. Examine her.
Examine this childs joints.
1.initial approach & attitude to the patient (WIPER)
2.Look to surroundings or attached Walking Aids, orthosis or splint
3.General comments for appearance and general observation
4Ds (Diseases , Distress , Dysmorphism ,Dimensions)
Comment on the presence of aids (eg wheelchair, orthoses, splint)
Comment on any obvious MSK abnormalities (eg arthrogryposis, hemihypertrophy)
Observe the childs posture (sitting, standing and lying)
4.Three screen questions: 1. Hurting : 2. Dressing dificulty : 3. Stair difficulty
5. pREMS: paediatric regional examination of the MSKS look, feel, move approach
fully expose the area of interest (Comment on obvious abnormalities -eg swollen knee).
If you ask the pt to undress, comment on whether they find this easy or difficult
if embarrass the pt, say to the examiner I would normally expose the completely
look at specific Joint ( At three levels)
Look for contraction deformities, erythema ,swelling and scar
Look for skin and muscle bulk around (wasting )
Asymmetry - Compare both sides
Comment about posture and movement where relevant
fell Palpate the joint and watch child face?
Feel skin temperature ,tenderness or effusion ,fluctuance , crepitus
swelling (fluid, soft tissue, bony) (Wrist and knee arthritis)
fell the TM joint when clench
Move first Active then passiveEstimate the range of movement dont measure it.
Ask child to move his joint first (passive) then by you (active)
Estimate the range of movement dont measure it.
Compare with range of normal joint , dont compare with yourself or caregiver
function may be assessed
for lower limb examination ask to stand up and walk with exposure
for upper limb examination ask to grasp large object ,drawing ,combing
for jaw - open and close mouth
for thoracolumbar - pick up objet from floor or taking off and putting on shoes , socks
Notes on specific joints

Wrists
Palpate to localise tenderness
Range of movement: flexion 80, extension 70, radial deviation 20, ulnar deviation 30

Elbows
Range of movement: flexion 135, extension 0, supination and pronation 90 (elbows flexed)

Shoulders
The range of movement can be easily tested as follows (this method also covers function):
- Put your hands above your head Tests flexion (90) and abduction (180)
- Give yourself a hug Tests adduction (45)
- Scratch your back Tests external rotation (45)
- Hide your hands behind your back Tests internal rotation (55) and extension (45)

Jaw and cervical spine

Look for acquired micrognathia and dental malocclusion
opening and closing your mouth , Feel for crepitus over the TMJ
Range of movement at cervical spine: flexion 45,extension 50,rotation 80,lateral flexion40

Thoracolumbar spine
Examine the child standing and bending forward for kyphoscoliosis
Feel for tenderness and check the range of movement:
- flexion(should be able to touch toes), extension (30at lumbar area)
- lateral bending (50to each side),lateral rotation(30 to each)
Functional evaluation: ask to pick up an object from floor or to puton their socks and shoes
Lower limbs
Examine the gait
Remember to look for leg length discrepancy
Make the child squat (to test for proximal muscle weakness)
Make the child stand on each leg in turn (to test for Trendelenburgs sign)

Hips
Look for muscle wasting
Note the resting position
Feel for tenderness
Measure true leg length between the anterior superior iliac spine and the medial malleolus
Range of movement: flexion 120, extension 30, abduction 50, adduction 30.
internal rotation 35, external rotation 45 (with Hip flextion) pain if hip pathology, be careful!

Knees
Look for quadriceps wasting
Feel for tenderness, raised local temperature and effusion
Range of movement: flexion 135, extension up to 10. Check for abnormal movement
You should know the manoeuvres for checking the stability of the ligaments in the knee.
effusion at knee
bulge test :fell knee when press over distal thigh ,milk from side and compare again
If the medial parapatellar fossa is seen to bulge out, this indicates a small effusion
patellar tap test: empty the suprapatellar pouch with one hand and dip the patella with the
thumb, of the other hand. If the patella is felt to tap the underlying bone and to bounce back
up, this indicates a medium sized effusion
cross fluctuation: place the thumb and index of one hand below the patella and with
the other hand press over distal thigh.If an impulse transmitted,this a large effusion

Ankles and feet

Range of movement at ankle: plantar flexion 50, dorsiflexion 20,Inversion (5),eversion (5)
Abduction (10) and adduction (20) ,plantar flexion (45) and extension (70)
Some joint deformities are more obvious when the patient is weight bearing.
function: ask the child to stand, and observe what happens to their feet and ankles.
Looking at their footwear may also provide some information about function.
Standing and walking on tiptoe and then on the heels can accentuate problems with the feet
If child has joint swelling
Describe in detail appearance/range of movement and its impact on function
type of abnormality (swelling ,stiffness ,deformities)
- painfull or painless
- unilateral /bilateral/multible
- associated redness ,erythmia ,change skin color
- associated muscle wasting.
- associated abnormal posture
- limitation of movement / hypermobile
- associated length discrepancy (legs)
- associated limp (legs)
- functional /dysfunction

To complete my examination I would also like to examine the patients.)

further exam joint above it and the joint below it
pGALS screening examination if you have not already done
Asses other systems feature of inflammatory arthritis
Eyes (blue sclera/marfan ) (redness ,pallor,visual impairment (uvitis)/JIA)
Skin:psoriasis/JIA,malor rash/SLE,dermatomyostitis,E.nodusum/IBD, striae/steroid
Nail pitting and hair loss ( psoriasis/JIA)
Dactylitis (SCD)
Check temperature(infection,inflammation)
Evidence of infection (reactive arthritis)
Exam palate and cardiac if suspected marfan syndrom
Exam abdomen for Hepatosplenomegaly(SJIA)
Exam for steroid toxicity(SJIA)
Look for evidence of overgrowth or undergrowth of the affected limb
- Measure leg length (from ASIS to medial mmalleeolus) for discripency
Measure wt ,ht and ploted in growth charts , OFC if relevant

Mention Dx site of abnormalities : muscle , bone , joint

Mention causative Dx : infection ,trauma , inflammation vasculitis or bleeding
comment on :additional investigations, Rx, involvement of other health professionals

Causes of a swollen joint

A swollen joint indicates arthropathy which may or may not mean inflammation (arthritis)
and may or may not cause arthralgia (pain).
The causes of a swollen joint are categorized according to pathological mechanism:
Trauma: accidental or non-accidental
Infection: septic arthritis, viral arthritis
Inflammation: postinfectious arthritis, juvenile idiopathic arthritis (JIA), SLE, DM.
Vasculitis: HSP
Haematological: sickle cell disease, haemophilia
Malignancy: leukaemia, Ewing sarcoma, osteosarcoma
Drug reactions
Investigations
Imaging
Imaging dominates the investigation of musculoskeletal disorders and is considered here.
plain radiographs:
provide clear views of bones and joints and useful information of soft tissues ,joint effusions.
If hip pathology is suspected, AP and frog-leg lateral views of the pelvis are required.
Ultrasound:
especially useful for imaging the hip joint. It is sensitive for detection of hip joint fluid but
has low specificity for distinguishing pus, blood, and inflammatory effusion.
It is the most useful initial investigation for detection of effusion or DDH
Radionuclide scan: 99 mTechnetium-labelled methylene diphosphonate
is taken up at skeletal sites with increased bone turnover or blood supply.
Magnetic resonance imaging: MRI
is more sensitive than radiology in detecting bone or joint infection.
It is the modality of choice for imaging the vertebral column and spinal cord
Blood tests and microbiological samples
often required and the indications for specific tests are considered with each disease.
If child has JIA
quick assessment of childwithjuvenilechronic arthritis
GENERAL EXAMINATION:
- Look for pallor, rash, lymphadenopathy, hepatosplenomegaly ,Temperature ( infection)
- Joint deformity ,and Muscle wasting
JOINTS:
1. Flex the neck to each side: looking for pain and restriction of movements
2. Open the jaw wide and move it from side to side - feel for click ,Look for micrognathia
3. Place both hands behind the head with elbows back. External rotation and abduction
4. Place both hands out in front, palms down, fingers straight, with elbows at 90 at the side.
Inspect swelling, deformity, and skin changes at wrists, MCP's and interphalangeal (PIPJ)
5. Turns the hands over (supination). Testing proximal and distal radioulnar joints.
Inspect the palmar aspects for wasting, skin changes, flexor tenosynovitis swelling
6. Make a tight fist with each hand. Observe ability to curl fingers tightly into palms.
7. Place the tip of each finger onto the tip of the thumb in turn.
8. Squeeze across the 2nd to 5th metacarpals. Look for tenderness
9. Passively flex the hip and knee while holding the knee.
10. Passively internally and externally rotates the hip.
11.Press downs the patella and palpate for effusion in each knee
12. Squeeze all metatarsals to test MTP joints

GAIT:
Any limping
Any leg length discrepancy due to chronic monoarthritis (especially knee)

GROWTH:
Check growth centiles (short stature as an effect of the disease or chronic use of steroid)

EYES:
Needs very careful assessment by ophthalmologist especially in case of pauci JIA:
Look for irregular pupil, cataract, and band keratopathy Iridocyclitis
Retinopathy (if patient using chloroquine)

SIGNS OF STEROID TOXICITY:

- Hypertension
- Growth retardation
- Features of Cushing's syndrome
- Osteoporosis
- Proximal myopathy
- Cataract
- Diabetes mellitus
- Gastric irritation
- Protein wasting, edema, metabolic alkalosis
- Susceptibility to infection
- Psychosis
If child has spinal defect
Describe in detail appearance/range of movement and its impact on function
Describe the deformitis /Curvature of the spine:
kyphosis Abnormal flexion curvature kyphotic spinal deformities
lordosis Abnormal extension curvature lordosis spinal deformities
scoliosis Abnormal LT/RT lateral curvature scilotic spinal deformity + high shoulder
whats the side to which the spine is convex
disappears pending forward touch their toes = postural (80%),fixed this structural(20%).
Abnormal limitation of lateral pending ,rotation or extension movement of spine
Abnormal hypermobile spinal flextion
Difficulty to pick up objet from floor or wear stock and shoes
At the end said..To complete my examination I would also like to ..
Further examination after finding of scoliosis
- Skin: look for neurocutaneous stigmata, and scars from previous surgery
- CNS examination spina bifida, poliomyelitis, Frederick's ataxia, and cerebral palsy
- Mention pubertal staging:, as scoliosis tends to worsen during puberty
- Respiratory examination if scoliosis is severe (look for evidence of respiratory failure)
Look for evidence of Marfans syndrome if the child is tall
- exam palate and cardiac
- measure wt ,ht and ploted in growth charts / measure Sitting height & arms span

Mention Dx of abnormalities : bone , connective tissue ,muscle ,neurogenic

Mention causative Dx : idiopalhic ,Marfan's Sprengel's deformity , Klippel-Feil syndrome
comment on :additional investigations, Rx, involvement of other health professionals
Investigations
Think about blood tests (inflammatory markers, antinuclear antibody and other
autoimmune antibodies) and imaging (plain X-ray, MRI, ultrasound)
Treatment
Consider both medical and non-medical treatment
physiotherapy, occupational therapy, orthopaedic involvement and orthotics
Causes of scoliosis
postural (80%)
idiopalhic (female)
unilateral muscle spasm and pain
unequal leg length.
fixed structural (20 %). There a gibbus or hump, due to increased convexity underlying
ribs (rotation of the vertebrae), on the side of convexity:
idiopathic
Ligament : Marfan's syndrome
Muscle : muscular dystrophy.
Bone: Sprengel's deformity , Klippel-Feil syndrome
Neurogenic: spina bifida, poliomyelitis, Frederick's ataxia, and cerebral palsy
Sprengel's shoulder
one scapula congenitaly fixed in a high position
limited abduction of the shoulder , there hypoplasia of the shoulder girdle muscles.
There may also be a cervical rib (and therefore brachial plexus compression)

Klippel-Feil syndrome-
the fundamental defect is fusion of the cervical spine.
results in short neck, low hairline and webbing of the skin of the neck (DDxTurner's).
There is restricted neck movement and sometimes torticollis.
There also cervical spina bifida and thoracic hemivertebrae(root or cord compression).
if flexion and external rotation posture ,assymetric and limitation of hip Abduction this CHD
In child with unilateral dislocation of hip look for the following signs:
o Leg posture: the thighs tend to be held in partial external rotation, flexion and abduction
o Limb shortening: above knee shortening on the affected side
o Asymmetry of the thighs: skin creases may be asymmetrical checked in supine and prone
o Flattening of the buttock: may appear on the affected side in prone position
o Limitation of abduction: persistent and less than 75 degree (the most important sign)
o 20% of children with CDH will not walk at 18 months of age ,80% will walk at normal age
o limp or fall towards the affected side
o After 2 years the child can not balance on the affected leg

In child with bilateral dislocation:

o The signs as described, although there is no normal hip for comparison
o A perineal gap may be present.

if child with leg length discrepancy.. Measurement of leg-length:

- Apparent shortening is measured between fixed point (xiphoid processor umbilicus) and the
tip of the medial malleolus.
- True shortening is measured from the anterior superior iliac spine to medial malleolus.

Causes of discrepancy:
1- Hemihypertrophy:
o Wilm's tumor
o Beckwith Wiedemann syndrome
o Neurofibromatosis
o Diastematomelia
o Klippel-Trenaunay-Weber syndrome
o Silver-Russell syndrome
o Local causes (cavernous hemangioma)
o Idiopathic
2- Juvenile chronic arthritis
3- Skeletal Dysplasia
4- Perth's disease
5- Slipped capital femoral epiphysis
6- Radiation therapy
7- Lymphatic system obstruction (e.g., elephantiasis)
Suggested approach to examining the legs in the MSK Station
1.initial approach & attitude to the patient (WIPER)

2.Look to surroundings or attached Walking Aids, orthosis or splint

3.General comments for appearance and general observation

4Ds (Diseases , Distress , Dysmorphism ,Dimensions)
Comment on the presence of aids (eg wheelchair, orthoses, splint)
Comment on any obvious MSK abnormalities (eg arthrogryposis, hemihypertrophy)
Observe the childs posture (sitting, standing and lying)

4. Expose the legs and look for any obvious abnormality (Ask about pain in the legs) .

5. gait : ask child to stand up and walk with exposure

Ask child to squat (proximal muscle weakness) Consider Gowers sign
Ask child to stand on each leg (trendelenburge sign)

If there are obvious pointers regard pathology, pREMS: look, feel, move approach .

If there are no obvious pointers regard pathology, perform Legssection of pGALS.

Ask child to stand : exposed legs from toes to hips
Ask about any pain anywhere and as you go through your examination
Inspect while standing: Compare and comment on
discrepancies ,quadriceps bulk , symmetry, knee swelling or deformity? foot arches
then Lying down (supine) and exposed the legs appropriately ,fell knee
Look joints movement on both sides: (Active/Passive)compare movement ranges
Firstly active bend and straighten your knees (feel for crepitus)
Then passive full flex knee and hip then flex hip to 90o with ext. and int. rotations
Then passive plantar flexion and dorsiflexion of foot and big toe
Then passive inversion and eversion of forefoot

if the pGALS screen is normal, proceed to

- a neurological examination of the legs
- Look at the spine
Summary for joints examination
The candidate should look, feel, and assess active and passive movement of joints in the
examination of hands, elbows, shoulders, spine (cervical, thoracic, lumbar), hips, knees,feet
and ankles.

The candidate should be able to:

Detect abnormalities at these regions.

Differentiate joint disease from periarticular lesions.

Define and describe joint abnormality in terms of joint inflammation and/or damage.

The candidate should be able to detect the following signs at non-axial joints:

increased warmth
swelling (fluid, soft tissue, bony)
fluctuance
tenderness
coarse crepitus
restriction of movement
stress pain
associated muscle wasting and weakness.

The candidate should be able to recognise the associated systemic and multisystem
feature of arthritis and connective tissue disease and the need to assess other systems as
appropriate.
If pt has Hyprmobile gait and joint need hypermobility test after pGALS
Preceding to hypermobility examination
General appearance of child for 4 Ds
marfanoid face? facial asymmetry, blue sclera
see if look tall/proportional or disproportional
Look for evidence that hypermobility is affecting function (orthoses)
comment on posture (sitting or standing in a way that places strain on joint ligaments)
Skin bruises
hands
Arachnodactyly (long spider finger- finger and thumb encircling wrist)
Extend 5th finger to extensor forearm (beighton score/1 for each)
oppose thumb to extensor forearm (beighton score/1 for each)
arm:
Scare or bruises
Brachial plus (bounding in AR )
Extend elbow(N 0) (beighton score/1 for each)
head and neck : high arch palat
chest
Deformities
Murmur (MVP/AR)
spine exam
Asses scoliosis; check the back of, for asymmetry of skin creases or shoulder level
lean forward touch floor by palm(beighton score/1 for all)
leg
Trophic scar
extend knee (beighton score/1 for each)
feet
Look pes planus (flat feet) normal up to 6 yrs
gait
May be flat foot and excessive pronation if sever

To complete my examination I would also like to examine the patients.)

Growth charts / Sitting height & arms span (Marfan syndrome) and Plot growth
Other systems related (cardiac)(palate)(eyes) looking for evidence of Marfan syndrome
Parent examination (EDS and marfan are AR)

BEIGHTON SCORE
1. Touch palms on the floor with soles flat and a straight leg (spine hypermobility) 1 point
2. Extend 5th metacarpophalangeal joint more than 90 degrees (1 point for each side)
3. Oppose thumb to forearm (1 point for each side)
4. Extend elbow more than 10 degrees beyond neutral (1 point for each side)
5. Extend knee more than 10 degrees beyond vertical (1 point for each side)
This gives a total of 9 potential points.
A score of more than 4 is suggestive of generalised hypermobility
Hand Examination (look , fell , move then function)
1.initial approach & attitude to the patient (WIPER)
2.Look to surroundings or attached Walking Aids, orthosis or splint
3.General comments for appearance and general observation
4. Ask whether they have any pain in their hands
general hand inspection :
Dorsal hands inspection
Exam both hands at once (comment on tremor and abnormal movement)
Shape and deformity
Arachnodactyly {Marfans, Homocystinuria, MEN 2B}
Polydyctyl ,syndyctyl or overlape (PWS< LMBS)
Short 4th/5th metacarpal {PseudohypoPTH, Turners}
Broad and short hands (down syndrome)
Large (Acromegaly)
Bony abnormalities: Absent Thumb (Holt-Oram Syndrome)
Asymmetry
Muscles and soft tissues
wasting (small hand muscles ,thenar,1st dorsal interosse)(myopathy ,arthritis ,CNS)
hand contraction deformity ,dupuytrens contaction (chronic liver diseases rare)
thickening synovial sheath leads to swelling on the dorsum of the wrist and the front
of the fingers and wrist in rheumatoid arthritis (tendon sheath arthritis)
spindling of proximal IPJ , disorganisation subluxation and wasting of small muscles.
Psoriatic arthritis involves the distal interphalangeal joints and nails
Tuberous and tendon xanthomas (H.cholestronemia)
SC nodules(RF)
Nails
Onycholitis {separation nail}
Clubbing (test)
Pitting (psoriasis)
Splinter hemorrhages (IE)
Koilonychia {Spoon nails iron defn anaemia}
Leukonychia {white lines } (malnutrition ,chemoRx)
Beaus lines {transverse linear depressions illness, trauma, malnutrition}
Brown Lines {Renal build up waste}
Nail bed telangiectasia { Dermatomyositis)
Haemorrhage in nail fold { Dermatomyositis ,rheumatoid arthritis and scleroderma }
Skin
Eczema ,Cotact dermatitis , Scars (preterm)
Stigma : Neurocutanouse syndrome I.E stigma
Posture Hand
Claw { Ulnar N}-Hyperextension MCP weakness interosse Muscles (brachial palsy)
Wrist drop- Radial N palsy
Ulnar deviation - {RH arthritis}
Hemiplegic - (flexed hand and arm) (central lesion)
2.Inspect nail from side for clubbing and hyperconvexity (turner)

3.Palmar hands inspection (same as dorsal)

For deformity , asymmetry , Muscles and soft tissues , Skin, Posture
muscle wasting (palmar thener, hypothenar) (myopathy ,arthritis)
pallor (anemia)
single simen creases (down syndrome)
Janeway Lesions and oslers nodes (IE)

Palpation (both sides) : Always ask about pain first

perfusion, Temp, Sweating
Palpate swelling (bony or soft tissues)
Squeeze the metacarpal joints to feel for tenderness
Could asses sensation
radial n (dosal anatomical snufflex hand sensation)
ulnar n (palmar sensation of medial 1st ,2nd and medial half of middle fingers)
median n(palmar sensation of thumb ,index and lateral half of middle fingers)

Move
Range of movement
MCP : flextion (90 o) ,extension (30)
PCP : flextion (100 o)
DCP : flextion (90 o) ,extension (10)
Quicq asses of hand movement
Make fist ,finger flexion at each joint (median and ulnar n.)
Make astar fingers spread out abduction (ulnar), and extended (radial)
Make circle with flex thumb and index finge (median n.)

Function
Doing and undoing buttons
Writing and draw apencil
Grip strength
Use spone and knife
Hold acup

Consider performing a pGALS screening examination if joint pathology found in the hand
Mention the need for a neurological examination if any motor or sensory disturbance is
found; pay close attention to the brachial plexus and cervical spine
Hand nerves
radial nerve
dosal anatomical snuffbox hand sensation
wrist ,thumb and finger extension
Radial N palsy Wrist drop

Ulnar nerve
palmar sensation of medial 1st ,2nd and medial half of middle fingers
finger flextion
Ulnar N palsy cause claw hand (Hyperextension MCP )
weak small palmar muscles (interosse Muscles) except thener emenence

median nerve (brachial plexus)

palmar sensation of thumb ,index and lateral half of middle fingers
thumb adduction and abduction
median N palsy cause weak small palmar muscles (thener emenence )

brachial palsy
upper brachial nerves palsy C5 ,C6)(erbs palsy)

assymetric moro
adduction and internal rotation shoulder
extension elbow
pronation forarm
flextion wrist (waiter hand)

lower brachial nerves palsy C8 ,T1)(klumps palsy)

The hand is flaccid with little or no control.
If the sympathetic fibers injured, ipsilateral ptosis and miosis can occur.
If Swollen distal IPJ in one hand + pits on the nail of this finger. This psoriatic arthritis

I would like to carry out further joint examination and look for the rash of psoriasis.

- I would like to do pGALS looking for asymmetric oligoarthritis of large and small joints
- I would like to look for the rash of psoriasis.
- I would like to examine for signs of eye disease

If Swollen wrists + restriction of movement at wrists + swelling of proximal IPJ this JIA

- I would like to do a detailed examination of all joints

- I would like to examine for signs of eye disease
- I would like to review the growth
- I would look for signs of steroid toxicity
- I would like to check the skin

If fixed flexion deformity of elbow + bruising, although the joint is not painful this haemophilia

- How does arthropathy reflect the seriousness of the haemophilia?

- How do you treat bleeding into the joint?

If ony deformities in the arms and legs + blue sclera. this osteogenesis imperfecta

- How does arthropathy reflect the seriousness of the haemophilia?

- How do you treat bleeding into the joint?
talipes equinovarus :
clubfoot deformity. It is characterized by 3 primary components:

1. entire foot planter flexion (equinus)

2. hind foot inversion (varus)
3. The forefoot adductus deformity

The birth prevalence is 0.9 per 1000 live births, with a ratio M to F of 2 : 1.
The typical idiopathic congenital clubfoot must be differentiated from similar deformity to:
o Spinal cord tethering
o Myelodysplasia
o Degenerative neurological conditions

Positional talipes from intrauterine compression is common.

It is of multifactorial inheritance, but may also be secondary to
oligohydramnios during pregnancy.
a malformation syndrome
a neuromuscular disorder such
spina bifida.
association with developmental dysplasia of the hip (DDH).

Treatment is started promptly, while the tissues are lax, with stretching and strapping or
serial plaster casts.
If this corrects the disorder, treatment can be discontinued or night splints used.
If the condition is severe, corrective surgery is usually necessary.
As the results of corrective surgery performed at a few weeks of age have been disappointing,
surgery is usually delayed to 6-9 months of age.
The condition needs to be differentiated from the rare congenital vertical talus, where the
foot is stiff and rocker-bottom in shape.
Many of these infants have other malformations.
The diagnosis can be confirmed on X-ray. Surgery is usually required.
Talipes calcaneovalgus
The foot is dorsiflexed and everted .
It usually results from intrauterine moulding and self-corrects.
Passive foot exercises are sometimes advised.
There is an association with developmental dysplasia of the hip
Patterns of Abnormalities
The candidate should be able to recognise the clinical presentation, and compose an
appropriate differential diagnosis for the following:

Acute monoarthritis

Chronic monoarthritis, Oligoarthritis and Polyarthritis

Scoliosis

Dislocated hip and developmental dysplasia at the hip

Contracture syndromes including arthrogryposis

Congenital deformities

Functional gait abnormalities

The limping child

The role of a musculoskeletal screening examination (pGALS)

The pGALS screening examination (paediatric Gait, Arms, Legs and Spine) is simple and quick
and helps to localise the site of joint problems.

pGALS is very useful to identify the pattern of joint involvement especially where symptoms
are illocalised

The pGALS screen findings help to focus a more detailed regional examination
Juvenile idiopathic arthritis (JIA)
Epidemiology

Age at onset extremely rare prior to 6 months

Peak age
1-3 years (girls)
8-12years (boys and girls)
Girls are affected more commonly than boys, particularly with respect to the
pauciarticular form of illness
Lower limb more commonly affected

Classification
Classification of juvenile idiopathic arthritis (JIA) according to International
League Against Rheumatism (ILAR)
Oligoarthritis (Extended /Persistence)

Polyarthritis (either RF + or -)

Psoriatic Arthritis

Enthesitis-related Arthritis

Systemic Onset
 Classification and clinical features of JIA (juvenile idiopathic arthritis)

Onset age Sex Articular pattern Extra-articular features

JIA subtype ratio Laboratory
(approximate %) (f : m) abnormalities

Oligoarthritis 1-6 years 5:1 1-4 (max) joints anterior uveitis 20%, ANA++
(persistent) (50%) knee, ankle or wrist leg length discrepancy
Prognosis excellent > 90%
Remission 60%
Oligoarthritis 1-6 years 5:1 > 4 joints > first 6 mo. anterior uveitis 20% ANA++
(extended) (8%) Asymmetrical asymmetrical growth
large and small joints Prognosis moderate
Polyarthritis 1-6 years 5:1 Symmetrical large Low-grade fever, ANA+
(RF -ve) (16%) and small joint arthritis, anterior uveitis 5%, ESR, WBC,
often with marked finger reduction of growth rate CRP,platelets
involvement
Prognosis moderate elevated
Cervical spine and TMJ
may be involved. Remission 20%
Polyarthritis 10-16 years 5 : 1 Symmetrical large Rheumatoid nodules 10% ANA+
(RF +ve) (3%) and small joint arthritis, No uveitis RF+
often with marked finger Prognosis poor ESR, WBC,
involvement CRP,platelets
Remission <5%
elevated
Systemic arthritis 1-10 years 1 : 1 Oligoarthritis or Acute illness, malaise, Anaemia raised
(9%) polyarthritis high daily fever initially, neutrophils and
have aches and pains in with salmon-pink, macular platelets, and
joints and muscles CRP.
rash, lymphadenopathy,
(arthralgia/myalgia) but
initially no arthritis hepatosplenomegaly,
serositis.
No uveitis
Prognosis variable to poor
Remission 50%
Diagnosis:

How do we make the diagnosis?

Systemic JIA is a clinical diseasethere is not one lab test that definitively makes the Dx

Does the patient have swollen joints not just pain?

How long has the swollen joint been there?

Are they below 16?

Do they have any other medical problems?

What is the fever pattern

Do they have signs of MAS?

Did you look for other possibilities?

The diagnosis of JRA is established by:

The presents of arthritis

The duration of the disease for at least 6 weeks
The exclusion of the possible diagnosis.

Although a presumptive diagnosis of systemic onset JRA can be established during the
onset of the systemic phase, a definitive diagnosis is not possible until arthritis develop.

Children must be <16 years old at time of onset of disease, the diagnosis of JRA does not
change when the child become adult.

The acute arthritides can affect the same joints as JRA, but have a shorter time course.

In particular JRA can be confused with the spondyloarhtropathies, which are associated
with the spinal involvement, and enthesitis, which is inflammation of tendinous insertions.

All of the paediatric spondyloarthropathies can present with peripheral arthritis before
other manifestations and initially may be diagnosed as JRA .

Because there are so many other causes of arthritis, these disorders need to be excluded
before providing a definitive diagnosis of JRA .
Differential diagnosis of Juvenile arthritis
Connective tissue diseases:
SLE
Juvenile dermatomyositis
Scleroderma with arthritis
Seronegative spondyloarthropathy:
Infectious arthritis:
Bacterial arthritis
Viral arthritis
Fungal arthritis
Lyme disease
vasculitis arthritis:
Henoch-Schonlein purpura
Kawasaki
Rheumatic arthritis:
Rheumatic fever
Reactive arthritis:
Reiter syndrome
IBD
Toxic synovitis
Orthopedic disorders:
Traumatic arthritis
Legg-Calve-Perthes disease
Slipped capital femoral epithesis
Mucloskeletal Pain Syndromes:
Growing pains
Hypermobilitis syndromes
Myofascial pain syndromes/fibromyalgia
Haematological/Oncological Disorders:
Leukemia
Lymphoma
SCD
Thalassemia
bones tumors
Metastatic bone disease
Differential diagnosis of systemic arthritis
Infection - bacterial/viral/protozoal (e.g. malaria), Mycoplasma and other (Lyme)
Kawasaki's disease
Rheumatic fever
Reactive arthritis - post-streptococcal, post-enteric, post-viral
Malignancy - leukaemia, neuroblastoma
Connective tissue disorders - systemic lupus erythematosus (SLE), P. nodosa (PAN)
Laboratory investigations in JRA:

CBC show Anaemia, elevated WBC, CRP, ESR and Platelets: in polyarticular and systemic
onset forms

ANA: in patients with pauciarticular to identify patients with risk for uveitis.

RF: in older patients and adolescents with polyarticular disease to identify children with
early onset adult rheumatoid arthritis.

Diagnostic arthrocentesis may be necessary to exclude supportive arthritis in children who

present with acute onset of monoarticular symptom.

The synovial fluid WBC count is typically < 50,000 to 100,000/mm3 and should be
predominantly lymphocytes rather than neutrophils seen with supportive arthritis and
should have gram negative stain and culture

Radiological:

The most common finding in early stage is a normal bone x-ray.

Over time peri-articular osteopenia resulting from decreased mineralization

Growth centres may be slow to develop, whereas there may be accelerated maturation of
growth plates or evidence of bony proliferation.

Erosions of bony articular surfaces may be late finding.

If the cervical bone is involved, fusion of C1-4 may occur, and atlanto-axial subluxation may
be demonstrable
Management
Approach Considerations

The ultimate goals in managing rheumatoid arthritis are to

suppressing inflammation to prevent or control joint damage
prevent loss of function
to decrease pain.
preventing blindness

JIA is a chronic disease characterized by periods of remission and flare.

Treatment is aimed at inducing remission with the least toxicity from medications with
hopes of inducing a permanent remission.
The success of therapy is monitored best with repeated physical examinations and history.
The number of joints involved and the duration of morning stiffness should demonstrate
continued decrease, with elimination reflecting success.
Surgery may be indicated in patients who are unresponsive to medical therapy.
Treatment A team-based approach

Management may include 1 or all of the following areas:

Pharmacologic therapy
Psychosocial interventions
Measures to enhance school performance
Improved nutrition to address anemia and osteoporosis
Physical therapy
Occupational therapy
Patient education and the support of an experienced paediatric
rheumatology team are essential.
The team should include a paediatric rheumatologist, and staff with specific
paediatric expertise in rehabilitation, disease education, nutrition, drug monitoring,
school liaison, family and social support and psychology.
In addition, there needs to be ready access to other paediatric specialties including
Ophthalmology
Orthopaedics
maxillo-facial surgery
nephrology
dermatology
psychiatry.
The inverted pyramid approach is preferred to the conventional pyramid approach as
early control of inflammation is achieved with a combination of rapid acting anti-
inflammatory medications & slower acting second line drugs.

Drugs used in treatment of JIA:

NSAIDs remain the first line, particularly ibuprofen. Doses are usually high and antacids
are occasionally required for gastrointestinal sideeffects
Aspirin products
Systemic corticosteroids(methylprednisolone, prednisone and prednisolone):
gain control of active polyarthritis with significant active arthritis
life-saving in systemic arthritis with pericarditis.
bridging therapy until other medications take effect.
intra-articular Corticosteroid injection (triamcinolone) in oligoarticular JIA.
Disease-modifying anti-rheumatic drugs (DMARD)
Methotrexate , Sulfasazine ,Cyclosporine
Thalidomide ,Azathioprine ,Leflunamide
used in patients whose arthritis does not come under complete control with NSAID alone.
Biologic agents
These drugs are targeted therapies directed against specific pathologic mechanisms
which are present in inflamed joints biological agents are increasingly being used for
those not adequately controlled on methotrexate or those intolerant of it.
This include
Anti-TNF :Etanercept/ Infliximab /Adalimumab (Humira TM)
Interleukin-1 receptor antagonist :Anikinra(Kineret TM)
Interleukin-2 receptor antagonist :canakinumab / Rilonacept
Interleukin-6 receptor antagonist : Tocilizumab (Actemra TM)

Stem Cell Transplant

Exercise and Other Nonpharmacologic Therapy
Physiotherapy and occupational therapy
- Hydrotherapy
- Passive movement
- Gentle exercises
- Splints
Surgical Treatment
Advances in medical treatment have reduced the need for surgical intervention in JIA.
Possible procedures include.
Synovectomy
Osteotomy and arthrodesis
Total hip and total knee replacements

Hospital Admission

Inpatient care is required for persisting fevers of unknown origin or when children with
known JIA have severe exacerbation of disease.
Admit for evaluation any child who loses the ability to walk for unknown reasons.
The development of pericarditis in children with systemic-onset JIA is usually an indication
for admission.
The rash of systemic JRA is diagnostic only after the diagnosis is
made (by exclusion).
Uveitis in JRA
SYSTEMIC LUPUS ERYTHEMATOSIS
Clinical Findings
The symptoms depend on the organ involved with immune complex deposition.
SLE is thought to affect women of child-bearing age, approximately 5% of SLE
presents in childhood, mostly around puberty.

SLE is rare in children younger than 9 years old

Females > Males 8:1 between ages 9 and 15 years

Systemic Manifestations These include

Weakness
Anorexia
Fever (70%)
Fatigue
weight loss.

Joint Symptoms (80-90%)

Joint symptoms are the most common presenting feature - nonerosive
Nondeforming arthritis may involve any joint, often in asymmetric manner.
Myositis may also occur and is more painful than the dermatomyositis.

Diagnosis
The diagnosis of lupus is confirmed by the combination of clinical and laboratory
manifestations revealing multisystem disease.
The presence of 4 of 11 criteria serially or simultaneously strongly suggests the
diagnosis which has 98% sensitivity and 97% specificity for SLE..
Patients suspected to have lupus who demonstrate fewer than 4 criteria should
receive appropriate medical treatment.
Revised Classification Criteria for Systemic Lupus Erythematosus
CRITERION DEFINITION
Malar rash Fixed erythema, flat or raised, over the malar eminences, tending to
spare the nasolabial folds
Discoid rash Erythematous raised patches with adherent keratotic scaling and
follicular plugging; atrophic scarring may occur in older lesions
Photosensitivity Rash as a result of unusual reaction to sunlight (elicited by patient
history or physician observation)
Oral ulcers Oral or nasopharyngeal ulceration, usually painless, observed by a
physician
Nonerosive involving two or more peripheral joints, characterized by tenderness,
Arthritis swelling, or effusion
Serositis Pleuritis: pleuritic pain or rub or evidence of pleural effusion or
Pericarditis:documented by ECG or rub or evidence of p. effusion
Renal disorder Persistent proteinuria >0.5 g/day or >3plus (+ + +) or
Cellular casts: may be RBC, hemoglobin, granular, tubular, or mixed
Neurologic Seizures:in the absence of offending drugs or known metabolic
disorder derangements (e.g., uremia, ketoacidosis, or electrolyte imbalance)
or
Psychosis:in the absence of offending drugs or known metabolic
derangements (e.g., uremia, ketoacidosis, or electrolyte imbalance)
Hematologic Hemolytic anemia, with reticulocytosis or
disorder Leukopenia: <4,000/mm3 total on two or more occasions or
Lymphopenia: <1,500/mm3 on two or more occasions or
Thrombocytopenia: <100,000/mm3
Immunologic Anti-DNA antibody to native DNA in abnormal titer or
disorder Anti-Smith:presence of antibody to Smith nuclear antigen or
Positive finding of antiphospholipid antibodies based on
(1) an abnormal serum level of lgG or lgM anticardiolipin antibodies;
(2) a positive test result for lupus anticoagulant( antiRa //anti La) or
(3) a false-positive serologic test for syphilis

Antinuclear An abnormal titer of antinuclear antibody by immunofluorescence or an

antibody equivalent assay at any time and in the absence of drugs known to be
associated with drug-induced lupus syndrome
 Differential Diagnosis

Many of the clinical manifestations of SLE are found in other inflammatory

illnesses and during acute or chronic infection diseases such as;-
rheumatic fever
rheumatoid arthritis
viral infections.
An overlap syndrome known as mixed connective tissue disease

Treatment
Patients with SLE should be treated by specialists in medical centers where both
medical and psychologic support can be given to both patients and their families
Prednisone, 0.51 mg/kg/d orally, has significantly lowered the mortality
rate in SLE and should be used in all patients with renal, cardiac, or CNS
involvement. followed by a slow steroid taper over 4-6 mo beginning 4-6 wk after
achieving a serologic remission.

Three circumstances in particular warrant renal biopsy:

A child with SLE and nephrotic syndrome-to distinguish membranous
glomerulonephritis from diffuse proliferative glomerulonephritis
Failure of high-dose corticosteroids to reverse deteriorating renal function
A prerequisite to entry into clinical therapeutic trials

Skin manifestations, arthritis, and fatigue may frequently be treated with antimalarials such
as hydroxychloroquine, 57 mg/kg/d orally.

If disease control is inadequate with prednisone or if the dose required produces intolerable
side effects, an immunosuppressant should be added. Either azathioprine, 23 mg/kg/d
orally, or cyclophosphamide, 0.51 g/m2, administered intravenously once a month, has
been most widely used. Recent studies indicate that mycophenolate mofetil may used in
place of intravenous cyclophosphamide to induce remission or sustain remission after
intravenous cyclophosphamide therapy.

Thrombotic events due to clotting antibodies require long-term anticoagulation.

Juvenile dermatomyositis
This is an unusual case and a rare condition in clinical practice.

The following will help to increase your awareness of the condition and will be of
practical use if you see a case.

20% present in childhood, usually the second decade

Acute or sub-acute onset

Painful tender muscles with lethargy, skin rashes and proximal muscle weakness either
shoulder girdle or lower limbs

Usually asymmetrical

Rash characteristic upper eyelids and upper cheeks (butterfly), rash also over elbows,
hyperaemia of nail beds

Can get dysphagia (adults), skin calcification

Raised ESR and CPK

Confirm diagnosis with muscle biopsy

Treatment difficult and with long-term steroids other immunosuppression

Osteogenesis imperfecta
Osteogenesis imperfecta is a disorder of connective tissue characterised by bone Fragility

- Osteogenesis imperfecta type I AD

- Osteogenesis imperfecta type II AR lethal syndrome
- Osteogenesis imperfecta type III AR
- Osteogenesis imperfecta type IV AD
Orthoses
Children with neurological disorders are very prone to developing joint contractures and
bony abnormalities, particularly spinal kyphoscoliosis.

Orthoses are useful in helping to prevent contractures, in maintaining a good position if

contractures have been repaired and in providing joint stability.

They are particularly useful in aiding individual children with mobility.

The type of orthosis depends on the childs individual needs, eg they may be

- anklefoot orthoses if there is just ankle and foot involvement,

- anklefoot orthoses extending to the knee if the knee is involved.
- thoracolumbar orthoses are available for a scoliosis.
- Wrist orthoses are also commonly used.
Consultation

Conflict resolution /drug error (methotrexate for arthritis)

SCENARIO

pharmacist informs you that a drug error has been made. Steven, 13, who is meant to be
receiving weekly methotrexate for arthritis, has instead received adaily dose over the
holiday weekend. Steven's mother is waiting in the parents' room and is aware a drug error
has been made. She is understandably upset over the potential consequences.

You must counsel Steven and his mother about the drug error and discuss
what will be done about it.
1.Greeting the mother
2.Introduce yourself with name, make a good rapport
3.explain that you have asked the named nurse also to attend and have made efforts
not to be disturbed, e.g. cannot be bleeped.
4.apologise for the error.
5.said we understand what you particularly worried about?
6.Explain what will be done about it . Risk management to deal with error, i.e.
notification of the critical incident (and that you will complete the form) and if they
wish to take the matter further to involve the patient advisory liaison service.
6.Management regarding effects of medication: blood tests, side effects.
Methotrexate's toxic effects are related to its interaction withthe folic acid pathway.
Side effects may be dose dependent or independent:
Mouth sores - dose dependent
Stomach upset (nausea, vomiting, diarrhoea) - dose dependent
Liver toxicity - dose independent
Pneumonitis - dose independent
Bone marrow toxicity
Headache
Drowsiness
Itchiness
Skin rash
Dizziness
Hair loss
Low white cell count.
Important blood tests include full blood count, liver function tests, urea and creatinine

7. Establish parental understanding, Do you have any questions for me?

8. thanks