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Exam 3: Pediatric musculoskeletal disorders

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Variations of normal posture
most resolve without any treatment
If severe, persistent, or painful, refer!
Genu Varum
Bow legs
Common in children or toddlers < 3 years old
Seldom needs treatment
Rickets
Blount's disease
Rickets
Abnormal x-ray of metaphyses
Blount's disease
Infantile tibia vara
Afro-Caribean children
Beaking of the proximal medial tibial epiphysis on x-ray
Orthotics or surgery
Genu Valgum
Knock-knees
Common in children between 2-7 years old
Usually resolves
Pes planus
Flat feet
Common in toddlers learning to walk-resolves (fat pad disappears)
Most people develop a medial longitudinal arch, some don't
Can occur with Ehlers-Danlos syndrome
Surgery only indicated in symptomatic adolescents
3 main causes of in-toeing
Metatarsus varus
Medial tibial torsion
Persistent anteversion of the femoral neck
Metatarsus varus
An adduction deformity of a highly mobile forefoot
Medial tibial torsion
At the lower leg
Tibia is laterally rotated less than normal in relation to the femur
Persistent anteversion of the femoral neck
At the hip, the femoral neck is twisted forward more than normal
Out-toeing
Uncommon
Infants 6-12 months of age
When bilateral, it is due to lateral rotation of the hips
Resolves spontaneously
Toe walking
Common in 1-3 year olds
Can be persistent, usually from habit
May be due to mild cerebral palsy
Can occur from tight Achilles tendons
Duchenne's muscular dystrophy
Limp
Developmental dysplasia of hip (DDH)
Often detected in newborn
If detected later-limp
Painful or painless
can refer pain to knee
Developmental dysplasia of the hip (DDH)
Previously called congenital dislocation of the hip (CDH)
Spectrum of disorders
Early detection important-conservative tx
Late diagnosis-hip dysplasia-complex treatment (surgery)
Neonatal screening
Barlow & Ortolani's maneuver
Asymmetry of skinfolds
Limited abduction of hip
Shortening of affected leg
Limp
Abnormal gait
If DDH suspected
Orthopedic consult
X-ray or US
Positioning device, puts hips in abduction
Satisfactory response usually
Can use splinting
Surgery occasionally
Transient synovitis (TX, irritable hip)
Most common cause of acute hip pain in children
Occurs in children of 2-12 years old
Often follows or is accompanied by viral infx
Presentation of TS
Sudden onset of pain in hip or a limp
No pain at rest
Decreased range of motion (especially external rotation)
Pain may refer to knee
Afebrile or mild fever
Child does not appear ill
Labs for TS
Neutrophil count & acute phase reactants are normal or slightly raised
Blood cultures negative
X-ray of joint normal
Small joint effusion of ultrasound
Tx for TX
bedrest
Septic arthritis
Child with high fever, child looks sick
Pain at rest
Minimal or no movement of hip
Neutrophil count & acute-phase reactant are markedly high
Aspirate joint under US guidance
Usually blood borne illness
bacteria associated with septic arthritis
Group B strep (strep pyogenes) and staph aureus
H. influenzae now uncommon due to immunization
Tx of septic arthritis
Ortho consult
Infectious disease consult
X-rays
Aspiration of the joint space under US guidance
C&S
IV antibiotics
Surgical drainage
Legg-Calve-Perthes disease
Avascular necrosis of proximal femur (Perthes disease)
Avascular necrosis followed by revascularization and reossification over 18-36 months
Male: Female (5:1)
5-10 years old
Insidious onset
Limp or hip pain
May mimic transient synovitis
Bilateral in 10-20%
X-rays of Legg-Calve-Perthes disease
Increased density in the femoral head
Subsequently becomes fragmented and irregular
Bone scan and MRI helpful also
Prognosis of Legg-Calve-Perthes disease
Good in most, especially in those with <6 years old with < half the epiphysis involved
Can result in DJD through adult life
Tx of Legg-Calve-Perthes disease
Bed rest
traction
cast
surgery
Slipped capital femoral epiphysis
Displacement of the epiphysis of the femoral head posteroinferiorly
Most common at 10-15 y/o during adolescent growth spurt (especially in obsese boys)
Skeletal maturation may be delayed
Presentation of slipped capital femoral epiphysis
Limp or hip pain
Can refer to knee
Restricted abduction and internal rotation of the hip
Onset may be acute following minor trauma
20% have it bilateral
Dx and Tx of Slipped capital femoral epiphysis
X-ray is diagnostic
Tx is surgery
Osgood-schlatter disease
Overuse syndrome
Physically active males around puberty
Detachment of cartilage from tibial tuberosity
Localized pain and swelling over tibial tubercle
Bilateral in 25-50%
Most resolve with reduced physical activity
Splint
Chondromalacia patella
Softening of the articular cartilage of the patella
Most often adolescent females
Pain with movement of patella
Pain with standing up from sitting or walking up stairs
Tx of chondromalacia patella
Rest and physiotherapy for quadriceps muscle strengthening
Osteochondritis dissecans
Separation of bone and cartilage from medial femoral condyle (other bones too) following avascular necrosis
Knee pain
Kids in sports
May have loose articular bodies
Treatment of osteochondritis dissecans
Rest, quadriceps exercises
Surgery?
Subluxation and dislocation of the patella
Feeling of instability or giving way of knee
Occurs suddenly
Reduction spontaneously or on gentle extension of knee
X-ray of subluxation and dislocation of patella
Needed to differentiate loose bodies from bone gx
Tx of subluxation and dislocation of patella
Immobilization or surgery
Painful knee due to injury
Menisci
Ligaments
MRI of knee
Tx: usually conservative
If infant or young child: fracture more likely (ligaments can be stronger than bones)
Postional talipes
From intrauterine compression
Common
Can be corrected to the neutral position with passive manipulation
Passive exercises
Talipes equinovarus (clubfoot)
Inverted/supinated
Forefoot adducted
Heel rotated inwards and in plantarflexion
Affected foot shorter and half muscles thinner
Foot position is fixed
Often bilateral
0.9 in every 1000 live births
Males: Females (2:1)
Talipes equinovarus (club foot)
Multifactoral inheritence
Also due to oligohydramnios
Can also be afeature of a malformation syndrome or a neuromuscular disorder (spina bifida)
Association with developmental dysplasia of hip (DDH)
Confirm dx with x-ray
Tx of club foot
Started early while tissues are lax
Stretching and strapping
Serial plaster casts
If severe-surgery
-surgery may be delayed to 6-9 months of age
Scoliosis
Lateral curvature of the spine
Rotation of the vertebral bodies-back asymmetry
Severe: can lead to cardiorepiratory failure from distortion of the chest
Causes of scoliosis
Idiopatic: most common
Secondary:
-neuromuscular imblanace: cerebral palsy, muscular dystrophy, polio
-Disorders of bone: Neurofibromatosis, connective tissue abnormalities (Marfan's), Postural (leg length discrepancy)
Screening for scoliosis
Can identify when child bends forward
X-ray
Tx of severe scoliosis
Spinal braces-efficacy questionable
Spinal surgery
Osteomyelitis
Infx of the metaphysis of the long bones
Most common sites are distal femur and proximal tibia
Usually due to hematogenous spread of the pathogen
Swelling, pain, warmth, immobile limb
May develop into septic arthritis
Most are S. aureus, also strep and H. flu
Sickle cell anemia: increase risk of staph and salmonella
Labs for osteomyelitis
+ blood cultures
WBC increased
Acute-phase reactants increased
7-10 days after infx
X-rays abnormal
-subperiosteal new bone formation
-localized bone rarefaction
Bone scan
Tx of osteomyelitis
IV antibiotics
Aspiration or surgical decompression
Surgical drainage
Splint
Mobilization
Juvenile rheumatoid arthritis (JRA)
Nonmigratory monarticular or polyarticular arthropathy
Tendency to involve large joints or proximal interphalangeal joings
Lasts > 3 months
Systemic manifestations of JRA
Fever
Erythematous rash (salmon-pink rash at the height of the fever)
Nodules
Leukocytosis
Sometimes: Iridocyclitis, pleuritis, pericarditis, anemia, fatigue, growth failure
HLA-DR5
associated with iritis and production of ANA antibodies
HLA-DR4
Associated with seropositive polyarticular disease
May be related to formation of antisuppressor cell antibodies, immune complex generation, chronic inflammation
3 major presentations of JRA
Acute febrile form (Still's disease)
Polyarticular form
Pauciarticular form
Still's disease
Evanescent salmon pink macular rash
Arthritis
Hepatosplenomegaly
Leukocytosis
Polyserositis
Episodic illness
Remission of systemic features usually within 1 year
Do not develop iridocyclitis
Polyarticular form
Resembles the adult disease
Crhonic pain and swelling of many joints in a symmetrical fashion
Both large and small joints
Systemic features of polyarticular form of JRA
Less prominent, but can include
-low grade fever
-faigure
-rheumatoid nodules
-anemia
These patients tend to have long-standing arthritis
May wax and wane
Iridocyclitis occurs occasionally
Older children may have + rheumatoid factor test
Pauciarticular form
Chronic arthritis of a few joints (usually large weight-bearing joints)
Asymmetrical
Synobitis mild and may be painless
Systemic features uncommon
Extra-articular involvement of pauciarticular form of JRA
Inflammation in the eye
Up to 30% of children with pauciarticular JRA develop insidious, asymptomatic iridocyclitis (can cause blindness)
Severity of the eye disease does not correlate with that of arthritis
Routine opthalmologic screening with slit lamp exam q 3 months if ANA+ (q6 months if ANA- for 4 years after onset of arthritis)
Lab findings for JRA
No completely reliable dx test available
RF+
ANA+
ESR increased
Joint fluid in JRA or other inflammatory arthritis (not infx)
Imaging: MRI may show joint damage earlier than x-ray
Objectives of tx of JRA
Restore function
Relieve pain
Maintain joint motion
Tx of JRA
NSAIDS
Physical therapy (stretching, position, joint protection)
Immunosuppressive agents
TNF inhibitors
Leflunomide (Arava)
Infliximab (Remicade)
NSAIDS for JRA
Naproxen
Ibuprofen
Diclofenac
Tolmetin
Indomethaicin
Immunosuppressive agents
Methotrexate
Sulfasalazine
Methylprednisolone
Prednisone
TNF inhibitors
Etanercept (newer) (Enbrel)
Leflunomide (Arava)
Mechanism of action not fully understood
Metabolite inhibitor pyrimidine neucleotide synthesis
ANtiproliferative for T-cells
Infliximab (Remicade)
Recombinant humanized monoclonal antibody to TNF-alpha
Prevents synovial and interstitial inflammation
Following pt with JRA
Pediatric opthamologist
Refer to specialist
Local corticosteroid injections into joints
Synovectomy
Joint replacement
Admit when: persistent fevers of unkknown origin, severe exacerbation of disease, inability to walk, development of pericarditis
Continued outpatient visits
Prognosis for JRA
In the primarily articular forms of JRA, disease activity diminishes progressively with age and ceases by puberty in about 85% of patients
In a few instances, the disorder will persist into adult life
Problems after puberty usually due to residual joint damage
Presentation in teen years usually continues into adult disease
Juvenile spondyloarthropathy
HLA B27 associated arthritis
Similar, but different than ankylosing spodylitis in adults
Mostly older boys
Large joint arthritis (esp in lower limbs)
Swollen digit (sausage finger)
HLA-B27 tissue type
+ family history
Juvenile spondyloarthropathy
Inflammation of insertion of tendons into bone (achilles tendon, plantar fascia, enthesitis)
May be sacroiliac and spinal
Acute symptomatic iritis may occur
Does not frequently progress to joint destruction or ankylosis in children as it does ina dults
Tx of juvenile spondyloarthropathy
NSAIDs
Methotrexate (Trexall)
Etenercept (Enbrel)
Infliximab (Remicade)
Achondroplasia
Autosomal dominant
50% are new mutations
Short stature (short limbs)
Large head
Frontal bossing
Depression of nasal bridge
Hands short and broad
Lumbar lordosis
Hydrocephalus sometimes (closely monitor head circumference)
Cognitively normal
Can be dx prenatally on US
Long bone surgery possible to increase height
Osteogenesis imperfecta
Brittle bone disease
Collagen metabolism problem
Bone fragility
Bowing and frequent fracture
Child abuse is usually suspected, then this is found
Type 1 osteogenesis imperfecta
Most common form
Autosomal dominant
Fractures occur during childhood
Blue sclera
Hearing loss
Type 2 osteogenesis imperfecta
Multiple freacture already present before birth
Often stillborn
Inheritance variable: mostly autosomal dominant or due to new mutations
Marfan's syndrome
Autosomal dominant
Disorder of connective tissue
Tall stature, long thin digits (Arachnodactyly), hyperextensible joints, high arched palate, dislocation of the lenses of the eyes, severe myopia, long thin limbs, arm span is greater than height, chest deformity and scoliosis, degeneration of the media and vessel walls
Rickets
Vitamin D deficiency
Dietary
X linked dominant genetic disorder
Liver failure, especially due to biliary atresia
Results in low serum 25 (OH) D
X linked genetics with Rickets
Familial (primary) hypophosphtemia
Kidney problem with problems in making vitamin D
Low serum calcium, low serum phosphate, elevated serum alkaline phosphhatase, hyperphosphaturia, NO hyperparathyroidism
Liver failure with Rickets
Poor bile salt secretion into bowel
Poor absorption of fat-soluble vitamins, including vitamin D
Osteoscarcoma
6th most common cancer in childhood, it is 3rd in adolescents and young adults
Distal femur >40%
-also proximal tibia, proximal humerus, and mid and proximal femur common
Bone pain over cancer and may have mass in that area: Several months prior to diagnosis
Labs for osteosarcoma
Elevated alk phos or LDH levels
Characteristic radiographic appearance (MRI)
Biopsy of osteosarcoma
To be done by surgeon doing the definitive procedure
Tries to salvage limb
Ewing sarcoma
Only 10% of primary malignant bone tumors
White males
Mostly in 20s
Pain at site of tumor, may have swelling and erythema
Fever, weight loss
Elevated LDH
Can metastasize
Biopsy
Chemo and surgery
Rhabdomyosarcoma
Painless and progressively enlarging mass
Soft tissue sarcoma
Anywhere on body
Abnormal function due to tumor growth