O: Paediatric Orthopaedics Flashcards
what are the 4 congenital disorders?
osteogenesis imperfecta, skeletal dysplasia, marfan’s syndrome, ehlers-danlos syndrome
what is osteogenesis imperfecta?
defect of maturation and organisation of T1 collagen
aetiology of osteogenesis imperfecta?
mutation to T1 collagen genes- COL1A1/ COL1A2
which types of osteogenesis imperfecta are the most severe?
T3 & T4
s/s of osteogenesis imperfecta?
multiple fragility fractures in childhood, short stature with multiple other deformities, blue sclerae, loss of hearing, scoliosis
what are some x-ray findings of osteogenesis imperfecta?
calloused bone healing and thin cortices
mx of osteogenesis imperfecta?
treat fractures, bisphosphonates. no cure
what should you be aware of when treating osteogenesis imperfecta?
non accidental injury and osteopenia as both may result in multiple fracture presentations
what is skeletal dysplasia?
dwarfism
types of skeletal dysplasia?
proportionate, disproportionate (limbs shorter than spine),
which is the most common type of skeletal dysplasia?
Achondroplasia: short limbs, prominent forehead, widened nose
what is Marfan’s syndrome?
autosomal dominant mutation of fibrillin gene
s/s of Marfan’s?
tall stature, disproportionate long limbs, ligamentous laxity, high arched palate, pacts excavatum
main complications of Marfan’s?
aortic aneurysm, cardiac myopathy
what is ehlers- dances syndrome?
heterogenous condition often autosomal dominant of abnormal elastin and collagen formation
s/s of Ehlers-danlos?
profound jt jypermobility,vascular fragility, ease of bruising, jt instability, scoliosis
what is cerebral palsy?
umbrella term for disorders that are apparent at birth that are characterised by non-progressive motor deficits
epi of cerebral palsy?
onset before 2-3yo
aetiology of cerebral palsy?
1/10 due to problems during labour- prematurity, hypoxia, intrauterine infections, cerebral insults, neonatal meningitis
s/s of cerebral palsy?
learning difficulties, failure to achieve developmental milestones, motor issues develop later- typically spastic displegia
what 2 other conditions are often associated with cerebral palsy?
autism and ADHD
what is scoliosis?
sideways curvature of spine
epi of scoliosis?
3% of adolescents, F>M
aetiology of scoliosis
widely unknown, hereditary
- Heuter-Volkmann’s Law
- triangular spine (growth spurt)
- trunk rotation while walking
what is Heuter-Volkmann’s Law
inc pressure across epiphyseal plate
3 main groups of scoliosis causes?
congenital, neuromuscular, myopathic (Deuchene Muscular Dystrophy)
ix for scoliosis
x-ray and forward bend test
mx for scoliosis
bracing, surgery
criteria for surgical intervention in scoliosis?
> 45º, thoracic/ lumbar spine, high chance of curve progression, cosmetically unacceptable, cerebral palsy/ spina bifida, breathing difficulties
risk factors for curve progression in scoliosis
pre-menarche, growth spurt, angle >30º, right thoracic curve in girls
what are 3 other spinal defects in kids?
kyphosis: outward curve of spine
spondylosis: stress fracture of isthmus of vertebral arch
spondylolisthesis: displacement of vertebra due to spondylosis
what is developmental dysplasia (DDH)
ball and socket jt of hip doesn’t form properly
what does DDH result in?
dislocation and sublimation of hip
epi of DDH?
8:1 F:M, 1st born, left hip
types of DDH?
early <3 months
late onset >3months
aetiology of DDH?
fhx, pregnancy- breech position, moulded baby, >4kg, twins, Down’s Syndrome
what is the usually pathophysiology of DDH?
socket is too shallow for femoral head
s/s of DDH?
fairly asymptomatic- mothers concerns, clicking hip