CORTEXT Paediatric Orthopaedics Flashcards
other names for osteogenesis imperfecta?
brittle bone disease
is osteogenesis imperfecta congenital or not?
congenital
what causes osteogenesis imperfecta
defect of the maturation and organisation of type 1 collagen
what type of inheritance is brittle bone disease most commonly?
autosomal dominant
clinical presentation of osteogenesis imperfecta?
short stature fragility fractures in childhood deformities blue sclerae loss of hearing learning difficulties
what do the bones of someone with osteogenesis imperfecta look like?
thin
thin cortices
osteopenic
fractures healed by callus
medical term for short stature?
skeletal dysplasia
what does disproportionate skeletal dysplasia mean?
short in stature with limbs proportionally shorter or longer than the spine
most common type of skeletal dysplasia?
achondroplasia
clinical presentation of achondroplasia?
disproportionately short limbs
prominent forehead
widened nose
main cause of connective tissue disorders?
genetic disorder of collagen synthesis affecting soft tissue
are hypermobile people more or less likely to get soft tissue injuries and dislocations?
yes
is marfans autosomal dominant or recessive?
dominant
clinical features of marfans
disproportionately long limbs ligamentous laxity scoliosis pectus excavatum eye problems aortic aneurysm high arched palate
clinical features of ehlers danlos syndrome?
joint hypemobility easy bruising joint instability scoliosis early onset OA poor wound healing cardiac abnormalities scoliosis/kyphosis
MSK features of down syndrome?
short stature
joint laxity
recurrent dislocation
how are muscular dystrophies typically inherited?
x linked recessive
what is significant about the route of inheritance of muscular dystrophies?
only affects boys as it is x linked recessive
clinical features of muscular dystrophies?
progressive muscle weakness and wasting
clinical features of duchenne muscular dystrophy?
difficulty standing progressive muscle weakness cant walk age 10 age 20 progressive cardiac and resp failure death in early 20s
how is the diagnosis of duchenne muscular dystrophy found?
serum creatine phosphokinase
abnormalities on muscle biopsy
usual onset of cerebral palsy?
2-3 years old
causes of cerebral palsy?
genetic problems brain malformation intrauterine infection in early pregnancy prematurity intracranial haemorrhage HYPOXIA AT BIRTH meningitis
which births have a higher incidence of brachial plexus injury?
twins
large babies
shoulder dystocia
most common type of brachial plexus palsy?
erb’s palsy
what nerve roots are injured in erb’s palsy?
C5 and 6
what nerves would lose their motor innervation if C5 and 6 nerve roots were damaged?
deltoid supraspinatus infraspinatus biceps brachialis
why is physio required in erb’s palsy?
to prevent contractures early on
why do children with erb’s palsy adopt a “waiter’s tip” position of the arm?
internal rotation of the humerus as the scapularis muscle do not hold it in place
what nerve roots of the brachial plexus are affected in klumpke’s palsy?
C8 and T1 roots
what causes klumpke’s palsy?
forceful adduction causing paralysis of the intrinsic hand muscles +/- finger and wrist flexors
are the fingers typically extended or flexed in klumpke’s palsy?
flexed
do children at birth typically have varus or valgus knees?
varus
do young children typically have varus or valgus knees?
valgus
what is the physiologic valgus angle for an adult?
6 degrees
what does a varus knee look like
bow legs
what does a valgus knee look like
knock knees
test for flat feet?
dorsiflexion of the big toe - pushing your toe up with the feet on the ground
what is DDH?
dislocation or subluxation of the femoral head during the perinatal period which affects development of the hip joint
which gender is more prone to DDH?
girls
which hip is DH more common in?
left
why can you get early arthritis in an untreated DDH?
a false acetabulum forms over the original which decreases joint space
what should be done in patients with a positive barlow or ortolani test?
ultrasound to detect dislocated/unstable/displaced hip
why can an x ray not be used for early DDH diagnosis
femoral head epiphysis is unossified until around 4-6 months
most common cause for transient synovitis of the hip?
viral URTI comes first
typical age for transient synovitis of hip?
2-10
which gender is more commonly affected from transient synovitis of the hip?
boys
most common cause of hip pain in children
transient synovitis of hip
how could you exclude transient synovitis from septic arthritis
if CRP is normal -> TS
how could you exclude transient synovitis from perthes?
radiographs
treatment for transient synovitis
short course of NSAIDs
rest
what conditions can predispose to SUFE?
hypothyroidism
renal disease
treatment for patellar tendonitis?
rest
physio
another word for talipes equinovarus?
clubfoot
what causes clubfoot?
abnormal alignment of the calcaneal, navicular and talar joints
what gender is clubfoot most common in
boys
why do clubfoot patients typiclly have ankle equinus?
contractures of the soft tissues cause plantarflexion (ankle equinus)
treatment for ankle equinus
early splintage by ponseti technique
treatment for skeletal dysplasia
supportive to prevent spinal cord and nerve compression, joint instability, deformity
is achondroplasia autosomal dominant or recessive?
dominant
where does connective tissue originate from embryologically?
mesoderm
main connective tissue disorders?
marfan’s
ehlers-danlos syndrome
most abundant protein in body?
collagen
what is the only cartilage that contains type 2 collagen?
hyaline cartilage
why do marfan’s patients have hypermobility?
overproduction of fibrillin
do children look normal at birth in duchenne muscular dystrophy?
yes
what causes duchenne muscular dystrophy
defect in dystrophin gene which is essential for calcium transport
do children with duchenne muscular dystrophy have a raised or normal CK?
raised
what does equinus mean
ankle plantarflexion
risk factors for talipes equinovarus?
FH
breech presentation
low amniotic fluid volume
what is the ponseti technique?
casting for 9 weeks
boots and bar 23hrs pd for 3 months
night time up to 4yrs
what investigation should you do if you suspect muscular dystrophy?
muscle biopsy
what is syndactly?
failure of digits to separate?
what age would surgical separation be done?
3 or 4
what tendon commonly needs divided in talipes equinovarus?
achilles
what are hypoplastic bones?
short bones
what is polydactyly?
extra digit
what is fibular hemimelia?
partial or complete absence of the fibula
associated symptoms of fibular hemimelia?
shortening of the leg
bowing of the tibia
deformity of ankle
treatment for mild cases of fibular hemimelia?
limb lengthening with circular frame external fixator
treatment for severe cases of fibular hemimelia?
amputation at 10 months tot 2yrs
what causes radial club hand?
absence of radius
what is macrosomia?
big baby in diabetic mum
most common type of cerebral palsy?
spastic
what part of the brain is affected in spastic cerebral palsy?
motor cortex
what part of the brain is affected in ataxic cerebral palsy?
cerebellum
what part of the brain is affected in athetoid cerebral palsy?
basal ganglia
which form of cerebral palsy causes repetitive movements?
dystonic
which form of cerebral palsy causes uncontrolled writhing?
athetoid
what part of the brain is affected in dystonic cerebral palsy?
basal ganglia
what is the distribution of cerebral palsy in an ipsilateral arm and leg called?
hemiplegic
what is the distrubution of cerebral palsy in the legs only called?
diplegic
treatment for cerebral palsy?
hip dislocation by THR/osteotomy baclofen botox rhizotomy surgical release of joint contractures
cause of spina bifida?
failure of fusion of posterior vertebral arch
what is the milder case of spina bifida called?
spina bifida occulta
what does the affected area look like in spina bifida occulta?
birth mark
skin tag
lipoma
hairy patch
associated symptoms of spina bifida occulta?
bladder/bowel problems
pes cavus
toe clawing
what is the severe form of spina bifida called?
spina bifida cystica
what herniates in spina bifida cystica?
meninges (meningocele) or roots of cauda equina (myelomeningocele)
which herniation brings the most neurological deficit?
myelomeningocele aka roots of cauda equina
associated symptoms of spina bifida?
hydrocephalus
what is hydrocephalus?
build up of cerebrospinal fluid in brain
treatment for hydrocephalus?
shunt
treatment for spinal bifida?
early closure within 48hrs birth
scoliosis correction
hip procedure
what is polio?
viral infection of anterior horn cells in spinal cord or brainstem
effect of lower motor nerve deficit?
weaknes
reduced tone
loss of reflexes
how does polio enter the body?
GI tract
flu like illness
clinical presentation
paralysis of group of muscles or one or more limbs within 2-3 days of contracting it
treatment for polio?
splint/brace
tendon transfer
joint fusion
leg lengthening
