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2 MSK > CORTEXT Paediatric Orthopaedics > Flashcards

CORTEXT Paediatric Orthopaedics Flashcards

1
Q

other names for osteogenesis imperfecta?

A

brittle bone disease

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2
Q

is osteogenesis imperfecta congenital or not?

A

congenital

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3
Q

what causes osteogenesis imperfecta

A

defect of the maturation and organisation of type 1 collagen

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4
Q

what type of inheritance is brittle bone disease most commonly?

A

autosomal dominant

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5
Q

clinical presentation of osteogenesis imperfecta?

A 
short stature
fragility fractures in childhood
deformities
blue sclerae
loss of hearing
learning difficulties
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6
Q

what do the bones of someone with osteogenesis imperfecta look like?

A

thin
thin cortices
osteopenic
fractures healed by callus

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7
Q

medical term for short stature?

A

skeletal dysplasia

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8
Q

what does disproportionate skeletal dysplasia mean?

A

short in stature with limbs proportionally shorter or longer than the spine

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9
Q

most common type of skeletal dysplasia?

A

achondroplasia

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10
Q

clinical presentation of achondroplasia?

A

disproportionately short limbs
prominent forehead
widened nose

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11
Q

main cause of connective tissue disorders?

A

genetic disorder of collagen synthesis affecting soft tissue

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12
Q

are hypermobile people more or less likely to get soft tissue injuries and dislocations?

A

yes

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13
Q

is marfans autosomal dominant or recessive?

A

dominant

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14
Q

clinical features of marfans

A 
disproportionately long limbs
ligamentous laxity
scoliosis
pectus excavatum
eye problems
aortic aneurysm
high arched palate
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15
Q

clinical features of ehlers danlos syndrome?

A 
joint hypemobility
easy bruising
joint instability
scoliosis
early onset OA
poor wound healing
cardiac abnormalities
scoliosis/kyphosis
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16
Q

MSK features of down syndrome?

A

short stature
joint laxity
recurrent dislocation

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17
Q

how are muscular dystrophies typically inherited?

A

x linked recessive

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18
Q

what is significant about the route of inheritance of muscular dystrophies?

A

only affects boys as it is x linked recessive

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19
Q

clinical features of muscular dystrophies?

A

progressive muscle weakness and wasting

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20
Q

clinical features of duchenne muscular dystrophy?

A 
difficulty standing
progressive muscle weakness
cant walk age 10
age 20 progressive cardiac and resp failure
death in early 20s
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21
Q

how is the diagnosis of duchenne muscular dystrophy found?

A

serum creatine phosphokinase

abnormalities on muscle biopsy

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22
Q

usual onset of cerebral palsy?

A

2-3 years old

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23
Q

causes of cerebral palsy?

A 
genetic problems
brain malformation
intrauterine infection in early pregnancy
prematurity
intracranial haemorrhage
HYPOXIA AT BIRTH
meningitis
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24
Q

which births have a higher incidence of brachial plexus injury?

A

twins
large babies
shoulder dystocia

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25
Q

most common type of brachial plexus palsy?

A

erb’s palsy

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26
Q

what nerve roots are injured in erb’s palsy?

A

C5 and 6

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27
Q

what nerves would lose their motor innervation if C5 and 6 nerve roots were damaged?

A 
deltoid
supraspinatus
infraspinatus
biceps
brachialis
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28
Q

why is physio required in erb’s palsy?

A

to prevent contractures early on

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29
Q

why do children with erb’s palsy adopt a “waiter’s tip” position of the arm?

A

internal rotation of the humerus as the scapularis muscle do not hold it in place

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30
Q

what nerve roots of the brachial plexus are affected in klumpke’s palsy?

A

C8 and T1 roots

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31
Q

what causes klumpke’s palsy?

A

forceful adduction causing paralysis of the intrinsic hand muscles +/- finger and wrist flexors

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32
Q

are the fingers typically extended or flexed in klumpke’s palsy?

A

flexed

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33
Q

do children at birth typically have varus or valgus knees?

A

varus

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34
Q

do young children typically have varus or valgus knees?

A

valgus

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35
Q

what is the physiologic valgus angle for an adult?

A

6 degrees

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36
Q

what does a varus knee look like

A

bow legs

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37
Q

what does a valgus knee look like

A

knock knees

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38
Q

test for flat feet?

A

dorsiflexion of the big toe - pushing your toe up with the feet on the ground

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39
Q

what is DDH?

A

dislocation or subluxation of the femoral head during the perinatal period which affects development of the hip joint

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40
Q

which gender is more prone to DDH?

A

girls

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41
Q

which hip is DH more common in?

A

left

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42
Q

why can you get early arthritis in an untreated DDH?

A

a false acetabulum forms over the original which decreases joint space

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43
Q

what should be done in patients with a positive barlow or ortolani test?

A

ultrasound to detect dislocated/unstable/displaced hip

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44
Q

why can an x ray not be used for early DDH diagnosis

A

femoral head epiphysis is unossified until around 4-6 months

45
Q

most common cause for transient synovitis of the hip?

A

viral URTI comes first

46
Q

typical age for transient synovitis of hip?

A

2-10

47
Q

which gender is more commonly affected from transient synovitis of the hip?

A

boys

48
Q

most common cause of hip pain in children

A

transient synovitis of hip

49
Q

how could you exclude transient synovitis from septic arthritis

A

if CRP is normal -> TS

50
Q

how could you exclude transient synovitis from perthes?

A

radiographs

51
Q

treatment for transient synovitis

A

short course of NSAIDs

rest

52
Q

what conditions can predispose to SUFE?

A

hypothyroidism

renal disease

53
Q

treatment for patellar tendonitis?

A

rest

physio

54
Q

another word for talipes equinovarus?

A

clubfoot

55
Q

what causes clubfoot?

A

abnormal alignment of the calcaneal, navicular and talar joints

56
Q

what gender is clubfoot most common in

A

boys

57
Q

why do clubfoot patients typiclly have ankle equinus?

A

contractures of the soft tissues cause plantarflexion (ankle equinus)

58
Q

treatment for ankle equinus

A

early splintage by ponseti technique

59
Q

treatment for skeletal dysplasia

A

supportive to prevent spinal cord and nerve compression, joint instability, deformity

60
Q

is achondroplasia autosomal dominant or recessive?

A

dominant

61
Q

where does connective tissue originate from embryologically?

A

mesoderm

62
Q

main connective tissue disorders?

A

marfan’s

ehlers-danlos syndrome

63
Q

most abundant protein in body?

A

collagen

64
Q

what is the only cartilage that contains type 2 collagen?

A

hyaline cartilage

65
Q

why do marfan’s patients have hypermobility?

A

overproduction of fibrillin

66
Q

do children look normal at birth in duchenne muscular dystrophy?

A

yes

67
Q

what causes duchenne muscular dystrophy

A

defect in dystrophin gene which is essential for calcium transport

68
Q

do children with duchenne muscular dystrophy have a raised or normal CK?

A

raised

69
Q

what does equinus mean

A

ankle plantarflexion

70
Q

risk factors for talipes equinovarus?

A

FH
breech presentation
low amniotic fluid volume

71
Q

what is the ponseti technique?

A

casting for 9 weeks
boots and bar 23hrs pd for 3 months
night time up to 4yrs

72
Q

what investigation should you do if you suspect muscular dystrophy?

A

muscle biopsy

73
Q

what is syndactly?

A

failure of digits to separate?

74
Q

what age would surgical separation be done?

A

3 or 4

75
Q

what tendon commonly needs divided in talipes equinovarus?

A

achilles

76
Q

what are hypoplastic bones?

A

short bones

77
Q

what is polydactyly?

A

extra digit

78
Q

what is fibular hemimelia?

A

partial or complete absence of the fibula

79
Q

associated symptoms of fibular hemimelia?

A

shortening of the leg
bowing of the tibia
deformity of ankle

80
Q

treatment for mild cases of fibular hemimelia?

A

limb lengthening with circular frame external fixator

81
Q

treatment for severe cases of fibular hemimelia?

A

amputation at 10 months tot 2yrs

82
Q

what causes radial club hand?

A

absence of radius

83
Q

what is macrosomia?

A

big baby in diabetic mum

84
Q

most common type of cerebral palsy?

A

spastic

85
Q

what part of the brain is affected in spastic cerebral palsy?

A

motor cortex

86
Q

what part of the brain is affected in ataxic cerebral palsy?

A

cerebellum

87
Q

what part of the brain is affected in athetoid cerebral palsy?

A

basal ganglia

88
Q

which form of cerebral palsy causes repetitive movements?

A

dystonic

89
Q

which form of cerebral palsy causes uncontrolled writhing?

A

athetoid

90
Q

what part of the brain is affected in dystonic cerebral palsy?

A

basal ganglia

91
Q

what is the distribution of cerebral palsy in an ipsilateral arm and leg called?

A

hemiplegic

92
Q

what is the distrubution of cerebral palsy in the legs only called?

A

diplegic

93
Q

treatment for cerebral palsy?

A 
hip dislocation by THR/osteotomy
baclofen
botox
rhizotomy
surgical release of joint contractures
94
Q

cause of spina bifida?

A

failure of fusion of posterior vertebral arch

95
Q

what is the milder case of spina bifida called?

A

spina bifida occulta

96
Q

what does the affected area look like in spina bifida occulta?

A

birth mark
skin tag
lipoma
hairy patch

97
Q

associated symptoms of spina bifida occulta?

A

bladder/bowel problems
pes cavus
toe clawing

98
Q

what is the severe form of spina bifida called?

A

spina bifida cystica

99
Q

what herniates in spina bifida cystica?

A

meninges (meningocele) or roots of cauda equina (myelomeningocele)

100
Q

which herniation brings the most neurological deficit?

A

myelomeningocele aka roots of cauda equina

101
Q

associated symptoms of spina bifida?

A

hydrocephalus

102
Q

what is hydrocephalus?

A

build up of cerebrospinal fluid in brain

103
Q

treatment for hydrocephalus?

A

shunt

104
Q

treatment for spinal bifida?

A

early closure within 48hrs birth
scoliosis correction
hip procedure

105
Q

what is polio?

A

viral infection of anterior horn cells in spinal cord or brainstem

106
Q

effect of lower motor nerve deficit?

A

weaknes
reduced tone
loss of reflexes

107
Q

how does polio enter the body?

A

GI tract

flu like illness

108
Q

clinical presentation

A

paralysis of group of muscles or one or more limbs within 2-3 days of contracting it

109
Q

treatment for polio?

A

splint/brace
tendon transfer
joint fusion
leg lengthening

2 MSK (61 decks)

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