CORTEXT Paediatric Orthopaedics Flashcards
1
Q
other names for osteogenesis imperfecta?
A
brittle bone disease
2
Q
is osteogenesis imperfecta congenital or not?
A
congenital
3
Q
what causes osteogenesis imperfecta
A
defect of the maturation and organisation of type 1 collagen
4
Q
what type of inheritance is brittle bone disease most commonly?
A
autosomal dominant
5
Q
clinical presentation of osteogenesis imperfecta?
A
short stature fragility fractures in childhood deformities blue sclerae loss of hearing learning difficulties
6
Q
what do the bones of someone with osteogenesis imperfecta look like?
A
thin
thin cortices
osteopenic
fractures healed by callus
7
Q
medical term for short stature?
A
skeletal dysplasia
8
Q
what does disproportionate skeletal dysplasia mean?
A
short in stature with limbs proportionally shorter or longer than the spine
9
Q
most common type of skeletal dysplasia?
A
achondroplasia
10
Q
clinical presentation of achondroplasia?
A
disproportionately short limbs
prominent forehead
widened nose
11
Q
main cause of connective tissue disorders?
A
genetic disorder of collagen synthesis affecting soft tissue
12
Q
are hypermobile people more or less likely to get soft tissue injuries and dislocations?
A
yes
13
Q
is marfans autosomal dominant or recessive?
A
dominant
14
Q
clinical features of marfans
A
disproportionately long limbs ligamentous laxity scoliosis pectus excavatum eye problems aortic aneurysm high arched palate
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
16
Q
MSK features of down syndrome?
A
short stature
joint laxity
recurrent dislocation
17
Q
how are muscular dystrophies typically inherited?
A
x linked recessive
18
Q
what is significant about the route of inheritance of muscular dystrophies?
A
only affects boys as it is x linked recessive
19
Q
clinical features of muscular dystrophies?
A
progressive muscle weakness and wasting
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
21
Q
how is the diagnosis of duchenne muscular dystrophy found?
A
serum creatine phosphokinase
abnormalities on muscle biopsy
22
Q
usual onset of cerebral palsy?
A
2-3 years old
23
Q
causes of cerebral palsy?
A
genetic problems brain malformation intrauterine infection in early pregnancy prematurity intracranial haemorrhage HYPOXIA AT BIRTH meningitis
24
Q
which births have a higher incidence of brachial plexus injury?
A
twins
large babies
shoulder dystocia
25
most common type of brachial plexus palsy?
erb's palsy
26
what nerve roots are injured in erb's palsy?
C5 and 6
27
what nerves would lose their motor innervation if C5 and 6 nerve roots were damaged?
```
deltoid
supraspinatus
infraspinatus
biceps
brachialis
```
28
why is physio required in erb's palsy?
to prevent contractures early on
29
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
30
what nerve roots of the brachial plexus are affected in klumpke's palsy?
C8 and T1 roots
31
what causes klumpke's palsy?
forceful adduction causing paralysis of the intrinsic hand muscles +/- finger and wrist flexors
32
are the fingers typically extended or flexed in klumpke's palsy?
flexed
33
do children at birth typically have varus or valgus knees?
varus
34
do young children typically have varus or valgus knees?
valgus
35
what is the physiologic valgus angle for an adult?
6 degrees
36
what does a varus knee look like
bow legs
37
what does a valgus knee look like
knock knees
38
test for flat feet?
dorsiflexion of the big toe - pushing your toe up with the feet on the ground
39
what is DDH?
dislocation or subluxation of the femoral head during the perinatal period which affects development of the hip joint
40
which gender is more prone to DDH?
girls
41
which hip is DH more common in?
left
42
why can you get early arthritis in an untreated DDH?
a false acetabulum forms over the original which decreases joint space
43
what should be done in patients with a positive barlow or ortolani test?
ultrasound to detect dislocated/unstable/displaced hip
44
why can an x ray not be used for early DDH diagnosis
femoral head epiphysis is unossified until around 4-6 months
45
most common cause for transient synovitis of the hip?
viral URTI comes first
46
typical age for transient synovitis of hip?
2-10
47
which gender is more commonly affected from transient synovitis of the hip?
boys
48
most common cause of hip pain in children
transient synovitis of hip
49
how could you exclude transient synovitis from septic arthritis
if CRP is normal -> TS
50
how could you exclude transient synovitis from perthes?
radiographs
51
treatment for transient synovitis
short course of NSAIDs
| rest
52
what conditions can predispose to SUFE?
hypothyroidism
| renal disease
53
treatment for patellar tendonitis?
rest
| physio
54
another word for talipes equinovarus?
clubfoot
55
what causes clubfoot?
abnormal alignment of the calcaneal, navicular and talar joints
56
what gender is clubfoot most common in
boys
57
why do clubfoot patients typiclly have ankle equinus?
contractures of the soft tissues cause plantarflexion (ankle equinus)
58
treatment for ankle equinus
early splintage by ponseti technique
59
treatment for skeletal dysplasia
supportive to prevent spinal cord and nerve compression, joint instability, deformity
60
is achondroplasia autosomal dominant or recessive?
dominant
61
where does connective tissue originate from embryologically?
mesoderm
62
main connective tissue disorders?
marfan's
| ehlers-danlos syndrome
63
most abundant protein in body?
collagen
64
what is the only cartilage that contains type 2 collagen?
hyaline cartilage
65
why do marfan's patients have hypermobility?
overproduction of fibrillin
66
do children look normal at birth in duchenne muscular dystrophy?
yes
67
what causes duchenne muscular dystrophy
defect in dystrophin gene which is essential for calcium transport
68
do children with duchenne muscular dystrophy have a raised or normal CK?
raised
69
what does equinus mean
ankle plantarflexion
70
risk factors for talipes equinovarus?
FH
breech presentation
low amniotic fluid volume
71
what is the ponseti technique?
casting for 9 weeks
boots and bar 23hrs pd for 3 months
night time up to 4yrs
72
what investigation should you do if you suspect muscular dystrophy?
muscle biopsy
73
what is syndactly?
failure of digits to separate?
74
what age would surgical separation be done?
3 or 4
75
what tendon commonly needs divided in talipes equinovarus?
achilles
76
what are hypoplastic bones?
short bones
77
what is polydactyly?
extra digit
78
what is fibular hemimelia?
partial or complete absence of the fibula
79
associated symptoms of fibular hemimelia?
shortening of the leg
bowing of the tibia
deformity of ankle
80
treatment for mild cases of fibular hemimelia?
limb lengthening with circular frame external fixator
81
treatment for severe cases of fibular hemimelia?
amputation at 10 months tot 2yrs
82
what causes radial club hand?
absence of radius
83
what is macrosomia?
big baby in diabetic mum
84
most common type of cerebral palsy?
spastic
85
what part of the brain is affected in spastic cerebral palsy?
motor cortex
86
what part of the brain is affected in ataxic cerebral palsy?
cerebellum
87
what part of the brain is affected in athetoid cerebral palsy?
basal ganglia
88
which form of cerebral palsy causes repetitive movements?
dystonic
89
which form of cerebral palsy causes uncontrolled writhing?
athetoid
90
what part of the brain is affected in dystonic cerebral palsy?
basal ganglia
91
what is the distribution of cerebral palsy in an ipsilateral arm and leg called?
hemiplegic
92
what is the distrubution of cerebral palsy in the legs only called?
diplegic
93
treatment for cerebral palsy?
```
hip dislocation by THR/osteotomy
baclofen
botox
rhizotomy
surgical release of joint contractures
```
94
cause of spina bifida?
failure of fusion of posterior vertebral arch
95
what is the milder case of spina bifida called?
spina bifida occulta
96
what does the affected area look like in spina bifida occulta?
birth mark
skin tag
lipoma
hairy patch
97
associated symptoms of spina bifida occulta?
bladder/bowel problems
pes cavus
toe clawing
98
what is the severe form of spina bifida called?
spina bifida cystica
99
what herniates in spina bifida cystica?
meninges (meningocele) or roots of cauda equina (myelomeningocele)
100
which herniation brings the most neurological deficit?
myelomeningocele aka roots of cauda equina
101
associated symptoms of spina bifida?
hydrocephalus
102
what is hydrocephalus?
build up of cerebrospinal fluid in brain
103
treatment for hydrocephalus?
shunt
104
treatment for spinal bifida?
early closure within 48hrs birth
scoliosis correction
hip procedure
105
what is polio?
viral infection of anterior horn cells in spinal cord or brainstem
106
effect of lower motor nerve deficit?
weaknes
reduced tone
loss of reflexes
107
how does polio enter the body?
GI tract
| flu like illness
108
clinical presentation
paralysis of group of muscles or one or more limbs within 2-3 days of contracting it
109
treatment for polio?
splint/brace
tendon transfer
joint fusion
leg lengthening
