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cortext > paediatric orthopaedics > Flashcards

paediatric orthopaedics Flashcards

1
Q

what causes osteogenesis imperfecta

A

a defect in the maturation and organisation of type 1 collagen

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2
Q

what type of genetic transmission is OI

A

autosomal dominant

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3
Q

how does OI present

A

multiple fragility fractures in childhood

short stature with multile deformities

blue sclera

loss of hearing

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4
Q

osteogenesis imperfecta differential diagnoses

A

NAI

osteopaenia

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5
Q

how do bones appear on xray in OI

A

thin, with thin cortices and osteopaenic

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6
Q

skeletal dysplasia is the medical term for

A

short stature

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7
Q

characteristics of achondroplasia

A

disproportionately short limbs

prominent forehead

widened nose

lax joints

normal mental development

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8
Q

skeletal dysplasias are associated with

A

learning difficulties

spine deformity

limb deformity

internal organ dysfunction

craniofacial abnormalities

etc

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9
Q

people with generalised ligamentous laxity are more prone to

A

soft tissue injuries (eg ankle sprains)

recurrent dislocations of joints (especially shoulder and patella)

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10
Q

marfan’s is autosomal _________

A

dominant

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11
Q

marfan’s is due to a defect in….

A

the fibrillin gene

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12
Q

marfan’s presenation

A

tall stature

disproportionately long limbs

ligamentous laxity

high arched palate

scoliosis

flattening of chest

eye problems

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13
Q

common cause of premature death in marfan’s

A

cardiac abnormalties

(aortic aneurysm, cardiac valve incompetence)

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14
Q

what causes Ehlers-Danlos syndrome

A

abnormal elastin and collagen formation

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15
Q

clinical features of ehlers-danlos syndrome

A

profound joint hypermobility

vascular fragility with ease of bruising

joint instability

scoliosis

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16
Q

complications of surgery in ehlers-danlos syndrome

A

bleeding

poor skin healing

wound dehiscence

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17
Q

down syndrome is associated with…

A

short stature

joint laxity

antlanto-axial instability

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18
Q

muscular dystropy inheritance is normally

A

x-linked recessive (only affects boys)

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19
Q

duchenne muscular dystrophy presentation

A

progressive muscle wasting and weakness

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20
Q

what causes DMD

A

a defect in the dystrophin gene involving calcium transport

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21
Q

DMD prognosis

A

unable to walk at 10 years old

progressive cardiac and respiratory failure by 20 years old

death typically in early 20s

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22
Q

DMD diagnosis

A

raised serum creatine phosphokinase

abnormalities on muscle biopsy

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23
Q

management of DMD

A

physiotherapy

splintage

deformitiy correction

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24
Q
A

gower’s sign (DMD)

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25
Q

cerebral palsy is due to

A

an insult to the immature brain before, during or after birth

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26
Q

causes of CP

A

genetic problems

brain malformation

intrauterine infection in early pregnancy

prematurity

intracranial haemorrhage

hypoxia during birth

meningitis

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27
Q

is cerebral palsy often caused by problems during labour?

A

no, only 1/10 cases are due to labour problems

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28
Q

extra bones, absent bones, short bones, and fusions of bones are all exmaples of

A

limb malformations

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29
Q

the commonest limb malformation is

A

syndactyly

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30
Q

what is syndactyly

A

two digits are fused due to failure of separation of the skin/soft tissues or phalanges of adjacent digits either partially of along the full length of the digit

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31
Q

what is polydactyly

A

an extra digit

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32
Q

what is fibular hemimelia

A

partial or complete absence of the fibula often with absence of the lateral foot rays leading to a shortened limb, bowing of the tibia and ankle deformity

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33
Q

absence or hypoplasia of the radius leads to underdevelopment of the hand, causing what type of deformity

A

club hand

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34
Q

obstetric brachial plexus palsy is most common in small babies

true/false

A

false - more common in big babies

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35
Q

shoulder dystocia (difficult delivery of the shoulder after the head with comression of the shoulder on pubic symphysis) may lead to

A

obstetric brachial plexus palsy

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36
Q

erb’s palsy affects which roots of the brachial plexus

A

C5 and C6

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37
Q

erb’s palsy results in loss of innervation to

A

deltoid, supraspinatus, infraspinatus, biceps and brachialis muscles

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38
Q

erb’s palsy presentation

A

internal rotation of the humerus (due to unopposed subscapularis)

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39
Q

klumpke’s palsy affects which nerve roots

A

C8 and T1

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40
Q

klumpke’s palsy results in

A

paralysis of the intrinsic hand muscles +/- finger and wrist flexors and possible horner’s syndrome

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41
Q

klumpke’s palsy presentation

A

fingers are flexed up

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42
Q

sit alone/crawl

A

6-9 months

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43
Q

stands (milestones)

A

8-12 months

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44
Q

walks (milestones)

A

14-17 months

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45
Q

jumps (milestones)

A

24 months

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46
Q

climbs stairs independently (milestones)

A

3 years

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47
Q

loss of primitive reflexes

A

1-6 months

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48
Q

head control (milestones)

A

2 months

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49
Q

speaking a few words (milestones)

A

9-12 months

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50
Q
A
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51
Q

eats with fingers, uses spoon (milestones)

A

14 months

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52
Q

stacks 4 blocks (milestones)

A

18 months

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53
Q

understands 200 words, learns arounf 10 words a day (milestones)

A

18-20 months

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54
Q

potty trained (milestones)

A

2-3 years

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55
Q

pathological varus or valgus is wehre alignement is…

A

considered outside the normal range (+/- 6 degrees of mean)

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56
Q

a valgus deformity results in…

A

a knock kneed appearance with a larger than normal gap between the ankles

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57
Q

a varus deformity will result in…

A

a bow-legged appearance

a larger than average gap between the knees

58
Q

most cases of valgus/varum deformity will resolve by age

A

10

59
Q

in-toeing refers to a child who…

A

when walking and standing will have feet that point toward the midline

60
Q

in-toeing can be caused by

A

femoral neck anteversion

internal tibial torsion

forefoot adduction

61
Q

excess femoral neck anteversion can give the appearance of

A

in-toeing

knock knees

62
Q

mobile/flexible flat feet are defined as

A

the medial arch forms with dorsiflexion of the great toe (Jack test)

63
Q

rigid flat footedness is defined as

A

a flat arch regardless of load or great toe dorsiflexion

64
Q

what does rigid flat foot imply?

A

an underlying bony abnormality (eg tarsal coalition)

underlying inflammatory or neurological disorder

65
Q

what causes developmental dysplasia of the hip?

A

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

66
Q

boys are more commonly affected by DDH

true/false

A

false - girls are more commonly affected

67
Q

which hip is DDH more common in?

A

left hip

68
Q

risk factors for DDH

A

family history

breech presentation

first born babies

down’s syndrome

other congenital disorders

69
Q

complications of DDH

A

if untreated a false acetabulum can form leading to a shortened limb

severe arthritis may occur at a young age

gait/mobility can be reduced

70
Q

signs of DDH

A

shortening

asymmetric groin/thigh skin creases

click or clunk on the otolani or barlow manoeuvres

71
Q

what is the ortolani test

A

reducing a dislocated hip with abduction and anterior displacement

(postive in DDH)

72
Q

what is the barlow test

A

dislocatable hip with flexion and posterior displacement

(postivie in DDH)

73
Q

why should xrays not be used in the investigation of DDH before the age of 4-6 months

A

the femoral head epiphysis is unossified until 4-6 months

use USS before this age

74
Q

persistent dislocation over the age of 18 months is likely to require

A

open reduction

75
Q

transient synovitis of the hip is

A

a self-limiting inflammation of the synovium of the hip joint

76
Q

risk factor for transient synovitis

A

upper respiratory tract infection

77
Q

what ages does transient synovitis affect

A

ages 2-10

78
Q

transient synovitis affects girls more commonly than boys

true/false

A

false - boys are more commonly affected

79
Q

what is the commonest cause of hip pain in children

A

transient synovitis

80
Q

transient synovitis presentation

A

restricted range of movement

may have a low grade fever

not systemically unwell or septic

81
Q

investigation of transient synovitis

A

xray to eclude perthes

CRP to exclude septic arthritis

MRI to exclude osteomyelitis

82
Q

transient synovitis treatment

A

NSAIDs and rest

83
Q

what is perthes disease

A

idiopathic osteochondritis of the femoral head

84
Q

perthes disease most commonly affects boys

true/false

A

true

85
Q

perthes disease is more common in active children of short stature

true/false

A

true

86
Q

perthes disease pathophysiology

A

the femoral head transiently loses its blood supply resulting in necrosis with subsequent abnormal growth

the femoral head may collapse

subsequent remodelling occurs, with the femoral head not fitting the acetabulum properly

87
Q

an incongruent joint in perthes disease can lead to

A

early onset arthritis

88
Q

perthes disease presentation

A

pain and a limp

usually unilateral

89
Q

clinical signs of perthes disease

A

loss of internal rotation, then

loss of abduction, then

positive Trendellenburg test

90
Q

treatment of perthes disease

A

no specific treatment

regular xray observation

avoidance of physical activity

91
Q

subluxation of the femoral head in perthes disease may require

A

osteotomy of the femur or acetabulum

92
Q

SUFE commonly affects

A

overweight prepubertal adolescent boys

93
Q

what is a SUFE

A

the femoral head epiphysis slips inferiorly in relation to the femoral neck

94
Q

risk factors for SUFE

A

hypothyroidism

renal disease

95
Q

SUFE presentation

A

pain and a limp

patients can present purely with pain in the knee

96
Q

why can pain from SUFE be felt in the knee

A

the obturator nerve supplies both the hip and knee joint

97
Q

SUFE treatment

A

urgent surgery to pin the femoral head to prevent further slippage

98
Q

severe slips in SUFE have a greater risk of

A

avascular necrosis

99
Q

why is knee extensor mechanism pain a fairly common occurence during adolescence?

A

body weight increases and sporting activites increase

100
Q

patellar tendonitis is usually self limiting

true/false

A

true

101
Q

what is osgood-schlatter’s disease

A

inflammation of the tibial tubercle apophysis

(apohpysitis is inflammation of a grwoing tubercle where a tendon attaches)

102
Q

what is osteochondritis dessicans?

A

a fragment of hyaline cartilage with variable amount of bone fragments breaks off the surface of the joint

103
Q

which joint is most commonly affected by osteochondritis dessicans?

A

the knee

104
Q

which bony feature is most commonly affected by osteochondritis dessicans?

A

medial femoral condyle

105
Q

osteochondritis dessicans presentation

A

poorly localised pain, effusion and occasionally locking

106
Q

how can you tell a lesion is at risk of breaking off in osteochondritis dessicans

A

fluid signal behind it on MRI

107
Q

which type of meniscal tears may benifit from meniscal repair?

A

peripheral or bucket handle

108
Q

what is talipes equinovarus also known as

A

club foot

109
Q

what causes talipes equinovarus

A

in utero abnormal alignment of the joints between the talus, calcaneus and navicular

110
Q

talipes equinovarus presentation

A

ankle equinovarus (plantarflexion), supination of the forefoot and varus alignment of the forefoot

111
Q

risk factors for talipes equinovarus

A

male gender

family history

breech presentation

oligohydramnios (low amniotic fluid content)

112
Q

talipes equinovaurs treatment

A

early splintage

(ponseti technique)

may require tenotomy of the achiles tendon

113
Q
A
114
Q

what is tarsal coalition

A

an abnormal bridge, of bony, fibrous or cartilaginous tissue, is formed between the calcaneus and navicular or between the talus and calcaneus

115
Q

what can tarsal coalition cause

A

a painful fixed flat foot

116
Q

what is scoliosis

A

a lateral curvature of the spine (and also rotational deformity)

117
Q

causes of scoliosis

A

idiopathic

neuromuscular disease

tumour

skeletal dysplasia

infection

118
Q

idiopathic scoliosis is more common in male/females

A

females

119
Q

indications for scoliosis surgery

A

pain

cosmesis

improve wheelchair posture

120
Q

respiratory complication of severe curves in scoliosis

A

restrictive lung defect

121
Q

what is spondylolisthesis

A

slippage of one vertabra over another

122
Q

where does sponylolisthesis usually occur

A

L4/5 or L5/S1 level

123
Q

causes of spondylolisthesis

A

developmental defect

recurrent stress fracture

124
Q

spondylolisthesis presentation

A

low back pain

radiculopathy

waddlig gait/flat back

125
Q

spondylolisthesis treatment

A

minor - rest and physio

major - stabilisation and reduction

126
Q

which nerve roots are damaged in Erb’s palsy?

A

C5 and C6

127
Q

what is the investigation of choice is DDH is suspected after postnatal checks?

A

ultrasound

128
Q

which of the following is not a typical feature of SUFE?

limp

pain in the hip (groin)

pain in the knee

gross shortening of the limb

loss of internal rotation

A

gross shortening of the limb

gross shortening would be more suggestive of a chronic condition, such as a missed DDH

129
Q

a 13 year old girl presents with new and progressive back pain and a change in the curvature of her spine

on examination she has a scoliosis with the curve concave to the right

what should you do?

A

refer to the local paediatric or orthopaeidc unit for review

back pain in children is a red flag, so have a low threshold for referral/investigation

130
Q

which of the following is not true in relation to perthes disease?

it is associated with tall stature

loos of internal rotation is often the earliest clinical sign

it is most common in boys aged 4-9

most cases are unilateral

A
131
Q

progresseive muscular weakness is the primary feautre of duchenne’s muscular dystrophy

true/false

A

true

132
Q

a 13 year old girl presents with 2 month history of new and progressive back pain in the lumbar region

there is no distinct point of tenderness or loss of flexibilty, but hte back appears to have lost its normal lordosis and looks quite ‘flat’

what now?

A

refer to the local paediatric or orthopaedic unit for review

back pain in children is always a red flag

this sounds like spondylolisthesis so may need urgent surgical repair

133
Q

osteogenesis imperfect is a genetic disorder with both autosomal dominant and recessive models of inheritance

true/false

A

true

134
Q

osgood-schlatter’s disease is usually a self-lmiting apophysitis and usually settles with time given rest

true/false

A

true

135
Q

around the age of 3, children commonly have a valgus alignment of around 10-15 degrees

true/false

A
136
Q

which of the following increase the risk of DDH

female sex

2nd baby

breech presentation

spina bifida

down syndrome

family history of DDH

maternal DM

A
137
Q

the most common cause of paediatric hip pain is

A

transient synovitis

138
Q

________ is an autosomal dominant or sporadic mutation of the fibrillin gene resulting in tall stature with disproportionately long limbs and ligamentous laxity

assocaited features include a high arched palate, scoliosis, pectus excavatum, eye problems (lens disclocation, retinal detachment), aortic aneurysm and cardiac valve incompetence

A

marfan’s syndrome

139
Q

the age at time of diagnosis of perthes disease of the hip is inversely proportional to the prognosis

true/false

A

true

140
Q

a 14 year old boy with pain at the top of both anterior shins

he is tall and thin and has recently grown

he has tender, prominent tibial tuberosities

whats up?

A

osgood-schlatter’s disease

141
Q
A

cortext (6 decks)

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