paediatric orthopaedics Flashcards
(141 cards)
1
Q
what causes osteogenesis imperfecta
A
a defect in the maturation and organisation of type 1 collagen
2
Q
what type of genetic transmission is OI
A
autosomal dominant
3
Q
how does OI present
A
multiple fragility fractures in childhood
short stature with multile deformities
blue sclera
loss of hearing
4
Q
osteogenesis imperfecta differential diagnoses
A
NAI
osteopaenia
5
Q
how do bones appear on xray in OI
A
thin, with thin cortices and osteopaenic
6
Q
skeletal dysplasia is the medical term for
A
short stature
7
Q
characteristics of achondroplasia
A
disproportionately short limbs
prominent forehead
widened nose
lax joints
normal mental development
8
Q
skeletal dysplasias are associated with
A
learning difficulties
spine deformity
limb deformity
internal organ dysfunction
craniofacial abnormalities
etc
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)
10
Q
marfan’s is autosomal _________
A
dominant
11
Q
marfan’s is due to a defect in….
A
the fibrillin gene
12
Q
marfan’s presenation
A
tall stature
disproportionately long limbs
ligamentous laxity
high arched palate
scoliosis
flattening of chest
eye problems
13
Q
common cause of premature death in marfan’s
A
cardiac abnormalties
(aortic aneurysm, cardiac valve incompetence)
14
Q
what causes Ehlers-Danlos syndrome
A
abnormal elastin and collagen formation
15
Q
clinical features of ehlers-danlos syndrome
A
profound joint hypermobility
vascular fragility with ease of bruising
joint instability
scoliosis
16
Q
complications of surgery in ehlers-danlos syndrome
A
bleeding
poor skin healing
wound dehiscence
17
Q
down syndrome is associated with…
A
short stature
joint laxity
antlanto-axial instability
18
Q
muscular dystropy inheritance is normally
A
x-linked recessive (only affects boys)
19
Q
duchenne muscular dystrophy presentation
A
progressive muscle wasting and weakness
20
Q
what causes DMD
A
a defect in the dystrophin gene involving calcium transport
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
22
Q
DMD diagnosis
A
raised serum creatine phosphokinase
abnormalities on muscle biopsy
23
Q
management of DMD
A
physiotherapy
splintage
deformitiy correction
24
Q
A
gower’s sign (DMD)
25
cerebral palsy is due to
an insult to the immature brain before, during or after birth
26
causes of CP
genetic problems
brain malformation
intrauterine infection in early pregnancy
prematurity
intracranial haemorrhage
hypoxia during birth
meningitis
27
is cerebral palsy often caused by problems during labour?
no, only 1/10 cases are due to labour problems
28
extra bones, absent bones, short bones, and fusions of bones are all exmaples of
limb malformations
29
the commonest limb malformation is
syndactyly
30
what is syndactyly
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
31
what is polydactyly
an extra digit
32
what is fibular hemimelia
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
33
absence or hypoplasia of the radius leads to underdevelopment of the hand, causing what type of deformity
club hand
34
obstetric brachial plexus palsy is most common in small babies
true/false
false - more common in big babies
35
shoulder dystocia (difficult delivery of the shoulder after the head with comression of the shoulder on pubic symphysis) may lead to
obstetric brachial plexus palsy
36
erb's palsy affects which roots of the brachial plexus
C5 and C6
37
erb's palsy results in loss of innervation to
deltoid, supraspinatus, infraspinatus, biceps and brachialis muscles
38
erb's palsy presentation
internal rotation of the humerus (due to unopposed subscapularis)
39
klumpke's palsy affects which nerve roots
C8 and T1
40
klumpke's palsy results in
paralysis of the intrinsic hand muscles +/- finger and wrist flexors and possible horner's syndrome
41
klumpke's palsy presentation
fingers are flexed up
42
sit alone/crawl
6-9 months
43
stands (milestones)
8-12 months
44
walks (milestones)
14-17 months
45
jumps (milestones)
24 months
46
climbs stairs independently (milestones)
3 years
47
loss of primitive reflexes
1-6 months
48
head control (milestones)
2 months
49
speaking a few words (milestones)
9-12 months
50
51
eats with fingers, uses spoon (milestones)
14 months
52
stacks 4 blocks (milestones)
18 months
53
understands 200 words, learns arounf 10 words a day (milestones)
18-20 months
54
potty trained (milestones)
2-3 years
55
pathological varus or valgus is wehre alignement is...
considered outside the normal range (+/- 6 degrees of mean)
56
a valgus deformity results in...
a knock kneed appearance with a larger than normal gap between the ankles
57
a varus deformity will result in...
a bow-legged appearance
a larger than average gap between the knees
58
most cases of valgus/varum deformity will resolve by age
10
59
in-toeing refers to a child who...
when walking and standing will have feet that point toward the midline
60
in-toeing can be caused by
femoral neck anteversion
internal tibial torsion
forefoot adduction
61
excess femoral neck anteversion can give the appearance of
in-toeing
knock knees
62
mobile/flexible flat feet are defined as
the medial arch forms with dorsiflexion of the great toe (Jack test)
63
rigid flat footedness is defined as
a flat arch regardless of load or great toe dorsiflexion
64
what does rigid flat foot imply?
an underlying bony abnormality (eg tarsal coalition)
underlying inflammatory or neurological disorder
65
what causes developmental dysplasia of the hip?
dislocation or subluxation of the femoral head during the perinatal period which affects the subsequent development of the hip joint
66
boys are more commonly affected by DDH
true/false
false - girls are more commonly affected
67
which hip is DDH more common in?
left hip
68
risk factors for DDH
family history
breech presentation
first born babies
down's syndrome
other congenital disorders
69
complications of DDH
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
signs of DDH
shortening
asymmetric groin/thigh skin creases
click or clunk on the otolani or barlow manoeuvres
71
what is the ortolani test
reducing a dislocated hip with abduction and anterior displacement
(postive in DDH)
72
what is the barlow test
dislocatable hip with flexion and posterior displacement
(postivie in DDH)
73
why should xrays not be used in the investigation of DDH before the age of 4-6 months
the femoral head epiphysis is unossified until 4-6 months
use USS before this age
74
persistent dislocation over the age of 18 months is likely to require
open reduction
75
transient synovitis of the hip is
a self-limiting inflammation of the synovium of the hip joint
76
risk factor for transient synovitis
upper respiratory tract infection
77
what ages does transient synovitis affect
ages 2-10
78
transient synovitis affects girls more commonly than boys
true/false
false - boys are more commonly affected
79
what is the commonest cause of hip pain in children
transient synovitis
80
transient synovitis presentation
restricted range of movement
may have a low grade fever
not systemically unwell or septic
81
investigation of transient synovitis
xray to eclude perthes
CRP to exclude septic arthritis
MRI to exclude osteomyelitis
82
transient synovitis treatment
NSAIDs and rest
83
what is perthes disease
idiopathic osteochondritis of the femoral head
84
perthes disease most commonly affects boys
true/false
true
85
perthes disease is more common in active children of short stature
true/false
true
86
perthes disease pathophysiology
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
an incongruent joint in perthes disease can lead to
early onset arthritis
88
perthes disease presentation
pain and a limp
usually unilateral
89
clinical signs of perthes disease
loss of internal rotation, then
loss of abduction, then
positive Trendellenburg test
90
treatment of perthes disease
no specific treatment
regular xray observation
avoidance of physical activity
91
subluxation of the femoral head in perthes disease may require
osteotomy of the femur or acetabulum
92
SUFE commonly affects
overweight prepubertal adolescent boys
93
what is a SUFE
the femoral head epiphysis slips inferiorly in relation to the femoral neck
94
risk factors for SUFE
hypothyroidism
renal disease
95
SUFE presentation
pain and a limp
patients can present purely with pain in the knee
96
why can pain from SUFE be felt in the knee
the obturator nerve supplies both the hip and knee joint
97
SUFE treatment
urgent surgery to pin the femoral head to prevent further slippage
98
severe slips in SUFE have a greater risk of
avascular necrosis
99
why is knee extensor mechanism pain a fairly common occurence during adolescence?
body weight increases and sporting activites increase
100
patellar tendonitis is usually self limiting
true/false
true
101
what is osgood-schlatter's disease
inflammation of the tibial tubercle apophysis
(apohpysitis is inflammation of a grwoing tubercle where a tendon attaches)
102
what is osteochondritis dessicans?
a fragment of hyaline cartilage with variable amount of bone fragments breaks off the surface of the joint
103
which joint is most commonly affected by osteochondritis dessicans?
the knee
104
which bony feature is most commonly affected by osteochondritis dessicans?
medial femoral condyle
105
osteochondritis dessicans presentation
poorly localised pain, effusion and occasionally locking
106
how can you tell a lesion is at risk of breaking off in osteochondritis dessicans
fluid signal behind it on MRI
107
which type of meniscal tears may benifit from meniscal repair?
peripheral or bucket handle
108
what is talipes equinovarus also known as
club foot
109
what causes talipes equinovarus
in utero abnormal alignment of the joints between the talus, calcaneus and navicular
110
talipes equinovarus presentation
ankle equinovarus (plantarflexion), supination of the forefoot and varus alignment of the forefoot
111
risk factors for talipes equinovarus
male gender
family history
breech presentation
oligohydramnios (low amniotic fluid content)
112
talipes equinovaurs treatment
early splintage
(ponseti technique)
may require tenotomy of the achiles tendon
113
114
what is tarsal coalition
an abnormal bridge, of bony, fibrous or cartilaginous tissue, is formed between the calcaneus and navicular or between the talus and calcaneus
115
what can tarsal coalition cause
a painful fixed flat foot
116
what is scoliosis
a lateral curvature of the spine (and also rotational deformity)
117
causes of scoliosis
idiopathic
neuromuscular disease
tumour
skeletal dysplasia
infection
118
idiopathic scoliosis is more common in male/females
females
119
indications for scoliosis surgery
pain
cosmesis
improve wheelchair posture
120
respiratory complication of severe curves in scoliosis
restrictive lung defect
121
what is spondylolisthesis
slippage of one vertabra over another
122
where does sponylolisthesis usually occur
L4/5 or L5/S1 level
123
causes of spondylolisthesis
developmental defect
recurrent stress fracture
124
spondylolisthesis presentation
low back pain
radiculopathy
waddlig gait/flat back
125
spondylolisthesis treatment
minor - rest and physio
major - stabilisation and reduction
126
which nerve roots are damaged in Erb's palsy?
C5 and C6
127
what is the investigation of choice is DDH is suspected after postnatal checks?
ultrasound
128
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
gross shortening of the limb
gross shortening would be more suggestive of a chronic condition, such as a missed DDH
129
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?
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
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
131
progresseive muscular weakness is the primary feautre of duchenne's muscular dystrophy
true/false
true
132
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?
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
osteogenesis imperfect is a genetic disorder with both autosomal dominant and recessive models of inheritance
true/false
true
134
osgood-schlatter's disease is usually a self-lmiting apophysitis and usually settles with time given rest
true/false
true
135
around the age of 3, children commonly have a valgus alignment of around 10-15 degrees
true/false
136
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
137
the most common cause of paediatric hip pain is
transient synovitis
138
\_\_\_\_\_\_\_\_ 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
marfan's syndrome
139
the age at time of diagnosis of perthes disease of the hip is inversely proportional to the prognosis
true/false
true
140
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?
osgood-schlatter's disease
141
