Paediatric MSK conditions

Question 1

what is developmental dysplasia of the hip?

Answer 1

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

Question 2

if DDH is left untreated what can occur?

Answer 2

shallow acetabulum, leading to shortened limb and limp

severe arthritis

Question 3

give 4 risk factors for DDH?

Answer 3

Girls > boys
Breech presentation
1st born
Multiple pregnancy

Question 4

which hip is more likely to be affected in DDH?

Answer 4

Left hip

Question 5

A baby presents with shortening of their left leg, and asymmetrical groin creases, what investigations would you do and what differential diagnosis would you be considering?

Answer 5

Ultrasound of hip
Ortolani/Barlows tests
DDH

Question 6

what are Ortolani and Barlows tests?

Answer 6

diagnostic for DDH if a clunk is heard
Ortolani = reducing a dislocated hip with abduction and anterior displacement
Barlows = dislocating hip with adduction and posterior displacement

Question 7

How is DDH managed?

Answer 7

Pavlik’s harness for 6 weeks - holds hips flexed and abducted

Question 8

What is Perthes disease?

Answer 8

idiopathic Osteochondritis of the femoral head - inflammation of the bone/cartilage which leads to the femoral head losing its blood supply, AVN and collapse/fracture as a result

Question 9

Who is most likely to be affected by Perthes disease?

Answer 9

Short boys who are active

Question 10

How does Perthes disease present?

Answer 10

Hip pain
Limp
Loss of internal rotation of the hip

Question 11

Management of Perthes disease?

Answer 11

regular X-rays and no exercise to monitor improvement

Question 12

what is transient synovitis of hip?

Answer 12

self limiting of the synovium of the hip joint, usually following an URTI

Question 13

How does transient synovitis of the hip present?

Answer 13

limp with knee/groin pain
history of URTI
reluctance to weight bear

Question 14

management of transient synovitis of hip?

Answer 14

once more serious causes have been excluded NSAIDs and rest

Question 15

what does SUFE stand for?

Answer 15

Slipped Upper Femoral Epiphysis

Question 16

What is SUFE?

Answer 16

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

Question 17

Who is most likely to get SUFE?

Answer 17

Overweight pre-pubertal boys

Question 18

What can develop as a result of SUFE?

Answer 18

avascular necrosis of the femoral head

Question 19

How would SUFE present?

Answer 19

pain - can sometimes only be in the knee
loss of internal rotation of the hip
limp

Question 20

Management of SUFE?

Answer 20

Pinning of the femoral head to prevent it slipping further

Question 21

What is osteogenesis imperfecta and what is it caused by?

Answer 21

Brittle bone disease - autosomal dominant defect in maturation and organisation of type I collagen

Question 22

What are blue sclera, loss of hearing and multiple fragility fractures signs of?

Answer 22

Osteogenesis imperfecta

Question 23

What will bones look like on X-Ray in osteogenesis imperfecta?

Answer 23

Bones will have thin cortices and be osteopenic

Question 24

What is the difference between proportionate and disproportionate skeletal dysplasias?

Answer 24

proportionate = limbs and spine are proportionately short 
disproportionate = limbs are disproportionately short/long compared to the spine

Question 25

What is the most common type of skeletal dysplasia and what are its typical features?

Answer 25

Achondroplasia | Disproportionately short limbs, lax joints & prominent forehead

Question 26

What is Ehlers-Danlos syndrome?

Answer 26

autosomal dominant condition where there is abnormal collagen and elastin formation - get joint hypermobility and instability

Question 27

What is Marfans syndrome?

Answer 27

autosomal dominant/sporadic condition affecting the fibrillin gene - tall with disproportionately long limbs

Question 28

What is duchenne's muscular dystrophy?

Answer 28

progressive muscular weakness due to a defect in the dystrophin gene

Question 29

what is a sign of duchenne's muscular dystrophy?

Answer 29

Gowers sign - walking hands up legs due to pelvic weakness

Question 30

Who is most likely to be affected by duchenne's?

Answer 30

Boys

Question 31

What is cerebral palsy?

Answer 31

neuromuscular condition due to insult to the developing brain before age 3

Question 32

Give 5 causes of cerebral palsy?

Answer 32

``` Brain malformation Intrauterine infection Premature birth Hypoxia during birth Menengitis ```

Question 33

What is ataxic cerebral palsy?

Answer 33

When the cerebellum is affected so there is reduced coordination and balance

Question 34

What is athetoid cerebral palsy?

Answer 34

when there is uncontrolled writhing motion, sudden changes in tone and difficulty controlling speech

Question 35

Give 3 MSK related complications of cerebral palsy?

Answer 35

Joint contractures Scoliosis Hip dislocation

Question 36

What is Spina Bifida?

Answer 36

a congenital disorder where 2 halves of the posterior vertebral arch fail to fuse in the first 6 weeks of gestation

Question 37

What is poliomyelitis?

Answer 37

viral infection affecting the anterior motor horn cells in the spinal cord/brain stem - this results in lower motor neurone deficit (paralysis)

Question 38

What is the most common type of obstetric brachial plexus palsy and which nerve roots are affected in it?

Answer 38

Erb's palsy - injury to C5 & C6 nerve roots | loss of innervation to trapezius, deltoid and some of the rotator cuffs resulting in "waiter tips" pose

Question 39

What is the more rare type of obstetric brachial plexus palsy where C8 and T1 roots are affected?

Answer 39

Klumpkes palsy

Question 40

What is talipes equinovarus?

Answer 40

clubfoot - congenital deformity where the calcaneus, talus and navicular are misaligned and there are contractors in the surrounding soft tissues

Question 41

Are boys or girls more likely to have clubfoot?

Answer 41

boys

Question 42

Give 2 risk factors for the development of clubfoot?

Answer 42

Breech presentation | Low amniotic fluid content

Question 43

Management of clubfoot?

Answer 43

Ponseti technique

Question 44

What is myositis ossificans?

Answer 44

post traumatic calcification of soft tissues - history of trauma with soft tissue swelling, hardness then develops over a couple of weeks

Question 45

What is the best imaging for suspected DDH?

Answer 45

Ultrasound