Clinical Paediatric orthopaedics Flashcards

1
Q

What is osteogenesis imperfecta?

A

brittle bones disease - defect of maturation and organisation of type 1 collagen

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2
Q

What the main causes of osteogenesis imperfecta?

A

Autosomal dominant with multiple fragility fractures of childhood, short stature with multiple deformities, blue sclerae and loss of hearing

  1. can have rarer autosomal recessive and are either fatal in the perinatal period or associated with spinal deformity
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3
Q

what is Skeletal dysplasia?

A

medical term for short stature/dwarfism - due to genetic error (hereditary or sporadic mutation)

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4
Q

What is the most common type of skeletal dyspasia?

A

achondroplasia - autosomal dominant however most cases are sporadic

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5
Q

What is Marfan’s syndrome?

A

Autosomal dominant or sporadic mutation of the fibrillin gene resulting in tall stature with disproportionately long limbs and ligamentous laxity

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6
Q

What are the associated features of Marfan’s syndrome?

A

High arched palate

scoliosis

Flattening of the chest

eye problems - lens dislocation and retinal detachment

aortic aneurysm

cardiac valve incompetence

spontaneous pneumothorax

apical blebs

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7
Q

What is Duchenne muscular dystrophy?

A

Defect in the dystrophin gene involved in calcium transport causes muscle weakness.

X linked recessive - only boys get it

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8
Q

When is Duchenne muscular dystrophy first observed in boys?

A

when they start to walk - find it difficult standing

Difficult to climb stairs

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9
Q

How do you diagnose Duchenne muscular dystrophy?

A

Raised CK

muscle biopsy

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10
Q

What is the commonest type of Obstetric brachial plexus palsy?

A

Erb’s palsy - injury to the upper nerve roots. causes loss of motor innervation of the deltoid, supraspinatus, infraspinatus, biceps and brachialis muscles.

internal rotation of the humerus and waiter’s tip posture

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11
Q

How do you manage Erb’s palsy?

A

physiotherapy

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12
Q

What is Klumpke’s palsy?

A

rarer type of obstetric brachial plexus

lower brachial plexus injury via forceful adduction results in paralysis of the intrinsic hand muscles and finger and wrist flexors and possible Horner’s syndrome

fingers normally flexed

worse prognosis than Erb’s

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13
Q

What does ‘in-toeing’ mean?

A

refers to a child who has their feet point towards the midline when walking or standing

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14
Q

What is femoral neck anteversion

A

describes the femoral neck pointing slightly forwards (anterverted). Excess femoral neck anterversion may give the appearance of in-toeing but is not at a greater magnitude

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15
Q

What is flat feet?

A

when children do not develop arched foot. does not necessarily carry any functional problems?

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16
Q

How do you differentiate between mobile and fixed flat feet?

A

Mobile/flexible- flattened medial arch forms with dorsiflexion of the great toe (jack test). Only present on weight bearing. It is a normal variant in children.

rigid- arch remains flat regardless of load or great toe dorsiflexion. implies underlying bony abnormality

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17
Q

What is developmental dysplasia of the hip? (DDH)

A

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

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18
Q

What is the prevalence DDH?

A

More common in girls than boys

affects up to 5 in 1000 babies

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19
Q

What are the clinical features of DDH?

A

more common in the left hip - due to the intrauterine position

Age <4

1/5 of cases of DDH are bilateral

Severe arthritis due to reduced contact area can occur at a young age

signs:

  1. Shortening, asymmetric groin/thick skin creases
  2. Click or clunk occurs with ortolani or Barlow manoeuvres
  3. Unstable hips with a positive ortolani test
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20
Q

What are the risk factors of DDH?

A

Positive family history of DDH

Breech presentation

first born babies

Down’s syndrome

Talipes

Arthrogryposis

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21
Q

How do you diagnose DDH?

A

positive Ortolani test ( reducing a dislocated hip with abduction and anterior displacement)

Positive Barlow test (dislocatable hip with flexion and posterior displacement)

US - detects a dislocated hip, unstable hip or a shallow acetabulum

X rays - cannot be used for early diagnsis as the femoral head epiphysis is unossified until 4-6 months but X-rays are investigation after this age

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22
Q

How do you manage DDH?

A

Pavlik harness- for dislocated or persistently unstable hips. keeps the hips in comfortable flexion and abduction - maintains reduction. Used up to 4-6 months of age

Open reduction for children >18 months

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23
Q

What is the transient synovitis of the hip?

A

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

most common cause of hip pain in childhood

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24
Q

What is the prevalence of transient synovitis of the hip?

A

Typical age 2-10
more common in boys

Commonly occurs shortly after an URTI

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25
Q

What are the clinical features of transient synovitis?

A

Child presents with limp or reluctance to weight bear on the affected side

Motion may be restricted - not as bad as septic arthritis

Fever

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26
Q

How do you diagnose transient synovitis?

A

Radiographs exclude Perthes disease

Normal CRP - excludes Septic arthritis

MRI - excludes osteomyelitis

aspiration of the hip - see if there is potential bacterial infection

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27
Q

How do you manage transient synovitis?

A

NSAIDs and rest

pain generally resolves within a few weeks

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28
Q

What is Perthes disease?

A

Idiopathic osteochondritis of the femoral head

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29
Q

What is the prevalence of Perthes disease?

A

Occurs between the age of 4-9 years old

more common in boys 5:1 - very active boys of short stature

30
Q

What is the pathophysiology of the Perthes disease?

A

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

31
Q

What are the signs and symptoms of Perthes disease?

A

Loss of internal rotation is usually the first clinical sign followed by loss of abduction

Children will present with pain and a limp

Most cases are unilateral and bilateral cases may represent an underlying skeletal dysplasia or a thrombophilia

positive trendellenburg test

32
Q

How do you manage Perthes disease?

A

X-RAY observation

avoidance of physical activity

50% of cases do well

Sometimes the femoral head may partially dislocate requiring an osteotomy

33
Q

What is a slipped upper femoral epiphysis? (SUFE)

A

A condition where the growth plate (physis) is not strong enough to support body weight and the femoral epiphysis slips due to the strain

34
Q

What is the prevalence of SUFE?

A

Mainly affects overweight pre-pubertal adolescent boys

girls are less commonly affected and hypothyroidism or renal disease may predispose SUFE

35
Q

What are the clinical features of SUFE?

A

Loss of internal rotation of the hip is the predominant clinical sign

Patients have pain and a limp

Beware: patients can presently purely with pain in the knee so you must ALWAYS examine the hip even if they do not complain about it.

X-rays changes can be subtle and a lateral view must be obtained

36
Q

How do you manage SUFE?

A

Urgent surgery to pin the femoral head to prevent further slippage

the greater the degree of the slip the worse the prognosis

some rquire hip replacements in adolescence or early adulthood.

gentle manipulation can be used for severe acute slips - risks avascular necrosis

chronic severe slips may require an osteotomy

37
Q

What is the epidemiology of anterior knee pain?

A

Common in adolescence especially in girls

38
Q

What are clinical features of Anterior knee pain?

A

Softening of the hyaline cartilage of the patella occurs

aeitiology is unclear may be due to muscle imbalance

39
Q

How do you manage anterior knee pain?

A

Physiotherapy is used to rebalance the muscles

Majority of cases are self-limiting

Resistant cases may require surgery to shift forces on the patella

40
Q

What is Talipes Equinovarus (club foot)

A

congenital deformity of the foot due to utero abnormal alignment of the joints

41
Q

What is the epidemiology of club foot?

A

Positive family history

Occurs in 1 in 800 births

Boys more likely than girls 2:1

Oligohyadraminos ( low aminiotic fluid) is a risk factor

42
Q

What are the signs and symptoms of club foot?

A

Joints between Talus, calcaneus and navicular bones have abnormal alignment in utero

  1. Ankle equinus deformity ( plantarflexion)
  2. Supination of the forefoot
  3. Varus alignment of the forefoot
43
Q

How do you diagnose club foot?

A

Diagnosis is usually obvious

can have delayed presentations ( uncommon in modern healthcare systems) - results in fixed deformity with the child walking on the outside of their foot.

44
Q

How do you manage club foot?

A

Ponseti technique - main treatment. foot placed in casts with 5 or 6 weekly cast changes.

For full correction tentomy of the achilles tendon ( minor technique) is required in 80% of cases

Boot brace then required to be used for 23 hours a day for 3 months and then then during sleep until the age of 3 or 4

45
Q

What is scoliosis?

A

Lateral curvature of the spine

46
Q

What are the causes of scoliosis?

A

Idiopathic is the most common

secondary causes include:

  1. Neuromuscular disease
  2. Tumour e.g. osteoid osteoma
  3. Skeletal dysplasia
  4. infection
    n. b. Idiopathic scoliosis more common in females and younger children with scoliosis usually with an underlying cause. May progress more readily to a severe curve
47
Q

How do you diagnose scoliosis?

A

Usually on appearance

MRI - immediate if painful scoliosis

Forward bend test

48
Q

What are the signs and symptoms of scoliosis?

A

Usually painless or mild pain

If painful must have urgent investigations e.g. for tumour or infection

49
Q

What are the risk factors for curve progression?

A

cobb angle >=30 degrees

pubertal growth spurt

Premenarchal girls

Right thoracic curves in girls

Left lumbar curves in boys

50
Q

How do you manage Scoliosis?

A

Mild non progressive scoliosis does not require surgery

Large curves require suregery for cosmesis or improve wheelchair posture.

51
Q

What can severe curves result in?

A

Restrictive lung defect and surgery may be required to prevent breathing difficulties

52
Q

What is spondylosthesis?

A

Slippage of one vertebra over another and usually occurs at the L4/L5 or L5/S1 level

53
Q

What is the cause of spondylolisthesis?

A

Due to a developmental defect

or

recurrent stress posterior elements which fails to heal

54
Q

What are the clinical features of spondyloisthesis?

A

Usually presents in adolescence

Low back pain complaint - may have a radiculopathy with severe slippage.

They may have a paradoxical flat back - muscle spasm

can present acutely with waddling gait

55
Q

How do you manage spondylosisthesis?

A

Rest and physiotherapy for minor degree slippage

More severe slips may require stabilisation and possibly reduction.

56
Q

compare the periosteum in children and in adults.

A

it is much thicker and tends to remain in tact in children

57
Q

which fractures heal faster adults or children?

A

children - thicker periosteum which is a rich source of osteoblasts

58
Q

what does a salter harris classification rank?

A

ranks physeal fractures from 1 to 5

the greater the classification the poorer the prognosis

59
Q

what is a salter harris I fracture?

A

pure physeal separation

best prognosis

60
Q

what is a salter harris II fracture?

A

most common physeal fracture

similar to Salter harris I but has a small metaphyseal fragment to physis and epiphysis

61
Q

what do salter harris III and IV fractures signify?

A

fractures are intra-articular and with the fracture splitting the physis, there is greater potential for growth arrest

62
Q

what is a salter-harris V injury?

A

compression injury to the physis with subsequent growth arrest

cannont be diagnosed on initial x-rays only detected once angular deformity has occured

63
Q

Give examples of common children distal radius fractures.

A

buckle fractures - are stable and require 3-4 weeks of splintage

greenstick fractures- may be angulated and may require manipulation and casting if there is significant deformity

salter harris fractures - occur in distal radius physis in older children. angulation with deformity requires manipulation

64
Q

why do both monteggia and galeazzi fracture-dislocations of the forearm go against usual principles of children’s fractures?

A

as they both require anatomic reduction and rigid fixation with plates and screws

65
Q

which type of supracondylar fractures of the elbow are more common?

A

extension type fractures - due to fall on to outstreched hand

less common: flexion type - due to fall onto the point of the flexed elbows

66
Q

How are supracondylar fractures managed?

A

Undisplaced fractures - splint

angulated,rotated or displaced fractures - closed reduction with wires to prevent deformity

67
Q

what are the causes of femoral shaft fractures in chidlren?

A

fall onto a flexed knee

indirect bending

rotational force

in children >2 more than half these cases are due to child abuse

68
Q

how do you manage femoral shaft fractures?

A

children >2 - gallows traction and early hip spica cast is appropriate

children 2-6 - thomas splint or hip spica cast

children 6-12 - femur is large enough to accomodate flexible intramedullary nails

children 12 and above - adult type intramedullary nail

69
Q

what is the femur a common site of?

A

benign and malignant tumours and so fractures may be pathological with osteolysis and cortical thinning

70
Q

which age group are undisplaced spiral fractures of the tibial shaft common in?

A

toddlers - also known as the toddler’s fracture

71
Q

how do you manage tibial fractures in children?

A

spiral undisplaced fractures - cast

cast is the main treatment

compartment syndrome risk is much lower for children

use intramedullary nails or external fixation for open fractures or very unstable fractures when stabilising them

adolescents with a closed proximal tibial physis can have adult type intramedullary nails