MSK & Orthopaedics Flashcards

1
Q

What is Perthes’ disease?

A

A degenerative condition affecting the hips of children, typically between 4-8 years

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2
Q

What is the pathophysiology of Perthes’ disease?

A

Avascular necrosis of the femoral head, specifically the epiphysis - impaired blood supply eventually causes femoral necrosis

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3
Q

What is the epidemiology of Perthes’ disease?

A

5x more common in males, 10% of cases are bilateral

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4
Q

What are the features of Perthes’ disease?

A
  • Hip pain: progressively over a few weeks
  • Limp
  • Stiffness and reduced range of hip movement
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5
Q

What are the diagnostic investigations for Perthes’ disease?

A
  • Plain x-ray
  • Technetium bone scan or MRI if normal x-ray and symptoms persist
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6
Q

What are the features of Perthes’ disease on x-ray?

A
  • Joint effusion: widening of the medial joint space
  • Asymmetrical femoral epiphyseal size
  • Blurring of the physeal plate
  • Radiolucency of proximal metaphysis
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7
Q

What are the complications of Perthes’ disease?

A
  • Osteoarthritis
  • Premature fusion of the growth plates
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8
Q

What staging is used to classify Perthes’ disease?

A

Catterall staging

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9
Q

What is the management of Perthes’ disease?

A
  • Keep the femoral head with the acetabulum (cast, braces)
  • If less than 6 years: observation
  • Older/ if failed to respond to conservative measures: surgical management
  • If severe deformities: surgical management
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10
Q

What is the prognosis of Perthes’ disease?

A

Most cases resolve with conservative management
Early diagnosis improves outcomes

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11
Q

What causes Perthes’ disease?

A

The trigger for avascular necrosis is unknown

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12
Q

What is muscular dystrophy?

A

Muscles appearing poorly nourished due to degeneration

Dys - bad
Trophy - nourish

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13
Q

How can muscular dystrophies and neuropathies be distinguished?

A

In muscular dystrophy, the neuromuscular junction is in tact but the muscles are dystrophied

In neuropathies that NMJs are not preserved

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14
Q

What are the dystrophinopathies?

A

Muscular dystrophies that occur due to mutations in the dystrophin gene (allelic disorders)

  • Duchenne muscular dystrophy
  • Becker muscular dystrophy
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15
Q

What mutation leads to duchenne muscular dystrophy?

A

Nonsense or frameshift mutation leading to no production of the dystrophin protein (one or both of the binding sites are lost)

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16
Q

What mutation leads to becker muscular dystrophy?

A

Misshapen dystrophin caused by missense mutations (both binding sites are preserved)

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17
Q

What is the difference between DMD and BMD?

A

DMD:
- More severe
- Symptoms tend to present by age 5
- Earlier onset complications

BMD:
- Less severe
- Symptomatic by age 10-20

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18
Q

What is the inheritance pattern of the dystrophinopathies?

A

X-linked recessive

2/3 of the time, the mother is a carrier
1/3 of the time, the mutation is sporadic

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19
Q

What is lyonization?

A

The inactivation of one x chromosome in females (as they inherit 2) to ensure that females only have one functional copy of the x chromosome in cells

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20
Q

What are manifesting carriers?

A

Carriers of an x-linked recessive genetic condition where more of the x-chromosomes with the functional copy of the gene undergo lyonization, leading to over-expression of the defective gene and manifestation of the disease

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21
Q

What is the function of the dystrophin protein?

A

It links intracellular actin to the dystrophin-associated protein complex on the muscle cell membrane, which links with the extracellular matrix, and provides structure (like a wooden support beam)

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22
Q

What happens to muscle cells without dystrophin?

A

The integrity of the sarcolemma (muscle cell membrane) is compromised leading to release of intracellular proteins eg. CK and cell death

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23
Q

What happens to muscle tissue in the short term, for a patient with dystrophinopathy?

A

Muscle regeneration:
- Cell death leads to muscle tissue loss
- Muscle fibres of different sizes are produced leading to some tissue regain

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24
Q

What happens to muscle tissue in the long term, for a patient with dystrophinopathy?

A

Muscle atrophy:
- Fat and fibrotic tissue infiltrates the muscle tissue
- Muscles become weak

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25
Q

How does Duchenne muscular dystrophy present?

A
  • Progressive proximal muscle weakness from 5 years old
  • Waddling gait
  • Pseudohypertrophy of the calfs
  • Gowers’ sign
  • 30% of patients have intellectual impairment
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26
Q

What is Gower’s sign?

A

When lying on their stomach, the child will use their arms to help themselves stand due to muscle weakness in the legs and upper hips

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27
Q

What are the later symptoms of dystrophinopathies?

A
  • Wheelchair use
  • Respiratory failure - diaphragmatic weakness
  • Scoliosis
  • Dilated cardiomyopathy
  • Arrhythmias
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28
Q

What is the cause of the cardiac symptoms in dystrophinopathies?

A

Dystrophin is expressed in cardiac muscle

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29
Q

How are dystrophinopathies diagnosed?

A
  • High serum creatine kinase
  • Genetic testing: DNA test, Western blot
  • Muscle biopsy: staining for dystophin (genetic testing has replaced this as the way to achieve a definitive diagnosis)
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30
Q

What is the medical management for dystrophinopathies?

A
  • Glucocorticoids - slow the degeneration, but there are side effects eg. weight gain
  • Ataluren - restores dystrophin synthesis (>=5 years old)
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31
Q

What is the prognosis for DMD?

A
  • Most children can’t walk by the age of 12
  • Patients typically survive to 25-30 years
  • Associated with dilated cardiomyopathy
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32
Q

What is the physiotherapy for Perthe’s disease?

A

Stretching of the quadriceps and hamstring muscles, strengthen quads and encourage hip abduction

Continue activity

33
Q

What is the supportive care for acute pain in Perthe’s?

A
  • Simple analgesia
  • Ice packs
34
Q

What is the supportive management for DMD?

A
  • Physiotherapy (prevent contractures)
  • Exercise and psychological support
  • Dietician (gastric feeding in some patients, vitamin D and calcium supplementation if bone fragility)
35
Q

Why can DMD lead to nocturnal hypoxia?

A

Weakness of the intercostal muscles

36
Q

What is the management for DMD with nocturnal hypoxia?

A

Overnight CPAP

37
Q

What is the management for DMD if left ventricular fraction drops?

A

Cardioprotective drugs (eg. carvedilol) and left ventricular assist devices

38
Q

What is the managment for DDH in <6 months old?

A

1st line
- Observation: monitor via repeat USS or X-ray

2nd line
- Splint/ Pavlik’s harness to keep hip flexed and abducted (follow up with x-ray at 6 months)

3rd line
- Surgery if conservative measures fail

39
Q

What is the surgery for DDH?

A

Reduction with spica casting

40
Q

What are the indications for USS at 6 weeks to check for DDH?

A
  • Breech presentation (36/40)
  • Breech delivery (including <36/40)
  • Family hx DDH
41
Q

What imaging is better for DDH >6 months?

A

Hip x-ray

42
Q

Which sex is at greater risk of DDH?

A

Female

43
Q

Does DDH tend to resolve?

A

Most resolve spontaneously by 3-6 weeks

44
Q

What is the initial management of fractures?

A

Initial pain management and immobilisation
- Oral ibuprofen and/or paracetamol
- IV opiods for severe pain
- Acute stage assessment and diagnostic imaging

45
Q

What is the management of distal radius fracture in ED?

A

Manipulation
Consider:
- A below-elbow plaster cast
- K-wire fixation if the fracture is completely displaced

46
Q

What is the first step in management of femoral fracture in ED?

A

Admit all children

47
Q

Femoral fracture managment 3-18 months?

A

Gallows traction

48
Q

Femoral fracture managment 1-6 years?

A

Straight leg traction with possible conversion to hip spica cast to enable early discharge

49
Q

Femoral fracture managment 4-12 years?

A

Elastic intramedullary nail

50
Q

Femoral fracture managment >11 years (weight >50kg)?

A
  • Elastic intramedullarly nails supplemented by endcaps

OR
- Lateral entry antegrade rigid intramedullary nail

OR
- Submuscular plating

51
Q

Who should patients with JIA be managed by?

A

Specialist paediatric rheumatology MDT

52
Q

Why should patients with JIA continue activity?

A

Inactivity leads to deconditioning, disability and decreased bone mass (continue activities like swimming and cycling)

53
Q

What is the management of JIA?

A

Pharmacological
WHO Pain ladder
- Simple analgesia
- NSAIDs (controlling pain and stiffness)
- Consider weak opiates

Intra-articular corticosteroids (or oral/IV)
- Adjunctive agents (avoided if possible due to growth suppression and osteoporosis)

DMARDs (when disease fails to respond to conventional treatment)
- 1st line: oral or SC methotrexate
- 2nd line: sulfasalazine

Other (biologics)
- Inflammatory cytokine blockage eg. TNF-alpha inhibitors, IL receptor antagonists, anti-emetics

54
Q

What are the complications of JIA?

A
  • Joint damage
  • Anterior uveitis
  • Osteoporosis
  • Growth failure
55
Q

When is Osgood-Schlatter referred to orthopaedic surgeons?

A

If symptoms don’t improve or worsen or persist to adulthood despite management

56
Q

What is the management of Osgood-Schlatter?

A

Pain relief
- Analgesia (paracetamol/ NSAIDs)
- Ice packs over tibial tuberosity (10-15 mins up to x3 per day)
- Protective knee pads (pain when kneeling)

57
Q

Should sporting activity be stopped in Osgood-Schlatter?

A
  • Reduce sporting activity
  • Change type of exercise to limit running and jumping
  • As sx decrease, gradually increase exercise levels
  • Low impact quadriceps exercises (cycling, swimming, straight leg raises)
58
Q

What is the management for osteochondritis dissecans?

A
  • Pain relief
  • Rest and quads exercises
  • Sometimes surgical intervention needed to remove intra-articular loose bodies
59
Q

What is the management for acute osteomyelitis?

A
  • High dose IV empirical abx (usually 2-4 weeks)
  • Clinical recovery (acute phase proteins normal) switch to oral abx
  • Alter abx regimen when blood cultures are back
  • Affected limb immobilised, analgesia given
60
Q

When should blood cultures be taken for osteomyelitis?

A

Before starting abx

61
Q

When can early transition to oral abx be started for children with osteomyelitis?

A

If they respond well, early transition to oral abx (after 3 days- 1 week) can be considered

62
Q

How long is the course of oral abx for osteomyelitis?

A

6 weeks

63
Q

When is surgical debridement considered for osteomyelitis?

A

If there’s dead bone or a biofilm

64
Q

How is chronic osteomyelitis staged?

A

Using Cierney-Mader classification

65
Q

What is the management for chronic osteomyelitis?

A
  • Surgical debridement
  • IV abx
  • Functional rehabilitation
66
Q

What is the management for reactive arthritis?

A
  • Self-resolving
  • Symptomatic relief: NSAIDs, steroids (severe), DMARDs (ongoing)
67
Q

What is the management of Rickets?

A

Calcium and vitamin D deficiency
- Daily calcium
- Daily ergocalciferol (V D2)/ cholecalciferol (V D3)

Pseudo-vitamin D deficiency (defect in 1-alpha hydroxylase)
- Calcitriol/ 1-alfacalcidiol

Dietary
- Oily fish, egg yolk

68
Q

What is given in hypophosphataemic rickets?

A

Phosphate salts

69
Q

What is the antibiotic course length for septic arthritis?

A

Initially IV for 2 weeks, followed by 4 weeks of oral abx

70
Q

Which abx are used for septic arthritis in a neonate <3 months?

A

IV cefotaxime

71
Q

Which abx are used for septic arthritis 3 months - <=5 years?

A
  • IV ceftriaxone
  • If penicillin allergic, give clindamycin
72
Q

Which abx are used for septic arthritis >=6 years?

A
  • IV flucloxacillin
  • If penicillin allergic, give clindamycin
73
Q

What are the oral step-down abx for septic arthritis?

A
  • Co-amoxiclav
  • Flucloxacillin
74
Q

Which methods are used for joint aspiration in septic arthritis?

A

Joint aspirated to dryness
- Closed needle aspiration
- Arthroscopically

75
Q

What is the management of SUFE?

A
  • Patient remains non-weight bearing, analgesia and IMMEDIATE ORTHO REFERRAL
  • Surgical repair: in situ fixation across the growth plate
76
Q

What are the x-ray findings of SUFE?

A

Trethowan’s sign: Klein line doesn’t intersect superior femoral epiphyses/ asymmetry between Klein line on either side

77
Q

What is the managment of transient synovitis?

A
  • Bed rest
  • Pain relief (paracetamol or NSAIDs)
  • Usually resolves after a few days
78
Q

What is the management of femoral fractures at birth or due to prematurity?

A

Simple padded splint

79
Q

What is the management of femoral fractures 0-6 months?

A

Pavlik’s harness or Gallows traction