Paediatrics: Birth defects/MSK Flashcards

1
Q

What is cerebral palsy?

A

A group of permanent movement disorders that occurs as a result of damage to a child’s CNS involved in motor control, leading to impaired movement and coordination - the lesion that occurs is non-progressive i.e. it does not get worse but lifelong

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2
Q

What are the three types of causes of cerebral palsy?

A

1) Antenatal/intrapartum
2) Postnatal
3) Idiopathic

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3
Q

What are antenatal/intrapartum causes of cerebral palsy?

A

1) Hypoxic-ischaemic encephalopathy
2) Infection

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4
Q

What are postnatal causes of cerebral palsy?

A

1) Meningitis
2) Trauma
3) Haemorrhage
4) Medication toxicity
5) Kernicterus - never event as significant jaundice should be identified and treated asap

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5
Q

How does CP present?

A

1) Depends on the site and extent of the lesion
2) Widespread brain damage affecting multiple areas of motor control will result in a wide variety of motor impairments

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6
Q

What are the categories of motor disorders of cerebral palsy (not exclusive and can overlap)?

A

1) Spastic - 90% of patients with cerebral palsy have some spastic features
2) Dyskinetic/athetoid
3) Ataxic

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7
Q

What is spastic cerebral palsy caused by?

A

Damage to the pyramidal pathway (pyramidal weakness)

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8
Q

What are the features of spastic cerebral palsy?

A

1) Increased tone and reflexes
2) Clasp knife
3) Flexed hip and elbow
4) Scissor gait
5) May be monoplegic, diplegic, hemiplegic

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9
Q

What is dyskinetic/athetoid cerebral palsy caused by?

A

Damage to the basal ganglia pathways

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10
Q

How does dyskinetic/athetoid cerebral palsy present?

A

1) Choreiform movements
2) Can exhibit signs of Parkinsonism

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11
Q

What is ataxic cerebral palsy caused by?

A

Damage to the cerebellar pathways

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12
Q

How does ataxic cerebral palsy present?

A

1) Uncoordinated movements
2) Signs of cerebellar lesions

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13
Q

What are complications of cerebral palsy?

A

1) Injuries from impaired balance/coordination
2) Aspiration pneumonias from impaired swallowing
3) Muscle wasting
4) Scoliosis and other MSK deformities from impaired posture and muscle control

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14
Q

How is cerebral palsy managed?

A

1) Specialist and MDT input to minimise and deal with complications of cerebral palsy
2) Medical management
3) Surgical management

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15
Q

What type of specialist and MDT input is offered to minimise and deal with complications of cerebral palsy?

A

1) Physiotherapy - to help with movement and strength exercises
2) Occupational therapy - may help with mobility aids, home adjustments and devices e.g. orthotics
3) Speech and language therapy - for swallowing assessments
4) Dieticians - if there are concerns regarding low intake due to swallowing difficulties

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16
Q

What are medical management options for cerebral palsy?

A

1) Baclofen - for spasms
2) Botox injections - for contractures

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17
Q

What are surgical management options for cerebral palsy?

A

1) Orthopaedic surgery - for MSK deformities/injuries/tendon releases
2) General surgery - if a PEG tube needs fitting

18
Q

What are the typical features of cerebral palsy presentation?

A

1) Delayed achievement of motor milestones
2) Hypertonia and brisk reflexes

19
Q

What is a risk factor for hypoxic ischaemic encephalopathy?

A

Infants > 42 weeks who get stuck during prolonged labour e.g. emergency instrumental delivery after prolonged labour

20
Q

How does Perthe’s disease present?

A

Limping child aged 4-10 years old

21
Q

What is the genetic background of Becker’s muscular dystrophy?

A

X-linked recessive inherited disorder

22
Q

How does Becker’s muscular dystrophy present?

A

1) Slowly progressive muscle weakness of the legs and pelvis
2) Regression of motor milestones e.g. walking

23
Q

What type of jaundice is not severe enough to cause kernicterus?

A

Physiological breast milk jaundice (jaundice on day 2, peaks at day 7 then resolves)

24
Q

Would congenital developmental dysplasia of the hip (CDH) prevent a child from learning to walk at an appropriate time?

A

No

25
Q

What is spinal muscular dystrophy?

A

Form of motor neurone disease - genetic condition which affects the nervous system and skeletal muscle

26
Q

How does spinal muscular atrophy present?

A

1) Floppiness at birth
2) Progressive weakness
Does not present with spasticity like in cerebral palsy however contractures may develop

27
Q

How does muscular dystrophy present?

A

1) Progressive weakness and loss of muscle mass
2) Caused by genetic mutation
Does not present with spasticity like in cerebral palsy

28
Q

In which age group is spinal stenosis more common?

A

Adults > 50 years

29
Q

When can spinal stenosis occur in babies and children?

A

When they are born with a small spinal canal

30
Q

How does spinal stenosis present in children?

A

Impingement of nerves at a specific level
1) Back pain
2) Progressive neurological symptoms e.g. numbness, weakness or tingling

31
Q

What is developmental dysplasia of the hip?

A

An abnormality of the hip joint where the ball of the femur and acetabulum of the pelvis do not articulate properly during development, meaning that the joint can dislocate easily and will continue to develop abnormally

32
Q

What are risk factors for developmental dysplasia of the hip (DDH)?

A

5 F’s
1) Female
2) Firstborn
3) Family history
4) Fanny first (breech)
5) Fluid (oligohydramnios)

33
Q

How do you screen for developmental dysplasia of the hip (DDH)?

A

Two manoeuvres:
1) Barlow - posterior dislocation
2) Ortolani - relocation on abduction of the hip

34
Q

What should be done if there are risk factors for DDH (breech or first degree FH) or abnormal hip examination (Barlow and Ortolani test)?

A

Hip ultrasound

35
Q

How do you manage developmental dysplasia of the hip (DDH)?

A

1) In mild cases, DDH will be self-limiting within the first few months of life
2) More severe cases may necessitate a Pavlik harness or surgery

36
Q

What would make you refer a child for hip ultrasound?

A

1) Abnormal hip examination - Barlow and Ortolani test
2) First degree FH of DDH or breech presentation

37
Q

Up until what age is simple gastro-oesophageal reflux (posseting) considered normal?

A

Up to the age of 1 year

38
Q

When would baby regurgitation be concerning?

A

If baby is not growing well or there is associated pain

39
Q

What can be done to prevent further neurological damage in HIE?

A

Cooling to 34 degrees at 6h of life

40
Q

What is HIE?

A

When a hypoxic event around birth causes irreversible brain damage which presents with irritability, hypotonia and seizures