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Clinical Medicine - OT > Week 4 - Paeds Spina Bifida and Muscular Dystrophy > Flashcards

Week 4 - Paeds Spina Bifida and Muscular Dystrophy Flashcards

1
Q

What is spina bifida?

A

A condition that results from a neural tube defect, where the vertebra that cover the spinal cord have one or more openings in the middle, allowing exposure and protrusion of nervous tissues and coverings, with varying levels of damage to the nerves.

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2
Q

How does the neural tube develop?

A

In the 3rd week, the embryonic dorsal ectoderm thickens on the lateral edges and grows toward each other, fusing along the midline. It is formed by the 4th week.

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3
Q

What is a neural tube defect?

A

Neural tube defects are major congenital anomalies that result from very early disruption in development of the brain and spinal cord

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4
Q

What are the 3 forms of neural tube defect?

A

Anencephaly
Encephalocele
Spina Bifida

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5
Q

What is ancencephaly?

A

a severe congenital condition in which a large part of the skull is absent along with the cerebral hemispheres of the brain. Incompatible with life.

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6
Q

What is encephalocele?

A

a sac-like protrusion or projection of the brain and the membranes that cover it through an opening in the skull. Encephalocele happens when the neural tube does not close completely during pregnancy

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7
Q

What are the 4 factors affecting the prognosis of neural tube defects?

A

The site
The extent of the defect
The type of nerve cells involved
The extent of secondary abnormalities

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8
Q

True or false; babies with Spina Bifida survive unless the condition is severe?

A

True

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9
Q

What determines the level of impairment in Spina Bifida?

A

The site and extent of brain tissue involved

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10
Q

What does spina bifida mean?

A

Split or divided spine

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11
Q

True or false; Spina Bifida can occur anywhere along the spine?

A

True

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12
Q

When does Spina Bifida occur?

A

When part of the vertebra does not completely join which results in the exposure of a part of the spinal cord and surrounding tissues.

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13
Q

True or false; Spina bifida refers to a group of defects that do not interfere with the development of the CNS?

A

False. Spina bifida defects DO interfere with the development of the CNS.

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14
Q

What are the 3 types of spina bifida?

A
  1. Spina Bifida Occulta
  2. Meningocele
  3. Myelomeningocele
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15
Q

Which type of spina bifida is the most common?

A

Spina bifida occulta

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16
Q

Which type of spina bifida is the rarest?

A

Meningocele

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17
Q

Which type of spina bifida is the rarest?

A

Myelomeningocele

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18
Q

Which 2 types of spina bifida and a type of Spina Bifida Cystica?

A

Meningocele and myelomeningocele

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19
Q

What is Spina Bifida Occulta?

A

A malformation of one or more vertebrae, with the skin intact. It is sometimes called “closed” spina bifida and is not associated with any symptoms.

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20
Q

What is Meningocele?

A

A protrusion of spinal fluid and the meninges through a gap in the spine due to a congenital defect with malformed vertebrae.

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21
Q

What is Myelomeningocele?

A

Malformed vertebrae where sac of fluid comes through an opening in the baby’s back. Part of the spinal cord and nerves are in this sac and are damaged.

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22
Q

What are the ectodermal abnormalities associated with spina bifida occulta?

A

A dermal pit
A depression with a tuft of hair
A fatty swelling

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23
Q

What percentage of the population have Spina Bifida Occulta?

A

10-20%

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24
Q

Is the spinal cord involved in Meningocele SB?

A

No, no spinal cord involvement

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25
Q

What are the symptoms of Meningocele SB?

A

A broad range, however often causes very few symptoms.

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26
Q

Where does Meningocele SB occur?

A

Anywhere along the length of the spine

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27
Q

How is Meningocele SB treated?

A

Surgery to correct it

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28
Q

Which form of Spina Bifida is the most frequently treated by OT?

A

Myelomeningocele

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29
Q

What impairment is Myelomeningocele asociarted with?

A

Motor and sensory impairment

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30
Q

Where can Myelomeningocele occur?

A

Anywhere along the spine

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31
Q

What determines the severity of symptoms of Myelomeningocele?

A

The size and level of the lesion

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32
Q

What other conditions is Myelomeningocele associated with?

A

Hydrocephalus and Chiari II malformation.

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33
Q

What is hydrocephalus?

A

A build-up of cerebrospinal fluid in the ventricles of the brain causing them to expand and push against brain tissue.

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34
Q

What is Chiari II malformation?

A

A condition in which brain tissue extends into the spinal canal, present at birth. It occurs when part of the skull is abnormally small or misshapen.

35
Q

What are the symptoms of Chiari II malformation?

A

pain and trouble swallowing

36
Q

What are the 5 identified risk factors of Spina Bifida?

A 
Nutrition
Genetic Syndrome
Twins
Environmental Agents
Noxious Agents
37
Q

True or false, a majority spina bifida cases occur in families with a history of neural tube defects?

A

False. Spina bifida occurs in families with no history of neural tube defects in a majority of cases

38
Q

What nutritional factor is important in preventing spina bifida?

A

Folate

39
Q

What is the underlying cause for neural tube defects?

A

There is no clear underlying cause

40
Q

What population has the highest prevalence of neural tube defect affected pregnancies?

A

Teenage women

41
Q

What is the prevalence of NTD in live births?

A

4.6 per 100,000

42
Q

What percentage of pregnancies affected by NTD were foetal deaths or managed terminations?

A

77%

43
Q

How is spina bifida diagnosed?

A

Screening test by blood at 16-18 weeks
Ultrasound
Amniocentesis after 1 week

44
Q

What percentage of affected pregnancies are diagnosed by testing?

A

85-90%

45
Q

What are the functional limitations of SB?

A 
Reduced mobility
Reduced skin sensation
Incontinence
Sexual dysfunction
Hydrocephalus
Chiari II malformation
Learning difficulties
Latex allergy
Feeding difficulties
46
Q

What % of people with SB also have hydrocephalus?

A

80-90%

47
Q

What % of people with SB have Chiari II malformation? 90%

A

90%+

48
Q

How does hydrocephalus present in babies and young children?

A

Looks like an enlargement of the head

49
Q

What % of children with Myelomeningocele have hydrocephalus?

A

90%

50
Q

How is hydrocephalus treated?

A

A VP shunt is inserted at a young age to drain excess CFS

51
Q

What are the signs of a malfunctioning VP shunt?

A 
Headache
Vomiting
Vision problems
Deterioration in performance
personality changes
Dizziness
Seizures
52
Q

What determines the need for surgery with Chiari II malformation?

A

The presence of symptoms

53
Q

What are the symptoms of Chiari II malformation?

A 
Balance problems
muscle weakness
dizziness
vision problems
difficulty swallowing
problems with voice production
ringing ears
fine motor difficulties
breathing problems
54
Q

What is the team involved in SB?

A 
Paediatrician
Paediatric Neurologist
Paediatric Orthopaedic surgeon
OT
PT
SP
Social worker
55
Q

True or false; in Spina Bifida, ambulation declines with age?

A

True

56
Q

What is skin management?

A

Prevention of pressure sores, burns and scrapes

57
Q

What is continence management?

A

Surgery, catheterisation, aids and diet.

58
Q

True or false; Children with a lesion at L4 or lower will walk without splints or crutches?

A

True

59
Q

How will a SB child with a sacral level lesion walk?

A

With or without gait aids

60
Q

How will a SB child with a lumbar lesion walk?

A

With gait aids and a wheelchair will be required for long distances and as they age.

61
Q

True or false; Children with higher lesions can walk with orthotics and won’t require a wheelchair as they get older?

A

False, they will require a wheelchair as they get older.

62
Q

Will a child with a Thoracic level lesion be able to walk?

A

No, it is likely they will need a wheelchair for mobility.

63
Q

What is muscular dystrophy?

A

A group of genetic diseases that cause progressive weakness and loss of muscle mass.

64
Q

Which muscles are primarily affected by MD?

A

Skeletal muscles

65
Q

How is MD characterised?

A

By weakness presenting from birth to late adulthood

66
Q

What is the most common serious muscle disease?

A

Muscular Dystrophy

67
Q

What are the 5 most common types of MD?

A
  1. Duchenne MD
  2. Becker MD
  3. Facioscapulohumeral Syndrome
  4. Distal MD
  5. Emery-Dreifuss MD
68
Q

What is the most common form of MD?

A

Duchenne MD

69
Q

True or false, Duchenne MD is not inherited?

A

False, it is a genetic condition

70
Q

What is the average age of diagnosis for Duchenne MD?

A

2-5 years

71
Q

What gender is more likely to be affected by Duchenne MD?

A

Males as it is an X linked condition

72
Q

What causes Duchenne MD?

A

A lack of protein in the muscle cell which causes the muscle to break down over time

73
Q

What does Duchenne MD do to the body?

A

Causes significant, progressive weakness over time of the muscles that support the spine and body, arms, legs, breathing muscles and heart

74
Q

What is the life expectancy of Duchenne MD?

A

Mid 20s

75
Q

What are the signs of Duchenne MD?

A 
Abnormal walking/running
Toe walking
Difficulty climbing
Difficulty getting up from floor
Frequent falls
Intellectual impairment
Delayed language development
Gowers sign
Enlarged and firmness of the calf, quads and triceps
76
Q

What is the most common sign of Duchenne MD?

A

Delayed walking

77
Q

True or false, in Duchenne MD, development in the first year of life is usually normal?

A

True

78
Q

What is Gower’s sign?

A

A sequence of manoeuvres used to rise from a supine position to compensate for progressive weakness of the muscles

79
Q

When is an apparent improvement in function seen in Duchenne MD?

A

Between the ages of 4 and 6

80
Q

When does function begin to decline in Duchenne MD?

A

6 onwards

81
Q

When is independent mobility usually lost in Duchenne MD?

A

Between the ages of 8 and 13 years

82
Q

How is Duchenne MD diagnosed?

A

Creatinekinase test
Electromyography
Muscle Biopsy

83
Q

What needs to be considered in the management of Duchenne MD?

A

The emotional, social, and educational needs of the child and family.

Clinical Medicine - OT (35 decks)

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