Spina Bifida

Question 1

Spina bifida

Answer 1

A vertebral defect characterised by non-closure of the posterior elements of the vertebral arch

Question 2

CNS manifestations of spina bifida

Answer 2

Spinal cord - sensory and motor deficits
Arnold-Chiari malformation
Hydrocephalus
Syringomyelia

Question 3

Genitourinary manifestations of spina bifida

Answer 3

Neurogenic bladder and bowel

Secondary renal impairment

Question 4

MSK manifestations of spina bifida

Answer 4

LL contractures/deformity
Scoliosis/spinal deformity
Hip dislocation
Increased risk of fracture

Question 5

Skin manifestations of spina bifida

Answer 5

Paraesthesia/anaesthesia
Reduced circulation
May result in pressure injury and reduced/delayed healing

Question 6

Other manifestations of spina bifida

Answer 6

Developmental delays
Learning difficulties
Cognitive deficits

Question 7

Signs and symptoms of Tethered Cord Syndrome

Answer 7

Decline in muscle strength
gait changes
Change in function
Progressive scoliosis 
Back and/or LL pain
Increasing LL deformities/contractures
Spasticity 
Change in urodynamics
Change in sensation

Question 8

When myelomeningoceole is present damage to the spinal cord typically occurs at the level of the lesion resulting in….

Answer 8

Motor deficits below the level of lesion
Sensory deficits below the level of lesion
Bladder/bowel dysfunction

Question 9

Neurosegmental Level of lesions

Answer 9

Thoracic - LL paralysis
L1 - weak hip flexion
L2 - Strong hip flexion and moderate adduction
L3 - strong hip flexion, adduction and knee extension
L4 - as above but with moderate/strong DF and inversion, knee flexion
L5 - as above but with strong knee flexion, DF and moderate eversion, hip abduction
S1 - as above with moderate/strong hip extension and weak ankle plantar flexion
S2- as above with strong PF and hip extension
S3 - as above with foot intrinsics

Question 10

Syringohydromyelia

Answer 10

Pain
Progressive spasticity
UL weakness, LL weakness, trunk weakness
Sensory changes
Scoliosis
Changing urodynamic function
Functional deterioration

Question 11

Arnold-Chiari Malformation

Answer 11

In infants
- Stridor, snoring, impaired vocal cord, high pitched cry, chewing and swallowing difficulties, nystagmus, tonal changes, recurrent aspirations
In older children
- sleep disturbances, nystagmus, headaches, neck pain or stiffness, increasing weakness, spasticity, reduced balance, loss of sensation, UL dysfunction and ataxia

Question 12

Learning difficulties with hydrocephalus

Answer 12

Eye- hand coordination
Attention
Memory 
Sequencing
Decision-making
Comprehension
Generalising 
Organisation
Problem-solving
Motivation

Question 13

Effect of hydrocephalus on development

Answer 13

GM delays
- hypotonia, delays in development of automatic reactions, prolonged need for propping in sitting, delays in achieving head control and trunk equilibrium, poor hip stability and delayed crawling
FM delays
- grip strength, abnormal hand position sense, difficulty with visual motor integration, poor motor quality and coordination

Question 14

Signs and symptoms of hydrocephalus - newborn to 18 months

Answer 14

Tense or bulging fontanelle
Increased head circumference 
Sunset eyes
Irritability
Poor feeding/vomitting
Drowsiness
Seizures
Asymmetrical postures

Question 15

Signs and symptoms of hydrocephalus - older children to adult

Answer 15

irritability, personality changes, headache, nausea and vomitting, seizures, decreased coordination and balance, blurred vision