Ch 10 - Peds: Spina Bifida and NMD Flashcards
Where are the highest and lowest incidence of Spina bifida?
Highest incidence occurs in the British Isles, Ireland, Wales, and Scotland
Lowest in Japan
What is spina bifida occulta?
Failure of fusion of the posterior elements of the vertebrae
What is the MC SC level involved with spina bifida occulta?
Lumbosacral or sacral region
MC L5 and S1 levels
What is menigocele?
Protruding sac contains meninges and spinal fluid
What is the MC SC level involved with spina bifida cystica?
75% lumbar and lumbosacral segments
What is myelomeningogele?
Protruding sac contains meninges, spinal cord, and spinal fluid
What is myelocele?
Cystic cavity is in front of the anterior wall of the spinal cord
What is myelomeningogele associated with?
Arnold-Chiari malformation Complicated by hydrocephalus in over 90% of the cases
What % of children with hydrocephalus requiring shunting?
> 80% require VP shunting
What are typical signs of a tethered cord?
Weakness Scoliosis Pain Ortho deformity Urologic dysfunction
What are presenting symptoms of Syringomyelia?
Deterioration of neuro function
Pain and temp sensory deficits
Loss of motor function esp UE
Inc spasticity Hyperreflexia
What is best to evaluate for Syringomyelia?
MRI
What is the MCC of death in myelodysplasia?
Central resp dysfunction
What is common with Proteus urinary tract infections?
Calcinosis
What is associated with greater chance of bowel continence?
Presence of bulbocavernosus or anocavernosus reflex
Describe Intellectual function in myelodysplasia.
The higher the lesion, the lower the IQ score
When is Independent self-catheterization may be achieved?
5-6 yo
Why is there a 500x inc risk of anaphylaxis in the OR with spina bifida?
Latex allergy
Describe ambulation in thoracic myelodyplasia.
Assistive devices for passive standing, usually started at 12 to 18 months
Describe ambulation in lower thoracic and lumbar myelodyplasia.
Reciprocal gait orthosis (RGO), used after the age of 3 years
Describe ambulation in lumbar myelodyplasia.
Bracing is usually not required, but patients may benefit from AFOs if plantar flexors are spastic or nonfunctional
Describe functional community ambulation by level.
Thoracic: 0-33%
High lumbar: 31%
Low Lumbar:38% by 15 yo,95% by 15-31 yo
Sacral: 100%
What is the employment rate among those with spina bifida?
25-50%
What is Gower’s sign?
Inability to rise from sitting position on the floor, normally due to proximal (pelvic girdle) muscle weakness