Spina Bifida Flashcards
Etiology
Maternal lack of folic acid in first trimester
Also associated with valproic acid (seizure medication)
Myelodysplasia
General terminology that encompasses all of the kids
SBO
Involves non-fusion of the halves of the vertebral arches
Without disturbance of the underlying neural tissue
Myelocele
Protruding sac containing meninges and CSF
NR and SC remain intact in their normal positions
No motor or sensory deficits, associated hydrocephalus my or other CNS problems
Lipomeningocele
Superficial fatty mass in the low lumbar or sacral level of the SC
Significant neurologic deficits and hydrocephalus are NOT expected
Myelomeningocele
Spinal cord
Meninges
CSF
Prenatal Testing and Dx
AFP - remain abnormally high after 14 weeks gestation
Fetal ultrasound
Fetal surgery
Repair the exposed spine in utero - assoc with decreased hydrocephalus, no significant changes in motor fx
Prognosis
Greater than 90% survival with early treatment
- Closure of spine
- Shunt for hydrocephalus
- Clean, intermittent catheterization
Impairments
Disruption of nerve conduction below level of the lesion
Joint contracture due to mm imbalances
Loss of sensation
Loss of bowel and bladder control
Associated Disorders
Neurological
Hydrocephalus
Arnold Chiari Malformation
Hydromyelia
Tethered cord
Decreased speech and cognitive function - cocktail party speech
Associated Disorders
MSK
Club feet
Congenital hip dislocation
Hydrocephalus
An abnormal accumulation of CSF in cranial vault
Causes…
Overproduction CSF
A failure in absorption of CSF
Obstruction in normal flow of CSF through brain structures and SC
Management of Hydrocephalus
90% of SB will develop this
Shunt placed within first several days to 6 mos
Thin, flexible tube that diverts CSF away from the lateral ventricles
VA shunt
Moves excess CSF from one lateral ventricle to the right atrium of the heart
VP shunt
Preferred treatment for hydrocephalus
Drains to abdomen
Chiari II Malformation
Common to children with Spina Bifida
Deformity of the cerebellum, medulla, and cervical spinal cord
The posterior cerebellum is herniated downward through foramen magnum
Brainstem structures also displaced in a caudal direction
Signs of shunt malfunction
Headache Irritability Fever unrelated to illness Nausea Increased spasticity in INN mm Increase difficulty with postural control Decreased school performance Decreased level of consciousness "Sunset" sign of the eyes
Sunset sign
Downward rotation of eyes within orbit so you only see half the iris and pupil
Hydromyelia
Excess CSF collects in pockets down the SC that creates areas of pressure and necrosis of the surrounding PERIPHERAL NERVES
Causes scoliosis
Progressive UE weakness and hypertonus
Requires shunting
Tether Spinal Cord
Adhesions anchor the SC at the sight of the original lesion
Cord is not free to slide upward and reposition
Excessive stretch causes metabolic changes and ischemia of the neural tissue
Associated degeneration in mm function
Signs of Tethered Spinal Cord
Rapidly progressive scoliosis
Hypertonus at one or several sites in the LEs
Changes in gait pattern
Changes in urologic function
Increase tone in passive ROM
Asymmetric changes in MMT results
Areas of decreasing strength
Discomfort in the back
Requires surgical repair***
Management of Neonate
Prevent infection
Closure of the back within 72 hours of birth
Developmental Issues
Mild/moderate developmental delay
Develops compensatory strategies for low tone/proprioceptive input
Movement and exploration is limited
Handling Strategies for Parents
Education should focus on gross, fine, and perceptual motor abilities
UE/LE, head, and trunk
Learning pace depends on child and family capacity to learn
Emphasize upright positions