Spina Bifida Flashcards
Clinical Signs of Spina Bifida
-Absence of motor and sensory function (usually B) below the level of the spinal defect
Loss of neural control of bowel/bladder function
Unilateral and asymmetric motor and sensory loss
-Higher motor or sensory level on one side than on other
-functional deficits may be partial or complete
What is hydrocephalus
abnormal accumulaation of CSF in cranial vault
What are causes of hydrocepalus
Overproduction of CSF
Failure in absorption of CSF fluid
Obstruction in normal flow of CSF through brain structures and spinal cord
What is Chiari II Malformation
- common to children with SB
- deformity of cerebellum, medulla, and C-spinal cord
- posterior cerebellum is herniated downward through foramen magnum
- brainstem structures also displaced in caudal direction
What are Prenatal Testing and Diagnosis?
- γ-Fetoprotein levels (AFP)
- ->Remain abnormally high after 14 weeks’ gestation
- Fetal ultrasound
What is fetal surgery and what is it associated with
repair the exposed spine in utero
associated with decreased hydrocephalus
What are the risks of fetal surgery
premature labor, placental abruption, and thinning of uterus
What are management ideas for the Neonate
- Studies support early and aggressive intervention
- Prevent infection
- Closure of the back within 72 hours of birth
What is the general philosophy of treatment
- DEPENDS on extent of neurologic impairment
- DEPENDS on extent of hydrocephalus
- Extend of kyphoscoliosis/scoliosis
Scoliosis in patients with Spina Bifida
- bracing may help decrease but not eliminate the problem
- function becomes difficult
- surgical option for fusion
Latex allergy in patients with spina bifida
- increased in children with spina bifida
- will need to make sure you are using latex free things (including Thera-Band)
- allergic reaction can develop with increased exposure
What is the perceptual motor and cognitive performance?
- wide variety of cognitive levels depending on treatment of hydrocephalus, episodes of cerebral infection, and the presence of other CNS abnormalities
- increased attention deficit
- decreased ocular function
What is the wheelchair mobility in patients with spina bifida?
- adapted strollers for young children
- standard wheelchair by school age
- power wheelchair and scooters as child ages
What should we say to pts about recreation and leisure activities?
- encourage full participation in recreation activities with adaptations as needed
- aquatics are also good
What are some common complaints of young adults with spina bifida?
- Obesity, incontinence, recurrent urinary tract infections, chronic decubiti, joint pain, hypertension, neurologic deterioration, and depression
- Social integration, vocational issues, and sexual counseling
What is the most common cause of morbidity in spina bifida?
urinary tract issues
___% of those who undergo neonatal surgery for back closure will develop hydrocephalus. These pts will require a shunt placement.
90
Ventriculoatrial (VA) shunt
Moves excess CSF from one lateral ventricle to the right atrium of the heart
Ventriculoperitoneal (VP) shunt
Preferred treatment for hydrocephalus. Shunt runs down to peritoneum where CSF is absorbed and the excess is excreted.
MMT should be performed preoperatively and postoperatively on day __, at __ months, and yearly thereafter.
10, 6
Name two types of contractures common with spina bifida.
Hip flexion due to unopposed hip flexors and ankle dorsiflexion due to unopposed dorsiflexors
Spina bifida is the ___ most common birth defect.
2nd
- 3.4 per 10,000 live births
What are possible causes of spina bifida?
- Has been linked to maternal lack of folic acid in first trimester
- Also associated with valproic acid (Seizure medication)
What are some early treatment options for a patient with spina bifida? How is the prognosis?
- Closure of spine (surgical), Shunt for hydrocephalus, Clean, intermittent catheterization for urination
- Greater than 90% survival with early treatment
Spina bifida can also be known as…..
Myelomeningocele Meningomyelocele Spina bifida aperta Spina bifida cystica Spinal dysraphism Myelodysplasia
What is spina bifida?
Spinal defect diagnosed by the presence of an external sac on the infant’s back
Where is the most common place for the external sac to be located?
In the lumbar region
What is contained in the external sac?
- Meninges and spinal cord tissue
- Protruding through a dorsal defect in the vertebrae
- Can be covered by membrane or exposed
What is spinal bifida occulta?
- Involves nonfusion of the halves of the vertebral arches
- Without disturbance of the underlying neural tissue
What is a myelocele?
- Protruding sac containing meninges and cerebrospinal fluid (CSF)
- Nerve roots and spinal cord remain intact and in their normal positions.
- No motor or sensory deficits
- Associated hydrocephalus, or other CNS problems
What is a lipomeningocele?
- Superficial fatty mass in the low lumbar or sacral level of the spinal cord
- Significant neurologic deficits and hydrocephalus are not expected.
Explain the embryology behind spina bifida.
- Caudal end of the neural tube closes on approximately day 25-26 of gestation
- Failure of the neural tube to close at any point along the caudal border initiates the defect of spina bifida cystica or myelomeningocele
Developmental Issues (3)
1) Mild/moderate developmental delay
2) Develops compensatory strategies for low tone/proprioceptive input
3) Movement and exploration is limited.
2 handling strategies for parents
1) Education should focus on gross, fine, and perceptual motor abilities.
2) Emphasize upright positions.
4 developmental concerns
1) Equilibrium and righting reactions
2) Prone positioning
3) Early weight bearing
4) Assessment of quality of movement
We should discourage the use of what 5 infant devices?
Infant walkers, jumper seats, swings, bouncer chairs, and the excessive use of infant car seats
We should encourage what type of active participation?
upright experiences by parents
Explain the principle of bracing that says: predictable level of mobility exists for children at each motor level
- Advocates establishing reasonable expectations for each child
- Many factors affect continuation and discontinuation of ambulation
Explain the principle of bracing that says: Early standing and gait training for as long as seems reasonable
Children attain their optimal level of performance, regardless of their motor level, and assist them to maintain this level for as long as is feasible
A well-defined orthotic approach should begin as early as…
the child’s first day of life.
What is the posture of the LEs in a child with thoracic level paralysis and what does this posture put them at risk for?
Posture of flaccid lower extremities and at risk for developing a frog-legged deformity
What type of bracing is required for a child with thoracic level paralysis?
total contact body brace
What should the total contact body brace include?
lower leg section to hold the ankle in a neutral or plantigrade position.
What type of bracing is required for a child with high lumbar paralysis?
high level of bracing for standing and ambulation
What are children with high lumbar paralysis at risk for?
hip subluxation/dislocation
