Spina Bifida Flashcards
Spina Bifida
- what is it = ?
- most commonly located in the what region = ?
Spina Bifida → Neural tube defect causing neurologic dysfunction.
→ Most commonly located in the lumbar/sacral region.
Spina Bifida
- List a few of the caustitve factors
→ No single etiology.
→ Genetic predisposition.
→ Environmental influences
- Low maternal folic acid levels (recommended 400 mcg pre-conception, 600 mcg during the first trimester
→ Maternal hyperthermia
- Medications: Valproic acid, antidepressants.
What is the prognosis for individuals with Spina Bifida?
→ > 90% survival with aggressive treatment.
→ Key treatments:
- Antibiotics for infection prevention.
- Early surgical closure of the back.
- Ventricular shunt placement.
- Clean, intermittent catheterization.
What are other synonymous terms for Spina Bifida?
→ Myelomeningocele.
→ Meningomyelocele.
→ Spina Bifida occulta/aperta.
→ Spina Bifida cystica.
→ Spinal dysraphism.
→ Myelodysplasia.
Describe the embryology of Spina Bifida.
→ Caudal neural tube closes by day 28 of gestation.
→ Failure to close at any point along the caudal border initiates Spina Bifida.
→ Clinical signs:
- Bilateral absence of motor/sensory function below the defect.
- Loss of bowel and bladder control.
How is Spina Bifida diagnosed prenatally?
→ Blood Test: Alpha-Fetoprotein (AFP) levels remain high after 16 weeks.
→ Amniocentesis: Detects 89% of neural tube defects (NTDs).
→ Ultrasound: ‘Lemon sign’ shows frontal bone flattening before 24 weeks.
What are the classifications of Spina Bifida?
2x2
→ Spina Bifida Occulta: Non-fusion of posterior vertebral arch; no neural tissue disturbance.
→ Spina Bifida Cystica:
- (a) Meningocele: Sac with meninges and CSF; no motor deficits.
- (b) Myelomeningocele: Sac with meninges, CSF, and spinal cord.
What is Hydrocephalus, and how does it relate to Spina Bifida?
→ Abnormal CSF accumulation in ventricles.
→ Causes:
- Overproduction of CSF.
- Failure of CSF absorption.
- Obstruction in normal CSF flow.
Explain Arnold-Chiari Malformation and its link to Spina Bifida.
→ Primary cause of hydrocephalus in Spina Bifida.
→ Deformity of cerebellum, medulla, and cervical spine.
→ Posterior cerebellum herniates through foramen magnum.
→ Surgical treatment: Posterior fossa decompression and cervical laminectomy.
What are the birth considerations for Spina Bifida?
→ C-section is preferred to reduce nerve trauma and infection risk.
→ Prevents contamination and facilitates timely surgical closure.
What are the risks and benefits of fetal surgery for Spina Bifida?
→ Benefits: Decreased hindbrain herniation and hydrocephalus.
→ Risks: Infant mortality, preterm labor, placental abruption, uterine wall thinning.
What is the general philosophy of treatment for Spina Bifida?
→ Back Surgery: Place neural tissue in vertebral canal, cover with skin/fascia.
→ Additional factors: Evaluate hydrocephalus and orthopedic deformities.
How is hydrocephalus managed in infants with Spina Bifida?
→ Monitor head circumference and signs like ‘sunsetting’ eyes, suture separation, bulging fontanel.
→ Shunt placement within days to 6 months after birth.
What types of shunts are used for hydrocephalus in Spina Bifida?
→ Ventriculoatrial (VA) Shunt: Moves CSF from ventricle to heart atrium.
→ Ventriculoperitoneal (VP) Shunt: Preferred; diverts CSF to peritoneum.
What are key aspects of physical therapy for infants with Spina Bifida?
→ Manual Muscle Testing (MMT): Pre- and post-surgery, starting day 10 and continuing yearly.
→ ROM Exercises: Daily, avoiding aggressive stretching.
What are effective positioning strategies for infants with Spina Bifida?
→ Prone Position: Across parent’s lap or forearm.
→ Upright Position: Over parent’s shoulder.
→ Supported Sitting: Monitor infant’s response to gravity.
How should sensory assessment be approached in Spina Bifida?
→ Light touch, deep pressure, temperature checks.
→ Educate parents on safety measures, like bath water temperature and skin protection.
What are developmental issues associated with Spina Bifida?
→ Gross/fine motor, perceptual motor, and cognitive deficits.
→ Delays in head and trunk control, righting, and equilibrium responses.
What are the typical developmental issues in children with Spina Bifida?
CNS deficits: gross motor, fine motor, perceptual motor, cognition.
→ 85% have low muscle tone
→ Mild to moderate developmental delay
→ Abnormal head and trunk control
→ Delayed righting and equilibrium responses
→ Limited movement and exploration
How do compensatory patterns affect children with Spina Bifida?
→ Head: Larger and heavier, shoulders elevated to stabilize head.
→ Upper Extremities: Overuse in prone-on-elbows (POE) position, difficulty shifting weight side-to-side, poor head control.
→ Trunk: Lack of rotation, leads to W-sitting.
→ Lower Extremities: Degree of paralysis varies based on motor and sensory loss.
What handling strategies should parents use for children with Spina Bifida?
→ Focus on upper/lower extremities, head, and trunk.
→ Address compensatory patterns by facilitating proper movement.
→ Encourage upright positions early.
→ Adapt to the child’s and family’s capacity for learning.
What are the key components of developmental intervention for Spina Bifida?
→ Equilibrium and righting reactions.
→ Dynamic sitting and prone positioning.
→ Early weight-bearing activities.
→ Quality of movement assessment.
What are the general philosophies of bracing for Spina Bifida?
→ Predictable levels of mobility exist based on lesion level.
→ Set reasonable expectations for the child’s performance.
→ Early standing and gait training is encouraged.
→ Support optimal performance for as long as possible.
What are the characteristics of thoracic-level paralysis in Spina Bifida?
→ Flaccid lower extremities, prone to “frog-leg” deformity (hips abducted, externally rotated, knees flexed, ankles plantarflexed).
→ No lower extremity movement against gravity.
→ Use total contact orthosis in infancy to stabilize pelvis and lumbar spine.
How is high lumbar paralysis (L1-L3) managed in Spina Bifida?
→ Muscle Activation: Some hip flexion/adduction, weak quads, and medial hamstrings (L3).
→ High-level bracing for standing and ambulation.
→ Standing device may decrease as hip control improves.
→ Risk of hip subluxation/dislocation; surgery not always recommended.
What orthotic and mobility options are used for thoracic and high lumbar paralysis (T12-L3)?
→ Orthotics: HKAFOs, RGOs, swivel standers.
→ Mobility: Rolling walkers, forearm crutches, swing-to or swing-through gait.
→ Wheelchair: Primary mode of mobility in adolescence.
Describe the characteristics of low lumbar paralysis (L4-L5) in Spina Bifida.
→ Strong hip flexors/adductors, glut medius, and tensor fascia lata.
→ Glut max and lateral hamstrings absent.
→ Strong quads, dorsiflexors; absent plantarflexors.
→ High risk for hip contractures and dislocation.
How are orthotics and gait managed for low lumbar paralysis (L4-L5)?
→ Orthotics: RGO (if CNS deficits), KAFO (weak quads), AFO.
→ Mobility: Rolling walker, forearm crutches by age 6.
→ Gait Characteristics: Hyperlordotic spine, glut medius gait, prone positioning to counteract hyperlordosis.
What are the characteristics of sacral-level paralysis in Spina Bifida?
→ S1-S2: Strong knee flexors and glut medius; weak glut max, gastroc/soleus.
→ S2-S3: Full innervation of muscles with good strength.
→ Orthosis: SMO or ground reaction AFO for weak gastroc-soleus.
→ Crouch gait is common.
What are common foot deformities in children with Spina Bifida?
→ Talipes equinovarus (clubfoot).
→ Pes equinus (toe-walking).
→ Rocker bottom foot.
→ Vertical talus.
→ High arch with toe-clawing.
→ Pes cavus and calcaneovarus deformities.
What are the symptoms and treatment of hydromyelia in Spina Bifida?
→ Excess CSF creates pressure and nerve necrosis.
→ Symptoms: Progressive scoliosis, upper extremity weakness, hypertonus.
→ Requires shunt revision or additional placement.
Describe the tethered spinal cord condition in Spina Bifida.
→ Adhesions anchor the spinal cord, preventing normal movement.
→ Leads to metabolic changes, neural ischemia, and muscle function loss.
→ Diagnosed with imaging, treated with neurosurgical release.
What are the symptoms of a tethered spinal cord in Spina Bifida?
→ Sudden lumbar lordosis increase.
→ Early scoliosis development.
→ Spasticity in sacral muscles.
→ Gait pattern change, urologic function decline, increased tone with passive ROM.
→ Asymmetric MMT changes, progressive lower extremity weakness.
How is scoliosis managed in children with Spina Bifida?
→ Thoracic level: always at risk; lumbar/sacral: evaluate as curve develops.
→ Bracing: Supports weak trunk, slows progression.
→ Surgical fusion may be needed; avoid sacral fusion to maintain flexibility.
→ Pre- and post-op PT assessment is critical.
What is the relationship between Spina Bifida and latex allergy?
→ Natural rubber is widely used in medical products.
→ Increased incidence in Spina Bifida (18-37% vs. 1% in general population).
→ Many children’s hospitals are latex-free.
How does Spina Bifida affect perceptual motor and cognitive performance?
→ Varies based on hydrocephalus management and CNS anomalies.
→ Attention deficits, distractibility, “cocktail party chatter.”
→ Decreased ocular function, visual perceptual, and spatial issues.
What wheelchair mobility options are available for children with Spina Bifida?
→ Adapted strollers for young children.
→ Hand-propelled devices (“Ready Racer”), electric cars, standard wheelchairs.
→ Power wheelchairs and scooters for older children.
→ Essential cushion for pressure relief.
What recreational activities can be adapted for children with Spina Bifida?
→ Encourage full participation with adaptations.
→ Adaptive sports programs.
→ Pairing teens with special needs children in recreational sports.
→ Adaptive bicycles, aquatics, and specialized camps.
