Spina Bifida Flashcards

1
Q

Spina Bifida

  • what is it = ?
  • most commonly located in the what region = ?
A

Spina Bifida → Neural tube defect causing neurologic dysfunction.

→ Most commonly located in the lumbar/sacral region.

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2
Q

Spina Bifida

  • List a few of the caustitve factors
A

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.
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3
Q

What is the prognosis for individuals with Spina Bifida?

A

> 90% survival with aggressive treatment.

→ Key treatments:
- Antibiotics for infection prevention.
- Early surgical closure of the back.
- Ventricular shunt placement.
- Clean, intermittent catheterization.

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4
Q

What are other synonymous terms for Spina Bifida?

A

→ Myelomeningocele.
→ Meningomyelocele.
→ Spina Bifida occulta/aperta.
→ Spina Bifida cystica.
→ Spinal dysraphism.
→ Myelodysplasia.

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5
Q

Describe the embryology of Spina Bifida.

A

→ 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.
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6
Q

How is Spina Bifida diagnosed prenatally?

A

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.

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7
Q

What are the classifications of Spina Bifida?

2x2

A

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.
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8
Q

What is Hydrocephalus, and how does it relate to Spina Bifida?

A

→ Abnormal CSF accumulation in ventricles.

→ Causes:

  • Overproduction of CSF.
  • Failure of CSF absorption.
  • Obstruction in normal CSF flow.
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9
Q

Explain Arnold-Chiari Malformation and its link to Spina Bifida.

A

→ 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.

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10
Q

What are the birth considerations for Spina Bifida?

A

→ C-section is preferred to reduce nerve trauma and infection risk.
→ Prevents contamination and facilitates timely surgical closure.

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11
Q

What are the risks and benefits of fetal surgery for Spina Bifida?

A

Benefits: Decreased hindbrain herniation and hydrocephalus.

Risks: Infant mortality, preterm labor, placental abruption, uterine wall thinning.

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12
Q

What is the general philosophy of treatment for Spina Bifida?

A

Back Surgery: Place neural tissue in vertebral canal, cover with skin/fascia.

Additional factors: Evaluate hydrocephalus and orthopedic deformities.

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13
Q

How is hydrocephalus managed in infants with Spina Bifida?

A

→ Monitor head circumference and signs like ‘sunsetting’ eyes, suture separation, bulging fontanel.

→ Shunt placement within days to 6 months after birth.

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14
Q

What types of shunts are used for hydrocephalus in Spina Bifida?

A

Ventriculoatrial (VA) Shunt: Moves CSF from ventricle to heart atrium.

Ventriculoperitoneal (VP) Shunt: Preferred; diverts CSF to peritoneum.

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15
Q

What are key aspects of physical therapy for infants with Spina Bifida?

A

Manual Muscle Testing (MMT): Pre- and post-surgery, starting day 10 and continuing yearly.

ROM Exercises: Daily, avoiding aggressive stretching.

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16
Q

What are effective positioning strategies for infants with Spina Bifida?

A

Prone Position: Across parent’s lap or forearm.

Upright Position: Over parent’s shoulder.

Supported Sitting: Monitor infant’s response to gravity.

17
Q

How should sensory assessment be approached in Spina Bifida?

A

→ Light touch, deep pressure, temperature checks.
→ Educate parents on safety measures, like bath water temperature and skin protection.

18
Q

What are developmental issues associated with Spina Bifida?

A

→ Gross/fine motor, perceptual motor, and cognitive deficits.
→ Delays in head and trunk control, righting, and equilibrium responses.

19
Q

What are the typical developmental issues in children with Spina Bifida?

A

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

20
Q

How do compensatory patterns affect children with Spina Bifida?

A

→ 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.

21
Q

What handling strategies should parents use for children with Spina Bifida?

A

→ 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.

22
Q

What are the key components of developmental intervention for Spina Bifida?

A

→ Equilibrium and righting reactions.
→ Dynamic sitting and prone positioning.
→ Early weight-bearing activities.
→ Quality of movement assessment.

23
Q

What are the general philosophies of bracing for Spina Bifida?

A

→ 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.

24
Q

What are the characteristics of thoracic-level paralysis in Spina Bifida?

A

→ 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.

25
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.
26
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.
27
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.
28
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.
29
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.
30
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.
31
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.
32
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.
33
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.
34
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.
35
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.
36
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.
37
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.
38
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.