Spina Bifida Overview

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Questions and Answers

What is the impact of a spinal defect in a fetus at 21 weeks of gestation?

Increased muscle tone and spastic paralysis

Complete sensory preservation and normal function

Cognitive impairments only

Flaccid paralysis and sensory loss (correct)

What is neurogenic bladder?

Bladder control due to strong neural innervation

Urinary condition from a spinal cord or nerve issue (correct)

Normal bladder function in brain-injured patients

Hydrocephalus affecting bladder control

What percentage of patients may experience neurogenic bladder dysfunction?

95% (correct)

75%

85%

65%

Which assessment tool is used for determining the functional level in Spina Bifida patients?

Spina Bifida Neurological Scale Signup and view all the answers

What condition is considered a primary cause of hydrocephalus?

Arnold-Chiari malformation Signup and view all the answers

What cognitive impact may patients with spinal defects experience?

Learning disabilities in 80% of cases Signup and view all the answers

What type of paralysis is more commonly associated with spinal defects?

Flaccid paralysis Signup and view all the answers

Which assessment evaluates the functional skill level and required caregiver assistance?

Pediatric Evaluation of Disability Inventory Signup and view all the answers

What is the primary cause of spina bifida occurring during embryonic development?

Inadequate closure of the neural tube Signup and view all the answers

Which type of spina bifida includes a sac that contains neural material?

Myelomeningocele Signup and view all the answers

Which of the following statements about meningocele is true?

It contains only CSF and meninges Signup and view all the answers

What is a recommended preventive measure against spina bifida for women of childbearing age?

Daily intake of folic acid Signup and view all the answers

What is the incidence of spina bifida in the general population?

1 in 1000 pregnancies Signup and view all the answers

Which of the following factors is NOT a suspected etiology for spina bifida?

Viral infections during pregnancy Signup and view all the answers

What is a common consequence for fetuses diagnosed with high spinal cord defects?

A tendency for termination of pregnancy Signup and view all the answers

What is one of the challenges associated with intra-uterine surgical repair of spina bifida?

Potential neurological deterioration of the fetus Signup and view all the answers

What is a common complication associated with spina bifida that can occur due to prolonged pressure from wheelchairs or bracing?

Pressure sores Signup and view all the answers

Which of the following impairments is likely to be observed in children with spina bifida?

Delayed motor development Signup and view all the answers

Which surgery is recommended for severe cases of spina bifida before 12 months of age?

Soft-tissue release surgery Signup and view all the answers

What intervention has been shown to improve gait speed in ambulatory children with spina bifida?

Treadmill training Signup and view all the answers

What are common foot deformities associated with spina bifida?

Clubfoot Signup and view all the answers

Which of the following is NOT a goal of the general physical therapy plan for individuals with spina bifida?

Cure neurological issues Signup and view all the answers

Which method is commonly used for the conservative management of clubfoot?

The Ponseti method Signup and view all the answers

What is one potential complication associated with a ventriculoperitoneal (VP) shunt?

Infection Signup and view all the answers

What percentage of children with spina bifida is likely to have a latex allergy?

18-40% Signup and view all the answers

During physical therapy, which precaution is NOT typically necessary due to the characteristics of spina bifida?

Enhanced muscle strength building Signup and view all the answers

How might spina bifida negatively affect a child's daily living activities?

Physical limitations hinder everyday activities Signup and view all the answers

What characterization describes the 'crouch standing' gait defect?

Persistent hip and knee flexion Signup and view all the answers

Which device is commonly used as a mobility aid for children with spina bifida?

Walkers Signup and view all the answers

What primary risk factor requires physical therapy patients with spina bifida to monitor their exercises closely?

Increased spasticity with exercise Signup and view all the answers

What percentage of children with spina bifida are likely to experience hydrocephalus?

80-95% Signup and view all the answers

Which of the following statements is true regarding the external closure of a defect in spina bifida management?

It reduces risk of damage to spinal nerves. Signup and view all the answers

What is a primary focus of the School Function Assessment (SFA)?

Understanding how impairments affect school-related tasks Signup and view all the answers

Which of the following is a key aspect of managing an adolescent with spina bifida?

Optimizing activity to minimize obesity risk Signup and view all the answers

What is one potential complication that children with spina bifida face?

Spinal and brain neurological deficits Signup and view all the answers

How can physiotherapists support children with spina bifida?

By promoting life-long management strategies Signup and view all the answers

What condition results from a defect in the embryonic neural tube?

Spina bifida Signup and view all the answers

What is one goal when training adolescents with spina bifida?

To train for self-reliance and pressure care Signup and view all the answers

What long-term effect can result from 'overuse' of certain joints in individuals with spina bifida?

Early degenerative changes in shoulders Signup and view all the answers

Which of the following assessments focuses on physical fitness and exercise training for individuals with spina bifida?

A systematic review by Oliveira et al. Signup and view all the answers

Study Notes

Spina Bifida Definition

Neural tube defect: This occurs in the embryo.
Synonyms:
- Spina bifida
- Myelomeningocele or meningomyelocele
- Meningocele

Spina Bifida Aetiology

Development: Happens around 21 days of embryonic development.
Cause: Failure of neural tube closure causing problems with spinal cord and vertebral development.
Result: Issues with the formation of skeletal and nervous tissues.

Types of Spina Bifida

Myelomeningocele:
- Neural material protrudes through spine
- Nerves at and below the sac are affected.
Meningocele:
- NO neural material in the sac
- Contains CSF and meninges, no neurological complications.
Spina Bifida Occulta:
- Nonfusion of vertebral arches
- No neural tissue disturbance
- May have a tuft of hair over area
- Occurs in 4-8% of the general population.

Spina Bifida Etiology

Genetics:
- Chromosomal abnormalities
- Racial variation in incidence
- Increased risk for siblings (2-3%)
Environmental:
- Teratogens (higher incidence in Celts, lower in African blacks)
Dietary Factors:
- High maternal alcohol intake
- Anti-epileptic medication use
- Low maternal folic acid levels before conception.
Cause: No definitive cause, likely to be multifactorial.

Spina Bifida Epidemiology

Incidence: Affects 1 in 1000 pregnancies.
Prenatal Diagnosis:
- Ultrasound at 18 weeks of gestation
- Alphafetoprotein levels screening, amniotic fluid analysis.
23% of pregnancies with prenatal diagnoses are terminated.
Survival: Lower survival rate for high SB defects in utero (primarily see mid-low thoracic and lumbar defects).
Delivery: C-section delivery is often preferred to avoid further damage to the neural sac.

Spina Bifida Prevention

Folic Acid:
- Consume daily in the months before conception and throughout pregnancy.
- 400 microgram folic acid tablet recommended daily.

Spina Bifida Intra-uterine Repair

Controversy: Debate about the benefits of surgical repair of the sac in utero.
Potential Benefits:
- May reduce further nerve damage
- May decrease the risk of Chiari II symptoms
Evidence: Limited evidence, repair performed in 21 out of 52 fetuses.

Spina Bifida Signs and Symptoms

Level Dependent:
- Paralysis distal to the defect (usually flaccid paralysis)
- Sensory loss (may not correlate with motor loss)
- Bowel and bladder dysfunction.

Spina Bifida Bladder and Bowel Dysfunction

Neurogenic bladder: 95% of patients experience this.
Urologist follow-up: Necessary to monitor and manage bladder function.
Training:
- Catheterization and self-catheterization training before age 7
- Importance of nursing involvement.

Spina Bifida Brain Signs and Symptoms

Associated Brain and Brainstem Symptoms:
- Cognitive, sensory and perceptual motor function may be impaired.
- Lower IQ than the general population
Learning Disabilities: 80% of individuals with SB.

Spina Bifida Arnold Chiari Malformation

Definition: Posterior lobes/cerebellum extends further inferiorly.
Location: Cerebellum and brainstem extend through the foramen magnum.
Effects: CNS abnormalities.
Connection to Hydrocephalus: Considered to be the primary cause of hydrocephalus.
Hydrocephalus: Abnormal accumulation of CSF.

Spina Bifida Physical Therapy Assessment

Classification Systems:
- Spina Bifida Neurological Scale (SBNS) - used to determine the motor level
Muscle Strength Testing: Evaluate key muscle groups.
Sensation Testing: Light touch and pinprick sensation tests.
Range of Motion (ROM): Assess ROM at each dermatomal level.

Spina Bifida Outcome Measures

Battelle Developmental Inventory:
Pediatric Evaluation of Disability Inventory (PEDI): Focuses on functional skills, caregiver assistance, and adaptations.
WeeFIM: Assesses functional impairments in preschool and young elementary school-aged children.
Spina Bifida Health-Related Quality of Life: Measures the quality of life for individuals with SB.

Spina Bifida Musculoskeletal Deformities

Stability and Alignment: Lack of stability and symmetrical alignment.
Motor Development: Delays in motor development (sitting at 11 months).
Postural Deviations and Contractures:
- Scoliosis, kyphosis, lordosis (more common in children with higher spinal lesions).
- Hip contracture, deformity of femur and acetabulum (occurs in 1/3 to 1/2 of children).

Spina Bifida Musculoskeletal Deformities (Continued)

Torsional Deformities: External or internal tibial torsion
Knee Deformities:
- Genu Varus (bowleggedness)
- Genu Valgus (knock-knees
- Knee flexion or extension contractures
- Thoracic and high lumbar SB: More than 70% develop knee contractures.
Foot Deformities: Common due to unopposed muscle action around the ankle.
- Calcaneovarus or calcaneovalgus foot due to absence of gastroc/soleus.
- Talipes equinovarus (clubfoot) due to L4 or L5 lesions.

Spina Bifida Musculoskeletal Deformities (Effects)

Negative Impact:
- Positioning
- Body image
- Weight bearing in sitting and standing
- Activities of Daily Living (ADLs)
- Energy expenditure.
- Mobility from infancy through adulthood.

Spina Bifida Gait Defects: 'Crouch Standing'

Characteristics:
- Persistent hip and knee flexion
- Increased lumbar lordosis.
Causes:
- Muscle weakness (insufficient soleus strength to maintain tibia vertical)
- Orthopaedic deformities (calcaneal valgus leads to tibial internal rotation and knee flexion)

Spina Bifida Decision to Walk or Not

Challenges:
- Increasing demands to walk further and faster
- Gait aids may only be suitable indoors
- Increased energy expenditure due to heavier body weight through adolescence
- Higher demands on balance due to increased height
- Development of obesity
- Reduction in gait due to foot ulcers, leading to immobility during healing.

Spina Bifida Medical/Surgical Management

External Closure of Defect: Reduces risk of CNS infection and further damage to spinal nerves within 24-72 hours after delivery.
Ventriculoperitoneal (VP) Shunt for Hydrocephalus:
- Hydrocephalus occurs in 80-95% of SB cases.
- Shunt insertion in the first week of life or within the first six months.
- Complications: Infection, blockage, multiple revision surgeries.

Spina Bifida Clubfoot

Prevalence: 30-50% of patients.
Conservative Treatment:
- Ponseti method: Serial manipulation and long-leg casting to gradually correct the deformity.
Surgical Treatment:
- Soft-tissue release surgery for severe cases between 10-12 months.

Spina Bifida Other Complications

Pressure Sores:
- 85-95% of individuals with SB by adulthood.
- Caused by pressure from wheelchairs, casts, braces, urine/stool soiling, friction, and shearing forces.
Osteoporosis:
- Decreased bone mineral density.
- Increased risk of fractures.
- Passive weight bearing has not been shown to reduce fracture rates.
Latex Allergy:
- 18-40% of children with SB (compared to 1-5% in the general population).
- Latex is found in gloves, wheelchairs, splints, and braces.

Spina Bifida Neurological Complications

Shunt Blockage: Drowsiness, headaches, seizures.
Tethering of Spinal Cord: Progression of neurological spinal symptoms (onset of upper limb weakness).
Spasticity: Can be pronounced.

Spina Bifida General Physical Therapy Plan of Care/Goals

ROM: Prevent loss of ROM in upper and lower extremities
Upper Extremity Overuse Injuries: Prevention.
Functional Mobility: Maximize independent functional mobility.
Quality of Life: Optimize health-related quality of life.
Health and Fitness: Maximize strength and aerobic capacity.

Spina Bifida Physical Therapy Interventions

Patient and Family Education: On long-term implications of SB, risks for obesity, osteoporosis, and urinary tract infections.
Exercises: ROM maintenance, strength improvement.
Functional Mobility Training: Transfers, wheeled mobility, ambulation.

Spina Bifida Physical Therapy Precautions

Decreased Cardiorespiratory Endurance:
Fracture Risk: Increased risk of fracture due to osteoporosis.
Hip Dislocation and Skin Breakdown: Increased risk due to decreased sensation.
Latex Allergy:
Incontinence During Exercise:
Thermoregulation Difficulties:
Fall Risk: Increased risk of falls
Spasticity: Increased spasticity with exercise.

Spina Bifida Management: Evidence-Based Practice

Upper Extremity Strength Training:
- Increases strength and independent wheelchair mobility in children.
Treadmill Training:
- Improves gait speed and aerobic fitness in ambulatory children.

Spina Bifida Mobility Training Using Braces

Hip KAFO (Knee-Ankle-Foot Orthosis):
RGO (Reciprocating Gait Orthosis):
KAFO (Knee-Ankle-Foot Orthosis):
AFO (Ankle-Foot Orthosis:

Spina Bifida Weight Bearing Opportunities

Stander: Allows for upright weight bearing.
Parallels: Provides support for weight bearing activities.

Spina Bifida Gait Aids

Walker:
Twisting Cable:

Spina Bifida School Time

Seating: Classroom seating and the need for adaptive seating or standing devices.
Transfers: Assessment of functional transfers (sit-to-stand, floor-to-stand).
School Function Assessment (SFA): Assesses how impairments affect tasks like moving around the school, interacting with others, and using classroom materials.
Wheelchairs:

Spina Bifida Adolescence

Mobility Needs: Assessment of energy expenditure and social/environmental goals.
Obesity Prevention: Optimize activity to minimize obesity risk.
Self-Reliance Training: Stretches, pressure care, continence management.
Overuse Management: Early degenerative changes in shoulders and other heavily used joints (OA, ligamentous instability).

Spina Bifida Summary

Neural Tube Defect: Results from a defect in the formation of the neural tube in the embryo.
Neurological Deficits: May present with spinal and brain deficits.
Musculoskeletal Deformities: Associated musculoskeletal deformities.
Complications: At high risk of complications due to abnormal movement during growth.
Management: Requires lifelong management by a multidisciplinary team including a physiotherapist.

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Description

Explore the key aspects of Spina Bifida, a neural tube defect that impacts embryonic development. This quiz covers its definitions, etiologies, types, and genetic factors associated with the condition. Test your understanding of this important topic in developmental biology and medicine.