Spina bifida Flashcards
define the diagnosis of spina bifida and co-existing conditions
describe the impairments associated with this diagnosis
discuss tests and measures for this diagnosis
identify common medial management procedures and PT interventions for this diagnosis
apply knowledge of PT management through a case exercise
what is spina bifida
Spina Bifida is a type of neural tube defect that occurs when one or more vertebrae fail to fuse at approximately 28 days of gestation, leaving the spinal cord unprotected in utero.
90-95% dont have a family history of spina bifida.
neural tube development
neurulation in weeks 3 and 4 of gestation
what is the difference between open and closed spinal dysraphism?
Open spinal dysraphism (OSD) – Meninges and/or nerve tissue are exposed to the external environment. Most prevalent.
Closed spinal dysraphism (CSD) – Meninges and nerve tissue are covered by skin. Mildest form of the condition.
what is spina bifida occult?
hair tuff at the level of the injury
what is spina bifida meningocele?
spinal fluid at the level of injury
what is lipomyelomeningocele
fatty tissue at the level of injury
what is myelomenigeocele?
spinal nerves
99% of all case
what can be used to diagnosis prenatal SB?
13-15 weeks - blood lab looking for alpha fetoprotein 80% reliable
16-24 weeks fetal ultrasound >90% reliable
16-18 weeks amniocentesis 100% accurate
how do you determine the complexity of SB?
Complications can range from minor to severe physical and mental disabilities.
Impact determined by:
Size/Location of malformation
Open vs closed
Which spinal nerves are involved
most common for L5-s1 to be affected
The higher the malformation occurs on the back, the greater the amount of nerve damage and loss of muscle function and sensation.
what are the causes and risk factors of sb?
Exact cause is still an unknown. There are likely multiple factors:
Genetic
Nutritional
Environmental
Folic acid (vitamin B9), when taken by the mother prenatally reduces the risk of neural tube defects by up to 70%.
FDA/CDC Recommendation – all women of childbearing age ingest 0.4 mg (400 µg) of folic acid every day. Dietary sources include dark leafy greens, whole grains, and legumes.
What are the neuromuscular, sensory, and motor deficits in spina bifida?
Loss of sensation
Loss of muscle strength and control
Loss of bowel/bladder control
Muscle contractures
what are thew skeletal impairments associated with SB?
Foot abnormalities (eg, club foot)
Shorter legs
Decreased bone density
Scoliosis
Hip subluxation and dislocation
what is hydrocephalus and what are the symptoms of it?
Caused by a disturbance of formation, flow or absorption of cerebrospinal fluid.
Approximately 25% at birth, which increases up to 90% who will require a ventriculoperitoneal (VP) shunt.
Signs & Symptoms:
Headaches
Nausea
Lethargy
Vision problems
Hoarse cry
Swallow issues
Changes in speech
Seizures
what are the other possible complications of SB?
Hydrocephalus
Arnold Chiari Malformation
Seizures
Cognitive impairments
Tethered Cord
Bowel/bladder complications
Latex allergy
73% have a latex allergy
what is the Arnold-chiari malformation type 2?
Portion of the brain stem descends into the cervical spine.
Signs & Symptoms:
Changes in breathing pattern
Swallowing problems (e.g., gagging)
Ocular muscle palsies
Weakness/spasticity in arms
Bradycardia
what is club foot?
20-50% of infants with spina bifida
Many factors:
Muscle imbalance
Contractures
Intrauterine positioning
Spasticity
what is tethered cord
20-50% of children with spina bifida and re-tethering occurs for 10-15%
Caused by the spinal cord becoming fastened to part of the vertebral column which results in the spinal cord becoming abnormally stretched with the child’s growth.
Signs and symptoms:
Weakness
Scoliosis
Pain
Orthopedic deformity
Urologic dysfunction
Change in function
how do you test SB patients for motor and sensory lost?
Motor Level determined by which muscle group can be graded a 3/5 MMT scale
name the muscles, involvement level and movement
what are the common assessments for body functions and structure? what do they test?
Goniometry – Range of motion
Myotomes and Dermatomes
Hand-Held Dynamometry or MMT– Muscle strength
Gait and Movement Analysis – Gait deviations
what are the common assessments for activity and participation and what do they asses?
Alberta Infant Motor Scale– Motor function
Pediatric Evaluation of Disability Inventory (PEDI) – goal setting
WeeFIM – validated for use with children with spina bifida
School Function Assessment – Participation in school
Spina Bifida Health-Related Quality of Life
what is the movement analysis looking for?
Observe differences in spontaneous leg movements
what does motor development look like compared to a normal child for the first 6 months
motor development is close to typical (head control and hand use) but less LE movement
what does rolling look like for a child with SB?
Rolling – Children with thoracic level SB usually roll by 18 months with compensatory strategies, many will progress to crawling
what does sitting look like for a child with SB?
Sitting – Children with mid-lumbar SB can sit with support with delay and increased lordosis, typical development in L4-L5 lesions
what does pulling to stand and cruising look like for a child with SB?
Pull to stand/cruise – Children with low lumbar lesions, may progress to ambulation with assistive devices with an average onset of age 3
what are the ambulation predictions in children with SB?
Lower motor level - Need good quadriceps strength
No history of shunt - Cognitive function necessary for ambulation
No history of hip or knee contracture surgery
what is the functional ability at motor level t6-t12?
Wheelchair for mobility
Non-functional ambulation and standing
what is the functional ability at motor level L1-L3?
Wheelchair for mobility
Limited household ambulation
what is the functional ability at motor level L3-L4?
Household ambulation
Limited community ambulation
Wheelchair for long distances
what is the functional ability at motor level L4-L5?
Household and community ambulation
Wheelchair for long distances
what is the functional ability at motor level S1-S2?
community ambulation
all ability to ambulate in the community
what might some gait patterns look life for a child with SB?
trendelenburg pattern:
Hip abductor weakness (Motor innervation L4-S1)
Associated with weak hip extensors and plantar flexors
Trunk sways side to side
Knee valgus
there might be excessive dorsiflexion causing the slap foot gait and crouch gait
when using thkfo or um canadanian cruches the gait will look like a swing through
gait patterns later in life look like a swing through gait. Using one crutch or walking along is exxesice trendlenburg and knee valves
what is the multidisplanary approach?
Rehab Medicine
Physical Therapy
Occupational Therapy
Neurosurgery
Orthopedics
Urology
Colorectal/GI
Nurse Clinic Coordinator
Nutritionist
Social Work
Neuropsychology
what is the first step in medical management for SB?
First Step
Fetal or Post-natal Surgery
The management of myelomeningocele study: Fetal surgery leads to improved mobility and fewer surgeries for shunt placement (Houtrow, 2020)
con: fetal surgery is a high risk for the baby and the mother
what is the next step after fetal surgery in medial management?
Surgery to place shunt
Small hollow tube that drains cerebrospinal fluid from the brain
Additional surgeries are needed as the child outgrows the shunt or it becomes clogged or infected
10-30% have seizure activity – associated with brain malformation or CSF shunt malfunction or infection
what are common orthopedic conditions seen in patients with SB?
Scoliosis and/or kyphosis
70% with lesions above L2
Once detected – obtain spine x-ray every 1-2 years, may consider surgery when Cobb angles >40 deg
Surgical options include fusion or growing rods
Hip subluxation and dislocation
Fractures 11-30% in children with SB related to osteopenia (most common: distal femur and femoral neck)
what does early intervention of SB look like?
Monitor joint alignment, muscle imbalance, and development of contractures
Use stretching, orthoses, and positioning splints to maintain alignment
Strengthen trunk and neck with tummy time and prone activities
Determine orthotics for ambulation as soon as they pull to kneel/stand
Power mobility can be introduced around 12 months of age
how does Partial Body Weight Supported Treadmill Training help with early intervention in patients with sb?
Enables parents to provide high repetition and functionally relevant activity
Builds muscle strength and motor control
Improves bone density
Enhancing sensory input led to more steps : velcro, friction and visual flow treadmill tracts
Children walked ~2 months sooner
what are the recommendations for a child with spina bifida? 1-5 years old
monitor for change:
Neurologic level, strength, gait, sensation, bowel/bladder function, and musculoskeletal changes
Get them up right and weight bearing everyday: Standing frame and mobility device if not pulling to stand
Discuss mobility options: Ambulation with orthotics and/or assistive device
Manual vs. power wheelchairs
use hkafo, KAFO, GRAFO, solid AFO, SMAFO
Stander, mobile stander, manual wheelchair, reverse walker, forearm crutches
what are the recommendations for a child with spina bifida? 6-12 years old
monitor for change: Neurologic level, strength, gait, sensation, bowel/bladder function, and musculoskeletal changes
discuss benefits of different mobility devices for different settings: school home community
flexibility and strengthening exercises: Emphasize UE strength to improve w/c mobility and functional activities
explore adaptive recreational activities: For promotion of daily physical activity and weight-bearing activities
ambulation in children with spina bifida?
Some children with spina bifida will gain the strength and motor capacity to walk. Average onset is age 3.
Most likely will need to use a mobility support device.
Many children will lose this capacity later in life. Average offset is age 11.
Motor Function into Adulthood with Spina Bifida
Considerations for PT management and family education:
Increasing body size
Joint degradation
Decrease in bone density
Painful movement
Motivation
Independent living: 30-60%
Employment rate: 25-50%
Discuss some key takeaways from the lecture.
Spina Bifida is a neural tube defect and the most prevalent type is myelomeningocele
There are several neuromuscular, sensory, motor, skeletal, and other medical complications associated with this diagnosis
Motor development is delayed and functional ability is dependent on the lesion level
Consider the materials/equipment you use since >70% of individuals with spina bifida have a latex allergy
Follow the Guidelines for Care from the Spina Bifida Association
Promote physical activity and weightbearing activities
