Spina Bifida Flashcards
At what day does the neural tube normally begin to close?
day 21
the neural tube completes closing at day?
28
what are the 4 types of mylodyplasia?
-occulta
-meningocele
-menigomyelocele (MM)
-myloschisis
name the types of mylodysplasia that do NOT cause paralysis vs. the ones that do
NO Paralysis:
-ocuculta (hair tuft only)
-meningecele (skin covered)
Paralysis:
-meningomylocele (may or may not be skin covered)
-myeloschisis ( not skin covered, spinal cord exposed)
Which is the most common type of myelodysplasia?
MM
MM may be associated with genetic abnormalities such as:
trisomy 13, 18 and 21
what are the common risk factors for MM?
-Alchohol intake
-Anticonvulsants
-Maternal pre-gestational IDDM
-Maternal BMI of > 29 (obesity not overweight)
What maternal condition is a risk factor for MM?
Maternal pre-gestational IDDM
(Insulin Dependant Diabetes Mellitus)
A supplement taken 3 months before conception to reduce the risk of MM, what is it?
Folic acid
What is the role of folic acid in preventing spina bifida?
it reduces the risk of neural tube defects when taken before pregnancy
At which stage of pregnancy can MM be diagnosed?
18 weeks
How is MM typically diagnosed during pregnancy?
-Maternal serum alpha-fetoprotein testing (> 2.5 MoM)
-Ultra sound
-Amniotic fluid analysis
Ultrasound is a diagnostic tool used to determine if there are any cranial malformations in a fetus with MM. What could this malformation be?
Chiari II
(posterior cerebellum displaced downward through the formamin magnum, and the brain stem structures also dispalced in the cudal direction (in the note))
(a deformity where the cerebellum and brain stem herniate through the formen magnum (a simpler definition))
Why is C-section birth safer for a fetus with SB?
less risk for infection and damage to nerual sac
Management of Myelomeningocele Study (MOMS): In-utero repair of the MM Sac
what are the key benefits of in-utero repair of the MM sac?
-less need for shunting
-reversal of hindbrain herniation (AKA: Chiari malformation)
-greater likelihood to walk
-motor function 2 or more levels better than expected by anatomical level
Risk factors of in-utero repair of the MM include:
-spontaneouse rupture of the mambrane
-premature delivery
-oligohydraminos (deficiency of amniotic fluid)
Name SOME musculoskeletal deformities in children with SB
-hip dysplasia, subluxation, dislocation
-Talipes equinovarus TEV (add-varus-supination-PF)
-calcaneal valgus (abd-valgus-pronation-DF, (flat feet))
what is a motor level?
it refers to the lowest intact, functional neuromuscular segment
in the myelodysplasia study group criteria (IMSG), what is type of assessment used to determine the motor level?
functional assessment of muscle groups, (must be more or equal to grade 3 to be considerd a motor level)
slide 12
what are common mesculoskletal contractures ?deformities associated with high level lesions? (thoracic -L2)
-hip flexion, abduction , external rotation contracture
-knee flexion contracture
-ankle PF contracture
-lordosis of the lumbar spine
-scoliosis (present in 90% of cases)
slide 14
slide 15
This type of standing frame is used for patients with lesions at which level?
Thoracic lesions (high level lesions)
weak hip movements are associated with lesions at which level?
L1-L2 (high lumbar)
What type of ambulation is possible for children with L1-L2 lesions?
Short-distance household ambulation using KAFOs or RGOs with upper limb support.
What is the primary mobility device used for community distances in L1-L2 lesions?
wheelchair
What orthotic devices are recommended for children with L1-L2 lesions?
KAFOs or RGOs and upper limb support
What is the primary characteristic of an L1 motor level lesion?
“Weak iliopsoas muscle (grade 2).”
What distinguishes an L2 motor level lesion from L1?
At L2, the iliopsoas, sartorius, and hip adductors are grade 3 or better.
L1-L2 lesions presentation
Exceeds criteria for L1, but does not meet L2 criteria
Reciprocating gate orthosis (RGO) is suitable for children with SB that have a lesion at what level
Upper lumbar
What are common musculoskeletal deformities in mid to low lumbar lesions (L3-L5)?
-Hip and knee flexion contractures
-increased lumbar lordosis
-scoliosis (present in 40% mid lumbar, 10% lower lumbar)
-genu and calcaneal valgum (genu valgum+flat feet)
-pronated feet when bearing weight. (Flexible flat feet)
-crouch gait (this is the cause of hip/knee contractures)
What is the primary gait pattern seen in children with mid to low lumbar lesions?
A pronounced crouch gait with weight-bearing primarily on the calcaneus.
What orthotic device is typically required for L3 motor level lesions?
KAFOs and crutches for household and some community ambulation; wheelchairs for longer distances.
How do children with L4 motor levels initially learn to walk?
With KAFOs and walkers
After learning to walk, how do children with L4 motor levels ambulate?
AFOs and forearm crutches, wheelchairs for longer distances
What percentage of individuals with L3 lesions achieve independent living as adults?
60%
What percentage of children with L4 lesions continue to ambulate as adults?
20%
Slide 22
Slide 22
What is a common characteristic of L5 lesions in terms of ambulation?
They can ambulate without orthoses but may need them to correct foot alignment and substitute for lack of push-off (toe off,PF)
What is the recommended assistive device for children with L5 lesions during community ambulation?
Upper limb support such as crutches to decrease energy expenditure and ensure safety
Unless upper limb support is used, what would be evident in children with L5 lesions?
Gluteal lurch
A wheel chair or bike can be used for long distances in the case of a lesion at
L5
What percentage of individuals with L5 lesions achieve independent living as adults?
80%
What are common musculoskeletal presentations in children with sacral lesions?
-mild hip and knee flexion contractures
-increased lumbar lordosis
-and ankle/foot deformities such as varus or valgus with a pronated or supinated forefoot
What is the typical gait pattern in children with sacral lesions?
Mild crouch gait, with weight-bearing primarily on the calcaneus unless plantar flexor muscles are at least grade 3/5.
What level of ambulation is possible for children with S1 motor levels?
They can walk without orthoses or upper limb support but have weak push-off when running or climbing stairs.
What is the recommended orthotic device for children with S1 lesions?
FO or AFO to improve foot alignment.
What is a common gait abnormality seen in children with S1 lesions?
Mild to moderate gluteal lurch.
What functional deficits are typically seen at S2 motor levels?
Decreased push-off strength and reduced stride length.
What are the physical characteristics of children with S2-S3 motor levels?
No obvious MOTOR deficits
Bowel and bladder dysfunction occurs in lesions at what level?
From T1-S5
No loss means?
That there is no bowel or bladder dysfunctions, meaning the sacral plexus was spared (not injured)
Why do sensory deficits in spina bifida not always correlate with motor levels?
Sensory loss may skip levels and does not always match the motor lesion level. Which is why it is important to test all dermatomes and multiple sites within a dermatome (for older kids)
What sensory tests are recommended for children with spina bifida?
Light touch, pinprick, vibration, and proprioception tests.
What is the primary educational focus for children with sensory deficits?
Teaching skin protection techniques and pressure relief to prevent injuries.
What co morbidity occurs in 25% or more of kids born with MM?
Hydrocephalus
What percentage of children with spina bifida develop hydrocephalus after back lesion closure?
60%
What percentage of children with hydrocephalus require shunt placement?
80-90%
What may Hydrocephalus affected?
Cognition
What device is commonly used to manage hydrocephalus?
A ventriculoperitoneal (VP) catheter /shunt.
How does a ventriculoperitoneal shunt work?
It drains excess cerebrospinal fluid (CSF) from the brain ventricles into the peritoneal cavity for reabsorption.
What are early signs of shunt dysfunction? SHORT NOTE
-changes in speech
-decrease school performance
-recurring headache
There are many more, check slide 28
who is 90% more likely to develop hydrocephalus?
children with spina bifida and chiari II malformation
Surgical Management of Chiari II Malformation includes:
Posterior fossa decompression and
Cervical spinal laminectomyThis surgery to reduce compression on the cervical spinal cord and brainstem.
What is a common cognitive personality trait in children with hydrocephalus?
“Cocktail party personality” – verbose speech with excessive and inappropriate use of jargon and clichés.
What are common causes of upper limb dyscoordination in children with spina bifida?
-cerebellar ataxia due to chiari II
- motor cortex or pyramidal tract damage (UMNL secondary to hydrocephalus)
-motor learning deficits
What is the cause of motor learning deficits in children with SB?
Use of upper limbs for balance and support rather than manipulation and exploration
Mention some co-morbidities of SB
-cognitive dysfunction
-language dysfunction
-latex allergy (73% gave it, so no balloons or gloves!)
- UL dis-coordination
-spasticity and seizures
-neurogenic bowel and bladder
-skin break down and obesity
How is neurogenic bladder typically managed in children with spina bifida?
With clean intermittent catheterization on a regular schedule.
What are common issues related to neurogenic bowel in children with spina bifida?
Incontinence, constipation, and impaction.
Why is it that only 5% of kids with MM develop voluntary bladder and bowel control?
Because in most cases S2 to S4 is not functioning
When is a bowel program successful?
In the presence of a cutaneous reflex
Which OM is used to set goals for kids with SB?
Canadian Occupational Performance Measure (COPM)
Some PT treatment for SB include:
-passive ROM (start early to avoid contractures)
Strengthening exercises (using body weight then progress to external weight )
What positions will a child with SB be restricted to post-op?
Prone and sidelying positions
What is the goal from treatment of spinal deformities?
Maintain a balanced trunk and pelvis to optimize function and posture.
What orthotic device is commonly used to manage spinal deformities in children with spina bifida?
Bivalve Silastic TLSO (thoracolumbosacral orthosis).
Why is surgical fusion often delayed until after age 10?
To avoid truncal shortening.
At what age is surgical fusion typically recommended for spinal deformities in girls vs. boys?
-Girls: 10-11 yo
-Boys: 12-13 yo
how can PT improve bome mineral density and ROM in children with SB?
standing program 5 days per week for 60-90 min/day
(does NOT improve muscle strength but may maintain it)
