Myelomeningocele Flashcards
(37 cards)
1
Q
Clinical manifestations of myelomeningocele
A
- Loss of sensory and motor function
- Orthopedic abnormalities
- Hydrocephalus
- Brain and SC abnormalities
- Cognitive impairment and learning issues
- Integumentary Impairment
- B&B dysfunction
- Disturbed growth and development
- Psychosocial issues
2
Q
nervous system developement/background
A
- The nervous system develops from a portion of the ectoderm called the neural plate
- Neural plate 🡪 neural tube 🡪 brain and spinal cord
- Closure begins in the cervical region and proceeds cranially and caudally
- Closure is completed by the 26th day
3
Q
etiology/causes of spina bifida
A
- A defect in the neural tube closure and overlying vertebral arches results in spina bifida
- Causes
- No definitive cause
- Genetic predisposition
- Genetic risk factors
- Celtic origin
- Previous spinal bifida births
- Genetic risk factors
- Environmental risk factors
- Lower socioeconomic status
- Deficiencies in folic acid and Vitamin A
- Environmental risk factors
4
Q
incidence and prenatal testing for spina bifida
A
- Incidence: 2/1000
- Prenatal testing
- AFP (alpha-fetoprotein) at abnormally high level in amniotic fluid past 14 weeks gestational age, compared with US results
5
Q
types of spina bifida
A
- Occulta
- Cystica
- Meningocele / myelocele
- Lipomeningocele
- Myelomeningocele
- Anencephaly
- Meningocele / myelocele
6
Q
definition and characteristics of spina bifida occulta
A
- Definition: failure of the vertebral arches to meet and fuse in 3rd month
- Commonly occurs at the lumbosacral area
- Characteristics
- Overlying skin may be marked by dimple, pigmentation, or patch of hair
- Usually associated with no neurological or musculoskeletal dysfunction
- Higher than normal incidence of urinary tract infections
7
Q
spina bifida cystica definition
A
Definition: failure of the neural tube and vertebral arches to close with cystic protrusion of the meninges with or without the spinal cord
8
Q
subtypes of spina bifida cystica
A
- Meningocele/myelocele: spinal cord remains in the vertebral canal but it may be abnormal
-
Lipomeningocele: superficial fatty mass in the low lumbar or sacral area
- high incidence of B&B dysfunction due to tethered cord
-
Myelomeningocele: extensive spinal cord abnormalities
- Most common in thoracic and lumbosacral regions
- Anencephaly: cranial end of neural tube does not fuse
9
Q
clinical manifestations of Myelomeningocele
A
- Loss of sensory and motor function
- Orthopedic abnormalities
- Hydrocephalus
- Brain and SC abnormalities
- Cognitive impairment and learning issues
- Integumentary Impairment
- B&B dysfunction
- Disturbed growth and development
- Psychosocial issues
10
Q
Loss of Sensory and Motor Function in Myelomeningocele depends on
A
- Degree of spinal cord abnormality
- Traction or stretch on the spinal cord
- Trauma to exposed neural tissue during delivery
- Postnatal damage resulting from drying or infection of the neural plate
11
Q
T/F: Loss of sensation below the level of the lesion is impaired but may not exactly match the level of the lesion and will results in deficits in kinesthetic, proprioceptive, and somatosensory problems.
A
True
12
Q
types of motor dysfunction with Myelomeningocele
A
- Complete loss of function below the level of the lesion 🡪 flaccid paralysis, loss of sensation, absent reflexes
- Below the level of the lesion if the spinal cord is intact: mixture of flaccidity and spasticity
- Flaccidity immediately below the lesion to the severed cord
13
Q
orthopedic problems with myelomeningocele causes and types
A
- Deformity/contractures
- Causes:
- Muscle imbalance
- Effect of stress
- Posture
- Gravity
- Congenital malformations
- Decreased sensation
- Neurological complications
- Causes:
- Types of congenital malformations
- Hemivertebrae
- Deformed vertebral bodies/ribs
- Missing ribs
- Lumbar kyphosis
- Club feet
- Rocker bottom feet
14
Q
hydrocephalus incidence, cause, and signs and symptoms with myelomeningocele
A
- Incidence: 80-90%
- Cause: blockage of flow of CSF; typically develops following closure of sack
-
S&S
- Increased head size
- Bulging fontanelles
- Irritability
- Lethargy
- Sunset eyes
15
Q
hydrocephalus intervention and what to watch for
A
- Intervention: VA/VP shunt or shunt revision
- Watch for S&S of shunt malfunction in a child with a shunt (see next slide)
16
Q
clinical manifestations in the infant of hydrocephalus
A
- Bulging fontanelles
- Vomiting
- Change in appetite
- Sunset sign
- Edema
- redness along the shunt tract
- Thinning of the skin over the scalp
- High pitched cry
- Irritability
- Lethargy
- Seizures
- Excessive growth of the head
17
Q
toddler clinical manifestations for hydrocephalus
A
HA
Vomiting
New nystagmus/ new squint
Edema
redness along shunt tract
Irritability
Lethargy
Seizures
18
Q
school aged children clinical manifestations of hydrocephalus
A
- HA
- Vomiting
- Memory changes
- Edema
- redness along shunt tract Personality changes
- Irritability
- Lethargy
- Seizures
19
Q
arnold chiari malformation
A
- Definition: brainstem (medulla, possibly pons and 4th ventricles) and cerebellum herniate through the foramen magnum into the upper cervical canal (C1-4)
- Symptoms
- Respiration
- Paralysis of vocal cords
- Bulbar dysfunction
- Apnea
- Swallowing
- Abnormal gag
- UE weakness
- Treatment: decompression surgery
20
Q
Hydromyelia
A
- Definition: dilation of the center canal of spinal cord
- May be focal, multiple, or diffuse
- Cause: may be a consequence of untreated or inadequately treated hydrocephalus (due to increased hydrostatic pressure from above)
- Effect: may cause pressure necrosis of the spinal cord, leading to muscle weakness and scoliosis
21
Q
Hydromelia symptoms and interventions
A
- Symptoms
- Rapidly progressive 🡪
- Scoliosis
- UE weakness
- Spasticity
- Ascending motor loss in the LE
- Interventions
- Shunt revision
- Posterior cervical decompression
- Central canal to pleural cavity with a flushing device
22
Q
tethered cord
A
- Definition: pathological fixation of the spinal cord in an abnormal caudal location
- Effect
- Mechanical stretch
- Distortion
- Ischemia
- Suspect when: any patient with abnormal neurulation
- Symptoms
- Decreased strength
- Development of LE spasticity
- Back pain at site of sac closure
- Change in urological function
- Intervention: release
23
Q
cog and learning issues characteristics
A
- Visual-motor perceptual deficits
- Difficulties with spatial relations
- Body image issues
- Difficulty with development of hand dominance
- Poor hand function
- “Cocktail party speech”
24
Q
considerations for integ
A
- Latex allergies
- Use latex free gloves, therapy balls, therabands, and tape
- Pressure sores
- Parental training
- Child training
25
bowel and bladder complications
* Teach self catheterization early if possible
* Difficulties due to:
* Body image issues
* Fine motor skills
* Perceptual skills
26
age appropriate spina bifida interventions
* ROM
* MMT
* Sensory testing
* Developmental testing
* Positioning and equipment needs
* Education for family/child
* Balance
* Coordination
* Reflexes
* Muscle tone
* Skin condition
27
prenatal issues with spina bifida
* Possible diagnosis
* Referral to early intervention
* Possible sac closure in utero
* C section to prevent trauma to neural sac
* PT intervention
* Education
* Assistance with referrals
28
newborn issues
* Sac closure within 24-48 hours
* Shunt procedure
* PT concerns
* Assess function to determine baseline pre and post surgery
* Identify muscle imbalances
* Possibly deforming forces
* Identify need for positioning, bracing, taping
* Consider state
* Alert vs. crying
* May need to use tactile or vestibular stimuli to alert the child
* Positioning
* Sidelying or prone to protect surgical incision
* Observe spontaneous movements
* Observe response to sensory input
* Differentiate between reflex and voluntary movement
29
infant toddler concerns for spina bifida
* PT concerns
* Facilitate developmental sequence
* Prevent deformity
* Home program
* Early weight bearing
* Be careful of alignment
* Skin care
* Emphasize balance and equilibrium in sitting/standing/core strength
* Importance of fine motor skills
30
toddler/preschool spina bifida concerns
* PT concerns
* Emphasize progressive LE weight baring and gait training activities
* Self care activities
* Programs
* EI
* School based
* Community
* \* Adapt normal equipment/activities as necessary
* Bladder training
* Strength assessment
* Antigravity control in developmental positions, during play, weighted toys
* Sensory considerations
* Assess light touch and position changes as cognitive and language skills permit
31
adolescent PT concerns for spina bifida
* Children become aware of their differences by age 6
* Constantly reevaluate
* Body grows and muscle strength/length do not keep up
* Equipment needs change
* Skin and ROM issues may increase as more time is spent sitting
* Independent community mobility is a necessity
* W/C prescriptions if not prescribed already
* Prepare family for significant changes in adolescence
32
thoracic levels
Possible muscle function:
* **Abdominals**
* **Intercostals**
* **Erector spinae**
Possible Secondary Impairments
* Flaccid LE (frog leg position)
Orthoses Needed
* Total contact orthosis for standing
* Resting AFO (with age and increased sitting)
Interventions
* Prone positioning
* Daily ROM
* Gentle wrapping
* Surgical intervention (ITB, hip ER, knee flexors)
33
L1-L3 Level
Possible muscle function:
* **Strong hip flexors**
* **Strong adductors**
Possible Secondary Impairments
* Require bracing to stand
* Hip dislocation and subluxation
Orthoses Needed
* Parapodium
* Swivel walker
* A Frame
* RGO
* HKAFO
34
L4-L5 Level
Possible Muscle Function
* **Strong hip flexion**
* **Strong hip adduction**
* **Some hip abduction (glute med/TFL)**
* **Strong quads**
* **Medial HS**
* **Anterior tibialis**
* **Some foot intrinsics**
Orthoses Needed
* Twister cable (due to rotation)
* KAFO if quads are weak
* Floor reaction AFO
35
S1-S5 Level
Possible Muscle Function:
* **Balance at hips/knees**
* **Hip extensors (full/partial innervation)**
* **Lateral HS (full/partial innervation)**
* **Stronger glute med**
* **Stronger medial HS**
* **Stronger quads**
* **Variation**
* **Gastroc-soleus may be weak**
* **Toe flexors may provide some plantarflexion but usually not strong enough for gait**
Possible Secondary Impairments
* Crouched gait with increased weight gain
* Hip instability
Orthoses Needed
* AFO needed for gastroc/soleus
Interventions
* Aggressive treatment for hip instability
36
general considerations psychosocial adjustment
* Family attitudes and environmental considerations will influence the child’s adjustment
* It is important to be given opportunities to interact with peers and age appropriate responsibilities
37
psychosocial adjustment
0-6 months:
parents may be in shock denial/may misinterpret information
6-18 months
* Very stressful
* Lots of doctor appointments and hospitalizations
2 years – school age
* Concerns about toilet training
* Social acceptability concerns
6 years
* Child more aware of disability
Adolescence
* Bowel and bladder management
* Sexual Issues
* Females: potential for orgasm and fertility
* Males: frequently sterile with small testicles and penises; potential for erection and ejaculation depends on the lesion
