Spina Bifida Flashcards
Spina bifida is the ____ most common birth defect, occurring __ per 10,000 live births
second
3.4
What is the proposed etiology of spina bifida?
maternal lack of folic acid during the first trimester
also associated with the seizure medication valproic acid
It is suggested that the pregnant woman takes ___ μg of folic acid during the months prior to conception and ___ μg through the first trimester
400
600
The survival rate of spina bifida is __% with early treatment
90
Excellent prognosis is due to what 3 things?
- he use of antibiotics to limit infection
- The surgical insertion of shunts to manage hydrocephalus
- Early and consistent use of clean, intermittent catheterization to completely empty the bladder
What is spina bifida aka?
Myelomeningocele
Describe the spinal defect that is present in spina bifida patients
There is an external sac on the infant’s back that contains meninges and spinal cord tissue that protrudes through a dorsal defect in the vertebrae
Where does the defect typically occur?
in the lumbar region
What is spina bifida occulta?
o A condition involving nonfusion of the halves of the vertebral arches, but without disturbance of the underlying neural tissue
How can spina bifida occulta be distinguished externally?
by a midline tuft of hair
There is a high rate of what 2 things in spina bifida occulta patients?
tethered cord and urinary tract disorders
What is myelocele?
A protruding sac containing meninges and CSF, but the spinal cord and nerve roots remain intact and in their normal positions which means there typically is no motor or sensory deficits, hydrocephalus, or other CNS problems
There is a high incidence of ______ and _____ dysfunction resulting from a tethered spinal cord
bladder and bowel
What is lipomeningocele?
A superficial fatty mass in the low lumbar or sacral level of the spinal cord
The caudal end of the neural tube closes on approximately day __ of gestation
26
What are the 4 clinical signs of spina bifida?
- Absence of motor and sensory function (usually bilateral) below the level of the spinal defect
- Loss of neural control of bowel and bladder function
- Higher motor or sensory level on one side than on the other
- Functional deficits may be partial or complete
What can be defined as an abnormal accumulation of CSF in the cranial vault?
Hydrocephalus
What is the keystone of Hydrocephalus?
bulging fontanels
What are the 4 causes of hydrocephalus?
- Overproduction of CSF
- A failure in absorption of CSF fluid
- Obstruction in the normal flow of CSF through the brain structures and spinal cord
- Arnold-Chiari malformation
What is considered to be the primary cause of hydrocephalus in children with spina bifida?
Arnold-Chiari malformation
What is Arnold-Chiari malformation?
It is a deformity of the cerebellum, medulla, and cervical spinal cord in which the cerebellum sinks into the posterior aspect of the cranium which results in the posterior cerebellum being herniated downward through the foramen magnum displacing brainstem structures caudally
Management of the neonate with spina bifida typically depends on the extent of what 3 things?
- neurologic impairment
- hydrocephalus
- kyphoscoliosis/scoliosis
Early and aggressive surgical intervention is supported and it is now acceptable for the child’s back to be closed within __ hours of birth
72
Prior to surgery is PT intervention necessary?
An evaluation may not be imperative, but an assessment can offer insight into the severity of any ortho problems present at birth
Hydrocephalus is a common side effect that occurs in __% of children with spina bifida
90
Typically how is hydrocephalus managed?
via shunting
What are the 2 types of shunts?
- Ventriculoatrial (VA) shunt
- Ventriculoperitoneal (VP) shunt
The VA shunt moves excess CSF from one lateral ventricle to where?
the right atrium of the heart
The VP shunt moves excess CSF from one lateral ventricle to where?
free abdominal space
Which shunting technique is preferred?
VP
When should MMT be performed before and after surgery?
- Before surgery
- 10 days after
- at 6 months
- yearly thereafter
What is the goal of early MMTs?
to assist the medical staff to identify the level of the back lesion by assessing LE movement or lack thereof
What is a motor level assigned according to?
The last intact nerve root found (lowest level)
What movements can be mistaken as voluntary in infants with thoracic level spina bifida?
Reflex movements at the knee (flexion) or ankle (PF/DF)
Why is this reflex movement a concern?
It is usually unopposed by an active antagonist at the same joint and can therefore cause deformity
Extreme tightness of which muscle group may be evident in the child with a motor level at L2-L3 or L3-L4?
hip flexors
Extreme tightness of which muscle group may be evident in the child with a motor level at L5?
dorsiflexors
Typically what position are ROM exercises performed in?
prone or side-lying
Post-op ROM exercises should be performed 2-3 times per day with the PT’s hands close to the joint being moved which will make a ____ lever arm
short
True or False
Aggressive stretching should always be avoided
True
True or False
You should stroke the plantar surface of the baby’s foot in order to get them to react
False
They have no sensation on the bottom aspect of their feet
Where should sensory stimulation be done at?
thigh or trunk
How can you tell if a child’s shoe or orthosis does not fit correctly?
When areas of redness last longer than 30 minutes
What is the earliest problems noted in a developing infant with spina bifida?
Instability of the head and upper body with delayed or weak acquisition of antigravity movement in all positions, balance, and equilibrium responses
Children with spina bifida who demonstrate poor neck stability may retain what reflex longer than a typically developing infant?
Moro (startle)
What are 4 reasons why upright positions are emphasized to parents?
- They learn best in these positions through environmental interaction
- Lung function is optimized
- Abdominal activation
- Vestibular Input
Why is early weight bearing essential?
Promotes bone health and joint formation
Describe the child with thoracic level paralysis
They have flaccid lower extremities and at risk for developing a frog-legged deformity (legs are abducted, ER, and flexed at the hips and knees with the feet in PF)
What type of orthosis should be worn in a child with thoracic level spina bifida?
total contact body brace
Describe the child with high lumbar level paralysis
Usually exhibit some active hip flexion and adduction, but usually no other strong movements at the knees or hips are present
What defect/problem is common in children with high lumbar level paralysis?
hip dislocations
True or False
ROM exercises should be discontinued following hip dislocation
False
Describe the child with low lumbar level paralysis
Usually have strong hip flexors and adductors and poor to good hip abduction strength.
What ____ deformity is common in low lumbar level paralysis?
clubfoot
What is the expected functional level in children with low lumbar level paralysis?
Ambulatory in life unless increased body weight, flexion contractures, poor CNS status, or further complications exist
What type of orthotics are required for ambulation in a child with low lumber level paralysis?
- RGO (if CNS deficit present)
- KAFO (weak quads)
- AFO
Describe muscle involvement in a child with sacral level paralysis
Retain use of all muscles with exception of glute max, gastroc, and foot intrinsics
What is the expected functional level in children with sacral level paralysis?
Independent gait with moderate to miimal deviations based on patterns of weakness
What type of orthotics are required for ambulation in a child with sacral level paralysis?
AFOs
What provides the best assessment of bracing needs?
3D Gait Analysis with MMT
What are 6 signs of CNS deterioration?
- Nausea/Vomiting
- Increased spasticity and tone
- Sunsetting/Doll eyes (unable to attain vertical gaze)
- Decreased balance
- Complaints of headaches
- Excessive crying
What are 2 conditions that may indicate CNS deterioration?
- hydromelia
- tethered spinal cord
What is hydromelia?
A collection of excess CSF in pockets down the spinal cord that created areas of pressure and necrosis of the surrounding peripheral nerves
What does hydromelia lead to?
scoliosis
What are the characteristics of hydromelia?
Progressive upper extremity weakness and hypertonus
How is hydromelia managed?
shunting
What is tethered spinal cord?
Adhesions that are anchored to the spinal cord at the site of the original lesion which does not allow the spinal cord to move resulting in excessive stretch and ischemia of neural tissue
How is tethered spinal cord managed?
surgically reparied
Children with spina bifida have an increased incidence of ____ allergy
latex
Children with spina bifida tend to have a wide variety of cognitive levels which seem to depend on what 3 things?
- treatment of hydrocephalus
- episodes of cerebral infection
- the presence of other CNS abnormalities
What type of special PT is recommended in children with spina bifida and why?
Helps to reduce tone and increase sensory involvement
What are the most common complications in the young adult with spina bifida?
- Obesity
- Incontinence
- Recurrent urinary tract infections
- Chronic decubiti
- Joint pain
- Hypertension
- Neurologic deterioration
- Depression
In conclusion what is the most common cause of morbidity in children with spina bifida?
urinary tract issues
