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______ ______ is a birth defect / congenital malformation in which the vertebral column is open; often with spinal cord involvement
spina bifida
spina bifida occurs in utero around ___-___ weeks gestation
3-4
the degree to which neural elements are involved & the level of the spinal cord affected define the _______ of the loss of motor & sensory function around and below that level
severity
diagnosis during pregnancy → _______ ________ = a protein produced by the baby & measured by the amount in the mother's blood
alpha-fetoprotein (AFP)
true or false: low levels of AFP indicate spina bifida
false - high
diagnosis during pregnancy → ultrasound imaging displays either _____ sign or _____ sign
banana; lemon
(banana / lemon) sign displayed on ultrasound:
- chiari II malformation
- cerebellum is tightly wrapped around brainstem
- form of SB tethering that prevents downward migration of posterior brain
banana
(banana / lemon) sign displayed on ultrasound:
- chiari II malformation
- concavity of the frontal lobe
- also called scalloping of the frontal lobes
lemon
_________ is another procedure used to diagnose SB during pregnancy where a sample of the amniotic fluid can be taken & levels of AFP are measured
amniocentesis
exposed spinal cords are exposed to the toxicity of the ______ ______ which can result in the degradation of the arteries supplying the spinal cord
amniotic fluid
true or false: damage to the spinal cord is progressive, so the earlier the intervention, the better
true
true or false: early intervention cannot stop the leak of CSF from the spinal cord
false
what is the main risk factor for spina bifida?
reduced folic acid intake
the following are _____ _____ for spina bifida:
- maternal anticonvulsant therapy
- maternal obesity
- diabetes mellitus
- fungal infection
risk factors
which type of spina bifida?
- least serious & most common
- discovered only on xray or scan
- most people never become aware
occulta
which type of spina bifida?
- next most serious type
- meninges pass back through the opening of the spine
- cyst-like swelling forms
meningocele
which type of spina bifida?
- most serious type
- spinal cord is enclosed in the cyst
myelomeningocele
_________ is the clinically most significant type in which the spinal neural tube fails to close during embryonic development
myelomeningocele
in myelomeningocele, the vertebrae at the level of the lesion lack ______ ______, and so are incomplete dorsally
neural arches
in myelomeningocele, the protruding sac contains _____ fluid, _______ and ______ tissue
spinal; meninges; neural
true or false: the etiology of myelomeningocele is unknown, but may be due to a combination of genetics & environment
true
there appears to be a link between limited ______ and _____ _____ intake and neural tube defects such as MMC
vitamin; folic acid
a lesion in the spinal cord continues to leak CSF which causes a congenital brain malformation → _____ _____ ________
chiari II malformation
chiari II malformation → when the _______ is displaced inferiorly beyond the foramen magnum, causing partial blockage of CSF
brainstem
excessive CSF builds up in the ventricles, causing pressure on the CNS tissue that can further compromise brain development → which ventricle is build up commonly seen in?
4th ventricle
________ → an excessive accumulation of CSF dilating the cerebral ventricles after the primary spinal site is closed
hydrocephalus
effects of _______ include impairments in:
- speed of processing
- immediate registration
- learning & memory
- organization
- high level language
hydrocephalus
hydrocephalus → the cumulative pattern of ______ deficits over time becomes apparent as the child is expected to be more functionally independent
cognitive
________ - ________ ______ → plastic catheter that routes excessive CSF from ventricles to the peritoneal cavity to be reabsorbed
ventricular-peritoneal shunt
true or false: a VP shunt can become clogged, shift, or become dysfunctional because of child's growth
true
_________ → abnormal widening of the central canal due to increased pressure of the CSF over the spinal cord or a result of the chiari II malformation
hydromyelia
hydromyelia results in increased weakness in the ____ muscles; particularly in the _____
UE; hands
during growth, the spinal cord may _____ as a result of the development of adhesions or bony spurs at the lesion closure site
tether
spinal cord tethering → excessive ______ to the spinal cord can cause metabolic changes & ischemia of neural tissue with degeneration of muscle function
stretch
the following are signs of _____ ______ ________:
- change in sensation or continence
- inability to perform tasks that child was previously capable of
- spasticity in muscles with sacral nerve root innervation
- development of scoliosis at a young age
- reduced activity tolerance
spinal cord tethering
surgical intervention → the protruding tissue is removed & spinal opening is closed either in-utero (_______ repair) or shortly after birth (______ repair)
prenatal; postnatal
in-utero / prenatal repair at ____ weeks of gestation was found to decrease need for postnatal VP shunting & improve motor function
26
the following are risks after ______ repair:
- ruptured membranes
- preterm delivery
- unknown urologic function
prenatal
_________ → result of a spastic bladder wall; pressure from this spasticity causes urine to reflux toward the kidney
hydronephoresis
true or false: pressure injuries are common complications of spina bifida due to impaired sensation
true
in SB, there may be decreased cardiorespiratory endurance and apnea which may be caused by _______ dysfunction & limited mobility
postural
impairments to the ______ ______ in SB include:
- balance
- sensation in trunk, UE, and LE
- innervation to UE or LE (weakness or paralysis)
- bowel & bladder control
- proprioception
- visual deficits including decreased depth perception
nervous system
impairments to the ______ _____ in SB include:
- impaired posture & gait
- decreased UE & LE strength
- LE paralysis
- contractures
- scoliosis or kyphosis
- hip dislocation
musculoskeletal system
______ ______ ______ → superior dislocation of the talonavicular joint; also known as rocker-bottom foot deformity
congenital vertical talus
orthopedic abnormalities associated with SB include knee flexion or extension contractures & (varus / valgus) deformities in addition to IR or ER of the hips
valgus
cessation of ambulation occurs during the later years of the _____ ______ of life
first decade
true or false: the level of the lesion is a large factor in ambulation cessation
true
typically, the more _____ the lesion, the later ambulation cessation occurs
caudal
children with ______- level lesions should be encouraged to ambulate with appropriate orthoses and assistive devices
lumbar
physical therapy interventions focus on strengthening innervated muscles & teaching ________ movement patterns along with postural control
compensatory
which outcome measure is used to determine if discrepancy is present and to monitor motor skills?
peabody developmental motor scales -2 (PDMS-2)
the PDMS-2 assess _____ and _____ motor development
gross; fine
what is the age range for the PDMS-2?
birth - 5 years
which type of test is the PDMS-2?
norm-referenced