Increase if mothers obese, diabetic, on antiseizure meds
Prognosis: __% survival perinatally due to __ __. Prevalence has __ due to increased survival.
90%, improved medical management (antibiotics, ventricular shunts and urinary management), increased.
Survival into adulthood is ~85%
How would you repair a lesion?
Surgical placement of neural tissue and meninges within the spinal canal.
Usually done within 24-48 hours of birth (prevents infections and limit the neurological injury)
Can a shunt and closure be performed at the same time?
Fetal (prenatal) surgery has these potential outcomes:
decreases continued neurological damage occurring during the last months of pregnancy.
decrease hindbrain herniation
What hospitals are presently conducting RCT of fetal surgery?
Children's Hospital of Philadelphia
University of California SF
Name some complicating conditions: (4)
Arnold Chiari II
Cranial nerve palsies (VI, IX, X)
Info about hydrocephalus...
25% born with this, add. 60% more will develop after surgical closure.
Associated with arnold chiari II
over 80% require CSF shunting (can be externally programmed to adjust the flow in shunt, variety of ventriculoperitoneal shunting procedures)
Fever, malaise, vomiting/change in appetite, recurring headache, changes in speech, decreased school performance/memory, are symptoms for what?
Hydrocephalus or shunt dysfunction
More: incontinence worsens, difficulties w/ visuomotor/perception, decreased activity level/difficult to arouse in morning, increase irritability, onset or increase incidence or seizures, edema&redness along shunt.
Malformation of cerebellum, pons resulting in caudal displacement of the brain stem through foramen magnum is...
Arnold Chiarii II
CSF flow is disrupted leading to hydrocephalus
possible compression of cranial nerves and brain stem (if severe enough)
Stretch pressure on brachial plexus (if severe enough)
*diff. reasonings for this disease (first two mentioned)
Management of Arnold Chiari depends on __ & __. Can lead to __. outcomes appear better with __ __. What does surgery involve?
severity & progression. Mortality. Early surgery.
Surgery involves decompressing brain stem (remove part of lamina in upper cervical area, may decompress the posterior fossa in older individuals)
Cn VI oculomotor weakness in __ __. CN IX and X = __ & __ dysfunction. What is the management for these CN palsies?
lateral recuts. Pharyngeal and laryngeal dysfunction.
Management - neurosurgical shunting/decompression may be necessary with mixed impact on CN palsies.
Progressive Neurological dysfunctions with myelodysplasia include...
tethered spinal cord
Definition, symptoms, and management for hydromyelia include:
Excessive CSF accumuluation in the spinal cord
Symptoms: Rapid increase scoliosis due to peripheral n. damage to spinal muscle innerv. Increase weakness & perhaps spasticity in the UE's.
Management - revision of VP shunt; spinal shunting
With increasing scoliosis you can suspect hydromyelia or thethered cord if lesion is below T12. Above T12 ....
Child may not have the musculature to support spine.
Symmetrical BOS in sit-to-stand, Sitting orthosis or spinal bracing, surgical fusion.
Hip dislocation: most common in high levels due to flaccidity (T12) or L2-3 due to unopposed action of hip flexors and adductors. characteristics?
Varied opinion on whether to surgically intervene as children may lose function.
If not painful, bilateral and pelvis remains symmetrical, surgery may not be indicated.
Convulsive disorders: Occur in __-__%. Happens secondary to __ __ ___ or __; residual injury or brain malformaiton. Management: ____ meds.
10-30%, CSF shunt malfunction or infection. Management: anticonvulsant meds.
Neurogenic bowel and bladder notes:
Fewer than 5% of children develop voluntary control of urinary and anal sphincter.
Training programs can be effectively administered by the time child is 7-8 y.o.
Ind. bowel programs managed by: diet, timing, and laxatives and stimulants.
Bladder: is clean intermittent catheterization effective? What can you do for infection concern? What else can you say about meds and therapy?
Effective. Infection concern: short courses of prophylactic antibiotics.
Meds to supplement program for spastic or hypotonic sphincters are 65% successful.
Variety of injection therapy&surgical alternatives if can not be managed more conservatively.
Increased demineralization due to flaccid paralysis and secondary stimulation from stand is what disease?
studies have not found that standing programs alone, significantly impact the progression of osteoporosis.
Motor paralysis: motor level is the highest intact functional neuromuscular segment, T or F
International myelodysplasia study group criteria for assigning motor levels that more accurate than traditional neurosegmental levels used for spinal cord injury.
What are the varieties of motor paralysis?
Complete, incomplete, "skip" levels (one or more caudal segments are functioning below last functional level), higher levels may have some degree of spasticity.
Children should be monitored as motor changes are a frequent sign of neurological complication.
What are some sensory losses?
Losses are not always consistent with motor levels.
Sensory impairment impact functional use (big impact)
Integumentary and safety concerns
Should be monitored as frequent sign of neurological complication.
Characteristics of musculoskeletal deformities...
Often have he primary impact on fx
Resulting from muscle fx imbalance & resulting positioning or compensations
impact on positioning, weight bearing, ADL, energy expenditure, mobility.
Focus for prevention and intervention in therapeutic programs.
Musculoskeletal deformities of the extremities and trunk include:
Extremities: position of comfort and in direction of muscle imbalance.
Trunnk: scoliosis, kyphosis, lordosis.
Motor incoordination is secondary to ?
Arnold chiari II - cerebellar ataxia.
Motor cortex or pyramidal tract dysfunction - primary or secondary to hydrocephalus
Motor learning deficits and cognitive dysfunction.
T or F: higher functioning is related to higher level lesions and uncomplicated neurological history.
False - lower level lesions.
Complicated by UE incoordination and memory deficits.
What are some aspects of the Hx part of the examination that should be touched on.
Family and prenatal, postnatal medical, developmental, previous interventions (PT and related support), educational, complaints and concerns that bring child of family for intervention.
What systems could be affected? (Important as associated with additional congenital abnormalities and impact of sensorineural deficits)
integumentary (skin breakdown; obesity; pt knowledge of skin care routine)
Cariopulmonary (secondary to activity level and musculoskeletal deformities of the spine)
Tests and measures: ROM, joint alignment and integrity can work on...