Pathology
when does myelodysplasia/spina bifida occur
occurs during neurulation (28th day after conception)
which type of spina bifida is a secondary neurulation defect that occurs from 28-32 days to 7 weeks of gestation, defect at L5-S1 from failure of vertebral arch to grow and fuse, only sign may be hemangioma dimple, lipoma, hair tuft
spina bifida occulta
test dermatomes to get an accurate baseline
document areas that are absent and decreased sensation for: light touch, pinprick, vibration, and thermal
evaluate proprioception and kinesthetic
decreased safety requires: education on checking water temperature and skin inspection/pressure relief techniques
which surgery is controversial and what are the requirements for it
hip surgery
prerequisite for hip surgery in children with MM:
lesion level at or below L3, fixed a pelvic obliquity caused by unilateral subluxation, dislocation interferes with sitting or standing, contributes to scoliosis,skin care is unmanageable, painful hip dysplasia in ambulatory
what are some interventions to improve function
strengthen weak muscles, increase endurance, increase efficiacy of performing tasks, protect weight-bearing joints, prolong mobility, work on age-appropriate developmental activities, work on activities that are problematic for the child, teach compensatory skills
is the main reason that spina bifida arises
most arise from failure of closure of caudal neuropore; 80% occur in lumbar area
which type of spina bifida is a sac with meninges that protrudes trough vertebral defect usually no paralysis
UE: shoulder girdle overuse
trunk: scoliosis (congenital or acquired), kyphosis, lordosis
LE: malalignment and contractures
what are some posture assessment could we find in standing
ankle pronation-calcaneal valgus
genu valgus
hip and knee flexion contractures
increased lumbar lordosis
what motor function is at the thoracic level, and what equipment could we use
intact innervation of: neck, upper limb, shoulder girdle, trunk (unless above T10)
weak lower abdominals, absent lower limb movement, decreased sitting balance, decreased respiratory function
Equipment: parapodium- for non functional ambulation, WC
what should pregnant women have in their diet to prevent spina bifida? what is the recommendation for how much they should take?
folic acid, they should consume 400 micrograms (0.4 mg) of folic acid daily-> higher recommendations of 4 mg from 3 months before pregnancy through first trimester for women with previous child with NTD
which type has a sac with spinal cord and/or nerve roots and meninges protrudes through vertebral defect, associated with nerve paralysis
spina bifida aperta- myelomeningocele
what is a chiari malformation and what are some symptoms
deformity of cerebellum, medulla and cervical spinal cord
posterior cerebellum herniates downward through foramen magnum
symptoms: neck p!, balance problems, muscle weakness, numbness, difficulty swallowing, vomiting, HAs, poor or weak cry
what is the minimum age for girls and boys when considering a spinal fusion
10-11 years old for girls
12-13 years old for boys
what motor function and equipment could we see for a high lumbar (L1-L2) level? L3?
L1-L2-> weak hip flexors, adductors, and rotators; potential for household ambulation; equipment: with UE support the use of KAFOs or RGOs; WC
L3-> strong hip flexion and adduction strength, potential for ambulation household and short community distances; equipment: assistive device, KAFOs, forearm crutches, WC
what are some common characteristics we could see in a child with spinal bifida
varyig degrees of muscle weakness, paralysis, loss of sensation in legs, and/or poor bladder and bowl control
some may have hydrocephalus
for motor paralysis what are 3 neuromuscular involvements that can happen
complete cord transection- manifest as normal function down to a particular lvl
incomplete lesions- mixed manifestations of spasticity and volitional control
skip lesions- more caudal segments are functioning despite the presence of one or more non-functioning segments interposed between the intact more cephalad spinal segments
why is skin breakdown important with children diagnoses MM
skin breakdown occurs in 85-95% of all children with MM by the time they reach young adulthood
physical therapy is important for education and instruction on strategies for prevention of skin breakdown and proper cushion for a wheelchair
what are some interventions we can perform for a child with spinal bifida
importance of maintaing joint ROM- perform without excessive force, to avoid fractures
bed mobility, ADL function, transfer, pain management, avoid contractures (reduce the risk for skin breakdown and possible amputation), avoid surgery
what is motor function and equipment at L4 level? L5?
L4: strong ankle dorsiflexion, knee flexion and extension; potential for functional ambulation with wheelchair for long distances; equipment: may initially need KAFOs and a walker, AFOs, forearm crutches, WC
L5: knee flexion strength, weak hip extension, hip abduction, plantarflexion; potential to be a functional ambulatory, may require a wheelchair during growth spurts, for long distances or on uneven surfaces; equipment: orthotics, upper limb support, WC
what are ways we can diagnosis it
maternal serum alpha fetoprotein screening
US in the 2nd trimester
Amniotic fluid analysis
73% of children with MM have been reported to have what?
a latex allergy
may also have allergies to kiwi, avocados and bananas
what is tethered cord syndrome
these attachments cause an abnormal stretching of the spinal cord
20-50% of children with MM defects repaired shortly birth will requires surgery to untether the spinal cord
what is ambulation interventions
symmetric neutral joint alignment in sitting and standing
correct fitting assistive devices
orthoses that provide total contact
what is motor functions and equipment for S1 level? S2, S2-S3?
S1: weak gastrocnemius/soleus, gluteus medius, gluteus maximus; potential for functional ambulation, equipment: orthotics may be used to improve alignment
S2, S2-S3: strong plantarflexors and gluteals, potential to be a functional ambulatory, orthotics may be needed to improve alignment