spina bifida

Question 1

what is it

Answer 1

most common neural tube defect
-2nd most common child abnormality after CP
-highest incidence in british, irelands , wales scotland
-lowest in japan
-hispanic

Question 2

what are the risk factors

Answer 2

polycentric inheritance
-environment

Question 3

what bout nutrition

Answer 3

folic acid deficiency

Question 4

what is the folic acid dosage

Answer 4

4000 mcg
1-3 months before pregnancy

Question 5

when does the neural tube close

Answer 5

28 days

Question 6

what is carniorachisisis

Answer 6

most severe
both brain and spinal cord remain open

Question 7

what is the double hit theory

Answer 7

maldevelopment of neural tube + exposure to amniotic fluid = damage to normal spinal cord tissue

Question 8

how is it diagnosed?

Answer 8

lab: AFP, 13-15 weeks, 80% reliable
imaging: US, 16-24 weeks, 90% reliable
procedure: amniocentesis: 16-18 weeks, 100% accurate

Question 9

what is lemon sign

Answer 9

lemon shaped skull

Question 10

what is banana sign

Answer 10

cerebellum looks like a banana because its wrapped around posterior brainstem due to downward traction of spinal cord
chiari II

Question 11

what is myelomenigocele

Answer 11

-arnold chiari type II
-hydrocephalus
-motor, sensory, bowel and bladder issues
-majority 75% lumbar- lumbosacral

Question 12

Which of the following types of orthotic would be most appropriate for a child with L4-L5 level spina bifida?

Answer 12

AFO

Question 13

what is meningocele

Answer 13

-incomplete skin coverage=CSF leak
-neurologic signs

Question 14

what is myelocele

Answer 14

-cystic cavity anterior to spinal cord

Question 15

what is occulta

Answer 15

-no neuro deficit
-rarely associated w lipoma or tethered cord
-L5-S1

Question 16

what are the neurosurgical complications with arnold chiari type II

Answer 16

-sleep
-apnea
-palpitations
-diplopia
-dysphagia

Question 17

what are the neurosurgical complications with hydrocephalus

Answer 17

-headaches
-nausea
-altereted mentality
-lethargy
-personality change
-vision
-hoarse cry
-swallow issues

Question 18

what are the neurosurgical complications with syringomyelia

Answer 18

-motor changes
-sensory changes
-reflex changes
-neck pain

Question 19

what are the neurosurgical complications with tethered cord

Answer 19

-motor changes
-sensory changes
-reflex change
-back pain
-scoliosis
-bowel or bladder issues
-sexual changes

Question 20

talk about arnold chiari type II malformation

Answer 20

-downward displacement of medulla, brainstem, cerebellar through foramen magnum

-present in 80-90% cases of MMC, hydroceph 90%

-imparied fine motor coordination and ataxia

-severe= respiratory dysfunction and arythmias

-suboccipital decompression surgery

Question 21

talk about sleep disordered breathing

Answer 21

-central apnea, periodic breathing, obstructive apnea, central hypoventilation

-central respiratory dyssfunction= most frequent single cause of death in SB

-81% of people with SB

-symptoms: sleepy, snoring, stridor, central apnea, aspiration

Question 22

talk about hydrocephalus

Answer 22

usually present at birth> symptomatci first week

-monitor head circumference, fontanelles

CSF shunt option

spontaneous arrest of hydrocephalus occurs in 50% of patients with SB by 15 years

Question 23

what is the sign or gaze palsy associated with severe hydrocephalus?

Answer 23

sunset sign
upward gaze palsy

Question 24

what is syringomyelia

Answer 24

fluid filled central cavity

-symptoms: change in motor and sensory function, increased reflexes, pain

-often associated w hydrocephalus so treat that so it can treat the syrinx

Question 25

talk about tethered cord

Answer 25

most common signs and symptoms:
weakness, pain, change in function, scoliosis, bowel/bladder

-neurosurgery de-terhting done more often for kids
-risk of loss of function depending on surgery copmliocations

re-tethering occurs 10-15%

Question 26

what are predictors for ambulation in kids with SB?

Answer 26

1. low motor level: need good quad strength
2. no history of shunt
3. no history of hip or knee contracture surgery

Question 27

what is a common gait pattern you might see in a patient with an L4 myelomeningocele?

Answer 27

trendelenburg

-hip abduct weakness L5-S1
-associated w weak hip ext and PF
-trunk sway
-knee valgus