spina bifida Flashcards
what is it
most common neural tube defect
-2nd most common child abnormality after CP
-highest incidence in british, irelands , wales scotland
-lowest in japan
-hispanic
what are the risk factors
polycentric inheritance
-environment
what bout nutrition
folic acid deficiency
what is the folic acid dosage
4000 mcg
1-3 months before pregnancy
when does the neural tube close
28 days
what is carniorachisisis
most severe
both brain and spinal cord remain open
what is the double hit theory
maldevelopment of neural tube + exposure to amniotic fluid = damage to normal spinal cord tissue
how is it diagnosed?
lab: AFP, 13-15 weeks, 80% reliable
imaging: US, 16-24 weeks, 90% reliable
procedure: amniocentesis: 16-18 weeks, 100% accurate
what is lemon sign
lemon shaped skull
what is banana sign
cerebellum looks like a banana because its wrapped around posterior brainstem due to downward traction of spinal cord
chiari II
what is myelomenigocele
-arnold chiari type II
-hydrocephalus
-motor, sensory, bowel and bladder issues
-majority 75% lumbar- lumbosacral
Which of the following types of orthotic would be most appropriate for a child with L4-L5 level spina bifida?
AFO
what is meningocele
-incomplete skin coverage=CSF leak
-neurologic signs
what is myelocele
-cystic cavity anterior to spinal cord
what is occulta
-no neuro deficit
-rarely associated w lipoma or tethered cord
-L5-S1
what are the neurosurgical complications with arnold chiari type II
-sleep
-apnea
-palpitations
-diplopia
-dysphagia
what are the neurosurgical complications with hydrocephalus
-headaches
-nausea
-altereted mentality
-lethargy
-personality change
-vision
-hoarse cry
-swallow issues
what are the neurosurgical complications with syringomyelia
-motor changes
-sensory changes
-reflex changes
-neck pain
what are the neurosurgical complications with tethered cord
-motor changes
-sensory changes
-reflex change
-back pain
-scoliosis
-bowel or bladder issues
-sexual changes
talk about arnold chiari type II malformation
-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
talk about sleep disordered breathing
-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
talk about hydrocephalus
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
what is the sign or gaze palsy associated with severe hydrocephalus?
sunset sign
upward gaze palsy
what is syringomyelia
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
talk about tethered cord
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%
what are predictors for ambulation in kids with SB?
- low motor level: need good quad strength
- no history of shunt
- no history of hip or knee contracture surgery
what is a common gait pattern you might see in a patient with an L4 myelomeningocele?
trendelenburg
-hip abduct weakness L5-S1
-associated w weak hip ext and PF
-trunk sway
-knee valgus
