Neural Tube Defects
Folic acid supplementation
400 mcg/day beans, peas, lentils, liver fortification of cereal grains
Prenatal screening for neural tube defects
Alpha-fetoprotein- increases Limited US of skull/spine
Neural tube defects
Failure of the neural plate to form the neural tube, first 28 days following conception
Management of neural tube defects
Fetal surgery After birth- nicu, protect from trauma, neurosurgical closer within 72 hours
Most common and severe form of spina bifida
Myelomeningocele
Hydrocephalus ongoing care
OFC, anterior fontanelle for tension/bulging, change in mentation, vomiting, EOM's, shunt failure
Teratogens for neural tube defects
anti-seizure meds (valproate) insulin resistance (DM 2 mom's 10-20x)
Arnold-Chiari (type II) malformation
association with myelomeningocele Cerebellar tonsils and brain stem push down through foramen magnum resulting in hydrocephalus
Neuro function of anencephaly
brainstem function
Associated malformations with anencephaly
cleft lip/palate omphalocele
Mobility in neural tube defects
continually address/assess, mobility aids assist walking and promote confidence, independence and fitness
Bowel function and neural tube defects
disorders of innervation in intestinal tract and anus (sphincter control, fecal incontinence)
Spina bifida pathogenesis
embryologic in nature, failure of caudal neuropore to close on day 27
Pathogenesis of anencephaly
embryologic, failure of rostral neuropore to close on day 25
Anencephaly
failure of development of most of the cranium and brain, open defect in the calvaria and skin exposing neural tube
Risk factors for neural tube defects
folate deficiency genetic factors (markers on 2nd chromosome) Teratogens amniotic bands maternal fever in 1st trimester
Spina bifida occulta
least severe form, spinal cord normal, one or more vertebrae may be deformed
Clinical features of anencephaly
major portions of CNS absent hypothalamus missing cerebellum, brainstem, optic nerves, spinal cord malformed
Myelomeningocele
meninges and spinal nerves protrude through an opening in the spine leading to severe nerve damage and profound physical manifestations
Associated with tethered cord
meningocele
Good prognosis following surgical repair
meningocele
Least common form of spina bifida
meningocele
Maternal fever in 1st trimester increases risk for
neural tube defects (febrile illness or hot tub)
Prognosis anencephaly
no mgmt options, nearly all liveborn infants die shortly after birth
Most individuals with myelomeningocele are cognitively
normal, learning disabilities are common
3 Classifications of Spina Bifida
occulta meningocele myelomeningocele
Decline in neural tube defects has occurred because of
periconceptual folic acid supplementation prenatal screening (AFP and US)
Positive prenatal screen for neural tube defects
pregnancy termination
Prevention of vesicoureteral reflux
prophylactic antibiotics (amoxicillin)
Neurogenic bladder issues
retained urine- increase of UTI's vesicoureteral reflux- hydronephrosis- renal damage
Hydrocephalus tx
shunt placement- ventriculoperitoneal shunt
Tuft of hair, lipoma or birth mark on lower back in lumbar region
spina bifida occulta
failure of fusion of vertebral arch
spina bifida occulta
Meningocele
spinal cord normally develops, meninges protrude like a cyst out of opening in spine, may be skin or membrane covered
clean intermittent catheterization
use catheter in bladder for complete evacuation
Meningocele and damage
usually no nerve damage but may have some minor physical disabilities
Physical manifestations of myelomeningocele
variable paresis of lower limbs with hypotonia muscle imbalance sensory loss bladder denervation bowel denervation scoliosis