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Neural Tube Defects (NTDs)
• Deformities involving CNS covering
• Abnormalities that are derived from the embryonic neural
tube (neural tube defects) constitute the largest group of
congenital anomalies with multifactorial inheritance.
 Neural Tube Defects
• Mainly embryologic
induction disorder,
failure to properly
form mesoderm-
neuroectoderm.

• DEFICITS:
sensorimotor
disturbance, dislocated
hips, talipes equinovarus
(clubfoot), and
hydrocephalus.
Epidemiology
▪ Globally, it is estimated that
approximately 300,000 babies
are born each year with NTDs
resulting in approximately
88,000 deaths and 8.6 million
disability-adjusted life years.

▪ In the United States, rates of

NTDs have declined from 1.3
per 1000 births (1990) to 0.3 per
1000 births after introduction of
mandatory food fortification with
folic acid in 1998
• In Atlanta 1990’s more
than 30% of affected
pregnancies were
terminated based on
prenatal test.

• In Sept. 1992 – US Public

Health Service: All woman
childbearing age, capable
of becoming pregnant –
should take 0.4 mg Folic
Acid per day for purpose
of reducing risk of NTD’S.
• In the United States,
3,000 pregnancies are
affected by neural tube
defects every year.
 Etiology
• failure of neural tube closure
during the embryo’s early
development (the first 3 to 5
weeks)
Evidenced implicates
multifactorial origin:
• including drugs
• radiation
• maternal malnutrition
• chemicals
• possibly a genetic mutation
in folate pathways
Increased Risk of NTDs
• Low Folic Acid Intake during pregnancy
• Pre-pregnancy maternal obesity
• previous NTD pregnancy
• use of antiepileptic drugs (AEDs) in pregnancy
Types of Neural Tube Defects:

Exencephaly
– a malformation
where the brain
is totally exposed
or herniated
through a skull
defect
Exencephaly
Types of Neural Tube Defects

Encephalocele
- a protrusion of
the brain and
meninges into a
fluid-filled sac
through a skull
defect
Large occipital encephalocele
 Types of Neural Tube Defects:

Cranioschisis – a skull
defect through which
various tissues protrude

Microcephaly - is a
disorder in which the brain
growth is so slow that it falls
more than three standard
deviation below normal
growth charts
The two most common types of
NTDs

•Anencephaly
•Spina Bifida (SB)
Types of Neural Tube Defects:
Anencephaly
• brain is degenerated to a spongiform mass with no
bony covering in a fetus
• absence of the cranial vault with cerebral hemispheres either
completely missing or greatly reduced in size.
Types of Neural Tube Defects:
Rachischisis or Spina
Bifida – fissures in the
spinal column that leaves
the meninges and spinal
cord exposed

• A neural tube defect

where there is an
incomplete closure of
the vertebrae & neural
tube.
Spina Bifida
• Two Types of Spina Bifida
(SB):

a. Spina Bifida Occulta –

failure of the posterior
vertebral arches to fuse,
most often at the 5th lumbar
or 1st sacral vertebrae, there
is no herniation of the spinal
cord or meninges

SC – Intact & not visible

Meninges – not exposed on
skin surface
Neurological deficits are not
usually present.
Spina Bifida Occulta

The child has long hairy tail with underline spina

bifida occulta.
Spina Bifida

b.Spina Bifida Cystica

– a defect in the
closure of the
posterior vertebral
arch resulting in one
of the following 2
anomalies:
Meningocele and
Myelomeningocele
(Meningomyelocele)
Spina Bifida Cystica

2 Anomalies:
1. Meningocele –
hernia protrusion of
a saclike cyst of
meninges filled with
spinal fluid –
lumbosacral area
SC – not involved!
Neurological deficit –
absent.
Spina Bifida Cystica –
“Myelomeningocele”
a. Protrusion of the
2.Myelomeningocele meninges, CSF, nerve roots,
(Meningomyelocele) – and a portion of the spinal
a sac-like extrusion cord occurs.
through a bony defect b. The sac (defect) is covered
containing meninges, by a thin membrane prone
CSF, and a portion of
the spinal cord or to leakage or rupture.
nerve roots c. Neurological deficits are
evident.
d. Most serious type of Spina
Bifida
Assessment

• Intrauterine Life
• Sonography
• Fetoscopy
• amniocentesis
(discovery of increase
AFP in amniotic fluid),
or analysis of AFP in
the maternal serum
Assessment

• After birth
• Ultrasound
• Transillumination (if
with lesion)
• Radiologic studies
• CT scans & MRIs
• Flat plate films of the
spinal column
• Neurological
examination of the
extremities
Clinical Manifestations:
• Depends on the location of the
defect and SC involvement
• The higher the defect, the greater
neurologic dysfunction
- sensory loss
- paralysis of the legs
- weakness
- affected bladder & bowel function
- orthopedic problems (hip and
joint deformities)
- trauma to the lower extremities
may occur
- Hydrocephalus
Therapeutic Management
• spina bifida occulta need no
immediate surgical correction
Meningocele,
Myelomeningocele, or
Encephalocele
• involves surgery to replace the
contents that are replaceable
• to close the skin disorder to prevent
infection surgery for neural tube
disorders done only after infant
survived the newborn period (usually
within 24 to 48 hours)
 NURSING MANAGEMENT:
Pre-operative
• monitor the newborn for integrity
of the sac & leakage of CSF
• assess the extremities for
deformities
• frequently assess the VS
• stay alert for signs of infection
• measure head circumference
• cover the sac on the newborn’s
back with a sterile saline
dressing
 NURSING MANAGEMENT:
• Place the infant in a prone position
with hips slightly flexed & legs abducted
• Assess the infant regularly for motor
deficits as well as bladder & bowel
involvement
• Frequently assess the vital signs & stay
alert for signs of infection
• Feed the infant with the head turned to
one side
• Tactile stimulation such as touching,
patting, and cuddling maybe comforting.
• Monitor for signs of increased ICP –
indicates Hydrocephalus
development
Postoperative:
- observe the wound healing
- note any signs of infection & CSF
leakage
- measure the head circumference
daily
- assess intake & output
- monitor the infant’s vital signs
carefully
- watch closely for symptoms of
infection, especially
meningitis
If a ventriculoperitoneal shunt was placed, watch for
hydrocephalus, increased intracranial pressure, or infection
Inspect the surgical site for
CSF leakage

Placed in the prone or side-

lying position (upright)

Splints maybe used to

maintain extremity
alignment.
• Nursing Diagnosis: Risk for
infection related to rupture or
bacterial invasion of the neural tube
sac.
• Outcome Evaluation: Neural tube
sac remain intact: axillary
temperature remains below 98.6 F
(37C)
• Nursing Diagnosis: Risk for
Imbalanced Nutrition, less than
body requirements, related to
difficulty assuming normal feeding
position.
• Outcome Evaluation: Child’s skin
turgor is good; weight is maintained
within 10% of birth weight; specific
gravity of urine remains between
1.003 and 1.030
• Nursing Diagnosis: risk for ineffective cerebral tissue
perfusion related to increased intracranial pressure.
• Outcome Evaluation: Child’s head circumference remains
within present percentile on growth chart; signs and symptoms
of increased intracranial pressure are absent.
• Nursing Diagnosis: Risk for impaired skin integrity related to
required prone positioning.
• Outcome Evaluation: Infant’s skin remains intact without
erythema or ulceration.
• Nursing Diagnosis: Impaired physical mobility
related to neural tube disorder.
• Outcome Evaluation: Child ambulates with the
least amount of accessory equipment possible.
• Nursing Diagnosis: Risk for impaired elimination
related to neural tube disorder.
• Outcome Evaluation: Child demonstrates ability
to independently manage bowel and bladder
elimination by school age
 Planning &
Implementation:

Providing emotional support

- keep parents informed about their child ’ s
status
- allow them to express their frustrations &
anger
- encourage them to become involved in the
child’s care in the hospital.
A cademics.
T ouches heart.
E ducates mind.
A rticulates vision.
M odels excellence