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Neural Tube Defects: Group 1

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Neural Tube

Defects
Group 1

 
ABUAN, MA. MINNA
APOLINARIO, DAVID ELIZER
BAJANDI, PRINCESS ELIZABETH JANINE
BARCENAS, JENNIFER
BERNARDINO, ERNANNE PATRICK
BRAZIL, LEO
CALICDAN, RAINIER
CANCILLER, GIAN ANGELO
CANIDO, MARY JOYCE
CASERO, AIZA
COLLADO, JEMMA
CORDERO, RONALD
Neural Tube Defects

 neural tube defects are birth defects of the brain


and spinal cord.
 arise early in fetal development and can cause life-
long complications of varying severity.
 between the 15th and 28th day, the neural tube
forms and then becomes the brain, spine and
vertebral column.  
Neural Tube Defects

 neural tube fusing process usually is complete by


28 days of gestation.
 if the tube does not close properly along its entire
length, then a NTD will form at the open location.
 two of the most common neural tube defects are
spina bifida and encephalocele.
Encephalocele
 congenital gap in the skull that usually results in a
protrusion of brain material.
 also called bifid cranium , cephalocele , craniocele .

 neural tube defect characterized by sac-like protrusions of


the brain and the membranes that cover it through
openings in the skull.
 classified as nasofrontal, nasoethmoidal, or naso-orbital

 severity of encephalocele varies, depending on its location


Encephalocele

 Occurrence: 1 per 5,000 live births worldwide.

 Exact cause is unknown.

 Seen more commonly in females than males.

 Fetuses with an front-type encephalocele are much more


likely to survive than those with an encephalocele on the
back of the head.
Diagnostic Evaluation

 Alpha-Fetoprotein (AFP), Maternal Serum Alpha-


Fetoprotein (MSAFP)
 measured in pregnant women, using maternal blood or
amniotic fluid
 done between 15 and 20 weeks of pregnancy
 level in maternal serum or amniotic fluid is compared with
levels expected at a given gestational age
Diagnostic Evaluation

 Ultrasound

 MRI - may also be recommended to get a more accurate


and detailed picture of the defect and any associated
anomalies.
Clinical Manifestations

 developmental delay

 vision problems

 mental and growth retardation

 seizures
Clinical Manifestations

 craniofacial abnormalities

 neurologic problems

 hydrocephalus

 spastic quadriplegia

 microcephaly

 ataxia
Management

 Reparative surgery – performed during infancy


 Goals:
 closure of open skin defects to prevent infection and desiccation of
brain tissue
 removal of nonfunctional extracranial cerebral tissue with water-
tight closure of the dura
 total craniofacial reconstruction with particular emphasis on
avoiding the long-nose deformity
Management

 Proper levels of folic acid have been shown to help prevent


such defects when taken before pregnancy, and early in
pregnancy.
 400 micrograms of folic acid daily
Nursing Diagnoses

 Ineffective Cerebral Tissue Perfusion related to increased


ICP before surgery
 Risk for Impaired Skin Integrity related to alterations in
LOC and enlarged head
 Anxiety of parents related to child undergoing surgery

 Ineffective Family Coping related to diagnosis and surgery


Spina Bifida

 also called spinal dysraphia

 malformation of the spine in which the posterior portion of


the laminae of the vertebrae fails to close
 occurs in approximately 1 per 1,000 live births in the United
States
 most common developmental defect of the central nervous
system
 more common in Caucasian than in non-Caucasian people.
Types of Spina Bifida

 Spina Bifida Occulta


 The defect is only in the vertebrae. The spinal cord and meninges
are normal.

 Meningocele
 The meninges protrude through the opening in the spinal canal.
This forms a cyst filled with CSF and covered with skin.

 Myelomeningocele (or Meningomyelocele)


 The spinal cord and cord membranes protrude through the defect
in the laminae of the vertebral column. Myelomeningoceles are
covered by a thin membrane.
MENINGOMYELOCELE
 lesion contains both the spinal cord and cord membranes.

 A bluish area may be evident on the top because of


exposed neural tissue.
 The sac may leak in utero or may rupture after birth,
allowing free drainage of CSF. This renders the child highly
susceptible to meningitis.
 Occurs four to five times more frequently than
meningocele.
 may affect as many as 1 out of every 800 infants
Etiology

 Exact cause of meningomyelocele is unknown.

 Low levels of folic acid in a woman's body before and


during early pregnancy is thought to play a part in this
type of birth defect.
 May recur if parents had:
 (1-2%) 1 child affected
 (10%) 2 children affected
Diagnostic Evaluation

 Prenatal ultrasound

 Fetal MRI

 AFP, MSAFP levels are abnormally high


Clinical Manifestations

 round, raised, and poorly epithelialized area may be noted


at any level of the spinal column
 the highest incidence of the lesion occurs in the
lumbosacral area
 Hydrocephalus (approximately 2/3 of children) due to
Arnold-Chiari malformation
 Loss of motor control and sensation below the level of the
lesion can occur
Clinical Manifestations

 Clubfeet

 Bladder dysfunction

 Fecal incontinence and constipation

 Developmental disabilities
Management

 Surgical Intervention
 Procedure: laminectomy and closure of the open lesion or
removal of the sac usually can be done soon after birth.
 Purpose:
 To prevent further deterioration of neural function.
 To minimize the danger of rupture and infection, especially
meningitis.
 To improve cosmetic effect.
 To facilitate handling of the infant.
Management

 Multidisciplinary Follow-Up for Associated Problems


 team may include a neurologist, neurosurgeon, orthopedic
surgeon, urologist, primary care provider, social worker,
physical therapist, a variety of community-based and
hospital staff nurses, and the child and family.
Prognosis

 Influenced by the site of the lesion and the presence and


degree of associated hydrocephalus. Generally, the higher
the defect, the greater the extent of neurologic deficit and
the greater the likelihood of hydrocephalus.
 In the absence of treatment, most infants with meningo
myelocele die early in infancy.
 Surgical intervention is most effective if it is done early in
the neonatal period, preferably within the first few days of
life.
Prognosis

 Even with surgical intervention, infants can be expected to


manifest associated neurosurgical, orthopedic, or urologic
problems.
 New techniques of treatment, intensive research, and
improved services have increased life expectancy and have
greatly enhanced the quality of life for most children who
receive treatment.
Nursing Diagnoses

 Neonates (Preoperative)
 Risk for Infection related to contamination of the
myelomeningocele site
 Impaired Urinary Elimination related to neurologic deficits
 Ineffective Tissue Perfusion: Cerebral related to potential
hydrocephalus
 Fear (parents) related to neonate with neurologic disorder
and to surgery
Nursing Diagnoses

 Infants and Children (Postoperative)


 Ineffective Thermoregulation following surgery
 Impaired Urinary Elimination related to sacral denervation
 Bowel Incontinence or Constipation related to impaired
innervation of anal sphincter and bowel musculature
 Disturbed Body Image related to the child's appearance,
difficulties with locomotion, and lack of control over
excretory functions

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