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Neural tube defect

Mrs. Abhilasha Saha


Associate professor- Paediatric Nursing
National medical college, Birgunj, Nepal
 Spina bifida is a birth defect that involves the
incomplete development of the spinal cord or
its coverings characterized by defective
closure of the bony encasement of the spinal
cord through which the spinal cord and
meninges may or may not protrude.

 The term spina bifida comes from Latin and


literally means "split" or "open" spine.
 Spine bifida also called spinal dysraphia,
refers to a malformation of the spine in which
the posterior portion of the laminae of the
vertebrae fails to close.

 It occurs in 1 per 1,000 live births.

 Most common developmental defect of the


CNS.
Types of Spina Bifida
The two forms of spina bifida are

 Spina bifida occulta and


 Spina bifida manifesta.
 Spina bifida occulta is the mildest form of
spina bifida (occulta means hidden). Most
children with this type of defect never have
any health problems, and the spinal cord is
often unaffected.
Spina bifida manifesta includes two types of
spina bifida:

 Meningocele,

 Myelomeningocele/ meningomyelocele.
 Meningocele involves the meninges, the
membranes responsible for covering and
protecting the brain and spinal cord. If the
meninges push through the hole in the
vertebrae (the small, ring-like bones that
make up the spinal column), the sac is
called a meningocele.
 Myelomeningocele is the most severe form of
spina bifida. It occurs when the meninges push
through the hole in the back, and the spinal cord
also pushes though. Most babies who are born with
this type of spina bifida also have hydrocephalus,
an accumulation of fluid in and around the brain.

Occurs four to five times more frequently than


meningocele
Causes
 Unknown etiology

 Geneticpredisposition triggered by
environmental factors- drugs ( valproic acid )
during pregnency

 Women and having one child affected


Causes
 Arrest in the orderly formation of the vertebral
arches and spinal cord between 4 _ 6th wk) of
pregnancy when the two sides of the
embryo's spine fail to join together, leaving
an open area.
 Incomplete closure of neural tube during 4th
wk of embryonic life.
 The neural tube forms adequately, then
rupture
 Maternal folic acid deficiency has been linked
to spina bifida, and researchers believe that
many cases can be prevented if women of
childbearing age consume 0.4 milligrams
(400 micrograms) of folic acid every day, and
continue to take it throughout the first
trimester.
Symptoms of Spina Bifida
 Babies who are born with spina bifida occulta
often have no outward signs or symptoms.
The spinal cord does not protrude through
the skin, although a patch of hair, a
birthmark, or a dimple may be present on the
skin over the lower spine.
Meningocele
 Fluid-filled sac visible on the back. The sac is
often covered by a thin layer of skin and can
be as small as a grape or as large as a
grapefruit.

 Seldom evidence of weakness of the legs or


lack of sphincter control
Myelomeningocele
 A sac-like mass that bulges from the back,
but a layer of skin may not always cover it. In
some cases, the nerves of the spinal cord
may be exposed. A baby who also has
hydrocephalus will have an enlarged head,
the result of excess fluid and pressure inside
the skull.
 Bladder dysfunction
 Fecal incontinence and constipation

 Developmental disabilities- average


intellectual
Diagnostic evaluation
 Prenatal detection- The alpha-fetoprotein
(AFP) test, performed between the 16th and
18th weeks of pregnancy, measures how
much AFP, which the fetus produces, has
passed in the mother's bloodstream. If the
amount is high, the test is repeated because
in many cases, high AFP readings are false.
If the second result is high, other tests will be
done to double-check and confirm the
diagnosis.
 CT scan and MRI
 Ultrasound

 Amniocentesis- A needle is inserted through


the mother's belly and into the uterus to
collect fluid that is tested for AFP.
 Clinical manifestations
Treatment
 Children with spina bifida occulta seldom
need treatment.

 Incases of spina bifida manifesta, treatment


depends on the type of spina bifida and its
severity.
 Babies with meningocele usually have an
operation during infancy in which doctors
push the meninges back and close the hole
in the vertebrae. Many will have no other
health problems later unless there is nerve
tissue involved with the sac.
 Babies with myelomeningocele need more
immediate attention and often have surgery
within the first 1 to 2 days after birth. During
this first surgery, doctors push the spine back
into the vertebrae and close the hole to
prevent infection and protect the spine.
 Procedure – laminectomy and closure of the
open lesion or removal of sac
A baby who also has hydrocephalus will need
an operation to place a shunt in the brain.
The shunt is a thin tube that helps to relieve
pressure on the brain by draining and
diverting extra fluid.
Nursing management
 Risk for impaired skin integrity r/t impaired
motor and sensory function
 Risk for infection r/t contamination of the
myelomeningocele site
 Altered urinary elimination r/t neurological
deficits
 Altered cerebral tissue perfusion r/t
hydrocephalus
 Fear r/t neurological disorder and surgery
 Ineffectivethermoregulation following surgery
 Bowel incontinence/ constipation r/t the
abnormal development of and damage to the
spinal cord
 Body image disturbance r/t child’s
appearance, difficulty in locomotion, lack of
control over excretory functions