SPINA BIFIDA

INTRODUCTION

Spina bifida is a birth defect that occurs when the spine and spinal
cord don't form properly. It falls under the broader category of neural tube
defects. It is part of a group of birth defects called neural tube defects caused by
a defect in the neural arch generally in the lumbosacral region, spina bifida is a
failure of the posterior laminae of the vertebrae to close; this leaves an opening
through which the spinal meninges and spinal cord may protrude The neural
tube is the embryonic structure that eventually develops into the baby's brain
and spinal cord and the tissues that enclose them. Normally, the neural tube
forms early in pregnancy, and it closes by the 28th day after conception. In
babies with spina bifida, a portion of the neural tube fails to develop or close
properly, causing defects in the spinal cord and in the bones of the spine. Spina
bifida can range from mild to severe, depending on the type of defect, size,
location and complications. When early treatment for spina bifida is necessary,
it's done surgically, although such treatment doesn't always completely resolve
the problem.

DEFINITION

Spina bifida is a congenital defect of the spine in which part of the

spinal cord and its meninges are exposed through a gap in the backbone. It often
causes paralysis of the lower limbs, and sometimes learning difficulties.

INCIDENCE
 The rate of myelomeningocele and other neural tube defects has declined
since the late 20th century. The average worldwide incidence of spina bifida is 1 case
per 1000 births, but marked geographic variations occur. According to the CDC, the
prevalence of spina bifida in the United States is higher in the white and Hispanic
populations (2 and 1.96, respectively, per 10, 000 live births) than in the black
population (1.74 per 10, 000 live births).Data from state and national surveillance
systems from 1983-1990 found the birth prevalence rate of myelomeningocele to be
slightly higher in females than in males (1.2:1).

RISK FACTORS

Spina bifida is more common among whites and Hispanics, and females
are affected more often than males. Although doctors and researchers don't
know for sure why spina bifida occurs, they have identified some risk factors:

1.Folate deficiency.

Folate (vitamin B-9) is important to the healthy development of a

baby. Folate is the natural form of vitamin B-9. The synthetic form, found in
supplements and fortified foods, is called folic acid. A folate deficiency
increases the risk of spina bifida and other neural tube defects.

2.Family history of neural tube defects.

Couples who've had one child with a neural tube defect have a
slightly higher chance of having another baby with the same defect. That risk
increases if two previous children have been affected by the condition. In
addition, a woman who was born with a neural tube defect has a greater chance
of giving birth to a child with spina bifida. However, most babies with spina
bifida are born to parents with no known family history of the condition.

3.Some medications.
 For example, anti-seizure medications, such as valproic acid seem
to cause neural tube defects when taken during pregnancy, possibly because
they interfere with the body's ability to use folate and folic acid.

4.Diabetes.

Women with diabetes who don't control their blood sugar well have
a higher risk of having a baby with spina bifida.

5.Obesity.

Pre-pregnancy obesity is associated with an increased risk of neural

tube birth defects, including spina bifida.

6.Increased body temperature.

Some evidence suggests that increased body temperature

(hyperthermia) in the early weeks of pregnancy may increase the risk of spina
bifida. Elevating your core body temperature, due to fever or the use of saunas
or hot tubs, has been associated with a possible slight increased risk of spina
bifida.

TYPES

Spina bifida can occur in different forms: spina bifida occulta,

meningocele or myelomeningocele. The severity of spina bifida depends on the
type, size, location and complications.

1. Spina bifida occulta

"Occulta" means hidden. The mildest form, spina bifida occulta results
in a small separation or gap in one or more of the bones of the spine (vertebrae).
Many people who have spina bifida occulta don't even know it, unless the
condition is discovered during an imaging test done for unrelated reasons. It is a
defect which results from failure of formation of bony arch around the spinal
cord, but spinal cord and meninges are normal. It is not visible externally and is
asymptomatic. This type of defects occurs in 5% cases of spina

2.Spina bifida cystica

Spina bifida cystica is a defect in the closure of posterior vertebral

arch with protrusion of spinal cord and meninges through the defect.

a. Meningocele

In a form of spina bifida called meningocele, the protective membranes

around the spinal cord (meninges) push out through the opening in the
vertebrae, forming a sac filled with fluid. But this sac doesn't include the spinal
cord, so nerve damage is less likely, though later complications are possible.

b. Myelomeningocele

Also known as open spina bifida, myelomeningocele is the most severe

form. The spinal canal is open along several vertebrae in the lower or middle
back. The membranes and spinal nerves push through this opening at birth,
forming a sac on the baby's back, typically exposing tissues and nerves. This
makes the baby prone to life-threatening infections.
 PATHOPHYSIOLOGY

Spina bifida occurs because of defect in the orderly closure of vertebral

column and formation of spinal cord during 4th to 6th week of gestation .

During the 3rd week of gestation, a depression forms in the dorsal of the
ectoderm in embryo.

This depression becomes deeper and its margins close dorsally to create
a neural tube.

The ends of the neural tube close by the end of the 4th week of gestation.

The walls of neural tube thicken and become spinal cord and brain.
 The neural canal become ventricles and central canal of the spinal cord

The vertebral column is formed simultaneously with the neural tube.

Occurrence of the defect due to neural tube fails to close normally or

rupture after having once closed

CLINICAL FEATURES

1.Paralysis.

If the opening occurs at the top of the spine, the patient’s legs are more
likely to be completely paralyzed, and there will be other problems with
movement elsewhere in the body.

2.Cognitive symptoms.

Problems occurring in the neural tube have a negative effect on brain

development; the main part of the brain (cortex), especially the frontal part does
not develop properly, leading to some cognitive problems.

3.Arnold-Chiari malformation.

There may also be Type 2 Arnold-Chiari malformation, an abnormal brain

development involving the cerebellum – this may affect the patient’s language
processing and physical coordination skills.

4.Birthmark.

There may be a small birthmark, dimple or tuft of hair on the skin where
the spinal defect is.
5.Learning difficulties:

People with spina bifida have normal intelligence. However, learning

difficulties can occur, leading to problems with attention, solving problems,
reading, understanding spoken language, making plans, and grasping abstract
concepts.

6.Bowel and urinary incontinence: These are common.

7.Meningitis:

There is a higher risk of meningitis among people with spina bifida. This
can be life-threatening.

8.Other problems: In time, the individual may experience skin problems,

gastrointestinal problems, latex allergies, and depression. Skin can be more
easily damaged if the person lacks sensation in the legs. They may injure or burn
themselves without realizing.

DIAGNOSTIC FINDINGS

1.AFP levels.

Elevated maternal alpha fetoprotein levels in the maternal seruma nd

the amniotic fluid indicates the probability of CNS abnormalities.

2.Ultrasonography.

Ultrasonographic examination of the fetus may show an incomplete neural

tube.

3.Clinical examination.
 Diagnosis of spina bifida is made from clinical observation and
examination.

4.Other imaging studies.

Additional evaluation of the defect may include magnetic resonance

imaging (MRI), computed tomography (CT), and myelography.

5.Amniocentesis

If a blood test shows high levels of AFP in the blood but the ultrasound is
normal, the doctor may offer amniocentesis. An analysis of the sample indicates
the level of AFP present in the amniotic fluid. A small amount of AFP is
normally found in amniotic fluid. However, when an open neural tube defect is
present, the amniotic fluid contains an elevated amount of AFP because the skin
surrounding the baby's spine is gone and AFP leaks into the amniotic sac.

MANAGEMENT

Surgical Management

Surgery before birth

Fetal surgery for myelomeningocele, the most severe form of spina bifida, is a
delicate surgical procedure where fetal surgeons open the uterus and close the
opening in the baby's back while they are still in the womb. Fetal spina bifida
surgery is shown to offer significantly better results than traditional repair after
birth. Because spinal cord damage is progressive during gestation, prenatal
repair of myelomeningocele may prevent further damage.
 Surgery after birth

If the baby has been diagnosed with spina bifida during pregnancy, need to
deliver at a high-risk pregnancy centre. After delivery, the sac cannot stay
outside of the body for a long time. It may tear or get infected, or it may already
be ruptured exposing its delicate contents. The baby will have surgery to repair
the sac within two or three days of birth. A neurosurgeon will do the operation.
A neurosurgeon is a doctor who operates on the brain and spinal cord.

The baby may need an MRI before their operation to help the surgeons to better
see the spinal cord. An MRI is a scan that takes special pictures of the inside of
the body. An MRI scan requires a person to be still while the pictures are being
taken. Some children may need medicine to help them keep still during the tests.

The purpose of the operation is to:

 close the skin over the sac

 prevent infection and further damage to the spinal cord

Nursing Management

Goals

The major goals for the patient are to:

 Prevent infection.
 Maintain skin integrity.
 Prevent trauma related to disuse.
 Increase family coping skills, education about the condition, and
support.
Nursing interventions

The newborn child has a condition such as spina bifida can naturally cause the
family to feel grief, anger, frustration, fear and sadness, however, nurses are there to
help the family cope and understand the defect the child has.

1.Prevent infection.

Monitor the newborn’s vital signs, neurologic signs, and behavior frequently;
administer prophylactic antibiotic as ordered; carry out routine aseptic technique;
cover the sac with a sterile dressing moistened in a warm sterile solution and change it
every 2 hours; the dressings may be covered with a plastic protective covering.

2.Promote skin integrity.

Placing a protective barrier between the anus and the sac may prevent
contamination with fecal material, and diapering is not advisable with a low defect.

3.Prevent contractures of lower extremities.

Newborns with spina bifida often have talipes equinovarus (clubfoot)

and congenital hip dysplasia (dislocation of the hips); if there is loss of motion in the
lower limbs because of the defect conduct range-of-motion exercises to prevent
contractures; position the newborn so that the hips are abducted and the feet are in a
neutral position; massage the knees and other bony prominences with lotion regularly,
then pad them, and protect them from irritation.

4.Proper positioning of the newborn.

Maintain the newborn in a prone position so that no pressure is placed on the sac;
after surgery, continue this positioning until the surgical site is well healed.

5.Promote family coping.

 Be especially sensitive to their needs and emotions; encourage family members to
express their feelings and emotions as openly as possible; provide privacy as needed
for the family to mourn together over their loss; encourage the family members to
cuddle and touch the newborn using proper precautions for the safety of the defect.

6.Provide family teaching.

Give family members information about the defect and encourage them to discuss
their concerns and ask questions; provide information about the newborn’s present
state, the proposed surgery, and follow-up care; information shall be provided in small
segments to facilitate comprehension; after the surgery, teach the family to hold the
newborn’s head, neck, and chest slightly raised in one hand during feeding; also teach
them that stroking the newborn’s cheeks helps stimulate sucking.
Pre-operative Care

1. Nursing Diagnosis: Risk of impaired skin integrity related to altered

motor and sensoryfunctions.

NursingInterventions

a.Avoid positioning the baby on back to prevent pressure on the sac.

b.Change the position of the baby frequently.

c.Provide meticulous skin and back care to keep the skin clean and dry.

d.Skin may be massaged periodically to stimulate blood circulation, with

special attention to bony prominences.

2. Nursing Diagnosis: Risk of injury and infection to the sac.

Nursing Interventions
 a. Do not position the baby on the back, as pressure over the sac may lead to
rupture of sac.

b. Clean the sac aseptically using normal saline.

c. Cover the sac with sterile gauze pieces dipped in normal saline. This
prevents the sac from dryness and rupture.

d. Change the wet saline gauze dressings every 2-4 hours, in order to keep the
sac moist.

e. Prevent contamination of the sac with urine and feces. So, do not put diaper,
as passing wine or stool inside the diaper keeps care giver unaware of soiling.

f.Frequently check the sac for any CSF leakage.

g.Prophylactic antibiotics must be administered to the infant.
h.Infant must be handled canefully while feeding or giving care, to prevent
injury to the sac.
i.While the infant is placed in side lying position, keep a pillow behind the
bad to prevent sudden rolling of infant on the bad. '
3. Nursing Diagnosis: Impaired bladder and bowel function related to
neurological deficit.

Nursing Interventions

a. Children with spina bifida may have the problem of retention of urine so
intermittent catheterization is done and also taught to parents.
b. Since bladder is an abdominal organ in infancy, pressure is applied gently on
the lower abdomer‘ to manually express urine.

c. Monitor for sign and symptoms of urinary tract infection like cloudy or foul-
smelling urine, fever etc. Administer prophylactic antibiotics to decrease the risk
of urinary tract infection among patients who are catheterized.

d. Administer increased amount of fluids to the child as it helps in flushing out

the bladder and urinary tract.

e. If the chiild has urine incontinence, then also catheterization is essential. In

case of bowel incontinence, keep the child clean and dry.

f. Provide, frequent buttod( care and perianal care to infants with fecal
incontinence, to prevent excoriation of perianal skin.

g. Provide soft bland food to the child having fecal incontinence. In case, the
child has constipation administer laxatives as prescribed.

4. Nursing Diagnosis: Inadequate cerebral tissue perfusion related to

complications like hydrocephalus.

Nursing Interventions

a. Monitor for signs of hydrocephalus like irritability, feeding difficulty, reduced

alertness, tense or bulging fontanelle etc.

b. Administer diuretics like Mannitol and Acetazolamide as prescribed, to reduce

the intra cranial pressure.
Postoperative Care
1.Nursing Diagnosis: Ineffective themioregulation related to surgery.

Nursing Interventions

a. Monitor the vital signs frequently after surgery.

b. Keep the infant warm, using radiant warmer or incubator.
c. Avoid exposing the child unnecessarily. w. Maintain thennoneutral
environment.

2. Nursing Diagnosis: Risk of development of infection related to surgery.

Nursing Interventions

a. Keep the operated site clean and dry. Observe for any drainage from the
operated site.

b. Keep the baby in prone position, to avoid pressure on the operated site.

c. Dressing of the site should be done using aseptic techniques.

d. Administer the prescribed antibiotics.

3. Nursing Diagnosis: Altered nutrition less than body requirement related to

disease process.

Nursing Intervention
a. Administer intravenous fluids as ordered.

b. . Maintain intake and output chart

c. Begin oral feeds as soon as infant starts tolerating the feeds.

d. Feed the infant in side lying position.

e. The baby can be held gently for feeding as soon as the surgical area is
sufficiently healed.

f. Burp the infant frequently between the feeds.

4. Nursing Diagnosis: Risk of development of complications related to the

disease process.

Nursing Intervention
a. In order to prevent development of hip or joint deformity, the nurse must pay
attention to the position in which the infant is placed.

b. An infant with associated hip dysplasia is placed in prone position with legs
slightly to moderately abducted. A pillow is placed between the knees to
counteract hip subluxation.

c.In order to reduce chances of CSF leakage from the incision site, the infant
may be placed in low trendelenburg's position as this helps in reducing CSF
pressure at the site.

d. Monitor for development of hyd rocephalus.

e. lf hydrocephalus is suspected, elevate the head end of bed to reduce the
intracranial pressure.

f. Provide passive range of motion exercises to prevent development of

contractures.
g. Educate the parents regarding surgeries that may be needed for correction of
deformities or . contractures .Such surgeries must be performed before the infant
begins to walk.

h. Clubfoot may be corrected by repeated application of plaster cast.

SUMMARY

As far we discussed about spina bifida, its definition,types,causes and clinical

features, diagnostic findings and management

CONCLUSION
Spina bifida is a birth defect that occurs when the spine and spinal cord
don't form properly. It falls under the broader category of neural tube defects.
The neural tube is the embryonic structure that eventually develops into the
baby's brain and spinal cord and the tissues that enclose them.Normally, the
neural tube forms early in pregnancy, and it closes by the 28th day after
conception. In babies with spina bifida, a portion of the neural tube fails to
develop or close properly, causing defects in the spinal cord and in the bones of
the spine.Spina bifida can range from mild to severe, depending on the type of
defect, size, location and complications. When early treatment for spina bifida
is necessary, it's done surgically, although such treatment doesn't always
completely resolve the problem
BIBLIOGRAPHY

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