SPINA BIFIDA

Definition

Spina Bifida is a congenital abnormality with developmental defect in the spinal column with
incomplete closure of vertebral canal due to failure in fusion of vertebral arches.

It pertains to the defective closure of the vertebral column and it is typically apparent or
noticeable at birth. It is most common in the areas such as lower thoracic, lumbar, or sacral
region and usually extends for 3 to 6 vertebral segments.

Simple Definition: Spina bifida is a birth defect and a type of neural tube defect that occurs
when the spine and spinal cord are not able to form and develop properly.

Spina bifida has the possibility to occur along the spine if the neural tube isn’t able to close or
develop well.

What is the neural tube?

Embryonic precursor to the central nervous system. It is a tube in the embryo which is
considered the 'first draft' of the brain and spinal cord. The neural tube later becomes the
baby=s spinal cord, spine, brain, and skull.

When the neural tube doesn’t close all the way, the backbone which provides protection to the
spinal cord doesn’t form and close as it normally should. Thus, this often results in damage to
the spinal cord and nerves.

The severity of spina bifida depends on particular factors such as: the size and location of the
opening in the spine and as well as whether the part of the spinal cord and nerves are affected.

Additional Notes:
It is one of the most serious neural tube defects compatible with prolonged life.

Prevalence:
This defect is one of the more common congenital anomalies overall, with an incidence in the
US of about 1/1500. Here in the Philippines, a study at the Philippine General Hospital showed
that the prevalence of spina bifida ranged from 4.4 to 8.12 out of 10,000 births or admissions.

There are several types or forms of Spina Bifida and the most common ones are: Spina Bifida
Occulta, Closed Neural Tube Defects, Myelomeningocele, and Meningocele.

Spina Bifida Occulta

It is considered to be as the mildest type of spina bifida and does not usually cause any
problems or disabilities. However, it is also deemed to be the most common type. In this type of
spina bifida, there is a small gap in the spine, but there is no opening/pouch/sac on the back.
The spine is open, but the spinal cord is not out of the usual placement. It is also known as the
“hidden spina bifida” due to the fact that an individual may not realize that they have this kind of
condition. In the cause of a newborn, there might be signs such as a tuft of hair, a small dimple,
or a birthmark.

Myelomeningocele

This type of spina bifida is the most severe form and the most serious type. It happens when
parts of the spinal cord and nerves come through the open part of the spine. It causes nerve
damage and other disabilities since a sac of fluid comes through an opening in the baby’s back
and also, part of the spinal cord and nerves are in this sac. In some cases, sacs are covered
with skin, but in other cases, tissue and nerves may be exposed. This type of spina bifida
causes moderate to severe disabilities, such as problems affecting how the person goes to the
bathroom, loss of feeling in the person’s legs or feet, and not being able to move the legs.
Basically:
 The spinal canal remains open along several vertebrae in the lower or middle back
 Both the membranes and the spinal cord or nerves protrude at birth, forming a sac
 Tissues and nerves usually are exposed, though sometimes skin covers the sac

Also, for this case, it is common for patients (most particularly infants) to develop
hydrocephalus.

Why?
The opening in the spinal cord also results in loss of the fluid surrounding the nervous system.
This causes the brain to be positioned further down into the upper spinal column than normal,
which is called an Arnold Chiari II malformation. When this happens, the normal flow of fluid out
of the brain is obstructed, causing Hydrocephalus, an excess of cerebrospinal fluid within the
brain.

Meningocele

It is a rare type of spina bifida which is characterized by a sac of spinal fluid bulging through an
opening in the spine. The spinal cord is in its normal place in the spinal canal. No nerves are
affected in this type, and the spinal cord isn't in the fluid sac. The sac is often covered by skin
and may require surgery. This type of spina bifida can cause minor disabilities. For babies with
this type of spina bifida, they might have some minor problems with functioning, including those
affecting the bladder and bowels.

Causes

The exact cause of spina bifida is still unknown until today. Nevertheless, there are certain
factors that could possibly add up to the cause of this condition. These include the following:
folate deficiency, genetics, use of certain medications, and maternal diabetes.

**Remember:

Folic acid (also known as vitamin B9)

 Occurs naturally in some foods, such as broccoli, peas and brown rice.
 It's also added to foods, such as some breakfast cereals. Folic acid tablets are available
from pharmacies and supermarkets, or a GP may be able to prescribe them for you.
Folic acid is essential for women’s health and is especially important during pregnancy

Folate deficiency or low folic acid intake during pregnancy is considered to be a very significant
factor that can highly increase the chances of a pregnant mother having a child with spina
bifida.
 This is due to the fact that within the first 28 days of pregnancy, the neural tube, which is
responsible for the development of the brain and spine, closes.
 Inadequate concentrations of folate can prevent closure of the neural tube, which can
lead to a neural tube defect.
 Why is it important?
o The fast rate of cellular and tissue growth during pregnancy requires greater
amount of dietary folate for proper DNA synthesis.

Another would be having a family history of spina bifida (or other kinds of neural tube defects)
increases the chances of the mother of having a baby with spina bifid as well.
 Over the years of research and study, small percentage of cases have been reported to
run in families.
 However, the condition does not have a clear pattern of inheritance although genetics is
still regarded to be one of the factors.
Taking certain medications has also been linked to an increased risk of having a baby with
spina bifida or other birth defects. Common medications that increases this risk would be
valproic acid and carbamazepine (which are commonly used to treat epilepsy, bipolar disorder,
and other mental health problems).

 How these medications cause birth defects (including spina bifida) can be quite
complicated
 But the simplest explanation for this is that teratogenesis occur wherein VPA (valproic
acid) crosses the placenta and interferes with normal development causing
developmental abnormalities in the fetus.

Signs and Symptoms

For children with minor problems or for those with mild spina bifida, it could be asymptomatic.
However, there can be several manifestations, signs, and symptoms for those with other types
of spina bifida, especially the severe ones.

Problems in Movement

Paralysis (varies from weakness or total paralysis) and sensory deficits could occur, especially if
the spinal cord and lumbosacral nerve roots are affected and is involved in the problem.

Bowel Problems
The rectal tone could be decreased. Many of those with spina bifida have limited or no control
over their sphincter muscles and have bowel incontinence.

Rectal tone = “sphincter tone” = pressure over anal sphincter

Fetal Incontinence = The inability to control bowel movements, causing stool (feces) to leak
unexpectedly from the rectum

Bladder Problems

The nerves that control the bladder are affected as well since they have not formed properly. It
can cause manifestations such as urinary incontinence, hydronephrosis, and kidney stones.
Paralysis also impairs bladder function, occasionally leading to a neurogenic bladder and,
consequently, urinary reflux.

Urinary Incontinence
 Involuntary leakage of urine. It means a person urinates when they do not want to.

Hydronephrosis
 Abnormal enlargement of a kidney, which may be caused by blockage of the ureter
(such as by a kidney stone) or chronic kidney disease that prevents urine from draining
into the bladder.

Urinary Reflux
 “Vesicoureteral Reflux”
 Urine flows backward from the bladder to one or both ureters and sometimes to the
kidneys.

Neurological Problems
Babies could also develop hydrocephalus which can lead on to further damage in the brain and
other complications. A number of those with spina bifida have also shown learning difficulties
but most have normal intelligence still.

Why?
The opening in the spinal cord also results in loss of the fluid surrounding the nervous system.
This causes the brain to be positioned further down into the upper spinal column than normal,
which is called an Arnold Chiari II malformation. When this happens, the normal flow of fluid out
of the brain is obstructed, causing Hydrocephalus, an excess of cerebrospinal fluid within the
brain.
Skin Problems

Spina bifida could cause reduced sensations since the nerves are already affected. There is
also a high probability that those with spina bifida can develop an allergic reaction to latex.

Regarding latex allergy

 The exact cause is still unknown
 However, the most accepter theory or concept is that is due to the early, intense, and
constant exposure to latex products due to several medical procedures or interventions.
 Children with spina bifida (SB) have a high degree of exposure to latex products as a
consequence of repeated surgical procedures, implantation of latex-containing materials
and catheterisation. The consequence is a higher incidence of latex allergic reactions.
 Multiple surgeries, diagnostic tests and examinations, also from bladder and bowel
programs.

Laboratory Tests or Diagnostic Tests

Imaging scans such as ultrasound, MRI, and CT scan can be required in order to have a clear
visualization of the infant’s spine and bones in the back. Aside from that, other tests that could
indicate spina bifida are also done.
 High-resolution level II ultrasound
o Similar to the standard ultrasound
o Main difference is that by the name itself, it produces more detailed and high-
resolution images.
o Used to confirm the diagnosis and determine the location of the lesion, and to
assess for any other birth defects including spina bifida itself.
 Ultrafast fetal MRI
o Images are quickly captured using a “snapshot” technique.
o Performed to confirm presence of the Chiari II malformation and to screen for
evidence of any other neurologic abnormalities which could indicate SB
 Amniocentesis
o Procedure in which a sample of amniotic fluid is obtained and removed from the
uterus for testing or treatment.
o Done to confirm the presence of elevated amniotic fluid alpha-fetoprotein
(AFAFP) levels and acetylcholinesterase (AChE), which indicate open neural
tube defects.
o AFP Levels
 Alpha-fetoprotein (AFP) s a protein produced in the liver of a developing
fetus.
 During a baby's development, some AFP passes through the placenta
and into the mother's blood.
 0–40 nanograms per milliliter (ng/mL) = Normal
 Because the skin surrounding the baby's spine is gone and AFP leaks
into the amniotic sac.
 Maternal serum alpha-fetoprotein (MSAFP) test
o Also done to confirm elevated AFP levels in the mother’s blood
o Blood test = blood sample
o AFP Levels also show up in the blood

Medical Management

Three medical procedures are often done to manage spina bifida and its symptoms or
complications and these include the following:
 Surgical repair of the spinal lesion
 In babies with spina bifida, nerves and membranes can push out of an opening in
the spine and form a sac. This damages the nerves and can lead to serious
 infections, so your baby will usually have surgery to repair the spine within 48
hours of birth.
 During surgery, the surgeon will put the spinal cord and any exposed tissues or
nerves back into the correct place. The gap in the spine is then closed and the
hole sealed with muscle and skin.
 Although this will repair the defect, unfortunately it cannot reverse any nerve
damage.
 Sometimes a ventricular shunt
 This is often done if the baby has developed hydrocephalus.
 The surgeon will implant a thin tube called a shunt to drain away excess fluid to
another part of the body, usually the stomach.
 The shunt will usually need to remain in place for the rest of the child's life.
 Further surgery may be needed if the shunt becomes blocked or infected and/or
the child grows out of the shunt and needs a larger one.
 Various measures for orthopedic and urologic complications
 This includes physiotherapy, occupational therapy, and employing the use of
mobility aids.
 In order for the child to develop and grow as normally as possible
 For them to adapt well

Nevertheless, we need to keep in mind that spina bifida and its effects along with the needed
treatment/management could vary according to its type and severity.

Not all people born with spina bifida have the same needs, so treatment will be different for
each person. Some people have problems that are more serious than others. People with
myelomeningocele and meningocele will need more treatments than people with spina bifida
occulta.

Nursing Management
 Responsibilities and tasks of a nurse
 Utilizing the nursing process or our ADPIE

1) Assessment
- The very first thing that nurses must do.

Routine newborn examination is conducted by the nurses while giving particular emphasis
and attention to any neurologic impairment. When gathering data during physical examination,
observe the movement and response to stimuli of the lower extremities; carefully measure the
head circumference and examine the fontanelles. It is also the responsibility of the nurse to
assess the knowledge of the parent/family members/guardians regarding the defect present in
the baby. Determine the family’s knowledge and understanding of the defect, as well as their
attitude concerning the birth of a newborn with such serious problems.

2) Diagnosis

Several nursing diagnosis can be formulated based on the data gathered from the assessment
phase such as:
 Risk for infection related to vulnerability of the myelomeningocele sac.
 Risk for impaired skin integrity related to exposure to urine and feces.
 Risk for injury related to neuromuscular impairment.
 Compromised family coping related to perceived loss of the perfect newborn.
 Deficient knowledge of the family caregivers related to the complexities of caring for a
newborn with serious neurologic and musculoskeletal defects.

3) Planning

As nurses, the major goals for the patient would be to:

 Prevent infection.
 Maintain skin integrity.
 Prevent trauma related to disuse.
 Increase family coping skills, education about the condition, and support.
4) Interventions
 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.
 Maintain 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.
 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.
 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.
 Increase family coping skills, education about the condition, and support.
 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.
 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.

5) Evaluation
- How effective ang management and care?

The expected results and outcomes from the patient include the following:
 Prevented infection.
 Maintained skin integrity.
 Prevented trauma related to disuse.
 Increased family coping skills, education about the condition, and support.