CHAPTER 2

SPINA BIFIDA MEDICAL CONCEPT

2.1 Definition
Spina bifida comes from the latin word divided spine, is a group of
neural tube defects that involves the brain and the spinal cord and/or
meninges. It occurs when the neural tube does not close during the babys
development.
Spina bifida is a birth defect that happens when a baby's backbone (spine)
does not form normally. As a result, the spinal cord and the nerves that branch
out of it may be damaged. Sometimes, the defect causes an opening in the
back, which is visible. The spinal cord and its coverings sometimes push
through this opening. Other times, there is no opening and the defect remains
hidden under the skin. Depending on the severity of the defect and where it is
on the spine, symptoms vary. Mild defects may cause few or no problems,
while more severe defects can cause serious problems, including weakness,
loss of bladder control, or paralysis
2.2 Clasification
1. Spina Bifida Occulta
Occulta is Latin for "hidden". This is the mildest form of spina
bifida. In occulta, the outer part of some of the vertebrae is not completely
closed. The splits in the vertebrae are so small that the spinal cord does
not protrude. The skin at the site of the lesion may be normal, or it may
have some hair growing from it; there may be a dimple in the skin, or a
birthmark. The incidence of spina bifida occulta is approximately 10-20%
of the population, and most people are diagnosed incidentally from spinal
X-rays.
2. Meningocele
Meningokel have milder symptoms than myelomeningokel because
the spinal cord is not out of the bone protector, meningocele is
meningens protruding through the vertebrae that are not intact and
palpable as a fluid-filled lumps under the skin and is characterized by the
prominence of the meninges, the spinal cord and cerebrospinal fluid.
Meningokel like sac at the waist, but here there are no protrusions corda
spinal nerves. Babies with this condition can have health problems if the
nerves around the spine are damaged. For example, if the nerves that
7

control the release of the bowels or bladder are affected, it may be

difficult for a child to control these body functions. They also might have
trouble moving certain muscles (paralysis).
3. Myelomeningocele
Myelomeningocele, also known as meningomyelocele, is the type of
spina bifida that often results in the most severe complications.In
individuals with myelomeningocele, the unfused portion of the spinal
column allows the spinal cord to protrude through an opening. The
meningeal membranes that cover the spinal cord also protrude through
the opening, forming a sac enclosing the spinal elements.
A baby with this type of spina bifida usually has some paralysis, and
muscle or bone problems as a result of the paralysis. This is due to the
abnormal development of nerves in the spine, or to nerves being
stretched as a result of the defect. It's also common for babies to
have hydrocephalus, a buildup of cerebrospinal fluid in and around the
brain. This causes the baby to have an enlarged head or bulging soft spot
at birth, which is the result of too much fluid and pressure inside the
skull.
2.3 Etiologi
1. Predisposing Factor
a. Rationale Age
Infants and children are more likely to have this condition because it
is a birth defect.

b. Heredity
Couples who have spina bifida are more likely to have a child with
spina bifida. Individuals who have had spina bifida are at risk of
having a child with the same condition.
2. Precipitating Factors
a. Rationale Nutrition
women who do not take folic acid or lack of folic acid prior to
pregnancy and/or during early pregnancy have a higher risk of
having spina bifida than women who take folic acid.
b. Anti Convulsants

Valproic acid and carbamazepine belong to a group of drugs called

folic acid antagonist. This group interferes with the bodys use of
folic acid.
c. Socio-Economic Factors
Socio-economic status of the person would also affect the risk of
having spina bifida because of the decrease compliance of nutrition
and supplements of the mother during pregnancy
d. Diabetes
Women who have diabetes before they become pregnant are at
higher risk of having a child with spina bifida and other types of
birth defects.
e. Increase Body Temperature
Studies suggest that if a womans body temperature goes up to 101 102F (38.33 38.88C) during early pregnancy, it would double the
risk of having a child with spina bifida.
2.4 Sign and Symptom
Symptoms of spina bifida is varies, depending on the severity of the
damage that occurs in the spinal cord and nerve roots are affected.
1. Paralysis
2. Orthopedic abnormalities (i.e., deformed feet, hip dislocation, scoliosis)
3. Bladder and bowel control problems, including incontinence, urinary
tract infections, and poor kidney function
4. Pressure sores and skin irritations
5. Abnormal eye movement
6. 68% of children with spina bifida have an allergy to latex, ranging from
mild to life-threatening.
7. An abnormal tuft of hair
2.5 Patofisiologi
During the first month of pregnancy the upper part of the spinal cord
and the brain is formed, followed by the formation of the lower spinal
cord at 5 6 weeks during pregnancy. An interruption in the formation
would result to failure in closure of the spinal cord leaving a defect in the
spine. Factors affecting failure of closure would include the in adequate
folic acid intake which is used by the body for cell production and
development; anticonvulsant which is a folic acid antagonist blocks the
absorption of folic acid. Having a history of diabetes before or during
pregnancy with high levels of glucose in the body would also affect the

10

closure of the spine as well as having a high body temperature during

pregnancy.
In the first month of pregnancy, the central nervous system of the baby
starts to develop. Any slight interruption of the development will lead to a
congenital defect. If it will occur, there will be a defect in the spinal cord
in which the neural tube will fails to close thus creating an opening and
forms a protrusion of the meninges through the spinal space and what is
now called the Meningocele. If treated through surgical repair of
meningocele it would lead to a good prognosis. But if not, the protrusion
will create an obstruction. Due to the obstruction, there will be an indirect
flow of cerebro-spinal fluid in and out of the spinal cord and it will
accumulate in the brain creating now a hydrocephalus. Hydrocephalus if
treated with VP shunt would either lead to a good prognosis or would
create a shunt complication including shunt malfunction, clogged VP
shunt or infection. If not treated, the cerebrospinal fluid will still continue
to accumulate in the brain and in the long term the person would develop
learning disabilities and would further complicate to mental retardation.
Meanwhile if there is a continuous accumulation of the CSF, it cannot
circulate properly and would force its way below the spinal cord. If that
happens there would be a displacement of the foramen magnum and
creates a herniation of the cerebellum, this condition is called Chiari II
malformation in which the person would experience headache, nausea and
vomiting, dizziness, increased intra cranial pressure. If not treated there
would be a compression of the spinal cord making the person feel choking
sensation, arm stiffness, difficulty in feeding, swallowing and breathing
and eventually would die.
.

11

2.6 Pathway
Precipitating Factors:
- Nutrition
- Medication
- Socio-economic factors
- Diabetes
- Increased body temp

Predisposing Factors:
- Age
- Heredity
1 st Month of
central

Central Nervous
System begins to form

Defect in the spinal

Protruding sac through the

defect- containing meninges
Dx:
- Translumination
- CT scan
- MRI

Meningocele

If treated:

Surgical repair of
menigocele

If not

No direct flow of CSF

to the spinal cord

12

Obstruction of prognosis
fluid in the brain

Acute pain

CSF unable to
circulate

s/sx:
- Increased us ICP
- Increase head
circumferenc

Hydrocephal

If treated

If not

- VP

Good

Shunt
complication

s/sx:
- Headache
- Nausea &
vomiting
- Fever

Accumulation of CSF
in the brain

fluid may possibly

forced

Displacement of
foramen

Fluid still
accumulated
in the
prognosis

Learning
disabilities

Mental
retardation

Chiari II

s/sx:
Headache
Muscle weakness
Increased ICP
Dizziness
- nausea
If not treated:

Compression of the
spinal

s/sx:
- Choking
- arm stiffness
- difficulty in feeding
- swallowing, and

13

2.7 Complication
Other complications of spina bifida associated with the birth include:
1. Paralysis Cerebri
2. Mental Retardation
3. Muscle Atrophy
4. Osteoporosis
5. Fracture (due to decreased muscle mass).
2.8 Support Examination
In the first trimester pregnant women do a blood test called Triple
Screen. This test is a screening test for spina bifida, Down syndrome and
other congenital abnormalities. 85% of women having a baby with spina
bifida will have high levels of serum alpha feytoprotein. After the baby is
born, the examination:
1. Spine x-rays to determine the extent and location of abnormalities.
2. Spinal Ultrasound can show the spinal cord and vertebrae
abnormalities
3. CT scan or MRI of the spine sometimes performed to determine the
location and extent of abnormality.
2.9 Management
1. Medical management
a. Myelomeningocele surgery to prevent rupture.
b. Surgical repair the spinal lesion and shunt CSS in infants
hidrocefalus conducted at birth.
c. Skin grafting is required when large lesions.
d. Prophylactic antibiotics to prevent meningitis.
e. Drugs that can be given is: Antibiotic, anticolinergic, laxatives
2. Nursing management
a. Pre-operasi
Immediately after birth exposed areas should be covered.
Keep the body temperature that can drop rapidly. muscle activity
in the lower limbs and anal spingter will be recorded by

14

Physiotherapists. Oksipito circle-frontal head is measured and

graphed.
b. Post-operasi
1.) General post-surgical treatment of neonates
2.) Providing food can be given orally 4 hours after surgery.
3.) If there is a suction drain the wound should be checked every
hour.
4.) keep the skin over the perineum and buttocks remain intact.
5.) Prolapse recti an early problems due to paralysis of the pelvis
muscles and try to wear the belt on the buttocks.
6.) The circle head is measured and graphed once or twice a week.
2.10

Outlook
After recovery from surgery, babies born with a

meningocele or myelomeningocele may need long-term

care to help treat any underlying conditions that result
from their spina bifida. Those with paralysis may
eventually need walking aids like leg braces, walkers, or
a wheelchair.
With the right medical care, kids can go on to lead
normal, active lives. The goal is to create a lifestyle for
them and their families in which their disability interferes
as little as possible with normal everyday activities.

2.11

Prevention
Many cases of spina bifida can be prevented if women

of childbearing age take 0.4 milligrams (400 micrograms)

of folic acid every day before pregnancy and continue to
take it throughout the first trimester. Women don't find
out that their pregnant until 4 to 5 weeks into the
pregnancy, it is important to start taking folic acid before

15

becoming pregnant. This provides the best protection for

an unborn baby. Good sources of folic acid include eggs,
orange juice, and dark green leafy vegetables. Many
multivitamins contain the recommended dose of folic
acid, too.