Spina Bifida Taylor Finnell, Natalie Pennington, Brooke Johnson

Spina Bifida
Taylor Finnell, Natalie Pennington, Brooke Johnson
Brigham Young University
SPINA BIFIDA
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 a mental handicap. Spina Bifida is the most common birth defect that results in life
long disabilities.
What is Spina Bifida:
Spina bifida is a neural tube defect. It is a disorder involving incomplete development of the
brain, spinal cord, and/or their protective coverings that is caused by the failure of the babies
spine to close properly during the first month of pregnancy. This results in physical as well as
mental disabilities throughout life. Infants born with spina bifida sometimes have an open lesion
on their spine where significant damage to the nerves and spinal cord has occurred. These
openings vary in severity. Although the spinal opening can be surgically repaired shortly after
birth, the nerve damage is permanent, resulting in varying degrees of paralysis of the lower
limbs.
All cases of spina bifida can be grouped into three categories varying from lowest level of
severity to highest: occulta, meningocele, and myelomeningocele. Occulta is the mildest form
where one or more vertebrae are malformed but skin still covers the malformation. Occulta often
will go undiagnosed because it is so mild. Meningocele is more severe than Occulta.
Meningocele is where meninges protrude from the spinal opening. Depending on the case,
different formations of skin cover this defect. Myelomeningocele is the most severe where the
spinal cord and the meninges bulge from the opening in the spine often being exposed on the
outside of the body.
Spina bifida occurs at the end of the first month of pregnancy and can usually be prevented if the
mother takes adequate amounts of folic acid starting four months prior to pregnancy.
Types of Spina Bifida:
Spina Bifida is among the most common neural tube defect. The term bifida is derived from the
Latin word bifidus, or “left in 2 parts.” The equivalent Greek term, is derived from rachis,
meaning spine, and schisis, meaning division. Spina bifida is classified in two forms, spina bifida
occulta, also known as hidden spina bifida, and spina bifida manifesta made up of the two
conditions, meningocele and myelomeningocele. Myelomeningocele is also commonly called
spina bifida cystica.
Spina Bifida Occulta:
Spina bifida occulta is the mildest form of spina bifida. The individual with spina bifida occulta
contains at least one malformed vertebra; however, the nerves and spinal cord are normal and are
covered by a layer of skin. Most children with this type of condition never have health problems
and the spinal cord is often unaffected. Some can have symptoms if the hidden defect is severe
enough, thus, the condition is often referred to as hidden spina bifida. Since many individuals
classified as having spina bifida occulta are never professionally diagnosed, due to a lack of
physically apparent symptoms, researchers have difficulty identifying the conditions prevalence.
However, professionals from the Spina Bifida Association estimate that somewhere from 10 to
20 percent of the population have spina bifida occulta. That means that of the 4 million babies
born in the US each year, 1,500 to 2,000 have spina bifida.
Spina bifida occulta can refer to a number of issues in the neural tube and spinal cord.
Individuals are categorized as having spina bifida occulta if they have any abnormality within the
spinal cord. Abnormalities refer to a spinal cord that ends too thick, a tethered spinal cord that
does not attach to the rest of the body, fatty lumps inside the cord, if the skin on the back and the
spinal cord are connected by tissue, etc. Some specific forms of spina bifida occulta include:
•
Lipomyelomeningocele and lipomeningocele: a tethered spinal cord, except it is attached
to a benign fatty tumor
•
Thickened filum terminale: the end of the spinal cord is too thick
•
Fatty filum terminale: there is a fatty lump at the inside end of the spinal cord;
•
Diastematomyelia (split spinal cord) and diplomyelia: the spinal cord is split in two,
usually by a piece of bone or cartilage; and
•
Dermal sinus tract (with involvement of the spinal cord): the spinal canal and the skin of
the back are connected by what looks like a band of tissue
Individuals with these forms of Spina Bifida Occulta, even if little is wrong with the spine,
may experience slight symptoms. Some of these symptoms
include pain, weakness, or numbness in the back or legs; or
change in bladder or bowel function. Eighty percent of
infants with mild neural tube defects will have a slight
physical manifestation. The condition could be shown as a
hairy patch, a fatty lump, a dark spot or birth mark, a skin
tract or sinus, or a hypopigmented spot (area with less skin
color).
Few people with spina bifida occulta will need treatment.
Most are able to participate in the same level of quality of
life as their peers.
Coinciding Difficulties:
Hydrocephalus
Professionals estimate that 70%-90% of children born with myelomeningocele and other
forms of spina bifida also suffer from hydrocephalus. This is a condition in which the primary
characteristic is excessive accumulation of fluid in the brain. Although hydrocephalus was once
known as "water on the brain," the "water" is actually cerebrospinal fluid (a clear fluid that
surrounds the brain and spinal cord). The excessive accumulation of cerebral spinal fluid results
in an abnormal widening of spaces in the brain called ventricles. This widening creates
potentially harmful pressure on the tissues of the brain.
The purpose of cerebral spinal fluid is to keep the
brain tissue buoyant, acting as a cushion or “shock
absorber”; acts as a vehicle for delivering nutrients
to brain and removing waste; and to flow between
the cranium and spine to compensate for changes
in intracranial blood volume. Medical conditions
that block the normal flow of cerebral spinal fluid
will result in an over-accumulation of fluid. The
resulting pressure of the fluid against the brain tissue causes hydrocephalus.
The most common treatment for hydrocephalus is to insert a tube, called a shunt, to drain excess
fluid from the head to another place where the body can remove it naturally, this usually occurs
in the abdomen. Shunts have valves that regulate both the direction and amount of fluid that is
drained. All shunts have three parts: a ventricular catheter to reach the area where there is too
much fluid, a valve to control flow (there are many types) and tubing to carry the fluid from one
place in the body to another. Almost all shunts are put in during the first days or weeks after
birth. Sometimes the shunt will be inserted at the time of the initial back closure.
Most people with Spina Bifida and shunted hydrocephalus will need a shunt for life. About 20
percent of people with Spina Bifida will need more than one shunt revision. If a shunt is not
implanted, the pressure buildup can cause conditions including brain damage, seizures, or
blindness.
Latex Allergies:
People who have Spina Bifida and catheterize; or have several surgeries from very early in life,
such as bladder surgery or shunt revisions, are at very high risk for allergy because of a
“cumulative” effect over time. Symptoms of latex sensitivity can be minor, but without warning,
may become life threatening. Research has found that up to 73% of children and adolescents
with spina bifida are sensitive to natural rubber latex. This is important to be aware of as latex is
the substance often used to make surgical gloves and bandages.
MYELOMENINGOCELE
Definition:
Myelomeningocele is the most common type of spina bifida and is also the most severe type. It is
a neural tube defect in which the bones of the spine do not completely form, resulting in an
incomplete spinal canal. This causes the spinal cord and meninges to stick out of the child’s
back. Myelomeningocele may affect as many as 1 out of every 800 infants.
Symptoms:

Partial or complete lack of sensation in the legs due to the nerve damage that is involved.
This may result in a patient using aids such as a wheelchair, leg braces, or crutches for
the entirety of their life.

Loss of bladder or bowel control. Spina bifida often inhibits a persons ability to
voluntarily relax their muscles needed to control bladder and bowel.

Weakness of the hips, legs, or feet of a newborn. These deformities are most commonly a
direct result of the tethered spinal cord.

Abnormal feet or legs, such as clubfoot.

Build-up of fluid inside the skull (hydrocephalus).

Hair at the back part of the pelvis.
Treatment:
The most common treatment for people with myelomeningocele is after birth surgery. This
surgery is to repair the defect and is usually recommended at an early age. Before surgery, the
infant must be handled carefully to reduce any damage to the exposed spinal cord.
Due to modern technology and an advancing medical world, current practices have started doing
pre-birth surgical repairs to children with early-detected spina bifida. Doctors can surgically
repair the child’s spinal and brain malformation before the child is ever born or completely
developed resulting in minimal disabilities to be present in life.
MENINGOCELE
Spina Bifida Manifesta includes two types of Spina Bifida: Meningocele and Myelomeningocele.
Meningocele is the term for the protruding sac that results from the meninges pushing through
the vertebrae of the spine. These meninges are the membranes responsible for protecting and
covering the spinal cord. The sac contains fluid and membranes, but in this form of Spina Bifida,
there is no protrusion of the spinal cord.
Treatment:
Meningocele repair: surgery to repair the abnormal opening where the sack protrudes from the
back. This is done by draining the fluid and closing the opening. If the meningocele is not
covered by a layer of skin, this surgery is necessary to decrease the risk of infection and to
protect the spinal column and the tissue inside.
TESTING AND DIAGNOSIS
Prenatal Tests:
These tests are a few common screening methods done in the second trimester of pregnancy (16
to 18 weeks gestation).

Maternal Serum Alpha Fetoprotein Screening (MSAFP):
This test measures the level of alpha-fetoprotein (AFP), a natural protein found in
the placenta. This can be done by testing the mother’s blood. It is common for some AFP
to cross the placenta, so a small amount in the mother’s bloodstream should cause no
alarm. Abnormally high levels of AFP correlate with Spina Bifida because it indicates
that the fetus may have an open neural tube defect that is allowing the AFP to be released
at a more rapid rate. However, this is not a definite indication of spina bifida. Rather, it is
an indication that further testing may be recommended. Proper gestastional dates are
important for accurate readings.

Ultrasound: This is used to check the anatomy of the fetus. An advanced ultrasound can
detect certain characteristics of spina bifida such as location and severity of spinal
defects.

Maternal Amniocentisis: Typically done if previous tests suggest spina bifida. In
amniocentisis, the doctor uses a needle to take a sample of amniotic fluid. This fluid is
then tested to determine the level of AFP present. If the levels are elevated, this indicated
the presence of a neural tube defect. To confirm this conclusion, a second test on the
same fluid can be done. While this can to confirm whether or not the fetus has spina
bifida, it can also increase the chance of miscarriage.
Postnatal Tests:
Mild cases of spina bifida occulta that are not diagnosed prenatally may be found during routine
MRIs or X-rays. Also, if the doctor suspects hydrocephalus, a CT scan or X-ray of the skull
would be used to detect fluid in the brain.
Risk Factors

Maternal age. Spina bifida is more commonly seen in teenage mothers.

History of miscarriage.

Birth order. First-born infants are at higher risk.

Socioeconomic status. Children born into lower socioeconomic families are at higher
risk for developing spina bifida. It is thought that a poor diet, lacking essential vitamins
and minerals, may be a contributing factor.

Race. Spina bifida is more common among whites and Hispanics.

Family history of neural tube defects

Folate deficiency. Folate is the natural form of vitamin B-9. The synthetic form is called
folic acid. Folic acid can be found in vitamins, supplements, and enriched foods.

Diabetes. If blood sugar is not controlled.

Obesity.

Some medications. Anti-seizure medications seem to cause neural tube defects when
take during pregnancy.
PHYSICAL EFFECTS
Levels of paralysis and physical disabilities vary in spina bifida due to the location of the defect.
The higher up the defect is on the spine, the more paralysis the child will most likely have. Spina
bifida can result in little or no paralysis to complete paralysis and dependence.
The image below shows where the different areas of spinal defect are and how they affect one’s
ability to control muscle movement.
PREVENTION
FOLIC ACID:
It is important to start taking all vitamins up to three months prior to pregnancy so that needed
vitamins are already in the mother's system. However, it is crucial that mothers and potential
mothers focus on taking Folic Acid supplements in regards to spina bifida. Statistically, 80-90%
of people who are diagnosed with spina bifida could have been prevented had the mother had
adequate levels of folic acid in her system.
Folic acid is an important vitamin in the development of a healthy fetus. It is recommended that
all women of childbearing age consume 400 micrograms of folic acid daily. Foods high in folic
acid include dark green vegetables, egg yolks, and some fruits. Many foods—such as some
breakfast cereals, enriched breads, flours, pastas, rice, and other grain products—are now
fortified with folic acid. Most multivitamins contain this recommended dosage of folic acid.
Consuming the correct amount of folic acid
Folic acid is crucial in the development of a fetus because folic acid is a key component in
making cells. Taking just 400 micrograms of folic acid a day will reduce the incidence of spina
bifida by over 70%.
THERAPEUTIC RECREATION IMPLICATIONS
Therapeutic Recreation plays a major part in many individuals’ lives. People with spina
bifida can benefit from TR in a myriad of ways, including increased self-awareness, muscle
strength, self-efficacy, and motivation. It is commonly utilized in rehabilitation of individuals.
One of the most important roles of Therapeutic Recreation in the lives of those with spina
bifida is possibly leisure education. There are so many things that can still be done from a
wheelchair, or any other aids that are used. A Recreation Therapist can push those they work
with to explore new boundaries and therefore assist them in discovering their true rather than
perceived limitations. There are many sports camps that serve people with spina bifida,
especially those with the more severe forms of spina bifida. Games such as wheelchair basketball
and tennis are common.
Works Cites
http://www.spinabifidaassociation.org
http://emedicine.medscape.com/article/311113-overview
http://kidshealth.org/parent/system/ill/spina_bifida.html
http://nervous-system.emedtv.com/spina-bifida-occulta/spina-bifida-occulta-p2.html
http://www.spinabifidaassociation.org
http://kidshealth.org/parent/system/ill/spina_bifida.html
http://emedicine.medscape.com/article/2040534-overview#a30
http://www.chw.org/display/PPF/DocID/22505/router.asp
http://www.webmd.com/parenting/baby/tc/spina-bifida-topic-overview?page=2
http://emedicine.medscape.com/article/2040534-overview#a30