Spina bifida (Latin: "split spine") is a developmental birth defect caused by the incomplete closure of

the embryonic neural tube. Some vertebrae overlying the spinal cord are not fully formed and
remain unfused and open. If the opening is large enough, this allows a portion of the spinal cord to
protrude through the opening in the bones. There may or may not be a fluid-filled sac surrounding
the spinal cord. Other neural tube defects include anencephaly, a condition in which the portion of
the neural tube which will become the cerebrum does not close, and encephalocele, which results
when other parts of the brain remain unfused.

Spina bifida malformations fall into four categories: spina bifida occulta, spina bifida cystica
(myelomeningocele), meningocele and lipomeningocele. The most common location of the
malformations is the lumbar and sacral areas . Myelomeningocele is the most significant form and it
is this that leads to disability in most affected individuals. The terms spina bifida and
myelomeningocele are usually used interchangeably.

Spina bifida can be surgically closed after birth, but this does not restore normal function to the
affected part of the spinal cord. Intrauterine surgery for spina bifida has also been performed and
the safety and efficacy of this procedure is currently being investigated. The incidence of spina bifida
can be decreased by up to 75% when daily folic acid supplements are taken prior to conception.

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Spina bifida is a birth defect that involves the incomplete development of the spinal cord or its
coverings. The term spina bifida comes from Latin and literally means "split" or "open" spine.

Spina bifida occurs at the end of the first month of pregnancy when the two sides of the embryo's
spine fail to join together, leaving an open area. In some cases, the spinal cord or other membranes
may push through this opening in the back. The condition usually is detected before a baby is born
and treated right away.

Types of Spina Bifida

The causes of spina bifida are largely unknown. Some evidence suggests that genes may play a role,
but in most cases there is no familial connection. A high fever during pregnancy may increase a
woman's chances of having a baby with spina bifida. Women with epilepsy who have taken the drug
valproic acid to control seizures may have an increased risk of having a baby with 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:

1. 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.

2. 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.

Because of the abnormal development of and damage to the spinal cord, a child with
myelomeningocele typically has some paralysis. The degree of paralysis largely depends on where
the opening occurs in the spine. The higher the opening is on the back, the more severe the paralysis
tends to be.

Children with spina bifida often have problems with bowel and bladder control, and some may have
attention deficit hyperactivity disorder (ADHD) or other learning difficulties, such as hand-eye
coordination problems.

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Diagnosis. The patient was diagnosed with a neuropathic foot ulcer, likely secondary to spina bifida.

Spina bifida. Spina bifida is one of several possible congenital anomalies of the spine. The term spinal
dysraphism encompasses any state in which there is incomplete fusion of the midline structures of
the back and may affect somatic ectodermal, neuroectodermal, or mesodermal tissues. Other
dysraphic states include meningocele, myelomeningocele, and diastematomyelia. Abnormalities that
may be associated with dysraphic states include tethered cord, intraspinal lipoma, fibrous bands,
intraspinal lipomas, dermal sinuses, dermoid cysts, lipomyelomeningoceles, and neurofibromas.[20]
Several of these anomalies may be associated together, since skin and nerve tissue both originate
from ectodermal embryonic tissues. This article will focus on the discussion of spina bifida and
neuropathic associations, which may eventually manifest as lower extremity ulcerations, as it did in
this patient.

In the United States, spina bifida is the most common major birth defect among live-born infants.
[14] The incidence is 0.15 percent among the White population and 0.04 percent among the Black
population. Girls are more frequently affected by this disease than are boys, and the etiology in most
cases is not known. Genetics may play a role, since there is an increased risk of recurrence in the
families of affected children. There is evidence that a deficiency of folic acid may be an etiologic
factor. Possible teratogenic factors include valproic acid, maternal gestational diabetes, and
maternal hyperthermia.[15]

Spina bifida cystica. Spina bifida cystica is the most common defect of incomplete fusion and is
manifested as a meningocele or myelomeningocele, which usually presents with neurologic signs
evident at birth.[15] This condition is believed to occur before the 26th day of gestation.[21] A
meningocele is a protrusion of the meninges without neural elements through a vertebral defect.
Meningoceles are usually cystic, covered by skin, and full of cerebrospinal fluid. Myelomeningocele
refers to a protrusion through a vertebral defect in which the spinal cord or spinal roots are also
herniated. The majority of meningoceles and myelomeningoceles occur in the lumbar and
lumbosacral regions, and the incidence is said to be between one and five per 100 live births, with
the highest incidence in the northern regions of the British Isles.[21]

Clinical features of these lesions relate to the extent and the region of the spinal cord that is
involved. Disturbances in lower-extremity motor function and bladder sphincter function may be
associated with these lesions. In fact, nearly 90 percent of infants with spina bifida have some type
of foot deformity, such as clubfeet, vertical talus deformity, or calcaneo valgus. Secondary congenital
contractures, known as arthrogryposis, may occur in utero because of lack of fetal movement.

Spina bifida occulta. Spina bifida occulta refers to a vertebral defect without herniation of the spinal
canal contents and is also most commonly found in the lumbosacral region. The incidence in normal
adults has been reported to be five percent to 17.3 percent. Often, these patients are asymptomatic
at birth, although 80 percent are associated with dermal lesions and vertebral defects. Cutaneous
markers may consist of any combination of lesions, which include hypertrichosis, hemangiomas,
lipomas, dimples, hyperpigmentation, hypopigmentation, teratomas, or acrochordons.[20]
Occasionally, a sinus tract may lead into an intraspinal cyst or epidermoid structure and may
predispose infants with this condition to recurrent gram-negative bacterial meningitis.[21] Magnetic
resonance imaging appears to be the most sensitive screening tool for dysraphism.[20]
Spina bifida is associated with varying degrees of neurologic and musculoskeletal impairment and
ranges from complete paralysis and/or paresthesia to minimal or no impairment.[15] The anatomic
level of the lesion generally correlates with the motor and sensory deficit. Although the occult
dysraphic state may go undetected in early childhood, as the spinal cord ascends to its normal
position in life (L1-L2), patients may later present with foot deformities, gait disturbances, scoliosis,
and sphincter dysfunction. The failure of the spinal cord to ascend secondary either to primary
malformation or to secondary tumor is known as tethering of the cord. Foot deformities make the
foot more vulnerable to ulceration via bony deformities and altered foot biomechanics.[11]

Bony deformities alter the natural protective fat pads located on the plantar surface of the foot,
which normally protect the foot by distributing weight-bearing forces evenly over a large area.
Excessive focal pressure developed during the midstance and heel-rise parts of the gait cycle may
cause skin loss or hypertrophy of the stratum corneum (callus) over bony prominences. These
changes in the foot increase the possibility of ulceration by two orders of magnitude.[1] Shearing,
pressure, and frictional forces also all play a role in the breakdown of the cutaneous barrier, since
they are concentrated over the abnormal bony prominences. The risk of ulceration is proportional to
the number of risk factors and is increased by 1.7 in persons with peripheral neuropathy, by 12 in
patients with peripheral neuropathy and foot deformity, and by 36 in those who have peripheral
neuropathy, foot deformity, and previous amputation, as compared with those without risk factors.
[10]

The most common cause of ulceration of the foot is the loss of protective sensation due to
peripheral neuropathy. Loss of pain sensation results in increased trauma to the foot and thus an
increased risk to develop skin ulceration.[5] Cutaneous pressure perception is measured with the
Semmes-Weinstein monofilament. A 4.17 monofilament may be felt in a person with normal
sensation and is equated with 1g of linear pressure. A 5.07 monofilament is equated to 10g of linear
pressure, and persons who cannot feel this filament are considered to have lost protective
sensation.[10,12,13]

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An infant with spina bifida and hydrocephalus will usually have delay in development. This may be
present from birth. It is due to several reasons

* Long periods of time in hospital after birth

* Parent's concerns about having infant with spina bifida and knowing how to handle the child
 * The infant's heavy head due to hydrocephalus making it difficult to gain head control.

* The infant's difficulty in learning to move due to muscle paralysis, contractures and deformities.

Carrying out a programme of treatment can lessen the delay in the development. The programme
should be aimed at encouraging normal development from the first few months of life. It should also
aim at making the child as independent as possible in the community as the child grows and
develops.

AIDS used in rehabilitation

All children should be given opportunity to stand and walk. The child can be stood as soon as he has
sufficient head control and balance in his body. Standing encourages the muscles that hold the body
up to walk. It prevents contractures and strengthens leg bones. If the child does not have muscle
strength in the legs, aids such as gaiters, forward lean standers or an upright stander may be used.

Learning to walk should be fun and not a task. The child needs to gain confidence slowly. Special aids
such as splints or calipers may be necessary to support the legs during walking. As the balance and
confidence with walking improves; the child may be able to use crutches or walking sticks for
support. For some children the effort of walking is too great. This may be difficult for the family to
understand and accept. They may need to talk about it and not push the child to walk. These
children may use a wheelchair or trolley for all or part of the body.

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Definition of Spina Bifida

Spina Bifida is a developmental anomaly characterized by defective closure of the bony encasement
of the spinal cord through which the spinal cord and meninges may or may not protrude.

If the anomaly is not visible, the condition is called spina bifida occulta.
If there is an external protrusion of the saclike structure, the condition is called spina bifida cystica,
and is further classified according to extent of neural involvement (e.g., meningocele,
meningomyelocele, or myelomeningocele).

Description of Spina Bifida

Spina bifida is the most frequently occurring permanently disabling birth defect. It affects
approximately one out of every 1,000 newborns in the U.S. More children are affected by this
condition than muscular dystrophy, multiple sclerosis, and cystic fibrosis combined.

Spina bifida is a disabling birth defect of the spinal column resulting from the failure of the spinal
column to close properly during the first month of pregnancy. In severe cases, the spinal cord
protrudes through the back. The condition may cause varying degrees of paralysis and loss of feeling
in the lower limbs, and bowel and bladder complications.

A large percentage of children born with spina bifida have hydrocephalus, the accumulation of fluid
in the brain. Hydrocephalus is controlled by a surgical procedure called shunting which relieves the
fluid buildup in the brain by redirecting it to the abdominal area.

Spina bifida occulta is a usually harmless form of spina bifida in which there is a small defect or gap
in a few of the small bones (vertebrae) that make up the spine. The spinal cord and nerves are
usually normal, and most affected individuals have no problems caused by this minor abnormality.

Meningocele is the rarest form of spina bifida in which a cyst or lump consisting of membranes
surrounding the spinal cord pokes through the open part of the spine. The spinal cord and the
nerves are usually normal. The cyst, which can be as small as a nut or as large as a grapefruit, can be
removed by surgery, allowing the baby to develop normally.

Meningomyelocele is the most severe form of spina bifida. In this form, the cyst holds nerve roots of
the spinal cord and often the cord itself. Or there may be no cyst, but only a fully exposed section of
the spinal cord and nerves. Spinal fluid may leak out, and the area often is covered with sores.
Affected babies are at high risk of infection until the back is closed surgically. In spite of surgery,
varying degrees of leg paralysis and bladder and bowel control problems remain.

Causes and Risk Factors of Spina Bifida

The most important risk factor for spina bifida is poor nutrition, especially a diet deficient in folic
acid.
There appears to be factors other than nutrition in the development of spina bifida. Genetics also
seems to play a role. People of Northern European and Hungarian ancestry have the highest rates of
the disease, and the condition tends to run in families, though not consistently. In fact, 90 to 95
percent of children with spina bifida are born to women who have no other children or anyone in
the family with the defect.

Treatment of Spina Bifida

Meningocele, which does not involve the spinal cord, can usually be repaired surgically to preserve
normal spinal function.

When a baby is born with the severest form of spina bifida, it usually is operated on at once - within
48 hours after birth. Doctors surgically release the spinal cord, replace it in the spinal canal and cover
it with muscle and skin. Even so, limb paralysis and bowel problems usually remain. Specialists in
orthopedics and urology are asked to treat these problems.

As soon after the operation as possible, a physical therapist teaches the parents how to exercise
their baby's legs and feet to prepare for walking with leg braces and crutches. Some children need a
wheelchair.

If the child develops hydrocephalus, fluid can be drained from the brain through surgical placement
of a shunt. The shunt runs under the skin into the chest or abdomen, and the fluid passes harmlessly
through the child's body.

With treatment, children with spina bifida usually can become active individuals. Most are able to
have children, although such a pregnancy is considered "high risk."

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CONCERN

learning disabilities associated with hydrocephalus

Spina Bifida is a malformation of the spinal column in which one or more of the vertebrae fail to
close completely. This leaves a gap or split in the vertebrae causing damage to the spinal column
and spinal cord. Spina Bifida, a neural tube defect, affects approximately 1 in every 1000 births in
Western Australia.

There are three types of spina bifida: occulta, meningocele and myelomeningocele.
Myelomeningocele is the form of spina bifida that people identify as spina bifida as it is the most
disabling.

Approximately 80% of children born with spina bifida will also develop a condition known as
hydrocephalus. Hydrocephalus is a life long medical condition, not a disease. This condition does not
commonly cause impairment to intellect.

Hydrocephalus is an abnormal accumulation of fluid within the cavities of the brain. Hydrocephalus
is caused when there is an obstruction to the flow of CSF or a failure of the CSF to be absorbed into
the bloodstream. CSF is produced continuously and when there is an obstruction or failure to
absorb, the CSF accumulates in the ventricles causing them to enlarge and pressure in the head to
increase.

The impact spina bifida has on the individual differs from person to person. While similarities can be
seen in individuals with lesions in the same area of the spine, there are no two people affected in
exactly the same way. The reason for this is that some nerves end at the site of the lesion, some
continue through the lesion with little effect, and others that continue through the lesion are
damaged. Damage to the nerves of the spinal cord often affect the lower body of the person with
spina bifida therefore impacting on mobility, the ability to manage their bowel and bladder and skin
sensation. People with spina bifida may also have nonverbal learning difficulties and an allergy to
latex.

How does spina bifida affect the individual?

Mobility and skin sensation

Almost all people with spina bifida will have their mobility affected in some way. The site of the
lesion along the spinal cord will determine the extent of paralysis and sensory loss. Generally
speaking, their higher the site of the lesion, the greater the impact on the individuals mobility. For
example, children with a lesion in the thoracic or high lumbar region will have paralysis of the legs
and variable weakness and sensory loss in the abdomen and lower body. These children will need to
use wheelchairs and will only manage to walk with extensive orthoses (splints). If the lesion is at the
L3 level, the child will be able to flex the hips and extend the knees and ankles, but have weak or
absent ankle-toe flexion and hip extension. These children will be able to walk with simpler forms of
orthoses. Some may need to use crutches. Often a wheelchair will be required for long distances,
such as when on excursions. A lesion in the sacral area usually causes only mild weakness in the
ankles or toes. A child with this lesion will be able to walk quite well with minimal or no orthoses.

As well as having their mobility affected, almost all people with spina bifida have limited or no
sensation in some parts of their body. Nerve damage through the site of the lesion will mean that
people with spina bifida will have little or no feeling in some areas of their skin. This will affect their
ability to feel pain, touch, heat and cold. Again, how this affects one person is different from another
person. It is vital to understand how each individual is affected to ensure the protection of their skin
from damage from heat, cold, pressure, scrapes and cuts. Even crawling on nylon carpet can cause
severe friction burns. All people with spina bifida will have greater loss of sensation at the back of
the legs than the front. Most people will also have loss of sensation around the anus, genitalia and
feet. It is important to remember that the motor and sensory function is not always symmetrical;
one side may be better than the other.

Continence

Approximately 90% of children born with spina bifida have a urinary system and bowel that do not
function properly due to nerve damage to the pelvic floor area. The nerve damage associated with
spina bifida means that these children cannot feel the sensations associated with bowel and bladder
management. They are unable to feel when their bowel or bladder is full and are unable to evacuate
at will. In the majority of cases the kidney and bowel are normal and the damage is only to the parts
of the system that control elimination. Children are often bowel and bladder incontinent, but some
children may have problems with just one of these areas. If their bowel and bladder are not correctly
managed, it is possible that this will predispose them to kidney damage and overloading of the
colon.

Incontinence and the difficulties experienced in its management can lead to severe psychological
problems such as poor body image and low self-esteem. A sense of isolation and alienation can also
develop, resulting in behavioural problems and poor academic achievement. As children grow older,
peers and school staff become less tolerant of the incontinence and associated odour. It is important
to note that children are unlikely to notice when they have ‘had an accident’. Such children are
unable to smell the odour, even though it may be quite offensive to others. The best analogy to
explain this situation is the wearing of perfume or aftershave: having put it on, you are unaware of
the fragrance, although those around you are able to detect it. Proper continence management, a
healthy diet and an awareness of the need to check and change pads regularly is essential. There are
also some very good deodorants on the market which have been designed to assist in minimizing the
odour.

Learning Difficulties

Many children who have spina bifida and/or hydrocephalus have specific learning difficulties, even
though most children fall within the normal range of intelligence. It appears that these problems are
related to the hydrocephalus and Chiari malformation. It is not uncommon for these children to be
incorrectly labelled as lazy, careless or clumsy. It is therefore important to recognise that these
issues are indeed part of the disability and are neurologically based. For children with neurological
abnormalities, intelligence scores alone are not particularly informative.

Early identification of children having problems is important. Teachers should watch out for
restlessness, distractibility and forgetfulness. It is also important to note if students appear to miss
the point of questions and explanations, or have difficulty keeping up. Fortunately the learning
difficulties experienced by many children with spina bifida and hydrocephalus are not severe. If
detected early and provided with extra assistance, these need not have a dramatic impact on the
students learning.

Parents are concerned about:

• The acceptance of their child by peers and into the classroom

• The reaction of other parents to their child being in a mainstream classroom

• Is the teacher ‘accepting’ or ‘happy’ to have a student with a disability or will they resent
having a student with a disability?

• The educational development of their child; are they progressing and are any difficulties
addressed?

• Developmental progress – independence, communication

• Access to the school environment – transportation to and from school, access to facilities,
classrooms, toilets, are there ramps, are doors wide enough, is the school multi-level and is there an
elevator?

• Will their child’s safety be a priority – is there a risk of injury from falling, being pushed, will
there be staff available in case of a seizure (for students with epilepsy)?

• Having the opportunity to ask questions, share concerns and problem solve with the teacher

• Will their child’s place be ‘at risk’ of not maintaining their place in the school if they raise
concerns about some issues or make complaints?

• Not knowing how to prepare their child for school

• Not knowing how or when to enrol their child in school – what is the process, when do we
start, how much information are we required to give?

• Who do they speak to if they have concerns and will they be addressed?